Hepatorenal glycogenosis (type I glycogenosis) and carcinoma of the liver

Zangeneh, Fereydoun; Limbeck, George A.; Brown, Barbara I.; Emch, James R.; Arcasoy, Müfit; Goldenberg, Victor E.; Kelley, Vincent C.

doi:10.1016/s0022-3476(69)80010-7

Cited by 64 publications

(18 citation statements)

References 12 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Они могут быть единичными и множе-ственными, нередко перерождаться в злокачественные. Период малигнизации опухоли может занимать до 28 лет [37,38]. В постпубертатный период на первый план выступает гиперурикемия с ее клиническими ослож-нениями.…”

Section: клиническая картинаunclassified

Glycogenosis in Children: Modern Aspects (Part I)

Сурков¹

2012

Vopr. sovr. pediatr.

View full text Add to dashboard Cite

show abstract

Section: клиническая картинаunclassified

Glycogenosis in Children: Modern Aspects (Part I)

Сурков¹

2012

Vopr. sovr. pediatr.

View full text Add to dashboard Cite

show abstract

“…While the tumors are relatively rare in the first decade of life they appear in all patients who pass through adolescence. Histologically both adenomas and carcinomas were diagnosed and in some cases the clinical course of the disease suggested transformation from adenomas into carcinomas (36,43,61,116). The increasing reports on the development of hepatic tumors in patients with inborn hepatic glycogenosis have been explained as a consequence of the supportive therapy which nowadays ensures that more and more infants with Type I gly- cogen storage disease are surviving into childhood and young adulthood (36).…”

Section: Relevance Of the Observations In Rodents To Hepatocarcinogenmentioning

confidence: 99%

Biological markers of preneoplastic foci and neoplastic nodules in rodent liver

et al. 1982

View full text Add to dashboard Cite

Foci of altered hepatocytes are regularly observed early during hepatocarcinogenesis in rodents. The abnormal hepatocytes may show a number of different phenotypes as characterized by various cytomorphological and cytochemical markers. The first appearance and the further development of the abnormal cell populations depend on the dose of the carcinogen given and on the duration of the carcinogenic treatment. According to cytochemical, morphometric and autoradiographic findings in rats receiving low doses (2-10% of the LD 50/kg bw/ day) of hepatocarcinogens for limited periods (iistop" experiments), glycogenotic (clear or acidophilic) hepatocytes indicate the first step of the neoplastic cell transformation which can be detected by these methods at present. The glycogenotic cells undergo a characteristic metamorphosis and give rise to basophilic tumor cells poor in glycogen, but rich in ribosomes. Under extreme experimental conditions, such as a single or repeated application of higher doses of one or several chemical carcinogens a puzzling picture emerges which is "reversible" to a large extent after withdrawal of the respective compounds. This observation points to a phenotypic instability of the cellular changes induced in certain experimental systems. Foci of altered hepatocytes persisting after withdrawal of the carcinogenic compounds are considered preneoplastic lesions. They may transform into neoplastic nodules which are also persistent and share a number of cytomorphological and cytochemical markers with the focal lesions. The persistent nodules progress to hepatocarcinomas after lag periods of weeks or months. However, the foci may also progress to hepatocarcinomas without passing a nodular intermediate stage. The development of both neoplastic nodules and carcinomas from the preneoplastic glycogen storage foci can proceed independent of further administration of carcinogen. The sequence of cellular changes during hepatocarcinogenesis derived from the experimental results in rodents is strongly supported by observations in humans, especially by the increasing reports on the appearance of hepatic tumors in patients who suffer from inborn hepatic glycogenosis.

show abstract

“…patient with type Ia GSD, by Zangeneh et al 6 in 1969, HCA in a 3-year-old patient was reported to have developed into HCC when the patient was 23 years of age. 12 Gordon et al 13 also reported a case of HCC localized in the same area as an HCA lesion identified 5 years previously.…”

mentioning

confidence: 98%

“…[1][2][3] The overall prevalence of adenomas in the patients with type Ia GSD reported so far in the literature may be close to 50%. [1][2][3][4][5][6][7][8] The natural course of HCA occurring in type Ia GSD liver has recently been made clear. The life-threatening factors in this tumor are twofold: malignant transformation and intratumoral bleeding.…”

mentioning

confidence: 99%

“…In such patients, HCCs have been diagnosed either at autopsy or at an advanced stage of malignancy. [4][5][6][7] In one series, in 49 reported cases of patients with type Ia GSD bearing HCA somewhere in the liver, there were at least 9 patients with 9 HCC (18%), the youngest patient being 6 years old at the time of the diagnosis of cancer. 5 However, in another series, no HCC was observed in 43 GSD patients bearing HCA.…”

mentioning

confidence: 99%

See 1 more Smart Citation