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PubMed2006

Pathology of pheochromocytoma and extra-adrenal paraganglioma.

The 2004 WHO classification of endocrine tumors defines pheochromocytoma as a tumor arising from chromaffin cells in the adrenal medulla. Closely related tumors in extra-adrenal sympathetic and parasympathetic paraganglia are classified as extra-adrenal paragangliomas. A pheochromocytoma is an intra-adrenal sympathetic paraganglioma. While arbitrary, this nomenclature serves to emphasize important distinctive properties of intra-adrenal tumors that must be taken into account in clinical practice and research. Those include an often adrenergic phenotype, a relatively low rate of malignancy, and a predilection to occur in particular hereditary syndromes. Current roles of pathology are limited to distinguishing primary or metastatic pheochromocytomas/paragangliomas from other endocrine or nonendocrine tumors, and flagging tumors that show features suggestive of malignant potential or syndromic disease. Future roles may involve more definitive assessment of malignancy, genotype-phenotype correlation, and identification of targets for therapy. Pathology practice currently rests mostly on interpretation of conventional histological sections stained with hematoxylin and eosin, with variable ancillary application of immunohistochemical staining. Malignancy is currently defined by the presence of metastases, not local invasion. Local invasion alone is a poor predictor of metastases, and the absence of apparent invasion does not preclude development of metastases. The two types of aggressive behavior might therefore have different biological underpinnings, and those will be resolved most effectively if consistent terminology is employed. In order to be optimally informative, pathology reports must employ consistent nomenclature and incorporate standard elements. Templates or checklists for minimal standard reporting are recommended by several pathology associations, but identification of some recommended and optional elements is currently subjective or inconsistent.

Adrenal Gland Neoplasms↗
PubMed2005

Biochemical and clinical manifestations of dopamine-producing paragangliomas: utility of plasma methoxytyramine.

Measurements of plasma-free normetanephrine and metanephrine provide a sensitive test for diagnosis of pheochromocytoma but may fail to detect tumors that produce predominantly dopamine. Such tumors are extremely rare, usually found as extraadrenal paragangliomas. This report describes measurements of plasma concentrations of free methoxytyramine, the O-methylated metabolite of dopamine, in 120 patients with catecholamine-producing tumors, including nine with extraadrenal paragangliomas secreting predominantly dopamine. In seven of these nine patients, tumors were found incidentally or secondary to the space-occupying complications of the lesions. Plasma concentrations of free methoxytyramine and dopamine were increased in all nine patients, including two with normal plasma and urinary normetanephrine and metanephrine and normal urinary outputs of dopamine. Relative increases above normal for plasma methoxytyramine (104-fold) and dopamine (56-fold) were much greater (P < 0.001) than those for urinary dopamine (3-fold). Insensitivity of the latter for identification of dopamine-secreting tumors was due to dependence of the urinary amine on renal extraction and decarboxylation of circulating 3,4-dihydroxyphenylalanine. Measurements of plasma-free methoxytyramine, in addition to normetanephrine and metanephrine, are unlikely to improve diagnosis of pheochromocytomas in hypertensive patients with symptoms of catecholamine excess but may be useful in selected patients for identification of tumors that produce predominantly dopamine.

Adrenal Gland Neoplasms↗
PubMed2006

Review: Should patients with apparently sporadic pheochromocytomas or paragangliomas be screened for hereditary syndromes?

CONTEXT: The recent identification of germline mutations of the mitochondrial complex II genes in variants of paraganglioma/pheochromocytoma syndrome has enlarged the number of known causative genes for hereditary pheochromocytoma. A question confronting clinicians is whether they should screen patients with apparently sporadic pheochromocytomas for unsuspected germline mutations of some or all of the seven genes known to cause hereditary paraganglioma or pheochromocytoma (NF1, VHL, RET, MEN1, SDHD, SDHC, and SDHB). A positive answer was suggested by a report that placed the estimate of hereditary disease in apparently sporadic pheochromocytoma as high as 24%. EVIDENCE ACQUISITION: We applied clinically useful criteria to a review of the literature, defining cases of apparently sporadic pheochromocytoma as those without a suspicious personal or family history, with a focal, unilateral pheochromocytoma, and presenting at age less than 50 yr. EVIDENCE SYNTHESIS: We reduced the overall estimate of unsuspected hereditary pheochromocytoma patients with apparently sporadic pheochromocytoma to approximately 17%. Mutations in only three genes (VHL, SDHB, and SDHD) accounted for almost this entire minority, and unsuspected RET mutation was rare. Costs, coverage by insurance, the potential effect on insurability, and deficient information for populations outside of referral centers should be considered before recommending genetic testing in patients with apparently sporadic presentations of pheochromocytomas. CONCLUSION: We recommend genetic testing for patients with an apparently sporadic pheochromocytoma under the age of 20 yr with family history or features suggestive of hereditary pheochromocytoma or for patients with sympathetic paragangliomas. For individuals who do not meet these criteria, genetic testing is optional.

