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Abstract: We present a case involving a 9-year-old boy diagnosed with metastatic carotid body paraganglioma. The metastases were detected in cervical lymph nodes and lungs using 68 Ga-DOTANOC PET/CT imaging. The patient received peptide receptor radionuclide therapy with 177 Lu-DOTATATE. Following 3 treatment cycles, a significant improvement was observed in the metastatic lesions. After 4 cycles, the patient achieved a complete response, with a cumulative administered activity of 16.65 GBq during the therapy. This case underscores the effectiveness of using 177 Lu-DOTATATE in managing metastatic carotid body paraganglioma, offering promising results in terms of tumor regression and overall therapeutic response., Competing Interests: Conflicts of interest and sources of funding: none declared., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

A highly vascular glomus tumor that develops from the paraganglion cells of the carotid body is called a carotid body tumor (CBT), also known as a chemodectoma or carotid body paraganglioma (CBP). It is situated near the carotid bifurcation, where the external and internal carotid arteries splay out characteristically. We present a case of a 30-year-old woman who had a slightly tender, slightly pulsatile, and slightly ballotable swelling over the lateral aspect of the neck on the right side. The surgical resection of the tumor was done based on the diagnosis made on clinical-radiological investigations as a carotid body tumor further confirmed by a histopathological study. We also provide a summary of the research on carotid body tumors clinical and imaging manifestations, assessment, and therapy., Competing Interests: The authors declare no competing interests., (Copyright: Swaragandha Shivaji Jadhav et al.)

Tumour to tumour metastases are uncommon, and we report a case of carotid body paraganglioma metastatic to a hepatocellular adenoma. A 54-year-old man presented after a CT chest for chronic cough that incidentally identified two liver lesions in segment 3 and caudate. The imaging findings were suspicious for atypical haemangiomas versus hepatocellular adenoma. The segment 3 lesion was biopsied, demonstrating beta-catenin activated hepatocellular adenoma. He underwent partial hepatectomy with pathology showing the beta-catenin activated hepatocellular adenoma contained a central area of paraganglioma. On closer review, the patient revealed a carotid body paraganglioma with lymph node metastases requiring resection 24 years earlier. He subsequently underwent left hepatectomy including the resection bed and caudate, which confirmed the caudate lesion as metastatic paraganglioma. This case demonstrates how paraganglioma can metastasise to liver decades after initial resection and provide insight into the diagnostic workup for hepatocellular adenoma with neuroendocrine features., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Paragangliomas 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 Presentation: We 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., Conclusions: The 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., (© 2022. The Author(s).)

Introduction and Objective: To 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., Methods: We 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., Results: In 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., Conclusion: Surgery 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., (Copyright © 2018. Published by Elsevier Editora Ltda.)

Carotid Body Tumor i.e. Paraganglioma is a challenging entity from the point of multidisciplinary diagnosis. The main treatment option i.e. surgery yields intraoperative risk,related to cranial nerve palsy and vascular morbidity.Bifurcation of Common Carotid Artery especially at the Carotid Body is the place where Head and Neck Paraganglioma is most frequently seen i.e. 60% of incidence [19]. Indeed, the knowledge of genetic germline SDH mutations, which cause deregulation of hypoxia-induced factors yields better understanding of the tumor nature. It is recommended to conduct selective neck dissection in regions IIA, IIB, III to exlude malignant transformation and metastasis, due to malignant potential of Carotid Body Tumors, especially in case of SDHB mutation. SDHD mutation is the main cause of hereditary HNPGLs. Computed tomography (CT), magnetic resonance imaging (MRI) and angiography yield thorough assessment of paraganglioma extension. In large size tumors, embolization of supplying artery under guidance of angiography may be considered. In case of Carotid Body Tumor, differential diagnosis should include: carotid artery aneurysm, lymphadenopathy, Schwannoma of the hypoglossal nerve or acessory thyroid gland.

