Aggressive Fibromatosis of the Chest Wall Mimicking Low Grade Fibrosarcoma – An Unusual Clinical Presentation

Patient presented with a progressively increasing large swelling on the chest wall since 2 years. On examination, firm to hard ill defined growth was found. Microscopic examination showed a cellular spindle cell tumour. Strong positivity for VIMENTIN and focal positivity for SMA and DESMIN was seen on Immunohistochemistry. Final Diagnosis of aggressive fibromatosis was made.

Papillary thyroid carcinoma (PTC) with desmoid-type fibromatosis (DTF) is characterized by genetic alterations of the fibroblasts. This report aims to describe the clinical, pathological, and immunohistochemical findings in 14 cases of PTC-DTF and to clarify the diagnostic significance of the variant. The patients included 9 women and 5 men, with a mean age of 49.3 years. PTCs with focal DTF components and with extensive DTF components included 7 cases each. On aspiration cytology, 12 cases were reported as suspicious for malignancy or malignant, and schwannoma or fibroma was suggested in 1 case each. The DTF components were histologically classified into 4 types, namely, central (4 cases), peripheral (1 case), mixed (7 cases), and diffuse type (2 cases). The stromal components were consistent with those of DTF. Immunohistochemically, fibroblasts in the DTF components showed nuclear and cytoplasmic expression for beta-catenin in 12 cases. We agree that PTC with nodular fasciitis-like stroma should be renamed to PTC-DTF.

A 19-year-old woman presented with painless swelling of the abdomen. During surgery, a giant mass measuring 37cmx26cmx12cm within the distal pancreas invading the spleen was noted. Distal pancreatectomy, splenectomy, and debulking surgery were performed. Histological examination showed that the tumor infiltrated the spleen and pancreatic parenchyma, and sections of the solid areas revealed a proliferation of spindle-shaped or stellate cells growing in fascicular and storiform patterns within a myxoid intercellular matrix. Cystic areas were representing the entrapped excretory pancreatic ductules. Interestingly, there were two ectopic adrenal tissues found incidentally in the peripheral portion of the tumor. The histopathologic and immunohistochemical features were consistent with a solid cystic desmoid tumor of the pancreas. Desmoid tumors of the pancreas are very rare, and if they present as a solid cystic lesion, their diagnosis may be difficult. We report the case for its rarity and huge size and to emphasize a regular follow-up because the long-term prognosis is currently unknown.

A 63-year-old woman with FAP and previous colectomy was in December 2012 referred for severe pain in her right shoulder. An MRI revealed a tumor measuring 7.2 x 2.2 cm in close relation to the right scapula.

Intraabdominal fibromatosis is a rare benign tumor that often affects the mesentery or retroperitoneum, and can infiltrate adjacent organs. However, it is rare for fibromatosis to arise from the small intestinal wall. A 27-year-old female with a tangible abdominal tumor is described. The computed tomography (CT) scan revealed a 7.5 cm, small intestine-associated tumor in the right abdomen. The patient received tumor resection and intestinal anastomosis. Further pathological examination confirmed the tumor as a duodenal fibromatosis that infiltrated the intestinal wall. To the best of our knowledge, this is the first report of a duodenum-derived fibromatosis that invaded the muscular layer of the intestine.

We report the case of a 16-year old patient presenting with giant, multinodular, mesenchymal tumor of the abdominal wall occupying the left abdominal region and measuring 25 cm on the vertical axis, 20 cm on the transverse axis, mobile when compared with the deep structures and gradually increasing in volume over childhood and neglected. After small biopsy, which showed desmoid tumor, the patient underwent complete surgical resection of the tumor with immediate reconstruction by free muscolo skin flap of the latissimus dorsi attached to the large blood vessels of the inguinal fold (left iliac artery and left external iliac vein), connected by termino lateral anastomosis. Flap survival was correctly performed and reconstruction was successful.