International Journal of Pediatric Otorhinolaryngology Extra
Volume 2, Issue 3 , Pages 144-146, September 2007

Peripheral T-cell NHL on the glabella in an infant

  • Serhan Küpeli

      Affiliations

    • Hacettepe University, Institute of Oncology, Department of Pediatric Oncology, Sihhiye, 06100 Ankara, Turkey
    • Corresponding Author InformationCorresponding author. Tel.: +903123052989.
    • ,
  • Ali Varan

      Affiliations

    • Hacettepe University, Institute of Oncology, Department of Pediatric Oncology, Sihhiye, 06100 Ankara, Turkey
    • ,
  • Canan Akyüz

      Affiliations

    • Hacettepe University, Institute of Oncology, Department of Pediatric Oncology, Sihhiye, 06100 Ankara, Turkey
    • ,
  • Diclehan Orhan

      Affiliations

    • Hacettepe University, Faculty of Medicine, Department of Pediatric Pathology, Turkey
    • ,
  • Yavuz Köksal

      Affiliations

    • Hacettepe University, Institute of Oncology, Department of Pediatric Oncology, Sihhiye, 06100 Ankara, Turkey
    • ,
  • Münevver Büyükpamukçu

      Affiliations

    • Hacettepe University, Institute of Oncology, Department of Pediatric Oncology, Sihhiye, 06100 Ankara, Turkey

Received 21 February 2007; received in revised form 14 April 2007; accepted 15 April 2007.

Article Outline

Summary 

To date, several pediatric NHL cases in unusual localizations such as orbita, lacrimal sac and eyelid have been published. This is the first case of PTCL reported in childhood involving glabellar region. Only sporadic case reports and small series have been reported related with PTCL in children. The present case is interesting both for the site of involvement, glabella, and in histopathologic subtype, peripheral T-cell lymphoma and also in clinical progress.

Keywords: NHL, Glabella, Infant

 

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1. Peripheral T-cell non-hodgkin lymphoma on the glabella in an infant 

T-cell non-Hodgkin lymphoma basically can be divided into two subtypes; precursor T-cell lymphoma and mature (peripheral) T-cell lymphoma [1]. To date, several pediatric NHL cases in unusual localizations such as orbita, lacrimal sac and eyelid have been published [2], [3], [4]. Peripheral T-cell lymphoma (PTCL) is a rare entity in childhood and has not been well defined as it is in adults. Only sporadic case reports and small series have been reported related with PTCL in children [5], [6], [7], [8]. The present case is interesting both for the site of involvement, glabella, and in histopathologic subtype, peripheral T-cell lymphoma and also in clinical progress.

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2. Case report 

A 22-month-old boy was admitted to our hospital with 20 days history of red-colored swelling in his forehead. Despite the antibiotic therapy, the swelling has continued to grow and the patient has been referred to our department. In physical examination of the child, a soft tissue mass on the glabella extending to the medial part of the right orbita without signs of inflammation and a lymphadenopathy, 1cm in diameter, in right anterior cervical region were noted. The magnetic resonance imaging investigation revealed a soft tissue mass on the glabella, extending to the right medial chantus and right part of the nose, with multiple lymph nodes in parotid glands, submandibular and jugular regions (Fig. 1).

Biopsy was performed from the lesion on the nose and histopathology showed diffuse infiltration of the subcutaneous tissue by neoplastic, atypical lymphocytes with hyperchromatic round-oval shaped nuclei (Fig. 2). Immunohistochemistry demonstrated LCA, CD45RO and CD3 positive cells whereas no immunoreactivity was found with CD20, CD79, Tdt, CD30, desmin, myogenin, myoglobin, S-100, NSE, ChrA or muscle specific actin. After the diagnosis of the PTCL the patient was given Vincristin (1.5mg/m2), Cyclophosphamide (300mg/m2) and Prednisone (60mg/m2) chemotherapy. One week after the first chemotherapy course the primary lesion reduced in size. The same protocol was planned to be given for two more times with intrathecal prophylaxis.

Two weeks after the first course, an enlargement in the lesion on glabella was realized and the patient was considered in state of progression clinically. A more intensive chemotherapy protocol consisting of Vincristin (2mg/m2), Cyclphosphamide (500mg/m2), Doxorubicine (60mg/m2), Prednisolone (60mg/m2), and Methotrexate (3g/m2) was started. After consolidation therapy a partial remission was ensured but during maintenance therapy a bone marrow relapse was determined and ALL Rez BFM-2002 protocol was started for salvage. During this therapy bone marrow infiltration was noted after two courses. In this relapse the bone marrow examination interestingly showed B-cell surface markers in neoplastic cells. An HLA identical donor was not found for bone marrow transplantation. The patient was lost in an attack of febrile neutropenia.

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3. Discussion 

Childhood lymphoblastic lymphomas are usually of T-cell type and present with a mediastinal mass, sometimes asociated with upper body lymphadenopathy. However PTCL, mostly occurring in adults, shows considerable morphological and immunophenotypic heterogeneity [9]. Peripheral T-cell lymphoma, generally presented with extranodal skin manifestations represents approximately 20% of NHLs in children [10]. In an article from the UK Children's Cancer Study Group involvement of the skin and soft tissue was reported in only 5 % of the T-lymphoblastic lymphomas. In literature individual case reports can be seen describing patients with NHL in unusual localizations [11]. This is the first case of PTCL reported in childhood involving glabellar region.

In our patient the starting hyperemic lesion on the glabella was confused with cellulitis and continuing enlargement despite antibiotherapy obligated radiologic investigation. Magnetic resonance imaging of the patient revealed findings compliant with a neoplasm and biopsy of the involved tissue showed mature T-cells in sampling area. As reported in another case with PTCL, differential diagnosis of cutaneous peripheral T-cell lymphoma should include cellulitis and abscess [6].

Immunohistochemistry showed T-cell surface marker positivity initially. In the last relapse however B cell surface markers were found positive. In the present case we could not explain whether the patient had the bilineage malignancy from the beginning or a secondary B cell malignancy occured.

Young children with PTCL have been reported to have high-stage disease and shorter relapse free survival (RFS). Bekkenk et al. [12] reported disappointing results even with bone marrow transplantation and intensive regimens in patients with PTCL unspecified. In a series of 22 children with PTCL, RFS was found significantly lower in patients under the age of 12 years [8]. In adults also PTCL has been reported to have poor prognosis especially with high-stage disease [9]. In a study of prognostic factors in a group of 82 patients age older than 70 years at diagnosis has been found adversely related with survival [12]. Young children were found to have a higher rate of expression of CD30 than those from adults [8]. But the reason for worse prognosis is still obscure. Although intensive chemotherapy regimens have been tried the patient developed recurrent relapses and died of neutropenic sepsis.

Varying and unpredictable clinical course of PTCL in childhood obligates close follow-up of such patients and investigation of new therapies.

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References 

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PII: S1871-4048(07)00028-7

doi:10.1016/j.pedex.2007.04.002

International Journal of Pediatric Otorhinolaryngology Extra
Volume 2, Issue 3 , Pages 144-146, September 2007

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