What is Sister Mary Joseph's nodule?

Sister Mary Joseph's Nodule

Sister Mary Joseph's nodule is a metastatic tumor deposit in the umbilicus that represents advanced intra-abdominal or pelvic malignancy with poor prognosis and median survival of only 4-14 months. 1, 2

Clinical Characteristics

• Definition: A palpable umbilical cutaneous lesion resulting from metastasis of an internal malignancy
• Appearance: Often presents as a firm, irregular nodule in the umbilical region, frequently >2cm in size 1
• Origin: Represents metastasis from primary tumors in the abdominal or pelvic cavity

Primary Tumor Sites

The most common primary sites include:

• Gastrointestinal tract: Stomach (41.1% of cases) is the most frequent primary site 1
• Genitourinary tract: Ovarian cancer is a common source in women 3, 4
• Other sites: Pancreas, colon, and other intra-abdominal organs

Pathological Features

• Histology: Adenocarcinoma is the predominant histological type (88.2% of cases) 1
• Differentiation: Most primary tumors (52.9%) are poorly differentiated 1
• Spread mechanism: Metastasis occurs through:
  • Direct peritoneal spread
  • Lymphatic channels
  • Venous channels
  • Embryonic remnants in the umbilical region

Clinical Significance

Sister Mary Joseph's nodule has critical clinical importance:

1. It serves as a visible marker of advanced internal malignancy
2. May be the first and sometimes only manifestation of an underlying cancer 5
3. Indicates poor prognosis with limited survival (median 28 weeks in one study) 1

Diagnostic Approach

When an umbilical nodule is identified:

• Biopsy: Essential for histopathological confirmation
• Imaging: CT scan of abdomen and pelvis to identify the primary tumor
• Additional workup: Based on suspected primary site (e.g., endoscopy for suspected gastric cancer)

Treatment Considerations

Treatment is often palliative due to advanced disease stage:

• Surgical options: Excision of the nodule may be performed for symptomatic relief
• Systemic therapy: Chemotherapy based on the primary tumor type
• Radiation: May be considered in selected cases
• Prognosis: Generally poor with high mortality rate (32.4% in-hospital mortality in one series) 1

Clinical Pearls

• The umbilicus is an often overlooked area during physical examination but warrants careful inspection 3
• High index of suspicion is crucial for early detection of this sign
• Recurrence in the umbilical nodule is common (26.1%) even after complete excision 1
• In rare cases, it may be the only sign of recurrence in previously treated malignancies 2

Sister Mary Joseph's nodule is named after Sister Mary Joseph Dempsey (1856-1939), who was the surgical assistant to Dr. William J. Mayo at St. Mary's Hospital in Rochester, Minnesota. She was the first to observe the association between umbilical nodules and advanced intra-abdominal malignancy.