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PubMedDate not supplied

Immunohistochemical features of paragangliomas.

Immunohistochemistry is part of the routine diagnosis of the neuroendocrine tumors. In our study, we included 52 paragangliomas with various localizations by routine histology and immunohistochemistry. In order to increase the diagnostic specificity, a complex immunohistochemistry panel has been performed consisting of Bcl-2, Ki-67, Bax and Pituitary Adenylate Cyclase-Activating Peptide (PACAP), somatostatin, VIP and Calcitonin Gene Related Peptide (CGRP). After heat induced antigen retrieval, the immunostaining was performed by StreptABC using DAB as a chromogen. We were the first to demonstrate the presence of Bax and PACAP in paragangliomas. Some of the used markers are of prognostic value. The relationship between Bcl-2 and Bax is decisive in generating the final response to the input apoptotic signals. The Ki-67 antigen staining has gained wide acceptance in prognostic evaluation of other tumor types. We noted a small number of Ki-67 positive cases, which signifies a low mitotic activity of these tumors and a relatively high number of Bax positivities (32.9%) and the much lower number of Bcl-2 positivities (11.39%), and could explain the benign behaviour of paragangliomas.

Biomarkers, Tumor↗

PubMed1992

Paraganglioma of the inferior laryngeal paraganglia. A case report.

Laryngeal paragangliomas are uncommon, usually benign neoplasms. The overwhelming majority of these tumors are supraglottic, arising from the superior laryngeal parasympathetic paraganglia. Few paragangliomas of the infraglottic region have been described. We describe the case of a 35-year-old man with a family history of carotid body tumors who presented with an infraglottic paraganglioma.

Adult↗

PubMed2002

Pheochromocytomas and paragangliomas in von Hippel-Lindau disease: a role for laparoscopic and cortical-sparing surgery.

HYPOTHESIS: Von Hippel-Lindau disease (VHL) is an autosomal-dominant disorder characterized by benign and malignant tumors involving the central nervous system, kidneys, pancreas, adrenal glands, and paraganglia. Appropriate management of pheochromocytomas and paragangliomas in VHL is evolving as we better understand the genetics and natural course of the disease and master advanced surgical techniques for adrenalectomy. DESIGN: Retrospective chart review. SETTING: Tertiary referral center. PATIENTS: A total of 109 patients identified at the Mayo Clinic, Rochester, Minn, with VHL (60 males and 49 females) between January 1, 1975, and June 30, 2000. Seventeen patients (16%) had an identifiable adrenal mass and 3 patients had paragangliomas. Follow-up was complete in all but 2 patients. MAIN OUTCOME MEASURES: Clinical presentation, preoperative evaluation, surgical management, and outcome. RESULTS: Three patients with paragangliomas and 13 of 17 patients with adrenal masses underwent surgical resection. Median age at time of diagnosis was 30 years (range, 16-47 years); 8 (40%) were asymptomatic. Fractionated urinary catecholamine and metanephrine concentrations were normal in one third of patients. Computed tomographic scanning identified 20 (83%) of 24 tumors. Adrenalectomies were performed as unilateral or bilateral, open or laparoscopic, and, finally, total or cortical-sparing. Seven (50%) of the patients underwent other concurrent abdominal procedures. There were no deaths, with an overall operative morbidity of 2 patients (14%). Only the 2 patients in whom bilateral total adrenalectomies were performed became corticosteroid dependent. No recurrences have been noted to date. CONCLUSIONS: A multidisciplinary approach is imperative for proper examination and monitoring of patients with VHL. Evaluation should begin early in life and always before elective surgery and childbirth. All adrenal masses in patients with VHL should be thoroughly evaluated and most should be resected. Early intervention and advanced surgical techniques better allow for cortical-sparing and laparoscopic procedures. With low recurrence rates, corticosteroid independence can be maintained for prolonged periods.

Adolescent↗

PubMed1975

Nonchromaffin paraganglioma (chemodectoma) of thyroid region.

A case of a paraganglioma located over the thyroid cartilage in the midline is reported. The tumor had all the appearances of a carotid body tumor, but its odd location could not be explained on the basis of previously known areas where paraganglionic tissues have been found. Since a paraganglioma has also hitherto been described within the thyroid gland, clinicians and pathologists alike are cautioned that unusually located paragangliomas may be expected in the future.

