Paraganglioma Research Articles

Head and Neck Malignant Paragangliomas: Experience from a Single Institution.

Objectives: 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. Methods: 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. Results: 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 66months. 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. Conclusions: 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.

Republished: Manual reduction of a radial artery loop under direct fluoroscopic visualization

Transradial access has become increasingly used in neurointerventions because it reduces access site complications. However, radial artery anomalies can be difficult to navigate, often necessitating conversion to femoral access. We describe the case of a female patient in her early 70 s who underwent preoperative embolization of a carotid body tumor via right transradial access. Her radial angiogram demonstrated the presence of a radial artery loop which was successfully navigated with a triaxial system but would not spontaneously reduce even after the guide catheter was advanced into the subclavian artery. However, manual manipulation of the catheters in the antecubital fossa under direct fluoroscopic visualization reduced the loop allowing the procedure to continue transradially. Although a majority of radial loops can be traversed and reduced using standard techniques, this case demonstrates that manual reduction can be successful when other measures fail. We recommend attempting this method before converting the access site.

A 15-year pheochromocytoma and paraganglioma experience in a single centre: a Singapore perspective.

Pheochromocytomas (PCC) and paragangliomas (PGL) are rare endocrine tumours. The objective of this study was to describe our experience with these two entities in a Singapore population. We identified patients with positive histopathological confirmations of PCC and PGL who were treated at a tertiary Singapore hospital between January 2000 and December 2015. The results were analysed for clinical presentations, treatment and long-term outcomes. A total of 27 cases (20 PCC, 7 PGL) were identified over a 15-year period. One case of PGL developed bilateral disease on follow-up. There were 17 male and 10 female patients with a median age of 57 (range 24-77) years. A positive family history was uncommon and present in only 3.7% of patients. Uniquely, the top three presenting symptoms were abdominal discomfort, palpitations and diaphoresis. Despite adequate preoperative preparation, intraoperative haemodynamic instability occurred in 70.4% and early postoperative hypotension occurred in 11.1% of patients. After surgery, hypertension was resolved in 41.2% (7/17) and diabetes mellitus in 60% (3/5). Disease recurrence was reported in 22.2% and distant metastases in 14.8%. At the end of the follow-up period (median 35 [range 3-148] months), 70.4% were still alive. PCC and PGL can present with a wide range of symptoms. Intraoperative haemodynamic instability was frequent despite good preoperative preparation. Disease recurrences and metastasis occurred in up to one-fifth of the patients. Genetic screening should be offered to patients with PCC and PGL.

Challenging Anesthesia in Pediatric Pheochromocytoma and Paraganglioma Tumors Resection

Abstract Pheochromocytoma (PCC) and paraganglioma (PGL) are chromaffin cell tumors that secrete catecholamines and are some of the rarest pediatric tumors. Perioperative care poses a challenge for the anesthetist. Hemodynamic regulation, surgery manipulation, and perioperative care require special attention. These tumors provide major treatment challenges as well as a high risk of hypertensive crisis-related cardiovascular consequences. We present a successfully managed case of PCC removal in a 13-year-old male. He presented with typical hypertensive crisis symptoms (i.e., vomiting, headaches, and seizures), and he was managed well to prepare him for surgery. We anticipated hemodynamic alterations during the surgery and controlled them with a combination of antihypertension, vasodilator, and epidural analgesia. The safe perioperative care of such patients requires good communication between an experienced multidisciplinary team of surgeons, pediatric endocrinologists, and anesthetists.

