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Cureus Aug 2022Head and neck paragangliomas (PGLs) most commonly derive from the carotid body, jugulotympanic, vagal, and laryngeal paraganglia. Thyroid PGLs originate in the inferior...
Thyroid Paraganglioma With Medullary Carcinoma: A Unique Combination in a Patient in Association With Multiple Endocrine Neoplasia Type 2B Syndrome With Prolonged Survival.
Head and neck paragangliomas (PGLs) most commonly derive from the carotid body, jugulotympanic, vagal, and laryngeal paraganglia. Thyroid PGLs originate in the inferior laryngeal paraganglion, which may lie inside the thyroid parenchyma. Intrathyroid PGLs are rare with approximately 75 cases reported to date, mostly as solitary lesions. The coexistence of thyroid PGL with medullary thyroid carcinoma (MTC) has not been reported. Here, we report a unique case of intrathyroid PGL concomitant with MTC in the context of multiple endocrine neoplasia type 2B syndrome. Interestingly, the patient showed a prolonged survival with good clinical response to tyrosine kinase inhibitors, despite her advanced metastatic MTC. We discuss the challenges in pathology, differential diagnosis, and genetic background for the development of these thyroid lesions.
PubMed: 36176816
DOI: 10.7759/cureus.28423 -
Journal of Vascular Surgery Dec 2010Our objective was to assess the short- and long-term outcome for patients after carotid body tumor (CBT) resection and discuss the potential pitfalls of the treatment.
OBJECTIVES
Our objective was to assess the short- and long-term outcome for patients after carotid body tumor (CBT) resection and discuss the potential pitfalls of the treatment.
METHODS
An analysis was undertaken of all patients who underwent CBT resection at Royal Brisbane and Women's Hospital and Greenslopes Private Hospital between 1982 and 2007. Primary tumor characteristics, surgical technique, and outcomes were recorded and analyzed.
RESULTS
A total of 49 consecutive CBT resections (2 recurrent tumors) were carried out in 39 patients (26 women [56%]) who were a mean age of 49 years (range, 17-75 years). A nontender neck mass was the presenting complaint in 85%, followed by screening in familial or contralateral tumors in 26%. Familial cases occurred in 11 patients (28%). There were no operative deaths. Complications occurred in 13 of the 49 operations (27%), predominantly temporary nerve palsies and were more likely to occur in tumors of large volume or in cases of removal of coexisting vagal tumors. Malignant disease was present in seven cases (15%). All patients have been followed-up postoperatively for a mean of 11 years (range, 2-26 years). Metachronous paragangliomas have been discovered in six patients, all with familial disease.
CONCLUSIONS
Early resection of carotid body tumors should be undertaken while still small to minimize the risk of neural injury, which increases with tumor size. In cases of bilateral CBT, we recommend that the smaller tumor be resected first, before the staged resection of the larger contralateral tumor. In familial or bilateral tumor cases, other synchronous and metachronous paragangliomas should be excluded. Mandatory lifelong follow-up is essential.
Topics: Adolescent; Adult; Aged; Carotid Body Tumor; Female; Humans; Male; Middle Aged; Neoplasm Recurrence, Local; Young Adult
PubMed: 21146747
DOI: 10.1016/j.jvs.2010.06.153 -
European Annals of Otorhinolaryngology,... Jun 2013First Bite Syndrome (FBS) is a rare pain syndrome sometimes occurring after surgery of the upper cervical region. It presents as excruciating pain, triggered at the...
INTRODUCTION
First Bite Syndrome (FBS) is a rare pain syndrome sometimes occurring after surgery of the upper cervical region. It presents as excruciating pain, triggered at the beginning of a meal by chewing, swallowing or even simple contact with generally acidic food, waning on subsequent bites and recurring with identical features after pausing for several minutes or at the next meal.
OBJECTIVES
Retrospective review of 17 patients who developed FBS after upper cervical surgery.
RESULTS
Seventeen patients developed FBS between 1999 and 2010 following surgery for paraganglioma in eight cases, vagal or sympathetic schwannoma in five cases (including one malignant tumour), pleiomorphic adenoma in three cases and Warthin's tumour of the deep lobe of the parotid in one case. The cervical sympathetic trunk was sacrificed in 10 cases and the external carotid artery was ligated in six cases. Horner's sign was observed postoperatively in 12 patients. The characteristic pain of FBS was triggered by chewing or simple contact with essentially acidic food.
