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 Table of Contents  
CASE REPORT
Year : 2014  |  Volume : 3  |  Issue : 5  |  Page : 420-422

Umbilical cutaneous nodule: A diagnostic dilemma


1 Department of Surgery, Kalpana Chawla Government Medical College, Karnal, India
2 Department of Surgery, SHKM GMC, Nalhar, Mewat, Haryana, India
3 Department of Pathology, Dr. RML Postgraduate Institute of Medical Sciences, New Delhi, India

Date of Web Publication5-Aug-2014

Correspondence Address:
Sunder Goyal
Department of Surgery, Kalpana Chawla Government Medical College, Karnal, Haryana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2278-0513.138070

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  Abstract 

Umbilical cutaneous nodule can be due to infection, skin ailments and tumors. It can be primary or secondary in origin. When it is secondary due to metastasis from visceral malignancy then it is named as Sister Mary Joseph nodule. It can be a presenting symptom and sign of hidden malignancy and thus poses a diagnostic dilemma for physician. Our case presented to us with ulcerated umbilical nodule and subsequent ultrasound revealed accompanied urinary bladder malignancy. Mostly, these nodules are adenocarcinoma but our was an unusual case of transitional cell carcinoma.

Keywords: Sister Mary Joseph nodule, transitional cell carcinoma, urinary bladder, visceral malignancy


How to cite this article:
Goyal S, Jain A, Goyal S. Umbilical cutaneous nodule: A diagnostic dilemma. Clin Cancer Investig J 2014;3:420-2

How to cite this URL:
Goyal S, Jain A, Goyal S. Umbilical cutaneous nodule: A diagnostic dilemma. Clin Cancer Investig J [serial online] 2014 [cited 2019 Jul 23];3:420-2. Available from: http://www.ccij-online.org/text.asp?2014/3/5/420/138070


  Introduction Top


Periumbilical cutaneous metastasis from hidden visceral malignancies has been named as " Sister Mary Joseph's (SMJ) nodule." It was named in respect of SMJ in 1928. SMJ was first observer to establish a correlation between umbilical nodules and carcinoma. She was first assistant of Dr. William J. Mayo a surgeon at St. Mary's hospital at Rochester, Minnesota (presently the Mayo Clinic). [1] Primary malignancy in the gastrointestinal tract and ovary can spread to umbilicus. Rarely, malignancy of endometrium, prostate, lung, urinary bladder and from unknown primaries can also spread to umbilicus. [2],[3] It is more common in females. In a report of 34 cases, male outnumbered females by the ratio of 1.4:1. [4] In one study, in 29% of umbilical metastasis, the origin of the primary lesion was unknown, [5] thus umbilical nodule poses a diagnostic dilemma for clinician.


  Case report Top


A 65-year-old woman presented with an ulcerated umbilical nodule with yellowish discharge for the last 2 months. On examination, there was a 2 cm × 1 cm hard ulcerated, brownish nodule occupying lower half of the umbilicus [Figure 1]. Urine examination showed field full of RBC/high power field. Blood investigations revealed hemoglobin level as 7 g, blood urea 45 mg/dl and serum creatinine 2.2 mg/dl. Fine-needle aspiration (FNA) cytology of the umbilical nodule revealed transitional cell carcinoma [Figure 2].
Figure 1: Umbilical ulcerated nodule in patient

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Figure 2: Fine-needle aspiration cytology of umbilical nodule showing transitional carcinoma of urinary bladder

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Subsequent ultrasound of the abdomen showed growth occupying whole of the urinary bladder with thickening at dome [Figure 3]. There were metastases in the liver. Due to poverty, patient refused for computerized tomography. Cystoscopy and biopsy of bladder growth was carried out under local anesthesia. Whole bladder was affected with growth including dome. Histopathology of bladder growth revealed transitional cell carcinoma. Adjuvant chemotherapy was planned due to disseminated disease. A combination of cisplatin, methotrexate and vinblastine was planned. Patient refused treatment due to the high cost of drugs and was lost to follow-up.
Figure 3: Ultrasound showing urinary bladder growth

