Head-and-neck paragangliomas: An overview of 54 cases operated at a tertiary care center

Singh, Shruti; Madan, Renu; Singh, Manoj Kumar; Thakar, Alok; Sharma, Suresh C.

doi:10.4103/sajc.sajc_339_18

Cited by 15 publications

(18 citation statements)

References 22 publications

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“…It is well known that HNPGLs occur more frequently in women than men ( Erickson et al 2001 , Smith et al 2017 , Rijken et al 2019 , Singh et al 2019 , Rana et al 2021 ) and this was also reflected in our cohort. We systematically analyzed clinical features of HNPGLs and demonstrated that tumors of jugular and tympanic origin are more common in women (88%) than men.…”

Section: Discussionsupporting

confidence: 76%

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

Richter

Qiu

Ghering

et al. 2022

Endocrine-Related Cancer

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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 presence of SDHx mutations, catecholamine production and other presentations. In this multicenter study, we collected clinical and biochemical data from 244 patients with 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 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 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.

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Section: Discussionsupporting

confidence: 76%

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

Richter

Qiu

Ghering

et al. 2022

Endocrine-Related Cancer

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“…Surgical resection represents the mainstay of treatment for HNP and is associated with the highest rates of local control. 19 , 20 However, due to the morbidity associated with surgery, recent studies have suggested that primary radiotherapy may provide similar local tumor control with reduced morbidity. 21 - 23 Others have suggested that conservative management with serial imaging and close follow-up may be preferred for patients with advanced age, multiple comorbidities, small asymptomatic tumors, or cases in which surgical resection carries high morbidity.…”

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confidence: 99%

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

et al. 2022

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Objective The 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 Design Retrospective cohort study. Setting National Cancer Database between 2004 and 2016. Methods This study included patients aged ≥18 years who were diagnosed with HNP. Clinical outcomes and clinicopathologic features were compared with regard to local tumor behavior. Results Our 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). Conclusion While 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.

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“…Carotid body tumors are the most frequent head and neck paragangliomas, although they are rare when all head and neck tumors are considered. 3,4 Their definitive treatment is surgery in expense of neurovascular complications. 5 The efficacy of angiography and embolization before CBT surgery was first reported by Schick et al 16 The aim of embolization is to control arterial supply of the tumor and by this way to decrease blood loss, to shorten the operation time, and to decrease overall surgical complications.…”

Section: Discussionmentioning

confidence: 99%

“…2 Head and neck paragangliomas constitutes less than 0.5% of all head and neck tumors and approximately 0.03% of all human tumors. 3 Head and neck paragangliomas are named according to their site of origin (eg, carotid body, jugular, vagal, tympanic, etc), and carotid body tumor (CBT) is the most frequent head and neck paraganglioma with a rate of 60% to 78%. 4 Although being mostly benign, malignancy rate of 6% to 10% is reported for CBT.…”

Section: Introductionmentioning

confidence: 99%

Is Preoperative Embolization Necessary for Carotid Paraganglioma Resection: Experience of a Tertiary Center

et al. 2020

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Purpose: This study was designed to investigate whether preoperative embolization is a useful procedure to decrease blood loss and neurovascular complications for carotid body tumor (CBT) surgery or not. Methods: Medical records of our tertiary care center from 2012 to 2019 were scanned for patients who underwent surgery for CBT, retrospectively. Age, gender, complaint and head and neck examination findings at the time of presentation, preoperative complete blood count parameters, imaging records (cervical magnetic resonance imaging and carotid artery angiography), Shamblin classification, tumor size, intraoperative findings, and postoperative complications were noted. Results: A total of 26 patients were operated due to CBT between 2012 and 2019 in our clinic; preoperative arterial embolization was performed to 15 (57.7%) patients, and 11 (42.3%) patients were operated without embolization. Youngest patient was 24 years old, while oldest was 69 years and mean age was 44.35 ± 12.73. (embolization group: ages ranging between 24 and 64 with a mean of 41.5 ± 11.02 years; in nonembolization group: ages ranging between 26 and 69 with a mean of 48.1 ± 14.3). Embolization status was not significantly related to cranial nerve injury, vascular injury, overall complications, and hematocrit decrease. Arterial injury is more likely to occur with increasing Shamblin class ( r = .39; P = .04). Tumor size is not found to be significantly related to cranial nerve injury, vascular injury, overall complications, and hematocrit decrease, but cranial nerve injury and vascular injury were more likely to occur in large tumors ( r = .34; P = .089 and r = .34; P = .087, respectively). Age was significantly and negatively correlated to vascular injury ( r = −.51; P = .05). Vascular injury was significantly correlated with gender (male predominance: r = −.64; P = .000). Conclusion: Although preoperative arterial embolization is considered to attenuate the complication risk, we found that there was no significant difference among the patients with or without embolization.

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Head-and-neck paragangliomas: An overview of 54 cases operated at a tertiary care center

Cited by 15 publications

References 22 publications

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

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

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

Is Preoperative Embolization Necessary for Carotid Paraganglioma Resection: Experience of a Tertiary Center

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