| Summary: | Intimal sarcoma is a grouping of uncommon pleomorphic sarcomas most commonly arising from the intimal layer of large named arteries. The venous system is rarely affected. Albeit, no specific societal guidelines exist, treatment follows the guidelines of soft tissue sarcoma and includes 1 cm margins with radiation therapy for resectable disease. Unfortunately, surveillance of patients is limited, given the high mortality. We present a 65-year-old male with a one-month history of a left neck mass. CT scan demonstrated a 3.7 cm homogenous neck mass. FNA was nondiagnostic, which was followed by an excisional biopsy. Intraoperatively, a solid firm well-circumscribed lesion emanating from the left external jugular vein (EJV) was resected en bloc. Pathology showed locally aggressive angiocentric neoplasm with epithelioid to spindle morphology infiltrating through the vessel wall consistent with intimal sarcoma.
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