Adult↗
PubMed1997

Functioning thoracic paraganglioma: association with Von Hippel-Lindau syndrome.

Functioning thoracic paraganglioma (pheochromocytoma) is unusual and therefore suggestive of a pathogenesis distinct from that of sporadic adrenal pheochromocytoma. To determine whether the pheochromocytoma-associated syndromes Von Hippel-Lindau disease (VHL) and multiple endocrine neoplasia type 2 (MEN 2) play a role in the development of thoracic functioning paragangliomas, germline DNA from five unselected patients with this rare tumor was analyzed for mutations in the genes that predispose to VHL and MEN 2. Genetic investigations and further clinical data revealed that three had VHL, with two different germline mutations of the vhl gene, but no individual was affected by MEN 2. Two of the three patients with VHL did not show any additional VHL-associated lesions. This result suggests that VHL should be considered in the differential diagnosis of thoracic pheochromocytoma, as such a diagnosis carries further important implications for the patient and family. Conversely, in patients suspected of a catecholamine-secreting tumor and known VHL, thoracic localization should be considered if an adrenal pheochromocytoma cannot be detected.

Adolescent↗
PubMed1984

Intrathecal paraganglioma of the cauda equina.

A 33-year-old obese woman with chronic pain in her lower back presented with weakness of her right quadriceps and diminution of her patellar reflex. Her myelogram revealed an intradural oval mass at level of the interspace between her 3rd and 4th lumbar vertebrae. Operation disclosed an encapsulated mass attached to her filum terminale. Histopathological examination showed a paraganglioma with typical dense core granules, confirmed by electron microscopic examination. We report our patient to call attention to intrathecal paragangliomas as a rare and treatable cause of the cauda equina syndrome.

Adult↗
PubMed1989

Paraganglioma of the orbit with extension to the middle cranial fossa: case report.

Intraorbital paragangliomas are rare. A case of intraorbital paraganglioma with extension to the middle cranial fossa is reported. Progressive throbbing, hemicranial headaches, unilateral proptosis, and visual failure are the presenting symptoms. The computed tomographic and operative findings suggested a meningioma. Surgical removal of the tumor resulted in total relief of symptoms and recovery of vision. The pathogenesis and pathological findings are discussed.

Adult↗
PubMed1993

Paragangliomas of the sellar region: report of two cases.

Two cases of paraganglioma arising from the parasellar region are presented. Both occurred in middle-aged women who sought treatment of headaches but who had no endocrinological dysfunction; one case was associated with ophthalmoplegia from cavernous sinus involvement. Diagnosis in both cases was confirmed by typical histological appearance and cytochemical demonstration of immunoreactive chromogranin in tumor cells. The pathological features and possible pathogenesis of parasellar paragangliomas are discussed.

Adult↗
PubMed2003

Flow cytometric DNA analysis for determination of malignant potential in adrenal pheochromocytoma or paraganglioma: an Indian experience.

BACKGROUND: We analyzed the histological features and DNA flow cytometric results in 34 patients with pheochromocytoma and paragangliomas and attempted correlation with the biological behavior for determination of the malignant potential of these tumors. METHODS: DNA analysis was done on a FACSort flow cytometer using paraffin-embedded tissues. Histopathological analysis was performed using parameters, i.e., cell size (large, medium, and small), cell size variation, mitotic rate, nuclear pleomorphism, golden yellow to brown pigment in the tumor, necrosis, and venous invasion. RESULTS: Six tumors had high (>5/10HPF) mitotic rate while venous invasion was seen in three tumors. Fifty percent (18/34) of patients had aneuploid tumors, and 68% (23/34) of patients had high (>10%) S-phase fraction tumors. Aneuploidy correlated with >5/10HPF mitotic rate (P <.05) and diploidy with golden yellow to brown pigment (P <.01). The patients with aneuploid tumor had a worse prognosis than patients with diploid tumors (P =.004). No such difference was observed with low and high S-phase fractions (P =.748), presence and absence of venous invasion (P =.927), and mitotic rate (P =.159). Nuclear pleomorphism and necrosis were not significant factors in prognosis. CONCLUSIONS: Flow cytometric DNA analysis of paragangliomas and pheochromocytomas correlated with biological behavior in the patients with regard to metastasis and overall survival in the patients.

Adrenal Gland Neoplasms↗
PubMed2001

Long-term oncological results in 47 cases of jugular paraganglioma surgery with special emphasis on the facial nerve issue.