Carotid body tumors (CBTs) are rare paragangliomas, comprising 0.5% of all head and neck tumors, and 65% of head and neck paragangliomas. A majority of CBTs occur sporadically, while 15% are familial or hyperplastic in the setting of chronic hypoxia. They usually present as unilateral, well-circumscribed rubbery masses, arising at the level of the carotid bifurcation. A majority of CBTs are painless and therefore may evade diagnosis for months to years. Symptomatic lesions occur due to progressive cranial nerve IX, X, or XII dysfunction, manifesting as hoarseness, dysphagia, vertigo, coughing, or odynophagia. Other local symptoms include neck discomfort, pulsatile tinnitus, hearing loss, or carotid sinus syndrome. Appropriate workup includes a thorough physical exam followed by radiographic imaging, vascular studies, and biochemical workup with 24 h urinary catecholamine or metanephrine analysis. The management of these tumors, which involves standalone surgical resection or following embolization for larger and more vascular tumors, will also be discussed.

Aim: To evaluate whether standard magnetic resonance imaging (MRI) could distinguish vagal paragangliomas (VPs) from carotid body tumours (CBTs) in the parapharyngeal space., Methods and Materials: Thirteen VPs in 13 patients and 29 CBTs in 26 patients were included in this study. MRI features were evaluated independently by two head and neck radiologists with 10 and 16 years of experience (X.Wang and Y.Chen)., Results: Significant differences were found in shape, direction of internal carotid artery (ICA) displacement, splaying of the carotid bifurcation, involvement of the jugular foramen, and maximum vertical diameters between VPs and CBTs (p=0.016, <0.001, <0.001, <0.001 and <0.001, respectively). Splaying of the carotid bifurcation was found in all the 29 CBTs for both observers, whereas only four VPs for observer 1 and two for observer 2 showed this feature. The ICA was displaced posteriorly in all the 29 patients with CBTs for both observers, and only three patients with VPs for observer 1 and two for observer 2. Involvement of the jugular foramen was found in seven patients with VPs for both observers, while none of patients with CBT showed this feature. With the combination of splaying of the carotid bifurcation and involvement of the jugular foramen, the multiple logistic regression model revealed the diagnostic accuracy was 95.2% for observer 1 and 97.6% for observer 2. With the combination of ICA displacement direction and involvement of the jugular foramen, the diagnostic accuracy was 97.6% for both observers., Conclusion: MRI features can effectively differentiate VPs from CBTs in the parapharyngeal space., (Copyright © 2019 The Royal College of Radiologists. All rights reserved.)

Background: To review the Shamblin classification of carotid body paragangliomas (CBPs) and the role of intra-arterial stenting in their surgical management., Methods: Retrospective case series of 20 patients with 28 CBPs that were surgically resected at our center. Intra-arterial stenting was performed in Shamblin II and II classes., Results: The mean follow-up was 47.8 months. Five (17.9%) tumors were Shamblin class I, 15 (53.6%) were class II, and 8 (28.6%) were class III. Thirteen (68.4%) CBPs were associated with other paragangliomas. The internal carotid artery (ICA) was stented preoperatively in eight (28.6%) cases and occluded in four (14.3%) cases. The tumor extended to the jugular foramen in six cases (21.4%). Intraoperatively, there was an ICA injury in one case of Shamblin II CBP in the present era., Conclusions: The proposed classification enables the clinician to plan the management of the ICA and the right approach. Stenting of the ICA gives a chance for complete tumor removal with arterial preservation., (© 2019 Wiley Periodicals, Inc.)

Tumors of the carotid body are uncommon neoplasms that originate in the neuroectoderm. These tumors are slow growing but well vascularized and very destructive. Between 5% and 13% of carotid body tumors are malignant, behaving aggressively at the local level, invading the adjacent lymph nodes, and resulting in remote metastases. We present the case of a 60-year-old man who was examined for pain and paresthesias in his arm. Magnetic resonance imaging showed a large tumor in the left carotid space. Histologic study of a biopsy specimen from the tumor revealed that it was a paraganglioma. Further studies to determine the extent of disease detected metastases in bone and lung, confirming the tumor's malignancy., (Copyright © 2018 SERAM. Publicado por Elsevier España, S.L.U. All rights reserved.)

