JL Yu, PL Khong, AKS Chiang, DKL Cheuk, SY Ha, GCF Chan

Abstract

Objective: Paediatric undifferentiated embryonal sarcoma (UES) and mesenchymal hamartoma (MH) of liver have similar imaging cystic imaging characteristic but have totally different clinical behaviour. We reviewed ours experience and compared that with the literature. Methods: Our patients' database over the past 25 years and all articles available from PubMed up to December 2016 were searched. Case reports recorded with demographic data, clinical information and investigations were recruited. The diagnosis of all cases had to be confirmed by histopathology. The data was analysed by SPSS 11.0 software. Results: We identified 2 cases each of UES and MH in our centre, and from the literature, there were a total of 219 cases of UES and 138 cases of MH. The age at diagnosis for UES ranged from 1-83 years but 39.73% were between 6-10 years, whereas 65.22% of MH were found at <2 years (range, newborn-73 years). The chief complaint for UES was abdominal distension with or without abdominal pain and 50.81% of them had accompanied systemic symptoms. In contrary, only 9.57% of MH had systemic symptoms. Interestingly, both tumours were more commonly found in the right hepatic lobe. The prognosis of these two entities was quite different, 41/103 (39.81%) of UES died, whereas only 7/110 (6.36%) cases with MH died. UES required additional chemotherapy whereas MH did not. With the limited information, some authors suggested that the ultrasonography findings were better concordant with the histology than computed tomography or magnetic resonance imaging. 54.9% UES patients' tumour was solid and only 30.36% were cystic. For MH, there was no significant difference in the nature of tumour. Conclusion: Similar imaging appearance and possible pathological overlap between UES and MH makes the initial diagnosis difficult to be established. Surgical biopsy and excision remained mandatory to confirm the diagnosis and to guide the subsequent treatment option.

目的：作者綜合自身的臨床診治經驗和相關文獻，並嘗試通過其臨床和影像表現，就兒童肝臟未分化胚胎性肉瘤（UES）和間葉性錯構瘤（MH）進行鑒別診斷。方法：搜集其主理的中心過去25年病人資料，和2016年12月後 PubMed的全部文獻。病例報告記錄包括一般記錄、臨床資料和檢查結果。所有病例的診斷必須由病理組織學確認。資料用SPSS 11.0軟體分析。結果：作者從中心內找到UES和MH各二例，從文獻彙集全部219例UES和 138例MH。UES的診斷年齡為1-83歲，其中39.72%為6-10歲；65.22%的MH在2歲前被發現（範圍從新生兒至73歲）、UES的主訴為腹脹，或伴有腹痛，其中50.81%伴有全身症狀。相反，僅有9.57%的MH伴有全身症狀。值得注意的是，兩種腫瘤都多見於肝臟右葉。兩種疾病的預後相當不同，41/103（39.81%）的UES 死亡，而僅有 7/110 （6.36%）的MH死亡。UES需要化療，而MH不用化療。用有限的資料，部份作者認為超聲檢查結果與組織學結果的符合度好於CT或MRI。UES病人的腫瘤中，54.9%為實體性，僅有30.36%為囊性。而MH病人中沒有恒定的這種差異。結論：雖然UES和MH具有相似的影像形態和病理學可能重疊，從二者臨床表現特徵的差別可以有助臨床鑒別診斷。然而，手術活檢和切除仍是必需的，用以確認診斷和指導後續治療。