Meta-Analysis of the Effects of Preoperative Embolization on the Outcomes of Carotid Body Tumor Surgery

ABSTRACT Background Carotid body tumors (CBTs) are situated at the bifurcation of the common carotid artery within the adventitia, and are reported to be the most common head and neck paragangliomas. Surgery is the gold standard for curative treatment of resectable CBTs and is recommended in otherwise healthy patients because of the risk of local complications related to tumor size and a small but definite risk of malignancy. Preoperative embolization has been shown to reduce potential intraoperative blood loss and provide the surgeon with greater ease and safety in excising the tumor, thus reducing the operation time and morbidity. However, other physicians have stated that although blood loss may be reduced after preoperative embolization, transfusion requirements are not affected, and that the embolization procedure adds a significant risk for stroke. Therefore, the purpose of the current study was to compare the surgical outcomes of patients undergoing CBT surgical resection with and without preoperative embolization. Aim To evaluate the need for preoperative embolization for the treatment of carotid body tumor. Methodology A meta-analysis study is done to compare the surgical outcomes of patients undergoing CBT surgical resection with and without preoperative embolization. Results Our meta-analysis for evaluation of the effects of preoperative embolization on the outcomes of carotid body tumor surgery, included (14) studies with a total number of patients (n = 477).The results of these studies showed no statistically significant difference between preoperative embolization group and non embolization group in carotid body surgery for (blood loss & operation time). Preoperative embolization did not reduce risk of postoperative complications. Conclusion Preoperative embolization shows no statistically significant reducing in blood loss and operation time, also embolization does not decrease incidence of postoperative complications. It seems that embolization should not be a routine part of carotid body tumor surgery especially with the known potential risks and complications of this procedure .

Shamblin III Chemodectoma: The vascular surgeon's point of view

Chemodectomas (CBTs) are the most frequently encountered tumours at the carotid bifurcation. Even if rare and commonly benign, their development close to the head and neck structures is often source of morbidity by compression and infiltration. Therefore, in order to avoid permanent neurologic or vascular complications these infrequent lesions need to be early removed. The total excision may be technically challenging and requires extensive expertise in surgical neck anatomy and a cooperative multidisciplinary approach. Almost a quarter of CBTs infiltrate carotid vessels so vascular expertise is pivotal in their resection. The present study aims to give an overview of vascular specificity and procedures required during surgical excision of such tumors to assist and guide treating physicians who encounter CBTs. This overview will particularly emphasize current therapeutic options: we discuss the referral criteria that should guide the decision about what type of preoperative care and surgical procedure should be offered to the affected patients. We also specify the epidemiologic data, screening recommendations and outcomes achieved with the different therapeutic approaches. The clinical, operative and follow-up data about a case of a Shamblin III carotid body tumour surgically removed by a multidisciplinary team at our Institution are also reported.

Special situations in pheochromocytomas and paragangliomas: pregnancy, metastatic disease, and cyanotic congenital heart diseases.

The aimof our study was to describe the epidemiology, diagnosis, and treatment of the most complex pheochromocytoma and paraganglioma (PGL) cases, including pheochromocytoma/PGL during pregnancy, in cyanotic congenital heart diseases (CCHDs), and metastatic pheochromocytoma. The English and Spanish literature was thoroughly evaluated searching for articles reporting clinical studies, case reports, or reviews of pheochromocytoma/PGL in pregnancy and in CCHD and metastatic pheochromocytoma/PGL. Particular settings in the diagnosis and management of pheochromocytoma and PGLs remain challenging. Those special situations include the diagnosis during pregnancy or in the context of CCHD since the typical clinical features of pheochromocytoma may be confounded with preeclampsia during pregnancy and with the complications commonly observed in CCHD. In addition, although some clinical and genetic features have been associated with higher risk of metastatic pheochromocytoma, the detection and prediction of the development of metastatic disease involve another complex situation that may require special hormonal determinations as plasmatic 3-methoxytyramine and nuclear medicine studies including 18FDG PET-CT or 18F-FDOPA PET-CT, among others. Furthermore, the selection of the most appropriate treatment in these situations, as well as the moment to carry it out, requires special care as limited evidence is available. This article reviews the epidemiology, diagnosis, and treatment of the pheochromocytoma/PGL during pregnancy, metastatic pheochromocytoma/PGL, and pheochromocytoma/PGL in CCHD. The diagnosis, and especially the treatment, of metastatic pheochromocytomas and pheochromocytoma/PGL during pregnancy and in CCHD is challenging. Thus, these cases should be management in reference centres by multidisciplinary teams specialized in the pheochromocytoma/PGL treatment.

