A. Sister Mary Joseph nodule
C. Stomach or colon
Sister Mary Joseph was born Julia Dempsey in 1856; she took the religious name “Mary Joseph” when she joined the Third Order Regular of St. Francis of the Congregation of Our Lady of Lourdes in Rochester, Minnesota. The main objective of that order was to establish St. Marys Hospital, the earliest incarnation of the now world-renowned Mayo Clinic. The hospital opened in 1889, and Sister Mary Joseph served as both the hospital superintendent and the first surgical assistant to Dr. William Mayo. During her experience in the operating room, Sister Mary Joseph was the first person to note that the presence of an umbilical nodule constituted a physical sign associated with an advanced intra-abdominal malignancy. In the 11th edition of the textbook Physical Signs in Clinical Surgery (1949), Dr. Hamilton Bailey attributed this observation to the nun, and the sign was thereafter known by the eponym Sister Mary Joseph nodule (SMJN).[1,2]
Over 400 cases of this entity have appeared in the medical literature, many of which appeared in a single large series or were reviewed in an exhaustive meta-analysis.[3,4] In men, umbilical metastases most commonly originate in the gastrointestinal tract, particularly the stomach or colon. In women, umbilical metastases most often arise from the ovary or endometrium. In rare instances, bronchogenic carcinoma, hepatocellular carcinoma, non-Hodgkin lymphoma, squamous cell carcinoma of the cervix, transitional cell carcinoma of the bladder, and adenocarcinoma of the prostate can lead to the SMJN phenomenon. In some 15% to 30% of cases, the primary site of origin remains unidentified.[3,6] It is noteworthy that nonumbilical cutaneous metastases from intra-abdominal or pelvic tumors typically occur late in the disease course, whereas an umbilical metastasis may be the earliest presenting sign of an underlying malignancy or recurrence of a previously treated neoplasm. Nonetheless, finding umbilical metastases generally portends a dismal prognosis, as the SMJN phenomenon is characteristically associated with widespread disease.[7,8] However, aggressive surgical and chemotherapeutic approaches may improve the prognosis, and in any given patient, intervention should be personalized.
The clinical features of SMJN are rather consistent: firm, indurated plaques or nodules, often with a vascular appearance (erythematous to violaceous). However, erosions, ulcerations, crusts, and fissures have been noted in a few cases. Because not every umbilical mass is metastatic in nature, there is a differential diagnosis. The latter include primary umbilical tumors (basal cell carcinoma, melanoma, myosarcoma), umbilical hernia, teratoma, epidermoid cyst, neurofibroma, dermatofibroma, umbilical endometriosis, and foreign body granuloma and omphalith.[9, 10] True SMJN should be positive on 18F-fluorodeoxyglucose positron-emission tomography/CT scanning, whereas “pseudo” SMJN is more often negative.