Efficacy of low-dose chemotherapy with methotrexate and vinblastine for patients with extra-abdominal desmoid-type fibromatosis: a systematic review

This systematic review aims to evaluate the efficacy of low-dose chemotherapy with methotrexate and vinblastine for patients with extra-abdominal desmoid-type fibromatosis. We searched the pertinent literature from January 1990 to August 2017. The search yielded 40 studies, 9 of which were included after the first and second screenings. There were three prospective case series but no randomized controlled trials among the nine studies. There was no case-control report (vs. no treatment). According to Response Evaluation Criteria in Solid Tumors criteria, the mean response rate (complete remission or partial response) was 36% (11-57%). Including stable disease, namely, clinical benefit was consistently as high as 85% (69-100%). Mean adverse event rate of G3 or G4 according to CTCAE was 31%. One study reported improvement of pain (87.5%) because of this chemotherapy. The efficacy of this chemotherapy was convincing. However, the overall evidence was weak, and this chemotherapy is not covered by insurance in Japan; we only weakly recommend low-dose chemotherapy with methotrexate and vinblastine in patients with extra-abdominal desmoid-type fibromatosis.

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.