Neck Paragangliomas Research Articles

Vagal paraganglioma extending to the brain: a case report with literature review

Introduction: Vagal paraganglioma (VP) is a rare tumor of the vagus nerve (VII). This study aims to present a rare case of VP extending to the brain. Case report: A 35-year-old woman presents with neck swelling for 1 year. On examination, there was an ill-defined hard mass involving the left side of the anterior neck, fixed in all directions. A neck ultrasound revealed an irregular mass attached to both carotid arteries. A computed tomography scan showed a large lobulated, intensely enhanced mass encasing left carotid carotid vessels. Total resection under general anesthesia was done. Histopathologic examination revealed VP. Discussion: VP is the rarest form of paraganglioma and accounts for <5% of all head and neck paragangliomas. The average age of diagnosis for these patients is reported to be 45 years. VP poses a therapeutic challenge due to its anatomic location, and surgery is often associated with a significant rate of postoperative morbidities, particularly loss or impaired vagus nerve function. Conclusion: VP is a rare tumor of the head and neck, even rarer in young adults. A standard management approach is absent for these cases.

Functional recovery after surgical approach of jugulotympanic glomus tumors

Abstract Paragangliomas are tumors of the neuroendocrine system, which arise from the neural crest and chemoreceptor cells. They can be found in the adrenal gland, in the aortic arch, in the jugular bulb or at the level of the cochlear promoter. Glomus tumors grow slowly, are well vascularized, invasive and locally aggressive. Because of their anatomic relation with the jugular bulb, the internal carotid artery, the facial nerve and the cranial nerves IX, X, XI, XII, the patients may develop important complications. Therapeutic options for head and neck paragangliomas are surgical treatment with endovascular embolization, conventional radiotherapy, stereotactic radiosurgery or a combination of these therapeutic methods. In case of paragangliomas, it is essential to take into consideration the size and localization of the tumor, in order to establish the proper therapeutic approach, either conservative, radiotherapy or surgical, due to the risk of postoperative nerve palsies, details that we try to cover in this review. In order to emphasise the importance of correct diagnosis, proper treatment, postoperative complications and postoperative functional recovery in the case of glomus tumors, we present the case of a 35-year-old female who was diagnosed and surgically treated for a right jugulotympanic paraganglioma. The postoperative cranial nerve injuries involved VII, IX and X nerves and the patient followed multiple protocols of functional recovery for the affected nerves. After specific therapy, the patient partially recovered the function of the right facial nerve; deglutition and voice were significantly improved.

Neurological Manifestations of Paragangliomas of the Head and Neck.

This paper will outline the clinical neurologic presentation and diagnostic evaluation of patients with paragangliomas of the head and neck. Contemporary management options will be outlined for these rare and complex tumors. The majority of recent publications and research on these tumors are dedicated to traditional and robotic image-guided radiosurgery in the treatment of head and neck paragangliomas. Paragangliomas are rare, slow-growing tumors of the head and neck which usually cause silent cranial nerve deficits or compensated mild speech or swallowing symptoms. While radiologic surveillance is often the best treatment option, subtotal resection with case-specific radiosurgery is commonly used in patients with large tumors.

Long-Term Outcomes in Head and Neck Paragangliomas Managed with Intensity-Modulated Radiotherapy.

Head & Neck Paragangliomas have been historically relying on surgery mostly, with worsened quality of life and major sequelae. Conventional external radiation therapy seems to offer an equivalent control rate with a low toxicity profile. The aim of this study was to assess the safety and efficiency of intensity-modulated radiation therapy in Head & Neck paragangliomas. This is a retrospective monocentric study conducted in a referral center, including all patients treated with IMRT, whether as an exclusive or post-operative treatment for a tympanic and jugular, carotid, or vagal paraganglioma. Data collection was performed through the manuscript and computerized medical files, including consultation, operative, imaging, pathological analyses, delineation, and treatment planning reports. Success was defined as the complete or partial regression or stabilization without progression, or relapse in accordance with the RECIST criteria. Acute toxicities and long-term sequelae were assessed. Our cohort included 39 patients included between 2011 and 2021: 18 patients treated for a TJ PG (45.9%), 11 patients for a carotid PG (28.4%), and 9 for a vagal PG (23.1%). Twenty-nine patients had IMRT as an exclusive treatment (74.4%), whereas 10 patients had a post-operative complementary treatment (25.6%). Median follow-up in our cohort was 2318 days (average=2200 days, 237-5690, sd=1281.9). Among 39 patients, 37 were successfully controlled with IMRT (94.8%), and the toxicity profile was low without any major toxicity. IMRT seems an ideal treatment, whether exclusive or post-operative for Head & Neck paragangliomas. 3 Laryngoscope, 133:607-614, 2023.

