How is PEComa treated?

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 malignant PEComa?

Malignant perivascular epitheliod cell tumor (PEComa) is a very rare entity composed of distinctive perivascular epitheliod cells with variable immunoreactivity for melanocytic and muscle markers. At present this neoplasm does not have a known normal cellular counterpart and the natural history is often unpredictable.

What does PEComa stand for?

Perivascular epithelioid cell tumour, also known as PEComa or PEC tumour, is a family of mesenchymal tumours consisting of perivascular epithelioid cells (PECs). These are rare tumours that can occur in any part of the human body.

What is a sugar tumor?

Histologically, clear cell “sugar tumor” consists of round or oval clear cells containing abundant membrane-bound glycogen. Some tumor cells may have a “spidery” appearance [1]. There is a mild variation in nuclear size and nuclear membranes, nucleoli may be prominent, but mitoses are usually absent.

What causes PEComa?

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 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 pulmonary Lymphangioleiomyomatosis?

Lymphangioleiomyomatosis (LAM) is a lung disease caused by the abnormal growth of smooth muscle cells, especially in the lungs and lymphatic system. This abnormal growth leads to the formation of holes or cysts in the lung.

Is PEComa genetic?

Perivascular epithelioid cell neoplasms (PEComa) are a family of rare mesenchymal tumors with hybrid myo-melanocytic differentiation. Although most PEComas harbor loss of function TSC1/TSC2 mutations, a small subset were reported to carry TFE3 gene rearrangements.

What is mesenchymal tumor?

Mesenchymal tissue neoplasms are soft tissue tumors, also known as connective tissue tumors, which are relatively frequent in domestic animals and have a high incidence in some species. These tumors may be located in all organs, with a higher or lower incidence in some tissues, as it will be shown.

Is LAM a tumor?

LAM is not cancer, but appears similar to other conditions in which benign tumors grow uncontrollably. LAM lung disease shares some features with a different condition called tuberous sclerosis.

Is LAM a terminal disease?

LAM is a progressive, frequently fatal, cystic lung disease that affects women almost exclusively, most often during childbearing years.

What is renal angiomyolipoma?

Angiomyolipoma (AML) is a benign renal neoplasm composed of fat, vascular, and smooth muscle. Two types are described: isolated angiomyolipoma and angiomyolipoma that is associated with tuberous sclerosis (see the images below). AML that is associated with tuberous sclerosis accounts for 20% of angiomyolipomas.