Cell Nucleus↗

PubMed1976

Vagal body tumor (nonchromaffin paraganglioma, chemodectoma, and carotid body-like tumor) with cervical node metastasis and familial association: ultrastructural study and review.

A case of a vagal body tumor with a solitary cervical node metastasis is described. There was a remarkable familial association: a biopsy-proven paraganglioma occurred in two members of the patient's family and there was a strong clinical suspicion of a paraganglioma in two others. In a review of the literature, eight patients with metastasizing vagal body tumors and eight patients in whom the vagal body tumor was a component of multicentric presentation of paragangliomas were found. Only one other patient in whom there was a familial occurrence involving a vagal body tumor has been reported. Ultrastructural study of the cervical node metastasis revealed the presence of light and dark chief cells containing scanty, membrane-bound, densecore, neurosecretory-type granules. Atypical granules were noted in a few of the dark cells. Nerve fibers, synaptic vesicles, and sustentacular cells, such as occur in normal paraganglionic tissue, were not observed in this tumor.

Adult↗

PubMed1979

Retroperitoneal paraganglioma and systemic amyloidosis: a case report.

The case of a 50-year-old woman with a retroperitoneal paraganglioma and nephrotic syndrome is presented. After the tumor was removed deposits of amyloid material were observed in the paraganglioma, kidneys, and liver. There was no thrombosis of the renal vein. The clinical picture (fever and nephrotic syndrome) was similar to that of patients with amyloidosis associated with neoplasms. The nephrotic syndrome disappeared 8 months after the operation. The patient has not had fever or nephrotic manifestations over the past 4 years and is asymptomatic at the present time. We believe this to be the first reported case of paraganglioma associated with amyloidosis. Nor have we found any references to clinical and biological remission of the nephrotic syndrome following surgical removal of the concomitant neoplasm.

Amyloidosis↗

PubMed1983

Paraganglioma of the cauda equina.

The authors present two cases of paraganglioma located in the cauda equina. Diagnosis was suspected by light microscopy and confirmed with the identification of characteristic neurosecretory vesicles. The presence of cytoplasmic filaments in both of our cases and of cilia in one case are very unusual findings in paraganglioma and seem to be characteristic of this location. The cauda equina is a extremely rare location for this type of tumor and, as far is known, only ten other cases have been reported. Paraganglioma should be considered as a diagnostic possibility in order not only to make a correct diagnosis, but also to prepare tissues for special stains and biological and electronmicroscopic studies.

Adult↗

PubMed1986

Paraganglioma of the cauda equina region. Clinicopathologic study of 31 cases with special reference to immunocytology and ultrastructure.

Thirty-one paragangliomas of the cauda equina region were studied (18 men and 13 women, ages 30-71 years [mean, 51 years]). Symptoms (1 day to 15 years in duration; mean, 48 months) included low back pain (87%), sensory/motor deficits (35%), urinary/fecal incontinence (13%), and paraplegia (6%). All patients studied had some myelographic block. Cerebrospinal fluid protein level ranged from 56 to 7000 mg/dl (mean, 1109 mg/dl). Most tumors were limited to the filum terminale, although one also involved the conus medullaris and two clearly arose from a caudal nerve root. All but one were entirely intradural. The tumor was totally excised in 26 cases; these patients remain disease-free. Of three patients whose tumors were excised subtotally, two received radiotherapy; the one non-radiated patient died of tumor-related complications. No autopsy was performed. One partially encapsulated tumor that had been subjected to biopsy and irradiation presented 1 year later with osseous invasion and retroperitoneal extension; 20 years after subtotal excision, this patient is alive but paraplegic. Morphologically, all tumors resembled paraganglioma at other sites. Cytologic atypia and mitotic activity generally were absent to mild. Fourteen (45%) cases showed ganglionic differentiation. All tumors tested were immunoreactive for neuron-specific enolase and neurofilament protein, and most showed somatostatin or serotonin reactivity. S-100 protein immunoreactivity was noted in sustentacular cells and, to a lesser extent, within chief cells and neurons. The authors conclude that paragangliomas are largely benign and encapsulated and respond to simple resection. When surgically feasible, gross total removal should be the goal of surgery. When subtotal resection is necessary or when local invasion leaves a question as to completeness of tumor removal, irradiation seems mandatory although far from guaranteeing prevention of recurrence. Biopsy alone is undesirable.

