Case Report: Cavernous Hemangioma of the Liver

Case Report: Cavernous Hemangioma of the Liver

Case Report: Cavernous Hemangioma of the Liver CONNIE T. DuPRE, MD, RUTH-MARIE E. FINCHER, MD ABSTRACT: Although cavernous hemangioma is the most co...

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Case Report: Cavernous Hemangioma of the Liver CONNIE T. DuPRE, MD,

RUTH-MARIE E. FINCHER, MD

ABSTRACT: Although cavernous hemangioma is the most common benign tumor of the liver, controversy persists regarding diagnosis and management of these lesions. With the development of multiple noninvasive modalities to visualize the liver, hepatic cavernous hemangiomas are recognized with increased frequency. The authors report the unusual case of a postmenopausal woman on no exogenous estrogen therapy who had a cavernous hemangioma that remained stable for approximately 10 years before dramatically increasing in size. This patient illustrates the vague symptoms associated with cavernous hemangiomas and the unpredictability of growth. Although estrogens have been reported trophic, this patient had no exogenous or endogenous estrogen supply, yet. her lesion reached massive proportions. Modalities necessary to assure accurate diagnosis and factors influential in management are discussed. KEY INDExING TERMS: Cavernous hemangioma; Hepatic tumors [Am J Med Sci 1992; 303(4):241244.]

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avernous hemangioma is the most common benign tumor of the liver with an estimated prevalence of up to 7% in the United States. 1,2 The only liver tumor more common is metastatic disease. However, the true prevalence of cavernous hemangioma is unknown because most lesions are small and asymptomatic, and are diagnosed incidentally or at autopsy. With the advent of modalities to noninvasively visualize the liver, previously undiagnosed, asymptomatic lesions are now detected. The internist, therefore, must differentiate between these common benign and malignant lesions to make appropriate diagnostic and therapeutic decisions. Although cavernous hemangiomas have been reported in the literature since 1861, the natural history, From the Medical College of Georgia School of Medicine, Department of Medicine, Section of General Internal Medicine, Augusta, Georgia. We thank Margaret Rebecca Williams for typing the manuscript. Correspondence: Connie T. DuPre, MD, Department of Medicine, HB-201O, Medical College of Georgia, Augusta, GA 30912. THE AMERICAN JOURNAL OF THE MEDICAL SCIENCES

particularly which factors influence growth, is not yet well-delineated.3,4 Hemangiomas are thought to be congenital malformations or hamartomas that increase in size with growth of the liver and thereafter by ectasia. 3- 5 Factors responsible for ectatic growth are illdefined. Estrogens have been postulated to be trophic but their role has not been confirmed. 2,6 Most lesions remain small although patients rarely develop massive lesions with impressive symptomatology. Because the stimuli for enlargement of cavernous hemangiomas are unknown, accurate prediction of growth cannot be made. Management, therefore, remains essentially empirical or anecdotal. We present the case of a postmenopausal woman whose cavernous hemangioma was noted incidentally during abdominal surgery. The hemangioma subsequently enlarged, then remained stable for several years before becoming massive and symptomatic. This case illustrates the vague symptomatology characteristic of hemangioma and the unpredictability of growth. Despite literature which suggests the trophic role of estrogens, this patient had no exogenous or endogenous estrogen supply.2,6 Case Report A 27-year-old woman underwent a hysterectomy and was intraoperatively noted to have a small liver mass. In 1983, at age 36, she presented with vague abdominal pain. An upper gastrointestinal series revealed extrinsic compression of the stomach. Computerized tomography of the abdomen revealed a liver mass in the right lobe. Angiogram showed the tumor blush characteristic of cavernous hemangioma, which involved the entire right lobe of the liver with extension to the left lobe. All laboratory studies were unremarkable including a complete blood count and automated analysis of serum chemistries. Because the lesion was bilateral, resection was not recommended. Sclerotherapy was suggested but the patient refused. She was seen in followup on several occasions without appreciable change in the size of her liver but with resolution of her symptoms. At the time of a clinic visit in 1983, the liver edge was palpable 7 cm below the right costal margin. The patient complained of hot flashes and anxiety, which were thought to be secondary to surgical menopause. Although she requested hormonal therapy, she received none because of the potential trophic role of estrogens. The patient remained asymptomatic until 1990 when at age 46 she presented to the clinic because of a 65-pound weight loss during the preceding 2 years. The patient's liver edge was then 21 cm below the right costal margin. A contrasted computerized tomographic scan of the abdomen was consistent with cavernous hemangioma although hepatoma could not be excluded (Figure 1). A nuclear medicine tagged red blood