Head and neck paragangliomas: the belfast trust experience

Searchable abstracts of presentations at key conferences in endocrinology ISSN 1470-3947 (print) | ISSN 1479-6848 (online)

Head and Neck Paragangliomas: An Update on the Molecular Classification, State-of-the-Art Imaging, and Management Recommendations.

Paragangliomas are neuroendocrine tumors that derive from paraganglia of the autonomic nervous system, with the majority of parasympathetic paragangliomas arising in the head and neck. More than one-third of all paragangliomas are hereditary, reflecting the strong genetic predisposition of these tumors. The molecular basis of paragangliomas has been investigated extensively in the past couple of decades, leading to the discovery of several molecular clusters and more than 20 well-characterized driver genes (somatic and hereditary), which are more than are known for any other endocrine tumor. Head and neck paragangliomas are largely related to the pseudohypoxia cluster and have been previously excluded from most molecular profiling studies. This review article introduces the molecular classification of paragangliomas, with a focus on head and neck paragangliomas, and discusses its impact on the management of these tumors. Genetic testing is now recommended for all patients with paragangliomas to provide screening and surveillance recommendations for patients and relatives. While CT and MRI provide excellent anatomic characterization of paragangliomas, gallium 68 tetraazacyclododecane tetraacetic acid-octreotate (ie, 68Ga-DOTATATE) has superior sensitivity and is recommended as first-line imaging in patients with head and neck paragangliomas with concern for multifocal and metastatic disease, patients with known multifocal and metastatic disease, and in candidates for targeted peptide-receptor therapy. Keywords: Molecular Imaging, MR Perfusion, MR Spectroscopy, Neuro-Oncology, PET/CT, SPECT/CT, Head/Neck, Genetic Defects © RSNA, 2022.

Somatic Mutation Profiling in Head and Neck Paragangliomas.

ContextHead and neck paragangliomas (HNPGLs) are rare neoplasms with a high degree of heritability. Paragangliomas present as polygenic diseases caused by combined alterations in multiple genes; however, many driver changes remain unknown.ObjectiveThe objective of the study was to analyze somatic mutation profiles in HNPGLs.MethodsWhole-exome sequencing of 42 tumors and matched normal tissues obtained from Russian patients with HNPGLs was carried out. Somatic mutation profiling included variant calling and utilizing MutSig and SigProfiler packages.Results57% of patients harbored germline and somatic variants in paraganglioma (PGL) susceptibility genes or potentially related genes. Somatic variants in novel genes were found in 17% of patients without mutations in any known PGL-related genes. The studied cohort was characterized by 6 significantly mutated genes: SDHD, BCAS4, SLC25A14, RBM3, TP53, and ASCC1, as well as 4 COSMIC single base substitutions (SBS)-96 mutational signatures (SBS5, SBS29, SBS1, and SBS7b). Tumors with germline variants specifically displayed SBS11 and SBS19, when an SBS33-specific mutational signature was identified for cases without those. Beta allele frequency analysis of copy number variations revealed loss of heterozygosity of the wild-type allele in 1 patient with germline mutation c.287-2A>G in the SDHB gene. In patients with germline mutation c.A305G in the SDHD gene, frequent potential loss of chromosome 11 was observed.ConclusionThese results give an understanding of somatic changes and the mutational landscape associated with HNPGLs and are important for the identification of molecular mechanisms involved in tumor development.

A Four-Generational Report on Hereditary Head and Neck Paraganglioma.

