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PubMed1978

Polypoid nonchromaffin paraganglioma of the duodenum.

A polypoid tumor was surgically removed from the second part of the duodenum of a 56-year-old male. The main body consisted of large epithelioid cells arranged in an adenoma like pattern of strands and nests. These cells were argyrophil and had marked nonspecific esterase activity. Unmyelinated nerves with proliferated Schwann cells accompanied these epithelioid cells together with scattered gangliocyte like elements. Ultramicroscopically, the epithelioid cells were seen to contain round electron dense granules, 150 nm in diameter on average. The tumor is considered to be a nonchromaffin paraganglioma, as it probable developed from paraganglion cells associated with small arteries or branches of the vagus nerve, or from the undifferentiated pluripotent APUD cells of the duodenum.

APUD Cells↗
PubMed1987

Paragangliomas of the craniocervical region. An immunohistochemical study on tyrosine hydroxylase.

An immunohistochemical study on tyrosine hydroxylase (TH), a rate-limiting enzyme in the catecholamine synthesizing pathway, was made on three craniocervical region paragangliomas, two of which showed metastases to the cervical lymph nodes. In all of the original tumors, the majority of tumor cells showed positive immunostaining for TH of variable intensity in their cytoplasm regardless of their cytological features such as cellular and nuclear pleomorphism. The finding suggests that most tumor cells are capable of production of catecholamines and are derived from chief cells in the normal paraganglia. In cervical lymph nodes, however, no positive immunostaining for TH was observed in metastatic tumor cells, in contrast with the findings in the original tumors. The absence of TH immunoreactivity in metastatic tumor cells appears to be noteworthy in considering their malignant potential. Application of the TH immunohistochemistry to further cases appears important for the better understanding of this neoplasm, a catecholamine-producing tumor.

Adult↗
PubMed1979

Paraganglioma in the cauda equina region.

Some new ultrastructural observations are documented in paraganglioma in the cauda equina region. These include the presence of nerve fibers in the tumor capsule, clear vesicles and tubulo-vesicular structures in some cytoplasmic processes in the tumor, fenestrated endothelial cells, and numerous tubular bodies in some endothelial cells.

Adult↗
PubMed1989

Immunocytochemical demonstration of IGF-II-like immunoreactivity in human paraganglioma of the craniocervical region.

Insulin-like-growth factor (IGF)-II-like immunoreactivity was examined in two carotid bodies and six extra-adrenal paragangliomas with use of monoclonal antibody against rat IGF-II, which crossreacts with human IGF-II. Chief cells but not sustentacular cells of the carotid body were positive at about 10% in one case and less than 1% in another case. Among four carotid body tumours, a possible vagal body tumour and one glomus jugulare tumour, all but the glomus jugulare tumour exhibited positive tumour cells irrespective of histological variations. The frequency of positive cells ranged from 20 to 60%. IGF-II like immunoreactivity, therefore, might be widely distributed in human extra-adrenal paraganglionic tissues and tumours, although its biological role in these cells remains to be elucidated.

Adult↗
PubMed1990

Non-functional malignant paraganglioma of the stomach.

We report the second case of a malignant paraganglioma of the stomach in a 56-year-old female patient. However, our case is the first investigated by immunohistochemistry and electron-microscopy. The tumour was characterized immunohistochemically by the presence of neurofilament protein, glial fibrillary acidic protein, S-100 protein, neuron-specific enolase, chromogranin A, ACTH, leu-enkephalin and vasoactive intestinal polypeptide, and ultrastructurally by demonstration of neurosecretory granules and paranuclear intermediate filament whorls. Despite massive metastatic spread in the abdominal cavity, the patient is still alive 4 years after initial diagnosis.

Female↗
PubMed1991

Immunohistochemical characterization of HLA-DR-antigen positive dendritic cells in phaeochromocytomas and paragangliomas as a prognostic marker.

