Cholangiocarcinoma with a metastatic umbilical lesion

David Harrington, infectious disease registrar, Christina George, dermatology registrar, Hazem Ibrahim, consultant dermatopathologist, Sanjay Bhagani, consultant in infectious diseases and HIV

A 59 year old man with a renal transplant and well controlled HIV infection presented with an enlarging, firm, and intermittently discharging crusted umbilical nodule (fig 1). He gave a history of mild, unspecified weight loss. On examination, he had hepatomegaly of 4 cm. Liver enzymes were raised, with raised alkaline phosphatase and normal bilirubin. Computed tomography of the abdomen showed an extrahepatic biliary mass, a large secondary liver lesion, intra- and extra-hepatic biliary duct dilatation, and a left groin nodal mass, consistent with metastatic cholangiocarcinoma. A skin biopsy of the umbilical nodule revealed poorly differentiated adenocarcinoma, confirming the diagnosis of a Sister Mary Joseph nodule. The patient developed obstructive jaundice, and repeat imaging showed that the disease had progressed. A palliative biliary stent was inserted and the patient died 3 months after diagnosis.

Fig 1

Sister Mary Joseph nodules are cutaneous metastatic umbilical deposits from primary intra-abdominal or pelvic malignancies. When seen, they should prompt investigation for underlying malignancy.


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