Sister Mary Joseph's nodule (SMJN) is an eponym used to describe the metastatic lesion of the umbilicus. Umbilical metastases are rare and its occurrence as a first manifestation of an asymptomatic primary malignancy is very rare. The gallbladder is the source of SMJN in 2%-3% of cases. SMJN can present in any one of the following ways; first manifestation of an occult primary malignancy, an indication of recurrence in a patient with a previous malignancy, or as progression of an underlying symptomatic primary disease. Our study emphasizes the importance of identifying this very useful and easily applicable clinical sign by careful physical examination of the abdomen, and also the need for proper clinical evaluation of any umbilical lesion, and the histological diagnosis. In the present study we report on a case of Sister Mary Joseph's Nodule as the presentation of an asymptomatic gallbladder carcinoma, and review the relevant literature

Deshmane, V

Kumar, V

Mukunda, M

Pampanagouda, SKM

Suryadevara, S

Cognitive Sciences ePrint Archive

1 
 
 
 
 
Case Report: 
Umbilical Metastasis as the Manifestation of an Asymptomatic Gallbladder Carcinoma 
Authors 
Pampanagouda SKM, Post Graduate Student in Surgical Oncology, 
Sailaja Suryadevara, Post Graduate Student in Surgical Oncology, 
Veerendra Kumar, Professor, Department of Surgical Oncology, 
Vijayalakshmi Deshmane, Professor, Department of Surgical Oncology, 
Malathi Mukunda, Professor, Department of Pathology, 
Jaipal, Professor, Department of Radiodiagnosis, 
Kidwai Memorial Institute of Oncology, Bangalore, India. 
 
Address for Correspondence 
Dr. Pampanagouda SKM, 
212/2-1, Balaji Road, 
Thyagarajnagar, 
Bangalore -560028, 
India.  
E-mail: drpampanagouda@yahoo.co.in 
 
Citation 
Pampanagouda SKM, Suryadevara S, Kumar V, Deshmane V, Mukunda M, Jaipal. Umbilical Metastasis as the Manifestation of an 
Asymptomatic Gallbladder Carcinoma. Online J Health Allied Scs. 2014;13(1):7. Available at URL: 
http://www.ojhas.org/issue49/2014-1-7.html 
Open Access Archives 
http://cogprints.org/view/subjects/OJHAS.html 
http://openmed.nic.in/view/subjects/ojhas.html 
 
Submitted: Mar 28, 2014; Accepted: Apr 24, 2014; Published: May 15, 2014 
 
Abstract: Sister Mary Joseph's nodule (SMJN) is an eponym 
used to describe the metastatic lesion of the umbilicus. 
Umbilical metastases are rare and its occurrence as a first 
manifestation of an asymptomatic primary malignancy is 
very rare. The gallbladder is the source of SMJN in 2%-3% 
of cases. SMJN can present in any one of the following ways; 
first manifestation of an occult primary malignancy, an 
indication of recurrence in a patient with a previous 
malignancy, or as progression of an underlying symptomatic 
primary disease. Our study emphasizes the importance of 
identifying this very useful and easily applicable clinical sign 
by careful physical examination of the abdomen, and also the 
need for proper clinical evaluation of any umbilical lesion, 
and the histological diagnosis. In the present study we report 
on a case of Sister Mary Joseph's Nodule as the presentation 
of an asymptomatic gallbladder carcinoma, and review the 
relevant literature. 
Key Words: Sister Mary Joseph's Nodule (SMJN); 
Umbilicus mass; Gall bladder carcinoma 
Introduction: 
SMJN has been reported mainly in association with primary 
abdomino-pelvic malignancies, and it is estimated that 1% to 
3% of gastrointestinal and gynecological tumors metastasize 
to the umbilicus. Umbilical metastasis from Gallbladder 
Carcinoma is usually found following laparoscopic 
cholecystectomy for an apparently benign gallbladder 
disease. Umbilical metastases were first reported by Walshe 
in 1846.(1) Sister Mary Joseph (1856-1939), was the surgical 
assistant for Dr William J Mayo, at St Mary's Hospital, 
Minnesota, between 1890-1915. She was the first to note the 
presence of a nodule at the umbilical region as being a sign 
of an advanced intra-abdominal neoplasm with unfavorable 
prognosis, and brought it to the attention of Dr William 
Mayo. She made this observation while preparing the 
patient's abdomen for surgery. Dr. Mayo reported this 
condition as "pants button umbilicus" in 1928.(2) The 
eponym was coined in her recognition by the British Surgeon 
Hamilton Bailey in 1949 in the 11th edition of his textbook 
entitled "Demonstration of Physical signs in Clinical 
Surgery".(3) 
Case Report: 
A 60 years old female presented with a swelling at the site of 
umbilicus since 1.5 months, and mild dull aching pain at that 
region since one month. There was no history of any 
variation in the size of the swelling. She had hysterectomy 
done 20 years ago and didn't have any other co-morbidities. 
On examination, a 4x3 cm, non tender nodule present at the 
site of umbilicus.(Figure 1) There was no palpable intra 
abdominal mass or organomegaly. USG of abdomen and 
pelvis revealed Gall bladder mass with calculi and a porta 
hepatis LN (1.6x1 cm) with hypoechoic lesion (3x2 cm) at 
the umbilicus. CT scan of the abdomen showed 
heterogeneous mass in the gall bladder with calculi,(Figure 2) 
with few necrotic LNs in the porta hepatis and para aortic 
region, and a soft tissue mass (metastatic) in the umbilical 
region.(Figure 3) Complete blood count, liver function test 
and renal function test were within normal limits. Serum CA 
19-9 was 29.94 U/ml (Normal- up to 27 U/ml). Esophago 
gastro duodenoscopy was normal. Fine needle aspiration 
cytology of umbilical lesion was positive for carcinoma, 
(Figure 4) and gall bladder mass showed features of 
adenocarcinoma. Umbilical lesion was positive for CK 7 and 
negative for CK 20. Immunohistochemistry of umbilical 
nodule was positive for CA 19-9.(Figure 5) After explaining 
the prognosis, patient opted for symptomatic treatment due to 
financial constraints, and was lost for follow up after 4 
months. 
 
