Cervical Paragangliomas Research Articles

Diagnosis and management of cervical paragangliomas: a report of six cases

Diagnosis and management of cervical paragangliomas: a report of six cases

Preoperative Embolization in Surgical Resection of Cervical Paragangliomas: Usefulness and Current Evidence.

Cervical paragangliomas (CPG) are slow-growing tumours, most of which are highly vascular, making surgical resection challenging. Preoperative embolisation of afferent arteries has been recommended to facilitate operative dissection, reduce perioperative blood loss, and shorten the duration of the operation. However, there is conflicting evidence on the benefits of preoperative embolisation on surgical outcomes, operative time, and bleeding. The objective of this study was to compare the perioperative parameters and outcomes like duration of surgery, blood transfusion, duration of stay in the ICU, cranial nerve injuries, and postoperative stroke between patients who underwent excision of CPGs with and without preoperative embolisation. This is a retrospective study conducted at the Division of Vascular Surgery, Sree Chitra Tirunal Institute of Medical Sciences and Technology, Thiruvananthapuram, Kerala, India. The study included a review of the medical records of 32 patients who underwent excision of CPGs.Patients who underwent surgical resection without preoperative embolisation (SR) were compared with those who underwent surgical resection with embolisation (SREMB). Statistical analysis was done in R statistical software (R Foundation for Statistical Computing, Vienna, Austria). Categorical variables were reported in absolute numbers and percentages continuous variables were compared with an unpaired Mann-Whitney U test. The chi-square test was used to compare the categorical data. Out of 32 patients included in this study, 13 (40.6%) patients were included in the SREMB group. Between the two groups, there were no significant differences in operative time (120 vs. 150 minutes; p = 0.59), blood transfusion requirement (0.69 vs. 0.37 units; p = 0.39) and ICU stays (1 vs. 1; p = 0.56).Postoperative cranial nerve injury was significantly more in the SREMB group (6 vs 1; p = 0.01) but stroke (0 vs 2; p = 0.50) was not statistically significant between the two groups. In this study, we found that there was insufficient evidence to support routine preoperative embolisation in CPG.Hence, preoperative embolisation should only be used in a very select group of patients.

Cervical paragangliomas: clinical presentation and Managment modalities

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

Bilateral Carotid Paraganglioma: Case Report; Successful Surgical Excision, Radiological Image and Review of the Literature

Introduction: Carotid body tumors are rare neoplasms that generally present as asymptomatic slow-growing masses in the neck, commonly called cervical paragangliomas, highly vascularized whose cells originate from the embryonic neural crest, carotid glomus tumor is uncommon, representing 1 out of every 30,000 head and neck tumors, they are located in the adventitia of the vessels, they usually grow surrounding the arteries of the carotid bifurcation and the nerves that surround it, in Ecuador the prevalence is at 55 years of age with greater frequency in women [1]. Clinical Case: A 33-year-old female patient with no significant clinical history, presented with a painful hard mass in the right cervical region that progressively increased in size, for the past 6 months. On physical examination, a tumor in the right cervical region of approximately 5 centimeters in diameter was found, with a hard consistency attached to deep planes, for which Eco Doppler and AngioTac of the neck were performed, which reported bilateral carotid paraganglioma. Excision of the glomus was necessary due to its size right carotid artery, surgery using a right cervico-lateral approach, identifying the vascular tumor mass of 4.8 x 2.9 x 2.8 cm in diameter, which was located in the carotid bifurcation. Removal of Shamblin II right carotid body paraganglioma by temporary clamping of the common carotid artery and the digital dissection method, there was no intraoperative massive bleeding or cranial nerve deficits in the postoperative period with favorable evolution, surgical wound in good condition during follow-up in outpatient consultation. Conclusion: Glomus carotid tumors are generally benign and diagnosed late. Imaging studies are essential for its diagnosis and staging.

