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1
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Papke DJ. Mesenchymal Neoplasms of the Liver. Surg Pathol Clin 2023; 16:609-634. [PMID: 37536892 DOI: 10.1016/j.path.2023.04.013] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 08/05/2023]
Abstract
Mesenchymal neoplasms of the liver can be diagnostically challenging, particularly on core needle biopsies. Here, I discuss recent updates in neoplasms that are specific to the liver (mesenchymal hamartoma, undifferentiated embryonal sarcoma, calcifying nested stromal-epithelial tumor), vascular tumors of the liver (anastomosing hemangioma, hepatic small vessel neoplasm, epithelioid hemangioendothelioma, angiosarcoma), and other tumor types that can occur primarily in the liver (PEComa/angiomyolipoma, inflammatory pseudotumor-like follicular dendritic cell sarcoma, EBV-associated smooth muscle tumor, inflammatory myofibroblastic tumor, malignant rhabdoid tumor). Lastly, I discuss metastatic sarcomas to the liver, as well as pitfalls presented by metastatic melanoma and sarcomatoid carcinoma.
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Affiliation(s)
- David J Papke
- Department of Pathology, Brigham and Women's Hospital, Harvard Medical School, 75 Francis Street, Boston, MA 02115, USA.
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2
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Kruk E, Kobryń K, Rykowski P, Szczepankiewicz B, Patkowski W, Zieniewicz K. Liver Transplantation for Unresectable Calcifying Nested Stromal Epithelial Tumor: Case Report With a 1-Year Follow-Up and Review of Literature. Front Surg 2022; 9:875782. [PMID: 35586511 PMCID: PMC9108170 DOI: 10.3389/fsurg.2022.875782] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Grants] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/14/2022] [Accepted: 03/29/2022] [Indexed: 11/13/2022] Open
Abstract
Introduction Calcifying nested stromal epithelial tumor (CNSET) is an extremely rare diagnosis among patients treated for primary hepatic neoplasms. There are only 45 cases reported worldwide. Histopathological characteristics are well-demarcated nests of spindle and epithelioid cells in a dense desmoplastic stroma with variable calcification and ossification. It is mostly diagnosed in children and young females. Treatment strategies implemented for the management of CNSET include radiofrequency ablation, transarterial chemoembolization, surgical resection, adjuvant and neoadjuvant chemotherapy, and liver transplantation. Given the small number of available cases, there are still no established standards of treatment for this neoplasm. Case Presentation A 28-year-old female diagnosed with CNSET presented mild abdominal pain, with normal laboratory values. The tumor was initially deemed unresectable, therefore, the patient was disqualified from liver resection. Further deterioration of the patient's clinical condition and local tumor progression led to qualification for liver transplantation. The patient underwent liver transplantation 1 year following initial diagnosis and a 12 months recurrence-free period was observed. During the course of treatment, she did not receive systemic chemotherapy, radiotherapy, or loco-regional treatment. Conclusion Multiple strategies have been implemented for the treatment of CNSET, with liver resection providing the best outcomes. Transarterial chemoembolization, radiofrequency ablation, and radiotherapy are reported to be insufficient in the management of this tumor. Various chemotherapy regimens turned out to be ineffective as well. There have been only eight reported cases of patients undergoing liver transplantation for CNSET, with tumor recurrence in two cases. CNSET appears to be a neoplasm with low malignancy potential, although an aggressive progression has subsequently been reported. Further investigation is still required in this field.
