裏細胞血管瘤－－一個引起脾臟腫大之罕見疾病

Case Report: Littoral Cell Angioma—A Rare Disease Causing Splenomegaly

成人脾臟腫大(splenomegaly)之鑑別診斷及相關處理，對基層臨床醫師而言，向來深具挑戰性。輕度脾臟腫大之患者一般缺乏明顯臨床症狀，往往是在接受例行性健康檢查或腹部超音波檢查時意外發現；然而，除了在少部分的孩童、青少年或體型瘦削之年輕人身上，此現象可被視為正常外，脾臟腫大皆需要更進一步評估與處理。造成脾臟腫大的病因相當繁多，其中以病毒感染、血液學疾病、循環性疾病及發炎性疾病最為常見。診斷有賴於詳細的病史詢問與理學檢查，搭配相關之實驗室檢查和影像學評估，治療則視其根本原因而決定。本文報告一位44歲的B型肝炎帶原女性，脾臟腫大持續長達兩年，因出現上腹痛及背部疼痛，至本院門診求診，理學檢查無異常發現；實驗室檢查亦無明顯異常數值。經安排腹部超音波後，發現除脾臟腫大外，還併有數顆低回音病灶；腹部電腦斷層檢查則顯示脾臟有多顆大小不一之腫瘤。轉介外科進行脾臟切除(splenectomy)後，病理報告結果為裏細胞血管瘤(littoral cellangioma)。本篇文章的目的，除討論此罕見的脾臟腫瘤外，亦要提醒基層醫師提高對脾臟腫大病因之警覺性，並了解相關鑑別診斷的臨床表現，進而對診斷、治療及轉診時機有更佳的掌握，如此才能給予病患更適切的照護品質。

The approach to a patient with splenomegaly has remained a challenge for primary care physicians because patients with mild splenomegaly do not usually present with significant symptoms and it is often discovered incidentally. Splenomegaly always requires further evaluation and management because it has been associated with a wide variety of causes, among which viral infections, hematologic, circulatory and inflammatory diseases are the most common. An accurate diagnosis is based on detailed history taking, physical examination and associated lab or image studies. Here we present the case of a 44-year-old female with a past medical history of chronic hepatitis B and a two year history of persistent splenomegaly. She came to our hospital with complaints of epigastralgia and low back pain. Physical examination did not reveal any positive finding and the lab results were unremarkable. Abdominal ultrasonography showed splenomegaly with several hypoechoic lesions; abdominal CT scan revealed multiple different-sized tumors. The patient was then referred to the Department of General Surgery for splenectomy. The pathology report showed a littoral cell angioma. In addition to introducing the littoral cell angioma, the purpose of this report is to remind primary care physicians to be more vigilant of the manifestations associated with splenomegaly and to have a better understanding of the appropriate diagnostic approach and subsequent management, so that delivery of a higher quality of care may be achieved.