• Advances in pediatric surgery
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• 제13권2호
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• pp.162-168
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• 2007

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.

• 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.

• Journal of Yeungnam Medical Science
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• 제36권1호
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• pp.54-58
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• 2019

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.

• 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.

• 대한암한의학회지
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• 제23권2호
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• pp.35-41
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• 2018

Objective: The purpose of this study is to report a metastatic adrenocortical carcinoma patient treated with wheel balanced cancer therapy (WBCT) regimen. Methods: A 52-year-old female patient diagnosed and incised with metastatic adrenocortical carcinoma visited the East West Cancer Center (EWCC) on Sep. 1st, 2018 for WBCT. The patient was treated with WBCT for an approximately 2 months, from Sep. 1st to Nov. 9th. Computed tomography (CT) was used to follow-up the tumor site. Laboratory analysis and National Cancer Institute Common Terminology Criteria for Adverse Events (NCI-CTCAE), version 5.0 were used to evaluate the safety of WBCT. Results: The surgical beds after surgery (Lt. Laparoscopic adrenalectomy) were maintained without recurrence at follow up chest CT, and related symptoms and quality of life (QOL) were improved during the WBCT. Conclusion: This case study suggests that WBCT may help to improve QOL of adrenocortical carcinoma patient.

• 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.

• Advances in pediatric surgery
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• 제15권2호
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• pp.103-112
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• 2009

Catheter related and perianal problems are common surgical complications encountered during the treatment of pediatric malignancies. However acute surgical abdominal emergencies are rare. The aim of this study is to review acute surgical abdominal complications that occur during the treatment of childhood malignancies. Out of a total of 1,222 patients who were newly diagnosed with malignant disease, between January 2003 and May 2008, there were 10 patients who required surgery because of acute abdominal emergencies. Their medical records were reviewed retrospectively. Hematologic malignancies were present in 7 patients (4 leukemia, 2 lymphoma, 1 Langerhans cell histiocytosis) and solid tumors in 3 patients (1 adrenocortical carcinoma, 1 desmoplastic small round cell tumor, 1 rhabdomyosarcoma). Seven patients had intestinal obstruction, two had gastrointestinal perforation and one, typhlitis. Intestinal obstructions were treated with resection of the involved segment with (N=2) or without (N=3) enterostomy. Two patients had enterostomy alone when resection could not be performed. Intestinal perforation was treated with primary repair. Typhlitis of the ascending colon was treated with ileostomy. Right hemicolectomy was necessary the next day because of the rapidly progressing sepsis. Three patients are now alive on chemotherapy and one patient was lost to followed-up. Among six patients who died, five died of their original disease progression and one of uncontrolled sepsis after intestinal perforation. Although rare, acute surgical abdominal complications can occur in childhood malignancies. Rapid and accurate diagnosis and appropriate operation are required for effective treatment of the complications.

• 한국임상수의학회지
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• 제26권1호
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• pp.95-100
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• 2009

A 15-year-old, spayed female maltese dog was presented with polydipsia, polyuria, polyphagia, abdominal distention, alopecia and hyperpigmentation. The complete blood counts were in normal range, and the serum biochemistry revealed elevated level of glucose and globulin. Mild hepatomegaly was seen on radiography of abdomen. Abdominal ultrasonography revealed the uniformly enlarged left adrenal gland measured 2.4 cm in diameter. ACTH stimulation test and LDDST revealed hyperadrenocorticism. HDDST revealed pituitary dependent hyperadrenocorticism. On CT images, isodense mass with contrast enhancing was seen in left adrenal gland. Cytologic result is consistent with benign tumor. Adrenal mass was surgically removed and evaluated. Histopathologic examination revealed adenocortical adenoma.