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Affiliation(s)
- Emilia Kruk
- Department of General, Transplant and Liver Surgery, Medical University of Warsaw, Warsaw, Poland
| | - Konrad Kobryń
- Department of General, Transplant and Liver Surgery, Medical University of Warsaw, Warsaw, Poland
- *Correspondence: Konrad Kobryń
| | - Paweł Rykowski
- Department of General, Transplant and Liver Surgery, Medical University of Warsaw, Warsaw, Poland
| | | | - Waldemar Patkowski
- Department of General, Transplant and Liver Surgery, Medical University of Warsaw, Warsaw, Poland
| | - Krzysztof Zieniewicz
- Department of General, Transplant and Liver Surgery, Medical University of Warsaw, Warsaw, Poland
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3
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Papke DJ, Dong F, Zhang X, Kozielski R, Basturk O, Fletcher CDM, Zhao L. Calcifying nested stromal-epithelial tumor: a clinicopathologic and molecular genetic study of eight cases highlighting metastatic potential and recurrent CTNNB1 and TERT promoter alterations. Mod Pathol 2021; 34:1696-1703. [PMID: 33994539 DOI: 10.1038/s41379-021-00822-w] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/09/2021] [Revised: 04/09/2021] [Accepted: 04/10/2021] [Indexed: 02/08/2023]
Abstract
Calcifying nested stromal-epithelial tumor (CNSET) is a rare hepatic tumor that occurs in children and young adults. With <40 cases in the literature, the mechanism for tumorigenesis and the biological behavior of CNSET remain uncertain. Here, we studied the clinicopathologic and molecular genetic features of eight CNSETs. Six patients (75%) were female, and the median age at presentation was 22.5 years (range 14-34 years). The median tumor size was 14 cm (range 2.7-18 cm). All tumors had fibrous stroma that contained organoid nests of epithelioid to spindled tumor cells with moderate amounts of palely eosinophilic cytoplasm and ovoid, vesicular nuclei. Five tumors showed calcifications, and one showed lymphovascular invasion. Necrosis was absent in all. Immunohistochemistry demonstrated nuclear β-catenin expression in five of five tested tumors and focal to diffuse nuclear WT-1 positivity in five of seven. Hepatocellular markers (HepPar-1, arginase-1, and albumin in situ hybridization) and neuroendocrine markers (synaptophysin, chromogranin, and INSM1) were uniformly negative. Next-generation sequencing demonstrated CTNNB1 alterations in all seven sequenced tumors. Sanger sequencing demonstrated TERT promoter mutations in all six sequenced tumors. Clinical follow-up was available for seven patients (median duration 4.4 years; range 1.2-6.2 years): four (57%) developed metastatic disease; all four developed lung metastases; and two also had abdominal metastases. All four patients with metastatic disease also had persistent or recurrent liver tumors. Three patients with metastases were alive with disease at the most recent follow-up and one died of disease. The other three patients with available follow-up did not develop metastasis or recurrence. One tumor treated with neoadjuvant chemotherapy showed no response, and another showed 90% tumor fibrosis; the latter patient remained disease-free at 6.2 years of follow-up. Our series demonstrates the presence of TERT promoter mutations and CTNNB1 alterations in all sequenced tumors and suggests that CNSET might perhaps be more aggressive than previously reported.
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Affiliation(s)
- David J Papke
- Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA
| | - Fei Dong
- Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA
| | - Xuchen Zhang
- Department of Pathology, Yale School of Medicine, New Haven, CT, USA
| | - Rafal Kozielski
- Department of Pathology and Jacobs School of Medicine and Biomedical Sciences, University at Buffalo, Buffalo, NY, USA
| | - Olca Basturk
- Department of Pathology, Memorial Sloan Kettering Cancer Center, New York City, NY, USA
| | - Christopher D M Fletcher
- Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA
| | - Lei Zhao
- Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, USA.
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4
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Runyan C, Patel M, Youssfi M, Taylor S, Biyyam D. Calcifying nested stromal-epithelial tumor of the liver: Case report of a rare primary liver tumor. Radiol Case Rep 2021; 16:2337-2342. [PMID: 34306277 PMCID: PMC8258647 DOI: 10.1016/j.radcr.2021.04.087] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/27/2021] [Revised: 04/30/2021] [Accepted: 04/30/2021] [Indexed: 12/21/2022] Open
Abstract
Calcifying nested stromal epithelial tumor is a very rare primary liver tumor in children. To our knowledge, few cases have been reported in literature. We describe the imaging appearance and histopathologic features of this tumor incidentally detected in a 2-year-old girl. This tumor should be considered in the differential when a large heterogeneous liver tumor with central scar and coarse/chunky calcifications is identified at imaging in the absence of elevated alpha-fetoprotein in a child.
