Papageorgiou Sotirios G, Mavroeidi Ioanna, Kostakis Marios, Spathis Aris, Leventakou Danai, Kritikou Evangelia, Oikonomopoulos Nikolaos, Kourkouti Chrysoula, Krania Maria, Bouchla Anthi, Thomopoulos Thomas, Tsakiraki Zoi, Markakis Konstantinos, Panayiotides Ioannis G, Thomaidis Nikolaos, Pappa Vasiliki, Foukas Periklis G, Peppa Melpomeni
Hematology Unit, Second Propaedeutic Department of Internal Medicine and Research Institute, University General Hospital "Attikon", School of Medicine, National and Kapodistrian University of Athens, 12462 Athens, Greece.
Endocrine Unit, Second Propaedeutic Department of Internal Medicine and Research Institute, University General Hospital "Attikon", School of Medicine, National and Kapodistrian University of Athens, 12462 Athens, Greece.
J Clin Med. 2023 Jul 31;12(15):5032. doi: 10.3390/jcm12155032.
Primary adrenal lymphoma (PAL) is a rare entity that presents as unilateral or bilateral rapidly growing adrenal masses, with signs and symptoms most commonly related to adrenal insufficiency due to the mass effect on the surrounding tissues. Although steroeidogenesis has not been previously described in PAL, we herein report two cases of PAL presenting as adrenal incidentalomas (AIs) that demonstrated autonomous cortisol production. A 52-year-old woman presented with lumbar pain; a computed tomography (CT) scan demonstrated a left AI measuring 8.5 × 15 × 10 cm. Similarly, an 80-year-old woman presented with lumbar pain, demonstrating in a CT scan a bilateral AI (right: 9 × 6.5 cm, left: 3.6 × 3.2 cm). Both cases underwent a full hormonal evaluation according to the algorithm for the investigation of AIs, demonstrating increased 24-h cortisol excretion, suppressed fasting adrenocorticotropic hormone (ACTH) levels, and non-suppressed serum cortisol levels in both the overnight and the low-dose dexamethasone suppression tests, indicating autonomous cortisol secretion and Cushing's syndrome. In a relatively short time, both patients developed night sweats, and their clinical picture deteriorated, while the CT scans showed increased dimensions of the masses with radiological characteristics compatible to lymphoma. Both patients underwent ultrasound-guided biopsies (FNBs), revealing infiltration of the left adrenal by diffuse large B-cell lymphoma in the first case, whereas bilateral adrenal infiltration from the same histological type was noted in the second case. Subsequently, they were treated with immunochemotherapy, but the second patient died from an infection shortly after the initiation of the treatment. To our knowledge, this is the first report of PAL presenting with Cushing's syndrome due to autonomous cortisol production, indicating that neoplastic lymphoid cells in PAL might acquire the potential for steroidogenesis; therefore, more cases of PAL should be analyzed so as to further elucidate the complex pathogenesis and the natural course of this entity.
原发性肾上腺淋巴瘤（PAL）是一种罕见的疾病，表现为单侧或双侧迅速增大的肾上腺肿块，其体征和症状最常与肿块对周围组织的压迫导致肾上腺功能不全有关。虽然此前尚未在PAL中描述过类固醇生成，但我们在此报告两例表现为肾上腺偶发瘤（AI）的PAL病例，这些病例显示出自主性皮质醇分泌。一名52岁女性因腰痛就诊；计算机断层扫描（CT）显示左肾上腺偶发瘤，大小为8.5×15×10 cm。同样，一名80岁女性因腰痛就诊，CT扫描显示双侧肾上腺偶发瘤（右侧：9×6.5 cm，左侧：3.6×3.2 cm）。两例均根据肾上腺偶发瘤的检查算法进行了全面的激素评估，结果显示24小时皮质醇排泄增加、空腹促肾上腺皮质激素（ACTH）水平降低，过夜及小剂量地塞米松抑制试验中血清皮质醇水平未被抑制，提示自主性皮质醇分泌及库欣综合征。在相对较短的时间内，两名患者均出现盗汗，临床症状恶化，而CT扫描显示肿块尺寸增大，具有与淋巴瘤相符的影像学特征。两名患者均接受了超声引导下活检（细针穿刺活检），第一例显示左肾上腺被弥漫性大B细胞淋巴瘤浸润，而第二例则发现双侧肾上腺均有相同组织学类型的浸润。随后，他们接受了免疫化疗，但第二名患者在治疗开始后不久因感染死亡。据我们所知，这是首例因自主性皮质醇分泌导致库欣综合征的PAL报告，表明PAL中的肿瘤性淋巴细胞可能获得了类固醇生成的能力；因此，应分析更多PAL病例，以进一步阐明该疾病复杂的发病机制和自然病程。