Objective: The aim of this study was to present our experience in the baseline evaluation of carotid body paragangliomas (CBP) with 68 Ga-DOTATATE PET-CT., Methods: Five patients (4F, 1M; age 24-73 years) with CBPs who underwent 68 Ga-DOTATATE PET-CT scan before the treatment were evaluated retrospectively. PET-CT images were analyzed visually as well as semiquantitatively, with measurement of maximum standardized uptake value (SUV max )., Results: All patients had unilateral CBP lesion, showed intense 68 Ga-DOTATATE uptake in PET-CT. Additionally, 68 Ga-DOTATATE avid lesions were found in two patients. One of them had focal intense uptake in thyroid gland and frontal cerebrum. The other one had intense uptake in bone and adrenal mass. Four patients were operated for unilateral primary CBP. Last patient was treated with peptide receptor radionuclide therapy ( 177 Lu-DOTATATE) for both metastatic pheochromocytoma and CBP., Conclusions: 68 Ga-DOTATATE PET-CT is a valuable imaging modality for staging of CBPs, detecting unknown lesions and changing the management of patients. It is also useful in demonstrating expression of SSTRs for PRRT opportunity.

Paragangliomas are tumors that originate from the extra-adrenal chromaffin and nonchromaffin cells of neural crest origin. Lymph node metastases are common, while distant metastases to lung, liver, and bone are rare events and usually occur in the presence of a long standing clinically evident primary tumor. Primary diagnosis of paraganglioma at a metastatic site without a known primary is a diagnostic challenge. We report a case of an adult woman with incidentally detected metastasis to bilateral lungs from an occult carotid body paraganglioma, which presented a cytopathological diagnostic dilemma on EBUS-TBNA from paratracheal lymph nodes. Diagn. Cytopathol. 2017;45:327-332. © 2016 Wiley Periodicals, Inc., (© 2016 Wiley Periodicals, Inc.)

A 13-year-old woman with a history of left malignant carotid body paraganglioma status postsurgical resection underwent 123I-MIBG scan for staging. The images demonstrated no definite evidence of MIBG-avid disease. However, there was asymmetric activity in the region of the brain with relatively less activity on the left compared with the contralateral right side on the head images, which was related to prior infarct revealed from the patient's history. This asymmetric MIBG activity persisted 8 years later.

Carotid body paragangliomas (CBPGLs) are a rare neoplasms of the neuroendocrine system that affect the carotid glomus. The aim of this study is to improve their management in our Departments. This retrospective analysis reports family history, clinical presentation, imaging diagnostics, Shamblin classification, surgical treatment, complications, and the outcome of seven patients with CBPGLs. All lesions were represented by a painless cervical mass, with no functional or bilateral neck tumors. One patient had two different localizations (the second one was a glomus tumor of the right prelachrymal sac), and a family history for CBPGL. All neck tumors were diagnosed during duplex ultrasound corroborated by magnetic resonance imaging (MRI), and by magnetic resonance angiography (MR-A). They presented a diameter between 3 and 5 cm (MRI). Complete subadventitial resection of the tumor was performed in all patients, with no preoperative embolization in any of the cases. The CBPGLs were confirmed on histopathology and immunohistochemistry. Lymph node metastasis was not found in any of the cases. Mortality and perioperative stroke rates were null. Transitory cranial nerve deficit occurred in one case without permanent palsy. After a follow-up of three years in each patient, there were no signs of tumor recurrence in any of the cases. Relatively early diagnosis of CBPGL was possible in our seven patients using multidisciplinary management. Preoperative planning of the surgical procedure by integrated diagnostic imaging was essential in our study to operate only Shamblin group II tumors, minimizing the known risk of complications associated with large CBPGL (group III).

Paragangliomas, or glomus tumors, are neoplasms arising from extra-adrenal chromaffin tissue. They frequently cause symptoms by over-production of catecholamines with known predilection to multicentricity. We describe the case of a patient with bilateral carotid body tumor who underwent a preoperative ⁶⁸Gallium labeled [1,4,7,10-tetraazacyclododecane-1,4,7,10-tetraacetic acid]-1-NaI3-Octreotide (⁶⁸Ga-DOTANOC) positron emission tomography/computed tomography (PET/CT) imaging for staging. This is a unique case in which multiple paraganglioma and pheochromocytoma were demonstrated in a single patient using ⁶⁸Ga-DOTANOC PET/CT., (Copyright © 2013 Elsevier España, S.L.U. and SEMNIM. All rights reserved.)

