PMC7236383_01
MRI of Case 1 (suprasellar germinoma). (A and B) representative sagittal and coronal views, respectively, at the time of presentation. (C) sagittal view after 2 doses of weekly vinblastine showing dramatic tumor response and (D) sagittal view after completion of vinblastine monotherapy and prior to definitive radiotherapy showing further tumor response.
vdaa048f0001.jpg
PMC7236383_01_vdaa048f0001.jpg
F1
MRI head scan revealed a 36 mm diameter lobulated enhancing suprasellar solid mass involving the optic chiasm (Figure 1A and B). Repeat MRI at this time, with spinal imaging, performed 22 days after the first scan, revealed a substantial decrease in size and enhancement of the suprasellar mass, which was now 21 x 19 mm in axial diameter (Figure 1C) compared with 36 x 28 mm originally. Weekly vinblastine monotherapy was therefore continued and further re-assessment scan showed continued response with a further decrease in size and enhancement of the suprasellar lesion (Figure 1D), prior to definitive radiotherapy.
Relative miR-371a-3p levels in cerebrospinal fluid (CSF) and serum from the cases. (A) CSF and (B) serum miR-371a-3p levels in Cases 1-4, compared with 4 previously published cases. The published cases comprised 2 positive controls (intracranial suprasellar malignant GCT cases, MGCT_IC#3 and MGCT_IC#2) and 2 negative controls (a suprasellar low-grade glioma case, B-non-GCT_IC#1, and a patient with intracranially disseminated high-risk neuroblastoma). N/A, not available. Error bars represent standard error of the mean.
vdaa048f0002.jpg
PMC7236383_04_vdaa048f0002.jpg
F2
Serum and CSF miR-371a-3p quantification at the time of relapse revealed levels of 44 870 and 4.88, respectively (Figure 2), consistent with levels observed in patients with proven intracranial malignant GCTs.
PMC7236383_02
Relative miR-371a-3p levels in cerebrospinal fluid (CSF) and serum from the cases. (A) CSF and (B) serum miR-371a-3p levels in Cases 1-4, compared with 4 previously published cases. The published cases comprised 2 positive controls (intracranial suprasellar malignant GCT cases, MGCT_IC#3 and MGCT_IC#2) and 2 negative controls (a suprasellar low-grade glioma case, B-non-GCT_IC#1, and a patient with intracranially disseminated high-risk neuroblastoma). N/A, not available. Error bars represent standard error of the mean.
vdaa048f0002.jpg
PMC7236383_04_vdaa048f0002.jpg
F2
Serum and CSF miR-371a-3p quantification at the time of relapse revealed levels of 44 870 and 4.88, respectively (Figure 2), consistent with levels observed in patients with proven intracranial malignant GCTs.
MRI of Cases 2 and 3 presenting with pituitary thickening and diabetes insipidus. Representative (A) coronal views of Case 2, (B) sagittal views of Case 2, and (C) coronal views of Case 3:at first presentation (left panels), 1 year later (central panels), and 2 years later (right panels). White arrows indicate the radiological abnormality.
vdaa048f0003.jpg
PMC7236383_03_vdaa048f0003.jpg
F3
New symptoms (hearing loss and ear discharge) resulted in a further MRI being performed which showed that the pituitary stalk had increased to 5 mm diameter over the 3-year period since the previous scan (Figure 3C) but nil else of note. Serial MRI over the following years revealed no change in the enhancing pituitary stalk thickening, which remained stable at 5 mm diameter (Figure 3C).
PMC7236383_03
Relative miR-371a-3p levels in cerebrospinal fluid (CSF) and serum from the cases. (A) CSF and (B) serum miR-371a-3p levels in Cases 1-4, compared with 4 previously published cases. The published cases comprised 2 positive controls (intracranial suprasellar malignant GCT cases, MGCT_IC#3 and MGCT_IC#2) and 2 negative controls (a suprasellar low-grade glioma case, B-non-GCT_IC#1, and a patient with intracranially disseminated high-risk neuroblastoma). N/A, not available. Error bars represent standard error of the mean.
vdaa048f0002.jpg
PMC7236383_04_vdaa048f0002.jpg
F2
Serum and CSF miR-371a-3p quantification at the time of relapse revealed levels of 44 870 and 4.88, respectively (Figure 2), consistent with levels observed in patients with proven intracranial malignant GCTs.
MRI of Cases 2 and 3 presenting with pituitary thickening and diabetes insipidus. Representative (A) coronal views of Case 2, (B) sagittal views of Case 2, and (C) coronal views of Case 3:at first presentation (left panels), 1 year later (central panels), and 2 years later (right panels). White arrows indicate the radiological abnormality.
vdaa048f0003.jpg
PMC7236383_03_vdaa048f0003.jpg
F3
New symptoms (hearing loss and ear discharge) resulted in a further MRI being performed which showed that the pituitary stalk had increased to 5 mm diameter over the 3-year period since the previous scan (Figure 3C) but nil else of note. Serial MRI over the following years revealed no change in the enhancing pituitary stalk thickening, which remained stable at 5 mm diameter (Figure 3C).
PMC7236383_04
Relative miR-371a-3p levels in cerebrospinal fluid (CSF) and serum from the cases. (A) CSF and (B) serum miR-371a-3p levels in Cases 1-4, compared with 4 previously published cases. The published cases comprised 2 positive controls (intracranial suprasellar malignant GCT cases, MGCT_IC#3 and MGCT_IC#2) and 2 negative controls (a suprasellar low-grade glioma case, B-non-GCT_IC#1, and a patient with intracranially disseminated high-risk neuroblastoma). N/A, not available. Error bars represent standard error of the mean.
vdaa048f0002.jpg
PMC7236383_04_vdaa048f0002.jpg
F2
Serum and CSF miR-371a-3p quantification at the time of relapse revealed levels of 44 870 and 4.88, respectively (Figure 2), consistent with levels observed in patients with proven intracranial malignant GCTs.
MRI of Case 4 with germinoma relapse of an intracranial malignant non-germinomatous germ cell tumor (NGGCT). Representative sagittal views at (A) original diagnosis of the NGGCT, (B) following completion of treatment with chemotherapy and radiotherapy, and (C) at the time of relapse with germinoma, with nodular enhancing cyst-like lesions posterior and inferior to the corpus callosum and involving the septum pellucidum. White arrows indicate the radiological abnormality.
vdaa048f0004.jpg
PMC7236383_04_vdaa048f0004.jpg
F4
MRI scan showed an enhancing suprasellar mass measuring 33 mm x 29 mm (Figure 4A). MRI imaging at that stage showed a good response with only a small non-enhancing 9 mm residual that was not felt to be resectable (Figure 4B). At 11 years of age, and 3 years following the end of treatment, routine surveillance MRI demonstrated an asymptomatic relapse outside the radiotherapy field, with nodular enhancing cyst-like lesions posterior and inferior to the corpus callosum and involving the septum pellucidum (Figure 4C), measuring 43 mm x 19 mm x 11 mm.