DESMOID TUMOR IN A PATIENT WITH FAMILIAR ADENOMATOSIS POLYPOSISIS

• DOI: https://doi.org/10.22533/at.ed.1594682424075

• Palavras-chave: Case Report; Demoid tumor; Aggressive fibromatosis

• Keywords: Case Report; Demoid tumor; Aggressive fibromatosis

• Abstract:

  Desmoid tumors (TD), also known as aggressive fibromatosis or musculo-aponeuritic fibromatosis, are rare, benign, non-metastatic, locally infiltrative neoplasms that originate from soft tissues or muscular-aponeurotic structures. Goal: To report a rare case of a patient with desmoid tumor with a history of familial adenomatosis polyposis (PAF). Case report: Male patient, 32 years old, with a family history of FAP, underwent protocolectomy with ileo-rectal reconstruction in 2021. In 2023, the patient noticed the presence of a solid, painless mass on the right flank. Computed tomography of the total abdomen was performed. with contrast, a large heterogeneous mass was revealed, measuring 11.5 x 9.5 x 12.0 cm (W x AP x T), suggestive of Desmoid Tumor. Submitted to tumor resection with rotation of a muscle flap and enterectomy with burial-anastomosis. Discussion/Final Considerations:  These tumors often occur in the abdominal wall. Imaging exams assist in diagnosis and can determine the approach modality. Surgical resection with a histologically negative margin has been indicated as an approach for this disease in most cases.