Giant Desmoid Tumor of the Abdominal Wall Associated with Familial Adenomatous Polyposis

In this paper a case of vast desmoid tumor of the abdominal wall associated with familial adenomatous polyposis is reported. Desmoid tumors represent a particular type of fibrous neoplasms with a higher prevalence in females. They are extremely rare in the sporadic form, while they are found in about 10% of patients affected by familial adenomatous polyposis. Despite their benign histology and the absence of metastatic potential, they can be considered as fibrosarcomas with a low level of malignancy because of their locally invasive nature. The treatment of choice is surgical excision, as wide as possible, aimed at preventing recurrences, which are frequent in this tumor type. The usefulness of complementary therapies such as radiotherapy, hormone or chemotherapy, is not entirely clear.

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Cytoreductive strategy for multiple intra-abdominal and abdominal wall desmoid tumors in familial adenomatous polyposis: report of three cases

Clinical Journal of Gastroenterology ◽

10.1007/s12328-012-0330-5 ◽

2012 ◽

Vol 5 (5) ◽

pp. 361-366 ◽

Cited By ~ 1

Author(s):

Koji Tanaka ◽

Yuji Toiyama ◽

Yoshinaga Okugawa ◽

Junichiro Hiro ◽

Aya Kawamoto ◽

...

Keyword(s):

Familial Adenomatous Polyposis ◽

Abdominal Wall ◽

Desmoid Tumors ◽

Adenomatous Polyposis

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Recurrent giant cranial desmoid tumor in a 3-year-old boy with familial adenomatous polyposis requiring bifrontoparietal cranioplasty: case report

Journal of Neurosurgery Pediatrics ◽

10.3171/2016.6.peds15741 ◽

2016 ◽

Vol 18 (6) ◽

pp. 703-707

Author(s):

Luyuan Li ◽

John N. Jensen ◽

Sara Szabo ◽

Peter VanTuinen ◽

Sean M. Lew

Keyword(s):

Familial Adenomatous Polyposis ◽

Desmoid Tumor ◽

Genetic Disorder ◽

Aggressive Fibromatosis ◽

Colorectal Polyps ◽

Desmoid Tumors ◽

Adenomatous Polyposis ◽

Connective Tissues ◽

Total Resection ◽

Cranial Fasciitis

Desmoid tumors, also known as aggressive fibromatosis, are locally infiltrating musculoaponeurotic neoplasms arising in connective tissues. Desmoid tumors may be associated with familial adenomatous polyposis (FAP), a genetic disorder that presents with hundreds to thousands of precancerous colorectal polyps. The authors report the case of an 18-month-old boy who underwent resection of a right temporal desmoid tumor (initially diagnosed as cranial fasciitis) and developed a bilateral frontoparietal calvarial desmoid tumor 2 years later. The patient underwent gross-total resection of the tumor that required a large cranioplasty. He was subsequently diagnosed with FAP. The patient has been without tumor recurrence for 9 years afterwards and has not required revision of his cranioplasty. This is the first report describing a recurrent cranial desmoid tumor in a pediatric patient with FAP. The authors believe, however, that some of the cases previously reported as cranial fasciitis are likely desmoid tumors pathobiologically and genetically.

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A Case of Abdominal Wall Desmoid Tumor Following Pancoloproctectomy for Familial Adenomatous Polyposis with von Recklinghausen's Disease.

Nihon Rinsho Geka Gakkai Zasshi (Journal of Japan Surgical Association) ◽

10.3919/jjsa.62.2455 ◽

2001 ◽

Vol 62 (10) ◽

pp. 2455-2460

Author(s):

Isao OHORI ◽

Makoto SASAKI ◽

Tomoharu MIYASHITA ◽

Ken ASANO ◽

Tatsuya KIMOTO

Keyword(s):

Familial Adenomatous Polyposis ◽

Abdominal Wall ◽

Desmoid Tumor ◽

Von Recklinghausen’S Disease ◽

Adenomatous Polyposis ◽

Von Recklinghausen's Disease ◽

Recklinghausen's Disease ◽

Recklinghausen’S Disease

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Desmoid tumor in patients with familial adenomatous polyposis

Arquivos de Gastroenterologia ◽

10.1590/s0004-28032010000400010 ◽

2010 ◽

Vol 47 (4) ◽

pp. 373-378 ◽

Cited By ~ 11

Author(s):

Raquel Franco Leal ◽

Patricia V. V. Tapia Silva ◽

Maria de Lourdes Setsuko Ayrizono ◽

João José Fagundes ◽

Eliane M. Ingrid Amstalden ◽

...

