Introduction: Paragangliomas are rare neuroendocrine tumors arising from extra-adrenal chromaffin cells, with an estimated incidence of 2–8 cases per million per year. These tumors originate from neural crest–derived cells of the sympathetic and parasympathetic paraganglia and may secrete catecholamines, resulting in malignant hypertension or symptoms such as headache, palpitations, and diaphoresis. They can occur anywhere along the paravertebral and para-aortic regions from the skull base to the pelvic floor. Case: We report a case of a 14-year-old male with no known premorbid conditions who presented with presyncope and a 1-month history of headache. On examination, he had severe hypertension (242/167 mmHg), tachycardia (127 bpm), and grade IV hypertensive retinopathy. Investigations showed preserved renal function with markedly elevated 24-hour urinary metanephrines (normetanephrine 90.75 µmol/L). Computed tomography revealed a lobulated, heterogeneously enhancing mass measuring 5.0 × 6.1 × 5.4 cm along the left margin of the abdominal aorta at the infrarenal level, suggestive of an extra-adrenal lesion. Gallium-68 PET scan demonstrated a somatostatin receptor–avid left peritoneal mass. The patient underwent exploratory laparotomy and tumor excision, complicated intraoperatively by blood pressure lability requiring nitroprusside and inotropic support. Postoperatively, he improved significantly and was able to wean off all antihypertensive medications. Histopathology confirmed left retroperitoneal paraganglioma. Conclusion Primary peritoneal paraganglioma is a rare but important cause of secondary hypertension, especially in young patients presenting with hypertensive emergency. High index of suspicion is essential for early diagnosis. Management requires a multidisciplinary approach with careful preoperative optimization to minimize perioperative complications. Surgical resection remains the definitive treatment and, as demonstrated in this case, can result in marked clinical improvement with resolution of hypertension.
Paraganglioma

Introduction: Mediastinal paragangliomas (PGLs) are extremely rare extra-adrenal neuroendocrine tumors, accounting for approximately 2% of all PGLs and commonly associated with pathogenic germline variants (PGVs), particularly those involving succinate dehydrogenase (SDH) mutations. Case: We report a 35-year-old female presenting with chronic cough and hemoptysis, accompanied by paroxysmal symptoms and new-onset hypertension. She had a history of pheochromocytoma treated 15 years earlier with right adrenalectomy and liver lobectomy due to intraoperative adrenal adherence to the liver. Histopathology confirmed adrenal pheochromocytoma, with no evidence of PGL in the liver. She remained in biochemical remission for 3 years but subsequently defaulted. Biochemical evaluation revealed markedly elevated 24-hour urinary normetanephrine (58,950 nmol/L; ~26-fold increase). Computed tomography of the thorax demonstrated a mediastinal mass compressing the right bronchus, resulting in luminal narrowing and segmental lung collapse. Endobronchial biopsy confirmed PGL (Ki-67 index 5%). Functional imaging with DOTATATE, FDG-PET, and MIBG demonstrated metastatic disease involving the lungs and lymph nodes. Genetic testing identified a heterozygous pathogenic SDHB mutation (c.79C>T; p.Arg27), consistent with autosomal dominant hereditary PGL-pheochromocytoma syndrome; family screening confirmed the same mutation in her father and two siblings. She underwent two sessions of bronchoscopic intervention, including cryoablation, balloon dilation, argon plasma coagulation, and intratumoral alcohol injection for airway control and hemoptysis. Multidisciplinary evaluation deemed complete surgical resection high risk and not feasible; therefore, peptide receptor radionuclide therapy was initiated. Conclusion This case demonstrates the aggressive and metastatic nature of SDHB-mutated PGLs. This group of patients require long term surveillance given the risk of developing new tumors even years or decades after primary tumor resection. It highlights the importance of genetic testing following pheochromocytoma surgery to guide targeted therapy, lifelong surveillance, and family screening within a multidisciplinary care approach.
Paraganglioma

Objective:Paragangliomas （PGLs） are chromaffin cell tumors originating from paraganglia and are classified as neuroendocrine neoplasms.They predominantly occur along the distribution area of the paraganglia, commonly occurring between the ages of 20 and 40, with a slight male predominance.They are most frequently found in the axial regions from the skull base to the pelvic cavity. Paragangliomas in the head and neck region typically lack endocrine functionality and primarily manifest through local mass effects. However, clinical signs and symptoms alone cannot reliably distinguish between metastatic and non-metastatic cases. Clinically apparent metastatic paragangliomas are relatively rare. Herein, we present a case of a paraganglioma located in the region of the jugular foramen with liver, bone, and lymph node metastases, and discuss the treatment and prognosis of head and neck paragangliomas.
Humans ; Head and Neck Neoplasms/pathology* ; Jugular Foramina/pathology* ; Liver Neoplasms/secondary* ; Lymphatic Metastasis ; Paraganglioma/pathology*

