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The American Journal of Case Reports Oct 2014Carotid body tumors are rare, highly vascularized neoplasms that arise from the paraganglia located at the carotid bifurcation. Surgery is the only curative treatment....
BACKGROUND
Carotid body tumors are rare, highly vascularized neoplasms that arise from the paraganglia located at the carotid bifurcation. Surgery is the only curative treatment. However, treatment of bilateral carotid body tumors represents a special challenge due to potential neurovascular complications.
CASE REPORT
We present the therapeutic management of a 34-year-old woman with bilateral carotid body tumors. The patient underwent surgical resection of the largest tumor. It was not possible to resect the tumor without sacrificing the ipsilateral vagal nerve. Due to unilateral vagal palsy, we decide to withhold all invasive therapy and to observe contralateral tumor growth with serial imaging studies. The patient is free of disease progression 5 years later.
CONCLUSIONS
Treatment of bilateral CBTs should focus on preservation of the quality of life rather than on cure of the disease. In patients with previous contralateral vagal palsies, the choice between surgery and watchful waiting is a balance between the natural potential morbidity and the predictable surgical morbidity. Therefore, to avoid bilateral cranial nerve deficits, these patients may be observed until tumor growth is determined, and, if needed, treated by radiation therapy.
Topics: Adult; Angiography; Biopsy; Carotid Artery, Common; Carotid Body Tumor; Diagnostic Imaging; Female; Follow-Up Studies; Humans; Quality of Life; Time Factors; Vascular Surgical Procedures
PubMed: 25278171
DOI: 10.12659/AJCR.891150 -
Ear, Nose, & Throat Journal Aug 2014Paragangliomas are uncommon tumors that arise from the parasympathetic neuroectodermal ganglionic cells and have been described in numerous anatomic locations, most...
Paragangliomas are uncommon tumors that arise from the parasympathetic neuroectodermal ganglionic cells and have been described in numerous anatomic locations, most commonly in the abdomen. Head and neck paragangliomas are classified into carotid body (most common), vagal, and jugulotympanic types. Computed tomography is the initial imaging modality of choice for the preoperative assessment of the extent of paragangliomas. Magnetic resonance imaging and selective angiography provide more detail of the surrounding tissues and vasculature. Surgical resection is the treatment of choice.
Topics: Female; Head and Neck Neoplasms; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Paraganglioma; Tomography, X-Ray Computed
PubMed: 25181670
DOI: 10.1177/014556131409300822 -
Cancer Dec 2014Paragangliomas of the head and neck are rare, slow-growing, generally benign tumors of neuroendocrine cells associated with the peripheral nervous system that commonly...
BACKGROUND
Paragangliomas of the head and neck are rare, slow-growing, generally benign tumors of neuroendocrine cells associated with the peripheral nervous system that commonly involve the carotid body, jugular bulb, vagal ganglia, and temporal bone. Treatment options include surgery, radiotherapy (RT), stereotactic radiosurgery (SRS), and observation. This article briefly reviews our 45-year institutional experience treating this neoplasm with RT.
METHODS
From January 1968 through March 2011, 131 patients with 156 benign paragangliomas of the temporal bone, carotid body, jugular bulb, or glomus vagale were treated with RT at a median dose of 45 Gy in 25 fractions. The mean and median follow-up times were 11.5 years and 8.7 years, respectively.
RESULTS
Five tumors (3.2%) recurred locally after RT, all within 10 years of treatment. The overall local control rates at 5 and 10 years were 99% and 96%, respectively. The cause-specific survival rates at 5 and 10 years were 98% and 97%, respectively. The distant-metastasis free survival rates at 5 and 10 years were 99% and 99%, respectively. The overall survival rates at 5 and 10 years were 91% and 72%, respectively. There were no severe complications.
CONCLUSION
RT for benign head and neck paragangliomas is a safe and efficacious treatment associated with minimal morbidity. Surgery is reserved for patients in good health whose risk of associated morbidity is low. SRS may be suitable for patients with skull base tumors <3 cm where RT is logistically unsuitable. Observation is a reasonable option for asymptomatic patients with a limited life expectancy.