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Figure 1. Computerized tomographic scan with intravenous contrast demonstrates enhancement of the cavernous hemangioma measuring 15 cm anteroposterioriy by 20 cm transversely by 28 cm longitudinally at its greatest dimensions. There is a smaller low-density area in the posterior aspect of the right lobe superiorly, which represents an extension of the mass or a "satellite lesion."

count (RBC) scan suggested a secondary tumor element in the left lobe. Magnetic resonance imaging revealed a cavernous hemangioma of the right and left lobes of the liver with a small satellite lesion (Figure 2). The hemangioma was massive, measuring 15 cm anterioriy, 22 cm transversely, and 30 cm longitudinally at its greatest diameter between the right and left hepatic lobes. A smaller lesion was present in the posteriomedial aspect of the right lobe measuring 2 by 4 by 5 cm. The patient was referred for surgical evaluation and liver transplantation was recommended.

Discussion

Hemangiomas are composed of a predominance of cystic ally dilated vascular spaces. The lining consists

of endothelial cells. The septate walls are composed of a nondescript fibrous tissue with relatively thin septae.7 Occasionally, cavernous hemangiomas contain clotted blood.3 ,4 Cavernous hemangiomas are most frequently detected in the third through fifth decades but can occur in any age group. Symptomatic lesions occur more commonly in women than men. 2- 4,S Influences on growth of cavernous hemangiomas remain speculative. Although they are capable of reaching enormous size as in our patient, lesions greater than 4 cm are unusual and are designated giant hemangiomas.3 ,4 Growth seems to occur by progressive ectasia, maintaining a dissectable plane between the hemangioma and normal liver parenchyma. 3,5 As they do not demonstrate infiltrative growth, further enlargement should not preclude resection. 3,4 One series reported that patients with lesions greater than 4 cm were symptomatic while those less than 4 cm were asymptomatic. 9 Another report concluded that most hemangiomas are asymptomatic unless they are greater than 10 cm in diameter.3 Our patient's 30-centimeter hemangioma is among the largest reported in the literature. Symptoms, when present, are often nonspecific and are related to growth and encroachment of viscera such as vague abdominal pain, abdominal fullness, early satiety, nausea, or emesis.s Fever may be present. Rare presentations include obstructive jaundice, biliary colic, gastric outlet obstruction, and spontaneous hemorrhage. 3,4 Pain is most likely related to stretching and inflammation of Glisson's capsule.3,4 Thrombocytopenia and hypofibrogenemia have also been associated with cavernous hemangioma of the liver presumably related to consumption of coagulation factors by active thrombosis. 3 Physical examination is generally unremarkable unless hepatomegaly is present. No specific blood test is

Figure 2. (Left) Proton-weighted image reveals an isodense mass measuring 15 cm anteroposterioriy by 22 cm transversely by 30 cm longitudinally between the left and right hepatic lobes. A smaller lesion is present in the posteromedial aspect of the right lobe. It measures 2 cm by 4 cm by 5 cm. (Right) T2-weighted image demonstrates the lesions as hyperintense, consistent with a cavernous hemangioma.

