DIFFUSE HEMANGIOMA OF THE SPLEEN

Transcription

1 DIFFUSE HEMANGIOMA OF THE SPLEEN ELLIS KELLERT, M.D. Ellis Hospital Laboratory, Schenectady, N. Y. One finds in the medical literature numerous reports of angioma of the spleen, a somewhat rare condition as compared with other lesions of the spleen. Small and large forms, benign and malignant, are described. As early as 1879 Langhans described a large, pulsating, cavernous angioma of the spleen in a man of thirty years. This tumor, apparently, was malignant, for metastases were found in the liver. Homans, in 1897, reported a cavernous angioma of the spleen removed from a woman of twenty-two years. Albrecht, in 1902, found that up to that date, 11 cases of angioma of the spleen had been reported, and in 1915 Dowd tabulated 13 cases and described an angioma of the spleen removed from a female patient. It contained a large blood cyst, 10 cm. in diameter. Homans' and Dowd's patients died within several months, and the clinical course suggested metastases to the peritoneum and liver. Lubarsch, in a series of 19,000 autopsies, found only 3 cases of angioma of the spleen. He is of the opinion that small angiomas are exceedingly rare. Recent text-books on pathology (Delafield and Prudden; Karsner) speak briefly of the condition, noting particularly the cavernous types of angioma. Krumbhaar, writing on tumors of the spleen, reported 28 cases, among which 2 angiomata were found as "inconspicuous autopsy findings." Krumbhaar also reported 40 cases of splenic neoplasm but did not include a large angioma. In recent years numerous case reports of extremely large spleens containing diffuse angiomas have led to the conviction that the condition occurs often enough to warrant consideration in the differential diagnosis of splenic hypertrophies. The majority of the reports describe single and multiple, large and small angiomas, in some instances associated with similar lesions in other organs. Miiller found, in a spleen weighing 260 gm., 33 nodules of capillary angiomata. Orlandi describes a diffuse hemangioma in a spleen weighing 800 em. removed from a woman forty-four years of age. It differed from spleens of similar type previously reported in that a sarcomatous element was lacking. Ugriumow records two cases 412

2 DIFFUSE HEMANGIOMA OF THE SPLEEN 41 3 of angioma in which the spleens weighed 2300 gm. and 1635 gm. respectively. Steden saw a spleen of 4125 gm., in which only a narrow rim of splenic tissue remained visible about the angioma. Ngher describes a spleen of 2500 gm., composed mainly of a tumor that on section was found strewn with small and large vessel lumina. Zeno and Cid report a spleen weighing 5800 gm., removed from a male Arab thirty years of age. Sections of the organ showed large blood sinuses separated by connective-tissue trabeculae. In some areas the lumens were filled by proliferating endothelium. A report by Schwarz describes a spleen weighing 3500 gm., removed from a woman thirty-five years of age. This spleen contained large amounts of caseous and hyaline material in the center but elsewhere was of an angiomatous character. The clinical data in our case are extremely meager. Mrs. M. R., aged fifty-two years, an Italian woman, speaking no English, stated that for a long time she had noticed increasing enlargement of the abdomen. Her early history contained nothing of special note. Since she was able to carry on her work, no attention was paid to the abdominal condition. She entered the Ellis Hospital Feb. 27, 1929, complaining of malaise, chills, and fever. Physical examination disclosed a thin, undernourished woman of dark brunette type, with an anxious expression. No skin changes, icterus, enlarged lymph nodes, or edema were found. The abdomen was markedly distended, and an enlarged spleen extending to the midline and into the pelvis could be palpated. It was tender, but no pulsation could be detected. The liver did not appear to be enlarged. There were no petechiae and no evidence of bleeding from the mucous membranes. The blood examination was as follows: erythrocytes, 3,482,000; leukocytes, 7,300; hemoglobin, 35 per cent (Newcomer); color index, 0.5; polymorphonuclears, 59 per cent; lymphocytes, 39 per cent; endothelial leukocytes, 1 per cent; basophiles, 1 per cent. Smears showed marked variation in size and shape of the red cells; few poikilocytes and microcytes. No normoblasts were found. The platelets appeared normally abundant. Clotting time and bleeding time were not prolonged. Repeated examination of smears revealed no malarial parasites, and permission for splenic puncture was refused. Blood culture proved sterile. Several urinary examinations showed a trace of albumin, specific gravity , and few leukocytes. The Wassermann test was negative. During the first few days the patient's temperature was quite irregular, varying from 99" to 103" F.; pulse 84 to 100; respirations 20. She did not complain of abdominal pain and did not vomit. On March 3, 1929, a splenectomy was performed by Dr. Albert Grussner. The greatly enlarged spleen was found adherent to the anterior abdominal wall and partly covered by a thickened, moist omentum, separation of which was followed by the escape of considerable thick yellowish pus that appeared to come from a pocket in the spleen. After much difficulty the spleen

