LETTER TO EDITOR
|Year : 2007 | Volume
| Issue : 3 | Page : 163-164
Neuroblastoma presenting as stridor in a neonate
Utkarsh Kohli1, Pawan Bhutani1, Rakesh Lodha1, Sandeep Agarwala2
1 Department of Pediatrics, All India Institute of Medical Sciences, Ansari Nagar, New Delhi - 110 029, India
2 Department of Pediatric Surgery, All India Institute of Medical Sciences, Ansari Nagar, New Delhi - 110 029, India
Department of Pediatrics, All India Institute of Medical Sciences, Ansari Nagar, New Delhi - 110 029
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Kohli U, Bhutani P, Lodha R, Agarwala S. Neuroblastoma presenting as stridor in a neonate. J Indian Assoc Pediatr Surg 2007;12:163-4
|How to cite this URL:|
Kohli U, Bhutani P, Lodha R, Agarwala S. Neuroblastoma presenting as stridor in a neonate. J Indian Assoc Pediatr Surg [serial online] 2007 [cited 2019 Sep 20];12:163-4. Available from: http://www.jiaps.com/text.asp?2007/12/3/163/34964
Stridor in the newborn period is an unusual presentation of neuroblastoma. , We report a two and a half month-old infant presenting with stridor due to extrinsic tracheal compression caused by a cervico-thoracic neuroblastoma.
This baby presented to us with complaints of noisy breathing and respiratory distress since day 15 of life and fever for two days. This child, term-born and the older of twin females, was apparently well till day 15 of life, when she was noticed to have noisy breathing. The child's symptoms were intermittent and nonprogressive and improved on prone positioning. There was no history of choking, difficulty in feeding, cyanosis, fever, recurrent vomiting, facial plethora and altered sensorium.
The child had earlier received steroids and antimicrobials for 20 days without a favorable response. There was no history of maternal hyperparathyroidism or phenytoin, phenobarbitone or diuretic intake during pregnancy. On examination, the child was found to have tachycardia and tachypnea with subcostal and intercostal retractions. Peripheries were warm and arterial blood oxygen saturation (SaO 2 ) on supplemental oxygen was 96%. There was a 2 x 2 cm, firm and nontender cervical mass on the right side. Respiratory examination revealed marked distress with prominent stridor and diffuse bilateral crepititions. No other systemic abnormality could be detected.
On day 3 of admission, the child's respiratory distress worsened and she was intubated and ventilated. A postadmission chest radiograph showed diffuse bilateral infiltrates and mediastinal widening. Contrast-enhanced computerized tomographic imaging of the neck and chest revealed a large mass extending from the neck to the abdomen in the posterior mediastinum and compressing the airway from three sides [Figure - 1],[Figure - 2]. The child was also started on intravenous antimicrobials at the time of admission as she had pneumonia; blood culture at admission was positive for Staphylococcus aureus . Child's serum calcium level and renal function was normal.
Ultrasound-guided fine needle aspiration cytology of the chest mass showed small round cells with scanty cytoplasm, suggestive of a primitive neuroectodermal tumor. Methyliodobenzylguanidine (MIBG) scans 24 and 72 h after contrast injection showed increased tracer concentration in the posterior mediastinum and right supraclavicular node, which was compatible with a neuroblastoma. Urinary vanillylmandelic acid (VMA) levels were normal. We offered chemotherapy to our patient as she had stage 4 disease, but the parents were unwilling for the same.
Neuroblastoma belongs to a group of small round cell tumors of neuroectodermal origin and accounts for 8-10% of pediatric cancers.  Although neuroblastomas can occur anywhere along the symapathetic chain, two thirds of the tumors are found in the abdomen. Infants have a higher incidence of cervical and thoracic tumors.  Neuroblastoma can give rise to diverse clinical manifestations because of variation in location of the primary tumor, distant metastasis by both lymphatic and hematogenous routes and paraneoplastic syndromes.  Thoracic tumors, though well known to cause stridor and other compressive symptoms, are often picked up accidentally on chest radiography performed for other indications. 
MIBG is an analog of norepinephrine that concentrates within secretory granules of catecholamine-producing cells. More than 95% of neuroblastomas secrete homovanillic acid (HVA) or VMA and hence, MIBG specificity for the diagnosis of neuroblastoma approaches 100% while sensitivity is reported to be around 90-95%. 
Treatment of neuroblastoma involves surgery, chemotherapy and radiotherapy, hence good coordination between these specialists is required. Other novel therapies that have been tried include [I 131 ]-MIBG, [I 125 ]-MIBG  and monoclonal antibody-based therapies for metastatic neuroblastomas. 
The present case highlights the importance of considering cervico-thoracic neuroblastoma as a diagnostic possibility in newborns presenting with stridor.
| References|| |
|1.||Tai CF, Lee KS, Chen IH. Primary cervical neuroblastoma in infants. J Formos Med Assoc 1997;96:561-4. [PUBMED] |
|2.||Antony R, Al-Rawas A, Irwin M. Stridor in a newborn. CMAJ 2005;173:601-2. [PUBMED] [FULLTEXT]|
|3.||Shah S, Ravindranath Y. Neuroblastoma. Indian J Pediatr 1998;65:691-705. |
|4.||Ravindranath Y, Cushing B. Neuroblastoma of neck and chest. In : Pochedly C, editor. Neuroblastoma: Clinical and biological manifestations. Elsevier Science Publishing Company Inc: New York, NY; 1982. p. 39-49. |
|5.||Pashankar FD, O'Dorisio MS, Menda Y. MIBG and somatostatin receptor analogs in children: Current concepts on diagnostic and therapeutic use. J Nucl Med 2005;46:55s-61s. [PUBMED] [FULLTEXT]|
|6.||Cheung NV, Kushner BH, Kramer K. Monoclonal antibody based therapy of neuroblastoma. Hematol Oncol Clin North Am 2001;15:853-66. |
[Figure - 1], [Figure - 2]