Cystic hemangioma of the spleen

CYSTIC HEMANGIOMA WILLIAM

W.

OF THE SPLEEN

REICH, M.D. AND LLOYD R.

VAN

TASSELL, M.D.

Berkeley, California

C

APILLARY and cavernous hemangiomas of the spIeen constitute a reIativeIy rare p&hoIogic entity. Most reported cases1-3 have been from autdpsy findings in which an incidence of about 0.1 per cent has been recorded. A few cases have been discovered at operation which has usuaIIy been performed for hemorrhage foIIowing trauma.4’5 Hemangioma and Iymphangioma are of particular interest in the present report because they may be the underlying basis for cystic changes giving rise to a variety of pathoIogic changes in the spleen. In general, it may be stated that cysts constitute a pathoIogic of the spIeen curiosity and are extremeIy rare in cIinica1 surgery. McCIure and AItemeier6 report 148 spIenic cysts in a review of the Iiterature up to 1941. As a cause of spIenomegaIy, cystic disease is responsibIe for a very Iow percentage and, apart from parasitic invasion (e.g., echinococcus disease), onIy 108 spIenic cysts were reported up to 1941 by Watts and Warthen. Deneens states that a review of the BeIIevue HospitaI statistics from 1904 to 1942 reveaIed no case of hemorrhagic cysts of the spIeen with a similar record at St. Vincent’s HospitaI for the period 1910 to 1940. McCIure and AItemeier’j add to their report a recent case of a patient operated upon by them at the Henry Ford HospitaI. PauIg beIieves that cysts of the spIeen are very rare. He states that AndraI reported the first case in 1829 and he then reports an operative case in which the spIeen presented one Iarge and several smaIIer cystic areas. In his case there was a history of maIaria and in the pathogenesis of this Iesion an antecedent maIaria1 infection with trauma appear to be prominent features. Andral’s case microscopicaIIy presented 840

cyst wa1l.s Iined by stratified epithelium. The same writer presents a simple cIassification of spIenic cysts as foIIows: (I ) Hydatid cysts of the spIeen; (2) muItipIe serous cysts associated with poIycystic disease of the kidneys; (3) singIe cysts consisting of (a) dermoid cysts, (b) epidermoid cysts and (c) serous and bIood cysts devoid of an epitheIia1 Iining and (4) MuItipIe serous cysts. McClure and AItemeier6 present a more eIaborate cIassification : (I ) true cystslined by a specific secretory membrane; (a) epitheIia1 consisting of derma1 and epiderma1; (6) endotheIia1 consisting of Iymphangioma, hemangioma, poIycystic disease and some serous cysts; (c) parasitic-Iined by protoplasmic matrix containing numerous nucIei making up x-hydatid cysts. (2) faIse cysts-no specific secretory Iining making up (a) hemorrhagic; (b) serous; (c) infIammatory with acute necrosis in infection and chronic tubercuIosis; (d) degenerative liquefaction of infarcted areas caused by emboIism or arteria1 thrombus. The subject of the present report appears to have true cysts of endotheIia1 origin. Gradations and changes in the pathoIogic picture, depending upon the age of the Iesion, areas studied, concommitant disease, etc:, makes overIapping of cIassification IikeIy. SweetlO like most writers emphasizes the reIative paucity of clinica symptoms associated with spIenic cysts. Apart from the acute picture of shock and hemorrhage which may occur with rupture, the chief compIaints are those referabIe to the presence of a mass in the hypochondrium; because of its size, this may give rise to varying degrees of discomfort and dysfunction due to pressure on neighboring viscera. DifferentiaI diagnosis of spIenomegaly ordinariIy presents no great diffr-