3 414 ELLIS KELLERT was removed, the peritoneum sponged as thoroughly as possible, and the very pendulous abdominal wall sutured. The infected omenturn, because of its widespread involvement, was not removed. The patient reacted well to the operation. The temperature dropped immediately to 99" F., rising to 100" F. at night. On the 11th day there Fra. 1. HEMANCIOMA OF SPLEEN: SURFACE MARKINGS AND INFARCT-LIKE AREA T,EADING TO ABSCESS FIG. 2. HEMANGIOMA OF SPLEEN: CROSS-SECTION SHOW IN^ ABSCESS, INFARCT, CUT VESSELS, SMALL CYSTS, AND GENERAL SPONUE-LIKE CHARACTER OF THE SURFACB was a sharp rise to 103" F., lasting but one day. Chill was absent at that time. Throughout the postoperative period the patient complained of abdominal pain and vomited occasionally. However, she improved steadily and left the hospital March 28, with normal temperature, pulse,

4 DIFFUSE HEMANGIOMA OF THE SPLEEN 415 and respiration. Cultures of the pus from the omentum yielded numerous colonies of hemolytic streptococci of the beta type (T. Smith and Brown). In view of the extensive omental and peritoneal infection with this organism, recovery was quite remarkable. On August 1, 1931, the patient was alive and in good health. The spleen measured 36 X 20 X 8 cm. and weighed 4770 gni. The capsule was of the usual slate-blue color and the surface covered with minute pitted areas and innumerable petechiae. The post,erior border near the middle was deeply notched, whereas the anterior margin was intact. On the convexity near the anterior margin were slightly depressed, broad, grayish areas, the larger of which resembled infarctions. Considerable fibrous streaking and roughening, particularly where the omentum had been adherent, were evident. Consistency was increased but the organ felt somewhat resilient. No vessel changes were found at the hilum. On section, the cut surface presented a most unusual appearance, not at all resembling spleen. The color was dark red and the surface so liberally sprinkled with cut vessels as to have a distinctly spongy character. The vascular spaces varied greatly in size, from 1 mm. to 15 mm. in diameter. Certain of the larger ones had distinct vessel walls and the lumina were filled with reddish or yellowish serum or blood. Near the center and leading to one of the depressed areas above mentioned was a large abscess of hour-glass shape, measuring 6 X 2 X 2 cm. The pus was yellowish-green and the lining of the cavity thick and fibrous. Cultures of this pus yielded hemolytic streptococci of the same type as those found in the omentum. Above the abscess was an old healed infarct. On histologic examination the most marked feature was the vascularity of the section. Small and large thin-walled vascular spaces almost 35

5 416 ELLIS KELLERT completely replaced the normal splenic tissue. Although many clusters of lymphoid cells were present, typical malpighian bodies were rarely encountered. The numerous small and large vascular spaces formed a picture closely simulating cavernous hemangioma. The vessel walls were indistinct, varied greatly in thickness, appeared fibrous, and did not contain myoglial tissue. The lining was of the flat endothelial type and FIGS HEMANGIOMA OF SPLEEN Figs. 4 and 5. Cells lining vascular spaces (X 300). Fig. 6. Cells in the intervascular tissue ( X 300). Fig. 7. Spaces near capsule of spleen (X 70). Figs. 8 and 9. Clusters of endothelial cells in cavernous spaces (X 300). inactive. The spaces were filled with granular pink-staining material; some with red blood cells and the various leukocytes of the blood. The intervascular connective tissue contained small vascular spaces, as in the normal spleen, numerous polynuclear and lymphocytic cells, few eosinophiles, and many endothelial cells. A moderate amount of old blood pigment was present. Oocasionally in a vascular space, large, round, multinucleated cells of endothelial type were found, and occasionally spaces suggesting cholesterin clefts.

