Histopathological and radiological findings of desmoid tumor of abdominal cavity

In this article, we reported a 28-year-old female with a desmoid tumor of the anterior abdominal wall who underwent surgical resection. Preoperative evaluation was done using abdominal ultrasound, computed tomography, and magnetic resonance imaging. The histopathological examination of the resected specimen revealed a desmoid tumor. Because of the heterogeneity of the desmoid tumors, treatment needs to be individualized. However, complete surgical resection with negative margins seems to be the treatment of choice for this tumor entity and postoperative radiotherapy can help reduces the local recurrence rate.

Desmoid tumors can be safely managed with watchful waiting, including either observation alone or tamoxifen/NSAIDs. Surgery at first presentation can be associated with significant treatment burden. All diagnoses of desmoid tumor within the Alberta Cancer Registry from August 2004 to September 2015 were identified. Patients with F.A.P. were excluded. Demographics, tumor characteristics and treatment and outcome data were collected. Outcomes were compared between immediate surgery and watchful waiting. The effect of abdominal wall site on progression and recurrence and the effect of microscopic margin on recurrence were assessed with Fisher’s exact test. Watchful waiting was successful in 58% of patients, and a further 28% only required one aggressive treatment thereafter, for a total of 86%. Surgery had a favorable recurrence rate (15%), but some recurrences were associated with significant treatment burden. Treatment should be tailored to individual patients in a multidisciplinary setting. A trial of observation appears warranted in most patients. Recurrence rate was not affected by positive margins.

Desmoid tumors (DTs) of the head and neck have typically been classified as extra-abdominal, although the anatomic challenges of the head and neck warrant consideration of these DTs as a special entity. We present a review of DTs and describe our series of five patients with DTs of the head and neck treated within 2 decades. In our series of 5 patients with head and neck DTs, no patient experienced a recurrence during a median follow-up of 47.5 months (range 13 to 150), although all had positive histologic margins. The functional integrity of vital structures over meticulous radicality of the tumor resection must be considered, especially in the head and neck.

This case series is focused to determine the treatment outcomes for pediatric patients of aggressive fibromatosis. It is a retrospective case series conducted on 7 patients presented to section of Orthopedics, department of surgery of our institute in Karachi. Out of 7 pediatric patients, there were 6 (85.7%) males and 1 (14.3%) female patient. The median age was 6 years IQR (5-11) years. Gluteal region was the most common site of disease. Four patients (57.1%) had positive tumor margins while three (42.9%) had negative margins. Out of 7 patients, 4 patients (57.1%) had recurrent disease and they had positive margins. The median Disease Free survival time was 14 months and there was no expiry of patients. The conclusion of our study was that aggressive fibromatosis is more prevalent in children below 15 years of age and disease burden is higher in male gender. Positive margins after surgery indicate a high risk for disease recurrence therefore; primary surgery with negative margins is the treatment of choice for children with AF. However, we recommend that multicenter trials should be conducted in the future to clarify the role of adjuvant treatment for patients with pediatric AF.

Extra-abdominal desmoid fibromatosis, also known as aggressive fibromatosis, is a slow growing benign tumour that arises from fascia or aponeuroses of muscles. The tumour characteristically grows along fascial planes and can infiltrate subcutaneous tissues and muscles. The pattern of subcutaneous spread seen on ultrasound has been described to resemble a branching staghorn. This represents the local invasion (from the deeper tumour) along fibrous septa into the subcutaneous fat. On CT the tumour is of soft tissue density, similar to muscle, with variable density. On MRI the tumour is again heterogeneous reflecting the mixture of collagen, spindle cells and extra-cellular matrix.

The purpose of this study is to clarify the relationship of the staining pattern of beta-catenin with the CTNNB1 mutation status and various clinical variables, and investigate the significance of immunohistochemical staining of beta-catenin in cases diagnosed with DF. Between 1997 and 2017, 104 cases diagnosed with DF from 6 institutions in Japan were enrolled in this study. For all cases, immunohistochemical staining of beta-catenin and gene mutation analysis of CTNNB1 were performed. Of 104 cases, 87 (84%) showed nuclear staining of beta-catenin, and 95 (91%) positive staining in cytoplasm. The proportion of cases showing strong nuclear staining of beta-catenin was significantly higher in the cases with S45F than in those with T41A or wild type (WT). The proportion of cases stained strongly in the cytoplasm rather than in the nucleus was significantly higher in the group of T41A than that of S45F or WT. Among 17 cases in which nuclear immunostaining were absent, CTNNB1 mutation was observed in 5 cases (29.4%). There were unignorable cases of DF with negative beta-catenin immunostaining despite a definitive clinical and pathological diagnosis of DF and/or positive CTNNB1 mutation.