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PubMed1993

The management of multiple paraganglioma of the head and neck.

During the years 1984 to 1991, of 32 patients who had one large glomus or carotid body tumour, six were found to have other paragangliomas. Excluded from the above total of 32 are patients whose only lesion was a solitary glomus tympanicum. No two patients had identical problems and no pattern emerged on which patient management in any particular case could be based. The major problems associated with the tumours themselves and their treatment is the risk to hearing and the lower cranial nerves. Individual cases and their treatment are discussed.

Adult↗
PubMed1993

Frozen section diagnosis in a jugulo-tympanic paraganglioma.

The light microscopy and immunohistochemical findings of a jugulo-tympanic paraganglioma occurring in a 29-year-old man are reported. Diagnostic difficulties from frozen sections are stressed and selected literature is briefly commented upon.

Adult↗
PubMed1983

Cauda equina paraganglioma.

Two paragangliomas resected from the cauda equina had similar morphology, both containing cells having dense core secretory granules with no associated sustentacular or supporting cells. The cells were arranged in characteristic lobules that were surrounded by basement membrane and separated by a fibrovascular stroma. This tumor, although rarely found elsewhere in the central nervous system, does occur in the region of the filum terminale, and causes a clinical syndrome typical of tumors in that area.

Adult↗
PubMed2003

Cauda equina paraganglioma: a review.

Although rare, cauda equina paraganglioma (CEP) is definitely established in the table of cauda equina tumours. There are no particular investigations to document its preoperative diagnosis, unless a vasomotor amine syndrome is present to guide the differential diagnostic spectrum. Magnetic resonance imaging is the examination of choice for the presurgical assessment, but there are still overlapping findings, mainly among CEP, ependymoma, schwannoma, and hemangioblastoma. It is also the only means for postoperative follow-up. Computed tomography may fail to reveal all intrathecal lesions. Total excision is the gold standard for lesions with such a benign or at least protracted clinical course, as observed with the CEP. In contrast to well-known oncologic principles in micro, ultrastructural, and immunohistochemical prediction analysis, these tumours behave in an independent fashion. Promising efforts are being conducted in immunohistochemistry and DNA search, but the mainstay of our knowledge regarding its natural history still stems from our sporadic observation of its clinical behaviour.

Cauda Equina↗
PubMed2003

Case report: intra-renal paraganglioma masquerading as a renal cyst.

A case is reported in which a 46-year-old normotensive male patient presented with large right-sided painless abdominal mass. On sonography there was a large cyst in the lower pole of right kidney. Intravenous urography showed distortion of pelvicollecting system and medial shift of upper right ureter. Exploration revealed a cystic mass (18 x 15 cm) located in lower pole of right kidney. It contained brownish fluid and because of suspicion of malignancy radical nephrectomy was carried out. Histological examination showed the cystic mass to be adrenal tissue infiltrated by neoplastic cells. Characteristic organoid nests of malignant cells ("Zellaballen" were present. Cells stained positively for chromogranin, neurone specific enolase (NSE), neurofilament, and synaptophysin. Sustentacular cells showed positive reaction to S-100 protein. No further treatment was offered. Patient remains well at 30 months follow-up. On medline search there is record of three cases of intra-renal paraganglioma in English literature and the present case appears to be the fourth one.

Cysts↗
PubMed2000

Mutations in SDHC cause autosomal dominant paraganglioma, type 3.

Nonchromaffin paragangliomas (PGLs) are usually benign, neural-crest-derived, slow-growing tumours of parasympathetic ganglia. Between 10% and 50% of cases are familial and are transmitted as autosomal dominant traits with incomplete and age-dependent penetrance.

Chromosomes, Human, Pair 1↗
PubMed2001

Treatment of metastatic carcinoid tumours, phaeochromocytoma, paraganglioma and medullary carcinoma of the thyroid with (131)I-meta-iodobenzylguanidine [(131)I-mIBG].

