| Popis: |
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. |
