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Year : 2015  |  Volume : 52  |  Issue : 4  |  Page : 675--676

Subcutaneous panniculitis-like T-cell lymphoma: A report of five cases with review of literature

Rekha A Nair, N Arunkumar, Priya Mary Jacob, Nileena Nayak 
 Department of Pathology, Regional Cancer Centre, Thiruvananthapuram, Kerala, India

Correspondence Address:
Rekha A Nair
Department of Pathology, Regional Cancer Centre, Thiruvananthapuram, Kerala
India




How to cite this article:
Nair RA, Arunkumar N, Jacob PM, Nayak N. Subcutaneous panniculitis-like T-cell lymphoma: A report of five cases with review of literature.Indian J Cancer 2015;52:675-676


How to cite this URL:
Nair RA, Arunkumar N, Jacob PM, Nayak N. Subcutaneous panniculitis-like T-cell lymphoma: A report of five cases with review of literature. Indian J Cancer [serial online] 2015 [cited 2020 Sep 22 ];52:675-676
Available from: http://www.indianjcancer.com/text.asp?2015/52/4/675/178416


Full Text

Sir,

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a cutaneous T-cell lymphoma of cytotoxic T-cell type infiltrating subcutaneous tissue with preferential sparing of epidermis and dermis.

[1] In the recent “WHO classification of tumors of hematopoietic and lymphoid tissues,” it is classified as a distinct entity under mature Natural Killer (NK)/T-cell neoplasms with exclusion of γδ T-cell lineage. The incidence in Asia has been reported to be around 1.3%.[2] We reviewed five cases of SPTCL which were reported over a period of 5 years from June 2006 to May 2012 at our center.

Out of the five patients, four were males and one was a female, with an age range of 18-78 years. Four of our patients presented with subcutaneous nodules involving the sites that included axilla, both legs, site of previous surgical scar, while one patient presented with a localized thickening of the skin in the medial and lateral aspect of right thigh. No prodromal symptoms were seen. Lab investigations including peripheral smear and bone marrow were within the normal limits except in one case, which presented with leucopenia and elevated liver enzymes.

All cases demonstrated a dense lymphoid infiltrate located in the subcutaneous tissue resembling lobular panniculitis at low power [Figure 1]. The overlying epidermis and dermis were uninvolved except in one case where there was dermal infiltration. The atypical lymphoid cells were pleomorphic small to medium sized to diffusely large T-cells with hyper-chromatic irregularly contoured nuclei. A diagnostic feature in SPTCL is rimming of fat cells by neoplastic cells [Figure 2], described as adipotropism,[3] which was seen in all our cases. Areas of karyorrhexis and cytophagocytosis were seen in two cases. Angiocentric infiltration was seen in one case, which also showed areas of necrosis and foreign body giant cell reaction. All cases showed cluster of differentiation (CD) 3 positive [Figure 3], CD8 positive [Figure 4], Granzyme B positive [Figure 5], Perforin positive [Figure 6], CD4 negative, CD56 negative immunophenotype. Two cases showed loss of CD5 and another two cases showed loss of CD7. Epstein-Barr virus (EBV) detection by EBV-encoded RNA (EBER) in situ hybridization was done in all the five cases and were negative.{Figure 1}{Figure 2}{Figure 3}{Figure 4}{Figure 5}{Figure 6}

