Sister Mary Joseph nodule (SMJN) is a sign of metastatic cancer from the viscera in the abdomen or pelvis. A 71-year-old male presented to the surgical outpatient clinic with a painful umbilical nodule and was later referred to the Department of Dermatology due to suspected omphalitis. Physical examination revealed a tender, subcutaneous, periumbilical nodule approximately 5mm in diameter. SMJN was suspected, and the patient underwent a biopsy and a contrast-enhanced computed tomographic (CT) scan of the abdomen and pelvis. Hematoxylin-eosin staining of the biopsy specimen confirmed small nests of enlarged atypical cells and a lumen structure in the dermis, and immunohistochemical staining was positive for carbohydrate antigen 19-9 (CA 19-9). CT scan of the abdomen showed a 3cm cystic lesion in the body of the pancreas and dilatation of the main pancreatic duct. Peritoneal dissemination of the tumor was strongly suspected due to the presence of fluid in the rectovesical pouch. The suspected presence of peritoneal dissemination made surgery inappropriate, and thus chemotherapy was recommended as a first-line treatment. He was subsequently referred for a second opinion. Histopathological examination of the umbilical tumor cannot always identify a primary lesion; therefore, systemic screening of malignancy using CT or magnetic resonance imaging should be considered. Some conditions, including omphalitis, mimic SMJN and have thus been reported as pseudo-Sister Mary Joseph nodule; therefore, SMJN should be distinguished from other conditions by meticulous examination of the periumbilical tumor. The importance of both timely biopsy of the periumbilical tumor and systemic screening using imaging modalities was highlighted by a report of the long survival of a patient with SMJN treated successfully by chemotherapy.Skin Research, 12: 292-295, 2013
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