Lin Yu-Wei, Hsu Yu-Hsin, Lee Ming-Yuan
Department of Pathology and Laboratory Medicine, Koo Foundation Sun Yat-Sen Cancer Center, Taipei, TWN.
Cureus. 2022 Aug 8;14(8):e27769. doi: 10.7759/cureus.27769. eCollection 2022 Aug.
Neuroblastoma (NB) is the most common extra-cranial cancer of early childhood and rarely occurs in adults. The clinical symptoms of NB can be diverse. We discuss a rare case of an adult NB presenting as acute leukemia. A 45-year-old woman presented with persistent hip pain, weight loss, anemia, and incidental fever for several months. Imaging studies showed diffuse bone marrow (BM) uptake and hypermetabolic lesions involving the left adrenal gland, bilateral axillary nodes, and left lateral aspect of the abdomen. Her 24-hour urine catecholamines were within the normal range. On the peripheral blood film, blast-like cells were noted, occupying approximately 2% of leukocytes. The BM imprints showed infiltration of blast-like cells with convoluted nuclei and scant cytoplasm in more than 85% of the total nucleated cells. Acute leukemia was initially suspected based on morphology. Blast-like cells were negative for myeloperoxidase, combined esterase, periodic acid-Schiff, CD45RB, CD68, and CD138. In a further study, these cells were positive for CD56, synaptophysin (SYN), and CD99 with negativity for desmin, myogenin, NKX-2.2, CD31, cytokeratin (AE1/AE3), Melan-A, ERG, S-100, and SALL4. Morphologically similar neoplastic cells in axillary node biopsy were positive for CD56, chromogranin A, SYN, and neurofilament, but negative for GFAP, CD246, and vimentin. Based on laboratory, pathological, and imaging studies, metastatic NB with BM and multifocal involvement was diagnosed. The differential diagnosis of metastatic small blue round cell tumors should be considered for adult patients with circulating blast-like cells, and an accurate diagnosis would enable the patient to receive appropriate and timely treatment.
神经母细胞瘤(NB)是幼儿期最常见的颅外癌症,在成人中很少发生。NB的临床症状可能多种多样。我们讨论一例罕见的成人NB表现为急性白血病的病例。一名45岁女性出现持续髋部疼痛、体重减轻、贫血和偶发发热数月。影像学检查显示弥漫性骨髓(BM)摄取以及累及左肾上腺、双侧腋窝淋巴结和腹部左侧的高代谢病变。她的24小时尿儿茶酚胺在正常范围内。在外周血涂片上,发现有原始样细胞,约占白细胞的2%。骨髓印片显示超过85%的有核细胞中有核呈卷曲状、胞质稀少的原始样细胞浸润。基于形态学最初怀疑为急性白血病。原始样细胞髓过氧化物酶、联合酯酶、过碘酸 - 希夫反应、CD45RB、CD68和CD138均为阴性。在进一步研究中,这些细胞CD56、突触素(SYN)和CD99呈阳性,而结蛋白、生肌调节因子、NKX - 2.2、CD31、细胞角蛋白(AE1/AE3)、Melan - A、ERG、S - 100和SALL4呈阴性。腋窝淋巴结活检中形态学相似的肿瘤细胞CD56、嗜铬粒蛋白A、SYN和神经丝呈阳性,但GFAP、CD246和波形蛋白呈阴性。基于实验室、病理和影像学研究,诊断为伴有骨髓和多灶性受累的转移性NB。对于有循环原始样细胞的成年患者,应考虑转移性小蓝圆细胞肿瘤的鉴别诊断,准确诊断将使患者能够接受适当及时的治疗。