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Medicina (Kaunas, Lithuania) May 2024: Paragangliomas of the head and neck are rare, slow-growing neuroendocrine tumors, benign in their vast majority, but with a possibility of developing distant... (Review)
Review
: Paragangliomas of the head and neck are rare, slow-growing neuroendocrine tumors, benign in their vast majority, but with a possibility of developing distant metastases. They show great inheritable character, and their behavior has proven to be unpredictable; therefore, they are considered malignant. This article aims to offer a more comprehensive presentation of the pathogenesis, epidemiology, diagnostic methods, imaging development, and treatment guidelines. We tried to bring together all the necessary data that, in our opinion, a head and neck practitioner should know when managing this type of tumor. Our main focus is on the most recent studies, with the purpose of a homogenous presentation of all current guidelines and approaches to this pathology. : Paragangliomas of the head and neck are still a disputed topic. One of the main reasons for that is their low incidence of 0.3 to 1 per 100,000 every year. The most frequent locations are the carotid body, the temporal bone, the jugular and mastoid foramen, and the vagal nerve. Their clinical presentation usually involves a painless lateral mass associated with symptoms such as hoarseness, hearing loss, tinnitus, and cranial nerve deficits. Up to 40% of them are inherited, mostly linked with mutations of succinate dehydrogenase complex. Imaging evaluation consists of CT and MRI, and new functional explorations such as F-FDA and F-FDG PET/CT, F-DOPA PET, I-MIBG, and Ga-DOTATE PET/CT. Measuring the catecholamine levels in the plasma and urine is mandatory, even though paragangliomas of the head and neck rarely display secretory behavior. Treatment mainly consists of surgery, with different approaches and techniques, but conservative management methods such as wait and scan, radiotherapy, proton therapy, and chemotherapy have proven their efficiency. The therapeutical decision lacks consensus, and current studies tend to recommend an individualized approach. Guidelines regarding long-term follow-up are still a matter of debate.
Topics: Humans; Head and Neck Neoplasms; Paraganglioma
PubMed: 38929531
DOI: 10.3390/medicina60060914 -
WMJ : Official Publication of the State... Sep 2023Radiation therapy may result in complications, including fibrosis, which can result in pain and difficulty with movement - especially in the neck.
BACKGROUND
Radiation therapy may result in complications, including fibrosis, which can result in pain and difficulty with movement - especially in the neck.
CASE PRESENTATION
A 52-year-old man with right-sided vague neck pain unresponsive to conservative management had a computed tomography scan that showed a vagal paraganglioma in the carotid sheath surrounding the right carotid arteries and internal jugular vein. Following radiation therapy, he noticed a new pain in his right jaw and neck worse with certain movements of the neck. Nonsurgical conservative measures including physical therapy and pharmacological management were unsuccessful. An ultrasound evaluation demonstrated fibrosis beneath the sternocleidomastoid muscle and in proximity to the carotid sheath. After careful trajectory planning using ultrasound imaging, a 25 G needle was introduced real time in proximity to the fibrosis. Using a dexamethasone/saline mixture under real-time ultrasound guidance, adhesions were released. After 3 injections, the patient reported greater than 90% pain relief, which lasted 4 months. Subsequently, he required similar injections approximately every 3 months to achieve greater than 75% pain relief.
CONCLUSIONS
This is a successful demonstration of the utility of ultrasound evaluation and guidance for adhesiolysis following radiation therapy.
Topics: Male; Humans; Middle Aged; Neck Pain; Radiation Fibrosis Syndrome; Ultrasonography; Pain Management; Ultrasonography, Interventional
PubMed: 37768775
DOI: No ID Found -
The American Journal of Case Reports Oct 2023BACKGROUND Paragangliomas are neuroendocrine neoplasms derived from paraganglia of the sympathetic and parasympathetic nervous systems. Parasympathetic ganglia-derived...
BACKGROUND Paragangliomas are neuroendocrine neoplasms derived from paraganglia of the sympathetic and parasympathetic nervous systems. Parasympathetic ganglia-derived tumors, also called non-chromaffin, are located almost exclusively in the neck and skull base and are usually non-secretory and inactive. A case of malignant glomus jugulare with a metastatic cervical lymph node is described here. CASE REPORT A 24-year-old woman was referred to an otolaryngology clinic for concern of voice change for 1 month, which was associated with right progressive hearing loss, pulsating tinnitus, and right facial weakness. A clinical examination revealed a reddish mass in the right ear behind an intact tympanic membrane with right facial weakness of House-Brackmann grade VI. A bedside flexible nasopharyngoscopy revealed an immobile right vocal fold. A computed tomography scan of the brain revealed a destructive lesion within the right jugular foramen. The patient underwent embolization followed by glomus tumor resection via infra-temporal fossa with Fisch type A approach. Pathology revealed that the tumor was an infiltrative epithelioid tumor with a spindle and nesting pattern separated by fibrovascular stroma. The submitted lateral neck lymph node revealed a metastatic tumor. CONCLUSIONS Glomus jugulare tumors are uncommon paragangliomas, and malignant behavior with metastasis is extremely rare. Metastatic tumors are often associated with facial and vagal nerves palsy. There are no histological features that distinguish malignant glomus jugulare tumors. Malignant neoplasms are characterized by the presence of metastases. Tumors of the glomus jugulare that are malignant are treated with surgery, radiotherapy, or both. However, our search of the literature revealed no clear guidelines, given the scarcity of cases. Moreover, the presence of metastasis increases the risk of death.
Topics: Female; Humans; Young Adult; Adult; Glomus Jugulare Tumor; Glomus Tumor; Paraganglioma; Facial Paralysis; Skull Base; Neoplasms, Second Primary; Sarcoma
PubMed: 37814445
DOI: 10.12659/AJCR.940138