The World Health Organization defines perivascular epithelioid cell tumors<unk> s) as mesenchymal tumors composed of histologically and immunohistochemically distinctive perivascular epithelioid cells (PECs).<unk> s were first discovered by Pea and Colleagues in 1991. Zamboni et al in 1996 suggested the name<unk>  for these neoplasms. They are a group of tumors that includes: angiomyolipoma (AML), lymphangioleiomyomatosis (LAM), and others. PEC has no normal counterpart, it expresses myogenic and melanocytes markers such as HMB45 and actin. Genetically they are linked to the tuberous sclerosis genes 1 and 2. There are no established risk factors for the<unk> s but the risk increases in patients with tuberous sclerosis. The symptoms depend upon the area involved and can include palpable abdominal mass/abnormal vaginal bleeding (uterus), flank pain (kidney), or dull abdominal pain in the right upper quadrant (liver). It may occur in any age group, but median age is 54 years and is more common in females. Clinically, most<unk> s are benign. CT scan and presence of PECs on histology are helpful in diagnosis. Surgery is the mainstay of treatment; however, other chemotherapeutic and immunotherapeutic drugs are under investigation.
PEComa
Neuroma
Ameloblastoma
Hemangioblastoma
Myxoma
Gemistocytic astrocytoma
Protoplasmic astrocytoma
Schwannoma