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.

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.

Is neurofibroma Sox10 positive?

In summary, Sox10 shows an increased specificity for tumors of neural crest origin compared with S100: Sox10 was positive in only 5 of 668 cases (99% specificity) in nonschwannian, nonmelanocytic tumors, whereas S100 was positive in 53 of 668 cases (91% specificity).

What causes PEComa?

Perivascular epithelioid cell tumors (PEComas) are rare tumors driven by tuberous sclerosis complex gene mutations causing up-regulation of mTOR.

How curable is soft tissue sarcoma?

Most people diagnosed with a soft tissue sarcoma are cured by surgery alone, if the tumor is low-grade; that means it is not likely to spread to other parts of the body. More aggressive sarcomas are harder to treat successfully.

What is 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.

What does Sox10 positive mean?

Sox10 also marks normal myoepithelial cells of salivary gland and bronchial and mammary ducts and lobules, and it stains the myoepithelial component of salivary gland neoplasms such as myoepithelioma or pleomorphic adenoma. Sox10 labels both benign melanocytes (nevus cells) and the vast majority of melanomas.

What is Pecoma?

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 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 are the treatment options for PEComas?

Radical resection remains the primary treatment method for PEComas because these tumours are characterised by high resistance to radiation and chemotherapy.

Is SOX10 a good marker of melanoma?

SOX10: a useful marker for identifying metastatic melanoma in sentinel lymph nodes SOX10 (Sry-related HMg-Box gene 10) is a key nuclear transcription factor in the differentiation of neural crest progenitor cells to melanocytes. It has been shown to be a sensitive and specific marker of malignant melanoma of multiple histologic types.

What does SOX10 stand for?

SOX10 (Sry-related HMg-Box gene 10) is a key nuclear transcription factor in the differentiation of neural crest progenitor cells to melanocytes. It has been shown to be a sensitive and specific marker of malignant melanoma of multiple histologic types.

What are the signs and symptoms of PEComas?

PEComas are most often diagnosed in middle-aged women as extensive tumours locat More ed in the abdominal cavity or pelvis, manifesting as pain and complaints related to pressure on nearby organs.