Difference between revisions of "Follicular dendritic cell sarcoma"

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{{ Infobox diagnosis
| Name      = {{PAGENAME}}
| Image      = Follicular dendritic cell sarcoma - alt -- very high mag.jpg
| Width      =
| Caption    = [[Micrograph]] showing a follicular dendritic cell sarcoma. The (benign) interspersed lymphocytes are a characteristic finding. [[H&E stain]].
| Synonyms  = follicular dendritic cell tumour, follicular dendritic cell neoplasm
| Micro      = oval or spindle-shaped cellular & nuclear morphology, variable architecture (sheets, fascicles, whorles, storiform pattern), nuclei with small nucleoli and clear or dispersed chromatin, multinucleated cells, interspersed small lymphocytes, +/-necrosis, +/-marked nuclear atypia, +/-abundant mitoses.
| Subtypes  =
| LMDDx      = [[histiocytic sarcoma]], [[thymoma]], other [[spindle cell lesions]]
| Stains    =
| IHC        = CD21 +ve, CD23 +ve, CD35 +ve, Ki-M4p +ve, Ki-FDRC1p +ve, vimentin +ve
| EM        =
| Molecular  =
| IF        =
| Gross      =
| Grossing  =
| Staging    =
| Site      =
| Assdx      =  [[Castleman disease|Castleman disease, hyaline-vascular type]]
| Syndromes  =
| Clinicalhx =
| Signs      =
| Symptoms  =
| Prevalence = very rare
| Bloodwork  =
| Rads      =
| Endoscopy  =
| Prognosis  =
| Other      =
| ClinDDx    =
| Tx        = excision
}}
'''Follicular dendritic cell sarcoma''', abbreviated '''FDCS''', is a very rare [[malignant]] tumour.
'''Follicular dendritic cell sarcoma''', abbreviated '''FDCS''', is a very rare [[malignant]] tumour.


It is also known as '''follicular dendritic cell tumour''' (abbreviated '''FDCT''').<ref name=pmid17515347>{{Cite journal  | last1 = Leipsic | first1 = JA. | last2 = McAdams | first2 = HP. | last3 = Sporn | first3 = TA. | title = Follicular dendritic cell sarcoma of the mediastinum. | journal = AJR Am J Roentgenol | volume = 188 | issue = 6 | pages = W554-6 | month = Jun | year = 2007 | doi = 10.2214/AJR.04.1530 | PMID = 17515347 }}</ref>
It is also known as '''follicular dendritic cell tumour''' (abbreviated '''FDCT'''),<ref name=pmid17515347>{{Cite journal  | last1 = Leipsic | first1 = JA. | last2 = McAdams | first2 = HP. | last3 = Sporn | first3 = TA. | title = Follicular dendritic cell sarcoma of the mediastinum. | journal = AJR Am J Roentgenol | volume = 188 | issue = 6 | pages = W554-6 | month = Jun | year = 2007 | doi = 10.2214/AJR.04.1530 | PMID = 17515347 }}</ref> and '''follicular dendritic cell neoplasm'''.<ref name=pmid19391666>{{Cite journal  | last1 = Denning | first1 = KL. | last2 = Olson | first2 = PR. | last3 = Maley | first3 = RH. | last4 = Flati | first4 = VR. | last5 = Myers | first5 = JL. | last6 = Silverman | first6 = JF. | title = Primary pulmonary follicular dendritic cell neoplasm: a case report and review of the literature. | journal = Arch Pathol Lab Med | volume = 133 | issue = 4 | pages = 643-7 | month = Apr | year = 2009 | doi = 10.1043/1543-2165-133.4.643 | PMID = 19391666 }}</ref>
 
Other possible names are: ''dendritic reticulum cell sarcoma'', (???) ''follicular dendritic cell neoplasm''. (???)