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Affiliation(s)
- Charles Runyan
- Department of Radiology, Phoenix Children's Hospital, 1919 E. Thomas Rd., Phoenix, AZ 85016, USA
| | - Mittun Patel
- Department of Radiology, Phoenix Children's Hospital, 1919 E. Thomas Rd., Phoenix, AZ 85016, USA
| | - Mostafa Youssfi
- Department of Radiology, Phoenix Children's Hospital, 1919 E. Thomas Rd., Phoenix, AZ 85016, USA
| | - Steve Taylor
- Department of Radiology, Phoenix Children's Hospital, 1919 E. Thomas Rd., Phoenix, AZ 85016, USA.,Department of Pathology, Phoenix Children's Hospital, 1919 E. Thomas Rd., Phoenix, AZ 85016, USA
| | - Deepa Biyyam
- Department of Radiology, Phoenix Children's Hospital, 1919 E. Thomas Rd., Phoenix, AZ 85016, USA
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5
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Babu MCS, Sreevalli A, Hassan SA, Champaka G, Lokanatha D, Jacob LA, Lokesh KN, Rudresha AH, Rajeev LK, Smitha S. Nested Stromal and Epithelial Tumor of the Liver: An Unusual Nonhepatocytic Entity. Indian J Med Paediatr Oncol 2020. [DOI: 10.4103/ijmpo.ijmpo_58_20] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/04/2022] Open
Affiliation(s)
- MC Suresh Babu
- Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
| | - A Sreevalli
- Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
| | - Syed Adil Hassan
- Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
| | - G Champaka
- Department of Pathology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
| | - D Lokanatha
- Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
| | - Linu Abraham Jacob
- Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
| | - KN Lokesh
- Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
| | - AH Rudresha
- Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
| | - LK Rajeev
- Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
| | - Saldanha Smitha
- Department of Medical Oncology, Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India
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6
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Geramizadeh B. Nested Stromal-Epithelial Tumor of the Liver: A Review. Gastrointest Tumors 2019; 6:1-10. [PMID: 31602372 PMCID: PMC6738243 DOI: 10.1159/000496339] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/09/2018] [Revised: 12/14/2018] [Indexed: 12/26/2022] Open
Abstract
BACKGROUND Nested stromal-epithelial tumor (NSET) is a rare liver tumor, which is most commonly seen in the pediatric age group. To the best of our knowledge, there has been no published review on this rare tumor in the English literature so far. SUMMARY In this review, we will discuss all the reported details of the published cases, including demography, clinical presentation, molecular histogenesis, imaging, gross pathology and histopathology, immunohistochemical findings, treatment modalities, and outcome of NSET of the liver. KEY MESSAGE Thirty-eight cases of NSET have been reported in the last 20 years in the English literature. This tumor produces a very large and calcified mass in the liver and characteristically can present as Cushing syndrome. NSET is a nonbiliary and nonhepatocytic tumor with biphasic differentiation into 2 components of epithelial and stromal cells. The epithelial cells are arranged as nests of mildly atypical epithelial cells with a few mitotic figures. The stromal component is composed of myofibroblasts and desmoplastic stroma which often shows ossification and calcification. Immunohistochemically, this tumor is positive for both epithelial and mesenchymal markers. The majority of the reported cases in the literature had benign behavior with an indolent course.
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Affiliation(s)
- Bita Geramizadeh
- *Bita Geramizadeh, Department of Pathology, Shiraz University of Medical Sciences, Shiraz 7194818476 (Iran), E-Mail
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7
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Benedict M, Zhang X. Calcifying Nested Stromal-Epithelial Tumor of the Liver: An Update and Literature Review. Arch Pathol Lab Med 2019; 143:264-268. [PMID: 30354275 DOI: 10.5858/arpa.2017-0346-rs] [Citation(s) in RCA: 12] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/05/2023]
Abstract
Calcifying nested stromal-epithelial tumor is a rare entity that has gone by a variety of names in the literature: ossifying malignant mixed epithelial and stromal tumor, ossifying stromal-epithelial tumor, and desmoplastic nested spindle cell tumor of the liver. To our knowledge, approximately 38 cases have been reported in the literature. The histogenesis is still largely unknown but histopathologically is characterized by nests of spindle and epithelioid cells in an organoid arrangement surrounded by a prominent dense myofibroblastic stroma with occasional psammomatous calcification and focal heterotopic ossification. Vascular invasion is rare and tumoral recurrence is uncommon with only a single reported case of metastasis leading to death. Treatment is mainly by surgical intervention with the role of chemotherapy seeming limited, but lack of data hinders a true recommendation. It is important to rule out other processes such as hepatoblastoma, calcified hemangioma, synovial sarcoma, metastatic gastrointestinal stromal tumor, desmoplastic small round cell tumor, among others, which appear similar radiographically and histopathologically.