This work is licensed under a 
Creative Commons Attribution- 
No Derivative Works 2.5 India License 
Online Journal of Health and Allied Sciences 
Peer Reviewed, Open Access, Free Online Journal 
Published Quarterly :  Mangalore, South India : ISSN0972-5997 
Volume 13, Issue 1; Jan-Mar 2014 
2 
 
 
Figure 1: Umbilical nodule. 
 
Figure 2: CT Abdomen showing Gall Bladder mass with calculi. 
 
Figure 3: CT abdomen showing Umbilical nodule. 
 
Figure 4: Fine needle aspiration cytology of SMJN 
stained with Papinicolaou stain. 
 
Figure 5: Umbilical nodule immunohistochemical 
staining positive for CA 19-9. 
Discussion: 
Umbilicus consists of a puckered scar through the linea alba 
at the former attachment of the umbilical cord. Umbilical 
region shows a rich arterial supply that includes the inferior 
epigastric artery and deep circumflex iliac artery: branches of 
the external iliac artery, and the superficial epigastric artery: 
branch of the internal mammary artery. Venous drainage 
from the umbilicus occurs to axillary vein via the lateral 
thoracic vein and to femoral vein through superficial 
epigastric vein. Small para umbilical veins connect with the 
portal system along the ligamentum teres. There is 
documented lymphatic communication between the 
umbilicus and axillary, para aortic, internal mammary, 
external iliac and inguinal lymph nodes. Tumor spread to the 
umbilical region has been postulated to occur through several 
ways. The most common route is contiguous spread from the 
peritoneal surface, and is considered to be the most important 
route for tumor spread from visceral malignancies. Other 
probable methods of tumor spread are through the 
hematogenous, lymphatic route, or along the remnants of 
embryological origin which are associated with the umbilical 
region such as urachus, ligamentum teres, vitello-intestinal 
duct remnant and the medial umbilical ligaments. 
The mean age of diagnosis of cases with SMJN is 50.6 years 
(range 18-87 years). SMJN could present as a painless or 
painful, firm to hard indurated lesion with irregular margins, 
or even necrotic, sometimes fissured or ulcerated with serous, 
mucinous, purulent, or bloody discharge, and occasionally is 
pruritic. The nodule has been described as white, bluish 
violet and brownish red. The nodule may be seen or felt deep 
in the sub cutis at the umbilical region. Patients may present 
with other clinical signs or symptoms consistent with the 
primary disease, and guide in identifying the source. SMJN is 
an initial presenting sign in up to 14-33% of the cases, where 
as in 40% of patients with a known neoplasm the nodule is an 
early sign of relapse.(4) If umbilical disease is the initial 
presentation of a metastatic tumor, then the chances of 
identifying the primary is less than 50%, and these patients 
are more likely to be women.(5) 
An umbilical lesion can be benign (57%) or malignant 
(43%). Benign lesions to be considered are umbilical hernia, 
pilonidal sinus, pyogenic granuloma, endometriosis, mycosis, 
urachal duct cyst, omphalitis, fibroma, foreign body 
granuloma, hypertrophic scar, lymphangioma, melanocytic 
nevi, papilloma, cutaneous epithelial inclusion cysts, keloid 
and myxoma. Primary umbilical neoplasms are melanoma, 
squamous cell carcinoma, basal cell carcinoma, 
adenocarcinoma and sarcoma. Known primary sites of origin 
for SMJN are stomach, colorectal, pancreas, lung, esophagus, 
liver, gallbladder, prostate, kidney, penis, bladder, 
3 
 