Twelve cases of phaeochromocytoma (PCC) and four cases of paraganglioma (PGG) were studied by immunohistochemistry and immunoelectron microscopy in order to demonstrate HLA-DR (Ia)-antigen-positive dendritic cells (IaDCs). Dense infiltration of IaDCs was detected in the majority of PCCs revealing high urinary or serum catecholamine levels, but in aggressively growing PCCs, a familial PCC and all PGGs, few IaDCs were demonstrated. Interestingly, these IaDCs were negative for S-100 protein. Although S-100-protein-positive sustentacular-like cells (SCs), morphologically similar to IaDCs, were also present, these were clearly distinguished from IaDCs by our double immunostaining method. Ultrastructurally, IaDCs had smooth or slightly indented nuclei and contained a moderate amount of endoplasmic reticulum, small mitochondria and vacuoles, extending elongated cytoplasmic processes. These results suggest that determination of the quantity of IaDCs is a highly effective method of assessing the character of PCCs, in particular, their prognosis.

Adrenal Gland Neoplasms↗
PubMed1992

Malignant paraganglioma of the uterus.

We report a malignant uterine paraganglioma in a 40-year-old female, who died 7 months after the initial diagnosis. On light microscopy the tumour showed a typical zellballen pattern as well as a pronounced cellular pleomorphism. In many tumour cells hyaline globules were demonstrated within the cytoplasm. Immunohistochemically the lesion was characterized by the presence of neuron-specific enolase, protein gene product 9.5 and synaptophysin, and electron microscopically by the occurrence of neurosecretory granules.

Adult↗
PubMed1989

Duodenal gangliocytic paraganglioma. Report of a case and review of the literature.

A case of gangliocytic paraganglioma (GP) of the ampulla of Vater is reported and the literature reviewed, with special attention to immunohistochemical studies. The present case, which occurred in a 56-year-old woman, shows the typical histological admixture of epithelioid, ganglion and spindle cells. Immunohistochemistry reveals strong reactivity for synaptophysin, Leu-7, somatostatin, S-100 protein and vimentin. A few ganglion cells are reactive for neurofilaments. Chromogranin A, myelin basic protein, desmin and cytokeratin are absent. Immunohistochemical data from literature regarding the cytoskeletal composition of GPs are not unequivocal: cytokeratin and neurofilament positivity is reported by some authors and denied by others. More uniformity is reported concerning the peptides produced by GPs: somatostatin and pancreatic polypeptide are the most frequently found antigens, followed by serotonin. General neuroendocrine markers like neuron specific enolase and protein gene product 9.5 are always positive, whereas chromogranins are rarely found. S-100 protein is always positive in the spindle cell component. Our data are in keeping with those previously reported and add the diffuse positivity for the Leu-7 antigen and the positivity of ganglion cells for synaptophysin. The nature of the tumour is still a matter of debate and it is difficult to agree with either of the proposed hypotheses--hamartoma/choristoma versus true neoplasm. However the recent reports of the occasional malignant evolution of GPs may support their true neoplastic nature.

Antigens, Differentiation↗
PubMed1991

Phaeochromocytoma and functioning paraganglioma in childhood and adolescence: role of iodine 131 metaiodobenzylguanidine.

Phaeochromocytomas and functioning paragangliomas are rare tumours in childhood and adolescence. We review our experience of 43 cases (24 men, 19 women) who were first diagnosed at the age of less than or equal to 18 years. All patients were evaluated at some point in their illness with iodine 131 metaiodobenzylguanidine (131I-mIBG) scintigraphy. Eight patients (19%) had bilateral adrenal tumours, 12 (28%) had solitary extra-adrenal tumours, and 8 (19%) had multiple tumours. In 10 patients (23%), the tumours were associated with a familial neurocristopathic syndrome. Thirteen of 24 (54%) unifocal tumours which were initially considered to be benign ultimately proved to be multi-focal and/or malignant. The final prevalence of malignancy was 60%--26 patients, of whom only 15 (57%) had obviously malignant tumours at the time of diagnosis. Primary tumour size greater than or equal to 5 cm was more commonly associated with a malignant course in adrenal but not extra-adrenal tumours. No other clinical, biochemical or morphological characteristic was significantly associated with malignancy. Although the high prevalence of malignancy in this series at least partly reflects referral bias, the need for lifelong follow-up of these patients is underscored. 131I-mIBG scintigraphy was positive in 36 patients (84%), with a somewhat lower false-negative rate (12%) than X-ray computed tomography (20%). Eight patients with malignant tumours received therapeutic doses of 131I-mIBG, with partial tumour responses in 3. Thus, 131I-mIBG is an efficacious, non-invasive, localising agent and may be considered as a palliative therapeutic agent when alternatives have failed.

