Introduction:
Extragastrointestinal stromal tumours (EGISTs) are very uncommon compared to their gastrointestinal counterparts. Most of them originate from the intestinal mesentery and the omentum.
Case report:
A 70 year-old Caucasian woman presented with a bulky abdominal mass which on laparotomy was found to originate from the lesser omentum and was completely resected. Histological examination revealed spindle cells with severe pleomorphism and high mitotic activity. Immunohistochemically, the tumour cells showed strong positivity for c-kit (CD117), DOG-1 and human haematopoietic progenitor cell antigen (CD34). An exon 11 deleterious mutation was identified and thus regular dosing of 400mg imatinib mesylate was initiated.
Discussion:
There have been only a few previous reports of EGISTs arising in the lesser omentum. Although EGISTs seem to have morphological and immunohistochemical similarities with GISTs, their pathogenesis, incidence, genetic background and prognosis are not completely known because they are extremely rare. It is strongly believed that such tumours originate from cells, which have similar pathological characteristics and biological behaviour as the intestinal cells of Cajal. In most series of EGISTs, a female predominance, a greater size and a higher mitotic index than GISTs were observed.
Conclusion:
EGISTs are very rare mesenchymal tumours which originate from cells outside the gastrointestinal tract and tend to have a more aggressive biological behaviour than their GI counterparts. Complete surgical resection is the most effective treatment associated with the use of imatinib in the presence of adverse prognostic factors. In any case a strict follow-up is necessary due to high recurrence rates.