Topics: Adult; Aged; Aged, 80 and over; Aortic Bodies; Carotid Body Tumor; Cohort Studies; Disease-Free Survival; Female; Glomus Jugulare Tumor; Head and Neck Neoplasms; Humans; Male; Middle Aged; Paraganglioma, Extra-Adrenal; Radiotherapy, Intensity-Modulated; Retrospective Studies; Temporal Bone; Treatment Outcome; Young Adult
PubMed: 25060724
DOI: 10.1002/cncr.28923 -
European Archives of... Aug 2015Paragangliomas (PGs) are slowly growing, usually benign neoplasms. The aim of the study was to analyze the incidence, diagnostic and therapeutic management of patients...
Paragangliomas (PGs) are slowly growing, usually benign neoplasms. The aim of the study was to analyze the incidence, diagnostic and therapeutic management of patients with multiple paragangliomas of the head and neck. A retrospective review of the records of 84 patients with head and neck PGs, diagnosed and treated in our institution was performed for the years 1983-2013 to identify patients with multiple tumors. Fourteen (16.6 %) patients developed multiple PGs, synchronous or metachronous, within 4-21 years of follow-up. Clinical data of these patients were reviewed to evaluate the diagnosis, location, stage and management strategy. There was a total number of 37 tumors in 14 patients. There were 20/37 (54.0 %) carotid PGs, 9/37 (24.3 %) jugular PGs and 8/37 (21.7 %) vagal PGs. Carotid PGs were observed in 12/14 (86 %) patients and in 8/14 (57 %) cases bilateral tumors occurred. Vagal PGs developed in 7/14 (50 %) patients and bilateral tumors were found in 1/14 (7 %) case. Jugular PGs occurred in 9/14 (64 %) patients. There were 30 synchronous tumors and seven metachronous PGs diagnosed 2-18 years after removal of the first tumor. Single metachronous mediastinal PG occurred. All patients had at least one tumor removed, with histopathological confirmation of the diagnosis. One patient had positive history of familial PGs. Carotid PGs are most common multiple paragangliomas. Radiological survey of the head and neck is required to detect multicentric tumors. Metachronous mediastinal and abdominal tumors may occur. Regular, prolonged follow-up is essential to identify metachronous PGs and possible postoperative gradual ICA occlusion.
Topics: Adolescent; Adult; Carotid Body Tumor; Diagnostic Imaging; Disease Management; Female; Follow-Up Studies; Glomus Jugulare Tumor; Glomus Tympanicum Tumor; Head; Head and Neck Neoplasms; Humans; Male; Middle Aged; Neck; Neoplasms, Multiple Primary; Paraganglioma; Poland; Retrospective Studies
PubMed: 24920325
DOI: 10.1007/s00405-014-3126-z -
Il Giornale Di Chirurgia 2014Objectives. Carotid Body Tumor (CBT) is a rare lesion of the neuroendocrine system but it is the most common form of head and neck paraganglioma (PGL). Our objective is...
Objectives. Carotid Body Tumor (CBT) is a rare lesion of the neuroendocrine system but it is the most common form of head and neck paraganglioma (PGL). Our objective is to discuss the optimal management of these lesions to provide the best outcome of patients treated by surgical resection. Patients and Methods. A retrospective evaluation was obtained by review of the records of 20 patients with 26 CBT treated at our institution between 2000 and 2012. Primary tumor characteristics, diagnostic protocols, surgical treatment, short and long-term outcomes were collected and analyzed. Results. A total of 26 CBTs resections were performed on 20 patients; the age range was 21-89 years. There was a female prevalence (14 women-80% and 6 men-20%). Familial cases occurred in 6 patients (30%); of these, 3 patients had bilateral lesions and 1 patient multiple paragangliomas. In all cases no lymph node metastasis was found. All lesions were grouped into three groups according to the latero-lateral diameter: Group I < 3 cm; Group II 3<>5cm; Group III >5cm. All patients were managed by surgical resection of the CBT. There were no operative deaths. Overall we found transitory neurological impairment in 15,3% and permanent neurological deficit in 7,6% of cases. No complications occurred in all resections of Group I tumors. In Group II only 1 resection was followed by dysphonia by recurrent nerve palsy (after vagal nerve en-bloc resection). In Group III only 1 resection was followed by permanent vagus nerve palsy. Conclusions. Surgical removal of the tumor is the only treatment that can ensure a complete eradication of the disease. Family screening is of great importance in patients with hereditary forms. Careful preoperative planning of surgical procedure by integrated diagnostic imaging and a full mastery of the surgical technique can minimize the risk of the most common postoperative complications. Lifelong follow-up is mandatory to make early diagnosis of recurrent disease.