==General==
==General==
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*Behave like a low-grade sarcoma.<ref name=pmid9606805>{{Cite journal  | last1 = Perez-Ordoñez | first1 = B. | last2 = Rosai | first2 = J. | title = Follicular dendritic cell tumor: review of the entity. | journal = Semin Diagn Pathol | volume = 15 | issue = 2 | pages = 144-54 | month = May | year = 1998 | doi =  | PMID = 9606805 }}</ref>
*Behave like a low-grade sarcoma.<ref name=pmid9606805>{{Cite journal  | last1 = Perez-Ordoñez | first1 = B. | last2 = Rosai | first2 = J. | title = Follicular dendritic cell tumor: review of the entity. | journal = Semin Diagn Pathol | volume = 15 | issue = 2 | pages = 144-54 | month = May | year = 1998 | doi =  | PMID = 9606805 }}</ref>
*Reported association with [[Castleman disease]] (hyaline-vascular type).<ref name=pmid9606805/>
*Reported association with [[Castleman disease]] (hyaline-vascular type).<ref name=pmid9606805/>
WHO 2001 classification of dendritic cell neoplasms:<ref name=pmid17636477>{{Cite journal  | last1 = Kairouz | first1 = S. | last2 = Hashash | first2 = J. | last3 = Kabbara | first3 = W. | last4 = McHayleh | first4 = W. | last5 = Tabbara | first5 = IA. | title = Dendritic cell neoplasms: an overview. | journal = Am J Hematol | volume = 82 | issue = 10 | pages = 924-8 | month = Oct | year = 2007 | doi = 10.1002/ajh.20857 | PMID = 17636477 }}</ref>
*[[Langerhans cell histiocytosis]].
*Langerhans cell sarcoma.
*Interdigitating dendritic cell sarcoma/tumour.
*Follicular dendritic cell sarcoma/tumour.
*Dendritic cell sarcoma, not specified otherwise.
==Gross==
Features:
*Often a head and neck lesion.{{fact}}


==Microscopic==
==Microscopic==
Line 25: Line 66:
DDx:
DDx:
*[[Histiocytic sarcoma]].<ref name=pmid17324277>{{Cite journal  | last1 = Alexiev | first1 = BA. | last2 = Sailey | first2 = CJ. | last3 = McClure | first3 = SA. | last4 = Ord | first4 = RA. | last5 = Zhao | first5 = XF. | last6 = Papadimitriou | first6 = JC. | title = Primary histiocytic sarcoma arising in the head and neck with predominant spindle cell component. | journal = Diagn Pathol | volume = 2 | issue =  | pages = 7 | month =  | year = 2007 | doi = 10.1186/1746-1596-2-7 | PMID = 17324277 | url = http://www.diagnosticpathology.org/content/2/1/7 }}</ref>
*[[Histiocytic sarcoma]].<ref name=pmid17324277>{{Cite journal  | last1 = Alexiev | first1 = BA. | last2 = Sailey | first2 = CJ. | last3 = McClure | first3 = SA. | last4 = Ord | first4 = RA. | last5 = Zhao | first5 = XF. | last6 = Papadimitriou | first6 = JC. | title = Primary histiocytic sarcoma arising in the head and neck with predominant spindle cell component. | journal = Diagn Pathol | volume = 2 | issue =  | pages = 7 | month =  | year = 2007 | doi = 10.1186/1746-1596-2-7 | PMID = 17324277 | url = http://www.diagnosticpathology.org/content/2/1/7 }}</ref>
*[[Spindle cell lesions]].
*[[Thymoma]].
*[[Spindle cell lesions]] such as [[sarcomatoid carcinoma]].
*[[Meningioma]].{{fact}}


===Images===
===Images===
*[http://www.ajronline.org/content/188/6/W554/F7.expansion FDCS (ajronline.org)].<ref name=pmid17515347/>
 
