Adrenal cortical carcinoma

　　肾上腺皮质癌是一种罕见的疾病，可在任何年龄发生。大多数 ACC 的根本原因尚不清楚，但 ACC 可能与遗传性肿瘤综合征有关，例如 Li-Fraumeni 综合征、Lynch 综合征、多发性内分泌肿瘤 1 型和家族性腺瘤性息肉病 [8]。大多数肾上腺肿瘤是单侧的，尽管双侧 ACC 也可能发生 [9]。功能性肾上腺皮质肿瘤会过量产生以下激素：皮质醇、醛固酮、睾酮和雌激素。因此，功能正常的 ACC 可出现与几种不同内分泌综合征相关的症状，包括库欣综合征和男性化 [10]。相比之下，非功能性 ACC 患者可能会出现多种非特异性症状，例如腹痛、疲劳和与占位效应相关的症状。然而，由于创新成像方式的发展，无症状肾上腺肿块的发现变得更加普遍[11]。建议术前评估皮质醇、促肾上腺皮质激素、硫酸脱氢表雄烯二酮、17-羟基孕酮、睾酮、雄烯二酮和雌二醇的基础水平，进行地塞米松抑制试验，以及评估尿中游离皮质醇水平 [3]。这些测试可能有助于确定肿瘤的肾上腺皮质起源，并提供有关肿瘤恶性潜能的信息。它们也是评估术后肾上腺功能不全风险所必需的，这些激素可以作为术后随访期间的肿瘤标志物[12]。影像学在ACC的诊断中起着至关重要的作用。具有高恶性风险的肾上腺肿瘤通常具有肿瘤大小(> 4 cm)、高密度(> 10 HU)、异质性、绝对洗脱值低于60%、相对洗脱值低于40%、坏死、出血和骨化[3]。在 CT 扫描中，ACC 可能看起来很大，边缘不规则，异质性，平均密度 > 10 HU，并有钙化、坏死和出血的迹象。大约 30% 的 ACC 肿瘤显示出钙化 [13]。在使用对比增强进行的 CT 扫描中，肿瘤通常表现出不均匀的增强，典型的特征是由于坏死的存在，与中心区域相比，周边区域的增强增强 [12]。浸润、静脉血栓和淋巴结肿大的识别明确提示恶性肿瘤。在磁共振成像 (MRI) 上，正常肾上腺组织在 T1 和 T2 加权图像上显示出低到中等的信号强度 [3]。 MRI 可用于通过使用脂肪抑制技术确定肿瘤的脂质含量。因此，MRI 可用于支持区分肾上腺腺瘤和 ACC。在 T1 Read more...

　　这个病例特别有趣，因为没有明显的疑似 ACC 的放射学特征，也没有在原始组织学上看到任何提示性迹象——即使在重复回顾性评估之后也是如此。尽管先前已经注意到缺乏指示肾上腺皮质恶性肿瘤的 CT 征象 (4, 5)，但缺乏特征性组织学的情况要少得多 (5)。 　　在绝大多数情况下，明确的原发性醛固酮增多症和局灶性 2 cm 肾上腺病变与良性病理有关 (4)。虽然在 ACC 中发现了低钾性高血压，但更常见的原因是类固醇生成途径中更近端的类固醇产生，而不是醛固酮。值得注意的是，在我们的患者诊断为原发性醛固酮增多症之前，高血压控制不佳的长期(约 20 年)以及随后对良性病理学的回顾和回顾性证实，因为这表明恶性转化而不是来自 ACC 的存在。一开始。 　　单纯分泌醛固酮的 ACC 异常罕见，因此，关于这种疾病患者预后的数据非常有限。通过与分泌皮质醇的 ACC 类比，我们患者的预后可能比非分泌性病变略好;显然，勤奋的随访和治疗性米托坦水平对于改善长期结果是必不可少的。 　　本报告强调需要持续监测因原发性醛固酮增多症通过手术“治愈”的患者，即使未怀疑为恶性肿瘤。 本文来源于互联网，如有侵权请联系删除。来源：https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4533278/

Following a diagnosis of adrenal cancer, there are several treatment options that may be used. Each of these techniques has unique advantages and disadvantages that will make it preferable for some cases and not others. As adrenal cancer is relatively rare, it can be worthwhile to collaborate with other endocrine specialists to discuss the best treatment options for the particular case. The treatment of adrenal cancer typically involves a multidisciplinary team a may include surgeons, endocrinologists, radiation oncologists, medical oncologists, nurses, psychologists, social workers, and other health professionals. Surgery The most common treatment for adrenal cancer is adrenalectomy or surgical removal of the adrenal gland. In this procedure, the cancer is removed as much as possible, including areas where the cancer has spread to such as nearby lymph nodes. The procedure can be performed in two main ways: through an incision in the back below the ribs or an incision in the front of the abdomen. The incision in the back is useful to remove small tumors but can be difficult for larger tumors. As such, the incision in the front of the abdomen is the most common method used in practice. If the cancer has metastasized to other areas of the body, such as the liver, surgical removal of these secondary tumors may also be needed. For small adrenal tumors, a laparoscope can also be inserted into the adrenal gland to view the tumor and remove it. This is most commonly used for smaller tumors and helps to reduce recovery time. However, it cannot be used for larger tumors as the whole tumor should be removed in one piece to reduce the risk of recurrence. Radiation therapy Radiotherapy, which involves a focused beam of high-energy radiation, can be used to target the region of the cancerous cells in the adrenal gland. This is usually used as adjuvant therapy, in addition to other techniques such as surgery. There are two main types of radiation therapy that may be used: external beam radiation therapy and internal radiation therapy (brachytherapy). External beam radiation therapy uses a machine outside of the body to direct the radiation towards the adrenal gland. The radiation is usually administered once or twice a day, five days a week for a treatment period of approximately 6 weeks. In this type of radiation therapy, the surrounding tissue that the radiation passes through before it reaches the tumor is also affected. Treatment times are kept short to minimize this, but some adverse effects may be experienced. Internal radiation therapy, also known as brachytherapy, uses small pellets of radioactive material, which are placed inside the body next to or inside the tumor. This is usually left inside the body for a few days then removed. The localization of the radiation helps to reduce exposure to the surrounding tissues. Chemotherapy Chemotherapy for patients with adrenal cancer may be administered via intravenous injection or oral medications. This technique is usually reserved for patients with stage 4 adrenal cancers because it can help to destroy cancer cells in several parts of the body simultaneously. For cancer contained in the adrenal gland, surgical removal is usually preferred. Mitotane is a common chemotherapeutic agent used for adrenal gland because it can block adrenal gland hormone production and destroys cancers cells. It is particularly useful for cancers caused by excessive hormone production. Like other chemotherapeutic agents, it also destroys some healthy cells in this process. Other chemotherapeutic agents, which are often used in combination with mitotane, may include: Cisplatin Doxorubicin Etoposide Streptozocin Paclitaxel 5-fluorouracil Vincristine Other medications There are several other medications that may be used in the treatment of adrenal cancer, primarily to reduce the production of hormones related to the tumor. These may include: Ketoconazole and Metyrapone to reduce the production of adrenal steroid hormones Spironolactone to decrease the effects of aldosterone Mifepristone to decrease the effects of cortisol Tamoxifen, Toremifene, and Fulvestrant to block the effects of estrogen Article Source: https://www.news-medical.net/health/Adrenal-Cancer-Treatment.aspx