[Off-pump Resection of Dopamine-secreting Cardiac Paraganglioma].

Dopamine-secreting paragangliomas is known to be rare. The average annual incidence rate was reported 0.8 per 100,000 person-years. Approximately 1 to 2% of paragangliomas occur in the chest. We describe a patient with a large dopamine-secreting cardiac paraganglioma, right adrenal tumor and carotid body tumor. A 26-year-old man with progressive exertional dyspnea was referred to our hospital for further management of multiple paragangliomas. Positron emission tomography (PET) and PET-computed tomography (CT) detected those three legions. The diameter of cardiac paraganglioma was over 45 mm and was biggest among three tumors. Firstly, therefore, we planned cardiac paraganglioma resection. Through left lateral thoracotomy in the 4th intercostal space via, cardiac paraganglioma was resected under cardiopulmonary bypass and beating heart. Postoperative course was uneventful. Tumor cells were positive for synaptophysin and chromogranin A. Free metanephrines in the serum and urinary fractionated metanephrines normalized after cardiac surgery while the other two tumors remained untreated.

[Clinical analysis of robotic-assisted surgery in the treatment of large pheochromocytoma/paraganglioma: a study of 29 cases].

This study aimed to compare the safety and clinical efficacy of Da Vinci robotic surgery (control group) versus traditional laparoscopic surgery in the treatment of large (tumor size >6 cm) pheochromocytomas/paragangliomas. The evaluation was based on intraoperative metrics such as operative time, blood loss, conversion to open surgery rates, postoperative drain duration, and length of postoperative hospital stay. A retrospective analysis was conducted on 29 patients (14 males and 15 females) who underwent Da Vinci robotic surgery for large (greater than 6 cm) pheochromocytomas/paragangliomas from October 2019 to September 2023. The average maximum tumor diameter was (7.2±1.6) cm. During the same period, 32 patients underwent laparoscopic resection of challenging pheochromocytomas/paragangliomas.Data showed no significant differences between the two groups, except for the norepinephrine levels. Operative time, blood loss, conversion rate, postoperative drainage duration, and length of hospital stay were recorded and compared between the two groups. The differences between the groups were analyzed using the t-test, and the conversion rates were compared using the chi-square (χ²) test. All surgeries were successfully performed. One patient underwent a one-stage bilateral tumor resection. In the Da Vinci robotic surgery group, the average operative time was (76±32) minutes, compared to (106±45) minutes in the control group (P=0.003). The average intraoperative blood loss was (95±75) ml, compared to (160±90) ml in the control group (P=0.019). The conversion rate to open surgery was 2 cases (6.9%) in the robotic group compared to 5 cases (15.6%) in the control group (P=0.031). The average postoperative drainage duration was (3.0±1.0) days, compared to (3.5±1.5) days in the control group (P=0.128). The average length of hospital stay was (4.5±1.2) days, compared to (4.7±2.2) days in the control group (P=0.657). Postoperative pathological diagnosis confirmed pheochromocytoma or paraganglioma. Blood pressure normalized within 1 to 3 months postoperatively, with an average systolic blood pressure of (116±13) mmHg(1 mmHg=0.133 kPa）and diastolic blood pressure of (73±5) mmHg in the robotic surgery group. Follow-up for 3 to 40 months showed normal levels of blood catecholamines and no recurrence or metastasis on follow-up imaging studies, including chest, abdominal, and pelvic CT scans. The average levels of 3-methoxy-norepinephrine, 3-methoxy-epinephrine, and 3-methoxy-tyramine were (0.42±0.21) nmol/L (normal value≤1.05 nmol/L), (0.11±0.07) nmol/L (normal value≤0.32 nmol/L), and (0.017±0.006) nmol/L (normal value≤0.036 nmol/L), respectively. This study demonstrates that for the treatment of large pheochromocytomas/paragangliomas, the Da Vinci robotic technique offers advantages such as high-definition visualization, precise operation, and flexibility. These advantages enable the safe and efficient completion of surgeries, as evidenced by shorter operative times, less blood loss, and lower conversion rates.

Cardiac Paraganglioma in a Young Patient Presents with Angina-like Symptoms: A Case Report and Literature Review.