Objective: To investigate the clinicopathological features of glomuvenous malformation (GVM). Methods: Thirty-one cases of GVM diagnosed at the Henan Provincial People's Hospital from January 2011 to December 2021 were collected. Their clinical and pathological features were analyzed. The expression of relevant markers was examined using immunohistochemistry. The patients were also followed up. Results: There were 16 males and 15 females in this study, with an average age of 11 years (range, 1-52 years). The locations of the disease included 13 cases in the limbs (8 cases in the upper limbs, 5 cases in the lower limbs), 9 cases in the trunks, and 9 cases in the foot (toes or subungual area). Twenty-seven of the cases were solitary and 4 were multifocal. The lesions were characterized by blue-purple papules or plaques on the skin surface, which grew slowly. The lumps became larger and appeared to be conspicuous. Microscopically, GVM mainly involved the dermis and subcutaneous tissue, with an overall ill-defined border. There were scattered or clustered irregular dilated vein-like lumens, with thin walls and various sizes. A single or multiple layers of relatively uniform cubic/glomus cells were present at the abnormal wall, with scattered small nests of the glomus cells. The endothelial cells in the wall of abnormal lumen were flat or absent. Immunohistochemistry showed that glomus cells strongly expressed SMA, h-caldesmon, and collagen IV. Malformed vascular endothelial cells expressed CD31, CD34 and ERG. No postoperative recurrence was found in the 12 cases. Conclusions: GVM is an uncommon type of simple venous malformation in the superficial soft tissue and different from the classical glomus tumor. Morphologically, one or more layers of glomus cells grow around the dilated venous malformation-like lumen, which can be combined with common venous malformations.
Male ; Female ; Humans ; Child ; Glomus Tumor/surgery* ; Endothelial Cells/pathology* ; Paraganglioma, Extra-Adrenal/pathology* ; Immunohistochemistry

Humans ; Laryngeal Neoplasms/surgery* ; Paraganglioma/surgery*

OBJECTIVE: To explore the surgical strategy and experience of reoperation for pheochromocytoma and paraganglioma which is very challenging. METHODS: The clinical data of 7 patients with pheochromocytoma and paraganglioma who underwent reoperation in Department of Urology, Peking University Third Hospital from August 2016 to February 2021 were analyzed retrospectively. There were 4 males and 3 females, with an average age of (44.1±11.5) years (28-60 years), 6 cases on the right side and 1 case on the left side. The causes of the operations included: (1) 2 cases of tumor recurrence after resection; (2) The primary operations failed to completely remove the tumors in 3 cases, because the tumors were large and closely related to blood vessels. (3) Pheochromocytoma and paraganglioma wasn't diagnosed before primary operation, therefore, drug preparation wasn't prepared. Two cases were interrupted by severe blood pressure fluctuations during the primary operations. Imaging evaluation, catecholamine biochemical examination and adequate adrenergic α receptor blockers were administrated in all the cases. The surgical approaches included open transperitoneal surgery in 4 cases, robot-assisted laparoscopy in 1 case and retroperitoneal laparoscopy in 2 cases. The innovative techniques included mobilization of the liver, inferior vena cava transection and anastomosis, and transection of left renal vein. RESULTS: The average tumor size was (8.0±3.2) cm (3.6-13.9 cm). The median interval between the reoperation and the primary operation was 9 months (IQR: 6, 19 months). The median operation time was 407 min (IQR: 114, 430 min) and the median blood loss was 1 500 mL (IQR: 20, 3 800 mL). Operations of 5 cases were performed successfully, and 1 case failed only by exploration during the operation. One case died perioperatively. There were 5 cases of intraoperative blood transfusion, the median transfusion volume of red blood cells was 800 mL (IQR: 0, 2 000 mL). One case experienced postoperative lymphorrhagia, and recovered after conservative treatment. The renal function was normal in 2 cases after resection and anastomosis of inferior vena cava or transection of left renal vein. The average postoperative hospital stay was (7.2±3.3) d (4-13 d). The median follow-up time of 6 patients was 33.5 months (IQR: 4.8, 48.0 months). The case who failed in the reoperation still survived with tumor and there was no recurrence in the rest of the patients. CONCLUSION The reoperation of pheochromocytoma and paraganglioma, which can not be resected in the primary operation or recurred postoperatively, is difficult with high risk of hemorrhage, and there is a risk of failure and perioperative death. Different surgical approaches and strategies need to be adopted based on the different situation.
Adrenal Gland Neoplasms/surgery* ; Adult ; Female ; Humans ; Laparoscopy ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; Paraganglioma/surgery* ; Pheochromocytoma/surgery* ; Reoperation ; Retrospective Studies

Humans ; Paraganglioma/complications* ; Retroperitoneal Neoplasms/diagnostic imaging* ; Retroperitoneal Space ; Tachycardia

