Are PEComas benign?

Most PEComas are benign but some can be malignant with the potential to spread to other parts of the body. PEComas can also be associated with the genetic condition tuberous sclerosis complex (TSC). In this condition, multiple tumors form in the body.

Are PEComas 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.

How is PEComa treated?

For most tumors, surgical resection is the first or only effective treatment. The most effective treatment for gastrointestinal PEComa is surgical resection. Surgical resection of the tumor with the adjacent tissue in the gastrointestinal tract is the mainstay of treatment of the primary tumor and of local recurrence.

What is a PEComa?

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.

How many people have PEComa?

These tumors are usually rare and mostly benign, and when they occur, the uterus is the second most common lesion site to be affected, after the retroperitoneum. Until now, approximately 100 PEComa cases have been reported, of which one-third of these affected the uterus.

How common is a 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 is angiomyolipoma of the kidney?

(AN-jee-oh-MY-oh-lih-POH-muh) A benign (noncancer) tumor of fat and muscle tissue that usually is found in the kidney. Angiomyolipomas rarely cause symptoms, but may bleed or grow large enough to be painful or cause kidney failure.

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.

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 common is PEComa?

Is a PEComa a sarcoma?

Malignant PEComa is an “ultra rare and aggressive” soft-tissue sarcoma that carries a high risk of metastases, explained Wagner, and no therapies are specifically approved for this indication.