Fig 8 - uploaded by Cihangir Erem
Content may be subject to copyright.
Glomus jugulare. Conventional angiogram with catheter in the right common carotid shows the intense tumour blush typical of glomus tumours.
Source publication
The purpose of this study was to determine the clinical characteristics of carotid body tumors and to analyze the benefits of different treatment modalities.
The clinical records of 13 patients with paragangliomas treated during the period of 1995-2005 were reviewed for age at diagnosis, sex, initial symptoms, duration of symptoms, evidence of secr...
Similar publications
Carotid body tumor presents as palpable mass in anterolateral aspect of the neck. However, bilateral sporadic carotid body tumor is a rare anomaly, reported to occur in 5% of population. We are going to report the case of a 60-year-old patient who presented to our department with painless, palpable mass bilaterally in anterolateral aspects of the n...
Abstract Objective: Toreview the presentation, management and outcome of carotid body tumors at a University Hospital in Karachi. Setting: The Aga Khan University Hospital, Karachi - a tertiary care hospital in Pakistan. Methods: The medical records of all the patients who,were,admitted between,1st January1991 to 1st January 2002, with a diagnosis...
Citations
... ey are distributed paraxially in the trunk mainly related to major arteries and cranial nerves of the pharyngeal arches origin. WHO classification has designated paragangliomas by location (i.e., carotid, vagal, jugular, laryngeal, and tympanic paragangliomas) [2]. ...
Background. Carotid body paragangliomas are rare neoplasms usually benign, however sometimes presenting as highly aggressive tumors. Surgery is the main line of treatment. Purpose. To study and describe clinical presentations, surgical approaches, postoperative complications, and treatment outcomes. Materials and Methods. A single-institution retrospective analysis of 19 cases with carotid body paragangliomas who were candidates for surgery from January 2009 through January 2019 with a mean follow-up period of 58.8 months. Results. The mean age was 46 years with the female predominance of 63%. The mean size of the tumor was 4.3 cm. All cases were presented with a painless pulsating neck lump located anteriorly at the level of the hyoid bone. Neck US was done in all cases as a primary screening investigation. CT scanning was the second main investigation performed in 17 cases (89.5%) revealing tumors attached to the carotid artery at its bifurcation. Urinary catecholamine metabolites were measured in all cases to rule out familial functioning types. 5 cases (26.3%) were malignant. All cases were surgically approached through transcervical transverse incision. 11, 5, and 3 cases were classified as Shamblin’s type II, III, and I, respectively. All tumors were R0 resected with nodal neck dissection conducted in the malignant group. Major complications occurred in 4 cases (21%) during tumor dissection from the adventitia of carotid bifurcation. ECA ligation was performed in one case (5.3%). 2 patients (10.5%) suffered XII nerve paralysis. Carotid artery blowout occurred in one patient (5.3%) and was immediately controlled. No operative mortality occurred. All patients were free of disease during the follow-up period. 4 malignant cases (21%) suffered a systemic relapse to bone and lung metastasis justifying adjuvant chemotherapy, radiotherapy, or both. Conclusions. Surgery is the treatment of choice for carotid body paragangliomas. Complete R0 resection should be justified especially in case of malignancy. Adjuvant chemotherapy or radiotherapy is an option for patients with primary malignancy or relapse.
1. Introduction
Paragangliomas (PGs) are paraganglionic tumors of chemoreceptor cell origin. This term “paraganglia” was first described by Kohn in the early 20th century and is the most appropriate nomenclature from the embryologic aspect [1].
They are distributed paraxially in the trunk mainly related to major arteries and cranial nerves of the pharyngeal arches origin. WHO classification has designated paragangliomas by location (i.e., carotid, vagal, jugular, laryngeal, and tympanic paragangliomas) [2].
Carotid body paragangliomas (CBPs) are also known as chemodectomas because of the physiological function of the carotid body as a chemoreceptor. “Nonchromaffin” refers to the histologic staining which distinguishes these paragangliomas from the chromaffin-reacting tissue of the adrenal medulla [3].