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culty but such conditions as polycystic kidney, retroperitonea1 bIastoma, mesenteric cysts, etc. must be considered either alone or in association with splenic cysts. The diagnosis of cystic disease per se must usually await surgical expIoration or necropsy although Sweet and others have pointed out the value of x-ray studies. Recause of the benign nature of the process, splenectomy for splenic cysts gives excellent results. However, the technical problems involved in splenectomy for any splenomegaly present a wide range, and in some cases they may be of such a nature as to preclude successful surgica1 extirpation of the tumor. These problems have been reviewed by BaIfour,‘l Henry12 Maingot, l3 Behrend’j and many other surgeons. summarized surgical RecentIy, Dunphyl” problems, reviewed surgica1 anatomy and presented some very important points of great technica significance in splenectomy. We agree with him that spIenectomy is often a relatively simple procedure in the case of a smaI1 spleen; here, even in the presence of severe trauma and hemorrhage, proper preparation coupled with good anesthesia wiI1 ensure a fairIy simple and successfu1 operative procedure. However, in long-standing pathoIogic states (Banti’s syndrome, spIenic anemias, cirrhosis, etc.) the development of numerous extremely vascular adhesions, the great friability of the spleen and the technical obstacIes presented by the size of the organ and its fixation to adjoining viscera (especially the stomach, coIon and diaphragm) may combine to render splenectomy extremely hazardous or impossible even for the most experienced and skiIIed surgeon. The present case is deemed worthy of presentation not onIy because of the rarity of the condition but aIso because of the associated pregnancy, the long history, the striking surgica1 and pathologic findings and also to reemphasize the surgica1 difflculties which one may anticipate. We beIieve it also serves to point out the great value of adequate pre- and postoperative care.

of SpIeen CASE

Mrs. B. C. Negro female, first admitted rick Memorial

A merir;,nJourd oFSurgrry 841 HISTORY

(Hospital No. 77$2), a married twenty-five years of age, was to the out-patient service, HerHospital, April 29, 1946. Her

chief complaint was a painful mass in the left hypochondrium of about seven month’s duration. Her family history revealed the following: the medical status of her parents was unobtainable; there were several siblings; there was no known history of tuberculosis, cancer or other familial diseases. In regard to the patient’s past history she denied that there were any serious illnesses or operations. The patient, on closer questioning, stated that she may have had malaria during her childhood in the South, and also stated that she had “spleen trouble” at the age of thirteen. Catamenia began at the age of 15; her periods were reguIar every twenty-six days, lasting for three days; there was a moderate flow and no dysmenorrhea. The patient had been married five years. She has two living children; she had one spontaneous miscarriage in 1944. Last menstrual period was on March 13, 1946. She had been troubled by nausea and painful breasts since the onset of her present amenorrhea; she thought she was probably pregnant. Physical examination revealed a fairly well nourished, young colored woman. Her weight was 143 pounds and blood pressure, 90 /60. System findings were unimportant except for abdominopelvic findings. There was a large, irregularly round, firm, slightly tender mass occupying the left hypochondrium. It extended from below the rib cage to the left flank, well below the umbilicus and mesially to the right of the midline. A notched surface was palpable. The liver was not enlarged to percussion in the right hypochondrium and duIlness merged with that of the mass on the left side. Abdominopelvic examination showed the uterus to be uniformly enlarged, soft and extending the symphysis; the mucous membranes vault were dusky. Clinical diagnosis: EarIy pregnancy;

above of the splenic

tumor. X-ray and Iaboratory findings revealed that a plain film showed extreme enrargement of the spIeen; the Ieft costophrenic angle was compressed and there were old, healed tuberculous foci in the left lung; the finding suggested Ieukemia or other 1ymphobIastoma. Urinalysis