Paragangliomas are rare benign neuroendocrine tumors, and 80% of all paragangliomas are either carotid body tumors or glomus jugulare tumors. We present a case of recurrent unresectable carotid body paraganglioma with nodal and T7 vertebral metastases in a 30-year-old man 6 years postsurgery detected with Ga DOTANOC PET/CT and was administered with peptide receptor radionuclide therapy using Lu DOTATATE. After 5 cycles of Lu DOTATATE (total cumulative activity of 750 mCi [27 GBq]), significant response at the primary site on Ga DOTANOC PET/CT and complete disappearance of nodal and T7 vertebral metastases were noted.

Objective: The goal of this study is to review our series of head and neck paragangliomas to identify factors that may help in predicting malignancy., Study Design: Case series with chart review., Setting: Academic medical center., Subjects and Methods: Subjects with head and neck paragangliomas at our institution from 1976 to current were reviewed. In addition to statistical comparisons of epidemiologic factors, pathologic and radiographic characteristics were reviewed., Results: Of the 84 subjects, there were seven malignant paragangliomas (8%). Age was found to be significantly different between the benign and malignant subgroups, with an average age of 54 ± 16 and 40 ± 12 years, respectively (P = 0.02). Pain was a presenting complaint in five patients with benign disease (6%), and five of the seven malignant patients (71%) presented with pain, showing a significant association between pain and disease type (P < 0.0001). The odds ratio for a patient with pain having a malignant tumor was 36 (95% CI: 5.5-234). Enlarging neck mass was noted in all cases of malignant disease, but only in 31 percent of cases of benign disease (P < 0.0001). In a secondary analysis of carotid body tumors alone, enlarging neck mass was not found to be significant between benign and malignant disease (P = 0.14). However, pain continued to be significantly different, with 67 percent of malignant lesions demonstrating pain, compared with only 11 percent of benign lesions (P = 0.01)., Conclusion: This study suggests that pain, a rapidly enlarging neck mass, and younger age are predictive factors of underlying malignancy, which should prompt one to consider an aggressive diagnostic and management approach., (Copyright © 2010 American Academy of Otolaryngology–Head and Neck Surgery Foundation. Published by Mosby, Inc. All rights reserved.)

Carotid paraganglioma (CP) is a relatively rare neoplasm, most commonly located in the head and neck. It is a slow-growing tumor and there has been some debate on the optimal techniques of excision. We report herein the case of a 35-year-old woman who presented with an asymptomatic mass in the right side of the neck. The diagnosis of CP was confirmed by computed tomography and carotid angiography. This tumor was successfully removed surgically without complication. Throughout the description of this case, we analyze the clinical, radiological, pathological and therapeutic particularities of this entity.

Paragangliomas of the organ of Zuckerkandl are rare tumors with only several case reports noted in the literature. Synchronous paraganglioma occur sporadically, and on occasion, in association with several genetic syndromes. Paraganglioma of the organ of Zuckerkandl with synchronous carotid body paraganglioma is described herein. Traditionally, surgical resection of abdominal paraganglioma involved an exploratory laparotomy. In this manuscript, we describe one of the first reported laparoscopic resections of this tumor and review the literature on multiple paragangliomas and their associated genetic syndromes.

Unlabelled: Paragangliomas are uncommon neoplasms with rare occurrence in the head and neck area., Objective: The purpose of this study was to report the experience of management of these rare tumors by a team of Ear, Nose, and Throat (ENT) surgeons working in the context of a sub-Saharan country., Material and Methods: We conducted a retrospective study in the Ear, Nose and throat Department of a Dakar university hospital. The study concerned 8 patients with a highly vascular tumor located in the neck and temporal bone. The preoperative investigations were computed tomography scanning (CT scan) using contrast injection in 88% and Doppler ultrasonography for the patients with a neck mass. All the patients underwent surgery except one. In 2 cases, the management with a team of vascular surgeons was necessary. Histological examination of the tumor was realized in all cases., Results: The tumor was located in the neck in 5 cases (62,5%) with a carotid body tumor in three patients (37,5%) and vagal location in two (25%). The temporal bone was involved in three patients with a tympano-jugular location (37,5%). All the neck masses were operated on via a unique cervical approach. The tympano-jugular tumors were treated by radical mastoidectomy in two patients. In the third patient with a tympano-jugular tumor, with important involvement of the temporal bone, only the neck extension was operated. The histological diagnosis of paraganglioma was confirmed in all patients. Radiation therapy was delivered in two patients (25%), to complete surgery in the event of extensive temporal bone tumor and exclusive in one case of an inoperable vagal tumor. The outcomes, marked early death in one patient (14%), were good in the short and mid term for the others patients., Conclusion: With the improvement of technological infrastructures, particularly with developing modern imaging, we have better knowledge of paragangliomas in the head and neck area, in our experience in a developing country. However, therapeutic approaches are still limited by modest humans and material resources.

