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Supraglottic laryngeal paraganglioma
European Annals of Otorhinolaryngology, Head and Neck diseases (2010) 127, 117—119
CLINICAL COMMENTARY
Supraglottic laryngeal paraganglioma G. Buiret a,∗, O. Merrot a, F. Le Breton b, G. Landry a, M. Poupart a, J.-C. Pignat a a
ENT Department, Croix-Rousse Hospital, Lyon Hospital Board (hospices civils de Lyon), Grande Rue de la Croix Rousse, 69004 Lyon, France b Anatomopathology Department, Croix-Rousse Hospital, Lyon Hospital Board (hospices civils de Lyon), Grande Rue de la Croix Rousse, 69004 Lyon, France Available online 13 May 2010
KEYWORDS Benign tumor; Larynx; Neuroendocrine tumor; Paraganglioma; Surgery
Summary Introduction: Laryngeal paraganglioma is a rare, mainly supraglottic, tumor. Clinical case: A 67-year-old woman was operated on in June 2009 for supraglottic laryngeal paraganglioma, with simple postoperative course. Discussion: Anatomopathologic features, malignant paraganglioma and the various possible treatments are presented and discussed. Surgical exeresis with an external approach should in our opinion remain the reference treatment, for optimal control of exeresis of this potentially hemorrhagic tumor with risk of recurrence in case of incomplete exeresis. © 2010 Elsevier Masson SAS. All rights reserved.
Introduction Laryngeal endocrine tumors are varied. Wick [1] classified them in terms of origin: neural (paraganglioma), and epithelial (carcinoid tumor). They are rare: some 80 individual cases of laryngeal paraganglioma have been reported, but no large series. In a review of the laryngeal paraganglioma literature, Barne [2] showed onset to be between the ages of 40 and 60 years, the sex ratio to be a 3:1 female predominance, and location to be mainly supraglottic (82 %) or subglottic (15 %).
∗
DOI of original article:10.1016/j.aforl.2010.04.002. Corresponding author. Tel.: +33 472071693; fax: +33 472071690. E-mail address: [email protected] (G. Buiret).
We present a further case of subglottic paraganglioma.
Clinical case A 67-year-old woman with a history of high blood pressure and type-II diabetes, non-smoker and non-drinker, consulted in May 2009 for dysphonia progressively worsening since December 2008, without dyspnea or swallowing disorder. Nasofibroscopy found right paralaryngeal submucosal tumefaction covering the glottis; the left vocal fold was mobile and the right not visible. Suspension laryngoscopy found arching of the right ventricular band; the glottis, subglottis, base of the tongue and hypopharynx were free. Biopsy found inflammatory remodeling without malignancy criteria.
1879-7296/$ – see front matter © 2010 Elsevier Masson SAS. All rights reserved. doi:10.1016/j.anorl.2010.04.002
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G. Buiret et al.
Figure 1
Axial, sagittal and frontal larynx scan cross-sections.
Discussion
Figure 2
Macroscopic view of tumor sectioned in half.
Cervicothoracic scan (Fig. 1) showed a 33 × 30 mm lesion with contrast uptake stretching from the right aryepiglottic fold, across the epiglottic cartilage and involving the preepiglottic space. The glottis was conserved, and there was no adenopathy or other cervical or thoracic location. The patient was operated on with a cervical approach in June 2009. After Paul André ‘‘L’’ incision and exeresis of the right half of the hyoid bone, chondrotomy of the superolateral third of the right thyroid cartilage disclosed a highly hemorrhagic lesion, which was progressively dissected. The tumor was resected and the pharyngectomy was progressively closed over a Redon aspiration drain. Tracheotomy was performed at the end of surgery. The sample was sectioned (Fig. 2): it was firm and brown, without intratumoral hemorrhage. Anatomopathology (Fig. 3) found a submucosal tumor pushing the mucosa to the surface, and confirmed the diagnosis of paraganglioma. The tumor reached the inferior and medial surgical margins. Frozen sections were negative. The patient resumed feeding on D6, and the tracheotomy was closed on D10. Further investigation of family history found that a paternal aunt had been operated on for paraganglioma. A whole-body 111 Indium pentetreotide scan was therefore performed and showed no other paraganglioma loci or metastasis; a familial form was investigated.
