ABSTRACT
Only 14 cases of extraskeletal myxoid chondrosarcoma (EMC) of the vulva have been documented in the literature. We report a case of a 63-year-old woman with EMC of the vulva confirmed by both EWSR1 and NR4A3 fluorescence in situ hybridization, the latter of which is a more specific probe for this entity. The unusual location of this tumor of prominent myxoid morphology gave rise to a wide differential diagnosis, which necessitated thorough histologic evaluation and confirmatory ancillary testing in the form of immunohistochemistry and cytogenetic studies. This article aims to review extraskeletal myxoid chondrosarcoma of the vulva and various diagnostic clues to help differentiate it from its histologic mimics. This is the fifth case of vulvar EMC in the literature with confirmation of a NR4A3 gene rearrangement.
Subject(s)
Humans , Female , Middle Aged , Soft Tissue Neoplasms , Vulva/abnormalities , Chondrosarcoma/pathology , RNA-Binding Protein EWS , Diagnosis, Differential , Nuclear Receptor Subfamily 4, Group A, Member 3ABSTRACT
Arteriovenous malformation (AVM) is a rare lesion in the uterus, which can lead to abnormal uterine bleeding. While AVM has been described in other organs in the literature, there is a paucity of pathology reports of the AVM in uterus. On gross examination, the uterus was markedly enlarged and partly distorted with a pedunculated solid mass, which on the cut surface showed multiple well-circumscribed hemorrhagic cysts ranging from 0.1 to 4.0 cm in size. Microscopically, they were malformed dilated vascular structures containing organized thrombi. We present this case of uterine AVM with gross and microscopic findings, which can serve as a crucial reminder for pathologists to keep in the differential diagnoses as a potential cause of abnormal uterine bleeding.