Paraganglia are clusters of cells originating from the neural crest with histological and cytochemical characteristics of neuroendocrine cells. Paragangliomas of the head and neck represent less than 0.5% of all head and neck tumors and they usually occur between the ages of 40 and 50. Paragangliomas in childhood and in adolescence are extremely rare; only 23 case reports have been reported in the recent literature. In childhood, the estimation of malignant potential is 3–10%; therefore, early diagnosis and treatment of carotid body paragangliomas are mandatory. However, due to the rarity of these lesions in young patients, they are often not included in the differential diagnosis of solid masses in the neck area, a fact that may lead to misdiagnosis or delay in treatment. We present, herein, two extremely rare cases of patients in adolescence who were diagnosed with a carotid body paraganglioma and were treated surgically in our unit. One of the patients was diagnosed and treated at the age of 15 years while the other had a long-standing tumor in the neck that was followed up by a general surgery outpatient service as a branchial cleft cyst at the age of 15 years and was eventually treated surgically 8 years later. Carotid body tumor was not considered in the initial differential diagnosis because of its rarity at this age range.
Paraganglia are clusters of cells with histological and cytochemical characteristics of neuroendocrine cells originating from the neural crest [
We present two cases of carotid body paragangliomas (CBP) that occurred during adolescence; one of the patients had a delayed treatment, as a neck mass initially observed at childhood was considered to be a branchial cleft cyst, for which he had annual surveillance at a general surgery outpatient service.
The clinical picture, the imaging investigations, the differential diagnosis, and the surgical treatment are discussed in the current manuscript, along with a review of similar published cases.
We searched the PubMed and Cochrane Library databases with no language restrictions. Studies or case reports were published between 1968 and 2018.
We performed a literature search with the keywords “Paraganglioma”, “Carotid body paraganglioma”, “Head and neck paraganglioma”, and “Chemodectoma”, without limitations to the dates or the article types. According to the database and the used terms, many studies and incidents were reported. Our research focuses on carotid body paragangliomas in adolescence, so more specific investigation with the keywords “adolescence carotid body paraganglioma” and “pediatric carotid body paraganglioma” has revealed only 23 published case reports.
Two reviewers independently assessed the risk of bias. They extracted the characteristics and results of the various studies that were discussed. In case of any disagreements that had occurred during data extraction, the third reviewer was asked to decide.
During the literature search, many investigations found to be referred to multiple incidents, including a number of pediatric people treated with CBP. In some of these series, there is no reference of the exact number of pediatric patients being treated, nor the preoperative, intraoperative, and postoperative management of these patients.
The search produced 21 papers that were reviewed (Table
Reported cases of CBPs in pediatric age group [
Year/author | Age/ Gender | Symptoms | Shamblin type | Size (mm) | Malignancy | Cranial nerve injury | Treatment | Follow-up period without complications (months) |
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1968/ Chambers et al [ | 12/M | NR | NR | 25X25 | No | No | Surgical Excision | 120 |
14/M | NR | 60X60 | 120 | |||||
9/F | NR | 15X20 | 12 | |||||
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1971/ Shamblin et al [ | 12/NR | NR | NR | NR | No | NR | Surgical Excision | NR |
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1980/ Newland et al [ | 13/F | Hypertension, | NR | 25X80 | NR | NR | Surgical Excision/ Radiotherapy, Chemotherapy | NR |
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1983/ Carney et al [ | 12/F | NR | NR | NR | No | No | Surgical Excision | Epithelioid myosarcoma- |
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1986/ Dickinson et al [ | 12/F | NR | NR | NR | No | XII permanent paralysis | Surgical Excision | NR |
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1988/ Hallett et al [ | 12/NR | Neck Mass Tumor | NR | NR | NR | Yes (not specified) | Surgical Excision | NR |
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1989/ Thompson et al [ | 14/F | Bilateral CBPs and Glomus Jugular Tumor | I | 20X20 | No | Right facial nerve paralysis | Surgical Excision | NR |
