Perivascular epithelioid cell tumour

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Perivascular epithelioid cell tumour
Renal angiomyolipoma, a type of PEComa
Synonyms	PEComa
Pronounce	N/A
Specialty	N/A
Symptoms	Variable, depending on location; may include pain, bleeding, or mass effect
Complications	Potential for malignancy
Onset	Variable
Duration	Chronic
Types	Angiomyolipoma, Clear-cell "sugar" tumor, Lymphangioleiomyomatosis
Causes	Unknown
Risks	Tuberous sclerosis complex
Diagnosis	Histopathology, Immunohistochemistry
Differential diagnosis	Sarcoma, Melanoma, Carcinoma
Prevention	N/A
Treatment	Surgical resection, Targeted therapy
Medication	mTOR inhibitors
Prognosis	Variable; depends on type and location
Frequency	Rare
Deaths	N/A

Perivascular epithelioid cell tumour (PEComa) is a rare type of tumor that is derived from perivascular epithelioid cells (PECs). These tumors can occur anywhere in the body, but are most commonly found in the kidney, liver, lung, and uterus.

History

The term "PEComa" was first proposed by Zamboni et al. in 1996 to describe a group of tumors with similar histological characteristics. These tumors were initially recognized due to their unique co-expression of melanocytic and smooth muscle markers.

Pathology

PEComas are characterized by the presence of perivascular epithelioid cells. These cells are unique in that they express both melanocytic and smooth muscle markers, a feature that is not seen in other types of tumors. The exact origin of PECs is still a matter of debate, but they are believed to originate from a common precursor cell that has the ability to differentiate into both melanocytes and smooth muscle cells.

Clinical Presentation

The clinical presentation of PEComas can vary widely depending on the location of the tumor. Symptoms can range from asymptomatic to severe, depending on the size and location of the tumor. In some cases, the tumor may be discovered incidentally during imaging studies for unrelated conditions.

Diagnosis

Diagnosis of PEComas is typically made through a combination of imaging studies, histopathological examination, and immunohistochemical staining. Imaging studies such as computed tomography (CT) or magnetic resonance imaging (MRI) can help to identify the location and size of the tumor. Histopathological examination of a biopsy sample can confirm the presence of PECs, and immunohistochemical staining can help to confirm the diagnosis by demonstrating the co-expression of melanocytic and smooth muscle markers.

Treatment

Treatment of PEComas typically involves surgical removal of the tumor. In some cases, chemotherapy or radiation therapy may also be used, particularly if the tumor is large or if it has spread to other parts of the body.

Prognosis

The prognosis for individuals with PEComas can vary widely depending on factors such as the size and location of the tumor, the individual's overall health, and the success of treatment. In general, however, PEComas are considered to be a type of low-grade malignancy, which means that they tend to grow slowly and are less likely to spread to other parts of the body compared to other types of tumors.

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