BackgroundThis article investigates the inheritance, penetrance, clinical presentation, and therapeutic outcomes of hereditary head and neck paragangliomas (HNPGLs) by offering a four-generational report of an 18-member family affected by this rare condition.MethodologyInformation was compiled by examination of patients and a review of medical records and correspondence (retrospective case series).ResultsSix members of the 18-member family were diagnosed with HNPGL between 2002 and 2018. A known pathogenic point mutation in subunit D of the succinyl dehydrogenase complex (SDHD, c.317G>T, p.Gly106Val) was responsible for the tumor phenotype. The mutation could be revealed in seven family members, three diseased adults, one healthy adult, and three healthy children, out of the nine who consented to gene testing. The median age at diagnosis was 33.5 years (range: 22-50 years). Five of the eight primary tumors were glomus caroticum, two were glomus jugulare, and one was a glomus vagale tumor. The therapeutic approaches were multimodal and included embolization therapy, surgery, radiation, and watchful waiting. Follow-up was reported for five of the six patients (mean follow-up of 34.8 months after primary therapy); three showed no disease progression or recurrence.ConclusionsThis study exemplifies the autosomal dominant, parent-of-origin-dependent inheritance and the high disease penetrance in hereditary paraganglioma-pheochromocytoma syndromes. Six out of a total of eight adult descendants (75%) of the original SDHD mutation carrier developed tumors, and the morbidity associated with the disease as well as its therapy was especially high in late-diagnosed, advanced cases. This substantiates the necessity for early radiologic surveillance and genetic testing.

Paragangliomas da Cabeça e Pescoço: A Experiência de um Centro Oncológico do Sul da Europa

Paragangliomas are usually benign slow-growing tumors, but they are locally invasive and can cause significant morbidity. The aim of this study was to characterize the presenting symptoms, secretory status, genetics, imaging features, treatment modalities, post-treatment complications and survival of patients with head and neck paragangliomas treated at a single institution. We retrospectively reviewed the clinical records of patients managed at our center between 1997 and 2020. Seventy-three patients were included in the study, encompassing 89 head and neck paragangliomas. Forty-eight patients (65.8%) were female and 15 (20.5%) had multiple tumor sites (including 10 patients with multicentric benign paragangliomas and five with disseminated malignant disease). Regarding location, our series encompassed 40 temporal bone paragangliomas (44.9%), 24 carotid body paragangliomas (27%), 22 vagal paragangliomas (24.7%), two laryngeal paragangliomas (2.2%) and one sinonasal paraganglioma (1.1%). Excessive catecholamine secretion was detected in 11 patients (15.1%). Sixty-four patients (87.7%) underwent genetic testing. Of those, 24 (37.5%) exhibited pathogenic succinate dehydrogenase complex germline mutations. Regarding patients who presented with untreated disease, 45 patients (66.2%), encompassing 55 tumors, underwent surgery as primary treatment modality, 20 (29.4%; 23 tumors) were initially treated with radiotherapy and three patients (4.4%, encompassing three solitary tumors) were kept solely under watchful waiting. Five-year overall survival was 94.9% and disease-free survival was 31.9%. Head and neck paragangliomas are rare, slow-growing but locally aggressive tumors resulting in high morbidity but low mortality rates.

Contemporary Radiotherapy for Fisch-Classified Jugulotympanic Paragangliomas - Does Tumor Category Matter? Outcomes From a 13-Year Experience