Paragangliomas are rare extra-adrenal neuroendocrine tumors originating from chromaffin tissue that present a diagnostic and therapeutic challenge due to their diverse clinical manifestations and low incidence. While these tumors often manifest as catecholamine-secreting functional tumors, their clinical presentation can vary, leading to delayed diagnosis and challenging management. This study presents the case of a 22-year-old patient with cardiac paraganglioma who initially presented with angina-like symptoms, highlighting the importance of considering this rare condition in young individuals with nonspecific complaints. Diagnostic imaging, including transthoracic echocardiography, CT angiography, and MRI, played a crucial role in identifying the tumor's location and vascularization. Surgical excision, including pulmonary artery graft and CABG, was the primary management approach, which was accompanied by intraoperative complications that later led to CCU admission, followed by postoperative complications, ultimately leading to the patient's death. This case highlights the significance of early recognition and management of complications following a surgical approach to treat paragangliomas.

Paediatric renal hilar paraganglioma.

Retroperitoneal neuroendocrine tumours are exceptionally rare. The excision of tumours located in the renal hilum near the renal vessels can be challenging. We report a case of a paraganglioma located at the renal hilum which was excised successfully in a child who presented with abdominal pain, breathlessness, left varicocele and hypertension.

Paraganglioma-induced pheochromocytoma crisis successfully treated by veno-arterial extracorporeal membrane oxygenation: a case report.

Pheochromocytoma crisis is rare but potentially fatal if not recognized early and properly managed. Here, a woman in her 20s with a paraganglioma-induced pheochromocytoma crisis, who was successfully treated by veno-arterial extracorporeal membrane oxygenation (VA-ECMO) and interval tumor resection, is described. In July 2022, the patient was brought to hospital with a complaint of sudden-onset of palpitations with vomiting. The patient developed cardiorespiratory failure with hypoxia. Computed tomography scan showed pulmonary oedema and a mass anterior to the inferior vena cava. She was transferred to the intensive care unit and treated with VA-ECMO. The patient's ECMO was withdrawn after 6 days without any complications. After hemodynamic stabilization, the patient underwent tumor resection 4 months later. The postoperative course was uneventful and she was discharged on postoperative day 7. Histopathological analysis confirmed a paraganglioma. VA-ECMO may play a significant role in saving lives and providing time for accurate diagnosis and specific treatment of a patient with pheochromocytoma crisis. Appropriate individual management can help avoid the occurrence of ECMO complications.

[Baroreflex failure syndrome after the resection of complex paragangliomas of head and neck: a case report].

Protection of cranial nerves is one of the major challenges in the resection of paragangliomas of head and neck, especially in complex paragangliomas. We report a case of bilateral jugular tumor with unilateral carotid body tumor. Baroreflex failure syndrome（BFS） occurred after staged resection of bilateral lesions. There is still a lack of effective treatment for this complication. More prudent and reasonable treatment strategy is important to reduce the incidence of BFS.

[Exploration of technique for preservation of external-middle ear structure in surgery of jugular foramen paraganglioma （appended 2 case reports）].

Objective:To investigate the feasibility and effect of the modified surgery of the classic infratemporal fossa type A approach for the surgical treatment of jugular foramen paraganglioma with preservation of the external and middle ear structures. Methods:The medical data of 2 patients with jugular foraminal paraganglioma treated by sublabyrinthic-transmastoid approach were retrospectively analyzed. The clinical feature, degree of tumor resection, postoperative facial nerve function and hearing retention, and the incidence of postoperative complications were evaluated. Results:Two patients were both female, and were pathologically confirmed as paraganglioma. The tumor of case 1 was staged as C2De1, and case 2 as C1De1. Tumors were completely resected in both patients. Case 1 suffered infection after surgery, with residual tympanic membrane perforation and mixed deafness. Case 2 developed mild facial paralysis（grade Ⅱ） after surgery, and recovered after symptomatic treatment. There was no tumor residue or recurrence during half a year of follow-up. Conclusion:Surgical treatment of certain paragangliomas in the jugular foramen with a combined sublabyrinthic-transmastoid and upper neck approach might achieve both complete resection of the tumor and preserving the structure and function of the outer-middle ear. This procedure is suitable for paragangliomas restricted in the jugular foramen area, with no or limited involvement of the internal carotid artery（C1 or C2）, and with no or mild hearing loss.