3-Iodobenzylguanidine↗
PubMed1989

Perioperative management for resection of a malignant non-chromaffin paraganglioma of the bladder.

The perioperative management of a 39-year-old patient with a rare, catecholamine producing, non-chromaffin paraganglioma of the bladder is presented. Although the management of this patient was comparable with a patient with a phaeochromocytoma, this case was complicated by marked release of catecholamines, high requirements for vasodilator therapy preoperatively, the presence of a malignant tumour with metastases and an atypical presentation. Preoperatively the diagnosis was a non-metastatic bladder phaeochromocytoma and the blood pressure was controlled with prazosin, nifedipine and propranolol. Anaesthesia was induced with fentanyl, lidocaine, thiopentone and vecuronium and was maintained with nitrous oxide, isoflurane, fentanyl and vecuronium. The patient was haemodynamically stable throughout the operative and postoperative period.

Adult↗
PubMed2004

Primary paraganglioma of the thyroid gland.

We describe the case of a 46-yr-old euthyroid woman, who was submitted to right lobectomy plus isthmusectomy because of a 30 mm large, rapidly growing thyroid nodule. Two cytological examinations of fine needle aspiration biopsy (FNAB) specimens were not diagnostic. Histology showed a neoplasm composed of nests of chief cells, almost completely replacing thyroid parenchyma, infiltrating the capsule and surgical resection margins, and invading perithyroid tissues. Immunohistochemical analysis revealed that the tumor stained positively to chromogranin, synaptophysin, NSE, S-100 protein and tyrosine hydroxylase, whereas no immunoreactivity was detected against cytokeratin, thyroglobulin, TTF-1, calcitonin and CEA. A diagnosis of thyroid paraganglioma (PG) was finally made. No complications developed following operation. Laboratory analysis and imaging study excluded multicentric disease, metastases to neck or extracervical organs, and multiple endocrine neoplasia (MEN). We report this unusual case, underscore its clinical and immunohistochemical features and discuss differential diagnosis.

Biomarkers, Tumor↗
PubMed2001

Functional imaging of malignant paragangliomas and carcinoid tumours.

Complete staging is mandatory for the management and therapy of neuroendocrine tumours. Various radiotracers are available but the best imaging strategy has yet to be defined. In this study we retrospectively compared 123I-MIBG, 111In-[D-Phe1]-DTPA-octreotide and 18F-FDG (PET) imaging in 15 patients with metastatic neuroendocrine tumours (11 carcinoid tumours, 4 paragangliomas). Planar images were acquired 1, 4, 24 and 48 h following the injection of 111In-[D-Phe1]-DTPA-octreotide and 123I-MIBG. Whole-body PET scans were performed 45 min after injection of 18F-FDG. 111In-[D-Phe1]-DTPA-octreotide was positive in 11/15 patients and identified 44 lesions, 18F-FDG PET was positive in 11/15 patients and identified 107 lesions and 123I-MIBG was positive in 8/15 patients and identified 67 lesions. No single scintigraphic technique identified all metastatic sites. In one patient all studies were negative. 18F-FDG PET identified more abnormal sites than the other two modalities. Combination of all three imaging modalities with X-ray CT helps to provide a more comprehensive map of the disease.

3-Iodobenzylguanidine↗
PubMed2000

Successful treatment with paclitaxel of a patient with metastatic extra-adrenal pheochromocytoma (paraganglioma). A case report and review of the literature.

