T H E B R I T I S H J O U R N A L O F S U R G E R Y

HIEMANGIOMA OF THE SPLEEN

CAVERNOUS HIEMANGIOMA RELATION TO OTHER CYSTIC TUMOURS. CASE REPORT OF GIANT

BY RODNEY SMITH AND N. F. C. GOWING, LONDON

STUDY of relevant literature brings the conclusion that haemangioma of the spleen is a condition of considerable rarity. Several authors have discussed the tumour under the general heading of ' splenic cysts ', of which Fowler (1940) reviewed 137 cases that had appeared in the literature up to 1939. Since that date other examples have been reported (McClure and Altemeier, 1942 ; Gallagher and Mossberger, 1942 j Harmer and Chalmers, 1946). Apart from the solitary cysts included in this classi- fication, occasional examples of multiple cysts are encountered in association with congenital polycystic disease of the kidneys, pancreas, and liver. In a recent review of polycystic disease of the kidney Rall and Ode1 (1949) reported multiple cysts of the spleen in 2 per cent of their cases coming to autopsy.

Solitary splenic cysts may be broadly classified into :-

I. Parasitic cysts-hydatids. 2. Non-parasitic cysts :-

a. Dermoids and epidermoids. b. Hzmanniomata and lvmphanniomata. - - - c. ' False 'cysts.

Hvdatid cvsts will not be further discussed. The cysts -are designated ' false ' if there is no demon- strable epithelial or endothelial lining. They form an indefinite group, for it is clear that such a lining, originally present, may become lost as a result of degenerative changes so that correct pathological classification becomes impossible. Nevertheless, it appears certain that many false cysts are without a lining from their inception, and the aetiological importance of injury and hzmorrhage into the spleen has been stressed by several authors. Tamaki (1948) states that a history of trauma is available in a high proportion of hzmorrhagic false cysts. It has also been pointed out that such cysts are relatively common in West African natives, amongst whom malaria and splenomegaly are widespread (Harmer and Chalmers, 1946). The role of embolism and thrombosis in the genesis of false cysts is discussed by Dabrzaniecki (1930).

Dermoids and epidermoids appear to be rarer than other varieties of non-parasitic cyst. Dermoids presumably take origin from embryonic cells segre- gated during early life, and epidermoids may have a similar pathogenesis. Bostick and Lucia (1949)~ however, in describing an epidermoid cyst of the spleen in a man of 20 years, suggest that the epithelial lining may arise by metaplasia of mesenchyme. They therefore propose the term ' metaplastic meso- dermal cyst ' for this variety, but the simple descrip- tive name ' epidermoid', which commits us to no special aetiological standpoint, would still seem preferable.

In common with the majority of vascular ' tumours ', the hzmangiomata and lymphangiomata of the spleen are to be regarded as malformations of congenital origin rather than as true neoplasms. As would be expected, a considerable proportion of

cases have angiomatoid malformations in other organs as well as the spleen, and it is perhaps of interest to note that several early papers (quoted by Dowd, 1915) reported angiomata of the spleen with ' metastases ', in the liver and elsewhere. In view of their probable origin in very early life, the age incidence of the angiomata of the spleen calls for comment. Although a few examples have been described in infancy, the majority of the reported cases have been in adults. It must be presumed that the spleen enlarges slowly over the years until it reaches a sufficient size to produce symptoms. Such an enlargement is not produced by cell-division in a neoplasm, but may result from : (a) Inflammatory swelling ; (b) Progressive dilatation of the cavernous spaces ; (c) Thrombosis in some channels leading to engorgement of other parts of the angioma; (d) Canalization of previously uncanalized vasoformative tissue included in the malformation.

I n most instances, and in the case here reported, factors (b) and (c) are probably the most important. By the time splenectomy is performed such changes may be so extensive that pathological diagnosis is rendered very difficult. Many sections may have to be examined before typical cavernous spaces lined by epithelial cells are found.

CASE REPORT W. W., a man of 50, was admitted to a hospital on

the outskirts of London after he had had a severe haema- temesis. He was successfully treated by conservative means, receiving a total of eight pints of whole blood by drip transfusion. Meanwhile physical examination had revealed a very large mass in the upper abdomen. The nature of this seemed obscure and when he had com- pletely recovered from his haemorrhage he was trans- ferred to St. George's Hospital for further investigation and treatment.