[A case report of giant jugular foramen paraganglioma].

To investigate the clinical experience of glomus jugulare paraganglioma by presenting a case of giant glomus jugulare paraganglioma. The clinical data of 1 case of giant glomus jugulare paraganglioma with unilateral anacousia and pulsatile tinnitus admitted to our department was retrospectively analyzed, and the relevant literature was reviewed to summarize the characteristics of the disease. The tumor tissue in the jugular venous foramen region was completely resected, with complete preservation of the facial nerve during the operation. There was no tumor recurrence during the 2-year postoperative follow-up period. With nonspecific clinical symptoms and a high rate of early misdiagnosis The giant glomus jugulare paraganglioma case only manifested as symptoms of unilateral anacousia and pulsatile tinnitus is clinically rare. The intraoperative safe resection of the tumor, maximum preservation of facial nerve function remains the focus of surgery.

A rare association of pheochromocytoma, paraganglioma, and pituitary adenoma (3PA): A case report and literature review.

RATIONALE: 3P association (3PA) is a rare condition with co-occurrence of pituitary adenoma and pheochromocytoma/paraganglioma. There have been less than a hundred documented cases of 3PA, which can be sporadic or related to genetic mutations. The present case report describes the first Iranian patient with 3PA and a 90th case of 3PA in the available literature. PATIENT CONCERNS AND INTERVENTIONS: A 36-year-old Caucasian male was admitted with headache and sudden increase in blood pressure. An abdominal CT scan revealed a retroperitoneal mass posterior to the inferior vena cava, later removed and diagnosed as a pheochromocytoma. Four years later, he noticed occasional mild headaches and a painless mass on the right side of his neck. The ultrasonography evaluations suggested a carotid body tumor, which was surgically removed. About a month after his second surgery, the severity of the patient's headaches worsened, and he developed right homonymous hemianopia. A brain MRI showed a mass in favor of macroadenoma, craniopharyngioma, or meningioma, and elevated prolactin level led to the diagnosis of macroprolactinoma. DIAGNOSES: Based on the provided history, this patient was diagnosed with 3PA, and a genetic study identified a positive succinate-dehydrogenase-complex subunit b mutation, possibly linked to his family history of carotid body tumor. OUTCOMES: He has remained symptom-free during his visits every 3 months. LESSONS: The number of cases diagnosed with 3PA worldwide is increasing. Using clinical and genetic assessments, we can timely diagnose and adequately monitor individuals with or at risk of 3PA.

[Simultaneous occurrence of facial neurinoma in internal auditory canal and middle ear paraganglioma in patient. Unusual combination and difficult surgical task]. / Odnovremennoe vozniknovenie nevrinomy litsevogo nerva vo vnutrennem slukhovom prokhode i paragangliomy srednego ukha u patsientki: neobychnoe sochetanie i neprostaya khirurgicheskaya zadacha.

CLINICAL CASE: The 59-year-old patient complained of hearing loss on the left, ear murmur for a long time, periodic pain and discomfort in the left ear, dizziness for 6 months. She was found to have concurrent vestibular schwannoma in the internal auditory canal and temporal bone paraganglioma. Both tumors were removed in one operation. The schwannoma was removed by translabirinth access due to preoperative deafness, while the glomus tumor was removed during this access. Postoperative biopsy showed the presence of two unrelated diseases: paraganglioma (ICD-0 code 8690/3) and schwannoma (ICD-0 code 9560/0).

[Application of internal carotid artery stent in glomus jugular paraganglioma surgery].

Objective:To summarize the application of internal carotid artery stent in glomus jugular paraganglioma surgery, and to provide an effective strategy for reducing the risk of internal carotid artery injury. Methods:This article reviewed the surgical cases of internal carotid artery stent implanting from 2018.06 to 2022.12, and discussed the stent placement method, treatment protocols, and perioperative management strategies. Results:A total of 5 patients underwent a comprehensive evaluation of the degree of internal carotid artery invasion using imaging techniques such as MRI, carotid CT angiography （CTA）, and digital silhouette angiography （DSA）. All patients were found to have varying degrees of internal carotid artery involvement. Stenting of the internal carotid artery was performed in all patients before surgery, and the stenting process went smoothly without any internal carotid artery injury. Three months after stenting, tumor resection or subtotal resection surgery was performed to avoid internal carotid artery injury during the surgery, and the surgical process was successfully completed. Postoperative follow-up from 4 months to 2 years showed that the internal carotid artery was patent after stent placement, with great endothelialization process and no stent-related complications. Conclusion:In patients with glomus jugular paraganglioma, when preoperative imaging shows internal carotid artery involvement, preoperative stenting is a safe and effective therapeutic strategy to reinforce the arterial wall structure, protect and maintain the integrity of the artery, and reduce the risk of vascular injury during the surgery. This article summarizes the experience of internal carotid artery stent in glomus jugular paraganglioma surgery, which provides an important reference for clinical practice.