Oncological and functional results were assessed in 47 type C and/or D jugular paraganglioma operated on between 1984 and 1998 using the classical infratemporal fossa type A approach (mean follow-up = 66 months). In 24 instances, however, the facial nerve was not re-routed. Total resection was achieved in 33 cases (70 per cent). In 25 patients available for follow-up this resulted in a 92 per cent cure rate while two patients (eight per cent) developed recurrences that are being followed-up clinically and radiologically. Sub-total resection, leaving infracentimetric tumour remnants after being coagulated, was achieved in 14 cases (30 per cent). In 11 patients available for follow-up, only three cases developed tumour regrowth (27 per cent) that was controlled by salvage irradiation or surgery while in the other cases tumour remnants remained stable (73 per cent). Symptomatic post-operative lower cranial nerve impairment was observed in 23 per cent. When results were analysed depending on whether the facial nerve had been re-routed (n = 18) or not (n = 24), the incidence of facial paralysis HB grade III or more at one year was 33 per cent and eight per cent, respectively. Total resection was achieved in 56 per cent when the facial nerve was re-routed versus 75 per cent when it was not, the difference being due to a higher incidence of large tumours in the first group. The present study suggests that: 1) surgical resection of jugular paraganglioma provides overall satisfactory results, i.e. a 86 per cent rate of either cure or tumour remnant stabilization, but carries a significant risk of iatrogeny; 2) complete tumour removal should not be attempted, especially in patients over 60 years of age with no pre-operative neurological deficits, since leaving infracentimetric tumour remnants has no major detrimental effect on the final outcome; 3) facial nerve transposition carries a significant risk of cosmetic sequelae while it does not provide significant advantages in terms of tumour resection and long-term oncological control.

Adolescent↗
PubMed2001

Functional imaging as an aid to decision-making in metastatic paraganglioma.

Malignant paraganglioma is a rare and slow growing tumour of neuroendocrine origin. At the time of diagnosis, the tumour is usually widespread, with limited therapeutic options. A variety of functional imaging studies are available for staging the disease, guiding therapy and monitoring treatment response. These include 123I-MIBG or 131I-MIBG, 111In-pentetreotide or 111In-lanreotide (somatostatin analogues), and 18F-FDG positron emission tomography. Various radionuclides, including 131I and 90Y, can be targeted to the tumour using MIBG or pentetreotide. Such targeted radionuclide therapy may provide valuable long-term palliation in such patients. We present two cases with metastatic paragangliomas who had widespread soft tissue and bone metastases. One patient was treatment naive and the second had received previous chemotherapy. The functional imaging work-up performed and the targeted radionuclide therapies considered in these patients are described. Both patients were treated with 131I-MIBG. Partial tumour response and complete symptomatic and hormonal response was achieved in one patient; in the second patient there was no change.

3-Iodobenzylguanidine↗
PubMed2004

Case report: drug interference with MIBG uptake in a patient with metastatic paraganglioma.

Metaiodobenzylguanidine (MIBG) labelled with iodine-131 ((131)I) has become a well established therapeutic tool for inoperable metastastic tumours of paraganglioma. There are different pharmacological substances known to interfere with MIBG-uptake which may result in a false negative MIBG scan. We present the case of a 26-year-old male polytoxicomanic patient with metastatic paraganglioma, who underwent MIBG therapy. During earlier therapies, MIBG uptake in the metastatic lesions was very high. A post-therapeutic whole-body scan subsequent to recent (131)I-MIBG therapy failed to detect the vast majority of metastatic lesions-except for two. (18)F-fluorodeoxyglucose (FDG) positron emission tomography (PET) showed metastases with a similar distribution to the initial MIBG scan. The possible reasons for the discrepancy in the findings of the MIBG scans and the (18)F-FDG-PET scan are discussed with special emphasis on drug intake prior to MIBG administration, increased MIBG turn-over and unknown drug mixture interference with MIBG uptake.

3-Iodobenzylguanidine↗
PubMed2006

Stable disease and improved health-related quality of life (HRQoL) following fractionated low dose 131I-metaiodobenzylguanidine (MIBG) therapy in metastatic paediatric paraganglioma: observation on false "reverse" discordance during pre-therapy work up and its implication for patient selection for high dose targeted therapy.

