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Blume H, Petre EN, Ziv E, Yuan G, Rodriguez L, Sotirchos V, Zhao K, Alexander ES. Safety and efficacy of transarterial therapies for pancreatic acinar cell carcinoma metastases. Clin Imaging 2025; 121:110463. [PMID: 40163953 DOI: 10.1016/j.clinimag.2025.110463] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/25/2025] [Revised: 03/08/2025] [Accepted: 03/25/2025] [Indexed: 04/02/2025]
Abstract
PURPOSE To evaluate the safety and efficacy of transarterial therapy, including hepatic arterial embolization (HAE) and transarterial radioembolization (TARE), for patients with hepatic metastases secondary to pancreatic acinar cell carcinoma (PACC). METHODS This retrospective, single-center study included patients with PACC liver metastases treated with transarterial therapy between 11/2013 and 2/2023. Nine patients with PACC were treated in a total of 18 sessions [HAE (n = 14), and TARE (n = 4)]. Patient demographics, tumor characteristics, and radiographic response were recorded. Local tumor progression-free survival (LTPFS) and overall survival (OS) were assessed via Kaplan-Meier analysis. Adverse events were graded according to Common Terminology Criteria for Adverse Events (CTCAE) v5. RESULTS Median LTPFS was 6.77 months (95 % CI: 3.23-26.33 months) after first treatment. Median assisted LTPFS in the six patients with multiple treatment sessions was 22.33 months (95 % CI: 3.67-31.93 months). Median OS was not reached (95 % CI: 0.17-NR). One-year OS from first treatment was 66.67 % (95 % CI: 28.17-87.83 %). Adverse events within one month of treatment occurred in 5/18 (27.8 %) sessions. Three of the five (60 %) reported complications were grade 1 and included mild post-embolization syndrome. One grade 3 complication occurred; pulmonary embolism associated with hypoxia and treated with anticoagulation. There was one death, grade 5, five days after treatment in a patient with a history of pancreaticoduodenectomy who developed a hepatic abscess complicated by sepsis. CONCLUSION This small retrospective study suggests that transarterial therapies for PACC provide acceptable local control and safety.
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Affiliation(s)
- Harrison Blume
- Memorial Sloan Kettering Cancer Center, Department of Radiology, 1275 York Avenue, New York, NY 10065, United States of America; Albert Einstein College of Medicine, 1300 Morris Park Avenue, Bronx, NY 10461, United States of America.
| | - Elena N Petre
- Memorial Sloan Kettering Cancer Center, Department of Radiology, 1275 York Avenue, New York, NY 10065, United States of America.
| | - Etay Ziv
- Memorial Sloan Kettering Cancer Center, Department of Radiology, 1275 York Avenue, New York, NY 10065, United States of America.
| | - Gavin Yuan
- Memorial Sloan Kettering Cancer Center, Department of Radiology, 1275 York Avenue, New York, NY 10065, United States of America; New York Medical College, 40 Sunshine Cottage Road, Valhalla, NY 10595, United States of America.
| | - Lee Rodriguez
- Memorial Sloan Kettering Cancer Center, Department of Radiology, 1275 York Avenue, New York, NY 10065, United States of America.
| | - Vlasios Sotirchos
- Memorial Sloan Kettering Cancer Center, Department of Radiology, 1275 York Avenue, New York, NY 10065, United States of America.
| | - Ken Zhao
- Memorial Sloan Kettering Cancer Center, Department of Radiology, 1275 York Avenue, New York, NY 10065, United States of America.
| | - Erica S Alexander
- Memorial Sloan Kettering Cancer Center, Department of Radiology, 1275 York Avenue, New York, NY 10065, United States of America.
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Shyr B, Chen T, Wang S, Chen S, Shyr Y, Shyr B. A Comparative Study of Pancreatic Acinar Cell Carcinoma: A Case-Control Study. Health Sci Rep 2025; 8:e70633. [PMID: 40226178 PMCID: PMC11985884 DOI: 10.1002/hsr2.70633] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Grants] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/12/2024] [Revised: 02/07/2025] [Accepted: 03/20/2025] [Indexed: 04/15/2025] Open
Abstract
Background and Aims Pancreatic acinar cell carcinoma (PACC) is rare. This study aims to elucidate its clinical features and survival outcomes. Methods Patients diagnosed with PACC were enrolled. A comparison between PACC and pancreatic ductal adenocarcinoma (PDAC) patients was conducted following propensity score matching (PSM). Results There were 11 resectable and nine unresectable PACC. The majority (60%) of PACC cases were located in the pancreatic head, with a median tumor size of 5.9 cm. Elevated serum lipase level was observed in 64.3% of cases. Regional lymph node involvement was found in 65.0%. The median survival was 20.0 months for resectable PACC patients compared to 6.7 months for unresectable cases. The 1-, 3-, and 5-year survival rates were 100%, 49.1%, and 32.0%, respectively, for resectable PACC patients, while for unresectable cases, they were 33.3%, 0%, and 0%. Resectable PACC patients exhibited lower rates of lymph node involvement (36.4% vs. 66.1%), lymphovascular invasion (LVI), 36.4% versus 72.8%, and perineural invasion (PNI), 45.5% versus 86.0%, compared to PDAC. Following PSM, there was no significant difference in survival between resectable PACC and PDAC. Conclusion PACC is associated with lower rates of lymph node involvement, LVI, and PNI, which might attribute to superior outcome when compared with PDAC reported in the literature. However, there is no survival difference between resecrable PACC and PDAC after PSM.
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Affiliation(s)
- Bor‐Shiuan Shyr
- Division of General Surgery, Department of Surgery and Therapeutic and Research Center of Pancreatic CancerTaipei Veterans General HospitalTaipeiTaiwan (ROC)
- National Yang Ming Chiao Tung UniversityTaipeiTaiwan (ROC)
| | - Ting‐Chung Chen
- Division of General Surgery, Department of Surgery and Therapeutic and Research Center of Pancreatic CancerTaipei Veterans General HospitalTaipeiTaiwan (ROC)
- National Yang Ming Chiao Tung UniversityTaipeiTaiwan (ROC)
| | - Shin‐E. Wang
- Division of General Surgery, Department of Surgery and Therapeutic and Research Center of Pancreatic CancerTaipei Veterans General HospitalTaipeiTaiwan (ROC)
- National Yang Ming Chiao Tung UniversityTaipeiTaiwan (ROC)
| | - Shih‐Chin Chen
- Division of General Surgery, Department of Surgery and Therapeutic and Research Center of Pancreatic CancerTaipei Veterans General HospitalTaipeiTaiwan (ROC)
- National Yang Ming Chiao Tung UniversityTaipeiTaiwan (ROC)
| | - Yi‐Ming Shyr
- Division of General Surgery, Department of Surgery and Therapeutic and Research Center of Pancreatic CancerTaipei Veterans General HospitalTaipeiTaiwan (ROC)
- National Yang Ming Chiao Tung UniversityTaipeiTaiwan (ROC)
| | - Bor‐Uei Shyr
- Division of General Surgery, Department of Surgery and Therapeutic and Research Center of Pancreatic CancerTaipei Veterans General HospitalTaipeiTaiwan (ROC)
- National Yang Ming Chiao Tung UniversityTaipeiTaiwan (ROC)
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Teramatsu K, Fujimori N, Murakami M, Yasumori S, Matsumoto K, Nakata K, Nakamura M, Koga Y, Oda Y, Ogawa Y. Pathological complete response with FOLFIRINOX therapy for recurrence of pancreatic acinar cell carcinoma. Clin J Gastroenterol 2024; 17:776-781. [PMID: 38761340 DOI: 10.1007/s12328-024-01983-2] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/05/2024] [Accepted: 05/06/2024] [Indexed: 05/20/2024]
Abstract
Pancreatic acinar cell carcinoma (PACC) is a very rare subtype of pancreatic cancer. Due to small number of patients, no standard chemotherapy protocol has been established. We experienced an extremely rare case of PACC with liver metastasis that showed a pathological complete response after modified FOLFIRINOX (mFFX) therapy. A 42-year-old man who underwent distal pancreatectomy for an 80 mm tumor at the pancreatic tail 3 years ago was referred to our hospital in September 2017 for the treatment of a recurrent liver tumor. Percutaneous biopsy revealed an acinar-neuroendocrine carcinoma, similar to the surgical specimen. He received eight cycles of irinotecan plus cisplatin chemotherapy. However, the tumor increased in size, and treatment was switched to mFFX therapy. The tumor in the liver shrank remarkably after nine cycles of mFFX therapy. Conversion surgery was selected, and the patient underwent hepatic left and caudate lobectomy 8 months after administration of mFFX. The resected specimen showed no viable tumor cells, indicating a pathological complete response. The histological diagnosis was reconsidered, and PACC was finally diagnosed via an additional immunohistological review. The patient has remained well with no recurrence for 6 years after surgery. This study is the first to report a case of pathological complete response with mFFX therapy for the recurrence of PACC.
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Affiliation(s)
- Katsuhito Teramatsu
- Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1, Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan
| | - Nao Fujimori
- Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1, Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan.
| | - Masatoshi Murakami
- Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1, Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan
| | - Sho Yasumori
- Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1, Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan
| | - Kazuhide Matsumoto
- Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1, Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan
| | - Kohei Nakata
- Department of Surgery and Oncology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
| | - Masafumi Nakamura
- Department of Surgery and Oncology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
| | - Yutaka Koga
- Department of Pathology, National Hospital Organization Kyushu Cancer Center, Fukuoka, Japan
| | - Yoshinao Oda
- Department of Anatomic Pathology, Graduate School of Medical Sciences, Kyushu University Hospital, Fukuoka, Japan
| | - Yoshihiro Ogawa
- Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, 3-1-1, Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan
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de Jesus VHF, Donadio MDS, de Brito ÂBC, Gentilli AC. A narrative review on rare types of pancreatic cancer: should they be treated as pancreatic ductal adenocarcinomas? Ther Adv Med Oncol 2024; 16:17588359241265213. [PMID: 39072242 PMCID: PMC11282540 DOI: 10.1177/17588359241265213] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/17/2024] [Accepted: 06/13/2024] [Indexed: 07/30/2024] Open
Abstract
Pancreatic cancer is one of the deadliest malignancies in humans and it is expected to play a bigger part in cancer burden in the years to come. Pancreatic ductal adenocarcinoma (PDAC) represents 85% of all primary pancreatic malignancies. Recently, much attention has been given to PDAC, with significant advances in the understanding of the mechanisms underpinning disease initiation and progression, along with noticeable improvements in overall survival in both localized and metastatic settings. However, given their rarity, rare histological subtypes of pancreatic cancer have been underappreciated and are frequently treated as PDAC, even though they might present non-overlapping molecular alterations and clinical behavior. While some of these rare histological subtypes are true variants of PDAC that should be treated likewise, others represent separate clinicopathological entities, warranting a different therapeutic approach. In this review, we highlight clinical, pathological, and molecular aspects of rare histological types of pancreatic cancer, along with the currently available data to guide treatment decisions.
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Affiliation(s)
- Victor Hugo Fonseca de Jesus
- Oncoclínicas, Department of Gastrointestinal Medical Oncology, Santos Dumont St. 182, 4 floor, Florianópolis, Santa Catarina 88015-020, Brazil
- Department of Medical Oncology, Centro de Pesquisas Oncológicas, Florianópolis, Santa Catarina, Brazil
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Patel RK, Parappilly M, Sutton TL, Behrens S, Schwantes IR, Johnson AJ, Pommier RF, Sheppard BC. Referral and treatment patterns in pancreatic acinar cell carcinoma: A regional population-level analysis. Am J Surg 2024; 231:55-59. [PMID: 37087362 DOI: 10.1016/j.amjsurg.2023.04.010] [Citation(s) in RCA: 1] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/15/2022] [Revised: 03/08/2023] [Accepted: 04/16/2023] [Indexed: 04/24/2023]
Abstract
BACKGROUND Pancreatic acinar cell carcinoma (PACC) is a rare exocrine tumor of the pancreas. We evaluated the effect disease stage, surgical intervention, and institutional volume status plays in survival. METHODS We queried the Oregon State Cancer Registry for patients with PACC from 1997 to 2018. Treatment and referral patterns were analyzed, and overall survival (OS) was evaluated with Kaplan-Meier and Cox-proportional hazard analysis. RESULTS 43 patients were identified. Median OS was 33.1 and 7.1 months in those with locoregional and metastatic disease respectively (p = 0.008). Surgical intervention was associated with improved OS (hazard ratio 0.28, p < 0.0001). High volume center (HVC) care trended towards improving OS. While the majority of cases were diagnosed at low volume centers (74%), referral to HVCs was rare (n = 4) and limited to advanced (stage III/IV) disease. CONCLUSION Stage and surgical resection influence survival outcomes in PACC, more data is needed to delineate the impact of institutional volume status.
