Conversion of virilization to feminization in a young girl with adrenal cortical carcinoma

Halmi, Katherine A.; Lascari, André D.

doi:10.1002/1097-0142(197104)27:4<931::aid-cncr2820270426>3.0.co;2-j

Cited by 37 publications

(15 citation statements)

References 17 publications

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“…The high levels of this androgen are probably due as much to the peripheral transformation of adrenal androgens as to direct production by the tumour (Saez et al, 1967;Saez et al, 1971)• Furthermore, it is necessary to emphasize that in literature there are numerous cases of tumour of the adrenals characterized by feminilization (Halmi & Lascari, 1971;Comité et al, 1984 ) •…”

Section: Discussionmentioning

confidence: 99%

Adrenocortical tumours in children: our experience with nine cases

Cacciari¹,

Cicognani²,

Pirazzoli³

et al. 1986

Acta Endocrinologica

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Nine cases of adrenocortical tumor are presented, six were males. Four were less than three years and five were between 5 and 10 years of age. Clinical virilization was found in 8 children, one had only signs of hypercortisolism and another showed signs of virilization and hypercortisolism simultaneously. Urinary 17-KS and 17-OHCS were high in all patients. Plasma levels of testosterone and of the other adrenal androgens were high in all the cases tested. Plasma level of cortisol was elevated only in few cases. In two out of five cases steroids were only partially suppressed by dexamethasone. Computed tomography and abdominal sonography have been useful tods for the localization of the tumour. The resection of the tumour, indipendent of hystopathological diagnosis, led to a complete normalization of the clinical and hormonal picture in eight cases evaluated at a distance of 2 months 10 years after surgery. In one case a hepatic metastasis was observed and removed three years after surgery. Adrenocortical tumours are rare (Javadpour et al., 1980) and the vast majority of them are hormone secreting. Although cases have been described which showed feminilization and hyperaldosteronism (Bacon & Lowrey, 1965; Bhettay & Bormici, 1977; Crane et al., 1961; Gauguly et al., 1980), virilization and hypercortisolism are most commonly found (Hayles et al., 1966). This paper illustrates our experience with nine cases of adrenocortical tumour, eight of whom showed inappropriate virilization with or without hypercortisolism and one of whom was a classic case of Cushing's syndrome.

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Section: Discussionmentioning

confidence: 99%

Adrenocortical tumours in children: our experience with nine cases

Cacciari¹,

Cicognani²,

Pirazzoli³

et al. 1986

Acta Endocrinologica

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“…Like Cushings syndrome, the signs and symptoms of Conns syndrome can be obscured by other manifestations. Feminization was also a rare form of presentation accounting for 2.2% of the cases (31)(32)(33)(34)(35)(36)(37)(38)(39). The most frequent sign was gynecomastia.…”

Section: Clinical Featuresmentioning

confidence: 99%

Adrenocortical tumors in children

Ribeiro

Michalkiewicz

Figueiredo

et al. 2000

Braz J Med Biol Res

111

117

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Childhood adrenocortical tumors (ACT) are rare. In the USA, only about 25 new cases occur each year. In Southern Brazil, however, approximately 10 times that many cases are diagnosed each year. Most cases occur in the contiguous states of São Paulo and Paraná. The cause of this higher rate has not been identified. Familial genetic predisposition to cancer (p53 mutations) and selected genetic syndromes (Beckwith-Wiedemann syndrome) have been associated with childhood ACT in general but not with the Brazilian counterpart. Most of the affected children are young girls with classic endocrine syndromes (virilizing and/or Cushing). Levels of urinary 17-ketosteroids and plasma dehydroepiandrosterone sulfate (DHEA-S), which are abnormal in approximately 90% of the cases, provide the pivotal clue to a diagnosis of ACT. Typical imaging findings of pediatric ACT consist of a large, well-defined suprarenal tumor containing calcifications with a thin capsule and central necrosis or hemorrhage. The pathologic classification of pediatric ACT is troublesome. Even an experienced pathologist can find it difficult to differentiate carcinoma from adenoma. Surgery is the single most important procedure in the successful treatment of ACT. The role of chemotherapy in the management of childhood ACT has not been established although occasional tumors are responsive to mitotane or cisplatin-containing regimens. Because of the heterogeneity and rarity of the disease, prognostic factors have been difficult to establish in pediatric ACT. Patients with incomplete tumor resection or with metastatic disease at diagnosis have a dismal prognosis. In patients with localized and completely resected tumors, the size of the tumor has predictive value. Patients with large tumors have a much higher relapse rate than those with small tumors.

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“…A variety of enzyme defects involving steroid metabolism have been found in virilizing adrenocortical tumours. The synthetic defects have included 30-hydroxysteroid dehydrogenation (Cameron et al, 1970;Saez et al, 1970;Halmi & Lascari, 1971), 110-hydroxylation (Villee et al, 1967;Saez et al, 1970), C17-20 desmolase reaction (Saez et al, 1970) and sulphate conjugation (Cameron et al, 1970). In our patient the enzyme defect, however, seemed to be none of these, but more likely that of 21-hydroxylation.…”

Section: Discussionmentioning

confidence: 70%

Ultrastructural and Steroidogenic Characteristics of an Androgen‐producing Adrenocortical Tumour

Huhtaniemi

Kahri

Pelkonen

et al. 1978

Clinical Endocrinology

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A 16-year-old female patient with an adrenal tumour was studied. Clinically she had progressive hirsutism, showed high urinary 17-oxosteroid excretion with normal plasma cortisol. Plasma C19-steroids, both unconjugated (including testosterone) and sulphate-conjugated, were greatly elevated. On surgical exploration an adrenal tumour was histologically an adenoma. On ultrastructural analysis the cells in all zones of the adjoining adrenal were considered normal. Although the tumour cells had the general appearance of a steroid-secreting cell their structure diverged from the cells of every subzone of the cortex. This was the case particularly with mitochondria and lipid inclusions. The only endogenous unconjugated steroids detected in the adjoining cortex were corticosterone and cortisol while in tumour tissue these were present in lesser amounts. The tumour tissue contained large amounts of C19-steroids, 11beta-hydroxy-androstenedione being quantitatively most significant. On the basis of the steroid profile an impaired defect of 21-hydroxylation in tumour cells leading steroid synthesis from corticosteroidogenesis to the C19 pathway is proposed.

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Conversion of virilization to feminization in a young girl with adrenal cortical carcinoma

Cited by 37 publications

References 17 publications

Adrenocortical tumours in children: our experience with nine cases

Adrenocortical tumours in children: our experience with nine cases

Adrenocortical tumors in children

Ultrastructural and Steroidogenic Characteristics of an Androgen‐producing Adrenocortical Tumour

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