FROM THE DEPARTMENTS OF DIAGNOSTIC RADIOLOGY AND PEDIATRICS, KOMMUNEHOSPITALET, DK-SOOO AARHUS, DENMARK.
HEMANGIOMA OF THE LIVER Report of two cases J. TH. JENSEN and T. KLINGE Angiography in hemangioma of the liver has been reported severa times in the last few years. The use of angiography both in establishing the primary diagnosis and in a follow-up examination has, however, been reported only once before (TAVERNIER et colI. 1972) after intervening surgery. In the two cases presented in this report a follow-up examination was carried out after steroid treatment in one patient, and after 6 years without treatment in the other case. Hemangiomas are thought to be embryonic sequestrations of unipotent angioblastic cells (ARIEL & PACK 1960). They are usually divided into the following groups: Hemangioendotheliomas, capillary and cavernous hemangiomas. The main difference between the groups is the amount of endothelial cells and the size of the blood filled spaces. Most authors describe the presence of a maturing process via the cavernous type to spontaneous remission, that corresponds with the occurrence of hemangiomas of mixed type in the liver, i.e. with centres of different degree of maturation. The lesion must be regarded as benign in most cases, although sometimes it has a clinically malignant course. The description of metastases by some authors is regarded by others as multicentric development of new growths and not as metastases. The exact incidence is not known. McLoUGHLIN (1971) has mentioned a frequency of approximately 0.35 per cent of cavernous hemangiomas in autopsies, but the tendency for spontaneous remission makes such a statement uncertain. Hemangioendotheliomas, particularly the mixed type, are most common in children. Submitted for publication 6 September 1974. Acta Radiologica Diagnosis 17 (1976) Fasc, 1 January
Downloaded from acr.sagepub.com at UNSW Library on July 27, 2015
61
62
J. TH. JENSEN AND T. KLINGE
The cavernous type more often occurs in adults with a predomination of females in a ratio of 9: 1 (PANTOJA 1968). The hemangiomas are usually small and multiple, but may be diffuse throughout one lobe or the whole liver. They are commonly accompanied by hemangiomas of the skin. The histology is described by CROCHER & CLELAND (1957), DEHNER & ISHAK (1971) and EDMONDSON (1956) and others. The clinical symptoms may be abdominal discomfort with enlargement of the liver, and cardiac failure in small infants. Thrombocytopenia and hemorrhage following rupture or necrosis may occur, but many angiomas are found accidentally at angiography. A large number of liver hemangiomas probably exist without any clinical symptoms (TAVERNIER et coll., REUTER & REDMAN 1972). A warning must be given against any attempt at biopsy because of the great danger of a massive hemorrhage (KAGAN et coll. 1971, PANTOJA). Angiography is thus the best and safest procedure for arriving at the correct diagnosis, and the best results are obtained with selective coeliac angiography.
Treatment. Concerning treatment, the natural tendency for spontaneous remission is well recognized. When surgery is contemplated (e.g. local exision, lobectomy or ligation of a feeding artery), angiography is of great value in determining the exact anatomy of the lesion (TAVERNIER et coll.). The use of radiation therapy is discussed by many authors and treatment with steroids is suggested in combination with radiation therapy by DEHNER & ISHAK. One successful case treated with steroids and cardiac treatment is reported by GOLDBERG & FONKALSRUD (1969). The prognosis is generally good but there is the possibility of cardiac failure, and hemorrhages may be fatal. Radiographic findings. An enlarged liver or an elevated or abnormally domed right hemidiaphragm may sometimes be found on a conventional film of the abdomen. Calcific deposits appearing as scattered irregular flecks of calcium, placed in the septa may occur. Calcified phleboliths are rarely seen (McLOUGHLIN, PANTOJA). The angiographic appearance has been described several times and in reviewing the literature the following points seem to be established: the best known and most common angiographic finding is that of the cavernous hemangioma (among others DUKEN et coll. 1971, McLOUGHLIN, PANTOJA, POLLARD et coll. 1966, TAVERNIER et coll.). The coeliac and the hepatic arteries are of normal size and appearance, but may be displaced around masses or crowded together. Late in the arterial phase, irregular areas of contrast medium appear to form labyrinths of pools or lakes, usually well demarcated, arranged in clusters or rings, often with an avascular centre. The retention of the contrast medium in these spaces lasts a long time, up to 30 seconds, and the venous phase is delayed. KAGAN et coil. and ROSCH & STECKEL (1972) report slight dilatation of the coeliac artery and the feeding hepatic arterial branches. The other condition, often described, is hemangioendothelioma in newborn and small infants (BERDON & BAKER 1969, Moss et coll. 1971, SELKE & CORWELL 1969, PANTOJA). The feeding coeliac and hepatic arteries are enlarged, the circulation rate is increased and bizarre irregular spaces surrounding large avascular areas are rapidly
Downloaded from acr.sagepub.com at UNSW Library on July 27, 2015
HEMANGIOMA OF THE LIVER
63
a b Fig. 1. Case 1. a) Abdominal aortography. Enlarged liver with stretched hepatic arteries of normal width. b) Capillary phase. Numerous irregular pools of contrast medium.
