Morphology

Lest we forget: Sister Mary Joseph and her nodule

Maria R. Gaiser, MD, Knut Schakel, MD, and Peter Helmbold, MD

Department of Dermatology, University of

Heidelberg, Heidelberg, Germany

Correspondence

Maria Rita Gaiser, MD

Department of Dermatology

University of Heidelberg

Vossstr. 2, 69115 Heidelberg

Germany

Email: maria-gaiser@gmx.de

Conflict of interest: None.

Case report

A 71-year-old woman presented to our outpatient clinicwith a new mass growing in her umbilicus. The solid andulcerated tumor was of red-violet color and had a size ofapproximately 2 cm (Fig. 1a). In addition, the patientshowed signs of ascites with a clearly bloated abdomen.Histopathological examination of the biopsy revealed acompletely ulcerated epidermis with an underlying dermalinfiltration of pleomorphic tumor cells with focal develop-ment of lumina (Fig. 1b). The lesion was diagnosed as ametastatic manifestation of a carcinoma of unknown ori-gin. Immunohistochemically, carcinoma cells showed ahigh expression of CA125 and CK7. The patient wasreferred to the Department of Gynecology for furtherexamination and diagnosed with metastatic ovarian carci-noma at an inoperable stage.

Discussion

Sister Mary Joseph nodule describes a metastatic skinmanifestation of an internal carcinoma. In the majority ofcases, this nodule is the only allusion to a malignanttumor in an otherwise healthy-appearing patient. Theunderlying malignancies are usually of gastrointestinal orgynecological origin and often at an inoperable stage attime of diagnosis. In general, metastatic skin manifesta-tions occur at a frequency of 4.7% in patients with carci-noma excluding melanoma,1 of which 10% affect theumbilicus. This region is an anatomic location that is pre-disposed to metastatic dissemination along the lymphaticvessels and/or the ligaments accompanying the ductus

(a)

(b)

Figure 1 (a) Clinical exploration revealed a solid, red-violet,ulcerated tumor in the umbilicus. (b) Histopathologicalanalysis demonstrated a dermal infiltration of pleomorphictumor cells with focal development of lumina (H&E,original magnification 9 200 lm)

ª 2013 The International Society of Dermatology International Journal of Dermatology 2013, 52, 587–588

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omphaloentericus (an embryonic relict), per continuitatemvia the anterior layer of the lesser omentum or hematogen.It may resemble a variety of other diagnoses, for examplegranuloma pyogenicum, teratoma, omphalith, or congeni-tal malformations of the ductus omphalomesentericus.Sister Mary Joseph, born in 1856 in New York as Julia

Dempsey, joined the Franciscan order of Rochester,Minnesota, in 1878. Eleven years later, she started work-ing at Saint Marys Hospital and became the first surgicalassistant to William Mayo, a gastrointestinal surgeon andone of the seven co-founders of the Mayo Clinic. Duringher 25 long years of work, she noted a co-occurrence ofumbilical tumors in patients with underlying advancedinternal carcinomas and brought it to Dr. Mayo’s atten-tion. In 1928 he reported her findings as pants buttonumbilicus during a lecture to the Cincinnati Academy ofMedicine. Nevertheless, it took 21 years until 1949 whenthe English surgeon Sir Hamilton Bailey would honor Sis-ter Mary Joseph for her observation in the 11th editionof his famous surgical textbook Demonstrations of Physi-cal Signs in Clinical Surgery with the new name SisterMary Joseph nodule.

As a tragic co-occurrence, the three long-time co-work-ers William Mayo, his brother Charles Mayo as well as Sis-ter Mary Joseph all died within three months in 1939.Sister Mary Joseph nodule is most often a sign of a bad

prognosis. Fewer than 15% patients are still alive aftertwo years.2 However, depending on the kind of underly-ing malignancy, the prognosis may be much better if trea-ted immediately.Sister Mary Joseph nodule is nothing new and is not

extraordinarily rare, but it is always important to thinkabout, especially for dermatologists who are active in der-matological surgery.

References

1 Lookingbill DP, Spangler N, Sexton FM. Skin involvementas the presenting sign of internal carcinoma. Aretrospective study of 7316 cancer patients. J Am Acad

Dermatol 1990; 22: 19–26.2 Powell FC, Cooper AJ, Massa MC, et al. Sister Mary

Josephs nodule: a clinical and histologic study. J Am Acad

Dermatol 1984; 10: 610–615.

International Journal of Dermatology 2013, 52, 587–588 ª 2013 The International Society of Dermatology

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