What causes PEComa?

The cause of sporadic cases of PEComas is not known. PEComas may be associated with the genetic condition tuberous sclerosis complex (TSC), an autosomal dominant genetic disorder. Dominant genetic disorders occur when only a single copy of a non-working gene is necessary to cause a particular disease.

Is 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.

What is PEComa sarcoma?

A family of rare tumors that form in the soft tissues of the stomach, intestines, lungs, female reproductive organs, and genitourinary organs. Most PEComas are benign (not cancer). They often occur in children with an inherited condition called tuberous sclerosis. Also called perivascular epithelioid cell tumor.

What is leiomyosarcoma?

Leiomyosarcoma, or LMS, is a type of rare cancer that grows in the smooth muscles. The smooth muscles are in the hollow organs of the body, including the intestines, stomach, bladder, and blood vessels. In females, there is also smooth muscle in the uterus.

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.

How is leiomyosarcoma detected?

How is leiomyosarcoma diagnosed? Doctors use imaging to detect leiomyosarcoma. These tests typically include MRIs or CTs. The scans can show the size of the tumor, where it started, whether any nearby organs or tissues are involved, and whether the tumor has spread to other parts of the body.