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PubMed1998

Hypercalcemia in malignant paraganglioma due to parathyroid hormone-related protein.

A 15-year-old boy had hypercalcemia in association with malignant retroperitoneal paraganglioma. He had suppressed circulating levels of intact parathyroid hormone, whereas parathyroid hormone-related protein (PTHrP) immunoreactivity was elevated in plasma. Both the serum 25-hydroxyvitamin D and 1,25-dihydroxyvitamin D levels were normal. Preoperatively the patient required control of hypercalcemia with intravenous pamidronate therapy. His circulating calcium and PTHrP concentrations became normal after a successful surgical resection of the primary retroperitoneal tumor. To our knowledge, this is the first reported case of elevated PtHrP levels in a patient with paraganglioma which resolved postoperatively.

Adolescent↗

PubMedDate not supplied

Vagal paraganglioma. A case report.

Vagal paragangliomas are rare tumors and difficult to differentiate from carotid body tumors or schwannomas of the vagus nerve. We present a patient who could be preoperatively diagnosed as having a vagal paraganglioma by ultrasonography and color Doppler sonography.

Cerebral Angiography↗

PubMed1999

Paraganglioma of the urinary bladder: A case report and review of the Japanese literature.

We describe a patient with primary paraganglioma of the urinary bladder associated with typical clinical symptoms. Systolic blood pressure frequently increased to 300 mm Hg immediately after micturition. Levels of urinary vanillylmandelic acid excretion and serum norepinephrine were significantly elevated. The tumor was removed by partial cystectomy. Histological examination of the tumor revealed paraganglioma of the urinary bladder. Compared with a review of 53 similar cases reported in Japan, the distribution of age, sex and tumor location in the bladder differed from those described worldwide.

Cystectomy↗

PubMed2002

Pheochromocytoma and multifocal functioning paraganglioma in a 9-year-old boy with von Hippel-Lindau disease.

We report the case of a 9-year-old boy presenting with multifocal paraganglioma partially involving the right suprarenal gland, abdominal paraganglia, and the left para-aortal retropleural region. Von Hippel-Lindau disease (VHL) was diagnosed exclusively by VHL-gene analysis. Other clinical signs of VHL were lacking. After a complaint-free period of 7 months the boy developed a metachronous paraganglioma adjacent to the opposite adrenal gland. Patients with VHL disease should undergo adrenal-sparing surgery.

Adrenal Gland Neoplasms↗

PubMed1996

Hypertension and unilateral renal ischemia (Page kidney) due to compression of a retroperitoneal paraganglioma.

We describe a 17-year-old hypertensive man with a left abdominal mass on the computed tomographic scan. Radiologic studies revealed a large tumor near the left renal hilus, resulting in renal compression and displacement toward the posterolateral abdominal cavity. Aortography excluded stenosis of the renal arterial vessels. Plasma and urinary catecholamines were normal and plasma renin activity (PRA) was high. The left renal vein renin was 2.1-fold higher than the right one. After resecting the tumor including the left kidney and adrenal, high blood pressure and elevated PRA returned to normal. Histologically, the tumor was a paraganglioma, and the affected kidney showed hyperplasia of the juxtaglomerular apparatus. Thus, the hypertension in our patient was probably due to renal ischemia extrinsically compressed by a non-functioning retroperitoneal paraganglioma (Page kidney).

Adolescent↗

PubMed1984

Paraganglioma involving the larynx.

Paraganglioma is a rare tumor in the upper respiratory tract. More than 30 paragangliomas have been reported arising in the larynx but only a few in the trachea. The organoid arrangements of the cell nests reproduce the classic 'Zellballen' of the normal gland. Neurosecretory granules can be seen at electron microscopy. Angiography may be a valuable diagnostic adjunct. Malignant degeneration may occur. The 44-year-old woman reported had a dumbbell-shaped tumor involving the subglottic larynx but with its largest portion outside the larynx and within the left lobe of the thyroid gland. The tumor may very well have arisen from the inferior laryngeal paraganglia. An important differential diagnosis is hemangiopericytoma, which was the preoperative diagnosis in this case. A laryngectomy including the left lobe of the thyroid gland was performed and there is no sign of local recurrence or metastases 7 years after operation.

Adult↗

PubMed1987

Paraganglioma of the supraglottic larynx.

Supraglottic paraganglioma is described in an 80-year-old woman who presented with a small shiny-red mass at the posterior aspect of the left aryepiglottic fold. Histological examination revealed the typical 'zellballen' pattern of extra-adrenal paragangliomas. The tumor was locally excised and did not recur at 24 months follow-up.

Aged↗

PubMed2002

Paraganglioma of the cervical sympathetic trunk.