Topics: Adult; Aged; Aged, 80 and over; Carotid Body Tumor; Female; Humans; Male; Middle Aged; Retrospective Studies; Time Factors; Young Adult
PubMed: 24690341
DOI: No ID Found -
Translational Medicine @ UniSa 2013Between 1972 and 2012, 25 patients presenting 32 paragangliomas of the neck were observed. Tumor locations included the carotid body (CBTs) in 21 patients and the vagus...
Between 1972 and 2012, 25 patients presenting 32 paragangliomas of the neck were observed. Tumor locations included the carotid body (CBTs) in 21 patients and the vagus nerve in 4. Four patients had bilateral CBT and one a bilateral vagal tumor; a metachronous bilateral jugulare paraganglioma was diagnosed in one patient with bilateral CBT Shamblin type III. Five patients presented CBTs type II and three type III. Preoperative embolization was performed in 5 CBTs, with no significant difference in blood loss. Twenty-nine paragangliomas were resected (with three internal carotid artery resection): there were no cerebrovascular accident or perioperative death. Nine patients (36%) had cranial nerve palsy prior to surgery and a postoperative nerve dysfunction occurred in four other tumors (16%). Persistent nerve deficits occurred in 3 patients (12%). No evidence of malignancy was shown, intraoperatively or during a postoperative follow-up period (9 months to 18 years; mean: 8 years).
PubMed: 24251239
DOI: No ID Found -
European Annals of Otorhinolaryngology,... Jun 2014The objective of this study was to report 11 cases of malignant head and neck paraganglioma and to compare their epidemiological, clinical, and genetic characteristics,... (Comparative Study)
Comparative Study
BACKGROUND
The objective of this study was to report 11 cases of malignant head and neck paraganglioma and to compare their epidemiological, clinical, and genetic characteristics, their natural history and their treatment with those of a series of 131 benign paragangliomas.
PATIENTS AND METHODS
Retrospective analysis of 142 patients with head and neck paraganglioma managed between 2001 and 2008. Age at the time of diagnosis, gender, primary tumour site, presence of other non-head/neck paragangliomas and/or metastases diagnosed by imaging (CT, MRI, Octreoscan or (18)F-FDG PET), histology, urinary catecholamine and metanephrine levels, family history, and genetic test results were recorded.
RESULTS
This series comprised 131 benign head and neck paragangliomas, mostly observed in women with a mean age at diagnosis of 45 years and a predominance of tympanojugular sites (followed by carotid and vagal sites) with 5% of secreting tumours and 20% of multifocal tumours. Eleven patients (7.7%) with a 1:1 sex ratio presented criteria of malignancy. These patients, with a lower mean age (38 years), predominantly presented carotid lesions with a higher rate of secreting and multifocal tumours, 27% and 46% respectively. The main sites of metastases were bone and lymph nodes. No tympanic paragangliomas were observed.
CONCLUSIONS
Malignant paragangliomas are mainly observed in young patients with multifocal tumours, particularly carotid tumours, and are predominantly related to subunit SDH-B mutation. The work-up in these high-risk patients must include whole body scintigraphy and spine MRI. Malignancy is not necessarily associated with a poor short-term prognosis due to the slow course of the disease.
