Clinical Review & Education
Clinical Problem Solving | PATHOLOGY
A Retropharyngeal Mass in a Young Boy Jason Showmaker, MD; Eliav Gov-Ari, MD
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Figure. A, Magnetic resonance image of the neck; B, firm encapsulated mass being dissected from the retropharyngeal space with extensions to the carotid space; C, low-power micrograph of a specimen from oropharyngeal mass showing a Schwannian-like background of eosinophilic, spindle-shaped cells (hematoxylin-eosin, original magnification ×40); D, high-power micrograph showing clusters of well-differentiated ganglion cells with large round nuclei, prominent nucleoli, and granular basophilic Nissle substance (hematoxylin-eosin, original magnification ×100).
A young boy was noted during routine adenotonsillectomy to have a large firm mass in the posteriolateral pharyngeal wall and was referred to a tertiary medical center. Postoperatively, he developed mild velopharyngealinsufficiency.Onflexibleendoscopy,theposteriorpharyngeal mass was clearly visible. Magnetic resonance imaging (MRI) of the neck revealed a 3 × 2 × 1-cm fusiform lesion attached to the medial aspect of the left carotid space, extending to the midline retropharyngeal space at the level of C2 to C4 (Figure, A). The mass showed homogenous enhancement with gadolinium contrast (not shown). The patient was taken to the operating room, and a transoral approach via horizontal incision in the level of orohypopharynx was performed. The firm encapsulated mass was easily identified and dissected free from surrounding tissue (Figure, B). The mass showed a narrow extension to the carotid space.
jamaotolaryngology.com
On gross examination, the mass was firm and encapsulated. Hematoxylin-eosin–stained slides at low power (original magnification ×40) revealed a Schwannian-like background of eosinophilic, spindle-shaped cells (Figure, C). At higher power (original magnification ×100), clusters of well-differentiated ganglion cells with large round nuclei, prominent nucleoli, and granular basophilic Nissle substance (endoplasmic reticulum and ribosomes present in the cytoplasm of neural cells) were seen (Figure, D). Postoperatively, the patient was noted to have unilateral mild drooping of his eyelid, and his left pupil was constricted (yet responded to light) on the side of the resected lesion. Repeated MRI 6 months later showed no recurrence. What is your diagnosis?
JAMA Otolaryngology–Head & Neck Surgery February 2014 Volume 140, Number 2
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archotol.jamanetwork.com/ by a Florida International University Medical Library User on 05/30/2015
173
Clinical Review & Education Clinical Problem Solving
Diagnosis Carotid space ganglioneuroma
Discussion Ganglioneuromas are a benign tumor of neural crest origin and represent the most histologically mature form of a rare group of tumors termed neuroblastic tumors. Neuroblastic tumors represent 6% of tumors in children and most commonly arise in the abdomen and thorax (specifically, the posterior mediastinum and retroperitoneum), while only 5% arise from the cervical region.1 Age at presentation varies. Neuroblastic tumors are divided into 3 types based on a spectrum differentiation toward mature neural crest elements. The metastatic potential increases with less mature types. Neuroblastomas represent the most primitive or least mature variant and have significant metastatic potential. Ganglioneuroblastoma has intermediate maturity and has a lower metastatic potential, whereas ganglioneuroma represents the most mature variant, with no essentially metastatic potential. Ganglioneuromas are considered to be relatively slowgrowing tumors and are often identified as an incidental intraoral or cervical swelling. When occurring in cervical sites, symptoms typically result from mass effect, causing dysphagia, neck pain, dysphonia, globus sensation, dyspnea, snoring, obstructive sleep-related apnea, cranial nerve palsy of nerves IX to XII, or Horner syndrome consisting of ipsilateral ptosis, meiosis, and anhidrosis. Owing to the ARTICLE INFORMATION
REFERENCES
Author Affiliations: University of Missouri School of Medicine, Columbia.
1. Cotton RT, Ballard ET, Going JA, et al. Tumors of the head and neck in children. In: Thawley SE, Panje WR, Batsakis JB, Lindberg RD, eds. Comprehensive Management of Head and Neck Tumors. Philadelphia, PA: W. B. Saunders Co; 1987:1792-1794.
Corresponding Author: Eliav Gov-Ari, MD, Department of Otolaryngology–Head and Neck Surgery, University of Missouri School of Medicine, One Hospital Dr, MA-314, Columbia, MO 65212 ([email protected]). Section Editor: Edward B. Stelow, MD. Published Online: December 19, 2013. doi:10.1001/jamaoto.2013.6068. Conflict of Interest Disclosures: None reported.