III | Large | No | ||||||
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1990/ Gounot et al [ | 14/M | Right neck mass | II | 35X25X15 | No | No | Subadventitial Excision | 12 |
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1991/ Ophir et al [ | 12/F | Bilateral CBPS and Unilateral Jugular Tumor | III | 30X40 | Family History (+) | Paralysis of IXth to XIIth cranial nerves | Surgical Excision | 96 |
II | 30X30 | No | ||||||
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1993/ Varudkar et al [ | 6/M | Swelling, Hypertension, Pheochromocytoma | NR | NR | No | No | Surgical Excision | NR |
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1999/ Hajnzic et al [ | 9/M | NR | NR | 50 | Metastatic Chest Paraganglioma, Pulmonary Metastasis | No | Surgical Excision | Death after 4 months |
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2000/ Wang et al [ | 10/NR | NR | NR | NR | No | Yes (not specified) | Surgical Excision, Preoperative Embolization | 2.9 |
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2001/ Plukker et al [ | 11/NR | NR | NR | NR | No | Yes (not specified) | Surgical Excision | NR |
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2003/ Takautz et al [ | 8.2/F | NR | NR | 25 | NR | No | Surgical Excision | 118 |
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2005/ Osborne et al [ | 16/F | Nonpulsatile Tumor | NR | NR | NR | NR | Preoperative Emboli, Subadventitial Dissection | NR |
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2007/ Zaupa et al [ | 15/M | Asymptomatic Mass | NR | NR | No | NR | Subadventitial Excision | NR |
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2007/ Sajid [ | 18/ NR | NR | NR | NR | NR | NR | Surgical Excision | NR |
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2008/ Georgiadis et al [ | 13/F | Painless Pulsatile Slow-Growing Mass | III | 50X43X30 | No | No | En Bloc Surgical Excision | 9 |
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2014/ Lopez-vasquez et al [ | 16/F | Asymptomatic Mass | NR | 40X30X30 | No | NR | Surgical Excision | NR |
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2017/Ifeoluwa et al [ | 8/F | Pulsatile Slow-Growing Mass | NR | NR | No | No | Preoperative embolization Surgical resection | Current time |
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2018/Hogan et al [ | 8/F | Neck mass | NR | NR | No | No | Preoperative embolization and balloon test occlusion | NR |
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2018/ our cases | 16/ F | Cervical Pain, Dizziness, Dysphagia, Breathlessness and Palpable Mass | II | 26x21x30 | No | No | Surgical Excision | 26 |
15/M | Asymptomatic Mass | II | 60X35 | No | No | Surgical Excision | 96 |
M: male, F: female, and NR: not reported.
We report two cases of carotid paraganglioma in young age that have been operated in our department in the last 14 years, with a diagnosis of carotid body paragangliomas diagnosed and treated during adolescence, by the same surgeon (first author).
A 16-year-old girl, with a free past medical history, presented with a palpable mass on the left side of the neck, complaining of dysphagia and cervical pain associated with dizziness. Neurologic examination was unremarkable and diagnostic tests for Epstein–Barr infection were negative. The patient underwent a thyroid ultrasound (US), which did not reveal any significant findings from the thyroid gland. However, a 27.5mm oval shaped, well-defined, hypoechoic, solid lesion was found at the left carotid triangle. The lesion showed high vascularity. Magnetic resonance imaging (MRI) of the neck followed showing an ovoid mass measuring 26x21x30mm between the left internal and external carotid arteries. A computed tomography angiography (CTA) was also performed with similar findings, suggesting the diagnosis of a CBP (Figures
3-dimensional (3D) cervical-computed tomography angiography (CTA), lyre sign (arrow).
CTA-carotid body paraganglioma (CBP), lyre sign (arrow).
Paraganglioma of carotid tumor after surgical resection.
Histopathological appearance of CBP (16-year-old teenage girl) showing “zellballen” growth pattern of paragangliomas with central round/oval chief cells containing abundant eosinophilic granular or vacuolated cytoplasm, uniform nuclei with dispersed chromatin-nests of cells (blue arrow). Prominent fibrovascular stroma separates nests (black arrow).
The patient had no postoperative neurologic symptoms except a transient episode of left parietal hypoesthesia. This was further investigated with an MRI of the brain and carotid ultrasound, which did not reveal sinister findings.
She had an uneventful postoperative recovery and was subsequently discharged on the 3rd postoperative day (POD).
