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 Table of Contents  
CASE REPORT
Year : 2014  |  Volume : 2  |  Issue : 2  |  Page : 225-227

Subcutaneous panniculitis-like T-cell lymphoma


Department of Pathology, Dr. D.Y Patil Medical College and Hospital, Pimpri, Pune, India

Date of Web Publication11-Nov-2014

Correspondence Address:
Banyameen Iqbal
Department of Pathology, Dr. D.Y Patil Medical College and Hospital, Pimpri, Pune
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2321-4848.144350

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  Abstract 

Panniculitis like T-cell lymphoma is a rare, aggressive variant of cutaneous T-cell lymphoma. The main dilemma lies in differentiating these lesions from simple panniculitis which is a benign lesion. We present the case of a 73 -year-old female patient, who presented with complaints of swelling in left axilla since 15 days, associated with throbbing pain and swelling of one arm. The initial diagnosis was paniculitis, but no clinical improvement was seen despite systemic antibiotic therapy. After two skin biopsies, the patient was diagnosed with panniculitis cutaneous T-cell lymphoma (PTCL). SPTCL can be a challenge to diagnose and can be misdiagnosed as benign panniculitis due to similarities in clinical and histological features between the two entities and for which immunohistochemical stains are required for confirmation of diagnosis.

Keywords: Panniculitis lymphoma, subcutaneous lymphomas, T cell lymphoma


How to cite this article:
Jain A, Iqbal B, Kambale T. Subcutaneous panniculitis-like T-cell lymphoma . Arch Med Health Sci 2014;2:225-7

How to cite this URL:
Jain A, Iqbal B, Kambale T. Subcutaneous panniculitis-like T-cell lymphoma . Arch Med Health Sci [serial online] 2014 [cited 2019 Oct 15];2:225-7. Available from: http://www.amhsjournal.org/text.asp?2014/2/2/225/144350


  Introduction Top


Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) belongs to the category of extranodal mature T and natural killer (NK)-cell neoplasms. [1],[2] SPTCL is a type of skin lymphoma in which there is infiltration by neoplastic cytotoxic T cells mimicking panniculitis. It was first described by Gonzalez et al., in 1991, when they presented eight cases of T-cell lymphoma which were localized primarily to the subcutaneous adipose tissue without any lymph node involvement. [3] The incidence of SPTCL is less than 1% of the non-Hodgkin lymphomas. [1]

SPTCL is most common in young females with a male to female ratio of 0.5. [4] The median age of this lesion is 36 years (range 9-79 years) with 19% of patients being 20 years or younger. [4] Clinically, it presents as multiple palpable nodules on the trunk or lower extremities, and less commonly on the face or upper extremities. [1]

Histologically, SPTCL is characterized by an infiltrate composed predominantly of atypical lymphocytes and histiocytes. Extensive tumor necrosis, karyorrhectic debris along with cytophagocytosis and angioinvasion characterize this disease. Apoptotic mechanisms are known to be involved in its pathogenesis. [5],[6],[7],[8]

We report a case of an old lady with SPTCL, who presented with a swelling in left axilla.


  Case Report Top


A 73-year-old lady presented with complaint of swelling in left axilla since 15 days associated with pain, throbbing in nature, and swelling of arm. On local examination, there was an ill-defined, 10 × 8 × 5 cm swelling seen in left axilla extending into chest wall. There was no ulceration, organomegaly, or palpable lymph nodes; however, the patient had pallor. The hematological investigations showed the following: Hb 8.6 gm/dl; total count 3700 cells/mm 3 ; differential count: Lymphocytes-47%, neutrophils-50%, basophils-0 %, eosinophils-2%, monocytes-1%; and erythrocyte sedimentation rate (ESR) was 28 mm at 1 hour; human immunodeficiency virus (HIV) status was non-reactive. Serological investigations for systemic lupus erythematosus (SLE) were negative. Ultrasonography (USG) done shows feature of panniculitis.

