Sister Mary Joseph Nodule : An Update on Characteristics and Outcomes

Objectives: We aimed to assess and update the characteristics of patients with Sister Mary Joseph Nodules (SMJN) to define patient outcomes in regards to type of cancer and mortality. Background: The SMJN is a well-known manifestation of intraabdominal malignancy. The majority of published case reports demonstrate that SMJNs typically arise from a gastrointestinal or gynecologic source. Furthermore, these patients have a dismal prognosis, with many patients dying within a few months of diagnosis. Methods: We reviewed patients seen at Mayo Clinic between January 1, 1992 and March 31, 2017 with the Sister Mary Joseph Nodule. Pathology reports were reviewed to confirm biopsy-proven umbilical metastasis. Abstracted data included basic demographics at diagnosis, site of primary malignancy, treatment, and survival. Results: 113 patients were identified. Median survival from the time of diagnosis of SMJN was 14.6 months (follow-up ranging from 0.4 to 231.5 months). 2-year mortality was 88.4%, and 5-year mortality was 95.4%. 47.8% had a primary gastrointestinal (GI) malignancy (31.5% of which were colorectal), 35.4% had a primary gynecologic malignancy (52.5% of which were ovarian), and 4.4% had a primary hematologic malignancy. Greater survival was associated with gynecologic compared to GI neoplasia (35.7 months vs. 15.7 months; p = 0.02). Treatment with both chemotherapy and surgery (average survival, 42.7 ± 8.6 months) was associated with improved survival compared to treatment with chemotherapy alone (mean 19.1 months; ± 1.8 months) or no treatment (mean 4.0 ± 0.24 months; p < 0.01). Conclusions: Most SMJNs are of a gastrointestinal (colonic) or gynecologic (ovarian) origin. SMJNs of gynecologic origin have better survival compared to tumors with a GI origin. Treatment with chemotherapy and surgery, regardless of primary tumor type is associated with better survival as compared to chemotherapy alone or to no treatment. ORIGINAL ARTICLE This is an EJBM Online First article published ahead of the final print article. The final version of this article will be available at www.einstein. yu.edu/ejbm EJBM 2018 www.einstein. yu.edu/ejbm E82 INTRODUCTION Metastatic disease protruding through the umbilicus is known as a Sister Mary Joseph nodule (SMJN). The association of metastatic intra-abdominal malignancy and umbilical nodules bears the namesake of Sister Mary Joseph Dempsey (1856-1939), who was one of Dr. William Mayo’s surgical assistants (Powell, 2011). She observed the relationship between umbilical nodules noted during pre-operative skin preparation and widespread intra-abdominal malignancy noted during surgery (Mayo, 1928). The SMJN is typically no greater than 5 cm in diameter, and is often described as hard, indurated, or firm. There may be fissuring, necrosis, or ulceration with associated bloody, serous, or mucinous discharge (Abu-Hilal & Newman, 2009). SMJN is an ominous sign reflecting an advanced stage of the underlying malignancy, and is often associated with a poor prognosis. There is a paucity of longitudinal data on the outcomes of patients with SMJN and whether the presence of this physical examination finding still portends a worse prognosis despite advancement in cancer chemotherapeutics. In order to better understand the modern prognostic implications of SMJN, the aims of this study were to better define the baseline characteristics of patients with SMJN, to assess prognosis stratified by cancer primary and to determine the effects of aggressive treatment in patients with SMJN.