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Affiliation(s)
- Mark Benedict
- From the Department of Pathology, Yale University School of Medicine, New Haven, Connecticut
| | - Xuchen Zhang
- From the Department of Pathology, Yale University School of Medicine, New Haven, Connecticut
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8
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Tsuruta S, Kimura N, Ishido K, Kudo D, Sato K, Endo T, Yoshizawa T, Sukeda A, Hiraoka N, Kijima H, Hakamada K. Calcifying nested stromal epithelial tumor of the liver in a patient with Klinefelter syndrome: a case report and review of the literature. World J Surg Oncol 2018; 16:227. [PMID: 30453967 PMCID: PMC6245822 DOI: 10.1186/s12957-018-1528-x] [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: 06/07/2018] [Accepted: 11/12/2018] [Indexed: 01/30/2023] Open
Abstract
BACKGROUND Calcifying nested stromal epithelial tumor (CNSET) is a primary neoplasm of the liver, characterized by well-demarcated nests consisting of spindle and epithelioid cells with calcification and bone formation. An association of Cushing syndrome with CNSET has drawn attention, but the origin of CNSET has not been clarified. CASE PRESENTATION We report here the case of a 20-year-old male with Klinefelter syndrome who underwent liver resection for an increasing liver tumor that was pathologically diagnosed with CNSET. He was postoperatively followed up and received several examinations, and recurrences and extrahepatic lymph node metastases were detected on the 64th day after surgery. Chemoembolization and chemotherapy were not effective, leading to tumor progression with development of progressive liver failure, and the patient finally died 164 days after hepatectomy. CONCLUSIONS This case suggests that an imbalance of hormones affects the genesis and progression of CNSET, and indicates the importance of closely following patients with CNSET by imaging with attention to hepatic recurrence and extrahepatic metastases.
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Affiliation(s)
- Satoru Tsuruta
- Department of Gastroenterological Surgery, Hirosaki University Graduate School of Medicine, 5, Zaifu, Hirosaki, Aomori, 036-8562, Japan
| | - Norihisa Kimura
- Department of Gastroenterological Surgery, Hirosaki University Graduate School of Medicine, 5, Zaifu, Hirosaki, Aomori, 036-8562, Japan.
| | - Keinosuke Ishido
- Department of Gastroenterological Surgery, Hirosaki University Graduate School of Medicine, 5, Zaifu, Hirosaki, Aomori, 036-8562, Japan
| | - Daisuke Kudo
- Department of Gastroenterological Surgery, Hirosaki University Graduate School of Medicine, 5, Zaifu, Hirosaki, Aomori, 036-8562, Japan
| | - Kentaro Sato
- Department of Gastroenterological Surgery, Hirosaki University Graduate School of Medicine, 5, Zaifu, Hirosaki, Aomori, 036-8562, Japan
| | - Tetsu Endo
- Department of Gastroenterology and Hematology, Hirosaki University Graduate School of Medicine, 5, Zaifu, Hirosaki, Aomori, 036-8562, Japan
| | - Tadashi Yoshizawa
- Department of Pathology and Bioscience, Hirosaki University Graduate School of Medicine, 5, Zaifu, Hirosaki, Aomori, 036-8562, Japan
| | - Aoi Sukeda
- Division of Pathology and Clinical Laboratories, National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan
| | - Nobuyoshi Hiraoka
- Division of Pathology and Clinical Laboratories, National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan
| | - Hiroshi Kijima
- Department of Pathology and Bioscience, Hirosaki University Graduate School of Medicine, 5, Zaifu, Hirosaki, Aomori, 036-8562, Japan
| | - Kenichi Hakamada
- Department of Gastroenterological Surgery, Hirosaki University Graduate School of Medicine, 5, Zaifu, Hirosaki, Aomori, 036-8562, Japan
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9