Symptoms: Middle Ear Mass and Hearing Loss

Symptoms: Middle Ear Mass and Hearing Loss

Long-Term Results of Gamma Knife Radiosurgery for Glomus Tumors: An Analysis of 32 Patients.

BackgroundGlomus jugulare tumors are rare slow-growing hypervascular tumors that arise from the paraganglia of the chemoreceptor system within the jugulare foramen of the temporal lobe. The historical standard treatment has been surgical resection, but because of their high vascularity and involvement with cranial nerves (CNs), Gamma Knife radiosurgery (GKRS) has been advocated as an alternative. The goal of this study is to update and report long-term results of GKRS to achieve local control and symptomatic improvement and to reduce morbidity and mortality when treating glomus jugulare tumors.Materials and MethodsThis study retrospectively collected and reviewed clinical and radiographic data of 32 patients with glomus jugulare tumors treated with GKRS at the Miami Neuroscience Center, South Miami, FL, from 1995 to 2019. For the 32 patients, the mean volume treated was 13.9 cc (0.23 to 40.0 cc), with an average of 8.6 isocenters. The median prescription dose was 12.84 Gy ± 2.07 Gy (range: 10-20 Gy). Follow-up data were available for 29 out of 32 patients, with a median clinical follow-up time of 37.3 months (range: 4.3-169.1 months). At follow-up, patients were evaluated for neurological signs and symptoms and radiographic evidence of progression of disease.ResultsThe median age of the cohort treated with GKRS was 60 years (range: 14-83 years). There were three males and 27 females. Presenting symptomatology was available for 30 out of 32 patients. The most common presenting symptom was hearing loss (21/30) and the most common CN deficit was in CN VIII (19/30). Out of 29 of the patients followed up, 28 patients had improvement (20/29) or resolution (8/29) of symptoms. At the most recent evaluation or contact, patients were without symptomatic progression of CN deficits.Radiographic tumor control was achieved in 28 out of 29 patients. One patient had a recurrence seven years after GKRS, which was treated with surgery. There were no complications, radionecrosis, or mortality reported from GKRS.ConclusionThese data confirm that GKRS is a reasonable upfront treatment option for glomus jugulare tumors. GKRS should be considered more frequently given its excellent long-term local control with low morbidity and risk of complications.

The Influence of Young Age on Difficulties in the Surgical Resection of Carotid Body Tumors

Simple SummaryThe aim of this study was to reveal the factors affecting the complexity and difficulties in performing surgery to resect carotid body tumors (CBTs). We analyzed 20 patients with 21 CBTs. We used the “same day surgery” procedure, including preoperative embolization of the feeding arteries in the morning and resection surgery in the afternoon of the same day. Four patients underwent resection of the carotid artery, followed by reconstruction. These four patients were between 18 to 23 years of age at the time of surgery. The mean blood loss and operative time in these patients differed significantly from those in older patients. These results indicated that young age may influence the difficulties faced in CBT surgery, resulting in an increased risk of carotid artery resection. The results obtained from our study could help surgeons safely and effectively perform resection surgery for CBTs.This study evaluated patient characteristics that affect the complexity and difficulties of performing surgery to resect carotid body tumors (CBTs). We retrospectively reviewed the medical records of 20 patients with 21 CBTs who were enrolled in the study. The median patient age was 46 years and the mean tumor diameter was 37.6 mm. The mean blood loss and operative time were 40.3 mL and 183 min, respectively. Four patients underwent resection of the carotid artery followed by reconstruction. These four patients were between 18 to 23 years of age at the time of surgery. The mean blood loss and operative time in these patients were 166 mL and 394 min, respectively, which differed significantly from those of older patients. Therefore, young age influenced the difficulties faced in surgical resection of CBT, with an increased risk of blood loss and carotid artery resection.

