Myoepithelial carcinoma is a rare malignant tumor arising from myoepithelial cells. The usual sites of occurrence are the oral cavity and pharynx with the majority of tumors arising from the salivary gland. However, there have been reported cases of myoepithelial carcinoma seen in the superficial soft tissue, upper respiratory tract, breast, skin, and GI tract. Deep soft tissue myoepithelial carcinoma is relatively uncommon.Due to the rarity of this malignancy, consensus on appropriate therapy remains sparse. However, complete resection and/or adjuvant chemotherapy and radiotherapy have been advocated for non-metastatic localized diseases. Sadly, the reported outcome in patients with metastatic disease remains dismal.In this case, we report a 79-year-old male incidentally found to have a deep soft tissue mass in the sacral area with a coexistent left axillary lymphadenopathy with biopsy and immunohistochemistry confirmation of metastatic myoepithelial carcinoma. He had a rapid clinical deterioration with subsequent fatality.
Sarcoma is an uncommon neoplasm of mesenchymal origin (1). The presentation is usually vague. It may present as a mass in the thigh or retroperitoneum, with resultant pain or paresthesia of the affected area. The diagnosis is very challenging due to its indistinct presentation. The prognosis remains poor due to delays in diagnosis and few available therapeutic options. We herein report the first case of superior vena cava (SVC) syndrome caused by spindle cell sarcoma.
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