ON ADMIssION.-He was a powerfully built man and showed no signs of recent severe loss of blood. His general condition appeared to be excellent and the only abnormal physical signs were related to the mass in his abdomen. This was a smooth, tense, cystic swelling occupying the whole of the epigastrium and centred to the left of the midline. Its lower margin was below the level of the umbilicus and the upward extension had caused such elevation of the left leaf of the diaphragm and mediastinal shift that the apex beat was palpable just medial to the right nipple. Radiographs (Figs. 663-664) showed the margins of the tumour clearly and also areas of calcification in its centre (Fig. 664 A). Examination with a barium meal showed gross displacement of the stomach forwards and to the right (Fig. 664).

A diagnosis was made of a cyst of the spleen or tail of the pancreas. Naturally the patient was asked how long he had had this enormous lump in his belly, but he expressed astonishment, saying that he had never noticed anything wrong.

AT oPERATIoN.-After induction with pentothal, anaesthesia was maintained with nitrous oxide and oxygen, relaxation being obtained with curare.

The abdomen was opened by a long left paramedian incision and the anterior surface of an enormous tense cyst of the spleen presented, covered by a ' capsule ' of

H W M A N G I O M A O F T H E S P L E E N 567

compressed splenic tissue. An onlooker at this point it and evacuate the contents. This temptation was suggested that in order to deliver the tumour from the resisted, as it was not considered desirable to have first- abdomen it would clearly be necessary first to puncture hand experience of the unfortunate end to the operation

FIG. 663.-Soft-tissue radiographs showing the outlines of the tumour and the elevation of the left diaphragm.

A B

FIG. 664.-A, Barium m-a1 showing the displacement of the stomach forwards and to the right. In B, calcification in the tumour can be seen.

568 T H E B R I T I S H J O U R N A L O F S U R G E R Y

described in The Citadel. The incision was enlarged by a transverse extension cutting across both rectus muscles, and it was then possible to deliver the enormous splenic

thickened capsule. The main bulk of the tumour con- sisted of dark red material with irregular paler areas and scattered foci of calcification (Fig. 665, C).

B

A FIG. 66~.-Gross appearance of specimen.

mass from the abdomen. The pedicle was easily secured and splenectomy was a simple procedure. The abdominal wall was repaired with stainless steel wire.

Convalescence was uneventful and the patient was discharged to a convalescent home after twelve days, having been ambulant from the day after operation. He reported for routine examination three months after his operation in excellent health and exhibiting no weakness of his abdominal wall. He had resumed his occupation as a meat porter, but was at the moment out on strike.

PATHOLOGICAL REPORT.- Macroscopical Appearance.-The splenic mass was

roughly spherical in shape but somewhat flattened antero- posteriorly. It weighed 8 lb. IZ 02. The notched

Histology.-Throughout the greater part of the angioma there was extensive thrombosis in the vascular channels, with destruction of the endothelium and partial organization. However, the outlines of the vascular walls could still be shown clearly in sections impregnated for reticulin, and a careful search revealed spaces lined by an intact endothelial layer. The appear- ances were those of a cavernous hremangioma of the spleen in which widespread thrombosis and degenerative changes had occurred. (Fig. 666.)

FIG. 666.-Microscopic appearance of the tumour. Low power. One of the few areas sufficiently free from necrosis and thrombosis to show vascular spaces lined by endothelium.

(H. and E.)

inferior border of the spleen could still be seen as a sharp ridge (Fig. 665, A, B). On cut section a narrow rim of compressed splenic tissue was apparent beneath the

REFERENCES BOSTICK, W. L., and LUCIA, S. B. (1949), Arch. Parh.

DABRZANIECKI, W. (193o), Ann. Surg., 92, 21s. DOWD, J. (ISIS), Ibid., 62, 177. FOWLER, R. H. (I940), Int. Abstr. Surg., 10, 213. GALLAGHER, P., and MOSSBERGER, J. I. (I942), Ann. Surg.,

HARMER, M., and CHALMERS, J. A. (1946), Brit. med. J.,

MCCLURE, R. D., and ALTEMEIER, W. A. (1942), Ann.

RALL, J. E., and ODEL, H. M. (I949), J . Amer. med. Sci.,

TAMAKI, H. T. (1948), Arch. Path. (Lab. Med.), 46,

(Lab. Med.), 41, 215 .

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