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  Discussion Top


The umbilical nodule can occur due to various diseases. About 38% umbilical nodules are primary tumors of the umbilicus; 32% are due to endometriosis and 30% are due to metastatic disease from a distant location. [6] Sometimes, the SMJ nodule is the sole indication of an occult malignancy. An SMJ nodule was discovered prior to the diagnosis of the primary tumor in 152 of 368 cases in one study. [7] In another study, 45 of 85 patients developed an SMJ nodule 1-12 months prior the diagnosis of the primary malignancy. [8] Sometime, umbilical metastases are the first sign of a systemic malignancy as in our case. Rarely discharge from umbilical nodule can be the first sign of recurrence in carcinoma ovary. [9] For these reasons, the presence of a peri-umbilical nodule necessitates assessment for an occult intra-abdominal malignancy.

The incidence of SMJ nodule is only 1-3% of all intra-abdominal or pelvic malignancies. Studies have shown that most of the umbilical lesions are adenocarcinoma, rarely a squamous carcinoma, melanoma or sarcoma, [10] but uncommonly it can be transitional cell carcinoma as in our case. [11]

Among all reported cases of SMJ nodule, 35-65% metastasize from gastrointestinal malignancies; 12-35%, from the genitourinary tract; 15-30%, from unknown sites; and 3-6%, from the lung and breast. [12] Barrow. in his study had reported that cancer of the stomach accounted for 25%, ovary 12.4%, colon and rectum 10.0%, pancreas 7.4%, gallbladder 3.4%, uterus, 3.0%, liver 2.4%, endometrium 1.4%, sarcoma 1.5% and small bowel 1.0%. [13] Rarely, it can be a metastatic nodule from malignant mesothelioma. [14]

The mechanism of metastasis of the primary tumor to umbilicus is not clear; however, several hypotheses have been proposed. First hypothesis is that spread is via peritoneal infiltration (the most common route) or through arteries, veins, or lymphatic channels. Another is that spread occurs through embryonic structures such as the urachus, round ligament of liver, vitello intestinal duct remnant, or obliterated vitelline artery. There is a connection between liver and umbilicus via venous and lymphatic channels and these channels seems to be means of mutual seedlings but which organ is affected first is not clear. [12]

Generally, SMJ nodule presents as a firm irregular nodule, size range from 1 cm to 1.5 cm, and may reach up to 10 cm in diameter. [12] It can be a fissured or ulcerated nodule, and depending from origin, it can secrete serous, mucinous, purulent, or bloody discharge. The lesion may be of different colors such as white, bluish violet, brownish red with or without itching. [15]

Tumors such as Paget's disease, angioma, and other ailments such as umbilical hernia, endometriosis, hypertrophic scar, keloid, mycosis, psoriasis, pyogenic granuloma, melanocytic nevi, epithelial inclusion cysts, epidermoid cysts, omphaliths, eczema, squamous cell carcinoma and basal cell carcinoma are differential diagnoses of an umbilical nodule. [16]

When a patient presents with an umbilical nodule, to establish an easy and early diagnosis, the following diagnostic tests like: Culture and special microbiology staining of ulcer specimen, FNA of the umbilicus for cytology, umbilical biopsy for histopathological examination, immunohistochemistry, imaging modalities (ultrasound, computed tomography, magnetic resonance imaging scan of the abdomen) and tumor markers such as carcinoembryonic antigen, prostate-specific antigen, and cancer antigen are essential. [10],[16]

The presence of SMJ nodule means disseminated disease and it means that it is not amenable to cure. Without treatment, life expectancy is about 2-11 months. However, some issues seem to control the prognosis of such patients like direct invasion by the peritoneum is associated with a worse prognosis. The prognosis is good when the metastasis is discovered before the primary tumor. [10]

Recent reports have suggested that prognosis is better with aggressive management, the mean survival being 17.6-21 months with surgery and adjuvant chemotherapy instead of surgery alone (7.4 months) or chemotherapy alone (10.3 months). However, surgery is usually recommended only in patients with a solitary umbilical metastasis. Surgery should be avoided in cases with widespread disseminated disease. In case of widespread disease, effective palliation can be attained with adjuvant chemoradiotherapy. Prognosis depends on the diseased organ. It has been noted that patients with ovarian cancer have a better survival rate than do patients with other malignancies. [10],[12]