CBPs are rare highly vascular tumors with an incidence of only 0.3% of all paragangliomas and 60% of head and neck paragangliomas, followed by jugulotympanic and vagal PGs [4].
CBPs are more prevalent in middle-aged females and typically present as slowly growing mass. They can remain asymptomatic for years. Clinically, the mass is typically vertically fixed to the carotid bifurcation (Fontaine sign) [5].
10% of CBPs may present with cranial nerve palsy (IX, X, recurrent laryngeal, and XI), involving the sympathetic chain causing Horner syndrome. Typically, they are solitary but can be multicentric, particularly in familial syndromes (Carney’s syndrome and MEN types II A and B) which constitute 28% of CBPs [6].
Most patients do not have functioning CBPs. However, symptomatic patients with headaches, excessive sweating, and palpitations should be evaluated with 24 h urine collection for catecholamine metabolites (metanephrine and VMA) as well as serum catecholamines. This is an important recommendation for proper anesthetic safety and appropriate alpha- and beta-blockade [7].
Shamblin described three different stages of CBPs. Type I consists of a small tumor easily dissected from the adjacent vessels in a periadventitial plane. Type II tumors are larger and partially surround the vessel. Type III tumors are large and completely surround the carotid bifurcation [8].
Fine-needle aspiration (FNA) of CBPs is generally not recommended as it may result in significant hemorrhage; however, aspirations in unsuspected cases may show moderate cellularity of small groups arranged in a “pseudorosette” pattern [9].
The majority of CBPs carry a benign behavior and only 6% are malignant. Malignancy is defined mainly by a spread to lymph nodes (LNs) or distant metastases. The reported 5-year survival rate based on the National Cancer Database is about 60% when regional LN metastases were found [10].
Several radiological investigations were identified to evaluate CBPs. Contrast-enhanced CT scans demonstrate an enhancing mass at the carotid bifurcation and detail any fine osseous changes. Contrast-enhanced MRI also shows a hyperintense T2-weighted image. Due to their vascularity, CBPs have a “salt-and-pepper” pattern on MRI caused by high-velocity flow voids (black dots) and foci of hemorrhage or slow flow (white dots). Angiography is sometimes used for patients undergoing resection demonstrating splaying of the internal and external carotid arteries “Lyre sign” [11–13].
¹²³I-MIBG scans aid in localization, especially in occult or familial types. ¹¹¹In octreotide (a somatostatin analog) may also be sensitive. Positron emission tomography (PET) using ¹⁸fluorodeoxyglucose (¹⁸FDG-PET) shows avid uptake by the tumor cells [14, 15].
Conventional therapy for CBPs is surgical resection. The basic principle of surgery involves locating and preserving the cranial nerves prior to dissecting the tumor as the XI nerve, ansa cervicalis, X nerve, and superior laryngeal nerves are often embedded within the capsule of the tumor. Large tumors may involve the sympathetic chain and IX nerve as well [16].
Radiotherapy (RT) or chemotherapeutics (CTH) theoretically may be successful in ceasing the growth of CBPs, but complete regression with these modalities alone is rare. Thus, it is indicated in large unrespectable and recurrent tumors [17].
2. Materials and Methods
Retrospective analysis of all cases presented at NCI-Cairo University with H&N CBP candidates for surgery from January 2009 to January 2019 was approved by the ethical committee. 19 cases were included. Data collected from patients archive at the statistical department included demographic features (age and sex), tumor characteristics (type, grade, stage, primary, or recurrent), type of surgery and sequelae (loss of nerve function), treatment (CTH, RT, or both), and outcome. Failure patterns were classified as local recurrence and distant metastasis.
3. Results
Due to the rarity of CBPs, only 19 cases were included. The best methodology to describe such rare tumors is “Descriptive Epidemiology” which evaluates separately age, sex, size of the tumor, presenting symptoms, different managements, complication, and outcomes.
3.1. Age and Tumor Size
Patients’ age ranged from 35 to 57 years with a mean age of 46 years. 7 cases were males (36.8%) and 12 cases were females (63.2%) with a male-to-female ratio of 1 : 1.7. The size of the tumor ranged from 3 to 10 cm with a mean size of 5.4 ± 2.1 cm. 13 cases (68.4%) were right-sided; however, this peculiar side does not seem to carry any significance as does bilaterality which is seen in the familial types.
