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Ear, Nose, & Throat Journal May 2024To summarize the clinicopathological and genetic features of malignant paragangliomas in head and neck cancer and to explore the appropriate treatment options for this...
To summarize the clinicopathological and genetic features of malignant paragangliomas in head and neck cancer and to explore the appropriate treatment options for this rare lesion. Six patients harboring head and neck malignant paraganglioma from Beijing Tongren Hospital were retrospectively reviewed. The clinicopathological characteristics, gene mutations, and prognosis of these patients were analyzed. Of these 6 patients, 3 were male and 3 were female; 4 patients harbored malignant carotid body tumors, and two had malignant vagal paragangliomas. Three patients had cervical lymph node metastasis, two presented with lung and bone metastasis, and 1 had lung and liver metastasis. Of the 6 patients, four underwent surgical resection, and the other two patients denied surgery and instead received chemotherapy with paclitaxel, ifosfamide, and dacarbazine. These 2 patients with vagal paraganglioma received postoperative radiotherapy. All 6 patients are still alive at the present time, with a median follow-up time of 66 months. Positive Ki-67 expression in tumor tissue ranged from 1% to 40%. Genetic mutations in SDHD, SDHB, ATR, and MAP3K13 were identified in 4 patients. After comprehensive treatment, head and neck malignant paraganglioma can attain a favorable prognosis. Genetic mutations are commonly detected in patients with malignant paragangliomas. This study also identified mutations in ATR and MAP3K13 in these patients.
Topics: Humans; Female; Male; Middle Aged; Retrospective Studies; Head and Neck Neoplasms; Adult; Mutation; Paraganglioma; Prognosis; Carotid Body Tumor; Succinate Dehydrogenase; Aged; Lymphatic Metastasis
PubMed: 34654328
DOI: 10.1177/01455613211052338 -
Acta Otorrinolaringologica Espanola Feb 2009Paragangliomas are rare tumors and most are benign: less than 10% show criteria for malignancy. In the head and neck, the main locations are carotid, jugulo-tympanic and... (Review)
Review
Paragangliomas are rare tumors and most are benign: less than 10% show criteria for malignancy. In the head and neck, the main locations are carotid, jugulo-tympanic and vagal. Surgery continues to be the mainstay of treatment but both external beam radiotherapy (EBRT) and stereotactic radiosurgery (SRS) have been shown to be effective therapeutic alternatives. EBRT requires large volumes to cover the lesion, while radiosurgery allows administration of a high dose in a smaller volume, with high precision, which should reduce the number of long-term complications. The results in terms of local control with both treatment modalities are good, as reflected in published series. The role of chemotherapy is restricted to malignant paragangliomas. The treatment used includes the combination of chemotherapy, which provides short-lasting responses, and the role of new molecular targets is currently being investigated. The present article reviews the indications and results of the non-surgical treatment of paragangliomas.
Topics: Head and Neck Neoplasms; Humans; Paraganglioma
PubMed: 19245783
DOI: No ID Found -
The Journal of International Advanced... Nov 2022This study aimed to review tumor control and cranial nerve function outcomes in patients with complex jugular paragangliomas and to refine the surgical strategies for...
BACKGROUND
This study aimed to review tumor control and cranial nerve function outcomes in patients with complex jugular paragangliomas and to refine the surgical strategies for complex jugular paragangliomas.
METHODS
We describe our experience with 12 patients with complex jugular paragangliomas diagnosed in our institution from January 2013 to June 2020. The main outcomes included tumor control, complications, and function of facial nerve and lower cranial nerves, postoperatively.
RESULTS
Gross-total resection was achieved for 9 (75%) patients, and subtotal resection was achieved for 3 (25%) patients. The surgical tumor control rate was 100% after a mean follow-up of 45.5 months (range, 13-111 months). Postoperatively, 10 patients (83.3%) obtained unchanged or improved facial nerve function. However, new lower cranial nerve deficits occurred in 2 patients (16.7%) due to surgical removal of the concurrent vagal paraganglioma and scar tissue enclosing the IX and XII nerves.
CONCLUSION
Our refined surgical techniques, including tension-free anterior facial nerve rerouting, sigmoid sinus tunnel-packing, and pushpacking techniques, could be a choice for the treatment of complex jugular paragangliomas to achieve tumor control and cranial nerves preservation. A 2-stage surgery should be applied to minimize the risk of bilateral cranial neuropathies and the influence on cerebral circulation in patients with bilateral paragangliomas. The preoperative endovascular intervention such as coil embolization or internal carotid artery stenting can be employed for the management of paragangliomas with internal carotid artery-associated lesions.
Topics: Humans; Carotid Stenosis; Treatment Outcome; Retrospective Studies; Stents; Glomus Jugulare Tumor; Paraganglioma
PubMed: 36349670
DOI: 10.5152/iao.2022.22675 -
Indian Journal of Otolaryngology and... Oct 2019Vagal paragangliomas are rare tumors. Advances in the imaging have aided the diagnosis and assessment of this disease. Surgery is the mainstay of the treatment however...
