Follicular dendritic cell sarcoma
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Follicular dendritic cell sarcoma, abbreviated FDCS, is a very rare malignant tumour.
| Follicular dendritic cell sarcoma | |
|---|---|
| Diagnosis in short | |
|
| |
| 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, CD35 +ve, Ki-M4p +ve, Ki-FDRC1p +ve, vimentin +ve |
| Associated Dx | Castleman disease, hyaline-vascular type |
| Prevalence | very rare |
| Treatment | excision |
It is also known as follicular dendritic cell tumour (abbreviated FDCT).[1]
Other possible names are: dendritic reticulum cell sarcoma, (???) follicular dendritic cell neoplasm. (???)
General
- Very rare.
- Behave like a low-grade sarcoma.[2]
- Reported association with Castleman disease (hyaline-vascular type).[2]
Microscopic
Features:[2]
- 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
IHC
Features:[2]
- CD21 +ve.
- CD35 +ve.
- Ki-M4p +ve
- Ki-FDRC1p +ve.
- Vimentin +ve.
- S-100 +ve/-ve.
- Muscle-specific actin +ve/-ve.
- EMA +ve/-ve.
See also
References
- ↑ 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.
- ↑ 2.0 2.1 2.2 2.3 Perez-Ordoñez, B.; Rosai, J. (May 1998). "Follicular dendritic cell tumor: review of the entity.". Semin Diagn Pathol 15 (2): 144-54. PMID 9606805.
- ↑ 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.
- ↑ URL: http://www.npplweb.com/wjsmro/fulltext/2/1. Accessed on: September 13, 2014.