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

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

Paragangliomas are relatively rare chromaffin cell tumors which may be cured through resection. Patients with paragangliomas may develop metastatic diseases. There is no consensus regarding refractory chemotherapy for treatment of metastatic disease. In this report, we presented a case of a 43-year-old woman who was admitted to the hospital with a history of episodic headaches, diaphoresis, and weakness. Elevated plasma catecholamine levels and a right paraaortic mass were observed on computed tomography. The mass was excised, and a diagnosis of paraganglioma was confirmed. After 20 months of follow-up, local recurrence and metastases were detected in the thorax, abdomen, and skeletal system. Plasma and urinary catecholamine levels were high. Chemotherapy was administered, and no improvement was observed. Therefore, following this palliative conventional chemotherapy, sorafenib was administered for three months, and, finally, positron emission tomography showed that the patient's lesions had completely regressed.
Abdomen ; Adult ; Chromaffin Cells ; Consensus ; Diagnosis ; Drug Therapy ; Female ; Follow-Up Studies ; Headache ; Humans ; Neoplasm Metastasis ; Paraganglioma* ; Paraganglioma, Extra-Adrenal ; Pheochromocytoma ; Plasma ; Positron-Emission Tomography ; Recurrence ; Thorax

Retroperitoneal paragangliomas are uncommon neuroendocrine tumors which are derived from extra-adrenal paraganglioma with various clinical signs and symptoms. Although most extra-adrenal paragangliomas are histologically benign, some tumors can synthesize and secrete excess catecholamine from the tumor. Excessive production of catecholamine causes numerous cardiovascular manifestations such as severe hypertension, cardiomyopathy, cardiac arrhythmias, and even multiorgan failure. It can lead to high risks of morbidity and mortality, especially in patients who are unrecognized or not adequately prepared. We present a female patient who was preoperatively undiagnosed of secreting retroperitoneal paraganglioma that caused cardiac tachyarrhythmia and severe intraopertive hypertension not controlled by usual antihypertensive agents. A secreting extra-adrenal paraganglioma should be included in differential diagnosis for patient who have incidentaloma and show wide range of hypertension with hemodynamic instability that is not well controlled by common antihypertensive drugs.
Antihypertensive Agents ; Arrhythmias, Cardiac ; Cardiomyopathies ; Diagnosis, Differential ; Female ; Hemodynamics ; Humans ; Hypertension ; Mortality ; Neuroendocrine Tumors ; Paraganglioma* ; Paraganglioma, Extra-Adrenal ; Tachycardia

Extra-adrenal paragangliomas and pheochromocytomas are rare but clinically important tumors, which produce, store, release, and metabolize catecholamines resulting in unexpected life-threatening effects. It is neither easy nor difficult to clinically diagnose these tumors despite the availability modern clinical methods because signs and symptoms such as recurrent episodes of paroxysmal hypertension and headache are nonspecific. Only a few cases of unexpected death in which the deceased had a non-diagnosed paraganglioma or pheochromocytoma have been reported. Death in these cases is usually sudden and occurs during emergency room care or during a major or minor abdominal operation, without prodromal symptoms. Death is considered to occur because of paroxysmal hypertension, cerebrovascular attacks, abrupt hemorrhage into the tumor parenchyma, or acute left ventricular failure. We report the case of a 73-year-old man who died 12 hours after undergoing decompression surgery for spinal stenosis. A medico-legal autopsy revealed that death in this case resulted from an undiagnosed paraganglioma around the abdominal aorta.
Aged ; Aorta, Abdominal ; Autopsy ; Catecholamines ; Death, Sudden ; Decompression ; Emergencies ; Headache ; Hemorrhage ; Humans ; Hypertension ; Paraganglioma ; Paraganglioma, Extra-Adrenal ; Pheochromocytoma ; Prodromal Symptoms ; Spinal Stenosis

Jugulotympanic paragangliomas (JTPs) known as glomus tumors, are neoplasms of variable invasiveness that arise from the paraganglia situated around the jugular bulb or middle ear. We now report a rare case of JTP in an 18-year-old male. Preoperative diagnoses through external auditory canal biopsy and radiologic examination both failed. Even using a frozen section, an informative finding was not obtained because mostly granulation tissue was present along with associated squeezing artifacts. On permanent histologic examination, small cell nests between many ectatic small vessels and fibrotic stroma were seen, and those cells were positive for CD56, synaptophysin and chromogranin. Because JTPs are rare and have rather different histologic findings - higher vascularity, smaller and less uniform tumor cells than other paragangliomas - they are easy to misdiagnose. However, remembering those differences may help the physician avoid missing JTPs.
Adolescent ; Artifacts ; Biopsy ; Ear Canal ; Ear, Middle ; Frozen Sections ; Glomus Jugulare ; Glomus Tumor ; Glomus Tympanicum ; Granulation Tissue ; Humans ; Male ; Paraganglioma ; Paraganglioma, Extra-Adrenal ; Synaptophysin

Jugulotympanic paragangliomas (JTPs) known as glomus tumors, are neoplasms of variable invasiveness that arise from the paraganglia situated around the jugular bulb or middle ear. We now report a rare case of JTP in an 18-year-old male. Preoperative diagnoses through external auditory canal biopsy and radiologic examination both failed. Even using a frozen section, an informative finding was not obtained because mostly granulation tissue was present along with associated squeezing artifacts. On permanent histologic examination, small cell nests between many ectatic small vessels and fibrotic stroma were seen, and those cells were positive for CD56, synaptophysin and chromogranin. Because JTPs are rare and have rather different histologic findings - higher vascularity, smaller and less uniform tumor cells than other paragangliomas - they are easy to misdiagnose. However, remembering those differences may help the physician avoid missing JTPs.
Adolescent ; Artifacts ; Biopsy ; Ear Canal ; Ear, Middle ; Frozen Sections ; Glomus Jugulare ; Glomus Tumor ; Glomus Tympanicum ; Granulation Tissue ; Humans ; Male ; Paraganglioma ; Paraganglioma, Extra-Adrenal ; Synaptophysin

Glomuvenous malformation (GVM), an uncommon variant of venous malformation (VM), reveals dilated venous channels lined by variable amounts of glomus cells histopathologically. It appears as compressible blue or blue-purple nodules or plaque(s), sometimes showing a familial tendency. GVM usually involves only the superficial layer of the cutis or subcutaneous tissue, unlike VM, which frequently involves deeper tissue. Although GVM shares features of VM and glomus tumor, their clinicopathological and/or genetic features are sufficiently distinctive to enable a differential diagnosis, which is important for management decisions. Herein, we report five patients who showed a large confluent patch or several scattered patches composed of tender bluish blebs. Two patients among them were siblings. Histopathological and immunohistochemical examinations confirmed GVM.
Blister ; Diagnosis, Differential ; Glomus Tumor ; Humans ; Paraganglioma, Extra-Adrenal ; Siblings ; Subcutaneous Tissue

Adolescent ; Catecholamines ; secretion ; Female ; Humans ; Lung Neoplasms ; diagnosis ; metabolism ; Paraganglioma, Extra-Adrenal ; diagnosis ; metabolism ; Stomach Neoplasms ; diagnosis ; metabolism

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