Sino-orbital desmoid tumor in a pediatric patient – Case report with review of literature

We report a case of a 2-year-old female who presented with bilateral progressive proptosis, visual loss, nasal obstruction, and breathing difficulty. Magnetic resonance imaging revealed a large sino-orbital mass that was extending to the orbital apex and skull base. An initial diagnosis of rhabdomyosarcoma was made elsewhere on the basis of the presence of round and spindle cell tumor. Subsequent biopsy with immunohistochemical staining was positive for nuclear staining with β-catenin, shifting the diagnosis to a myofibroblastic tumor, favoring desmoid-type fibromatosis. With image guidance, near complete excision of tumor was performed by a multidisciplinary team, while respecting danger zones such as the skull base and the optic nerve. Following a recurrence over 2 months, additional excision was performed with a 6-month treatment of methotrexate and vinblastine.

We present the case of a 25-year-old female with a history of palpable breast mass who was evaluated with ultrasound, diagnostic mammography, MRI, and CT. Ultrasound-guided biopsy revealed fibromatosis, and MRI ultimately revealed that the mass was arising from the pectoralis major muscle and extensively involved the chest wall.

Desmoids are rare soft-tissue tumors of the abdominal wall that may sporadically occur extra-abdominally. It manifests as clonal fibroblastic proliferation with an infiltrative tendency and capacity to recur without metastasizing. An adolescent male presented with a gradually increasing globular, non-tender, firm, non-pulsatile swelling (8 × 5 × 3 cm3) in the left popliteal fossa that had been present for five months. Following thorough investigation with imaging and Tru-cut biopsy, finally, an excisional biopsy was done. Histopathological examination confirmed a desmoid tumor, and the patient received adjuvant radiotherapy. At the one-year postoperative follow-up, there was no recurrence; the patient had been explained the prognosis.

We present a case of a desmoid tumor recurrence in a patient with a history of a resected desmoid tumor of the right neck area with free surgical margins six months earlier. The neoplasm was found to invade the parapharyngeal space, and wide excision was performed including most of the sternocleidomastoid muscle, the thrombosed internal jugular vein, and the infiltrated spinal accessory nerve. The histopathologic findings displayed free microscopic margins, with close margins at the site of the parapharyngeal space extension. After 3 months, there was no sign of tumor recurrence. After 6 months, local tumor recurrence was identified on clinical examination and imaging. The decision of the Oncology Board was further treatment with radiotherapy. Response to treatment was satisfactory, and the patient was on close follow-up for twelve months. Appropriate patient counseling and close follow-up are warranted in all cases.

A 46-year-old female patient presented to our clinic with postprandial nausea and vomiting. A contrast-enhanced abdominal computerized tomography revealed a mass lesion with a size of 100 mm × 80 mm which originated from the distal pancreas and compressed the gastric pilor externally. Upon exploration the distal part of duodenum, proximal jejunum, and pancreatic mass were noted to form a conglomerated structure. Therefore, the fourth part of the duodenum, a 25 cm part of the proximal jejunum, distal pancreas, and the spleen were excised en-bloc. The pathology report of the specimen indicated fibromatosis with a diameter of 55 mm that originated from the muscularis propria of the duodenum and extended into the pancreatic parenchyma. There was also an incidentally detected 10 mm paraduodenal hydatid cyst. No tumor recurrence was detected at a follow-up period of 24 mo. In conclusion, the most ideal treatment of desmoid-type fibromatosis is surgical resection of the mass lesion with clean surgical borders. Although rare, this tumor may originate from the intestinal wall. Histopathological verification is of great significance for a proper diagnosis.