BONE SARCOMA IN POLYOSTOTIC FIBROUS DYSPLASIA
BONE SARCOMA IN POLYOSTOTIC FIBROUS DYSPLASIA
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DOI：
10.1097/00000658-194506000-00012
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发表时间：
1945-01-01
影响因子：
9
通讯作者：
STEWART, FW
中科院分区：
文献类型：
--
作者：
COLEY, BL;STEWART, FW
在过去的十年里。现在通常被称为骨纤维异常增殖症的综合征已经越来越被认识到。在1934年，Goldhamerl发现了一种疾病，他将其描述为单侧纤维性骨营养不良伴早发性青春期，同年，Borak和Doll 2描述了他们所称的单侧雷克林豪森骨病伴早发性青春期。在这个国家里，几乎同时出现了Lichelman的论文，3他使用了”多骨纤维性发育不良”这个术语，Albright，Butler，汉普顿和史密斯4以及Albright，Scoville和Sulkowitch 5，他们将这个过程定义为一种综合征，其特征是播散性纤维性骨炎，色素沉着和内分泌功能障碍，女性早熟。在1942年，Lichelman和Jaffe，6发表了大量病例的报告，除了骨骼病变外，还描述了他们的附属特征。本文作者无意再次描述该综合征。他们的唯一目的是将两例由骨纤维异常增殖症引起的恶性骨肿瘤记录在案。在此，据他们所知，这一事件还没有被描述，虽然案件无疑存在。在我们的第一个病例中，最初是在1929年发现的，尽管描述了特征性色素沉着，但很自然地没有认识到该综合征。直到大约十年后，这种情况下达到适当的临床病理分类。患者JW，编号40833，女性，42岁，立陶宛血统。她过去的经历平淡无奇。入院前5个月，她注意到左肩轻微僵硬。在接下来的一个月里，这种情况有所增加，并出现疼痛，尤其是在运动时。显然，她在第一次出现症状后大约两个月才注意到肩部肿胀。肿胀变得越来越严重，在进入纪念医院前两周，患者进入另一家医院，在那里做出恶性骨肿瘤的推定诊断，于是她被转诊到纪念医院。在入院前的两个月里，病人体重减轻了8磅，偶尔头痛，并主诉左前胸壁有烧灼感。初步检查的相关部分涉及局部情况和X线检查结果。左肩胛骨是一个大的肿瘤肿块，坚硬，圆形，无压痛，可能有些波动。整个肩区、前后胸壁为褐色色素沉着部位，在颈部向前和向后向上延伸。这种色素沉着基本上是单侧的，但略超过中线（图2和3）。I和2）。左臂活动明显受限;外展基本不存在，前位运动仅为中度。
WITHIN THE PAST DECADF. the syndrome now commonly designated as fibrous dysplasia of bone has become increasingly recognized. In I934, Goldhamerl recognized a disease which he characterized as osteodystrophia fibrosa unilateralis with pubertas praecox, and, in the same year, Borak and Doll2 described whatthey called unilateral Recklinghausen's disease of bone with pubertas praecox. Almost simultaneously in this country appeared papers by Lichtenstein, 3 who employed the term" polyostotic fibrous dysplasia," and by Albright, Butler, Hampton and Smith4 and Albright, Scoville and Sulkowitch, 5 who defined the process as a syndrome characterized by osteitis fibrosa disseminata, areas of pigmentation, and endocrine dys-function, with precocious pubertyin females. In 1942, Lichtenstein and Jaffe, 6 published an account of a large number of cases, withdescriptions of their accessory features in addition to the skeletal lesions. The present authors have no intention of again describing the syndronle. Their sole purpose is to place on record two instances of malignant bone tumor arising on the basis of fibrous dysplasia. Heretofore, so far as they know, this event has not been described, although cases doubtless exist. In our first case, initially seen in I929, the syndrome was, quite naturally, not recognized, although thecharacteristic pigmentation is described. Not until about ten years later did this case reach the proper clinicopathologic category.CASE REPORTS Case I.-The patient, JW, No. 40833, was a female, age 42, of Lithuanian ancestry. Her past history was uneventful. Five months prior to admission, she noted slight stiffness in her left shoulder. This increased during the following month and pain developed, especially on motion. Apparently, shefirst noted swelling in the shoulder region about two months after her first symptom. The swelling became pro-gressively worse andtwo weeks prior to admission to the Memorial Hospital, the patient entered another hospital where a presumptive diagnosis of malignant bone tumor was made, whereupon she was referred to Memorial. During the two months prior to admission, the patient had lost about eightpounds in weight, sufferedfrom occasional headaches and complained of a burning sensation over the left anterior chest wall. The pertinent part of the initial examination concerns the local condition and the radiographic findings. The left scapula was the site of a large tumor mass, firm, rounded, nontender, possibly somewhat fluctuant. The entire shoulder region, anterior and posterior chest walls were the site ofa brownish pigmentation which extended upward both anteriorly and posteriorly in the neck region. This pigmentation was essentially unilateral but reached slightly beyond the midline (Figs. I and 2). t There was marked limitation of motion of the left arm; abduction was essentially absent and anteropostero motions were but moderate.