Contribution of radiation therapy of head and neck paragangliomas: About 6 cases presentation
Öz
The neck’s paraganglions are bilateral nodular structures, with the same embryological origin which is the cephalic neural crest. They have a branchiomeric distribution. They have an important secreting function during the embryonic life, and then regress when the adrenal medulla starts functioning. Then only carotid and aortic paraganglions have chemoreceptor, baroreceptor and endocrine proved functions. The paragangliomas are tumors developed at the expense of paraganglions by proliferations of the chief cells (of type I) most of the time. Generally these tumors exhibit a slow growth rate, most often presenting asymptomatically as a space occupying mass lesion witnessed clinically or radiographically. The secreting tumors are very rare (5%). The benignity is the rule but the localization near noble structures makes it a highly risky tumor. This disease is often monofocal but it may also be part of a multifocal disease (6%). Advances in imaging have facilitated the diagnosis and the assessment of this disease. Diagnosis is generally made through a combination of clinical findings and radiographic studies. Surgery is the treatment of choice through total subadventitial resection, and rebuilding of the carotid axis when necessary. Surgery may lead to significant morbidity, resulting from major cranial nerve injury and especially at a late stage in the evolution of the disease. The preoperative embolization can facilitate ablation and reduce morbidity. Precisely, external radiotherapy can de indicated for recurrences, tumoral operating residues, and counter indications.
Anahtar Kelimeler
Kaynakça
- 1. Baysal BE. Hereditary paraganglioma targets diverse paraganglia. J Med Genet. 2002;39;617–22.
- 2. Van der Mey AG, Maaswinkel-Mooy PD, Cornelisse CJ. Genomic imprinting in hereditary glomus tumors: evidence for new genetic theory. Lancet. 1989;2:1291-4.
- 3. Opocher G, Schiavi F. Genetics of pheochromocytomas and paragangliomas. Best Pract Res Clin Endocrinol Metab. 2010;24:943-56.
- 4. van der Mey AG, Maaswinkel-Mooy PD, Cornelisse CJ, Schmidt PH, van de Kamp JJ. Genomic imprinting in hereditary glomus tumors: evidence for new genetic theory. Lancet. 1989;ii:1291–4.
- 5. Heutink P, van der Mey AG, Sandkuijl LA, et al. A gene subject to genomic imprinting and responsible for hereditary paragangliomas maps to chromosome 11q23-qter. Hum Mol Genet. 1992;1:7–10.
- 6. Niemann S, Müller U. Mutations in SDHC cause autosomal dominant paraganglioma, type 3. Nat Genet. 2000;26:268–70.
- 7. Astuti D, Latif F, Dallol A, et al. Gene mutations in the succinate dehydrogenase subunit SDHB cause susceptibility to familial pheochromocytoma and to familial paraganglioma. Am J Hum Genet. 2001;69:49–54.
- 8. Hao HX, Khalimonchuk O, Schraders M, Dephoure N, Bayley JP, Kunst H, Devilee P, Cremers CW, Schiffman JD, Bentz BG, Gygi SP, Winge DR, Kremer H, Rutter J. SDH5, a gene required for flavination of succinate dehydrogenase, is mutated in paraganglioma. Science. 2009;28:1139–42.
Ayrıntılar
Birincil Dil
İngilizce
Konular
Klinik Tıp Bilimleri
Bölüm
Olgu Sunumu
Yazarlar
Sanae Ghammad
*
0000-0002-7940-8396
Morocco
Allouche Fadwa
Bu kişi benim
0000-0002-6793-4911
Morocco
Terrab Fatima Zahra
Bu kişi benim
0000-0002-9257-1964
Morocco
Chebihi Hassani Ghita
Bu kişi benim
0000-0003-2714-1957
Morocco
Alami Zenab
Bu kişi benim
0000-0003-3349-1793
Morocco
Bouhafa Thouria
Bu kişi benim
0000-0002-9857-1594
Morocco
Hassouni Khalid
Bu kişi benim
0000-0002-1442-255X
Morocco
Yayımlanma Tarihi
25 Şubat 2019
Gönderilme Tarihi
29 Ekim 2018
Kabul Tarihi
20 Aralık 2018
Yayımlandığı Sayı
Yıl 2019 Cilt: 3 Sayı: 2