摘要和推荐小结
摘要
肾上腺皮质癌（ACC）是一种罕见肿瘤，大多数情况下可产生类固醇激素，具有不同的预后。本指南的目的是为临床医生提供基于GRADE(Grading of Recommendations Assessment, Development and Evaluation）系统的、ACC患者临床管理的最佳循证建议。我们预先确定了四个主要的临床问题，我们认为这些问题对于ACC患者的管理特别重要，并进行了系统的文献检索：
组织病理学如何诊断ACC？
ACC中最好的预后标志物是什么？
辅助治疗是否能预防疾病复发或降低根治性切除后的死亡率？
大体上不能不完全切除、复发或转移性疾病的最佳治疗选择是什么？
小组内也讨论了其他相关问题。
选定的建议包括：
我们推荐，所有疑似和经证实的ACC患者在多学科专家团队会议上进行讨论。
我们推荐，每位患有（或疑似）ACC的患者都应进行仔细的临床评估，包括详细的内分泌检查以确定自主激素过量，及针对肾上腺的影像检查。
我们推荐，（疑似）ACC的肾上腺手术应仅由有经验的肾上腺手术和肿瘤手术的外科医生进行，目的是进行完整的整块切除术（包括切除寡转移病灶的转移性疾病）。
我们建议，所有疑似ACC应由专业的肾上腺病理学家使用Weiss评分、以及提供Ki67指数进行评估。
我们建议在根治性手术后患有高复发风险的患者（ENSAT III期，或R1切除，或Ki67>10％）进行辅助性米托坦（mitotane）治疗。
对于不适合完全手术切除的晚期ACC，局部治疗措施（例如放疗、射频消融，化学栓塞）具有特殊价值。然而，我们建议不要在广泛转移性疾病的情况下常规使用肾上腺手术。在这些患者中，我们建议根据预后参数，使用米托坦（mitotane）单药治疗或选择使用米托坦、依托泊苷，多柔比星和顺铂。对治疗具有良好反应的特定患者，可以随后考虑手术。
对于无病间隔至少12个月但复发的患者，完全切除/消融似乎是可行的，我们推荐手术或其他局部治疗。
此外，我们提供有关米托坦治疗和其他支持治疗管理的详细建议。最后，我们对未来研究方向提出建议。
1. 建议摘要
Summary of recommendations
1.1 总体建议
Overarching recommendations
R.1.1 我们推荐所有患有疑似和经证实的肾上腺皮质癌（ACC）的患者，至少在初次诊断时在多学科专家团队会议上进行讨论（包括在肾上腺肿瘤诊治方面经验丰富的专家，应至少包括以下学科：内分泌、肿瘤、病理、放射、外科）。此外，该团队应该能够获得介入放射学、放射治疗、核医学和遗传学以及姑息治疗团队的肾上腺专科知识信息。
R.1.1. We recommend that all patients with suspected and proven adrenocortical carcinoma (ACC) are discussed in a multidisciplinary expert team meeting (including health care providers experienced in care of adrenal tumors, including at least the following disciplines: endocrinology, oncology, pathology, radiology, surgery) at least at the time of initial diagnosis. In addition, this team should have access to adrenal-specific expertise in interventional radiology, radiation therapy, nuclear medicine and genetics as well as to palliative care teams
R.1.2 我们建议，在治疗决策的任何时候，都应考虑参加临床试验（如果有）。此外，我们鼓励患者参与登记和收集生物样本，此常作为结构化研究计划的一部分，旨在确定诊断，预后和治疗反应的生物标志物。
R.1.2. We suggest that at any time of decision making regarding therapy, enrollment in a clinical trial (if available) should be considered. Furthermore, we encourage patients’ participation in registries and the collection of biological material as part of structured research programs aimed at defining biomarkers of diagnosis, prognosis and treatment response.
