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.
Is 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.
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 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 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.
What happens when sarcoma spreads to the lungs?
The metastatic tumor is the same type of cancer as the primary tumor. For example, if soft tissue sarcoma spreads to the lung, the cancer cells in the lung are actually soft tissue sarcoma cells. The disease is metastatic soft tissue sarcoma, not lung cancer.
Can sarcoma in the lungs be cured?
Stage IV sarcomas are rarely curable. But some patients may be cured if the main (primary) tumor and all of the areas of cancer spread (metastases) can be removed by surgery. The best success rate is when it has spread only to the lungs.
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.
What are the radiologic findings characteristic of malignant PEComa of the lung?
According to most literatures, malignant PEComa of the lung also appeared radiologically as a solitary, well-defined nodule like benign clear cell sugar tumor, without cystic changes and calcification. Only one recently reported case showed spindle cell morphology and significant calcification.
What are the types of PEComa tumors?
PEComa tumors may be benign, malignant, or they may be of uncertain malignant potential The most common type of PEComas are angiomyolipoma (AML) and lymphangioleiomyomatosis (LAM) type of tumors. Other forms of PEComas are generally rare. These tumors are seen among a wide age range of individuals, usually adults
What is perivascular epithelioid cell tumors (PEComa)?
Perivascular Epithelioid Cell Tumors (PEComa) is an extraordinarily rare mesenchymal neoplasm especially the malignant type originating from the lung. To date, only 8 cases of malignant or malignant potential pulmonary PEComa had been documented. Firm diagnostic criteria for malignant pulmonary PEComa need urgently to be established.
What are the signs and symptoms of malignant PEComa?
The signs and symptoms of Malignant PEComa may include: In the initial stages, most tumors are small-sized and may not exhibit any signs and symptoms. The size can range from 5-30 cm. Large tumors can affect the functioning of the organ that is involved, such as the kidney or liver.
