Difference between revisions of "Thymus"

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===General===
===General===
*Extremely rare - only 17 case reports as of 2011.<ref name=pmid20034984>{{Cite journal  | last1 = Lu | first1 = HS. | last2 = Gan | first2 = MF. | last3 = Zhou | first3 = T. | last4 = Wang | first4 = SZ. | title = Sarcomatoid thymic carcinoma arising in metaplastic thymoma: a case report. | journal = Int J Surg Pathol | volume = 19 | issue = 5 | pages = 677-80 | month = Oct | year = 2011 | doi = 10.1177/1066896909355458 | PMID = 20034984 }}</ref>
*Extremely rare - only 17 case reports as of 2011.<ref name=pmid20034984>{{Cite journal  | last1 = Lu | first1 = HS. | last2 = Gan | first2 = MF. | last3 = Zhou | first3 = T. | last4 = Wang | first4 = SZ. | title = Sarcomatoid thymic carcinoma arising in metaplastic thymoma: a case report. | journal = Int J Surg Pathol | volume = 19 | issue = 5 | pages = 677-80 | month = Oct | year = 2011 | doi = 10.1177/1066896909355458 | PMID = 20034984 }}</ref>
*Two reports of transformation into [[thymic carcioma]].<ref name=pmid20034984/>
*Two reports of transformation into [[thymic carcinoma]].<ref name=pmid20034984/>


===Microscopic===
===Microscopic===
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DDx:
DDx:
*[[Thymic Carcinoma]] with sarcomatoid differentiation.
*[[Thymic carcinoma]] with sarcomatoid differentiation.


===IHC===
===IHC===

Revision as of 20:55, 22 December 2011

Thymus is an annoying little organ that is in the mediastinum. It is often removed in pediatric cardiac surgery 'cause it is in the way. In adults, it is commonly removed 'cause the patient has myasthenia gravis.

Overview

  • Thymus involutes after childhood.
  • Location: anterior mediastinum.
  • Important for development of the immune system.
  • May contain within it parathyroid.[1]
  • May be absent due to genetic abnormalities, e.g. Di George syndrome.

Normal histology

General

Features:[2]

  • No germinal centres.
  • Hassall's corpusle (thymic corpusle).
    • Round eosinophilic thingy.
    • Thought to arise from medullary epithelial cells (see cell types).[1]

Cell types

Cells of the thymus (short version):

  1. Cortical epithelial cells.[1]
    • Epithelioid.
    • Abundant cytoplasm.
    • Pale nuclei with small nucleoli.
  2. Medullary epithelial cells.[1]
    • Spindle morphology.
    • Scant cytoplasm.
    • Oval dark nuclei.
  3. T lymphocytes.

Other cells:

  • Macrophages.
  • Dendritic cells.
  • Other WBCs: B lymphocytes, neutrophils, eosinophils.
  • Myoid cells.

Note:

  • Thymic tumours are derived from the epithelial component of the thymus, i.e. the cortical epithelial cells and medullary epithelial cells.

Images:

IHC and thymus

Types A, AB, B:[3]

  • CK7-, CK20-, CAM5.2+, CK5/6+, p63+, CD5-.

Type C:[4]

  • CD5+.

All types:[5]

  • CD1a (immature T cells, Langerhans cells, dendritic cells[6]), CEA +ve (focal), vimentin -ve.

Others (immature T cells):

  • TdT.
  • CD99.

Anterior mediastinum mass DDx

4 Ts (mnemonic):


Thymus and stress

  • Stress -> increased endogenous steroid -> lymphocyte death -> increased tingible body macrophages.[7]

Specific conditions

Thymic follicular hyperplasia

  • AKA thymic follicular hyperplasia.

Features:[8]

  • Follicular centres in the thymus.

Associations:[8]

Thymoma

General

  • Strong association with autoimmune disease, esp. myasthenia gravis.

Classification

The WHO published a widely used system - WHO classification:[9]

Type A
  • AKA Spindle cell or medullary.
  • Arise from medullary epithelial cells.
  • Good prognosis.

IHC:

  • Usu. keratin+.
Type AB
  • Like Type A... but with foci of lymphocytes.
Type B1
  • Near normal, expanded cortex.

