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Sister Mary Joseph Nodule as a Presenting Sign of Pancreatobiliary Adenocarcinoma

by Michael L. Shelling, MD; Magalys Vitiello, MD; Emma L. Lanuti, MD; Senen Rodriguez, MD; Francisco A. Kerdel, BSC, MBBS

Michael L. Shelling, MD is from Department of Dermatology and Cutaneous Surgery, University of Miami Miller School of Medicine, University of Miami Hospital, Miami, Florida. Magalys Vitiello, MD, is from Florida Academic Dermatology Centers, Miami, Florida; Woodhull Medical Center, Brooklyn, New York. Emma L. Lanuti, MD, is from Department of Dermatology and Cutaneous Surgery, University of Miami Miller School of Medicine, University of Miami Hospital, Miami, Florida. Senen Rodriguez, MD, is from Department of Pathology, University of Miami Miller School of Medicine, University of Miami Hospital, Miami, Florida. Francisco A. Kerdel, BSC, MBBS, is from Department of Dermatology and Cutaneous Surgery, University of Miami Miller School of Medicine, University of Miami Hospital, Miami, Florida; Florida Academic Dermatology Centers, Miami, Florida

DISCLOSURE: Drs. Shelling, Vitiello, Lanuti, and Rodriguez report no relevant conflicts of interest. Dr. Kerdel has received grants, participated in advisory boards, and is a speaker for: Abbott, Amgen, Astellas, Celgene, Pfizer, Merck, Centocor, Genentech, and Stiefel.

ABSTRACT
Sister Mary Joseph nodules represent metastatic cancer of the umbilicus. These malignancies are usually associated with the ovary and gastrointestinal tract. The authors report the case of a Sister Mary Joseph nodule originating from the bifurcation of the common hepatic duct. Umbilical nodules should prompt clinical evaluations, as these tumors are usually associated with poor prognosis. (J Clin Aesthet Dermatol. 2012;5(10):44–46.)

A 79-year-old man presented to the emergency room with a two-month history of mild periumbilical pain and an enlarging umbilical mass. For the past two weeks, he had experienced worsening right upper quadrant pain and abnormally dark urine, light-colored stool, and yellowing of his skin. Over this same period of time, he experienced anorexia and had lost nearly 15 to 20 pounds. He denied having similar complaints in the past and reported a normal colonoscopy nearly two years earlier.

On physical exam, the patient had a 3x3cm hard, firm, ulcerated umbilical nodule with a diffuse infiltration of the entire periumbilical area (Figure 1). He also had right upper quadrant tenderness with a firm liver edge palpated 2cm below the costal margin.

ASSESSMENT
The worsening clinical symptoms and apparent jaundice prompted an extensive diagnostic workup. Hepatitis panel was negative for hepatitis B and C. Liver function tests were abnormally elevated and tumor markers demonstrated a markedly elevated CA19-9, carcinoembryonic antigen (CEA), and alpha-fetoprotein (AFP). The clinical picture was consistent with obstructive jaundice.

A right upper quadrant ultrasound showed an enlarged liver with mild coarse echo texture and dilated intrahepatic bile ducts with a hypoechoic lesion in the right lobe of the liver. Computed tomography (CT) scan of the abdomen and pelvis showed an enlarged liver with an ill-defined mass in the gallbladder fossa with hypoattenuated lesions in the superior aspect of the liver and retrocrural lymph nodes. The large umbilical lesion was easily appreciated on crosssectional image from the CT scan (Figure 2). Magnetic resonance cholangio-pancreatography revealed a large area of altered signal intensity in the central portion of the liver with epicenter in the region of the gallbladder fossa with involvement of the liver parenchyma and extension to the porta hepatis. There was evidence of biliary obstruction at the level of the liver hilum with marked dilatation of the intrahepatic biliary ducts and a hypervascular lesion in the head of the pancreas.

A shave biopsy was performed on the ulcerated umbilical tumor. Histological sections (Figure 3) revealed anaplastic glands without necrosis in the dermis, surrounded by a marked desmoplastic reaction. They had no relation to the epidermis or skin adnexals and were morphologically consistent with a well-to-moderately differentiated adenocarcinoma of pancreaticobiliary origin.

