Tomonari Shimagaki1, Keishi Sugimachi2, Yohei Mano2, Emi Onishi2, Yuki Tanaka3, Rie Sugimoto3, Kenichi Taguchi4, Masaru Morita5, Yasushi Toh5. 1. Department of Hepatobiliary and Pancreatic Surgery, National Hospital Organization Kyushu Cancer Center, 3-1-1 Notame, Minami-ku, Fukuoka, 811-1395, Japan. rgwsn6911@gmail.com. 2. Department of Hepatobiliary and Pancreatic Surgery, National Hospital Organization Kyushu Cancer Center, 3-1-1 Notame, Minami-ku, Fukuoka, 811-1395, Japan. 3. Department of Hepato-Biliary-Pancreatology, National Hospital Organization Kyushu Cancer Center, Fukuoka, 811-1395, Japan. 4. Department of Pathology, National Hospital Organization Kyushu Cancer Center, Fukuoka, 811-1395, Japan. 5. National Hospital Organization Kyushu Cancer Center, Fukuoka, 811-1395, Japan.
Abstract
BACKGROUND: Undifferentiated embryonal sarcoma of the liver (UESL) is a rare malignant mesenchymal tumor that usually occurs in children and is rarely diagnosed in adults. CASE PRESENTATION: The case was a female in her late 20s who presented with a huge liver mass found upon the examination of fever. Imaging analysis showed a well-defined mass measuring 9 cm in the largest dimension in the right posterior segment of the liver. The patient underwent right hemi-hepatectomy. Histopathological studies revealed that the circumscribed tumor was composed of a proliferation of atypical epithelioid to spindle-shaped cells with pleomorphic nuclei arranged in haphazard pattern. Histopathological features observed in immunohistochemical analyses confirmed a final diagnosis of UESL. Genome analysis using FoundationOne CDx revealed 11 somatic mutations including TP53 (R196*) and STK11 (F354L). Adjuvant chemotherapy with ifosfamide and etoposide was performed, and the case has been followed up without recurrence for 1 year after hepatectomy. CONCLUSIONS: A UESL should be considered in the differential diagnosis of large and well-defined solid liver lesions. Although the prognosis of UESL is extremely unfavorable, aggressive surgical resection with adjuvant chemotherapy and genomic analysis may be helpful for ensuring long-term survival.
BACKGROUND: Undifferentiated embryonal sarcoma of the liver (UESL) is a rare malignant mesenchymal tumor that usually occurs in children and is rarely diagnosed in adults. CASE PRESENTATION: The case was a female in her late 20s who presented with a huge liver mass found upon the examination of fever. Imaging analysis showed a well-defined mass measuring 9 cm in the largest dimension in the right posterior segment of the liver. The patient underwent right hemi-hepatectomy. Histopathological studies revealed that the circumscribed tumor was composed of a proliferation of atypical epithelioid to spindle-shaped cells with pleomorphic nuclei arranged in haphazard pattern. Histopathological features observed in immunohistochemical analyses confirmed a final diagnosis of UESL. Genome analysis using FoundationOne CDx revealed 11 somatic mutations including TP53 (R196*) and STK11 (F354L). Adjuvant chemotherapy with ifosfamide and etoposide was performed, and the case has been followed up without recurrence for 1 year after hepatectomy. CONCLUSIONS: A UESL should be considered in the differential diagnosis of large and well-defined solid liver lesions. Although the prognosis of UESL is extremely unfavorable, aggressive surgical resection with adjuvant chemotherapy and genomic analysis may be helpful for ensuring long-term survival.
Authors: Rosa María Pérez-Gómez; Danny Soria-Céspedes; Beatriz de León-Bojorge; Carlos Ortiz-Hidalgo Journal: Appl Immunohistochem Mol Morphol Date: 2010-03
Authors: Biagio Ricciuti; Kathryn C Arbour; Jessica J Lin; Amir Vajdi; Natalie Vokes; Lingzhi Hong; Jianjun Zhang; Michael Y Tolstorukov; Yvonne Y Li; Liam F Spurr; Andrew D Cherniack; Gonzalo Recondo; Giuseppe Lamberti; Xinan Wang; Deepti Venkatraman; Joao V Alessi; Victor R Vaz; Hira Rizvi; Jacklynn Egger; Andrew J Plodkowski; Sara Khosrowjerdi; Subba Digumarthy; Hyesun Park; Nuno Vaz; Mizuki Nishino; Lynette M Sholl; David Barbie; Mehmet Altan; John V Heymach; Ferdinandos Skoulidis; Justin F Gainor; Matthew D Hellmann; Mark M Awad Journal: J Thorac Oncol Date: 2021-11-02 Impact factor: 15.609