A case report of desmoid tumour—a forgotten aspect of FAP?

Xuereb, Sarah; Xuereb, Rachel; Buhagiar, Chiara; Gauci, Jonathan; Magri, Claude

doi:10.1016/j.ijscr.2016.11.052

Cited by 5 publications

(5 citation statements)

References 11 publications

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“…DTs usually occur in individuals between 15 and 60 years with a female preponderance [9]: 93% (n � 28) in our study and 67% in other series [10]. But, there is an equal incidence in males and females in DTs associated with FAP [11]. Pregnancy is associated with DTs due to high estrogenic levels [12].…”

Section: Discussionmentioning

confidence: 43%

The Management of Desmoid Tumors: A Retrospective Study of 30 Cases

Zenzri

Yahyaoui

Charfi

et al. 2020

International Journal of Surgical Oncology

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Objectives. Desmoid tumor also called aggressive fibromatosis is a rare type of benign tumor. It is a mesenchymal malignancy without metastatic potential. The standard management is resection, but other options including observation may be discussed. Desmoid-type fibromatosis may occur throughout the body, but the abdominal wall is the most common site. The aim of our study was to assess the clinicoepidemiological profile, prognostic factors, and treatment outcome of desmoid tumors. Methods. A monocentric retrospective study was conducted over a period of 19 years between February 2000 and November 2019 at the oncology department of Salah Azaïz Institute. Our study concerns 30 patients with desmoid tumor. All data regarding patients were obtained from the medical record. Results. Thirty patients were included. The median age was 35 years with a female predominance (sex ratio = 0.07). A palpable mass was the most common complaint (n = 27). Median tumor size was 5 cm. The principal site of involvement was the abdominal wall (n = 14). Surgery was performed in 27 patients. The histopathology reports listed 14 (52%) cases with negative margins and 13 (48%) cases with positive margins. Radiation therapy was performed in 2 patients. One patient received tamoxifen. Local recurrence occurred in 11 patients. Two patients died of their desmoid tumor. Abdominal wall tumors have less risk of recurrence compared with other sites (p=0.047). Macroscopic margin involvement (R2) was the only prognostic factor influencing disease-free-survival (p=0.034). Conclusion. Desmoid tumors are aggressive tumors with a tendency for local recurrence. Abdominal wall tumors have less risk of recurrence. Macroscopic margin involvement was the only prognostic factor that affects disease-free-survival.

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Section: Discussionmentioning

confidence: 43%

The Management of Desmoid Tumors: A Retrospective Study of 30 Cases

Zenzri

Yahyaoui

Charfi

et al. 2020

International Journal of Surgical Oncology

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“…The strategy of therapy which may be selected consists of surgery, pharmacotherapy or conservative treatment (follow-up). In some cases, the mass is considered unresectable and pharmacotherapy is selected (12). In conclusion, in this case, the desmoid tumor prevented oral intake of food by the patient.…”

Section: Discussionmentioning

confidence: 88%

A successful operation for giant intra‑abdominal desmoid tumors associated with familial adenomatous polyposis: A case report

et al. 2022

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Desmoid tumors are benign proliferations of spindle cells originating in fibro-aponeurotic tissue. Many patients with familial adenomatous polyposis (FAP) die from desmoid tumors, which can arise spontaneously but often appear to be surgically induced by prophylactic colectomy. Desmoid tumors are the second most common cause of death in patients with FAP, second to colorectal cancer. Many patients can live a long life with desmoid tumors without symptoms, but when symptoms (ranging from bowel or ureteric obstruction to bowel perforation with abscess and fistula) appear or there is a risk of functional impairment, a wide spectrum of therapies (local and systemic) are valuable in improving the symptoms and controlling the disease. A half-Japanese, half-Caucasian male, who had been diagnosed with intra-abdominal desmoid tumors associated with FAP at age 13, was treated using abdominal wall incision for decompression and chemotherapy from the age of 38. The therapeutic outcome was progressive disease, based on the modified response evaluation criteria in solid tumors (mRECIST), and when he visited our hospital at age 41 the desmoid tumor had invaded the small bowel with a fistula to the abdominal wall. We performed a palliative operation to improve his symptoms, which were fever, abdominal pain, vomiting, and difficulty eating. As the tumor was extremely large and had invaded the small intestine, massive resection including the small intestine was required. To prepare for anticipated massive bleeding, a balloon catheter was placed in the superior mesenteric artery just prior to surgery. Although the operation was extremely difficult, following surgery the patient regained his ability to eat and when discharged was ambulatory and without short-bowel syndrome. We report our experience treating one of the largest reported intraperitoneal desmoid tumors. Resection resulted in a good postoperative course, with improved quality of life and prognosis.

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“…[1][2][3] These tumors are common in familial adenomatous polyposis (FAP) and are characterized by their propensity for slow growth and invasion of adjacent organs, but do not show metastatic tendency. 4,5 Desmoid tumors causing bowel obstruction have been reported but remain a rare finding. 6 Surgical wide excision is currently the treatment of choice, with the goal of achieving free resection margins.…”

Section: Discussionmentioning

confidence: 99%

“…Most intra‐abdominal desmoid tumors affect small bowel mesentery, usually measure between 5 and 10 cm, and occur in a wide age range of patients without gender or race predilection 1‐3 . These tumors are common in familial adenomatous polyposis (FAP) and are characterized by their propensity for slow growth and invasion of adjacent organs, but do not show metastatic tendency 4,5 . Desmoid tumors causing bowel obstruction have been reported but remain a rare finding 6 .…”

Section: Discussionmentioning

confidence: 99%