Henderson D W, Raven J L, Pollard J A, Walters M N
Pathology. 1976 Oct;8(4):329-41. doi: 10.3109/00313027609101496.
A case of desseminated alveolar rhabdomyosarcoma in an 18-year-old male with leuco-erythroblastic anaemia is described. Numerous bizarre malignant cells, including frequent multinucleated giant cells, were seen in bone marrow aspirates, and osteolytic lesions appeared late in the clinical course. The primary site of the neoplasm remained undertermined during life and also at necropsy, which revealed minute pulmonary metastases and extensive lymph nodal, pleural and skeletal deposits. The diagnosis was confirmed on necropsy tissue by ultrastructural examination which demonstrated numerous thin (5 nm) and thick (15 nm) intracytoplasmic filaments in tumour cells, sometimes organized in bundles; scattered dense Z-band-like bodies, and rod-shaped structures were also seen. The fine structure of the rhabdomyosarcoma in the present case is compared with previous ultrastructural studies. Elongated, thick intracytoplasmic filaments whose diameter corresponds to that of myosin myofilaments are strong evidence for rhabdomyoblastic differentiation and are considered to be the sine qua non of a positive electron microscopic diagnosis of rhabdomyosarcoma. Orgaized bundles of filaments and Z-band-like dense bodies are usually present, and rod-shaped structures are found infrequently, but none of these are necessary for the ultrastructural diagnosis.
本文描述了一例18岁男性播散性肺泡横纹肌肉瘤合并幼粒 - 幼红细胞性贫血的病例。在骨髓穿刺物中可见大量形态怪异的恶性细胞，包括常见的多核巨细胞，溶骨性病变在临床病程后期出现。肿瘤的原发部位在生前及尸检时均未明确，尸检发现有微小的肺转移以及广泛的淋巴结、胸膜和骨骼转移。通过超微结构检查在尸检组织中确诊，结果显示肿瘤细胞内有大量细（5纳米）、粗（15纳米）的胞质丝，有时成束排列；还可见散在的致密Z带样小体及杆状结构。将本病例横纹肌肉瘤的精细结构与以往的超微结构研究进行了比较。直径与肌球蛋白肌丝相当的伸长、粗大胞质丝是横纹肌母细胞分化的有力证据，被认为是横纹肌肉瘤电镜阳性诊断的必要条件。通常存在成束的细丝和Z带样致密小体，杆状结构较少见，但这些对于超微结构诊断都不是必需的。
