• Clinical and Experimental Pediatrics
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• 제51권9호
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• pp.1018-1022
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• 2008

선천 부신 과다형성 환자에서 고환 부신 잔류 종양은 흔하게 발생한다. 대개 이 종양은 적절한 corticosteroid 억제 치료로 호전될 수 있다. 저자들은 양측성 고환 부신 잔류 종양을 보인 21-hydroxylase 결핍증 환아에게서 corticosteroid를 투여하였으나 반응하지 않아 고환 적출술을 시행한 사례를 경험하였기에 보고하는 바이다.

• Asian Pacific Journal of Cancer Prevention
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• 제16권12호
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• pp.5031-5036
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• 2015

Background: Adrenal tumors are relatively uncommon, and have different presentations, so we decided to evaluate the clinico-pathological characteristics of benign and malignant tumors in a ten-year period. Materials and Methods: This cross sectional-analytical study was conducted on adrenal resection samples taken during 2004-2014 in three hospitals in Yazd province. Data were analyzed using SPSS software, version 17. Chi-square and Fisher's exact test were used as appropriate Results: A total of 71 patients with adrenal tumors were analyzed, including 32 (45.1%) men and 39 (54.9%) women with an overall mean age $37.7{\pm}19.9$ (range: 6-75 years). Some 50.7% of lesions were benign and 49.3% were malignant. Neuroblastoma was the most malignant lesion (32.3%) followed by adrenocortical carcinoma (8.4%). Among the benign lesions pheochromocytoma was the most common (25.3%) followed by adrenocortical adenoma (12.6%). While 64% of tumors were functional 36% were non functional. Significant correlation was seen between the age of patient and type of tumor (P=0.001). In patients between 14-40 years old no malignant lesions was found, although under 14 years old all of the tumors were malignant. Malignant lesions mostly presented with abdominal pain, abdominal mass and anorexia (57.2%, 45.7% and 45.7%) respectively. Benign lesions mostly presented with paroxysmal hypertension, headache and abdominal pain (61.2%, 47.2% and 44.4%) respectively. Conclusions: Since the trend of adrenal tumors is on the rise based on this and other studies, suspected cases should undergo prompt hormonal and radiological assessment. Early diagnosis and treatment could prevent tumor progression and reduce mortality and morbidity rates.

• Clinical and Experimental Pediatrics
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• 제54권3호
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• pp.137-140
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• 2011

Testicular adrenal rest tumors (TARTs) are considered to be formed from aberrant adrenal tissue that has become hyperplastic because of elevated adrenocorticotropic hormone (ACTH) in male patients with congenital adrenal hyperplasia (CAH). A 6-year-old boy presented with testicular enlargement and pubic hair. He was diagnosed with CAH complicated by precocious puberty. However, he was not followed-up. At the age of 17, he visited the outpatient clinic because of testicular enlargement and short stature. His right and left testicles were $10{\times}6$ cm and $7.5{\times}4.5$ cm, respectively. His height was 155.1 cm (standard deviation score (SDS), -2.90). The diagnosis of CAH due to 21 hydroxylase deficiency was confirmed by mutation analysis of CYP21A2. Histological examination of the testes showed large, polygonal, eosinophilic cells with round nuclei and prominent nucleoli, which were suggestive of TARTs. He was treated with dexamethasone for 3 weeks and tumors regressed. Subsequently, dexamethasone was replaced by prednisolone and $9{\alpha}$-fludrocortisone; thereafter, the reduced testis size has been maintained.

• Nuclear Medicine and Molecular Imaging
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• 제42권sup1호
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• pp.130-133
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• 2008

Adrenal tumors are increasingly detected by widespread use of anatomical imaging such as a, MRI, etc. For these adrenal tumors, differentiation between malignancy and benignancy is very important. In diagnostic assessment of adrenal tumor, $^{18}F-FDG$ PET and PET-CT have been reported to have high diagnostic performance, especially, very excellent performance in evaluation of adrenal metastasis in the oncologic patient. In cases of adrenal incidentalomas, $^{18}F-FDG$ PET or PET-CT is helpful if a or chemical-shift MRI is inconclusive. $^{18}F-FDG$ PET and PET-CT may be applied to the patients with MIBG-negative pheochromocytomas. In summary, $^{18}F-FDG$ PET and PET-CT are expected to be effective diagnostic tools in the management of adrenal tumor.