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Meletani T, Cantini L, Lanese A, Nicolini D, Cimadamore A, Agostini A, Ricci G, Antognoli S, Mandolesi A, Guido M, Alaggio R, Giuseppetti GM, Scarpelli M, Vivarelli M, Berardi R. Are liver nested stromal epithelial tumors always low aggressive? World J Gastroenterol 2017; 23:8248-8255. [PMID: 29290661 PMCID: PMC5739931 DOI: 10.3748/wjg.v23.i46.8248] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 06/20/2017] [Revised: 07/27/2017] [Accepted: 08/25/2017] [Indexed: 02/06/2023] Open
Abstract
Nested stromal-epithelial tumor (NSET) is a non-hepatocytic and non-biliary tumor of the liver consisting of nests of epithelial and spindled cells with associated myofibroblastic stroma and variable intra-lesional calcification and ossification, which represents a very rare and challenging disease. Most of the reported cases have been treated with surgery, obtaining a long survival outcome. Here, we report the case of a 31-year-old Caucasian man who underwent surgery at our institution for a large, lobulated, multinodular mass of the right hemi-liver. The histological exam confirmed the diagnosis of NSET. After 6 mo from surgery, a liver recurrence was described and a chemo-embolization was performed. After a further disease progression, based on the correlation between the histological features of the disease and those of the hepatoblastoma, a similar chemotherapy regimen (with cisplatin and ifosfamide/mesna chemotherapy, omitting doxorubicin due to liver impairment) was administered. However, infection of the biliary catheter required a dose modification of the treatment. No benefit was noted and a progression of disease was radiologically assessed after only four cycles. The worsening of the clinical status prevented further treatments, and the patient died a few months later. This case report documents how the NSET might have an aggressive and non-preventable behavior. No chemotherapy schedules with a proved efficacy are available, and new data are needed to shed light on this rare neoplasm.
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MESH Headings
- Adult
- Antineoplastic Combined Chemotherapy Protocols/therapeutic use
- Diagnosis, Differential
- Fatal Outcome
- Hepatectomy
- Hepatoblastoma/diagnosis
- Hepatoblastoma/pathology
- Humans
- Liver/diagnostic imaging
- Liver/pathology
- Liver/surgery
- Liver Neoplasms/diagnosis
- Liver Neoplasms/pathology
- Liver Neoplasms/therapy
- Male
- Neoplasm Recurrence, Local/diagnosis
- Neoplasm Recurrence, Local/pathology
- Neoplasm Recurrence, Local/therapy
- Neoplasms, Complex and Mixed/diagnosis
- Neoplasms, Complex and Mixed/pathology
- Neoplasms, Complex and Mixed/therapy
- Neoplasms, Glandular and Epithelial/diagnosis
- Neoplasms, Glandular and Epithelial/pathology
- Neoplasms, Glandular and Epithelial/therapy
- Positron-Emission Tomography
- Tomography, X-Ray Computed
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Affiliation(s)
- Tania Meletani
- Medical Oncology, University Hospital and Polytechnic University Ancona, 60126 Marche, Italy
| | - Luca Cantini
- Medical Oncology, University Hospital and Polytechnic University Ancona, 60126 Marche, Italy
| | - Andrea Lanese
- Medical Oncology, University Hospital and Polytechnic University Ancona, 60126 Marche, Italy
| | - Daniele Nicolini
- Hepatobiliary and Transplantation Surgery, Department of Clinical and Experimental Medicine, University Hospital and Polytechnic University Ancona, 60126 Marche, Italy
| | - Alessia Cimadamore
- Section of Pathological Anatomy, Department of Biomedical Sciences and Public Health, University Hospital and Polytechnic University Ancona, 60126 Marche, Italy
| | - Andrea Agostini
- Department of Radiology, University Hospital and Polytechnic University Ancona, 60126 Marche, Italy
| | - Giulia Ricci
- Medical Oncology, University Hospital and Polytechnic University Ancona, 60126 Marche, Italy
| | - Stefania Antognoli
- Medical Oncology, University Hospital and Polytechnic University Ancona, 60126 Marche, Italy
| | - Alessandra Mandolesi
- Section of Pathological Anatomy, Department of Biomedical Sciences and Public Health, University Hospital and Polytechnic University Ancona, 60126 Marche, Italy
| | - Maria Guido
- Pathology Department, Padova University, 35121 Padova, Italy
