Feminizing Adrenal Adenoma in a Five Year Old Boy

Leditschke, J. F.; Arden, Felix

doi:10.1111/j.1440-1754.1974.tb02923.x

Cited by 8 publications

(7 citation statements)

References 7 publications

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“…In literature only single cases have been reported in adults[ 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 19 21 22 23 24 25 25 ] and in pediatric population. [ 19 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 ] The largest published series does not exceed two[ 7 25 ] and three cases. [ 10 ] In our department we recorded five cases but only four have been published.…”

Section: Introductionmentioning

confidence: 99%

Feminizing adrenocortical tumors: Literature review

Chentli

Bekkaye

Azzoug

2015

Indian J Endocr Metab

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Feminizing adrenal tumors (FAT) are extremely rare tumors prevailing in males. Clinical manifestations are gynecomastia and/or other hypogonadism features in adults. They are rarer in pediatric population and their main manifestation is peripheral sexual precocity.In women genital bleeding, uterus hypertrophy, high blood pressure and/or abdomen mass may be the only manifestations.On the biological point, estrogen overproduction with or without increase in other adrenal hormones are the main abnormalities. Radiological examination usually shows the tumor, describes its limits and its eventual metastases. Adrenal and endocrine origins are confirmed by biochemical assessments and histology, but that one is unable to distinguish between benign and malignant tumors, except if metastases are already present. Immunostaining using anti-aromatase antibodies is the only tool that distinguishes FAT from other adrenocortical tumors. Abdominal surgery is the best and the first line treatment. For large tumors (≥10 cm), an open access is preferred to coeliosurgery, but for the small ones, or when the surgeon is experienced, endoscopic surgery seems to give excellent results. Surgery can be preceded by adrenolytic agents such as ortho paraprime dichloro diphenyl dichloroethane (Mitotane), ketoconazole or by aromatase inhibitors, but till now there is not any controlled study to compare the benefit of different drugs. New anti-estrogens can be used too, but their results need to be confirmed in malignant tumors resistant to classical chemotherapy and to conventional radiotherapy. Targeted therapy can be used too, as in other adrenocortical tumors, but the results need to be confirmed.

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Section: Introductionmentioning

confidence: 99%

Feminizing adrenocortical tumors: Literature review

Chentli

Bekkaye

Azzoug

2015

Indian J Endocr Metab

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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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“…Less commonly, ACC co-secretes glucocorticoids and androgens. Rarely patients with ACCs present with feminization and features of hyperaldosteronism (10). Our patient's tumour was considerably large (>200cm³) with very high androgen levels by the time of diagnosis.…”

Section: Referencesmentioning

confidence: 62%

A case of childhood adrenocortical carcinoma

Colombage

Muthukuda²,

Bulugahapitiya

et al. 2016

Sri Lanka J. Diabetes Endocrinol. Metab.

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We describe a one year and seven months old baby girl who presented with a short history of pubic hair growth. The child was otherwise healthy and there were no other features of virilization. Her axillary hair and breast development were prepubertal (Tanner 1). Hormonal evaluation revealed markedly raised androgen levels.). There were no features of hypercortisolism or hyperaldosteronism. USS abdomen revealed a left sided adrenal mass measuring 6.8cm x 6cm. A left sided adrenalectomy was performed and the histology revealed features of an adrenocortical carcinoma with areas of necrosis, lymphatic and vascular invasion. Child was treated with adrenalectomy followed by mitotane therapy. This case highlights the importance of suspecting ACC in a young child presenting with pubarche in order to avoid delay in making the diagnosis.

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Feminizing Adrenal Adenoma in a Five Year Old Boy

Cited by 8 publications

References 7 publications

Feminizing adrenocortical tumors: Literature review

Feminizing adrenocortical tumors: Literature review

Adrenocortical tumors in children

A case of childhood adrenocortical carcinoma

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