A Huge Sister Mary Josephs Nodule From Signet Ring Cell Gastric Carcinoma Showing Good Response to FOLFOX-Based Chemotherapy Regimen

Abstract

Metastatic involvement of the umbilicus as a result of a visceral carcinoma is a rare entity known as Sister Mary Josephs nodule (SMJN). The most common primary sites are the gastrointestinal and gynecological tract. The occurrence of SMJN is commonly associated with advanced peritoneal disease and poor prognosis. The average survival time at the appearance of an umbilical metastasis is estimated at 10 months, and only 13% of patient will be alive at 2 years. Treatment usually involves systemic chemotherapy and palliative care, but the possibility of surgical resection should be considered especially if good response to systemic treatment is achieved in selected patients to maintain or to improve the quality of life. We describe here an unusual case of a 60-year-old man referred with enlarging umbilical lesion, identified as SMJN, that was metastasized from advanced signet ring cell carcinoma of the stomach with generalized peritoneal carcinomatosis. The nodule had rapid progression, and in a few days had reached 15 cm. Chemotherapy was started immediately and patient achieved good clinical and radiological response. A resection of the umbilical nodule was discussed but the patient unfortunately died following a massive pulmonary embolism. This case is unique in view of the unusual size of the SMJN in our patient and the good response to chemotherapy. We present this case to increase physicians awareness for careful evaluation of the umbilical area and encourage them to look for a primary digestive tumor if an umbilical nodule is observed. Therapeutic response to primary chemotherapy may encourage a multimodal approach allowing resectability of the metastatic umbilical nodule.

J Med Cases. 2022;13(3):115-118
doi: https://doi.org/10.14740/jmc3895