Adrenocortical carcinoma in children: Clinical aspects and prognosis

Sabbaga, Cesar Cavalli; Avilla, Sylvio Gilberto Andrade; Schulz, Claudio; Garbers, João Carlos; Blucher, Decio

doi:10.1016/0022-3468(93)90341-h

Cited by 81 publications

(47 citation statements)

References 7 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…All patients ≤4 years at diagnosis were alive without evidence of disease after at least a 5-year follow-up, whereas all patients aged 10 years or older died from their disease. The strong association between age and outcome was also reported in other series (3)(4)(5)8). We acknowledge that in the present study disease stage was lower in the younger subgroup which may explain the better outcome, yet perhaps age and stage of disease are directly correlated.…”

Section: Authors ----------------------------------Hormonalsupporting

confidence: 79%

“…however, these features do not seem to be unique to patients under 4 years of age (20). The age-related survival benefit does also seem to be present in patients from the brazilian region where the p53 germline mutation is prevalent (5,21). This could be compatible with extra-tumoral factors beneficial to survival in the youngest patients.…”

Section: Authors ----------------------------------Hormonalmentioning

confidence: 96%

“…Virilization and precocious puberty are the most common symptoms, reported in 80-100% of patients (3)(4)(5)(6).…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Adrenocortical carcinoma in children: First population-based clinicopathological study with long-term follow-up

et al. 2014

View full text Add to dashboard Cite

Abstract. Adrenocortical carcinoma (ACC) is rare in both adult and pediatric populations. Literature suggests significant differences between children and adults in presentation, histological properties and outcome. The aim of this first nationwide study on pediatric ACC was to describe the incidence, presentation, pathological characteristics, treatment and survival in The Netherlands. All ACC patients aged <20 years at diagnosis and registered in the population-based Netherlands Cancer Registry between 1993 and 2010 were included. Clinical data were extracted from medical records. Archival histological slides were collected via the Dutch Pathology Registry (PALGA). We compared our findings to all clinical studies on pediatric ACC that were found on PubMed. Based on the results, 12 patients were identified: 8 females and 4 males. The median age was 4

show abstract

Section: Authors ----------------------------------Hormonalsupporting

confidence: 79%

Section: Authors ----------------------------------Hormonalmentioning

confidence: 96%

See 1 more Smart Citation

Adrenocortical carcinoma in children: First population-based clinicopathological study with long-term follow-up

et al. 2014

View full text Add to dashboard Cite

show abstract

“…Seventy-five percent of the children present in stage I / II, 10% in stage III, and the remaining in stage IV. Surgery is the cornerstone of treatment in stages I -III of adrenocortical carcinoma, open surgery remains the gold standard and laparoscopic surgery is discouraged in view of excess locoregional recurrences [8].…”

Section: /3 Discussionmentioning

confidence: 99%

A Rare Case of Virilizing Adrenocorical Carcinoma in a Child Presented with Peripheral Precocious Puberty

Al-Jumaili¹

2015

EMIJ

View full text Add to dashboard Cite

Objective: To report a rare case of virilizing adrenocortical carcinoma presenting with peripheral precocious puberty.Methods: we present the initial clinical findings, laboratory tests and medical imaging findings with surgical procedure, tumor staging and histopathological study. Post-operative follows up schedule and management. In addition a brief review literature Results: A 8.5 year old boy presented with pubarche and other virilizing features that dated back to one year. Physical examination revealed; height 121 cm, weight 30 kg, Tanner Stages for pubic hair, penile length and testes volume were IV, IV, ІI respectively. Laboratory findings gonadotropins were low, testosterone high so the precocious puberty was gonadotropins independent ( peripheral) either adrenal or testicular, so abdominal ultrasonography performed that showed well defined mass at right suprarenal region (49×48) mm., and abdominal magnetic resonance demonstrated a mass at right adrenal gland region measures (5×5) cm then dynamic computed tomography of adrenal glands revealed: right adrenal mass (5×4x4) cm well defined. Scrotal ultrasonography revealed both testes slightly enlarged in size with normal echogenicity. Based on the above data the interpretation was adrenal androgen-secreting tumor either adenoma or carcinoma. Therefore, the patient underwent right open adrenalectomy: grossly; encapsulated mass, no invasion to surroundings, size (5×4x4). Histopathology study was consistent with adrenocortical carcinoma confirmed by Immunohistochemistry. Postoperatively: glucocorticoid replacement therapy was initiated, and he was scheduled for follow -up examination. Conclusion:This case illustrates the rare occurrence of adrenocortical carcinoma in this age group and to our knowledge is the first case report in Iraq.

show abstract

“…These tumours occur more commonly in girls and usually present before the age of 6 years [6,7,73,74,75]. Virilisation in female children and pseudoprecocious puberty in male children are the commonest clinical features.…”

Section: Adrenocortical Carcinomamentioning

confidence: 99%

Adrenal pathology in childhood: a spectrum of disease

Paterson

2002

Eur Radiol

View full text Add to dashboard Cite

Adrenal lesions in children may present with an asymptomatic adrenal mass lesion, an endocrinopathy, a hypertensive or metabolic crisis or a paraneoplastic syndrome. Some of the underlying disease processes remain localised within the adrenal gland or may develop into disseminated disease. The role of the radiologist is to confirm the adrenal location of a lesion and to document any other foci of disease. Ultrasound, CT, MR imaging and metaiodobenzylguanidine scintigraphy are the most commonly utilised imaging modalities for examining the adrenal glands and their pathologies in children. This paper reviews the spectrum of adrenal disease in childhood and presents the information as a series of radiological appearances: anomalies of adrenal shape, location, number and size, cystic adrenal masses, solid adrenal mass lesions, and adrenal calcifications are all examined. The radiological findings must always be interpreted in conjunction with the patient's age, the clinical history, the findings on physical examination and the available biochemical data.

show abstract

Adrenocortical carcinoma in children: Clinical aspects and prognosis

Cited by 81 publications

References 7 publications

Adrenocortical carcinoma in children: First population-based clinicopathological study with long-term follow-up

Adrenocortical carcinoma in children: First population-based clinicopathological study with long-term follow-up

A Rare Case of Virilizing Adrenocorical Carcinoma in a Child Presented with Peripheral Precocious Puberty

Adrenal pathology in childhood: a spectrum of disease

Contact Info

Product

Resources

About