A 15-year-old teenage boy initially presented in a district hospital, with a swelling at the right side of his neck, without any significant clinical symptoms. Although full details of the work-up performed at the time are not available, he was diagnosed with a branchial cleft cyst and was offered surveillance with follow-up imaging. Eight years later, he visited our unit. Physical examination revealed a painless palpable well-defined mass (Figure
Painless palpable well-defined mass in the right carotid triangle.
Digital subtraction angiography (DSA) revealed a carotid body paraganglioma in the right carotid bifurcation.
Paraganglioma of carotid tumor after surgical resection was 3 cm in diameter.
Histopathological appearance of CBP (15-year-old teenage boy) showing “zellballen” growth pattern of paragangliomas (blue arrow). Prominent fibrovascular stroma separates nests (black arrow).
The surgical technique adopted in both patients consisted of an oblique incision along the sternal head of the left sternocleidomastoid muscle under general anesthesia. Our strategy was to expose, dissect, and isolate the proximal common carotid artery using a vascular tape. The ansa cervicalis was also exposed early in the operation. The carotid bifurcation was exposed in a caudocranial approach. The proximal external and the proximal internal carotid arteries were isolated and controlled with vessel loops. Dissection was extended to the level of the digastor muscle in order to expose and control the distal internal carotid artery. During this process, the hypoglossal nerve was identified and preserved, by following the ansa cervicalis. The external carotid artery was cross-clamped temporarily. Finally, the tumor was removed with sharp dissection from the bifurcation with meticulous technique in order to avoid injury to the internal carotid artery and the cranial nerves. The reported plane of dissection reported as a white interface plane between the tumor and the vessels was not identified in the first patient as the tumor was severely adhering to the vessel wall and was typically found in the second patient. Following the removal of the CBP, the carotid sheath and platysma were approximated and the skin closed with a continuous subcuticular suture, after performing meticulous hemostasis (Figures
The right carotid triangle with carotid body paraganglioma.
The carotid body paraganglioma of the right carotid triangle was completely excised, with preservation of the hypoglossal and vagus nerve.
Both patients had an annual postoperative follow-up with cervical ultrasound and carotid duplex ultrasonography with no evidence of local recurrence. Familial disease was excluded clinically, by screening the patient’s first degree relatives with ultrasound imaging.
Paragangliomas of the head and neck are quite rare tumors, and although the exact incidence is unclear they are considered to represent less than 0.5% of all head and neck tumors [
First recognized by Arias-Stella, CBPs occur in individuals under chronic stimulation by low environmental atmospheric partial pressure of oxygen, leading to hyperplasia of the chief cells of the carotid body [
In our review of the literature we recognized 23 cervical paragangliomas involving the carotid body in children and adolescents. Mean patient age at the time of treatment was 12 years, with range 6 to 18 years (Table
Two of the patients had bilateral CBPs and at the same time presented jugular tumor [
The incidence of familial paragangliomas is approximately 10%, and about 30% of them are caused by mutations in succinate dehydrogenase (SDH) gene [
Diagnosis of these tumors can be quite challenging and most patients overlook the symptoms of the disease or are misdiagnosed with cervical lymphadenopathy, salivary gland pathology, neurofibromas, brachial cleft cysts, or lipomas [
The most important preoperative imaging is CTA or MRA, while digital subtraction angiography (DSA) is the mainstay of the diagnosis, with 100% reported accuracy, whereas the “lyre sign”, an enhancing vascular oval mass widening the angle of the bifurcation, is essentially pathognomonic of a CBP [
Benign tumors were found in 14 patients [
Complete surgical removal of the tumor is the gold standard operation. Although this is quite straightforward in Shamblin I tumors, locally advanced tumors constitute a challenge. Radical resection is crucial due to the possibility of malignancy. According to Luna-Ortiz et al., all tumors Shamblin class II and even tumors infiltrating vessels (Shamblin class III) must be managed surgically [
Most patients identified by our review had CBPs that were classified as Shamblin II and III [
Neck paraganglioma in childhood is extremely rare incidence, with only a few published cases in literature. However, CBPs must be included in the differential diagnosis of pulsatile cervical mass and imaging with US and CT or MRI needs to follow, for evaluating the nature of the mass and the relation with the adjacent vessels and the anatomic structures.
The authors declare that there are no conflicts of interest regarding the publication of this paper.