While most cases of paraganglioma are sporadic, there exist well-identified familial syndromes (type II A and B multiple endocrine neoplasia, Carney complex) and genes have been identified (PGL 1, 2 and 3) with autosomal dominant transmission of varying penetrance, inactivated on the maternal side [3,4]: affected males run a 50 % risk of having an affected child, while affected females do not have affected children but transmit a silent mutation. According to Pelliteri et al. [4], the reference imaging technique for head and neck paraganglioma is angioMRI, which was not performed in the present case, diagnosis being suspected only peroperatively. Angio-MRI provides better vascular mapping than angioscan, which on the other hand shows better definition of bone contact. Endoscopic biopsy is difficult [4] as it must be deep, and thus potentially hemorrhagic, as the lesion is submucosal. Anatomopathology shows two types of cell: the principal type is polygonal with a rounded nucleus and eosinophil cytoplasm, with rare mitoses, arranged in alveoli in a ‘‘Zellballen’’ pattern (which is characteristic but not pathognomic). Around the Zellballen are found thin, spindle-shaped sustentacular cells with long nuclei.
Figure 3
Microscopic view of tumor.
Supraglottic laryngeal paraganglioma The tumor is highly vascularized and non-encapsulated. On immunomarking, the principal cells are fixed by neuroendocrine (chromogranin and synaptophysin) but not epithelial stains (cytokeratin, carcinoembryonic antigen). The sustentacular cells, unlike the principal cells, fix S-100 protein. There are no anatomopathologic criteria specific to malignancy in paraganglioma: only clinical evolution confirms metastasis. Several putative cases of malignant laryngeal paraganglioma have been described in the literature, but only one is accepted as such [5]. According to Manolidis et al. [6], 1—3 % of paragangliomas secrete, and are only to be explored for if the patient is symptomatic. The reference treatment for laryngeal paraganglioma is surgical exeresis with a cervical approach (lateral pharyngolaryngectomy [4,5,7]) without cervical curage as there are no ganglions to ablate [5]. Angiography with preoperative embolization is possible. Sesterhenn et al. [8] and Papacharalampous et al. [9] reported endoscopic exeresis, which we would not, however, recommend due to the risk of uncontrollable bleeding in case of hemorrhage. Isolated external radiotherapy does not provide tumor regression but merely halts growth [4,10]. In the case of laryngeal location with reduced airway, there is a further risk of radioinduced edema. Postoperative radiotherapy does not feature as a possible option in the literature.
Conflict of interest None.
119
References [1] Wick MR. Neuroendocrine neoplasia. Current concepts. Am J Clin Pathol 2000;113:331—5. [2] Barne L. Paraganglioma of the larynx. A critical review of literature. ORL J Otorhinolaryngol Relat Spec 1991;53: 220—34. [3] Rubin AD, Cheng SS, Bradford CR. Laryngeal paraganglioma in a patient with multiple head and neck paragangliomas. Otolaryngol Head Neck Surg 2005;132:520—2. [4] Pellitteri PK, Rinaldo A, Myssiorek D, Jackson CG, Bradley PJ, Devaney KO, et al. Paragangliomas of the head and neck. Oral Oncology 2004;40:563—75. [5] Ferlito A, Siver CE, Bradford CR, Rinaldo A. Neuroendocrine neoplasms of larynx: an overview. Head Neck 2009;31:1634—46. [6] Manolidis S, Shohet JA, Jackson CG, Glassock III ME. Malignant glomus tumors. Laryngoscope 1999;109:30—4. [7] Konowitz PM, Lawson W, Som PM, Urken ML, Breakstone BA, Biller HF. Laryngeal paraganglioma: update on diagnosis and treatment. Laryngoscope 1988;98:40—9. [8] Sesterhenn AM, Folz BJ, Lippert BM, Jänig U, Werner JA. Laser surgical treatment of laryngeal paraganglioma. J Laryngol Otol 2003;117:641—6. [9] Papacharalampous G, Korres S, Tzagaroulakis M, Segas I, Fedekidis A. Paraganglioma of the larynx. Med Sci Monit 2007;13:CS145—8. [10] Myssiorek D, Halaas Y, Silver C. Laryngeal and sinonasal paragangliomas. Otolaryngol Clin North Am 2001;34: 971—82.
