Surgical Management of Desmoid Tumor of the Female Pelvis: A Case Report.

A 25 years married women having one child delivered vaginaly presented in the department of Obs & Gynae, Bangabandhu Sheikh Mujib Medical University, Bangladesh on April 2013 with pelvic pain and discomfort. On laparotomy a pseudocystic lesion 12×10cm in size was found over the right paracolic gutter and extending down into the pelvis involving the right parametrium. No abnormality was found in the uterus or tubes. The histological examination revealed a desmoidtumor of the pelvis. The patient’s recovery was uneventful.

The case is presented on 28-year-old female in her fifth week of gestation, in whom an abdominal wall tumour was found attached to left adnexa and uterus while performing a prenatal ultrasound. When she presented with abnormal uterine activity at 38.2 weeks of gestation, she was admitted and obstetrics decided to perform a caesarean section. Histopathology reported a desmoid fibromatosis. A contrast enhanced abdominal computed tomography scan was performed, showing a tumour of 26×20.5x18cm, with well-defined borders in contact with the uterus, left adnexa, bladder and abdominal wall, with no evidence of infiltration to adjacent structures. A laparotomy, with tumour resection, hysterectomy and left salpingo-oophorectomy, components separation techniques, polypropylene mesh insertion, and drainage was performed. There is no evidence of recurrence after 6 months follow-up.

There is continuing controversy over the significance of prognostic factors in sporadicdesmoid-type fibromatosis (DF). Further, only limited data is available in the literature concerning second recurrence in patients with recurrent DF. The purpose of this study was to identify prognostic factors influencing recurrence-free survival (RFS) and to determine rates of local recurrence (LR) in patients with primary and recurrent DF. Recurrence in primary and recurrent DF is characterized by different predictive factors and benefits from distinct therapeutic strategies. Immunohistochemical markers are valuable tools in the diagnosis of DF, although have limited potential in predicting the outcome.

We report an unusual case of a 9-month-old Japanese girl who prior to presenting at our hospital underwent debulking surgery twice with chemotherapy for desmoid-type fibromatosis of the tongue at another hospital. We performed a partial glossectomy and simultaneous reconstruction with local flap and achieved microscopic complete resection.

Patients were enrolled if they were treated with methotrexate and vinblastine chemotherapy for recurrences after surgical excision or newly diagnosed aggressive fibromatosis that was not amenable to surgical resection at the Korea University Medical Center from May 2008 to February 2016. Low-dose chemotherapy with methotrexate and vinblastine for 1 year was effective and well tolerated by adult patients with aggressive, recurrent fibromatosis.

R0 resection is the most effective treatment for chest AF. Postoperative radiotherapy did not reduce the recurrence rate.