We report the case of a 17-year-old man who presented with left-sided Horner's syndrome. Magnetic resonance imaging revealed a spindle-shaped cervical tumor in the left paravertebral space. During operation, a tumor originating from the left sympathetic trunk was found. The histopathologic analysis showed a sympathetic paraganglioma. The sympathetic trunk is an extremely rare location for a cervical paraganglioma; only a few cases have been reported in the literature.

Adolescent↗

PubMedDate not supplied

Catecholamine-secreting infratemporal fossa paraganglioma.

Extraadrenal paragangliomas are tumors of the paraganglion system, usually arising from the carotid bodies, the glomus jugulare, or the glomus tympanicum. These tumors are capable of secreting catecholamines which can cause severe hypertensive crises. This paper reports a case of a patient who suffered a cerebral vascular accident due to hypertension resulting from a catecholamine-secreting infratemporal fossa paraganglioma. Any patient with a history of paroxysmal hypertension, headaches, and palpitations should be evaluated for a catecholamine-secreting tumor. Diagnosis and management of these tumors is best accomplished by a team including a radiologist, an endocrinologist, an anesthesiologist, a pathologist, an otolaryngologist--head and neck surgeon, and when the tumor invades the cranial cavity, a neurosurgeon. In this case, precise radiographic mapping of the tumor extent and its blood supply, control of hypertension with adrenergic blocking agents, and depletion of catecholamine stores using alpha-methyl-p-tyrosine allowed total extirpation through a craniofacial approach and a successful outcome.

Catecholamines↗

PubMedDate not supplied

Neuroendocrine markers in paragangliomas of the head and neck.

Eighteen paragangliomas of the head and neck (11 carotid body tumors, four glomus tympanicum tumors, three glomus jugulare tumors) were studied retrospectively. Tissue from each of these tumors was immunostained for the presence of serotonin, a variety of neuropeptide hormones, and the enzyme neuron-specific enolase (NSE). Seven tumors were studied by electron microscopy. The clinical and laboratory records were reviewed for evidence of endocrine activity or metabolic imbalance. All tumors displayed diffuse and intense immunostaining for NSE. In addition, a wide variety of hormonal substances could be identified. Those most frequently demonstrated were serotonin and leu-enkephalin. Ten of the 11 carotid body tumors demonstrated immunoreactivity for multiple hormones. By electron microscopy all tumors contained a heterogeneous population of membrane-bound neurosecretory granules. None of these tumors was associated with a clinically apparent endocrine syndrome. We conclude that paragangliomas of the head and neck are neuroendocrine tumors that are capable of synthesizing a variety of hormonal substances. These hormonal substances rarely elicit a clinically apparent endocrine or metabolic imbalance. All of the tumors demonstrated immunostaining for NSE. Future studies on serum levels of NSE may provide useful diagnostic and follow-up data.

Carotid Body Tumor↗

PubMed1995

Laryngeal paraganglioma versus atypical carcinoid tumor.

Paraganglioma and atypical carcinoid tumor of the larynx are two neuroendocrine neoplasms that have often been confused in the past, and even in the present, in the literature. The clinicopathological profile of the two lesions is presented and the differential diagnosis is discussed. A correct diagnosis is of paramount importance, since treatment and prognosis depend on diagnostic accuracy and differ for the two lesions. Paraganglioma of the larynx is usually benign, whereas atypical carcinoid tumor is malignant and has an aggressive clinical course.

Adolescent↗

PubMedDate not supplied

Multiple simultaneous paragangliomas of the head and neck in association with multiple retroperitoneal pheochromocytomas.

A patient in whom multiple, familial, catecholamine-secreting head and neck paragangliomas and retroperitoneal pheochromocytomas were identified is reported. There were at least nine primary and possibly five recurrent neoplasms, the most reported in a single patient. In patients with family history of pheochromocytoma or paraganglioma or with multiple tumors, careful laboratory and angiographic studies are indicated to discover additional lesions.

Adult↗

PubMedDate not supplied

Paraganglioma of the sphenoid sinus appearing as labile hypertension.

Paragangliomas, also known as glomus tumors, are unusual, generally benign tumors most commonly found in association with major vessels and cranial nerves. Only a small number of these tumors have been described arising in the paranasal sinuses, and none of these has been physiologically active. The following case report describes a metabolically active paraganglioma arising in the sphenoid sinus.

Aged↗

PubMed1990

Extra-adrenal non-functioning paraganglioma.

Extra-adrenal non-functioning retroperitoneal paragangliomas are rare tumors. We herein report a case of retroperitoneal tumor which posed a diagnostic and management problem. Review of the literature shows only 16 previously reported cases of extra-adrenal paraganglioma which underwent complete surgical excision.

