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Retroperitoneal unicentric Castleman disease presenting with paraneoplastic pemphigus
Sree Vani Paladugu1, Cherring Tandup2, Suvradeep Mitra3
1General Surgery, Post Graduate Institute of Medical Education and Research, Chandigarh, India.
BMJ Case Reports
|December 4, 2024
Summary
Castleman disease, a rare benign lymph node hyperplasia, can present atypically with mucocutaneous lesions. Surgical excision of a retroperitoneal tumor led to the definitive diagnosis and successful treatment.
Area of Science:
- Oncology
- Dermatology
- Pathology
Background:
- Castleman disease is a rare lymphoproliferative disorder with diverse clinical presentations.
- Distinguishing Castleman disease from other conditions can be challenging due to its varied histology and presentation.
Observation:
- A young adult male presented with mucocutaneous lesions resembling common dermatological conditions.
- Failure of conventional treatment for skin lesions prompted further investigation for an underlying systemic disease.
- Immunoprecipitation and histology suggested paraneoplastic pemphigus, with abdominal imaging revealing a retroperitoneal tumor.
Findings:
- A retroperitoneal tumor was identified, supporting a paraneoplastic etiology for the mucocutaneous lesions.
- Preoperative biopsy of the tumor was inconclusive, necessitating surgical intervention.
- Complete surgical excision of the retroperitoneal tumor confirmed Castleman disease as the final diagnosis.
Implications:
- This case highlights the diagnostic challenges posed by atypical presentations of rare diseases like Castleman disease.
- A systematic diagnostic approach, including advanced imaging and surgical pathology, is crucial for accurate diagnosis.
- Successful surgical management of the underlying Castleman disease resolved the associated paraneoplastic syndrome.