Rare Carotid Bifurcation Tumor

Introduction: Paraganglioma or chemodectoma of the carotid corpuscle or carotid glomus is a relatively rare neoplasm of slow progression, late diagnosis and uncertain prognosis. This tumor originates from the chemoreceptors of the carotid bifurcation and clinically presents as a laterocervical mass. It represents the majority of paragangliomas of the head and neck (60-70%). Observation: We report the observation of a 56-year-old female patient who consulted for a right laterocervical swelling without any other associated clinical signs. A complete radiological workup (Angioscanner/Angiography) will confirm the vascular nature of the lesion, characterize the size of the mass, its nature, its mapping, and its relationship to adjacent organs. Surgical excision had confirmed the diagnosis on histopathological examination. From this observation, we review the clinical, radiological and histopathological features of this tumor, as well as its uncertain evolutionary mode and the therapeutic modalities. Conclusion: Lateral cervical paragangliomas remain rare and poorly known affections which require an early diagnosis and a multidisciplinary management: ENT surgeon, vascular surgeon, oncologist and radiotherapist according to the evolutive forms.

Giant carotid chemodectoma treated with a combination of surgery and CyberKnife radiotherapy: a case report and review of the literature

BackgroundParagangliomas are rare vascular neuroendocrine tumors that develop in the extra-adrenal paraganglion tissue. They occur most commonly at the carotid bifurcation, where they are known as carotid body tumors. Most paragangliomas are benign, locally aggressive, infiltrative tumors. Approximately 10% of patients with paragangliomas develop distant metastases, 10% present with multiple or bilateral tumors (mostly carotid body tumors), and 10% have a family history of paragangliomas. The malignant transformation of carotid body tumors has been reported in 6% of cases.Case presentationWe present the case of a 64 year-old Caucasian woman with a gigantic glomic tumor mass in the neck. Twenty years before the consultation, the patient had undergone an unsuccessful attempt to remove the mass. Over the last 3 years, the patient had felt enlargement of the mass at an increased rate, almost doubling the prior size. Angio magnetic resonance imaging showed a 9 cm paratracheal mass on the left cervical side that laterally displaced the sternocleidomastoid muscle and 2 c m of the trachea. Due to the change in the tumor behavior, the maxillofacial team at Ruber International Hospital decided to remove the tumor surgically after embolization. During the surgery the tumor was gently dissected from the carotid an removed from the carotid bifurcation uneventfully. Two small nodes adhering tightly to the internal carotid adventitia and the posterior torn hole were left in place to avoid any potentially life-threatening complications. The final biopsy confirmed the initial diagnosis of carotid body paraganglioma and showed a Ki-67 expression of 19%. Due to the aggressive growth behavior and high Ki-67 expression of the tumor, the patient was referred to the CyberKnife Unit of Ruber International Hospital for treatment of the remaining nodes.ConclusionsThe management of cervical paragangliomas is difficult and remains a challenge. Although the likelihood of tumor control is high with surgical or radiotherapeutic treatments, we currently lack consensus regarding the best treatment option. Nevertheless, in selected complex cases, such as the case we present, the combination of surgery and radiosurgery may allow complete local tumor control with minimal morbidity.

Head and neck paragangliomas: Ten years of experience in a third health center. A cohort study

Head and neck paragangliomas are rare vascular tumors derived from the paraganglionic system, located at the carotid body, jugular vein, tympanic cavity and vagal nerve.From 2010 to 2020, a cohort of 26 patients divided in two groups, 15 with cervical paragangliomas and 11 with temporal bone paragangliomas, was reviewed by analysing the medical history, the epidemiological and clinical parameters, the imaging results and classification, the modality of treatment and outcome.Cervical paragangliomas present as firm and pulsatile mass with the characteristic aspect of “salt and pepper” on MRI T1 weighted sequences. The most common type on Shamblin classification was the type II. Total surgical resection was performed in 93,33% of cases. The sensitivity of MRI in the diagnosis of vagal paragangliomas was up to 75%, with a specificity of 90,91% and the correlation of the MRI results and the findings of surgical exploration is significant with p ⩽ 0.02.Temporal bone paragangliomas appear as pulsatile mass behind the tympanic membrane, causing variable hearing loss in 90,90% of the cases. The facial nerve is the most frequently affected cranial nerve, in 36,36% of the cases. The main type according to FISH classification is the type B. Embolization was performed in all type C tumors. Surgery was the first line treatment while the inoperable patients were considered for radiotherapy.The aim of this study is to report the main clinical features of head and neck paragangliomas, the imaging tools and findings evaluating their sensitivity and specificity and the treatment protocol and outcome.