| | - Rita Alaggio
- Pathology Department, Padova University, 35121 Padova, Italy
| | - Gian Marco Giuseppetti
- Department of Radiology, University Hospital and Polytechnic University Ancona, 60126 Marche, Italy
| | - Marina Scarpelli
- Section of Pathological Anatomy, Department of Biomedical Sciences and Public Health, University Hospital and Polytechnic University Ancona, 60126 Marche, Italy
| | - Marco Vivarelli
- Hepatobiliary and Transplantation Surgery, Department of Clinical and Experimental Medicine, University Hospital and Polytechnic University Ancona, 60126 Marche, Italy
| | - Rossana Berardi
- Medical Oncology, University Hospital and Polytechnic University Ancona, 60126 Marche, Italy
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10
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Tehseen S, Rapkin L, Schemankewitz E, Magliocca JF, Romero R. Successful liver transplantation for non-resectable desmoplastic nested spindle cell tumor complicated by Cushing's syndrome. Pediatr Transplant 2017. [PMID: 28626929 DOI: 10.1111/petr.13000] [Citation(s) in RCA: 8] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/17/2023]
Abstract
Desmoplastic spindle cell tumors of liver are rare tumors of low malignant potential characterized by well-demarcated nests of spindle and epithelioid cells in a dense desmoplastic stroma. While surgery remains the definitive treatment, there have been reports of tumor recurrence locally and metastasis which respond poorly to chemotherapy. Hepatic transplant has been attempted in cases of recurrence or large size of primary tumor. Long-term follow-up and imaging surveillance are required as these tumors have shown a tendency for recurrence many years after initial therapy.
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Affiliation(s)
- Sarah Tehseen
- Department of Pediatrics, Division of Pediatric Hematology Oncology (Aflac Cancer and Blood Disorders Center), Emory University, Atlanta, GA, USA.,Department of Pediatrics, Division of Pediatric Gastroenterology, Children's Healthcare of Atlanta, Emory University, Atlanta, GA, USA
| | - Louis Rapkin
- Department of Pediatrics, Division of Pediatric Hematology Oncology (Aflac Cancer and Blood Disorders Center), Emory University, Atlanta, GA, USA.,Department of Pediatrics, Division of Pediatric Gastroenterology, Children's Healthcare of Atlanta, Emory University, Atlanta, GA, USA
| | | | - Joseph F Magliocca
- Department of Surgery, Division of Liver Transplantation, Emory University, Atlanta, GA, USA
| | - Rene Romero
- Department of Pediatrics, Division of Pediatric Hematology Oncology (Aflac Cancer and Blood Disorders Center), Emory University, Atlanta, GA, USA.,Department of Pediatrics, Division of Pediatric Gastroenterology, Children's Healthcare of Atlanta, Emory University, Atlanta, GA, USA
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11
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Garg I, Baladron Zenetti MJ, Kendi AT. Nested Stromal-epithelial Tumor of Liver with Recurrent Extrahepatic Metastasis: Role of Fluorodeoxyglucose Positron Emission Tomography/Computed Tomography. Indian J Nucl Med 2017; 32:372-373. [PMID: 29142366 PMCID: PMC5672770 DOI: 10.4103/ijnm.ijnm_60_17] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 01/19/2023] Open
Abstract
Nested stromal-epithelial tumor (NSET) is a very rare nonhepatocytic and nonbiliary primary tumor of the liver. An 8-year-old boy was incidentally detected with hepatic lesions, involving both lobes of the liver for which he later underwent orthotopic liver transplant. The hepatic lesions were confirmed to be NSET following histopathological examination of explant liver specimen. He later developed recurrence with multiple metastatic lesions, including multi-station nodal and right talar bone involvement. We here present the case highlighting the importance of 18F-fludeoxyglucose positron emission tomography/computed tomography in the management of this rare tumor, in particular for monitoring disease progression and/or recurrence.
