The patient is a 62-year-old male who presented to an outpatient medical clinic complaining of a growing, slightly painful, periumbilical mass, and mild lower gastrointestinal discomfort over the last 4 months. On examination, the patient appeared cachectic with an erythematous soft nodule within the umbilicus. Laboratory evaluation revealed anemia (Hct: 28%) and colonoscopy detected a tumor in the sigmoid colon. Both biopsies of the sigmoid mass and the umbilical nodule revealed the presence of adenocarcinoma. Due to the advanced stage of the disease, the patient received chemotherapy as his primary treatment.
Umbilical tumors may be the first sign of an underlying cancer or of a recurrence of a previous cancer . Metastatic cancer of the umbilicus is known as Sister Mary Joseph’s nodule . It is encountered in about 1–3% of patients with an intra-abdominal and/or pelvic malignancy, with gastric carcinoma being the most common cause in men, and ovarian carcinoma the most common cause in women .
This condition was named for Sister Mary Joseph (1856–1939), who was a daughter of Irish immigrants and was born in Salamanca, New York. From 1890 to 1915, Sister Mary Joseph was the first surgical assistant to William James Mayo, and in September 1892, she was appointed nursing superintendent of St. Mary’s Hospital. She noted the association between paraumbilical nodules observed during skin preparation for surgery and metastatic intraabdominal cancer confirmed at surgery [2, 4]. Hamilton Bailey coined the term “Sister Mary Joseph’s nodule” in 1949 in her recognition .
Metastasis to the umbilical region has been hypothesized to occur in several ways, with the most important including hematogenous spread (through extensive arterial and venous networks). Additional possibilities include spread due to lymphatic communication between the umbilicus and the axillary, para-aortic, inguinal, external iliac, internal mammary nodes. Another theorized route of metastatic spread is via the ligaments of embryonic origin such as the vitelline duct, which connects the umbilicus to the ileum . From the umbilicus, tumor cells are then most often spread through the rest of the body .
Sister Mary Joseph’s nodule is significant because it may be the first and only presenting sign of malignancy, as has been demonstrated in about 30% of cases . The clinical appearance is often that of a painful umbilical nodule, with irregular margins, and a hard consistency. The surface has also been typically described to be necrotic-appearing with bloody, serous, or purulent discharge. The size of the nodule is most often less than 5cm but has been documented to reach as large as 10cm in diameter [4, 6, 7].
The differential diagnosis of umbilical lesions is extensive and can be divided into benign causes vs. primary malignancies vs. an umbilical nodule due to metastases (Sister Mary Joseph’s nodule). Benign causes include cysts, umbilical hernias, skin tags, teratomas, angiomas, abscess, pyogenic granulomas, formation of an omphalith (due to concretions of the umbilicus), or endometriosis. Primary umbilical malignancies include basal cell carcinomas, melanomas, and mesenchymal tumors . To make the diagnosis of Sister Mary Joseph’s nodule, physicians rely on the histopathologic examination of biopsies taken from the umbilical tumor, which can also be used to detect primary origin of the cancer [1, 8].
A retrospective study from the Mayo Clinic reviewed 85 cases of umbilical tumors due to metastases from 1950 and 1982. Of the 85 patients that were reviewed, 40 occurred in men and 45 occurred in women. Twelve patients (14%) had umbilical nodules as their initial presentation of internal malignancy while 45 (53%) developed umbilical nodules within 12 months of diagnosis . A study by Dubreil et al reviewed 368 cases of umbilical metastases . In 152 out of 368 cases (41%), umbilical metastasis was discovered before the primary cancer, and in 97 out of the 152 cases (64%), the nodule was the only initial presenting sign of malignancy. The study by Dubreil et al also specifically evaluated the location of the primary malignancy leading to umbilical metastases. Out of a review of 368 cases of umbilical nodules, 96 were from metastasis of adenocarcinoma of the stomach (30% of male cases, 9% of female cases), 74 were from adenocarcimona of the rectum, colon, or small bowel (25% of male cases, 12% of female cases), 59 cases were linked to the ovary (64% of female cases), while others were due to squamous cell carcinoma (4%), the cervix (4%), the pancreas (10%), the gallbladder (2%), the breast, the lung, the prostate, and the penis. 41 out of 368 cases (11%) were of unknown etiology .
Since Sister Mary Joseph’s nodule is usually a reflection of metastatic disease, the majority of patients with a Sister Mary Joseph’s nodule have a poor prognosis and die within 10 months following discovery of the nodule. While we may not be able to change the course of advanced disease, it is imperative that we are aware of Sister Mary Joseph’s nodule, and its association with malignancy [3, 4, 7, 8].
Dr. Keri Herzog is a 3rd year resident at NYU Langone Medical Center
Peer reviewed by Michael Poles, GI Section Editor, Clinical Correlations
Image courtesy of Wikimedia Commons.
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