Bilateral adrenocortical hyperplasia in children with Cushing's syndrome

Neville, A. Munro; Symington, T.

doi:10.1002/path.1711070204

Cited by 26 publications

(1 citation statement)

References 69 publications

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“…Lediglich 5-15 % manifestieren sich in den ersten beiden Lebensdezennien (19,33,35,51). Knapp 1/3 von ihnen sind als gutartige Adenome, mindestens 2/3 als Karzinome zu klassifizieren (47). Der Prozentsatz der letztgenannten beträgt in der Gesamtzahl bösartiger Neoplasien bei Kindern allerdings nur 0,2-0,4 (5,65).…”

Section: Introductionunclassified

Das Nebennierenrinden-Karzinom im Kindes- und Jugendalter*

Knöpfle¹,

Födisch²,

M.³

et al. 1986

Klin Padiatr

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The presented paper includes the medical records of two girls with adrenal cortical carcinomas on the one hand and the detailed analysis of a collected series of 150 equal cases in pediatric literature on the other hand. In our patients there were a primarily metastatic, non-functioning tumour and a locally invasive, hormone-secreting neoplasm respectively. The latter one produced a Cushing's syndrome with signs of virilization. In this case surgical removal was followed by postoperative irradiation and adjunctive cytostatic therapy with cyclophosphamide and adriamycin for one year. But tumour recurrence occurred within 6 months. A treatment with the antitumour agents aminogluthetimide and o,p' DDD was transiently effective. However drug-induced side effects necessitated the stop of this regimen after 5 months. The analysis of 150 case reports revealed a peak between 1 and 3 years of age, a striking prevalence of the female sex in all age groups, and a metastasizing rate of about 30% at the time of diagnosis. Regional structures, liver and lung were the main locations of metastatic lesions. The occurrence of second primary neoplasms and the association with a group of other conditions particularly congenital disorders have proved to be a remarkable feature of the disease. In primary adrenal cortical tumours the determination of the neoplasm's biological behaviour by morphologic criteria alone can often be very problematical. This fact is especially stressed.

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Section: Introductionunclassified

Das Nebennierenrinden-Karzinom im Kindes- und Jugendalter*

Knöpfle¹,

Födisch²,

M.³

et al. 1986

Klin Padiatr

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Evidence of 11 beta-hydroxylase deficiency in childhood adrenocortical tumors. The plasma corticosterone/11-deoxycorticosterone ratio as a possible marker for malignancy

Doerr

Sippell

Drop

et al. 1987

Cancer

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In search for a biochemical marker to differentiate between adrenocortical carcinoma (AC) and ade-noma (AA), plasma levels of the following steroids were studied preoperatively and postoperatively: 1 I-deoxycorticosterone (DOC), corticosterone (B), 11-deoxycortisol (S), and cortisol (F). Levels were measured by Sephadex LH-20 chromatography and specific radioimmunoassays. The subjects included eight children ages 2 years, 5 months to 9 years, 10 months. There were three girls and 5 boys with pseudoprecocious puberty due to adrenocortical tumors (histologically, four were AC and four, AA). The preoperative showed that DOC and S levels were elevated in all patients, F levels were elevated in four of eight children when compared with age-matched controls, whereas B was normal. Postoperatively, all levels returned to normal. The ratios of B/DOC and F/S as an index of adrenal I 1 beta-hydroxylase activity were calculated. The preoperative ratios of B/DOC were markedly decreased in all patients with AC compared to controls (7.7,4.1,5.9,1.9 versus 23.5, median), but normal in three of four patients with AA (16.2,29.6, 16.1). The F/S ratios were significantly lower in AC and AA when compared with controls. The data indicate a deficiency in 1 I beta-hydroxylation in cases of adrenocortical tumors. Despite a still limited number of patients, the decreased B/DOC ratios may possibly indicate malig-nancy and could be helpful in distinguishing by biochemical means between benign and malignant adrenocortical tumors.

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Functional Cortical Neoplasms

Zarrinpar¹,

Yeh

2009

Endocrine Neoplasia

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Bilateral adrenocortical hyperplasia in children with Cushing's syndrome

Cited by 26 publications

References 69 publications

Das Nebennierenrinden-Karzinom im Kindes- und Jugendalter*

Das Nebennierenrinden-Karzinom im Kindes- und Jugendalter*

Evidence of 11 beta-hydroxylase deficiency in childhood adrenocortical tumors. The plasma corticosterone/11-deoxycorticosterone ratio as a possible marker for malignancy

Functional Cortical Neoplasms

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