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Infant with an unusual pharyngeal mass.

The differential diagnosis of an infant with a pharyngeal mass includes congenital masses (i.e., dermoid cysts, lymphangiomas, and teratomas), lymphadenitis, hemangiomas, and soft-tissue malignancies. The most common malignancy in the first year of life is neuroblastoma. (1) These tumors most frequently present suprarenally (2) and rarely in the aerodigestive tract. We report the presentation and management of an infant with an unusual pharyngeal mass diagnosed as a neuroblastoma.

A 19-week-old infant girl presented with noisy breathing of 4 to 6 weeks' duration. The symptoms worsened when the child cried or was agitated. She had been feeding well without episodes of apnea or cyanosis and was demonstrating appropriate weight gain. The patient was delivered at full term with no complications and had no prior hospitalizations.

Flexible fiberoptic laryngoscopy revealed a bulging right lateral pharyngeal wall and a normal epiglottis, supraglottis, and vocal folds. Magnetic resonance imaging (MRI) confirmed a pharyngeal mass from C1 to C7 measuring 1.6 x 5.1 x 6.4 cm with heterogeneous enhancement and a mass effect with displacement of the oropharyngeal airway (figure 1). The MRI also revealed an enlarged level II right cervical lymph node measuring 12.5x8.5x3.0 mm. The differential diagnosis included neuroblastoma, rhabdomyosarcoma and, less likely, neurofibroma.

The infant underwent airway evaluation and an excisional biopsy. Direct laryngoscopy revealed a normal supraglottis, vocal folds, and postcricoid space. A posterior bulge of the right hypopharynx was noted. The rest of the airway examination down to the bronchi was normal. The neck was then explored, subplatysmal flaps raised, and the medial border of the right sternocleidomastoid identified. The mass was noted deep to the sternocleidomastoid and posterior to the internal jugular vein and vagus nerve. It was removed without difficulty (figure 2). Additionally, level II lymph nodes were dissected and removed.

Frozen section of these nodes was consistent with a diagnosis of neuroblastoma. The incision was closed in the standard fashion and a suction drain left in place. The patient had an uneventful recovery, with the drain removed on the fourth postoperative day. A diagnosis of neuroblastoma with metastasis to one of seven resected nodes was confirmed (figure 3).

A complete staging evaluation was then performed by the oncology service. Bilateral bone marrow biopsies and aspirates demonstrated no evidence of neuroblastoma. Cytogenetic analysis of the tumor revealed no amplification of the MYCN oncogene, which occurs in approximately 22% of cases and is associated with a poor prognosis. (3) A radiolabeled I-131 metaiodobenzylguanidine (MIBG) scan revealed no areas of distant metastasis. Similarly, MRI of the neck, chest, abdomen, and pelvis revealed no suspicious lesions. Urine catecholamines secreted by the tumor were elevated at the time of presentation but normalized after tumor excision.

The patient was classified as having a stage 2B neuroblastoma, given the localized tumor with complete gross resection and ipsilateral lymph node involvement. No chemotherapy was indicated. Recommended follow-up was for MRI scans every 3 months and monthly urine catecholamines to evaluate for disease recurrence.

Neuroblastomas have a highly variable presentation, ranging from aggressive metastasis to spontaneous regression. (4) Primary tumors in the neck or upper chest can cause Horner syndrome (ptosis, miosis, anhidrosis) or airway compromise. (4) Other presenting symptoms include abdominal discomfort and distension, bone pain, watery diarrhea, periorbital ecchymoses due to orbital metastases, subcutaneous skin nodules, and opsoclonus-myoclonus syndrome.

Treatment of neuroblastoma depends on risk stratification and staging. Adverse prognostic features include patients >12 months of age, MYCN gene amplification, metastatic spread, and a poorly differentiated tumor with a high mitotic-karyorrhexis index. (5) In low-risk patients such as the one described in this report, surgical excision and observation is the mainstay of therapy.

This case highlights the need for a multidisciplinary approach to the treatment of children with a malignant neck mass. While surgical excision was the mainstay of treatment in this infant, staging of the tumor and long-term follow-up is required by pediatric oncology.

References

(1.) Linet MS, Ries LA, Smith MA, et al. Cancer surveillance series: Recent trends in childhood cancer incidence and mortality in the United States. J Natl Cancer Inst 1999;91(12):1051-8.

(2.) Orbach D, Sarnacki S, Brisse HJ, et al. Neonatal cancer. Lancet Oncol 2013;14(13):e609-20.

(3.) Cohn SL, Pearson AD, London WB, et al. The International Neuroblastoma Risk Group (INRG) classification system: An INRG Task Force report. J Clin Oncol 2009;27(2):289-97.

(4.) Maris JM. Recent advances in neuroblastoma. New Engl J Med 2010;362(23):2202-11.

(5.) Ward E, DeSantis C, Robbins A, et al. Childhood and adolescent cancer statistics, 2014. CA Cancer J Clin 2014;64(2):83-103.

Benjamin B. Shields, MD; Erin E. Lampson, MD; Anita L. Sengupta, MD; Tanya C. Watt, MD; Ron B. Mitchell, MD

From the Department of Otolaryngology (Dr. Shields and Dr. Mitchell), the Division of Pediatric Hematology-Oncology, Children's Medical Center (Dr. Lampson), and the Department of Pathology (Dr. Sengupta), University of Texas Southwestern Medical Center, Dallas.

Caption: Figure 1. Coronal section of T2-weighted MRI reveals a large, hyperintense pharyngeal mass compressing the airway.

Caption: Figure 2. A: A large pharyngeal mass is removed via excisional biopsy. B: Gross sections of the mass show an encapsulated mass with a vaguely nodular cut surface.

Caption: Figure 3. A: Low-power histology of the mass (hematoxylin and eosin stain, original magnification x4) shows a nodular proliferation of small neuroblasts in a background of fibrillary neuropil. B: High-power histology of the mass (hematoxylin and eosin stain, original magnification x.20) shows small neuroblasts in a background of fibrillary neuropil. One maturing ganglion cell with three nuclei is seen in the lower left of the image (arrow).
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Title Annotation:PEDIATRIC OTOLARYNGOLOGY CLINIC
Author:Shields, Benjamin B.; Lampson, Erin E.; Sengupta, Anita L.; Watt, Tanya C.; Mitchell, Ron B.
Publication:Ear, Nose and Throat Journal
Article Type:Case study
Date:Jun 1, 2018
Words:934
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