A Study of 4 S Neuroblastomas.

Elanthirayan, P (2006) A Study of 4 S Neuroblastomas. Masters thesis, Madras Medical College, Chennai.

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Abstract

INTRODUCTION : Neuroblastoma is one of the commonest solid tumor in the paediatric age group with an overall poor prognosis. Stage 4S Neuroblastomas as the S stands for is a special group among neuroblastomas. Interesting to note that age is a major criteria which differentiates between two groups of patients at either end of the prognostic spectrum.The neuroblastomas presenting after 1yr with all other features of 4 S seem to behave so,different from the actual 4S Neuroblastomas. These 4S NBs have many unique characteristics. Most important of them being tumor regression and tumor maturation. Another interesting fact about these tumors is that it defies the conventional definition of metastatic disease.Because in 4 S NB’s despite their widespread metastasis prognosis is considered as good and the chances that such tumors will regress is very good. There have also been disputing claims that these are incidentalomas and cannot be classified as tumors However there are enough studies to prove that these are true tumors very similar to their non 4 S counterparts and are capable of metastazing and even sometimes progressing rapidly ,with poor outcome. Though most of these tumors regress and disappear some progress on to stage 4 with resultant poor prognosis.There are few pointers to predict tumor progression and regression.This phenomenon is much less studied and understood.This study aims to evaluate the natural course of the disease and to identify the changing patterns of disease regression and maturation. The present scenario of unpredictable disease pattern and behavior gives rise to the question of whether to treat these individuals or not. Chemotherapy with its attendant complications in this age group is very risky.On the other hand, to wait and watch a tumor which has metastasized is a difficult decision.There have been various studies supporting and opposing chemotherapy in 4 S Neuroblastomas in the literature.This study aims to formulate a protocol for identifying between the cases to be treated from those which need to be just observed and followed up.In our setup,where molecular studies and genetic markers are not easily available,we aim to formulate a simplified selection criteria,based on the available resources to identify the patients requiring chemotherapy. AIM : 1. To study the natural course of disease and its regression pattern in 4 S Neuroblastomas. 2. To study the role of Chemotherapy in 4S neuroblastomas. MATERIALS AND METHODS : This is a combined prospective and retrospective study done at Department of Paediatric Surgey,ICH, spanning a four year period from 1st June 2001 to 30th May 2005.All case records of the 52 patients with neuroblastoma ,who presented within this period were taken up for he study.In this, we had ten cases of stage 4S Neuroblastoma .The youngest age of presentation was 2 months.The oldest patient was 11 months. Sex distribution was equal,comprising gof five male and five female.The mode of presentation varied widely,commonest being an abdominal mass. CONCLUSION : This study of 4S Neuroblastomas in our institution over past 4 years establishes the following things. In our study all cases (100%) have shown either tumor maturation or tumor regression. The subgroup of < 3 months have done remarkably well in our study unlike other studies. The secondary effects which are the causes of morbidity and mortality in these cases have not been observed in our study group. Most importantly, a set disease pattern has been identified in the 4 S Neuroblastomas. Though the duration of disease regression varied with an average period of 6.8 months.All these cases followed a set pattern with three phases. Phase I of regression of secondary disease. Phase II of slow tumor regression and phase III of rapid tumor regression. With regard to the role of chemotherapy in these cases, our study concludes there is no role for chemotherapy in these cases as a routine. Chemotherapy does not seem to alter the duration of disease neither does it initiate the process of maturation or tumor regression. In fact the duration of disease regression has been slightly higher in the chemotherapy group. Based on all these we formulate the following indications for chemotherapy in 4SNB. Age > 6 months as presentation with 1) Disease progression in form of new lesions / enlarging masses. 2) Failure of disease regression identified by the non progression from Phase I to Phase II. This six month age cut off has been proposed based on studies proving increased chances of disease progression,in cases presenting beyond this age. Apart from these, any secondary effects of disease, which is life threatening ( Eg, rapidly enlarging liver ),warrants chemotherapy,in any age group.

Item Type: Thesis (Masters)
Uncontrolled Keywords: 4 S Neuroblastomas.
Subjects: MEDICAL > Paediatric Surgery
Depositing User: Kambaraman B
Date Deposited: 28 Jul 2017 02:56
Last Modified: 28 Jul 2017 02:56
URI: http://repository-tnmgrmu.ac.in/id/eprint/2281

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