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Affiliation(s)
- Ishan Garg
- Department of Radiology, Mayo Clinic College of Medicine, Rochester, MN, USA
| | | | - Ayse T Kendi
- Department of Radiology, Mayo Clinic College of Medicine, Rochester, MN, USA
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12
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Weeda VB, de Reuver PR, Bras H, Zsíros J, Lamers WH, Aronson DC. Cushing syndrome as presenting symptom of calcifying nested stromal-epithelial tumor of the liver in an adolescent boy: a case report. J Med Case Rep 2016; 10:160. [PMID: 27306557 PMCID: PMC4910226 DOI: 10.1186/s13256-016-0951-2] [Citation(s) in RCA: 12] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/26/2016] [Accepted: 05/12/2016] [Indexed: 12/18/2022] Open
Abstract
BACKGROUND Ectopic adrenocorticotropic hormone-producing primary liver tumors are rare, especially in children. We report the case of an adolescent boy of mixed Dutch and Moroccan descent with an adrenocorticotropic hormone-producing calcifying nested stromal-epithelial tumor with long-term follow-up. Thus far, only two such cases have been reported. CASE PRESENTATION A 16-year-old boy of mixed Dutch and Moroccan descent presented with Cushing syndrome and a palpable abdominal mass. A calcifying nested stromal-epithelial tumor was diagnosed. Postoperatively, his plasma adrenocorticotropic hormone concentration normalized. He remains in complete remission 13 years after tumor resection. CONCLUSIONS Calcifying nested stromal-epithelial tumor should be in the differential diagnosis of liver tumors, especially if associated with Cushing syndrome as significant morbidity and mortality may be associated. Literature on the topics involved is comprehensively reviewed.
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Affiliation(s)
- V B Weeda
- Tytgat Institute at the Academic Medical Center, Meibergdreef 69-71, S-building, 1105 BK, Amsterdam, The Netherlands. .,Pediatric Surgical Center of Amsterdam, Meibergdreef 9, 1105, AZ, PO BOX 22660, Amsterdam, The Netherlands.
| | - Ph R de Reuver
- Pediatric Surgical Center of Amsterdam, Meibergdreef 9, 1105, AZ, PO BOX 22660, Amsterdam, The Netherlands
| | - H Bras
- Department of Pathology, Academic Medical Center, Meibergdreef 9, 1105, AZ, Amsterdam, The Netherlands
| | - J Zsíros
- Division of Pediatric Oncology, Emma Children's Hospital at the Academic Medical Center, Meibergdreef 9, 1105, AZ, Amsterdam, The Netherlands
| | - W H Lamers
- Tytgat Institute at the Academic Medical Center, Meibergdreef 69-71, S-building, 1105 BK, Amsterdam, The Netherlands
| | - D C Aronson
- Pediatric Surgical Center of Amsterdam, Meibergdreef 9, 1105, AZ, PO BOX 22660, Amsterdam, The Netherlands.,Department of Paediatric Surgery, Leeds Children's Hospital at the Leeds Teaching Hospitals NHS Trust, Leeds, West Yorkshire, LS1 3EX, UK
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Misra S, Bihari C. Desmoplastic nested spindle cell tumours and nested stromal epithelial tumours of the liver. APMIS 2016; 124:245-51. [PMID: 26994733 DOI: 10.1111/apm.12502] [Citation(s) in RCA: 11] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/29/2015] [Accepted: 11/27/2015] [Indexed: 01/10/2023]
Abstract
Desmoplastic nested spindle cell tumour of liver (DNSTL), nested stromal-epithelial tumour (NSET) and calcifying nested stromal-epithelial tumour (CNSET) are recently described entities with similar morphology, immunohistochemistry and molecular genetics. These are rare entities with only three large case series described till date. These tumours commonly present in the paediatric age group. NSETs, in addition have been described to be associated with ectopic adrenocorticotropic hormone (ACTH) production and Cushingoid features. It is important to discuss this rare group of tumours with a low malignant potential as the most common radiological differential diagnosis is hepatoblastoma, which has a relatively poorer prognosis. Thus, a pathologist needs to keep this entity in mind, so as to offer a correct histological diagnosis.
