Laryngeal paraganglioma in a patient with multiple head and neck paragangliomas

2005 ◽  
Vol 132 (3) ◽  
pp. 520-522 ◽  
Author(s):  
Adam D. Rubin ◽  
Sara S. Cheng ◽  
Carol R. Bradford
Keyword(s):  
Head And Neck ◽  
Neuroendocrine Tumors ◽  
Carotid Body ◽  
Additional Case ◽  
Carotid Body Tumor ◽  
Head And Neck Paragangliomas ◽  
Synchronous Lesion

Paragangliomas of the larynx are rare neuroendocrine tumors, with fewer than 70 cases reported in the literature. 1 Typically, laryngeal paragangliomas are not found in patients with multicentric or familial paragangliomas. Only 1 case of laryngeal paraganglioma has been reported in a patient with a synchronous lesion elsewhere (carotid body tumor). 2 We report an additional case of a patient with a laryngeal and multiple other paragangliomas.

S135 – Head and Neck Paragangliomas and von Hippel-Lindau Disease

2008 ◽  
Vol 139 (2_suppl) ◽  
pp. P122-P122
Author(s):  
Carsten Christof Boedeker ◽  
Gerd Jurgen Ridder ◽  
Wolfgang Maier ◽  
Hartmut Ph Neumann
Keyword(s):  
Head And Neck ◽  
Carotid Body ◽  
Medical Literature ◽  
International Medical ◽  
Carotid Body Tumor ◽  
Pancreatic Cysts ◽  
Kidney Tumors ◽  
Von Hippel Lindau ◽  
Von Hippel Lindau Disease ◽  
Head And Neck Paragangliomas

Objectives To review our experience with head and neck paragangliomas (HNPs) in patients with von Hippel-Lindau disease (VHL). HNPs are rare tumors, 30% of which have been shown to be hereditary neoplasms. Since 2000,4 different paraganglioma syndromes (PGL 1–4) have been described. VHL is characterized by retinal angiomas, haemongioblastomas of the central nervous system, kidney tumors, pancreatic cysts, and pheochromocytomas. Only 4 cases of VHL patients with HNPs have been published in the international medical literature. We systematically reviewed our VHL registry for patients with HNPs. Methods In 1983 we founded a registry for VHL patients. Until 2008, 395 index cases could be registered. First-degree relatives were offered genetic screening as well. Results Three VHL patients presented with HNPs. One boy (mutation VHL 505 T>C) developed a metastatic pheochromocytoma at age 7. 8 years after treatment, he presented with a right-sided carotid body tumor. Another male patient was diagnosed with a tumor of the right carotid body at age 33 (VHL 404 T>A). Interestingly, there have been no other tumors so far. The third patient was diagnosed with a left carotid body tumor at age 33 (VHL 505 T>C). Currently, she has multiple haemangioblastomas of the spinal cord. Conclusions The 3 cases presented demonstrate the importance of awareness of HNPs as one of the manifestations in VHL. So far this rare association has found very little attention in international medical literature. We suggest that VHL patients should undergo a thorough otolaryngological examination of the head and neck.

scholarly journals Tinnitus With Unexpected Spanish Roots: Head and Neck Paragangliomas Caused by SDHAF2 Mutation

2020 ◽  
Vol 4 (3) ◽  
Author(s):  
Laura Maria Roose ◽  
Niels J Rupp ◽  
Christof Röösli ◽  
Nadejda Valcheva ◽  
Achim Weber ◽  
...  
Keyword(s):  
Genetic Testing ◽  
Head And Neck ◽  
Carotid Body ◽  
Loss Of Function ◽  
Negative Family History ◽  
Paraganglioma Syndrome ◽  
Plasma Free Metanephrines ◽  
The Right ◽  
Head And Neck Paragangliomas

Abstract It is estimated that up to 40% of all head and neck paragangliomas (HNPGL) have a hereditary background with the most common mutations being found in the succinate dehydrogenase (SDH) genes. SDHAF2 mutation leads to the rare paraganglioma syndrome 2. The authors present the case of a 15-year-old male patient with 2, non-secretory HNPGLs, presenting with left-sided, pulsatile tinnitus, and hearing loss. Imaging led to the suspicion of a jugulotympanic paraganglioma on the left, as well as a carotid body tumor on the right. After resection of the jugulotympanic tumor, histology confirmed the presence of a paraganglioma; immunohistochemistry furthermore suggested a loss of SDHB expression. Genetic testing revealed a rare germline, loss-of-function mutation in the SDHAF2 gene, previously described to cause hereditary paraganglioma syndrome 2. Twenty months after the first operation, the patient underwent a resection of the right carotid body paraganglioma. Plasma-free metanephrines/catecholamines always remained within the reference range; the patient is under regular follow-up, and his relatives will be screened. Our findings emphasize the relevance of genetic testing in patients with HNPGL, also with negative family history, especially when the patients present at a young age and with multiple lesions.

