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PubMedDate not supplied

Primary paraganglioma of the lung. Report of two cases and review of the literature.

Two cases of primary paragangliomas of the lung are presented. The first occurred in a 69-year-old woman and was a grossly and histologically benign tumour. The other case occurred in a 33-year-old woman who had metastases to the peribronchial lymph nodes. This seems to be the second malignant case reported. No evidence of recurrence nor metastatic disease was found at follow-up, 8 months for the first case and 7 years for the second, respectively. The morphological and immunohistochemical findings are discussed and the literature comprising 20 cases is reviewed.

Aged↗
PubMed2000

Mutations in SDHD, a mitochondrial complex II gene, in hereditary paraganglioma.

Hereditary paraganglioma (PGL) is characterized by the development of benign, vascularized tumors in the head and neck. The most common tumor site is the carotid body (CB), a chemoreceptive organ that senses oxygen levels in the blood. Analysis of families carrying the PGL1 gene, described here, revealed germ line mutations in the SDHD gene on chromosome 11q23. SDHD encodes a mitochondrial respiratory chain protein-the small subunit of cytochrome b in succinate-ubiquinone oxidoreductase (cybS). In contrast to expectations based on the inheritance pattern of PGL, the SDHD gene showed no evidence of imprinting. These findings indicate that mitochondria play an important role in the pathogenesis of certain tumors and that cybS plays a role in normal CB physiology.

Alleles↗
PubMed1989

Functional, malignant intrathoracic paraganglioma.

This paper describes a case of functional, malignant branchiomeric paraganglioma, the third such to be reported. The patient presented with malignant hypertension and symptoms suggestive of excessive catecholamine secretion.

Adolescent↗
PubMed1990

Raised dopamine metabolites in a case of malignant paraganglioma.

This paper describes the case of a malignant retroperitoneal paraganglioma with extensive metastases. The patient presented with a supraclavicular mass and an absence of hypertension. Exclusively raised dopamine metabolites were detected which may be a marker of a malignant process and account for the lack of hypertension.

Adult↗
PubMed1989

Malignant primary pulmonary paraganglioma.

The histological, immunocytochemical, and electron microscopic findings in a case of malignant primary pulmonary paraganglioma are reported. The existence of this rare tumour is evidence for the presence of pulmonary chemoreceptors.

Female↗
PubMed1993

Tracheal paraganglioma.

The trachea is an unusual site for paragangliomas, only four cases having been documented previously. A fifth case is presented here, together with immunohistochemical evidence that the tumour is biologically benign.

Female↗
PubMed1979

Radiological evaluation of paragangliomas of the head and neck.

Paragangliomas can be differentiated angiographically from most other tumors in the head and neck by their profuse vascularity; specific classification is based on their position and displacement of adjacent vessels. Subtraction usually aids in diagnosis. In most cases, bilateral carotid angiography should be performed, since the ipsilateral carotid artery may have to be ligated and an asymptomatic contralateral tumor may also be found during surgery. Studies of multiple vessels will often show an additional blood supply. Computed tomography can demonstrate the tumor's vascular nature and position and is valuable in follow-up after radiation therapy.

Aged↗
PubMed2004

Endoscopic ultrasound findings in duodenal gangliocytic paraganglioma.

Gangliocytic paraganglioma is an uncommon benign neurogenic tumour of the digestive tract that is usually located in the descending duodenum. Due to the unusual location, such lesions are frequently overlooked on routine radiological examinations and initial endoscopy. With the recent development of endoscopic ultrasound, lesions in this location can be more easily detected and better viewed. A case of a duodenal gangliocytic paraglioma detected by endoscopic ultrasound is presented; this is possibly only the second such case to be reported in the literature.

Adult↗
PubMed1994

Paraganglioma of the lung--developed after exposure to nuclear radiation by the Tschernobyl atomic reactor accident?