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Affiliation(s)
- Ranish K Patel
- Oregon Heath & Science University (OHSU), Department of Surgery, Division of General Surgery, Portland, OR, 97239, USA
| | - Michael Parappilly
- Department of Cell, Developmental and Cancer Biology, Oregon Health & Science University, 2720 S. Moody Ave., Mailcode KC-CDCB, Portland, OR, 97201, USA
| | - Thomas L Sutton
- Oregon Heath & Science University (OHSU), Department of Surgery, Division of General Surgery, Portland, OR, 97239, USA
| | - Shay Behrens
- Oregon Heath & Science University (OHSU), Department of Surgery, Division of General Surgery, Portland, OR, 97239, USA
| | - Issac R Schwantes
- Oregon Heath & Science University (OHSU), Department of Surgery, Division of General Surgery, Portland, OR, 97239, USA
| | - Alicia J Johnson
- Oregon Heath & Science University (OHSU), Department of Surgery, Division of General Surgery, Portland, OR, 97239, USA
| | - Rodney F Pommier
- OHSU Department of Surgery, Division of Surgical Oncology, Knight Cancer Institute, Portland, OR, 97239, USA
| | - Brett C Sheppard
- Oregon Heath & Science University (OHSU), Department of Surgery, Division of General Surgery, Portland, OR, 97239, USA.
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Ikezawa K, Urabe M, Kai Y, Takada R, Akita H, Nagata S, Ohkawa K. Comprehensive review of pancreatic acinar cell carcinoma: epidemiology, diagnosis, molecular features and treatment. Jpn J Clin Oncol 2024; 54:271-281. [PMID: 38109477 PMCID: PMC10925851 DOI: 10.1093/jjco/hyad176] [Citation(s) in RCA: 7] [Impact Index Per Article: 7.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/26/2023] [Accepted: 11/29/2023] [Indexed: 12/20/2023] Open
Abstract
Pancreatic acinar cell carcinoma is a rare form (0.2-4.3%) of pancreatic neoplasm with unique clinical and molecular characteristics, which largely differ from pancreatic ductal adenocarcinoma. Pancreatic acinar cell carcinoma occurs more frequently in males and can occur in children. Serum lipase is elevated in 24-58% of patients with pancreatic acinar cell carcinoma. Pancreatic acinar cell carcinomas tend to be large at diagnosis (median tumour size: ~5 cm) and are frequently located in the pancreas head. Radiologically, pancreatic acinar cell carcinoma generally exhibits a solid appearance; however, necrosis, cystic changes and intratumoral haemorrhage can occur in larger lesions. Immunostaining is essential for the definitive diagnosis of pancreatic acinar cell carcinoma. Compared with pancreatic ductal adenocarcinoma, pancreatic acinar cell carcinoma has a more favourable prognosis. Although radical surgery is recommended for patients with pancreatic acinar cell carcinoma who do not have distant metastases, the recurrence rate is high. The effectiveness of adjuvant therapy for pancreatic acinar cell carcinoma is unclear. The response to FOLFIRINOX is generally favourable, and some patients achieve a complete response. Pancreatic acinar cell carcinoma has a different genomic profile compared with pancreatic ductal adenocarcinoma. Although genomic analyses have shown that pancreatic acinar cell carcinoma rarely has KRAS, TP53 and CDKN2A mutations, it has a higher prevalence of homologous recombination-related genes, including BRCA1/2 and ATM, than pancreatic ductal adenocarcinoma, suggesting high sensitivity to platinum-containing regimens and PARP inhibitors. Targeted therapies for genomic alternations are beneficial. Therefore, genetic testing is important for patients with pancreatic acinar cell carcinoma to choose the optimal therapeutic strategy.
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Affiliation(s)
- Kenji Ikezawa
- Department of Hepatobiliary and Pancreatic Oncology, Osaka International Cancer Institute, Osaka, Japan
| | - Makiko Urabe
- Department of Hepatobiliary and Pancreatic Oncology, Osaka International Cancer Institute, Osaka, Japan
| | - Yugo Kai
- Department of Hepatobiliary and Pancreatic Oncology, Osaka International Cancer Institute, Osaka, Japan
| | - Ryoji Takada
- Department of Hepatobiliary and Pancreatic Oncology, Osaka International Cancer Institute, Osaka, Japan
| | - Hirofumi Akita
- Department of Gastroenterological Surgery, Osaka International Cancer Institute, Osaka, Japan
| | - Shigenori Nagata
- Department of Diagnostic Pathology and Cytology, Osaka International Cancer Institute, Osaka, Japan
| | - Kazuyoshi Ohkawa
- Department of Hepatobiliary and Pancreatic Oncology, Osaka International Cancer Institute, Osaka, Japan
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Bellotti R, Paiella S, Primavesi F, Jäger C, Demir IE, Casciani F, Kornprat P, Wagner D, Rösch CS, Butturini G, Giardino A, Goretzky PE, Mogl M, Fahlbusch T, Kaiser J, Strobel O, Nießen A, Luu AM, Salvia R, Maglione M. Treatment characteristics and outcomes of pure Acinar cell carcinoma of the pancreas - A multicentric European study on radically resected patients. HPB (Oxford) 2023; 25:1411-1419. [PMID: 37563033 DOI: 10.1016/j.hpb.2023.07.897] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/04/2023] [Revised: 07/04/2023] [Accepted: 07/20/2023] [Indexed: 08/12/2023]
Abstract
BACKGROUND Acinar cell carcinomas (ACC) belong to the exocrine pancreatic malignancies. Due to their rarity, there is no consensus regarding treatment strategies for resectable ACC. METHODS This is a retrospective multicentric study of radically resected pure pancreatic ACC. Primary endpoints were overall survival (OS) and disease-free survival (DFS). Further endpoints were oncologic outcomes related to tumor stage and therapeutic protocols. RESULTS 59 patients (44 men) with a median age of 64 years were included. The median tumor size was 45.0 mm. 61.0% were pT3 (n = 36), nodal positivity rate was 37.3% (n = 22), and synchronous distant metastases were present in 10.1% of the patients (n = 6). 5-Years OS was 60.9% and median DFS 30 months. 24 out of 31 recurred systemically (n = 18 only systemic, n = 6 local and systemic). Regarding TNM-staging, only the N2-stage negatively influenced OS and DFS (p = 0.004, p = 0.001). Adjuvant treatment protocols (performed in 62.7%) did neither improve OS (p = 0.542) nor DFS (p = 0.159). In 9 cases, radical resection was achieved following neoadjuvant therapy. DISCUSSION Radical surgery is currently the mainstay for resectable ACC, even for limited metastatic disease. Novel (neo)adjuvant treatment strategies are needed, since current systemic therapies do not result in a clear survival benefit in the perioperative setting.
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Affiliation(s)
- Ruben Bellotti
- Department of Visceral, Transplant and Thoracic Surgery, Center of Operative Medicine, Medical University of Innsbruck, 6020 Innsbruck, Austria
| | - Salvatore Paiella
- General and Pancreatic Surgery Unit, Pancreas Institute, University of Verona, Verona, Italy
| | - Florian Primavesi
- Department of Visceral, Transplant and Thoracic Surgery, Center of Operative Medicine, Medical University of Innsbruck, 6020 Innsbruck, Austria; Department of General, Visceral and Vascular Surgery, Salzkammergut Hospital, 4840 Vöcklabruck, Austria
| | - Carsten Jäger
- Department of Surgery, Klinikum Rechts der Isar, School of Medicine, Technical University of Munich, Munich, Germany
| | - Ihsan E Demir
- Department of Surgery, Klinikum Rechts der Isar, School of Medicine, Technical University of Munich, Munich, Germany
| | - Fabio Casciani
- General and Pancreatic Surgery Unit, Pancreas Institute, University of Verona, Verona, Italy
| | - Peter Kornprat
- Department of General Surgery, Medical University of Graz, Graz, Austria
| | - Doris Wagner
- Department of General Surgery, Medical University of Graz, Graz, Austria
| | | | | | | | - Peter E Goretzky
- Department of Surgery, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Berlin 13353, Germany
| | - Martina Mogl
- Department of Surgery, Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Berlin 13353, Germany
| | - Tim Fahlbusch
- St. Josef Hospital, Department of General and Visceral Surgery, Ruhr-University Bochum, Germany
| | - Jörg Kaiser
- Department of General, Visceral and Transplantation Surgery, Heidelberg University Hospital, Heidelberg, Germany
| | - Oliver Strobel
- Department of General, Visceral and Transplantation Surgery, Heidelberg University Hospital, Heidelberg, Germany; Division of Visceral Surgery, Department of General Surgery, Medical University of Vienna, Vienna, Austria
| | - Anna Nießen
- Department of General, Visceral and Transplantation Surgery, Heidelberg University Hospital, Heidelberg, Germany
| | - Andreas M Luu
- St. Josef Hospital, Department of General and Visceral Surgery, Ruhr-University Bochum, Germany; Klinikum für Allgemein, Viszeral- und Minimalinvasive Chirurgie, HELIOS Klinikum, Krefeld, Germany
| | - Roberto Salvia
- General and Pancreatic Surgery Unit, Pancreas Institute, University of Verona, Verona, Italy
| | - Manuel Maglione
- Department of Visceral, Transplant and Thoracic Surgery, Center of Operative Medicine, Medical University of Innsbruck, 6020 Innsbruck, Austria.
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Kniepeiss D, Talakić E, Portugaller RH, Fuchsjäger M, Schemmer P. Non-colorectal liver metastases: A review of interventional and surgical treatment modalities. Front Surg 2022; 9:945755. [PMID: 36406370 PMCID: PMC9666734 DOI: 10.3389/fsurg.2022.945755] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/16/2022] [Accepted: 10/13/2022] [Indexed: 12/31/2023] Open
Abstract
Liver metastases (LM) occur in up to 90% either simultaneously with the diagnosis of the primary tumor or at a later time-point. While resection of colorectal LM and resection or transplantation of neuroendocrine LM is part of a standard therapy with a 5-year patient survival of up to 80%, resection of non-colorectal and non-neuroendocrine LM is still discussed controversially. The reason for it is the significantly lower survival benefit of all different tumor entities depending on the biological aggressiveness of the tumor. Randomized controlled trials are lacking. However, reviews of case series with ≥100 liver resections are available. They show a 5-year patient survival of up to 42% compared to only <5% in patients without treatment. Risk factors for poor survival include the type of primary tumor, a short interval between resection of the primary tumor and liver resection, extrahepatic manifestation of the tumor, number and size of the LM, and extent of liver resection. Overall, it has recently been shown that a good patient selection, the technical advances in surgical therapy and the use of a risk score to predict the prognosis lead to a significantly better outcome so that it is no longer justified not to offer liver resection to patients with non-colorectal, non- endocrine LM. Since modern therapy of LM is multimodal, the optimal therapeutic approach is decided individually by a multidisciplinary team consisting of visceral surgeons, oncologists, interventional radiologists and radiologists as part of a tumor board.
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Affiliation(s)
- Daniela Kniepeiss
- General, Visceral and Transplant Surgery, Medical University of Graz, Graz, Austria
- University Transplant Center Graz, Medical University of Graz, Graz, Austria
| | - Emina Talakić
- University Transplant Center Graz, Medical University of Graz, Graz, Austria
- Department of Radiology, Division of General Radiology, Medical University of Graz, Graz, Austria
| | - Rupert Horst Portugaller
- University Transplant Center Graz, Medical University of Graz, Graz, Austria
- Division of Neuroradiology, Vascular and Interventional Radiology, Department of Radiology, Medical University of Graz, Graz, Austria
| | - Michael Fuchsjäger
- University Transplant Center Graz, Medical University of Graz, Graz, Austria
- Department of Radiology, Division of General Radiology, Medical University of Graz, Graz, Austria
| | - Peter Schemmer
- General, Visceral and Transplant Surgery, Medical University of Graz, Graz, Austria
- University Transplant Center Graz, Medical University of Graz, Graz, Austria
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Calimano-Ramirez LF, Daoud T, Gopireddy DR, Morani AC, Waters R, Gumus K, Klekers AR, Bhosale PR, Virarkar MK. Pancreatic acinar cell carcinoma: A comprehensive review. World J Gastroenterol 2022; 28:5827-5844. [PMID: 36353206 PMCID: PMC9639656 DOI: 10.3748/wjg.v28.i40.5827] [Citation(s) in RCA: 29] [Impact Index Per Article: 9.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/25/2022] [Revised: 09/14/2022] [Accepted: 10/14/2022] [Indexed: 02/06/2023] Open
Abstract
Acinar cell carcinoma (ACC) is a rare pancreatic malignancy with distinctive clinical, molecular, and morphological features. The long-term survival of ACC patients is substantially superior to that of pancreatic adenocarcinoma patients. As there are no significant patient series about ACCs, our understanding of this illness is mainly based on case reports and limited patient series. Surgical resection is the treatment of choice for patients with the disease restricted to one organ; however, with recent breakthroughs in precision medicine, medicines targeting the one-of-a-kind molecular profile of ACC are on the horizon. There are no standard treatment protocols available for people in which a total surgical resection to cure the condition is not possible. As a result of shared genetic alterations, ACCs are chemosensitive to agents with activity against pancreatic adenocarcinomas and colorectal carcinomas. The role of neoadjuvant or adjuvant chemoradiotherapy has not been established. This article aims to do a comprehensive literature study and present the most recent information on acinar cell cancer.