OBJECTIVE: Meta-iodo-benzyl-guanidine labelled with 131-iodine [(131)I-mIBG] has been used extensively for imaging tumours originating from the neural crest but experience with its therapeutic use is limited, particularly for non-catecholamine secreting tumours. In order to assess the therapeutic response and potential adverse effects of the therapeutic administration of (131)I-mIBG, we have reviewed all patients who had received this form of treatment in our department. DESIGN: Retrospective analysis of the case notes of patients with neuroendocrine tumours who received treatment with (131)I-mIBG and were followed-up according to a defined protocol in a given time frame. PATIENTS: Thirty-seven patients (18 with metastatic carcinoid tumours, 8 metastatic phaeochromocytoma, 7 metastatic paraganglioma and 4 metastatic medullary carcinoma of the thyroid) treated with (131)I-mIBG over a 15-year period were included in this analysis. MEASUREMENTS: The symptomatic, hormonal and tumoural responses before and after (131)I-mIBG therapy over a median follow-up duration of 32 months (range 5-180 months) were recorded. Of the 37 patients (22 males; median age 51 years, range 18-81 years), 15 were treated with (131)I-mIBG alone whereas the other 22 received additional therapy. RESULTS: A total of 116 therapeutic (131)I-mIBG doses were administered [mean cumulative dose 592 mCi (21.9 GBq); range 200-1592 mCi (7.4-58.9 GBq)]. None of the patients showed a complete tumour response. However, 82% of patients treated with (131)I-mIBG alone and 84% who received additional therapy showed stable disease over the period of follow-up. Overall survival during the period of the study was 71%. The overall 5-year survival rate was 85% (95% confidence interval, 72-99%) for all patients and 78% (95% confidence interval, 55-100%) for the carcinoid group alone, according to Kaplan-Meier analysis. Symptomatic control was achieved in all the patients treated with (131)I-mIBG alone, and in 72% of those receiving additional therapy. Hormonal control was noted in 50% and 57% of patients, respectively. (131)I-mIBG therapy was safe and well tolerated. Serious side-effects necessitating the termination of (131)I-mIBG therapy were seen in only 2 of our patients. CONCLUSIONS: (131)I-mIBG therapy produces symptomatic and hormonal improvement and moderate tumour regression/stabilization in patients with metastatic neuroendocrine tumours with minimal adverse effects. It may be a valuable alternative or additional therapeutic option to the currently available conventional treatment modalities.

3-Iodobenzylguanidine↗
PubMed2003

Genetic analysis of mitochondrial complex II subunits SDHD, SDHB and SDHC in paraganglioma and phaeochromocytoma susceptibility.

BACKGROUND: Germline mutations in three subunits of mitochondrial complex II (SDHB, SDHC and SDHD) may be associated with susceptibility to phaeochromocytoma (PC) and/or head and neck paraganglioma (HNPGL). METHODS: To further define the role of SDH subunit mutations in these disorders, we analysed a series of 22 probands with PC and evidence of genetic susceptibility (seven with familial PC only, one with familial PC and HNPGL, 10 sporadic cases with multiple PC and four cases of isolated paediatric onset PC) for germline SDHB, SDHC and SDHD mutations. In addition, we analysed 34 cases of HNPGL (30 isolated cases with single tumours, three isolated cases with multiple tumours and one familial case with multiple tumours) for somatic and germline mutations in SDHB, SDHC and SDHD. RESULTS: We identified four germline mutations (three SDHB and one SDHD, three novel) in the 22 PC probands. Combining these results with our previous series, we have detected germline SDHB or SDHD mutations in 2/12 (17%) of familial PC only kindreds, 4/5 (80%) of familial PC and HNPGL cases, 1/10 of sporadic multiple PC cases and 2/4 (50%) of paediatric PCs. No somatic mutations were detected in the HNPGL tumours, but four cases with multiple HNPGL had the common P81L germline SDHD mutation. Intriguingly a silent SNP (c.204C > T) in SDHD was significantly more common in HNPGL cases (6/34) than in controls (1/100, P = 0.0011). Combining our results with those from two other large studies in which both SDHB and SDHD have been analysed, SDHB mutations were most commonly associated with phaeochromocytoma susceptibility and SDHD with the development of HNPGL (P = 0.025). However, germline SDHB and SDHD mutations demonstrate considerable phenotypic variability and genotype-phenotype correlations are complex. CONCLUSION: The significantly lower frequency (P = 0.028) of germline SDH subunit mutations in familial PC only cases compared to those with familial PC and HNPGL suggests that further PC susceptibility gene(s) remain to be identified.