Golden-Angle Radial Sparse Parallel (GRASP) MRI differentiates head & neck paragangliomas from schwannomas

PurposeThe aim of this study was to demonstrate the value of DCE MRI with high spatiotemporal resolution (GRASP) for differentiating paragangliomas and schwannomas in the head and neck. MethodsIn a retrospective PACS search of in total 410 patients who had undergone head & neck GRASP-MRI, we identified 6 patients with biopsy proven cervical paragangliomas (n = 3) and schwannomas (n = 3). Conventional MRI features were evaluated, lesion size was determined. Postprocessing in 4D-GRASP datasets was performed (1) based on reconstructions with a temporal resolution (Tres) of 4.1 s, qualitative time-intensity curve classification and semiquantitative parameter (Tpeak, PH, ERmax and Slopemax) analysis, and (2) voxel-based mapping and qualitative and semiquantitative perfusion modeling based on reconstructions with a Tres of 1.6 s. Additionally, GRASP perfusion analysis was performed in another set of 5 patients with presumed cervical paragangliomas (n = 3) and schwannomas (n = 2) based on conventional imaging criteria and was correlated with conventional imaging findings. Due to the small sample size, both groups were compared qualitatively. ResultsIn the time intensity curve classification of 4D GRASP reconstructions (Tres 4.1 s), biopsy proven paragangliomas were consistently characterized by a type-III rapid inflow wash-out pattern, compared to a type-I inflow pattern in the schwannoma group. In both temporal resolutions, semiquantitative analysis of time intensity curves demonstrated rapid wash-in, wash-out, and higher peak signal intensities in paragangliomas compared to schwannomas. In 5 presumed (non-biopsy-proven) paragangliomas and schwannomas, time intensity curves improved diagnostic certainty. ConclusionsVisual time intensity curve classification and semi-quantitative analysis of GRASP-MRI were, in this small retrospective series, sufficient to differentiate cervical paragangliomas from schwannomas. Utilization of this technique may further improve diagnostic confidence in lesions lacking conventional imaging features.

Surgical treatment options for cervical paragangliomas

The therapies available for the rare tumor entity of cervical paraganglioma (PG) are currently undergoing aparadigm shift. The treatment of choice for small carotid body tumors, malignant and active endocrine tumors is surgical resection; however, for locally advanced carotid body tumors and vagal PG, surgical therapy should be critically evaluated. Due to the immediate proximity of these hypervascularized tumors to the caudal cranial nerves, there is a risk of severe nerve damage with a significant impairment of quality of life after resection, particularly for locally advanced cervical PG, emphasizing further the importance of arestrictive surgical strategy. External radiotherapy can provide an equivalent primary therapeutic option with respect to the rate of recurrence and is accompanied by alower morbidity. The slow rate of tumor progression and the multifocality of the familial variant of cervical PG or significant comorbidities in older, asymptomatic patients warrant aless aggressive treatment strategy for these tumors. When await and scan approach is implemented, aclosely monitored radiological and clinical re-evaluation is of upmost importance. In amultidisciplinary approach the following critical points require consideration before atherapy is implemented,: size and location of the tumor, progression rate, genetic background, patient age and general condition, relevant comorbidities, the presence of synchronous PG and/or vasoactive catecholamine-producing tumors. Although best practice algorithms for the treatment of cervical PG have already been devised, recent innovative developments have led to more patient-tailored, individualized treatment approaches.

