A surgical approach to adrenocortical tumors in children: the mainstay of treatment

Background

Adrenocortical tumors (ACTs) are rare in the pediatric population. The pathogenesis, prognostic indicators, and management of these tumors are still unclear because of its infrequent occurrence. This case series presents the surgical experience of the authors’ center over 29 years.

Methods

The medical records of children treated for ACTs between 1974 and 2003 were reviewed. Information on age, sex, presenting symptoms, hormonal levels, pathology, stage, treatment, and outcome was obtained.

Results

Nine children (5 girls, 4 boys) were treated for ACTs. The median age at presentation was 29 months (range, 5 months to 11 years). Endocrine dysfunction was found in 8 patients. Four presented with virilizing symptoms, 4 presented with both virilizing and Cushing’s symptoms, and 1 patient with Beckwith-Wiedemann syndrome was identified during routine screening. One was an adenoma, and 8 were carcinomas. Of the carcinomas, 3 were stage I, and 5 were stage II. The mean tumor weight was 125 g (range, 42 g to 336 g) with a mean volume of 139 cm³ (range, 30 cm³ to 626 cm³). All patients had complete excision of the tumor with spillage occurring in 2 cases. Lymph node biopsies were done in all but 2 patients. Two patients were treated with chemotherapy because of large tumor size and nodal involvement. All patients are doing well including those with tumor spillage.

Conclusions

This study shows that surgical excision continues to be the mainstay of treatment for ACTs. Extensive lymph node biopsy in small ACTs can probably be avoided given the generally good outcome with surgery alone. The role of adjuvant chemotherapy remains unclear because most of the children in our series were effectively treated with surgical resection only. Patients should be enrolled in multicenter trials to assess the added value of chemotherapy.     

Minimally invasive adrenalectomy and malignant adrenal tumours

Summary   Background: Minimally invasive adrenalectomy is the standard procedure for the treatment of small benign adrenal neoplasms. The question of whether this technique is also suitable for treating adrenal malignancies has recently been discussed with controversy. Methods: A literature search was performed to gather published experience and opinions concerning the role of endoscopic adrenalectomy in the treatment of primary adrenal malignancies and adrenal metastases. Results: Adrenocortical carcinomas (ACCs) have been approached laparoscopically, and this technique has even been advocated for large tumours. Several small ACCs have been successfully removed laparoscopically. Oncological catastrophes with peritoneal carcinomatosis have been reported. Reoperation after laparoscopic adrenalectomy for unsuspected ACC was usually not performed. There are no data demonstrating a survival benefit of systematic lymphadenectomy. Adrenal metastases are firm and rarely penetrate the capsule of the adrenal gland. Several reports on endoscopic adrenalectomy for metastases have been published. Conclusions: Laparoscopic adrenalectomy for ACC should be considered with great reluctance. If ACC is diagnosed postoperatively upon histological examination after laparoscopic adrenalectomy for a presumably benign tumour, reoperation is not mandatory provided oncological principles were respected during the primary operation. Adrenal metastases confined to the adrenal gland can be removed laparoscopically.      

Incidence of adrenal involvement and assessing adrenal function in patients with renal cell carcinoma: Is ipsilateral adrenalectomy indispensable during radical nephrectomy?