Adolescent↗
PubMed1999

Tumour size: the only predictive factor for 131I MIBG uptake in phaeochromocytoma and paraganglioma.

BACKGROUND: 131I Meta-iodobenzylguanidine (131I MIBG) scintigraphy can detect chromaffin tumours with a high specificity but its sensitivity remains limited. In this study, the influence of clinical features and tumour pathology on the results of 131I MIBG of patients with phaeochromocytomas and paragangliomas was examined. METHODS: The records of 104 patients operated on for chromaffin tumours who had pre-operative 131I MIBG were reviewed. Demographic data, clinical features, biochemical results and pathology of tumours were analysed. The size of the tumour was assessed by the three measured diameters of the specimen and its calculated volume. Univariate relationship between tumours' characteristics and the results of 131I MIBG were examined. RESULTS: Out of 119 tumours, 104 (87%) were detected by 131I MIBG. Mean +/- SD largest diameter and volume of the tumours were 6.0 +/- 2.4 cm (range 0.8-11.5 cm) and 68 +/- 74 cm3 (range 0.2-421 cm3). Results of 131I MIBG were significantly correlated with both the largest diameter of the tumour (P < 0.01) and by its volume (P < 0.001). 131I MIBG was negative in 35.5% of tumours < 20 cm3 and in 2.6% of those < 20 cm3. No other criteria were correlated with positive 131I MIBG, including aetiology, pathology or catecholamine secretion pattern. CONCLUSIONS: The result of 131I MIBG scanning in patients with chromaffin tumours is significantly correlated with the size of the tumour.

3-Iodobenzylguanidine↗
PubMed2003

[Gangliocytic paraganglioma of the ampulla of Vater].

A case of a bleeding duodenal gangliocytic paraganglioma (GPG) of the Papilla Vateri is reported. The 47 year old patient was treated successfully by partial pancreaticoduodenectomy. Histologically the GPG is characterized by three types of cells: epitheloid cells, ganglion-like cells and spindle cells. So far 81 cases are reported in the literature. Most cases of GPGs are benign, metastasis or recurrence is possible. This case showed an infiltrative growth pattern indicating the true neoplastic nature of this tumor.

Ampulla of Vater↗
PubMed1988

[Magnetic resonance tomographic studies of paragangliomas of the glomus caroticum and glomus jugulare using Gd-DTPA].

The value of magnetic resonance tomography in the diagnosis of paragangliomas in the head and neck has been studied and compared with CT and angiography. Magnetic resonance tomography on its own equals the accuracy of CT, but the use of Gd-DTPA improves diagnostic accuracy. In 19 patients with a glomus jugulare tumour, MR tomography with Gd-DTPA accurately diagnosed all tumours larger than 5 mm. In seven patients with a carotid body tumour, it was possible to arrive at an exact differential diagnosis. Sensitivity was better than that of CT or of sonography. Where there is clinical suspicion of a glomus tumour, magnetic resonance tomography with Gd-DTPA should be the first investigation; if there is a positive finding, angiography should be carried out to demonstrate the circle of Willis.

Carotid Body Tumor↗
PubMed1994

Paraganglioma-like (hyalinizing trabecular) adenoma of the thyroid revisited.