Keyword(s):

Familial Adenomatous Polyposis ◽

Median Time ◽

Desmoid Tumor ◽

Treatment Options ◽

Tumor Resection ◽

Signs And Symptoms ◽

Desmoid Tumors ◽

Adenomatous Polyposis ◽

Antiinflammatory Drugs ◽

Surgical Data

CONTEXT: Desmoid tumors constitute one of the most important extraintestinal manifestations of familial adenomatous polyposis. The development of desmoids is responsible for increasing morbidity and mortality rates in cases of familial adenomatous polyposis. OBJECTIVES: To evaluate the occurrence of desmoid tumors in familial adenomatous polyposis cases following prophylactic colectomy and to present patient outcome. METHODS: Between 1984 and 2008, 68 patients underwent colectomy for familial adenomatous polyposis at the School of Medical Sciences Teaching Hospital, University of Campinas, SP, Brazil. Desmoid tumors were found in nine (13.2%) of these patients, who were studied retrospectively by consulting their medical charts with respect to clinical and surgical data. RESULTS: Of nine patients, seven (77.8%) were submitted to laparotomy for tumor resection. Median age at the time of surgery was 33.9 years (range 22-51 years). Desmoid tumors were found in the abdominal wall in 3/9 cases (33.3%) and in an intra-abdominal site in the remaining six cases (66.7%). Median time elapsed between ileal pouch-anal anastomosis and diagnosis of desmoid tumor was 37.5 months (range 14-60 months), while the median time between colectomy with ileorectal anastomosis and diagnosis was 63.7 months (range 25-116 months). In 6/9 (66.7%) patients with desmoid tumors, the disease was either under control or there was no evidence of tumor recurrence at a follow-up visit made a mean of 63.1 months later (range 12-240 months). CONCLUSIONS: Desmoid tumors were found in 13.2% of cases of familial adenomatous polyposis following colectomy; therefore, familial adenomatous polyposis patients should be followed-up and surveillance should include abdominal examination to detect signs and symptoms. Treatment options include surgery and clinical management with antiestrogens, antiinflammatory drugs or chemotherapy.

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Mesenteric desmoid tumor in the patient after restorative proctocolectomy due to familial adenomatous polyposis – a case report

Polish Journal of Surgery ◽

10.5604/01.3001.0011.8171 ◽

2018 ◽

Vol 90 (3) ◽

pp. 53-58

Author(s):

Adam Nałęcz ◽

Krzysztof Dębski ◽

Marek Dobosz ◽

Magdalena Krauze

Keyword(s):

Case Report ◽

Familial Adenomatous Polyposis ◽

Desmoid Tumor ◽

Familial Polyposis ◽

Case Reports ◽

Surgical Excision ◽

Individual Case ◽

Entire Body ◽

Adenomatous Polyposis ◽

Fibrous Tumor

Desmoid (desmoid tumor) is a cytologically benign fibrous tumor that originates from musculoskeletal structures of the entire body [1]. The term "desmoid", first introduced by Muller in 1838, derives from the Greek word desmos, which means tendinous [2]. The etiopathogenesis of desmoid is not fully understood, most reports of publications regarding its treatment are based on individual case reports. The prognosis is good, and healing is achieved mainly through surgical excision of the lesion. The aim of the paper is to present a case of a 33-year-old woman with diagnosed familial polyposis in the colon, who has been identified with mesenteric desmoid.

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Giant Intra-Abdominal Desmoid Tumor in a Young Male without History of Surgery, Trauma, or Familial Adenomatous Polyposis

Case Reports in Surgery ◽

10.1155/2018/9825670 ◽

2018 ◽

Vol 2018 ◽

pp. 1-5

Author(s):

Noritoshi Mizuta ◽

Kozo Tsunemi

Keyword(s):

Familial Adenomatous Polyposis ◽

Desmoid Tumor ◽

Treatment Plan ◽

Abdominal Cavity ◽

Beta Catenin ◽

Desmoid Tumors ◽

Adenomatous Polyposis ◽

History Of Surgery ◽

History Of ◽

Abdominal Desmoid Tumor

Desmoid tumors are rare, monoclonal myofibroblastic neoplasms that occur in the extremities, the trunk, and the abdominal cavity. We present a case that is significant for its rarity and for consideration of its treatment plan. A 17-year-old male reported swelling of his abdomen and abdominal pain. He was referred to our hospital with no history of surgery, trauma, or familial adenomatous polyposis. A large tumor in the abdominal cavity was detected by computed tomography, and surgical resection was performed. The tumor was thought to have developed from the anterior lobe of the transverse colon mesentery. It weighed 5.9 kg. Tumor cells with collagen fibers were observed in histopathological examination, but heteromorphism and the nuclear fission image were not apparent. Immunostaining revealed beta-catenin expression in the tumor cell nucleus. Diagnosis was an intra-abdominal desmoid tumor. Currently, there are no signs of recurrence. In this case, preoperative diagnosis was difficult, but surgery was the optimal treatment according to the symptoms. Desmoid tumors have invasive development and common local recurrence, so sufficient range of resection including normal tissue and strict follow-up are necessary.

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