1.2 疑似ACC的诊断程序
1.2. Diagnostic procedures in suspected ACC
R.2.1 ACC的诊断并不总是很明显。我们推荐尽快使用所有必需的诊断工具及时判断肾上腺占位的良恶性。
R.2.1. The diagnosis of ACC is not always obvious. We recommend establishing as soon as possible whether an adrenal mass is malignant, using all required diagnostic tools in a timely fashion.
R.2.2 我们推荐每位患有（疑似）ACC的患者都要经过仔细评估，包括病史、临床症状和肾上腺激素过量的表现。
R.2.2. We recommend that every patient with (suspected) ACC should undergo careful assessment including case history, clinical examination for symptoms and signs of adrenal hormone excess.
R.2.3 我们推荐所有疑似ACC的患者进行详细的激素检查，以确定糖皮质激素、性激素、盐皮质激素和肾上腺皮质类固醇激素前体的潜在自主过量。此外，必须排除嗜铬细胞瘤。
R.2.3. We recommend that all patients with suspected ACC undergo a detailed hormonal work-up to identify potential autonomous excess of glucocorticoids, sex hormones, mineralocorticoids and adrenocortical steroid hormone precursors. In addition, a pheochromocytoma must be excluded.
R.2.4 我们推荐所有疑似ACC的患者进行针对肾上腺的影像检查。
R.2.4. We recommend adrenal-focused imaging in all patients with suspected ACC.
R.2.5 我们推荐，除了腹腔 - 骨盆横断面成像（CT或MRI）之外，对高度怀疑ACC的患者进行胸部CT检查，因为结果可能会影响治疗决策。
R.2.5. We recommend in any case where there is high suspicion for ACC performing a chest CT, in addition to an abdominal-pelvic cross-sectional imaging (CT or MRI), because the results might influence therapeutic decision making.
R.2.6 我们建议仅在临床怀疑转移性病变的情况下进行额外的影像检查（例如骨和脑成像）。
R.2.6. We suggest performing additional imaging (e.g. bone and brain imaging) only in case of clinical suspicion of metastatic lesions.
R.2.7 我们推荐不要在疑似ACC患者的诊断检查中使用肾上腺活检，除非有证据表明转移性疾病无法进行手术，并且需要获得组织病理学证据以指导肿瘤管理。
R.2.7. We recommend against the use of an adrenal biopsy in the diagnostic work-up of patients with suspected ACC unless there is evidence of metastatic disease that precludes surgery and histopathologic proof is required to inform oncological management.
1.3 局部疑似ACC的手术
1.3. Surgery for suspected localized ACC
R.3.1 我们推荐，对于疑似/确诊ACC的肾上腺手术应仅由肾上腺和肿瘤手术经验丰富的外科医生进行。
R.3.1. We recommend that adrenal surgery for suspected/confirmed ACC should be performed only by surgeons experienced in adrenal and oncological surgery.
R.3.2 我们推荐对怀疑为ACC的所有肾上腺肿瘤进行全面整体切除，包括肿瘤周围/肾上腺腹膜后腹膜脂肪。对于疑似ACC，我们推荐不要进行摘除术和部分肾上腺切除术。如果怀疑邻近器官被侵犯，我们建议整体切除。然而，我们建议不要在没有直接肾脏侵犯的情况下进行同侧肾脏的常规切除。
R.3.2. We recommend complete en bloc resection of all adrenal tumors suspected to be ACC including the peritumoral/periadrenal retroperitoneal fat. We recommend against enucleation and partial adrenal resection for suspected ACC. If adjacent organs are suspected to be invaded, we recommend en bloc resection. However, we suggest against the routine resection of the ipsilateral kidney in the absence of direct renal invasion.