The aim of the present study was to explore the association between paraganglioma-like adenomas of the thyroid (PLAT) and chronic lymphocytic thyroiditis (CLT) or Hashimoto's thyroiditis, and to discuss its possible relationship to papillary thyroid carcinoma. Six cases were analyzed by standard histopathological and immunohistochemical techniques. All 6 cases (all females) had clinical and/or histological evidence of CLT. Only one patient had 2 PLATs. The PLATs were devoid of inflammation and sharply demarcated from the surrounding CLT. There is cyto-morphological overlap with papillary thyroid carcinoma (nuclear grooves and pseudo-inclusions). We conclude that PLAT is associated with CLT more frequently than any other thyroid lesion, and feel that this is more than merely a chance association. PLAT shares several cytological features with papillary carcinoma and cases have been seen where they have occurred in the same thyroid. PLAT could therefore represent an unusual variant of papillary carcinoma.

Adenoma↗
PubMedDate not supplied

Malignant hypertension and paraganglioma in a 14-year-old girl.

A 14-year-old girl presented with malignant hypertension. Physical examination and abdominal computerized tomography revealed a paraaortic mass. Urinary catecholamines, serum renin, and aldesterone levels were high. The mass was totally excised and a paraganglioma of the organ of Zuckerkandl was confirmed. The hypertension resolved after surgery.

Adolescent↗
PubMed1989

Gangliocytic paraganglioma.

A study of gangliocytic paragangliomas (GPs) of the gastrointestinal tract from 51 patients showed characteristic microscopic features: epithelioid cells with an endocrine growth pattern, spindle cells, and ganglion cells. Forty-nine tumors were located in the duodenum, 1 in the jejunum, and 1 in the pylorus. Twenty-one patients were female, 28 male, and for two the sex was unknown. The average age at presentation was 54 years (range, 23-83). No patient had a recurrence. No neuroendocrine syndrome was found in any patient or patient's family. Immunohistochemical stains in 33 cases yielded the following (proportion positive): S-100 protein 94%, synaptophysin 94%, neuron-specific enolase 94%, pancreatic polypeptide 88%, somatostatin 75%, chromogranin 72%, neurofilament protein 64%, keratin 52%, leu-enkephalin 48%, serotonin (one case), and gastrin (one case). Antisera usually stained one or two of the three major cell types. Pancreatic-type tissue was identified in or near 28 tumors, including the pyloric and jejunal lesions and two in the distal duodenum. The authors conclude that GP is benign; is not associated with endocrine syndromes; contains autonomic, neural, and endocrine cell types; and is related to pancreatic development.

Adult↗
PubMed1989

Multiple extra-adrenal paragangliomas associated with catecholamine cardiomyopathy.

We describe a patient with multiple extra-adrenal paragangliomas who presented with clinical features of a dilated cardiomyopathy. Hypertension was not a major feature of the illness despite excessive catecholamine secretion by the tumours. Following surgical resection the changes of dilated cardiomyopathy did not regress on echocardiography and the patient died of left ventricular failure.

Abdominal Neoplasms↗
PubMed1986

Sudden death due to a paraganglioma of the organs of Zuckerkandl.

A 20-year-old woman died suddenly in a hospital emergency room after presenting with nausea, vomiting, back pain, and hypertension. At autopsy, an extra-adrenal pheochromocytoma (paraganglioma) of the organs of Zuckerkandl was found, with microscopic focal myocardial necrosis similar to that described in death from adrenal pheochromocytomas. Tumors of the organs of Zuckerkandl are extremely rare; less than 100 such cases have been reported in the world's literature, and only six, including the present case, have presented as a sudden, unexpected death. The symptoms of catecholamine storm may mimic those of acute drug intoxications, leading to misdiagnosis by both clinical physicians and pathologists.

Adult↗
PubMed2004

Pheochromocytoma and functional paraganglioma.

PURPOSE: Pheochromocytomas and paragangliomas are rare tumors of chromaffin cell origin. Their identification is likely increasing owing to the increased use of radiographic images detecting incidental adrenal masses. RECENT FINDINGS: The pathophysiology of hypertension induced by the release of catecholamines and newly discovered peptides has been shown to be more complex than the concept of episodic catecholamine release. SUMMARY: This review looks at the most recent advances in the physiology and molecular basis of these tumors.

Adrenal Gland Neoplasms↗