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Affiliation(s)
| | - Taher Daoud
- Department of Diagnostic Radiology, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, United States
| | - Dheeraj Reddy Gopireddy
- Department of Diagnostic Radiology, University of Florida College of Medicine Jacksonville, Jacksonville, FL 32209, United States
| | - Ajaykumar C Morani
- Department of Diagnostic Radiology, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, United States
| | - Rebecca Waters
- Department of Pathology and Lab Medicine, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, United States
| | - Kazim Gumus
- Department of Research and Diagnostic Radiology, University of Florida College of Medicine Jacksonville, Jacksonville, FL 32209, United States
| | - Albert Russell Klekers
- Department of Diagnostic Radiology, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, United States
| | - Priya R Bhosale
- Department of Diagnostic Radiology, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, United States
| | - Mayur K Virarkar
- Department of Diagnostic Radiology, University of Florida College of Medicine Jacksonville, Jacksonville, FL 32209, United States
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Survival Outcome and Prognostic Factors for Pancreatic Acinar Cell Carcinoma: Retrospective Analysis from the German Cancer Registry Group. Cancers (Basel) 2021; 13:cancers13236121. [PMID: 34885230 PMCID: PMC8656891 DOI: 10.3390/cancers13236121] [Citation(s) in RCA: 8] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/30/2021] [Revised: 11/29/2021] [Accepted: 12/01/2021] [Indexed: 12/18/2022] Open
Abstract
Simple Summary Less than 1% of all pancreatic malignancies are acinar cell carcinomas. Based on data from the German Cancer Registry Group, we performed a comparative analysis of characteristics and prognostic factors of pancreatic acinar cell carcinoma and the most common type of pancreatic cancer—pancreatic ductal adenocarcinoma. Compared to pancreatic ductal adenocarcinoma, patients with pancreatic acinar cell carcinoma were younger at the time of diagnosis and the percentage of males was higher. The prognosis of patients with pancreatic acinar carcinoma was better than that of patients with pancreatic ductal adenocarcinoma. Surgical resection was the strongest positive prognostic factor for pancreatic acinar cell carcinoma. The study shows that pancreatic acinar cell carcinoma has features distinct from pancreatic ductal adenocarcinoma. Radical resection should be advocated, whenever feasible. Abstract Background: Pancreatic acinar cell carcinoma (PACC) is a distinct type of pancreatic cancer with low prevalence. We aimed to analyze prognostic factors and survival outcome for PACC in comparison to pancreatic ductal adenocarcinoma (PDAC), based on data from the German Cancer Registry Group. Methods: Patients with PACC and PDAC were extracted from pooled data of the German clinical cancer registries (years 2000 to 2019). The distribution of demographic parameters, tumor stage and therapy modes were compared between PACC and PDAC. The Kaplan–Meier method and Cox regression analysis were used to delineate prognostic factors for PACC. Propensity score matching was used to compare survival between PACC and PDAC. Results: There were 233 (0.44%) patients with PACC out of 52,518 patients with pancreatic malignancy. Compared to PDAC, patients with PACC were younger (median age 66 versus 70, respectively, p < 0.001) and the percentage of males was higher (66.1% versus 53.3%, respectively, p < 0.001). More patients were resected with PACC than with PDAC (56.2% versus 38.9%, respectively, p < 0.001). The estimated overall median survival in PACC was 22 months (95% confidence interval 15 to 27), compared to 12 months (95% confidence interval 10 to 13) in the matched PDAC cohort (p < 0.001). Surgical resection was the strongest positive prognostic factor for PACC after adjusting for sex, age, and distant metastases (hazard ratio 0.34, 95% confidence interval 0.22 to 0.51, p < 0.001). There was no survival benefit for adjuvant therapy in PACC. Conclusions: PACC has overall better prognosis than PDAC. Surgical resection is the best therapeutic strategy for PACC and should be advocated even in advanced tumor stages.
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11
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Dreikhausen L, Schulte N, Belle S, Weidner P, Moersdorf J, Reissfelder C, Ebert MP, Zhan T. Pancreatic Acinar Cell Carcinoma with Germline BRCA2 Mutation and Severe Pancreatic Panniculitis: A Case Report. Visc Med 2021; 37:447-450. [PMID: 34722729 DOI: 10.1159/000515267] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/01/2020] [Accepted: 02/15/2021] [Indexed: 11/19/2022] Open
Abstract
Pancreatic acinar cell carcinoma (ACC) is a rare malignant disease that displays distinct differences to pancreatic ductal adenocarcinoma. Here, we report the case of a patient with ACC and underlying breast cancer susceptibility gene 2 (BRCA2) germline mutation that developed severe pancreatic panniculitis (PP) during the course of the disease. The patient received a multimodal therapy including surgery, systemic chemotherapy, and targeted therapy with the PARP inhibitor olaparib, resulting in an overall survival of 47 months. Findings from this case are compared to the current knowledge on management of ACC and paraneoplastic PP.
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Affiliation(s)
- Lena Dreikhausen
- Department of Medicine II, University Medical Center Mannheim, Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany
| | - Nadine Schulte
- Department of Medicine II, University Medical Center Mannheim, Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany
| | - Sebastian Belle
- Department of Medicine II, University Medical Center Mannheim, Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany
| | - Philip Weidner
- Department of Medicine II, University Medical Center Mannheim, Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany
| | - Johannes Moersdorf
- Department of Dermatology, University Medical Center Mannheim, Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany
| | - Christoph Reissfelder
- Department of Surgery, University Medical Center Mannheim, Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany
| | - Matthias P Ebert
- Department of Medicine II, University Medical Center Mannheim, Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany
| | - Tianzuo Zhan
- Department of Medicine II, University Medical Center Mannheim, Medical Faculty Mannheim, Heidelberg University, Mannheim, Germany
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Kniepeiss D, Talakić E, Schemmer P. Interventionelle und chirurgische Therapie non-kolorektaler Lebermetastasen. TUMORDIAGNOSTIK & THERAPIE 2021; 42:585-597. [DOI: 10.1055/a-1557-7043] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/12/2025]
Abstract
ZusammenfassungIn der Vergangenheit wurden Resektionen oder Transplantationen wegen non-kolorektaler Lebermetastasen durch die Abwägung von geringem Überlebensvorteil und Komplikationsrate eher zurückhaltend indiziert. Mittlerweile zählt die Leberchirurgie jedoch zu den komplikationsarmen Standardverfahren in der Tumortherapie, die unter Einbettung in multimodale Therapiekonzepte zu einer deutlichen Steigerung des Patientenüberlebens führen.
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Affiliation(s)
- Daniela Kniepeiss
- Klinische Abteilung für Transplantationschirurgie an der Universitätsklinik für Chirurgie; Universitätsklinikum Graz, Österreich
| | - Emina Talakić
- Klinische Abteilung für Allgemeine Radiologie, Universitätsklinik für Radiologie, Medizinische Universität Graz, Österreich
| | - Peter Schemmer
- Fachbereich für Allgemein-, Viszeral- und Transplantationschirurgie Graz an der Universitätsklinik für Chirurgie; Medizinische Universität Graz, Österreich
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13
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Balouchi-Anaraki S, Ahmadvand S, Safaei A, Ghaderi A. 4H12, a Murine Monoclonal Antibody Directed against Myosin Heavy Chain-9 Expressed on Acinar Cell Carcinoma of Pancreas with Potential Therapeutic Application. IRANIAN BIOMEDICAL JOURNAL 2021; 25:310-322. [PMID: 34425650 PMCID: PMC8487684 DOI: 10.52547/ibj.25.5.310] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 06/21/2021] [Accepted: 08/03/2021] [Indexed: 11/18/2022]
Abstract
BACKGROUND Pancreatic acinar cell carcinoma (PACC) is a rare type of pancreatic exocrine neoplasm that is frequently diagnosed at late stages with a high rate of metastasis. Identification of new biomarkers for PACC can improve our knowledge of its biology, early detection, or targeted therapy. In this study, hybridoma technology was used to generate mAbs against Faraz-ICR, a pancreatic acinar cell carcinoma cell line. METHODS Cell ELISA and flow cytometry were used for screening, and the 4H12 hybridoma clone was selected for further analysis. The 4H12 mAb was specific for myosin heavy chain-9 (MYH9) as determined by Immunoprecipitation, Western blot, and mass spectrometry. RESULTS This antibody reacted variably with other cancer cells, in comparison to Faraz-ICR cell. Besides, by immunohistochemical staining, the acinar cell tumor, which was the source of Faraz-ICR, showed high MYH9 expression. Among 21 pancreatic ductal adenocarcinoma cases, nine (42.8%) expressed MYH9 with low intensity, while 10 (47.8%) and 2 (9.5%) cases expressed MYH9 with moderate to strong intensities, respectively. The 4H12 mAb inhibited the proliferation of Faraz-ICR cells in a dose-dependent manner from 0.75 to 12.5 μg/ml concentrations (p < 0.0001 and p < 0.002). IC50 values were achieved at 12.09 ± 4.19 µg/ml and 7.74 ± 4.28 µg/ml after 24- and 48-h treatment, respectively. CONCLUSION Our data suggest that the 4H12 mAb can serve as a tool for investigating the role of MYH9 pancreatic cancer biology and prognosis.
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Affiliation(s)
- Sima Balouchi-Anaraki
- Department of Immunology, School of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran
- Shiraz Institute for Cancer Research, School of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran
| | - Simin Ahmadvand
- Shiraz Institute for Cancer Research, School of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran
| | - Akbar Safaei
- Department of Pathology, School of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran
| | - Abbas Ghaderi
- Department of Immunology, School of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran
- Shiraz Institute for Cancer Research, School of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran
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14
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Zhou W, Han X, Fang Y, Han S, Cai Y, Kuang T, Lou W, Wang D. Clinical Analysis of Acinar Cell Carcinoma of the Pancreas: A Single-Center Experience of 45 Consecutive Cases. Cancer Control 2021; 27:1073274820969447. [PMID: 33121259 PMCID: PMC7791459 DOI: 10.1177/1073274820969447] [Citation(s) in RCA: 8] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022] Open
Abstract
Backgrounds: Acinar cell carcinoma of the pancreas is a rare malignancy, and its features
remain unclear. We aimed to analyze the clinical characteristics, treatment
and prognosis of acinar cell carcinoma with our institutional case
series. Methods: Patients diagnosed with acinar cell carcinoma in our hospital between 2005
and 2019 were reviewed. Investigations on clinicopathological features,
treatment details and long-term survival were performed. Results: A total of 45 pathologically confirmed acinar cell carcinomas were
identified. The median age at diagnosis was 58 years with a male-to-female
ratio of 3.1:1. There were 24 (53.3%) localized, 5 (11.1%) locally advanced
and 16 (35.6%) metastatic cases, with a pancreatic head-to-body/tail ratio
of 1:1.4 for all the primary lesions. In the localized group, there were 10
pancreatoduodenectomy, 12 distal pancreatectomy, 1 total pancreatectomy, and
1 distal pancreatectomy combined with proximal gastrectomy. Among the
locally advanced and metastatic cases, 13 patients received chemotherapy, 1
received concurrent radiochemotherapy, 1 underwent synchronous resection of
primary tumor and liver metastasis, 1 underwent palliative operation, 1
underwent exploratory laparotomy, and 4 required no treatment. The median
overall survival of this series was 18.9 months with a 5-year survival rate
of 19.6%. Moreover, the resected acinar cell carcinoma patients were
associated with prolonged survival compared with the unresected cases (36.6
vs. 8.5 months, P < 0.001). Conclusions: Surgical resection could improve the long-term survival of acinar cell
carcinoma patients, which might also improve the prognosis of selected
metastatic cases. Large-scale studies are needed to further clarify the
biological behavior and clinical features, and to seek the optimal
treatments.
