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PubMed1998

Mediastinal paraganglioma: a case report.

Mediastinal paragangliomas are unusual neoplasms that have been described in the medical literature. The microscopic features of paragangliomas are well established and are essentially similar, regardless of the anatomic site, although some minor differences have been noted according to the location of the tumor. We present a case of nonfunctioning anterior mediastinal paraganglioma in a 33-year-old woman. The mediastinal tumor was accidentally discovered on a thoracic tomographic scan performed for an unrelated minor nasal discomfort. Diagnosis was confirmed by 123I-metaiodobenzylguanidine scintigraphy and mediastinoscopy. Complete resection of the tumor was done through a median sternotomy and the patient received intraoperative radiotherapy.

3-Iodobenzylguanidine↗

PubMed2004

Malignant retroperitoneal paraganglioma: case report and review of the literature.

Paraganglioma is a rare neuroendocrine neoplasm observed in patients of all ages but it has not been characterized in children. The authors describe a retroperitoneal paraganglioma diagnosed by chance in an 11-year-old boy. Many aspects of retroperitoneal paraganglioma are still under investigation. The treatment of choice is radical resection. Surgery may be possible following chemotherapeutic debulking with cyclophosphamide, vincristine, and dacarbazine. 131I-MIBG radiotherapy has proved increasingly useful in reducing the pain associated with disseminated disease and also in facilitating surgical resection in cases that appear inoperable. Conventional radiotherapy is purely palliative and used to reduce the pain of bone metastases.

3-Iodobenzylguanidine↗

PubMed2001

Gene mutations in the succinate dehydrogenase subunit SDHB cause susceptibility to familial pheochromocytoma and to familial paraganglioma.

The pheochromocytomas are an important cause of secondary hypertension. Although pheochromocytoma susceptibility may be associated with germline mutations in the tumor-suppressor genes VHL and NF1 and in the proto-oncogene RET, the genetic basis for most cases of nonsyndromic familial pheochromocytoma is unknown. Recently, pheochromocytoma susceptibility has been associated with germline SDHD mutations. Germline SDHD mutations were originally described in hereditary paraganglioma, a dominantly inherited disorder characterized by vascular tumors in the head and the neck, most frequently at the carotid bifurcation. The gene products of two components of succinate dehydrogenase, SDHC and SDHD, anchor the gene products of two other components, SDHA and SDHB, which form the catalytic core, to the inner-mitochondrial membrane. Although mutations in SDHC and in SDHD may cause hereditary paraganglioma, germline SDHA mutations are associated with juvenile encephalopathy, and the phenotypic consequences of SDHB mutations have not been defined. To investigate the genetic causes of pheochromocytoma, we analyzed SDHB and SDHC, in familial and in sporadic cases. Inactivating SDHB mutations were detected in two of the five kindreds with familial pheochromocytoma, two of the three kindreds with pheochromocytoma and paraganglioma susceptibility, and 1 of the 24 cases of sporadic pheochromocytoma. These findings extend the link between mitochondrial dysfunction and tumorigenesis and suggest that germline SDHB mutations are an important cause of pheochromocytoma susceptibility.

Amino Acid Sequence↗

PubMed1977

Malignant vagal paraganglioma: report of a case and review of literature.

A case of malignant vagal paraganglioma is presented. The literature on the subject is briefly reviewed. This report brings to 48 the number of vagal paragangliomas now in the medical literature. Eight of these cases are found to be acceptable examples of malignant paraganglioma with evidence of lymph-node or distant metastases, and pertinent findings from these cases are summarized. Salient features of other cases showing extensive local or vascular invasion are also reviewed.

Adult↗

PubMed1992

A gene subject to genomic imprinting and responsible for hereditary paragangliomas maps to chromosome 11q23-qter.

