International Journal of Pediatric Otorhinolaryngology Extra
Volume 6, Issue 3 , Pages 146-148, September 2011

Neuroblastoma and life-threatening tracheal obstruction: A case report and literature review

  • Claire Kinsella

      Affiliations

    • Department of Haematology/Oncology, Our Lady's Children's Hospital, Crumlin, Dublin 12, Ireland
    • Corresponding Author InformationCorresponding author at: 23 Tyrconnell Place, Tyrconnell Road, Inchicore, Dublin 8, Ireland. Tel.: +353 86 1962384.
    • ,
  • John Russell

      Affiliations

    • Department of Otorhinolaryngology, Our Lady's Children's Hospital, Crumlin, Dublin 12, Ireland
    • ,
  • Dave Mannion

      Affiliations

    • Department of Anaesthesiology, Our Lady's Children's Hospital, Crumlin, Dublin 12, Ireland
    • ,
  • Michael Capra

      Affiliations

    • Department of Haematology/Oncology, Our Lady's Children's Hospital, Crumlin, Dublin 12, Ireland

Received 22 April 2010; received in revised form 31 May 2010; accepted 1 June 2010. published online 08 July 2010.

Article Outline

Abstract 

Thoracic neuroblastoma is a rare cause of life-threatening tracheal compression in paediatric oncology. We describe the case of a 5-month old boy with a 2-week history of progressive stridor, a right supraclavicular mass, a paravertebral/mediastinal mass on imaging and elevated urinary catecholamines. In view of the patient's threatened airway, a multidisciplinary management plan was agreed upon prior to obtaining a biopsy to confirm the diagnosis of neuroblastoma. Preoperative microlaryngobronchoscopy (MLB) demonstrated critical tracheal compression necessitating intubation. Neuroblastoma was confirmed on histology. Urgent cytoreductive treatment was initiated with excellent response allowing extubation 14 days later.

Keywords: Neuroblastoma, Tracheal obstruction

 

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1. Introduction 

Airway compression by a mediastinal mass is a medical emergency. Despite the improvement in prognosis for children with cancer over the last few decades, mortality from tumour-related life-threatening complications or treatment thereof continues to be an entity. Such complications include: infection, metabolic abnormalities, haematological deficiencies and mechanical/space-occupying-related problems.

Masses in the anterior mediastinum, as compared to the middle or posterior mediastinum have a greater propensity for causing airway compression. It would be uncommon for masses originating in the posterior mediastinum to cause significant airway compression. We report a case of significant tracheal compression as a result of thoracic neuroblastoma in a young infant.

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2. Case report 

A 5-month old boy with mild stridor since birth presented to his general practitioner (GP) with progression of the stridor and reluctance to feed over the preceding 2 weeks. Following an ineffective 3-day course of oral steroids he was referred to the local paediatric service where a chest X-ray demonstrated a paravertebral mediastinal mass with CT chest revealing a 5cm×3cm mass extending from the apex of the right thorax into the supraclavicular region, causing left tracheal deviation and compressing and distorting the innominate vein (Fig. 1). He was immediately referred to a tertiary paediatric centre for further management.

Examination revealed a well-looking infant but with significant biphasic stridor at rest. No tracheal tug or subcostal recession was evident. A firm 1–2cm ill defined mass in the right supraclavicular area was palpable. No signs of superior vena cava obstruction were evident. Vital signs, including oxygen saturation, were normal as was the remainder of the examination, abdominal ultrasound, full blood count and relevant biochemistry. However, urinary catecholamines were elevated (HMMA=47.3mmol/mol, normal range <14).

With a presumptive clinical diagnosis of neuroblastoma, and in view of the patient's threatened airway, a multidisciplinary (radiology, anaesthesiology, oncology, otorhinolaryngology, surgery, and pathology) management plan was agreed upon prior to obtaining a biopsy to confirm the diagnosis. Under elective conditions in the operating room, he underwent a microlaryngobronchoscopy (MLB), demonstrating critical distal tracheal extrinsic compression (see Fig. 2), necessitating immediate intubation with a size 3.5 endotracheal tube (ETT), with the distal tip present at the carina.

  • View full-size image.
  • Fig. 2. 

    Microlaryngobronchoscopy images at presentation (a) demonstrating critical distal tracheal compression with resolution thereof 2 weeks later following chemotherapy (b).

