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首页> 外文期刊>Modern Pathology >Epithelioid Leiomyosarcoma in a Non-Immunocompromised Infant: Additional Differential Diagnosis of Pediatric |[ldquo]|Round Cell Tumors|[rdquo]|
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Epithelioid Leiomyosarcoma in a Non-Immunocompromised Infant: Additional Differential Diagnosis of Pediatric |[ldquo]|Round Cell Tumors|[rdquo]|

机译：非免疫功能低下婴儿的上皮样平滑肌肉瘤：小儿| ldquo |圆细胞瘤| rdquo |的附加鉴别诊断

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We report an 18-month-old Japanese girl with purely epithelioid leiomyosarcoma presenting as a huge intraabdominal mass. The patient had been well from birth and had shown no signs of immunodeficiency. She was negative for human immunodeficiency virus. Blood examination revealed elevated serum neuron specific enolase (NSE). Histologically, the tumor was comprised of solid growths of round or polygonal cells with vesicular nuclei and often vacuolated cytoplasm rich in glycogen. The tumor cells were positive for vimentin, NSE, and MIC2, and were negative for desmin and neurofilament. The age, clinical presentation, and histologic findings mostly favored Ewing's sarcoma/primitive neuroectodermal tumor. Silver stain, however, demonstrated well-developed reticulin fibers often outlining individual tumor cells. An expanded panel of immunostains showed that the tumor cells were intensely positive for smooth muscle actin, and ultrastructural study revealed abundant fine cytoplasmic filaments with focal subsarcolemmal densities, various amounts of glycogen, and irregularly arranged, thick basal lamina. The diagnosis of epithelioid leiomyosarcoma was made. Following reduction in tumor size by chemotherapy, the serum NSE level was normalized. From the surgical finding, the primary site was presumed to be the urachus or the urinary bladder dome. Although extremely rare, epithelioid leiomyosarcoma should be added in the list of differential diagnoses of pediatric "round cell tumors."
机译：我们报道了一个18个月大的日本女孩，其上皮样平滑肌肉瘤呈巨大腹腔肿块。该患者从出生起就身体健康，没有免疫缺陷的迹象。她对人类免疫缺陷病毒阴性。血液检查发现血清神经元特异性烯醇化酶（NSE）升高。从组织学上讲，该肿瘤由圆形或多边形细胞的坚固生长组成，这些细胞具有囊泡核，并且常空泡化富含糖原的细胞质。肿瘤细胞中波形蛋白，NSE和MIC2呈阳性，而结蛋白和神经丝呈阴性。年龄，临床表现和组织学发现大多偏爱尤因肉瘤/原始神经外胚层肿瘤。然而，银染显示出成熟的网状蛋白纤维，通常勾勒出单个肿瘤细胞的轮廓。扩大的免疫染色组显示，肿瘤细胞对平滑肌肌动蛋白呈强阳性，超微结构研究显示丰富的细胞质细丝，具有局灶性次结膜下层密度，各种糖原和不规则排列的厚基底层。诊断为上皮样平滑肌肉瘤。通过化疗减少肿瘤大小后，血清NSE水平恢复正常。根据手术发现，推测主要部位是天疱疮或膀胱穹顶。尽管极少见，上皮样平滑肌肉瘤应加在小儿“圆形细胞肿瘤”的鉴别诊断中。

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