Primary bilateral adrenal lymphoma masquerading as a metastatic melanoma: An unusual presentation of a rare disease

J. Stephen Stalls , Anas Mohamed , Masood Hassan , Kim Geisinger , Anuradha Kanungo , Moiz Vora , Anokhi Patel , Andrew Weil
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Abstract

This report describes a 70-year-old male with bilateral primary adrenal lymphoma (PAL) characterized as non-germinal center diffuse large B-cell lymphoma (DLBCL). PAL is a very rare, aggressive disease with a poor prognosis. Our patient presented with B symptoms, chills, and nausea. Imaging studies of his abdomen revealed rapidly enlarging bilateral adrenal masses. Computed tomography (CT) guided left adrenal mass core biopsy showed diffuse sheets of neoplastic cells with irregular nuclear contours, vesicular to hyperchromatic chromatin, and prominent nucleoli. The neoplastic cells demonstrated an immunohistochemistry (IHC) profile consistent with DLBCL. Markers assessing for melanoma and neuroendocrine tumors were negative. Fluorescence in situ hybridization (FISH) revealed BCL6 rearrangement. The diagnosis of primary adrenal DLBCL, non-germinal center subtype, was rendered. The patient’s chemotherapy is ongoing; six cycles of R-CHOP chemotherapy (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) induced significant clinical and radiological response.

The differential diagnosis is broad in patients with adrenal insufficiency and bilateral adrenal masses, and our case clinically centered on metastatic melanoma in the differential. Thorough pathologic evaluation of tissue, including an extensive IHC panel, was warranted for this patient due to the rarity of PAL and due to the morphologic overlap between melanoma and DLBCL.

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伪装成转移性黑色素瘤的原发性双侧肾上腺淋巴瘤:罕见疾病的不寻常表现
本文报告一例70岁男性双侧原发性肾上腺淋巴瘤(PAL),表现为非生发中心弥漫性大b细胞淋巴瘤(DLBCL)。PAL是一种非常罕见的侵袭性疾病,预后不良。我们的病人表现为B型症状,寒战和恶心。腹部影像学检查显示双侧肾上腺肿物迅速增大。计算机断层扫描(CT)引导左侧肾上腺肿块核心活检显示弥漫性肿瘤细胞片,核轮廓不规则,染色质呈泡状至深染,核仁突出。肿瘤细胞表现出与DLBCL一致的免疫组化(IHC)特征。评估黑色素瘤和神经内分泌肿瘤的标志物为阴性。荧光原位杂交(FISH)显示BCL6重排。诊断原发性肾上腺大细胞淋巴瘤,非生发中心亚型。病人的化疗仍在进行中;6个周期的R-CHOP化疗(利妥昔单抗、环磷酰胺、阿霉素、长春新碱和强的松)诱导了显著的临床和放射反应。肾上腺功能不全和双侧肾上腺肿块的鉴别诊断是广泛的,我们的病例临床集中在转移性黑色素瘤的鉴别诊断上。由于PAL的罕见性和黑色素瘤与DLBCL之间的形态学重叠,该患者需要对组织进行彻底的病理评估,包括广泛的免疫组化检查。
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来源期刊
Human Pathology: Case Reports
Human Pathology: Case Reports Medicine-Pathology and Forensic Medicine
CiteScore
0.50
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0.00%
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0
审稿时长
16 weeks
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