Through a midline neck incision a firm, lobulated, blue-coloured supraclavicular mass was visible and found to be encasing the internal jugular and subclavian vessels. Immediate frozen section confirmed the presence of malignancy with small-undifferentiated round blue cells. In view of the threatened airway and reliance on ETT to maintain airway patency, urgent cytoreductive treatment was required to decompress the critical tracheal compression. Chemotherapy was likely to be an effective, and a safer alternative to attempted surgical resection or radiotherapy. A femoral central venous line was inserted and bilateral bone marrow aspirates/biopsies were performed before the patient was transferred, still intubated to the paediatric intensive care unit (PICU). Chemotherapy, utilizing vincristine, adriamycin and cyclophosphamide was immediately commenced. Subsequent MIBG, and normal bone marrow investigations, confirmed the presence of localized disease only and MYCN was not amplified, therefore stratifying the patient into a favourable category.

His course in PICU was eventful: fluid overload, electrolyte abnormalities, febrile neutropenia, and coagulase negative staphylococcus infection ensued. He remained intubated for 14 days at which stage, following radiological evidence of tumour shrinkage with less tracheal deviation (see Fig. 2a), he was electively taken back to the operating room for possible elective extubation. This was successful with repeat MLB demonstrating tracheal patency (see Fig. 2).

He received five further cycles of chemotherapy with continued tumour shrinkage confirmed on imaging. A persistent residual small tumour in the right thoracic apex was resected. Histology revealed necrotic tumour only. Surgical complications included a right Horner's syndrome and a well compensated right partial vocal cord palsy, questioning the need of surgery in such a chemotherapy-sensitive tumour. He remains well and tumour-free 10 months following his presentation.

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3. Discussion 

Neuroblastoma, the most common extracranial malignancy in the first year of life [1], is of neural crest origin and therefore arises from any site along the sympathetic nervous system chain. Important prognostic variables include: stage, age and MYCN status. The higher the stage, with the exception of stage IV, the less favourable the prognosis [2]. Infants less than 1 year, possibly even up to 18 months, have a more favourable prognosis than older patients [3]. MYCN amplification is associated with a poor prognosis [4]. The abdomen is the primary site in over 65% of patients however in infants thoracic and cervical primary tumours are more common than in older children [5]. Thoracic, as compared to abdominal neuroblastoma has a more favourable prognosis [6].

Airway obstruction secondary to neuroblastoma is a rare entity with retropharyngeal, cervical and mediastinal-sited neuroblastoma been reported. To our knowledge, retropharyngeal and cervical neuroblastoma has been reported in four and eleven cases respectively to date [7], [8], [9], [10], [11], [12]. In one case the latter presented prenatally [10]. Mediastinal neuroblastoma arises almost exclusively within the posterior mediastinum, although there is an isolated case of neuroblastoma arising within the anterior mediastinum [13]. In contrast to anterior mediastinum-sited lesions, posterior masses generally do not cause airway obstruction. They generally present as an incidental finding or in some cases with discomfort or pain.

Our patient developed life-threatening airway obstruction as a result of the posterior mediastinal neuroblastoma extending anteriorly to compress his distal trachea.

Initial management of a patient with a tumour causing life-threatening airway compression involves immediate measures to protect airway patency. Once the airway has been secure urgent, appropriate and effective oncology-directed treatment to relieve the mechanical extrinsic compression of the airway is required. The following factors need to be considered in deciding on the most applicable treatment modality to use: tumour prognosis, sensitivity of tumour to treatment modality, treatment response time, available resources and predicted potential acute and chronic treatment-related morbidity. In our patient the tumour-related prognosis of his neuroblastoma was favourable relative to the site, stage, histological/biological features and age of patient. This together with the fact that neuroblastoma is a chemotherapy-sensitive tumour was the deciding factor for chemotherapy rather than surgical decompression or emergency radiotherapy. The vascular and airway involvement of this tumour negated the possibility of a complete resection, notwithstanding the potential morbidity of attempting resection in this challenging site. Although potentially effective, the long-term morbidity of radiotherapy to this area at such an age detracts from its use. With our radiotherapy facility being off-site, the risk of transporting an intubated infant with a life-threatening compromised airway further negates the use of such treatment.

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4. Summary 

Thoracic neuroblastoma arising in the posterior mediastinum is an extremely rare cause of life-threatening tracheal compression in paediatric oncology. A multidisciplinary approach is essential in managing these patients to secure airway patency and immediately thereafter to begin to decompress the extrinsic compression. In this case the chemotherapy was successful with extubation been possible within 14 days after chemotherapy administration.

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References 

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PII: S1871-4048(10)00046-8

doi:10.1016/j.pedex.2010.06.001

International Journal of Pediatric Otorhinolaryngology Extra
Volume 6, Issue 3 , Pages 146-148, September 2011

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