6 DIFFUSE HEMANGIOMA OF THE SPLEEN 417 This case is remarkable because of the widely diffuse character of the hemangioma and the great weight of the spleen. By virtue of the histology and subsequent clinical course, this angiomatous change must be regarded as a benign condition. That a similar neoplasm may become malignant is indicated by A. W. Wright, who reported a malignant hemangioma of the spleen with multiple liver metastases. The spleen weighed 520 gm. and contained, at the upper end, a large spherical mass which had ruptured and caused fatal hemorrhage. The histology suggested an old lesion in the spleen becoming active and producing metastases. Somewhat similar is the case reported by Schlopsnies. Splenic angioma may frequently terminate as a malignant neoplasm, for an actual capsule is rarely present. Pool and Stillman make the following observation, "If angiomata are present in several organs or tissues it is not conclusive evidence that they are of metastatic distribution, since it is well known that angiomata are commonly multiple." Nevertheless, the histology of the malignant forms is quite distinctive as compared with the benign neoplasm. Perhaps this splenic angiomatous change is erroneously regarded as a tumor. The absence of distinct nodules and of a connective-tissue framework, coupled with retention of the splenic outline, indicate a non-neoplastic lesion; possibly an embryonic anomaly or hamartoma, as suggested by Lubarsch. Apparently all gradations occur, from simple localized or widely diffuse telangiectatic change, to more or less encapsulated angiomatous nodules, and finally to highly malignant endotheliomat ous tumors with metastases. ALBRECHT, H.: Uber das Cavernom der Milz, Ztschr. f. Heilk. 23: 97, DELAFIELD, F., AND PRUDDEN, T. M. : Text-book of Pathology, Revision by F. C. Wood, William Wood & Co., New York, 1927, 14 ed., p DOWD, C. N.: Cavernous angioma of the spleen, Ann. Surg. 62: 177, HOMANS, J.: Report of a case of cavernous angioma of the spleen, Ann. Surg. 25: 732, KARSNER, H. T.: Human Pathology, J. B. Lippincott Co., Philadelphia, KRUMBHAAR, E. B.: Tumors of the spleen, S. Clin. North America 7: 61, KRUMBHAAR, E. B.: Incidence and nature of splenic neoplasms, Ann. Clin. Med. 5: 833, 1927.

7 418 ELLIS KELLERT LANGHANS, T. : Pulsirende cavernose Geschwulst der Milz mit metastatischen Knoten in der Leber, Virchow's Arch. f. path. Anat. 75: 273, LURARSCH, 0. : Gewiichse und nicht parasitke Zysten der Milz, Handb. d. spez. path. Anat. u. Histol. 2: 699, MULLER, H.: ffber multiple Capillarangiome der Milz, Virchow's Arch. f. path. Anat. 238: 76, N~~HER, H. : ffber Hamangiom der Milz, Deutsche Ztschr. f. Chir. 191 : 87, ORLANDI, N. : Primares diffuses Hamangioendotheliom der Milz, Virchow's Arch. f. path. Anat. 269: 152, POOL, E. H., AND STILLMAN, R. G.: The Surgery of the Spleen, D. Appleton & Co., New York, 1923, p SCHLOPSNIES, W. : ~ ber ein systematisiertes angioplastisches Sarkom in Milz, Leber und Knochenmark, Virchow's Arch. f. path. Anat. 274: 85, SCHWARZ, I?. : ffber ein primares Hamangiom der Milz von seltener Grosse, Beitr. z. klin. Chir. 150: 130, SHENNAN, T. : Histologically non-malignant angioma with numerous metastases, J. Path. and Bact. 19: 139, STEDEN, E. : uber ein aussergevohnlich grosses kavernoses Hamangiom der Milz, Arch. f. klin. Chir. 130: 616, THEILE: ffber Angiome und sarkomatiise Angiome der Milz, Virchow's Arch. f. path. Anat. 178: 296, UGRIUMOW, B.: ffber zwei seltene FBlle von Gefassneubildungen in der Milz und Leber, Virchow's Arch. f. path. Anat. 259: 366, WRIGHT, A. W.: Primary malignant hemangioma of the spleen with multiple liver metastases, Am. J. Path. 4: 507, ZENO. A, AND CID, J. M. : H6mangio-endothdliome de la rate, Ann. d'anat. path. 7: 583, 1930.