Umbilical nodule in cholangiocarcinoma. Sister Mary Joseph Nodule.

A 17-year-old girl was admitted with complaints of jaundice for 2 months. She also had constant dull ache in right upper abdomen for 3 months. There was no other bowel or urinary complaint. Physical examination showed icterus, and bilateral pedal edema. Abdominal examination revealed umbilical nodule [Figure 1], abdominal distension, mild hepatomegaly, and presence of shifting dullness. Left supraclavicular lymph node was not palpable. Her total bilirubin was 22 mg/dL with conjugated component of 17.7 mg/dL and serum alkaline phosphatase was 1128 IU. Contrast-enhanced computed tomography suggested a small mass-forming cholangiocarcinoma in mid-common bile duct with proximal biliary dilatation. Fine-needle aspiration cytology from umbilical nodule showed metastatic adenocarcinoma. The patient did not consent to undergo endoscopic retrograde cholangiopancreaticography for brush cytology and biliary stenting.

Figure 1

Umbilical nodule

QUESTIONS

Q1. What is this sign known as [Figure 1]?

Q2. What are the conditions in which this sign is seen?

ANSWERS

This sign is known as Sister Mary Joseph Nodule. Historically, Sister Mary Joseph (1856–1939) was the nursing superintendent and surgical assistant of Dr. William Mayo at St Mary's Hospital in Rochester, Minnesota (at present, the Mayo Clinic), who noticed the presence of an umbilical nodule in intra-abdominal malignancies.[1]

The most common sites of intra-abdominal malignancies associated with Sister Mary Joseph Nodule are the stomach and colon in males, and ovaries in females. Other reported primary sites are pancreas, biliary tract, fallopian tube, endometrium, and rarely from the lung and prostate.[2]

Spread of metastatic carcinoma to the umbilical region has been hypothesized to occur by either contiguous spread of peritoneal cancer, hematogenous spread through arterial and venous systems or lymphatic spread (mainly pancreatic carcinoma), or extension along ligaments of embryonic origin (round ligament of liver, urachus, vitello intestinal duct remnant, and the obliterated vitelline artery).[3] Our patient had extrahepatic cholangiocarcinoma, which was likely to have spread through falciform ligament to manifest as a metastatic umbilical nodule.