A Family Doctor's Tale - PECOMA CANCER

DOC I HAVE PECOMA CANCER 


 Actually this is one cancer which is seldom seen but I read about it in the journals. Because it was so interesting I have decided to write about it.

Perivascular epithelioid cell tumor (PEComa) is a neoplasm composed chiefly of HMB-45-positive(a marker which is relatively specific for melanoma) epithelioid cells with clear to granular cytoplasm and a perivascular distribution.
 
Such tumors have been reported in different organs

Although rare, only 20 + cases worldwide, PEC tumors (PEComas) should be regarded as a tumor of uncertain malignant potential. 

The median patient age is 46 years (range, 15-97 years).
There is a marked female predominance (22 females, 4 males).

Sites of involvement included
1.the omentum or mesentery (6 cases),
2.uterus (4 cases),
3.pelvic soft tissues (3 cases),
4.abdominal wall (2 cases),
5.uterine cervix (2 cases),
6.vagina,(1 case).
7.retroperitoneum,(1 case).
8.thigh,(1 case).
9.falciform ligament,(1 case).
10.scalp,(1 case).
11.broad ligament,(1 case).
12.forearm, (1 case).
13.shoulder,(1 case).
14.neck (1 case).

The tumors ranged from 1.6 to 29 cm in size (median, 7.8 cm).
Tumors were
1.epithelioid (N = 9),
2.spindled (N = 7), or
3.mixed (N = 10). 

Symptoms and Signs:
Among the common warning signs are:
1.weight loss,
2.pain in the bones
3.swelling of lymph nodes
4.Persistent fever
5.loss of appetite
6.generalised weakness and pallour

Causes of Pecoma  Cancer are unknown.
Certain genetic abnormalities may be present.
They are more common in women.

Diagnosis of Pecoma Cancer is by:
1.HMB-45-positive(a marker which is relatively specific for melanoma)is present in all cases
2.Perivascular epithelioid cell present on biopsy

Treatment of Pecoma Cancer:
The onset of PEComa Cancer has been known to be very rapid.
Because of its vascular origin, it can spread very fast.

Surgery will be useful if detected at the early stage.

Chemotherapy and radiation therapy are the main treatment for Pecoma cancer.

Besides these treatments, stem cells infusion also give patients and their families another source for a cure. 

The prognosis of Pecoma Cancer is:
Generally poor because onset is fast and spread diffuse .