Mesenchymal Tumors Can Derive from Ng2/Cspg4-Expressing Pericytes with β-Catenin Modulating the Neoplastic Phenotype.

The cell of origin for most mesenchymal tumors is unclear. One cell type that contributes to this lineages is the pericyte, a cell expressing Ng2/Cspg4. Using lineage tracing, we demonstrated that bone and soft tissue sarcomas driven by the deletion of the Trp53 tumor suppressor, or desmoid tumors driven by a mutation in Apc, can derive from cells expressing Ng2/Cspg4.

Herein, we report a 57-year-old woman who presented with recurrent epigastric pain and weight loss. An abdominal computed tomography scan showed an approximately 10-cm mass over the pancreatic head region and dilatation of the pancreatic duct. Tumor biopsy and bypass surgery were performed. A DT was confirmed on pathologic analysis. After resection, we prescribed treatment with the COX-2 inhibitor celecoxib. The patient showed complete remission and there was no local recurrence or distant metastasis within the 24-month follow-up period.

Only five cases of the desmoid tumors arising in the adult larynx have been reported in the English literature. In this case, repeated surgery and radiation were suspected as the causes. Also, the present report is the first to describe desmoid tumor co-existing with recurrent squamous cell carcinoma in the larynx.

The differential diagnosis of DTs is broad, and the diagnosis is often delayed due to nonspecific presentations. Immunohistochemistry is crucial in the accurate histological diagnosis, which guides treatment. Chemotherapy and radiation have a role in the management of both primary and recurrent lesions, but surgical resection remains the cornerstone of treatment.

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.