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Affiliation(s)
- Wentao Zhou
- The Research Institution of General Surgery, Zhongshan Hospital, Fudan University, Shanghai, China.,Department of Pancreatic Surgery, Zhongshan Hospital, Fudan University, Shanghai, China
| | - Xu Han
- Department of Pancreatic Surgery, Zhongshan Hospital, Fudan University, Shanghai, China
| | - Yuan Fang
- Department of Pancreatic Surgery, Zhongshan Hospital, Fudan University, Shanghai, China
| | - Siyang Han
- The Research Institution of General Surgery, Zhongshan Hospital, Fudan University, Shanghai, China
| | - Yumeng Cai
- Department of Pathology, Zhongshan Hospital, Fudan University, Shanghai, China
| | - Tiantao Kuang
- The Research Institution of General Surgery, Zhongshan Hospital, Fudan University, Shanghai, China
| | - Wenhui Lou
- Department of Pancreatic Surgery, Zhongshan Hospital, Fudan University, Shanghai, China
| | - Dansong Wang
- The Research Institution of General Surgery, Zhongshan Hospital, Fudan University, Shanghai, China
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15
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Shaib WL, Zakka K, Huang W, Chen Z, Alese OB, Wu C, Akce M, El-Rayes BF. Survival Outcomes of Acinar Cell Pancreatic Cancer: A National Cancer Database Analysis. Pancreas 2021; 50:529-536. [PMID: 33939665 DOI: 10.1097/mpa.0000000000001788] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/10/2022]
Abstract
OBJECTIVES Acinar cell pancreatic carcinomas (ACPCs) are rare neoplasms accounting for 1% to 2% of pancreatic tumors in adults. The objective of this study is to evaluate the benefit of chemotherapy in the adjuvant setting in resected ACPC and in the palliative setting for metastatic ACPC. METHODS Data were obtained from all US hospitals that contributed to the National Cancer Database between 2004 and 2014. Cases were identified using the histology code 8550. RESULTS A total of 593 patients with ACPC were identified. The mean age was 64.4 years (range, 18-90 years), with a male preponderance (72.8%, n = 432). Localized stage disease comprised 52.3% (n = 310) of patients. Among localized ACPC patients, 88.0% (n = 191) underwent surgery and 50.6% (n = 91) received adjuvant chemotherapy. The 5-year overall survival in those who received adjuvant treatment was slightly higher than those who did not receive adjuvant treatment (46.7% vs 44.8%, P = 0.3271). Among advanced-stage ACPC patients, 67.6% received chemotherapy, which translated into improved 5-year overall survival compared with no chemotherapy (8.1% vs 0%, P < 0.0001). CONCLUSIONS Chemotherapy in the palliative setting for advanced-stage ACPC patients was associated with improved survival. Adjuvant therapy did not translate into significant survival benefit.
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Affiliation(s)
- Walid L Shaib
- From the Department of Hematology and Oncology, Winship Cancer Institute
| | - Katerina Zakka
- From the Department of Hematology and Oncology, Winship Cancer Institute
| | - Weixing Huang
- Winship Research Informatics, Biostatistics, Emory University, Atlanta, GA
| | - Zhengjia Chen
- Winship Research Informatics, Biostatistics, Emory University, Atlanta, GA
| | - Olatunji B Alese
- From the Department of Hematology and Oncology, Winship Cancer Institute
| | - Christina Wu
- From the Department of Hematology and Oncology, Winship Cancer Institute
| | - Mehmet Akce
- From the Department of Hematology and Oncology, Winship Cancer Institute
| | - Bassel F El-Rayes
- From the Department of Hematology and Oncology, Winship Cancer Institute
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16
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Huang X, Li M, Zhang L, Xiong J, Lu H, Tian B. Clinical characteristics and treatment analysis of pancreatic acinar cell carcinoma: A single institutional comparison to pancreatic ductal adenocarcinoma. Surg Oncol 2021; 37:101528. [PMID: 33611029 DOI: 10.1016/j.suronc.2021.101528] [Citation(s) in RCA: 5] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/08/2020] [Revised: 01/12/2021] [Accepted: 01/25/2021] [Indexed: 02/08/2023]
Abstract
BACKGROUND Pancreatic acinar cell carcinoma (PACC) is a rare malignancy that accounts for less than 1% of primary pancreatic neoplasms. Currently, the lack of large-scale clinical studies limits our understanding of PACC. The aim of this study was to investigate the clinical characteristics and prognosis of PACC. METHODS In a retrospective analysis, 52 patients with PACC and 355 patients with pancreatic ductal adenocarcinoma (PDAC) who underwent surgical interventions were evaluated. Clinical characteristics and treatment outcomes were compared between the two groups. RESULTS The mean age was lower for patients with PACC than for those with PDAC (mean: 50.8 ± 10.9 versus 59.4 ± 10.9 years; p < 0.001). Except for alpha-fetoprotein (AFP), tumour markers were also lower in the PACC group than the PDAC group. In regard to tumour characteristics, maximum diameters of the primary tumour [median (range): 5.0 cm (1.0-18.2 cm) versus 3.5 cm (0.6-15.0 cm); p < 0.001] and hepatic metastatic lesions [6.7 cm (1.5-12.6 cm) versus 1.2 cm (0.3-3.3 cm); p < 0.001] were larger in patients with PACC than patients with PDAC, but vascular invasion [23.1% (12/52) versus 35.5% (126/355); p = 0.044] and perineural invasion [7.7% (4/52) versus 56.1% (199/355); p < 0.001] were more common in patents with PDAC than in patients with PACC. For treatment, radical resection was performed in 57.7% of patients with PACC, which increased the 5-year survival rate to 31.8%. In regard to prognosis, the 5-year survival rate was 21.4% for PACC and 9.7% for PDAC (p < 0.0001). CONCLUSIONS PACC is more indolent than PDAC, which makes early diagnosis more difficult. Although the stage may be advanced at diagnosis, the overall survival (OS) of PACC is much better than that of PDAC, and the prognosis greatly improves after radical resection.
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Affiliation(s)
- Xing Huang
- Department of Pancreatic Surgery, West China Hospital, Sichuan University, No. 37 Guoxue Alley, Chengdu, Sichuan, China.
| | - Mao Li
- Department of Pancreatic Surgery, West China Hospital, Sichuan University, No. 37 Guoxue Alley, Chengdu, Sichuan, China.
| | - Ling Zhang
- Department of Pancreatic Surgery, West China Hospital, Sichuan University, No. 37 Guoxue Alley, Chengdu, Sichuan, China.
| | - Junjie Xiong
- Department of Pancreatic Surgery, West China Hospital, Sichuan University, No. 37 Guoxue Alley, Chengdu, Sichuan, China.
| | - Huimin Lu
- Department of Pancreatic Surgery, West China Hospital, Sichuan University, No. 37 Guoxue Alley, Chengdu, Sichuan, China.
| | - Bole Tian
- Department of Pancreatic Surgery, West China Hospital, Sichuan University, No. 37 Guoxue Alley, Chengdu, Sichuan, China.
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Miksch RC, Schiergens TS, Weniger M, Ilmer M, Kazmierczak PM, Guba MO, Angele MK, Werner J, D'Haese JG. Pancreatic panniculitis and elevated serum lipase in metastasized acinar cell carcinoma of the pancreas: A case report and review of literature. World J Clin Cases 2020; 8:5304-5312. [PMID: 33269263 PMCID: PMC7674712 DOI: 10.12998/wjcc.v8.i21.5304] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/28/2020] [Revised: 07/28/2020] [Accepted: 09/28/2020] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Pancreatic panniculitis is an extremely rare condition associated with different underlying pancreatic disorders and characterized by subcutaneous fat necrosis induced by elevated serum lipase levels. These lesions usually affect the lower extremities and may precede abdominal symptoms of pancreatic disease. Acinar cell carcinoma (ACC) of the pancreas is a rare pancreatic neoplasm, accounting for only 1%-2% of pancreatic tumors in adults.
CASE SUMMARY We present the case of a 72-year-old man with ACC of the pancreatic head and synchronous liver metastases. Both the primary tumor and liver metastases were resected. Serum lipase was elevated before surgery and decreased to normal postoperatively. Rising serum lipase levels at follow-up led to the diagnosis of hepatic recurrence. This disease progression was then accompanied by pancreatic panniculitis, with subcutaneous fat necrosis and acute arthritis. To the best of our knowledge, only 4 cases have been reported in the literature and each showed a similar association of serum lipase levels with pancreatic panniculitis and progression of ACC.
CONCLUSION Clinical symptoms and progression of ACC may correlate with serum lipase levels, suggesting potential usefulness as a follow-up biomarker.
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Affiliation(s)
- Rainer Christoph Miksch
- Department of General, Visceral, and Transplantation Surgery, Ludwig-Maximilians-University Munich, Munich 81377, Bavaria, Germany
| | - Tobias S Schiergens
- Department of General, Visceral, and Transplantation Surgery, Ludwig-Maximilians-University Munich, Munich 81377, Bavaria, Germany
| | - Maximilian Weniger
- Department of General, Visceral, and Transplantation Surgery, Ludwig-Maximilians-University Munich, Munich 81377, Bavaria, Germany
| | - Matthias Ilmer
- Department of General, Visceral, and Transplantation Surgery, Ludwig-Maximilians-University Munich, Munich 81377, Bavaria, Germany
| | - Philipp M Kazmierczak
- Department of Radiology, Ludwig-Maximilians-University Munich, Munich 81377, Germany
| | - Markus O Guba
- Department of General, Visceral, and Transplantation Surgery, Ludwig-Maximilians-University Munich, Munich 81377, Bavaria, Germany
| | - Martin K Angele
- Department of General, Visceral, and Transplantation Surgery, Ludwig-Maximilians-University Munich, Munich 81377, Bavaria, Germany
| | - Jens Werner
- Department of General, Visceral, and Transplantation Surgery, Ludwig-Maximilians-University Munich, Munich 81377, Bavaria, Germany
| | - Jan G D'Haese
- Department of General, Visceral, and Transplantation Surgery, Ludwig-Maximilians-University Munich, Munich 81377, Bavaria, Germany
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18
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Clarke EM, Stevens SG, Bennett T, Crowley P, Starkey G. The surgical management of metastatic pancreatic acinar cell carcinoma and associated pancreatic panniculitis-A case report and literature review. Int J Surg Case Rep 2020; 76:539-544. [PMID: 33207427 PMCID: PMC7599369 DOI: 10.1016/j.ijscr.2020.10.062] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/18/2020] [Revised: 10/13/2020] [Accepted: 10/13/2020] [Indexed: 02/07/2023] Open
Abstract
Pancreatic acinar cell carcinoma (ACC) may present with pancreatic panniculitis. Complete surgical resection of ACC can successfully treat pancreatic panniculitis. Aggressive surgery for ACC can lead to prolonged disease-free survival. Introduction Pancreatic panniculitis is a rare manifestation of benign and malignant pancreatic disease. The presentation of pancreatic panniculitis is non-specific and thus diagnosis is often delayed. When associated with malignancy, pancreatic panniculitis confers a poor prognosis. This case demonstrates the successful surgical management of this paraneoplastic phenomenon following resection of the underlying pancreatic acinar cell carcinoma and associated liver metastasis. Presentation of case A 71-year-old female with debilitating subcutaneous lower limb lesions had a delayed diagnosis of pancreatic panniculitis. A formal diagnosis of pancreatic acinar cell carcinoma with liver metastasis was established and the disease was determined to be resectable. Pre-operatively, serum lipase measured 10,825 U/L. The patient proceeded to an open left hemihepatectomy and radical distal pancreatectomy with complete resection of malignant disease. Six days post-operatively the serum lipase levels normalised, and the panniculitis began to settle. The patient proceeded to adjuvant FOLFORINOX chemotherapy. Twenty months post-surgery, the patient remains disease-free and without any evidence of panniculitis. Discussion Due to the rarity of pancreatic acinar cell carcinoma, guidelines based on prospective data do not exist. Most management is based on retrospective analyses. A survival benefit may be achieved with more aggressive surgical management compared to other pancreatic cancer types. Pancreatic acinar cell carcinoma may show a slower rate of disease progression, an increased likelihood of resectability of disease at presentation and is more likely to undergo potentially curative resection. Conclusion Aggressive surgical management of resectable metastatic pancreatic acinar cell carcinoma can treat pancreatic panniculitis and provide sustained disease-free survival from pancreatic cancer.