Background: Paragangliomas of the head and neck represent rare tumors of neural crest origin that arise from chemoreceptors. They are highly vascular neoplasms that are benign in the majority of cases. There are controversial discussions concerning the different treatment options for cervical paragangliomas., Patients: Between January 1992 and November 2003 a total of 13 patients with 15 cervical paragangliomas were treated at the Department of Otorhinolaryngology - Head and Neck Surgery, University of Freiburg. Our study includes eight female and five male patients with a mean age of 39.9 years (range: 20 - 79 years). There were 14 carotid body tumors and one vagal paraganglioma., Results: 14 tumors were resected and one underwent primary radiation. In four patients we noted a total of 8 permanent, postoperative nerve deficits. During follow-up no recurrent tumors were seen., Conclusion: Complete surgical removal represents the therapy of choice for the majority of cervical paragangliomas. In cases of multiple head and neck paragangliomas or in patients with underlying diseases, primary radiation should be discussed as a treatment option.

Paragangliomas are neuroendocrine tumors derived from the extra-adrenal paraganglia of the autonomic nervous system. Within the head and neck, they are generally defined and named according to their site of origin, and may be found frequently neighboring vascular structures. Physiologic activity is rare in these neoplasms and they may exhibit patterns of inheritance which predispose their occurrence in families, often with multicentricity. These tumors generally exhibit a slow rate of growth, most often presenting asymptomatically as a space occupying mass lesion noted clinically or radiographically. The most common paraganglioma of the head and neck is the carotid body tumor followed by the jugulo-tympanic and vagal varieties. Other rare sites where this tumor may occur include; the larynx, sinonasal chambers and orbit. Diagnosis is generally made through a combination of clinical findings and radiographic studies. Magnetic resonance represents the most important imaging modality for the evaluation and characterization of suspected head and neck paraganglioma. Definitive management for these lesions should be carefully considered in relation to both tumor and patient-oriented factors, especially in regard to the potential morbidity of treatment. Surgery and radiation therapy represent the main treatment modalities for paraganglioma. The selection of treatment depends on the size, location, and biologic activity of the tumor as well as the overall fitness of the patient. Although radiotherapy may be effective in arresting growth of these tumors, rarely is the neoplasm eliminated without surgical resection. Surgery may be associated with significant morbidity, primarily as a consequence of incurring major cranial nerve injury. Patient selection (relative to age and medical condition) should be carefully considered prior to recommending aggressive surgery for paragangliomas of head and neck, especially in those patients at risk for disabling surgical morbidity.

Background: Paragangliomas are highly vascular tumors of neural crest origin that involve the walls of blood vessels or specific nerves within the head and neck. They may be multicentric, and they are rarely malignant. Surgery is the preferred treatment, and these tumors frequently extend to the skull base. There has been controversy concerning the role of preoperative angiography and embolization of these tumors and the benefits that these procedures offer in the evaluation and management of paragangliomas., Methods: Forty-seven patients with 53 paragangliomas were treated from the period of 1990-2000. Initial evaluation usually included CT and/or MRI. All patients underwent bilateral carotid angiography, embolization of the tumor nidus, and cerebral angiography to define the patency of the circle of Willis. Carotid occlusion studies were performed with the patient under neuroleptic anesthesia when indicated. The tumors were excised within 48 hours of embolization., Results: Carotid body tumors represented the most common paraganglioma, accounting for 28 tumors (53%). All patients underwent angiography and embolization with six patients (13%), demonstrating complications (three of these patients had embolized tumor involving the affected nerves). Cerebral angiography was performed in 28 patients, and 5 of these patients underwent and tolerated carotid occlusion studies. The range of mean blood loss according to tumor type was 450 to 517 mL. Postoperative cranial nerve dysfunction depended on the tumor type resected. Carotid body tumor surgery frequently required sympathetic chain resection (21%), with jugular and vagal paraganglioma removal frequently resulting in lower cranial nerve resection. These patients required various modes of postoperative rehabilitation, especially vocal cord medialization and swallowing therapy., Conclusions: The combined endovascular and surgical treatment of paragangliomas is acceptably safe and effective for treating these highly vascular neoplasms. Adequate resection may often require sacrifice of one or more cranial nerves, and appropriate rehabilitation is important in the treatment regimen., (Copyright 2002 Wiley Periodicals, Inc.)