Cervical paragangliomas: experience of 114 cases in 14 years

Introduction and objectiveTo report a single center experience with carotid body paraganglioma cases that were treated by the same surgeon in a city with high prevalence of paragangliomas due to high altitude. MethodsWe retrospectively investigated the demographic, clinicopathological and radiological data of 104 patients diagnosed with cervical paragangliomas between 2003 and 2017. The patients were classified according to the Shamblin classification. ResultsIn this study a total of 104 patients (33 male and 71 female, with a mean age of 54.6±13 years) diagnosed with cervical paragangliomas located on carotid bifurcation between 2003 and 2017 were included. Among those patients, 10 presented with bilateral tumors and in total, 114 paragangliomas were managed in this period. The mean diameter of the tumors was 5.12±1.45cm. Malignant tumor was determined in only one (0.9%) patient. All patients were operated. In 12 patients with the tumor diameter larger than 5cm, preoperative coil embolization was achieved. In 14 patients, preoperative angiographic embolization was employed and in 4 patients intraoperative sclerosing agent injections were performed. Facial paralysis was observed in 2 patients and dysphagia was present in 1 patient, Horner syndrome was seen in 1 patient and hoarseness was reported in 7 patients after operation. All those complications improved during follow-up. Mortality was not reported in any cases. ConclusionSurgery is the definitive treatment for patients with cervical paragangliomas. Although, it may be difficult in patients with the advanced Shamblin types, in experienced hands, complication rates are very low.

EP-1174: Outcomes in patients with cervical paragangliomas treated with intensity-modulated radiotherapy

EP-1174: Outcomes in patients with cervical paragangliomas treated with intensity-modulated radiotherapy

Surgical treatment of cervical paragangliomas

To present own experience in surgical treatment of cervical paragangliomas. 9 patients with cervical paragangliomas were enrolled. Endovascular embolization of supplying vessels followed by maximally radical surgery and subsequent carotid arteries repair involved into tumoral process reduces the risk of complications. The most careful attitude to caudal group of cranial nerves adjacent or involved into neoplasm allows to minimize postoperative neurological complications.

Surgical Management of Carotid Body Tumors: A 15-Year Single Institution Experience Employing an Interdisciplinary Approach

Cervical paragangliomas are rare neoplasms that arise from extraadrenal paraganglia in close association with the cranial nerves and extracranial arterial system of the head and neck, and therefore surgical extirpation can be challenging. A retrospective study was conducted of all patients undergoing surgical excision of a cervical paraganglioma between 2000 and 2015. The demographic characteristics, clinical features, surgical approach, and outcomes were reviewed. A total of 20 cervical paragangliomas were excised in 17 patients. There were 14 female and 3 male patients with a mean age of 56.6 ± 17.0 at the time of operation. Twelve patients had unilateral tumors and 5 patients had bilateral tumors. Familial involvement was confirmed by history or direct genetic analysis in 8 (47%) of the 17 patients. There were no malignant paragangliomas, and only 3 patients had tumors that were determined to be functional. Tumor size ranged from 1.3 to 6.0 cm. Two patients required combined arterial resection as part of complete excision of the tumor. There were no permanent operative cranial nerve injuries, no recurrences, minimal morbidity, and no mortality. In conclusion, optimal management of cervical paragangliomas should include a thorough preoperative evaluation, accurate definition of the surgical anatomy, and exclusion of synchronous paragangliomas. A combined therapeutic approach by a multidisciplinary team including surgeons and interventional radiologists provides safe and effective management of cervical paragangliomas with very low morbidity and excellent outcomes.

Cervical Paraganglioma Mimicking Thyroid Nodule: A Rare Clinical Case.