[The surgical outcomes in management of jugular foramen paragangliomas with tension free anterior rerouting of the facial never and tunnel packing of the inferior petrous sinus].

Objective:The aim of this study is to evaluate the safety and efficacy of surgical interventions ofjugular foramen paragangliomas（JFP） utilizing modified surgical techniques, tensionfree anterior rerouting of the facial nerve and tunnel-packing or push-packing of the inferior petrous sinus. Methods:A retrospective analysis was conducted on a cohort of 88 patients diagnosed with JFP and treated at the Eye Ear Nose and Throat Hospital of Fudan University（in Shanghai, China） from October 2010 to June 2021. The surgical outcomes were analyzed for tumor classification, intraoperative conditions, and function of the postoperative facial nerve（FN） and lower cranial nerve（LCN）. Results:The study included a total of 88 patients, gross total resection was achieved in 70 patients（79.5%）, near total resection was obtained in 17 patients（19.3%）, and one patient undergoing subtotal resection. The average of intraoperative blood loss was 448.3 mL. Additionally, 24 patients underwent surgical total anterior rerouting（TAR）, 18 patients underwent surgical total FN tension free anterior rerouting（TF-TAR）, and 18 patients underwent surgical FN partial FN tension free anterior rerouting（TF-PAR）. Good postoperative FN function（House-Brackmann Ⅰ-Ⅱ） was achieved in 62.5% of TAR group. In the TF-TAR and PF_TAR groups, good postoperative FN function was demonstrated in 88.9% patients. It showed a significantly improvement of the FN function following application of tension-free FN anterior rerouting technique（P=0.007）. Twenty patients（22.7%） suffered from at least one LCN deficit in the preoperative evaluation. The postoperative LCN deficits was correlated with the Fisch classification of tumors, which showed a lower incidence of LCN dysfunction in classes C1-C2（4.9%, 2/41cases） and poorer outcomes of LCN dysfunction in classes C3-D（8.5%,4/47cases ）, it was likely less impacted the LCN function in the early stage tumor. Conclusion:The application of modified surgical techniques of FN tension-free anterior rerouting and tunnel-packing of the inferior petrous sinus has been shown to effectively preserve the function of the FN and LCN, decrease intraoperative blood loss, and ultimately improve patients' postoperative quality of life.

[Reconstruction of internal carotid artery in the resection of paraganglioma of head and neck].

Objective:This study aims to discuss the necessity，surgical method and effect of reconstruction of internal carotid artery （ICA） in the resection of paraganglioma of head and neck. Methods:We retrospectively analyzed the data of the patients who underwent head and neck paraganglioma resection and ICA reconstruction in Peking Union Medical College Hospital from May 2015 to August 2023. The demographic characteristics, preoperative examinations, diagnoses, surgical techniques, and follow-up information were collected. Results:Six patients were enrolled, including four females and two males, with an average age of（39.8±13.0） years. All the patients presented mainly complaining local masses with or without pain. Four cases underwent surgery through Fisch infratemporal fossa approach type A+B, and one through approach type A and one through transcervical approach. Five patients had vascular reconstruction using the great saphenous vein while one patient used an artificial vessel. The average follow-up period was（43.8±31.6） months. One patient had tumor recurrence after 3 years and underwent second surgery to remove the residual tumor, and the other 5 patients had no tumor recurrence. One patient had acute bridging vessel embolism, and the other was found to have occlusion of the bridging vessel at follow-up. Conclusion:Carotid revascularization during resection of paraganglioma of head and neck is an important technique to completely remove the tumor, cure the disease, ensure intracranial blood supply, and reduce cerebrovascular complications. The "pre-reconstruction technique " is an effective method to minimize the duration of brain blood flow interruption, compared to traditional carotid revascularization. Close observation is necessary, along with regular radiological examinations to assess the patency of transplanted vessels, and the residual or recurrent tumors.