filled. The fast venous filling, after about one second, indicates the presence of arteriovenous shunting. Moss et coll. found numerous pathologic vessels in the arterial phase. Most of these children had cardiac insufficiency and several died, but in many cases no cardiac malformation was found. It seems that the great amount of blood shunted through the liver overburdens the heart. The last condition, the capillary hemangioma, resembles the hemangioendothelioma and several authors do not distinguish between the two types. According to TAVERNIER, the capillary hemangioma has normal-sized feeding arteries. The seemingly avascular masses, surrounded by irregular vascular spaces with a rapid shunting of blood to the veins, resemble the appearances of hemangioendothelioma, but there may be a more homogeneous appearance of the liver as well. The avascular hemangioma (WATSON & BALTAXE 1971) may be of this type. The avascular masses are thought to be areas with a large amount of endothelium compressing the vascular channels. The angiographic appearance with mixed endothelio-capillary. and cavernous elements corresponds to the theory of maturing. The proportion between the two elements decides the type, but the sudden jump from arteriovenous shunt to delayed venous filling in the cavernous type is difficult to understand and no explanation for this is found in the literature. It could be caused by the increasing width of the vascular bed, when small capillaries are replaced by wide cavernouslakes. At the same time there may be some thrombosis in' the irregular arteriovenous shunts. Extensive intravascular coagulation in hemangiomas of the liver is described by DIJKEN et colI.
Downloaded from acr.sagepub.com at UNSW Library on July 27, 2015
64
J. TH. JENSEN AND T. KLINGE
a
b
Fig. 2. Same case as in Fig. 1. a) Selective coeliac angiography 2.5 yearslater. Regression following steroidtreatment. b) Capillary phase. Only two small areas of cavernous hemangiomas.
Case reports Case 1. A girl born three weeks prematurely was fully developed with a weight of 3040 g. At 3 weeks of age hemangiomas of the skin were observed and one week later, a tendency to vomit developed. When 5 months old the girl became agitated with coughing, and one month later a right abdominal mass was detected, extending into the left hypochondrium. The girl was admitted to hospital and an anaemia was found, Hb 8.2 g %. Cardiac and liver function was normal. Angiography was performed via the femoral artery. Abdominal aortography was carried out with injection of 12 ml of Isopaque 260. The proximal part ofthe aorta was displaced to the left. The liver was enlarged and the hepatic arteries stretched, but of normal size (Fig. 1 a). In the capillary phase numerous irregular pools werefilled, partly diffuse and partly arranged in nests with a maximum diameter of 22 mm with an avascular centre. Later some diffuse filling occurred, but no veins were observed during the 11 seconds the examination lasted (Fig. 1 b). The spleen was enlarged and had, in the capillary phase, an appearance similar to that of the liver, suggesting that it also contained a hemangioma. Treatment with steroids was begun. After 3 months the hemangiomas of the skin began to fade and after a further 3 months, regression of the abdominal mass was observed. The treatment lasted 11 months with reduction of the dose after 3 months. Steroid face (as in Cushing's disease) and reduction in growth rate developed, but both returned to normal when the treatment ended. At 3 years of age the girl was re-admitted. She was 5 ern shorter in height than the average for her age, but otherwise appeared healthy. There was no hepatic or splenic mass. Angiography now demonstrated marked regression with only a few small hemangiomas of cavernous type. It was carried out as a selective coeliac angiography, using the same type and amount of contrast medium as at the previous angiography. The aorta was in a normal position. The liver and spleen were reduced in size compared with the first examination. The hepatic arteries were corkscrew-shaped, indicating a reduction in size of the liver (Fig. 2 a). The capillary phase had a normal appearance except for two small circles, maximum
Downloaded from acr.sagepub.com at UNSW Library on July 27, 2015
65
HEMANGIOMA OF THE LIVER
a
b
Fig. 3. Case 2. a) Selective coeliac angiography. Ectatic hepatic artery, instant filling of a large hemangioma. b) Capillary phase, irregular areas of contrast medium.