OBJECTIVE: To determine the value of ipsilateral adrenalectomy with radical nephrectomy, by investigating the clinical aspects of adrenal involvement and adrenocortical function in patients with renal cell carcinoma (RCC). PATIENTS AND METHODS: The demographic, clinical and pathological data of adrenal involvement were reviewed in 247 patients with RCC. To evaluate adrenocortical function, 14 patients (adrenalectomy in eight, adrenal-sparing in six) had a rapid adrenocorticotropic hormone (ACTH) stimulation test before and 2 weeks after surgery. RESULTS: There was adrenal involvement with RCC in seven of the 247 (2.8%) patients (a solitary adrenal metastatic tumour in four and direct extension into the adrenal gland in three). All adrenal involvement was detectable on abdominal computed tomography before surgery, and these patients had a large primary renal tumour of > pT2 and/or distant metastasis. Plasma cortisol levels declined significantly more in response to the rapid ACTH stimulation test in those treated by adrenalectomy than in those with spared adrenal glands at 2 weeks after surgery (P < 0.05), while there was no significant difference between the groups before surgery. CONCLUSIONS: These results suggest that unconditional ipsilateral adrenalectomy with radical nephrectomy for RCC should be avoidable, and thus preserve the reserve of adrenocortical function, as preoperative imaging, especially thin-slice multidetector helical computed tomography, can detect adrenal involvement with RCC in most cases. Unilateral adrenalectomy might cause an irreversible impairment of the reserve of adrenocortical function.     

Application of intravascular ultrasonography for intracaval tumor thrombectomies in adrenal metastasis from hepatocellular carcinoma: Report of two cases

(Received for publication on Aug. 3, 1998; accepted on May 27, 1999).     

Primary adrenocortical tumors in autopsy records —A survey of “cumulative reports in Japan” from 1973 to 1984—

All cases of primary adrenocortical tumors, including those found incidentally, recorded in the Pathological Autopsy Case Annuals of Japan during a 12 year period from 1973 to 1984 were surveyed. Age- and sex-specific incidences of lesions were estimated from the population, mortality and autopsy statistics for the same period. There were 101 adenomas, 77 of which were presumed to be non-functional, and 222 carcinomas. The age-specific distribution profiles of both the benign and malignant tumors showed single peaks in the sixties and could not be distinguished from one another. The estimated age-specific distribution of morbidity of the non-functional adenomas showed a peak at about 10 years younger than that found in the autopsy records, although it was again similar to that of carcinoma derived from various clinical reports. The morbidity of the non-functional adenomas per 100,000 population was almost equal to that of breast cancer in Japan, being highest in the fifties age group with more than 4,000 people a year being suggested to have adenoma in this age bracket.     

男性肾上腺生殖综合征的治疗体会(附17例报告)

目的:探讨男性肾上腺生殖综合征的治疗特点。方法:对我院10年收治的17例男性肾上腺生殖综合征患者的临床资料进行回顾性分析,其中先天性肾上腺增生(CAH)15例,肾上腺肿瘤2例;21羟化酶缺陷(21-OHD)与11β羟化酶缺陷(11-βOHD)患者服用皮质激素,17羟化酶缺陷(17-OHD)者除了服用糖皮质激素外,还服用雌激素及接受性腺切除,肾上腺肿瘤患者行手术切除肿瘤。结果:坚持服药的5例21-OHD患者的性早熟症状与实验室检查恢复正常,早期治疗的2例身高改善明显,1例患者的睾丸肾上腺残余肿块服药后明显缩小,3例精液检查显示有精子,但是数量与质量较差,1例停药的患者随诊时发现左睾丸有结节,恢复药物后结节质地变软。1例11β-OHD患者药物治疗后性早熟体征与血压恢复正常,6例17-OHD患者药物治疗与睾丸切除后的女性体征维持较好。肾上腺肿瘤患者随诊未见复发。结论:补充激素治疗可以改善CAH患者的身高与睾丸功能,肾上腺肿瘤需行手术切除肿瘤。     

Nonfunctioning adrenocortical carcinoma in a young girl

A 9 year old Japanese girl was admitted complaining of left hypochondrial pain and a large upper left abdominal tumor. There were no clinical or laboratory signs of hormonal abnormality. Intravenous pyelography showed marked compression and deformity of the kidney by a tumor. This tumor was excised together with the left kidney. The pathological diagnosis was adrenocortical carcinoma. Postoperatively, the child was given neither irradiation nor chemotherapy. Twenty-one months after the surgery, there was a hepatic metastasis, and she died 40 months after surgery from a combination of hepatic metastases and local tumor recurrence.     

