诊断为双侧原发性肾上腺弥漫性大 B 细胞淋巴瘤的患者在治疗前发现肾上腺功能不全:病例报告

orhan Ayyildiz , sengul baran , hazal sahin , abdullah karakus
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引用次数: 0

摘要

病例报告简介:肾上腺是恶性肿瘤经常转移的器官之一。然而,肾上腺的原发性恶性肿瘤却很少见。原发性肾上腺淋巴瘤(PAL)占结节外淋巴瘤的比例不到1%。75%的病例为双侧发病。CASEA 57 岁男性患者,无已知病史,主诉体重减轻(13 千克,超过体重的 10%)、震颤、食欲不振和下背部疼痛,前往一家外部中心就诊。在外部中心进行评估后,胸部 CT 显示双侧肾上腺肿块。PET CT 初步诊断为恶性肿瘤;右侧肾上腺约 11.6 × 8.1 厘米(SUVmax:25.6),左侧肾上腺约 10.1 × 7.2 厘米(SUVmax:24.左侧主动脉旁(SUVmax:11.7)淋巴结大小为 1.6 × 1.2 厘米,在主动脉间链中观察到几个轻度高代谢淋巴结。原发性恶性肿瘤的储存是在两个肾上腺中确定的异质结构的致密高代谢肿块区域的前景中进行评估的。在确诊为嗜铬细胞瘤后,对右侧肾上腺肿块病变进行的真切病理检查显示,其形态学和组织化学结果与弥漫大 B 细胞淋巴瘤一致,于是患者被转诊至我院。血常规中,血红蛋白:9.7g/dL Hct:31.2% MCV:96 fL Platlet:126.000 / mm3。生化指标方面,肌酐:1.83mg/dL 尿素:80 mg/dL Na:139 mmol/L K:4.36 mmol/L Ca:10.2mg/dL 尿酸:9.4mg/dL LD:343U/L。除脱水外,没有任何肾上腺功能不全的迹象。患者开始接受水合和别嘌醇治疗。随访期间,尿素和肌酐水平降至正常范围。患者的基础皮质醇为 12.02 µg/dL,促肾上腺皮质激素为 83.8ng/L。对患者进行了小剂量(1µg)促肾上腺皮质激素试验,初步诊断为肾上腺功能不全,并计划对其原发疾病进行化疗。患者在 30-60-90 分钟和 120 分钟时的皮质醇水平分别为 8.23-10.35-8.93-9.75 µg/dL,诊断为肾上腺功能不全,于是开始氢化可的松治疗。在对患者进行随访期间,开始了 R-CHOP 化疗,由于患者在化疗过程中使用了泼尼松龙,因此在化疗期间停用了氢化可的松,并进行了孤立的泼尼松龙治疗。鞘内化疗(Mtx、地塞米松、阿拉伯胞嘧啶)期间,在脑脊液中检测到中枢神经系统受累。鞘内治疗最初每周进行三次,后来改为每周两次。由于细胞离心机未检测到细胞,鞘内化疗进行了 4 次。在接受了6个周期的R-CHOP化疗后,患者因高风险疾病于2023年12月接受了自体外周干细胞移植。随访期间,患者总体情况良好,目前正在接受替代剂量氢化可的松治疗,病情缓解。
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ADRENAL INSUFFICIENCY DETECTED BEFORE TREATMENT IN A PATIENT DIAGNOSED WITH BILATERALLY PRIMARY ADRENAL DIFFUSE LARGE B CELL LYMPHOMA:A CASE REPORT

Case report: INTRODUCTION

Adrenal glands are one of the organs where malignancies frequently metastasize. However, primary malignancies of the adrenal glands are rare. Primary adrenal lymphomas (PAL) account for less than 1% of extranodal lymphomas. It is seen bilaterally in 75% of cases. The most common subtype is diffuse large B-cell lymphoma.

CASE

A 57-year-old male patient with no known history of disease applied to an external center with complaints of weight loss (13 kg, more than 10% of body weight), tremors, loss of appetite and lower back pain. As a result of the evaluations made at an external center, thorax CT showed bilateral adrenal masses. PET CT was taken with the preliminary diagnosis of malignancy; In the right adrenal gland, approximately 11.6 × 8.1cm (SUVmax: 25.6) and in the left adrenal gland, approximately 10.1 × 7.2cm (SUVmax: 24.4) in size, heterogeneous dense hypermetabolic solid mass sections with necrotic areas were seen and left paraaortic (SUVmax: 11.7) lymph node with dimensions of 1.6 × 1.2 cm and a few mildly-intensely hypermetabolic lymph nodes were observed in the interaortocaval chain. Primary malignancy storage was evaluated in the foreground of dense hypermetabolic mass regions of heterogeneous structure defined in both adrenal glands. After pheochromocytoma was diagnosed, the patient was referred to our clinic after the tru-cut pathology performed on the mass lesions in the right adrenal gland revealed that the morphological and histochemical findings were consistent with diffuse large B-cell lymphoma.

The patient's vital signs were stable upon admission. In the hemogram, Hgb: 9.7g/dL Hct: 31.2% MCV: 96 fL Platlet: 126.000 / mm3. In biochemistry, creatinine:1.83mg/dL urea:80 mg/dL Na:139 mmol/L K:4.36 mmol/L Ca:10.2mg/dL uric acid:9.4mg/dL LD:343U/L. There was no sign of adrenal insufficiency other than dehydration. The patient was started on hydration and allopurinol treatments. During follow-up, urea and creatinine levels decreased to normal limits. The patient's basal cortisol was 12.02 µg/dL and ACTH was 83.8ng/L. Low-dose (1µg) ACTH test was performed on the patient, for whom chemotherapy was planned for his primary disease, with the preliminary diagnosis of adrenal insufficiency. The patient's cortisol levels were detected as 8.23-10.35-8.93-9.75 µg/dL at 30-60-90 and 120 minutes, respectively, and hydrocortisone treatment was started with the diagnosis of adrenal insufficiency. During the follow-up of the patient, R-CHOP chemotherapy was started, and since the patient had prednisolone in the chemotherapy course, hydrocortisone was discontinued during chemotherapy and isolated prednisolone treatment was given. Central Nervous System involvement was detected in the cerebrospinal fluid during intrathecal (IT) chemotherapy (Mtx, Dexamethasone, Cytosine Arabinosine). Intrathecal therapy was initially administered 3 times a week and subsequently twice a week. Since no cells were detected in the cytocentrifuge, intrathecal chemotherapy was given 4 times. After 6 cycles of R-CHOP chemotherapy, the patient underwent Autologous peripheral stem cell transplantation because of high risk disease in December 2023. The patient, whose general condition is good during follow-up, is currently being followed in remission under replacement dose hydrocortisone treatment.

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CiteScore
2.40
自引率
4.80%
发文量
1419
审稿时长
30 weeks
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