소아 일차성 부신 피질 종양의 임상상

채영준,문석배,정성은,이성철,박귀원,Chai, Young-Joon,Moon, Suk-Bae,Jung, Sung-Eun,Lee, Seong-Cheol,Park, Kwi-Won 대한소아외과학회 2007 소아외과 Vol.13 No.2

Adrenocortical tumors are very rare in children and the clinical course is not clearly understood. The aim of this study is to review the clinical characteristics and courses of pediatric adrenocortical tumors. The medical records of patients who underwent surgery for primary adrenal tumor at the Department of Surgery, Seoul National University hospital, from Jan. 1986 to Feb. 2006 were reviewed. There were 10 adrenocortical tumor patients; 5 had adrenocortical adenoma and 5 adrenocortical carcinoma. All of the adrenocortical adenomas presented as functioning tumors, i.e., Cushing syndrome or virilization. However, only 2 patients had functioning adrenocortical carcinoma. Median size of adenoma was 5 (3.3-6) cm, and carcinoma 12.5 (6.5-13) cm. Adenomas were smaller than 6 cm and carcinomas were larger than 6.5 cm. Surgical resection alone cured all adrenocortical adenoma patients, and they were all alive without recurrence. Three of 5 adrenocortical carcinoma patients died of tumor recurrence despite radical surgery and chemotherapy. There were 2 long-term survivals for adrenocortical carcinoma, one patient survived 10 years without recurrence until he died of newly developed osteosarcoma, and the other patient is alive without recurrence for 20 years. As the prognosis of pediatric adrenocortical carcinoma is poor, peri-operative aggressive chemotherapy is suggested in addition to radical surgery.

The Fine Needle Aspiration Cytology of a Metastatic Pulmonary Adrenocortical Carcinoma Mimicking Primary Large Cell Carcinoma of the Lung

하승연,김나래,정동해,이재익 대한병리학회 2010 Journal of Pathology and Translational Medicine Vol.44 No.5

Adrenocortical carcinoma is a rare neoplasm and it has an invariably lethal prognosis. We report here on the fine needle aspiration cytologic findings of a solitary metastatic pulmonary adrenocortical carcinoma in a 24-year-old woman. The aspirate smears were very cellular and they were composed of a monomorphic population of large polyhedral cells with abundant granular or vacuolated cytoplasm, and the cells were predominantly singly scattered in a necrotic background. Multinucleated pleomorphic tumor cells were also found. Pleomorphic nuclei with thickened nuclear membranes were impinging on the cell membranes. Mitotic activity was occasionally seen. The cytologic findings of pleomorphic cells with microvacuolated cytoplasm and the presence of vague gland-like sheets, as well as the patient’ history of undergoing adrenalectomy for primary adrenocortical carcinoma helped the pathologist reach the diagnosis of metastatic adrenocortical carcinoma. Here, we focus on the cytologic differential points of metastastic pulmonary adrenocortical carcinoma and primary pulmonary carcinoma, especially large cell carcinoma.

부신기능저하증을 동반한 양측 부신피질암 증례 1예

김민주,김정희,김태용,김상완 대한내분비학회 2011 Endocrinology and metabolism Vol.26 No.3

Adrenocortical carcinoma is often functional and presents with signs and symptoms of adrenal steroid hormone excess. Adrenal insufficiency secondary to bilateral adrenocortical carcinoma is a particularly rare complication. We recently encountered a case of bilateral adrenocortical carcinoma complicated by adrenal insufficiency. A 52-year-old male was transferred to this hospital complaining of general weakness and weight loss. A bilateral adrenal mass was detected on abdomen CT. Plasma cortisol and aldosterone failed to rise during the rapid ACTH stimulation test. The CT-guided adrenal biopsy revealed findings consistent with adrenocortical carcinoma. Left hemiparesis was developed and brain metastasis was detected via brain MRI. Despite the application of gamma knife surgery and chemotherapy, the disease progressed and the patient died

Estrogen-secreting adrenocortical carcinoma

You Jeong,Sung Chul Cho,Hee Joon Cho,Ji Soo Song,Joon Seog Kong,Jong Wook Park,Yun Hyi Ku 영남대학교 의과대학 2019 Yeungnam University Journal of Medicine Vol.36 No.1