Paragangliomas of the head and neck are slow growing tumors which rarely show malignant progression. Familial transmission has been described consistent with an autosomal dominant mode of inheritance. Clinical manifestations of hereditary paragangliomas are determined by the sex of the transmitting parent. All affected individuals have inherited the disease gene from their father, expression of the phenotype is not observed in the offspring of an affected female until subsequent transmittance of the gene through a male carrier. This finding strongly suggests that genomic imprinting is involved. We report the results of a linkage study on a large Dutch pedigree with hereditary paragangliomas. Highly significant evidence for genetic linkage to chromosome 11q23-qter with the anonymous DNA marker D11S147 was detected with a peak lod score of 6.0 at a recombination fraction theta = 0.0. Likelihood calculations yielded an odds ratio of 2.7 x 10(6) in favor of genomic imprinting versus the absence of genomic imprinting.

Chromosome Mapping↗

PubMed2000

Treatment of progressive metastatic glomus jugulare tumor (paraganglioma) with gemcitabine.

Paragangliomas are rare tumors of the paraganglia composed of specialized neural crest cells arising in association with sympathetic ganglia. Here we report a case of progressive, metastatic paraganglioma (glomus jugulare tumor) responsive to single agent gemcitabine. In addition, a brief review of chemotherapy for paraganglioma follows the case presentation.

Adolescent↗

PubMed1999

Sudden death in a child due to an intrathoracic paraganglioma.

A 12-year-old boy under treatment for asthma was found dead in his home. The autopsy revealed a large posterior mediastinal mass that completely compressed the upper lobe of the right lung and the associated airways. This mass extended from the right costovertebral sulcus into the thoracic spinal canal through the spinal foramen and compressed the spinal cord. It was located in the epidural space and was adherent to a nerve root. The histologic and immunocytochemical features were that of a paraganglioma. Although neurogenic tumors are the most common posterior mediastinal masses in the pediatric population, paragangliomas are rare, and spinal involvement has not been described in children. In addition, sudden death has not been reported in association with any of the 13 cases of posterior mediastinal paraganglioma described in the literature as involving the spine. This case illustrates an unusual cause of sudden death in a pediatric patient due to a benign neoplasm.

Asthma↗

PubMed1979

Laryngeal paraganglioma. Case report with ultrastructural analysis and literature review.

Laryngeal paraganglioma is an infrequently reported tumor; only 16 examples have been recorded in the English literature. All but one laryngeal paraganglioma originated superiorly in the larynx; involvement of the ipsilateral aryepiglottic fold is common. Male patients predominate (11:5). The average age of patients at the time of diagnosis was 47 years, and symptoms had been present for an average duration of 5.8 years (range 6 months to 27 years). Attempted biopsy has resulted in significant hemorrhage in three cases. As illustrated by the present case, the Grimelius argyrophil stain is a useful diagnostic procedure. Electron microscopy confirmed the presence of neurosecretory granules with core diameters ranging from 110 to 140 nm. Surgical resection is the preferred treatment and has been possible in 14 cases; nine patients are alive and free of tumor for an average of 3 years. Compared to other head and neck paragangliomas, these have a more malignant course with a 25% mortality; tender subcutaneous metastases are commonly observed in these patients.

Adult↗

PubMed1980

Paraganglioma of the thyroid gland.

A case of a paraganglioma located within the thyroid gland is reported, and the light- and electron-microscopic features are described. Review of the literature reveals only one other case of intrathyroidal paraganglioma, which was associated with bilateral carotid body tumors. Regarding the present case, no similar tumors have been noted elsewhere. The findings support the view that the thyroid gland should be included among the unusual sites at which primary paragangliomas may occur.

Female↗

PubMed1997

Thyroid paraganglioma: a clinicopathologic and immunohistochemical study of three cases.

We report three cases of intrathyroidal paraganglioma. The patients were adult women without significant personal or family histories that presented with an asymptomatic thyroid nodule. The tumors were single, well-circumscribed solid masses, 2 cm in greatest diameter, located within one thyroid lobe. Microscopically, they were encapsulated and showed the typical nesting (Zellballen) pattern of paraganglioma in other sites. Two of the tumors were composed of small- to medium-sized cells with granular amphophilic cytoplasm, and the third consisted of relatively large cells having a similar staining quality. Immunohistochemically, all tumors showed positivity for neuron-specific enolase, chromogranin A, and synaptophysin. S-100 protein-positive sustentacular cells were demonstrated in each case. Negative staining for epithelial markers, thyroglobulin, carcinoembryonic antigen, calcitonin, calcitonin gene-related peptide, serotonin, vimentin, and Congo red excluded other tumors that were considered in the differential diagnosis, such as medullary carcinoma, hyalinizing trabecular adenoma, atypical follicular adenoma, Hurthle-cell neoplasm, and metastatic carcinoid tumor. The patients were alive and well without evidence of recurrent disease at the time of the last follow-up. The previous literature on these tumors is discussed. We conclude that intrathyroidal paraganglioma exists and that this tumor can be distinguished from other similar-appearing neoplasms in this organ.

