Desmoid Infilterating Ileum, a Rare Complication

Vallabha, Tejaswini; Sindgikar, Vikram; Baloorkar, Ramakanth; Dhamangoankar, Mandar; Karjol, Uday

doi:10.1007/s12262-012-0564-y

Cited by 3 publications

(9 citation statements)

References 3 publications

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“…Intra-abdominal desmoid tumours represents a type of fibroblastic proliferation with a tendency for local invasion, a high recurrence rate and no metastatic potential. 1 Based on the location, desmoid tumours are divided into extra-abdominal, intra-abdominal and abdominal wall desmoids. 1 Among all groups, intra-abdominal desmoids have the highest local recurrence rates.…”

Section: Discussionmentioning

confidence: 99%

“…1,2 It is predominantly a benign tumour without metastatic potential, but it has a local aggressive effect on surrounding structures and it is associated with a high recurrence rate. 1,3 Desmoid type fibromatosis (DTF) is radiologically difficult to distinguish from mesenchymal tumours, which have malignant potential, so misdiagnosis may lead to inadequate therapy. 2 This current case report describes a 37-year-old female patient with epigastric pain and a palpable abdominal mass caused by DTF.…”

Section: Introductionmentioning

confidence: 99%

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Aggressive fibromatosis of the right colon mimicking a gastrointestinal stromal tumour: a case report

et al. 2021

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Aggressive fibromatosis is a rare type of intra-abdominal desmoid tumour that usually involves the small bowel mesentery. It is a locally-invasive lesion, with a high rate of recurrence, but without metastatic potential. Aggressive fibromatosis is seen more often in young female patients. This case report presents the radiological, intraoperative and histopathological findings from a 37-year-old female patient that presented with epigastric pain and a palpable mass in the right hemiabdomen. Histological and immunohistochemical examinations of the resected tumour, including positive staining for beta-catenin, confirmed a postoperative diagnosis of desmoid type fibromatosis. This specific case showed that desmoid type fibromatosis of the colon can mimic gastrointestinal stromal tumours (GIST) based on its clinical presentation, computed tomography and magnetic resonance imaging findings. Differential diagnosis between desmoid type fibromatosis and GIST is clinically very important due to the different treatments and follow-up protocols that are implemented for these lesions.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Aggressive fibromatosis of the right colon mimicking a gastrointestinal stromal tumour: a case report

et al. 2021

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“…According to their location, desmoid tumors are divided into extra-abdominal, intraabdominal, and in the abdominal wall [3]. Church (1995) classified them into four groups: tumors that resolve spontaneously (10% of cases), tumors with cycles of progression and resolution (approximately 30%), tumors that remain stable (approximately 50%) and neoplasms with rapid, aggressive progression (10%) [6].…”

Section: Discussionmentioning

confidence: 99%

“…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%

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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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A rare cause of small bowel obstruction caused by duodenum-derived aggressive fibromatosis with β-catenin T41A mutation: A case analysis

Zhao,

Wang

2024

Medicine

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Rationale: Aggressive fibromatosis (AF) is a fibroblastic/myofibroblastic tumor known for its locally aggressive properties. Intra-abdominal AF primarily occurs in the small intestine mesentery, ileocolic mesocolon, omentum, retroperitoneum, and pelvis, and rarely originates from the intestinal wall. Here, we report a rare case of small bowel obstruction caused by duodenum-derived AF with β-catenin (CTNNB1) T41A mutation. Patient concerns: A 35-year-old male had a 4-month history of abdominal pain, nausea, and vomiting, which gradually worsened over time. Diagnoses: Based on the results of CT examination, histopathology and Sanger sequencing, the patient was diagnosed with small bowel obstruction caused by duodenum-derived AF. Interventions: Due to the extensive adhesion between the tumor and surrounding tissue, it is extremely challenging to completely remove the tumor through surgical resection with negative margins in this case. In order not to damage the function of surrounding vital organs, gastrojejunostomy was performed to relieve the symptoms of small bowel obstruction. Outcomes: The patient experienced a successful recovery. It is important to note that this patient is still at risk of local recurrence and requires regular follow-up. Lessons: The best treatment should be taken based on the individual patient to relieve symptoms and improve quality of life. Moreover, histopathology plays a crucial role in diagnosing and differentiating duodenum-derived AF. The detection of mutations in exon 3 of the CTNNB1 has become strong evidence for diagnosing duodenum-derived AF.

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Desmoid Infilterating Ileum, a Rare Complication

Cited by 3 publications

References 3 publications

Aggressive fibromatosis of the right colon mimicking a gastrointestinal stromal tumour: a case report

Aggressive fibromatosis of the right colon mimicking a gastrointestinal stromal tumour: a case report

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

A rare cause of small bowel obstruction caused by duodenum-derived aggressive fibromatosis with β-catenin T41A mutation: A case analysis

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