R.3.3 开放手术是确诊或高度怀疑ACC的标准手术方法。因此，我们推荐对所有影像学检查结果可疑恶性肿瘤，和有局部侵袭证据肿瘤，进行开放手术。然而，对于<6 cm而没有任何局部侵袭的证据的肿瘤，并且外科医生在这些类型的手术中有足够经验，腹腔镜肾上腺切除术（尊重肿瘤手术的原则）是合理的。
R.3.3. Open surgery is the standard surgical approach for confirmed or highly suspected ACC. Therefore, we recommend open surgery for all tumors with radiological findings suspicious of malignancy and evidence for local invasion. However, for tumors <6 cm without any evidence of local invasion, laparoscopic adrenalectomy (respecting the principles of oncological surgery) is reasonable if the surgeon has sufficient experience in these types of surgery.
R.3.4 对于高度怀疑或已证实的ACC，我们建议在进行肾上腺切除术治疗时，常规行局部区域淋巴结切除术。手术应该包括（至少）肾周和肾门淋巴结。应切除术前影像学或术中发现的所有可疑或肿大的淋巴结。
R.3.4. We suggest that routine loco-regional lymphadenectomy should be performed with adrenalectomy for highly suspected or proven ACC. It should include (as a minimum) the periadrenal and renal hilum nodes. All suspicious or enlarged lymph nodes identified on preoperative imaging or intraoperatively should be removed.
R.3.5 我们推荐，在肿瘤扩展侵犯至大血管时，由多学科外科团队依据情况做出个性化治疗决策。在专家中心进行审查之前，不应将此类肿瘤视为“无法切除”。
R.3.5. We recommend that individualized treatment decisions are made in cases of tumors with extension into large vessels based on multidisciplinary surgical team. Such tumors should not be regarded ‘unresectable’ until reviewed in an expert center.
R.3.6 如果第一次手术是次优选择的，并且在大体上切除不完整（R2切除），我们建议在多学科专家团队中讨论再次手术。
R.3.6. If the first surgery was suboptimal and macroscopically incomplete (R2 resection), we suggest to discuss repeat surgery in a multidisciplinary expert team.
R.3.7 我们推荐对所有接受ACC手术的高皮质醇血症患者进行围手术期氢化可的松置换。
R.3.7. We recommend perioperative hydrocortisone replacement in all patients with hypercortisolism that undergo surgery for ACC.
1.4 病理检查
1.4. Pathological work-up
R.4.1 我们推荐ACC的诊断应通过组织病理学确认（+++O）。
R.4.1. We recommend that the diagnosis of ACC should be confirmed by histopathology (+++0).
R.4.2 我们建议所有肾上腺肿瘤，不应轻易分类，所有疑似ACC，应由肾上腺病理学家（++OO）审查。
R.4.2. We suggest that all adrenal tumors, which cannot be readily classified, and all suspected ACC, should be reviewed by an expert adrenal pathologist (++OO).
R.4.3 我们建议进行类固醇生成因子-1（SF1）免疫组化，用于区分原发性肾上腺皮质肿瘤和非肾上腺皮质肿瘤（+OOO）。
R.4.3. We suggest the use of immunohistochemistry for steroidogenic factor-1 (SF1) for the distinction of primary adrenocortical tumors and non-adrenocortical tumors (+OOO).
R.4.4 我们推荐使用Weiss系统，基于九种组织学标准的组合，可应用于HE染色(苏木精-伊红)切片，以区分良性和恶性肾上腺皮质肿瘤（++OO）。
R.4.4. We recommend the use of the Weiss system, based on a combination of nine histological criteria that can be applied on hematoxylin and eosin-stained slides, for the distinction of benign and malignant adrenocortical tumors (++OO).
R.4.5 我们推荐对肾上腺皮质肿瘤（++OO）的每个切除标本使用Ki67免疫组化。
R.4.5. We recommend the use of Ki67 immunohistochemistry for every resection specimen of an adrenocortical tumor (++OO).
