Difference between revisions of "Castleman disease"

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#redirect [[Lymph node pathology#Castleman disease]]
'''Castleman disease''', abbreviated '''CD''', is a rare [[Lymph node pathology|pathology of the lymph node]].
 
It is also known as '''angiofollicular lymph node hyperplasia''' and '''giant lymph node hyperplasia'''.<ref>URL: [http://www.mayoclinic.com/health/castleman-disease/DS01000 http://www.mayoclinic.com/health/castleman-disease/DS01000]. Accessed on: 17 June 2010.</ref>
 
==General==
*Benign.
*Hyaline vascular variant - a pathology of the follicular dendritic cells.<ref>{{Cite journal  | last1 = Cokelaere | first1 = K. | last2 = Debiec-Rychter | first2 = M. | last3 = De Wolf-Peeters | first3 = C. | last4 = Hagemeijer | first4 = A. | last5 = Sciot | first5 = R. | title = Hyaline vascular Castleman's disease with HMGIC rearrangement in follicular dendritic cells: molecular evidence of mesenchymal tumorigenesis. | journal = Am J Surg Pathol | volume = 26 | issue = 5 | pages = 662-9 | month = May | year = 2002 | doi =  | PMID = 11979097 }}</ref>
 
===Classification===
CD is grouped by histologic appearance:<ref name=Ref_ILNP228>{{Ref ILNP|228}}</ref>
#Hyaline vascular (HV) variant (described by Castleman).
#*Usually unicentric.
#*Typically mediastinal or axial.
#*More common than plasma cell variant; represents 80-90% of CD cases.
#*May be associated with [[follicular dendritic cell neoplasia]].<ref name=Ref_WMSP_596>{{Ref WMSP|596}}</ref>
#Plasma cell (PC) variant.
#*Usually multicentric, may be unicentric.
#*Abundant plasma cells.
#*Associated with [[HHV-8]] infection (the same virus implicated in ''Kaposi's sarcoma'').
 
Notes:
*The subclassification of CD is in some flux. Some authors advocate splitting-out ''HHV-8'' and ''multicentric'' as separate subtypes.<ref name=pmid19546611>{{Cite journal  | last1 = Cronin | first1 = DM. | last2 = Warnke | first2 = RA. | title = Castleman disease: an update on classification and the spectrum of associated lesions. | journal = Adv Anat Pathol | volume = 16 | issue = 4 | pages = 236-46 | month = Jul | year = 2009 | doi = 10.1097/PAP.0b013e3181a9d4d3 | PMID = 19546611 }}</ref>
 
==Microscopic==
===Hyaline-vascular variant===
Features:<ref>URL: [http://www.ispub.com/journal/the_internet_journal_of_otorhinolaryngology/volume_9_number_2_11/article/a_rare_case_of_castleman_s_disease_presenting_as_cervical_neck_mass.html http://www.ispub.com/journal/the_internet_journal_of_otorhinolaryngology/volume_9_number_2_11/article/a_rare_case_of_castleman_s_disease_presenting_as_cervical_neck_mass.html]. Accessed on: 15 June 2010.</ref><ref name=Ref_ILNP236>{{Ref ILNP|236}}</ref>
*Pale concentric (expanded) mantle zone lymphocytes - '''key feature'''.
**"Regressed follicles" - germinal center (pale area) is small.
*"Lollipops":
**Germinal centers fed by prominent (radially penetrating sclerotic) vessels; lollipop-like appearance.
*Two germinal centers in one follicle.
*Hyaline material (pink acellular stuff on H&E) in germinal center.
*Sinuses effaced (lost).
*Mitoses absent.
 
====Images====
<gallery>
Image:Castleman_disease_-_high_mag.jpg | CD HVV - "lollipop" sign - high mag. (WC)
Image:Castleman_disease_-_intermed_mag.jpg | CD HVV - showing expanded mantle zone - intermed. mag. (WC)
</gallery>
www:
*[http://path.upmc.edu/cases/case115.html CD HVV - case 1 - several images (upmc.edu)].
*[http://path.upmc.edu/cases/case301.html CD HVV - case 2 - several images (upmc.edu)].
 
