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Primary Mediastinal Yolk Sac Tumor-A Report of Two Cases and Literature Review

前縱膈腔原發卵黃瘤之二病歷報告及相關文獻回顧

摘要


卵黃囊瘤是屬於Germ cell tumor的惡性非畸胎瘤(nonteratomatous)一種,絕大部份原發於生殖腺,極少數會有原發於生殖腺外,其中以縱膈腔內為常見,病患年齡分佈以年輕男性居多,病患常以胸痛,呼吸困難,咳嗽及體重減輕來表現,有些是毫與徵狀,是在常規身體檢查意外發現。其胸部X光發現常是典型之縱膈腔腫瘤來表現,在胸部電腦斷層上是呈現不均質腫瘤,在打顯影劑後可能會有不均質或邊緣顯影,至於病理診斷方面,若僅以病理切片之表現尤其是細針抽吸切片,可能會誤判為分化不良型腺癌,須配合血清胎兒蛋白上升及切片之免疫染色,有時須藉助外科手術以取得適當檢體也同時儘量切除腫瘤,以利於以platinum為基礎之化學治療的進行。 最近我們連續遇到兩名病人其在外院診斷為分化不良型腺癌而在本院最終診斷是前縱膈腔卵黃囊瘤,藉這兩個病例描述其臨床表現,影像學發現,診斷及治療經過並回顧歷年來與此種病歷相關的文獻報告。

並列摘要


Yolk sac tumors, also called endodermal tumors, are a kind of germ cell tumor, mostly found in the gonad. Extragonadol germ cell tumors are rare, and are usually found in the mediastinum. Mediastinal tumors are more prevalent in young males. Clinical presentations including chest pain, dyspnea, cough and body weight loss are noted, though asymptomatic cases with a huge anterior mediastinal mass, disclosed by chest radiography, are also seen. Due to the small sample size available for pathological study, yolk sac tumors are often misdiagnosed and consequently mismanaged. We came across 2 cases of primary anterior mediastinal tumors referred from a local hospital, where the initial diagnoses by fine needle aspiration biopsy were undifferentiated adenocarcinomal. However, at our hospital the markedly elevated serum alfa-fetoprotein (AFP) and normal β-human chorionic gonadotropin (β-hCG) of these two young males led to a suspicion of yolk sac tumors before surgical intervention. After surgical resection of the tumor, the specific pathologic characteristics and immunohistochemical staining confirmed the yolk sac tumor diagnosis. We present these 2 cases and review the associated literature.

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