174
neural crest cell origin, tumors have the potential for functional excretion of catecholamines, which may result in systemic symptoms, including hypertension, diarrhea, sweating, and flushing.2-4 Histologically, ganglioneuromas consist of mature ganglions in a background of Schwann cells. Metaiodobenzylguanidine (MIBG) scintigraphy is a localization protocol developed in the 1980s for neuroendocrine tumors. MIBG enters neuroendocrine cells via active uptake by the epinephrine transporter and is stored in neurosecretory granules; it has been used for detection and follow-up of neuroendocrine tumors.5 Several imaging options exist and include computed tomography (CT), MRI, MIBG, and ultrasonography. Magnetic resonance imaging shows low-to intermediate intensity on T1-weighted images and heterogeneous high intensity on T2-weighted images and considerable enhancement with contrast.6 Contrast CT may show hypoattenuation and is useful for showing the anatomic relationships of the mass. When identified, these lesions are typically excised because the natural history of neuroblastic tumors is unknown and histological analysis may identify the presence of immature cells, necessitating further evaluation by a medical oncologist.Mostcommonly,surgicalexcisionisachievedthroughatranscervical excision; however, in selected cases a transoral approach may be considered when tumors involve the retropharyngeal space. Excision invariably results in ipsilateral Horner syndrome that is transient in most cases. Ganglioneuromas are rare lesions in the cervical region and occur infrequently in the retropharyngeal space.
2. Olsen KD. Tumors and surgery of the parapharyngeal space. Laryngoscope. 1994;104 (5, pt 2)(suppl 63):1-28. 3. Enzinger FM, Weiss SW. Soft Tissue Tumors. St Louis, MO: Mosby; 1998:828-831.
4. Hughes KV III, Olsen KD, McCaffrey TV. Parapharyngeal space neoplasms. Head Neck. 1995;17(2):124-130. 5. Nakajo M, Shapiro B, Copp J, et al. The normal and abnormal distribution of the adrenomedullary imaging agent m-[I-131]iodobenzylguanidine (I-131 MIBG) in man: evaluation by scintigraphy. J Nucl Med. 1983;24(8):672-682. 6. Anil G, Tan TY. Imaging characteristics of schwannoma of the cervical sympathetic chain: a review of 12 cases. AJNR Am J Neuroradiol. 2010;31(8):1408-1412.
JAMA Otolaryngology–Head & Neck Surgery February 2014 Volume 140, Number 2
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archotol.jamanetwork.com/ by a Florida International University Medical Library User on 05/30/2015
jamaotolaryngology.com
Clinical Problem Solving | PATHOLOGY
A Retropharyngeal Mass in a Young Boy Jason Showmaker, MD; Eliav Gov-Ari, MD
A
B
C
D
Figure. A, Magnetic resonance image of the neck; B, firm encapsulated mass being dissected from the retropharyngeal space with extensions to the carotid space; C, low-power micrograph of a specimen from oropharyngeal mass showing a Schwannian-like background of eosinophilic, spindle-shaped cells (hematoxylin-eosin, original magnification ×40); D, high-power micrograph showing clusters of well-differentiated ganglion cells with large round nuclei, prominent nucleoli, and granular basophilic Nissle substance (hematoxylin-eosin, original magnification ×100).
A young boy was noted during routine adenotonsillectomy to have a large firm mass in the posteriolateral pharyngeal wall and was referred to a tertiary medical center. Postoperatively, he developed mild velopharyngealinsufficiency.Onflexibleendoscopy,theposteriorpharyngeal mass was clearly visible. Magnetic resonance imaging (MRI) of the neck revealed a 3 × 2 × 1-cm fusiform lesion attached to the medial aspect of the left carotid space, extending to the midline retropharyngeal space at the level of C2 to C4 (Figure, A). The mass showed homogenous enhancement with gadolinium contrast (not shown). The patient was taken to the operating room, and a transoral approach via horizontal incision in the level of orohypopharynx was performed. The firm encapsulated mass was easily identified and dissected free from surrounding tissue (Figure, B). The mass showed a narrow extension to the carotid space.
jamaotolaryngology.com
On gross examination, the mass was firm and encapsulated. Hematoxylin-eosin–stained slides at low power (original magnification ×40) revealed a Schwannian-like background of eosinophilic, spindle-shaped cells (Figure, C). At higher power (original magnification ×100), clusters of well-differentiated ganglion cells with large round nuclei, prominent nucleoli, and granular basophilic Nissle substance (endoplasmic reticulum and ribosomes present in the cytoplasm of neural cells) were seen (Figure, D). Postoperatively, the patient was noted to have unilateral mild drooping of his eyelid, and his left pupil was constricted (yet responded to light) on the side of the resected lesion. Repeated MRI 6 months later showed no recurrence. What is your diagnosis?
