Perivascular epithelioid cell tumor of pancreas on the background of chronic liver disease: a case report and review of literature

Authors

  • Rohan N. Umesh Institute of Surgical Gastroenterology and Liver Transplantation, Government Stanley Medical College and Hospital Chennai, Tamil Nadu, India
  • Saravanan Janakiraman Institute of Surgical Gastroenterology and Liver Transplantation, Government Stanley Medical College and Hospital Chennai, Tamil Nadu, India
  • Selvaraj Thangasamy Institute of Surgical Gastroenterology and Liver Transplantation, Government Stanley Medical College and Hospital Chennai, Tamil Nadu, India
  • Jeswanth Sathyanesan Institute of Surgical Gastroenterology and Liver Transplantation, Government Stanley Medical College and Hospital Chennai, Tamil Nadu, India

DOI:

https://doi.org/10.18203/2349-2902.isj20232344

Keywords:

Perivascular epithelioid cell tumour of the pancreas, PEComa of the pancreas, Distal pancreatosplenectomy, HMB 45, SMA

Abstract

Perivascular epithelioid cell tumours, known as PEComas, of the pancreas are an extremely rare group of neoplasms. This heterogenous entity includes angiomyo­lipo­ma (AML), clear cell sugar tumour (CCST), lymphangioleio­myoma (LAM), and another group of lesions originating from the soft tissues and organs with similar histological and immunophenotype characteristics. To date, only 23 cases have been reported throughout the world with different clinical presentations. We report a 52-year-old lady who presented with a symptomatic mass in the body of the pancreas on the background of hepatitis B virus (HBV)-related chronic liver disease (CLD). She presented with abdominal pain of 2 months' duration. On initial computed tomography (CT) imaging, it revealed a homogenously enhancing lesion in the body of the pancreas. It was T2 isointense on magnetic resonance imaging (MRI), and there was no uptake present in the 68Ga DOTONAC scan. Endoscopic ultrasound with fine needle aspiration (FNA) showed paucicellular material with occasional dysplastic cells. On account of suspicion of a non-functional neuroendocrine tumour, she underwent a distal pancreatosplenectomy. Intra-operatively, the lesion was encapsulated and hard in consistency, and the rest of the pancreas was soft with an undilated MPD. She had a smooth postoperative recovery. Final histopathology revealed benign PEComa of the distal body and tail of the pancreas with HMB 45 and SMA positivity on IHC. Currently, she has been on regular follow up for the last 12 months. PEComas usually present as a histological surprise and are a diagnosis of exclusion. However, knowledge of this exceptionally rare tumour makes diagnosis at the pre-operative level more certain. Resection must be considered, as it leads to a good prognosis and survival.

References

Geng C, Cao Z, Bacacao B, Cao Z. Perivascular epithelioid cell tumor of the pancreas: case report and literature review. Int J Clin Exp Pathol. 2021;14(5):653-61.

Mizuuchi Y, Nishihara K, Hayashi A, Tamiya S, Toyoshima S, Oda Y, et al. Perivascular epithelial cell tumor (PEComa) of the pancreas: a case report and review of previous literatures. Surg Case Rep. 2016;2(1):59.

Hirabayashi K, Nakamura N, Kajiwara H, Hori S, Kawaguchi Y, Yamashita T, et al. Perivascular epithelioid cell tumor (PEComa) of the pancreas: immunoelectron microscopy and review of the literature. Pathol Int. 2009;59(9):650-5.

Martignoni G, Pea M, Reghellin D, Zamboni G, Bonetti F. PEComas: the past, the present and the future. Virchows Arch. 2008;452(2):119-32.

Folpe AL, Mentzel T, Lehr HA, Fisher C, Balzer BL, Weiss SW. Perivascular epithelioid cell neoplasms of soft tissue and gynecologic origin: a clinicopathologic study of 26 cases and review of the literature. Am J Surg Pathol. 2005;29(12):1558-75.

Jiang H, Ta N, Huang XY, Zhang MH, Xu JJ, Zheng KL, et al. Pancreatic perivascular epithelioid cell tumor: A case report with clinicopathological features and a literature review. World J Gastroenterol. 2016;22(13):3693-700.

Zamboni G, Pea M, Martignoni G, Zancanaro C, Faccioli G, Gilioli E. Clear cell “sugar” tumor of the pancreas: a novel member of the family of lesions characterized by the presence of perivascular epithelioid cells. Am J Surg Pathol. 1996;20:722-30.