842

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Van TasselI-Hemangioma

was within normaI Iimits; repeat bIood counts showed mild secondary anemia and moderate Ieukopenia with normal distribution of Ieukocytes; fragiIity tests were within the norma range; pIatelets, 249,000; blood was group III-B and Rh positive. Tentative diagnosis: SplenomegaIy of unknown etiology, compIicated by pregnancy. The patient was admitted to the hospita1 JuIy 22, 1946. In view of her generahy good condition it was decided to postpone transfusion unti1 surgery and thereafter as a diffrcuIt operative course was anticipated. She was operated upon on JuIy 23, 1946, under nitrousoxide-oxygen ether. Saline was commenced before the abdomen was opened and this was folIowed with two units of whole bIood. A left rectus incision extending from the costal margin to the superior border of theiIium was made. As soon as the abdomen was opened 25 mg. of intocostrin were administered by injection into the perfusion ffuid. On opening the peritoneum the spIeen presented as a huge cystic mass widely dispIacing the stomach and the coIon. A Iarge knob-Iike tumor or cyst occupied the Iower poIe of the spIeen and the upper surface was deep under the dome of the diaphragm. There were whitish somewhat raised areas scattered over the remainder of the surface, and the organ was denseIy adherent to the diaphragm, liver, lateral abdominal wall, transverse colon, stomach and the omentum. These adhesions were very thick, tough and extremeIy vascuIar. The splenic tumor was compIetely immobiIe unti1 dissection of its attachments was commenced. The gastrosplenic omentum was doubly Iigated as high up as possible in seria1 segments with 2 chromic and carefuhy cut between ligatures. Because of very dense adhesions in the Iesser sac, exposure and contro1 of the major vesseIs through this approach did not appear feasible. Freeing of adhesions was therefore proceeded with, using sharp and bIunt dissection of smaI1 zones with secure hemostasis at each step. The peritoneum of the left parieta1 region above the lienorenal ligament was carefuIIy incised and stripped down with gauze. The right hand was then insinuated between the upper pole of the mass and the diaphragm; here bIunt dissection of adhesions had to be accomplished blindly. It was then found possible to push the huge spIenic tumor into the wound after which mesia1 rotation exposed the broad pedicIe with

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the main blood vessels. These were carefuIIy isolated and doubly ligated with No. 4 chromic, a doubIe clamp was pIaced distaIIy and the pedicIe cut through. The pedicle was then transfixed and tied with a third ligature which was then tacked down to the splenic bed. As soon as the spIeen was dehvered two Iarge hot saIine packs were snugIy appIied to the tumor bed. It was remarkable and very gratifying to note, upon removal of the packs, that the field was dry. The opening in the gastrocoIic omenturn was repaired with pIain o catgut. The abdomen was closed in layers with interrupted sutures to muscle, fascia and skin and with four heavy silk retenion sutures. The patient’s condition was excehent during the entire procedure. Systolic bIood pressure ranged from I 20 to I IO and diastoIic pressure from 80 to 60 mm. The puIse averaged 90 and respirations 20. The entire procedure took one hour and forty-five minutes. On returning to the ward the patient was given another unit of whoIe blood, and later that day 1,000 cc. of IO per cent glucose in saIine. The postoperative course was remarkably uneventful. The highest postoperative temperature was IOI’F. for one day and the pulse and respiration remained within normal limits. She was taking Iiquids on the first postoperative day, a Iight diet on the second day and was ambuIatory and on a genera1 diet on the fourth postoperative day. The patient compIained of some back pains and peIvic discomfort resembIing Iabor pains on the ninth day; these subsided with sedation. She left the hospita1 on the tweIfth day. The stitches were removed and the wound appeared to be heaIing niceIy per primam. She reported subsequentIy to the obstetrica service of the out-patient department and pursued an uneventfu1 prenata course. On November 27, 1946, she was deIivered with Iow forceps of a 7 pound baby gir1. Her puerperal status and course were entireIy uneventful. The patient has reported subsequently to our CIinic. She was reviewed December 30, 1946, at which time she was feeIing fine and had no further compIaints. PathoIogicaI report revealed the folIowing: specimen was IabeIIed ‘spleen.’ It was a large, dusky, cystic mass, measuring about 22 by 15 by 8 cm. On cut sections it appeared very congested and was riddled by variousIy sized cystic cavities, these varying in size from a few mm. to severa cm. in diameter. Microscopic examination of sections from different areas

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VOL. I.XXV, No. 6

FIG. FIG. FIG. FIG.

