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PubMed1976

So-called "minute pulmonary chemodectoma": a tumor not related to paragangliomas.

Twenty-six cases of so-called "minute pulmonary chemodectoma" are presented. The patient population showed a marked female preponderance, and there appeared to be an association of the lesion with pulmonary injury from a variety of causes including cardiac failure, chronic bronchitis and emphysema, and thromboemboli. Half the cases had multiple tumors. Microscopically, the tumors consisted of nests of cells in the interstitial tissue near small veins. Argentaffin and argyrophil stains failed to demonstrate cytoplasmic granules in any case. By electron microscopy, the nests were composed of large cells with broadly interdigitating processes connected by many well-formed desmosomes. The cytoplasm was filled with numerous 60-A filaments. The Golgi apparatus was prominent, while other organelles were sparse. No secretory granules were identified. It is concluded that the fine structure and lack of silver-positive granules are inconsistent with the morphology of previously reported paragangliomas, but that there is a resemblance at the light and electron microscopic level to meningeal arachnoed cells and the cells of meningiomas.

Adolescent↗
PubMed1977

Primary pulmonary paraganglioma: report of a case and review of the literature.

A case of primary paraganglioma of the lung in a 63-year-old woman is presented. The tumor arose from a segmental pulmonary artery and had typical epithelioid cell nests in a "Zellballen" pattern surrounded by reticulum. An electron microscopic study revealed cytoplasmic neurosecretory granules. As in eight previously reported cases, in the English literature, the tumor was benign. The tumor has occurred in seven women and two men with an age range of 43 to 69 years. All patients were asymptomatic, and the diagnosis has never been made prior to operation.

Cytoplasmic Granules↗
PubMed1980

Malignant carotid body paraganglioma: light and electron microscopic study of the tumor and its metastases.

A right carotid body paraganglioma (CBP) was removed from a 30-year-old female after finding metastases to cervical lymph nodes. The tumor and its metastases were studied by light and electron microscopy to determine the neoplastic cell type. Light microscopy confirmed the presence of chief cells but was inadequate alone to exclude sustentacular cells. By electron microscopy, only chief cells were found in both the primary and secondary tumors. This is the first report of an ultrastructural study of a metastasis from a malignant CBP. From our observation, we suggest that CBP be defined as a proliferation of chief and sustentacular cells. Electron microscopy is essential to determine the cell types present and thereby help classify the lesion as a tumor or hyperplasia of the carotid body.

Adult↗
PubMed1981

Tracheal paraganglioma: a case report with review of the literature.

Only three cases of tracheal paraganglioma have been reported in the literature. This paper describes an additional case, which showed pharmacological and ultrastructural evidence of hormone secretion. It is suggested that this tumor is derived from true paraganglia located in the trachea and not from misplaced or aberrant paraganglionic tissue. Hemoptysis was the only presenting symptom in two of the four reported cases and significant bleeding occurred during biopsy in the other two cases. The long-term prognosis appears good if complete resection is possible.

Biopsy↗
PubMed1981

Nonfunctional paraganglioma of the larynx: clinical and pathological considerations.

Paraganglioma of the larynx is a rare neoplasm. A review of the 16 previously reported cases in English medical literature is presented along with the details of an additional case. The tumor was analyzed by light and electron microscopy. The anatomy and embryology of the laryngeal paraganglia and controversies in nomenclature are discussed. Hoarseness, pain, dysphagia, and neck mass are the cardinal symptoms of this neoplasm; other clinical characteristics are reviewed. Partial laryngectomy and excision via lateral pharyngotomy and extralaryngeal approaches are the most commonly employed methods of treatment. More aggressive therapy including radical neck dissection, total laryngectomy, and radiation therapy have been used for malignancies.

Aged↗
PubMed1989

Duodenal gangliocytic paraganglioma with lymph node metastasis in a 17-year-old boy.

A case of duodenal gangliocytic paraganglioma (DGP) in a 17-year-old boy is presented. In this case a lymph node in the peripancreatic region was involved by a metastatic tumor. A review of the literature on DGP indicates that this case represents the youngest patient and is the second case of DGP with metastasis. Immunohistochemical staining for neuron-specific enolase (NSE), neurofilament (NF), pancreatic polypeptide, and somatostatin showed positive results for epithelioid and ganglion-like cells, whereas spindle cells showed immunoreactivities for S-100 protein, NSE, and NF. The histogenesis of DGP is discussed.

Adolescent↗
PubMed2000

Paraganglioma of the thyroid: two cases that clarify and expand the clinical spectrum.

