Research Article
Volume 8 Issue 12 - 2021
Primary Leiomyosarcoma of the Pancreas associated with Portal Hypertension
Guillermo André Carnevale1*, Adolfo Wulfson2, Claudio Guerrina3, Guillermo D’ottavio4, Nidal Mawuasi5, Miguel Muñoz6 and Germán Sánchez Granel7
1Hospital Italiano Rosario (H.I.R.), Rosario, Argentina
2Postgraduate Teacher in Gastroenterology and Endoscopy at the Faculty of Medical Sciences, Rosario (U.N.R.), Gastroenterology Rosario (GR), Argentina
3Hospital Dr. Clemente Álvarez (H.E.C.A.), Hospital Provincial Centenario, Rosario, Argentina
4Gastroenterologist, (H.I.G.), Rosario, Argentina
5Gastroenterologist, Haifa, Israel
6Clinical Oncology, Rosario Cancer Center, Rosario, Argentina
7Quantum, Pathology, Rosario, Argentina
*Corresponding Author: Guillermo Andrés Carnevale, Hospital Italiano Rosario (H.I.R.), Rosario, Argentina.
Received: February 16, 2021; Published: November 30, 2021




Abstract

Introduction: Primary Pancreatic Leiomyosarcoma is a rare tumour. It probably originates from the smooth muscle of the pancreatic ducts or the small pancreatic vessels. Given its rarity and the scarce published cases, only few data are available regarding its epidemiological characteristics, evolution and therapeutic strategies. Common elements of the previously published cases were poor prognosis and aggressive course with early haematogenous metastasis.

Case Report: A 61-year-old man presented with subacute abdominal pain and weight loss. Abdominal computerized tomography showed a large solid mass that encompasses the body and head of the pancreas in addition to multiple solid nodular lesions in the liver compatible with metastases.

Pathology Report: Primary pancreatic leiomyosarcoma, immunologically-stained positive for caldesmon, smooth muscle actin. Chemotherapy was given with initial improvement. The patient survived for 27 months.

Conclusion: The rarity and the ominous course of primary pancreatic leiomyosarcoma impose great medical challenge. It also lacks specific clinical and imaging characteristics and evidence-based treatment strategy. The immunohistochemically diagnosis proves to be essential, due to the important differential diagnosis. Its ominous prognosis corresponds to its aggressive course and early haematogenous metastases.

 

Keywords: Primary Leiomyosarcoma of the Pancreas; Rare Tumors of the Pancreas; Pancreas

References

  1. Søreide JA., et al. “Primary Leiomyosarcoma of the Pancreas a Case Report and a Comprehensive Review”. Journal of Gastrointestinal Cancer 4 (2016): 358-365.
  2. Du X., et al. “Primary pancreatic leiomyosarcoma: a retrospective analysis of clinical characteristics and prognosis of this rare disease”. Hepatogastroenterology120 (2012): 2644-2649.
  3. Nesi G., et al. “Primary leiomyosarcoma of the pancreas: a case report and review of literature”. Archives of Pathology and Laboratory Medicine 125 (2001): 152-155.
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  5. Zalatnai A., et al. “Pancreatic leiomyosarcoma. Case report with immunohistochemical and flow cytometric studies”. Virchows Archiv 5 (1998): 469-472.
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  7. Izumi H., et al. “Leiomyosarcoma of the pancreas: report of a case”. Surgery Today 41 (2011): 1556-1561.
  8. Aihara H., et al. “A small leiomyosarcoma of the pancreas treated by local excision”. HPB (Oxford)3 (2002): 145-148.
  9. Hur YH., et al. “Primary leiomyosarcoma of the pancreas”. Journal of Korean Surgical Society 1 (2011): S69-S73.
  10. Xu J., et al. “Clinical characteristics and prognosis of primary leiomyosarcoma of the pancreas: a systematic review”. World Journal of Surgical Oncology 11 (2013): 290.
  11. Zhang H., et al. “Primary leiomyosarcoma of the pancreas: study of 9 cases and review of literature”. The American Journal of Surgical Pathology 12 (2010): 1849-1856.
  12. Barros A., et al. “Extragastrointestinal stromal tumors (EGIST): a series of case reports”. Hepatogastroenterology107-108 (2011): 865-868.
Citation: Guillermo André Carnevale., et al. “Primary Leiomyosarcoma of the Pancreas associated with Portal Hypertension”. EC Gastroenterology and Digestive System 8.12 (2021): 17-21.

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