Case 1 A 54-year-old woman presented with a 5-month history of an asymptomatic, 2.5-cm, erythematous, ulcerated nodule involving the umbilicus (Fig. 1). Biopsy demonstrated metastatic adenocarcinoma (Fig. 2) and abdominal ultrasound showed a large ovarian tumor (Table 1). Further evaluation revealed pleural metastases. Figure 1. Erythematous nodule of the umbilicus Download figure to PowerPoint Figure 2. Proliferation of glandular structures composed of tumor cells with prominent nucleoli and numerous mitoses (hematoxylin and eosin, ×4000) Download figure to PowerPoint Table 1. Primary malignancy in five patients with Sister Mary Joseph's nodule Case Age (years) Gender Primary malignancy 1 54 Female Ovarian adenocarcinoma 2 58 Male Colorectal adenocarcinoma 3 42 Male Pancreatic adenocarcinoma 4 58 Male Ileocecal sarcoma 5 58 Female Cholangiocarcinoma Case 2 A 58-year-old man was hospitalized with a 6-month history of diffuse abdominal pain, a 2-week history of a cutaneous nodule, and a 5-day history of bloody diarrhea. Cutaneous examination revealed a 1.5-cm, erythematous to violaceous, infiltrative, and verrucous nodule of the umbilicus (Fig. 3). Colonoscopy showed a pedunculated polyp of the right colon and a loss of substance in the anal area; histologic examination demonstrated well-differentiated adenocarcinoma. Abdominal ultrasound showed moderate ascites. A diagnosis of colorectal adenocarcinoma (Table 1) with umbilical metastasis and peritoneal carcinomatosis was made. Figure 3. Irregular, infiltrative, verrucous nodule of the umbilicus Download figure to PowerPoint Case 3 A 42-year-old man, with a history of surgery 5 months earlier for a perforated gastric ulcer, was hospitalized with a 2-month history of abdominal pain and general deterioration. Clinical examination showed a 15-cm infiltrative area at the central abdomen with coalescing ulcerated papules at the umbilicus (Fig. 4), which developed during the onset of abdominal pain. Histologic evaluation revealed metastatic adenocarcinoma to the skin. Further evaluation supported a diagnosis of pancreatic cancer with peritoneal carcinomatosis (Table 1). Figure 4. Tumor infiltrating the central abdomen, with overlying umbilical nodule Download figure to PowerPoint Case 4 A 58-year-old man presented with a 3-month history of abdominal pain and a 1-month history of a painful, bilobed nodular lesion involving the umbilicus. Clinical examination showed a nontender, uniform, and fixed abdominal mass. Colonoscopy revealed a cecal mass. Histologic examination of the cecal mass and umbilical lesion confirmed a diagnosis of ileocecal sarcoma (Table 1). Further evaluation demonstrated peritoneal metastases. Case 5 A 58-year-old woman was hospitalized with a 1-year history of an umbilical lesion, abdominal pain, vomiting, and general deterioration. Examination revealed a 2-cm hard, fixed, nontender, umbilical tumor and hepatomegaly. Endoscopy showed a gastric ulcer without histologic evidence of malignancy. Additional evaluation demonstrated a gallbladder tumor, hepatic metastases, and peritoneal carcinomatosis (Table 1). Biopsy of the umbilical lesion revealed poorly differentiated adenocarcinoma.