Malignant neoplasms arising primarily in the spleen are found so infrequently as to be considered pathologic curiosities and so have been of little interest to the clinician. Textbooks of pathology and surgery merely mention the fact that they do occur. The largest group previously reported consists of thirty-two cases, which would lead one to infer that this disease is practically as rare as Gaucher's primary splenomegaly; but search of the literature shows a total of about 100 cases of malignant tumors. In more than 40,000 surgical cases at the Boston City Hospital, there occurred but two primary malignant tumors of the spleen. At the Massachusetts General Hospital, the diagnosis of primary sarcoma of the spleen has been made in four cases. The first came to operation and was reported by Warren. In the other three instances, the diagnosis was made by Cabot and others on the basis of clinical findings
SMITH CE, RUSK GY. ENDOTHELIOMA OF THE SPLEEN: A STUDY OF TWO CASES, WITH REVIEW OF THE LITERATURE OF PRIMARY MALIGNANCY OF THE SPLEEN. Arch Surg. 1923;7(2):371–414. doi:10.1001/archsurg.1923.01120020137009
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