Primary Reticulum-Cell Sarcoma of the Spleen

Primary splenic neoplasms are of unusual occurrence (2). On reviewing 11,707 autopsies and 68,820 surgical specimens, Bostick (1) encountered only 7 cases. Such tumors are derived from endothelium, lymphoblasts, reticulocytes, fibroblasts, smooth muscle, mesothelium, and embryonic inclusions (1). Malignant variations are rare. Langenstrass (4) encountered a single primary sarcoma in 6,400 autopsies. Primary malignant lymphomas though certainly uncommon, comprise a considerable portion of the primary tumors of the spleen (5). The organ of origin of solitary or multiple lymphoma which in the advanced stages has become diffuse in the reticuloendothelial system may go unrecognized. In these advanced stages one loses the criteria that distinguish a lymphoma as being primary in the spleen. In this respect, two points are worth mentioning: 1. The only diagnostic criterion of a primary lymphoma of the spleen is the absence of any extrasplenic lymphomatous lesion at the time of recognition of the splenic tumor. 2. Late secondary involvement of the spleen in a case of lymphoma is usually diffuse. Limitation of the disease to part of the spleen, while there is evidence of diffuse lymphoma in other organs, is suggestive, but not diagnostic, of a primary splenic lymphoma. In view of such criteria, one is inclined to be hesitant in accepting as genuine some of the reported cases of reticulum-cell sarcoma primary in the spleen (3). Splenic cysts, like splenic tumors, are also rare. Textbooks classify them as single or multiple, true (with a lining) or false (without a lining), lymphangiectatic, hemangiomatous, hydatid, dermoid, posttraumatic, and post-infarction. No classification was found that includes lymphoma as an underlying cause of cyst formation. Our purpose in this report is twofold: (a) to present a case of primary reticulum cell splenic lymphoma breaking down to produce a large pseudocyst; (b) to describe the peculiar clinical, radiologic, and operative features of this tumor, thus drawing attention to a diagnostic and prognostic pitfall in the management of apparently benign cysts of the spleen. Case report Clinical History: The patient was a 50-year-old housewife whose illness began two years prior to admission with a sensation of a shifting mass in the left lower abdominal quadrant. This was more apparent upon change of position. After eight months, a nontender, painless mass became palpable in the same region. Five months prior to admission the patient began to experience daily chills and fever reaching 38.5° C. During her illness she lost weight and felt very weak. The only other symptoms were a tendency to constipation and frequent gaseous distention relieved by eructation or passage of gas per rectum. Physical Examination: On admission the patient appeared weak and emaciated. The skin was pale and dry and the tongue was raw and red. The chest moved freely.