Glomus caroticum tumors: A case report of an operated giant carotid body tumor with a review of our experience in 47 patients

Open Medicine ◽  
2010 ◽  
Vol 5 (4) ◽  
pp. 411-416 ◽  
Author(s):  
Sureyya Talay ◽  
Mustafa Abanoz ◽  
Mehmet Ali Kaygin ◽  
Ozgur Dag ◽  
Umit Halici ◽  
...  
Keyword(s):  
Case Report ◽  
Head And Neck ◽  
Carotid Body ◽  
Neuroendocrine System ◽  
Carotid Body Tumor ◽  
Tumor Mass ◽  
Glomus Caroticum ◽  
Clinical Presentations ◽  
Giant Tumor ◽  
Neuroectodermal Origin

AbstractGlomus caroticum tumors, usually used as an alternative term for carotid body tumor, are of neuroectodermal origin and a part of the extra adrenal neuroendocrine system pathologies. These abnormalities are the most frequently detected paraganglioma in the localization of the head and neck. In our report, we present a giant tumor mass on the left side which was operated on successfully with a review of our experience retrospectively. Between the dates of June 1995 and October 2009, 47 patients, all of which had a glomus caroticum tumor, underwent to surgery. Tumor presented a wide variety of size and clinical presentations.

scholarly journals Head-and-neck paragangliomas are associated with sleep-related complaints, especially in the presence of carotid body tumors

2011 ◽  
Vol 16 (2) ◽  
pp. 527-534 ◽  
Author(s):  
Bas Havekes ◽  
Florine Kastelein ◽  
Agatha A. van der Klaauw ◽  
Nicolette van Duinen ◽  
Jeroen C. Jansen ◽  
...  
Keyword(s):  
Head And Neck ◽  
Carotid Body ◽  
Carotid Body Tumors ◽  
Head And Neck Paragangliomas

Octreotide Scintigraphy for the Detection of Paragangliomas

2000 ◽  
Vol 122 (3) ◽  
pp. 358-362 ◽  
Author(s):  
Fred F. Telischi ◽  
Andres Bustillo ◽  
Michelle L. H. Whiteman ◽  
Aldo N. Serafini ◽  
Michael J. Reisberg ◽  
...  
Keyword(s):  
Head And Neck ◽  
Neuroendocrine Tumors ◽  
False Negative ◽  
Neck Region ◽  
Synchronous Tumors ◽  
Head And Neck Region ◽  
Octreotide Scintigraphy ◽  
Octreotide Scan ◽  
Head And Neck Paragangliomas

Paragangliomas are neuroendocrine tumors located primarily in the head and neck region, but they can also occur at other sites. Confirming the preoperative diagnosis and detecting synchronous tumors may be difficult in some patients. Octreotide is a somatostatin analog that, when coupled to a radioisotope, produces a scintigraphic image of tumors expressing somatostatin type 2 receptors. Paragangliomas, like many neuroendocrine tumors, have been found to have a high density of somatostatin type 2 receptors on the cell surface. This study compared the results of preoperative octreotide scintigraphy with the histopathology in 21 patients who underwent surgery for presumed head and neck paragangliomas. Octreotide scan findings were positive in 16 patients with paragangliomas and negative in 3 patients with other pathology. One false-positive and 1 false-negative result were obtained. Thus, this test had an accuracy of 90%, a sensitivity of 94%, and a specificity of 75%. Previously unidentified synchronous tumors were identified in 5 patients. On the basis of this series of patients, octreotide scintigraphy appears to be a reliable test to detect paragangliomas and may be quite helpful in preoperative planning.

scholarly journals Potential Biomarkers of Metastasizing Paragangliomas and Pheochromocytomas