R.4.6 我们推荐疑似ACC的病理报告应至少包含以下信息：Weiss评分（包括确切的有丝分裂计数）、确切的Ki67指数、切除状态和病理性肿瘤分期（表明组织和器官和/或周围侵犯与否）和淋巴结状态（+OOO）。
R.4.6. We recommend that the pathology report of a suspected ACC should at least contain the following information: Weiss score (including the exact mitotic count), exact Ki67 index, resection status and pathological tumor stage (indicating invasion or not of the capsule and/or surrounding tissue and organs) and nodal status (+OOO).
1.5 分期分类和预后因素
R.5.1 在初步诊断时，我们推荐使用欧洲肾上腺肿瘤研究网络（ENSAT）分期分类（+++O）。
R.5.1. At initial diagnosis, we recommend using the European Network for the Study of Adrenal Tumours (ENSAT) staging classification (+++O).
R.5.2 在初步诊断时，我们推荐在评估预后和治疗方案时考虑以下因素：肿瘤分期、切除状态、Ki67指数（或有丝分裂计数）、自主皮质醇分泌和患者的一般状况（++OO）。
R.5.2. At initial diagnosis, we recommend taking the following factors into account when assessing the prognosis and treatment options: tumor stage, resection status, Ki67 index (or mitotic count), autonomous cortisol secretion and the patient’s general condition (++OO).
R.5.3 在随访期间，我们推荐在每次评估时重新评估预后，以指导治疗策略（++OO）。
R.5.3. During follow-up, we recommend re-assessing prognosis at each evaluation, to guide treatment strategy (++OO).
1.6 随访期间影像和激素评估方法和时间间隔
1.6. Methods and time interval for imaging and hormonal assessment during follow-up
R.6.1 我们推荐患者定期对腹部、骨盆和胸部进行断面影像检查，以确定疾病的复发或进展。
R.6.1. We recommend following patients with regular cross-sectional imaging of the abdomen, pelvis and chest for disease recurrence or progression.
R.6.2 完全切除后，我们建议每3个月进行影像检查持续2年，然后的3年内每3-6个月复查。小组的大多数专家建议继续进行超过5年的影像随访，但可调整监测方式。
R.6.2. After complete resection, we suggest radiological imaging every 3 months for 2 years, then every 3–6 months for a further 3 years. The majority of the panel suggests continuation of follow-up imaging beyond 5 years, but surveillance should then be adapted.
R.6.3 对于晚期ACC，我们推荐根据预后因素、预期治疗效果、治疗相关毒性，以及可用的替代治疗方案，进行监测。
R.6.3. For advanced ACC, we recommend surveillance based on prognostic factors, expected treatment efficacy and treatment-related toxicity, as well as the available alternative treatment options.
R.6.4 在所有患者中，我们推荐定期检查激素分泌情况。
R.6.4. In all patients, we recommend regular screening for hormone secretion.
1.7 辅助治疗
1.7. Adjuvant therapy
R.7.1 对于具有不确定恶性潜能的肾上腺肿瘤，我们推荐不进行辅助治疗（+OOO）。
R.7.1. For adrenal tumors with uncertain malignant potential, we recommend against adjuvant therapy (+OOO).
R.7.2 我们建议在手术后没有肉眼可见的残留肿瘤、但具有高复发风险的患者中进行辅助性米托坦治疗（+OOO）。然而，对于低/中度复发风险的患者（I-II期，R0切除和Ki67≤10％），我们不建议或反对辅助治疗，并且应针对辅助治疗选择进行单独讨论。
R.7.2. We suggest adjuvant mitotane treatment in those patients without macroscopic residual tumor after surgery but who have a perceived high risk of recurrence (+OOO). However, we cannot suggest for or against adjuvant therapy for patients at low/moderate risk of recurrence (stage I–II, R0 resection and Ki67 ≤10%) and adjuvant therapy options should be discussed on an individual basis.
R.7.3 一旦决定米托坦(mitotane)治疗，我们推荐在手术后尽快开始使用（+OOO）。
R.7.3. Once the decision for mitotane treatment is established, we recommend starting mitotane as soon as clinically possible after surgery (+OOO).