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Affiliation(s)
- Edward M Clarke
- Department of Surgery, Austin Hospital, Melbourne, Victoria, Australia.
| | - Sean G Stevens
- Department of Surgery, Austin Hospital, Melbourne, Victoria, Australia
| | - Tim Bennett
- Department of Rheumatology, Eastern Health, Box Hill, Melbourne, Australia
| | - Peter Crowley
- Dorevitch Pathology, Heidelberg Laboratory, Melbourne, Australia
| | - Graham Starkey
- Department of Surgery, Austin Hospital, Melbourne, Victoria, Australia; Department of Surgery, Warringal Private Hospital, Heidelberg, Melbourne, Australia
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19
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Niger M, Prisciandaro M, Antista M, Monica MAT, Cattaneo L, Prinzi N, Manglaviti S, Nichetti F, Brambilla M, Torchio M, Corti F, Pusceddu S, Coppa J, Mazzaferro V, de Braud F, Di Bartolomeo M. One size does not fit all for pancreatic cancers: A review on rare histologies and therapeutic approaches. World J Gastrointest Oncol 2020; 12:833-849. [PMID: 32879662 PMCID: PMC7443847 DOI: 10.4251/wjgo.v12.i8.833] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/31/2020] [Revised: 05/01/2020] [Accepted: 05/20/2020] [Indexed: 02/05/2023] Open
Abstract
Exocrine pancreatic neoplasms represent up to 95% of pancreatic cancers (PCs) and are widely recognized among the most lethal solid cancers, with a very poor 5-year survival rate of 5%-10%. The remaining < 5% of PCs are neuroendocrine tumors that are usually characterized by a better prognosis, with a median overall survival of 3.6 years. The most common type of PC is pancreatic ductal adenocarcinoma (PDAC), which accounts for roughly 85% of all exocrine PCs. However up to 10% of exocrine PCs have rare histotypes, which are still poorly understood. These subtypes can be distinguished from PDAC in terms of pathology, imaging, clinical presentation and prognosis. Additionally, due to their rarity, any knowledge regarding these specific histotypes is mostly based on case reports and a small series of retrospective analyses. Therefore, treatment strategies are generally deduced from those used for PDAC, even if these patients are often excluded or not clearly represented in clinical trials for PDAC. For these reasons, it is essential to collect as much information as possible on the management of PC, as assimilating it with PDAC may lead to the potential mistreatment of these patients. Here, we report the most significant literature regarding the epidemiology, typical presentation, possible treatment strategies, and prognosis of the most relevant histotypes among rare PCs.
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Affiliation(s)
- Monica Niger
- Medical Oncology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
| | - Michele Prisciandaro
- Medical Oncology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
| | - Maria Antista
- Medical Oncology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
| | - Melissa Anna Teresa Monica
- First Pathology Division, Department of Pathology and Laboratory Medicine, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
| | - Laura Cattaneo
- First Pathology Division, Department of Pathology and Laboratory Medicine, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
| | - Natalie Prinzi
- Medical Oncology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
| | - Sara Manglaviti
- Medical Oncology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
| | - Federico Nichetti
- Medical Oncology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
| | - Marta Brambilla
- Medical Oncology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
| | - Martina Torchio
- Medical Oncology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
| | - Francesca Corti
- Medical Oncology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
| | - Sara Pusceddu
- Medical Oncology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
| | - Jorgelina Coppa
- Hepato-biliary-pancreatic Surgery and Liver Transplantation Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
| | - Vincenzo Mazzaferro
- Hepato-biliary-pancreatic Surgery and Liver Transplantation Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
- Università degli studi di Milano, Milan 20133, Italy
| | - Filippo de Braud
- Medical Oncology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
- Università degli studi di Milano, Milan 20133, Italy
| | - Maria Di Bartolomeo
- Medical Oncology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
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20
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Abstract
OBJECTIVES Acinar cell carcinoma of the pancreas is a rare tumor with limited data. We aim to evaluate the characteristics, treatments, and outcomes of pancreatic acinar cell carcinoma after 2005. METHODS We retrospectively reviewed patients with pancreatic acinar cell carcinoma treated in Peking University Cancer Hospital and Institute (2005-2018) and identified cases from Surveillance, Epidemiology, and End Results database (2005-2015). RESULTS A total of 306 cases in our institute (n = 11) and Surveillance, Epidemiology, and End Results database (n = 295) were identified. The median age was 67 years, and 73.5% were male. The 5-year survival was 36.8% for all patients (median, 27 months). About 37% underwent surgical resection. The 5-year survival was 65.6% for resected patients as compared with 16.9% for unresected ones (P < 0.0001). Among locoregional and metastatic diseases, surgery significantly prolonged survival as well (P = 0.0003). Stage IV patients who received chemotherapy had a better survival than those without it (median, 16 vs 3 months; P = 0.0019). Aging, stage IV, and no surgery were independent predictors of poor overall survival. CONCLUSIONS For pancreatic acinar cell carcinoma, surgery is a potentially curative treatment contributing to long-term survival and suggested even in advanced diseases. Chemotherapy improved survival for metastatic patients.
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Di Marco M, Carloni R, De Lorenzo S, Grassi E, Palloni A, Formica F, Brocchi S, Filippini DM, Golfieri R, Brandi G. Long-term survival of two patients with recurrent pancreatic acinar cell carcinoma treated with radiofrequency ablation: A case report. World J Clin Cases 2020; 8:1241-1250. [PMID: 32337198 PMCID: PMC7176612 DOI: 10.12998/wjcc.v8.i7.1241] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/19/2019] [Revised: 01/08/2020] [Accepted: 03/11/2020] [Indexed: 02/05/2023] Open
Abstract
BACKGROUND Pancreatic acinar cell carcinoma (PACC) is a rare type of malignant pancreatic cancer that represents approximately 1% of all pancreatic neoplasms. Due to its very low incidence, only a few retrospective studies are available. Although surgery is the first choice for treatment, most patients experience recurrence (mainly in the liver) and there are no clear recommendations for patients with advanced disease.
CASE SUMMARY We report two patients with PACC treated with surgery who experienced tumour recurrence in the liver. Patient 1 carried a germline mutation in the APC gene. Both patients were treated with gemcitabine plus oxaliplatin and gemcitabine plus capecitabine as first- and second-line therapies, respectively. After a favourable response to chemotherapy, the patients underwent radiofrequency ablation of the remaining liver metastases. For patient 1, we documented a relapse in the liver after a disease-free period of 9 mo, and treatment with gemcitabine plus capecitabine was restarted. The patient achieved a complete response, and he remains alive without evidence of disease recurrence after six years. After radiofrequency ablation, patient 2 experienced disease-free survival for 21 mo, when peritoneal relapse was diagnosed and treated with chemotherapy. The patient achieved a stable disease state for nearly two years; nevertheless, further progressive disease was documented, and he died seven years after the first relapse.
CONCLUSION PACC presents different biological behaviours than pancreatic adenocarcinoma. Multidisciplinary treatment involving local ablative therapies may be considered for PACC.
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Affiliation(s)
- Mariacristina Di Marco
- Department of Experimental, Diagnostic, and Specialty Medicine - DIMES, Sant'Orsola-Malpighi Hospital, University of Bologna, Bologna 40138, Italy
| | - Riccardo Carloni
- Department of Experimental, Diagnostic, and Specialty Medicine - DIMES, Sant'Orsola-Malpighi Hospital, University of Bologna, Bologna 40138, Italy
| | - Stefania De Lorenzo
- Department of Experimental, Diagnostic, and Specialty Medicine - DIMES, Sant'Orsola-Malpighi Hospital, University of Bologna, Bologna 40138, Italy
| | - Elisa Grassi
- Medical Oncology, Ospedale degli Infermi, Faenza 48018, Italy
| | - Andrea Palloni
- Department of Experimental, Diagnostic, and Specialty Medicine - DIMES, Sant'Orsola-Malpighi Hospital, University of Bologna, Bologna 40138, Italy
| | - Francesca Formica
- Department of Experimental, Diagnostic, and Specialty Medicine - DIMES, Sant'Orsola-Malpighi Hospital, University of Bologna, Bologna 40138, Italy
| | - Stefano Brocchi
- Radiology Unit, Department of Diagnostic Medicine and Prevention, Sant'Orsola-Malpighi Hospital, University of Bologna, Bologna 40138, Italy
| | - Daria Maria Filippini
- Department of Experimental, Diagnostic, and Specialty Medicine - DIMES, Sant'Orsola-Malpighi Hospital, University of Bologna, Bologna 40138, Italy
| | - Rita Golfieri
- Radiology Unit, Department of Diagnostic Medicine and Prevention, Sant'Orsola-Malpighi Hospital, University of Bologna, Bologna 40138, Italy
| | - Giovanni Brandi
- Department of Experimental, Diagnostic, and Specialty Medicine - DIMES, Sant'Orsola-Malpighi Hospital, University of Bologna, Bologna 40138, Italy
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22
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He C, Zhang Y, Cai Z, Duan F, Lin X, Li S. Nomogram to Predict Cancer-Specific Survival in Patients with Pancreatic Acinar Cell Carcinoma: A Competing Risk Analysis. J Cancer 2018; 9:4117-4127. [PMID: 30519311 PMCID: PMC6277614 DOI: 10.7150/jca.26936] [Citation(s) in RCA: 18] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/27/2018] [Accepted: 07/22/2018] [Indexed: 02/07/2023] Open
Abstract
Background: The objective of this study was to evaluate the probability of cancer-specific death of patients with acinar cell carcinoma (ACC) and build nomograms to predict overall survival (OS) and cancer-specific survival (CSS) of these patients. Methods: Data were extracted from the Surveillance, Epidemiology, and End Results (SEER) database. Patients diagnosed with ACC between 2004 and 2014 were retrospectively collected. Cancer-specific mortality and competing risk mortality were evaluated. Nomograms for estimating 1-, 2- and 3-year OS and CSS were established based on Cox regression model and Fine and Grey's model. The precision of the 1-, 2- and 3-year survival of the nomograms was evaluated and compared using the area under receiver operating characteristic (ROC) curve (AUC). Results: The study cohort included 227 patients with ACC. The established nomograms were well calibrated, and had good discriminative ability, with a concordance index (C-index) of 0.742 for OS prediction and 0.766 for CSS prediction. The nomograms displayed better discrimination power than 7th or 8th edition Tumor-Node-Metastasis (TNM) stage systems in training set and validation set for predicting both OS and CSS. The AUC values of the nomogram predicting 1-, 2-, and 3-year OS rates were 0.784, 0.797 and 0.805, respectively, which were higher than those of 7th or 8th edition TNM stage systems. Regard to the prediction of CSS rates, the AUC values of the nomogram were also higher than those of 7th or 8th edition TNM stage systems. Conclusion: We evaluated the 1-, 2- and 3-year OS and CSS in patients with ACC for the first time. Our nomograms showed relatively good performance and could be considered as convenient individualized predictive tools for prognosis.
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Affiliation(s)
- Chaobin He
- Department of Hepatobiliary and Pancreatic Surgery, State Key Laboratory of Oncology in South China, Collaborative Innovation Center for Cancer Medicine, Sun Yat-sen University Cancer Center, Guangzhou 510060, China
| | - Yu Zhang
- State Key Laboratory of Ophthalmology, Zhongshan Ophthalmic Center, Sun Yat-sen University, Guangzhou, Guangdong, 510060, P.R. China
| | - Zhiyuan Cai
- Department of Hepatobiliary and Pancreatic Surgery, State Key Laboratory of Oncology in South China, Collaborative Innovation Center for Cancer Medicine, Sun Yat-sen University Cancer Center, Guangzhou 510060, China
| | - Fangting Duan
- Department of Hepatobiliary and Pancreatic Surgery, State Key Laboratory of Oncology in South China, Collaborative Innovation Center for Cancer Medicine, Sun Yat-sen University Cancer Center, Guangzhou 510060, China
| | - Xiaojun Lin
- Department of Hepatobiliary and Pancreatic Surgery, State Key Laboratory of Oncology in South China, Collaborative Innovation Center for Cancer Medicine, Sun Yat-sen University Cancer Center, Guangzhou 510060, China
| | - Shengping Li
- Department of Hepatobiliary and Pancreatic Surgery, State Key Laboratory of Oncology in South China, Collaborative Innovation Center for Cancer Medicine, Sun Yat-sen University Cancer Center, Guangzhou 510060, China
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23
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Ohara Y, Oda T, Enomoto T, Hisakura K, Akashi Y, Ogawa K, Owada Y, Domoto Y, Miyazaki Y, Shimomura O, Kurata M, Ohkohchi N. Surgical resection of hepatic and rectal metastases of pancreatic acinar cell carcinoma (PACC): a case report. World J Surg Oncol 2018; 16:158. [PMID: 30075727 PMCID: PMC6091145 DOI: 10.1186/s12957-018-1457-8] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/27/2018] [Accepted: 07/26/2018] [Indexed: 02/07/2023] Open
Abstract
Background Pancreatic acinar cell carcinoma (PACC), a rare variant of pancreatic malignancy, is generally managed the same way as pancreatic ductal adenocarcinoma (PDAC). Surgical resection is the gateway to curing it; however, once it metastasizes (usually to the liver, lungs, lymph nodes, or peritoneal cavity), systemic chemotherapy has been the only option, but with unfavorable results. Case presentation A 67-year-old man with symptoms of loss of appetite and weight underwent surgery for malignancy of the pancreatic tail extending into the entire pancreas. The pathological diagnosis was PACC following total pancreatectomy. Twenty-four months after the pancreatectomy, a solitary liver metastasis was treated by partial hepatectomy, and, subsequently, 4 months later, he presented with melena. Further examination revealed a type-2 rectal tumor. Histological examination following biopsy revealed it to be rectal metastasis of PACC, and it was treated by abdominoperineal resection. Subsequently, the patient did not have tumor recurrence as of 40 months after pancreatectomy. Conclusions This is a rare case of PACC presenting with metachronal metastases in the liver and rectum, and we successfully treated them by surgical resections. Since the malignant behavior of PACC is usually less than that of PDAC, surgical resection could be an option even for metastatic lesions when the number and extent of metastases are limited.
