Article

Hepatic angiomyolipoma: Two case reports of caudate-based lesions and review of the literature

Department of Surgery, St. Vincent Medical Center, Los Angeles, California 90057-1904, USA.
Liver transplantation and surgery: official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society 02/1997; 3(1):46-53. DOI: 10.1002/lt.500030107
Source: PubMed

ABSTRACT

Two case reports of hepatic angiomyolipoma, both originating in the caudate lobe, are reported with a review of the literature. The liver is the second most common site of angiomyolipoma, an uncommon benign tumor of mixed mesenchymal origin. It is commonly diagnosed following abdominal pain or as an asymptomatic mass discovered on abdominal ultrasound or computed tomography scan. Of 74 cases reported, the lesions ranged from 0.3 to 36 cm in diameter and are noted between the first and eighth decade, with predominant female predilection. The right lobe is the most common site, with lesions arising in the caudate lobe comprising only five cases. The natural history of the hepatic lesion is unknown. Malignant invasion or metastatic disease has not been documented. Hepatic and renal angiomyolipoma can occur concurrently (13 of 60 cases), although the majority are not biopsy proven. Multicentric hepatic disease occurs. The correlation between tuberous sclerosis and hepatic angiomyolipoma is not confirmed histologically and occurs rarely. These lesions have a characteristic radiographic appearance due to high fat content. Histologically, angiomyolipoma are characterized by an admixture of adipose tissue, blood vessels, and smooth muscle cells. These lesions cannot reliably be differentiated from a malignant lesion based on clinical history, radiologic examination, and/or pathologic interpretation. If clinical suspicion for malignancy is low, then careful observation with serial radiologic follow-up is performed. The treatment for a symptomatic or suspicious lesion is resection, if feasible. Liver transplantation may be considered for large or centrally located lesions not amenable to resection.

Full-text preview

Available from: onlinelibrary.wiley.com
  • Source
    [Show abstract] [Hide abstract]
    ABSTRACT: Angiomyolipoma (AML) is a benign mesenchymal tumor that has been frequently reported in the kidney but rarely in the liver. Hepatic AML may be clinically, radiologically and morphologically difficult to distinguish from hepatocellular carcinoma or other hepatic lesions, even though the number of cases has been increasing recently due to improved imaging techniques. Histologically it consists of smooth muscle cells, adipose cells and abnormal blood vessels. It is commonly diagnosed following abdominal pain but may also be asymptomatic, has a predominant female predilection, highly variable size and occurs in subjects with a wide age range. The right lobe is the most common site, and multicentricity has been reported. Here we report a case of the myomatous variant of AML, accidentally discovered in a young woman with no clear features on radiographic examination, which was diagnosed by means of fine needle aspiration biopsy (FNAB) and then surgically removed. Although careful observation with serial radiological follow-up is an option in these cases, we chose the surgical approach because of the risk of rupture due to the large size of the lesion and the risk of malignant behavior or transformation. In case of the myomatous variant composed of irregular cells with epithelioid appearance, hepatocellular carcinoma with fatty changes or the possibility of other malignant tumors must be ruled out by immunohistochemistry (HMB-45), even in biopsy specimens.
    Full-text · Article · Jan 2004 · Tumori
  • [Show abstract] [Hide abstract]
    ABSTRACT: Some of these relatively rare tumors occur in all age groups and both sexes, but several show a predilection for one sex or one age group. Many are small and are discovered incidentally at laparotomy or at autopsy. Almost all, however, may grow sufficiently large to produce symptoms and signs. In many instances these are related to pressure on adjacent viscera and organs, but in some cases (hemangioma, hepatocellular adenoma) the sudden onset of pain and other symptoms may herald complications such as thrombosis, infarction, or rutpure. Rapid increase in the size of some tumors, (bile duct cystadenoma, mesenchymal hamartoma) may be due to accumulation of fluid. Congestive heart failure is a frequent mode of presentation in infants with hemangioma or hemangioendothelioma. Large cavernous hemangiomas of the liver may occasionally lead to hemorrhagic manifestations from thrombocytopenia or hypofibrinogenemia. An uncommon manifestation of benign tumors of the liver is portal hypertension, which has only been reported in association with multiple nodular hyperplasia. Most of the benign tumors that are sufficiently large to be symptomatic remain undiagnosed prior to exploratory laparotomy. Tests of hepatic function are frequently normal or nondiagnostic. Upper and lower gastrointestinal radiographic series may show compression or displacement of various viscera and organs depending on the location and the size of the tumor. The hepatic scan will frequently reveal a filling defect or defects. Echograms are helpful in distinguishing solid from fluid filled tumors. By far the most useful technique for preoperative diagnosis, however, is hepatic angiography, since some benign tumors (e.g., hemangioma, hepatocellular adenoma and focal nodular hyperplasia) have characteristic if not pathognomonic vascular patterns. Even with sophisticated radiographic techniques, however, a definitive diagnosis may not be possible. Percutaneous hepatic biopsy may not be diagnostic because of the small size or location of the tumor, may be hazardous in the case of highly vascularized tumors (for example nodular hyperplasia or hepatocellular adenoma), and is contraindicated in the case of hemangioma or hemangioendothelioma. Laparotomy, open biopsy, and surgical resection should not be delayed, because of the danger of life threatening complications such as internal bleeding from rupture of the tumor. Little is known of the etiology and pathogenesis of benign tumors of the liver. Some, such as the infantile hemangioendothelioma, mesenchymal hamartoma and benign teratoma, are of undoubted congenital origin, but the intrauterine factors leading to their occurrence are unknown. Other tumors, such as the hemangioma and nodular hyperplasia, can occur in infants or children, so that they too may be congenital. The majority, however, first come to medical attention in adulthood and the inciting factors leading to their development and growth remain undetermined. Evidence suggests that hemangiomas are more frequent in multipara and may grow during pregnancy and under the influence of estrogen therapy. More recently a possible relationship between hepatocellular adenoma and oral contraceptives has been raised in a number of reports.
    No preview · Article · Aug 1975 · Medical Clinics of North America
  • [Show abstract] [Hide abstract]
    ABSTRACT: : Angiomyolipomas are benign mesenchymal tumours, mostly of renal origin. Hepatic angiomyolipomas are rare, and radiological and pathological diagnoses may be difficult. We report on the first case of hepatic angiomyolipoma recurrence known to us, 6 years after surgical treatment of the initial tumour. Morever, this hepatic recurrence was associated with renal angiomyolipoma without any stigmata of tuberous sclerosis. Eur J Gastroenterol Hepatol 12:579-582 (C) 2000 Lippincott Williams & Wilkins, Inc.
    No preview · Article · Jun 2000 · European Journal of Gastroenterology & Hepatology
Show more