===Plasma cell variant===
Features:<ref name=Ref_ILNP236>{{Ref ILNP|236}}</ref>
*Interfollicular sheets of plasma cells - '''key feature'''.
*Active germinal centers - mitoses present.
*Sinus perserved.
 
==IHC==
Hyaline-vascular variant:
*Stains to exclude [[mantle cell lymphoma]]:
**Cyclin D1.
 
Plasma cell variant:
*HHV-8 +ve.
 
==See also==
*[[Lymph node pathology]].
 
==References==
{{Reflist|2}}
 
[[Category:Diagnosis]]
[[Category:Lymph node pathology]]

Revision as of 01:36, 26 December 2013

Castleman disease, abbreviated CD, is a rare pathology of the lymph node.

It is also known as angiofollicular lymph node hyperplasia and giant lymph node hyperplasia.[1]

General

  • Benign.
  • Hyaline vascular variant - a pathology of the follicular dendritic cells.[2]

Classification

CD is grouped by histologic appearance:[3]

  1. Hyaline vascular (HV) variant (described by Castleman).
  2. Plasma cell (PC) variant.
    • Usually multicentric, may be unicentric.
    • Abundant plasma cells.
    • Associated with HHV-8 infection (the same virus implicated in Kaposi's sarcoma).

Notes:

  • The subclassification of CD is in some flux. Some authors advocate splitting-out HHV-8 and multicentric as separate subtypes.[5]

Microscopic

Hyaline-vascular variant

Features:[6][7]

  • Pale concentric (expanded) mantle zone lymphocytes - key feature.
    • "Regressed follicles" - germinal center (pale area) is small.
  • "Lollipops":
    • Germinal centers fed by prominent (radially penetrating sclerotic) vessels; lollipop-like appearance.
  • Two germinal centers in one follicle.
  • Hyaline material (pink acellular stuff on H&E) in germinal center.
  • Sinuses effaced (lost).
  • Mitoses absent.

Images

  • CD HVV - "lollipop" sign - high mag. (WC)

  • CD HVV - showing expanded mantle zone - intermed. mag. (WC)

www:

Plasma cell variant

Features:[7]

  • Interfollicular sheets of plasma cells - key feature.
  • Active germinal centers - mitoses present.
  • Sinus perserved.

IHC

Hyaline-vascular variant:

Plasma cell variant:

  • HHV-8 +ve.

See also

References

  1. ↑ URL: http://www.mayoclinic.com/health/castleman-disease/DS01000. Accessed on: 17 June 2010.
  2. ↑ Cokelaere, K.; Debiec-Rychter, M.; De Wolf-Peeters, C.; Hagemeijer, A.; Sciot, R. (May 2002). "Hyaline vascular Castleman's disease with HMGIC rearrangement in follicular dendritic cells: molecular evidence of mesenchymal tumorigenesis.". Am J Surg Pathol 26 (5): 662-9. PMID 11979097.
  3. ↑ Ioachim, Harry L; Medeiros, L. Jeffrey (2008). Ioachim's Lymph Node Pathology (4th ed.). Lippincott Williams & Wilkins. pp. 228. ISBN 978-0781775960.
  4. ↑ Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 596. ISBN 978-0781765275.
  5. ↑ Cronin, DM.; Warnke, RA. (Jul 2009). "Castleman disease: an update on classification and the spectrum of associated lesions.". Adv Anat Pathol 16 (4): 236-46. doi:10.1097/PAP.0b013e3181a9d4d3. PMID 19546611.
  6. ↑ URL: http://www.ispub.com/journal/the_internet_journal_of_otorhinolaryngology/volume_9_number_2_11/article/a_rare_case_of_castleman_s_disease_presenting_as_cervical_neck_mass.html. Accessed on: 15 June 2010.
  7. ↑ 7.0 7.1 Ioachim, Harry L; Medeiros, L. Jeffrey (2008). Ioachim's Lymph Node Pathology (4th ed.). Lippincott Williams & Wilkins. pp. 236. ISBN 978-0781775960.
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