Gangliocytic paraganglioma: A case report.

Paragangliomas (PGLs) are rare and encapsulated neuroendocrine tumors (NET), located in the adrenal gland or the extra-adrenal paraganglia. Extra-adrenal PGLs may develop a gangliocytic component with ganglion cells which are called gangliocytic paragangliomas (GPs). The most common location is the duodenum, and they appear with digestive symptoms or as an incidental finding. We described a 43 years old patient, with epigastric pain, nausea and vomiting. The CT-scan reveals a nodular image in the duodenum. An ultrasound-guided FNA was performed and the pathological report revealed neuroendocrine cell groups and neural tissue. Surgery was the chosen treatment. As the patient did not present lymphatic or pancreatic parenchyma invasion, radiotherapy (RT) was not administered. The management of GPs is not well established and multidisciplinary team approach is recommended to lead to therapeutic options. Surgical resection is still key in the treatment, and adjuvant RT may be considered in cases of lymph node invasion.

The intrabulbar or extrabulbar growth pattern and its surgical outcomes of jugular foramen paragangliomas.

OBJECTIVE: This study is to define a subclassification system of jugular foramen paragangliomas (JFPs) and to demonstrate corresponding microsurgical outcomes of JFPs. STUDY DESIGN: Retrospective study. SETTING: A single-center study. METHODS: We conducted a retrospective review of the clinical data of 44 patients with JFPs who underwent surgical management. Extrabulbar(Be) tumor and intrabulbar(Bi) tumor are defined based on the growth patterns, receiver operating characteristic (ROC) curves of the imaging profile were generated and was confirmed based on intraoperative findings. Area Under Curve (AUC), accuracy, sensitivity, and specificity for diagnostic imaging were revealed. We also compared the correlation between the two growth patterns with Fisch's classification, blood loss, lower cranial nerves (LCNs) deficit. RESULTS: There are 27 (69%) cases of Bi tumor and 17 (39%) cases of Be tumor. Significant radiomics features between the two growth patterns were demonstrated, ROC curves achieved excellent AUCs for MRI sequences (T1W1 MRI, MR contrast-enhanced sequence, MR complex sequences and MR complex + DSA by 0.833, 0.833, 0.875, 0.944) and had statistically significant in diagnosis of two growth patterns (P<0.05). There was no statistical correlation between growth patterns of JFPs and intra-operative blood loss. Preoperative LCNs deficits and Fisch's classification of tumors were correlated with the growth patterns of JFPs (P < 0.05). CONCLUSION: We proposetd two growth patterns of JFPs in term of the inferior petrous sinus involvement. Identification of Bi or Be growth patterns preoperatively is helpful to design optimal surgical strategies and minimize postoperative complications.

Combined underwater endoscopic and microscopic surgery for tympanic paraganglioma: A case report.

The resection of middle ear paragangliomas can be challenging given their vascular nature and the small volume of the tympanic cavity, particularly when the tumor in the hypotympanum is close or attached to the internal carotid artery (ICA). We performed combined underwater endoscopic and microscopic surgery for a Class B1 middle ear paraganglioma according to the modified Fisch classification. The suspicious bone in the hypotympanum and around the petrous ICA was drilled with underwater endoscopy. The feeding arteries, the caroticotympanic and inferior tympanic arteries, were suctioned and cauterized under microscopy. To the best of our knowledge, no case of middle ear paraganglioma treated with underwater endoscopy has been reported. Underwater endoscopy, providing a clear operative field with blood and bone dust irrigation, is a good indication for middle ear paragangliomas. In contrast, microscopic preparation for unexpected bleeding is important, particularly when the tumor closely extends to vital structures, such as the ICA or the jugular bulb.

Surgical results, technical notes and complications of jugular foramen lesions via retroauricular infratemporal fossa approach.