3.2. Symptomatology
The most common presentation was a painless pulsating neck lump located anteriorly to the sternomastoid at the level of the hyoid bone. The lumps were horizontally mobile but not vertically with palpable thrill and bruit heard in all cases. They progressed slowly over a period of 7–12 months except 3 patients for whom there was a progressive neck lump for more than 5 years. Patient complaints are shown in Table 1.
Pulsating painless neck lump
19 cases (100%)
Hoarseness (recurrent laryngeal nerve)
7 cases (36.8%)
Odynophagia (IX nerve)
5 cases (26.3%)
Lymphadenopathy (malignant)
3 cases (15.8%)
Dysarthria and saliva dripping (XII nerve)
1 case (5.3%)
... A bruit can be heard, though absence of which cannot rule out a CBT. Around 10% of the tumour can present with cranial nerve palsies involving the hypoglossal, glossopharyngeal, recurrent laryngeal, spinal accessory or the sympathetic chain [3] .This can cause complaints of pain, hoarseness of voice, tongue paresis, Horner's syndrome and dysphagia. Fever, though uncommon in CBTs, literature has reported it as one of the cause of Pyrexia of Unknown Origin [4] . ...
... A apresentação clínica e exame físico do TUCC é relativamente pobre, sendo que na maioria das vezes se apresenta apenas como uma massa palpável no triângulo anterior do pescoço e somente 10% dos casos cursam com alterações de nervos cranianos (ISIK et al, 2007). Conforme os casos descritos, nenhum dos pacientes apresentou paralisia de nervos cranianos na evolução natural da doença; os sintomas referentes à disfonia e disfagia apareceram após a intervenção cirúrgica. ...
O tumor de corpo carotídeo é uma neoplasia rara de caráter predominantemente benigno que afeta o glomus carotídeo, mais comumente em pessoas de meia idade, mas também crianças. Sua apresentação clínica é variável, desde formas assintomáticas até paralisia de nervos cranianos. Ao exame físico pode ser encontrado apenas elevação de região cervical no triângulo do pescoço. O diagnóstico é confirmado por meio de exames de imagens e o tratamento de escolha é a remoção cirúrgica. Este relato aborda três pacientes diagnosticados com tumor de seio carotídeo e acompanhados na cidade de Cascavel-PR.
... CBTs are paragangliomas of the carotid bodies occuring at the bifurcation of common carotid in to ECA and ICA. These are also called as chemodectomas [2][3][4]. These cervical paragangliomas arise in close relation to the extracranial arterial system and cranial nerves. ...
Background: Carotid body tumors (CBTs) are benign, but they are quite controversial for the management because of their location and symptoms. Cervical CBTs are slightly uncommon paragangliomas. These are highly vascular tumors. Their location is at the bifurcation of common carotid artery (CCA) which is adjacent to many cranial nerves. Surgical management involves a lot of morbidity and mortality. Case Report: A 48-year-old female reported with painless small swelling on the right side of the neck region just below the angle of the mandible. The radiological evaluation by ultrasonography, contrast-enhanced computerized tomography, and magnetic resonance angiography revealed the swelling as carotid body tumor. The patient had been advised surgical management as presently the entity was almost without any symptoms. Conclusion: The cross-sectional imaging plays a great role in the diagnosis and management of CBTs as they are associated with many complications. The treating surgeon had to be very careful about surgical extirpation.
... Каротидная нехромаффинная параганглиома (хемодектома) относится к числу редких заболеваний. Она развивается из каротидного тельца, расположенного в области бифуркации сонной артерии [1,2]. По данным литературы, частота ее встречаемости составляет 0,5-0,9% среди внеорганных опухолей шеи [3][4][5] и 37% -среди неврогенных опухолей шеи [6,7]. ...
Objective : to enhance the efficiency of diagnosis and treatment of carotid paragangliomas, by introducing of a multidisciplinary approach into clinical practice.
