Abstract
Primitive neuroectodermal tumors (PNETs) exhibit chromosomal translocations in common with those of Ewing’s sarcoma. They usually originate in bone or soft tissue but rarely arise in the vulva. The current case report presents a case of PNET originating in the vulva in a 60-year-old female, who previously underwent enucleation of a vulvar tumor in another hospital. The pathologist suspected a histopathological diagnosis of PNET, and simple vulvectomy and resection of the inguinal lymph nodes were performed. An ~3 cm mass recurred in the right side of the vulva four years following the initial surgery and the tumor was excised. The tumor comprised small, round-to-oval nuclei and stained positively for MIC-2, synaptophysin, neuron-specific enolase and neurofilament antibodies. To date, the patient remains alive and with no evidence of disease four years following multidisciplinary treatment, despite PNETs usually exhibiting a poor prognosis. This is due to the small tumor size and the absence of distant metastasis.
Highlights
Primitive neuroectodermal tumors (PNETs) were first discovered by Hart and Earle in 1973 [1]
PNETs are classified as central or peripheral types based on their site of origin, they usually originate in bone or soft tissue, but rarely arise in the vulva
In the vast majority of cases, PNETs have been shown to express at extremely high levels, an antigen determined by the MIC2 gene
Summary
A 60‐year‐old female visited Osaka City Sumiyoshi Hospital (Osaka, Japan) for evaluation of a mass in the right side of the vulva. Enucleation of the vulvar tumor was performed and the suspected diagnosis was hemangiopericytoma. The tumor exhibited characteristics of borderline malignancy and the patient was referred to Osaka City University Hospital (Osaka, Japan) for additional therapy. The tissue excised at the previous hospital was histopathologically examined by the pathologist and the suspected diagnosis was PNET. Four years following the initial surgery, a mass in the right side of the vulva was observed. MRI imaging revealed a 3‐cm mass in the right side of the vulva. The patient remains alive with no evidence of disease four years following the final medical treatment
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