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

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

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

Gangliocytic paragangliomas (GPs) are rare tumors of the duodenum, presenting as single sessile or pedunculated polypoid masses. Clinical manifestations of duodenal GPs can vary from an incidental finding at endoscopy to frequent upper gastrointestinal bleeding caused by mucosal ulceration and abdominal pain. GPs are considered benign, but the disease can recur and spread to regional lymph nodes. A 41-year-old female presented with abdominal pain. Upper gastrointestinal endoscopy revealed a subepithelial tumor of the ampulla of Vater in the second portion of the duodenum. The tumor was resected using the endoscopic mucosal resection technique. The tumor was diagnosed as benign GP of the duodenum using histological and immunohistochemical staining procedures.
Adult ; Ampulla of Vater/chemistry/*pathology/surgery ; Biopsy ; Duodenal Neoplasms/chemistry/*pathology/surgery ; Duodenoscopy ; Female ; Humans ; Immunohistochemistry ; Paraganglioma/chemistry/*pathology/surgery ; Treatment Outcome ; Tumor Markers, Biological/analysis

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

OBJECTIVE: To investigate the clinical feature, diagnose and therapeutic methods of paraglottic space primary paraganglioma. METHOD: One case of paraglottic space primary paraganglioma was reported and the relevant literatures were reviewed. RESULT: One case showed a hoarse voice, who was cured after the surgery of neck incision. NSE and CgA were positively expressed. CONCLUSION Paraganglioma of the paraglottic space is very rare. The diagnosis of paraglottic space primary paraganglioma bases on histopathology and immunohistochemistry. The immunohistochemistry and clinical character must be comprehensively analyzed to increase the diagnosis accuracy.
Female ; Glottis ; pathology ; Humans ; Middle Aged ; Paraganglioma ; pathology ; surgery

Paraganglioma is a rare neuroendocrine neoplasm observed in patients of all ages, with an estimated incidence of 3/1,000,000 population. It has long been recognized that some cases are familial. The majority of these tumors are benign, and the only absolute criterion for malignancy is the presence of metastases at sites where chromaffin tissue is not usually found. Some tumors show gross local invasion and recurrence, which may indeed kill the patient, but this does not necessarily associate with metastatic potential. Here, we report a case of vertebral metastatic paraganglioma that occurred 19 months after the patient had undergone partial cystectomy for urinary bladder paraganglioma. We believe this to be a rarely reported bone metastasis of paraganglioma arising originally within the urinary bladder. In this report, we also provide a summary of the general characteristics of this disease, together with progress in diagnosis, treatment, and prognosis.
Chromogranin A ; metabolism ; Cystectomy ; Female ; Humans ; Middle Aged ; Paraganglioma ; metabolism ; pathology ; secondary ; surgery ; Phosphopyruvate Hydratase ; metabolism ; Prognosis ; Radionuclide Imaging ; Spinal Neoplasms ; diagnostic imaging ; secondary ; surgery ; Synaptophysin ; metabolism ; Technetium ; Thoracic Vertebrae ; Tomography, X-Ray Computed ; Urinary Bladder Neoplasms ; metabolism ; pathology ; surgery

OBJECTIVETo investigate the clinical presentation, pathologic features, treatment and prognosis of prostatic paraganglioma.

METHODSWe retrospectively studied a case of prostatic paraganglioma and reviewed relevant literature. The patient was a 39-year-old man, admitted for repeated hematospermia for over 12 months. After misdiagnosed as having prostate cancer, he underwent suprapubic prostatectomy, with the tumor completely removed.

RESULTSPostoperative pathological examination confirmed the tumor to be prostatic paraganglioma, which was non-functional, with the immunohistochemical results of NSE (+), CGA (+), S100 (+), CK (-) and Desmin (-). Postoperative blood pressure was stable. Two weeks after surgery, the urethral catheter was removed and the patient discharged. No recurrence was found during 48 months of follow-up.

CONCLUSIONLacking specific clinical characteristics, paraganglioma of the prostate is easily misdiagnosed, and can be confirmed only by postoperative pathology and immunohistochemistry. For the treatment of this rare tumor, little experience has been accumulated, and further studies are needed.

Adult ; Humans ; Immunohistochemistry ; Male ; Paraganglioma ; pathology ; surgery ; Prostatic Neoplasms ; pathology ; surgery