Material and methods . A total of 15 cases of paragangliomas have been followed up for 10 years. The age of the patients was 18 to 58 years (mean age 38±12 years). Primary multiple sites of chemodectomas were noted in 2 cases. Bilateral involvement was observed in one case. In the other, two unilateral vascular tumors successively developed with an interval of almost 6 years. A set of studies was conducted, which embraced ultrasound duplex scanning (USDS) of the neck vessels, computed tomography (CT), and selective carotid angiography (SCAG) in various combinations. The investigators made a successive examination of the symmetrical areas of the face and neck and polypositional scanning of the zone of interest and studied quantitative parameters, including linear blood flow velocity (LBFV) and volumetric blood flow velocity. Native and contrast-enhanced X-ray CT and magnetic resonance imaging (MRI) were carried out. SCAG was performed as digital subtraction angiography.
Results. USDS can confirm the hypervascular nature of the tumor and its close relationship with the carotid artery branches and measure blood flow velocity (LBFVmean, 0.46 m/sec). X-ray CT and MRI examinations in combination with vessel contrasting make it possible to accurately determine the localization and topography of the tumor, its association with the carotid artery system and to type afferent vessels. SCAG is a highly informative technique that can identify the afferent vessels of the tumor and its relationship with the great vessels and, if technically possible, perform selective microembolization of the afferent tumor vessels, which will further facilitate surgical treatment, reducing the risk of intraoperative bleeding.
Conclusion. The multidisciplinary approach to diagnosing carotid paragangliomas in the maxillofacial area and neck makes it possible to determine the type, size, and velocity characteristics, to verify the diagnosis, and to work out optimal treatment policy. Among the non-invasive techniques, CT angiography and MR angiography are most valuable for final diagnosis. Selective angiography of the branches of the external carotid arteries remains the gold standard and is the final and most important stage of the study, which make it possible to detail angioarchitectonics, to determine afferent vessels, and to plan further treatment, including endovascular embolization. The diagnosis of carotid paragangliomas requires a multidisciplinary approach with the equal attraction of endovascular diagnosis and treatment specialists, maxillofacial surgeons, angiologists, ultrasound and radiology diagnosticians, and pathologists both at the stages of diagnosis and subsequent treatment. This problem can be effectively solved in a multidisciplinary hospital that has skilled staff with sufficient clinical experience in diagnosing vascular masses and is equipped with modern equipment, which allows optimization of further treatment.
... Approximately 10% of the cases present with cranial nerve palsy with paralysis of the hypoglossal, glossopharyngeal, recurrent laryngeal, or spinal accessory nerve, or involvement of the sympathetic chain, therefore been associated with pain, hoarseness, dysphagia, Horner syndrome, or shoulder drop [12]. ...
Introduction
Carotid body tumors also called carotid paragangliomas are rare neuroendocrine neoplasms derived from neural crest cells, approximately 3% of all paragangliomas occur in the head and neck area (Xiao and She, 2015); although they represent 65% of the head and neck paragangliomas (Georgiadis et al., 2008).
Presentation of case
We present the therapeutic management of a 65-year-old woman with bilateral carotid body tumors. The patient presented to medical clinic for unrelated signs and symptoms of weight loss, dyspepsia, and epigastric pain. Physical examination showed bilateral non-tender neck masses for which imaging studies were ordered resulting in the diagnosis of bilateral carotid tumor. Surgical resection was staged with one week of distance between each tumor resection.
Discussion
Carotid Body Tumors can arise from the paraganglia located within the adventitia of the medial aspect of the carotid bifurcation.
Resection is the only curative treatment. Carotid body tumors resection represents a special challenge due to potential neurovascular complications.
Conclusions
Surgical resection of carotid body tumors represents a special challenge to the surgeon because of the complex anatomical location of the tumor, including close relationship with the cranial nerves, involvement of the carotid vessels and large vascularization of the tumor. With the advance of diagnosis and improvement in surgical techniques as well as the understanding of biological behavior of tumors, surgical treatment has become a safer alternative for treating these tumors.