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Affiliation(s)
- Yusuke Ohara
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Faculty of Medicine, University of Tsukuba, 1-1-1 Tennodai, Tsukuba, Ibaraki, 305-8575, Japan
| | - Tatsuya Oda
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Faculty of Medicine, University of Tsukuba, 1-1-1 Tennodai, Tsukuba, Ibaraki, 305-8575, Japan.
| | - Tsuyoshi Enomoto
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Faculty of Medicine, University of Tsukuba, 1-1-1 Tennodai, Tsukuba, Ibaraki, 305-8575, Japan
| | - Katsuji Hisakura
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Faculty of Medicine, University of Tsukuba, 1-1-1 Tennodai, Tsukuba, Ibaraki, 305-8575, Japan
| | - Yoshimasa Akashi
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Faculty of Medicine, University of Tsukuba, 1-1-1 Tennodai, Tsukuba, Ibaraki, 305-8575, Japan
| | - Koichi Ogawa
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Faculty of Medicine, University of Tsukuba, 1-1-1 Tennodai, Tsukuba, Ibaraki, 305-8575, Japan
| | - Yohei Owada
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Faculty of Medicine, University of Tsukuba, 1-1-1 Tennodai, Tsukuba, Ibaraki, 305-8575, Japan
| | - Yu Domoto
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Faculty of Medicine, University of Tsukuba, 1-1-1 Tennodai, Tsukuba, Ibaraki, 305-8575, Japan
| | - Yoshihiro Miyazaki
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Faculty of Medicine, University of Tsukuba, 1-1-1 Tennodai, Tsukuba, Ibaraki, 305-8575, Japan
| | - Osamu Shimomura
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Faculty of Medicine, University of Tsukuba, 1-1-1 Tennodai, Tsukuba, Ibaraki, 305-8575, Japan
| | - Masanao Kurata
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Faculty of Medicine, University of Tsukuba, 1-1-1 Tennodai, Tsukuba, Ibaraki, 305-8575, Japan
| | - Nobuhiro Ohkohchi
- Department of Gastrointestinal and Hepato-Biliary-Pancreatic Surgery, Faculty of Medicine, University of Tsukuba, 1-1-1 Tennodai, Tsukuba, Ibaraki, 305-8575, Japan
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24
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Ang C, Herran LA, Lagunes DR, Klimstra DS, Kemeny NE. A Case Report of a Patient with Advanced Acinar Cell Carcinoma of the Pancreas: Long-Term Survival with Regional, Systemic and Targeted Therapy. TUMORI JOURNAL 2018; 99:e61-4. [DOI: 10.1177/030089161309900230] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
Abstract
Acinar cell carcinoma of the pancreas is an uncommon malignancy for which there are no prospective, randomized data to guide therapy. We describe the clinical course and management of a patient with advanced pancreatic acinar cell carcinoma who is alive seven years after diagnosis using systemic and regional chemotherapies as well as molecularly targeted agents.
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Affiliation(s)
- Celina Ang
- Department of Medicine, Gastrointestinal Oncology Service, Memorial Sloan-Kettering Cancer Center, New York, NY
| | | | - Diane Reidy Lagunes
- Department of Medicine, Gastrointestinal Oncology Service, New York, NY, USA
| | - David S Klimstra
- Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, NY, USA
| | - Nancy E Kemeny
- Department of Medicine, Gastrointestinal Oncology Service, Memorial Sloan-Kettering Cancer Center, New York, NY
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25
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Al-Hader A, Al-Rohil RN, Han H, Von Hoff D. Pancreatic acinar cell carcinoma: A review on molecular profiling of patient tumors. World J Gastroenterol 2017; 23:7945-7951. [PMID: 29259370 PMCID: PMC5725289 DOI: 10.3748/wjg.v23.i45.7945] [Citation(s) in RCA: 53] [Impact Index Per Article: 6.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/03/2017] [Revised: 09/17/2017] [Accepted: 10/26/2017] [Indexed: 02/06/2023] Open
Abstract
Pancreatic carcinomas with acinar differentiation are rare, accounting for 1%-2% of adult pancreatic tumors; they include pancreatic acinar cell carcinoma (PACC), pancreatoblastoma, and carcinomas of mixed differentiation. Patients with PACC have a prognosis better than pancreatic ductal adenocarcinomas but worse than pancreatic neuroendocrine tumors. Reports of overall survival range from 18 to 47 mo. A literature review on PACCs included comprehensive genomic profiling and whole exome sequencing on a series of more than 70 patients as well as other diagnostic studies including immunohistochemistry. Surgical resection of PACC is the preferred treatment for localized and resectable tumors. The efficacy of adjuvant treatment is unclear. Metastatic PACCs are generally not curable and treated with systemic chemotherapy. They are moderately responsive to chemotherapy with different regimens showing various degrees of response in case reports/series. Most of these regimens were developed to treat patients with pancreatic ductal adenocarcinomas or colorectal adenocarcinomas. Review of PACC's molecular profiling showed a number of gene alterations such as: SMAD4, BRAF, BRCA2, TP53, RB1, MEN1, JAK-1, BRCA-1, BRCA-2, and DNA mismatch repair abnormalities. PACCs had multiple somatic mutations with some targetable with available drugs. Therefore, molecular profiling of PACC should be an option for patients with refractory PACC.
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Affiliation(s)
- Ahmad Al-Hader
- Division of Hematology/Oncology, Indiana University School of Medicine, Indianapolis, IN 46202-3082, United States
| | - Rami N Al-Rohil
- Department of Pathology, Vanderbilt University School of Medicine, Nashville, TN 37232, United States
| | - Haiyong Han
- Molecular Medicine Division, Translational Genomics Research Institute (TGen), Phoenix, AZ 85004, United States
| | - Daniel Von Hoff
- Molecular Medicine Division, Translational Genomics Research Institute (TGen), Phoenix, AZ 85004, United States
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26
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Jäkel C, Bergmann F, Toth R, Assenov Y, van der Duin D, Strobel O, Hank T, Klöppel G, Dorrell C, Grompe M, Moss J, Dor Y, Schirmacher P, Plass C, Popanda O, Schmezer P. Genome-wide genetic and epigenetic analyses of pancreatic acinar cell carcinomas reveal aberrations in genome stability. Nat Commun 2017; 8:1323. [PMID: 29109526 PMCID: PMC5673892 DOI: 10.1038/s41467-017-01118-x] [Citation(s) in RCA: 51] [Impact Index Per Article: 6.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/02/2017] [Accepted: 08/18/2017] [Indexed: 02/08/2023] Open
Abstract
Pancreatic acinar cell carcinoma (ACC) is an aggressive exocrine tumor with largely unknown biology. Here, to identify potential targets for personalized treatment, we perform integrative genome-wide and epigenome-wide analyses. The results show frequently aberrant DNA methylation, abundant chromosomal amplifications and deletions, and mutational signatures suggesting defective DNA repair. In contrast to pancreatic ductal adenocarcinoma, no recurrent point mutations are detected. The tumor suppressors ID3, ARID1A, APC, and CDKN2A are frequently impaired also on the protein level and thus potentially affect ACC tumorigenesis. Consequently, this work identifies promising therapeutic targets in ACC for drugs recently approved for precision cancer therapy.
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Affiliation(s)
- Cornelia Jäkel
- Division of Epigenomics and Cancer Risk Factors, German Cancer Research Center (DKFZ), Im Neuenheimer Feld 280, 69120, Heidelberg, Germany
| | - Frank Bergmann
- Institute of Pathology, University Hospital Heidelberg, Im Neuenheimer Feld 224, 69120, Heidelberg, Germany
| | - Reka Toth
- Division of Epigenomics and Cancer Risk Factors, German Cancer Research Center (DKFZ), Im Neuenheimer Feld 280, 69120, Heidelberg, Germany
| | - Yassen Assenov
- Division of Epigenomics and Cancer Risk Factors, German Cancer Research Center (DKFZ), Im Neuenheimer Feld 280, 69120, Heidelberg, Germany
| | - Daniel van der Duin
- Division of Epigenomics and Cancer Risk Factors, German Cancer Research Center (DKFZ), Im Neuenheimer Feld 280, 69120, Heidelberg, Germany
| | - Oliver Strobel
- Department of General and Visceral Surgery, University Hospital Heidelberg, Im Neuenheimer Feld 110, 69120, Heidelberg, Germany
| | - Thomas Hank
- Department of General and Visceral Surgery, University Hospital Heidelberg, Im Neuenheimer Feld 110, 69120, Heidelberg, Germany
| | - Günter Klöppel
- Institute of Pathology, Technical University Munich, Trogerstr. 18, 81675, Munich, Germany
| | - Craig Dorrell
- Department of Pediatrics, Papé Family Pediatric Research Institute, Oregon Stem Cell Center, Oregon Health and Science University, Portland, OR, 97239, USA
| | - Markus Grompe
- Department of Pediatrics, Papé Family Pediatric Research Institute, Oregon Stem Cell Center, Oregon Health and Science University, Portland, OR, 97239, USA
| | - Joshua Moss
- Department of Developmental Biology and Cancer Research, Institute for Medical Research Israel-Canada, The Hebrew University-Hadassah Medical School, 9112102, Jerusalem, Israel
| | - Yuval Dor
- Department of Developmental Biology and Cancer Research, Institute for Medical Research Israel-Canada, The Hebrew University-Hadassah Medical School, 9112102, Jerusalem, Israel
| | - Peter Schirmacher
- Institute of Pathology, University Hospital Heidelberg, Im Neuenheimer Feld 224, 69120, Heidelberg, Germany
| | - Christoph Plass
- Division of Epigenomics and Cancer Risk Factors, German Cancer Research Center (DKFZ), Im Neuenheimer Feld 280, 69120, Heidelberg, Germany
| | - Odilia Popanda
- Division of Epigenomics and Cancer Risk Factors, German Cancer Research Center (DKFZ), Im Neuenheimer Feld 280, 69120, Heidelberg, Germany
| | - Peter Schmezer
- Division of Epigenomics and Cancer Risk Factors, German Cancer Research Center (DKFZ), Im Neuenheimer Feld 280, 69120, Heidelberg, Germany.
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27
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Glazer ES, Neill KG, Frakes JM, Coppola D, Hodul PJ, Hoffe SE, Pimiento JM, Springett GM, Malafa MP. Systematic Review and Case Series Report of Acinar Cell Carcinoma of the Pancreas. Cancer Control 2017; 23:446-454. [PMID: 27842335 DOI: 10.1177/107327481602300417] [Citation(s) in RCA: 42] [Impact Index Per Article: 5.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/17/2022] Open
Abstract
BACKGROUND Acinar cell carcinoma of the pancreas is a rare malignancy representing less than 1% of all pancreatic malignancies. METHODS We report on a case series of 21 patients with acinar cell carcinoma of the pancreas treated at a high-volume quaternary center. A systematic review of the medical literature was performed that described typical therapeutic management approaches for acinar cell carcinoma of the pancreas and reported on disease control and survival rates. Data for the case series were obtained from a prospective database. RESULTS In our systematic review of 6 articles, study patients had a median age of 61 years, 66% were male, 52% had stage I/II disease, and 55% of lesions were located in the pancreatic head. The rates of median survival were approximately 47 months after resection with adjuvant therapy, 38 months for nonmetastatic, locally unresectable disease, and 17 months for metastatic disease treated with chemotherapy. Combination fluoropyrimidine-based chemotherapy regimens had better rates of disease control than other therapies. Our case series included 21 study patients, 14 of whom required resection and 7 who had metastatic disease. The rates of median survival were 40.2 ± 31.9 months in those who underwent surgery and were treated with adjuvant therapy and 13.8 ± 11.3 months for patients with metastatic disease. CONCLUSIONS Multidisciplinary treatment for acinar cell carcinoma of the pancreas should be considered due to the rarity of the disease and its lack of high-level therapeutic data. Progress in the molecular analysis of this tumor may improve outcomes through the use of personalized therapy based on underlying tumor mutations.
