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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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Oda N, Tabata M, Uno M, Umeda Y, Kato H, Kubo T, Senoo S, Yagi T, Fujiwara T, Maeda Y, Kiura K. Dramatic Response to Carboplatin Plus Paclitaxel in Pancreatic Mucinous Cystadenocarcinoma with Liver Metastasis. Intern Med 2021; 60:2967-2971. [PMID: 33814494 PMCID: PMC8502668 DOI: 10.2169/internalmedicine.6730-20] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/06/2022] Open
Abstract
Mucinous cystic neoplasm (MCN) of the pancreas is a rare cystic tumor occurring in the pancreatic body and tail in young to middle-aged women that is pathologically characterized by an ovarian-like stroma. Chemotherapy for recurrent/advanced pancreatic MCN has been based on chemotherapy regimens for pancreatic ductal adenocarcinoma, but the prognosis is poor. We herein report a 37-year-old woman with pancreatic mucinous cystadenocarcinoma with liver metastasis that responded dramatically to carboplatin plus paclitaxel therapy (CBDCA+PTX). CBDCA+PTX may be a treatment option for recurrent/advanced pancreatic MCN with an ovarian-like stroma.
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Affiliation(s)
- Naohiro Oda
- Department of Allergy and Respiratory Medicine, Okayama University Hospital, Japan
- Department of Internal Medicine, Fukuyama City Hospital, Japan
| | - Masahiro Tabata
- Center for Clinical Oncology, Okayama University Hospital, Japan
| | - Masatoshi Uno
- Department of Internal Medicine, Kaneda Hospital, Japan
| | - Yuzo Umeda
- Department of Hepato-Biliary-Pancreatic Surgery, Okayama University Hospital, Japan
| | - Hironari Kato
- Department of Gastroenterology and Hepatology, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Japan
| | - Toshio Kubo
- Center for Clinical Oncology, Okayama University Hospital, Japan
| | - Satoru Senoo
- Department of Allergy and Respiratory Medicine, Okayama University Hospital, Japan
| | - Takahito Yagi
- Department of Hepato-Biliary-Pancreatic Surgery, Okayama University Hospital, Japan
| | - Toshiyoshi Fujiwara
- Department of Gastroenterological Surgery, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Japan
| | - Yoshinobu Maeda
- Department of Hematology, Oncology and Respiratory Medicine, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Japan
| | - Katsuyuki Kiura
- Department of Allergy and Respiratory Medicine, Okayama University Hospital, Japan
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Ovarian Metastasis from Pancreatic Ductal Adenocarcinoma. World J Surg 2021; 45:3157-3164. [PMID: 34236477 DOI: 10.1007/s00268-021-06209-x] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 06/10/2021] [Indexed: 10/20/2022]
Abstract
BACKGROUND Pancreatic ductal adenocarcinoma (PDAC) has a high propensity for systemic dissemination. Ovarian metastases are rare and poorly described. METHODS We identified PDAC cases with ovarian metastasis from a prospectively maintained registry. We reported on the association between outcomes and clinicopathologic factors. Recurrence-free (RFS) and overall survival (OS) were calculated using Kaplan-Meier analysis. RESULTS Twelve patients with PDAC and synchronous or metachronous ovarian metastases were identified. Nine patients (75%) underwent pancreatectomy for localized PDAC and developed metachronous ovarian recurrence. The median OS for all patients was 25.4 (IQR:15.4-82.9) months. For the nine patients with metachronous ovarian metastasis, the median RFS and OS were 14.2 (IQR:7.2-58.3) and 44.6 (IQR:18.6-82.9) months, respectively. Nodal disease, poor grade, vascular invasion in the pancreatic primary, and bilateral ovarian disease tended to confer worse outcomes. CONCLUSION Patients with resected PDAC and ovarian recurrence tend to have a comparable disease course to more common patterns of recurrence. Primaries with nodal disease, poorer grade, vascular invasion, and bilateral ovarian disease were indicative of more aggressive disease biology. The ideal management remains largely unknown, and future collaborative efforts should optimize therapeutic strategies.
