Perl Regine Mariette, Häring Almut, Horger Marius Stefan, Pfannenberg Christina, Gatidis Sergios
Department of Diagnostic and Interventional Radiology, University-Hospital of Tuebingen, Hoppe-Seyler-Straße 3, 72076, Tübingen, Germany.
Internal Medicine II, Department of Haematology, Oncology, Clinical Immunology and Rheumatology, University-Hospital of Tuebingen, Otfried-Müller-Straße 10, 72076, Tübingen, Germany.
EJNMMI Res. 2020 Feb 18;10(1):11. doi: 10.1186/s13550-020-0602-x.
The undifferentiated embryonic sarcoma of the liver (UESL) is a rare, aggressive tumor mainly affecting children. Since UESL has no specific clinical symptoms or imaging characteristics, many cases of UESL are diagnosed late. The paraneoplastic leukemoid reaction (PLR) is a very rare concomitant of oncological patients associated with poor prognosis. This report describes the clinical course of a patient combining these two rare entities and describes the diagnostic challenges and dynamics of paraneoplastic syndrome.
We report a case of UESL in a 46-year-old male who became clinically conspicuous due to pronounced B symptoms. CT and MRI showed a suspicious unifocal liver lesion. As the histological analysis of a tissue sample did not reveal a clear result, an 18F-FDG-PET-CT examination was performed. In addition to a high glucose metabolism of the liver lesion, an increased glucose metabolism in the entire bone marrow was observed. This finding was considered as pronounced paraneoplasia and laparotomy with liver segment resection followed. Immediately after resection of the tumor the paraneoplastic symptoms completely declined and the patient showed no signs of recurrence in the 1-year follow-up.
Although UESL is rare and predominantly affects children, this diagnosis should always be considered for unclear unifocal cystic liver lesions, regardless of the patient's age, as appropriate treatment has a good prognosis.
肝未分化胚胎性肉瘤（UESL）是一种罕见的侵袭性肿瘤，主要影响儿童。由于UESL没有特定的临床症状或影像学特征，许多UESL病例诊断较晚。副肿瘤性类白血病反应（PLR）是肿瘤患者非常罕见的伴随情况，与预后不良相关。本报告描述了一名合并这两种罕见病症的患者的临床病程，并描述了副肿瘤综合征的诊断挑战和动态变化。
我们报告一例46岁男性的UESL病例，该患者因明显的B症状而在临床上引人注目。CT和MRI显示肝脏有一个可疑的单发病灶。由于组织样本的组织学分析未得出明确结果，遂进行了18F-FDG-PET-CT检查。除了肝脏病灶的高糖代谢外，还观察到整个骨髓的糖代谢增加。这一发现被认为是明显的副肿瘤现象，随后进行了剖腹手术并切除肝段。肿瘤切除后，副肿瘤症状立即完全消退，患者在1年的随访中未出现复发迹象。
尽管UESL罕见且主要影响儿童，但对于不明原因的肝脏单发病灶性囊肿，无论患者年龄如何，均应始终考虑这一诊断，因为适当的治疗预后良好。