Adult↗

PubMedDate not supplied

Intrasellar paraganglioma with suprasellar extension: case report.

Paragangliomas are usually benign tumors which can be found in many sites of the body, from the base of the skull down to the pelvic floor. In the central nervous system the sellar region is very rarely involved; only three well studied cases have been reported to date. We present the cytological, histological, histochemical, immunocytochemical and ultrastructural features of an intrasellar and suprasellar paraganglioma in an 84-year-old man.

Aged↗

PubMed1987

Pathologic features of abdominal and thoracic paragangliomas in F344/N rats.

Seventeen paragangliomas were identified in a retrospective review of 200 NTP/NCI carcinogenicity studies in F344/N rats that served either as control or treated animals. Most tumors were grossly visible and located in the retroperitoneum adjacent to the vertebrae and aorta near the kidneys. Three microscopically detected paragangliomas were found at the base of the heart. Microscopically, neoplastic cells were in nests separated by reticulin fibers and capillaries. Argyrophil granules were in the cytoplasm of the retroperitoneal and mediastinal paravertebral tumors. Dense granules were found in the one tumor examined ultrastructurally. Some tumors had areas of necrosis and tumor emboli were present in the lumen of the abdominal aorta and vena cava adjacent to the tumor with metastases present in pulmonary vessels. The incidence of retroperitoneal neoplasms was 3 times more frequent in male than in female F344/N rats.

Abdominal Neoplasms↗

PubMed2001

Lessons to be learned: a case study approach. Paraganglioma of the urinary bladder.

Primary paraganglioma arises infrequently in the urinary bladder. We present here the clinicopathological, immunohistochemical and ultrastructural findings in a 23-year-old Saudi female. She was hospitalised because of gross haematuria over the previous seven days. The intravenous urogram revealed a filling defect in the urinary bladder. Cystoscopy disclosed a non-papillary tumour arising from the roof of the bladder neck. Transurethral resection was, accordingly, performed--but marked fluctuation in blood pressure was observed during the procedure. The histopathological findings of trabeculae and small nests of plump oval to spindle cells with a clear to acidophilic cytoplasm, forming an organoid pattern and demonstrating inconspicuous mitotic activity, were diagnostic of paraganglioma of the urinary bladder, the diagnosis was confirmed by means of immunohistochemical and ultrastructural studies. The endocrine markers chromogranin A and neuron-specific enolase were positive in chief cells; sustentacular cells at the periphery of neoplastic cell clusters were positive for S-100 protein. Neurosecretory granules were identified by electron microscopic examination. It is emphasised that, currently, there are no anatomico-pathological criteria to distinguish benign and malignant forms of the disease. Post-surgical recovery was uneventful in this case. Recurrence and metastases, though infrequent, have been reported in the literature; therefore, radiation therapy may be an important adjunct--and long-term follow-up is mandatory.

Adult↗

PubMed2006

Pheochromocytomas and secreting paragangliomas.

Catecholamine-producing tumors may arise in the adrenal medulla (pheochromocytomas) or in extraadrenal chromaffin cells (secreting paragangliomas). Their prevalence is about 0.1% in patients with hypertension and 4% in patients with a fortuitously discovered adrenal mass. An increase in the production of catecholamines causes symptoms (mainly headaches, palpitations and excess sweating) and signs (mainly hypertension, weight loss and diabetes) reflecting the effects of epinephrine and norepinephrine on alpha- and beta-adrenergic receptors. Catecholamine-producing tumors mimic paroxysmal conditions with hypertension and/or cardiac rhythm disorders, including panic attacks, in which sympathetic activation linked to anxiety reproduces the same signs and symptoms. These tumors may be sporadic or part of any of several genetic diseases: familial pheochromocytoma-paraganglioma syndromes, multiple endocrine neoplasia type 2, neurofibromatosis 1 and von Hippel-Lindau disease. Familial cases are diagnosed earlier and are more frequently bilateral and recurring than sporadic cases. The most specific and sensitive diagnostic test for the tumor is the determination of plasma or urinary metanephrines. The tumor can be located by computed tomography, magnetic resonance imaging and metaiodobenzylguanidine scintigraphy. Treatment requires resection of the tumor, generally by laparoscopic surgery. About 10% of tumors are malignant either at first operation or during follow-up, malignancy being diagnosed by the presence of lymph node, visceral or bone metastases. Recurrences and malignancy are more frequent in cases with large or extraadrenal tumors. Patients, especially those with familial or extraadrenal tumors, should be followed-up indefinitely.

Adrenal Gland Diseases↗