Ohsawa M, Tomita Y, Hashimoto M, Yasunaga Y, Kanno H, Aozasa K
Department of Pathology, Osaka University Medical School, Japan.
Mod Pathol. 1996 May;9(5):534-43.
Initial manifestation of malignant lymphoma in the adrenal gland is a rare event, and clinical and pathologic features are not fully understood. We conducted a nationwide study in Japan, and 20 patients with malignant lymphoma that showed initial and main manifestation in the adrenal gland were identified. Clinical and pathologic findings were summarized. In addition, the presence of the Epstein-Barr virus (EBV) genome in the tumor cells was examined by using polymerase chain reaction (PCR) and in situ hybridization (ISH), together with the immunohistochemical evaluation of the expression of latent membrane protein-1 (LMP-1). There were 13 men and seven women; their ages at admission ranged from 40 to 87 years (median, 65 yr). Fever, anemia, and elevation of lactic dehydrogenase levels were the common presenting findings. One patient had acquired immunodeficiency syndrome. Adrenal tumors were bilateral in 15 patients and unilateral (all in the left site) in five. Prognosis was very poor; all but two patients died within 1 year after admission. Histologically diffuse large cell type was the commonest type (14 specimens). Immunohistologically, 16 specimens were B-cell type, and one was T-cell type. Another three specimens showing no positive reaction for any antibodies were also judged as B-cell type on purely morphologic grounds. Prominent intravascular proliferation of tumor cells was found in five patients. PCR for EBV genomes gave positive results in five patients; the virus was subtyped as A in three patients and as B in two. The ISH provided positive signals in nine samples, including all five specimens positive for PCR. Four of the nine cases with detectable EBV by PCR and/or ISH expressed LMP-1. The present study shows that adrenal lymphoma is EBV associated and has a B-cell phenotype.
肾上腺恶性淋巴瘤的初始表现较为罕见，其临床和病理特征尚未完全明确。我们在日本开展了一项全国性研究，共识别出20例以肾上腺为初始及主要表现部位的恶性淋巴瘤患者。现将临床和病理结果进行总结。此外，我们还通过聚合酶链反应（PCR）和原位杂交（ISH）检测肿瘤细胞中EB病毒（EBV）基因组的存在情况，并对潜伏膜蛋白-1（LMP-1）的表达进行免疫组化评估。患者中男性13例，女性7例；入院时年龄在40至87岁之间（中位数为65岁）。发热、贫血和乳酸脱氢酶水平升高是常见的临床表现。1例患者患有获得性免疫缺陷综合征。15例患者的肾上腺肿瘤为双侧性，5例为单侧性（均位于左侧）。预后非常差；除2例患者外，所有患者在入院后1年内死亡。组织学上，弥漫大细胞型最为常见（14例标本）。免疫组化结果显示，16例标本为B细胞型，1例为T细胞型。另外3例对任何抗体均无阳性反应的标本，仅基于形态学判断也被判定为B细胞型。5例患者肿瘤细胞出现明显的血管内增殖。EBV基因组PCR检测结果显示5例患者呈阳性；其中3例患者的病毒亚型为A，2例为B。ISH检测在9个样本中呈阳性信号，包括所有5例PCR检测阳性的标本。在9例通过PCR和/或ISH检测可检测到EBV的病例中，有4例表达LMP-1。本研究表明，肾上腺淋巴瘤与EBV相关，具有B细胞表型。
