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PubMed1978

Mediastinal paragangliomas (aortic body tumor): a report of four cases and a review of the literature.

Four cases of paraganglioma originating in the supra-aortic or aortico-pulmonary bodies are described. A review of the reported examples of aortic body tumors and a study of these four cases indicate that there is a high incidence of aggressive tumor growth in the mediastinum, with resultant important morbidity or death in 16 of 35 cases. The morphologic features of aortic body tumors are identical to those of paragangliomas of other locations, and the occurrence of invasive growth and/or metastasis cannot be predicted on histologic grounds.

Adolescent↗

PubMed1979

Aortico-pulmonary paraganglioma: report of a case with ultrastructural study and review of the literature.

The light microscopic and ultrastructural features of an aortico-pulmonary paraganglioma (A-PP) are presented. The tumor was characterized by organoid clustering of neoplastic chief cells to form Zellballen. Argyrophilic granules were demonstrated within chief cell cytoplasm using a modified Grimelus technique. Ultrastructurally, three distinct cell types were present within the tumor: endothelial cells, pericytes and neoplastic chief cells. Membrane-bound neurosecretory granules were present and measured 100 to 2000 nm in diameter. "Light" and "dark" chief cells were less distinct than previously reported in other head and neck paragangliomas. Analysis of the 36 documented A-PP reported in the English literature reveals that the tumor has been either incompletely excised or has been considered unresectable in one-third of the cases. The reported surgical mortality is 9%, or approximately equal to the incidence of malignant behavior. The treatment of choice is surgical resection but when this is not possible, radiation may be a useful adjunct in therapy.

Adolescent↗

PubMed1979

The triad of gastric epithelioid leiomyosarcoma, functioning extra-adrenal paraganglioma, and pulmonary chondroma.

The triad of gastric epithelioid leiomyosarcoma, functioning extra-adrenal paraganglioma, and pulmonary chondroma was found in two young female Mayo Clinic patients, and two of these tumors occurred in another two. Review of the world literature disclosed nine patients who probably had two or more of the three neoplasms. We are also aware of two other patients with two of the three tumors. The association of gastric epitheloid leiomyosarcoma, functioning extra-adrenal paraganglioma, and pulmonary chondroma may well constitute a syndrome because of 1) the improbability of the coincidental occurrence of this triad of tumors, 2) the multicentricity of the tumors in the organs or system affected, and 3) the development of the tumors at a young age. Because two of the components of the triad are potentially lethal, it is important that patients less than 35 years of age who have any one of the three tumors be examined periodically to search for the others.

Adolescent↗

PubMed1979

A nonfunctioning paraganglioma of vagus nerve: an ultrastructural study.

The clinical, histological, and ultrastructural aspects of a cervical paraganglioma of the vagus nerve, in a 66-year-old white man, have been discussed in detail. Ultrastructurally, the tumor chief cells contained characteristic membrane-bound and dense-cored neurosecretory granules which ranged in size from 85 millimicron to 190 millimicron. Unlike earlier ultrastructural reports, the present study showed the presence of sustentacular or supporting cells. These cells were smaller, darker, polymorphic, and were commonly located at the periphery of a single or group of chief cells. Furthermore, unlike earlier reports on vagal paragangliomas, nonmyelinated nerve fibers and an occasional axon were identified in the present fine structure study.

Aged↗

PubMed1982

Immunoreactive somatostatin in catecholamine-producing extra-adrenal paraganglioma.

A patient with catecholamine-producing extra-adrenal paraganglioma was found to have a high concentration of immunoreactive somatostatin (IR-SRIF) in the peripheral blood plasma (47.0 pg/ml, normal; 13.3 +/- 5.3 pg/ml, mean +/- SD). After removal of the tumor, the plasma SRIF level fell within normal range (8.7 pg/ml). In the resected tumor, a considerable amount of IR-SRIF (71.0 ng/g wet weight)( was also detected and SRIF-positive tumor cells were demonstrated by the immunoperoxidase technique. Chromatographic analysis of an extract of the tumor showed the presence of two IR-SRIF components of large molecular size besides a component consistent with authentic tetradecapeptide SRIF (SRIF 14), and one of these had the same molecular size as octacosapeptide SRIF (SRIF 28). This seems to be the first report of a catecholamine-producing extra-adrenal paraganglioma that produces IR-SRIF with molecular heterogeneity.

Adrenal Gland Neoplasms↗

PubMed1983

Paraganglioma of cauda equina. A report of a case and review of the literature.

