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


METHODS
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.


CONCLUSIONS
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.


CONCLUSIONS
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.
Department of General and Hepato-Pancreatic Surgery, University Hospital "Alexandrovska"-Sofia, Medical University-Sofia, 1 G.Sofiiski Str., 1431 Sofia, Bulgaria. Electronic address: elena_arabadjieva@abv.bg.
Elena
E
Arabadzhieva
Department of General and Hepato-Pancreatic Surgery, University Hospital "Alexandrovska"-Sofia, Medical University-Sofia, 1 G.Sofiiski Str., 1431 Sofia, Bulgaria.
Atanas
A
Yonkov
Department of General and Hepato-Pancreatic Surgery, University Hospital "Alexandrovska"-Sofia, Medical University-Sofia, 1 G.Sofiiski Str., 1431 Sofia, Bulgaria.
Sasho
S
Bonev
Department of General and Hepato-Pancreatic Surgery, University Hospital "Alexandrovska"-Sofia, Medical University-Sofia, 1 G.Sofiiski Str., 1431 Sofia, Bulgaria.
Dimitar
D
Bulanov
Department of General and Hepato-Pancreatic Surgery, University Hospital "Alexandrovska"-Sofia, Medical University-Sofia, 1 G.Sofiiski Str., 1431 Sofia, Bulgaria.
Ivanka
I
Taneva
Department of General and Clinical Pathology, Medical University-Sofia, 2 Zdrave Str., 1431 Sofia, Bulgaria.
Vesela
V
Ivanova
Department of General and Hepato-Pancreatic Surgery, University Hospital "Alexandrovska"-Sofia, Medical University-Sofia, 1 G.Sofiiski Str., 1431 Sofia, Bulgaria.
Violeta
V
Dimitrova
CD117; CD34; Extragastrointestinal stromal tumor; Familial multiple lipomatosis; Radical resection