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Wang SD, Dai MH, Zhao YP. Primary pancreatic cystadenocarcinoma with ovarian metastases in a 38-year-old female: case report. Transl Cancer Res 2020; 9:7652-7656. [PMID: 35117364 PMCID: PMC8798069 DOI: 10.21037/tcr-20-2361] [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: 06/19/2020] [Accepted: 10/28/2020] [Indexed: 11/06/2022]
Abstract
Pancreatic cystadenocarcinoma with ovarian metastases is rare and easily misdiagnosed as primary ovarian cancers. Here we report 38-year-old female manifested tumors in pancreas and ovary concurrently, which was difficult to distinguish the primary site. She was admitted to hospital because of abdominal distension and a palpable mass in the lower abdomen. Abdominal ultrasound showed a lesion in pancreas and two masses in bilateral ovaries. Computed tomography (CT) revealed the hypo-enhancing pancreatic mass and the large pelvic lesion simultaneously. The largest cross-sectional of the right mass was 12×15.1 cm and 15.4×18.3 cm for the left side, probably malignant lesions. In addition, the level of the serum CA19-9 and CA125 were higher than the normal level. Positron emission tomography CT (PET-CT) revealed there might be the cystadenocarcinoma in the pancreatic tail with multiple metastatic lesions implanted in the pelvic. After comprehensive examination, she received bilateral salpingo-oophorectomy and biopsy of the pancreatic tumor. The pathological finding revealed that it was pancreatic cystadenocarcinoma with ovarian metastases. Postoperatively, she received the chemotherapy and the follow-up continued for 26 months until she died. This case reminded doctors that pancreatic primaries should be paid attention when dealing with metastatic ovarian malignancies although it was rare. Choosing effective diagnostic method and timely surgical intervention are essential to improve prognosis.
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Affiliation(s)
- Shun-Da Wang
- Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Meng-Hua Dai
- Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
| | - Yu-Pei Zhao
- Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China
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Wang SD, Zhu L, Wu HW, Dai MH, Zhao YP. Pancreatic cancer with ovarian metastases: A case report and review of the literature. World J Clin Cases 2020; 8:5380-5388. [PMID: 33269273 PMCID: PMC7674732 DOI: 10.12998/wjcc.v8.i21.5380] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/14/2020] [Revised: 08/30/2020] [Accepted: 09/17/2020] [Indexed: 02/06/2023] Open
Abstract
BACKGROUND Pancreatic cancer with ovarian metastases is rare and easily misdiagnosed. Most patients are first diagnosed with ovarian cancer. We report a rare case of ovarian metastases secondary to pancreatic adenocarcinoma. We also review the literature to analyze the clinical characteristics of, diagnostic methods for, and perioperative management strategies for this rare malignancy.
CASE SUMMARY A 48-year-old woman with an abdominal mass presented to our hospital. Computed tomography revealed lesions in the pancreas and lower abdomen. Radiological examination and histological investigation of biopsy specimens revealed either an ovarian metastasis from a pancreatic neoplasm or two primary tumors, with metastasis strongly suspected. The patient simultaneously underwent distal pancreatectomy plus splenectomy by a general surgeon and salpingo-oophorectomy with hysterectomy by a gynecologist. Histological examination of the surgical specimen revealed a pancreatic adenocarcinoma (intermediate differentiation, mucinous) and a metastatic mucinous adenocarci-noma in the ovary.
CONCLUSION For this rare tumor, surgical resection is the most effective treatment, and the final diagnosis depends on tumor pathology.
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Affiliation(s)
- Shun-Da Wang
- Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China
| | - Liang Zhu
- Department of Radiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China
| | - Huan-Wen Wu
- Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China
| | - Meng-Hua Dai
- Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China
| | - Yu-Pei Zhao
- Department of General Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing 100730, China
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Pancreaticobiliary metastasis presenting as primary mucinous ovarian neoplasm: A systematic literature review. Gynecol Oncol Rep 2019; 28:109-115. [PMID: 30997376 PMCID: PMC6453658 DOI: 10.1016/j.gore.2019.03.012] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/07/2019] [Revised: 03/19/2019] [Accepted: 03/20/2019] [Indexed: 01/13/2023] Open
Abstract
True primary mucinous ovarian carcinomas are rarer than originally thought and their clinical behavior and treatment response are different than more common epithelial ovarian carcinomas. Secondary ovarian neoplasms often mimic the clinical and histological features of mucinous ovarian cancer making their diagnosis, and therefore treatment, more difficult. Misdiagnosis can have a significant impact on both treatment and prognosis. The majority of these secondary ovarian neoplasms arise from the gastrointestinal tract, with mucinous histology often of pancreaticobiliary origin. Our study objective was to review current evidence distinguishing pancreaticobiliary ovarian metastasis from primary mucinous ovarian carcinoma. We utilized a PubMed search using MeSH terms and selected articles were reviewed, synthesized and summarized. Thirty-nine articles were included in the review. The clinical, gross, histological and immunohistochemical features distinguishing primary mucinous ovarian carcinomas from pancreaticobiliary ovarian metastasis were identified. Compared to primary mucinous ovarian carcinoma, metastatic pancreaticobiliary tumors are more often bilateral, <10 cm, have irregular external surface and surface implants, display an infiltrative pattern of invasion and stain for MUC1 and CK17. Primary ovarian mucinous tumors rarely (<3%) have signet ring cells or involvement of the hilum. Metastatic mucinous tumors mimic their primary mucinous ovarian counterparts and their clinical and histopathological features overlap in many ways. However, these metastatic tumors have features that can help differentiate them from primary mucinous carcinoma. With a high index of suspicion and knowledge of the reviewed features, distinguishing these tumors will continue to become easier.