Adult↗

PubMed1987

Extraadrenal paragangliomas. An immunocytochemical and ultrastructural report.

Although the majority of extraadrenal paragangliomas are nonfunctional, some of these tumors are associated with hormone production and clinical symptoms, notably hypertension. The authors have investigated 22 paragangliomas, five of which were diagnosed as clinically functional in a light microscopic immunocytochemical and electron microscopic study (nine cases). Histologically, all the paragangliomas exhibited similar features, with a "Zellballen" pattern of polygonal cells. All 22 cases were strongly immunoreactive to protein gene product 9.5 (PGP 9.5) antisera and moderately reactive to antineuron-specific enolase (NSE) sera. Ten cases (five functional) were focally immunoreactive to antichromogranin sera. Seven cases (four functional) were immunoreactive to neuropeptide Y and enkephalin antisera, and six (five functional) to tyrosine hydroxylase antisera. The clinically functional tumors expressed at least two of the antigens, enkephalin, neuropeptide Y, or tyrosine hydroxylase, whereas none of the 17 nonfunctional possessed more than one of these. Electron microscopic study revealed cells from all the nine cases studied to contain secretory granules. Granule sizes ranged from 100 to 280 nm and the morphologic examination of the secretory granules generally showed a dense core with a membrane-bound halo of variable size. Secretory granules were observed in the five functional cases and these were larger (220-280 nm) than those seen in the nonfunctional tumor cells (100-180 nm). Also, tumor cells from the functional cases contained numerous dilated mitochondrial profiles.

Adult↗

PubMed1991

An unusual organoid tumor. Alveolar soft part sarcoma or paraganglioma?

The histogenesis of alveolar soft part sarcoma (ASPS) is a subject of continued debate. Although many recent reports suggest a muscle origin, others advocate a neuroendocrine derivation. A tumor in the chest wall of a 16-year-old woman was diagnosed and treated as ASPS. The light microscopic, electron microscopic, and immunohistochemical findings showed features of both ASPS and paraganglioma. In addition, this lesion was positive for antibody to glucagon, a characteristic of neither ASPS nor paraganglioma, although seen in a few gangliocytic paragangliomas. This case demonstrates the need for continued inquiry into the histogenesis of ASPS.

Adolescent↗

PubMed1994

A patient with malignant paraganglioma that simultaneously produces adrenocorticotropic hormone and interleukin-6.

BACKGROUND: To the authors' knowledge, there have been no reports describing a case of malignant paraganglioma that produces both adrenocorticotropic hormone (ACTH) and interleukin-6 (IL-6). METHODS: The clinical course and pathophysiology of a patient with Cushing's syndrome induced by ectopic ACTH syndrome caused by a cervical malignant paraganglioma with elevated plasma levels of IL-6 was investigated. RESULTS: Ultrasonography, computed tomography, and magnetic resonance imaging of the neck revealed the presence of a tumor around the area from which the blood sample that showed the highest levels of ACTH and IL-6 was obtained by selective catheterization. Immunohistochemical staining of the removed tissue demonstrated the presence of ACTH and IL-6 proteins in the tumor cells. Reverse transcriptase-polymerase chain reaction also revealed the existence of IL-6 mRNA in those cells. CONCLUSION: The malignant paraganglioma of ectopic ACTH syndrome may produce IL-6. The present investigation provides new observations concerning ectopic ACTH syndrome.

ACTH Syndrome, Ectopic↗

PubMed2006

A novel G106D alteration of the SDHD gene in a pedigree with familial paraganglioma.