R.7.4 在以可接受的方式耐受米托坦治疗的无复发的患者中，我们建议给予辅助米托坦至少2年，但不超过5年（+OOO）。
R.7.4. In patients without recurrence who tolerate mitotane in an acceptable manner, we suggest to administer adjuvant mitotane for at least 2 years, but not longer than 5 years (+OOO).
R.7.5 专家组未就辅助放射治疗达成明确的共识。然而，我们建议不要在I-II期和R0期切除术（+OOO）患者中常规使用放射治疗。专家组建议在R1或Rx切除或III期患者的个体化基础治疗中可考虑除米托坦治疗外的放射治疗。
R.7.5. The panel did not come to a definitive consensus on adjuvant radiation therapy. However, we suggest against the routine use of radiation therapy in patients with stage I–II and R0 resection (+OOO). The panel suggests considering radiation in addition to mitotane therapy on an individualized basis therapy in patients with R1 or Rx resection or in stage III.
R.7.6 如果给予辅助放射治疗，我们推荐在手术后尽快开始治疗，并以50-60Gy的剂量向术前肿瘤床进行放射治疗，分次剂量约为2Gy（+OOO）。
R.7.6. If adjuvant radiation therapy is administered, we recommend starting treatment as soon as clinically possible after surgery and to deliver radiation therapy at the dose of 50–60 Gy to the previous tumor bed in fractionated doses of approximately 2 Gy each (+OOO).
R.7.7 专家组未就细胞毒性药物的辅助使用达成明确的共识。我们建议不要常规在佐剂环境中使用细胞毒性药物。然而，小组建议在具有非常高复发风险的患者中考虑选择辅助化疗。
R.7.7. The panel did not come to a definitive consensus on adjuvant use of cytotoxic drugs. We suggest against the routine use of cytotoxic drugs in the adjuvant setting. However, the panel suggests considering adjuvant chemotherapy in selected patients with very high risk for recurrence.
1.8 治疗复发和/或晚期ACC
1.8. Treatment of recurrent and/or advanced ACC
R.8.1 对于在初诊时出现有限的腹腔内转移的患者，如果完全切除所有病变似乎可行，我们建议进行手术治疗（+OOO）。在腹外病变有限的情况下，我们建议将肾上腺肿瘤切除与针对其他病变的长期肿瘤控制的治疗相结合（+OOO）。在所有复发和/或晚期ACC患者中，我们推荐尽快开始使用米托坦治疗（+OOO）。
R.8.1. For patients presenting at time of initial diagnosis with limited intra-abdominal metastases, we suggest surgical therapy if complete resection of all lesions seems feasible (+OOO). In case of limited extra-abdominal lesions, we suggest adrenal tumor resection in conjunction with therapy aiming at long-term tumor control of the other lesions (+OOO). In all patients, we recommend to start mitotane therapy as soon as clinically possible (+OOO).
R.8.2 专家组确信，除手术外，其他局部治疗措施（例如放射治疗、射频消融、冷冻消融、微波消融、化学栓塞）对于晚期ACC的治疗具有价值。我们建议基于肿瘤病变部位、部位相关专业知识、预后因素和患者偏好对选择方法的进行个体化决策（+ OOO）。
R.8.2. The panel is convinced that in addition to surgery other local therapeutic measures (e.g. radiation therapy, radiofrequency ablation, cryoablation, microwave ablation, chemoembolization) are of value for therapy of advanced ACC. We suggest individualization of the decision on the method of choice based on the localization of the tumor lesion(s), local expertise, prognostic factors and patient’s preference (+OOO).
R.8.3 我们建议在首次诊断即有广泛转移性疾病的情况下，不要常规使用肾上腺手术（+OOO）。
R.8.3. We suggest against the routine use of adrenal surgery in case of widespread metastatic disease at the time of first diagnosis (+OOO).