成人肾上腺皮质癌的诊断和治疗（附25例报告）

目的：探讨成人肾上腺皮质癌（ACC）的诊断及治疗方法。方法：对1986年～2012年期间我科收治的25例成人肾上腺皮质癌患者的临床资料进行回顾性分析,结合文献就其临床表现、影像学特征、诊断及鉴别诊断、治疗等问题进行讨论。本组25例术前均行B超、IVP、CT、MRI等影像检查。结果：14例行手术治疗并经术后病理证实。术后平均生存10．2个月,3年生存率为14．3％（2／14）,5年生存率为0。结论：肾上腺皮质癌恶性程度高、浸润性强,早期诊断和治疗是改善预后的关键,手术根治性切除仍是目前治疗的最佳方法。     

RECURRENT ADRENOCORTICAL CARCINOMA IN A 4 YEAR OLD GIRL

Adrenocortical tumours are rare in the paediatric age group. This is a report of a 4 year old girl with recurrent adrenocortical carcinoma emphasizing the role of surgery as the definitive form of therapy even in recurrent cases.     

肾上腺皮质癌9例报告

目的：探讨肾上腺皮质癌的诊断和治疗方法。方法：回顾性分析9例肾上腺皮质癌患者的临床资料,其中男7例,女2例。年龄5个月～63岁,平均39岁。术前全部行B超及CT检查,有内分泌异常者2例,分别为Cushing syndrome及原发性醛固酮增多症。无内分泌异常者7例。结果：手术完整切除9例。术后病理检查：I期1例;Ⅱ期4例;Ⅲ期3例;Ⅳ期1例。随访4～60个月,平均生存30个月,1例因肺部转移而术后26个月死亡,1例失去随访,其余7例均存活至今。结论：肾上腺皮质癌恶性程度较高,预后较差,根治性切除是治疗肾上腺皮质癌最有效的方法。化疗及放疗可作为术后辅助治疗。早期诊断和早期治疗可提高患者生存率。     

肾上腺恶性肿瘤的诊治（附34例报告）

目的：探讨肾上腺恶性肿瘤的诊断和治疗方法。方法：回顾性分析34例肾上腺恶性肿瘤的临床资料。其中肾上腺皮质癌13例;恶性嗜铬细胞瘤8例;肾上腺转移癌8例;皮质癌并发同侧肾盂癌1例;节神经母细胞瘤1例;恶性纤维组织瘤1例;脂肪肉瘤1例;肾上腺髓外浆细胞瘤1例。全部34例患者中,有临床症状者23例,有内分泌功能改变者22例。结果：所有患者均接受手术治疗,其中29例肿瘤全切,4例肿瘤小部分或包膜残留,1例仅探查取活检。32例获得随访,时间为2个月-5年。死亡11例,死亡患者生存期为5-29个月,平均21个月。结论：对于肾上腺恶性肿瘤患者。手术切除是首选治疗方法,并且术后的随访很重要。肾上腺皮质癌患者总体预后较差,化疗和放疗可作为术后辅助治疗。恶性嗜铬细胞瘤患者预后较好,MIBG和酚苄明可改善患者生存。对于转移性肾上腺肿瘤患者,手术切除孤立的转移灶可明显提高患者的5年生存率。     

Newly developed hypertension as an early marker of recurrence of adrenocortical carcinoma with high renin expression

Abstract:   Adrenocortical carcinoma can recur frequently after successful surgery but no adequate markers can detect this recurrence. Here, we present a recurrence of adrenocortical carcinoma with a high renin expression, after successful surgery, where hypertension has developed again. Tumor recurrence was observed via ¹⁸F-fluorodeoxyglucose positron emission tomography and coregistered computed tomography. Based on our findings, follow-up blood pressure assessment may effectively predict the recurrence of adrenocortical carcinoma.     