P14.67 Long-term results of gamma knife radiosurgery for treatment of intracranial glomus jugulare tumors

Abstract BACKGROUND Glomus Jugulare tumors are benign but locally aggressive ones that represent a therapeutic challenge. Previous studies about the use of Gamma Knife Radiosurgery (GRS) in those tumors have documented good results that needed larger number of patients and longer follow up periods to be confirmed. MATERIAL AND METHODS Between August 2001 and December 2017, 70 patients with glomus jugulare tumors were treated at the Gamma Knife Center, Cairo. They were 46 females and 24 males. The mean age was 48 years (16–71 years). Nineteen of these patients were previously operated, 5 were partially embolized, 3 underwent embolization and subsequent surgery and 43 had gamma knife as their primary treatment. Volume-staged gamma knife radiosurgery was used in 10 patients and single-session in 60 patients, with a total of 86 sessions. The mean target volume was 12.7 cm3 (range 0.2 to 34.5 cm3). The mean tumor volume was 15.5 cm3 (range 0.2 to 105 cm3). The mean prescription dose was 14.5 Gy (range 12 to 18 Gy). RESULTS The mean follow up period was 60 months (range 18 to 206 months), and by the time of the data analysis, two of the patients were dead (66 and 24 months after GK treatment). The tumor control was 98.6% (69/70). Thirty-two tumors became smaller and 37 were unchanged. The symptoms improved in 36 patients, were stable in 32 patients, and worsened in 2 patients who developed a transient facial palsy and worsened hearing. Symptomatic improvement began before any reduction in tumor volume could be detected, where the mean time to clinical improvement was 7 months whereas the mean time to tumor shrinkage was 18 months. CONCLUSION This study about the long term follow up of the GKR for the intracranial glomus jugulare tumors confirmed that this is a highly effective and safe treatment. This data shows that the clinical improvement is not correlated with the radiological volume reduction.

Pitfalls in the management of subglottic paragangliomas at unusual location: a case report and literature review

BackgroundSubglottic paragangliomas (PGs) are exceptionally rare and unpredictable, occasionally presenting at an atypical location. There are three different clinical forms of subglottic PGs: intraluminal (tracheal PGs), extraluminal (thyroid PGs) and the mixed type (both intraluminal and extraluminal, mixed-subglottic PGs). These tumors are usually misdiagnosed as other relatively common primary thyroid or laryngotracheal tumors, and the treatment is troublesome.Case presentationA 22-year-old male patient with subglottic PGs has been successively misdiagnosed as thyroid tumors and subglottic hemangiomas, and lastly underwent local extended lumpectomy and laryngotracheal reconstruction with a pedicled thoracoacromial artery perforator flap (PTAPF). The patient was decannulated successfully after the second-stage tracheal reconstruction with a local flap, and no evidence of local recurrence and distant metastasis of the tumor until now.ConclusionSubglottic PGs can be easily misdiagnosed as laryngotracheal or thyroid tumors when presented at an atypical location. It is essential for otolaryngologists and head and neck surgeons to remain vigilant against these tumors. If the tumor is not diagnosed or removed completely, patients may encounter a risk of lethal paroxysm, which is incredibly troublesome.

Carotid Body Tumor, Cardiac Paraganglioma, Adrenal Pheochromocytoma: A Contest with Hypercatecholamineemia

This patient was an elderly man with left carotid body tumor, cardiac paraganglioma and right adrenal pheochromocytoma at the same time. This is a rare case with a complex condition, and the diagnosis and treatment were difficult. After two rounds of discussion in the multidisciplinary team, our hospital has successively removed the lesions of the heart and the right adrenal, and the patient was discharged smoothly after the operations. The diagnosis and treatment of this patient reflects the significance of the multidisciplinary team on the diagnosis and treatment of difficult diseases.

Carotid Body Tumor, an Experience with 100 Patients, a Brief Report

Carotid Body Tumor, an Experience with 100 Patients, a Brief Report

574P Clinical impact of the GAPP score and SDHB negativity in patients with pheochromocytoma/paraganglioma

Pheochromocytoma (PCC) and paraganglioma (PGL) are rare tumors occurring in the adrenal medulla and extra-adrenal tissues, respectively. The clinicopathological features have not been fully elucidated, especially in Asian cohorts.

