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Year : 2018  |  Volume : 15  |  Issue : 4  |  Page : 229-231

A classical case of mucinous appendiceal adenocarcinoma presenting with Sister Mary Joseph nodule

Department of General Surgery, Apollo BGS Hospitals, Mysore, Karnataka, India

Date of Web Publication5-Dec-2018

Correspondence Address:
Keyur Yogesh Patel
Department of General Surgery, Apollo BGS Hospitals, Adichunchanagiri Road, Kuvempunagar, Mysore - 570 023, Karnataka
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/am.am_65_18

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Primary appendiceal adenocarcinomas are rare malignant neoplasms of the appendix. Carcinomas of the appendix are usually well-differentiated mucinous adenocarcinoma, which tend to produce pseudomyxoma peritonei, also known as gelatinous ascites, and do not show metastatic spread until late in the disease process. Umbilical metastasis from visceral malignancies described as Sister Mary Joseph nodule (SMJN) is rare and represents only 10% of all cutaneous metastases. To the best of our knowledge, only four cases of adenocarcinoma of the appendix metastasizing to the umbilicus have been reported in the medical literature. Here, we present a case of mucinous appendiceal adenocarcinoma which occurred in a 75-year-old female, who presented with SMJN.

Keywords: Primary appendiceal adenocarcinomas, pseudomyxoma peritonei, Sister Mary Joseph nodule, umbilical metastasis

How to cite this article:
Srinath S, Patel KY, Jayanth B N. A classical case of mucinous appendiceal adenocarcinoma presenting with Sister Mary Joseph nodule. Apollo Med 2018;15:229-31

How to cite this URL:
Srinath S, Patel KY, Jayanth B N. A classical case of mucinous appendiceal adenocarcinoma presenting with Sister Mary Joseph nodule. Apollo Med [serial online] 2018 [cited 2020 Oct 24];15:229-31. Available from: https://www.apollomedicine.org/text.asp?2018/15/4/229/246892

  Introduction Top

Primary appendiceal adenocarcinomas are very rare malignant neoplasms accounting for 0.05%–0.2% of all appendectomies and only 6% of all malignant tumors of the appendix.[1] It constitutes <0.5% of all gastrointestinal neoplasms.[2] Pseudomyxoma peritonei (PMP) is a rare condition, more common in females during 40–50 years of age with an incidence of one in million per year. Although PMP has been reported as originating from many intra-abdominal organs, most cases arise from the appendix. The frequency of cutaneous metastases from internal malignancies ranges from 5% to 9%.[3] However, umbilical metastasis from visceral malignancies described as Sister Mary Joseph nodule (SMJN) is rare and represents only 10% of all cutaneous metastasis.[3] To the best of our knowledge, only four cases of adenocarcinoma of the appendix metastasizing to the umbilicus have been reported in the medical literature.[4],[5],[6] We hereby present a classic case of mucinous appendiceal adenocarcinoma presenting with SMJN in a 75-year-old female.

  Case Report Top

A 75-year-old female presented to the emergency room with complaints of severe abdominal pain and abdominal distension of 3-day duration. She denied any complaints of nausea, vomiting, fever, chronic constipation, or recent weight loss. She was a known case of hypertension and had no other medical comorbidities, with nil surgical history. On examination, her vitals were stable and abdomen was grossly distended, diffusely tender in all quadrants, absent bowel sounds, no palpable mass per abdomen, and had no signs of peritonitis. She had a 3 cm × 3 cm nodule over umbilicus which was firm, irreducible, had smooth surface, and no discharging sinus. All routine investigations were normal. Ultrasonography abdomen revealed gross ascites with septations which limited the assessment by the study. Contrast-enhanced computed tomography [Figure 1] abdomen showed calcified lesion abutting the cecum; the appendix was not visualized, calcific sediments over parietal peritoneum, scalloping of liver surface, and confirmed the subcutaneous origin of the umbilical nodule [Figure 2]. Hence, diagnosis of PMP [Figure 3] with suspected umbilical metastasis secondary to suspected appendicular mucinous cystadenocarcinoma was made which was confirmed on histopathological examination of specimen postsurgery [Figure 4].
Figure 1: Contrast-enhanced computed tomography abdomen coronal sections

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Figure 2: Intraoperative findings

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Figure 3: Gelatinous material drained from the peritoneal cavity, i.e., pseudomyxoma peritonei

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Figure 4: Final histopathology of appendicular mass and umbilical nodule