<h3>Purpose/Objective(s)</h3> Jugulotympanic paragangliomas (JTPGs), recognized as slow-growing tumors, represent a treatment challenge because of their known vascular nature, tendency to be locally invasive, location near many vital structures, and their potential to cause very disabling symptoms. This study aimed to examine the outcomes of patients with Fisch-classified JTPGs treated with conventional radiotherapy including stereotactic radiosurgery. <h3>Materials/Methods</h3> Twenty-four consecutive patients with head and neck paragangliomas diagnosed between November 2003 and May 2017 were identified from the radiation oncology and radiosurgery databases and lists of patients presented for discussion at the weekly Department of Otolaryngology-Head and Neck Surgery multidisciplinary conference. After excluding individuals with carotid body glomus tumors or missing clinical and radiological follow-up information, 13 patients (with a mean age of 61.8 years; 6 glomus tympanicum and 7 glomus jugulare PGs; 10 women and 3 men;) composed the JTPG study participants. Non-invasive stereotactic radiosurgery or external beam radiotherapy was used to treat JTPGs in 11 patients and 2 patients, respectively. The investigation endpoints included subjective and objective responses to treatment (i.e., exhibited symptoms and signs of disease and the tumor) and observed toxicity. <h3>Results</h3> With regard to the risk designation of JTPGs, seven (54%) belonged to the Fisch A-B categories and six (46%) to C-D categories. During a median follow-up of 36 months (range 7-204) after-therapy, subjective responses consisted of eight patients (62%) who were symptom-free, and five individuals (38%) who did not improve. At a median radioimaging follow-up of 34 months (range 6-204), eight (62%) treated neoplasms were stable, two (15%) showed continued tumor growth, and three (23%) were smaller following radiation treatment. One participant (8%) experienced transient otalgia and tinnitus after external beam irradiation; no patient developed radiation oncogenesis. Long-term follow-up (from 75 to 204 months) was documented in six people (46%). Characterization and effects of treatment were not significantly different between the compared Fisch categorized groups of JTPGs. <h3>Conclusion</h3> The observed rates of tumor control (85%) and treatment-related toxicity (8%), and the long-term disease progression-free survival of some patients suggest that contemporary radiotherapy is efficacious and safe when applied for Fisch-classified JTPGs.

Update from the 5th Edition of the World Health Organization Classification of Head and Neck Tumors: Familial Tumor Syndromes.

The initiative of the 5th edition of the WHO classification of the Head and Neck Tumours establishing a new section dedicated to familial/heritable tumor syndromes with tumors and lesions in the head and neck region was much needed to better understand the tumours, diseases, and associated syndromes, as well as establish recommendations for monitoring and treating these patients. (WHO Classification of Tumours Editorial Board. Head and Neck tumours. Lyon (France): International Agency for Research on Cancer; 2022. https://publications.iarc.fr/ ). Within the newly established chapter on genetic tumor syndromes, we have described the main manifestations on the head and neck region in 15 syndromes. This review highlights the important findings within these syndromes, especially on the update on syndromes with tumors involving the head and neck region, as Gorlin syndrome/nevoid basal cell carcinoma syndrome associated with odontogenic keratocysts; Brooke-Spiegler syndrome/familial cylindromatosis and the associated membranous-type salivary gland basal cell adenoma, PTEN hamartoma tumor syndrome/Cowden syndrome with associated facial skin and mucosal lesions and characteristic multinodular thyroid lesions, Von Hippel Lindau syndrome and the associated middle ear endolymphatic sac tumor, as well as the fascinating genetic aspects of the diverse Head and Neck Paragangliomas. We will also discuss hyperparathyroidism-jaw tumor syndrome is characterized by parathyroid tumors in association with fibro-osseous jaw tumors, as well as head and neck desmoid tumors associated with familial adenomatous polyposis with Gardner syndrome variant familial, multicentric head and neck squamous cell carcinoma, tuberous sclerosis and neurofibromatosis type 1-associated head and neck lesions.

Update from the 5th Edition of the World Health Organization Classification of Head and Neck Tumors: Overview of the 2022 WHO Classification of Head and Neck Neuroendocrine Neoplasms.

