Desmoid Type Fibromatosis of the Breast Masquerading as Breast Carcinoma: Value of Dynamic Magnetic Resonance Imaging and its Correlation (ENTIRE ARTICLE)

Desmoid type fibromatosis of the breasts is a rare stromal tumour of the breast that constitutes less than 0.2% of all breast tumours. Bilateral and multi-centric lesions are extremely rare, with only less than 10 cases reported in literatures. Although benign, it is locally aggressive with recurrence in up to almost one-third of the cases. This case of bilateral multi-centric breast fibromatosis was the first case in our centre. 19-year old woman, with a paternal aunt diagnosed with breast cancer at 30-year-old, presented to our institution with the chief complaint of retracted nipples for 1 year, she denied any history of trauma to her chest. Sonography showed bilateral suspicious hypoechoic masses. MRI was performed for further evaluation due to its extensive involvement of both breasts. The aim of this review is to illustrate the main clinical, radiological and histopathological characteristics of this rare disease to increase awareness of this entity and discuss the role of MRI.

Aggressive fibromatosis (AF) is a rare soft tissue tumor derived from mesenchymal tissue characterized by invasion into the surrounding muscle and soft tissue growth without distant metastasis. Although AF originates from the mesentery, omentum, and pelvis and has been described, primary AF originating from the colon has rarely been reported. In this case, an 18-year-old male patient with sudden left lower abdominal pain for 10 hours with nausea and vomiting was admitted to the hospital for an emergency medical examination. Imaging revealed a mass located near his sigmoid colon that was considered interstitial or malignant. The patient underwent urgent exploratory surgery, and perforation of the tumor was found. Histopathological examination showed that the tumor was colonic AF. Considering the patientÂ’s medical history of acute abdomen pain, we assume this to be a rare case of colonic AF with perforation.

Intra-abdominal desmoid-type fibromatosis (DF) can present sporadically, in sites of previous trauma, surgical scars and irradiation, or in association with familial adenomatous polyposis and Gardner syndrome. Intra-abdominal DF is uncommon and especially rare after a common surgery like cholecystectomy. We report a rare case of a 67-year-old male who presented with a locally aggressive intra-abdominal DF in the gallbladder fossa, status post cholecystectomy. This progressively enlarging infiltrative enhancing solid mass in the gallbladder fossa on serial computed tomography and magnetic resonance imaging demonstrated gastric outlet obstruction, biliary obstruction, portal vein narrowing and encasement of hepatic artery. Diagnosis of DF in this postoperative setting was delayed and challenging due to uncharacteristic clinical presentation.

This was a 44-year-old, para 2 (2 alive) who was referred to our gynaecology clinic from a secondary health facility on account of slowly increasing abdominal swelling of 2 years duration. There was no nausea, vomiting or diarrhea. There was associated mild to moderate dull lower abdominal pain that did not radiate to any other part of the body. There was no change in her monthly menstrual flow. Physical examination revealed a pelvic mass about 32 weeks pregnancy size and firm. Computed Topography scan showed a huge pedunculated sub-serous uterine fibroid. A huge mass adherent to the anterior surface of the body of the uterus was completely surgically excised at exploratory laparotomy. Histology of the excised tumour revealed a definitive histological diagnosis of pelvic desmoid tumour. The patient was clinically stable and discharged home 10 days post operation and was followed-up on out-patient gynaecology clinic basis. Pelvic desmoid tumour should be considered as a differential diagnosis in premenopausal women who present with abdominal swelling.

Intra-abdominal desmoid tumor is rare with no particular imaging features, so preoperative diagnosis is quite difficult. R0 resection is essential for treatment but often requires extensive surgical trauma, which can be a risk for recurrence. A laparoscopic approach for this tumor was effective, with the resulting diagnosis and resection being less traumatic. The first report of successful laparoscopic complete resection and reconstructive procedures were demonstrated.