• Clinical and Experimental Reproductive Medicine
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• 제47권4호
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• pp.319-322
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• 2020

Adrenal rest tumors are a rare extra-adrenal complication of congenital adrenal hyperplasia (CAH) in women although they are more commonly found in the testes of male patients with CAH. An ovarian adrenal rest tumor (OART) may coexist with CAH or imitate its symptoms without CAH. In this case report, we present the case of a woman with OART without CAH, whose main complaint was infertility and who had a baby after successful surgical treatment.

• Advances in pediatric surgery
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• 제5권2호
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• pp.152-158
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• 1999

Adrenal cortical tumors are rare in adults and children. Most are malignant and functional. The principal clinical features are virilization, Cushing's syndrome, hyperaldosteronism and feminization. Recently, we treated a case of virilizing adrenal cortical tumor in a 26 month-old boy. The diagnosis was made by hormone assay, abdominal CT and tissue pathology. Right adrenalectomy was successful performed. Pathologic examination revealed an adrenal cortical adenoma with vascular invasion.

• Advances in pediatric surgery
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• 제6권2호
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• pp.160-165
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• 2000

저자들은 확정되지 않은 악성의 가능성을 가진 부신 피질 방추세포 종양 (adrenal cortical spindle cell neoplasm of indeterminate malignant potential)을 동반한 Beckwith-Wiedemann 증후군을 경험하였기에 문헌고찰과 함께 보고하는 바이다.

• Clinical and Experimental Pediatrics
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• 제50권3호
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• pp.302-305
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• 2007

최근 해상도가 항진된 방사선학적 검사가 널리 사용되면서, 21-수산화효소 결핍증에 의한 선천성 부신 피질 과형성증 환아에서 부신피질종양이 발견되는 경우가 많아지고 있다. 일반적으로 적절한 스테로이드 호르몬 치료를 받은 21-수산화효소 결핍증 소아에서 부신피질 종양의 발생률는 매우 드물다. 저자들은 생후 초기부터 적절한 용량의 스테로이드 치료로 잘 조절되고 있는 염분소실형 21-수산화효소 결핍증을 가진 12세 여아에서 부신 종양이 우연히 발견된 1례를 보고하고자 한다.

• Clinical and Experimental Pediatrics
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• 제60권2호
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• pp.31-37
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• 2017

Steroid 21-hydroxylase deficiency is the most prevalent form of congenital adrenal hyperplasia (CAH), accounting for approximately 95% of cases. With the advent of newborn screening and hormone replacement therapy, most children with CAH survive into adulthood. Adolescents and adults with CAH experience a number of complications, including short stature, obesity, infertility, tumor, osteoporosis, and reduced quality of life. Transition from pediatric to adult care and management of long-term complications are challenging for both patients and health-care providers. Psychosocial issues frequently affect adherence to glucocorticoid treatment. Therefore, the safe transition of adolescents to adult care requires regular follow-up of patients by a multidisciplinary team including pediatric and adult endocrinologists. The major goals for management of adults with 21-hydroxylase deficiency are to minimize the long-term complications of glucocorticoid therapy, reduce hyperandrogenism, prevent adrenal or testicular adrenal rest tumors, maintain fertility, and improve quality of life. Optimized medical or surgical treatment strategies should be developed through coordinated care, both during transition periods and throughout patients' lifetimes. This review will summarize current knowledge on the management of adults with CAH, and suggested appropriate approaches to the transition from pediatric to adult care.

• Advances in pediatric surgery
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• 제1권1호
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• pp.85-94
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• 1995

Precocious puberty is difficult to define because of the marked variation in the age at which puberty begins normally, onset of puberty before 8 years of age in girls and 9 years in boys may be considered precocious. The etiology of precocious puberty in boys is usually idiopathic, but can result from adrenal and testicular tumors. The hepatoblastoma that produces hCG is a very rare functioning tumor known to cause precocious puberty in boys. Recently, author experienced one case of virilizing adrenal cortical adenoma in 22 month-old boy, one case of adrenal cortical carcinoma in 28 month-old boy, and one case of virilizing hCG-producing hepatoblastoma in 7 year-old boy and reviewed literatures.