size 8 mm, with a dense collection of pools of contrast medium (Fig. 2 b). The veins filled at the end of the examination, which lasted for 10 seconds. Case 2. A girl, born prematurely, with a birth weight of 2500 g. There was some slight postnatal jaundice and some oedema of the right leg which disappeared spontaneously. At 2 years of age she was examined because of hemangioma-like changes in the left arm and leg. When 3 years old hepatomegaly was detected. At 6 years of age she was again admitted to hospital. Hemangiomas in the face and left arm were noticed, and at angiography a huge hemangioma of the liver was found. The angiography was carried out with Isopaque 350, starting with 20 ml in the abdominal aorta. After seeing the huge process, 40 ml were injected selectively into the coeliac axis demonstrating some ectasy of the hepatic artery. Rapid filling of numerous vessels was seen throughout almost the whole liver, which was enlarged (Fig. 3 a). Irregular areas of contrast medium appeared in some places, arranged in circles or rings, and at other sites scarcely visible (Fig. 3 b). Pool forming was slight, and a delicate venous filling was observed. In the lateral part of the right lobe, a rim of about 3 em was avascular, bordered by a capsular artery. It was thought to represent the residue of normal hepatic tissue, in which the vessels were not filled because the abnormal tissue 'swallowed up' all the contrast medium. No shunting was present. The patient was in good condition without any symptoms, so no treatment was given. Six years later she was re-admitted. In the meantime there had been no symptoms and she had developed normally. Cutaneous hemangiomas of the face and left arm were still present, as well as hepatomegaly. Angiography revealed that the hepatic hemangioma had grown in size, not only in absolute terms but also relative to the size of the patient. This angiography was performed with the same contrast medium as previously with 25 ml injected selectively in the coeliac artery. There was still ectasy of the common hepatic artery (Fig. 4 a), the vascularity of the liver had the same appearance, with the same lateral border as seen previously (Fig. 4 b). No venous filling was observed. 5 -765843
Downloaded from acr.sagepub.com at UNSW Library on July 27, 2015
66
J. TH. JENSEN AND T. KLINGE
a
b
Fig. 4. Same case as in Fig. 3. a) Selective coeliac angiography 6 years later. Further growth of the hemangioma. b) Capillary phase has the same appearance as 6 years earlier.
Discussion
Both patients had hemangiomas of the skin and an abdominal mass. The first presented with anemia and vomiting but the second had no symptoms at all. With the exception of the anaemia no pathology was found in laboratory tests of blood and urine in both cases. The first patient, at her first angiography, had a large hemangioma involving the whole liver and probably the spleen as well. The hemangioma resembled the one described by BERDON & BAKER, as a 'giant hepatic hemangioma'. It was large and had many irregular vascular pools, but only some were arranged in the characteristic configuration of the cavernous hemangioma. There were avascular areas but no dilatation of arteries or shunting to veins as is characteristic in hemangioendotheliomas. The hemangioma could be in an early stage of maturation, having left the endotheliomatous form but not quite reached the cavernous form. At the second examination, after steroid treatment for II months, extreme regression had occurred, as only two minute cavernous elements were left. The rest of the liver and spleen was normal. In the second patient the hemangioma was of a more capillary type. The absence of shunting and shortage of pools were not concordant with hemangioendothelioma
Downloaded from acr.sagepub.com at UNSW Library on July 27, 2015
HEMANGIOMA OF THE LIVER
67
or cavernous hemangioma, but it also differed from the usual description of the capillary hemangioma. This patient was not treated and experienced no discomfort, although the hemangioma grew in size. The main differential diagnosis is of hepatoma, where the feeding arteries are dilated, new-formed irregular tumour vessels are present and the circulation is rapid with early venous filling (Moss et coll., TAVERNIER et coll.). The second differential diagnosis is multiple metastases, which also have wide feeding arteries with irregular courses and tapering, tumour vessels, pools and rapid venous filling. These lesions are often multinodular (TAVERNIER et coIl., WATSON & BALTAXE 1971).
Conclusion
Different angiographic appearances of the hepatic hemangiomas, one previously not described, were encountered in these two patients. The maturation may vary between different parts of the tumour which results in varying width and course of the arteries, avascular areas alternate with irregular arrangements of lakes or pools; and the veins may be rapidly filled by shunts or their filling may be delayed. The presence of variation may explain the differences in previous descriptions and the lack of knowledge of the condition mentioned by Moss et coIl. The fact that under steroid treatment, the lesion regressed, and without treatment, increased in size like a tumour, is interesting even with the process of maturation in mind. Yet nothing conclusive can be said about treatment from this fact. No microscopy was obtained in our cases, and any attempt at biopsy has to be condemned. Angiography should replace histology in the diagnosis and control of this condition
SUMMARY Two cases of hepatic hemangiomas are described,diagnosed by angiography and controlled by repeat angiography after 2.5 and 6 years, respectively. The radiologic appearance is described and although it varies, angiography must be considered the safest method of diagnosis; biopsy is contraindicated. Steroid therapy seems to be a possible mode of treatment.
