Clinical Predictors and Prognostic Model for Pediatric Lymphoblastic Lymphoma Treated With Uniform BFM90 Protocol: A Single-Center Experience of 65 Patients From Asia

Patel, Amol; Tiwari, Akash; Biswas, Bivas; Sharma, Meher Chand; Sreenivas, Vishnubhatla; Bakhshi, Sameer

doi:10.1016/j.clml.2019.01.008

Cited by 8 publications

(4 citation statements)

References 17 publications

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“…The lack of association between the diagnostic interval and outcomes in bone sarcomas is compatible with that reported in prior studies from the Western world 8,9 . Better survival outcomes with a longer diagnostic interval have also been reported in certain other paediatric tumours—central nervous system (CNS) tumours and in lymphoblastic lymphoma 10,30 . On the other hand, delayed surgery following neoadjuvant chemotherapy and delay in the completion of the chemotherapy course in bone sarcomas have been reported to result in poorer treatment outcomes, with more local recurrences in the former situation and worse survival in the latter 8,31 .…”

Section: Discussionsupporting

confidence: 82%

“…8,9 Better survival outcomes with a longer diagnostic interval have also been reported in certain other paediatric tumours-central nervous system (CNS) tumours and in lymphoblastic lymphoma. 10,30 On the other hand, delayed surgery following neoadjuvant chemotherapy and delay in the completion of the chemotherapy course in bone sarcomas have been reported to result in poorer treatment outcomes, with more local recurrences in the former situation and worse survival in the latter. 8,31 The aforementioned delays are frequent in developing countries on account of financial and infrastructural limitations.…”

Section: Discussionmentioning

confidence: 99%

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Determinants and impact of diagnostic interval in bone sarcomas: A retrospective cohort study

Sasi

Ganguly

Biswas

et al. 2022

Pediatric Blood & Cancer

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Background: Diagnostic delays in cancers are frequent in developing countries due to poor health infrastructure. Existing literature from developed countries suggests that diagnostic interval in bone sarcomas is primarily dictated by tumour biology with no impact on survival. This study evaluates the social and biological determinants of the diagnostic interval in bone sarcomas in a resource-challenged setting and assesses its impact on treatment outcomes. Methods:A retrospective single-institutional study was conducted on patients with high-grade bone sarcomas recorded in the sarcoma clinic database between 2003 and 2018. Baseline clinical characteristics and treatment outcomes were recorded. Logistic regression was performed to assess the impact of baseline clinical and social characteristics (distance from treating centre and rural vs. urban residence) on the diagnostic interval. Further, the impact of diagnostic interval on histologic response to neoadjuvant chemotherapy, amputation requirement in extremity sarcomas and survival was evaluated.Results: A total of 1227 patients were included for analysis. The median diagnostic interval was 4 months (3-7 months). Age above 18 years, Ewing sarcoma (ES) diagnosis, absence of fever at presentation and tumour size above 7.5 cm were predictors of a longer diagnostic interval (>4 months). The length of the diagnostic interval did not impact amputation requirement or survival outcomes. However, the proportion of patients with good necrosis post-neoadjuvant chemotherapy was lower among patients with longer diagnostic intervals (25% vs. 34⋅16%; p-value = .04). Conclusion:Tumour characteristics rather than social factors determined the diagnostic interval. Diagnostic interval did not impact survival outcomes even in a resource-constrained setting.

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Section: Discussionsupporting

confidence: 82%

Section: Discussionmentioning

confidence: 99%

Determinants and impact of diagnostic interval in bone sarcomas: A retrospective cohort study

Sasi

Ganguly

Biswas

et al. 2022

Pediatric Blood & Cancer

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“…Poor prognostic factors include adult, female patients presenting with high LDH levels, CNS involvement, and refractory cases. The clinical factors which are responsible for poor outcome include superior mediastinal syndrome (SMS), pleural infusions at initial presentation, SMS with baseline ECOG PS >2, high leucocyte count, low serum albumin, and duration of symptoms ( 23 , 24 ). The genetic abnormalities which are associated with good prognosis include chromosomal rearrangements involving TAL1 [t(1:14)], t(1:17), TALX1, HOXA (CALM-AF10), deletion CDKN2A/2B, mutation in NOTCH1 and those associated with poor prognosis include rearrangements of TLX3 and mutation involving EZH2 ( 27 ).…”

Section: Differential Diagnosismentioning

confidence: 99%

Pediatric mediastinal lymphoma

Mallick¹,

Jain²,

Ramteke³

2020

Mediastinum

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The mediastinum is the visceral compartment of thoracic cavity divided into the superior and inferior mediastinum, further inferior compartmentalize into anterior, middle, and posterior mediastinum. Lymphoma in the mediastinum may be primary or secondary to systemic disease. Lymphoma may arise from lymphoid organs-like thymus, mediastinal lymph nodes or other mediastinal organs like heart, lung, pleura, and pericardium. It comprises about 12% of all the mediastinal tumors in adults however, it constitutes 50% of the pediatric mediastinal mass. Anatomically lymphoma most commonly involves anterior mediastinum. Among the pediatric mediastinal lymphomas, lymphoblastic lymphoma (LBL) predominate followed by Hodgkin lymphoma (HL), primary mediastinal large B cell lymphoma (PMBCL) and very rarely Grey zone lymphoma. Other types of non-HLs (NHLs) are rare among pediatric population. Radiologically and clinically present as an anterior mediastinal mass with symptoms of dyspnea, cough, and superior vena cava syndrome. Also, clinically and radiologically all the pediatric mediastinal mass shares the overlapping features, hence, for treatment and prognostic points of view its essential to differentiate the three entities, i.e., LBL, HL and PMBCL. The pathological diagnosis of pediatric mediastinal lymphomas is quite challenging for general histopathologists. In this review, we describe the pathology, genetics, differential diagnosis, treatment, prognosis, and a simplified histopathological and immunophenotypical approach to differentiate the pediatric mediastinal lymphomas.

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“…To bridge this gap, a retrospective analysis of 65 consecutive patients with pediatric LBL over 15 years, treated with BFM90 protocol, was carried out and the outcomes were assessed in terms of overall survival (OS) and event-free survival (EFS). [1] At the end of induction, 57% of patients had a complete remission, 28% had a partial remission, 6% had a progressive disease, and 3% had a stable disease. Treatment-related mortality occurred in three patients overall and 20 patients relapsed.…”

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confidence: 95%

Commentary on Clinical Predictors and Prognostic Model for Pediatric Lymphoblastic Lymphoma Treated with Uniform BFM90 Protocol: A Single-Center Experience of 65 Patients from Asia

Chitikela

Pushpam

2020

Indian Journal of Medical and Paediatric Oncology

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Clinical Predictors and Prognostic Model for Pediatric Lymphoblastic Lymphoma Treated With Uniform BFM90 Protocol: A Single-Center Experience of 65 Patients From Asia

Cited by 8 publications

References 17 publications

Determinants and impact of diagnostic interval in bone sarcomas: A retrospective cohort study

Determinants and impact of diagnostic interval in bone sarcomas: A retrospective cohort study

Pediatric mediastinal lymphoma

Commentary on Clinical Predictors and Prognostic Model for Pediatric Lymphoblastic Lymphoma Treated with Uniform BFM90 Protocol: A Single-Center Experience of 65 Patients from Asia

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