peritoneum, ovary, endometrium, breast, cervix, vagina, 
fallopian tube and lymphoma. 
Fine needle aspiration (FNA) is an easy and reliable 
minimally invasive method for diagnosis, and should be 
considered as an initial diagnostic test to evaluate an 
umbilical neoplasm. Once umbilical nodule is discovered, 
FNA or biopsy is mandatory to establish the diagnosis, and to 
guide search for the possible site of origin with appropriate 
investigations.  Excision of the umbilical mass to confirm the 
diagnosis may be required as a guide for further 
management. Immunohistochemical analysis may define the 
cellular origin in 72 % of cases of unknown primary tumor. 
Sometimes the nodule may not be evident clinically and 
identified only on imaging studies, especially in obese 
patients. 
Cutaneous metastasis occurs in 5%-9% of all cases of 
malignant disease. Metastasis to the umbilicus is rare and 
represents only 10% of tumors which metastasize to the 
skin.(6) Primary umbilical tumors account for 17% of cases, 
whereas metastatic lesions constitute 83% of all malignant 
umbilical tumors. Malignant melanoma is the most common 
primary umbilical malignancy. Among all reported cases of 
umbilical metastases, 35-65% metastasize from 
gastrointestinal malignancies; 12-35% from the genitourinary 
tract; 15-30% from unknown sites; and 3-6% from other 
malignancies such as those of the lung and breast.(7) In men, 
the commonest primary site is the digestive tract, of which 
stomach is the most common site, whereas gynecological 
malignancies, particularly epithelial ovarian tumors, are the 
most common primary sites in women. Among 
gastrointestinal malignancies the common sites in decreasing 
order of frequency are: stomach (25%), colorectal (10%), and 
pancreas (7%). Histologically, the most common tumor type 
is adenocarcinoma (75%). SMJN is more commonly seen in 
females, and is probably due to a large number of 
gynecological malignancies that might involve the umbilicus 
in addition to the gastrointestinal cancers. 
The average time between manifestation of skin lesion and 
diagnosis of cancer is 3 months, and the mean life expectancy 
is 2-11 months without the treatment, and less than 15% of 
the patients survive more than 2 years. Some of the studies 
have shown better survival for patients if they are treated 
aggressively with a combination of surgery and adjunctive 
therapy (21 months) instead of surgery alone (7.4 months), 
chemotherapy alone (10.3 months), or no treatment (2.3 
months).(8) However, appropriateness of such therapy is 
determined by the clinical state of the patient and the 
treatment should be individualized. It is known that the 
survival is better in patients who detected such a metastasis 
before definitive treatment of the primary tumor rather than 
as a recurrence of it. A better survival rate is possible in 
patients with primary ovarian carcinoma and in patients with 
metastases confined only to the umbilicus. 
Summary: 
Even though, SMJN is a rare clinical sign, the ability to 
identify this lesion may save a patient an unnecessary 
diagnostic and therapeutic intervention. Given the variable 
appearance of the lesion, high index of suspicion is necessary 
to make an accurate diagnosis. In this era of advanced 
technology, SMJN still remains as an interesting and useful 
diagnostic clinical sign. 
References: 
1. Walshe WH. The nature and treatment of cancer. The 
British and Foreign medical review. 1846:293-311. 
2. Mayo WJ. Metastases in cancer. Proceedings of the 
Staff Meetings of the Mayo Clinic 1928;3:327. 
3. Bailey H. Demonstration of Physical Signs in Clinical 
Surgery, 11th edition. Baltimore, Williams and Wilkins, 
1949, pp 227. 
4. Srinivasan R. Metastatic cutaneous and subcutaneous 
deposits from internal carcinoma. An analysis of cases 
diagnosed by fine needle aspiration. Acta Cytol. 
1993;37:894-898. 
5. Papala JA, Selim MA. Metastatic vs primary malignant 
neoplasms affecting the umbilicus : clinicopathologic 
features of 77 tumors. Annals of Diagnostic Pathology. 
2011;15:237-242. 
6. Lookingbill D, 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. 
7. Falchi M, Cecchini G, Derchi LE. Umbilical metastasis 
as first sign of cecal carcinoma in a cirrhotic patient 
(Sister Mary Joseph nodule). Report of a case 
[Italian] Radiol Med. 1999;98:94-96. 
8. Gabriele R, Conte M, Egidi F, Borghese M. Umbilical 
metastases: current view point. World J Surg Oncol  
2005;3:13. 


Umbilical Metastasis as the Manifestation of an Asymptomatic Gallbladder Carcinoma

Abstract

Similar works

Full text

Available Versions

Cognitive Sciences ePrint Archive