Objective. Paraganglioma is a rare neuroendocrine tumor. When it is located in the neck, it is commonly misdiagnosed as other thyroid neoplasms. Case Report. We report a case of cervical paraganglioma in a 55-year-old female. Patient was admitted to our clinic with goiter and neck pain. Thyroid ultrasonography revealed a 20 mm solitary, heterogeneous nodule located in the upper pole of left thyroid lobe. Fine needle aspiration cytology was nondiagnostic. She underwent left lobectomy and histopathology showed paraganglioma. Discussion. Cervical paragangliomas should be considered in the differential diagnosis of thyroid nodules.

Treatment of cervical paragangliomas: Is surgery the only way?

PurposeTo analyze the results after surgery or stereotactic radiotherapy (SRT) in the treatment of cervical paragangliomas. Against this background, the decision-making algorithm used in the treatment of carotid body tumors (CBTs) and vagal paragangliomas (VPs) was reevaluated relative to the existing literature on the topic. Materials and methodsRetrospective study between 2000 and 2012. A total of 27 CBTs and nine VPs in 32 patients were treated. Shamblin class I: 59.3% (n=16); class II: 29.6% (n=8); class III: 11.1% (n=3). Treatment modalities were surgery, radiotherapy, or observation. The end points for analysis were long-term tumor control and integrity of the cranial nerves. Results21 CBTs and seven VPs underwent surgery; SRT was performed in three CBTs and two VPs. Three CBTs were clinically observed. Permanent nerve paresis followed after surgery for CBTs in five patients (20%) and in all patients with VPs. No impaired cranial nerve function resulted after SRT. The median follow-up period was 4.7years. The tumor control rate after therapy for CBTs and VPs was 100%. One CBT that received clinical observation showed slow tumor progression. ConclusionsA surgical procedure should be regarded as the treatment of choice in patients with small CBTs. In larger CBTs, particularly in elderly patients with unimpaired cranial nerves, radical surgery should be regarded critically. As surgery for VPs caused regularly impairment of cranial nerves with functional disturbances of various degrees a comprehensive consultation with the patient is mandatory and nonsurgical strategies should be discussed.

Zespół Verneta w przebiegu wieloogniskowego przyzwojaka szyjnego – opis przypadku

Cervical paragangliomas are rare tumors of the neuroectodermal origin. Extensive paragangliomas press on the regional bone and neurovascular structures. 90-years-old woman was admitted to our department with sinister deafness and smooth painless tumor in the left external auditory meatus. The patient has suffered from sinister tinnitus and progresive sinister hypacusis for 11 years, hoarseness for 9 years, dysphagia for 4 years and the left humeral articulation's mobility dysfunction for 1 year. In diagnostic imaging two good vascularized tumors were shown in the left jugular foramen and in the left carotid bifurcation. We diagnosed the sinister glossopharyngeal, vagus and accessory nerves paralysis in multifocal cervical paraganglioma. Authors concluded that in a case of the one side hypacusis and the ipsilateral lower cranial nerve lesion the temporal bones CT shuold be the first step in the diagnostic process. The characteristic clinical picture of cervical paragangliomas permits diagnose them without histopathology.

Treatment of Cervical and Cranial Base Paragangliomas With Conformal Fractionated Radiation Therapy: A 25-year Experience in a Single Institution

Treatment of Cervical and Cranial Base Paragangliomas With Conformal Fractionated Radiation Therapy: A 25-year Experience in a Single Institution

Treatment of cervical paragangliomas

Review of the treatment of cervical paraganglioma. Review of the literature based on a Medline database. The treatment of choice consists of a multidisciplinary approach based on a detailed clinical, laboratory and radiological work-up. Vascular reconstruction may be necessary when the tumour invades the artery wall. The main complication of surgery is damage to cranial nerves involved in speech and/or swallowing. Treatment of bilateral tumours must be conducted in two stages. The first side to be operated depends on the sites and size of the tumours, as the primary objective is to avoid bilateral vagus nerve palsy. Radiotherapy has been used to treat paragangliomas for several years and achieves tumour stabilization in the majority of cases. Potential indications reported in the literature are: inoperable tumours, recurrence after surgery, some bilateral tumours and malignant tumours. Surgery is the standard treatment for cervical paraganglioma. Radiotherapy can be proposed when surgery is contraindicated.

