Oncocytic adrenocortical carcinoma

The new minimally invasive transobturator sling for surgical treatment of female genuine stress urinary incontinence is designed to reproduce the natural suspension of the urethral fascia while eliminating the need for retropubic needle passage. We report 3 cases of bladder perforation during the transobturator sling procedure. All injuries were identified intraoperatively by cystoscopy, and successful reinsertion of the mesh was accomplished. Transurethral bladder drainage with a Foley catheter was maintained for 5 to 7 days postoperatively. All 3 patients recovered uneventfully. Routine intraoperative cystoscopy is, therefore, recommended for the identification of bladder injuries during the transobturator sling procedure.     

An eleven-year experience with adrenocortical carcinoma.

BACKGROUND. Key issues in the treatment of adrenocortical carcinoma are the value of adjuvant therapy, the value of reoperation, and the search for effective chemotherapeutic agents. The present series was reviewed to evaluate these issues. METHODS. We present a retrospective series of 73 patients with adrenocortical carcinoma treated at a single institution. RESULTS. Twenty patients had carcinomas that were unresectable, and 53 patients underwent complete resections. Ten patients received adjuvant therapy (mitotane, seven patients; radiation, three patients). Forty-five (85%) patients had recurrence, including all who received adjuvant therapy. Mean disease-free intervals for those who did and did not receive adjuvant therapy were equivalent at 2.4 years. Nineteen patients with recurrent disease received chemotherapy, and 26 patients underwent 51 reoperations to resect recurrent and metastatic disease. The overall 5-year survival rate, which was 35%, was 47% for patients with complete resection. Stage and resectability were prognostic factors. Mean survival time for patients with recurrent disease treated medically was 19 months compared with 56 months for patients who underwent reoperation. Mitotane had a 24% partial response rate. Other chemotherapeutic agents were ineffective. CONCLUSIONS. We conclude that an aggressive surgical approach to recurrent and metastatic disease should be adopted and that patients should be resected free of disease whenever possible. Currently no effective chemotherapy exists, and the value of adjuvant therapy remains unproved.     

Non functioning adrenocortical carcinoma. A rare case.

Adrenocortical carcinoma is a rare tumor and experience in its diagnosis and treatment is limited. We present a case of non functioning adrenocortical carcinoma in which the pre-operative diagnosis of a kidney tumor had been made and a radical nephrectomy was performed.     

P-glycoprotein expression and multidrug resistance in adrenocortical carcinoma.

BACKGROUND. The response of adrenocortical carcinoma (ACC) to adjuvant chemotherapy has been disappointing with no significant impact on survival. The normal adrenal cortex has very high levels of P-glycoprotein, an energy-dependent efflux pump of a variety of structurally unrelated chemotherapeutic agents. P-glycoprotein has been implicated as a cause of multidrug resistance in a variety of neoplasms. The purpose of this study was to evaluate P-glycoprotein expression in ACC. METHODS. Eleven patients with ACC had paraffin-embedded tumor evaluated for P-glycoprotein expression. These were analyzed by immunohistochemistry assay with a battery of four anti-P-glycoprotein antibodies (MRK-16, JSB-1, UIC-2, MDR). RESULTS. All eleven cases showed intense, predominantly membrane immunoreactivity for P-glycoprotein. In 10 of the cases, most tumor cells were immunoreactive with at least three antibodies, and six of 11 cases were positive for all four antibodies. In this small series no correlation existed between P-glycoprotein expression and tumor grade, stage of disease, or survival. CONCLUSIONS. All 11 cases of ACC studied showed P-glycoprotein expression, which was similar to the normal adrenal cortex. This possible mechanism of multidrug resistance may help explain the significant chemoresistance seen in ACC.     

Feminizing adrenocortical carcinoma in man

A 37-year-old male with adrenocortical tumor presenting marked gynecomastia and impotence as initial symptoms was experienced. Abnormal elevations in urinary 17-KS, 17-OHCS and blood estrogen levels were noted. Total surgical removal of the tumor weghing 800g was performed. Postoperatively the gynecomastia disappeared and he regained libido with no signs of recurrence one year later.     