Biomarkers, Tumor↗

PubMed2001

Periampullary gangliocytic paraganglioma.

A33-year-old man presented with features of obstructive jaundice. Enterohepatic retrograde cholangiopancreatography examination revealed a periampullary growth. After pancreatoduodenectomy, histologic examination of the growth revealed three elements: a spindle cell element, large ganglion cells, and smaller paraganglioma-like cells arranged in small nests and groups. A diagnosis of gangliocytic paraganglioma was given. Gangliocytic paraganglioma is a rare benign duodenal tumor, with uncertain histogenesis. It is possible to identify the three components that are essential for its diagnosis on routine hematoxylin and eosin sections without the use of immunohistochemical markers. Because this lesion may present clinically as a periampullary carcinoma, it is important to recognize and diagnose this rare, but benign, entity.

Adult↗

PubMed1997

Preoperative embolization in the management of neck paragangliomas.

Surgery of neck paragangliomas carries inherent risks of excessive blood loss and cranial nerve injury. Preoperative embolization has been used to lessen the morbidity of surgery. We sought to characterize our experience with preoperative embolization by evaluating safety, efficacy, and surgical data. During a period of 22 years (1974 to 1996), 19 consecutive patients with 27 histopathologically confirmed neck paragangliomas were surgically treated at the Oulu University Hospital. All patients underwent preoperative arteriography and 17 patients had cervical ultrasonography (US). Eleven patients with 15 tumors were operated on without embolization and nine patients with 12 tumors were preoperatively embolized with 150- to 250-microm polyvinyl alcohol (PVA) particles. The mean blood loss during surgery in the nonembolized group was 1374 mL (range, 100 to 4500 mL) and the mean operation time was 4 hours and 48 minutes (range, 1.5 to 9 hours). In the embolized group the mean blood loss was significantly less (588 mL; range, 100 to 1800 mL; P = 0.04) and the mean operation time shorter (3 hours 24 minutes; range, 2 to 5 hours; P = 0.05). No embolic complications were recorded after the embolization. We conclude that preoperative embolization of neck paragangliomas 3 cm or greater in diameter with PVA particles is safe. Embolization to minimize operative bleeding facilitates surgery, shortens the operation time, and lessens the surgical risks.

Adolescent↗

PubMed1998

111Indium pentetreotide scan detection of familial paragangliomas.

Approximately 10% of head and neck paragangliomas are familial. Magnetic resonance imaging (MRI) is reported to be the best method for screening these families. 111Indium pentetreotide (Octreoscan) scintigraphy has been shown to be a sensitive indicator of neural crest tumors such as paragangliomas. Early diagnosis and treatment of familial paragangliomas (FP) would decrease the morbidity of tumor excision. Patients from two kindred with FP were scanned 24 hours after intravenous injection with approximately 6 millicuries of 111Indium pentetreotide. The entire body was imaged. Areas of increased uptake were further imaged with MRI. Altogether, five patients had positive 111Indium pentetreotide scans. Two unsuspected glomus tympanicums and bilateral carotid body tumors were identified. Three people tested had no abnormal uptake. There were no untoward reactions to the nuclide. 111Indium pentetreotide scanning is a safe, noninvasive method for early diagnosis of FP and is useful in detecting multicentric lesions. Patients suspect for FP are undergoing 111Indium pentetreotide imaging and followed up with MRI of the involved site if positive.

Adult↗

PubMed1996

Intrasellar paraganglioma: report of a case in a sibship of von Hippel-Lindau disease.