Fine needle aspiration cytology (FNAC) was attempted which showed hemorrhage with inflammatory cells comprising of lymphocytes and macrophages, finding were suggestive of inflammatory pathology [Figure 1]. An incisional biopsy was performed and submitted for histopathology. Grossly, the biopsy consisted of gray-white tissue fragments measuring 15 × 1 × 0.5 cm in diameter. Histopathological examination shows subcutaneous fat with predominant lobular infiltration of atypical lymphoid cells [Figure 2]. The interstitium of fat lobules was replaced by pleomorphic small-medium and medium-large atypical lymphocytes with irregular nuclei having dense chromatin. Characteristically, there was rimming of individual fat cells by surrounding neoplastic lymphocytes. Mitotic figures and karyorrhectic debris were noted. Histiocytes were seen amidst these lymphocytes. However, no erythrophagocytosis was seen and no granuloma was observed. Immunohistochemical study showed CD3 and CD5 immunoreactivity and CD30 and CD20 negativity of the lymphoid cells [Figure 3]. A diagnosis of SPTCL was made and the patient was referred to oncology center.
Figure 1: FNAC showing hemorrhage and inflammatory cells only (leishman ×40)

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Figure 2: Figure showing subcutaneous fat with predominant lobular infiltration by atypical lymphoid cells (H and E ×100)

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Figure 3: Figure showing high power view of atypical cells showing CD3 positivity

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  Discussion Top


SPTCL is a rare entity; accounting for less than 1% of all non hodgkins lymphoma (NHLs). [1] SPTCL has been recognized since 1997 and was included as a separate entity since 2001. World Health Organization/ European Organisation for Research and Treatment of Cancer (WHO/EORTC) classification of hematolymphoid classification; in 2008 WHO classification, it continues to be recognized as a distinct entity. [5] SPTCL is most common in young adults and has a female preponderance. [4] In our case, patient is 73-year-old female. Its association in immunosuppressed states has been reported. [9] However, our patient was immunocompetent. Presentation of this disease is mostly at a dermatology clinic, with multiple asymptomatic subcutaneous nodules and plaques, predominantly affecting the legs or trunk, and less commonly the faces or arms, [6] in our case a 10 × 8 × 5 cm swelling was seen in left axilla and extending into chest wall.

Laboratory findings may not be severe even though more than 50% may show cytopenias. [10] In our case, patient also present with cytopenia. Total count 3,700 cells/mm3; with differential count: Lymphocytes-47%, neutrophils-50%, eosinophils-2%, monocytes-1%.

Histopathologically, there is dense infiltration of subcutaneous fat tissue by small to medium and medium to large sized lymphocytes, predominantly in lobular pattern. Dermal invasion is absent or very minimal. The neoplastic lymphocytes show atypical features like hyperchromatic and angulated nuclei. The lymphomatous infiltrate may be admixed with histiocytes, plasma cells, and neutrophils, mimicking benign panniculitis. Characteristic feature of rimming of individual fat spaces by neoplastic lymphocytes will provide a diagnostic clue to this entity although it can be seen in other primary and secondary cutaneous lymphomas. [8],[10] Our case also presented with similar finding. Angioinvasion may also be a feature. [7] In our case, angioinvasion is not seen. The neoplastic lymphoid cells are of cytotoxic T-cell phenotype, which are CD3+, CD4−, CD8+, and CD56 [4],[7],[8] In our case, we had CD3 +, CD 45+, and CD20−. Staining for apoptosis shows numerous positive apoptotic bodies which suggest apoptosis as the mechanism of cell death in these tumors. [8] SPTCLs are lymphomas for which treatment modalities are as yet not well defined. The cell of origin is the cytotoxic T-cell. [8] Treatment used in the past includes systemic chemotherapy, radiotherapy, and high-dose chemotherapy with stem cell support. McGinnis KS et al., recently described DeniLeukin diftitox, which is a recombinant fusion protein that combines human interleukin 2 and diphtheria toxin as an effective, relatively non-toxic therapy for panniculitic T-cell lymphoma. [11]


