Subcutaneous panniculitis-like T-cell lymphoma in children. Literature review and case reports

Subcutaneous panniculitis-like T-cell lymphoma (SPTL) is a rare tumor from αβ mature cytotoxic T-lymphocytes, which primarily affects the subcutaneous adipose tissue and has morphological manifestations similar to panniculitis. SPTL frequency is less than 1 % of all NHL. It occurs in all age groups, but only 19–20 % are patients younger 20 years. Median age of patients is 35–36 years. To date are only a few cases in children described. In this article we described 3 SPTL cases in patients 1, 10 and 17 years old with typical clinical presentation and detailed analysis.

1. Jaffe E.S., Harris N.L., Vardiman J.W. et al. (еds.). Hematopathology. Elsevier Saunders Publishers, St. Louis, MO, USA 2010.

2. Swerdlow S.H., Campo E., Harris N.L. et al. WHO Classification of Tumours, Volume 2; IARC WHO Classification of Tumours, No 2. Lyon, France, IARC Press, 2008. 439 p.

3. Hytiroglou P., Phelps R.G., Wattenberg D.J., Strauchen J.A. Histiocytic cytophagic panniculitis: molecular evidence for a clonal T-cell disorder. J Am Acad Dermatol 1992;27 (2 Pt 2):333–6.

4. Willemze R., Jansen P.M., Cerroni L. et al. Subcutaneous panniculitis-like T-cell lymphoma: definition, classification, and prognostic factors: an EORTC Cutaneous Lymphoma Group Study of 83 cases. Blood 2008;111(2):838–45.

5. Parveen Z., Thompson K. Subcutaneous panniculitis-like T-cell lymphoma: redefinition of diagnostic criteria in the recent World Health Organization-European Organization for Research and Treatment of Cancer classification for cutaneous lymphomas. Arch Pathol Lab Med 2009Feb;133(2):303–8.

6. Mauch P.M., Armitage J.O., Coiffier B. et al. (eds.). Non-Hodgkin’s Lymphomas. Philadelphia, PA: Lippincott Williams & Wilkins, 2004.

7. Cerroni L., Gatter K., Helmut K. Skin Lymphoma: The Illustrated Guide, 3rd Edition. Wiley-Blackwell, 2011. 288 p.

8. Gonzalez C.L., Medeiros L.J., Braziel R.M., Jaffe E.S. T-cell lymphoma inolving subcutaneous soft tissue. A clinicopathologic entity commonly associated with hemophagocytic syndrom. Am J Surg Pathol 1991;15(1):17–27.

9. Hoque S.R., Child F.G., Whittaker S.G. et al. Subcutaneous panniculitis-like T-cell lymphoma: clinicopathological, immunophenotypic and molecular analysis of six patients. Br J Dermatol 2003;148: 516–25.

10. Burg G., Kempf W., Cozzio A. et al. WHO/EORTC classification of cutaneous lymphomas 2005: histological and molecular aspects. J Cutan Pathol 2005;32:647–74.

11. Hahtola S., Burghart Е., Jeskanen L. et al. Clinicopathological characterization and genomic aberration in subcutaneous panniculitis-like T-cell lymphoma. J Invest Dermatol 2008;128:2304–9.

12. Willemze R., Dreyling M; ESMO Guidelines Working Group. Primary cutaneous lymphoma: ESMO clinical recommendations for diagnosis, treatment and follow-up. Ann Oncol 2009;20 (suppl 4):iv115-iv118.