European Annals of Otorhinolaryngology, Head and Neck diseases 132 (2015) 109
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Letter to the editor Letter on the article “Malignant head/neck paragangliomas. Comparative study” Correspondance à propos de l’article : « Histoire naturelle des paragangliomes malins de la tête et du cou. Analyse comparative » To Editor, Mediouni et al. [1], in their recent article, described their experience in the treatment of malignant head and neck paragangliomas (HNPs) and, in particular, compared the epidemiological, clinical and genetic characteristics, natural history and treatment with those of a series of 131 benign HNPs. We congratulate the authors for the interesting manuscript that offers many opportunities for further research. Approximately 10% to 15% of HNP cases are familial. The majority of these cases are caused by mutations in SDHB or SDHD with mutations in SDHC occurring less commonly [2]. The occurrence of malignancy is also significantly different in sporadic and familial cases. Its overall frequency is reported to be 2 to 5% for jugulotympanic paragangliomas, 6% for carotid body and between 16 and 19% for glomus vagale tumours [3]. Surgery is recommended for all patients, if they are in good general health and a global evaluation does not provide data for its contraindication [4]. The incidence of tumour recurrence in the follow-up ranges from 3 to 20% [5]. We agree with the authors that familial cases and SDHB mutations are more frequently associated with malignancy and the risk of recurrence. Currently, the study protocol of HNPs requires the use of nuclear medicine. The same authors have used 111 In-pentetreotide scintigraphy (Octreoscan), 18 F-Fluorodeoxyglucose-Positron Emission Tomography (18 F-FDG-PET) or 123 I-MIBG scintigraphy, but none of these methods has proved to be sufficiently sensitive to detect the presence of bone metastases. Do you think that the use
DOIs of original articles: http://dx.doi.org/10.1016/j.anorl.2014.04.003, http://dx.doi.org/10.1016/j.anorl.2013.05.003 http://dx.doi.org/10.1016/j.anorl.2013.12.002 1879-7296/© 2014 Elsevier Masson SAS. All rights reserved.
of MRI should become of choice in these patients? How frequently should be performed? In addition, the authors describe a metastatic disease at diagnosis in 3 patients and during follow-up in the other 8 patients. The prognosis was worse in patients with liver metastases. Do you think that it was just a coincidence? Finally, the authors correctly state that no consensus has been reached concerning the histological criteria of malignancy (multiple mitotic figures, nuclear polymorphism and capsular effraction). We observed in our experience a correlation between malignancy and the presence of metastasis and recurrence rate. Have you observed this correlation? References [1] Mediouni A, Ammari S, Wassef M, et al. Malignant head/neck paragangliomas. Comparative study. Eur Ann Otorhinolaryngol Head Neck Dis 2014;131:159–66. [2] Neumann HPH, Pawlu C, Peczkowska M, et al. Distinct clinical features of paraganglioma syndromes associated with SDHB and SDHD gene mutations. JAMA 2004;292:943–51. [3] Lee JH, Barich F, Karnell LH, et al. National Cancer Data Base report on malignant paragangliomas of the head and neck. Cancer 2002;94:730–7. [4] Bozzani A, Arici V, Ragni F, et al. Clinical suspicion of bilateral carotid body paraganglioma and an unexpected histologic diagnosis. Ann Vasc Surg 2014;28:262 [e9–11]. [5] Jackson CG, McGrew BM, Forest JA, et al. Lateral skull base surgery for glomus tumors: long-term control. Otol Neurotol 2001;22:377–82.
A. Bozzani ∗ V. Arici F. Ragni Vascular Surgery, Foundation I.R.C.C.S. Policlinico San Matteo, Viale Camillo Golgi, 19, 27100 Pavia, Italy ∗ Corresponding author. E-mail address: [email protected] (A. Bozzani)
Available online at
ScienceDirect www.sciencedirect.com
Letter to the editor Letter on the article “Malignant head/neck paragangliomas. Comparative study” Correspondance à propos de l’article : « Histoire naturelle des paragangliomes malins de la tête et du cou. Analyse comparative » To Editor, Mediouni et al. [1], in their recent article, described their experience in the treatment of malignant head and neck paragangliomas (HNPs) and, in particular, compared the epidemiological, clinical and genetic characteristics, natural history and treatment with those of a series of 131 benign HNPs. We congratulate the authors for the interesting manuscript that offers many opportunities for further research. Approximately 10% to 15% of HNP cases are familial. The majority of these cases are caused by mutations in SDHB or SDHD with mutations in SDHC occurring less commonly [2]. The occurrence of malignancy is also significantly different in sporadic and familial cases. Its overall frequency is reported to be 2 to 5% for jugulotympanic paragangliomas, 6% for carotid body and between 16 and 19% for glomus vagale tumours [3]. Surgery is recommended for all patients, if they are in good general health and a global evaluation does not provide data for its contraindication [4]. The incidence of tumour recurrence in the follow-up ranges from 3 to 20% [5]. We agree with the authors that familial cases and SDHB mutations are more frequently associated with malignancy and the risk of recurrence. Currently, the study protocol of HNPs requires the use of nuclear medicine. The same authors have used 111 In-pentetreotide scintigraphy (Octreoscan), 18 F-Fluorodeoxyglucose-Positron Emission Tomography (18 F-FDG-PET) or 123 I-MIBG scintigraphy, but none of these methods has proved to be sufficiently sensitive to detect the presence of bone metastases. Do you think that the use
DOIs of original articles: http://dx.doi.org/10.1016/j.anorl.2014.04.003, http://dx.doi.org/10.1016/j.anorl.2013.05.003 http://dx.doi.org/10.1016/j.anorl.2013.12.002 1879-7296/© 2014 Elsevier Masson SAS. All rights reserved.
of MRI should become of choice in these patients? How frequently should be performed? In addition, the authors describe a metastatic disease at diagnosis in 3 patients and during follow-up in the other 8 patients. The prognosis was worse in patients with liver metastases. Do you think that it was just a coincidence? Finally, the authors correctly state that no consensus has been reached concerning the histological criteria of malignancy (multiple mitotic figures, nuclear polymorphism and capsular effraction). We observed in our experience a correlation between malignancy and the presence of metastasis and recurrence rate. Have you observed this correlation? References [1] Mediouni A, Ammari S, Wassef M, et al. Malignant head/neck paragangliomas. Comparative study. Eur Ann Otorhinolaryngol Head Neck Dis 2014;131:159–66. [2] Neumann HPH, Pawlu C, Peczkowska M, et al. Distinct clinical features of paraganglioma syndromes associated with SDHB and SDHD gene mutations. JAMA 2004;292:943–51. [3] Lee JH, Barich F, Karnell LH, et al. National Cancer Data Base report on malignant paragangliomas of the head and neck. Cancer 2002;94:730–7. [4] Bozzani A, Arici V, Ragni F, et al. Clinical suspicion of bilateral carotid body paraganglioma and an unexpected histologic diagnosis. Ann Vasc Surg 2014;28:262 [e9–11]. [5] Jackson CG, McGrew BM, Forest JA, et al. Lateral skull base surgery for glomus tumors: long-term control. Otol Neurotol 2001;22:377–82.
A. Bozzani ∗ V. Arici F. Ragni Vascular Surgery, Foundation I.R.C.C.S. Policlinico San Matteo, Viale Camillo Golgi, 19, 27100 Pavia, Italy ∗ Corresponding author. E-mail address: [email protected] (A. Bozzani)
