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Lymphoma - Non B cell neoplasms

Hodgkin lymphoma

Nodular sclerosis classical Hodgkin lymphoma


Reviewer: Dragos Luca, M.D. (see Reviewers page)
Revised: 29 October 2011, last major update August 2011
Copyright: (c) 2001-2011, PathologyOutlines.com, Inc.

Definition
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● Subtype of classical Hodgkin lymphoma (CHL) characterized by collagen bands that surround at least one nodule, and Hodgkin Reed-Sternberg (HRS) cells with lacunar type morphology (WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, 4th Edition, Lyon 2008)

Epidemiology
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● 70% of classical Hodgkin lymphoma in Europe and USA
● More common in rich countries, highest risk in those with high socioeconomic status
● Similar incidence in males and females (the only classical Hodgkin lymphoma subtype without male predominance)
● Peaks at 15-34 years of age

Sites
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● Cervical lymph nodes, mediastinum (80%), bulky disease (54%), spleen or lung (8-10%), bone (5%), bone marrow (3%), liver (2%)

Clinical features
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● Most present with stage II disease (2/3 have stage I or II disease)
● B symptoms: 40%

Case reports
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● 4 year old girl with juvenile rheumatoid arthritis and EBV+ disease (Hum Pathol 2000;31:253)
● 21 year old woman with primary pulmonary disease (Arch Pathol Lab Med 2003;127:e49)
● 54 year old immunocompetent patient with primary intracerebral EBV+ disease (Am J Surg Pathol 1999;23:477)

Treatment
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● Better overall prognosis than other types of classical Hodgkin lymphoma
● >90% survival at 5 years for early stage disease
● Adverse prognostic indicator: massive mediastinal disease

Gross images
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Various images

Micro description
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● Nodular growth pattern with broad fibroblast-poor birefringent collagen bands surrounding at least one nodule
● Usually confined within thickened lymphonodular capsule
● Highly variable numbers of HRS cells, small lymphocytes and other inflammatory cells; often numerous eosinophils, histiocytes and neutrophils; occasional foamy macrophages
● Mitoses uncommon
● HRS cells have more lobated nuclei, smaller lobes, less prominent nucleoli, more cytoplasm than other types of classical Hodgkin lymphoma
● Lacunar cells: formalin fixation artifact of delicate folded or multilobate nuclei surrounded by abundant pale cytoplasm often disrupted or retracted during cutting of sections with formalin fixation (but not B5 or Zenkers), leaving a lacune (empty hole); associated with necrosis and histiocytes (necrotizing granuloma-like)
● Syncytial variant: prominent aggregates of lacunar cells
● Grading by number of HRS cells (British National Lymphoma Investigation: 1-scattered, 2-aggregates in >25% of nodules) and number of eosinophils (German HL Study Group: >5%) is for research protocols but not for routine clinical purposes

Micro images
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Various images

   
Syncytial variant

   
Lacunar cells


Mummified cell

   

Bands of collagen

   
Reed-Sternberg cells with bilobed mirror-image nuclei, prominent nucleoli and abundant amphophilic cytoplasm


Syncytial variant: figure 1-mediastinal/lung mass; 2-atypical cells with amphophilic cytoplasm, multilobated nuclei with irregular outlines and prominent nucleoli; 3-CD15+; 4-S100 negative

Positive stains
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● HRS cells: CD15+, CD30+, fascin, vimentin; EBV+ in 10-40% of cases

Negative stains
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● HRS cells: B cell markers (CD19, CD20), T cell markers (CD3, CD4, CD5), CD34, CD45, EMA

Virtual slides
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Nodular sclerosis

Differential diagnosis
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● Anaplastic large cell lymphoma (T cell markers+, CD15-, EMA+, CD45+), mediastinal large B cell lymphoma, metastatic carcinoma, melanoma, seminoma

End of Lymphoma - Non B cell neoplasms > Hodgkin lymphoma > Nodular sclerosis classical Hodgkin lymphoma


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