The incidence of paraganglioma in the paediatric population is exceedingly rare, accounting for < 0.1% of childhood cancers. We report here the response and toxicity profile in a case of malignant paraganglioma which was treated with what is currently perceived as an unconventional and non-standard approach, using three consecutive low doses of 131I-MIBG (a cumulative dose of 11 647.6 MBq). The patient had a stable disease at the end of 42 months follow-up following the first treatment with 131I-MIBG. Excellent symptomatic and hormonal responses were observed. The only adverse effect was mild nausea in the first 24 h after therapy. In addition to the potentially primary end point of radiological and biochemical response measurement, we, in this paper, endeavoured to look for a quality of life evaluation for this form of treatment. Given the rarity of this condition, the experience gained by this therapeutic approach is intriguing from response and toxicity standpoints and may be extrapolated to malignant pheochromocytoma as well. An apparent "reverse discordance" between 131I-MIBG scintigraphy and 99Tc(m)-MDP bone scan encountered during pre-therapy work up is also described with possible explanations. This draws attention to an important clinical issue in selecting patients for high dose targeted therapy.

3-Iodobenzylguanidine↗
PubMed1990

Radiation therapy for paragangliomas of the temporal bone.

Treatment of paragangliomas of the temporal bone (glomus jugulare and glomus tympanicum tumors) is controversial, with both surgery and radiation therapy having their advocates. This paper discusses the experience at the University of Arizona Health Sciences Center in treating 10 cases of this uncommon tumor between 1971 and 1988. Seven of 10 cases were initially treated using irradiation and achieved complete tumor control for a mean of 67 months (range = 23-107 months). Two patients, one treated surgically and the other by embolization, had recurrences and were salvaged by radiation, and neither has recurred. The final patient is disease-free 9 months after embolization and surgery. There have been no serious sequelae of treatment. We conclude that moderate-dose irradiation can safely control most temporal bone paragangliomas.

Female↗
PubMed1986

Paraganglioma of the heart. The value of magnetic resonance imaging in the preoperative evaluation.

Although the 131I-metaiodobenzylguanidine scan has proven reliable in identifying mediastinal paragangliomas, further localization has usually required dynamic computerized tomographic scanning which requires rapid bolus injection of contrast material. In the case presented herein, magnetic resonance imaging provided accurate preoperative localization and added important anatomic detail that was not appreciated with dynamic computerized tomograms or with other studies. Magnetic resonance imaging can accurately localize cardiac paragangliomas without injection of contrast material and may provide more detailed information for better guidance for surgical excision.

3-Iodobenzylguanidine↗
PubMed1988

Magnetic resonance imaging of mediastinal paraganglioma.

The magnetic resonance (MR) findings are presented of a nonfunctioning mediastinal paraganglioma on both T1- and T2-weighted magnetic resonance images. The MR characteristics of this tumor differentiate it from previously reported functioning mediastinal paragangliomas (pheochromocytoma) and from other benign mediastinal neoplasms, although mediastinal bronchogenic carcinoma may produce a similar appearance.

Adult↗
PubMed2006

New developments in the detection of the clinical behavior of pheochromocytomas and paragangliomas.

Pheochromocytomas (PCC) are catecholamine-producing tumors that are, by definition, located in the adrenal medulla. Extra-adrenal catecholamine-producing tumors are called paragangliomas (PGL), which should be distinguished from head and neck paragangliomas, which are of parasympathetic origin. As is true for many (neuro)endocrine tumors, but unlike most other epithelial tumors, histopathological analysis does not allow a distinction to be made between PCC and PGL that will follow a benign course and those that have metastasized or will do so, a condition associated with poor prognosis. Therefore, many studies have been undertaken in the past decade, with the aim of providing a marker or a set of markers that allows clinical behavior in PCC and PGL to be predicted. Despite promising results in some areas, such as histopathological scoring systems, the use of the MIB-1 labeling index, and the analysis of telomerase activity, no single test or combination of tests has thus far yielded sufficiently high sensitivity and specificity to result in widespread acceptance in every day clinical practice. The relative rarity of PCC and PGL combined with a frequency of malignancy from as low as 2% up to 25% has hampered the power of past research and can only be overcome by multicenter collaborative efforts. In this article, recent attempts at marker detection, such as those mentioned above, as well as emerging knowledge on the molecular abnormalities in benign and malignant PCC and PGL will be presented.

Adrenal Gland Neoplasms↗
PubMed2006

Recent insights into the molecular pathogenesis of pheochromocytoma and paraganglioma.

Pheochromocytomas and paragangliomas are rare tumors derived from chromaffin cells. These tumors can arise in the context of hereditary cancer syndromes such as von Hippel- Lindau disease, multiple endocrine neoplasia type 2, and neurofibromatosis 1. Recent studies indicate that germ line mutations of genes encoding specific succinate dehydrogenase (SDH) subunits also predispose individuals to pheochromocytomas and paragangliomas. This review focuses on the genetics of these tumors and suggests a possible link between familial pheochromocytomas/paraganglioma genes and control of neuronal apoptosis during embryological development.

Adrenal Gland Neoplasms↗