Sister Mary Joseph Nodule, Umbilical Sentinel for an Endometrioid Endometrial Carcinoma in Postmenopause-Challenges for a Multidisciplinary Approach

Abstract

A 82 years old, hypertensive, obese, 1 delivery, menopause at 47 yrs, non-smoker, with history of vaginal bleeding in January 2018, is sent by the dermatologist for an irregular umbilical tumor of 2/1 cm, recurrent at 6 weeks post-ablation, microscopically considered as a carcinomatous metastasis. Abdomino-pelvic MRI rises the suspicion of uterine carcinoma, which is confirmed by endometrial biopsy. It is done extrafascial total hysterectomy with bilateral salpingo-oophorectomy, and pelvic lymph dissection and ablation, plus ablation of a skin-adipose-conjunctive peri-and subumbilical area of 5/4 cm. There were no peritoneal metastases, or other viscera abnormalities. The optic microscopy shows moderate differentiated endometrioid endometrial carcinoma, with areas of squamous differentiation, invasion in external myometrial half, without peritoneal invasion, with vessels metastatic embolism, positive pelvic lymph nodes with moderate differentiated endometrioid endometrial carcinoma and desmoplastic reaction; left ovary with mature teratoma. Umbilical specimen has malignant invasion with cribriform and tubular pattern, areas of squamous differentiation, and malignant emboli in vessels. The patient suffered radiotherapy, under oncologic monitoring. The follow up at 6,12,18 and 36 months was with no recurrence in genital, pelvis, peritoneum, abdominal wall. Sister Mary Joseph Nodule (SMJN) has an old history: first observations of assistant catholic nun Mary Joseph Dempsey (Saint Mary’s Hospital, Rochester, Minnesota, USA), William Mayo (1928) description, Hamilton Bailley (1949) characterization. Literature associates SMJN to cancers originating in gastro-intestinal, colonic tract, respiratory, urinary and genital tract: primary ovarian and endometrial cancers; Romanian case is the 36th with endometrial origin. Umbilical invasion may be due to direct vessels’ embolization by malignant endometrial cells, via lymphatics which run along the obliterated umbilical vein, or via the remnant structures of the falciform and umbilical ligaments. Patient is under oncologic monitoring on hormone therapy (Megesin®), with good prognosis up to four years of follow up, different from literature mentioned bad prognosis.