ADRENAL SPARING SURGERY DURING RADICAL NEPHRECTOMY IN PATIENTS WITH RENAL CELL CANCER: A NEW ALGORITHM

PURPOSE: Ipsilateral adrenalectomy is usually performed during radical nephrectomy because of renal cell cancer. Because renal tumors are detected more often in the earlier stages due to widespread use of ultrasound and computerized tomography, we define a subset of patients who would be eligible for adrenal sparing surgery. In a retrospective analysis we evaluated whether parameters obtained preoperatively are able to predict adrenal metastasis. MATERIALS AND METHODS: A total of 866 consecutive patients who underwent nephrectomy and ipsilateral adrenalectomy from 1983 to 1999 were evaluated. Preoperative parameters, including tumor size, location, clinical stage, number of tumors, and patient age and sex, were retrospectively compared with the histological results. Univariate and multivariate analyses were performed. RESULTS: A total of 27 (3.1%) adrenal metastases were noted in the 866 patients, and 63% were on the left side and 37% on the right side. Mean tumor size was 10 cm. with versus 6 cm. without adrenal involvement. Of the 27 patients 21 had multiple metastases at diagnosis and only 6 (0.7% of all 866) presented with solitary ipsilateral adrenal metastasis. Univariate and multivariate analyses revealed tumor size and M stage as best preoperative predictors of adrenal involvement. CONCLUSIONS: Adrenal sparing surgery is possible, and we suggest a new algorithm. If maximum tumor size measured by computerized tomography is less than 8 cm. and staging examination does not show organ or lymph node metastases, adrenalectomy is not necessary because of oncological reasons. This algorithm has to be validated by a prospective analysis.     

Rare case of left adrenal cortical carcinoma with level 3 inferior vena cava thrombus via adrenal vein

Introduction

Adrenocortical carcinoma (ACC) is a rare malignancy with an incidence of 0.7–2.0 cases/million habitants/year. It shows a bimodal pattern of age distribution with a higher incidence in the first decade and then between 40 and 50 years. Women are most frequently affected (55–60%).

METASTATIC ADRENOCORTICAL CARCINOMA TREATED BY REPEATED RESECTION: A CASE REPORT OF LONG-TERM SURVIVAL OVER 18 YEARS

A case of recurrent and metastatic nonfunctioning adrenocortical carcinoma in a 40-year-old woman is reported. The patient received 4 laparotomies and 1 thoracotomy for recurrent and metastatic disease after removal of the primary adrenal tumor. She has been alive for over 18 years following multiple surgery for diseases after the first adrenalectomy. In selected patients with recurrence and/or metastasis, repeated surgical resection offers the possibility of a cure or extended palliation.     

Clinical and molecular aspects of adrenocortical tumourigenesis

Adrenal masses are a common problem affecting 3-7% of the population. The majority turn out to be benign adrenocortical adenomas, which may be functional or non-functional. Much more rarely, these masses represent a primary adrenal carcinoma. It is becoming increasingly recognized that of the benign functioning adenomas or hyperplasias, the majority will hypersecrete aldosterone and this will be more frequently detected when hypertensive populations are screened for this disease. In contrast, the incidence of primary adrenocortical carcinoma has remained steady and for this disease, surgery represents the mainstay of treatment. The advent of laparoscopic adrenal surgery has lowered the threshold size for recommending surgery for asymptomatic adrenal masses and as such, an increased proportion of adrenocortical cancers are being resected and detected at an earlier stage. Recent progress has been made in our understanding of the key genetic changes which underpin the biology of this disease. Progression from adrenal adenoma to carcinoma involves a monoclonal proliferation of cells which, among other defects, have undergone chromosomal duplication at the 11p15.5 locus leading to overexpression of the IGF2 gene and abrogation of expression of the CDKN1C and H19 genes. TP53 is involved in progression to carcinoma in a subset of patients and the frequency of ACTH receptor deletion needs to be more fully explored. Other key oncogenes and tumour suppressor genes remain to be identified although the chromosomal loci in which they lie can be identified at 17p, 1p, 2p16 and 11q13 for tumour suppressor genes and chromosomes 4, 5 and 12 for oncogenes.