  Conclusion Top


SPTCL is a rare cytotoxic lymphoma which can be a challenge to diagnose and can be misdiagnosed as benign panniculitis due to similarities in clinical and histological features between the two entities. This may lead to delay in the diagnosis. Characteristic rimming of neoplastic lymphoid cells around individual adipocyte should raise a high index of suspicion of this lesion, which can be confirmed on immunohistochemical stains. Delay in diagnosis can lead to worsening of symptoms and higher probability of morbidity. In order to observe the course of the disease, long-term follow-up of these patients is important.


  Acknowledgement Top


There is neither conflict of interest between authors nor financial or material support from any firm, funding organization, or sponsor in design and conduct of the study; collection, management, analysis, and interpretation of the data; and preparation, review, or approval of the manuscript.

 
  References Top

1.Jaffe ES, Ralfkiaer E. Pathology and Genetics of Tumours of Haematopoietic and Lymphoid Tissues. Subcutaneous panniculitis-like T-cell lymphoma. In: World Health Organization Classification of Tumours. Lyon: IARC Press; 2001. p. 212-3.  Back to cited text no. 1
    
2.Chan JK. Tumours of the lymphoreticular system, including spleen and thymus. In: Fletcher CD, editor. Diagnostic Histopathology of Tumors. 2 nd ed. Philadelphia: Churchill Livingstone; 2000. p. 1182-3.  Back to cited text no. 2
    
3.Gonzalez CL, Medeiros LJ, Braziel RM, Jaffe ES. T-cell lymphoma involving subcutaneous tissue: A clinicopathologic entity commonly associated with hemophagocytic syndrome. Am J Surg Pathol 1991;15:17-27.  Back to cited text no. 3
    
4.Willemze R, Jansen PM, Cerroni L, Berti E, Santucci M, Assaf C, et al, EORTC Cutaneous Lymphoma Group. 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
    
5.Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, et al. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues. 4 th ed. Lyon: IARC Press; 2008. p. 294-5.  Back to cited text no. 5
    
6.Hoque SR, Child FJ, Whittaker SJ, Ferreira S, Orchard G, Jenner K, et al. SPTCL: A clinicopathologic, immunophenotypic and molecular analysis of six patients Br J Dermatol 2003;148:516-25.  Back to cited text no. 6
    
7.Salhany KE, Macon WR, Choi JK, Elenitsas R, Lessin SR, Felgar RE, et al. Subcutaneous panniculitis-like T-cell lymphoma: Clinicopathologic, immunophenotypic and genotypic analysis of alpha/beta and gamma/ delta subtypes. Am J Surg Pathol 1998;22:881-93.  Back to cited text no. 7
    
8.Kumar S, Krenacs L, Medeiros J, Elenitoba-Johnson KS, Greiner TC, Sorbara L, et al. Subcutaneous panniculitic T-cell lymphoma is a tumor of cytotoxic T lymphocytes. Hum Pathol 1998;29:397-403.  Back to cited text no. 8
    
9.Kaplan MA, Ferry JA, Harris NL, Jacobson JO. Clonal analysis of post transplant lymphoproliferative disorders, using both episomal Epstein-Barr virus and immunoglobulin genes as markers. Am J Clin Pathol 1994;101:590-6.  Back to cited text no. 9
    
10.Parveen Z, Thompson K. Subcutaneous panniculitis like T-cell Lymphoma: Redefinition of diagnostic criteria in the recent World Health Organisation-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. 10
    
11.McGinnis KS, Shapiro M, Junkins-Hopkins JM, Smith M, Lessin SR, Vittorio CC, et al. Denileukin diftitox for the treatment of panniculitic lymphoma. Arch Dermatol 2002;138:740-2.  Back to cited text no. 11
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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Case Report
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