In order to identify the primary etiology, both a colonoscopy and esophago-gastro-duodenoscopy were performed, which did not show any underlying pathology. Subsequently, an endoscopic retrograde cholangiopancreatography revealed a tumor within the biliary tree consistent with a Klatskin tumor, an adenocarcinoma located at the bifurcation of the common hepatic duct. Once the diagnosis of metastatic cholangiocarcinoma was established, palliative stenting of the biliary tree and hepatic ducts were done to alleviate the obstruction.

DISCUSSION
Metastatic cancer of the umbilicus is usually associated with cancers of the ovary and gastrointestinal tract. This rare manifestation of metastatic disease was recognized and best appreciated by a surgical assistant of Dr. William Mayo, Sister Mary Joseph, whose name has since been used to describe these umbilical nodules.[1–3] These metastatic lesions are predominantly from gastrointestinal (52%) and gynecological neoplasms (28%), most commonly from the stomach (23%), colon (15%), pancreas (10%), and ovary (16%), and less frequently from the uterus, cervix, gallbladder, and small intestine.[4] In a large retrospective study, cutaneous involvement with internal carcinomas occurred in approximately five percent of cases, with a large number of these lesions identified in the umbilicus.[5] Importantly, metastatic lesions can reach the umbilicus via propagation through lymphatic ducts, the venous network, arterial spread, contiguous extension, extension along the ligaments of embryologic origin, or even through iatrogenic seeding with laparoscopy.[2,6,7] These different pathways may help explain why there is such a wide array of malignant tumors that can produce these nodules.

The presence of these lesions is often a poor prognostic factor, as these patients frequently have advanced metastatic disease at the time of initial diagnosis. In several studies, the average survival after the appearance of these nodules was approximately 10 to 11 months.2,7 In fact, one of the earlier reports by Clements et al[8] suggested that the presence of this finding would essentially preclude a patient from curative surgical resection.[8] In this case, the patient initially developed the umbilical nodule, but only presented for further evaluation and diagnostic workup after the development of obstructive jaundice and worsening abdominal pain. At the time of diagnosis, he was found to have suspected metastatic disease involving the pancreas and liver with widespread involvement of lymph nodes, as may be seen in many of these cases.

It is essential for all physicians to be aware that an umbilical nodule may be the first presenting sign of internal malignancy and should prompt further clinical evaluation. In particular, this case demonstrates the value of a skin biopsy in determining the diagnosis of an unknown internal malignancy, as this may direct further workup and possibly spare the patient from additional invasive testing. In addition, the presence of Sister Mary Joseph nodule is often a predictor of poor prognosis, thus recognizing this finding and establishing the diagnosis early in the course of the disease allows earlier treatment and possibly improves overall survival. Not surprisingly, this umbilical metastasis may also represent the first sign of recurrent cancer.

REFERENCES
1. Key JD, Shephard DAE, Waiters W. Sister Mary Joseph’s nodule and its relationship to diagnosis of carcinoma of the umbilicus. Minn Med. 1976;59:561–566.
2. Powell FC, Cooper AJ, Massa MC, Goellner JR, Danie Su WP. Sister Mary Joseph’s nodule: a clinical and histologic study. J Am Acad Dermatol. 1984;10:610–615.
3. Albano EA, Kanter J. Sister Mary Joseph’s Nodule. N Engl J Med. 2005;352:1913.
4. Galvañ VG. Sister Mary Joseph’s nodule. Ann Intern Med. 1998;128:410.
5. Lookingbill DP, Spangler N, Sexton FM. Skin involvement as the presenting sign of internal carcinoma. A retrospective study of 7316 cancer patients. J Am Acad Dermatol. 1990;22:19–26.
6. Zeligman I, Schwilm A. Umbilical metastasis from carcinoma of the colon. Arch Dermatol. 1974;110:911–912.
7. Dubreuil A, Dompmartin A, Barjot P, Louvet S, Leroy D. Umbilicial metastasis or Sister Mary Joseph’s nodule. Int J Dermatol. 1998;37:7–13.
8. Clements AB. Metastatic carcinoma of the umbilicus. JAMA. 1952;150:556.

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