Acute Ischemia of Extremity as a First Manifestation of Peripheral Artery Leiomyosarcoma: Report of a Case and Review of the Literature.

Leiomyosarcoma is an aggressive soft tissue sarcoma derived from smooth muscle cells. Of all soft tissue sarcomas, approximately 5-10% are leiomyosarcomas. Vascular leiomyosarcoma constitutes about 2% of all leiomyosarcomas and involves veins 5 times more than arteries. When they arise from a major blood vessel, symptoms of vascular compromise or leg edema may be present. Because they are rare, definite diagnosis is often delayed. We present the case of an 88-year-old man who was admitted to our department with acute limb ischemia stage 4 according to Rutherford's criteria. His personal medical history included arterial hypertension under medication with nonspecific conduction disturbances showed in the electrocardiography. The duplex scan revealed the presence of thrombotic material to the distal superficial femoral and popliteal artery, whereas the presence of popliteal artery aneurysm was excluded. After the initial diagnostic approach, he underwent 2 unsuccessful embolectomy procedures. During the amputation procedure, a 6-cm mass was palpated in the popliteal fossa, and it was excised. The immunohistopathologic study revealed a grade 3 according to the French Federation Nationale des Centers de Lutte Contre le Cancer classification leiomyosarcoma. The patient was discharged 10 days later and referred to an oncologic center. He returned 6 months later with edema of the amputated limb and inguinal lymphadenopathy. Specimen of the inguinal lymph nodes was sent for histopathologic examination, which indicated the recurrence of the disease. Leiomyosarcomas should be taken into consideration in elderly patients presenting with acute limb ischemia.