ZUSAMMENFASSUNG Zwei Hille mit einem Hamangiom der Leber werden beschrieben. Sie wurden mit Angiographie untersucht und 2,5 bzw. 6 Jahre spater mit erneuerter Angiographie kontrolliert. Obwohl die Rontgenbefunde variieren, muss Angiographie als die sicherste diagnostische Methode angesehen werden; Biopsie ist kontraindiziert. Die Steroidtherapie wird als eine mogliche Behandlungsmethode vorgeschlagen.
Downloaded from acr.sagepub.com at UNSW Library on July 27, 2015
68
J. TH. JENSEN AND T. KLINGE
RESUME Description de 2 cas d'hemangiome hepatique diagnostiques per angiographie et controles par des angiographies repetees au bout de 2,5 et 6 ans. Les auteurs decrivent l'aspect radiologique; bien qu'il soit variable, I'angiographie doit etre consideree comme la methode de diagnostic la plus inoffensive. La biopsie est contre-indiquee, Les auteurs signalent une eventuelle technique de traitement avec des steroides.
REFERENCES ARIEL J. M. and PACK T.: Cancer and allied diseases of infancy and childhood. Little, Brown & Co. Toronto 1960. BERDON W. E. and BAKER D. H.: Giant hepatic hemangioma with cardiac failure in the newborn infant. Radiology 92 (1969), 1523. CROCHER D. W. and CLELAND R. S.: Infantile hemangioendothelioma of the liver. Pediatrics 19 (1957), 596. DEHNER L. P. and ISHAK K. G.: Vascular tumours of the liver in infants and children. Arch. Path. 92 (1971), 101. DUKEN B. G., HART H. CH., IMHAF J. W. and SLUITER J. T. F.: Benign hemangioma of the liver. The significance of selective angiography. Radial. Clin. BioI. 40 (1971), 50. EDMONDSON H. A.: Differential diagnosis of tumours and tumour like lesions of liver in infancy and childhood. Amer. J. Dis. Child. 91 (1956), 168. FREDENS M.: Angiography in primary hepatic tumours in children. Acta radiol. Diagnosis 8 (1969), 193. GOLDBERG S. J. and FONKALSRUD E.: Successful treatment of hepatic hemangioma with corticosteroids. J. Amer. med. Ass. 208 (1969), 2473. KAGAN A. R., JAFFE H. L. and KENNAMER R.: Hemangioma of the liver treated by irradiation. J. nucl. Med. 12 (1971), 835. McLoUGHLIN M. J.: Angiography in cavernous hemangioma of the liver. Amer. J. Roentgenol. 113 (1971), 50. Moss A. A., CLARK R. E., PALULEINSKAS A. J. and DELoRIMIER A. A.: Angiographic appearance of benign and malignant hepatic tumours in infants and children. Amer. J. Roentgenol. 113 (1971), 61. PANTOJA E.: Angiography in liver hemangioma. Amer, J. Roentgenol. 104 (1968), 874. POLLARD J. J., NEBESAR R. A. and MATTASO L. F.: Angiographic diagnosis of benign diseases of the liver. Radiology 86 (1966), 276. REUTER S. R. and REDMAN H. C.: Gastrointestinal angiography, p. 110. W. B. Saunders Co. Philadelphia, London, Toronto 1972. ROSCH J. and STECKEL R. J.: Selective angiography of the abdominal viscera. In: Golden's diagnostic radiology, Section 18. p, 36. The Williams & Wilkins Co., Baltimore 1972. SELKE A. C. and CORWELL S. H.: Infantile Hepatic Hemangioendotheliomas. Amer. J. Roentgenol. 106 (1969), 200. TAVERNIER J., DIARD F., DELORME G., LARROUDE CH. et GRELET PH.: L'angiographie des hemangiomes du foie. J. Radiol. Electrol. 53 (1972),493. VIDEBAEK AA.: Hemangio-endothelioma of the liver. Acta peediat, 33 (1964), 129. WATSON R. C. and BALTAXE H. A.: The angiographic appearance of primary and secondary tumours of the liver. Radiology 101 (1971), 539.