Topics: Adolescent; Adult; Aged; Bone Neoplasms; Diagnostic Imaging; Female; Genetic Testing; Head and Neck Neoplasms; Humans; Liver Neoplasms; Lung Neoplasms; Lymphatic Metastasis; Male; Middle Aged; Mutation; Neck Dissection; Neoplasm Recurrence, Local; Neoplasms, Multiple Primary; Paraganglioma; Retrospective Studies; Sex Distribution; Succinate Dehydrogenase; Thyroid Neoplasms; Young Adult
PubMed: 24239180
DOI: 10.1016/j.anorl.2013.05.003 -
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 -
Journal of Vascular Surgery Feb 2013Carotid body tumors are considered rare. However, there has been an increase in the number of these tumors managed at our center in recent years. Delayed presentation...
OBJECTIVE
Carotid body tumors are considered rare. However, there has been an increase in the number of these tumors managed at our center in recent years. Delayed presentation with large tumors is common. We studied the clinical profile, interventions, and outcomes of these tumors and assessed the factors influencing operative neurological morbidity and recurrence.
METHODS
This retrospective study was conducted at the Christian Medical College in Vellore, a tertiary care center in south India. We analyzed the inpatient and outpatient records of patients diagnosed to have carotid body tumors undergoing excision from January 1, 2005 to December 31, 2011. Patients diagnosed to have vagal paragangliomas were excluded.
RESULTS
Thirty-four of 48 tumors were excised from 32 patients (11 female, 21 male). Average age at presentation was 38.2 years, and three patients had familial bilateral tumors. All patients presented with a painless neck mass. There were 27 Shamblin group III, six Shamblin group II, and one Shamblin group I tumor. Eleven Shamblin group II/III tumors were associated with transient cranial nerve palsy or paresis (32.3%). Two Shamblin group III tumors were associated with perioperative stroke (5.8%). Preoperative embolization was done in 17 tumors, 12 of which were associated with neurological complications (two stroke, nine nerve palsy, one hemianopia). One patient underwent thrombolysis for a middle cerebral artery thrombus and recovered completely on follow-up, and another with a capsuloganglionic infarct managed conservatively had minimal persistent disability. Three patients had persistent nerve palsy (8.8%). Although complications were more common in patients with higher Shamblin group tumors, the difference was not statistically significant.
CONCLUSIONS
The overall rate of neurological complications is higher with tumors of higher Shamblin groups. Preoperative embolization was not effective in reducing neurological complications. The rates of postoperative stroke and permanent cranial nerve palsy after resection of large tumors are acceptable.
Topics: Adult; Carotid Body Tumor; Chi-Square Distribution; Cranial Nerve Diseases; Embolization, Therapeutic; Female; Humans; India; Male; Nervous System Diseases; Retrospective Studies; Stroke; Time Factors; Treatment Outcome; Tumor Burden; Vascular Surgical Procedures
PubMed: 23336858
DOI: 10.1016/j.jvs.2012.06.114 -
Intractable & Rare Diseases Research Aug 2012Carotid body tumour is a rare disease, a slow growing highly vascular tumour of the carotid body tissue and the most common type of the paraganglioma. This article... (Review)
Review
Carotid body tumour is a rare disease, a slow growing highly vascular tumour of the carotid body tissue and the most common type of the paraganglioma. This article reviews the pathological, clinical and ultrasound features of carotid body tumours and discusses the role of duplex ultrasound in the diagnosis and assessment of this condition. The initial presentation of carotid body tumour is usually a painless palpable neck mass. Some patients may experience local pressure symptoms as well as symptoms from vagal, hypoglossal and cervical sympathetic nerve impingement. Percutaneous needle aspiration or incisional biopsy is contraindicated for the diagnosis of carotid body tumours. Duplex ultrasound, computed tomography scan, magnetic resonance scan and angiography are commonly used diagnostic tools for this condition. Complete surgical excision of carotid body tumour is the treatment of choice as radiation therapy and chemotherapy are unsatisfactory. Based on vascularity and location, duplex ultrasound scan is able to diagnose carotid body tumour and differentiate it from many other masses in the neck. This non-invasive, inexpensive and readily available diagnostic tool can be used as a first-line imaging modality for the diagnosis and assessment of carotid body tumours.
PubMed: 25343084
DOI: 10.5582/irdr.v1.3.129