This case report describes the history of a patient with an aggressive course of a metastatic extra-adrenal pheochromocytoma (paraganglioma) who received different combination chemotherapy regimens with no or short-lasting clinical benefit. However, during treatment with single-agent paclitaxel, there was a significant clinical improvement, a partial biochemical response and a minor roentgenologic response, which was sustained for 1 year. In this report we present this case and also review the literature on the chemotherapy used for this rare disease over the past 15 years. To enable the activity of paclitaxel against this neoplasm to be determined, more patients need to be treated.

Adult↗
PubMed2002

Retroperitoneal paraganglioma in a child: resection and vena caval reconstruction.

Retroperitoneal paragangliomas (PG) are tumors of neuroectodermal origin rarely seen in children. A 10-year-old boy presented with back pain and a palpable mass secondary to a large right-sided retrocaval PG inferomedial to the kidney. Preoperative chemotherapy and perioperative embolisation were instigated to reduce tumor vascularity. En-bloc resection of the inferior vena cava below the left renal vein and above the caval bifurcation was achieved. Caval reconstruction was completed using a complex autograft of anterior uninvolved vena cava and right renal vein. At 4-year follow-up, the child remains tumor-free with normal caval flow and patency.

Child↗
PubMed1999

Repositioning the hereditary paraganglioma critical region on chromosome band 11q23.

Hereditary paragangliomas (PGL, glomus tumors, MIM no.168000) are mostly benign, slow-growing tumors of the head and neck region. The gene (or genes) affecting risk to PGL are subject to genomic imprinting: children of affected fathers exhibit an autosomal dominant pattern of disease inheritance, whereas children of affected mothers rarely if ever develop the disease through maternal transmission. We previously confined the disease gene to an approximately 6 Mb critical region on chromosome band 11q23 (PGL1). Based on haplotype analysis of an extended Dutch pedigree, a 2 Mb sub-region between D11S938 and D11S1885 was proposed as the PGL1 critical interval. In this study, we excluded this interval by analysis of two new single tandem repeat polymorphisms (STRP) contained therein. Instead, we predicted a non-overlapping, more proximal 2 Mb critical interval between D11S1647 and D11S897, and evaluated this new region using nine STRP (D11S1986, five new, closely-linked STRP, D11S1347, D11S3178, and D11S1987). Consistent with our prediction, we observed substantial haplotype-sharing within the Dutch pedigree. We also analyzed four new American PGL families. A recombination event detected in one family further defined D11S1347 as the new telomeric border. We observed significant haplotype-sharing within this new interval among three unrelated American PGL families, strongly suggesting that they originated from a common ancestor. Thus, we confined PGL1 to an approximately 1.5 Mb region between D11S1986 and D11S1347, and showed identity-by-descent sharing for a group of American PGL families.

Chromosome Banding↗
PubMed2002

Management of carotid body paragangliomas and review of a 30-year experience.

Carotid body tumors are rare neoplasms and must be considered in the evaluation of all lateral neck masses; early surgical removal has been recommended. In this study, the medical records of 29 patients with 34 carotid body paragangliomas who were treated at our institution between 1971 and 2001 were retrospectively reviewed. An overview is provided of this lesion, including diagnosis, classification, metastatic potential, possible secretory function, operative techniques, and nonsurgical methods of management. Carotid body tumors may be familial and are more often bilateral in these instances; five patients (17%) had bilateral tumors in this series. The criterion for malignancy is demonstrated by metastatic tumor in lymph nodes or distant organs. Three patients (10%) had malignant tumors, one with hepatic metastases. One patient (3%) in our series exhibited abnormal serotonin production. Vascular reconstruction was necessary in eight cases (28%). No stroke occurred, however, two arterial thromboses (7%), five permanent cranial nerve deficits (17%), and one death (3%) from massive pulmonary embolism were seen. Our experience demonstrates that early operative management is warranted to avoid the possibility of eventual metastasis and progressive local invasion to the point of inoperability.

Adult↗