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

To determine the clinicopathological and imaging features in 18F-fluoro-2-deoxyglucose (18F-FDG) positron emission tomography and computed tomography (PET/CT) for paraganglioma of testis, and to increase the diagnostic accuracy.
 Methods: A case of paraganglioma of testis with multiple lymph node and lung metastasis were reported. PET/CT and pathological findings in the case were retrospectively analyzed.
 Results: The patient presented with high blood pressure, high level of catecholamine, and urinary vanillylmandelic acid. The patient underwent 18F-FDG PET/CT, which showed the features including the right testis nodule with a star lesion nearby, the right spermatic cord, the lymphadenopathy of bilateral inguinal and retroperitoneum, the posterior basal segment of right lung nodule, and a lot of brown adipose tissues (BAT) in the whole body with intense FDG uptake. 18F-FDG PET/CT showed that the intense FDG uptake of the BAT disappeared after the excision of the right testis and metastasis of paraganglioma.
 Conclusion: PET/CT shows great value in localization diagnose, clinical staging and curative evaluation. PET/CT plays a helpful role in revealing the BAT with 18F-DG avidity in the patients with paraganglioma with elevated blood pressure, high level catecholamine, and urinary vanillylmandelic acid.
Fluorodeoxyglucose F18 ; Humans ; Lymph Nodes ; diagnostic imaging ; pathology ; Lymphatic Metastasis ; Male ; Neoplasm Staging ; Paraganglioma ; diagnostic imaging ; surgery ; Positron Emission Tomography Computed Tomography ; Retrospective Studies ; Sensitivity and Specificity ; Testicular Neoplasms ; diagnostic imaging ; surgery ; Testis ; diagnostic imaging ; surgery

Adult ; Humans ; Male ; Paraganglioma ; diagnosis ; surgery ; Seminal Vesicles ; pathology ; surgery ; Tomography, X-Ray Computed

Mediastinal paragangliomas are very rare neuroendocrine tumors. Complete resection is the standard treatment of a paraganglioma because of the tumor's potential malignancy and poor response to chemo- or radiotherapy. However, the highly vascular nature of the tumor and its characteristic anatomic location make complete resection difficult. We report a case of an anterior mediastinal paraganglioma, which was incidentally found on a chest computed tomography scan for chronic cough work-up of a 55-year-old woman. Complete resection was accomplished using video-assisted thoracoscopic surgery, and the patient recovered without any complications.
Cough ; Female ; Humans ; Mediastinal Neoplasms ; Middle Aged ; Neuroendocrine Tumors ; Paraganglioma* ; Radiotherapy ; Thoracic Surgery, Video-Assisted* ; Thorax

Adult ; Antigens, CD ; metabolism ; Antigens, Differentiation, Myelomonocytic ; metabolism ; Appendectomy ; Appendiceal Neoplasms ; complications ; metabolism ; pathology ; surgery ; Appendicitis ; etiology ; surgery ; Diagnosis, Differential ; Female ; Granular Cell Tumor ; complications ; metabolism ; pathology ; surgery ; Humans ; Paraganglioma ; metabolism ; pathology ; Phosphopyruvate Hydratase ; metabolism ; S100 Proteins ; metabolism

Adult ; CD56 Antigen ; metabolism ; Carcinoma ; metabolism ; pathology ; surgery ; Carcinoma, Medullary ; pathology ; Carcinoma, Papillary ; metabolism ; pathology ; DNA-Binding Proteins ; metabolism ; Diagnosis, Differential ; Female ; Humans ; Paraganglioma ; metabolism ; pathology ; Thyroid Neoplasms ; metabolism ; pathology ; surgery ; Thyroidectomy ; methods ; Transcription Factors ; Triglycerides ; metabolism

Isolated metastatic deposits of papillary thyroid carcinoma to the parapharyngeal space are rare. Herein, we describe the case of a young woman who presented with a right-sided oropharyngeal mass that was initially diagnosed as a parapharyngeal space paraganglioma. The patient opted for conservative treatment as she was asymptomatic and wished to avoid the risk of neurovascular morbidity associated with surgery. After 20 years, the patient sought treatment again for the oropharyngeal mass, which had progressively increased in size and was causing difficulty in swallowing. Repeat imaging of the affected area revealed that the mass had increased significantly in size; it also revealed the presence of a previously absent small lesion in the right lobe of the thyroid. Excision of the parapharyngeal space tumour and near-total thyroidectomy were performed; the excised specimens showed features of the follicular variant of papillary thyroid carcinoma. As papillary thyroid carcinoma that metastasises to the parapharyngeal space can masquerade as a paraganglioma, clinicians should bear in mind that an isolated metastatic deposit in the parapharyngeal space could be the first sign of occult papillary thyroid carcinoma.
Adult ; Carcinoma ; diagnosis ; diagnostic imaging ; surgery ; Carcinoma, Papillary ; Diagnosis, Differential ; Diagnostic Errors ; Female ; Humans ; Neoplasm Metastasis ; Paraganglioma ; diagnosis ; diagnostic imaging ; surgery ; Pharyngeal Neoplasms ; diagnosis ; diagnostic imaging ; surgery ; Thyroid Neoplasms ; diagnosis ; diagnostic imaging ; surgery ; Thyroidectomy ; Tomography, X-Ray Computed ; Treatment Outcome ; Whole Body Imaging

Gangliocytic paraganglioma (GP) is a rare, benign tumor which is usually found in the duodenum. We here report four recent cases of GP, with successful endoscopic resection in three cases, including a lesion on the ampulla of Vater. In all cases, each lesion had a stalk that facilitated removal using an endoscopic approach. Endoscopic mucosal resection is a feasible and safe treatment if the location, depth, and lymph node status are all favorable and is also helpful for definite diagnosis of unknown duodenal mass. To avoid morbidity resulting from open surgical resection, careful inspection for the peduncle of the GP will help determine the feasibility of endoscopic resection.
Aged ; Ampulla of Vater/pathology ; Chromogranin A/metabolism ; Colonoscopy ; Duodenal Neoplasms/pathology/*surgery ; Endoscopy, Gastrointestinal ; Female ; Humans ; Immunohistochemistry ; Intestinal Mucosa/pathology/surgery ; Male ; Middle Aged ; Neuroendocrine Tumors/pathology/surgery ; Paraganglioma/pathology/*surgery ; S100 Proteins/metabolism ; Synaptophysin/metabolism ; Tomography, X-Ray Computed