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Affiliation(s)
- Evan S Glazer
- Department of Gastrointestinal Oncology, Moffitt Cancer Center, Tampa, FL, USA.
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28
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Kruger S, Haas M, Burger PJ, Ormanns S, Modest DP, Westphalen CB, Kleespies A, Angele MK, Hartwig W, Bruns CJ, Kirchner T, Werner J, Heinemann V, Boeck S. Acinar cell carcinoma of the pancreas: a rare disease with different diagnostic and therapeutic implications than ductal adenocarcinoma. J Cancer Res Clin Oncol 2016; 142:2585-2591. [PMID: 27629876 DOI: 10.1007/s00432-016-2264-7] [Citation(s) in RCA: 24] [Impact Index Per Article: 2.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/11/2016] [Accepted: 09/06/2016] [Indexed: 02/07/2023]
Abstract
PURPOSE Acinar cell carcinoma (ACC) of the pancreas is a very rare cancer, constituting 1 % of all malignant non-endocrine pancreatic tumors. Only very limited data exist to guide treatment in patients with advanced ACC. METHODS Between 2000 and 2015, 15 patients with ACC were diagnosed and/or treated at our high-volume comprehensive cancer center. Medical records and correlating serum levels of the potential serum tumor markers CA 19-9, CEA and lipase were analyzed retrospectively. RESULTS A substantial antitumor activity was observed for treatment regimens containing 5-FU and oxaliplatin with partial responses or prolonged disease stabilizations (>12 months) observed in 6 out of 7 patients (86 %). Activity was also observed for single-agent 5-FU and its oral prodrugs. Serum lipase levels were elevated in 7 of 12 patients with advanced disease (58 %), whereas CEA and CA 19-9 seemed to be of minor importance for ACC (elevated pre-treatment levels in 4/12 and 3/12 cases, respectively). In selected patients, repeated serum lipase measurements were available and accurately predicted response to chemotherapy and relapse after surgery. CONCLUSIONS 5-FU- and oxaliplatin-containing regimens are active in advanced ACC. Lipase kinetics may be a useful novel tool to monitor the course of disease as well as treatment effects in ACC.
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Affiliation(s)
- Stephan Kruger
- Department of Internal Medicine III, Comprehensive Cancer Center, Klinikum Grosshadern, Ludwig-Maximilians-University of Munich, Marchioninistr. 15, 81377, Munich, Germany
| | - Michael Haas
- Department of Internal Medicine III, Comprehensive Cancer Center, Klinikum Grosshadern, Ludwig-Maximilians-University of Munich, Marchioninistr. 15, 81377, Munich, Germany
| | - Philipp Johannes Burger
- Department of Internal Medicine III, Comprehensive Cancer Center, Klinikum Grosshadern, Ludwig-Maximilians-University of Munich, Marchioninistr. 15, 81377, Munich, Germany
| | - Steffen Ormanns
- Institute of Pathology, Ludwig-Maximilians-University of Munich, 81377, Munich, Germany
| | - Dominik Paul Modest
- Department of Internal Medicine III, Comprehensive Cancer Center, Klinikum Grosshadern, Ludwig-Maximilians-University of Munich, Marchioninistr. 15, 81377, Munich, Germany
| | - Christoph Benedikt Westphalen
- Department of Internal Medicine III, Comprehensive Cancer Center, Klinikum Grosshadern, Ludwig-Maximilians-University of Munich, Marchioninistr. 15, 81377, Munich, Germany
| | - Axel Kleespies
- Department of General, Visceral, Transplantation, Vascular and Thoracic Surgery, Klinikum Grosshadern, Ludwig-Maximilians-University of Munich, 81377, Munich, Germany
| | - Martin Kurt Angele
- Department of General, Visceral, Transplantation, Vascular and Thoracic Surgery, Klinikum Grosshadern, Ludwig-Maximilians-University of Munich, 81377, Munich, Germany
| | - Werner Hartwig
- Department of General, Visceral, Transplantation, Vascular and Thoracic Surgery, Klinikum Grosshadern, Ludwig-Maximilians-University of Munich, 81377, Munich, Germany
| | - Christiane Josephine Bruns
- Department of General, Visceral and Tumor Surgery, University of Cologne, 50937, Köln-Lindenthal, Germany
| | - Thomas Kirchner
- Institute of Pathology, Ludwig-Maximilians-University of Munich, 81377, Munich, Germany
| | - Jens Werner
- Department of General, Visceral, Transplantation, Vascular and Thoracic Surgery, Klinikum Grosshadern, Ludwig-Maximilians-University of Munich, 81377, Munich, Germany
| | - Volker Heinemann
- Department of Internal Medicine III, Comprehensive Cancer Center, Klinikum Grosshadern, Ludwig-Maximilians-University of Munich, Marchioninistr. 15, 81377, Munich, Germany
| | - Stefan Boeck
- Department of Internal Medicine III, Comprehensive Cancer Center, Klinikum Grosshadern, Ludwig-Maximilians-University of Munich, Marchioninistr. 15, 81377, Munich, Germany.
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29
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Bergmann F. [Pancreatic acinar neoplasms : Comparative molecular characterization]. DER PATHOLOGE 2016; 37:191-195. [PMID: 27807633 DOI: 10.1007/s00292-016-0235-z] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 10/20/2022]
Abstract
Pancreatic acinar cell carcinomas are biologically aggressive neoplasms for which treatment options are very limited. The molecular mechanisms of tumor initiation and progression are largely not understood and precursor lesions have not yet been identified. In this study, pancreatic acinar cell carcinomas were cytogenetically characterized as well as by molecular and immunohistochemical analyses. Corresponding investigations were carried out on pancreatic ductal adenocarcinomas and pancreatic neuroendocrine neoplasms augmented by functional analyses. We show that pancreatic acinar cell carcinomas display a microsatellite stable, chromosomal unstable genotype, characterized by recurrent chromosomal imbalances that clearly discriminate them from pancreatic ductal adenocarcinomas and neuroendocrine neoplasms. Based on findings obtained from comparative genomic hybridization, candidate genes could be identified, such as deleted in colorectal cancer (DCC) and c-MYC. Furthermore, several therapeutic targets were identified in acinar cell carcinomas and other pancreatic neoplasms, including epidermal growth factor receptor (EGFR), L1 cell adhesion molecule (L1CAM) and heat shock protein 90 (HSP90). Moreover, L1CAM was shown to play a significant role in the tumorigenesis of pancreatic ductal adenocarcinoma. Functional analyses in cell lines derived from pancreatic neuroendocrine neoplasms revealed promising anti-tumorigenic effects using EGFR and HSP90 inhibitors affecting the cell cycle and in the case of HSP90, regulating several other oncogenes. Finally, based on mutational analyses of mitochondrial DNA, molecular evidence is provided that acinar cell cystadenomas (or better cystic acinar transformation) represent non-clonal lesions, suggesting an inflammatory reactive non-neoplastic nature.
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Affiliation(s)
- F Bergmann
- Pathologisches Institut, Universitätsklinikum Heidelberg, Im Neuenheimer Feld 224, 69120, Heidelberg, Deutschland.
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Callata-Carhuapoma HR, Pato Cour E, Garcia-Paredes B, Fernandez RM, Mendoza Fernandez ML, Fernandez AM, De La Rosa CA, Sotelo Lezama MJ, Cabezas-Camarero S, Sastre Varela J. Pancreatic acinar cell carcinoma with bilateral ovarian metastases, panniculitis and polyarthritis treated with FOLFIRINOX chemotherapy regimen. A case report and review of the literature. Pancreatology 2015; 15:440-4. [PMID: 25959244 DOI: 10.1016/j.pan.2015.04.006] [Citation(s) in RCA: 15] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 12/14/2014] [Revised: 04/09/2015] [Accepted: 04/14/2015] [Indexed: 02/08/2023]
Abstract
Pancreatic acinar cell carcinoma (PACC) is a rare pancreatic tumor, with an estimated frequency of less than 1% of pancreatic malignancies. There are no prospective studies to guide diagnostic or therapeutic algorithms. We report the case of a 36 year-old woman, diagnosed of a pancreatic tumor with liver and peritoneal metastases that was initially managed as a neuroendocrine tumor with temozolomide and capecitabine. After two cycles a severely painful arthritis developed in her left ankle with panniculitis and extensive fat necrosis, and CT scan demonstrated progressive disease. Pathology of the primary was reassessed establishing the diagnosis of PACC. The patient started treatment with FOLFIRINOX regimen, achieving clinical benefit and disease stabilization. We also briefly reviewed the literature on this rare subtype of pancreatic tumor.
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Pitman MB, Centeno BA, Ali SZ, Genevay M, Stelow E, Mino-Kenudson M, Castillo CFD, Schmidt CM, Brugge WR, Layfield LJ. Standardized terminology and nomenclature for pancreatobiliary cytology: The Papanicolaou Society of Cytopathology Guidelines. Cytojournal 2014; 11:3. [PMID: 25191517 PMCID: PMC4153338 DOI: 10.4103/1742-6413.133343] [Citation(s) in RCA: 44] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/15/2014] [Accepted: 02/06/2014] [Indexed: 02/07/2023] Open
Abstract
The Papanicolaou Society of Cytopathology has developed a set of guidelines for pancreatobiliary cytology including indications for endoscopic ultrasound (EUS) guided fine-needle aspiration (FNA) biopsy, techniques of EUS-FNA, terminology and nomenclature of pancreatobiliary disease, ancillary testing and post-biopsy treatment and management. All documents are based on the expertise of the authors, a review of the literature, discussion of the draft document at several national and international meetings over an 18 month period and synthesis of online comments of the draft document on the Papanicolaou Society of Cytopathology web site [www.papsociety.org]. This document selectively presents the results of these discussions and focuses on a proposed standardized terminology scheme for pancreatobiliary specimens that correlate cytological diagnosis with biological behavior and increasingly conservative patient management of surveillance only. The proposed terminology scheme recommends a six-tiered system: Non-diagnostic, negative, atypical, neoplastic [benign or other], suspicious and positive. Unique to this scheme is the “neoplastic” category separated into “benign” (serous cystadenoma) or “other” (premalignant mucinous cysts, neuroendocrine tumors and solid-pseudopapillary neoplasms (SPNs)). The positive or malignant category is reserved for high-grade, aggressive malignancies including ductal adenocarcinoma, acinar cell carcinoma, poorly differentiated neuroendocrine carcinomas, pancreatoblastoma, lymphoma and metastases. Interpretation categories do not have to be used. Some pathology laboratory information systems require an interpretation category, which places the cytological diagnosis into a general category. This proposed scheme provides terminology that standardizes the category of the various diseases of the pancreas, some of which are difficult to diagnose specifically by cytology. In addition, this terminology scheme attempts to provide maximum flexibility for patient management, which has become increasingly conservative for some neoplasms.
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Affiliation(s)
- Martha B Pitman
- Address: Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA
| | - Barbara A Centeno
- H. Lee Moffitt Cancer Center and Research Institute, Tampa, Florida, USA
| | - Syed Z Ali
- The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
| | | | - Ed Stelow
- University of Virginia Medical Center, Charlottesville, Virginia, USA
| | - Mari Mino-Kenudson
- Address: Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA
| | | | - C Max Schmidt
- Deparment of Surgery, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA
| | - William R Brugge
- Deparment of Surgery, Indiana University Medical Center, Indianapolis, USA
| | - Lester J Layfield
- Department of Medicine, Division of Gastroenterology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA
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Chaudhary P. Acinar Cell Carcinoma of the Pancreas: A Literature Review and Update. Indian J Surg 2014; 77:226-31. [PMID: 26246707 DOI: 10.1007/s12262-014-1049-y] [Citation(s) in RCA: 35] [Impact Index Per Article: 3.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/06/2013] [Accepted: 02/21/2014] [Indexed: 12/13/2022] Open
Abstract
Pancreatic acinar cell carcinoma is a rare tumour, accounting for only about 1 % of all pancreatic tumours. The long-term survival for patients with acinar cell carcinoma is significantly better than the long-term survival of patients with pancreatic adenocarcinoma. As no large series of patients with acinar cell carcinomas exist, our understanding of this disease comes mainly from small case series and case reports. Aggressive surgical resection with negative margins is associated with long-term survival in these more favourable pancreatic cancers. There are no clear treatment guidelines for patients in whom complete surgical resection with curative intent is not possible. Acinar cell carcinomas are chemoresponsive to agents that have activity against pancreatic adenocarcinomas and colorectal carcinomas because of the shared genetic alterations between these cancers. The role of neoadjuvant or adjuvant chemoradiotherapy remains unproven. The aim of this article is to present current knowledge on acinar cell carcinoma and comprehensive review of available literature.
