Clinical, Hormonal and Pathological Findings in a Comparative Study of Adrenocortical Neoplasms in Childhood and Adulthood

Mendonça, Berenice Bilharinho; Lucon, Antônio Marmo; Menezes, C A V; Saldanha, Luís Balthazar; Latrônico, Ana Claudia; Zerbini, Claudia; Madureira, Guiomar; Domenice, Sorahia; Albergaria, Maria Adelaide P.; Camargo, Marcia H. A.; Halpern, Alfredo; Liberman, Bernardo; Arnhold, Ivo Jorge Prado; Bloise, W; Andriolo, Adagmar; Nicolau, Wilian; Silva, Frederico A.Q.; Wroclaski, Eric; Arap, Sami; Wajchenberg, B. L.

doi:10.1097/00005392-199512000-00006

Cited by 15 publications

(34 citation statements)

References 11 publications

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“…In all series of ACT, female patients clearly predominate and males account for 10-35% of the reported cases (30,33,34). In our series, five men with androgen-secreting tumors accounted for 10% of the ACT-AS cases (data not shown).…”

Section: Act-assupporting

confidence: 42%

“…The measurement of 17OHP and compound S are of great interest considering that ACT-AS often present with defective steroid biosynthesis enzymes causing elevated levels of steroid precursors (30,31). Baseline 17OHP was indeed high in 67% of the ACT-AS (median 6 ng/ml or 18.2 nmol/l) and 11% of the NTAE (median 2.6 ng/ml or 7.9 nmol/l), and compound S was high in 85% of ACT-AS and 0% of the NTAE.…”

Section: Discussionmentioning

confidence: 99%

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Sex steroids in androgen-secreting adrenocortical tumors: clinical and hormonal features in comparison with non-tumoral causes of androgen excess

d’Alva

Abiven-Lepage

Viallon

et al. 2008

European Journal of Endocrinology

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Objective: Adrenocortical tumors (ACT) account for no more than 0.2% of the causes of androgen excess (AE). Conversely, these rare tumors have a very poor prognosis. It is difficult and important to exclude this diagnosis whenever there is AE. Design: Retrospective investigation of androgen profiles in a large consecutive series of androgensecreting (AS) ACT to assess their relative diagnostic value. Methods: A total of 44 consecutive female patients with ACT-AS and a comparison group of 102 women with non-tumor causes of AE (NTAE). Results: Patients with ACT-AS were older than the ones with NTAE (37.7 vs 24.8 years; P!0.001) and the prevalence of hirsutism, acne, and oligo/amenorrhea were not different. Free testosterone was the most commonly elevated androgen in ACT-AS (94%), followed by androstenedione (90%), DHEAS (82%), and total testosterone (76%), and all three androgens were simultaneously elevated in 56% of the cases. Androgen serum levels became subnormal in all ACT-AS patients after complete tumor removal. In NTAE, the most commonly elevated androgen was androstenedione (93%), while all three androgens were elevated in only 22% of the cases. Free testosterone values above 6.85 pg/ml (23.6 pmol/l) had the best diagnostic value for ACT-AS (sensitivity 82%, confidence interval (CI): 57-96%; specificity 97%, CI: 91-100%). Basal LH and FSH levels were significantly lower in the ACT-AS group. Conclusion: Free testosterone was the most reliable marker of ACT-AS. However, the large overlap of androgen levels between ACT-AS and NTAE groups suggests that additional hormonal and/or imaging investigations are required to rule out ACT-AS in case of increased androgens.European Journal of Endocrinology 159 641-647

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Section: Act-assupporting

confidence: 42%

Section: Discussionmentioning

confidence: 99%

Sex steroids in androgen-secreting adrenocortical tumors: clinical and hormonal features in comparison with non-tumoral causes of androgen excess

d’Alva

Abiven-Lepage

Viallon

et al. 2008

European Journal of Endocrinology

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“…Six of our patients presented with features of virilization, confirming other studies of paediatric populations where virilizing features were predominant 2,7 . Cushingoid features were seen in two patients, one with virilization and one with hemihypertrophy.…”

Section: Discussionsupporting

confidence: 88%

Adrenal cortical tumours: 25 years’ experience at the Royal Children's Hospital, Melbourne

McDonnell

Zacharin

2003

J Paediatrics Child Health

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“…Although high mitotic rate and DNA ploidy index may correlate with poor survival, not a single histopathologic feature has proven to be a reliable predictor of recurrence and metastasis [6-8]. Also, Weiss classification tends to be inaccurate for tumoral behavior prediction in children [9,10]. …”

Section: Introductionmentioning

confidence: 99%

Assessment of the role of transcript for GATA-4 as a marker of unfavorable outcome in human adrenocortical neoplasms

et al. 2004

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Background: Malignant neoplasia of the adrenal cortex is usually associated with very poor prognosis. When adrenocortical neoplasms are diagnosed in the early stages, distinction between carcinoma and adenoma can be very difficult to accomplish, since there is yet no reliable marker to predict tumor recurrence or dissemination. GATA transcription factors play an essential role in the developmental control of cell fate, cell proliferation and differentiation, organ morphogenesis, and tissue-specific gene expression. Normal mouse adrenal cortex expresses GATA-6 while its malignant counterpart only expresses GATA-4. The goal of the present study was to assess whether this reciprocal change in the expression of GATA factors might be relevant for predicting the prognosis of human adrenocortical neoplasms. Since human adrenal cortices express luteinizing hormone (LH/hCG) receptor and the gonadotropins are known to up-regulate GATA-4 in gonadal tumor cell lines, we also studied the expression of LH/hCG receptor.

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Clinical, Hormonal and Pathological Findings in a Comparative Study of Adrenocortical Neoplasms in Childhood and Adulthood

Cited by 15 publications

References 11 publications

Sex steroids in androgen-secreting adrenocortical tumors: clinical and hormonal features in comparison with non-tumoral causes of androgen excess

Sex steroids in androgen-secreting adrenocortical tumors: clinical and hormonal features in comparison with non-tumoral causes of androgen excess

Adrenal cortical tumours: 25 years’ experience at the Royal Children's Hospital, Melbourne

Assessment of the role of transcript for GATA-4 as a marker of unfavorable outcome in human adrenocortical neoplasms

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