CLINICAL COMMENTARY
Supraglottic laryngeal paraganglioma G. Buiret a,∗, O. Merrot a, F. Le Breton b, G. Landry a, M. Poupart a, J.-C. Pignat a a
ENT Department, Croix-Rousse Hospital, Lyon Hospital Board (hospices civils de Lyon), Grande Rue de la Croix Rousse, 69004 Lyon, France b Anatomopathology Department, Croix-Rousse Hospital, Lyon Hospital Board (hospices civils de Lyon), Grande Rue de la Croix Rousse, 69004 Lyon, France Available online 13 May 2010
KEYWORDS Benign tumor; Larynx; Neuroendocrine tumor; Paraganglioma; Surgery
Summary Introduction: Laryngeal paraganglioma is a rare, mainly supraglottic, tumor. Clinical case: A 67-year-old woman was operated on in June 2009 for supraglottic laryngeal paraganglioma, with simple postoperative course. Discussion: Anatomopathologic features, malignant paraganglioma and the various possible treatments are presented and discussed. Surgical exeresis with an external approach should in our opinion remain the reference treatment, for optimal control of exeresis of this potentially hemorrhagic tumor with risk of recurrence in case of incomplete exeresis. © 2010 Elsevier Masson SAS. All rights reserved.
Introduction Laryngeal endocrine tumors are varied. Wick [1] classified them in terms of origin: neural (paraganglioma), and epithelial (carcinoid tumor). They are rare: some 80 individual cases of laryngeal paraganglioma have been reported, but no large series. In a review of the laryngeal paraganglioma literature, Barne [2] showed onset to be between the ages of 40 and 60 years, the sex ratio to be a 3:1 female predominance, and location to be mainly supraglottic (82 %) or subglottic (15 %).
∗
DOI of original article:10.1016/j.aforl.2010.04.002. Corresponding author. Tel.: +33 472071693; fax: +33 472071690. E-mail address: [email protected] (G. Buiret).
We present a further case of subglottic paraganglioma.
Clinical case A 67-year-old woman with a history of high blood pressure and type-II diabetes, non-smoker and non-drinker, consulted in May 2009 for dysphonia progressively worsening since December 2008, without dyspnea or swallowing disorder. Nasofibroscopy found right paralaryngeal submucosal tumefaction covering the glottis; the left vocal fold was mobile and the right not visible. Suspension laryngoscopy found arching of the right ventricular band; the glottis, subglottis, base of the tongue and hypopharynx were free. Biopsy found inflammatory remodeling without malignancy criteria.
1879-7296/$ – see front matter © 2010 Elsevier Masson SAS. All rights reserved. doi:10.1016/j.anorl.2010.04.002
118
G. Buiret et al.
Figure 1
Axial, sagittal and frontal larynx scan cross-sections.
Discussion
Figure 2
Macroscopic view of tumor sectioned in half.
Cervicothoracic scan (Fig. 1) showed a 33 × 30 mm lesion with contrast uptake stretching from the right aryepiglottic fold, across the epiglottic cartilage and involving the preepiglottic space. The glottis was conserved, and there was no adenopathy or other cervical or thoracic location. The patient was operated on with a cervical approach in June 2009. After Paul André ‘‘L’’ incision and exeresis of the right half of the hyoid bone, chondrotomy of the superolateral third of the right thyroid cartilage disclosed a highly hemorrhagic lesion, which was progressively dissected. The tumor was resected and the pharyngectomy was progressively closed over a Redon aspiration drain. Tracheotomy was performed at the end of surgery. The sample was sectioned (Fig. 2): it was firm and brown, without intratumoral hemorrhage. Anatomopathology (Fig. 3) found a submucosal tumor pushing the mucosa to the surface, and confirmed the diagnosis of paraganglioma. The tumor reached the inferior and medial surgical margins. Frozen sections were negative. The patient resumed feeding on D6, and the tracheotomy was closed on D10. Further investigation of family history found that a paternal aunt had been operated on for paraganglioma. A whole-body 111 Indium pentetreotide scan was therefore performed and showed no other paraganglioma loci or metastasis; a familial form was investigated.
