表現異常的肝臟神經母細胞瘤

Abstract

我們報告了一例4個月大嬰兒肝臟中轉移性神經母細胞瘤的病例，神經母細胞瘤起源於原始神經脊，其產生交感神經細胞，包括交感神經節（在頸部、後縱隔、腹膜後和腹部）和腎上腺髓質細胞，因此，神經母細胞瘤可發生於腎上腺和交感神經，包括顱內、眼眶、胸、腹、盆等部位，一半以上見於腹部，臨床表現多取決於原發灶和轉移灶的位置，本病例在腹部超音波、電腦斷層與核磁共振檢查發現腫瘤類似肝母細胞瘤，後來病理切片證實為神經母細胞瘤。儘管肝母細胞瘤是兒童中最常見的原發性肝臟腫瘤，通常在出生後的前 3 年內會被診斷出來，對於嬰兒肝臟腫瘤，除肝母細胞瘤外，還必須考慮轉移性神經母細胞瘤，本文還討論了嬰兒神經母細胞瘤的臨床病理特徵和治療。肝臟中有多個不同大小的高迴聲實性腫塊作為神經母細胞瘤之臨床表現是非常罕見的，我們希望本文能提高對嬰兒肝臟神經母細胞瘤的認識以及臨床超音波醫師對於嬰兒腹部佔位性病變的瞭解。  Neuroblastoma is a rare cancer type that mostly affects infants and young chil-dren. It can spread to other organs, including bone marrow, bone, lymph nodes, liver, and skin. The purpose herein is to present the case of a four-month-old male infant with no prior systemic disease. The patient’s father discovered the abdominal disten-sion two months before presentation at our center. Plain abdominal radiograph showed hepatomegaly; abdominal ultrasound, computed tomography (CT) and magnetic resonance imaging (MRI)revealed multiple solid masses of varying sizes affecting two liver lobes, which we suspected as hepatoblastoma or metastatic neuroblastoma. The patient then underwent open biopsy and pathology indicated neuroblastoma. He then received chemotherapy. Three months later, follow-up liver CT showed significant tumor resolution and urine vanillylmandelic acid (VMA)had dropped to a normal value (1.14 mg/day). The patient is presently stable. Hepatoblastoma is the most common primary liver tumor in children and is typically diagnosed within the first three years of life; nevertheless, in addition to hepatoblastoma, metastatic neuro-blastoma must also be considered in infants.