Facts About Perivascular Epithelioid Cell Tumor

Posted by pinkismith on November 14th, 2017

What is Perivascular Epithelioid Cell Tumors? They are basically mesenchymal neoplasms defined by the presence of immunohistochemically and histologically distinctive perivascular epithelioid cells. This tumor family is known to show marked female predominance and includes angiomyolipoma, lymphangioleiomyomatosis, clear cell sugar tumor, and a group of rare, morphologically and immunophenotypically similar tumors. These tumors often start from a variety of visceral and soft tissue locations. However, the perivascular epithelioid cell has no known normal tissue counterpart. Below are some facts you may want to know about this rare disease.

There are subsets of this disease that has been collectively termed perivascular epithelioid cell tumors.

Pecomas are usually composed of cells with clear to the granular eosinophilic cytoplasm and focal perivascular accentuation.

In most cases, the treatment that is used is wide excision.

Perivascular epithelioid cell tumors are usually benign, but a subset behaves in a malignant manner.

The disease is rare, and few malignant cases have been reported. However, strong criteria for malignancy have yet to be ascertained.

Perivascular epithelioid cell tumor has no known normal cellular counterpart at the moment, but research is ongoing to ascertain any possibility.

Differential diagnostic of Pecoma

It is challenging to diagnose epithelioid. In fact, epithelioid smooth muscle tumors are the most difficult differential diagnosis of PEComa-NOS. To this day, controversy still exists as to what the minimum criteria for the diagnosis of malignant Pecoma are. According to studies conducted on uterine tumors, there was a demonstration of epithelioid morphology, clear cell areas, etc. Some researchers have argued that these lesions represented epithelioid smooth muscle tumors which are entirely different from Pecomas. As previously mentioned, there are many subsets of Pecoma and these are responsible for the difficulty in properly diagnose and differentiate them.

Treatment

At this time, there is no optimal treatment. However, surgery is the main treatment that is often adopted to treat the disease. There are other forms of treatments being proposed. However, it is only by properly diagnosing the different subsets that a treatment method will be proposed. Since there are many subsets, it is believed that surgery help in most cases.

Surgery is also the main treatment for local recurrences and metastases. The effectiveness of other therapies for perivascular epithelioid cell tumor remains unclear.