Mouton S C, Rosenberg H S, Cohen M C, Drut R, Emms M, Kaschula R O
Department of Pathology, Red Cross Children's Hospital, Rondebosch, Cape Town, South Africa.
Pediatr Pathol Lab Med. 1996 Jul-Aug;16(4):607-24.
Five childhood malignant ectomesenchymomas are reported from three centers in three countries. The children were all younger than 3 years (four less than 12 months), four were boys, and four tumors were sited in the pelvis or external genitalia. All tumors had distinctive light microscopic features of rhabdomyosarcoma and three also demonstrated small numbers of included neuronal cells. Immunohistochemical studies and transmission electron microscopy revealed the additional presence of neurogenic components in the remaining two patients. An additional epithelial component was found by immunohistochemistry in one tumor, which suggests a pluripotential origin that still requires karyotypic investigation. Aggressive chemotherapy and adequate surgical excision have thus far been effective in providing disease-free follow-up for periods of 7 to 50 months. It is implied that because the biological behavior and morphology closely resemble those of rhabdomyosarcoma and because the neurogenic component is often inconspicuous, many malignant ectomesenchymomas may be misdiagnosed as rhabdomyosarcomas.
来自三个国家三个中心的报告称有5例儿童恶性外胚层间叶瘤。这些儿童均小于3岁（4例小于12个月），4例为男孩，4例肿瘤位于骨盆或外生殖器。所有肿瘤均具有横纹肌肉瘤独特的光镜特征，3例还显示含有少量神经细胞。免疫组化研究和透射电镜显示，其余2例患者还存在神经源性成分。免疫组化在1例肿瘤中发现了额外的上皮成分，提示其具有多能性起源，仍需进行核型研究。积极的化疗和充分的手术切除迄今已有效地实现了7至50个月的无病随访。这意味着，由于其生物学行为和形态与横纹肌肉瘤极为相似，且神经源性成分往往不明显，许多恶性外胚层间叶瘤可能被误诊为横纹肌肉瘤。