Life ◽  
2021 ◽  
Vol 11 (11) ◽  
pp. 1179
Author(s):  
Anastasiya Snezhkina ◽  
Vladislav Pavlov ◽  
Alexey Dmitriev ◽  
Nataliya Melnikova ◽  
Anna Kudryavtseva
Keyword(s):  
Head And Neck ◽  
Neuroendocrine Tumors ◽  
Current Knowledge ◽  
Therapeutic Targets ◽  
The Body ◽  
Diagnostic Dilemma ◽  
Malignancy Risk ◽  
Criteria Of Malignancy ◽  
Head And Neck Paragangliomas ◽  
Potential Biomarkers

Paragangliomas and pheochromocytomas (PPGLs) are rare neuroendocrine tumors originating from paraganglionic tissue in many sites of the body. Most PPGLs are characterized by nonaggressive behavior but all of them have the potential to metastasize. PPGLs represent a great diagnostic dilemma as it is difficult to recognize tumors that are likely to be metastasizing; criteria of malignancy can be found both in benign and metastatic forms. This review aims to analyze the current knowledge of the nature of metastasizing PPGLs paying particular attention to head and neck paragangliomas (HNPGLs). Potential predictors of the malignancy risk for PPGLs were summarized and discussed. These data may also help in the development of diagnostic and prognostic strategies, as well as in the identification of novel potential therapeutic targets for patients with PPGLs.

Recurrence and Progression of Head and Neck Paragangliomas after Treatment

2020 ◽  
Vol 162 (4) ◽  
pp. 504-511
Author(s):  
Kevin J. Contrera ◽  
Valeda Yong ◽  
Chandana A. Reddy ◽  
Sara W. Liu ◽  
Robert R. Lorenz
Keyword(s):  
Head And Neck ◽  
Carotid Body ◽  
Lower Risk ◽  
Care Center ◽  
Tertiary Care ◽  
Cox Proportional Hazards ◽  
Risk Of Recurrence ◽  
Glomus Jugulare ◽  
Carotid Body Tumors ◽  
Head And Neck Paragangliomas

Objective To characterize the recurrence of head and neck paragangliomas and the factors associated with disease progression after treatment. Study Design Retrospective cohort study. Setting Tertiary care center. Subjects and Methods In total, 173 adults with 189 paragangliomas (41.3% carotid body, 29.1% glomus jugulare, 19.0% glomus tympanicum, and 10.6% glomus vagale) treated between 1990 and 2010 were evaluated to determine the incidence and risk of recurrence using Cox proportional hazards. Results The mean (SD) follow-up duration was 8.6 (9.1) years. The incidence was 2.92 recurrences per 100 person-years. The rate of recurrence was 8.2% (95% confidence interval [CI], 3.7-12.7) after 4 years and 17.1% (95% CI, 10.2-24.0) after 10 years. Glomus jugulare tumors were more likely to recur (hazard ratio [HR], 3.69; 95% CI, 1.70-8.01; P < .001) while carotid body tumors were less likely (HR, 0.44; 95% CI, 0.21-0.97; P = .041). Radiation had a lower risk of recurrence or progression compared to surgical excision (HR, 0.30; 95% CI, 0.10-.94; P = .040). Recurrence was associated with right-sided paragangliomas (HR, 3.60; 95% CI, 1.63-7.75; P = .001). The median time to recurrence was 18.4 years. Six (3.2%) patients developed metastasis, which was more common with local recurrence (9.5% vs 1.4%, P = .015). Conclusions Recurrence is more common with glomus jugulare tumors and less common with carotid body tumors. Radiation may have a lower risk of recurrence or progression than surgery for some paraganglioma types. Metastasis is rare but more likely with recurrent disease. Surveillance neck imaging is recommended every several years for decades after treatment.

Multiple glomus tumours

1992 ◽  
Vol 106 (6) ◽  
pp. 538-543 ◽  
Author(s):  
Dimitris G. Balatsouras ◽  
Panayotis N. Eliopoulos ◽  
Costas N. Economou
Keyword(s):  
Head And Neck ◽  
Carotid Body ◽  
Glomus Tumour ◽  
Diagnosis And Management ◽  
Glomus Tympanicum ◽  
Primary Neoplasms ◽  
The Right ◽  
Head And Neck Paragangliomas

AbstractA patient with multiple, synchronous, non-familial head and neck paragangliomas is reported. There were three primary neoplasms, a glomus tympanicum and glomus vagale on the right side and a glomus tumour of the carotid body on the left. Such a combination has never been reported previously.The reports of all the series with paragangliomas in the literature, as well as the reports of single cases with multiple tumours during the last three decades, are reviewed. Specific problems in diagnosis and management of multiple glomus tumours are discussed.

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