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  Discussion Top

SMJN is described as nodule or mass that can be either seen or felt in the periumbilical region and usually corresponds to umbilical metastasis from visceral malignancies. Sister Mary Joseph (1856–1939), the senior nurse and surgical assistant of Dr. William Mayo at St Mary's Hospital (now Mayo Clinic) in Rochester, Minnesota, was the first one to observe that many patients of intra-abdominal malignancies also had umbilical nodule. Storer in 1864 described the first case of umbilical metastasis even before the origin of the term SMJN. However, it was only in 1949; Sir Hamilton Bailey coined the term “Sister Mary Joseph's Nodule” in his book Demonstration of Physical Signs in Clinical Surgery to describe this entity.[7] The clinical features of SMJN can vary from painless firm irregular small nodule to sometimes painful ulcerated mass with exudate-like pus, blood, mucinous, or serous fluid. Sometimes, it can present as a diffuse subcutaneous induration with indistinct borders. SMJN constitutes 83%–85% of all malignant umbilical tumors, being much more common than primary malignant umbilical tumors. Among these, the most prominent histological type is adenocarcinoma (75%) with other histological variants such as squamous cell carcinoma, undifferentiated tumors, carcinoid, sarcoma, mesothelioma, melanoma, and lymphoma being extremely rare. Rich lymphatic and vascular supply of umbilicus, along with its association with embryologic remnants, makes it a potential site for metastasis of neoplastic cells.[8] In addition, direct extension from a contiguous tumor and direct implantation following laparoscopy may contribute to the spread of tumors to the umbilicus.[9] Often, it could represent the initial presenting symptom/sign for an underlying internal malignancy. Benign conditions such as endometriosis, hernia, myxoma, epithelial inclusion cysts, omphalithiasis, and abscesses can mimic SMJN.

Imaging modalities such as ultrasonography, computed tomography (CT), magnetic resonance imaging, and positron emission tomography provide assistance to establish the diagnosis. Tissue diagnosis by biopsy, either excisional or fine-needle aspiration, helps identifying the possible primary site.[10] Immunohistochemical marker studies and ultrastructural examination have been utilized in identifying origin of anaplastic tumors. In approximately 72% of the cases of unknown primary tumor, immunohistochemical analysis helped to determine the cellular origin.[11] In addition, the use of electron microscopy and cytogenetic analysis enhances the diagnostic sensitivity and specificity. Establishing the diagnosis of SMJN is ominous indicating an advanced stage of cancer with a grave prognosis. Long-term survival of patients is uncommon.

Berger, in 1882, first described primary appendicular neoplasm. Primary appendiceal cancer is rare accounting for <0.5% of all gastrointestinal tumors.[2] Three histological variants of the primary appendicular adenocarcinoma have been illustrated, including mucinous adenocarcinoma, intestinal type (goblet and colonic), and signet-ring cell cancers. Patients with appendiceal malignancies generally present with the symptoms suggestive of acute appendicitis or as a palpable abdominal mass. However, our patient lacked any symptoms and signs of acute appendicitis. Liu et al. reported a case similar to our case of appendiceal carcinoma presenting with only initial sign of SMJN.[5] The sex distribution is usually equal. Overall 5-year survival is 44% for appendicular mucinous tumors, 52% for the colonic subtype, and 20% for the signet-ring cell subtype.[12] When associated with intraperitoneal dissemination and accumulation of mucinous implants, the prognosis is similar to that of peritoneal carcinomatosis with 5-year survival being <10%.[12]

  Conclusion Top

Clinicians must exercise caution while dealing with umbilical lesions that do not exactly fit to the diagnosis of hernia and meticulously evaluate for an underlying malignancy.

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Conflicts of interest

There are no conflicts of interest.

  References Top

O'Donnell ME, Badger SA, Beattie GC, Carson J, Garstin WI. Malignant neoplasms of the appendix. Int J Colorectal Dis 2007;22:1239-48.  Back to cited text no. 1
Ozakyol AH, Sariçam T, Kabukçuoğlu S, Cağa T, Erenoğlu E. Primary appendiceal adenocarcinoma. Am J Clin Oncol 1999;22:458-9.  Back to cited text no. 2
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.  Back to cited text no. 3
Papalas JA, Selim MA. Metastatic vs. primary malignant neoplasms affecting the umbilicus: Clinicopathologic features of 77 tumors. Ann Diagn Pathol 2011;15:237-42.  Back to cited text no. 4
Liu H, Yuan SS, Lin KJ, Huang CK, Hung CM, Chen YS. Unusual presentation of appendiceal adenocarcinoma: Report of a case. Formosan J Surg 2008;41:217-20.  Back to cited text no. 5
Podugu A, Alvarez A, Pimentel R, Ukleja A. Mucinous appendiceal adenocarcinoma presenting as Sister Mary Joseph nodule: Case report and brief review of literature. J Gastrointest Dig Syst 2015;5:1000339.  Back to cited text no. 6
Bailey H. Demonstrations of Physical Signs in Clinical Surgery. New York: Williams and Wilkins; 1949. p. 227.  Back to cited text no. 7
Goodheart RS, Cooke CT, Tan E, Matz LR. Sister Mary Joseph's nodule. Med J Aust 1986;145:477-8.  Back to cited text no. 8
Yusuf M, Azhar R. Umbilical metastasis from oesophageal squamous cell carcinoma: A case report. Internet J Gastroenterol 2004;3.  Back to cited text no. 9
Gupta RK, Naran S, McHutchison AG, Lallu S, Fauck RJ. Diagnosis of umbilical metastasis (Sister Mary Joseph's nodule) by fine-needle aspiration: An immunocytochemical study. Diagn Cytopathol 1994;10:126-9.  Back to cited text no. 10
Schneider V, Smyczek B. Sister Mary Joseph's nodule. Diagnosis of umbilical metastases by fine needle aspiration. Acta Cytol 1990;34:555-8.  Back to cited text no. 11
Pai RK, Longacre TA. Appendiceal mucinous tumors and pseudomyxoma peritonei: Histologic features, diagnostic problems, and proposed classification. Adv Anat Pathol 2005;12:291-311.  Back to cited text no. 12


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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