This article reviews the pathologic features and the behavior of 39 small, round, or oval cell sarcomas occurring in the soft tissues and considered histologically indistinguishable from Ewing's sarcoma of bone. The tumors affected chiefly young adults (median age 20 years) and most commonly involved the soft tissues of the lower extremity and the paravertebral region. Microscopically, they consisted of solidly packed small, round, or ovoid cells of great uniformity, arranged in sheets or lobules separated by strands of fibrous connective tissue. The nucleus of the tumor cells contained finely divided chromatin, a distinct nuclear membrane, and frequently a minute nucleolus. The scanty ill-defined cytoplasm contained varying amounts of glycogen. Sometimes the histologic picture was dominated by a "peritheliomatous" pattern, or by large areas of necrosis or hemorrhage. Followup data ranging from 1 month to 14 years were available in 35 of the 39 cases (93%). Of these, 13 were alive and 22 had died. In the majority of the fatal cases, the clinical course was rapid; metastatic lesions developed within a few months after the primary tumor was excised. The lungs and the skeleton were the two most common sites of metastasis. Cure may be achieved by wide local excision of the tumor at an early stage of the disease, combined with radiation therapy and chemotherapy.
Ninety-seven patients who had received their primary and definitive surgical treatment for a soft-tissue sarcoma during the years 1956--1976 were studied with respect to local recurrence, metastasis, and survival. The aim of the treatment was to eradicate the tumor while preserving good function. The influence of host and tumor properties and different diagnostic and surgical procedures on the prognosis was studied by means of nonparametric multivariate analysis. Ninety-four percent of the tumors were located in the extremities. Malignant fibrous histiocytoma was the most common histologic type. A four-grade scale of histologic malignancy was used. Eighty-eight percent of the tumors were Grade III or IV. In 85% of the patients with an extremity lesion, local extirpation was carried out. The overall local recurrence rate was 21.7% and in 76 patients who underwent an adequate surgical procedure it was 6.6%. The overall five-year survival rate was 59%. The survival depended on the local control of the primary tumor, which was related to the adequacy of the surgical procedure and to the histologic grade of malignancy. No patient with a Grade I or II tumor died. The five-year survival rate for patients with Grade III tumors was 68% and for patients with Grade IV tumors 47%.
Summary. Focal muscular degeneration in two diabetics is described. In both instances the muscular lesion was excised on the grounds of clinical suspicion of tumour. The gross and microscopical pictures agreed with those of muscular degeneration following ischaemia. Accordingly, the vascular lesions that were demonstrated, diabetic mieroangiopathy and arteriosclerosis, m a y have been of importance in the pathogenesis.Rdsumd. Une ddgdndreseence museulaire locale a 6t@ observde ehez deux diabdtiques. Dans les deux eas la ldsion musculaire a @t@ excis@e, une tumeur ayant dt6 eonsid@r@e possible. L'apparence maeroscopique et mieroscopique dtait celle d'une dggdndrescence museulaire cons@-cutive ~ une isch@mie. Nous avons done eonsid6r6 que les ldsions vasculah'es ddmontr@es, microangiopathie diabgtique et art@rioseldrose, ont probablement eontribud ~ la pathog6n~se de ces d6gdn6reseences musculaires.Zusammenfassung. Bei zwei Diabetikern werden umsehriebene muskulgre Degenerationsherde gesehildert. In beiden F/illen wurden die krankhaft ver/~nderten Muskelgebiete wegen des klinisehen Verdaehtes auf Tumor exzidiert. Die makro-und mikroskopischen Befunde waren die gleiehen wie bei Muskeldegeneration nach Iseh~mie. Demnaeh waren fiir die Pathogenese m6glieherweise die naehgewiesenen Gef~13ver/inderungen, die diabetische Mikroangiopathie und Arteriosklerose, yon Bedeutung.
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