CASE PRESENTATION
An 85-year-old woman presented with painless, erythematous nodules on the umbilicus that developed over 1 month. At the time of presentation, the patient had significant unintentional weight loss, poor appetite, and right lower quadrant tenderness. Surgical history was significant for hysterectomy without oophorectomy 10 years ago. Physical examination of the abdomen revealed a 3-cm pink-to-violaceous tumor with several small "satellite" dermal papules with superimposed ulceration at the umbilicus and extending onto the lower abdomen (Figure 1). Punch biopsies revealed a malignant epithelial neoplasm with glandular differentiation in the dermis, elongated branching tubules and solid nests and cords of cells, and scattered mitotic figures and apoptotic cells are present (Figure 2). A panel of immunohistochemical stains revealed the following: AE1/AE3 positive, cytokeratin 7 positive, and CK20 negative, which is classically noted in an ovarian neoplasm (Selves et al., 2018). Biopsy results were consistent with metastatic adenocarcinoma. Immunohistochemical stains are used in poorly differentiated tumors to differentiate carcinoma, melanoma, sarcoma, neural neoplasms, and lymphomas. A panel of antibodies is recommended rather than a single stain because of possible heterogeneous staining (Selves et al., 2018). Stains are selected based on the clinical history and results of ancillary studies, including imaging. Computerized tomography of the abdomen and pelvis showed a large pelvic mass obstructing the sigmoid colon. When coupled with changes seen in histopathology, clinical history, and imaging, the possibility of metastatic serous ovarian carcinoma was raised. Differential diagnosis includes hernia and metastatic nodules of a gastrointestinal (GI) neoplasm, negated by the absence of goblet cells and GI epithelium on hematoxylinand eosin stain and CK7+/CK20 immunohistochemistry profile. The patient was subsequently managed by a multidisciplinary surgical and medical oncology team. However, the patient succumbed to the aggressive nature of her cancer and died 7 months after diagnosis. Sister Mary Joseph's nodule is a time-tested clinical sign representing metastatic nodules of advanced intra-abdominal malignancy.
DISCUSSION
About 1%-10% of internal malignancies present with cutaneous metastases, 16% of which are localized to the umbilicus (Vernemmen et al., 2022). Sister Mary Joseph's nodules were named after the surgical assistant who first linked umbilical nodules with abdominopelvic cancers in 1949 (Gabriele et al., 2005). Classically, Sister Mary Joseph's Nodules presents as a 0.5- to 2-cm painful hard nodule on the umbilicus with an irregular border; however, it may present as a blue-black, friable, ulcerated lump with drainage as large as 10 cm (Gabriele et al., 2005). Benign and malignant tumors can affect the umbilicus and warrant a thorough workup. Metastatic umbilical nodules can often be the only presenting sign of an occult malignancy (Gabriele et al., 2005). There is little clinical differentiation between a primary and secondary umbilical tumor. Primary umbilical tumors are rare and include basal cell carcinoma, malignant melanoma, and omphalomesenteric duct carcinoma (Chalya et al., 2013). Benign umbilical lesions include epidermal inclusion cysts, endometriosis, congenital malformations, and granulomas, among others (Chalya et al., 2013). On the basis of the available literature, the average survival time for a patient with this clinical finding is 10 months in the setting of metastatic disease, which portends a poor prognosis (Chiang & Lin, 2015). In men, GI tract malignancies (35%-65%) are the most common cause, whereas in women, ovarian and endometrial malignancies (34%) tend to be the culprits (Chalya et al., 2013). In rare cases, the primary tumor can originate in the breast, small bowel, liver, gall bladder, prostate, kidney, lung, or other sites (Chalya et al., 2013). Approximately 15%-30% of cases have an unidentifiable primary tumor (Chalya et al., 2013). Several possible pathways can lead to metastases to the umbilicus, including lymphatic, arterial, and venous drainage, as well as a contiguous spread (Gabriele et al., 2005). Sister Mary Joseph's nodules suggest a more significant cancer burden and a poor prognosis. A high degree of suspicion is warranted when evaluating umbilical lesions as this may be the only presenting sign of an occult malignancy.
REFERENCES