A case of a cauda equina paraganglioma in a 13-year-old boy is described. A review of literature revealed six similar reported cases. All were intradural extramedullarly tumors located in the cauda equina-filum terminale region. In all, the presenting and dominant symptom was low back pain, while neurologic deficit was mild or absent. Excessive CSF protein levels appeared to be a characteristic feature of the disease. Histologically, the tumor displayed the typical "Zellballen" pattern, however mild nuclear pleomorphism and some mitotic figures were noted. As it is impossible to predict the biological behaviour of paragangliomas from the histologic appearance, complete surgical resection with close follow-up is indicated in such cases.

Adolescent↗

PubMed1986

High-altitude paragangliomas diagnostic and therapeutic considerations.

Forty-one cervical paragangliomas that occurred in 40 patients born and living in Mexico City, Mexico, were studied. Tumors were most common in women (38/40); were unilateral (39/40), without a side preference; were of the nonhereditary type; and were not malignant. Tumor imaging with radionucleaide angiographic scanning was noninvasive, innocuous, and reliable for screening, whereas selective carotid angiography allowed for definitive diagnosis and clinical stratification of patients. Surgical resection was performed in 29 patients; in 23 cases, en bloc resection was accomplished without a vascular compromise, whereas in 4 patients, a portion of the external carotid artery had to be ligated and resected. Two other patients required a vascular graft to restore blood flow to the internal carotid. Seven patients had postoperative transient cranial nerve palsies, and one had an incomplete hemiplegia. Permanent nerve damage occurred in seven patients. It was concluded that high-altitude paragangliomas are hyperplastic growths that result from adaptation to hypoxia. They are rarely true neoplasm and, in general, are not associated with functional loss. In view of this and of the high morbidity rate associated with surgical removal, it was recommended that these patients be referred to research centers where efforts toward the elucidation of the etiology and pathophysiology of these tumors can be carried out. Surgery should be reserved for symptomatic cases or cases in which the diagnosis remains in doubt. The decision to operate must weigh the following factors: The tumor's benign nature, its slow growth rate, the technical difficulties associated with its resection, the high postoperative morbidity rate, and the general condition of the patient.

Altitude Sickness↗

PubMed1990

Malignant paraganglioma of the posterior mediastinum. A case report and review of the literature.

A case of malignant paraganglioma arising from the posterior mediastinum and presenting with a chest wall metastasis is reported with immunocytochemical and ultrastructural documentation. The pathobiologic features of this rare disease are reassessed with a review of the literature. Cytoarchitectural features do not provide reliable information regarding malignant potential. However, evidence of local invasiveness at surgical exploration and/or lack of initial surgical control are likely to be associated with synchronous or metachronous metastatic disease. Although there are clinicopathologic differences warranting distinction between benign paragangliomas arising in the anterior and posterior mediastinal compartments, there is no apparent pathobiologic difference with regard to the malignant group.

Chromogranins↗

PubMed1990

Multiple spinal metastases from paraganglioma.

Isolated vertebral body metastases from paraganglioma are exceedingly rare. They have been reported to occur in the presence of active primary tumor in the neck, local recurrence, or widespread metastases. A unique case of carotid body tumor (paraganglioma) is reported with the following features: (1) multiple vertebral body metastases (C6, T9, and L3) presenting with spinal cord compression, and no evidence of local recurrence or other metastatic disease; (2) absence of mitoses on the original specimen or the metastatic deposit; and (3) a prolonged interval (9 years) to the development of symptomatic metastases.

Adult↗

PubMed1992

Neuropeptide Y secretion from a malignant extraadrenal retroperitoneal paraganglioma.

A patient with a malignant extraadrenal retroperitoneal paraganglioma had elevated levels of immunoreactive neuropeptide Y (NPY) in the peripheral blood (5988 pg/ml; normal, 123 +/- 30 pg/ml [mean +/- standard error of the mean]). A 6-month course of chemotherapy allowed surgical removal of the previously unresectable primary tumor. Postoperatively, the plasma NPY level initially fell to 1089 pg/ml; continued chemotherapy caused an additional decrease to 440 pg/ml. Four months after surgery, the plasma NPY level increased to 940 mg/ml, coincident with hepatic metastases. This case is the first report of a NPY-secreting clinically nonfunctional malignant extraadrenal paraganglioma. Determination of circulating NPY levels may be useful in the diagnosis and follow-up of patients with neuroendocrine tumors.

Female↗

PubMed1993

Mediastinal paragangliomas. A clinicopathologic and immunohistochemical study of 16 cases.