This review article provides a brief overview of the new WHO classification by adopting a question-answer model to highlight the spectrum of head and neck neuroendocrine neoplasms which includes epithelial neuroendocrine neoplasms (neuroendocrine tumors and neuroendocrine carcinomas) arising from upper aerodigestive tract and salivary glands, and special neuroendocrine neoplasms including middle ear neuroendocrine tumors (MeNET), ectopic or invasive pituitary neuroendocrine tumors (PitNET; formerly known as pituitary adenoma) and Merkel cell carcinoma as well as non-epithelial neuroendocrine neoplasms (paragangliomas). The new WHO classification follows the IARC/WHO nomenclature framework and restricts the diagnostic term of neuroendocrine carcinoma to poorly differentiated epithelial neuroendocrine neoplasms. In this classification, well-differentiated epithelial neuroendocrine neoplasms are termed as neuroendocrine tumors (NET), and are graded as G1 NET (no necrosis and < 2 mitoses per 2mm2; Ki67 < 20%), G2 NET (necrosis or 2-10 mitoses per 2mm2, and Ki67 < 20%) and G3 NET (> 10 mitoses per 2mm2 or Ki67 > 20%, and absence of poorly differentiated cytomorphology). Neuroendocrine carcinomas (> 10 mitoses per 2mm2, Ki67 > 20%, and often associated with a Ki67 > 55%) are further subtyped based on cytomorphological characteristics as small cell and large cell neuroendocrine carcinomas. Unlike neuroendocrine carcinomas, head and neck NETs typically show no aberrant p53 expression or loss of RB reactivity. Ectopic or invasive PitNETs are subtyped using pituitary transcription factors (PIT1, TPIT, SF1, GATA3, ER-alpha), hormones and keratins (e.g., CAM5.2). The new classification emphasizes a strict correlation of morphology and immunohistochemical findings in the accurate diagnosis of neuroendocrine neoplasms. A particular emphasis on the role of biomarkers in the confirmation of the neuroendocrine nature of a neoplasm and in the distinction of various neuroendocrine neoplasms is provided by reviewing ancillary tools that are available to pathologists in the diagnostic workup of head and neck neuroendocrine neoplasms. Furthermore, the role of molecular immunohistochemistry in the diagnostic workup of head and neck paragangliomas is discussed. The unmet needs in the field of head and neck neuroendocrine neoplasms are also discussed in this article. The new WHO classification is an important step forward to ensure accurate diagnosis that will also form the basis of ongoing research in this field.

SDHB knockout and succinate accumulation are insufficient for tumorigenesis but dual SDHB/NF1 loss yields SDHx-like pheochromocytomas.

SUMMARYInherited pathogenic succinate dehydrogenase (SDHx) gene mutations cause the hereditary pheochromocytoma and paraganglioma tumor syndrome. Syndromic tumors exhibit elevated succinate, an oncometabolite that is proposed to drive tumorigenesis via DNA and histone hypermethylation, mitochondrial expansion, and pseudohypoxia-related gene expression. To interrogate this prevailing model, we disrupt mouse adrenal medulla SDHB expression, which recapitulates several key molecular features of human SDHx tumors, including succinate accumulation but not 5hmC loss, HIF accumulation, or tumorigenesis. By contrast, concomitant SDHB and the neurofibromin 1 tumor suppressor disruption yields SDHx-like pheochromocytomas. Unexpectedly, in vivo depletion of the 2-oxoglutarate (2-OG) dioxygenase cofactor ascorbate reduces SDHB-deficient cell survival, indicating that SDHx loss may be better tolerated by tissues with high antioxidant capacity. Contrary to the prevailing oncometabolite model, succinate accumulation and 2-OG-dependent dioxygenase inhibition are insufficient for mouse pheochromocytoma tumorigenesis, which requires additional growth-regulatory pathway activation.

Paragangliomas of the head and neck.

Paragangliomas are rare neuroendocrine tumors that can be found from the skull base to the pelvis. Head and neck paragangliomas have been historically treated with surgery. However, surgical resection adds risk of injury to vascular structures and cranial nerves that can lead to morbidity such as hoarseness, dysarthria, dysphagia, or aspiration. Recently, improved understanding of the behavior of these tumors and increasing experience in non-surgical treatments, such as observation and radiation therapy, have changed the paradigms of management of this entity. Multiple series now show a trend toward a more conservative management, with a higher percentage of patients being observed or treated with radiotherapy. Several factors should be taken into consideration when deciding the most appropriate treatment for head and neck paragangliomas, starting by differentiating carotid body tumors from non-carotid body tumors. In general, surgical resection is normally recommended for carotid body tumors as the complications from treatment are usually minimal. In contrast, for non-carotid body tumors, surgery is often associated with significant functional impairment due to cranial nerve paralysis. As such, non-surgical treatment is now usually recommended for this subset of head and neck paragangliomas. In young patients with no comorbidities and a small to medium carotid body tumors, surgery should be considered. Moreover, surgery should be offered for secreting tumors, malignant tumors, tumors with rapid growth or increase in symptomatology, and when radiotherapy cannot be performed. Conversely, conservative management with active surveillance or radiotherapy can be offered in the remaining cases in order to avoid unnecessary morbidity while still providing acceptable tumor control.