Downloaded from acr.sagepub.com at UNSW Library on July 27, 2015
HEMANGIOMA OF THE LIVER Report of two cases J. TH. JENSEN and T. KLINGE Angiography in hemangioma of the liver has been reported severa times in the last few years. The use of angiography both in establishing the primary diagnosis and in a follow-up examination has, however, been reported only once before (TAVERNIER et colI. 1972) after intervening surgery. In the two cases presented in this report a follow-up examination was carried out after steroid treatment in one patient, and after 6 years without treatment in the other case. Hemangiomas are thought to be embryonic sequestrations of unipotent angioblastic cells (ARIEL & PACK 1960). They are usually divided into the following groups: Hemangioendotheliomas, capillary and cavernous hemangiomas. The main difference between the groups is the amount of endothelial cells and the size of the blood filled spaces. Most authors describe the presence of a maturing process via the cavernous type to spontaneous remission, that corresponds with the occurrence of hemangiomas of mixed type in the liver, i.e. with centres of different degree of maturation. The lesion must be regarded as benign in most cases, although sometimes it has a clinically malignant course. The description of metastases by some authors is regarded by others as multicentric development of new growths and not as metastases. The exact incidence is not known. McLoUGHLIN (1971) has mentioned a frequency of approximately 0.35 per cent of cavernous hemangiomas in autopsies, but the tendency for spontaneous remission makes such a statement uncertain. Hemangioendotheliomas, particularly the mixed type, are most common in children. Submitted for publication 6 September 1974. Acta Radiologica Diagnosis 17 (1976) Fasc, 1 January
Downloaded from acr.sagepub.com at UNSW Library on July 27, 2015
61
62
J. TH. JENSEN AND T. KLINGE
The cavernous type more often occurs in adults with a predomination of females in a ratio of 9: 1 (PANTOJA 1968). The hemangiomas are usually small and multiple, but may be diffuse throughout one lobe or the whole liver. They are commonly accompanied by hemangiomas of the skin. The histology is described by CROCHER & CLELAND (1957), DEHNER & ISHAK (1971) and EDMONDSON (1956) and others. The clinical symptoms may be abdominal discomfort with enlargement of the liver, and cardiac failure in small infants. Thrombocytopenia and hemorrhage following rupture or necrosis may occur, but many angiomas are found accidentally at angiography. A large number of liver hemangiomas probably exist without any clinical symptoms (TAVERNIER et coll., REUTER & REDMAN 1972). A warning must be given against any attempt at biopsy because of the great danger of a massive hemorrhage (KAGAN et coll. 1971, PANTOJA). Angiography is thus the best and safest procedure for arriving at the correct diagnosis, and the best results are obtained with selective coeliac angiography.
Treatment. Concerning treatment, the natural tendency for spontaneous remission is well recognized. When surgery is contemplated (e.g. local exision, lobectomy or ligation of a feeding artery), angiography is of great value in determining the exact anatomy of the lesion (TAVERNIER et coll.). The use of radiation therapy is discussed by many authors and treatment with steroids is suggested in combination with radiation therapy by DEHNER & ISHAK. One successful case treated with steroids and cardiac treatment is reported by GOLDBERG & FONKALSRUD (1969). The prognosis is generally good but there is the possibility of cardiac failure, and hemorrhages may be fatal. Radiographic findings. An enlarged liver or an elevated or abnormally domed right hemidiaphragm may sometimes be found on a conventional film of the abdomen. Calcific deposits appearing as scattered irregular flecks of calcium, placed in the septa may occur. Calcified phleboliths are rarely seen (McLOUGHLIN, PANTOJA). The angiographic appearance has been described several times and in reviewing the literature the following points seem to be established: the best known and most common angiographic finding is that of the cavernous hemangioma (among others DUKEN et coll. 1971, McLOUGHLIN, PANTOJA, POLLARD et coll. 1966, TAVERNIER et coll.). The coeliac and the hepatic arteries are of normal size and appearance, but may be displaced around masses or crowded together. Late in the arterial phase, irregular areas of contrast medium appear to form labyrinths of pools or lakes, usually well demarcated, arranged in clusters or rings, often with an avascular centre. The retention of the contrast medium in these spaces lasts a long time, up to 30 seconds, and the venous phase is delayed. KAGAN et coil. and ROSCH & STECKEL (1972) report slight dilatation of the coeliac artery and the feeding hepatic arterial branches. The other condition, often described, is hemangioendothelioma in newborn and small infants (BERDON & BAKER 1969, Moss et coll. 1971, SELKE & CORWELL 1969, PANTOJA). The feeding coeliac and hepatic arteries are enlarged, the circulation rate is increased and bizarre irregular spaces surrounding large avascular areas are rapidly
Downloaded from acr.sagepub.com at UNSW Library on July 27, 2015
HEMANGIOMA OF THE LIVER
63
a b Fig. 1. Case 1. a) Abdominal aortography. Enlarged liver with stretched hepatic arteries of normal width. b) Capillary phase. Numerous irregular pools of contrast medium.