BACKGROUND: Paragangliomas of the mediastinum are rare neoplasms. Because of their rarity, the spectrum of morphologic and immunohistochemical features and biologic behavior of these tumors in this location has not been delineated. METHODS: A retrospective study of 16 cases of mediastinal paragangliomas was undertaken to evaluate their clinicopathologic and immunohistochemical features. RESULTS: The patients were 10 men and 6 women whose ages ranged from 16-69 years of age (mean, 42.5 years). Twelve tumors were located in the posterior mediastinum, and three tumors were located in the anterior mediastinum. In one case, the exact location of the tumor within the mediastinum was not available. Microscopically, the nesting pattern (zellballen) was the most commonly encountered. Areas of stromal hyalinization were also frequent and were marked in two tumors. In two cases there was a prominent spindle cell component, and two cases showed granular cell changes in the cytoplasm of the tumor cells. Cellular pleomorphism was observed in the majority of cases. Occasional mitoses were seen in a few cases. Immunohistochemical studies in 10 cases showed chromogranin reactivity in 10 of 10 cases, S-100 protein in 9 of 10 cases, leu-enkephalin in 6 of 10 cases, and neurofilament protein in 5 of 10 cases. Keratin was negative in all cases studied. All patients underwent surgical resection of their tumors, and one patient underwent adjuvant radiation therapy. Follow-up information ranging from 1-168 months (mean, 84.5 months) was obtained in 12 patients. Nine patients (75%) were alive and well at last follow-up. In one patient the tumor metastasized to spinal space and bone marrow, and another patient died with metastases to lymph node, lung, and pelvis 14 years after initial diagnosis. CONCLUSIONS: The only parameter found to correlate with aggressive behavior was the extent of circumscription and/or local infiltration of the tumor at initial resection. No clinical, histologic, or immunohistochemical features were otherwise found to separate aggressive from indolent clinical behavior. Regular follow-up is recommended in these patients due to the potential for these tumors to metastasize.

Adolescent↗

PubMed1993

Malignant paraganglioma presenting as Cushing syndrome with virilism in childhood. Production of cortisol, androgens, and adrenocorticotrophic hormone by the tumor.

BACKGROUND: A 12-year-old girl with intractable retroperitoneal paraganglioma experienced increased appetite, acne, obesity, "moon face," and enlargement of the clitoris during the course of the tumor. Plasma cortisol, serum testosterone, and dehydroepiandrosterone sulfate (DHEA-S) levels were increased to 34.1 micrograms/dl, 2.0 ng/ml, and 6.628 ng/ml, respectively. Adrenocorticotrophic hormone (ACTH) levels were not increased, and results of dexamethasone suppression tests were negative. Her condition was diagnosed as Cushing syndrome with virilism. Plasma cortisol levels were increased to a level of 107.1 micrograms/dl before death. METHODS: Tumor samples were obtained at the time of autopsy. The concentrations of cortisol, androgens, ACTH, and catecholamines were assayed in the tumor extracts. The indirect immunoperoxidase procedure was performed on fixed tissues for cortisol, DHEA-S, testosterone, and ACTH. RESULTS: Extracts of the tumor masses contained steroid hormones: the amount of immunoreactive cortisol was 1.64 micrograms/g wet weight; the amount of immunoreactive testosterone was 25.60 ng/g wet weight; immunoreactive DHEA-S, 579.00 ng/g wet weight; and immunoreactive ACTH, 891.00 pg/g wet weight in the metastatic mass of the lung. Immunohistochemically, immunoreactive cortisol, testosterone, and DHEA-S were detectable in the tumor cells. The adrenal gland was atrophic. CONCLUSIONS: The patient is the first reported with malignant paraganglioma with the capacity to produce cortisol, androgens, and ACTH.

Adrenocorticotropic Hormone↗

PubMed1994

Allelotype of head and neck paragangliomas: allelic imbalance is confined to the long arm of chromosome 11, the site of the predisposing locus PGL.

Paragangliomas of the head and neck region are usually slow growing, benign tumors. A considerable fraction has a positive family history, and the predisposing locus, PGL, has recently been assigned to 11q22-q23. The inheritance pattern of the disease suggests that PGL undergoes maternal genomic imprinting. We have investigated 26 tumor samples from 22 patients with head and neck paragangliomas for the occurrence of loss of heterozygosity (LOH) on all non-acrocentric autosome arms. LOH was found only on chromosome 11, with a marked clustering on the distal half of the q-arm. However, in many cases the resulting allelic imbalance relative to normal DNA was weak, suggesting that only part of the tumor showed this abnormality. In all eight cases where we were able to determine the parental origin, the allele undergoing loss was maternally derived. Clonality analysis with a polymorphic marker for the X-chromosome indicated that two of three informative female cases were polyclonal, although a number of tumors carry aneuploid stemlines in DNA flow cytometry. We conclude that either tumor heterogeneity or polyclonality may explain the partial allele loss events seen in certain cases.