OBJECTIVE: Plasma free metanephrines (MNs) have been widely used as an initial test for pheochromocytoma and paraganglioma (PPGL). PPGL without MNs elevation has been reported on rare occasions. The objective of this study was to analyze the clinical profile of sporadic PPGL patients with normal MNs. METHODS: In the study, 104 patients with sporadic PPGL diagnosed by histopathology in Peking University First Hospital from March 2015 to January 2020 were enrolled. All the patients had plasma MNs result, of whom, eight (7.69%) were with normal MNs. The reasons for their medical visits, clinical manifestations, the levels of plasma free MNs, 3-methoxytyramine (3-MT), catecholamines and chromogranin A (CgA), and the imaging findings were documented. Their preoperative diagnosis, perioperative medical management, and intraoperative blood pressure were analyzed. All the data mentioned above were compared with the MNs elevated group. The postoperative follow-up for MNs normal patients were applied. RESULTS: For the eight PPGL patients with normal plasma MNs, the most common clinical symptoms were sweating (3/8), abdominal and back pain (3/8), headache (2/8), palpitations (2/8), and fatigue (2/8). There were no significant differences in plasma free 3-MT and catecholamines' diagnostic positive rate between the MNs normal group and MNs elevated group, but the rate for plasma CgA was significantly decreased in the MNs normal group (2/5 vs. 41/43, P=0.005). No significant difference was found for the incidence of typical findings by enhanced CT between the two groups. In these eight MNs normal patients, six were diagnosed with PPGL by the previous history of PPGL, typical symptoms and CT findings, or elevation of 3-MT, CgA levels or positive results of PET-CT; two patients were misdiagnosed as nonfunctioning adenoma or primary aldosteronism. All these MNs normal patients underwent preoperative management with alpha adrenergic receptor blockers, of whom, one had an average intraoperative arterial pressure < 60 mmHg during surgery. The median follow-up time for the eight patients was 1.5 (0.5－4.5) years. No evidence of new tumors was found on the enhanced CT scans. Two MNs normal patients' plasma 3-MT and (or) CgA decreased to normal. CONCLUSION For patients with adrenal or retroperitoneal tumors, typical symptoms or a previous history of PPGL, normal plasma MNs is not a sufficient exclusion for PPGL. Plasma 3-MT, catecholamine, CgA results and the imaging findings are helpful for the diagnosis of PPGL. We recommend patients with suspected MNs normal PPGL take alpha adrenergic receptor blockers as preoperative blockade, but should avoid overdose. Postoperative follow-up for patients with normal MNs should focus on the positive biochemical markers before surgery.
Adrenal Gland Neoplasms ; Humans ; Metanephrine ; Paraganglioma ; Pheochromocytoma ; Positron Emission Tomography Computed Tomography

Acute Kidney Injury ; diagnostic imaging ; etiology ; Aged ; Humans ; Male ; Necrosis ; diagnostic imaging ; etiology ; Paraganglioma ; complications ; Postoperative Complications ; Retroperitoneal Neoplasms ; complications ; Skin ; diagnostic imaging ; pathology ; Tomography, X-Ray Computed

A 36-year-old male patient initially presented with hypertension, tinnitus, bilateral carotid masses, a right jugular foramen, and a periaortic arch mass with an elevated plasma dopamine level but an otherwise normal biochemical profile. On surveillance MRI 4 years after initial presentation, he was found to have a 2.2-cm T2 hyperintense lesion with arterial enhancement adjacent to the gallbladder, which demonstrated avidity on ⁶⁸Ga-DOTATATE PET/CTand retrospectively on ¹⁸F-FDOPA PET/CT but was nonavid on ¹⁸F-FDG PET/CT. Biochemical work-up including plasma catecholamines, metanephrines, and chromogranin A levels were found to be within normal limits. This lesion was surgically resected and was confirmed to be a paraganglioma (PGL) originating from the gallbladder wall on histopathology. Pheochromocytoma (PHEO) and PGL are rare tumors of the autonomic nervous system. Succinate dehydrogenase subunit D (SDHD) pathogenic variants of the succinate dehydrogenase complex are usually involved in parasympathetic, extra-adrenal, multifocal head, and neck PGLs. We report an unusual location of PGL in the gallbladder associated with SDHD mutation which could present as a potential pitfall on ¹⁸F-FDOPA PET/CT as its normal excretion occurs through biliary system and gallbladder. This case highlights the superiority of ⁶⁸Ga-DOTATATE in comparison to ¹⁸F-FDOPA and ¹⁸F-FDG in the detection of SDHD-related parasympathetic PGL.ClinicalTrials.gov Identifier: NCT00004847.
Adult ; Autonomic Nervous System ; Biliary Tract ; Catecholamines ; Chromogranin A ; Dopamine ; Gallbladder ; Head ; Humans ; Hypertension ; Magnetic Resonance Imaging ; Male ; Neck ; Paraganglioma ; Pheochromocytoma ; Plasma ; Positron-Emission Tomography and Computed Tomography ; Retrospective Studies ; Succinate Dehydrogenase ; Tinnitus