A 1.5 year old female is brought to the clinic with complaints of excessive enlargement of head, intolerance to feeds and severe malnourishment. MRI imaging was suggestive of a medulloblastoma causing obstructive hydrocephalus. Which of the following is an example of irrational management of the patient?
Ans. d. Radiotherapy 35-40 Gy was given to the whole craniospinal axis (Ref: Harrison 19/e p602, 18/e p3388; Sabiston 19/e p1888; Schwartz 9/e p1540: healthprofessionals/page5)In this child of age 1.5 years suffering from medulloblastoma, efforts should be made to omit or delay the radiation, given the profound impact of radiation at this age, so the example of irrational management should be radiotherapy 35-40 Gray given to the whole craniospinal axis.Medulloblastoma:Surgery is considered a standard part of treatment for histologic confirmation of tumor type and as a means to improve outcome.Total or near-total resections are considered optimal, if they can be performed safely.For children younger than 3 years, efforts should be made to omit or delay the radiation, given the profound impact of radiation at this age.BCNU and vincristine are primarily used for recurrences, in poor-risk patients, and in children < 3 years to avoid radiation therapyQ.Palliative ventriculoperitoneal shunt is done in pediatric patients of medulloblastoma with hydrocephalus (www. ncbi.nlm.nih.gov/m/.../19327082/)MedulloblastomaHighly malignant tumor found in cerebellumQ and infratentorial locationOccur predominantly in childrenQ (peak incidence at 3-4 yearsQ)Medulloblastoma is most radiosensitive brain tumorQMC site: Vermis (75%)MC site in adults: Lateral cerebellar hemisphereClinical Characteristics:Child usually presents with features of increased intracranial tensionQ.Adults present with ataxia and unilateral dysmetria as lateral origin is more commonQ.Metastasis:Dissemination through CSF is common leading to drop metastasisQ.Metastasis outside CNSQ affects bone, lymph node and liver.Tumor dissemination is most important prognostic factorQ.Treatment:Despite of extreme radiosensitivity, it should be surgically excisedQ.Surgical excision should be followed by radiotherapy and chemotherapyQ.BCNU and vincristine are primarily used for recurrences, in poor-risk patients, and in children < 3 years to avoid radiation therapyQ.Prognosis:Patients without a residual tumor and negative CSF seeding have 5-year survival rate > 75%.
Craniotomy and sub-total excision of the tumour. Surgeon leaves the layer of the tumour adherent with colliculus
First ventriculoperitoneal shunt was done
CCNU and vincristine were given as chemotherapy
Radiotherapy 35-40 Gy was given to the whole craniospinal axis
Central Nervous System