Paraneoplastic pemphigus associated with a pericardial ectopic thymoma

Area of Science:

• Autoimmune diseases
• Oncology
• Cardiology

Background:

• Paraneoplastic Pemphigus (PNP) is a rare autoimmune blistering disease associated with various neoplasms.
• Commonly associated malignancies include hematologic malignancies, Castleman's disease, carcinoma, and thymoma.
• Identifying and treating the underlying malignancy is crucial for inducing remission in PNP.

Observation:

• A young female patient diagnosed with PNP presented with no known history of malignancy.
• Cross-sectional imaging revealed a pericardial mass lesion extending into the transverse pericardial sinus.
• Excisional biopsy was performed for histopathological examination.

Findings:

• Histopathology confirmed the presence of a pericardial ectopic thymoma.
• This case highlights an unusual presentation of thymoma associated with PNP.

Implications:

• This case underscores the importance of comprehensive oncological workup in patients with suspected PNP, even in the absence of a prior malignancy diagnosis.
• The findings suggest that ectopic thymoma should be considered in the differential diagnosis of pericardial masses in patients with PNP.
• Early detection and treatment of the underlying neoplasm are critical for managing PNP and improving patient outcomes.