While most cases of paraganglioma are sporadic, there exist well-identified familial syndromes (type II A and B multiple endocrine neoplasia, Carney complex) and genes have been identified (PGL 1, 2 and 3) with autosomal dominant transmission of varying penetrance, inactivated on the maternal side [3,4]: affected males run a 50 % risk of having an affected child, while affected females do not have affected children but transmit a silent mutation. According to Pelliteri et al. [4], the reference imaging technique for head and neck paraganglioma is angioMRI, which was not performed in the present case, diagnosis being suspected only peroperatively. Angio-MRI provides better vascular mapping than angioscan, which on the other hand shows better definition of bone contact. Endoscopic biopsy is difficult [4] as it must be deep, and thus potentially hemorrhagic, as the lesion is submucosal. Anatomopathology shows two types of cell: the principal type is polygonal with a rounded nucleus and eosinophil cytoplasm, with rare mitoses, arranged in alveoli in a ‘‘Zellballen’’ pattern (which is characteristic but not pathognomic). Around the Zellballen are found thin, spindle-shaped sustentacular cells with long nuclei.
Figure 3
Microscopic view of tumor.
Supraglottic laryngeal paraganglioma The tumor is highly vascularized and non-encapsulated. On immunomarking, the principal cells are fixed by neuroendocrine (chromogranin and synaptophysin) but not epithelial stains (cytokeratin, carcinoembryonic antigen). The sustentacular cells, unlike the principal cells, fix S-100 protein. There are no anatomopathologic criteria specific to malignancy in paraganglioma: only clinical evolution confirms metastasis. Several putative cases of malignant laryngeal paraganglioma have been described in the literature, but only one is accepted as such [5]. According to Manolidis et al. [6], 1—3 % of paragangliomas secrete, and are only to be explored for if the patient is symptomatic. The reference treatment for laryngeal paraganglioma is surgical exeresis with a cervical approach (lateral pharyngolaryngectomy [4,5,7]) without cervical curage as there are no ganglions to ablate [5]. Angiography with preoperative embolization is possible. Sesterhenn et al. [8] and Papacharalampous et al. [9] reported endoscopic exeresis, which we would not, however, recommend due to the risk of uncontrollable bleeding in case of hemorrhage. Isolated external radiotherapy does not provide tumor regression but merely halts growth [4,10]. In the case of laryngeal location with reduced airway, there is a further risk of radioinduced edema. Postoperative radiotherapy does not feature as a possible option in the literature.
Conflict of interest None.
119
References [1] Wick MR. Neuroendocrine neoplasia. Current concepts. Am J Clin Pathol 2000;113:331—5. [2] Barne L. Paraganglioma of the larynx. A critical review of literature. ORL J Otorhinolaryngol Relat Spec 1991;53: 220—34. [3] Rubin AD, Cheng SS, Bradford CR. Laryngeal paraganglioma in a patient with multiple head and neck paragangliomas. Otolaryngol Head Neck Surg 2005;132:520—2. [4] Pellitteri PK, Rinaldo A, Myssiorek D, Jackson CG, Bradley PJ, Devaney KO, et al. Paragangliomas of the head and neck. Oral Oncology 2004;40:563—75. [5] Ferlito A, Siver CE, Bradford CR, Rinaldo A. Neuroendocrine neoplasms of larynx: an overview. Head Neck 2009;31:1634—46. [6] Manolidis S, Shohet JA, Jackson CG, Glassock III ME. Malignant glomus tumors. Laryngoscope 1999;109:30—4. [7] Konowitz PM, Lawson W, Som PM, Urken ML, Breakstone BA, Biller HF. Laryngeal paraganglioma: update on diagnosis and treatment. Laryngoscope 1988;98:40—9. [8] Sesterhenn AM, Folz BJ, Lippert BM, Jänig U, Werner JA. Laser surgical treatment of laryngeal paraganglioma. J Laryngol Otol 2003;117:641—6. [9] Papacharalampous G, Korres S, Tzagaroulakis M, Segas I, Fedekidis A. Paraganglioma of the larynx. Med Sci Monit 2007;13:CS145—8. [10] Myssiorek D, Halaas Y, Silver C. Laryngeal and sinonasal paragangliomas. Otolaryngol Clin North Am 2001;34: 971—82.