Primary ovarian and metastatic pancreaticobiliary tumors present similarly. Histologic findings can differentiate primary from metastatic ovarian tumors. Bilaterality, size, surface appearance can differentiate primary versus metastatic.
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Brunetti O, Aprile G, Marchetti P, Vasile E, Casadei Gardini A, Scartozzi M, Barni S, Delfanti S, De Vita F, Di Costanzo F, Milella M, Cella CA, Berardi R, Cataldo I, Scarpa A, Basile D, Mazzuca F, Graziano G, Argentiero A, Santini D, Reni M, Cascinu S, Silvestris N. Systemic Chemotherapy for Advanced Rare Pancreatic Histotype Tumors: A Retrospective Multicenter Analysis. Pancreas 2018; 47:759-771. [PMID: 29771769 DOI: 10.1097/mpa.0000000000001063] [Citation(s) in RCA: 22] [Impact Index Per Article: 3.1] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
Abstract
OBJECTIVES Two issues were put forth by clinicians in the management of the advanced stages of rare variants of pancreatic ductal adenocarcinoma and other exocrine histotypes with peculiar clinical and pathological features: Do chemotherapy regimens recommended in pancreatic ductal adenocarcinoma patients have a clinical activity in rare pancreatic tumors? Or should other chemotherapy combinations be considered in this subset of patients? METHODS We conducted a multicenter retrospective study that collected data from 2005 to 2016 at 14 Italian cancer centers with the aim to evaluate tumor response and time to progression for first- and second-line and overall survival. RESULTS Of approximately 4300 exocrine pancreatic cancer patients, 79 advanced cases affected by rare histological types were identified, with pancreatic acinar cell cancer (n = 23), pancreatic adenosquamous cancer (n = 16), and mucinous cystic neoplasm with an associated invasive mucinous cystadenocarcinoma (n = 15) most represented. Survival analyses for each subgroup in relation with the different chemotherapy regimens showed the lack of statistical significance correlations. CONCLUSIONS Because of the lack of clinical trials in patients affected by these rare pancreatic histotypes, only their molecular classification would help clinicians in future therapeutic choice.
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MESH Headings
- Adenocarcinoma, Mucinous/drug therapy
- Adenocarcinoma, Mucinous/pathology
- Aged
- Antineoplastic Combined Chemotherapy Protocols/therapeutic use
- Carcinoma, Acinar Cell/drug therapy
- Carcinoma, Acinar Cell/pathology
- Carcinoma, Adenosquamous/drug therapy
- Carcinoma, Adenosquamous/pathology
- Carcinoma, Pancreatic Ductal/drug therapy
- Carcinoma, Pancreatic Ductal/pathology
- Cystadenocarcinoma, Mucinous/drug therapy
- Cystadenocarcinoma, Mucinous/pathology
- Humans
- Kaplan-Meier Estimate
- Middle Aged
- Pancreatic Neoplasms/drug therapy
- Pancreatic Neoplasms/pathology
- Prognosis
- Retrospective Studies
- Treatment Outcome
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Affiliation(s)
| | | | - Paolo Marchetti
- Medical Oncology Unit, Sant'Andrea Hospital, University of Rome La Sapienza, Rome
| | - Enrico Vasile
- Medical Oncology Unit, Azienda Ospedaliero-Universitaria Pisana, Pisa
| | - Andrea Casadei Gardini
- Department of MedicalOncology, Istituto Scientifico Romagnolo per lo Studio e Cura dei Tumori (IRST) IRCCS, Meldola
| | | | - Sandro Barni
- Medical Oncology Unit, ASST Bergamo Ovest, Treviglio
| | - Sara Delfanti
- Medical Oncology Unit, Fondazione IRCCS Policlinico San Matteo, Pavia
| | | | | | - Michele Milella
- Medical Oncology 1, IRCCS Regina Elena National Cancer Institute, Rome
| | | | - Rossana Berardi
- Medical Oncology Unit, Università Politecnica Marche - Ospedali Riuniti Ancona, Ancona
| | - Ivana Cataldo
- Department of Pathology and Diagnostics, University of Verona, ARCNET, Verona
| | - Aldo Scarpa
- Department of Pathology and Diagnostics, University of Verona, ARCNET, Verona
| | - Debora Basile
- Department of Medical Oncology, University and General Hospital, Udine
| | - Federica Mazzuca
- Medical Oncology Unit, Sant'Andrea Hospital, University of Rome La Sapienza, Rome
