Glypican‑3 expression in malignant small round cell tumors

Abstract

Malignant small round cell tumors usually progress rapidly and show resistance to chemotherapy, and it is often difficult to make a definitive diagnosis based on their histological morphology. Glypican-3 (GPC3) is a highly tumor-specific antigen, and the overexpression of GPC3 was reported in many pediatric and adult malignancies. In the present study, we investigated the GPC3 expression in pediatric malignant small round cell tumors to assess its role in the differential diagnosis of the tumors. Immunohistochemistry was performed to assess the expression of GPC3 in samples from 84 rhabdomyosarcomas (RMSs; 44 alveolar and 40 embryonal RMSs), 62 Ewing sarcomas (EWSs), 35 neuroblastomas (NBs) and two desmoplastic small round cell tumors (DSRCTs). We performed a reverse transcription-quantitative polymerase chain reaction for GPC3 to determine the GPC3 mRNA expression in samples from 66 frozen tumors (23 RMSs, 28 EWSs and 15 NBs). The serum expression levels of GPC3 were analyzed in pre-operative blood samples from two RMS and eight NB patients. In total, 25% (21/84) of the RMSs and 3% (1/35) of the NBs exhibited a focal expression of GPC3, whereas, the other specimens showed no GPC3 expression. The GPC3 mRNA expression level of the RMSs with positive GPC3 expression (n=6) was significantly higher compared with the RMSs without such expression (n=17). A total of two cases of NB showed high serum levels of GPC3, but neither tumor showed immunoreactivity for GPC3. The immunohistochemical overexpression of GPC3 may be a candidate ancillary parameter in the differential diagnosis of RMS from EWS and DSRCT.

A rare case report of renal ewing sarcoma/primitive neuroectodermal tumor with ACTH production

“…It occurs mostly in the long bones and pelvis, and rarely in the kidney [ 1 , 2 ]. Small round blue cell tumors are histologically characterized by proliferation of small round tumor cells with sparse cytoplasm, which are often difficult to distinguish by standard histology or immunohistochemistry [ 3 , 4 ]. The disease usually has an insidious onset, rapid progress, easy recurrence and metastasis, and poor prognosis.…”

Single-cell imaging of T cell immunotherapy responses in vivo

“…In total, six surface antigens were identified as potential epitopes for RMS immunotherapy, including EGFR. A retrospective study using immunostaining of RMS patient samples ( Dobrenkov et al, 2016 ; Ganti et al, 2006 ; Grass et al, 2009 ; Majzner et al, 2019 ; Saraf et al, 2019 ; Shibui et al, 2019 ; Thway et al, 2011 ) revealed EGFR expression in a substantial fraction of human RMS ( n = 316 out of 476 primary patient samples and n = 9 of 12 cell line/PDXs; Fig. 6, B–D ; and Table 1 ).…”

The prognostic value of arginase-1 and glypican-3 expression levels in patients after surgical intrahepatic cholangiocarcinoma resection

“…Wang et al [ 33 ] reported that GPC-3 directly upregulated β-catenin to promote the proliferation and growth of lung squamous cell carcinoma. GPC-3 has also been reported to enhance the proliferation of nephroblastoma, hepatoblastoma, and melanoma cells [ 34 , 35 ]. In our study, we found the similar results that high GPC-3 expression levels were associated with tumor size and poor OS and DFS.…”