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題 名 | Papillary Cystic Neoplasm of the Pancreas in Children: Report of Three Cases=兒童胰臟乳頭囊狀瘤:三病例報告 |
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作 者 | 趙舜卿; 江文山; 林思偕; 駱至誠; 林博彥; |
書刊名 | 臺灣兒科醫學會雜誌 |
卷 期 | 41:2 民89.03-04 |
頁 次 | 頁101-105+114 |
分類號 | 417.62472 |
關鍵詞 | 乳頭囊狀瘤; 胰臟; Papillary cystic neoplasm; Pancreas; |
語 文 | 英文(English) |
中文摘要 | 本文目的在探討兒童胰臓乳頭囊狀瘤的臨床特征、診斷、方法與其預後。我們研究本院過去10年間小於15歲兒童胰臓乳頭囊狀瘤的臨床表征、診斷與病理組織的現象,並探討其手術治療與預後。研究結果計有3例個案全爲女性,年齡分別爲11,13、與13歲。在組織學上皆具有典型的胰臓乳頭囊狀瘤的特征。臨床的主要症狀爲腹痛與腹部腫瘤,無貧血或全身不適之症狀。在過去四十年的文獻回顧中,計有35例兒童胰臓乳頭囊狀瘤,他們主要表征也是腹痛與腹部腫瘤,較少見的臨床表征有貧血、全身不適與低血壓。本院的3個案與中之案例均無特定的腫瘤指標異常。這3位病童均接受腹部超音波與電腦斷層檢查,發現腫瘤含有硬組織與囊狀,爲一質地不均勻的腫瘤。所有腫瘤均有纖維外膜包被。一位病童接受血管攝影檢查,發現此特別獎具有豐富血液支配,而支配腫瘤的主要血管來自脾 動脈的胰臓分枝。所有病童均接受手術切除治療,術中無發現腫瘤轉移的個案。腫瘤的大小平均爲11.3公分,它們分別位於頭部、體部與尾部。這些結果與過去文獻中的案例相似,惟過去案例尾部侵犯的個案佔多婁。本院各案在臨床追蹤中㬟無腫瘤複發與死亡。過去文獻的個案只有兩例發現併有腫瘤轉移,但無死亡的案例。本研究的結論顯示胰臓乳頭囊狀瘤爲一低程度惡性的腫瘤,病童經過手術根除治療者均有長期有存活率。 |
英文摘要 | Papillary cystic neoplasm of the pancreas is very rare in children. There were only 35 pediatric cases reported in the literature. We herein report three children who had papillary cystic neoplasm of pancreas. They were female teenagers, and were pathologically diagnosed. The major presenting symptoms were abdominal pain and abdominal mass. Serum tumor markers of these patients showed normal results. A CT scan of these patients showed that this tumor was of pancreatic origin. These 3 tumors were localized to head, body, and tail, respectively. The mean maximal diameter of these tumors was 11.3±3 cm. Sonography and CT examination showed that the tumor was a heterogeneous mass with solid and cystic components. Anglo graphy of this tumor showed a hypervascular mass with blood supply mainly from pancreatic branch of splenic artery. They all underwent tumor resection. All tumors contained some degree of internal hemorrhage or cystic degeneration and all were well encapsulated. Histologically, tumor cells generally showed solid and pseudopapillary growth around the fibro vascular stalks. No metastasis, mortality or recurrence was noted during follow-ups. In conclusion, CT scan helps to make a prospective diagnosis of papillary cystic neoplasm of pancreas. Our study confirmed that a papillary neoplasm of the pancreas is a low-grade malignant tumor. Surgical resection of the tumor is the mainstay of effective management. |