Adrenocortical carcinoma is a rare type of endocrine malignancy with an annual incidence of approximately 1–2 cases per million. The majority of these tumors secrete cortisol, and a few secrete aldosterone or androgen. Estrogen-secreting adrenocortical carcinomas are extremely rare, irrespective of the secretion status of other adrenocortical hormones. Here, we report the case of a 53-year-old man with a cortisol and estrogen-secreting adrenocortical carcinoma. The patient presented with gynecomastia and abdominal discomfort. Radiological assessment revealed a tumor measuring 21×15.3×12 cm localized to the retroperitoneum. A hormonal evaluation revealed increased levels of estradiol, dehydroepiandrosterone sulfate, and cortisol. The patient underwent a right adrenalectomy, and the pathological examination revealed an adrenocortical carcinoma with a Weiss’ score of 6. After surgery, he was treated with adjuvant radiotherapy. Twenty-one months after treatment, the patient remains alive with no evidence of recurrence.

Estrogen-secreting adrenocortical carcinoma

Jeong, You,Cho, Sung Chul,Cho, Hee Joon,Song, Ji Soo,Kong, Joon Seog,Park, Jong Wook,Ku, Yun Hyi Yeungnam University College of Medicine 2019 Yeungnam University Journal of Medicine Vol.36 No.1

Adrenocortical carcinoma is a rare type of endocrine malignancy with an annual incidence of approximately 1-2 cases per million. The majority of these tumors secrete cortisol, and a few secrete aldosterone or androgen. Estrogen-secreting adrenocortical carcinomas are extremely rare, irrespective of the secretion status of other adrenocortical hormones. Here, we report the case of a 53-year-old man with a cortisol and estrogen-secreting adrenocortical carcinoma. The patient presented with gynecomastia and abdominal discomfort. Radiological assessment revealed a tumor measuring $21{\times}15.3{\times}12cm$ localized to the retroperitoneum. A hormonal evaluation revealed increased levels of estradiol, dehydroepiandrosterone sulfate, and cortisol. The patient underwent a right adrenalectomy, and the pathological examination revealed an adrenocortical carcinoma with a Weiss' score of 6. After surgery, he was treated with adjuvant radiotherapy. Twenty-one months after treatment, the patient remains alive with no evidence of recurrence.

알도스테론 분비를 동반한 부신피질암 1예

이유홍,박태진,김혜진,김대중,이관우,김명욱,김장희,이태희,정윤석 대한내분비학회 2008 Endocrinology and metabolism Vol.23 No.1

Primary aldosteronism is a syndrome characterized by hypokalemic alkalosis and hypertension. Aldosterone- producing adenomas and bilateral adrenal hyperplasia are common causes of this syndrome. An aldosterone- producing adrenocortical carcinoma is a very rare cause of primary aldosteronism. Recently we experienced a case of an aldosterone-producing adrenocortical carcinoma.A 41-year-old female was admitted for evaluation of a retroperitoneal mass. Because of hypokalemia and a history of hypertension, we evaluated the patient for primary aldosteronism. The high ratio of plasma aldosterone to renin activity suggested the possibility of the presence of primary aldosteronism. We performed adrenal vein sampling for differential diagnosis of an aldosterone-producing tumor from a retroperitoneal mass. The adrenal vein sampling showed that the primary aldosteronism was due to an aldosterone-producing tumor from the left adrenal gland. Surgical findings indicated that the retroperitoneal mass originated from the left adrenal gland and the pathological diagnosis for the mass was an adrenocortical carcinoma. In conclusion, the results from the adrenal vein sampling, as well as the surgical and pathological findings demonstrate that this case was an aldosterone-producing adrenocortical carcinoma. (J Kor Endocr Soc 23:56~61, 2008)

남성호르몬 분비 부신피질암

성병주,김성주,김한석,김두용,김택상,류현열 고신대학교의과대학 2007 고신대학교 의과대학 학술지 Vol.22 No.1

Adrenocortical carcinoma ranks among the least common malignant endocrine tumors, accounting for 0.02% of all annual cancers reported in USA. Given the generally advanced stage at diagnosis, the overall 5-year survival remains poor varying between 20 and 45%. Recently we treated a case of testosterone producing adrenocortical carcinoma. Here we report a case of a functioning adrenocortical carcinoma in a woman with a review of the relevant literature.

