Tissue culture, electron microscopic and enzyme histochemical investigations on a sympathetic ganglioneuroblastoma

Gullotta, F.; Fliedner, E.; Wüllenweber, R.; Orf, G.

doi:10.1007/bf00684833

Cited by 9 publications

(11 citation statements)

References 22 publications

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“…Also, in unrelated tissue samples prepared during the same period as the tumours, the mitochondrial morphology appeared normal. The morphological features of the CC are similar to those in earlier studies 13, 24. CC were usually oval‐ or polygonal‐shaped with ovoid nuclei and besides the numerous mitochondria, the cytoplasm was relatively devoid of other organelles.…”

Section: Resultssupporting

confidence: 83%

“…Ultrastructural examination of CC revealed unusually high numbers of tightly packed mitochondria with a swollen aspect and loss of inner membranes in both SDHD ‐linked and sporadic tumours. Identical observations were also made in earlier ultrastructural studies on paragangliomas 13, 24. Possibly, the increased number of mitochondria may reflect a compensatory mechanism to maintain sufficient respiratory chain activity and chemoreception after impairment of complex II function.…”

Section: Discussionsupporting

confidence: 65%

“…Possibly, the primary defect in these tumours is in a different part of the putative pathway that results in the development of paragangliomas. This could also explain the reported contradictory results of positive SDH activity in two sporadic tumours 24.…”

Section: Discussionmentioning

confidence: 96%

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SDHD mutations in head and neck paragangliomas result in destabilization of complex II in the mitochondrial respiratory chain with loss of enzymatic activity and abnormal mitochondrial morphology

Dekker

Hogendoorn

Kuipers‐Dijkshoorn

et al. 2003

The Journal of Pathology

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Hereditary head and neck paragangliomas are tumours associated with the autonomic nervous system. Recently, mutations in genes coding for subunits of mitochondrial complex II, succinate-ubiquinone-oxidoreductase (SDHB, SDHC, and SDHD), have been identified in the majority of hereditary tumours and a number of isolated cases. In addition, a fourth locus, PGL2, has been mapped to chromosome 11q13 in an isolated family. In order to characterize phenotypic effects of these mutations, the present study investigated the immunohistochemical expression of the catalytic subunits of complex II (flavoprotein and iron protein), SDH enzyme activity, and mitochondrial morphology in a series of 22 head and neck paragangliomas. These included 11 SDHD-, one SDHB-, two PGL2-linked tumours, and eight sporadic tumours. In the majority of the tumours (approximately 90%), the enzyme-histochemical SDH reaction was negative and immunohistochemistry of catalytic subunits of complex II showed reduced expression of iron protein and enhanced expression of flavoprotein. Ultrastructural examination revealed elevated numbers of tightly packed mitochondria with abnormal morphology in SDHD-linked and sporadic tumours. Immuno-electron microscopy showed localization of the flavoprotein on the remnants of the mitochondrial inner membranes, whereas virtually no signal for the iron protein was detected. These results indicate that the function of mitochondrial complex II is compromised in the majority of head and neck paragangliomas.

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Section: Resultssupporting

confidence: 83%

Section: Discussionsupporting

confidence: 65%

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SDHD mutations in head and neck paragangliomas result in destabilization of complex II in the mitochondrial respiratory chain with loss of enzymatic activity and abnormal mitochondrial morphology

Dekker

Hogendoorn

Kuipers‐Dijkshoorn

et al. 2003

The Journal of Pathology

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“…The interval between the initial symptoms and tumor diagnosis ranged from 1 day 24,80 to 20 years 15 (mean interval 150.80 Ϯ 12.65 weeks); in 56 cases the duration was not mentioned or not specific. 12,17,30,35,40,45,51,60,73,75,77,78,87 The duration of symptoms was longer in patients with uni-or bilateral sciatica (194.6 weeks), whereas the shortest duration of symptoms was observed in cases involving sudden motor deficit 24,77 and urinary retention. 18 Localization.…”

Section: Results Of the Literature Reviewmentioning

confidence: 99%

Paragangliomas of the lumbar region

Gelabert-González¹

2005

Journal of Neurosurgery: Spine

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“…Other sites of paragangliomas include the mediastinum, retroperitoneum, and visceral organs such as the adrenal glands and lungs [ 11. The exceedingly rare intracranial paragangliomas have been found at the petrous ridge [2], pineal region [3], and sella turcica [4]. To our knowledge, 22 cases of intraspinal paragangliomas have been recorded [5-171. A case reported by Gullotta and Helpas [8] was later included by Boker et al in their series of seven cases [ 171. We report herein a paraganglioma combined with glioma in the region of the conus medullaris and cauda equina.…”

Section: Introductionmentioning

confidence: 99%

Combined paraganglioma and glioma of conus medullaris and cauda equina

Shuangshoti¹,

Suwanwela²,

Suwanwela³

1984

Journal of Surgical Oncology

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A mixed paraganglioma and glioma occurred in the conus medullaris and cauda equina of a woman. The tumor was subdural, encapsulated, and vascular. It was first manifested with symptoms and signs of compression of the cauda equina when she was 19 years of age and was excised when she was 24. The tumor was attached to the conus medullaris, the dura mater, and a nerve root of the cauda equina. Striking light and dark cells with an organoid arrangement mingled with a few glial fibrillary acidic protein (GFAP)-positive neuroglia forming a combined paraganglioma and glioma. Twenty-three paragangliomas were reported in the literature including the present case. The sex and age of recorded 19 patients demonstrated ten males and nine females with an average age of 46.3 years. Twenty tumors were subdurally situated at the conus medullaris and cauda equina. Three neoplasms were epidural and thoracic in location.

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Tissue culture, electron microscopic and enzyme histochemical investigations on a sympathetic ganglioneuroblastoma

Cited by 9 publications

References 22 publications

SDHD mutations in head and neck paragangliomas result in destabilization of complex II in the mitochondrial respiratory chain with loss of enzymatic activity and abnormal mitochondrial morphology

SDHD mutations in head and neck paragangliomas result in destabilization of complex II in the mitochondrial respiratory chain with loss of enzymatic activity and abnormal mitochondrial morphology

Paragangliomas of the lumbar region

Combined paraganglioma and glioma of conus medullaris and cauda equina

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