Multiple Desmoid Tumors In A Patient With Gardner's Syndrome - Report Of A Case

INTRODUCTION Desmoid tumor (DT) is a common manifestation of Gardner's Syndrome (GS), although it is a rare condition in the general population. DT in patients with GS is usually located in the abdominal wall and/or intra-abdominal cavity. PRESENTATION OF CASE We report a case of a 32 years-old female patient with familial adenomatous polyposis (FAP), who was already submitted to total colectomy and developed multiple DT, located in the abdominal wall and in the left breast. The patient underwent several surgical procedures, with a multidisciplinary team of surgeons. Wide surgical resections of the left breast and the abdominal wall tumors were performed in separate steps. Polypropylene mesh reconstruction and muscle flaps were needed to cover the defects of the thoracic and abdominal walls. After partial necrosis of the adipose-cutaneous flap in the abdomen that required a new skin graft, she had a satisfactory outcome with complete healing of the surgical incisions. DISCUSSION DT is frequent in GS, however, breast localization is very rare, with few cases reported in the literature. Recurrence of DT is not negligible, even after a wide surgical resection. GS patients must be followed up closely, and clinical examination, associated with imaging studies, should be performed to detect any signs of tumor. CONCLUSION DT represents one of the most significant causes of the morbidity and mortality that affects FAP patients following colectomy. In general, the surgical procedures to excise DT are highly complex, requiring a multidisciplinary team. © 2014 The Authors.57370374Lee, B.D., Lee, W., Oh, S.H., A case report of Gardner syndrome with hereditary widespread osteomatous jaw lesions (2009) Oral Surg Oral Med Oral Pathol Oral Radiol Endodontol, 107 (3), pp. 68-72Jonathan, B., Claire, H., Mary, T., Gardner syndrome - Review and report of a case (2005) Oral Oncol Extra, 41, pp. 89-92Fotiadis, C., Tsekouras, D.K., Sfiniadakis, J., Genetzakis, M., Zografos, G.C., Gardner's syndrome: A case report and review of the literature (2005) World Journal of Gastroenterology, 11 (34), pp. 5408-5411Gómez García, E.B., Knoers, N.V., Gardner's syndrome (familial adenomatous polyposis): A cilia-related disorder (2009) Lancet Oncol, 10 (7), pp. 727-735Cristofaro, M.G., Giudice, A., Amantea, M., Gardner's syndrome: A clinical and genetic study of a family (2013) Oral Surg Oral Med Oral Pathol Oral Radiol, 115 (3), pp. 1-6Gu, G.L., Wang, S.L., Wei, X.M., Diagnosis and treatment of Gardner syndrome with gastric polyposis: A case report and review of the literature (2008) World J Gastroenterol, 14 (13), pp. 2121-2123(2003) Breast Imaging Reporting and Data System, Breast Imaging Atlas, , American College Of Radiology 4th ed. American College of Radiology Reston, VAMerg, A., Lynch, H.T., Lynch, J.F., Hereditary colon cancer-Part i (2005) Curr Probl Surg, 42 (4), pp. 195-256Mao, C., Huang, Y., Howard, J.M., Carcinoma of the ampulla of Vater and mesenteric fibromatosis (desmoid tumor) associated with Gardner's syndrome: Problems in management (1995) Pancreas, 10 (3), pp. 239-245Cruz-Correa, M., Giardiello, F.M., Familial adenomatous polyposis (2003) Gastrointestinal Endoscopy, 58 (6), pp. 885-894. , DOI 10.1016/S0016-5107(03)02336-8, PII S0016510703023368Juhn, E., Khachemoune, A., Gardner syndrome: Skin manifestations, differential diagnosis and management (2010) Am J Clin Drematol, 11 (2), pp. 117-122Turina, M., Pavlik, C.M., Heinimann, K., Recurrent desmoids determine outcome in patients with Gardner syndrome: A cohort study of three generations of an APC mutation-positive family across 30 years (2013) Int J Colorectal Dis, 28 (6), pp. 865-872Brown, C.S., Jeffrey, B., Korentager, R., Desmoid tumors of the bilateral breasts in a patient without Gardner syndrome: A case report and review of literature (2012) Ann Plast Surg, 69 (2), pp. 220-222Leal, R.F., Silva, P.V.V.T., Ayrizono, M.L.S., Desmoid tumor in patients with familial adenomatous polyposis (2010) Arq Gastroenterol, 47, pp. 373-378Rammohan, A., Wood, J.J., Desmoid tumour of the breast as a manifestation of Gardner's syndrome (2012) Int J Surg Case Rep, 3 (5), pp. 139-142Escobar, C., Munker, R., Thomas, J.O., Update on desmoid tumors (2012) Ann Oncol, 23 (3), pp. 562-569Camargo, V.P., Keohan, M.L., D'Adamo, D.R., Clinical outcomes of systemic therapy for patients with deep fibromatosis (desmoid tumor) (2010) Cancer, 116 (9), pp. 2258-2265Xu, H.M., Han, J.G., Ma, S.Z., Related citations treatment of massive desmoid tumour and abdominal wall reconstructed with meshes in Gardner's Syndrome (2010) J Plast Recontr Aesthet Surg, 63 (6), pp. 1058-106