filled. The fast venous filling, after about one second, indicates the presence of arteriovenous shunting. Moss et coll. found numerous pathologic vessels in the arterial phase. Most of these children had cardiac insufficiency and several died, but in many cases no cardiac malformation was found. It seems that the great amount of blood shunted through the liver overburdens the heart. The last condition, the capillary hemangioma, resembles the hemangioendothelioma and several authors do not distinguish between the two types. According to TAVERNIER, the capillary hemangioma has normal-sized feeding arteries. The seemingly avascular masses, surrounded by irregular vascular spaces with a rapid shunting of blood to the veins, resemble the appearances of hemangioendothelioma, but there may be a more homogeneous appearance of the liver as well. The avascular hemangioma (WATSON & BALTAXE 1971) may be of this type. The avascular masses are thought to be areas with a large amount of endothelium compressing the vascular channels. The angiographic appearance with mixed endothelio-capillary. and cavernous elements corresponds to the theory of maturing. The proportion between the two elements decides the type, but the sudden jump from arteriovenous shunt to delayed venous filling in the cavernous type is difficult to understand and no explanation for this is found in the literature. It could be caused by the increasing width of the vascular bed, when small capillaries are replaced by wide cavernouslakes. At the same time there may be some thrombosis in' the irregular arteriovenous shunts. Extensive intravascular coagulation in hemangiomas of the liver is described by DIJKEN et colI.
Downloaded from acr.sagepub.com at UNSW Library on July 27, 2015
64
J. TH. JENSEN AND T. KLINGE
a
b
Fig. 2. Same case as in Fig. 1. a) Selective coeliac angiography 2.5 yearslater. Regression following steroidtreatment. b) Capillary phase. Only two small areas of cavernous hemangiomas.
Case reports Case 1. A girl born three weeks prematurely was fully developed with a weight of 3040 g. At 3 weeks of age hemangiomas of the skin were observed and one week later, a tendency to vomit developed. When 5 months old the girl became agitated with coughing, and one month later a right abdominal mass was detected, extending into the left hypochondrium. The girl was admitted to hospital and an anaemia was found, Hb 8.2 g %. Cardiac and liver function was normal. Angiography was performed via the femoral artery. Abdominal aortography was carried out with injection of 12 ml of Isopaque 260. The proximal part ofthe aorta was displaced to the left. The liver was enlarged and the hepatic arteries stretched, but of normal size (Fig. 1 a). In the capillary phase numerous irregular pools werefilled, partly diffuse and partly arranged in nests with a maximum diameter of 22 mm with an avascular centre. Later some diffuse filling occurred, but no veins were observed during the 11 seconds the examination lasted (Fig. 1 b). The spleen was enlarged and had, in the capillary phase, an appearance similar to that of the liver, suggesting that it also contained a hemangioma. Treatment with steroids was begun. After 3 months the hemangiomas of the skin began to fade and after a further 3 months, regression of the abdominal mass was observed. The treatment lasted 11 months with reduction of the dose after 3 months. Steroid face (as in Cushing's disease) and reduction in growth rate developed, but both returned to normal when the treatment ended. At 3 years of age the girl was re-admitted. She was 5 ern shorter in height than the average for her age, but otherwise appeared healthy. There was no hepatic or splenic mass. Angiography now demonstrated marked regression with only a few small hemangiomas of cavernous type. It was carried out as a selective coeliac angiography, using the same type and amount of contrast medium as at the previous angiography. The aorta was in a normal position. The liver and spleen were reduced in size compared with the first examination. The hepatic arteries were corkscrew-shaped, indicating a reduction in size of the liver (Fig. 2 a). The capillary phase had a normal appearance except for two small circles, maximum
Downloaded from acr.sagepub.com at UNSW Library on July 27, 2015
65
HEMANGIOMA OF THE LIVER
a
b
Fig. 3. Case 2. a) Selective coeliac angiography. Ectatic hepatic artery, instant filling of a large hemangioma. b) Capillary phase, irregular areas of contrast medium.
