Primary Desmoid Tumor of the Small Bowel: A Case Report and Literature Review

Ebeling, Peter A.; Fun, Tristan; Beale, Katherine; Cromer, Robert; Kempenich, Jason W.

doi:10.7759/cureus.4915

Cited by 3 publications

(6 citation statements)

References 17 publications

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“…First, the pure mass effect of the growing tumor with compression of the surrounding structures causes symptoms such as ureteric obstruction with hydronephrosis, intestinal obstruction, and vascular or neural compression. Second, direct infiltration of the surrounding tissues can lead to ischemia, perforation, fistula formation in hollow organs, and gastrointestinal or intratumoral bleeding [3,[8][9][10][11][12].…”

Section: Discussionmentioning

confidence: 99%

“…In contrast to our case, for patients with asymptomatic, nonprogressive desmoid tumors the front-line approach is a dynamic monitoring with a type of wait-and-see policy. This strategy is applicable because of the possibility of self-limitation of the tumors, even their spontaneous regression [5,8,9].…”

Section: Discussionmentioning

confidence: 99%

“…When a symptomatic, resectable desmoid tumor is diagnosed, especially in cases with an intraabdominal location, the treatment is mainly surgical, and the purpose is complete tumor removal with negative microscopic margins [5,8,9]. Therefore, the intraoperative frozen sections are useful for evaluating the resection margins [16].…”

Section: Discussionmentioning

confidence: 99%

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Ruptured desmoid tumor imitating acute appendicitis – a rare reason for an emergency surgery

et al. 2019

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Background: Desmoid tumors, also known as aggressive fibromatosis, are extremely rare, accounting for less than 3% of soft-tissue sarcomas and less than 0,03% of all neoplasms. The diagnosis is usually delayed because of the lack of specific symptoms, and can sometimes lead to serious and, even fatal complications. Case presentation: We report the case of a 27-year-old male patient presenting with the clinical picture of acute appendicitis. During the operation, we found a tumor in the jejunum with a necrotic zone and perforation on its surface, causing hemorrhagic effusion into the abdominal cavity and subsequent peritonitis. The tumor was removed with negative margins via resection of the small bowel. The final histological result showed aggressive fibromatosis. Conclusions: Aggressive fibromatosis remains a serious problem with the possibility of locally aggressive behavior with high rates of recurrence. Sometimes, its clinical and macroscopic recognition can be immensely tricky. As shown by our patient, on rare occasions, desmoid tumors can lead to acute surgical abdomen requiring an emergency operation.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Ruptured desmoid tumor imitating acute appendicitis – a rare reason for an emergency surgery

et al. 2019

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“…Other risk factors include abdominal surgery/trauma and women of childbearing age due to the influence of estrogen on desmoid tumor growth [ 8 ]. DTFs can occur anywhere in the body, most commonly in the abdominal wall and extremities, and rarely from the abdominal organs or mesentery [ 9 ]. While standard of care for resectable malignant peritoneal mesothelioma is cytoreduction and heated intraperitoneal chemotherapy (HIPEC), treatment for DTF ranges from observation to surgery.…”

Section: Introductionmentioning

confidence: 99%

Desmoid-type fibromatosis in the setting of malignant peritoneal mesothelioma: a case report of two rare diseases

et al. 2022

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Background Desmoid-type fibromatosis (DTF) is a rare benign lesion that usually arises from the abdominal wall or extremities and rarely from the mesentery or intrabdominal organs. Malignant peritoneal mesothelioma is also a rare, yet aggressive disease. To our knowledge, this is the first case report of desmoid-type fibromatosis in the setting of malignant peritoneal mesothelioma. Case presentation An early 30-year-old female was referred to our center for large intra-abdominal mass concerning for recurrent malignant peritoneal mesothelioma after previous cytoreductive surgery with hyperthermic intraperitoneal chemotherapy and adjuvant chemotherapy. Further investigation revealed a large mesenteric mass, which was resected en bloc with the cecum and terminal ileum. Pathologic findings confirmed a surprising diagnosis of desmoid-type fibromatosis. Conclusions No adjuvant therapy was offered to this patient due to negative tumor margins; however, close follow-up will be provided for recurrence of both malignant peritoneal mesothelioma and desmoid-type fibromatosis, which can be differentiated in the future via biopsy in this patient.

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“…These tumors most commonly affect young adults aged [25][26][27][28][29][30][31][32][33][34][35], and have a female-to-male preference of 2:1 (6). DTF can occur anywhere in the body, but they most commonly arise from the abdominal wall or extremities and rarely from the abdominal organs or mesentery (7). We present the workup and management of an unusual case of a 24-year-old male with a large sporadic 31 × 25 × 12 cm retroperitoneal desmoid tumor arising from the ileocolic mesentery requiring surgical intervention due to mass effect on abdominal organs.…”

Section: Introductionmentioning

confidence: 99%

A Large Sporadic Intra-abdominal Desmoid-Type Fibromatosis in a Young Male: A Case Report

et al. 2020

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Desmoid type fibromatosis (DTF) is a rare benign tumor of connective tissue origin. While these tumors are typically not malignant, they can exhibit aggressive growth patterns causing mass effect on surrounding organs. These tumors typically present in the extremities and abdominal wall, rarely occurring in the mesentery, and abdominal organs. Due to the rarity of this tumor and the variable size and origin, it is difficult to provide exact prognosis, recurrence, and treatment efficacy regarding desmoid tumors arising from the ileocolic mesentery. We present a case of a young male with a sporadic 31 × 25 × 12 cm DTF arising from the ileocolic mesentery that was complicated by mass effect on bowel and intra-abdominal organs requiring surgical intervention. On presentation, the patient exhibited weight gain and abdominal pressure. Abdominal distension without tenderness on palpation was noted on physical examination. The tumor biopsy confirmed the diagnosis of DTF. No evidence of familial adenomatous polyposis or Gardner syndrome was identified. The tumor was surgically excised and intimately associated with the bowel requiring ileocolonic resection with primary anastomosis. At 3-months follow up, surveillance MRI showed no residual or recurrent lesion. A multidisciplinary approach to this patient's diagnosis and treatment allowed for an accurate diagnosis, efficient treatment, and follow up plan.

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Primary Desmoid Tumor of the Small Bowel: A Case Report and Literature Review

Cited by 3 publications

References 17 publications

Ruptured desmoid tumor imitating acute appendicitis – a rare reason for an emergency surgery

Ruptured desmoid tumor imitating acute appendicitis – a rare reason for an emergency surgery

Desmoid-type fibromatosis in the setting of malignant peritoneal mesothelioma: a case report of two rare diseases

A Large Sporadic Intra-abdominal Desmoid-Type Fibromatosis in a Young Male: A Case Report

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