Heywood G, Smyrk TC, Donohue JH. Primary angiomyolipoma of the pancreas. Pancreas. 2004;28:443-5.

Ramuz O, Lelong B, Giovannini M, Delpero JR, Rochaix P, Xerri L, et al. Sugar tumor of the pancreas: a rare entity that is diagnosable on preoperative fine-needle biopsies. Virchows Arch. 2005:446:555-9.

Périgny M, Larochelle O, Hammel P, Sauvanet A, Dokmak S, Belghiti J. Pancreatic perivascular epithelioid cell tumor (PEComa). Ann Pathol. 2008;28:138-42.

Gleeson FC, de la Mora Levy JG, Zhang L, Levy MJ. The differential broadens. EUS FNA appearance and cytological findings of pancre-atic angiomyolipoma. JOP. 2008;9:67-70.

Baez JC, Landry JM, Saltzman JR, Qian X, Zinner MJ, Mortelé KJ. Pancreatic PEComa (sugar tumor): MDCT and EUS features. JOP. 2009;10:679-82.

Zemet R, Mazeh H, Neuman T, Freund HR, Eid A. Asymptomatic pancreatic perivascular epithelial cell tumor (PEComa) in a male patient: report and literature review. JOP. 2011;12:55-8.

Nagata S, Yuki M, Tomoeda M, Kubo C, Yoshizawa H, Kitamura M, et al. Perivascular epithelioid cell neoplasm (PEComa) originating from the pancreas and metastasizing to the liver. Pancreas. 2011;40:1155-7.

Xie FL, Song YN, Qu LJ, Yu YH, Zheng ZY. Clinicopathologic features of perivascular epi-thelioid cell tumor of the pancreas. World J Gastroenterol. 2011;19:964-8.

Finzi G, Micello D, Wizemann G, Sessa F, Capella C. Pancreatic PEComa: a case report with ultrastructural localization of HMB-45 within melanosomes. Ultrastruct Pathol. 2012;36:124-9.

Al-Haddad M, Cramer HM, Muram T, Wang X, Pitt HA. Perivascular epithelioid cell tumor: an unusual pancreatic mass diagnosed by EUS-FNA. Gastrointest Endosc. 2013;78:165-7.

Okuwaki K, Kida M, Masutani H, Yamauchi H, Katagiri H, Mikami T, et al. A resected perivascular epithelioid cell tumor (PEComa) of the pancreas diagnosed using endoscopic ultrasound-guided fine-needle aspiration. Intern Med. 2013;52:2061-6.

Mourra N, Lazure T, Colas C, Arrive L, de Gramont A. Perivascular epithelioid cell tumor: the first malignant case report in the pancreas. Appl Immunohistochem Mol Morphol. 2013;21:e1-4.

Yunyuan L, Binbin L, Hongwei C, Chunyan X. Perivascular epithelioid cell tumor (PEComa) of the pancreas: a clinicopathological analysis. Diag Pathol. 2016;23:172-5.

Petrides C, Neofytou K, Khan AZ. Pancreatic perivascular epithelioid cell tumour presenting with upper gastrointestinal bleeding. Case Rep Oncol Med. 2015;431215.

Yang T, Xiao L, Zhihong Z, Hongxia L. Melanin-rich pancreatic PEComa: a case report and review of literature. Diag Pathol. 2016;23:1845-9.

Zizzo M, Castro Ruiz C, Ugoletti L, Bonacini S, Giunta A, Valli R, et al. Primary pancreatic pecoma: is surgery always the best choice? JOP. 2017;18:241-3.

Wei J, Liu X, Fu Y, Gao W, Jiang K, Zhang Z, et al. Primary perivascular epithelioid cell tumor of the pancreas: a case report. Pancreatology. 2016;16:S30.

Jiang H, Ta N, Huang XY, Zhang MH, Xu JJ, Zheng KL, et al. Pancreatic perivascular epithelioid cell tumor: a case report with clinicopathological features and a literature review. World J Gastroenterol. 2016;22:3693-700.

Hartley CP, Kwiatkowski DJ, Hamieh L, Lefferts JA, Smith KD, Lisovsky M. Pancreatic PEComa is a novel member of the family of tuberous sclerosis complex-associated tumors: case report and review of the literature. Virchows Arch. 2016;469:707-10.

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Published

2023-07-28

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Section

Case Reports