Bale P M, Parsons R E, Stevens M M
Hum Pathol. 1983 Jul;14(7):596-611. doi: 10.1016/s0046-8177(83)80203-2.
The main problems in the diagnosis of rhabdomyosarcoma are 1) distinction of undifferentiated examples from other small cell malignancies, especially soft-tissue Ewing's tumor and lymphoma; 2) distinction of spindling examples from fibrosarcoma, leiomyosarcoma, malignant fibrous histiocytoma, polyhistioma, and other sarcomas; 3) recognition of minimal criteria on small samples such as needle biopsy specimens or frozen sections; and 4) recognition of rhabdomyosarcoma in uncommon sites such as bone (mandible), perineum, retroperitoneum, and chest. In 95 pediatric cases diagnosed and treated at Royal Alexandria Hospital for Children--45 after the introduction of combined therapy--minimal diagnostic criteria were assessed. Cross-striations were found in only one third of cases; longitudinal myofibrils were more common and more helpful. There was much overlap between histologic types, and the microscopic patterns had little bearing on prognoses in preadolescent children. Fourteen cases could not be further differentiated ("embryonal sarcoma, probably rhabdomyosarcoma")--nine small-cell tumors; four tumors from genitourinary tract or head for which very small biopsy specimens were available, and one spindling retroperitoneal neoplasm. In all, slight evidence suggested embryonal rhabdomyosarcoma; this evidence included oat-shaped nuclei and, in a few cells, deeply eosinophilic cytoplasm, small elongated processes, or myxoid or alveolar foci--features that exclude lymphoma and Ewing's tumor. In six cases that were originally classified as poorly differentiated or undifferentiated, later material confirmed the presence of rhabdomyosarcoma by showing a predominantly well-differentiated (pleomorphic) or alveolar pattern after therapy. In 14 remaining undifferentiated cases, immunoperoxidase staining with antihuman-myoglobin serum was positive in five. With combined therapy there was 100 per cent survival among patients with paratesticular, limb, and stage I and stage II tumors; considerably improved survival among patients with head and neck, pelvic, and stage III tumors; and 100 per cent mortality among patients with intra-abdominal and stage IV tumors.
1）将未分化的病例与其他小细胞恶性肿瘤区分开来，尤其是软组织尤因肉瘤和淋巴瘤；2）将梭形的病例与纤维肉瘤、平滑肌肉瘤、恶性纤维组织细胞瘤、多组织细胞瘤及其他肉瘤区分开来；3）在小样本（如针吸活检标本或冰冻切片）中识别最低诊断标准；4）在不常见部位（如骨（下颌骨）、会阴、腹膜后和胸部）识别横纹肌肉瘤。在皇家亚历山德拉儿童医院诊断并治疗的95例儿科病例中——45例是在联合治疗引入后——评估了最低诊断标准。仅三分之一的病例发现有横纹；纵向肌原纤维更常见且更有帮助。组织学类型之间有很大重叠，显微镜下的模式对青春期前儿童的预后影响很小。14例无法进一步区分（“胚胎性肉瘤，可能为横纹肌肉瘤”）——9例小细胞肿瘤；4例来自泌尿生殖道或头部的肿瘤，活检标本非常小，还有1例梭形腹膜后肿瘤。总体而言，有轻微证据提示为胚胎性横纹肌肉瘤；这些证据包括燕麦形核，以及少数细胞中嗜酸性深染的细胞质、小的细长突起或黏液样或肺泡样病灶——这些特征可排除淋巴瘤和尤因肉瘤。在最初被归类为低分化或未分化的6例病例中，后来的材料通过显示治疗后主要为高分化（多形性）或肺泡样模式，证实了横纹肌肉瘤的存在。在其余14例未分化病例中，用抗人肌红蛋白血清进行免疫过氧化物酶染色，5例呈阳性。采用联合治疗，睾丸旁、肢体以及I期和II期肿瘤患者的生存率为100%；头颈部、盆腔和III期肿瘤患者的生存率有显著提高；腹腔内和IV期肿瘤患者的死亡率为100%。