JAMA Otolaryngology–Head & Neck Surgery February 2014 Volume 140, Number 2
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archotol.jamanetwork.com/ by a Florida International University Medical Library User on 05/30/2015
173
Clinical Review & Education Clinical Problem Solving
Diagnosis Carotid space ganglioneuroma
Discussion Ganglioneuromas are a benign tumor of neural crest origin and represent the most histologically mature form of a rare group of tumors termed neuroblastic tumors. Neuroblastic tumors represent 6% of tumors in children and most commonly arise in the abdomen and thorax (specifically, the posterior mediastinum and retroperitoneum), while only 5% arise from the cervical region.1 Age at presentation varies. Neuroblastic tumors are divided into 3 types based on a spectrum differentiation toward mature neural crest elements. The metastatic potential increases with less mature types. Neuroblastomas represent the most primitive or least mature variant and have significant metastatic potential. Ganglioneuroblastoma has intermediate maturity and has a lower metastatic potential, whereas ganglioneuroma represents the most mature variant, with no essentially metastatic potential. Ganglioneuromas are considered to be relatively slowgrowing tumors and are often identified as an incidental intraoral or cervical swelling. When occurring in cervical sites, symptoms typically result from mass effect, causing dysphagia, neck pain, dysphonia, globus sensation, dyspnea, snoring, obstructive sleep-related apnea, cranial nerve palsy of nerves IX to XII, or Horner syndrome consisting of ipsilateral ptosis, meiosis, and anhidrosis. Owing to the ARTICLE INFORMATION
REFERENCES
Author Affiliations: University of Missouri School of Medicine, Columbia.
1. Cotton RT, Ballard ET, Going JA, et al. Tumors of the head and neck in children. In: Thawley SE, Panje WR, Batsakis JB, Lindberg RD, eds. Comprehensive Management of Head and Neck Tumors. Philadelphia, PA: W. B. Saunders Co; 1987:1792-1794.
Corresponding Author: Eliav Gov-Ari, MD, Department of Otolaryngology–Head and Neck Surgery, University of Missouri School of Medicine, One Hospital Dr, MA-314, Columbia, MO 65212 ([email protected]). Section Editor: Edward B. Stelow, MD. Published Online: December 19, 2013. doi:10.1001/jamaoto.2013.6068. Conflict of Interest Disclosures: None reported.
174
neural crest cell origin, tumors have the potential for functional excretion of catecholamines, which may result in systemic symptoms, including hypertension, diarrhea, sweating, and flushing.2-4 Histologically, ganglioneuromas consist of mature ganglions in a background of Schwann cells. Metaiodobenzylguanidine (MIBG) scintigraphy is a localization protocol developed in the 1980s for neuroendocrine tumors. MIBG enters neuroendocrine cells via active uptake by the epinephrine transporter and is stored in neurosecretory granules; it has been used for detection and follow-up of neuroendocrine tumors.5 Several imaging options exist and include computed tomography (CT), MRI, MIBG, and ultrasonography. Magnetic resonance imaging shows low-to intermediate intensity on T1-weighted images and heterogeneous high intensity on T2-weighted images and considerable enhancement with contrast.6 Contrast CT may show hypoattenuation and is useful for showing the anatomic relationships of the mass. When identified, these lesions are typically excised because the natural history of neuroblastic tumors is unknown and histological analysis may identify the presence of immature cells, necessitating further evaluation by a medical oncologist.Mostcommonly,surgicalexcisionisachievedthroughatranscervical excision; however, in selected cases a transoral approach may be considered when tumors involve the retropharyngeal space. Excision invariably results in ipsilateral Horner syndrome that is transient in most cases. Ganglioneuromas are rare lesions in the cervical region and occur infrequently in the retropharyngeal space.
2. Olsen KD. Tumors and surgery of the parapharyngeal space. Laryngoscope. 1994;104 (5, pt 2)(suppl 63):1-28. 3. Enzinger FM, Weiss SW. Soft Tissue Tumors. St Louis, MO: Mosby; 1998:828-831.
4. Hughes KV III, Olsen KD, McCaffrey TV. Parapharyngeal space neoplasms. Head Neck. 1995;17(2):124-130. 5. Nakajo M, Shapiro B, Copp J, et al. The normal and abnormal distribution of the adrenomedullary imaging agent m-[I-131]iodobenzylguanidine (I-131 MIBG) in man: evaluation by scintigraphy. J Nucl Med. 1983;24(8):672-682. 6. Anil G, Tan TY. Imaging characteristics of schwannoma of the cervical sympathetic chain: a review of 12 cases. AJNR Am J Neuroradiol. 2010;31(8):1408-1412.
JAMA Otolaryngology–Head & Neck Surgery February 2014 Volume 140, Number 2
Copyright 2014 American Medical Association. All rights reserved.
Downloaded From: http://archotol.jamanetwork.com/ by a Florida International University Medical Library User on 05/30/2015
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