Head/neck paragangliomas: focus on tumor location, mutational status and plasma methoxytyramine.

Head and neck paragangliomas (HNPGLs) are tumors of parasympathetic origin that occur at variable locations and are often secondary to germline mutations in succinate dehydrogenase (SDH) subunit genes. Occasionally, these tumors produce catecholamines. Here, we assessed whether different locations of HNPGLs relate to the presence of SDHx mutations, catecholamine production and other presentations. In this multicenter study, we collected clinical and biochemical data from 244 patients with HNPGLs and 71 patients without HNPGLs. We clarified that jugulotympanic HNPGLs have distinct features. In particular, 88% of jugulotympanic HNPGLs arose in women, among whom only 24% occurred due to SDHx mutations compared to 55% in men. Jugulotympanic HNPGLs were also rarely bilateral, were of a smaller size and were less often metastatic compared to carotid body and vagal HNPGLs. Furthermore, we showed that plasma concentrations of methoxytyramine (MTY) were higher (P < 0.0001) in patients with HNPGL than without HNPGL, whereas plasma normetanephrine did not differ. Only 3.7% of patients showed strong increases in plasma normetanephrine. Plasma MTY was positively related to tumor size but did not relate to the presence of SDHx mutations or tumor location. Our findings confirm that increases in plasma MTY represent the main catecholamine-related biochemical feature of patients with HNPGLs. We expect that more sensitive analytical methods will make biochemical testing of HNPGLs more practical in the future and enable more than the current 30% of patients to be identified with dopamine-producing HNPGLs. The sex-dependent differences in the development of HNPGLs may have relevance to the diagnosis, management and outcomes of these tumors.

Z-scan analysis and theoretical studies of dopamine under physiological conditions

Dopamine (DA) is a widely researched catecholamine best known for its role in motor, motivation, addiction, and reward. Disruption in dopamine homeostasis and signaling within the central nervous system (CNS) can lead to disorders such as attention deficit hyperactivity disorder (ADHD), schizophrenia, Parkinson’s disease, and obsessive–compulsive disorder. In the periphery, circulating DA is stored in blood platelets, and its disruption correlates with pathological conditions such as head and neck paragangliomas, Huntington’s chorea, and schizophrenia. Various methods to sensitively and selectively detect dopamine have been reported, but sparse attempts have been made to exploit its intrinsic properties. Previously, we have harnessed dopamine’s natural mid-ultraviolet auto-fluorescence to carry out its label-free imaging in live brain tissues. Recently, we used the closed-aperture (CA) Z-scan method to provide the first line of evidence on the existence of dopamine nonlinearity. Here, we utilized this simple, sensitive, and straightforward CA Z-scan technique and coupled this with theoretical simulations to further investigate the nonlinear photophysical properties of DA under physiological conditions. Our combined approach revealed that the nonlinear property of dopamine is governed by the thermo-optical effects, and the CA Z-scan profiles can be modulated by parameters such as phase-shift, orders of absorption, and time dependency. Simple and physiologically relevant systems, such as the platelets, are amenable to Z-scan analysis, thereby empowering us to scrutinize in the future if nonlinearity and its alterations, if any, have a direct bearing on DA homeostasis and associated diseases.

Management of jugular tympanic paraganglioma: a case report.

Paragangliomas could be localized from the skull base to the pelvic floor. Tympanic localization represents the most common benign tumor of the middle ear. Diagnosis is based on clinical signs with a great contribution of radiology. A 40-year-old male presented with isolated tinnitus of the right ear evolving for 18 months. Examination revealed a red bulging right-sided tympanic membrane and a conductive hearing loss. Tomodensitometry and Magnetic resonance imagery showed findings in favor of a right jugular tympanic paraganglioma. The tumor was classified type B according to FISCH classification. The patient underwent surgery consisting in tympanotomy using a retro auricular access route. The postoperative course was uneventful. There was no recurrence during the one-year follow-up. Jugular tympanic paraganglioma diagnosis is guided by a combination of epidemiological, clinical and radiological features. Treatment is still not consensual, but surgery still have its indications in localized forms of head and neck paragangliomas (HNP´s).