| | - Giusi Graziano
- Scientific Direction, Cancer Institute "Giovanni Paolo II," Bari
| | | | | | - Michele Reni
- Department of Medical Oncology, IRCCS San Raffaele Scientific Institute, Milan
| | - Stefano Cascinu
- Modena Cancer Center, University of Modena and Reggio Emilia, Azienda Ospedaliera-Universitaria di Modena, Modena, Italy
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Abstract
Evidence-based guidelines on the management of pancreatic cystic neoplasms (PCN) are lacking. This guideline is a joint initiative of the European Study Group on Cystic Tumours of the Pancreas, United European Gastroenterology, European Pancreatic Club, European-African Hepato-Pancreato-Biliary Association, European Digestive Surgery, and the European Society of Gastrointestinal Endoscopy. It replaces the 2013 European consensus statement guidelines on PCN. European and non-European experts performed systematic reviews and used GRADE methodology to answer relevant clinical questions on nine topics (biomarkers, radiology, endoscopy, intraductal papillary mucinous neoplasm (IPMN), mucinous cystic neoplasm (MCN), serous cystic neoplasm, rare cysts, (neo)adjuvant treatment, and pathology). Recommendations include conservative management, relative and absolute indications for surgery. A conservative approach is recommended for asymptomatic MCN and IPMN measuring <40 mm without an enhancing nodule. Relative indications for surgery in IPMN include a main pancreatic duct (MPD) diameter between 5 and 9.9 mm or a cyst diameter ≥40 mm. Absolute indications for surgery in IPMN, due to the high-risk of malignant transformation, include jaundice, an enhancing mural nodule >5 mm, and MPD diameter >10 mm. Lifelong follow-up of IPMN is recommended in patients who are fit for surgery. The European evidence-based guidelines on PCN aim to improve the diagnosis and management of PCN.
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Stewart BA, Adjei A, Zhang X, Oxenberg JC. Metastatic adenocarcinoma of the pancreas presents as metastases to the axillary/supraclavicular region as the first sign of the disease. Ann Hepatobiliary Pancreat Surg 2017; 21:247-251. [PMID: 29264591 PMCID: PMC5736748 DOI: 10.14701/ahbps.2017.21.4.247] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/26/2017] [Revised: 08/13/2017] [Accepted: 08/13/2017] [Indexed: 02/07/2023] Open
Abstract
Mucinous tumors of the pancreas are rare and the diagnosis of invasive carcinoma can be a dilemma. While metastatic disease from mucinous cystadenocarcinoma (MCAC) and invasive intraductal papillary mucinous neoplasms (IPMN) have been reported, no extraperitoneal mucinous cystic metastatic disease has been described. When metastatic, the overall survival rates for invasive adenocarcinoma, mucinous cystadenocarcinoma (MCAC) and invasive intraductal papillary mucinous neoplasms (IPMN) are similar. The best improvement in the overall and progression free survival has been demonstrated with FOLFIRINOX (folinic acid – fluorouracil – irinotecan – oxaliplatin) for metastatic adenocarcinoma and Gemcitabine based regimens for MCAC. However, the variable responses of metastatic mucinous lesions have been observed and the overall prognosis remains poor. We describe a case of a patient who presented with metastatic adenocarcinoma of the pancreas as cystic masses in the supraclavicular and axillary regions. Additionally, this patient was initially treated with FOLFIRINOX and continues to have stable primary and metastatic disease after 18 months from the diagnosis.
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Affiliation(s)
- Blake A Stewart
- Department of General Surgery, Geisinger Wyoming Valley Medical Center, Wilkes-Barre, PA, USA
| | - Abdul Adjei
- Department of Hematology/Oncology, Geisinger Wyoming Valley Medical Center, Wilkes-Barre, PA, USA
| | - Xiaohong Zhang
- Department of Pathology, Geisinger Wyoming Valley Medical Center, Wilkes-Barre, PA, USA
| | - Jacqueline C Oxenberg
- Department of Surgical Oncology, Geisinger Wyoming Valley Medical Center, Wilkes-Barre, PA, USA
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