CONCLUSION
FBS must be identified by the head and neck surgeon and distinguished from the usual postoperative pain. The generally accepted hypothesis is that of sympathetic denervation with parasympathetic secretory hyperactivity, but Horner's sign was present in only 12 of the 17 patients of our series, suggesting that other pathogenic mechanisms may be involved. FBS is difficult to treat, but the pain gradually becomes less severe. The patient must be informed about this rare complication that can impact on postoperative quality of life.
Topics: Head and Neck Neoplasms; Horner Syndrome; Humans; Mastication; Muscle Cramp; Neck Dissection; Pain, Postoperative; Parathyroid Neoplasms; Peripheral Nervous System Neoplasms; Quality of Life; Retrospective Studies; Syndrome; Treatment Outcome
PubMed: 23477880
DOI: 10.1016/j.anorl.2012.08.001 -
BMC Medical Genetics Jun 2010Germline mutations of the succinate dehydrogenase subunit B gene (SDHB) predispose carriers for paragangliomas, and current estimates of the chance of mutation carriers...
BACKGROUND
Germline mutations of the succinate dehydrogenase subunit B gene (SDHB) predispose carriers for paragangliomas, and current estimates of the chance of mutation carriers actually developing tumors (penetrance) are high. We evaluate the phenotype and penetrance of a germline SDHB mutation in a large and clinically well-characterized paraganglioma family.
METHODS
Following identification of the mutation in a 31 year old index-patient, extensive clinical screening was performed in mutation carriers to evaluate the presence of head and neck, thoracic and abdominal paragangliomas. Presymptomatic DNA testing was performed in 19 family members.
RESULTS
DNA analysis detected 14 further SDHB mutation carriers. Three mutation carriers (median age 78 years) declined clinical surveillance, but had no clinical signs or symptoms associated with paragangliomas. The remaining 11 mutation carriers (mean age 53, range 37-76 years) consented to clinical screening. In only two, aged 43 and 48 years, were subclinical vagal paragangliomas identified.
CONCLUSIONS
Only three of the fifteen mutation carriers in this family have developed paraganglioma, which results in a calculated penetrance of 26% at 48 years of age. This figure is lower than current estimates, and we conclude that the co-operation of this family allowed an almost complete attainment of mutation carriers, and the extensive clinical evaluation carried out allowed us to identify all affected individuals.
Topics: Genes; Germ-Line Mutation; Humans; Mutation; Paraganglioma; Paraganglioma, Extra-Adrenal; Penetrance; Phenotype; Succinate Dehydrogenase
PubMed: 20540712
DOI: 10.1186/1471-2350-11-92 -
Journal of Vascular and Interventional... Apr 2008Paragangliomas are tumors of neural crest origin commonly arising from the carotid body, vagal nerve, or jugular bulb. The definitive treatment for these tumors is...
BACKGROUND AND PURPOSE
Paragangliomas are tumors of neural crest origin commonly arising from the carotid body, vagal nerve, or jugular bulb. The definitive treatment for these tumors is surgical resection, often augmented with pre-operative embolization due their highly vascular nature. We present our experience examining the efficacy and safety of endovascular embolization of these rare tumors.
METHODS
A review of patient's diagnosed with paragangliomas who underwent pre-operative embolization over a 5-year period (2002-2007) was conducted. The tumor subtype, efficacy of embolization, method of embolization, and rate of complication were noted.
RESULTS
A total of 38 patients underwent selective arterial embolization of their paraganglioma using polyvinyl alcohol (PVA) particles ranging in size from 100-1000 microns. The tumor subtypes treated were carotid body (n = 20), glomus vagale (n = 10), and glomus jugulare (n = 8). The average age at presentation was 44 years (range, 15-81). Twenty-two patients were female and sixteen were male. The most common artery embolized was the ascending pharyngeal branch of the external carotid artery. Post-embolization angiography revealed an average decrease in blood flow to tumor of 75%. With the exception of transient facial pain documented in 1 patient, there were no known complications from embolization.
CONCLUSIONS
The endovascular embolization of paragangliomas using PVA prior to surgical resection is a very safe and efficacious procedure that may reduce operative blood loss and associated morbidity.
PubMed: 22518217
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 -
Brazilian Journal of Otorhinolaryngology 2005Paragangliomas are tumors of the autonomic nervous system, arising from paraganglionic tissue. Paragangliomas of the head and neck region are very rare. In the head and...