肾上腺皮质癌的治疗进展

肾上腺皮质癌恶性程度高,预后差.除手术治疗外,目前已开展包括米托坦、胰岛素样生长因子Ⅰ受体拮抗剂,酪氨酸激酶受体抑制剂以及血管生成抑制剂等多种治疗方法.同时肾上腺皮质癌的生物学机制研究也取得一定进展,为分子靶向治疗提供了相关依据.本文就目前肾上腺皮质癌的治疗进展予以综述.     

Spontaneous rupture of adrenocortical carcinoma

Massive hemorrhage from an adrenocortical carcinoma seldom occurs in the retroperitoneal or abdominal cavity. We report a case of spontaneous rupture of primary adrenocortical carcinoma occurring in an adolescent. A right adrenalectomy with complete removal of the tumor was performed successfully.     

Cytotoxic treatment of adrenocortical carcinoma

Adrenocortical carcinoma (ACC) is a rare, aggressive tumor that is often detected in an advanced stage. Medical treatment with the adrenotoxic drug mitotane has been used for decades, but critical prospective trials on its role in residual disease or as an adjuvant agent after surgical resection are still lacking. The concept of a critical threshold plasma level of the drug must be confirmed in controlled studies. Because individual responsiveness cannot be predicted, the use mitotane is still advised for nonresectable disease. In case of cortisol or other steroid overproduction, several drugs (e.g., ketoconazole or aminoglutethimide) may be used. Chemotherapy with single agents (e.g., doxorubicin or cisplatin) have been disappointing, with low response rates (< 30%) and a short response duration. Part of this refractoriness may be explained by the fact that ACC tumors express the multidrug-resistance gene MDR-1. Chemotherapy with multiple agents has been tested in smaller series and has resulted in significant side effects. The best results were achieved by the combination of etoposide, doxorubicin, and cisplatin associated with mitotane, achieving a response rate of 54%, including individual complete responses. To be able to make progress in treating advanced ACC disease, adjuvant multicenter trials must be encouraged. When mitotane-based therapies are used, monitored drug levels are mandatory.     

Contemporary management of adrenocortical carcinoma

Context

Adrenocortical carcinoma (ACC) is a rare and typically aggressive malignancy. Available recommendations are based primarily on retrospective series or expert opinions, and only few prospective clinical studies have yet been published.

Objective

To combine the available evidence for diagnostic work-up and treatment of ACC to a contemporary recommendation on the management of this disease.

Evidence acquisition

We conducted a systematic literature search for studies conducted on humans and published in English using the Medline/PubMed database up to 31 January 2011. In addition, we screened published abstracts at meetings and several Web sites for recommendations on ACC management.

Evidence synthesis

In patients with suspected localised ACC, a thorough endocrine and imaging work-up is followed by complete (R0) resection of the tumour by an expert surgeon. In experienced hands, laparoscopic adrenalectomy is probably as effective and safe for localised and noninvasive ACC as open surgery. Most clinicians agree that mitotane should be used as adjuvant therapy in the majority of patients, as they have a high risk for recurrence. An international panel has suggested using tumour stage, resection status, and the proliferation marker Ki67 as guidance for or against adjuvant therapy. In patients with advanced disease at presentation or recurrence not amenable to complete resection, a surgical approach is frequently inadequate. In these cases, mitotane alone or in combination with cytotoxic drugs is the treatment of choice. The most promising regimens (etoposide, doxorubicin, cisplatin plus mitotane, and streptozotocin plus mitotane) are currently compared in an international phase 3 trial, and results should be available by the end of 2011. Several targeted therapies are under investigation and may lead to new treatment options. Management of endocrine manifestations with steroidogenesis inhibitors is required in patients suffering uncontrolled hormone excess.

Conclusions

Detailed recommendations are provided to guide the management of patients with ACC.     

