Is a PEComa malignant?

Perivascular epithelioid cell neoplasms (PEComa) are rare mesenchymal tumors that can occur in any part of the body and have unpredictable pathological behavior. They are usually benign, but may be malignant.

Is PEComa treatable?

PEComa exhibits a wide variety of behavior, from benign disease treatable by excision alone, to most recently described malignant disease with a poor chemotherapeutic response and prognosis [6].

How is PEComa treated?

PEComa is a rare neoplasm, and no standardized treatment has been established. Currently, surgery is the mainstay of treatment for primary PEComa at presentation, as well as for local recurrences and metastases, with the aim of obtaining clear resection margins.

How common is PEComa?

Omental PEComas are very rare. To the best of our knowledge, only two prior cases of omental PEComa or perivascular epithelioid cell tumor have been reported.

What causes a PEComa?

The origin of PEComas remains yet unknown but studies suggest that they arise from neural stem cells that present with the ability to differentiate into both myoid and melanocytic cells,.

What is metastatic PEComa?

Perivascular epithelioid cell tumors (PEComa) are a family of rare mesenchymal tumors composed of histologically and immunohistochemically distinctive perivascular epithelioid cells. [1] We report a case of metastatic PEComa with favorable but short-lived response to mammalian target of rapamycin (mTOR) inhibition.

What causes PEComa?

Most PEComas are benign (not cancer). They often occur in children with an inherited condition called tuberous sclerosis. Also called perivascular epithelioid cell tumor.

Is PEComa hereditary?

PEComas are related to the genetic alterations of tuberous sclerosis complex (TSC), an autosomal dominant genetic disease due to losses of TSC1 (9q34) or TSC2 (16p13. 3) genes which seem to have a role in the regulation of the Rheb/mTOR/p70S6K pathway.

Is PEComa genetic?

PEComas constitute a genetically diverse group that includes neoplasms harboring TFE3 gene rearrangements and those with TSC2 mutations, indicating alternative tumorigenic pathways.

Is PEComa benign or cancerous?

Most PEComas are benign or non-cancerous. In rare cases, some PEComas may be malignant, or cancerous, with no approved therapies until recently. The symptoms associated with PEComas depend on the location where the tumor manifests and symptoms can be nonspecific, similar to other forms of cancer.

What is a perivascular mesenchymal tumor (PEComa)?

PEComas are mesenchymal tumors composed of characteristic perivascular cells that show focal association with blood vessel walls and usually express melanocytic and smooth muscle markers [3].

What do we know about the clinicopathological features of PEComa?

Abstract PEComa (perivascular epithelioid cell tumor) is a rare liver tumor. Decisions regarding patient management are currently based on a few small case series. The aim of this study was to report the clinicopathological features of PEComa in order to provide guidance for management, complemented by our own experience.

What is PEComa and how can it be treated?

There is no cure for advanced malignant PEComa, but it is treatable. In most cases, the main goal oftreatment is to help shrink and control cancer growth as long as possible. PEComa=perivascular epithelioid cell tumor. Next: What is FYARRO and how can it help?