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

Metastatic paraganglioma treatment options are limited. Peptide receptor radionuclide therapy (PRRT) has been introduced as a novel management option for metastatic neuroendocrine tumors demonstrating safety, efficacy, and increased quality of life.We present two cases of marked progression of metastatic paraganglioma following initial partial response to PRRT. Given their positivity on ⁶⁸Ga-DOTATATE PET/CT and ¹¹¹In-octreotide SPECT, they underwent PRRT. Imaging following treatment revealed significant improvement in size and intensity, with some foci nearly completely resolved in one patient, and disease regression with a decrease in the number and size of bone and liver lesions in the second patient.Within months, repeat imaging in both patients revealed extensive metastatic disease with new lesions, which eventually lead to their deaths. The mechanism for rapid disease progression after partial response is not well understood, although it could be related to initially high Ki-67 levels or ¹⁸F-FDG PET/CT SUV(max) values. However, naturally rapid disease progression despite PRRT response cannot be excluded. This finding warrants the importance of proper patient counseling along with early and accurate pre-PRRT assessment, taking into consideration the above potential risk factors for therapy response in order to personalize treatment regimens and achieve maximum patient benefit.
Counseling ; Disease Progression ; Humans ; Liver ; Neuroendocrine Tumors ; Paraganglioma ; Positron-Emission Tomography and Computed Tomography ; Receptors, Peptide ; Risk Factors ; Tomography, Emission-Computed, Single-Photon

Gangliocytic paraganglioma is a very rare tumor that is usually located in the duodenum. It is characterized by benign behavior and favorable outcomes; however, it sometimes shows regional lymph node metastasis. The clinical management of gangliocytic paraganglioma has not yet been standardized. A 36-year-old Korean man visited Kosin University Gospel Hospital for the management of a duodenal subepithelial mass that was detected incidentally. Endoscopic mucosal resection was performed without any adverse events. Pathological examination of the resected specimen revealed gangliocytic paraganglioma. The patient has remained in good health during the 6 months of follow-up after the procedure.
Adult ; Duodenum ; Follow-Up Studies ; Humans ; Lymph Nodes ; Neoplasm Metastasis ; Paraganglioma

Vagal paragangliomas (VPGLs) represent <5% of all head and neck paragangliomas (PGLs) and show a 17% to 20% risk of malignancy. We present a rare case of a 50-year-old gender with a left VPGL in her neck. To date, approximately 200 cases have been reported. The tumor showed web-like adhesions and arterial supply from the external carotid artery. We performed en bloc resection including a part of the vagus nerve. The superior laryngeal nerve was preserved with the “human communicating nerve” which maintains neural communication in >70% of humans, providing motor fibers to the larynx. The patient recovered uneventfully and was discharged on the 3rd postoperative day. These tumors are therapeutically challenging owing to their proximity to vital neck and skull base structures. Early detection decreases surgical morbidity and mortality. Preservation of viable neural tissue is important in advanced disease.
Carotid Artery, External ; Head ; Humans ; Laryngeal Nerves ; Larynx ; Middle Aged ; Mortality ; Neck ; Paraganglioma ; Skull Base ; Vagus Nerve