Sister Mary Joseph nodule (SMJN) is an uncommon pattern of superficial periumbilical tumor metastasis, with the primary tumor most commonly associated with gynecological or gastrointestinal origins. This manifestation can represent extensive tumor development from any of the intra-abdominal or pelvic structures. Therefore, SMJN carries a poor prognosis, with a two-year survival rate of only 13.5 percent regardless of the etiology of primary cancer. In this case, a 67-year-old man with metastatic mucinous adenocarcinoma of the appendix involving the umbilicus presenting more than five years after the initial cancer diagnosis is reported. The features of patients with metastatic appendiceal carcinoma presenting as SMJN are also reviewed. With the inclusion of our patient, there are six patients who have documented SMJN due to appendiceal carcinoma: two men, two women, and two patients without demographic data. The patients ranged from the ages of 31 to 68 years, with a median age of 56.5 years at cancer diagnosis and 59 years at SMJN diagnosis. In 75 percent of the cases, SMJN was the initial clinical manifestation of a previously unsuspected appendiceal carcinoma and presented clinically one to seven months (median of five months) before the pathologic confirmation of the metastatic appendiceal carcinoma. The likelihood of SMJN presenting as the initial clinical feature of appendiceal cancer may increase in patients with extensive intraperitoneal metastasis in the form of pseudomyxoma peritonei or carcinomatosis. Therefore, the observation of a solitary umbilical nodule should prompt an investigation for an underlying primary neoplasm, as the prognosis after tumor metastasis to the umbilicus is poor.
Keywords: appendiceal; appendix; carcinoma; cutaneous; joseph; mary; metastasis; mucinous; nodule; sister.