Outcome of children and adolescents with lymphoblastic lymphoma

Dear Editor,Lymphoblastic lymphoma (LL) of the mediastinum is a rare tumour accounting for 2 % of non-Hodgkin lymphomas. It is a diagnostic and therapeutic emergency. Positive diagnosis is based on microscopic examination. They are classified as T LL in 85 to 90 % of the cases. Few survival studies have been reported, and our knowledge about these tumours is based on rare case reports and case studies [1]. Our aim was to assess the most relevant factors influencing the survival of patients presenting mediastinal LL.We conducted a retrospective single-centre study over a 16-year period between 2000 and 2015 including all patients presenting mediastinal LL. Patients with other mediastinal lesions were ruled out. Two age groups were identified: the pediatric age lower than 15 years and the adult age superior to 15 years. The tumours were subdivided into localized tumours including stages I and II according to the Ann Arbor classification of lymphoma and disseminated tumours including stages III and IVaccording to the same classification. The nature of the specimen, the gross exam, the microscopic features and the crowding artefacts were recorded. The immunohistochemical study was performed in all cases using a manual method performed by the same technician. The different antibodies included CD3, CD5, CD99, CD1a, CD45, Tdt, Ki-67, CD20, CK, EMA, CD79a, CD15 and CD30 antibodies. A multivariate analysis was performed to compare the sex, the gender and the age in relation to the global survival of the patients using cox model. The SPSS software (version 15.0) was used.Thirty-one T cell LL of the mediastinum were diagnosed. The mean age of the patients was 26 years. A male predominance was noticed with 19 men and 12 women. The global survival was estimated to 85 % at 8 months and 70 % at 12 months with a mean survival of 24 months. The multivariate analysis revealed that the sex was a relevant prognostic factor (p = 0.045). Table 1 illustrates the different results related to the odds ratio, the confidence interval and the p values.This study highlights the bad prognosis of these tumours with a global survival of 24 months versus 43 months in the literature. Many prognostic factors have been reported in the literature [2,3]. The young age is considered as a prognostic factor [4,5]. This was not proved in our study (p = 0.46). The female sex is considered as a prognostic factor either in our study or in the literature review (p = 0.045) [6]. In opposition to the literature, the stage does not seem to play a key role (p = 0.88) [7].Other prognostic factors have been reported in the literature including mir17, mir 19, MYC, bcl2, bcl6 and the c-myc without a real consensus [8,9].Our survival study revealed the prognostic relevance of the gender in LL. The prognostic impact of the subtype of the lymphoma was not highlighted because all the cases diagnosed were of T cell subtype. This fact is concordant to the literature review because the majority of mediastinal LL are of T cell subtype.

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“…Many prognostic factors have been reported in the literature [2,3]. The young age is considered as a prognostic factor [4,5].…”

Section: Dear Editormentioning

confidence: 99%

Multivariate analysis of prognostic factors of lymphoblastic lymphomas of the mediastinum

Mlika

Khanfir

Mansouri³

et al. 2016

Ann Hematol

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“…LBL is the second most common subtype of non-Hodgkin lymphoma in children. 13 Of note, only approximately 10% of LBL express B-cell markers in contrast to approximately 85% of acute lymphoblastic leukemia (ALL). 14 The distinction between leukemia and lymphoma is arbitrary and based on the extent of involvement of bone marrow (BM).…”