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Pitman MB, Centeno BA, Ali SZ, Genevay M, Stelow E, Mino-Kenudson M, Fernandez-del Castillo C, Max Schmidt C, Brugge W, Layfield L. Standardized terminology and nomenclature for pancreatobiliary cytology: the Papanicolaou Society of Cytopathology guidelines. Diagn Cytopathol 2014; 42:338-50. [PMID: 24554455 DOI: 10.1002/dc.23092] [Citation(s) in RCA: 161] [Impact Index Per Article: 14.6] [Reference Citation Analysis] [Abstract] [Key Words] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/03/2013] [Accepted: 01/08/2014] [Indexed: 12/20/2022]
Abstract
The Papanicolaou Society of Cytopathology has developed a set of guidelines for pancreatobiliary cytology including indications for endoscopic ultrasound (EUS)-guided fine needle aspiration (FNA) biopsy, techniques of EUS-FNA, terminology and nomenclature of pancreatobiliary disease, ancillary testing, and postbiopsy treatment and management. All documents are based on the expertise of the authors, a review of the literature, discussions of the draft document at several national and international meetings over an 18-month period and synthesis of online comments of the draft document on the Papanicolaou Society of Cytopathology web site (www.papsociety.org). This document selectively presents the results of these discussions and focuses on a proposed standardized terminology scheme for pancreatobiliary specimens that correlate cytological diagnosis with biological behavior and increasingly conservative patient management of surveillance only. The proposed terminology scheme recommends a six-tiered system: Nondiagnostic, Negative, Atypical, Neoplastic (benign or other), Suspicious and Positive. Unique to this scheme is the "Neoplastic" category separated into "benign" (serous cystadenoma), or "Other" (premalignant mucinous cysts, neuroendocrine tumors, and solid-pseudopapillary neoplasms). The positive or malignant category is reserved for high-grade, aggressive malignancies including ductal adenocarcinoma, acinar cell carcinoma, poorly differentiated neuroendocrine carcinomas, pancreatoblastoma, lymphoma, and metastases. Interpretation categories do not have to be used. Some pathology laboratory information systems require an interpretation category, which places the cytological diagnosis into a general category. This proposed scheme provides terminology that standardizes the category of the various diseases of the pancreas, some of which are difficult to diagnose specifically by cytology. In addition, this terminology scheme attempts to provide maximum flexibility for patient management, which has become increasingly conservative for some neoplasms.
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Affiliation(s)
- Martha B Pitman
- Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts
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Sumiyoshi T, Shima Y, Okabayashi T, Kozuki A, Nakamura T. Comparison of pancreatic acinar cell carcinoma and adenocarcinoma using multidetector-row computed tomography. World J Gastroenterol 2013; 19:5713-5719. [PMID: 24039366 PMCID: PMC3769910 DOI: 10.3748/wjg.v19.i34.5713] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/12/2013] [Revised: 03/30/2013] [Accepted: 07/13/2013] [Indexed: 02/06/2023] Open
Abstract
AIM: To distinguish acinar cell carcinoma (ACC) from pancreatic adenocarcinoma (AC) by comparing their computed tomography findings.
METHODS: Patients with ACC and AC were identified on the basis of results obtained using surgically resected pancreatectomy specimens. The preoperative computer tomographic images of 6 acinar cell carcinoma patients and 67 pancreatic adenocarcinoma patients in 4 phases (non-contrast, arterial, portal venous, and delayed phase) were compared. The scan delay times were 40, 70, and 120 s for each contrast-enhanced phase. The visual pattern, tomographic attenuation value, and time attenuation curve were assessed and compared between AC and ACC cases using the χ2 test, Wilcoxon signed-rank test, and Mann Whitney U test.
RESULTS: The adenocarcinomas tended to be hypodense in all 4 phases. The acinar cell carcinomas also tended to be hypodense in the 3 contrast-enhanced phases, although their computed tomographic attenuation values were higher. Further, 5 of the 6 acinar cell carcinomas (83%) were isodense in the non-contrast phase. The time attenuation curve of the adenocarcinomas showed a gradual increase through the 4 phases, and all adenocarcinomas showed peak enhancement during the delayed phase. The time attenuation curve of the acinar cell carcinomas showed peak enhancement during the portal venous phase in 4 cases and during the arterial phase in 2 cases. None of the 6 acinar cell carcinomas showed peak enhancement during the delayed phase.
CONCLUSION: The tumor density in the non-contrast phase and time attenuation curve pattern clearly differ between acinar cell carcinomas and adenocarcinomas, and multidetector-row computed tomography can thus distinguish these tumors.
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Acinar cell carcinoma of the pancreas: computed tomography features--a study of 15 patients. ACTA ACUST UNITED AC 2013; 38:137-43. [PMID: 22349806 DOI: 10.1007/s00261-012-9868-4] [Citation(s) in RCA: 31] [Impact Index Per Article: 2.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/17/2022]
Abstract
OBJECTIVE Evaluation of the imaging features of pathology-proven acinar cell carcinomas (ACCs) of the pancreas using computed tomography (CT). METHODS We reviewed the CT features, clinical presentations, and clinical outcomes of 15 patients (9 men, 6 women, mean age 62.3) with pathology-proven pancreatic ACCs. An abdominal radiologist retrospectively evaluated each patient's initial imaging study with respect to the lesion's size, location, attenuation (Hounsfield units) on arterial and venous phase images, peripancreatic lymphadenopathy, and distant metastases. Additional parameters studied included biliary and pancreatic ductal dilatation, intratumoral hemorrhage, calcification, the presence of cystic/necrotic components, and whether the tumor was intraparenchymal or exophytic. RESULTS The ACCs in this series were evenly distributed between the head/uncinate and the tail, were predominantly exophytic (73%), tended to be large (average size 5.1 cm), and were mostly hypodense to the surrounding pancreas on both the arterial and venous phase images. A sizeable proportion demonstrated a cystic or necrotic component (53%) and/or an enhancing capsule (53%). Of those lesions in the head or uncinate process, very few resulted in pancreatic (28%) or biliary (14%) ductal dilatation. None of the lesions in this series showed internal calcification or intratumoral hemorrhage. CONCLUSION While a prospective diagnosis is difficult, ACCs have several features which can differentiate them from ductal adenocarcinoma, including their large size, lack of biliary or pancreatic ductal dilatation, exophytic nature, and the presence of an enhancing capsule.
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Stotz M, Eisner F, Szkandera J, Absenger G, Kornprat P, Lackner C, Samonigg H, Gerger A, Pichler M. Clinico-pathological characteristics and clinical outcome of different histological types of pancreatic cancer in a large Middle European series. J Clin Pathol 2013; 66:753-7. [PMID: 23750038 DOI: 10.1136/jclinpath-2012-201394] [Citation(s) in RCA: 14] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/07/2023]
Abstract
AIMS Pancreatic cancer (PC) is a heterogeneous disease in terms of histological and molecular subtypes. The aim of this study was to evaluate the prognostic impact of different histological subtypes on cancer-specific survival (CSS) in a large single-centre Middle European cohort. METHODS We retrospectively studied the records of 400 consecutive PC patients who were treated from 2004 to 2010 at a single tertiary academic centre. The association of histological subtypes and parameters such as tumour stage, tumour grade, levels of tumour markers carcinoembryonic antigen and CA19-9 at diagnosis, was studied. CSS was calculated using the Kaplan-Meier method, and the influence of each parameter on CSS was assessed with univariate and multivariable Cox proportional models. RESULTS The survival time was significantly shorter in the ductal adenocarcinoma and acinar histological subtypes compared to neuroendocrine differentiation (p<0.001). No survival difference was observed between ductal adenocarcinomas and patients with a histological variant of ductal adenocarcinoma, namely, mucinous non-cystic adenocarcinoma (p=0.7). In multivariable analysis, ductal adenocarcinoma (HR=3.1, CI 1.6 to 6.1, p=0.001) and acinar carcinoma (HR=3.2, CI 1.3 to 8.5, p=0.016) were identified as independent predictors for CSS. CONCLUSIONS Our findings suggest that the main histological subtype is an independent predictor of CSS in patients with PC. Thus, our data underline the importance of routine assessment of histological type in PC for individual risk assessment. However, no clinical rationale for the subdivision of ductal adenocarcinoma and mucinous non-cystic adenocarcinoma can be supported by our study.
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Affiliation(s)
- Michael Stotz
- Division of Oncology, Department of Internal Medicine, Medical University of Graz, Graz, Austria
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Toll AD, Hruban RH, Ali SZ. Acinar cell carcinoma of the pancreas: clinical and cytomorphologic characteristics. KOREAN JOURNAL OF PATHOLOGY 2013; 47:93-9. [PMID: 23667367 PMCID: PMC3647135 DOI: 10.4132/koreanjpathol.2013.47.2.93] [Citation(s) in RCA: 18] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Download PDF] [Figures] [Subscribe] [Scholar Register] [Received: 02/07/2013] [Revised: 02/28/2013] [Accepted: 03/05/2013] [Indexed: 12/14/2022]
Abstract
Acinar cell carcinoma is a rare malignant epithelial neoplasm with predominantly exocrine acinar differentiation and is seen primarily in older men (mean age, 62 years). The presenting symptoms are usually non-specific, and jaundice is often not present. Symptoms relating to the overproduction and release of lipase into the circulation are present in 10-15% of patients. Characteristic cytomorphologic features include a population of cells with minimal pleomorphism, eccentrically placed nuclei with a single prominent nucleoli and moderate hyperchromasia. The cytoplasm is finely granular, and the background may contain granular debris secondary to cytolysis. A significant proportion of the cases also have a minor neuroendocrine component or scattered neuroendocrine cells. Approximately 50% of patients have metastatic disease at presentation, often restricted to the regional lymph nodes and liver. The prognosis is poor, only slightly better than that of pancreatic ductal adenocarcinoma.
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Affiliation(s)
- Adam D Toll
- Department of Pathology, The Johns Hopkins Hospital, Baltimore, MD, USA
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Lowery MA, Klimstra DS, Shia J, Yu KH, Allen PJ, Brennan MF, O'Reilly EM. Acinar cell carcinoma of the pancreas: new genetic and treatment insights into a rare malignancy. Oncologist 2011; 16:1714-20. [PMID: 22042785 DOI: 10.1634/theoncologist.2011-0231] [Citation(s) in RCA: 90] [Impact Index Per Article: 6.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022] Open
Abstract
BACKGROUND Acinar cell carcinoma (ACC) of the pancreas is a rare neoplasm, accounting for 1% of all pancreatic neoplasms. There remains a lack of data regarding the use of systemic therapy in this disease. We present a series of 40 consecutive cases of ACC of the pancreas treated at Memorial Sloan-Kettering Cancer Center, with an emphasis on evaluation of activity of new therapeutic agents. METHODS Patients reviewed at our institution from January 2000 through January 2011 were identified from an institutional database with prior institutional review board approval. Pathology was confirmed in all cases as ACC or a closely related entity. RESULTS Forty patients were identified; 29 were male (73%). The median age at diagnosis was 65 years (range, 16-87 years). The median overall survival (OS) time for patients with localized, resectable disease was 56.9 months and the OS time for patients with metastatic ACC (n = 18) was 19.6 months. Six patients with metastatic or recurrent ACC had a partial response to chemotherapy and five patients had stable disease for ≥6 months on systemic chemotherapy. Clinical observation was made of a patient with ACC and hereditary nonpolyposis colorectal cancer and a patient with ACC and a BRCA1 germline mutation. CONCLUSIONS ACC is moderately chemoresponsive to agents that have activity in pancreatic adenocarcinoma and colorectal carcinoma. A potential association between germline mutations in DNA mismatch repair genes and ACC warrants further evaluation.
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Affiliation(s)
- Maeve A Lowery
- Gastrointestinal Oncology Service, Memorial Sloan-Kettering Cancer Center, New York, New York 10065, USA
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