Desmoplastic small round cell tumor: A rare cause of a progressive brachial plexopathy

Abstract

Our findings confirm the occurrence of DSRCT as a primary peripheral nerve tumor. Despite its usually very aggressive clinical course, prolonged recurrence-free survival may be reached. Histomorphology and immunoprofile of DSRCT may lead to misdiagnosis as small cell carcinoma.

Multimodal treatment of an extremely rare desmoplastic small round cell tumor primary to the brachial plexus – A case report and review of literature

“…However, to the best of our knowledge, only two previously published cases of primary DSRCT have been reported arising directly from the BP. [10,13] Our patient, a 42-year-old female with rapidly worsening pain, weakness, and numbness in her right arm over 5 months, had an initial open yet inconclusive biopsy. After biopsy, her pain becomes intolerable and she lost considerable motor and sensory function.…”

“…In both cases of BP-DSRCT described previously, the patients were male and the time from initial symptoms to diagnosis was prolonged over several years, due both to a slowly growing mass and initially negative or equivocal investigations. [10,13] Given the rarity of DSRCT, the differential diagnosis included multiple entities such as neurogenic thoracic outlet syndrome,[10] neurofibroma, and schwannoma. [13] Contrary to the other reported cases, our patient`s tumor had an explosive clinical progression within a few months.…”

“…Mathys et al . [10] also performed an open biopsy, followed by radical resection and adjuvant RT, but no chemotherapy; however, due to aggressive resection, the function of the involved the arm was completely lost.…”

“…To date, only two cases of DSRCT arising from the brachial plexus (BP) have been described, one each by Mathys et al . [10] and Miwa et al . [13] In this paper, we add a third case.…”