OBJECTIVE: The objective of this study was to evaluate the clinical effect and safety of the postauricular infratemporal fossa approach (ITFA) in resecting jugular foramen lesions. METHODS: All 25 patients undergoing microsurgery via postauricular ITFA from March 2015 to May 2023 in the Department of Neurosurgery, Tangdu Hospital, Air Force Military Medical University were included. The clinical and radiological data were retrospectively analyzed. Regular follow-up was carried out. RESULTS: The mean age of all patients was 50.5±8.9 years, and 14 of them were female and 11 were male. Among the cases, lower cranial nerve schwannoma accounted for 60 % (15/25) of all tumors, jugular foramen paraganglioma accounted for 20 % (5/25), and the remaining 20 % included meningioma, chondrosarcoma, plasmacytoma, and salivary gland tumors. Total tumor resection was performed in 18 cases, subtotal tumor resection in 7 cases and partial resection in 1 case. Seven patients underwent gamma knife radiotherapy after surgery. Transient lower cranial nerve dysfunction occurred in 8 patients, and permanent lower cranial nerve dysfunction occurred in 2 patients after surgery. One patient developed facial paralysis, and one patient presented hearing loss. CONCLUSIONS: The postauricular ITFA achieved a relatively high total tumor resection rate and a lower incidence of neurological functional disorders. It is an alternative and suitable surgical approach for resecting jugular foramen lesions. Maximizing the preservation of neurological function is preferred, especially when radical resection cannot be achieved. Stereotactic radiotherapy could be used for residual tumors.

Analysis of clinical and pathological characteristics of retroperitoneal paraganglioma and associated prognostic factors.

BACKGROUND AND OBJECTIVES: The aim of this study is to explore the long-term prognostic risk factors associated with patients diagnosed with retroperitoneal paraganglioma (RPGL) and examine their clinical and pathological characteristics. METHODS: Expressions of biomarkers were identified using immunohistochemistry (IHC) and case databases were retrospectively searched. Survival analysis was performed using Kaplan-Meier and Cox risk regression to identify the factors that influence the postoperative progression-free survival of patients with RPGL. RESULTS: A total of 105 patients, most of whom had tumors situated in the paraaortic region, and whose average tumor size was 8.6 cm, were enrolled in this study. The average follow-up duration was 51 months, with a mortality rate of 19% and a recurrence and metastasis rate of 41.9%. Tumors were assessed using the modified Grading system for Adrenal Pheochromocytoma and Paraganglioma (GAPP), and SDHB, S-100, and Ki-67 were stained using IHC in all cases. Out of the total cases examined, negative in SDHB expression were observed in 18.1% of cases, S-100 expression was negative in 36.2% of cases, and endovascular tumor enboluswas present in approximately 25.7% of cases. The results of the univariate analysis indicated that several factors significantly influenced the progression-free survival of patients with PGL as follow: maximum tumor diameter (>5.5 cm), tumor morphological features, tumor grading (modified GAPP score > 6), SDHB negative, S-100 negative, and expression of proliferation index Ki-67 (>3%) (X2 = 4.217-27.420, p < 0.05). The results of the multivariate analysis indicated that negative of S-100 (p = 0.021) and SDHB (p = 0.038), as well as intravascular tumor thrombus (p = 0.047) expression were independent risk factors for progression-free survival in patients. CONCLUSION: RPGL is characterized by diverse biological features and an elevated susceptibility to both recurrence and metastasis. Both SDHB and S-100 can be employed as traditional IHC indicators to predict the metastatic risk of PGL, whereas the tumor histomorphology-endovascular tumor enbolus assists in determining the metastasis risk of RPGL.

[Paragangliomas of the head and neck]. / Paragangliome im Kopf-Hals-Bereich.

Paragangliomas represent a heterogeneous group of rare neuroendocrine tumors with marked variability in symptoms and disease course. Due to the close proximity to neurovascular structures, paragangliomas of the head and neck region can cause a variety of symptoms. To this day, there are no reliable prognostic factors that can predict a potentially malignant course. All patients with newly diagnosed paragangliomas should undergo an early diagnostic workup and regular follow-up examinations in specialized centers. While radical resection was previously regarded as standard treatment for paragangliomas, radiotherapy and active surveillance (watch-and-scan strategy) have become equally important over the years. Low-threshold techniques for molecular pathology analysis of the mutation-specific behavior of paragangliomas are nowadays available.