BACKGROUND: Paragangliomas (PGs) can, on rare occasions, arise within the thyroid parenchyma presumably from displaced laryngeal paraganglia. On the basis of a limited number of reported cases, thyroid PGs invariably affect women, they are always benign, and they are usually mistaken for some other more common thyroid lesion. METHODS: We describe the histopathologic features, immunohistochemical findings, and clinical characteristics of two thyroid PGs. RESULTS: One tumor was incidentally discovered in a 55-year-old man during evaluation of a carotid bruit. The other tumor aggressively invaded the trachea and esophagus of a 52-year-old woman with a presumed long-standing nodular goiter. In both cases, the initial pathologic evaluation suggested medullary thyroid carcinoma. Both patients are alive without recurrent disease after surgical resection. CONCLUSIONS: These cases emphasize the need to consider PG in the differential diagnosis of neuroendocrine thyroid tumors, even in those tumors involving men or behaving in a locally aggressive fashion. Failure to do so carries grave implications regarding patient prognosis and management.

Chromogranins↗
PubMed2001

Novel mutations and the emergence of a common mutation in the SDHD gene causing familial paraganglioma.

Familial paragangliomas (PGL) are slow-growing, highly vascular, generally benign neoplasms, usually of the head and neck, that arise from neural crest cells. This rare autosomal dominant disorder is highly penetrant and influenced by genomic imprinting through paternal transmission. Timely detection of these tumors may afford the affected individual the opportunity to avoid the potential serious morbidity associated with surgical removal and the mortality that may accompany local and distant metastases. Linkage to two distinct chromosomal loci, 11q13.1 and 11q23, has been previously reported. Recently, germline mutations in SDHD, a mitochondrial complex II gene on chromosome 11q23, have been demonstrated. We evaluated members of seven families with PGL, five previously studied and shown to have linkage to chromosome 11q23. The entire coding region of the SDHD gene was sequenced and yielded four novel mutations and one mutation shared in three of our unrelated families. Novel mutations found included a truncating mutation in exon 2, as well as a missense mutation, a deletion, and an insertion in exon 4. Three of our families had a common mutation in exon 3 (P81L) that has been reported and thought to be a founder mutation. A restriction enzyme assay was developed for initial screening of this mutation. Molecular analysis is now available and recommended for presymptomatic diagnosis in those at-risk individuals and for confirmatory diagnosis in those having PGL.

Chromosomes, Human, Pair 11↗
PubMed2004

Genomic imprinting and environment in hereditary paraganglioma.

Hereditary paraganglioma (PGL) is characterized by the development of slow-growing and vascularized tumors in the paraganglionic system. PGL is caused by germ line heterozygous inactivating mutations in the SDHB (PGL4), SDHC (PGL3), or SDHD (PGL1) genes, which encode three of the four subunits of mitochondrial complex II (succinate dehydrogenase; SDH). Common tumor sites include the carotid body in the neck and paraganglia in the abdomen. The risk of tumor development associated with SDHD mutations is determined by the sex of the transmitting parent, because only a paternal transmission leads to tumorigenesis in the progeny. This transmission pattern suggests operation of genomic imprinting on the SDHD gene. There is also evidence that the risk of tumor development increases at higher altitudes among SDHD mutation carriers. Accordingly, the increased prevalence of SDHD mutations in the Netherlands, attributable to multiple founder mutations, has been explained in part by the low altitudes in this country, which presumably reduce gene penetrance and relax the natural selection. Thus, PGL caused by SDHD mutations represents an unusual example of an inherited monogenic tumor syndrome because the risk of tumorigenesis shows an absolute dependence on the sex of the transmitting parent and may be modified by a ubiquitous environmental factor.

Atmospheric Pressure↗
PubMed1989

Carotid body tumours and other cervical paragangliomas: diagnosis and management in 25 patients.

This report of a series of 25 patients with cervical paragangliomas reviews the experience of one surgeon over a period of 13 years. A total of 26 resections were performed with no mortality and no hemiplegia. Two patients required carotid reconstruction. Eight patients had a permanent cranial nerve palsy but in seven this was due to involvement of the nerve by the tumour. Lymph node involvement occurred in two patients. Only one patient has developed recurrent tumour. These tumours are locally malignant and resection offers a chance of cure.

Adolescent↗
PubMed1987

Gangliocytic paraganglioma of the duodenum: report of cytologic, histologic, immunohistochemical, and ultrastructural features of a case.

A case of gangliocytic paraganglioma of the duodenum is reported, including light microscopic, ultrastructural, and immunohistochemical characteristics. The cytologic appearance, as might be seen in fine-needle aspiration smears, indicates a neoplasm with three cellular components (epithelioid, ganglion, and spindle cells) seen histologically by light and electron microscopy.

Duodenal Neoplasms↗
PubMed1986

Malignant retroperitoneal paragangliomas.

The authors reviewed the clinical features of 13 cases of malignant retroperitoneal paragangliomas reported in the English literature and report the 14th case. The reported case represents the first such tumor occurring in the familial form of the disease. Good response to combination chemotherapy in the reported case emphasizes the need to study the role of chemotherapy in the management of this neoplasm.

