Outcome of adrenocortical tumors in children

Hanna, Angela M.; Pham, Tuan H.; Askegard-Giesmann, Johanna R.; Grams, Jayleen; Iqbal, Corey W.; Stavlo, Penny; Moir, Christopher R.

doi:10.1016/j.jpedsurg.2007.12.022

Cited by 48 publications

(41 citation statements)

References 27 publications

(43 reference statements)

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“…37 So far, the best response rate achieved in adult patients has been reported with etoposide, doxorubicin and cisplatin in combination with mitotane. 38 Although the efficacy of chemotherapeutic agents as single or combination therapies in pediatric patients was uncertain, 6,10,39,40 newer results involving 254 cases from the International Pediatric Adrenocortical Tumor Registry suggest a role for chemotherapeutic agents in the treatment of patients with advanced disease. 5 Novel drugs directed against targets altered in adrenocortical carcinomas such as epidermal growth factor receptor (EGFR; ErbB-1; HER1), Insulin-like Growth Factor 1 (IGF-1) Receptor, Vascular endothelial growth factor ( VEGF) are currently under investigation in ongoing randomized international trials in adults.…”

Section: Adrenal Tumors Of the Cortex And Their Treatmentmentioning

confidence: 99%

“…2,4 The female to male ratio varies slightly (1.6-1.9:1). 5,6 Adrenocortical tumors derive from the cortical cells of the adrenals and may produce steroids autonomously. 4,7 In children, unlike in adults, about 90% of all adrenocortical tumors are hormonally active.…”

Section: Introductionmentioning

confidence: 99%

“…2,4 Overall, Cushing's syndrome is the most common initial clinical manifestation of adrenocortical tumors in children leading to the typical combination of distinct clinical findings, namely rapid weight gain associated with growth retardation or growth arrest. 6,9 In children, a significant number of adrenocortical neoplasms presenting with Cushing's syndrome are malignant, while the opposite is true for adults. 8 Treatment of malignant tumors of the adrenal cortex in childhood is still ineffective 10 with reported 5-year event-free survival rates of 35-55%.…”

Section: Introductionmentioning

confidence: 99%

“…8 Treatment of malignant tumors of the adrenal cortex in childhood is still ineffective 10 with reported 5-year event-free survival rates of 35-55%. 2,5,6 This report aims to raise awareness of the broad clinical spectrum of Cushing's syndrome in childhood as well as to discuss challenges in diagnosis and management. In addition, the concept is introduced of in vitro chemosensitivity testing as a tool to design drug treatment for highly malignant tumors such as the adrenocortical carcinoma.…”

Section: Introductionmentioning

confidence: 99%

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Atypical presentation of a hormonally active adrenocortical tumor in an adolescent leading to delayed diagnosis

Hagemann¹,

Calderari²,

Perren³

et al. 2011

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Adrenocortical tumors are rare in children and present with variable signs depending on the type of hormone excess. We herein describe the unusual presentation of a child with adrenocortical tumor and introduce the concept of in vitro chemosensitivity testing. cAsE rEPOrt: A 10.5-year-old girl presented with hypertrichosis/hirsutism and weight loss. the weight loss and behavioral problems, associated with halted puberty and growth, led to the initial diagnosis of anorexia nervosa. However, subsequent weight gain but persisting arrest in growth and puberty and the appearance of central fat distribution prompted further evaluation. rEsULts AND FOLLOW-UP: 24h-urine free cortisol was elevated. Morning plasma ActH was undetectable, while cortisol was elevated and circadian rhythmicity was absent. thus a hormonally active adrenal cortical tumor (Act) was suspected. On magnetic resonance imaging (MrI) a unilateral, encapsulated tumor was found which was subsequently removed surgically. tissue was investigated histologically and for chemosensitivity in primary cell cultures. Although there were some risk factors for malignancy, the tumor was found to be a typical adenoma. Despite this histology, tumor cells survived in culture and were sensitive to cisplatin in combination with gemcitabine or paclitaxel. At surgery, the patient was started on hydrocortisone replacement which was unsuccessfully tapered over 3 months. Full recovery of the hypothalamus-pituitaryadrenal axis occurred only after 3 years. cONcLUsIONs: the diagnosis of a hormonally active adrenocortical tumor is often delayed because of atypical presentation. cortisol replacement following unilateral tumor excision is mandatory and may be required for months or years. Individualized chemosensitivity studies carried out on primary cultures established from the tumor tissue itself may provide a tool in evaluating the effectiveness of chemotherapeutic drugs in the event that the adrenocortical tumor may prove to be carcinoma.

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Section: Adrenal Tumors Of the Cortex And Their Treatmentmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Atypical presentation of a hormonally active adrenocortical tumor in an adolescent leading to delayed diagnosis

Hagemann¹,

Calderari²,

Perren³

et al. 2011

View full text Add to dashboard Cite

show abstract

“…Virilizing adrenal tumors show growth acceleration, deepening of voice, premature pubic and axillary hair development, and clitoral or penile enlargement. Isolated Cushing syndrome, Conn syndrome, and pure feminization occur very rarely in children [6,13,18,24].…”

Section: Introductionmentioning

confidence: 99%

Virilizing adrenocortical carcinoma in a child with Turner syndrome and somatic TP53 gene mutation

Lee

Hong

et al. 2009

Eur J Pediatr

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Virilizing adrenocortical carcinoma and Turner syndrome have opposite clinical manifestations in some aspects. Here, we report on the first case of virilizing adrenocortical carcinoma in a girl with Turner syndrome. A 2 10/12-year-old girl presented pubic hair of Tanner stage III with clitomegaly, deepening of her voice, and tall stature. No other morphologic anomaly was found. Biochemical assessment revealed normal electrolytes with pronounced elevation of adrenal androgens. She was found to have a large mass of the left adrenal gland on abdominal computed tomography scan. She underwent complete resection of the mass, and pathology was consistent with adrenocortical carcinoma. She was tested for TP53 gene mutation, and we found a de novo TP53 gene mutation (Val143Ala) as well as a 45,X karyotype.

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Early and late renal adverse effects after potentially nephrotoxic treatment for childhood cancer

Knijnenburg

Mulder

Meeteren

et al. 2013

Cochrane Database of Systematic Reviews

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Outcome of adrenocortical tumors in children

Cited by 48 publications

References 27 publications

Atypical presentation of a hormonally active adrenocortical tumor in an adolescent leading to delayed diagnosis

Atypical presentation of a hormonally active adrenocortical tumor in an adolescent leading to delayed diagnosis

Virilizing adrenocortical carcinoma in a child with Turner syndrome and somatic TP53 gene mutation

Early and late renal adverse effects after potentially nephrotoxic treatment for childhood cancer

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