size 8 mm, with a dense collection of pools of contrast medium (Fig. 2 b). The veins filled at the end of the examination, which lasted for 10 seconds. Case 2. A girl, born prematurely, with a birth weight of 2500 g. There was some slight postnatal jaundice and some oedema of the right leg which disappeared spontaneously. At 2 years of age she was examined because of hemangioma-like changes in the left arm and leg. When 3 years old hepatomegaly was detected. At 6 years of age she was again admitted to hospital. Hemangiomas in the face and left arm were noticed, and at angiography a huge hemangioma of the liver was found. The angiography was carried out with Isopaque 350, starting with 20 ml in the abdominal aorta. After seeing the huge process, 40 ml were injected selectively into the coeliac axis demonstrating some ectasy of the hepatic artery. Rapid filling of numerous vessels was seen throughout almost the whole liver, which was enlarged (Fig. 3 a). Irregular areas of contrast medium appeared in some places, arranged in circles or rings, and at other sites scarcely visible (Fig. 3 b). Pool forming was slight, and a delicate venous filling was observed. In the lateral part of the right lobe, a rim of about 3 em was avascular, bordered by a capsular artery. It was thought to represent the residue of normal hepatic tissue, in which the vessels were not filled because the abnormal tissue 'swallowed up' all the contrast medium. No shunting was present. The patient was in good condition without any symptoms, so no treatment was given. Six years later she was re-admitted. In the meantime there had been no symptoms and she had developed normally. Cutaneous hemangiomas of the face and left arm were still present, as well as hepatomegaly. Angiography revealed that the hepatic hemangioma had grown in size, not only in absolute terms but also relative to the size of the patient. This angiography was performed with the same contrast medium as previously with 25 ml injected selectively in the coeliac artery. There was still ectasy of the common hepatic artery (Fig. 4 a), the vascularity of the liver had the same appearance, with the same lateral border as seen previously (Fig. 4 b). No venous filling was observed. 5 -765843
Downloaded from acr.sagepub.com at UNSW Library on July 27, 2015
66
J. TH. JENSEN AND T. KLINGE
a
b
Fig. 4. Same case as in Fig. 3. a) Selective coeliac angiography 6 years later. Further growth of the hemangioma. b) Capillary phase has the same appearance as 6 years earlier.
Discussion
Both patients had hemangiomas of the skin and an abdominal mass. The first presented with anemia and vomiting but the second had no symptoms at all. With the exception of the anaemia no pathology was found in laboratory tests of blood and urine in both cases. The first patient, at her first angiography, had a large hemangioma involving the whole liver and probably the spleen as well. The hemangioma resembled the one described by BERDON & BAKER, as a 'giant hepatic hemangioma'. It was large and had many irregular vascular pools, but only some were arranged in the characteristic configuration of the cavernous hemangioma. There were avascular areas but no dilatation of arteries or shunting to veins as is characteristic in hemangioendotheliomas. The hemangioma could be in an early stage of maturation, having left the endotheliomatous form but not quite reached the cavernous form. At the second examination, after steroid treatment for II months, extreme regression had occurred, as only two minute cavernous elements were left. The rest of the liver and spleen was normal. In the second patient the hemangioma was of a more capillary type. The absence of shunting and shortage of pools were not concordant with hemangioendothelioma
Downloaded from acr.sagepub.com at UNSW Library on July 27, 2015
HEMANGIOMA OF THE LIVER
67
or cavernous hemangioma, but it also differed from the usual description of the capillary hemangioma. This patient was not treated and experienced no discomfort, although the hemangioma grew in size. The main differential diagnosis is of hepatoma, where the feeding arteries are dilated, new-formed irregular tumour vessels are present and the circulation is rapid with early venous filling (Moss et coll., TAVERNIER et coll.). The second differential diagnosis is multiple metastases, which also have wide feeding arteries with irregular courses and tapering, tumour vessels, pools and rapid venous filling. These lesions are often multinodular (TAVERNIER et coIl., WATSON & BALTAXE 1971).
Conclusion
Different angiographic appearances of the hepatic hemangiomas, one previously not described, were encountered in these two patients. The maturation may vary between different parts of the tumour which results in varying width and course of the arteries, avascular areas alternate with irregular arrangements of lakes or pools; and the veins may be rapidly filled by shunts or their filling may be delayed. The presence of variation may explain the differences in previous descriptions and the lack of knowledge of the condition mentioned by Moss et coIl. The fact that under steroid treatment, the lesion regressed, and without treatment, increased in size like a tumour, is interesting even with the process of maturation in mind. Yet nothing conclusive can be said about treatment from this fact. No microscopy was obtained in our cases, and any attempt at biopsy has to be condemned. Angiography should replace histology in the diagnosis and control of this condition
SUMMARY Two cases of hepatic hemangiomas are described,diagnosed by angiography and controlled by repeat angiography after 2.5 and 6 years, respectively. The radiologic appearance is described and although it varies, angiography must be considered the safest method of diagnosis; biopsy is contraindicated. Steroid therapy seems to be a possible mode of treatment.