*[http://www.webpathology.com/image.asp?case=391&n=1 DRCS (webpathology.com)].
==Related images==
<gallery>
Image: Follicular dendritic cell sarcoma -- very low mag.jpg | FDCS - very low mag. (WC)
Image: Follicular dendritic cell sarcoma -- low mag.jpg | FDCS - low mag. (WC)
Image: Follicular dendritic cell sarcoma - alt -- low mag.jpg | FDCS - low mag. (WC)
Image: Follicular dendritic cell sarcoma -- intermed mag.jpg | FDCS - intermed. mag. (WC)
Image: Follicular dendritic cell sarcoma -- high mag.jpg | FDCS - high mag. (WC)
Image: Follicular dendritic cell sarcoma -- very high mag.jpg | FDCS - very high mag. (WC)
Image: Follicular dendritic cell sarcoma - alt -- very high mag.jpg | FDCS - very high mag. (WC)
</gallery>
====www====
*[http://www.ajronline.org/doi/full/10.2214/AJR.04.1530 FDCS (ajronline.org)].<ref name=pmid17515347/>
*[http://www.npplweb.com/manuscripts/2MXAJX4VWD/0/imgfolder/WJSMRO_2_1_Fig2.jpg FDCS (npplweb.com)].<ref>URL: [http://www.npplweb.com/wjsmro/fulltext/2/1 http://www.npplweb.com/wjsmro/fulltext/2/1]. Accessed on: September 13, 2014.</ref>
*[http://path.upmc.edu/cases/case55/dx.html FDCT - several crappy images (upmc.edu)].
*[http://path.upmc.edu/cases/case55/dx.html FDCT - several crappy images (upmc.edu)].
*[http://path.upmc.edu/cases/case399.html FDCS - several images (upmc.edu)].
*[http://path.upmc.edu/cases/case399.html FDCS - several images (upmc.edu)].
Line 44: Line 99:
*Muscle-specific actin +ve/-ve.
*Muscle-specific actin +ve/-ve.
*EMA +ve/-ve.
*EMA +ve/-ve.
Additional stains:<ref name=pmid21055030>{{Cite journal  | last1 = Yin | first1 = WH. | last2 = Yu | first2 = GY. | last3 = Ma | first3 = Y. | last4 = Rao | first4 = HL. | last5 = Lin | first5 = SX. | last6 = Shao | first6 = CK. | last7 = Liang | first7 = Q. | last8 = Guo | first8 = N. | last9 = Chen | first9 = GQ. | title = [Follicular dendritic cell sarcoma: a clinicopathologic analysis of ten cases]. | journal = Zhonghua Bing Li Xue Za Zhi | volume = 39 | issue = 8 | pages = 522-7 | month = Aug | year = 2010 | doi =  | PMID = 21055030 }}</ref>
*CD23 +ve.
*D2-40 +ve.
Note:
*The interspersed lymphocytes are B cells.<ref name=pmid21835430>{{Cite journal  | last1 = Lorenzi | first1 = L. | last2 = Lonardi | first2 = S. | last3 = Petrilli | first3 = G. | last4 = Tanda | first4 = F. | last5 = Bella | first5 = M. | last6 = Laurino | first6 = L. | last7 = Rossi | first7 = G. | last8 = Facchetti | first8 = F. | title = Folliculocentric B-cell-rich follicular dendritic cells sarcoma: a hitherto unreported morphological variant mimicking lymphoproliferative disorders. | journal = Hum Pathol | volume = 43 | issue = 2 | pages = 209-15 | month = Feb | year = 2012 | doi = 10.1016/j.humpath.2011.02.029 | PMID = 21835430 }}</ref>
===Images===
<gallery>
Image: Follicular dendritic cell sarcoma - CD23 -- high mag.jpg | FDCS - CD23 - high mag. (WC)
Image: Follicular dendritic cell sarcoma - CD23 -- very high mag.jpg | FDCS - CD23 - very high mag. (WC)
Image: Follicular dendritic cell sarcoma - CD21 -- high mag.jpg | FDCS - CD21 - high mag. (WC)
Image: Follicular dendritic cell sarcoma - CD21 -- very high mag.jpg | FDCS - CD21 - very high mag. (WC)
</gallery>


==See also==
==See also==

Latest revision as of 15:50, 10 September 2018

Follicular dendritic cell sarcoma
Diagnosis in short

Micrograph showing a follicular dendritic cell sarcoma. The (benign) interspersed lymphocytes are a characteristic finding. H&E stain.
Synonyms follicular dendritic cell tumour, follicular dendritic cell neoplasm
LM oval or spindle-shaped cellular & nuclear morphology, variable architecture (sheets, fascicles, whorles, storiform pattern), nuclei with small nucleoli and clear or dispersed chromatin, multinucleated cells, interspersed small lymphocytes, +/-necrosis, +/-marked nuclear atypia, +/-abundant mitoses.
LM DDx histiocytic sarcoma, thymoma, other spindle cell lesions
IHC CD21 +ve, CD23 +ve, CD35 +ve, Ki-M4p +ve, Ki-FDRC1p +ve, vimentin +ve
Associated Dx Castleman disease, hyaline-vascular type
Prevalence very rare
Treatment excision

Follicular dendritic cell sarcoma, abbreviated FDCS, is a very rare malignant tumour.

It is also known as follicular dendritic cell tumour (abbreviated FDCT),[1] and follicular dendritic cell neoplasm.[2]

General

  • Very rare.
  • Behave like a low-grade sarcoma.[3]
  • Reported association with Castleman disease (hyaline-vascular type).[3]

WHO 2001 classification of dendritic cell neoplasms:[4]

  • Langerhans cell histiocytosis.
  • Langerhans cell sarcoma.
  • Interdigitating dendritic cell sarcoma/tumour.
  • Follicular dendritic cell sarcoma/tumour.
  • Dendritic cell sarcoma, not specified otherwise.