Vagal paragangliomas are rare tumors. Advances in the imaging have aided the diagnosis and assessment of this disease. Surgery is the mainstay of the treatment however radiotherapy has been tried in the selected cases. Surgery is associated with some mortality but with a lot of morbidity in view of cranial nerve deficit. The necessity to prevent additional cranial nerve deficits needs thorough preoperative evaluation and influences the therapeutic approach. This study involves the retrograde evaluation of five patients of vagal paraganglioma histopathologically confirmed, all the patients were operated via trancervical approach. Preoperatively three of five had 9, 11 and 12th cranial nerve involvement, one had horner's syndrome. Postoperative vocal rehabilitation was done in all the patient by medialisation thyroplasty Issiki type 1. Though vagal paragangliomas are associated with mortality and morbidity. Surgical treatment should aim at reducing mortality. Vagal nerve morbidity can be taken care as a planned and staged procedure mainly involving restoring voice as rest of concerns are taken care by compensation from opposite nerves.
PubMed: 31741999
DOI: 10.1007/s12070-018-1340-5 -
Acta Otorrinolaringologica Espanola 2015Head and neck paragangliomas are rare tumours of a neuroendocrine nature. The aim of this study was to show our experience in the management of these kind of tumours.
INTRODUCTION
Head and neck paragangliomas are rare tumours of a neuroendocrine nature. The aim of this study was to show our experience in the management of these kind of tumours.
METHODS
This was a retrospective study of head and neck paragangliomas diagnosed between 1978 and 2014. A total of 126 patients with 162 tumours were analysed. The paragangliomas included 88 jugulotympanic tumours, 53 carotid tumours and 21 vagal paragangliomas.
RESULTS
Mean age at diagnosis was 53.1 years; 87 patients were female (69.1%) and 39, male (30.9%). Multifocality was present in 24 patients (19.1%). Germline mutations were found in 20 patients analysed; SDHD and SDHB were the most frequent. Surgery was performed on 72 isolated paragangliomas: these were 9 carotid, 21 tympanic, 37 jugular and 5 vagal paragangliomas. There were 25 isolated tumours that were observed periodically: 7 carotid, 3 tympanic, 9 jugular and 6 vagal paragangliomas; 5 jugular tumours were irradiated. Multifocal paragangliomas were individually treated, with a total of 26 surgical procedures and 36 tumours resected, 9 irradiated and 12 tumours periodically observed. Postoperative cranial nerve deficits in isolated carotid paragangliomas were lower (15%) compared with jugular tumours (45.5%, P=.04). Nerve deficit was found more frequently in tumours with intradural extension (100%) than in extradural tumours (37.5%, P=.007).
CONCLUSIONS
Management of head and neck paragangliomas include surgery, radiotherapy and wait and scan policies. A combination of all of them is usually needed in patients with multifocal paragangliomas.
Topics: Adult; Cranial Nerve Injuries; Dura Mater; Female; Genetic Predisposition to Disease; Germ-Line Mutation; Head and Neck Neoplasms; Humans; Male; Middle Aged; Neoplasm Invasiveness; Neoplastic Syndromes, Hereditary; Paraganglioma; Postoperative Complications; Retrospective Studies; Watchful Waiting
PubMed: 25638014
DOI: 10.1016/j.otorri.2014.11.002 -
International Journal of Surgery Case... 2018Paraganglioma of head and neck is a rare tumor and vagal schwannoma is even rarer. The majority of patients with vagal schwannoma presents with a lateral neck mass. Its...
BACKGROUND
Paraganglioma of head and neck is a rare tumor and vagal schwannoma is even rarer. The majority of patients with vagal schwannoma presents with a lateral neck mass. Its management is delicate as the need to confirm the diagnosis by histopathology via a biopsy is contraindicated. Here, is a case of a young female with vagal schwannoma complicated with hoarseness after the biopsy of the mass, which persists after extirpation of the tumor.
CASE DESCRIPTION
A 22-year old lady presented with a history of a right neck mass for a 5-months duration. Clinical examination revealed a mass at level II neck region which measures 3.0 cm × 2.0 cm and it was mobile, non-pulsatile and had smooth surfaced. CT scan and angiogram showed that the mass arose between the carotid artery and vagal nerve and it was a highly vascular lesion. A CT scan-guided biopsy performed but complicated with neck hematoma and patient developed hoarseness. On follow up, her hoarseness persists and her tissue biopsy came back as schwannoma. She was counseled regarding surgery versus radiation for her treatment and she agreeable for surgery. Hence, surgical excision was performed and intraoperatively the mass visualized arising from the vagal nerve. Postoperatively however, her voice did not improve.
CONCLUSION
Vagal schwannoma is a rare paraganglioma of head and neck and the best treatment is still controversial. Unnecessary investigation and procedure should be avoided in order to reduce morbidity as well as improves patient's quality of life.
PubMed: 30366175
DOI: 10.1016/j.ijscr.2018.10.025 -
Annals of Medicine and Surgery (2012) Oct 2021and importance: Head and neck paragangliomas are slowly growing benign tumors and they originate from specialized neural crest cells. We report an unusual combined...
