Sporadic Desmoid-Type Fibromatosis Occurring at Laparoscopic Trocar Site: An Unusual Entity

Desmoids are known to occur in previous surgical scars. However, previous laparoscopic port site/trocar site is an extremely uncommon site for sporadic desmoids, with only two cases of sporadic laparoscopic trocar site desmoids (occurring in absence of FAP) reported in world literature. We thus describe a case of sporadic DF, occurring at the site of umbilical port 8 months following laparoscopic cholecystectomy for cholelithiasis, treated successfully by margin-negative resection. To the best of our knowledge, this appears to be the first such case in Indian literature and the first being reported after laparoscopic cholecystectomy. In the era of laparoscopic surgery, one must be aware of the existence of such an entity after laparoscopy as it may be confused with conditions such as metastatic deposit, port site tumour recurrence, port site hernia, scar site endometriosis, soft tissue sarcoma etc. and may result in diagnostic dilemma.

Radiation therapy (RT) is often used in the treatment of unresectable or recurrent aggressive fibromatosis (also known as desmoid tumor) typically with excellent local control. Prior reports have suggested that local control in pediatric patients with aggressive fibromatosis is poor. We aimed to report a long-term single-institution experience with the radiotherapeutic treatment of these tumors with a focus on age-dependent outcomes. A total of 101 patients treated with RT for aggressive fibromatosis between 1975 and 2015 at a single institution were identified. Overall survival was excellent (98% and 95% at 5 and 10 years, respectively); local control was likewise excellent (82% and 78% at 5 and 10 years, respectively). Patients aged <20 years at diagnosis had significantly worse 5-year local control than those aged >40 years at diagnosis (72% vs 97%; hazard ratio, 9.0; P = .009). RT for aggressive fibromatosis offers excellent local control and should remain the standard of care for patients with unresectable or recurrent disease. Younger patients have diminished local control relative to older patients, suggesting possible biological differences contributing to radioresistance in the pediatric and young adult population.

Spindle cell lesions of the breast cover a wide spectrum of diseases ranging from reactive tumor-like lesions to high-grade malignant tumors. The recognition of the benign spindle cell tumor-like lesions (nodular fasciitis; reactive spindle cell nodule after biopsy, inflammatory pseudotumor/inflammatory myofibroblastic tumor; fascicular variant of pseudoangiomatous stromal hyperplasia) and tumors (myofibroblastoma, benign fibroblastic spindle cell tumor, leiomyoma, schwannoma, spindle cell lipoma, solitary fibrous tumor, myxoma) is crucial to avoid confusion with morphologically similar but more aggressive bland-appearing spindle cell tumors, such as desmoid-type fibromatosis, low-grade (fibromatosis-like) spindle cell carcinoma, low-grade fibrosarcoma/myofibroblastic sarcoma and dermatofibrosarcoma protuberans.

We present a case of a recurrent sporadic intra-abdominal (mesenteric) desmoid tumor. Such an entity is rare with few cases reported in the literature. Treatment options regarding intra-abdominal desmoid tumors will be discussed.

A 55-year-old female patient reported with an intraoral well-localised asymptomatic swelling on the right side of the mandible in relation to the right mandibular first molar along with a history of trauma 6_months back. The microscopic examination revealed a well-circumscribed lesion consisting of spindle cells arranged in storiform pattern associated with the buccal cortical plate. Immunohistochemistry (IHC) revealed faint nuclear positivity for beta-catenin. The above findings led to our diagnosis of periosteal desmoid, which is rarely reported in the head-and-neck region. The major challenge in the diagnosis of such lesions is good clinicopathological correlation as the differential diagnosis of spindle-cell lesions is vast and needs IHC confirmation.

An extensive 35 _ 20 cm sized full-thickness abdominal wall defect was created after resection of aggressive abdominal fibromatosis in a 19-year-old male patient. Immediate reconstruction was not possible due to prolonged operation time and resulting severe bowel edema. The purpose of this study is to introduce our experience of extensive full-thickness abdominal wall reconstruction using only ipsilateral deep inferior epigastric vessels.