Primary vulvar Ewing's sarcoma/primitive neuroectodermal tumor in a post-menopausal woman: a case report.
Ewing's sarcomas/peripheral primitive neuroectodermal tumors (ES/pPNETs) are high-grade malignant neoplasms rarely found outside the skeletal system. Only 12 cases of vulvar ES/pPNET have so far been reported, all involving children or women of child-bearing age. We describe the case of a 52-year-old woman who was admitted to our hospital for the local excision of a 4cm vulvar mass, originally thought to be a Bartholin's gland cyst. It was subsequently found to consist of small round cells positive for anti-CD99 antibody, thus suggesting a diagnosis of ES/pPNET. The demonstration of EWSR1 gene translocations by means of fluorescent in situ hybridization excluded small-cell carcinoma, squamous cell carcinoma of the small type, Merkel cell carcinoma, and lymphoblastic lymphoma. After surgery, the patient received six cycles of polychemotherapy and radiotherapy; she is still alive and well after 1 year of follow-up. Our findings underline the crucial role of molecular biology techniques in the differential diagnosis of small round cell tumors in these unusual locations.
Boldorini
Renzo
R
Department of Pathology, Maggiore Hospital, University School of Medicine Amedeo Avogadro, Novara, Italy.
Riboni
Francesca
F
Cristina
Silvia
S
Allegrini
Sara
S
Valentini
Stefano
S
Muscarà
Maurizio
M
Ruspa
Giovanni
G
D000071443:12E7 Antigen; D015703:Antigens, CD / Q000378:metabolism; D000970:Antineoplastic Agents / Q000627:therapeutic use; D002148:Calmodulin-Binding Proteins / Q000235:genetics; D015815:Cell Adhesion Molecules / Q000378:metabolism; D003131:Combined Modality Therapy; D003937:Diagnosis, Differential; D005260:Female; D006801:Humans; D017404:In Situ Hybridization, Fluorescence; D008875:Middle Aged; D018241:Neuroectodermal Tumors, Primitive, Peripheral / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D017698:Postmenopause; D034802:RNA-Binding Protein EWS; D016601:RNA-Binding Proteins / Q000235:genetics; D011878:Radiotherapy; D012512:Sarcoma, Ewing / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D014178:Translocation, Genetic; D014846:Vulvar Neoplasms / Q000235:genetics / Q000473:pathology* / Q000628:therapy
D000071443:12E7 Antigen; D015703:Antigens, CD; D000970:Antineoplastic Agents; C061828:CD99 protein, human; D002148:Calmodulin-Binding Proteins; D015815:Cell Adhesion Molecules; C500986:EWSR1 protein, human; D034802:RNA-Binding Protein EWS; D016601:RNA-Binding Proteins