  Conclusion Top


Sister Mary Joseph nodule is an uncommon manifestation of visceral and other malignancies. It can be a presenting symptom or sign of undiagnosed underlying malignancy, or an alarming symptom or sign of widespread disease. Although similar cases have been reported in the literature, this manifestation of the transitional carcinoma of urinary bladder is rare and clinician must be conscious of this rare clinical entity so that they can diagnose the primary cancer in early stage and thus can stop its progression and avoid its recurrence.

 
  References Top

1.Steensma DP. Sister (Mary) Joseph′s nodule. Ann Intern Med 2000;133:237.  Back to cited text no. 1
    
2.Deb P, Rai RS, Rai R, Gupta E, Chander Y. Sister Mary Joseph nodule as the presenting sign of disseminated prostate carcinoma. J Cancer Res Ther 2009;5:127-9.  Back to cited text no. 2
    
3.Spiess PE, Kassouf W, Tukaram K, Roy I. Metastatic umbilical mass from transitional cell carcinoma of the bladder. Can J Urol 2005;12:2856-8.  Back to cited text no. 3
    
4.Chalya PL, Mabula JB, Rambau PF, McHembe MD. Sister Mary Joseph′s nodule at a University teaching hospital in northwestern Tanzania: A retrospective review of 34 cases. World J Surg Oncol 2013;11:151.  Back to cited text no. 4
    
5.Hsu JT, Lin CY, Jan YY, Chen HM, Chen MF. An umbilical mass as the initial presentation of pancreatic carcinoma. Chang Gung Med J 2006;29:17-20.  Back to cited text no. 5
    
6.Soares LC, Almeida JP. Sister Mary Joseph nodule and peritoneal carcinomatosis from squamous cell cervical carcinoma. Proc Obstet Gynecol 2013;3:1-6.  Back to cited text no. 6
    
7.Powell FC, Cooper AJ, Massa MC, Goellner JR, Su WP. Sister Mary Joseph′s nodule: A clinical and histologic study. J Am Acad Dermatol 1984;10:610-5.  Back to cited text no. 7
    
8.Khan AJ, Cook B. Metastatic carcinoma of umbilicus: "Sister Mary Joseph′s nodule". Cutis 1997;60:297-8.  Back to cited text no. 8
    
9.Gupta P, Kumari A, Kriplani A, Bhatla N. Sister Mary Joseph′s nodule as the first and only sign of recurrence in a case of stage Ia carcinoma ovary. BMJ Case Rep 2014;2014.  Back to cited text no. 9
    
10.Serhrouchni KI, El Fatemi H, Chbani L, Harmouch T, Amarti A. Umbilical metastasis: A report case and review of the literature. Pathol Discov 2013;1:3.  Back to cited text no. 10
    
11.Rajan N, Makhuli ZN, Humphrey DM, Batra AK. Metastatic umbilical transitional cell carcinoma from a bladder diverticulum. J Urol 1996;155:1700.  Back to cited text no. 11
    
12.Palaniappan M, Jose WM, Mehta A, Kumar K, Pavithran K. Umbilical metastasis: A case series of four Sister Joseph nodules from four different visceral malignancies. Curr Oncol 2010;17:78-81.  Back to cited text no. 12
    
13.Barrow MV. Metastatic tumors of the umbilicus. J Chronic Dis 1966;19:1113-7.  Back to cited text no. 13
[PUBMED]    
14.Malani A, Gupta C, Kapadia L. Unusual presentation of a rare tumor: Umbilical metastasis Sister Mary Joseph′s nodule of malignant peritoneal mesothelioma. Internet J Fam Pract 2005;4:1.  Back to cited text no. 14
    
15.Urbano FL. Sister Joseph′s nodule. Hosp Physician 2001;37:33-5.  Back to cited text no. 15
    
16.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. 16
    


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  [Figure 1], [Figure 2], [Figure 3]



 

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