Carotid body tumors: case series of extremely rare head and neck paragangliomas

Carotid body tumors, also commonly known as nonchromaffin paragangliomas and chemodectomas, is a slowly growing neoplasm originating from carotid body chemoreceptors. Herein we have presented a case series of surgical treatment of patients with carotid body tumors in National Scientific Center of Surgery named after AN Syzganov (2009-2020). Total number of patients was 10 with 11 neck mass, of which 7 (70%) were women, the average age was 47 (31-73) years, one case we faced with a bilateral location. The main complaint was slow-growing neck mass. On computed tomography angiography most of cases (70%) were of the 3th type according to the Shamblin grade with average size of 5.9 (4-8) cm. The main treatment was surgical excision in all cases. Mean duration of surgery 111.5 (75-190) min and hospital stay days 12.1(8-20) were registered. Total number of complications were 2 (20%), where in 1 case surgery complicated by bleeding more than 500 ml, and 1 patient presented dysphonia after the surgery, which resolved by time. Duration of surgery and hospital stay days were directly associated with size of mass and distance to base of skull. Pathology results showed that two patients (20%) had malignant cells, one of them with metastases to the nearest lymph nodes. Surgical resection is the treatment of choice for carotid body tumors. The large size of the masses and involvement of the carotid arteries and cranial nerves in the process directly affect the surgical approach and increase the risk of complications.

Local Tumor Behavior Associated With Survival Among Patients With Paraganglioma of the Head and Neck.

ObjectiveThe purpose of this study was to evaluate the utility of ICD-O-3–classified local tumor behavior as a prognosticator of head and neck paraganglioma (HNP) outcomes.Study DesignRetrospective cohort study.SettingNational Cancer Database between 2004 and 2016.MethodsThis study included patients aged ≥18 years who were diagnosed with HNP. Clinical outcomes and clinicopathologic features were compared with regard to local tumor behavior.ResultsOur study included 525 patients, of which the majority had HNP classified as locally invasive (45.9%) or borderline (37.9%). The most common anatomic sites involved were the carotid body (33.7%), intracranial regions (29.0%), or cranial nerves (25.5%). Carotid body tumors were exclusively locally invasive, whereas intracranial and cranial nerve HNP were overwhelmingly benign or borderline (94% and 91%, respectively). One-fourth of patients underwent pathologic analysis of regional lymph nodes, of which the majority were positive for metastasis (80.6%). Metastasis to distant organs was twice as common in patients with locally invasive tumors vs benign (15% vs 7.1). For benign disease, surgery with radiotherapy (adjusted hazard ratio [aHR], 40.45; P = .006) and active surveillance (aHR, 24.23; P = .008) were associated with worse survival when compared with surgery alone. For locally invasive tumors, greater age (aHR, 1.07; P < .0001) and positive surgical margins (aHR, 4.13; P = .010) were predictors of worse survival, while combined surgery and radiotherapy were predictors of improved survival vs surgery alone (aHR, 0.31; P = .027).ConclusionWhile criteria for tumor behavior could not be defined, our results suggest that such a classification system could be used to enhance HNP risk stratification and guide clinical management decisions.

Treatment decision and estimation of growth of head and neck paragangliomas

PurposeHead and neck paragangliomas are slow growing tumors where observation has become more widely accepted. Tumor growth rate as well as predictors of increased tumor growth were analyzed with the goal to identify factors to better predict disease progression and counsel patients. Materials and methodsMulti-institutional retrospective cohort study from 2011 to 2020. Results130 head and neck paragangliomas in 125 patients were analyzed. 38 were observed (30.4%), 16 radiated (12.8%), and 71 underwent surgery (56.8%). Surgical patients were significantly younger (p = 0.038) and with more genetically mediated paragangliomas (p = 0.026). Significantly more patients were asymptomatic in the observation group (p = 0.005). Of the 39 observed tumors, 43.6% (n = 17) grew with a tumor doubling time of 5.67 years. More than half of the observed paragangliomas had no growth. When examining symptoms postoperatively and at follow-up, the surgical cohort had significantly more worsening symptoms (p = 0.007) and new cranial neuropathies (p = 0.031). ConclusionsHead and neck paragangliomas have slow growth rates if they grow at all. Patients in the surgical cohort had more clinical symptoms at presentation and worsening postoperative symptoms.