R.8.4 对于诊断晚期ACC，不符合局部治疗条件的患者，我们建议根据预后参数进行米托坦(mitotane)单药治疗或米托坦(mitotane) + EDP治疗（+++O）。
R.8.4. In patients with advanced ACC at the time of diagnosis not qualifying for local treatment, we recommend either mitotane monotherapy or mitotane + EDP depending on prognostic parameters (+++O).
R.8.5 对于复发性疾病且(复发前)无病间隔至少12个月的患者，完全切除/消融似乎是可行的，我们推荐手术或其他局部治疗（+OOO）。我们推荐干预后尽快开始使用米托坦。
R.8.5. In patients with recurrent disease and a disease-free interval of at least 12 months, in whom a complete resection/ablation seems feasible, we recommend surgery or alternatively other local therapies (+OOO). We recommend starting mitotane as soon as possible after the intervention.
R.8.6 如果上次手术/局部区域治疗和复发之间的时间间隔小于6个月，我们推荐EDP-M作为一线治疗，而不是重复进行局部区域检测（++OO）。
R.8.6. We recommend EDP-M as first-line treatment if the time interval between last surgery/loco-regional therapy and recurrence is less than 6 months (++OO), rather than repeat loco-regional measures.
R.8.7 对于所有其他复发性疾病患者，需要采用个体化治疗方法。
R.8.7. For all other patients with recurrent disease, an individualized approach is needed.
R.8.8 在使用米托坦单药治疗过程中疾病进展的患者中，我们推荐加用EDP（+++ O）。
R.8.8. In patients who progress under mitotane monotherapy, we recommend to add EDP (+++O).
R.8.9 对药物治疗有反应的患者（包括实现长期稳定的疾病情况），我们建议重新考虑旨在长期控制肿瘤的局部措施。在实现对疾病控制一般良好、病变进展缓慢的患者中也可以考虑这种方法。
R.8.9. In patients who respond to medical therapy (including achievement of long-term stable disease), we suggest re-considering local measures aiming at long-term tumor control. Such an approach could also be considered in patients attaining a generally good control of the disease, in which a limited number of lesions are progressing.
R.8.10 对于在EDP-M治疗下仍有疾病进展的患者，我们建议考虑其他治疗，包括个体化临床试验（+OOO）。
R.8.10. In patients who progress under EDP-M, we suggest considering additional therapies including clinical trials on an individual basis (+OOO).
R.8.11 目前尚不清楚米托坦停药的最佳时机，专家组无法就此问题提出具体建议。
R.8.11. The optimal timing of mitotane discontinuation is currently unknown and the panel could not come to a specific recommendation on this issue.
1.9 对米托坦的特殊考虑
1.9. Special considerations on mitotane
R.9.1 我们推荐根据患者的表现状况以及第一周的耐受性，采用逐渐增加剂量的方案开始使用米托坦治疗。
R.9.1. We recommend starting therapy with mitotane in an escalating regimen depending on the performance status of the patient as well as the tolerability in the first weeks.
R.9.2 我们推荐监测米托坦的血药浓度。一般目标是米托坦血浆水平达到高于14mg/L（+OOO）。
R.9.2. We recommend monitoring of blood concentration of mitotane. The general aim is to reach a mitotane blood level above 14 mg/L (+OOO).
R.9.3 我们推荐所有接受米托坦治疗的患者接受糖皮质激素替代治疗（皮质醇过度分泌者除外）。我们建议使用氢化可的松/醋酸可的松。由于类固醇清除率增加，以及皮质醇结合球蛋白增加，通常需要标准替代剂量的两倍。
R.9.3. We recommend glucocorticoid replacement in all patients treated with mitotane (except those with ongoing cortisol excess). We suggest using hydrocortisone/cortisone acetate for this purpose. Due to increased steroid clearance and increase cortisol-binding globulin at least twice the standard replacement dose is usually required.