Head and neck paragangliomas are tumors derived from parasympathetic paraganglia. Familial cases account for 10% or more of these tumors, and mutations of the genes encoding subunits for the mitochondrial respiratory chain complex II, SDHD, SDHB, and SDHC, have been reported. We analyzed mutations in the all four SDH genes, SDHA through SDHD, in a Japanese family with cervical paraganglioma that include a father with bilateral tumors and his daughter with a malignant left carotid body tumor with nodal metastasis. This pedigree harbored a germline G106D alteration in exon 4 of the SDHD gene that has not previously been reported to date. The tumors of the father expressed biallelic SDHD, but the SDHD expression was highly suppressed by an unknown mechanism(s) in tumors of his daughter, and the wild-type allele was predominantly suppressed in the metastatic node. These results suggest that the missense dysfunction of SDHD prepares neoplastic condition and that expressional silencing, particularly of the wild-type allele, plays an important role in the malignant transformation of the paragangliomas. Our results may lead to a better understanding of this disease and to the development of methods for prevention of this disease.

Alleles↗

PubMed2002

Radiosurgery for paraganglioma of the temporal bone.

PURPOSE: To report the outcome of patients with paraganglioma of the temporal bone treated with stereotactic radiosurgery at the University of Florida. METHODS AND MATERIALS: Between January 1997 and June 1999, five patients with paraganglioma of the temporal bone were treated with Linac-based stereotactic radiosurgery at the University of Florida. The ages of the three female and two male patients were between 40 and 88 years (median, 49 years). Four patients were treated at initial presentation, and one had recurrent disease. Treatment volumes ranged from 4.9 cm3 to 18.4 cm3, with a mean of 10.84 cm3. The dose applied to the margin of the tumor varied from 12.5 to 15 Gy (median, 15 Gy). The treatment dose was specified to the 80% isodose shell in two cases and to the 70% isodose shell in three cases. The median follow-up time was 27 months, ranging from 14 to 50 months. RESULTS: One of four previously untreated patients had a relapse at the primary tumor site. Treatment failure occurred at the field margin 6 months after radiosurgery; the patient was subsequently treated with fractionated stereotactic radiotherapy and at the time of analysis had no evidence of disease, 21 months after initiation of salvage therapy. The patient treated at the time of recurrence after conventional radiotherapy had a local recurrence 40 months after radiosurgery. At the time of this recurrence, the patient had biopsy-proven metastatic disease in two cervical lymph nodes, and no salvage therapy was performed. All patients were alive at the time of the analysis, one with disease present. Presenting symptoms improved in two patients and stabilized in one. The two patients who had local recurrence develop had worsening of their symptoms. One patient had a cranial nerve V palsy develop 6 months after treatment, which resolved after a few months. CONCLUSIONS: In this series, the results with stereotactic radiosurgery are discouraging compared with our results with conventional fractionated radiotherapy in patients with paraganglioma of the temporal bone.

Adult↗

PubMed2005

Pheochromocytoma and functional paraganglioma syndrome: no longer the 10% tumor.

Pheochromocytomas and abdominal paragangliomas are catecholamine-producing tumors of the sympathetic nervous system, while head and neck paragangliomas are non-secreting tumors of parasympathetic origin. Recent developments in clinical and molecular research on these tumor forms have significantly clarified their genetic backgrounds and challenged the view of "pheochromocytoma as the 10% rule tumor." Firstly, a larger proportion of these tumors are today discovered in normotensive patients during imaging carried out for other reasons than suspicion of pheochromocytoma. Secondly, although the differential diagnosis between malignant and benign tumors remains a challenge, the risk of malignancy well exceeds the classical 10% in patients with extra-adrenal disease, and/or carriers of germ-line SDHB mutations. Finally, up to a third of patients carry a germ-line mutation in a gene predisposing to pheochromocytoma and/or paraganglioma. Identification of a constitutional mutation in RET, VHL, SDHD, or SDHB has implications for clinical screening and follow-up for both the patient and for relatives at risk who can be identified by screening for the same mutation. Genetic testing in apparently sporadic cases is therefore regarded as beneficial, especially in patients diagnosed before 50 years of age, and in patients with bilateral, multifocal, malignant and/or extra-adrenal disease.

Abdominal Neoplasms↗

PubMed1984

Combined paraganglioma and glioma of conus medullaris and cauda equina.