Adult↗
PubMed2006

Pediatric paraganglioma: an early manifestation of an adult disease secondary to germline mutations.

BACKGROUND: Paraganglioma (PGL) and phaeochromocytoma (PCC) are chemotherapy and radiation-resistant neuroendocrine tumors that arise from sympathetic tissue, and rarely occur in children. PCC may be associated with predisposing (germline) conditions like the multiple endocrine neoplasia type 2 (MEN2; OMIM 164761), von Hippel-Lindau syndrome (VHL; OMIM 193300), and rarely neurofibromatosis type 1 syndrome (NF1; OMIM 162200) and multiple endocrine neoplasia type 1 (MEN1; OMIM 131100). PGL, on the other hand, may be related to predisposing germline conditions like the familial PGL syndrome and the NF1 syndrome. In adult studies, one of the highest predisposing factors for germline mutation among patients presenting apparently sporadic PCC/PGL was their age at presentation. The aim of this study was to determine the rate of germline mutations among the rare patients presenting with sporadic PGL during childhood. PROCEDURE: In this study, we report the genetic analysis for predisposing conditions for the only three PGL cases retrospectively identified at our pediatric institution in the last 20 years. RESULTS: None had NF1 clinical associated lesions. Mutation screening of genes associated to VHL (VHL), MEN (RET), and familial PGL (SDH-B, -C, and -D) showed that all cases had germline deletions in the SDHB gene. We report a novel mutation, c.778 del C. Importantly, several non-symptomatic relatives were found to be carriers, thus ensuring them a clinical follow-up. CONCLUSION: According to our findings, PGL presenting during childhood represents an early manifestation of an adult disease caused by predisposing germline mutations. These results underline the importance of genetic studies in pediatric PGLs.

Adolescent↗
PubMed1987

Planned simple carotid resection in surgery of paraganglioma fixed to the carotid artery.

Of 25 surgically treated cases (1956-1986) of paraganglioma, eleven underwent simple carotid resection. The collateral cerebral crossflow was promoted preoperatively with the use of compressing exercises of the carotid artery and controlled by rheoencephalography. There was no operative mortality. One died with a malignant lesion, and nine survived 3 months to 21 years without cerebrovascular complications. It appears that this procedure is worthwhile in selected cases.

Acupuncture Therapy↗
PubMed1993

Analysis of a second family with hereditary non-chromaffin paragangliomas locates the underlying gene at the proximal region of chromosome 11q.

The gene for autosomal, dominantly inherited, non-chromaffin paragangliomas has previously been mapped at 11q23-qter by linkage analysis of a single family. In the present study, we have used genetic markers from 11q for the analysis of two distantly related pedigrees with the same disorder. Linkage analysis and haplotyping indicate that the gene underlying the disorder in the present family is located on chromosome 11q proximal to the tyrosinase gene locus (11q14-q21). Closely linked markers are the human homologue of the murine INT2 protooncogene and the anonymous DNA marker D11S527. A maximum lod score of 5.4 (theta = 0.0) has been obtained for linkage between the disorder and the chromosomal region defined by these markers. The human INT2 gene can be regarded as a candidate for the disorder on the basis of its expression pattern during embryogenesis in the mouse. However, haplotype analysis indicates that this gene is probably not the predisposing genetic factor in the present family.

Chromosome Mapping↗
PubMed1995

Fine mapping of a putatively imprinted gene for familial non-chromaffin paragangliomas to chromosome 11q13.1: evidence for genetic heterogeneity.

Autosomal, dominantly inherited, non-chromaffin paragangliomas are tumors of the head and neck region occurring with a frequency of 1:30,000. Genomic imprinting probably influences the expression of the disorder, because tumor development is limited to individuals who have inherited the trait from their father. By linkage analysis and haplotyping of a single large family in which the pattern of inheritance is consistent with genomic imprinting, we have mapped the gene to a 5 cM region of chromosome 11q13.1 between D11S956 and PYGM. A maximum lod score of 7.62 at theta = 0.0 was obtained for D11S480. This interval does not overlap with a recently assigned locus for glomus tumors in other families: 11q22.3-q23.3. Furthermore, analysis of a second family showing the imprinting phenomenon resulted in the exclusion of the 5 cM area as the location of the disease gene, whereas an indication for linkage was obtained (Z = +2.65) with markers from the distal locus. These observations argue for the presence of two distinct imprinted genes for glomus tumors on 11q. A model for tumor initiation and progression is presented based on all available information.

Adult↗
PubMed1989

Melanotic paraganglioma of the orbit: a case report.

A paraganglioma of the orbit in a 21-year-old woman is presented, containing oculo-cutaneous melanin in many tumor cells, occasionally adjacent to neurosecretory granules, and in macrophages. This tumor expands the list of neuroectodermal tumors with potential melaninization.

Adult↗