Desmoid tumors (deep fibromatosis) of the mesentery are rare mesenchymal tumors. They are often misdiagnosed, especially with a previous history of resection for gastrointestinal stromal tumor (GIST). Immunohistochemistry can help differentiate between these two tumors. In this article, we present a case we had encountered: a Desmoid tumor developing in a patient with a history of GIST 3 years ago. It is the first case of GIST with subsequent development of Desmoid tumor to be reported in Saudi Arabia. We discuss the two entities of Desmoid tumor and GIST by comparing their definitions, clinical presentations, histological features, immunohistochemistry stains, molecular pathogenesis, prognosis, and treatment. We also discuss the relationship between GIST and the subsequent development of Desmoid tumors and compare our case with case reports in literature.
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http://dx.doi.org/10.1155/2018/1082956 | DOI Listing |
BJR Case Rep
January 2025
Dubai Health, Dubai 1853, United Arab Emirates.
Desmoid tumours are rare benign tumours that show locally aggressive and invasive features leading to potential complications. They can be quite challenging for the treating surgeon if they occur adjacent to neurovascular structures. The aetiology of these tumours is still unclear, but the incidence is higher in females and in patients with a history of trauma or surgical procedures, raising the possibility of genetic and hormonal factors as well as post-traumatic or post-operative inflammatory changes promoting the formation of desmoid fibromatosis.
View Article and Find Full Text PDFJpn J Clin Oncol
January 2025
Department of Clinical Oncology, Graduate School of Medicine, Akita University, Hondo 1-1-1, Akita, 010-8543, Japan.
Familial adenomatous polyposis (FAP) is an inherited disorder that follows an autosomal dominant inheritance pattern and is caused by a germline pathogenic variant in the APC gene. FAP also has extracolonic manifestations, including osteomas, brain tumors, and congenital hypertrophy of the retinal pigmented epithelium. Desmoid tumor is a rare soft-tissue tumor often associated with FAP.
View Article and Find Full Text PDFBreast J
January 2025
Australian National University School of Medicine and Psychology, Canberra, ACT 2600, Australia.
Breast desmoid tumour is a rare type of benign breast disease that presents like malignancy. Current guidelines are based on limited evidence derived from case reports and small case series and recommend resection with microscopically-negative margin (R0). There is a high risk of recurrence despite negative surgical margins.
View Article and Find Full Text PDFFront Oncol
December 2024
Angeles Breast Center, Hospital Ángeles Valle Oriente, San Pedro Garza Garcia, Nuevo Leon, Mexico.
Background: Desmoid-type fibromatosis of the breast is a rare, benign, but locally aggressive tumor that typically affects women. Its presentation in male patients is exceedingly rare, and even more so following a cosmetic procedure such as liposuction. This case report describes a unique presentation of breast fibromatosis in a male patient, who developed the condition after undergoing liposuction for cosmetic purposes to define the pectoral area.
View Article and Find Full Text PDFCureus
November 2024
Otolaryngology - Head and Neck Surgery, Aintree University Hospital, Liverpool, GBR.
Aggressive fibromatosis is a rare, benign proliferative disease with unknown aetiology and high recurrence rate. To date, there are only eight reported cases affecting the larynx. Four were managed with total laryngectomy, whilst spontaneous regression happened in one case.
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