Pseudo Sister Mary Joseph\'s nodule

THE AMERICAN JOURNAL OF GASTROENTEROLOGY © 1999 by Am. Coll. of Gastroenterology Published by Elsevier Science Inc.

Vol. 94, No. 7, 1999 ISSN 0002-9270/99/$20.00 PII S0002-9270(99)00292-0

Pseudo Sister Mary Joseph’s Nodule Rafael Amaro, M.D., Jeffrey A. Goldstein, M.D., Cynthia M. Cely, M.D., and Arvey I. Rogers, M.D., M.A.C.G. Division of Gastroenterology, University of Miami School of Medicine, and the Miami VA Medical Center, Miami, Florida

ABSTRACT The Sister Mary Joseph’s nodule is a significant finding in the physical examination. It is sometimes the only indication of an intra-abdominal metastatic malignancy. We report a patient who presented with an umbilical nodule that was discovered to be an omphalith. A review of the literature discusses the Sister Mary Joseph’s nodule and this unusual finding. (Am J Gastroenterol 1999;94:1949 –1950. © 1999 by Am. Coll. of Gastroenterology)

nodule, a thorough digital examination of the umbilicus revealed dark debris that was hardened and dry and that was impacted deeply in a long umbilicus, the evacuation of which obviated the need for biopsy of the nodule (which no longer existed). The abdominal CT scan was cancelled and the patient was treated for presumptive diabetic gastroparesis. A subsequent scintigraphic gastric emptying study confirmed the diagnosis of gastroparesis and, on follow-up, the patient has improved clinically and has started gaining weight.

INTRODUCTION The Sister Mary Joseph’s nodule is an important diagnostic finding, as it often is the first clue, and sometimes the only physical indication, of an advanced intra-abdominal neoplasm. It is associated most often with gastrointestinal or gynecological malignancies (1). The lesion is described as a firm, raised, nontender, and nonpruritic subcutaneous nodule. We report a man who presented with an umbilical nodule that was thought to represent this nodule but that was discovered to be an omphalith. We thought that it would be of interest to review what is known about the Sister Mary Joseph’s nodule as well as its differential diagnosis. As well, we include information known about omphaliths.

CASE REPORT A 65-yr-old white man with a history of diabetes mellitus type II, hypertension, peripheral vascular disease, peripheral neuropathy, gastroesophageal reflux disease, and a depressed mood presented with a 6-month history of significant weight loss and early satiety. He had been constipated for 1 month but denied abdominal pain, melena, or rectal bleeding. He was taking fosinopril, simvastatin, aspirin, lansoprazole, and insulin. He had no history of smoking or alcohol use and no family history of colon cancer. Physical exam was remarkable for peripheral neuropathy and a palpable, subcutaneous, hard, nontender, 1.5-cm periumbilical nodule. With the suspicion of a Sister Mary Joseph’s nodule associated with an underlying malignancy, the patient was scheduled for a biopsy, colonoscopy, and a CT scan of the abdomen. Recent esophagogastroduodenoscopy had revealed grade I reflux esophagitis. Colonoscopy did not show masses or polyps. In preparation for the biopsy of the

DISCUSSION This patient presented with an omphalith that was confused with a Sister Mary Joseph’s nodule (the pseudo Sister Mary Joseph’s nodule). An omphalith, a derivation of the Greek words omphalos (navel) and lithos (stone), results from the concretion of keratinous and sebaceous material in the umbilical region (2, 3). Usually asymptomatic, it may go unnoticed for many years, although it can cause surrounding secondary infection or ulceration with associated symptoms (4). The pathogenesis is thought to be inadequate hygiene allowing accumulation of keratin and sebum in a deep umbilical cleft, which after exposure and evaporation of moisture will adopt its usual stonelike consistency. After extraction, a black surface can be noticed (possibly secondary to oxidation of lipids), with a white, moistened underside that may release a putrid odor. Microscopic examination will show laminated keratin, frequently interspersed with amorphous material that may contain hair and bacteria (4). We could not find a case of an omphalith described in the gastroenterology literature, but we consider that we should be aware of this entity and should include it in our differential diagnosis of umbilical nodules to avoid an extensive and unproductive workup searching for an underlying intra-abdominal malignancy. Sister Mary Joseph was born as Julia Dempsey in Salamanca, NY. Her parents were Irish immigrants who had moved to Minnesota before the Civil War. She belonged to the order of the Sisters of Saint Francis, practiced nursing at Saint Mary’s Hospital, and was the first person to draw the attention of Dr. William Mayo to the presence of a periumbilical nodule as a sign of an abdominal metastatic malignancy. Apparently nothing was published about her ob-

