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Aggressive Angiomyxoma in a Young Lady

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Aggressive Angiomyxoma in a Young Lady

Ali AAEM, Sharaf Eldeen AKS, Hussein SM, Gerais A

University of Khartoum, Faculty of Medicine, Khartoum, Sudan 

Angiomyxoma is a rare mesenchymal tumor, with unknown aetiological factors. It commonly arises in the lower pelvis of   women in their reproductive years.
 It is very similar to a myxoma but with a notable vascular component, hence the name. It displaces and grows around structures rather than infiltrating  
them,has a high potential to recur locally but does not metastasise. (1)
Patients often present with an asymptomatic slow-growing mass.
The hallmark in histopathology is vessels of varying calibre haphazardly 
 scattered throughout the tumour     parenchyma, whereas mitotic figures are scarce.(2)
The tumor cells are characteristically positive for estrogen and progesterone receptors, suggesting a hormonal role in the development of the tumor.
Chromosomal translocation has also been described.
Surgery is the treatment of choice, despite the increased associated morbidity.
Treatment with GNRH agonists is an emerging therapy with good response, owing to the hormone-dependent growth. (1)
A single,32 years old lady presented with abdominal pain and swelling, which started 2 months earlier.  
She was diagnosed in a rural hospital, as a fibroid, underwent laparotomy and found to be a retro pubic mass, not removed.
A CT scan showed an ovarian mass, laparotomy done again. A biopsy was taken, leaving the mass in place.
Then she came to our clinic, but with no reports of histopathology.
U/S scan reported a sub serous fibroid. Another abdominal CT showed a normal uterus and a mass posteriorly in the pouch of Douglas.
An angiogram was suggested.
A third laparotomy was done to find a big mass posterior to the  bladder and attached to the pubic bone.
Mass was removed but surgery was complicated by bladder injury and massive haemorrhage, managed properly.
Postoperatively, the patient developed sepsis, treated and discharged after a week in a good condition.
Histopathology reports concluded a benign Angiomyxoma.
Patient came again in 35 days, with mild suprapubic tenderness/S showed a small retro pubic haematoma, managed conservatively.
Generally, the patient is in a good condition, asymptomatic and with good control of micturition.
This case illustrates the probability of rare tumours arising and giving symptoms, signs and radiological  findings suggestive of the more common
pelvic tumours. In addition, the increased morbidity associated with the abnormal growth and recurrence of these benign tumours make
them comparable to malignant ones.
Soft tissue tumors: Aggressive Angiomyxoma, Atlas of Genetics and Cytogenetic in Oncology and Haematology.

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