Sister Mary Joseph's nodule: Three case reports

Background An umbilical metastatic lesion is called 'Sister Mary Joseph's nodule'. It is an uncommon clinical or radiographic finding, and it is rare as the first sign of a malignant disease. Case presentation We report three cases of Sister Mary Joseph's nodule. In the three cases presented, the primary tumor was an adenocarcinona of the sigmoid colon, a carcinoma of the bladder, and an adenocarcinoma of the gallbladder, respectively. Conclusion The differential diagnosis of an umbilical lesion should always include metastatic disease apart from benign lesions and primary neoplasms.


Background
An umbilical lesion can be either benign or malignant. A malignant umbilical mass can represent a primary or metastatic lesion. The term 'Sister Mary Joseph's nodule' was first used by Sir Hamilton Bailey, in 1949 [1], in order to describe the entity of metastatic umbilical lesions. We present three cases of Sister Mary Joseph's nodule.

Case 1
A 62-year old man presented with a growing periumbilical mass and a mild lower abdominal pain during the last two months. He had symptoms of fatigue, and loss of weight and constipation alternating with diarrhea during the last six months. Patient was receiving medication for hypertension. On clinical examination there was a slight tenderness over the lower abdomen and an umbilical lesion fixed to the underlying tissues. Further evaluation, revealed anemia (Hct: 30%). Colonoscopy detected a tumor of the sigmoid colon. Computed tomography (Figure 1) showed a mass measured 3 cm, with characteristics of non-homogeneous soft structures, located to the anterior abdominal wall, at the region of the umbilicus, involving the adjacent portion of rectus abdominis muscle and adipose tissue. Both biopsies of umbilical and sigmoid tumors revealed the presence of adenocarcinoma. Considering the advanced stage of the disease, patient received chemotherapy as primary treatment.

Case 2
A 64-year old female presented with haematuria and dysuria during the last 4 months. She had no other medical or surgical history. The assessment with ultrasound detected an established left hydronephrosis and a mass at the left lateral wall of the bladder protruding into its cavity. Cysteoscopic biopsy of the mass showed a transitional cell carcinoma. Further assessment with computed tomography (CT-scan) confirmed the presence of the tumor at the left lateral wall of the bladder, and revealed pelvic lymph nodes involvement and an umbilical mass measured 2 cm (Figure 2). Percutaneous biopsy of the umbilical mass also showed transitional cell carcinoma. Patient underwent cystectomy and chemotherapy.

Case 3
A 54-year old male presented with an umbilical painless mass. He was receiving medication for diabetes mellitus. Patient's physical examination revealed no other abnormality. Pathologic examination of percutaneous biopsy specimen showed malignant epithelial tumor, with characteristics of gall bladder or pancreatic origin adenocarci-noma. Neither upper gastrointestinal endoscopy nor colonoscopy detected any abnormality. Computed tomography of the abdomen showed a gall bladder with increased wall thickness and intraluminal invasive mass, peritoneal lesions, ascites, and an umbilical mass involving the adjacent adipose tissue (Figure 3). Patient received surgical treatment. The pathologic examination of the surgical specimen showed adenocarcinoma of the gallbladder. Patient's further treatment was chemotherapy.

Discussion
The occurrence of Sister Mary Joseph's nodule is uncommon, and as first sign of malignancy is rare [2]. The intestinal and genitourinary tracts represent the most common, but not the only ones, primary malignant sites [3]. However, the study of an umbilical mass as unique clinical finding should be directed by the suspicion of being a metastatic deposit [4], having also in mind the potential of a primary malignant umbilical lesion or a benign disease [5]. In addition, an umbilical mass in a patient with known malignancy, especially of the abdomen, should be evaluated as potential spreading of the primary disease, a fact that can influence the therapeutic decision making [4,6]. Finally, the approach of surgery should be under careful consideration in these cases, because Sister Mary Joseph's nodule represents the spreading of a neoplasm, which is accompanied by low survival rates [4].

Conclusion
An umbilical mass can represent a benign lesion, such as a cyst or an abscess, but also a primary or metastatic malignant tumor. Although not common, the last two CT-scan of the abdomen: This image shows an umbilical mass measured 3 cm, with indefinite margins