23 August 2007 Ė Case of the Week #93
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Case of the Week #93
A 63 year old woman with an unknown past medical history presented with a right axillary mass and lymphadenopathy.† The axillary mass was excised.
What is your diagnosis?
Castlemanís disease, hyaline-vascular variant
Castlemanís disease, also known as giant lymph node hyperplasia or angiofollicular hyperplasia, is a rare lymphoproliferative disorder of unknown etiology with hyaline vascular, plasma cell and mixed variants.† Most cases are polyclonal (Histopathology 2006;48:233).† It may be solitary or multicentric (Acta Oncol 2004;43:698).† Solitary masses often are mediastinal and asymptomatic, and 90% are hyaline-vascular subtype.† Multicentric disease presents with generalized lymphadenopathy, and is almost always plasma cell subtype.† It may be associated with the POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, M-protein and Skin).† In this case, although the disease appeared to be multicentric (the lymph nodes were not biopsied), the subtype was hyaline-vascular.
The hyaline vascular (angiofollicular) subtype has prominent follicles within lymphoid tissue but no sinuses, and a low power targetoid pattern (image).† The appearance may resemble a lollipop (image).† The follicles have a marked vascular proliferation with thick and hyalinized vessel walls and hyalinized germinal centers which resemble thymic Hassallís corpuscles or splenic white pulp, but are actually regressed (involuted) germinal centers.† They often have an onion-skin pattern of lymphocytes (image), and may contain large cells with prominent nucleoli (CD21+, CD35+ follicular dendritic cells, Diagn Cytopathol 2000;22:230).† Frequently, a capillary is found that penetrates the follicle.† On fine needle aspirate, large histiocytic cells with nuclei resembling crumpled tissue paper are seen in a background of small lymphocytes (Cytopathology 2007;18:168)
The plasma cell variant also has follicular hyperplasia with large germinal centers, but with diffuse plasma cells between follicles.† It is associated with HHV8 infection (AJSP 2003;27:91).† In addition, a plasmablastic variant has been described (Blood 2000;95:1406)
The differential diagnosis includes follicular lymphoma, follicular hyperplasia, progressive transformation of germinal centers (APMIS 2005;113:288), HIV lymphadenopathy and mantle cell lymphoma.
Surgical excision is usually adequate for solitary lesions.† For multicentric lesions, the optimal treatment is unknown, although anti-IL6 therapy has shown promising results (Intern Med 2007;46:771)
Nat Pernick, M.D., President
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