Alleles↗

PubMed2003

A novel mutation in the SDHD gene in a family with inherited paragangliomas--implications of genetic diagnosis for follow up and treatment.

BACKGROUND: Early detection of paragangliomas (PGs) has been linked to low morbidity after surgical resection. Recent identification of causative genes (SDHB, SDHC, and SDHD) has made it possible to detect individuals at high risk for tumors. METHODS: We identified a three-generation family, with four individuals affected with PGs. Because pedigree analysis suggested maternal imprinting (the phenotype is present only if inherited through the paternal line), the SDHD gene (PGL1) was screened. RESULTS: A novel mutation that causes skipping of exon 3 was identified. Ten of the seventeen tested individuals carried the mutation. All six clinically unaffected individuals inherited the mutation from their mother. Five of them are men, with a 50% risk for affected progeny. CONCLUSIONS: To allow early treatment with low morbidity, genetic counseling is needed when familial paraganglioma is suspected. Asymptomatic carriers should be followed by cervical MRI. In addition, because pheochromocytomas may occur, catecholamine excretion can be performed. This screening should probably be proposed at 5 to 10 years of age.

Adult↗

PubMedDate not supplied

Paraganglioma of the thyroid.

We present a case of paraganglioma of the thyroid. The preoperative evaluation, surgical management, and histopathology are described. Four similar cases that have been reported are summarized, and a hypothesis for the origin of a paraganglioma within the thyroid capsule is proposed.

Aged↗

PubMedDate not supplied

Cytogenetic analysis of tissues from patients with familial paragangliomas of the head and neck.

BACKGROUND: Paragangliomas of the head and neck are slow-growing tumors that originate from neural crest cells. Between 7% and 9% of these tumors have a familial occurrence. The suspected gene for familial paragangliomas (FP) is transmitted with an autosomal dominant mode of inheritance with incomplete penetrance, and appears to exhibit genomic imprinting. It has been demonstrated by family studies that individuals who inherit the gene(s) from their father will develop the disease. Through linkage analysis, the gene(s) for FP has been postulated to be located on the long arm of chromosome 11. The discovery of many different genes has been elucidated through the cytogenetic analysis of affected individuals who carry specific chromosome aberrations. This project was designed to look for chromosome abnormalities in several second-generation family members to further assist in the localization of the gene(s) for FP. METHODS: This study involved the cytogenetic evaluation of lymphocytes, fibroblasts, and tumor cells of several second-generation family members from a three-generation family with FP of the head and neck to look for chromosome abnormalities generally, and for abnormalities of chromosome 11 specifically. Standard cytogenetic techniques were used for lymphocyte and fibroblast cultures. Tumor cells were cultured in a collagen matrix with F12 medium supplemented with 3% L-glutamine and 10% fetal calf serum. RESULTS: There were no detectable abnormalities of chromosome 11 in any of the cells. However, nonrandom abnormalities of chromosomes 5 and 7 were seen in some of the tumor cells of one FP patient. To our knowledge, this is the first article which demonstrated the ability to successfully culture FP of the head and neck.

Adult↗

PubMed2006

Bone metastases from head and neck paragangliomas: uncommon MR findings in an uncommon condition--report of three cases.

Malignancy (defined as metastatic disease) has been reported in nearly 5% of head and neck paragangliomas. Metastases preferentially target the lymph nodes, lung, liver, or bone. We describe three patients with multiple silent bone metastases exhibiting a fatty halo at MRI that coexisted with expansive bone lesions. In all cases, (123)I-metaiodobenzylguanidine ((123)MIBG) scintigraphy showed no abnormal tracer uptake, whereas (111)In-pentetreotide scintigraphy visualized a few silent bone metastases. These findings indicate that MRI should be included in the staging of paraganglioma patients with risk factors for malignancy.

3-Iodobenzylguanidine↗

PubMed1980

Malignant pheochromocytoma (paraganglioma) of the organ of Zuckerkandl: a study of two cases.

Ten percent of pheochromocytomas are found in extra-adrenal locations and are called paragangliomas. In recent years we have had the opportunity to study two patients with malignant paragangliomas of the organ of Zuckerlandl. Only 13 cases of such neoplasms have been reported previously. Our cases are of interest because of their features of recurrence, distant metastases, response to adjuvant therapy, and long survival.

Autonomic Nervous System↗