Lesion consists of:

  • >2/3 lymphocytes, <1/3 cortical epithelial cells.
Type B2
  • Neoplastic cells with some resemblance to cortical epithelial cells.
    • Epithelioid cells with distinct nucleoli.
    • May be perivascular.
  • Large population of lymphocytes.

Lesion consists of:

  • <2/3 but >1/3 lymphocytes, >1/3 but <2/3 cortical epithelial cells.

Notes:

  • Most common B type.
Type B3
  • Neoplastic cells with some resemblance to cortical epithelial cells.
    • Polygonal/round shape.
    • Form sheets (of cells) - key feature.
  • Lymphocytes - less than in Type B2.
  • AKA well-differentiated thymic carcinoma.

Lesion consists of:

  • <1/3 lymphocytes, >2/3 cortical epithelial cells.

Note:

  • Neoplastic cells derived from the thymus with cytologic features of malignancy are thymic carcinomas.

Images:

Microscopic

Features:

  • Lymphocytes.
  • Spindle cells.

DDx:

Images:

Staging

There is a system by Masaoka and colleagues.[10]

Metaplastic thymoma

  • AKA thymoma with pseudosarcomatous stroma.[11]

General

Microscopic

Features:[11]

  1. Epithelioid cells.
  2. Spindle cells.
  • Few lymphocytes.

DDx:

IHC

CD5 -ve.[11]

Thymic carcinoma

  • AKA Thymic tumour type C.

General

  • Rare.
  • Usually arise de novo, i.e. thymoma is not generally a precursor.

Microscopic

Features:[13]

  • Cytologically malignant - variable morphology.
  • +/-Squamous differentiation.

DDx:

Images:

IHC

Features:[13]

  • CD5 +ve.[14]
  • CD7+ve.
  • CD117 +ve.
  • TTF-1 -ve.

See also

References

  1. 1.0 1.1 1.2 1.3 Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease (7th ed.). St. Louis, Mo: Elsevier Saunders. pp. 706. ISBN 0-7216-0187-1.
  2. URL: http://www.kumc.edu/instruction/medicine/anatomy/histoweb/lymphoid/lymph03.htm. Accessed on: 17 June 2010.
  3. CJS. January 2010.
  4. CJS. January 2010.
  5. CJS. January 2010.
  6. URL: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1886385/pdf/amjpathol00102-0156.pdf. Accessed on: 26 August 2010.
  7. Toti P, De Felice C, Stumpo M, et al. (September 2000). "Acute thymic involution in fetuses and neonates with chorioamnionitis". Hum. Pathol. 31 (9): 1121–8. PMID 11014581.
  8. 8.0 8.1 Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease (7th ed.). St. Louis, Mo: Elsevier Saunders. pp. 707-8. ISBN 0-7216-0187-1.
  9. Mills, Stacey E; Carter, Darryl; Greenson, Joel K; Oberman, Harold A; Reuter, Victor E (2004). Sternberg's Diagnostic Surgical Pathology (4th ed.). Lippincott Williams & Wilkins. pp. 1264. ISBN 978-0781740517.
  10. Masaoka, A.; Monden, Y.; Nakahara, K.; Tanioka, T. (Dec 1981). "Follow-up study of thymomas with special reference to their clinical stages.". Cancer 48 (11): 2485-92. PMID 7296496.
  11. 11.0 11.1 11.2 URL: http://surgpathcriteria.stanford.edu/thymus/thymoma/metaplastic_thymoma.html. Accessed on: 22 December 2011.
  12. 12.0 12.1 Lu, HS.; Gan, MF.; Zhou, T.; Wang, SZ. (Oct 2011). "Sarcomatoid thymic carcinoma arising in metaplastic thymoma: a case report.". Int J Surg Pathol 19 (5): 677-80. doi:10.1177/1066896909355458. PMID 20034984.
  13. 13.0 13.1 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 147. ISBN 978-0781765275.
  14. Cotran, Ramzi S.; Kumar, Vinay; Fausto, Nelson; Nelso Fausto; Robbins, Stanley L.; Abbas, Abul K. (2005). Robbins and Cotran pathologic basis of disease (7th ed.). St. Louis, Mo: Elsevier Saunders. pp. 708. ISBN 0-7216-0187-1.