A mixed paraganglioma and glioma occurred in the conus medullaris and cauda equina of a woman. The tumor was subdural, encapsulated, and vascular. It was first manifested with symptoms and signs of compression of the cauda equina when she was 19 years of age and was excised when she was 24. The tumor was attached to the conus medullaris, the dura mater, and a nerve root of the cauda equina. Striking light and dark cells with an organoid arrangement mingled with a few glial fibrillary acidic protein (GFAP)-positive neuroglia forming a combined paraganglioma and glioma. Twenty-three paragangliomas were reported in the literature including the present case. The sex and age of recorded 19 patients demonstrated ten males and nine females with an average age of 46.3 years. Twenty tumors were subdurally situated at the conus medullaris and cauda equina. Three neoplasms were epidural and thoracic in location.

Adult↗

PubMed2003

SDHD mutations in head and neck paragangliomas result in destabilization of complex II in the mitochondrial respiratory chain with loss of enzymatic activity and abnormal mitochondrial morphology.

Hereditary head and neck paragangliomas are tumours associated with the autonomic nervous system. Recently, mutations in genes coding for subunits of mitochondrial complex II, succinate-ubiquinone-oxidoreductase (SDHB, SDHC, and SDHD), have been identified in the majority of hereditary tumours and a number of isolated cases. In addition, a fourth locus, PGL2, has been mapped to chromosome 11q13 in an isolated family. In order to characterize phenotypic effects of these mutations, the present study investigated the immunohistochemical expression of the catalytic subunits of complex II (flavoprotein and iron protein), SDH enzyme activity, and mitochondrial morphology in a series of 22 head and neck paragangliomas. These included 11 SDHD-, one SDHB-, two PGL2-linked tumours, and eight sporadic tumours. In the majority of the tumours (approximately 90%), the enzyme-histochemical SDH reaction was negative and immunohistochemistry of catalytic subunits of complex II showed reduced expression of iron protein and enhanced expression of flavoprotein. Ultrastructural examination revealed elevated numbers of tightly packed mitochondria with abnormal morphology in SDHD-linked and sporadic tumours. Immuno-electron microscopy showed localization of the flavoprotein on the remnants of the mitochondrial inner membranes, whereas virtually no signal for the iron protein was detected. These results indicate that the function of mitochondrial complex II is compromised in the majority of head and neck paragangliomas.

Adult↗

PubMed2004

Multiparameter DNA flow-sorting demonstrates diploidy and SDHD wild-type gene retention in the sustentacular cell compartment of head and neck paragangliomas: chief cells are the only neoplastic component.

Head and neck paragangliomas are considered to be biphasic tumours, composed of two distinct cell types: chief cells and sustentacular cells. A substantial number of these tumours show mutations in the SDHD gene located at chromosome 11q23. Although there is general agreement that paragangliomas are a neoplastic proliferation of chief cells, the nature of sustentacular cells is still a matter of debate. To clarify the nature of sustentacular cells further, multiparameter DNA flow cytometry was performed utilizing S-100 labelling as a selective marker of the sustentacular fraction simultaneously with DNA content measurement in six head and neck paragangliomas. S-100-positive fractions and other tumour-cell populations were flow-sorted and restriction digestion analysis of SDHD mutations was performed on each fraction. Flow cytometry demonstrated that the S-100 labelled cells were diploid. Restriction digestion analysis in informative cases revealed retention of the wild-type SDHD allele in S-100-positive fractions and loss of the wild-type allele in S-100-negative fractions. These data strongly suggest that sustentacular cells should be regarded as a non-neoplastic cell population that may be induced as a tumour-specific stromal component by chief cells.

Allelic Imbalance↗

PubMed2007

Cervical paraganglioma--a case report and review of all cases reported to the Manchester Children's Tumour Registry 1954-2004.

We report a 6-year-old male with left-sided ptosis, aniscoria and an initially missed slow growing left-sided neck mass, which was surgically excised when he was 9 years old and confirmed to be a paraganglioma. Seven years later he developed recurrent symptoms and was found to have a recurrence in the anterior mediastinum. We also report on all cases of cervical paragangliomas registered with the Manchester Children's Tumour Registry (MCTR) for the 50-year period 1954-2004. Paragangliomas are very rare tumours in the head and neck but should be considered in the differential diagnosis of neck masses especially when presenting with Horner syndrome. Recurrent symptoms and signs of hypertension herald recurrence. As these tumours can form part of a familial syndrome, long-term follow-up is necessary. Family members should be screened for early detection.

Adolescent↗