Solid Neoplasms of Childhood MCQs

Ans. is 'a' i.e., Rhabdomyosarcoma Rhabdomyosarcoma o The most common soft tissue sarcoma in children is rhabdomyosarcoma. o The common site of involvement are : - o Head & Neck (25%) 2"d most common Extremities Genitourinary (22%) —> 2nd most common Retroperitoneum

Ans. is 'c' i.e., Fragile-X-syndrome o Down's syndrome is the most common genetic cause of mental retardation, and fragile-X is second to Down's. Also know o Most common inherited cause of mental retardation is fragile-X- syndrome (because down's syndrome is congenital cause but not inherited).

Ans. is 'a' i.e., Astrocytoma o The most common cerebellar tumor in adults is metastasis, whereas in children the most common tumor is an astrocytoma.

Dinutuximab is a monoclonal antibody that targets a diasialoganglioside, GD2, which has ubiquitous expression on neuroblastoma cells Incorporation of the mAb into consolidative therapy after autologous stem cell transplant improves the 2-yr event-free survival from 46.5% to 66.5%. Dinutuximab is approved by the U.S. Food and Drug Administration (FDA) as a standard of care for this subset of patients. Reference: Nelson 21 st Edition, Chapter 525

Antimotility agents (loperamide) are contraindicated in children with dysentery and probably have no role in the management of acute watery diarrhea in otherwise healthy children. Similarly, antiemetic agents, such as the phenothiazines, are of little value and are associated with potentially serious side effects (lethargy, dystonia, malignant hyperpyrexia). Incorrect Options: Option B- Ondansetron is safe and a useful adjunct in troublesome vomiting. Option C- Probiotics can be given but are not routinely indicated. Option D- Remember: Ciprofloxacin is the drug of choice in childhood dysentery. Reference: Nelson Textbook of Pediatrics, 20th Edition, Page Number-1873; OP Ghai Essential Pediatrics, 10th Edition, Page Number-314.

Among the given options, Neuroblastoma frequently produces bony metastasis. Bony metastases in Neuroblastoma often occurs to orbital bones, producing proptosis and/or Raccoon Eyes/Panda Eyes. Vertebral spread is also reported. As per Nelson 21 st Edition, Metastatic spread in Neuroblastoma, which is more common in children >1 yr old at diagnosis, occurs through local invasion or distant hematogenous or lymphatic routes. The most common sites of metastasis overall in Neuroblastoma are the regional or distant lymph nodes, long bones and skull, bone marrow, liver, and skin. Kindly note that bone scan is needed in Neuroblastoma as well as Clear cell sarcoma variant of Wilms tumour in children. Reference: Nelson 21 st Edition, Chapter 525; OP Ghai 10 th Edition, Page 637-639

Clues to the diagnosis of Wilms Tumour here. Clue 1- Pediatric malignancy in young children. Wilms tumour occurs in children aged 2-3 years. Clue 2- Metastases to lung, lymph nodes and liver is seen in Wilms tumour Clue 3- Blind biopsy is avoided since it can upstage the disease, as per Nelson 21 st Imaging studies include plain abdominal radiography, abdominal ultrasonography (US), and CT of the abdomen to define the intrarenal origin of the mass and differentiate it from adrenal masses (e.g., neuroblastoma) and other masses in the abdomen Clue 4- Responds to chemotherapy with Vincristine, Actinomycin-D and Doxorubicin- Seen in Wilms Tumour. After radical nephrectomy, most patients receive chemotherapy using the said agents. Reference: Nelson 21 st Edition, Chapter 526; OP Ghai 10 th Edition, Pages 636-638

Most brain tumours in children are infratentorial, hence option A is correct. About other options Brain tumours are relatively frequent in childhood among malignancies and comprise most common solid malignancy in childhood. Signs of raised ICP can develop commonly with midline brain tumours like medulloblastoma Seizures may or may not be present in all cases Reference: OP Ghai 10 th Edition, Pages 642-643