Development Of Pecoma

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Perivascular epithelioid cell tumor can appear in any part of the body, but it is a rare disease. It is so rare that before 2010, only about 50 cases of Pecoma affecting different parts of the body had been reported in the literature. The tumors can affect both men and women of any age, but occurrences are more frequent among female. They usually range between 35 and 45 years of age.

To diagnose, a differential diagnosis could be applied, and these may include an inflammatory pseudotumor – it shares a similar situational and macroscopic appearance. However, an inflammatory pseudotumor is an internal accumulation of cells which are usually caused by concomitant infection or autoimmune disorder. Also, local necrotic areas may be detected. Inflammatory myofibroblastic tumors are not the same as an inflammatory pseudotumor. The former is a neoplasm with smooth muscle components, but the later is not. Also, an asymptomatic tumor has often been diagnosed incidentally through unrelated radiological tests.

Characteristics of Pecoma
There are many hypotheses regarding the cell of origin and possibilities, and these include neural crest, smooth muscle, and even pericytic. Characteristically, the tumor often manifests in a haphazard manner around a vascular lumen. Some cells surrounding the vessels are normally epitheliod and identified with their spindle-shaped and a clear to the pale granular cytoplasm. Pecoma is highly vascular and has thin-walled vessels that blend with the neoplastic cells easily. The neoplastic spindle cells themselves have a more eosinophilic cytoplasm. Immunostaining and granular characteristics that are consistent with melanocytic and smooth muscle.

What are symptoms of Pecoma?
Most symptoms of Pecoma are common with other diseases. However, symptoms of Pecoma affecting the lung are very uncommon. In one case, the tumor was detected as a result of left-sided thoracic pain after some rigorous physical exercise. In retrospection, the pain can possibly be explained by the actual location of the tumor which was on the chest wall.

In the case of the patient above, history and physical examination conducted initially suggested pneumothorax, which led to the process of radiological diagnostic. In some other cases, regular symptoms like fever and anemia are very common. In one reported case of Pecoma of the lung, the patient developed a severe haemothorax, which required immediate emergency treatment.
For the treatment, resection has often been applied due to its effectiveness at relieving Pecoma.

By: Christine carners

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Article Tags: AADI Bioscience

Submitted On Dec 06, 2017. Viewed 39 times.

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