6533b7d6fe1ef96bd1267142

RESEARCH PRODUCT

Malignant PEComa With Metastatic Disease at Diagnosis and Resistance to Several Chemotherapy Regimens and Targeted Therapy (m-TOR Inhibitor).

José M RayonJulia CruzAndres PovedaIsidro MachadoAntonio Llombart-boschJavier Lavernia

subject

0301 basic medicineAdultPathologymedicine.medical_specialtyPerivascular Epithelioid Cell Neoplasmsmedicine.medical_treatmentAntineoplastic AgentsDiseasePathology and Forensic MedicineTargeted therapy03 medical and health sciences0302 clinical medicineMedicineHumansMolecular Targeted TherapyChemotherapybusiness.industryTOR Serine-Threonine Kinasesmedicine.diseasePerivascular Epithelioid Cell Tumors030104 developmental biologyDrug Resistance Neoplasm030220 oncology & carcinogenesisImmunohistochemistrySurgeryFemaleSarcomaAnatomyDifferential diagnosisbusiness

description

Perivascular epithelioid cell tumors (PEComas) are infrequent neoplasms with peculiar myomelanocytic differentiation. The aggressive abdominopelvic variant is rare, with only a small number of published cases. We present an additional case of this unusual variant, which showed an aggressive histologic and clinical behavior with multiple liver metastases and resistance to several therapies. We also discuss the histological and immunohistochemical profiles as well as the differential diagnosis.

10.1177/1066896917701245https://pubmed.ncbi.nlm.nih.gov/28459168