R.9.4 我们推荐定期监测米托坦引起的不良反应，并对其进行适当治疗。为了增加米托坦的耐受性，我们建议在严重毒性副作用发生前即开始支持疗法。
R.9.4. We recommend regular monitoring of mitotane-induced adverse effects and to treat them appropriately. To increase tolerability of mitotane, we suggest starting supportive therapy ideally before severe toxicity occurs.
R.9.5 我们推荐了解米托坦的显着药物相互作用（例如，CYP3A4的强诱导作用）。应检查所有伴随用药的CYP3A4相互作用，并在必要时替换药物。应建议其他诊治人员在未经咨询的情况下，不要开展其他药物治疗。
R.9.5. We recommend being aware of significant drug interactions of mitotane (e.g. due to strong induction of CYP3A4). All concomitant medication should be checked for CYP3A4 interactions and substituted for an alternative if necessary and available. Other care providers should be advised not to initiate other drug therapies without consultation.
1.10 其他支持疗法
1.10. Other supportive therapies
R.10.1 我们推荐采用药物治疗来控制所有临床相关的激素生成性ACC患者的激素过量分泌。
R.10.1. We recommend medical therapy to control hormone excess in all patients with clinically relevant hormone-producing ACC.
R.10.2 我们推荐对骨转移患者进行抗（骨）再吸收治疗。
R.10.2. We recommend therapy with anti-resorptive treatment in patients with bone metastasis.
R.10.3 我们推荐姑息性放疗用于晚期/转移性ACC患者的症状缓解。
R.10.3. We recommend palliative radiation for symptom palliation in advanced/metastatic ACC patients.
R.10.4 我们推荐将姑息治疗纳入所有晚期ACC患者的标准肿瘤治疗。
R.10.4. We recommend integrating palliative care into standard oncology care for all patients with advanced ACC.
R.10.5 我们建议为生育年龄的女性患者提供生育保护咨询。生育咨询不仅应限于接受细胞毒性化疗的患者，还应限于计划开始进行米托坦治疗的患者。
R.10.5. We suggest counseling for fertility protection in female patients in reproductive age. Fertility counseling should not only be restricted to patients undergoing cytotoxic chemotherapy, but also given to patients who plan to embark on mitotane therapy.
1.11 遗传咨询
1.11. Genetic counseling
R.11.1 对于患有ACC的成年人，我们建议至少进行基本的临床遗传评估，探讨个人史和家族史，以获得遗传性易感综合征的证据。
R.11.1. For adults with ACC, we recommend at least a basic clinical genetic evaluation, exploring personal and family history for evidence of a hereditary predisposition syndrome.
R.11.2 小组不推荐也不反对用于体细胞突变的肿瘤基因检测。
R.11.2. The panel does not recommend for or against genetic tumor testing for somatic alterations.
1.12 妊娠和ACC
1.12. Pregnancy and ACC
R.12.1 当妊娠期间诊断出怀疑是ACC的肾上腺肿块时，无论妊娠处于何期，我们推荐立即进行手术切除。
R.12.1. When an adrenal mass suspected to be an ACC is diagnosed during pregnancy, we recommend prompt surgical resection regardless of pregnancy trimester.
R.12.2 应告知患者与ACC相关的妊娠相关问题，尤其是当前诊断或过去诊断的ACC。
R.12.2. Patients should be informed on pregnancy-related concerns specific to the current or past diagnosis of ACC.
R.12.3 我们推荐在进行米托坦治疗时避免妊娠。
R.12.3. We recommend avoiding pregnancy while being on mitotane treatment.
European Society of Endocrinology Clinical Practice Guidelines on the management of adrenocortical carcinoma in adults, in collaboration with the European Network for the Study of Adrenal Tumors, European Journal of Endocrinology
内分泌代谢病疾病 @CK医学科普
内分泌代谢病知识架构 @CK医学科普
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