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Table 1. Primary Site of Umbilical Metastasis Primary Site

Patients, n (%)

Digestive system Stomach Colon or rectum Pancreas Gallbladder Small bowel Other (liver, esophagus) Gynecological Ovary Uterus Cervix Breast Other (fallopian tube, vulva, vagina) Urinary (prostate, kidney, bladder, penis) Lung Other (mesothelioma, myeloma, lymphoma) Unknown

213 (52.3) 95 (23.3) 61 (14.9) 37 (9.0) 10 (2.4) 8 (1.9) 2 (0.4) 114 (28) 68 (16.7) 24 (5.8) 10 (2.4) 7 (1.7) 5 (1.1) 8 (1.9) 5 (1.2) 4 (0.8) 63 (15.4)

Modified from: Galvan˜ VG. Sister Mary Joseph’s nodule. Ann Intern Med 1998;128: 410.

servation until Hamilton Bailey named this nodule after her in 1949 (5, 6). A review of four large series (2, 7–9) reveals that the stomach is the most common primary malignant site (18 – 28%), followed by ovary (8 –24%), colon (10 –18%) and pancreas (7–15%). The primary site is not identified in 15–29% of cases. Table 1 summarizes a recent MEDLINE search of cases reported in the last 30 yr confirming these percentages (1). Metastasis to the umbilicus may occur through different routes, including: direct extension from the anterior peritoneal surface; lymphatic spread from the axillary, inguinal, and para-aortic nodes; and venous communication through the lateral thoracic vein, internal mammary veins, and portal venous system. It may also occur via arterial embolization, or along ligaments of embryonic origin (the round ligament of the liver, the median umbilical ligament from the urachus, and a fibrous obliterated vitelline

artery). Tumor implantation at the umbilicus after laparoscopic cholecystectomy for unsuspected gallbladder carcinoma has also been reported (10). In addition to umbilical metastases, the differential diagnosis of a Sister Mary Joseph’s nodule includes primary malignancies such as basal cell carcinoma, malignant melanoma, and myosarcoma, as well as various nonneoplastic entities including endometriosis, papillomas, teratomas, foreign body granulomas, epidermoid cysts, nevi, dermatofibromas, and neurofibromas (7). Reprint requests and correspondence: Arvey I. Rogers, M.D., Division of Gastroenterology, University of Miami and Miami VA Medical Center, 1201 NW 16th Street, Miami, FL 33125-1624. Received June 1, 1998; accepted Sep. 17, 1998.

REFERENCES 1. Galvan˜ VG. Sister Mary Joseph’s nodule. Ann Intern Med 1998;128:410. 2. Steck WD, Helwig EB. Tumors of the umbilicus. Cancer 1965;18:907–15. 3. Powell FC, Su WPD. Dermatoses of the umbilicus. Int J Dermatol 1988;27:150 – 6. 4. Swanson SL, Woosley JT, Fleischer AB, et al. Umbilical mass. Arch Dermatol 1992;128:1265–70. 5. Bailey H. Demonstrations of physical signs in clinical surgery, 11th ed. Baltimore: Williams & Wilkins, 1949. 6. Hill M, O’Leary JP. Vignettes in medical history: Sister Mary Joseph and her node. Am Surg 1996;62:328 –9. 7. Powell FC, Cooper AJ, Massa MC, et al. Sister Mary Joseph’s nodule: A clinical and histologic study. J Am Acad Dermatol 1984;10:610 –5. 8. Barrow MV. Metastatic tumors of the umbilicus. J Chron Dis 1966;19:1113–7. 9. Heatley MK, Toner PG. Sister Mary Joseph’s nodule: A study of the incidence of biopsed umbilical secondary tumours in a defined population. Br J Surg 1989;76:728 –9. 10. Nally C, Preshaw RM. Tumour implantation at umbilicus after laparoscopic cholecystectomy for unsuspected gallbladder carcinoma. Can J Surg 1994;37:243– 4.