肾上腺皮质癌的诊断与治疗

目的：提高肾上腺皮质癌的诊治水平。方法：结合临床资料及有关文献回顾性分析３例肾上腺皮质癌的临床表现,病理分期,诊断治疗和预后。结果：１例女性患者为Ⅰ期功能型肿瘤,经手术治疗生存３年;２例男性患者为Ⅳ期无功能肿瘤,１例手术生存２年７个月,最后死于无关疾病,１例行穿刺活检生存４个月死亡。结论：外科手术是肾上腺皮质癌唯一有效的治疗方法,早期诊断,彻底手术是治疗本病的关键。     

A case of virilizing adrenocortical carcinoma

A 36-year-old woman who had experienced two pregnancies consulted our hospital, because of scant menses and virilization. A 24-hour excretion of 17-ketosteroids and 17-hydroxy-corticosteroids demonstrated a decrease in 11-hydroxylase. A computed tomogram showed a huge inhomogenous tumefaction in the left adrenal. Left selective renal angiography revealed a large adrenal tumefaction. Selective adrenal venous samplings revealed that testosterone and dehydroepiandrosterone (DHA) were produced in response to stimulation by 0.25 mg exogenous adrenocorticotropic hormone (ACTH). After left adrenalectomy was performed, a diagnosis of adrenocortical carcinoma was made by pathological examination. This is the first report of a patient with a virilizing adrenocortical carcinoma, which produced testosterone and DHA in response to exogenous ACTH stimulation.     

Flow cytometry in feminizing adrenocortical carcinoma

A case of feminizing adrenal cortical neoplasm is presented in which automated flow cytometry determination of deoxyribonucleic acid content was used to confirm malignancy.     

肾上腺皮质癌诊断和治疗

目的 总结肾上腺皮质癌诊治体会。方法 １９７７～１９９８年收治２１例肾上腺皮质癌患者。针对首发症状,有无内分泌功能紊乱,影像学检查,治疗方法及随访资料进行分析。结果 ２１例中有内分泌功能紊乱者１０例,主要以柯兴综合征为主,性征异常者及原发性醛固酮增多症相对少见。影像学显示肿瘤４～１９ｃｍ,平均８．５ｃｍ。ＣＴ和ＭＲＩ揭示肿瘤边界不规则,内部质地不均匀。根治性手术治疗１７例,２年生存率３０％;肿瘤未     

原发性肾上腺皮质腺癌的CT表现

目的 总结原发性肾上腺皮质腺癌的CT表现,以提高其诊断准确性.方法 回顾性分析经手术或穿刺活检后病理证实的原发性肾上腺皮质腺癌17例的影像资料,所有的患者术前均做CT平扫及动脉期和门静脉期双期增强扫描.结果 全部病例单发,右侧9例,左侧8例,直径3～17 cm;6例肿瘤内可见点状、条索状或结节状钙化,10例见较大范围的液化、坏死和囊变区,4例肿瘤以实质为主伴小囊状低密度区.平扫肿瘤CT值为32～47 HU,平均37.3 HU.动脉期增强扫描,肿瘤实性成分强化较明显,肿瘤实质成分CT值在37～72 HU,平均50.9 HU,8例见显著强化的不规则的肿瘤血管.门静脉期肿瘤实质成分强化更明显,强化范围扩大,实质部分CT值为37～98 HU,平均83.4 HU.结论 原发性肾上腺皮质腺癌的CT表现具有一定的特征性,CT双期增强扫描在原发性肾上腺皮质癌的诊断中有重要的诊断价值.     

肾上腺皮质腺癌的诊断与治疗

目的 探讨肾上腺皮质腺癌的早期诊断、手术及围手术期处理。方法 回顾分析了1990年至2001年间31例肾上腺皮质腺癌临床资料．并与1978年至1989年同种病例作比较。结果 近10年患者病理分期更趋向早期，更易行根治性切除，5年的存活率明显提高。结论 影像学的发展，使患者易早期诊断；围手术期的充分准备是减少手术并发症及死亡率的关键。