Paragangliomas are tumors of the autonomic nervous system, arising from paraganglionic tissue. Paragangliomas of the head and neck region are very rare. In the head and neck, the most common sites of origin of this neoplasm are the carotid body, the jugular bulb and the vagal body. Paragangliomas of the nose and paranasal sinuses are very uncommon. The authors referred one case of nasal paraganglioma in a 45-year-old male patient, who was submitted to surgical excision, and included clinical findings, diagnostic criteria, treatment, prognosis and literature review. The importance of reporting this case refers to the rare incidence of paragangliomas in the nasal cavity and paranasal sinuses.
Topics: Humans; Male; Middle Aged; Nose Neoplasms; Paraganglioma
PubMed: 16450456
DOI: 10.1016/s1808-8694(15)31317-3 -
Sao Paulo Medical Journal = Revista... May 2001Protein marker positivity can assist in the definition of the therapeutic approach towards head and neck paragangliomas. The establishment of the therapeutic approach...
CONTEXT
Protein marker positivity can assist in the definition of the therapeutic approach towards head and neck paragangliomas. The establishment of the therapeutic approach should incorporate the results of such an investigation.
OBJECTIVE
To establish criteria for benignity and malignancy of vagal and jugular-tympanic paragangliomas, via the study of the relationships of sex, age, tumor size, duration of complaints, site, family history, presence of metastases, treatment, histological architecture and cell type with the immunohistochemical reactions to S100 protein, chromogranin and AgKi67.
DESIGN
A retrospective study of histological and clinical records.
SETTING
The Heliópolis and Oswaldo Cruz tertiary general hospitals, São Paulo.
SAMPLE
8 cases of head and neck paragangliomas.
MAIN MEASUREMENTS
Determination of degree of positivity to paragangliomas via immunohistochemical reactions.
RESULTS
1). The protein markers for the principal cells (AgKi67 and chromogranin) were sensitive in 100% of the tumors when used together. 2). S100 protein was well identified in the cytoplasm and nucleus of sustentacular cells and underwent reduction in the neoplasias.
CONCLUSIONS
Chromogranin was proven to be a generic marker for neuroendocrine tumors; S100 protein was positive in all 8 cases and the AgKi67 had low positivity in all cases.
Topics: Adult; Aged; Biomarkers, Tumor; Chromogranins; Female; Head and Neck Neoplasms; Humans; Immunohistochemistry; Male; Middle Aged; Neoplasm Proteins; Neuroendocrine Tumors; Paraganglioma; Retrospective Studies
PubMed: 11391454
DOI: 10.1590/s1516-31802001000300006 -
Case Reports in Veterinary Medicine 2020Pheochromocytomas are catecholamine-secreting tumors that are composed of neuroectoderm-derived chromaffin cells. An 8-year-old miniature dachshund with abdominal...
Pheochromocytomas are catecholamine-secreting tumors that are composed of neuroectoderm-derived chromaffin cells. An 8-year-old miniature dachshund with abdominal distension was diagnosed with a neuroendocrine tumor with invasion from the caudal vena cava to the right ventricular cavity. The dog died due to hypotensive shock from the vagal reflex, and on autopsy, an extra-adrenal pheochromocytoma (paraganglioma) was diagnosed in the caudal abdomen. At autopsy, the tumor plug of the caudal vena cava was confirmed. To the best of our knowledge, this is the first case report that echo-captured the extension of pheochromocytoma in the right ventricle and shows it in a figure and video file.
PubMed: 32095312
DOI: 10.1155/2020/5382687 -
Indian Journal of Otolaryngology and... Dec 1999Non - Chromaffin Paraganlioma arises in paraganglia of chemoreceptor system. It has been reported in the following sites-aortic bodies, superior vagal ganglion,...
Non - Chromaffin Paraganlioma arises in paraganglia of chemoreceptor system. It has been reported in the following sites-aortic bodies, superior vagal ganglion, auricular branch of vagus, inferior vagal nodose ganglion, superior laryngeal nerve, mandible, ciliary ganglion, bifurcation of pulmonary artery, pleura, femoral artery, pleura, femoral artery, retroperitoneal tissue, mesentry, coccyx and pineal body. Paraganglions of nasal cavity is a very rare occurance. A very few cases were reported before this.
PubMed: 23119624
DOI: 10.1007/BF02996436