Objectives: To assess the potential of color Doppler sonography to identify cervical mass lesions as paragangliomas., Study Design: Prospective evaluation., Methods: Fifteen patients with 18 paragangliomas (13 carotid body tumors, 3 vagal and 2 temporal) presenting as a mass in the neck were investigated using color Doppler sonography., Results: All paragangliomas presented in B-mode sonography as solid, well-defined, and hypoechoic tumors. Color Doppler imaging revealed hypervascularity in 15 (82%) tumors. No flow signal was detected in 3 carotid body tumors at standard velocity encoding (30 cm/s). Only carotid body tumors could be assessed in their full extent. Temporal and vagal paragangliomas were only partially visible. Carotid body tumors showed a splaying of the carotid bifurcation with displacement of the external carotid anteriorly and both the internal carotid and the internal jugular vein posteriorly. Anterior displacement of both carotid arteries and posterior displacement of the internal jugular vein was found in the 3 vagal paragangliomas. The caudal tumor extension of the 2 temporal paragangliomas was recognized within the expanded lumen of the internal jugular vein. According to the direction of tumor growth and vascular supply, the intratumoral flow signal was predominantly directed upward in carotid body tumors and downward in vagal and temporal paragangliomas., Conclusions: Based on the appearance in the B-mode, the hypervascularity, the relationship toward the carotid arteries and the internal jugular vein, and the intratumoral flow direction, color Doppler sonography was able to establish the diagnosis and type of a paraganglioma.

The authors present 6 cases of carotid glomus tumours referred to their attention over the past 10 years. Their experience shows that colour ultrasonography (using both morphological and functional evaluation) is the diagnostic key for defining the treatment of paraganglioma. More recent experience highlights the role of 111In Pentreotride using a multidisciplinary therapeutic approach.

A case of a 62-year-old Austrian man having a 25-year history of a right-sided carotid body tumor (CBT) is presented. Three months before being transferred to the University of Vienna for tumor resection the patient developed symptoms of tinnitus, progressive ipsilateral hearing loss and dysphagia. Pure-tone audiometry demonstrated a 50 dB right sensorineural hearing loss. A 6 x 6 x 4 cm firm, pulsatile mass was found in the right carotid triangle and extending towards the base of the skull. One week after radical tumor resection all preoperative symptoms disappeared and hearing of the right ear recovered. Review of the available literature showed that hearing loss and tinnitus are unusual symptoms of a CBT. Our findings suggest that routine audiometric evaluations in such cases of CBT patients should be obtained in order to determine the real incidence of audiological disorders.

Background: Current experience with carotid body tumors suggesting a high prevalence of associated cervical paragangliomas prompted this review., Patients and Methods: An 8-year retrospective study of patients with carotid body tumors was undertaken, detailing presentation, diagnosis, and treatment., Results: Eleven patients harboring 17 carotid body tumors were discovered. All patients had a neck mass. Seven patients (64%) had bilateral carotid body tumors. Six (55%) reported a positive family history-4 were first-generation relatives, 5 had bilateral tumors, and 3 had other head and neck paragangliomas. Angiography documented 4 associated vagal and 2 glomus jugulare paragangliomas in addition to the carotid body tumors. Precise surgical care limited blood loss to an average of 590 cc. The carotid artery was repaired during 5 resections (29%). Cranial nerve injury occurred in 3 cases, all following vagal body or glomus jugulare resection. Every patient is currently alive, stroke free, and functioning without major disability., Conclusions: Patients with carotid body tumors have a propensity for multiple head and neck paragangliomas. Angiography is diagnostic. The need for associated paraganglioma resection dramatically increases the risk of cranial nerve injury.

