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Table of Contents    
LETTER TO EDITOR
Year : 2016  |  Volume : 64  |  Issue : 5  |  Page : 1035-1036

Pediatric subcutaneous panniculitis-like T-cell lymphoma presenting as paraparesis: An unreported manifestation


Department of Pathology, Command Hospital (CC) Lucknow, Uttar Pradesh, India

Date of Web Publication12-Sep-2016

Correspondence Address:
R Ranjan
Department of Pathology, Command Hospital (CC) Lucknow, Uttar Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.190275

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How to cite this article:
Ranjan R, Singh RK, Kumar N, Dutta V. Pediatric subcutaneous panniculitis-like T-cell lymphoma presenting as paraparesis: An unreported manifestation. Neurol India 2016;64:1035-6

How to cite this URL:
Ranjan R, Singh RK, Kumar N, Dutta V. Pediatric subcutaneous panniculitis-like T-cell lymphoma presenting as paraparesis: An unreported manifestation. Neurol India [serial online] 2016 [cited 2020 May 28];64:1035-6. Available from: http://www.neurologyindia.com/text.asp?2016/64/5/1035/190275




Sir,

Subcutaneous panniculitis-like T-cell lymphomas (SPTCLs) are extremely rare non-Hodgkin's lymphomas (NHLs) and are uncommon in the pediatric age group.[1] They are broadly divided into two groups based on the T-cell receptor expression; α/β SPTCL and γ/δ SPTCL.

We report a case of SPTCL in an 11-year-old girl presenting with paraparesis and cutaneous lesions for 1 month. The parents sought medical attention when the child developed paraparesis. She had nodules over the occipital region, right parietal region, anterior abdominal wall, and the lumbar spine. The muscle power in the lower limbs was diminished. Magnetic resonance imaging of the spine showed heterogeneously enhancing epidural lesions with a soft tissue component at D6–D8 vertebrae causing cord compression. An enhancing soft tissue lesion with an osseous defect in the occipital bones and associated soft tissue component was also reported. A clinical differential diagnosis of a sarcoma or metastatic disease was entertained. In view of the progressing paraparesis, a decompression surgery was performed and resulted in a marked symptomatic improvement.

The biopsy showed a subcutaneous lesion with extensive necrosis and sheets of monomorphic malignant small round cells. A preferential perivascular and periadipocytic location of these cells were noted [Figure 1]. These cells on immunohistochemistry were positive for leucocyte common antigen (LCA), cluster difference (CD) 3, and CD8 and were negative for CD 20, terminal deoxynucleotidyl transferase (TDT), CD 99, CD 4, CD30, CD 56, anaplastic lymphoma kinase protein, cytokeratin (CK), vimentin, chromogranin, and synaptophysin. A diagnosis of SPTCL-NHL type was made.
Figure 1: Photomicrograph showing the atypical lymphoid cells with adipocyte rimming.

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The natural history of SPTCL is divided into two courses, one, an aggressive course with the T cell receptor (TCR) γ/δ (SPTCL-GD) with phenotype CD4−, CD8−, CD56+ and the other, a relatively indolent clinical course TCR α/β (SPTCL-AB) with phenotype CD4−, CD8+, CD56−.[2],[3] The two groups differ significantly with respect to the age group of patients, presentation, histopathology, immunohistochemistry, and outcome. On histopathology, the SPTCL-AB lesions are centered predominantly in the subcutis with sparing of the deep dermis and the epidermis; the atypical lymphoid cells are small-to-medium sized with associated giant cells, and histiocytes are occasional with much less frequency of angioinvasion and angiodestruction. The rimming of individual adipocytes by neoplastic T-cells is common. Cases of SPTCL-GD have a higher median age and show a large-sized cell, more necrosis, and angiodestruction. The overall survival in cases of SPTCL-AB and SPTCL-GD is 82 and 11%, respectively.[4]

SPTCL presents in the form of skin lesions or occasionally as lymphadenopathy, hepatosplenomegaly, pleural effusions, and hemophagocytosis. Only an occasional case of SPTCL-GD has been reported to have metastasis to the lungs, liver, kidneys, and the central nervous system.[4]

SPTCL is a rare NHL in pediatric patients and has never been reported with paraparesis. This case indicates that paraparesis can also be a manifestation of this enigmatic disease.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Chan JKC. Tumors of lymphoreticular system. In: Christopher D. M. Fletcher, editor. Fletcher Diagnostic Histopathology of Tumors. 3rd ed. Philadelphia, USA: Elsevier; 2007. p. 1122-3.  Back to cited text no. 1
    
2.
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 2009;133:303-8.  Back to cited text no. 2
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3.
Bittencourt AL, Vieira MD, Carvalho EG, Cunha C, Araujo I. Subcutaneous panniculitis-like T-cell lymphoma (SPTL) in a child with spontaneous resolution. Case Rep Oncol Med 2011;2011:639240.  Back to cited text no. 3
[PUBMED]    
4.
Willemze 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.  Back to cited text no. 4
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