Although uncommon, paragangliomas are known to affect the central nervous system. Those that arise in the sellar region are particularly rare. We report the clinicopathological features of a paraganglioma occurring in the setting of von Hippel-Lindau disease, a disorder in which paragangliomas are known to occur. We also present a review of the literature.

Adolescent↗

PubMed1995

Metastatic paraganglioma causing spinal cord compression.

SUMMARY OF BACKGROUND DATA: Paragangliomas are neoplasms that arise from the autonomic nervous system-associated paraganglia. Most often they are benign tumors. However, infrequently they have been reported to metastasize. Involvement of the spine is rare and usually is intradural at the level of the cauda equina. OBJECTIVES: The authors report two cases of metastatic paraganglioma to the spine, both of which resulted in myelopathy from extradural spinal cord compression. Also provided is a review of the literature. CONCLUSIONS: Paragangliomas rarely metastasize to the spine. Management of the two reported patients involved spinal cord decompression followed by spinal stabilization procedures. Long-term ambulatory status was maintained in both patients. Radiation therapy may be an important adjunctive treatment after surgical removal of these tumors.

Adult↗

PubMed2002

Thoracic paraganglioma presenting with spinal cord compression and metastases.

We report a case of a 41-year-old man with a primary thoracic spine paraganglioma presenting with spinal cord compression and distant metastasis. Paragangliomas arising within the spinal canal are rare and when they occur are usually found in the intradural, extramedullary compartment and in the lumbosacral region. Thoracic paragangliomas are very rare, with only five prior cases reported in the literature and with distant metastasis seen in only one of these. In this case, the tumor was entirely extradural and produced extensive sclerosis of the surrounding vertebrae. The clinical, radiographic, and pathologic features of this case are detailed and the relevant literature is reviewed.

Adult↗

PubMed2003

Cerebellopontine angle paraganglioma.

BACKGROUND: Acoustic neuroma account for 80% of cerebellopontine angle tumors. Paraganglioma is a rare tumor in this region. OBJECTIVE: The authors report a case of cerebellopontine angle paraganglioma arising from the anterior inferior cerebellar artery. RESULTS: The clinical and radiologic features were indistinguishable from those of a neuroma, and only the intraoperative view showed a vascular tumor. Definitive diagnosis was made by histopathologic methods. CONCLUSIONS: This unique paraganglioma location may be explained by existing embryologic data. The minimal retrosigmoid approach, assisted by endoscopy, allows safe and complete removal of the tumor.

Cerebellopontine Angle↗

PubMed2003

KI-67 AND hTERT expression can aid in the distinction between malignant and benign pheochromocytoma and paraganglioma.

The clinical and histopathological distinction between benign and malignant pheochromocytomas and paragangliomas is difficult, and reliable diagnostic markers are lacking. Here we have evaluated the prognostic value of human telomerase reverse transcriptase (hTERT) gene expression detected by reverse transcription PCR (RT-PCR); telomerase activity (TA) measured by TRAP (telomeric repeat amplification protocol) assay; immunohistochemical staining for Ki-67/MIB-1; and the mRNA expression of matrix metalloproteinase (MMP)-2 and EMMPRIN (extracellular matrix metalloproteinase inducer) analyzed by in situ hybridization in 32 primary pheochromocytomas or abdominal paragangliomas. hTERT was expressed in 7/11 malignant tumors (defined as presence of metastasis and/or extensive local invasion) as compared with in 2/21 benign tumors. All of the benign tumors showed <1% proliferative activity, as measured by Ki-67/MIB-1 staining. In all three patients with malignant tumors who developed metastases and/or invasive local recurrence during follow-up, the tumors were positive for either hTERT expression or Ki-67/MIB-1 immunoreactivity. TA was not a significant discriminator between benign and malignant tumors, and the value of EMMPRIN and MMP-2 as predictive markers was limited. In conclusion, the findings imply that the combined use of Ki-67/MIB-1 and hTERT, in addition to histopathology, provides a highly specific tool to identify benign pheochromocytoma and abdominal paraganglioma cases that are not at risk of developing recurrent or metastatic disease.

Abdominal Neoplasms↗