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兒童期原發性肝母細胞瘤:一罕見病例報告

Primary Hepatoblastoma in Childhood: A Rare Case Report

摘要


兒童期原發性肝母細胞瘤是一較罕見的疾病且常發生在3歲以下兒童,而較好發在肝臟右葉且大約有33%侵犯肝臟左右葉。本病例報告是一位4歲男童,住院前一個月,因咳嗽,流鼻水而至診所求診,經醫師積查發現腹部有腫塊而至本院求診,因腹部腫塊和臉色蒼白而住院,家族史無特殊發現。住院時理學檢查發育正常,結膜和嘴唇蒼白,腹部觸診肝臟腫大在右肋下緣12公分大,心臟和肺部皆正常,神經檢查正常。血液檢查血色素低,白血球和血小板增加;生化檢查正常除鹼性燐酸鹽酵素昇高;血清甲型胎兒蛋白明顯增加;乙型肝炎血清標記正常;靜脈注射腎盂攝影檢查正常;腹部超音波和腹部電腦斷層攝影檢查顯示肝臟左右葉有多結節病灶併有中心壞死,而在腹部超音波指引下實行針吸式活體組織切片,組織病理診斷為肝母組胞瘤一胚胎型。兒童期原發性肝母細胞瘤侵犯肝臟左右葉合併中心壞死,而發生在3歲以上的病例較罕見,故特此提出報告以供大家參考。

並列摘要


Primary hepatoblastoma is a rare disease in childhood and usually presents before the age of 3 years. The tumors predominantly involve the right lobe of the liver and in 33% of the patients the tumor involved both lobes. This is a report of a 4 year-old boy who was well until 1 month prior to admission when symptoms of an upper respiratory tract infection developed with a lingering cough, followed by rhinorrhea. At that time be visited a LMD and an abdominal mass was diagnosed on pbysical examination. He was then transferred to our hospital and admitted because of the abdominal mass and anemia. No history of abdominal disease was reported in the family. Upon hospitalization, the physical examination revealed a normally developed child with palor of the conjunctiva and lips. The liver was palpable 12 cm below the right costal margin. Heart and lung were normal as was the neurological examination Routine blood studies revealed anemia thrombocytosis, and leukocytosis. The serum alpha fetoprotein was markedly increased but serum surface hepatitis B markers were normal. The IVP was also normal. The Echo and CT scan of the entire abdomen disclosed multiple nodules in the hepatic parenchyma with variable sized hypodense lesions. Under sonoguided biopsy. the diagnosis of hepatoblastoma, embryonal type, was established. Pri-mary hepatoblastoma of the liver involving both lobes with central necrosis is a rare disease over 3 years of age. We herein report and discuss one case.

並列關鍵字

Hepatoblastoma Alpha-fetoprotein

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