Sotelo-Avila C, Gonzalez-Crussi F, deMello D, Vogler C, Gooch W M, Gale G, Pena R
Semin Diagn Pathol. 1986 May;3(2):151-63.
The clinical and pathologic features of 14 children with rhabdoid tumors are presented. Eight patients had primary renal neoplasms and six had extrarenal tumors. The eight renal rhabdoid tumors were identified among 514 primary renal neoplasms collected at four pediatric institutions. Six patients were under 1 year of age; five children died of tumor-related causes, four of them within 4 months of diagnosis and one 17 months postnephrectomy. Another patient died of sepsis 12 days postnephrectomy. One is alive 13 months postnephrectomy, and one was lost to follow-up evaluation. The most common sites of metastasis were the lymph nodes (seven children) and the lungs (three patients). Three infants with renal rhabdoid tumors had, in addition, intracranial masses, two of which manifested clinically before the detection of the renal tumors, in one confirmed to be a primitive neuroectodermal tumor. Five of the 6 extrarenal tumors were identified among 155,926 surgical pathology specimens examined in the same children's hospitals over the same period of time; the remaining extrarenal rhabdoid tumor was received in consultation from a community hospital. The extrarenal rhabdoid tumors occurred in the dorsum of the right foot, liver, soft tissue of the right chest wall, left temporal lobe, left leg, and left thoracic paraspinal region. The ages ranged from 6 weeks to 15 years and two months. Three patients died of tumor-related causes within 4 months of diagnosis; one was a term stillborn. Two are alive, 1 month and 70 months postdiagnosis. Common sites for metastases included the lungs (three patients), and liver and lymph nodes (two children each). Patients with renal and extrarenal rhabdoid tumors are of similar age, have a similar clinical course, with early metastases and poor response to therapy. Primitive neuroectodermal intracranial tumors have been identified in several reported patients with renal rhabdoid tumors; similar brain tumors have not been documented in patients with extrarenal rhabdoid tumors. The histogenesis of this tumor remains unknown.
本文介绍了14例横纹肌样瘤患儿的临床和病理特征。8例患者患有原发性肾肿瘤，6例患有肾外肿瘤。8例肾横纹肌样瘤是在4家儿科机构收集的514例原发性肾肿瘤中发现的。6例患者年龄在1岁以下；5名儿童死于肿瘤相关原因，其中4例在诊断后4个月内死亡，1例在肾切除术后17个月死亡。另1例患者在肾切除术后12天死于败血症。1例患者在肾切除术后13个月存活，1例失访。最常见的转移部位是淋巴结（7例患儿）和肺（3例患者）。3例患有肾横纹肌样瘤的婴儿还患有颅内肿块，其中2例在肾肿瘤被发现之前就有临床症状，1例经证实为原始神经外胚层肿瘤。6例肾外肿瘤中的5例是在同一儿童医院同期检查的155926份外科病理标本中发现的；其余1例肾外横纹肌样瘤是从一家社区医院会诊获得的。肾外横纹肌样瘤发生于右足背、肝脏、右胸壁软组织、左颞叶、左腿和左胸段脊柱旁区域。年龄范围从6周至15岁零2个月。3例患者在诊断后4个月内死于肿瘤相关原因；1例为足月死产儿。2例存活，诊断后分别为1个月和70个月。常见的转移部位包括肺（3例患者）、肝脏和淋巴结（各2例患儿）。患有肾和肾外横纹肌样瘤的患者年龄相似，临床病程相似，早期转移且对治疗反应不佳。在几例报告的肾横纹肌样瘤患者中已发现原始神经外胚层颅内肿瘤；肾外横纹肌样瘤患者中尚未记录到类似的脑肿瘤。该肿瘤的组织发生仍然未知。
