Humans ; Laparoscopy ; methods ; Male ; Middle Aged ; Paraganglioma, Extra-Adrenal ; pathology ; surgery ; Retroperitoneal Neoplasms ; pathology ; surgery

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

Extraadrenal paragangliomas involving the spine is less common and usually takes the form of intradural compression of the cauda equina. The authors report three cases of spinal paragangliomas resulting in extradural spinal cord compression and their MR findings. The MR imaging revealed a well-demarcated extradural mass with low to intermediate signal intensity on T1-weighted images and intermediate to high signal intensity on T2-weighted images compared to paravertebral muscles. After Gd-DTPA administration, heterogeneous and intense enhancement was found. Multiple punctate and serpiginous structures of signal void due to high-velocity flow were noted around and within the tumors on all sequences. In one case, the signal void structures were well corresponded with feeding arteries on angiography. These may be the characteristic findings of the extraadrenal paraganglioma involving the spine.
Adult ; Aged ; Female ; Human ; Magnetic Resonance Imaging ; Male ; Middle Age ; Paraganglioma, Extra-Adrenal/*diagnosis/pathology ; Spinal Neoplasms/*diagnosis/pathology

Paraganglioma in pregnancy is an extremely rare condition and its diagnosis is often delayed because the clinical symptoms can mimic those of preeclampsia or gestational hypertension. Here, we report the case of a 32-year-old, gravida 2, para 1 woman who presented with severe headache, palpitation, and sweating at 37 weeks' gestation. Although emergent cesarean section was performed on the assumption of severe preeclampsia, blood pressure fluctuated and heart rate remained tachycardiac. We suspected that she might have thromboembolic lesion in the chest or pheochromocytoma. Chest and abdominal computed tomography revealed a 4 cm mass in the left para-aortic space. Serum and urinary catecholamine levels were found to be significantly increased. She underwent laparoscopic mass removal and the pathology confirmed paraganglioma. When typical paroxysmal hypertension is accompanied by headache, palpitation, and sweating during pregnancy, adrenal tumors should be considered.
Adult ; Blood Pressure ; Cesarean Section ; Diagnosis ; Female ; Headache ; Heart Rate ; Humans ; Hypertension ; Hypertension, Pregnancy-Induced ; Paraganglioma ; Paraganglioma, Extra-Adrenal* ; Pathology ; Pheochromocytoma ; Pre-Eclampsia* ; Pregnancy ; Sweat ; Sweating ; Thorax