The Utility of 68Ga-DOTATATE PET/CT in Localizing Primary/Metastatic Pheochromocytoma and Paraganglioma: Asian Indian Experience.

Purpose:Pheochromocytoma and paraganglioma (PGL), together called PPGL, are rare tumors with a limited number of studies on the diagnostic performance of 68Ga-DOTA (0)-Tyr (3)-octreotate positron emission tomography-computed tomography (68Ga-DOTATATE PET/CT) from the Asian-Indian subcontinent.Materials and Methods:In this retrospective study, PPGL suspects (n = 87) who had undergone at least contrast-enhanced computed tomography (CECT) and 68Ga-DOTATATE PET/CT, were included. Lesion-wise, patient-wise, and region-wise sensitivities of 68Ga-DOTATATE PET/CT, 18F fluorodeoxyglucose positron emission tomography CT (18F-FDG PET/CT, n = 53), 131I-metaiodobenzylguanidine (131I-MIBG, n = 37), and CECT were compared, and diagnostic performance of 68Ga-DOTATATE PET/CT in the detection of PPGL was calculated.Results:68Ga-DOTATATE PET/CT had significantly higher lesion-wise sensitivity than 131I-MIBG for both primary (94% vs 75%, P = 0.004) and metastatic disease (85% vs 59%, P = 0.001) and higher sensitivity than CECT for metastatic lesions (83% vs 43%, P = 0.0001). The lesion-wise sensitivity of 68Ga-DOTATATE PET/CT was similar to 18F-FDG PET/CT for both primary tumors (94% vs 85%, P = 0.08) and metastatic lesions (82% vs 84%, P = 0.76) in the whole cohort but tended to be inferior in the head to head comparison.Conclusion:68Ga-DOTATATE PET/CT had higher sensitivity for detection of PPGL than 131I-MIBG (primary and metastatic) and CECT (metastatic) but similar to 18F-FDG PET/CT (primary and metastatic).

Development and validation of an improved classification and risk stratification system for carotid body tumors: Multinational collaborative cohort study.

This study aims to develop and validate a new classification system that better predicts combined risk of neurological and neurovascular complications following CBT surgery, crucial for treatment decision-making. Multinational retrospective cohort study with 199 consecutive cases. A cohort of 132 CBT cases was used to develop the new classification. To undertake external validation, assessment was made between the actual complication rate and predicted risk by the model on an independent cohort (n=67). Univariate analyses showed statistically significant associations between developing a complication and the following factors: craniocaudal dimension, volume, Shamblin classification, and Mehanna types. In the multivariate prognostic model, only Mehanna type remained as a significant risk predictor. The risk of developing complications increases with increasing Mehanna type. We have developed and then validated a new classification and risk stratification system for CBTs, which demonstrated better prognostic power for the risk of developing neurovascular complications after surgery.

Treatment Outcomes of Advanced Carotid Body Tumors in a High-Altitude Tertiary Referral Center

Chronic hypoxia-driven hyperplasia of the carotid body is considered a major risk factor for carotid body tumor (CBT). Our large referral base in a high-altitude region provides an ideal opportunity to examine the morbidity of CBT resection in a diverse sample of presentations.

Safety of Nonoperative Management of Carotid Body Tumors

Carotid body tumors (CBTs) are rare neuroendocrine paragangliomas that are typically asymptomatic and benign with a low rate of biochemical functionality. Historically, early surgical excision was recommended to prevent development of CBT-related complications. Yet, CBT resection can result in significant injury to cranial nerves and the carotid artery. Recent work has shown successful primary observation without resection of noncarotid body, cranial paragangliomas (glomus jugulare, tempanyomastoid, vagal body) with slow growth and a low rate of neuropathies.

Does Shamblin classification affect the clinical outcomes following carotid body tumor excision?

Cardiovascular Surgery and Interventions publishes original articles on topics in cardiovascular surgery, cardiovascular anesthesia and cardiology. These encompass all relevant clinical, surgical and laboratory studies, editorials, current and collective reviews, technical know-how papers, case reports, interesting images, “How to Do It” papers, correspondence and commentary.