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

Paraganglioma is one of the pheochromocytomas, and this arises in the extra-adrenal tissue. It is a rare tumor of neural crest origin; it accounts for at least 10% of all the pheochromocytomas. Paragangliomas produce catecholamines and on the basis of this, they are classed as either functional or nonfunctional. We report here on our experience with laparoscopic removal of functional paragangliomas of the pelvic cavity and we include a brief review of literatures.
Catecholamines ; Laparoscopy ; Neural Crest ; Paraganglioma* ; Paraganglioma, Extra-Adrenal ; Pelvis ; Pheochromocytoma

This rare tumor has been diagnosed postoperatively in a 64-years-old male patient. Herein a case of extra-adrenal paraganglioma of the retroperitoneum is presented with brief review of literatures.
Humans ; Male ; Paraganglioma, Extra-Adrenal*

Paragangliomas are neoplasms that arise from extra-adrenal paraganglia, microscopic islands of cells derived from the neural crest. In the head and neck region, paraganglionic tissue is distributed in the superior and inferior paraganglia, the carotid body, the vagal body, and the jugulotympanic region. Approximately 10% of patients with a paraganglioma have a family history of such tumors. Multiple lesions can be found in 26% of patients with family history. Patients with multiple paragangliomas have a higher risk of having functional pheochromocytoma and should undergo a pretreatment screening for vasopressor substances. Similarly, patients with a family history should undergo four-vessel arteriography to rule out any multiple and clinically unrecognized lesions. The authors have experienced three cases of multiple paragangliomas, which were managed surgically. Two cases were bilateral carotid body tumors and two cases were accompanied by glomus jugulare. Of these, one case had family history. We report three cases of multiple paragangliomas with a review of literatures.
Angiography ; Aortic Bodies ; Carotid Body ; Carotid Body Tumor ; Glomus Jugulare ; Head ; Humans ; Islands ; Mass Screening ; Neck ; Neural Crest ; Paraganglioma* ; Pheochromocytoma

Pheochromocytoma is a catecholamines secreting tumor that usually appears in the adrenal medulla, sympathetic ganglia and extra-adrenal chromaffin tissue. About 10% of this disease is detected in the extra-adrenal chromaffin tissue which is called paraganglioma. The three major clinical symptoms of pheochromocytoma are headache, syncope and hypertension. Approximately 0.1% of hypertensive patients have pheochromocytoma. The extra-adrenal paraganglioma is found in abdominal sympathetic nerve ganglion or Zukerkandle's organ with great frequency, but it also appears, albeit rare, in the cervical ganglion, thoracic cavity, bladder, and pelvic cavity. Some cases of paraganglioma in bladder are being reported internationally, but domestic reports are rare. We report a case of paraganglioma originating in the urinary bladder of patient who visited with acute renal failure associated with malignant hypertension. We treated him with phenoxybenzamine and later with partial cystectomy. High blood pressure was well controlled and acute renal failure was resolved.
Acute Kidney Injury* ; Adrenal Medulla ; Catecholamines ; Cystectomy ; Ganglia, Sympathetic ; Ganglion Cysts ; Headache ; Humans ; Hypertension ; Hypertension, Malignant ; Paraganglioma* ; Paraganglioma, Extra-Adrenal ; Phenoxybenzamine ; Pheochromocytoma ; Syncope ; Thoracic Cavity ; Urinary Bladder*

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

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

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

No abstract available.
Paraganglioma*