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Arabadzhieva E, Yonkov A, Bonev S, et al. A rare combination between familial multiple lipomatosis and extragastrointestinal stromal tumor. Int J Surg Case Rep. 2015;14:117-20doi: 10.1016/j.ijscr.2015.07.027.
Arabadzhieva, E., Yonkov, A., Bonev, S., Bulanov, D., Taneva, I., Ivanova, V., & Dimitrova, V. (2015). A rare combination between familial multiple lipomatosis and extragastrointestinal stromal tumor. International journal of surgery case reports, 14117-20. https://doi.org/10.1016/j.ijscr.2015.07.027
Arabadzhieva, Elena, et al. "A rare combination between familial multiple lipomatosis and extragastrointestinal stromal tumor." International journal of surgery case reports vol. 14 (2015): 117-20. doi: https://doi.org/10.1016/j.ijscr.2015.07.027
Arabadzhieva E, Yonkov A, Bonev S, Bulanov D, Taneva I, Ivanova V, Dimitrova V. A rare combination between familial multiple lipomatosis and extragastrointestinal stromal tumor. Int J Surg Case Rep. 2015;14:117-20. doi: 10.1016/j.ijscr.2015.07.027. Epub 2015 Jul 31. PMID: 26263450; PMCID: PMC4573610.
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Int J Surg Case Rep. 2015;14:117-20. doi: 10.1016/j.ijscr.2015.07.027. Epub 2015 Jul 31.

A rare combination between familial multiple lipomatosis and extragastrointestinal stromal tumor.

International journal of surgery case reports

Elena Arabadzhieva, Atanas Yonkov, Sasho Bonev, Dimitar Bulanov, Ivanka Taneva, Vesela Ivanova, Violeta Dimitrova

Affiliations

  1. Department of General and Hepato-Pancreatic Surgery, University Hospital "Alexandrovska"-Sofia, Medical University-Sofia, 1 G.Sofiiski Str., 1431 Sofia, Bulgaria. Electronic address: [email protected].
  2. Department of General and Hepato-Pancreatic Surgery, University Hospital "Alexandrovska"-Sofia, Medical University-Sofia, 1 G.Sofiiski Str., 1431 Sofia, Bulgaria.
  3. Department of General and Clinical Pathology, Medical University-Sofia, 2 Zdrave Str., 1431 Sofia, Bulgaria.

PMID: 26263450 PMCID: PMC4573610 DOI: 10.1016/j.ijscr.2015.07.027

Abstract

INTRODUCTION: Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract. Rarely, GISTs can be located in mesentery, retroperitoneal space, omentum or pancreas. In these cases, the neoplasm is defined as "extra-gastrointestinal stromal tumors" (EGISTs).

PRESENTATION OF CASE: We reported a case of a 63-year-old male patient diagnosed by computer tomography with large intraabdominal tumor with vague origin, postoperatively determined as an EGIST. The diagnosis was confirmed by immunohistochemical study. The patient had multiple, subcutaneous, painless lipomas localized in the arms, forearms, thighs, abdomen and thorax. Because of the family history and the clinical presentation the disease was determined as familial multiple lipomatosis (FML). We performed radical tumor resection with distal pancreatectomy and splenectomy, and abdominoplasty, removing redundant skin and underlying subcutaneous fat tissue with multiple lipomas.

DISCUSSION: FML is a rare hereditary benign disease. On the other hand, only few cases with familial GIST have been reported. In cases with extensive abdominal involvement, the primary origin of EGIST may be impossible to determine so the differential diagnosis is very difficult.

CONCLUSION: Although we could not prove correlation between the observed diseases, they are extremely rare and their combination is unusual which makes the presented case valuable and interesting.

Copyright © 2015 The Authors. Published by Elsevier Ltd.. All rights reserved.

Keywords: CD117; CD34; Extragastrointestinal stromal tumor; Familial multiple lipomatosis; Radical resection

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