I. 2. 3. 4.

Van

Superior and Inferomesial Longitudinal Cross section

TasselI-Hemangioma

of Spleen

IateraI surface of spIenic tumor; relative surface showing cystic masses. section showing Iarge and small cysts. showing cysts and heavy scarring.

were examined. There was almost complete destruction of the splenic puIp. This had been repIaced by broad bands of fibrinous and fibrous tissue which appeared to surround vascuIar channels Iined by endothehum. In some areas a capiIIary hemangiomatous network was we11 demonstrated. There was no evidence of malignancy. In other levels the genera1 pattern of a pIexiform hemangioma was simiIarIy noted. Wide trabeculae dominated the picture with small isIands of brood vesseIs spreading and compressing the tissue. In the larger spaces a liquefaction necrosis had converted the picture from red brood ceIIs and vesseIs into a homogeneous acidophiIic matrix. Diagnosis: Cystic degeneration in pIexiform hemangioma of the spIeen. (Figs. I, 2, 3 and4.) SUMMARY

The clinica history which went back to earIy chiIdhood is extremeIy interesting. AIso notable is the reIative paucity of compIaints referabIe to the growth unti1

A me&an

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843

size indicated.

it had reached huge proportions. The possibihty of a long-standing Iatent malaria1 infection may be inferred aIthough no hematologic or pathoIogic evidence was found in our study. It is interesting to speculate on the possible r6Ie of trauma in the initiation of hemorrhages and cystic degeneration in an organ which is the site of capiIIary and cavernous hemangiomas. These conditions wouId undoubtedly render the spIeen unusuahy susceptibIe to injury. Again, however, no definite history of trauma was elicited but forgotten injuries may we11 have occurred repeatedIS during the Iife of the patient. A comparison of the histoIogic features of our case with those we have had the opportunity to review in the Iiteratnre Ieads us to remark on the unusually striking features of hemangioma depicted in this case. The association of a reIativeIy advanced pregnancy as a troubIesome compIication is of

844American~~~~~~~or surgery Reich,

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TasseII-Hemangioma

cIinica1 interest, and the favorabIe outcome for mother and chiId indicates to what a degree nature wiII toIerate a pathoIogic state and a surgica1 insuIt of great magnitude without rehnquishing its purpose. REFERENCES I. KRUMBHAAR, E. B. Tumors of spleen with report on 28 recent cases. S. Clin. North America, 7: 61, ‘927. 2. KELLERT, E. Diffuse hemangioma of the spleen. Am. J. Cancer, 16: 412, rg32. 3. PINES, B. and RABINOVITCH,J. Hemangioma of the spleen. Arch. Patb., 33: 487, 1942. 4. NESLER, A. B., FABER, L. and LEIK, D. K. Hemangioma of the spleen with spontaneous rupture. J. Iowa M. SW., zg: 566, 1939. 3, CALLANDER,’C. L. Surgical Anatomy, Philadelphia, Ig43. W. B. Saunders Co.

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6. MCCLURE, R. D. and ALTEMEIER, W. A. Cysts of spIeen. Ann. Surg., I 16: 98, 1942. 7. WAI-~S, T. D. and WARTHEN, H. J. Non-parasitic cysts of spleen; report of case. Soutb. Surgeon, IO: 34, 194’. 8. DENEEN, E. V. Hemorrhagic cyst of the spIeen. Ann. Surg., I 16: 103, 1942. 9. PAUL, M. Cysts of the spIeen. Bit. J. Surg., 30: 336, 1942. IO. SWEET, R. H. SingIe true cysts of the spleen. New England J. Medy,.z28: 70f, 1943. of sdenectomv. Surf., II. BALFOUR. D. Techniaue Gynec. .& Obst., 23: I; IgI6. 12. HENRY, A. K. The removal of large spleens. &it. J. Surg., 27: 464, 1940. ‘3. MAINGOT, R. Abdominal Operations. Vol. I. New York, Ig4o. D. AppIeton-Century Company. J. 14. BEHREND, M. Surgery of spIenic tumors. Internat. Coil. Surgeons, g: 664, 1946. ‘5. DUNPHY, J. E. Splenectomy for trauma. Am. J. Surg., 71: 450, 1946.

CANCER of the Islets of Langerhans is a fast-growing tumor, occurring usuaIIy in the taiI of the pancreas of middIe-aged men, with or without hypoglycemia. UnIess metastases are discovered, usually in the liver and neighboring Iymph nodes, the maIignant nature of the growth is likely to escape detection by the surgeon. (Richard A. Leonardo, M. D.)