A 36-year-old Russian patient had been exposed to severe nuclear radiation during the Tschernobyl accident and developed Cushing's syndrome in September 1991. After bilateral adrenalectomy a small centrally localized lung tumor in the left segment S6 was diagnosed, and the syndrome was correlated to primary lung cancer. Thoracotomy and resection of the segments S6 revealed a primary ACTH-producing lung tumor of the cell type of a paraganglioma. The patient lost his symptoms after curative tumor resection. The case is discussed under the aspects of tumor etiology, its clinical course and immunohistochemical findings.

Adrenocorticotropic Hormone↗
PubMed1996

Paraganglioma of the urinary bladder.

A paraganglioma of the urinary bladder in a 60-year-old woman presented with irritative voiding symptoms, without hematuria or hypertension. Sonography revealed a well-limited ovoid mass of the posterior wall, and cystoscopy showed that it was covered by normally appearing mucosa. Treatment consisted of transurethral resection, and the patient has been followed for 2 years without recurrence. Histologically the lesion consisted of small nests of spindle cells with clear to acidophilic cytoplasm; mitotic activity was inconspicuous. Immunohistochemical analyses revealed that the tumor cells were strongly positive for neuron-specific enolase and chromogranin A, and negative for cytokeratin, vimentin, neurofilaments, glial fibrillary acid protein and HMB 45. Sustentacular cells at the periphery of neoplastic cell clusters were positive for S-100 protein.

Biomarkers, Tumor↗
PubMedDate not supplied

Pitfalls in fine needle aspiration cytology of extraadrenal paraganglioma. A report of 2 cases.

BACKGROUND: Fine needle aspiration (FNA) is commonly used as an initial diagnostic tool in the evaluation of mass lesions. However, extraadrenal paragangliomas (EAPs) are not commonly seen on FNA and, when encountered, can pose a diagnostic challenge since they have a wide variety of morphologic patterns. CASES: Two intraabdominal EAPs were misdiagnosed as an anaplastic adenocarcinoma of the pancreas with giant cell features and papillary adenocarcinoma of the pancreas. The sources of the pitfalls were inaccurate radiologic localization, misinterpretation of vascular-rich tissue fragments as papillary structures, presence of acinarmicroglandular structures, marked cellular pleomorphism (including multinucleation), prominent nucleoli and some cells with "squamoid" cytoplasm. CONCLUSION: The cytologic features of EAP, although suggestive, are not specific. A high index of suspicion and knowledge of clinical information, exact anatomic location and cytologic morphology combined with appropriate ancillary studies are the key to an accurate diagnosis.

Aged↗
PubMed1996

Confinement of PGL, an imprinted gene causing hereditary paragangliomas, to a 2-cM interval on 11q22-q23 and exclusion of DRD2 and NCAM as candidate genes.

Paragangliomas of the head and neck region, also known as glomus tumours, are mostly benign tumours of neuro-ectodermal origin. We mapped the familial form by linkage analysis in 6 families to chromosome region 11q22-q23, between the markers STMY and CD3D which currently span a 16-cM interval. Here, we performed detailed haplotype analysis of this region in a single Dutch multibranch 7-generation family. A region of 2 cM between the markers D11S938/D11S4122 and D11S1885 was shared between all patients of whom disease haplotypes could be reconstructed. In support of this localization, a recombination observed in a small French family with 2 affected nieces places the PGL gene proximal to marker D11S908, genetically coincident with D11S1885.

Chromosome Mapping↗
PubMed1986

Pain-inducing laryngeal paraganglioma: report of the ninth case and review of the literature.

There are two distinct forms of laryngeal paraganglioma. The patient with the rare Type II has exquisite throat pain that is often clinically confusing. The small size or submucosal location of the lesion often results in a delay in correct diagnosis and treatment. The lesion is very aggressive, even if tiny, generally with widespread metastasis. This tumor should be considered in the differential diagnosis of severe persistent throat pain, especially in the absence of abnormalities on physical or radiologic examination.

Humans↗