Hepatic adenoma in Fanconi anemia treated with oxymetholone

Mulvihill, John J.; Ridolfi, Ren L.; Schultz, Frederick R.; Borzy, Michael S.; Haughton, Peter B.T.

doi:10.1016/s0022-3476(75)80087-4

Cited by 70 publications

(19 citation statements)

References 13 publications

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“…The principal clinical features described in patients on androgen therapy in whom hepatocellular tumors devel¬ oped hepatomegaly, abdominal dis¬ comfort, and abnormal liver function test results. Two of the neoplasms ruptured into the peritoneal cavity.1 ·" However, in two previous cases,5 9 as well as in ours, abnormal liver func¬ tion test results were not accompa¬ nied by clinical signs or symptoms of liver involvement. One patient's tu¬ mor8 apparently was not only clini¬ cally, but also biochemically, silent.…”

Section: Report Of a Casesupporting

confidence: 48%

Multiple Hepatic Tumors and Peliosis Hepatis in Fanconi's Anemia Treated With Androgens

Shapiro¹,

Ikeda²,

Ruebner³

et al. 1977

Arch Pediatr Adolesc Med

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\s=b\We report the case of a 13-year-old boy who was known to have Fanconi's anemia for five years. For treatment of this condition he was given androgens and corticosteroids. Two months before his death, severe varicella developed complicated by pneumonia, jaundice, and prolonged fever; all of which resolved during a five-week hospitalization. Three weeks later he died of Clostridium septicum sepsis caused by necrotizing enterocolitis. At autopsy he was found to have multiple hepatocellular neoplasms. A striking feature of the neoplasms was cholestasis. The liver also showed peliosis hepatis. The association of the use of certain androgenic steroids with hepatic neoplasms histologically resembling hepatocarcinomas, but characterized by lack of metastases and apparent reversibility, suggests the desirability of a new nomenclature for these hepatocellular lesions.

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Section: Report Of a Casesupporting

confidence: 48%

Multiple Hepatic Tumors and Peliosis Hepatis in Fanconi's Anemia Treated With Androgens

Shapiro¹,

Ikeda²,

Ruebner³

et al. 1977

Arch Pediatr Adolesc Med

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“…In further support of this observation is the occurrence of tumors during pregnancy or in the immediate postpartum period (12,13). Additional case reports have associated liver neoplasms with androgenic-anabolic steroids (14,15) and adrenal carcinoma (7). The behavior of tumors incompletely resected also supports their dependency on steroids.…”

Section: Resultsmentioning

confidence: 81%

Human liver tumors in relation to steroidal usage.

Barrows¹,

Christopherson²

1983

Environ Health Perspect

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Since 1973 a number of investigators have reported an association between liver neoplasia and steroid usage. Through referral material we have examined the histology of over 250 cases of hepatic neoplasia, most in patients receiving steroid medications. The majority have been benign, predominantly focal nodular hyperplasia (55%) and hepatocellular adenoma (39%). The average age was 31.4 years; 83% had significant steroid exposure with an average duration of 71 months for focal nodular hyperplasia and 79.6 months for hepatocellular adenoma. The type of estrogenic agent was predominantly mestranol; however, during the period mestranol was the most frequently used synthetic steroid. A distinct clinical entity of life threatening hemorrhage from the lesion occurred in 31% of patients with hepatocellular adenoma and 9% of patients with focal nodular hyperplasia. Recurrence of benign tumors has occurred in some patients who continued using steroids and regression has been observed in patients who had incomplete tumor removal but discontinued steroid medication. Medial and intimal vascular changes have been present in a large number of the benign tumors. The relationship of these vascular changes to oncogenesis is unclear, but similar lesions have been described in the peripheral vasculature associated with steroid administration. A number of hepatocellular carcinomas have also been seen. Of significance is the young age of these patients and lack of abnormal histology in adjacent nonneoplastic liver. A striking number of the malignant hepatocellular tumors have been of the uncommon type described as "eosinophillic hepatocellular carcinoma with lamellar fibrosis." The epidemiology of liver lesions within this series is difficult to assess, since the material has been referred from very diverse locations.

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“…The occurrence of liver tumors such as adenoma and hepatocellular carcinoma in FA patients is well documented [Bernstein et al, 1971;Cattan et al, 1974;Corberand et al, 1975;Mulvihill et al, 1975;Sarna et al, 1975;Shapiro et al, 1977;Lesna et al, 1976;Henderson et al, 1974;Schmidt et al, 19841. However, it is difficult to determine its true incidence, for they are often found coincidently at autopsy.…”

Section: Treatment and Prognosismentioning

confidence: 99%

Fanconi anemia, dyskeratosis congenita, and WT syndrome

Shahidi

Opitz²,

Bernstein³

1987

Am. J. Med. Genet.

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The association of congenital anomalies and pancytopenia is encountered in several clinical syndromes. Among these, Fanconi anemia is by far the most prevalent, and consequently best known. As a result, other similar conditions, such as dyskeratosis congenita and particularly WT syndrome, are often mistaken for Fanconi anemia. However, at a closer look, the type of congenital anomalies, the mode of inheritance, cytogenic and other laboratory findings allow clear differentiation between these 3 syndromes.

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Hepatic adenoma in Fanconi anemia treated with oxymetholone

Cited by 70 publications

References 13 publications

Multiple Hepatic Tumors and Peliosis Hepatis in Fanconi's Anemia Treated With Androgens

Multiple Hepatic Tumors and Peliosis Hepatis in Fanconi's Anemia Treated With Androgens

Human liver tumors in relation to steroidal usage.

Fanconi anemia, dyskeratosis congenita, and WT syndrome

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