ZUSAMMENFASSUNG Zwei Hille mit einem Hamangiom der Leber werden beschrieben. Sie wurden mit Angiographie untersucht und 2,5 bzw. 6 Jahre spater mit erneuerter Angiographie kontrolliert. Obwohl die Rontgenbefunde variieren, muss Angiographie als die sicherste diagnostische Methode angesehen werden; Biopsie ist kontraindiziert. Die Steroidtherapie wird als eine mogliche Behandlungsmethode vorgeschlagen.
Downloaded from acr.sagepub.com at UNSW Library on July 27, 2015
68
J. TH. JENSEN AND T. KLINGE
RESUME Description de 2 cas d'hemangiome hepatique diagnostiques per angiographie et controles par des angiographies repetees au bout de 2,5 et 6 ans. Les auteurs decrivent l'aspect radiologique; bien qu'il soit variable, I'angiographie doit etre consideree comme la methode de diagnostic la plus inoffensive. La biopsie est contre-indiquee, Les auteurs signalent une eventuelle technique de traitement avec des steroides.
REFERENCES ARIEL J. M. and PACK T.: Cancer and allied diseases of infancy and childhood. Little, Brown & Co. Toronto 1960. BERDON W. E. and BAKER D. H.: Giant hepatic hemangioma with cardiac failure in the newborn infant. Radiology 92 (1969), 1523. CROCHER D. W. and CLELAND R. S.: Infantile hemangioendothelioma of the liver. Pediatrics 19 (1957), 596. DEHNER L. P. and ISHAK K. G.: Vascular tumours of the liver in infants and children. Arch. Path. 92 (1971), 101. DUKEN B. G., HART H. CH., IMHAF J. W. and SLUITER J. T. F.: Benign hemangioma of the liver. The significance of selective angiography. Radial. Clin. BioI. 40 (1971), 50. EDMONDSON H. A.: Differential diagnosis of tumours and tumour like lesions of liver in infancy and childhood. Amer. J. Dis. Child. 91 (1956), 168. FREDENS M.: Angiography in primary hepatic tumours in children. Acta radiol. Diagnosis 8 (1969), 193. GOLDBERG S. J. and FONKALSRUD E.: Successful treatment of hepatic hemangioma with corticosteroids. J. Amer. med. Ass. 208 (1969), 2473. KAGAN A. R., JAFFE H. L. and KENNAMER R.: Hemangioma of the liver treated by irradiation. J. nucl. Med. 12 (1971), 835. McLoUGHLIN M. J.: Angiography in cavernous hemangioma of the liver. Amer. J. Roentgenol. 113 (1971), 50. Moss A. A., CLARK R. E., PALULEINSKAS A. J. and DELoRIMIER A. A.: Angiographic appearance of benign and malignant hepatic tumours in infants and children. Amer. J. Roentgenol. 113 (1971), 61. PANTOJA E.: Angiography in liver hemangioma. Amer, J. Roentgenol. 104 (1968), 874. POLLARD J. J., NEBESAR R. A. and MATTASO L. F.: Angiographic diagnosis of benign diseases of the liver. Radiology 86 (1966), 276. REUTER S. R. and REDMAN H. C.: Gastrointestinal angiography, p. 110. W. B. Saunders Co. Philadelphia, London, Toronto 1972. ROSCH J. and STECKEL R. J.: Selective angiography of the abdominal viscera. In: Golden's diagnostic radiology, Section 18. p, 36. The Williams & Wilkins Co., Baltimore 1972. SELKE A. C. and CORWELL S. H.: Infantile Hepatic Hemangioendotheliomas. Amer. J. Roentgenol. 106 (1969), 200. TAVERNIER J., DIARD F., DELORME G., LARROUDE CH. et GRELET PH.: L'angiographie des hemangiomes du foie. J. Radiol. Electrol. 53 (1972),493. VIDEBAEK AA.: Hemangio-endothelioma of the liver. Acta peediat, 33 (1964), 129. WATSON R. C. and BALTAXE H. A.: The angiographic appearance of primary and secondary tumours of the liver. Radiology 101 (1971), 539.
Downloaded from acr.sagepub.com at UNSW Library on July 27, 2015