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of value in confirming the diagnosis. Ultrasound appearance is variable but is most often described as hyperechoic.3 Ultrasound cannot differentiate hemangioma from hepatocellular carcinoma, focal nodular hyperplasia, hepatic adenoma, or a solitary metastatic lesion. 3 Computerized tomography has proved most useful in the diagnosis of hemangiomas. The uncontrasted scan demonstrates the hemangioma as a well-demarcated hypodense mass. Following contrast, the center of the hemangioma remains hypodense. There is a peripheral zone of enhancement that varies in thickness and sometimes has a corrugated inner margin (tumor blush).3 Selective hepatic angiography demonstrates a characteristic pattern consisting of normal-sized hepatic arteries without neovascularity or "corkscrewing." Classic for cavernous hemangiomas is the prompt filling of the large blood filled spaces of the hemangioma with contrast medium producing the so-called "cotton-wool" appearance that surrounds the feeding hepatic arteries. 4 However, there have been reports of normal angiograms in patients with cavernous hemangioma. 2,10,ll The nuclear medicine sulphur colloid-tagged RBC study is specific for cavernous hemangioma. The sulphur colloid binds to normal liver and, therefore, demonstrates the hemangioma as a cold defect on the scan. The tagged RBC study demonstrates vascularity of a lesion. The correlation of a cold defect on sulphur colloid scan with increased uptake on the tagged RBC study confirms the diagnosis. 2,l1 Magnetic resonance imaging has been shown to have a high degree of specificity in the diagnosis of hepatic hemangiomas but expense limits its application. 2 ,3,l1 Because of the risk of significant hemorrhage, needle biopsy of these lesions should not be performed and diagnosis cannot be made by this procedure.3,10-12 Since the original description in the English literature of cavernous hemangiomas, "A Clinical Treatise on Disease of the Liver,"3 several surgical reviews have been published. Trastek 1 reviewed 49 cases of cavernous hemangioma, which measured at least 4 cm at the Mayo Clinic from 1960 through 1980. Thirty-six patients were observed without surgical intervention. The mean size of the lesions was 8.8 cm. During the followup period of as much as 15 years (mean 5.5), four lesions increased in size but no patient noted increased symptoms or spontaneous hemorrhage. The authors concluded that the natural history of these lesions is benign and should be considered when determining management of asymptomatic lesions or of lesions large enough to pose significant operative risk. Since Trastek'sl original publication, the followup period has been extended to 21 years (mean 12.5 years).3 During the extended followup, 25 patients have remained asymptomatic and four have died of unrelated causes. The two patients with symptomatic lesions at time of THE AMERICAN JOURNAL OF THE MEDICAL SCIENCES

initial presentation have remained symptomatic with little appreciable growth of the lesion. 1o Surgical resection is considered the therapy of choice for symptomatic lesions.2,3,1O,12 This recommendation has been based primarily on anecdotal evidence, weighing the risk of potential further growth of a symptomatic lesion with the possibility of spontaneous life-threatening rupture. There are only 21 cases of spontaneous life-threatening rupture of cavernous hemangioma of the liver in the literature. 1- 3 Although there is a risk of life-threatening rupture, the literature suggests it is rare. Therefore, the degree of symptomatology experienced by the patient remains influential in determining management. Although estrogens have been postulated, the trophic factors for growth of hemangiomas remain unknown. Sinanan 2 reviewed the 20-year experience with neoplastic lesions of the liver in Seattle and found 12 patients with liver hemangiomas. Eight were women with prior or concurrent estrogen use, six of whom were on replacement and two on birth control pills. Seven of the 12 patients were observed for 2-6 years. Estrogen therapy was discontinued in two patients during the followup period and all the hemangiomas remained unchanged clinically and symptomatically. No regression of any lesion was noted. Mathieu6 described an association between estrogen use, focal nodular hyperplasia, and cavernous hemangioma and postulated a potential trophic role of oral contraceptives. Another study reviewed 15 patients with cavernous hemangiomas of the liver ranging from 2.5 to 20 cm in diameter, all of which were resected.1O Four of the 15 were multifocal. All symptoms resolved following resection and patients remained asymptomatic during the period of followup of 2-7 months. There were no operative complications. Because of little operative morbidity and no mortality, the authors concluded resection could be performed in patients in whom the diagnosis is equivocal. The authors reported that despite the relative specificity of noninvasive radiologic modalities, even arteriography could not assure the diagnosis of cavernous hemangioma. Two patients at that center preoperatively thought to have hemangiomas actually had malignancies. Therefore, symptoms may prompt partial resection and establishment of a definitive diagnosis prior to surgery may not be essential. The authors emphasized that their experience could not be generalized and should be reserved for specialized liver centers. Davis 11 reported three cases of symptomatic cavernous hemangiomas of the liver with normal angiograms. In one of the largest studies undertaken, Iwatsuki and Starzp2 reviewed 547 consecutive patients who underwent hepatic resection from 1964 to 1989. Of the 547, 219 had benign lesions excised for therapeutic reasons or because malignancy could not be excluded. Of the 219 benign lesions noted, 114 were cavernous hemangiomas. Patients were divided into four groups 243

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based on their presenting symptomatology. Group 1 presented with severe pain associated with spontaneous rupture or hemorrhage into the necrotic center of a hemangioma; group 4 patients were asymptomatic. However, group 4 patients had a history of a malignant lesion; therefore, their diagnosis could not be absolutely confirmed preoperatively. There were no operative deaths; however, 14 ofthe 114 resected had significant complications including three with postoperative bleeding requiring reexploration, four with prolonged bile leak, five with subphrenic abscess, and two with acute viral hepatitis. The followup was limited to a single contact which ranged from 3 months to 15 years postresection with a median of 4.5 years. Of the original 114 patients, 94 were contacted. Eighty-seven percent of the patients in group 1, 95.5% of the patients in group 2, and 78.6% ofthe patients in group 3 were free of symptoms at time of followup. Four percent of patients in group 2 and 21.4% of patients in group 3 complained of continued symptomatology despite resection. The authors concluded that it was safe to observe asymptomatic, incidentally noted cavernous hemangiomas as long as the lesion was small and diagnosis was certain. Spontaneous rupture of cavernous hemangiomas is uncommon and percutaneous needle biopsy of suspected hemangioma should be avoided because of the danger of life-threatening hemorrhage and fact that diagnosis cannot be confirmed by this procedure. They further concluded that asymptomatic lesions should be serially studied and any change in the character or size of the lesions should be investigated. They added that noninvasive modalities can often be incorrect and any equivocal lesion should be excised. Their results concurred with those of Nichols3 who felt that lesions greater than 10 cm should be excised because of increased incidence of central necrosis with hemorrhage and possible rupture. The majority of reviews advocate the excision of large symptomatic cavernous hemangiomas because of the minimal operative morbidity and mortality. Excision in the majority of cases results in relief of symptoms. There is anecdotal evidence to support the excision of asymptomatic hemangiomas greater than 10 cm because of the associated risk of central necrosis and hemorrhage. However, which patients with lesions of this size will exhibit this complication cannot be predicted. These reviews underscore the necessity of multiple noninvasive modalities to confirm the diagnosis

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and the need for excision in cases in which the diagnosis remains equivocal after noninvasive evaluation. Our patient exemplifies the unpredictable nature of cavernous hemangiomas. Despite the multiple published reviews, there are no factors known to predict growth. Our patient was vaguely symptomatic in 1983 but her symptoms remained stable for approximately 7 years before her lesions reached massive proportions and resulted in dramatic symptomatology. Noninvasive evaluation with multiple radiologic modalities confirmed the diagnosis of cavernous hemangioma. Davisl l concluded that the combination of the technium Tc99m tagged RBC study and dynamic contrast computed tomography confirms the diagnosis in more than 90% of the cases. Magnetic resonance imaging is sensitive and specific but because of cost, its use should be limited. We conclude that the internist is obligated to evaluate all suspected cavernous hemangiomas noninvasively and refer all equivocal lesions for excision. Further, it is probably appropriate to serially observe cavernous hemangioma that are asymptomatic but large, and recommend excision only with the development of symptoms. Further studies are needed to delineate factors that predict growth. References 1. Trastek VF, Van Heerden JA, Sheedy PF, Adson MA: Cavernous hemangiomas of the liver: Resect or observe? Am J Surg 145:

49-53, 1983. 2. Sinanan MN, Marchioro T: Management of cavernous hemangioma of the liver. Am J Surg 157:519-522, 1989. 3. Nichols FC III, Van Heerden JA, Weiland LH: Benign liver tumors. Surg Clin N Am 69:297-314, 1989. 4. Grieco MB, Miscall BG: Giant hemangiomas of the liver. Surg Gynecol Obstet 147:783-786,1978. 5. Adson MA: Surgery symposium. Mass lesions of the liver. Mayo Clin Proc 61:362-368, 1986. 6. Mathieu D, Zafrani ES, Anglade MC, Dhumeaux D: Association of focal nodular hyperplasia and hepatic hemangioma. Gastroenterology 97:154-157, 1989. 7. Ochsner JL, Halpert B: Cavernous hemangioma of the liver. Surgery 43:577-582, 1958. 8. Shockman AT, Wenger JA, Kohn NN: Hemangioma of the liver. Gastroenterology 45:425-428, 1963. 9. Adam YG, Huvous AG, Fortner JG: Giant hemangiomas of the liver. Ann Surg 172:239, 1970. 10. Starzl TE, Koep LJ, Weil R, Fennell RH, Iwatsuki S, Kano T, Johnson M: Excisional treatment of cavernous hemangioma of the liver. Ann Surg 192:25-27, 1980. 11. Davis WD, Ferrante WA, Tutton RH, Bowen JC: Hepatic hemangioma with normal angiograms. Three case reports. JAMA 263:983-986, 1990. 12. Iwatsuki S, Todo S, Starzl TE: Excisional therapy for benign hepatic lesions. Gynecol Obstet 171:240-246, 1990.

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