선천성 부신피질과형성증 환자에서 동반된 부신피질암

조민성,진혜영,최진호,김대연,이정주,유한욱 대한소아내분비학회 2010 Annals of Pediatirc Endocrinology & Metabolism Vol.15 No.3

Adrenocortical carcinoma is a very rare condition in childhood. There are only a few reports about adrenocortical carcinomas associated with congenital adrenal hyperplasia. A two-month-old male baby presented at the local clinic with skin hyperpigmentation, dehydration, hyponatremia, and hyperkalemia. He was diagnosed with congenital adrenal hyperplasia and had been treated with hydrocortisone and 9α-fludrocortisone until 1 year of age. At age 5.8 years, he visited an outpatient clinic because of tall stature and secondary sexual characteristics, and hydrocortisone was administered. At age 16 years he was admitted for treatment of an adrenal tumor, incidentally detected during the evaluation of hematuria. Serum adrenocorticotrophic hormone and cortisol levels were 33.5 pg/mL and 5.2 μg/dL, respectively. 17α-hydroxyprogesterone level was 6.3 ng/mL and dehydroepiandrosterone sulfate level was 31.2 μg/dL. Abdominal ultrasonography and computed tomography revealed a solid mass of 7.7 × 5.6 × 6.2 cm in the left adrenal gland. It was totally removed by surgery, and the histopathology was compatible with adrenocortical carcinoma. 선천성 부신피질과형성증 환자에서 부신 피질 종양이 종종 발견되고 있는데 부신피질암은 매우 드물게 보고되고 있다. 생후 2개월에 피부의 과다 색소침착, 탈수와 저나트륨혈증, 고칼륨혈증으로 선천성 부신피질과형성증 진단을 받고 1세까지 스테로이드를 복용하다가 임의로 중단 후, 5세에 성조숙증이 발현하여 다시 스테로이드로 치료받던 환자가 16세경 혈뇨를 주소로 외래 방문하였다. 혈액 검사상 부신피질자극호르몬 33.5 pg/mL, 코티솔 5.2 μg/dL, 17-알파히드록시프로게스테론(17α-hydroxyprogesterone, 17-OHP) 6.3 ng/ mL, dehydroepiandrosterone sulfate (DHEA-S) 31.2 μg/dL 였고 초음파와 복부 전산화단층촬영상 왼쪽 부신에 7.7 × 5.6 × 6.2 cm의 종괴가 발견되어 수술적 제거를 받았고 조직학적으로 부신 피질 암종으로 진단받은 1례를 보고 한다.

코티졸, 알도스테론을 동시에 분비하는 부신피질암 1예

하지윤,김민경,차윤진,김승규,윤지영,이광원,박준성,조은석,안철우,박종숙 영남대학교 의과대학 2012 Yeungnam University Journal of Medicine Vol.29 No.2

Adrenocortical carcinomas are rare and frequently aggressive tumors that may be functional (hormone-secreting) and may cause Cushing’s syndrome or virilization, or non-functional and manifest as an abdominal mass. This paper reports the case of a 77-year-old woman with cortisol- and aldosterone-secreting adrenal carcinoma. The patient complained of general weakness, a moon face, and weight gain. She also had hypokalemia and hypertension. Her endocrinological data showed excessive aldosterone production and non-suppressible cortisol production in a low-dose dexamethasone suppresion test. Her abdominal CT showed a right adrenal mass. She underwent right adrenalectomy, and her histology revealed the presence of adrenocortical carcinoma. After adrenalectomy, her hypokalemia returned to normal and she is being treated with hydrocortisone.

코르티졸, 안드로겐, 알도스테론을 동시에 분비하는 부신피질암 1예

최재호,소예리,황유철,정인경,안규정,정호연,양승애 대한내분비학회 2011 Endocrinology and metabolism Vol.26 No.3

Primary adrenocortical carcinoma is a rare tumor, and is characterized by a peri-tumor mass effect and hormone excess signs. Adrenocortical carcinoma most commonly secretes cortisol, but tumors that secrete other adrenal hormones (aldosterone, androgen)are rare. Herein, we report the case of a 70-year-old woman with cortisol, androgen, and aldosterone-secreting adrenal carcinoma. The patient complained of generalized weakness, moon face, and central obesity. On laboratory examination, hypokalemia and metabolic alkalosis was detected. On the hormone test, cortisol, DHEA-S, and aldosterone were all increased. Abdominal CT showed a large right adrenal mass. She underwent right adrenalectomy and the histology revealed the presence of an adrenocortical carcinoma. After adrenalectomy, the patient was treated with hydrocortisone and mitotane