Dr Ira Shah,
MD, DCH (Gold Medalist), FCPS, DNB

Dr. Vishal

A 3 ½ years old female child born of non consanguineous marriage presented with intermittent fever since 5 months and pain in right thigh associated with limping since last 3 months requiring Thomas Ring splinting with traction twice. She also had lump on the right parietal & occipital area since last 7 days.

5 months back, in view of unexplained fever, positive Mantoux test & ESR of 130 mm at end on 1 hour she was started on 4 drugs Antituberculous therapy (ATT). She had a family history positive for Koch's as father had received ATT for 9 months three years back.

On examination, she was febrile and had pallor, bilateral cervical and occipital lymphadenopathy. There were restriction of movements on the right hip joint but there was no evidence of inflammation Spine examination was normal. On systemic examination, she had hepatosplenomegaly. Thus, a differential diagnosis of Koch's hip or malignancy was considered.

Her investigations revealed anemia (Hb-6.8 gm %) with normal WBC and platelet count. (White cell count = 8700 cells/cumm, polymorphs = 60%, lymphocytes = 24%, monocytes = 14% and eosinophils = 2%, platelet count = 3,13,000 cells/cumm, Reticulocytes = 4.1%). Her ESR was elevated (114 mm at end of 1 hour). She had elevated serum LDH and serum uric acid levels. (S.LDH = 1004 mg/dl and S. uric acid = 4.3 mg/dl). A skeletal survey revealed multiple lytic areas in femur & ischium, vertebrae & skull suggestive of multiple metastasis of round cell tumor. Ultrasound of abdomen & Hip showed hepatosplenomegaly with multiple lymphadenopathy displacing portal vein & Inferior vena cava. There was mild right Hip joint effusion with no internal echoes. A lymph node biopsy was done that showed metastasis of malignant round cell tumor most likely Neuroblastoma or rhabdomyosarcoma. Immunohistochemistry revealed Neuronale or neuroendocrine differentiation of tumor cells expressing chromogrenin strongly & negative for desmin, myoglobin, myo D-1, synapsophysin & S-100 thus confirming the diagnosis of Neuroblastoma. A bone marrow examination was done that showed malignant cells on aspirate and biopsy showed fibrosis with small round crushed cells favoring a diagnosis of Neuroblastoma. A repeat ultrasound of the abdomen showed large mass above the upper pole of Left kidney, displacing it downwards with specks of calcification confirming the tumor to be neuroblastoma. Her 24 hours urinary VMA was elevated [36.5 ng (Normal = 1.9-9.8)]. Patient was started on chemotherapy and is on regular follow-up.

Neuroblastoma is one of the most common cancers affecting children today, forming 8%-10% of the total seen from birth through age 14 years. Neuroblastoma occurs with slightly more frequency in males than in females. The median age at diagnosis is 2 years. It develops from the tissues that form the sympathetic nervous system. Neuroblastoma typically begins in the abdominal area either in the adrenal gland (located just above the kidney) or around the spinal cord in the neck, chest, or pelvis. Although neuroblastoma often is present at birth, it generally is not detected until the tumor begins to grow and compress the surrounding organs. Cancer cells can metastasize (spread) quickly to other areas of the body, such as lymph nodes, liver, lungs, bones, the central nervous system and bone marrow. Close to 70 percent of children diagnosed with neuroblastoma will have metastatic disease. It has a wide range of "virulence"; children with localized neuroblastoma can be cured by surgery alone but those with bone metastases usually have a fatal outcome (CANCER, 59:1853-1859, 1987).

The most common symptoms include the following:

1. An abdominal mass, either felt during an examination or seen as a swollen abdomen .


2. Uncontrolled eye movement caused by the tumor


3. Swelling and bruising of the area around the eyes, caused by metastases (tumor spread)


4. Compression of kidney or bladder by the tumor may cause changes in urination


5. Pain, limping or weakness may be present from bone involvement


6. Anemia or bruising may be present if there is bone marrow involvement


7. Paralysis and weakness may be present if there is spinal cord involvement


8. Diarrhea caused by a substance produced by the tumor (vasoactive intestinal peptide or VIP) may be present


9. Fever

Treatment includes a wide range of approaches. Depending on individual circumstances, these approaches are used either alone or in combination: