Argani P, Erlandson R A, Rosai J
Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, New York 10021, USA.
Am J Clin Pathol. 1997 Nov;108(5):537-43. doi: 10.1093/ajcp/108.5.537.
We report three cases of neuroblastoma arising within the thymus of elderly patients. All tumors consisted of primitive neuroblasts showing focal gangliocytic differentiation within nests of neuropil. All stained for neuroendocrine markers but were negative for cytokeratins and for the MIC2 gene product. One tumor was associated with the syndrome of inappropriate secretion of antidiuretic hormone, an endocrinopathy we found in three of five case reports of thymic neuroblastoma in adults. Immunohistochemical stains confirmed production of antidiuretic hormone by this tumor. One patient died of progressive disease, one patient is disease free at 18 months, and the other patient died of unrelated causes, a spectrum that reflects the variable clinical behavior others have reported. The possible histogenesis of these purely neural tumors includes malignant transformation of a mediastinal teratoma, aberrantly located sympathetic ganglia, neuroectodermal cells native to the normal thymus, and precursors of thymic epithelial cells that have differentiated along neural lines.
我们报告了3例发生于老年患者胸腺的神经母细胞瘤病例。所有肿瘤均由原始神经母细胞组成，在神经毡巢内可见局灶性神经节细胞分化。所有肿瘤均对神经内分泌标志物呈阳性染色，但细胞角蛋白和MIC2基因产物呈阴性。1例肿瘤与抗利尿激素分泌不当综合征相关，在成人胸腺神经母细胞瘤的5例病例报告中，我们发现有3例存在这种内分泌病。免疫组织化学染色证实该肿瘤可产生抗利尿激素。1例患者死于疾病进展，1例患者在18个月时无疾病，另1例患者死于无关原因，这一系列情况反映了其他人所报告的不同临床行为。这些纯神经源性肿瘤的可能组织发生包括纵隔畸胎瘤的恶性转化、位置异常的交感神经节、正常胸腺固有的神经外胚层细胞以及沿神经谱系分化的胸腺上皮细胞前体。
