An Ominous Sign: Mucinous Ovarian Carcinoma with Sister Mary Joseph Nodule

Abstract

Despite two centuries of progress in its surgical and oncological management, ovarian cancer remains the most lethal of the gynaecological cancers, claiming the lives of nearly 185,000 women globally each year. Historically considered a single disease, there is growing recognition that ovarian cancer is in fact a spectrum of malignancies with distinct cellular origins, molecular driver pathways and clinicopathological features. Mucinous ovarian carcinoma (mOC) is a rare histological subtype that presents a particular challenge in accurate diagnosis and management. Frequently confused with metastatic deposits from extra-ovarian mucinous tumours, the true incidence of primary mOC is estimated to be between 3-5%. Typically affecting younger women, prognosis for late-stage disease is abysmal with a median survival of <15 months. This case report describes a 38-year-old patient who presented with rapidly worsening abdominal distension. Subsequent debulking surgery removed a mass weighing 2.4kg, confirmed by histopathology as a high grade mucinous ovarian carcinoma with a mural nodule of anaplastic carcinoma. Evidence behind the current guidelines for management will be discussed, addressing our recent understanding of mOC as a separate disease from other histotypes and the consequent challenges in interpreting data from large multicentre trials in which patients with mOC are poorly represented. Moreover, using the Sister Mary Joseph nodule (SMJN) as an example, this case also highlights the importance of the physical examination and the value of subtle (and sometimes missed) clinical signs that provide important clues about the extent of a patient’s underlying disease and prognosis.