Gross

Features:

Microscopic

Features:[3]

  • Oval or spindle-shaped cellular & nuclear morphology.
  • Variable architecture (sheets, fascicles, whorles, storiform pattern).
  • Nuclei:
    • Small nucleoli.
    • Clear or dispersed chromatin.
  • Multinucleated cells.
  • Interspersed small lymphocytes - distinctive feature.
  • +/-Necrosis.
  • +/-Marked nuclear atypia.
  • +/-Abundant mitoses.

DDx:

Images

Related images

  • FDCS - very low mag. (WC)

  • FDCS - low mag. (WC)

  • FDCS - low mag. (WC)

  • FDCS - intermed. mag. (WC)

  • FDCS - high mag. (WC)

  • FDCS - very high mag. (WC)

  • FDCS - very high mag. (WC)

www

IHC

Features:[3]

  • CD21 +ve.
  • CD35 +ve.
  • Ki-M4p +ve
  • Ki-FDRC1p +ve.
  • Vimentin +ve.
  • S-100 +ve/-ve.
  • Muscle-specific actin +ve/-ve.
  • EMA +ve/-ve.

Additional stains:[7]

  • CD23 +ve.
  • D2-40 +ve.

Note:

  • The interspersed lymphocytes are B cells.[8]

Images

  • FDCS - CD23 - high mag. (WC)

  • FDCS - CD23 - very high mag. (WC)

  • FDCS - CD21 - high mag. (WC)

  • FDCS - CD21 - very high mag. (WC)

See also

References

  1. ↑ 1.0 1.1 Leipsic, JA.; McAdams, HP.; Sporn, TA. (Jun 2007). "Follicular dendritic cell sarcoma of the mediastinum.". AJR Am J Roentgenol 188 (6): W554-6. doi:10.2214/AJR.04.1530. PMID 17515347.
  2. ↑ Denning, KL.; Olson, PR.; Maley, RH.; Flati, VR.; Myers, JL.; Silverman, JF. (Apr 2009). "Primary pulmonary follicular dendritic cell neoplasm: a case report and review of the literature.". Arch Pathol Lab Med 133 (4): 643-7. doi:10.1043/1543-2165-133.4.643. PMID 19391666.
  3. ↑ 3.0 3.1 3.2 3.3 Perez-Ordoñez, B.; Rosai, J. (May 1998). "Follicular dendritic cell tumor: review of the entity.". Semin Diagn Pathol 15 (2): 144-54. PMID 9606805.
  4. ↑ Kairouz, S.; Hashash, J.; Kabbara, W.; McHayleh, W.; Tabbara, IA. (Oct 2007). "Dendritic cell neoplasms: an overview.". Am J Hematol 82 (10): 924-8. doi:10.1002/ajh.20857. PMID 17636477.
  5. ↑ Alexiev, BA.; Sailey, CJ.; McClure, SA.; Ord, RA.; Zhao, XF.; Papadimitriou, JC. (2007). "Primary histiocytic sarcoma arising in the head and neck with predominant spindle cell component.". Diagn Pathol 2: 7. doi:10.1186/1746-1596-2-7. PMID 17324277. http://www.diagnosticpathology.org/content/2/1/7.
  6. ↑ URL: http://www.npplweb.com/wjsmro/fulltext/2/1. Accessed on: September 13, 2014.
  7. ↑ Yin, WH.; Yu, GY.; Ma, Y.; Rao, HL.; Lin, SX.; Shao, CK.; Liang, Q.; Guo, N. et al. (Aug 2010). "[Follicular dendritic cell sarcoma: a clinicopathologic analysis of ten cases].". Zhonghua Bing Li Xue Za Zhi 39 (8): 522-7. PMID 21055030.
  8. ↑ Lorenzi, L.; Lonardi, S.; Petrilli, G.; Tanda, F.; Bella, M.; Laurino, L.; Rossi, G.; Facchetti, F. (Feb 2012). "Folliculocentric B-cell-rich follicular dendritic cells sarcoma: a hitherto unreported morphological variant mimicking lymphoproliferative disorders.". Hum Pathol 43 (2): 209-15. doi:10.1016/j.humpath.2011.02.029. PMID 21835430.
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