During the period 1971-1993, 33 patients were treated for cervical chemodectomas, 41 tumors were diagnosed. Seven patients had multicentric tumors. All patients expect one with bilateral carotid tumor were treated surgically. A carotid resection was performed in 4 patients. Six patients were given radiation therapy: five patient after surgery and one treated by irradiation alone. During the follow-up period one patient died of bone metastasis 6 year after a partial surgery and radiation therapy, and one had local recurrence treated with irradiation. The surgical management is the major treatment of these tumors, it revealed no postoperative cerebrovascular accident and limited complications secondary to unavoidable nerves sacrifice. Radiation therapy is performed only on particular cases: surgery contra-indications and nonradical resection.

We have applied a new method of preoperative embolization to an intravagal and to carotid body paraganglioma, using estrogen dissolved in absolute alcohol and polyvinyl alcohol particles, which diffusely embolizes vessels from capillaries to main feeders. Total resection of the tumors after embolization was successively performed without postoperative complications; total blood loss was 205 and 130 ml, respectively, and the surgical time was approximately 3 hours in both cases. The technique, characteristics, and advantages of this method are discussed.

Paragangliomas have neuroendocrine characteristics. We previously described successful in vivo visualization of various tumors of neuroendocrine origin after injection of the radiolabeled somatostatin analogue octreotide. In this study, we report the results of 111In-octreotide scintigraphy in 34 patients referred because of known paragangliomas or in whom a paraganglioma was suspected and compared the results of octreotide scintigraphy with the outcomes of other imaging techniques used in the diagnosis or follow-up of these patients. Fifty of 53 (94%) known localizations in 25 patients with paragangliomas were visualized. In two patients, three localizations were missed during octreotide scintigraphy. Unexpected additional paraganglioma sites, not detected or not investigated with conventional imaging techniques, were found in 9 of 25 patients (36%) with known paragangliomas. In four of them, the supposed tumor localizations were thereafter also demonstrated with other imaging modalities. In eight of nine patients who were referred because of symptoms consistent with paraganglioma or follow-up after surgical removal of a paraganglioma, neither routine imaging nor octreotide scintigraphy revealed any abnormalities indicative of paraganglioma. We conclude that: (1) virtually all paragangliomas can be visualized using in vivo 111In-octreotide scintigraphy and (2) because conventional imaging is usually limited to the site where a paraganglioma is clinically suspected, octreotide scintigraphy, because of the information it provides on potential tumor sites in the whole body, may be useful in detecting multicentricity or metastases in patients with paraganglioma.

Two patients with vascular tumors adjacent to the carotid branch of the vagus nerve are presented. Contrary to expectations, the tumours proved to be intravagal paragangliomata. Both were treated surgically.

Diagnostic and surgical features of lateral cervical chemodectomas are discussed in relation to a personal series of 16 patients with carotid body tumors and 10 with tumors of the intravagal glomus. Clinically, these affections are poor in symptomatology, those of the carotid glomus presenting as an isolated cervical tumor, intravagal glomus tumors as parapharyngeal masses of the restrostyloid type. Arteriographic findings are discussed in detail, as they constitute the only pathognomonic signs, enable differentiation between the two tumor sites, and supply essential pre-operative data: location of the pedicles, possible invasion of arterial walls, and the limits of the tumor. Surgical approach should be wide to enable control of the distal end of the internal carotid artery, if necessary under the base of the cranium, excision of the tumor usually being possible by employing the sub-adventitial resection technique.

High-resolution computed tomography with intravenous contrast enhancement is the initial procedure performed on individuals who present with signs and symptoms of a paraganglioma of the head and neck. Clinical information about these entities is reviewed and technical considerations are discussed.

We believe that paragangliomas of the carotid body should be excised in all patients unless formidable medical conditions prohibit the use of general anesthesia. If these tumors are left unresected, they will eventually grow to invade the skull and kill the patient. With definitive diagnostic modalities available, in addition to comparatively low-risk anesthesia, autotransfusion, and replacement of the carotid artery, there are few patients who cannot be surgically treated. The important point is that although such a tumor is rare, its presence must always be considered when dealing with a lateral neck mass. Carotid arteriography should be performed when a carotid body tumor is suspected.

Extra-adrenal paraganglioma (chemodectoma) is a rare tumor; the tumor of the carotid body is the most frequently observed chemodectoma. Histochemical analysis of the glomus cell shows catecholamine in all cases, but functional activity of those tumors is an exception. The diagnosis of carotid body tumor can be confirmed by carotid arteriography. Surgery is the treatment of choice. Malignant degeneration of chemodectoma has been observed in a few cases.