Castleman disease - unicentric vascular hyaline type

Case contributed by Dr Adrià Roset Altadill

Presentation

Presented with multiple oral ulcerative mucosal lesions that were resistant to various treatments.

Patient Data

Age: 70 years
Gender: Female

Bilobulated and well-defined right axillary mass that shows homogeneous intense enhancement, with a small central hypoattenuating region. Note the presence of some prominent feeding vessels in the posterior aspect of the lesion. 

Case Discussion

The radiologic features together with the clinical presentation were highly suggestive for a unicentric Castleman disease associated with paraneoplastic pemphigus

Surgical excision of the axillary mass was performed and the sample was sent to the anatomopathological analysis. The report described:

"Mix of lymphoid tissue with tumor proliferation of dendritic cells. There are germinal centers surrounded by an expanded mantle area arranged in circular layers ("onion skin" appearence). Also, a marked vascular tumor proliferation with small vessels of hyalinized walls stands out. Findings in keeping with a dendritic follicular cell tumor associated with vascular hyaline Castleman's disease."

After the removal of the Castleman disease mass, the dermatologic lesions progressively remitted.

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