• 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.

• 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.

• 대한병리학회지
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• 제52권6호
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• pp.416-419
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• 2018

We describe a case of a 61-year-old Korean man who was diagnosed with renal cell carcinoma that was discovered on abdominopelvic computed tomography obtained after the patient complained of back pain. A radical nephrectomy was performed, and the surgical specimen showed a relatively well-circumscribed and yellowish lobulated hard mass. Microscopically, the tumor showed sheets and nests of hypercellular pleomorphic cells with thick fibrous septation, frequent mitoses, and areas of adrenal cortical-like tissue. Immunohistochemical staining revealed that the tumor cells were positive for inhibin-${\alpha}$, vimentin, synaptophysin, and melan A. It also revealed that the tumor cells were negative for pan-cytokeratin, epithelial membrane antigen, paired box 8, ${\alpha}$-methylacyl-coenzyme A racemase, CD10, cytokeratin 7, carbonic anhydrase 9, c-Kit, renal cell carcinoma, transcription factor E3, human melanoma black 45, desmin, smooth muscle actin, S-100, chromogranin A, CD34, anaplastic lymphoma kinase, and integrase interactor 1. Based on these histopathological and immunohistochemical findings, we diagnosed the tumor as intrarenal adrenocortical carcinoma arising in an adrenal rest. Several cases of intrarenal adrenocortical carcinoma have been reported, although they are very rare. Due to its poor prognosis and common recurrence or metastasis, clinicians and pathologists must be aware of this entity.

• 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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• 제6권2호
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• pp.156-159
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• 2000

Neuroblastoma arises from the embryonic tissue of the adrenergic rest. It is commonly found in children and mostly in nonrenal tissue. We present a case of intrarenal neuroblastoma which was initially thought to be a Wilms' tumor. The patient was a 18 months-old girl treated with radical nephrectomy and adjuvant chemotherapy after operation. The neoplasm within the kidney in children cannot always indicate Wilms' tumor. Neuroblastoma of the adrenal gland or retroperitoneal tissue may often compress or invade the kidney directly or arise from the kidney. Clinical aspects that differentiate between neuroblastoma and Wilms' tumor are discussed with a review of the literatures.

• 한국임상수의학회지
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• 제26권3호
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• pp.268-272
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• 2009

An-11-year-old male Shih-tzu was admitted to emergency care unit of Haemaru Referral Animal Hospital with signs of dyspnea, anuria and depression. There were abnormalities on complete blood count and serum chemistry included leukocytosis with mild left shift, mild azotemia, and increased ALT activity. Fluid therapy(0.9% saline, 40 ml/hr) and antibiotics were immediately initiated. The patient began to vomit after 5 hours' rest and pale mucous membrane, bradycardia, and hypertension were noted. Abdominal ultrasonography revealed enlarged left adrenal gland and thrombus in caudal vena cava(CVC). Result of ACTH stimulation test was normal. Cytology of ultrasound-guided FNA smears showed numerous naked nuclei, which was suggestive of adrenal medullar tumor. Concentrations of 24 hour urine metanephrine and normetanephrine was moderately increased compared to those of a control dog. Adrenal mass was surgically removed, and biopsy of the CVC mass was obtained. After surgery the patient began to recover but the dog acutely developed cardiopulmonary arrest and died. On histopathology the adrenal mass and biopsy of the CVC mass were consistent with pheochromocytoma. On electron microscopic view norepinephrine specific granules were found in the cytoplasm of tumor cells.