... Cranial nerve palsy is an obvious possible presentation due to proximity of nerves like hypoglossal, glossopharyngeal, recurrent laryngeal, or spinal accessory nerve, or even involvement of the sympathetic chain and their associated clinical features like tongue movements, hoarseness of voice, Horner syndrome, and dysphagia. [11] As this is a paraganglionoma, in cases of functional carotid body tumors (CBTs), symptoms similar to those of pheochromocytoma, such as paroxysmal hypertension, palpitations, and diaphoresis, are seen. ...
A carotid body tumor (jugular paraganglioma) commonly presents as a painless slow growing neck mass, and its diagnosis calls for strong clinical suspicion mostly showing up incidentally on imaging and requiring detailed workup before going in for an intervention so as to determine the exact extent and vascularity of these masses as well as the need for preoperative embolization. We report here a case of carotid body tumor in a 42 year old man, its evaluation and management.
... 7-10% cases may be familial and 35-50% of familial cases may be multicentric [6,7]. Hyperplastic CBT is more common in patients with chronic hypoxia, patients living in high altitude and in patients of chronic obstructive pulmonary disease [11]. ...
... Symptoms of carotid body tumour[6,10,11]. ...
Carotid body tumours (CBT) are rare and highly vascular neoplasm
arising at the bifurcation of common carotid artery splaying the internal
and external carotid arteries. Surgery for carotid body tumour
may be associated with severe cardiovascular perturbation due to
its location, vicinity to vital structures, involvement of nerves and
stimulation of richly innervated chemoreceptors and carotid sinus.
Here the authors describe a case of bilateral carotid body tumour
in a 30 year old male patient posted for surgical removal of the tumour
and sudden hemodynamic instability due to injury to external
carotid artery.
... Так, по данным исследования J.C. Jansen et al., с использованием метода последовательной обработки данных средняя скорость роста опухоли составляет 0,83 мм/год [16]. В 10 % случаев хемодектома сочетается с параличом черепно-мозговых нервов (подъязычного, языкоглоточного, возвратного гортанного нервов), вовлечением ветвей шейного сплетения, симптомом Горнера, дисфагией [9]. Редким симптомом опухоли каротидного гломуса является лихорадка [12]. ...
Carotid chemodectoma (“chemodectoma caroticum”, a carotid glomus tumor) is a benign slow-growing, vascularized tumor that is one of the most common paragangliomas of the head and neck. The ultrasound examination of 18 000 patients referred for various reasons revealed 2 cases of carotid chemodectoma verified by angiography. The paper gives the current ideas of the rate, etiology, pathomorphology, and clinical manifestations of chemodectoma, as well as its major ultrasound differential diagnostic criteria.
... In most of the case, paragangliomas are inherited representing around 10-50%. [13,14] If CBT is inherited, it usually identified in younger age group, in second to fourth decade. ...
Aim: The aim was to perform a retrospective audit of rare vascular neoplasm-Carotid body tumor (CBT) in our cancer center. Materials and Methods: An institutional retrospective audit was performed in Curie Manavata Cancer Centre, Nasik, India from 30 th June 2013 to 30 th June 2014. Total of 5 patients were diagnosed with CBT in a period of 6 months. Results: Out of 5 patients who were diagnosed with CBT, 2 (40%) were males and 3 (60%) were females with a ratio of 1:1.5. The types of CBT were familial in 2 patients (40%), sporadic in 2 patients (40%) and hyperplastic in 1 patient (20%). The clinical presentation of the 5 cases was, 3 patients (60%) had a painless, progressive swelling, 2 patients (40%) had pre-auricular pain, and 1 patient (20%) was identifi ed in routine follow-up. All patients underwent surgical excision of CBT with no need to pre-operative embolization. 40% of patients had cranial nerve injury and hematoma formation. 1 out of 5 patients received post-operative adjuvant radiotherapy due to large tumor size and lymphatic spread. No recurrence was found after a follow-up of 6 months. Conclusion: Surgical management of CBT remains a challenge for head and neck surgeon. Despite a reduction in stroke and mortality, there can be a signifi cant morbidity associated inadvertent adjacent cranial nerve injuries. However, our audit shows these tumors can be managed well with meticulous planning and execution of the surgery.
