片側肥大症を伴った小児腫瘍性疾患の2症例の経験

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It is well-known that congenital hemihypertrophy has a tendency to be associated with other abnormalities and/or tumors. Here we reported such cases. Case 1 : A 5-month-old boy with hemihypertrophy of left side extremities, first noted at the age of two months, was transferred to the authors' hospital. He had abnormal findings on the right lobe of liver which was the contralateral side of hemihypertrophy. Hepatic hemangioma was diagnosed, but a malignant tumor was undeniable because he had sufferred from congenital segmental hemihypertrophy. After the liver was partially resected, capillary hemangioma was histologically evidenced. Case 2 : A one-month-old girl was transferred to the authors' hospital because of right hemihypertrophy noted from birth. She had the cutaneous hemangiomas and varicosities. Her diagnosis was Klippel-Weber syndrome and she had been followed. At the age of 2 years and 6 months, a huge tumor in right lower abdominal cavity was palpated under thick subcutaneus tissue. Right ovarian cyst was diagnosed. Six months later, right oohorectomy was done. Serouscystadenoma of ovary with borderline malignancy was histologically diagnosed. One month after the oohorectomy, she suddenly died from asphyxiation at her home. Autopsy was not done. She had multiple abnormalities : hemihypertrophy, macrodactyly, subcutaneous mass, exostosis, epidermal nevus, varicosities, scoliosis, macrocephaly, mental retardation, asymmetric eye size, high myopia, epibulbal tumor, optic disc pallor, strabismus, cataract, nystagmus, chorioretinal degeneration. Her condition was thought to be Proteus syndrome, originally named by Wiedemann et al. in 1983.
日本小児外科学会の論文
1991-08-20

片側肥大症を伴った小児腫瘍性疾患の2症例の経験

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