Median age at presentation of SPTCL is 35 years.[4] In our series, the median age was 57 years. In SPTCL, the subcutaneous nodules are usually localized to the extremities and trunk. The nodules vary from 0.5 cm to several centimeters in diameter. Necrosis and ulceration are rare. Systemic symptoms are seen in up to 50% of patients, while lab abnormalities like cytopenias and elevated liver enzymes are seen in up to 20% of the cases. In a resident short review by Zaheeda et al., peripheral cytopenias are seen in more than 50% cases of SPTCL.[5] In our study population, one case had leucopenia and elevated liver enzymes, while rest of them showed normal counts and normal levels of liver enzymes. Hemophagocytic syndrome is reported but the incidence is less common than in cutaneous lymphomas of γδ T-cell origin.[1] In our study population, two cases showed evidence of hemophagocytosis. Rimming of fat cells by neoplastic lymphoid cells, described as adipotropism by Willemze et al.[3] were seen in all our cases. Karyorrhexis, cytophagocytosis, fat necrosis may be seen.[6] Out of the five cases, two cases showed evidence of karyorrhexis and cytophagocytosis. In SPTCL, the atypical lymphoid cells are invariably positive for CD3, CD8, Granzyme B, Perforin and negative for CD4 and CD56.[4] All our cases showed the same immunophenotype. Negative expression of CD56 helps in differentiating cutaneous γδ T-cell lymphoma.[4] The loss of CD2, CD5 and/or CD7 are seen in a proportion of cases.[3] In our series, two cases showed loss of CD5 and another two showed loss of CD7. The pathologist can encounter problems in differentiating from benign panniculitis and primary cutaneous NK/T-cell lymphoma. Benign panniculitis in contrast to SPTCL, shows aggregates of CD20 positive B cells, admixed with variable proportion of CD3, CD4, CD8 positive T-cells. Primary cutaneous NK/T-cell lymphomas are invariably CD56 positive and show a predominant dermal infiltrate with angioinvasion, angiodestruction and EBV positivity. In our series, all cases were EBV negative by in situ hybridization. The distinction from primary cutaneous NK/T-cell lymphoma carries significance as the 5 years overall survival rate is just 11% when compared to 82% in case of SPTCL.[5] Hemophagocytic syndrome and lymph node dissemination indicates a poor prognosis. In a case report of SPTCL involving breast by Uematsu et al. the role of magnetic resonance imaging (MRI), elastography, Fluorodeoxyglucose positron emission tomography (FDG-PET) and digital mammography had been analyzed.[7] Among these, dynamic contrast enhanced MRI was found to be useful in detecting subcutaneous fat necrosis, a primary radiological feature of SPTCL. As it does not rule out other causes of fat necrosis, a core needle biopsy is absolutely essential in diagnosing the same. Therapeutically, combination chemotherapy like cyclophosphamide, doxorubicin, vincristine and prednisone (CHOP) had been traditionally used, while aggressive cases are treated with anthracycline based regimens.[4] We present these cases to signify the importance of differentiating it from other aggressive cutaneous T-cell lymphomas, owing to their response to combination chemotherapy.

References

1Jaffe E. T-cell and natural killer-cell lymphomas An update based on the 2008 WHO classification. Hematol Meet Rep (formerly Haematol Rep) [Internet]. 2009 Jun 11; [Cited 2013 Nov 16]; 3(1):[about 0 p.]. Available from: http://www.pagepress.org/journals/index.php/hmr/article/view/521.
2Vose J, Armitage J, Weisenburger D, International T-Cell Lymphoma Project. International peripheral T-cell and natural killer/T-cell lymphoma study: Pathology findings and clinical outcomes. J Clin Oncol 2008;26:4124-30.
3Willemze R, Jansen PM, Cerroni L, Berti E, Santucci M, Assaf C, et al. Subcutaneous panniculitis-like T-cell lymphoma: Definition, classification, and prognostic factors: An EORTC cutaneous lymphoma group study of 83 cases. Blood 2008;111:838-45.
4Jaffe ES, Gaulard P, Ralfkiaer E, Cerroni L, Meijer CM. Subcutaneous panniculitis-like T-cell lymphoma. In: Swerdlow SH, editor. WHO Classification of Tumors of Haematopoietic and Lymphoid Tissues. 4th ed. Lyon: IARC Press; 2008. p. 294-5.
5Parveen 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 2009;133:303-8.
6Kong YY, Dai B, Kong JC, Zhou XY, Lu HF, Shen L, et al. Subcutaneous panniculitis-like T-cell lymphoma: A clinicopathologic, immunophenotypic, and molecular study of 22 Asian cases according to WHO-EORTC classification. Am J Surg Pathol 2008;32:1495-502.
7Uematsu T, Kasami M. 3T-MRI, elastography, digital mammography, and FDG-PET CT findings of subcutaneous panniculitis-like T-cell lymphoma (SPTCL) of the breast. Jpn J Radiol 2012;30:766-71.