INTRODUCTION
and importance: Head and neck paragangliomas are slowly growing benign tumors and they originate from specialized neural crest cells. We report an unusual combined glomus vagal and jugular tumor that was rarely described in the literature to the best of our knowledge.
CASE PRESENTATION
A 51 years old female with no pathological history was presented to our ENT department with 6 months' history of a right latero cervical swelling gradually increasing in size associated with a swallowing difficulties and hoarseness. Preoperatively clinical examination had found vagal and hypoglossal nerve paralysis. Cervical CT scan and MRI had shown glomus jugular tumor. The patient underwent a surgical excision with severe swallowing difficulties and facial palsy in the immediate postoperative period with a mild recovery afterwards.
CLINICAL DISCUSSION
Paragangliomas of the mesotympanum and jugular foramen most commonly present as a vascular middle ear mass. The most common presenting symptom is pulsatile tinnitus occurring in 80% followed by hearing loss (60%). Dysfunction of cranial nerves traversing the jugular foramen may be commonly encountered with resultant abnormalities of speech, swallowing and airway function. Vagal paragangliomas are the least common of the three primary craniocervical paragangliomas. The most common presenting sign is the presence of a painless neck mass accompanied occasionally by dysphagia and hoarseness. The association of both glomus vagal and jugular tumor is rarely described in the literature to the best of our knowledge.
CONCLUSION
Head and neck paragangliomas are slowly growing benign tumors and they originate from specialized neural crest cells. Vagal paragangliomas are the least common of the three primary craniocervical paragangliomas. The association of both glomus vagal and jugular tumor is rarely described in the literature to the best of our knowledge. The choice of treatment depends on the location, size, and also biologic activity of the tumor as well as the physical condition of the patient.
PubMed: 34691440
DOI: 10.1016/j.amsu.2021.102918 -
AJNR. American Journal of Neuroradiology 1986Preoperative embolization was performed in 39 patients with 44 paragangliomas of the head and neck. Because of their complex vascular supply and their relation to vital... (Review)
Review
Preoperative embolization was performed in 39 patients with 44 paragangliomas of the head and neck. Because of their complex vascular supply and their relation to vital structures such as the internal carotid artery and the lower cranial nerves, paragangliomas of the temporal bone represent challenging lesions to both the neuroradiologist and the otoneurosurgeon. Detailed classification by high-resolution CT and recognition of the multi- or monocompartmental vascular composition and of dangerous situations by selective angiography are essential prerequisites for safe and effective devascularization of paragangliomas of the temporal bone. Major complications that may occur if embolic material reaches intraaxial vessels through anastomoses between external carotid artery branches and the internal carotid and/or the vertebral artery can be avoided with the use of specific precautionary techniques. Palsies of the facial and lower cranial nerves can also be avoided if reabsorbable material is used for embolization of vessels supplying cranial nerves in asymptomatic patients. In selected cases with significant supply from the internal carotid artery, special interventional techniques, including embolization of the pericarotid tumor portion through the caroticotympanic artery and pre- or peroperative balloon occlusion of the petrous internal carotid artery, allow radical removal of extensive paragangliomas of the temporal bone. Techniques and selection of materials for embolization of carotid body, vagal body, and other paragangliomas of the head and neck mainly depend on the vascular composition of the tumor and on the specific vascular territory in which the tumor is located. In this series, preoperative embolization significantly improved surgical conditions of paragangliomas of any location in the head and neck and proved to represent an essential prerequisite for successful surgery of extensive paragangliomas of the temporal bone.
Topics: Adult; Aged; Embolization, Therapeutic; Female; Head and Neck Neoplasms; Humans; Male; Middle Aged; Paraganglioma; Preoperative Care
PubMed: 3096120
DOI: No ID Found -
Journal of Surgical Case Reports Apr 2021Paragangliomas (PGs) are extremely rare multicentric neoplasms. Hereditary or familial PGs are associated with germline mutations in succinate dehydrogenase genes, seen...
Paragangliomas (PGs) are extremely rare multicentric neoplasms. Hereditary or familial PGs are associated with germline mutations in succinate dehydrogenase genes, seen in one-third of cases. Primary PGs of the thyroid are uncommon neuroendocrine neoplasms that account for 0.012% of all head and neck lesions. Although majority of these tumors are solitary, familial PGs are associated with synchronous tumors (carotid/vagal). We report an interesting case of primary thyroid PG in a patient with a previous history of a right carotid body, right vagal PGs and positive familial history, confining the differential diagnosis to recurrent lesions, which is the most common occurrence or new primary or a metastatic lesion. However, long interval and surgical anatomy suggests the diagnosis to be a primary lesion. In conclusion, although these lesions present multicentrically present at varying intervals, their occurrence at anatomically distinct sites should raise the concern for a new primary PG.
PubMed: 33936587
DOI: 10.1093/jscr/rjab102 -
Annals of Medicine and Surgery (2012) Jun 2021Head and neck paragangliomas are rare vascular tumors derived from the paraganglionic system, located at the carotid body, jugular vein, tympanic cavity and vagal nerve....
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.
PubMed: 34094530
DOI: 10.1016/j.amsu.2021.102412