Section: Introductionmentioning

confidence: 99%

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2022

JPNC

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Lymphomas involving the mediastinum occur in a wide age range and represent heterogeneous histological subtypes with various clinical symptoms and complex radiological findings. However, this cross sectional study that describes the clinical, pathological and radiological features of Bangladeshi pediatric patients aged less than 18 years. The study conducted in National Institute of Cancer Research and Hospital with 71 primary pediatric mediastinal masses, diagnosed between 2014 and 2018 and evaluated at enrollment or admission in the department of Pediatric Hematology and Oncology (PHO). The presence of extra thoracic lymph node involvement was excluded from the study. All patients were underwent computerized tomography (CT) or ultra sound (USG) guided needle biopsy. Only diagnosed cases of lymphoma (N-38) with mediastinal mass on chest radiography or CT scan were taken for analysis.Mediastinal lymphoma was 38 in number which, 16.30% of total (N-233) pediatric lymphoma of PHO. The median age of the patients was 11.43 years with mostly (68.42%) in 10-17 years age group. Males and females were equal in number. Common symptoms of the patients were fever, cough, dyspnea, chest pain. Most common side involved by tumors were anterior mediastinum 68.42% (N-26) followed by middle mediastinum 50% (N-19), Posterior mediastinum 10.53% (N-4) and superior mediastinum 7.9% (N-3). More than one mediastinal anatomical side involvement was in 34.21% (N-13) cases. Tissue biopsy revealed non Hodgkin Lymphoma were 86.84% (N-33), Hodgkin Diseases 10.53% (N-4) and T-lymphoblastic lymphoma (LBL) 2.63% (N-1). All tumors were malignant with 73.68% metastasis.Pediatric mediastinal mass is a troublesome condition for doctors during emergency management at presentation, for interventional radiologists during tissue biopsy, pathologists during histopathological examination and oncologists during treatment planning. A few publications narrated the pediatric mediastinal oncological conditions but in Bangladesh no such study has conducted before addressing the pediatric mediastinal lymphoma. So we conducted this study to show the importance of development of infrastructure to manage these type of tumors successfully.

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Pediatric primary mediastinal lymphoma – a descriptive study of a single cancer center of Bangladesh

Ghosh¹,

Al-Amin²,

Fan³

2022

JPNC

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Lymphomas involving the mediastinum occur in a wide age range and represent heterogeneous histological subtypes with various clinical symptoms and complex radiological findings. However, this cross sectional study that describes the clinical, pathological and radiological features of Bangladeshi pediatric patients aged less than 18 years. The study conducted in National Institute of Cancer Research and Hospital with 71 primary pediatric mediastinal masses, diagnosed between 2014 and 2018 and evaluated at enrollment or admission in the department of Pediatric Hematology and Oncology (PHO). The presence of extra thoracic lymph node involvement was excluded from the study. All patients were underwent computerized tomography (CT) or ultra sound (USG) guided needle biopsy. Only diagnosed cases of lymphoma (N-38) with mediastinal mass on chest radiography or CT scan were taken for analysis. Mediastinal lymphoma was 38 in number which, 16.30% of total (N-233) pediatric lymphoma of PHO. The median age of the patients was 11.43 years with mostly (68.42%) in 10-17 years age group. Males and females were equal in number. Common symptoms of the patients were fever, cough, dyspnea, chest pain. Most common side involved by tumors were anterior mediastinum 68.42% (N-26) followed by middle mediastinum 50% (N-19), Posterior mediastinum 10.53% (N-4) and superior mediastinum 7.9% (N-3). More than one mediastinal anatomical side involvement was in 34.21% (N-13) cases. Tissue biopsy revealed non Hodgkin Lymphoma were 86.84% (N-33), Hodgkin Diseases 10.53% (N-4) and T-lymphoblastic lymphoma (LBL) 2.63% (N-1). All tumors were malignant with 73.68% metastasis. Pediatric mediastinal mass is a troublesome condition for doctors during emergency management at presentation, for interventional radiologists during tissue biopsy, pathologists during histopathological examination and oncologists during treatment planning. A few publications narrated the pediatric mediastinal oncological conditions but in Bangladesh no such study has conducted before addressing the pediatric mediastinal lymphoma. So we conducted this study to show the importance of development of infrastructure to manage these type of tumors successfully.

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Outcome of children and adolescents with lymphoblastic lymphoma

Cited by 4 publications

References 26 publications

Multivariate analysis of prognostic factors of lymphoblastic lymphomas of the mediastinum

Multivariate analysis of prognostic factors of lymphoblastic lymphomas of the mediastinum

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Pediatric primary mediastinal lymphoma – a descriptive study of a single cancer center of Bangladesh

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