Al Meshaan Mohd Khaled, Nayef Marwan, Kwaider Talal, Otto Wolfgang, Katchy Ken C
J Med Case Rep. 2009 Apr 29;3:6840. doi: 10.1186/1752-1947-3-6840.
Peripheral primitive neuroectodermal tumors of the urinary bladder are rare and tend to occur in an older age group than do their counterparts in bones and soft tissue.
We report a case of peripheral primitive neuroectodermal tumor of the urinary bladder in a 67-year-old woman of Arab origin. She had undergone transurethral resection followed by chemotherapy because of pulmonary metastasized muscle-invasive squamous cell carcinoma of the bladder in 2005. One year later, she first presented with a history of repeated hematuria in our institution. Performing cystoscopy any tumor could be detected. Control cystoscopy two months later showed a tumor mass of 3 cm in diameter at another location than described for the first tumor. After perforating by transurethral resection partial bladder resection had to be done. Tissue specimen after pathological analysis revealed a peripheral primitive neuroectodermal tumor with tumor cells reactive to cluster of differentiation 99, neuron-specific enolase and S100 protein and stained negative for other markers such as cytokeratins, epithelial membrane antigen, desmin, smooth muscle actin, chromogranin and leucocyte common antigen. Staging computerized tomography was especially free from any hint on organ metastasis, but the patient died due to a cardiac problem only a few months later.
To the best of our knowledge, we report the eighth case of bladder peripheral primitive neuroectodermal tumors in literature and the first concerning an Arab patient. It is also the first presentation of a peripheral primitive neuroectodermal tumor patient with a history of squamous cell carcinoma of the bladder. As in other cases, expression of single-chain-type 1 glycoprotein and neural markers was positive and the disease was at an advanced stage at the time of diagnosis.
膀胱外周原始神经外胚层肿瘤罕见，与骨骼和软组织中的同类肿瘤相比，发病年龄往往更大。
我们报告一例膀胱外周原始神经外胚层肿瘤，患者为一名67岁的阿拉伯裔女性。2005年，她因膀胱肺转移肌层浸润性鳞状细胞癌接受了经尿道切除术，随后进行化疗。一年后，她首次因反复血尿前来我院就诊。膀胱镜检查未发现任何肿瘤。两个月后的复查膀胱镜检查显示，在与第一个肿瘤不同的位置有一个直径3厘米的肿瘤肿块。经尿道切除穿孔后，不得不进行部分膀胱切除术。病理分析后的组织标本显示为外周原始神经外胚层肿瘤，肿瘤细胞对分化簇99、神经元特异性烯醇化酶和S100蛋白呈反应性，而对其他标志物如细胞角蛋白、上皮膜抗原、结蛋白、平滑肌肌动蛋白、嗜铬粒蛋白和白细胞共同抗原染色为阴性。分期计算机断层扫描未发现任何器官转移迹象，但患者仅几个月后就因心脏问题死亡。
据我们所知，我们报告了文献中第八例膀胱外周原始神经外胚层肿瘤病例，也是首例涉及阿拉伯患者的病例。这也是首例有膀胱鳞状细胞癌病史的外周原始神经外胚层肿瘤患者。与其他病例一样，单链1型糖蛋白和神经标志物的表达呈阳性，且在诊断时疾病已处于晚期。
