Invasive and the largest pancreatic tumour of Rare Intraabdominal Desmoid-type fibromatosis with curative resection: case report

fibromatoses (Desmoid-type) tumour is rare benign fibrous tumours account approximately for 0.03% of all neoplasms. The Pancreas origin of this tumour is even more rarely reported subset with around 5% only. Establishing the diagnosis is fundamentally based on characteristic pathological and immunohistochemical studies. Surgical resection of the tumour is the mainstay of intervention. We aim to report the success in cure a rare and the largest pancreatic desmoid-type intraabdominal fibromatosis.

We report a case of a 41-years-old female with a sporadic abdominal wall desmoid tumor. After a period of 12-months observation, chemotherapy was given with Doxorubicin and Dacarbazine to facilitate minimal surgery. The patient received 4 cycles of chemotherapy. Treatment was well tolerated and resulted in a significant reduction of the tumor size from 48mm to 25mm, 48% reduction. The tumor was surgically resected, and the histopathology from the resected specimen revealed a partial pathological response. At 6 months post-surgery, there is no recurrence or surgical complications.

Desmoid tumors (DT) are rare non-metastatic neoplasms that occur through myofibroblast proliferation in musculoaponeurotic or fascial structures of the body, being commonly diagnosed in young women during pregnancy or in the post-partum period. We present the case of a 38-year-old woman, who recently gave birth, manifesting non-specific abdominal symptoms. Computed tomography indicated the presence of a solitary tumor arising from the intestinal wall or from the mesentery. Surgery confirmed the diagnosis, revealing a tumor that was localized at the level of the jejunal mesentery, having about 7 cm in diameter, in tight contact with the duodenum and the mesenteric vessels. “En bloc” resection of the tumor was performed, together with the involved enteral loops followed by end-to-end anastomosis of the jejunum. Histopathological examination of the surgical specimen sustained the diagnosis of desmoid tumor.

Here we present a 78-year-old male with desmoid-type fibromatosis arising in the dermis of the right medial calf with a pathogenic mutation in CTNNB1 and a variant of unknown significance in APC.

We made a pathological diagnosis gross total tumor resection by the international collaboration with Myanmar, Cambodia, and Japan. The case was a 5-year-old Burmese boy. A recurrent growing tumor was found in his right buttock, which caused gait disturbance and aesthetic problems. In order to remove this giant desmoid fibromatosis (DF), the oncologist consulted our department. Because DF had localized outside the pelvic ring, it could be resected with the embolization of the branch of the internal iliac artery. In operation, the sciatic nerve was preserved and a gross total resection was performed with R1 surgery. The patient is alive without any functional disorders and recurrences and is maintaining a good QOL. This is the first report on treating DF in Myanmar by an international collaboration team.

This paper aimed to (1) to compare the outcomes of extra-abdominal desmoid tumor fibromatosis (DTF) after different treatment modalities, (2) to assess prognostic factors for recurrence following surgical excision, and (3) to assess prognostic factors for progression during observation. This was a retrospective multicenter study under the patronage of the European Musculoskeletal Oncology Society (EMSOS). Three hundred eighty-eight patients (240 female, 140 male) with a mean age of 37.6 (±18.8 SD, range: 3-85) were included in the study. Two hundred fifty-seven patients (66%) underwent surgical excision of ADF, 70 patients (18%) were observed without therapy, the residual patients had different conservative treatments. There were no significant differences in terms of tumour recurrence or progression between the different treatment groups. After surgical excision, younger age, recurrent disease and larger tumour size were risk factors for recurrence, while tumours around the shoulder girdle and painful lesions were at risk of progression in the observational group. Local recurrence rate after surgery was similar to progression rates under observation. Hence, observation in DTF seems to be justified, considering surgery in case of dimensional progression in 2 consecutive controls (3 and 6 months) and in painful lesions, with particular attention to lesions around the shoulder girdle.