title: HMB-45 negative multifocal malignant perivascular epithelioid cell tumor of the soft tissue responding to sirolimus: First case report from India.
abstract: Perivascular epithelioid cell tumor (PEComa) is a group of sarcomas that exhibit a myomelanocytic phenotype and possess a unique cell type in the perivascular epithelioid cell. Traditionally HMB-45 immunoreactivity is the first criteria required to consider a tumor to be PEComa. We report a case of multifocal PEComa with negative HMB-45 marker. The patient presented with three big ulceroproliferative lesions; two over right thigh and one over the scalp in the right frontal region. The patient was prescribed with oral sirolimus to which good response was seen. To the best of our knowledge, this is the first case of HMB-45 negative multifocal malignant PEComa from India.
authors.affiliation: Department of Radiation Oncology, Acharya Tulsi Regional Cancer Treatment and Research Institute, Sardar Patel Medical College and Associated Group of Hospitals, Bikaner, Rajasthan, India.
authors.forename: Akhil
authors.initials: A
authors.lastname: Kapoor
authors.forename: Surender
authors.initials: S
authors.lastname: Beniwal
authors.forename: Mukesh Kumar
authors.initials: MK
authors.lastname: Singhal
authors.forename: Narender
authors.initials: N
authors.lastname: Kumar
authors.forename: Vanita
authors.initials: V
authors.lastname: Kumar
authors.forename: Harvindra Singh
authors.initials: HS
authors.lastname: Kumar
mesh_terms: D000328:Adult; D014408:Biomarkers, Tumor / Q000378:metabolism; D006801:Humans; D007194:India; D008297:Male; D058950:Melanoma-Specific Antigens / Q000378:metabolism*; D054973:Perivascular Epithelioid Cell Neoplasms / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology*; D011379:Prognosis; D020123:Sirolimus / Q000627:therapeutic use*; D012983:Soft Tissue Neoplasms / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology*; D058951:gp100 Melanoma Antigen
chemical_list: D014408:Biomarkers, Tumor; D058950:Melanoma-Specific Antigens; C548637:PMEL protein, human; D058951:gp100 Melanoma Antigen; D020123:Sirolimus