Impact of preoperative embolization on outcomes of carotid body tumor resections

This study assessed neurovascular complications in the surgical management of carotid body tumors (CBTs), with emphasis on those treated with and without preoperative embolization. We reviewed the clinical data of all consecutive patients with CBTs treated by surgical resection at our institution from 1985 to 2010. Outcomes were compared between Shamblin class II and III CBTs treated with preoperative embolization (EMB group) and with no preoperative embolization (NEMB group). A total of 131 patients (80 women, 51 men), who were aged 48 years (range, 16-84 years), had resection of 144 CBTs and 12 concurrent cervical paragangliomas. This included 18 patients who had bilateral resections and 29 with familial CBTs. Succinate dehydrogenase (SDHx) mutations were confirmed in 17 patients. Mean tumor volume was 20.5 cm(3) (range, 0.8-101.3 cm(3)), and there were two biochemically active CBTs (1%). There were 71 Shamblin II and 33 Shamblin III. The EMB group underwent 33 CBT resections, and the NEMB group underwent 71. There were more patients in the EMB group with bilateral (48% vs 22%; P = .01) and familial (34% vs 14%; P = .01) CBT; otherwise, patient demographics, Shamblin class, and tumor diameter and volumes were similar. No strokes or other major complications occurred after preoperative embolization with polyvinyl alcohol particles 1 day before surgery. The EMB group required less extensive procedures (simple excision in 97% vs 82%, P = .03; internal carotid artery clamping in 15% vs 37%, P = .04) and had less blood loss (mean estimated blood loss, 263 vs 599 mL; P = .002) than the NEMB group. However, there were no significant differences in operative time (250 vs 265 minutes; P = .49), temporary cranial nerve injury (52% vs 38%; P = .21), clinically apparent cranial nerve deficits after 1 year (12% vs 7%; P = .46), deaths (0% vs 0%; P > .99), stroke (0% vs 1%; P > .99), or postoperative length of stay (4.1 vs 4.2 days; P = .91). Large CBTs can be resected safely with or without preoperative embolization. Preoperative embolization may simplify the conduct of the operation and reduce blood loss but does not decrease rates of cranial nerve injury, although most are temporary.

Natural History of Cervical Paragangliomas<subtitle>Outcomes of Observation of 43 Patients</subtitle><alt-title>Natural History of Cervical Paragangliomas</alt-title>

To evaluate the outcomes, growth rate, and symptoms of nonoperated cervical paragangliomas. Retrospective review of clinical and radiologic records and images. Tertiary academic medical center. We studied all patients presenting with cervical paragangliomas between 1993 and 2010 who were observed rather than operated on. Growth of tumors and need for surgical removal. Forty-three patients with 47 tumors were identified. Mean patient age was 56 years (age range, 17-86 years). Thirty patients were asymptomatic (70%) (22 diagnosed by imaging for other reasons and 8 for workup of a neck mass), and only 5 presented with cranial nerve abnormalities (12%). No patients presented with lymphadenopathy, rapid growth, or pain. Reasons for observation were patient preference (n=15; 35%), advanced age of patient (n=12; 28%), and preexisting contralateral cranial nerve deficits (n=11; 26%). Twenty-eight of the 47 tumors were suspected carotid body tumors based on imaging (60%), and 19 were suspected vagal tumors (40%). The mean greatest dimension at presentation was 2.6 cm (range, 1-7.2 cm). During a mean follow-up of 5 years (range, 1-17 years), 19 tumors remained stable in size (42%); 17 grew (38%); and 9 regressed (20%). Of the 17 tumors that grew, the mean growth was 0.2 cm/y. Observation of cervical paragangliomas is an option in selected patients who do not present with worrisome symptoms. The natural history of paragangliomas in these patients is typically little to no growth over time. Regular follow-up is important to ensure minimal change and stable symptoms.