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Affiliation(s)
- Sunayana Misra
- Department of Pathology, Institute of Liver and Biliary Sciences, Delhi, India
| | - Chhagan Bihari
- Department of Pathology, Institute of Liver and Biliary Sciences, Delhi, India
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Schaffer LR, Shehata BM, Yin J, Schemankewitz E, Alazraki A. Calcifying nested stromal-epithelial tumor (CNSET) of the liver: a newly recognized entity to be considered in the radiologist's differential diagnosis. Clin Imaging 2015; 40:137-9. [PMID: 26589005 DOI: 10.1016/j.clinimag.2015.10.003] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/29/2015] [Revised: 09/24/2015] [Accepted: 10/16/2015] [Indexed: 02/08/2023]
Abstract
Calcifying nested stromal-epithelial tumor (CNSET), an extremely rare tumor found in the liver, was first described in 2001 by Ishak et al. The characteristic imaging features include large size, well-circumscribed, enhancing mass with calcification. To our knowledge, since 2001, there have been 29 reported. Typically arising from the right hepatic lobe, it is primarily found in children and shows clear predilection for females. Emphasizing imaging, we report a 14-year-old female with Beckwith-Wiedemann syndrome who presented with CNSET.
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Affiliation(s)
- Lauren R Schaffer
- Children's Healthcare of Atlanta and Emory University, Department of Pathology
| | - Bahig M Shehata
- Children's Healthcare of Atlanta and Emory University, Department of Pathology
| | - Julie Yin
- Children's Healthcare of Atlanta and Emory University, Department of Pathology
| | - Erwin Schemankewitz
- Children's Healthcare of Atlanta and Emory University, Department of Pathology
| | - Adina Alazraki
- Children's Healthcare of Atlanta and Emory University, Departments of Pediatrics and Radiology and Imaging Sciences.
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Procopio F, Tommaso LD, Armenia S, Quagliuolo V, Roncalli M, Torzilli G. Nested stromal-epithelial tumour of the liver: An unusual liver entity. World J Hepatol 2014; 6:155-159. [PMID: 24672646 PMCID: PMC3959117 DOI: 10.4254/wjh.v6.i3.155] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/02/2013] [Revised: 12/16/2013] [Accepted: 01/20/2014] [Indexed: 02/06/2023] Open
Abstract
Nested stromal-epithelial tumours (NSETs) of the liver have been reported to be extremely unusual primary hepatic neoplasms. To date, few cases have been described in the literature. NSETs have been defined as non-hepatocytic and non-biliary tumours of the liver consisting of nests of epithelial and spindled cells, myofibroblastic stroma and variable intralesional calcification and ossification. Here, we report a case of a young female who underwent liver resection for a large hepatic lesion that proved to be a calcifying NSET on pathological examination. Details about the clinical and histopathological features of the tumour are reported.
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Finegold MJ, López-Terrada DH. Hepatic Tumors in Childhood. PATHOLOGY OF PEDIATRIC GASTROINTESTINAL AND LIVER DISEASE 2014:547-614. [DOI: 10.1007/978-3-642-54053-0_14] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 01/04/2025]
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Malowany JI, Merritt NH, Chan NG, Ngan BY. Nested stromal epithelial tumor of the liver in Beckwith-Wiedemann syndrome. Pediatr Dev Pathol 2013; 16:312-7. [PMID: 23570373 DOI: 10.2350/13-02-1300-cr.1] [Citation(s) in RCA: 19] [Impact Index Per Article: 1.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 11/20/2022]
Abstract
ABSTRACT Beckwith-Wiedemann syndrome (BWS) is an imprinting disorder characterized by overgrowth, congenital malformation, and tumor predisposition. Children with BWS have a higher incidence of tumors, commonly intra-abdominal tumors such as Wilms tumor, hepatoblastoma, and adrenal cortical carcinoma. Here, we describe the first case of a rare hepatic malignancy of nested stromal epithelial tumor (NSET) of the liver in a child with BWS. A 22-month old girl with BWS had a new incidental liver mass. Her alpha-fetoprotein levels were normal. She underwent a liver segmentectomy. Histopathologic features combined with immunohistochemistry results (positivity for pankeratin [AE1/3], CD56, CK19, CD117, CD99 [weak membranous pattern], β-catenin, and WT1-COOH [focal]), were diagnostic of NSET of the liver. This is the first case of NSET of the liver associated with BWS. Its occurrence at such an early age is consistent with the tumor predisposition of BWS.
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Affiliation(s)
- Janet I Malowany
- Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Ontario, M5S 1A8, Canada
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