# An Unusual Presentation of Cholangiocarcinoma: The Sister Mary Joseph Nodule—A Case Report

**Authors:** Toni Esposito, Niharika Singh, Riddhish Sheth, George Keckeisen

PMC · DOI: 10.3390/reports9010051 · Reports - Clinical Practice and Surgical Cases · 2026-02-03

## TL;DR

A rare case of cholangiocarcinoma presented as a Sister Mary Joseph nodule, highlighting the importance of early recognition for better outcomes.

## Contribution

Reports a rare association between Sister Mary Joseph nodules and cholangiocarcinoma, emphasizing clinical awareness.

## Key findings

- SMJN can be an unusual presentation of advanced intrahepatic cholangiocarcinoma.
- Delayed recognition of SMJN may lead to late diagnosis and poor prognosis.
- Early detection of CCA is critical due to its rising incidence and mortality.

## Abstract

Background and Clinical Significance: Sister Mary Joseph nodules (SMJN) are rare extra-intestinal manifestations of metastatic intra-abdominal and pelvic malignancies, often indicating advanced disease and poor prognosis. Their association with cholangiocarcinoma (CCA) is particularly uncommon, with only a limited number of reported cases. Case Presentation: We present a case report of a 65-year-old previously healthy male who presented for an elective umbilical hernia repair. Additional findings of elevated direct bilirubin and a history of fatigue in the patient prompted further evaluation with biopsy and imaging, which revealed advanced-stage intrahepatic CCA. Following the diagnosis, the patient underwent biliary stenting and chemotherapy. Conclusions: The variable presentation of SMJN, along with its frequent misdiagnosis, often delays recogni-tion and management of the underlying malignancy. This case of SMJN in the setting of CCA highlights the complex interplay between intra-abdominal and pelvic malignancies and their impact on different organ systems. With the rising incidence and mortality rates associated with CCA, early recognition is essential to improving patient outcomes. This underscores the need for increased clinical awareness and further research, thus support-ing the development of this case report.

## Linked entities

- **Diseases:** cholangiocarcinoma (MONDO:0019087)

## Full-text entities

- **Genes:** GPT (glutamic--pyruvic transaminase) [NCBI Gene 2875] {aka AAT1, ALT, ALT1, GPT1, SGPT}, KRT7 (keratin 7) [NCBI Gene 3855] {aka CK7, K2C7, K7, SCL}, KRT19 (keratin 19) [NCBI Gene 3880] {aka CK19, K19, K1CS}
- **Diseases:** gastrointestinal cancers (MESH:D005770), metastatic carcinoma (MESH:C538445), liver flukes (MESH:D017093), ovarian, colorectal, and pancreatic malignancies (MESH:D010051), granuloma (MESH:D006099), lymphoma (MESH:D008223), abdominal malignancies (MESH:D000007), intra-abdominal and pelvic malignancies (MESH:D000082122), primary sclerosing cholangitis (MESH:D015209), primary liver cancer (MESH:D006528), basal cell carcinoma (MESH:D002280), carcinogenic (MESH:D011230), umbilical lesion (MESH:D014496), epithelial cyst (MESH:D009375), metastases (MESH:D009362), gastrointestinal stromal tumors (MESH:D046152), lethargy (MESH:D053609), bile flow obstruction (MESH:D002779), intrahepatic and extrahepatic biliary dilation (MESH:D015529), fatigue (MESH:D005221), jaundice (MESH:D007565), mesenchymal tumors (MESH:C535700), CCA (MESH:D018281), Carcinogenesis (MESH:D063646), gastric carcinomas (MESH:D013274), squamous cell carcinoma (MESH:D002294), pruritus (MESH:D011537), umbilical mass (MESH:C536030), neuroendocrine tumors (MESH:D018358), Nodule (MESH:D016606), right upper quadrant pain (MESH:D010146), SMJN (MESH:D058288), skin lesion (MESH:D012871), gastric, colon, or ovarian (MESH:D013276), injury to (MESH:D014947), inflammation (MESH:D007249), cirrhosis (MESH:D005355), malignant melanoma (MESH:D008545), umbilical hernia (MESH:D006554), hepatitis B and C (MESH:D006509), adenocarcinoma (MESH:D000230), carcinoma (MESH:D009369)
- **Chemicals:** formalin (MESH:D005557), eosin (MESH:D004801), Hematoxylin (MESH:D006416), H&amp;E (MESH:D006371), bilirubin (MESH:D001663)
- **Species:** Clonorchis sinensis (oriental liver fluke, species) [taxon 79923], Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

4 figures with captions in the complete paper: https://tomesphere.com/paper/PMC12922057/full.md

## References

12 references — full list in the complete paper: https://tomesphere.com/paper/PMC12922057/full.md

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Source: https://tomesphere.com/paper/PMC12922057