Sister Mary Joseph's nodule at a University teaching hospital in northwestern Tanzania: a retrospective review of 34 cases

Abstract

BackgroundSister Mary Joseph’s nodule is a metastatic tumor deposit in the umbilicus and often represents advanced intra-abdominal malignancy with dismal prognosis. There is a paucity of published data on this subject in our setting. This study was conducted to describe the clinicopathological presentation and treatment outcome of this condition in our environment and highlight challenges associated with the care of these patients, and to proffer solutions for improved outcome.MethodsThis was a retrospective study of histologically confirmed cases of Sister Mary Joseph’s nodule seen at Bugando Medical Centre between March 2003 and February 2013. Data collected were analyzed using descriptive statistics.ResultsA total of 34 patients were enrolled in the study. Males outnumbered females by a ratio of 1.4:1. The vast majority of patients (70.6%) presented with large umbilical nodule > 2 cm in size. The stomach (41.1%) was the most common location of the primary tumor. Adenocarcinoma (88.2%) was the most frequent histopathological type. Most of the primary tumors (52.9%) were poorly differentiated. As the disease was advanced and metastatic in all patients, only palliative therapy was offered. Out of 34 patients, 11 patients died in the hospital giving a mortality rate of 32.4%. Patients were followed up for 24 months. At the end of the follow-up period, 14(60.9%) patients were lost to follow-up and the remaining 9 (39.1%) patients died. Patients survived for a median period of 28 weeks (range, 2 to 64 weeks). The nodule recurred in 6 (26.1%) patients after complete excision.ConclusionSister Mary Joseph’s nodule of the umbilicus is not rare in our environment and often represents manifestation of a variety of advanced intra-abdominal malignancies. The majority of the patients present at a late stage and many with distant metastases. The patient's survival is very short leading to a poor outcome. Early detection of primary cancer at an early stage may improve the prognosis.

Sister Mary Joseph's nodule as the first and only sign of recurrence in a case of stage Ia carcinoma ovary

“…This sign can be the first sign of disease or an indicator of recurrence. Previously published reports have shown it to be a marker of extensive disease 3 4. However, in our case, it was novel to see no sign of intra-abdominal disease macroscopically as well as on microscopy of peritoneal biopsies.…”

Ungewöhnliche Manifestation eines Prostatakarzinoms

“…Dies gilt im Speziellen für den Sister-Mary-Joseph-Knoten, nicht zuletzt auch aus dem Grund, dass sich eine Vielzahl verschiedener Neoplasien hinter dieser Läsion verbergen können [1]. …”

“…Unter Berücksichtigung der publizierten Kasuistiken ist das Auftreten eines Sister-MaryJoseph-Knotens hier ein eher spätes Ereignis bei zumeist fortgeschrittenem Tumorbefund [1,2,6,8]. Auch im hier präsentierten Fall war das Prostatakarzinom bereits ca.…”

“…10 Jahre bekannt, war jedoch assoziiert mit einem biochemischen Rezidiv, welches sich erst in den letzten Monaten entwickelt hatte. Bezüglich der Größe und des übrigen klinischen und makroskopischen Befunds existieren verschiedene Angaben, jedoch sind erythematöse schmerzlose Knoten keine Seltenheit [1,8]. Da die Tumoren, wie auch im vorliegenden Fall, regelmäßig <2 cm sein können, ist die Gefahr groß, diese Tumormanifestation zu übersehen oder als benignen Befund einzuschätzen.…”

“…Dieses ist aber fatal: das Auftreten einer Umbilikalmetastase ist mit einer ungünstigen Prognose assoziiert. Die mittlere Überlebenszeit von Patienten mit einem Sister-Mary-JosephKnoten beträgt unter 11 Monate [1,2]. In den letzten Jahren hat sich die PET-CTUntersuchung in der Uroonkologie als wichtige Säule bei der Diagnostik etabliert [4].…”