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Long survival in a child with a mutated K27M-H3.3 pilocytic astrocytoma.

Hochart A, Escande F, Rocourt N, Grill J, Koubi-Pick V, Beaujot J, Meignan S, Vinchon M, Maurage CA, Leblond P - Ann Clin Transl Neurol (2015)

Bottom Line: We report the first case of a child with a H3F3A K27M mutated pilocytic astrocytoma, who presented with a 10 years survival, and underwent spontaneous malignant transformation.H3F3A K27M mutations are rarely observed in benign neoplasms and may be associated with an adverse outcome.This mutation might not be the major driver that led to the onset of tumorigenesis, and we could consider that the associated TP53 mutation, would be required for malignant transformation.

View Article: PubMed Central - PubMed

Affiliation: Pediatric Oncology Unit, Oscar Lambret Center Lille, France.

ABSTRACT
We report the first case of a child with a H3F3A K27M mutated pilocytic astrocytoma, who presented with a 10 years survival, and underwent spontaneous malignant transformation. The complex tumoral chromosomal rearrangements were consistent for genomic instability and for the histopathological features of malignant transformation into glioblastoma. H3F3A K27M mutations are rarely observed in benign neoplasms and may be associated with an adverse outcome. This mutation might not be the major driver that led to the onset of tumorigenesis, and we could consider that the associated TP53 mutation, would be required for malignant transformation.

No MeSH data available.


Related in: MedlinePlus

Light microscopy data from the first tumor sample resected in 2002 (left column) and the recurrence sample (right column), showing routine hematoxylin–erythrosin–saffron staining (first line) and immunohistochemistry of Ki67, EGFR, Olig2 and p53 (lines 2–5). (A and F) the first tumor exhibited hallmarks of an pilocytic astrocytoma, that is, piloid cells, Rosenthal fibers, eosinophilic granular bodies, and glomeruloid vascular proliferation, whereas in the second tumor, the cells were poorly differentiated. There was severe nuclear atypia and numerous mitoses. (B and G) Olig2 immunoreactivity is present in both tumors but is stronger in the recurrence. (C and H) The Ki67 index was 5% in the first tumor, but reached an overall 30% on the recurrence with a foci of 70%. (D and I) p53 labeling was faint in the first tumor, but strong in the second tumor (70% of the stained nuclei). (E and J) EGFR labeling appeared in the second tumor, as a membrane signal on 20% of tumor cells, but is weak to intermediate in intensity.
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fig02: Light microscopy data from the first tumor sample resected in 2002 (left column) and the recurrence sample (right column), showing routine hematoxylin–erythrosin–saffron staining (first line) and immunohistochemistry of Ki67, EGFR, Olig2 and p53 (lines 2–5). (A and F) the first tumor exhibited hallmarks of an pilocytic astrocytoma, that is, piloid cells, Rosenthal fibers, eosinophilic granular bodies, and glomeruloid vascular proliferation, whereas in the second tumor, the cells were poorly differentiated. There was severe nuclear atypia and numerous mitoses. (B and G) Olig2 immunoreactivity is present in both tumors but is stronger in the recurrence. (C and H) The Ki67 index was 5% in the first tumor, but reached an overall 30% on the recurrence with a foci of 70%. (D and I) p53 labeling was faint in the first tumor, but strong in the second tumor (70% of the stained nuclei). (E and J) EGFR labeling appeared in the second tumor, as a membrane signal on 20% of tumor cells, but is weak to intermediate in intensity.

Mentions: Microscopic features of a glioblastoma were present at examination of the tumor that was resected at recurrence in 2012 (Fig.2). Tumor cells had a stellate or elongated morphology, with a brisk mitotic activity, necrosis and vascular festoons. They were labeled by anti-Olig2 (70%) but not with anti-IDH1 (R132H) antibodies, and the Ki67 index reached 30% of the tumor cell nuclei. In addition, p53 immunoreactivity was strong, and 20% of the cell tumors were EGFR-immunoreactive. CD34 labeling remained negative. Immunofluorescence staining revealed a reduction in H3K27 methylation.


Long survival in a child with a mutated K27M-H3.3 pilocytic astrocytoma.

Hochart A, Escande F, Rocourt N, Grill J, Koubi-Pick V, Beaujot J, Meignan S, Vinchon M, Maurage CA, Leblond P - Ann Clin Transl Neurol (2015)

Light microscopy data from the first tumor sample resected in 2002 (left column) and the recurrence sample (right column), showing routine hematoxylin–erythrosin–saffron staining (first line) and immunohistochemistry of Ki67, EGFR, Olig2 and p53 (lines 2–5). (A and F) the first tumor exhibited hallmarks of an pilocytic astrocytoma, that is, piloid cells, Rosenthal fibers, eosinophilic granular bodies, and glomeruloid vascular proliferation, whereas in the second tumor, the cells were poorly differentiated. There was severe nuclear atypia and numerous mitoses. (B and G) Olig2 immunoreactivity is present in both tumors but is stronger in the recurrence. (C and H) The Ki67 index was 5% in the first tumor, but reached an overall 30% on the recurrence with a foci of 70%. (D and I) p53 labeling was faint in the first tumor, but strong in the second tumor (70% of the stained nuclei). (E and J) EGFR labeling appeared in the second tumor, as a membrane signal on 20% of tumor cells, but is weak to intermediate in intensity.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4402089&req=5

fig02: Light microscopy data from the first tumor sample resected in 2002 (left column) and the recurrence sample (right column), showing routine hematoxylin–erythrosin–saffron staining (first line) and immunohistochemistry of Ki67, EGFR, Olig2 and p53 (lines 2–5). (A and F) the first tumor exhibited hallmarks of an pilocytic astrocytoma, that is, piloid cells, Rosenthal fibers, eosinophilic granular bodies, and glomeruloid vascular proliferation, whereas in the second tumor, the cells were poorly differentiated. There was severe nuclear atypia and numerous mitoses. (B and G) Olig2 immunoreactivity is present in both tumors but is stronger in the recurrence. (C and H) The Ki67 index was 5% in the first tumor, but reached an overall 30% on the recurrence with a foci of 70%. (D and I) p53 labeling was faint in the first tumor, but strong in the second tumor (70% of the stained nuclei). (E and J) EGFR labeling appeared in the second tumor, as a membrane signal on 20% of tumor cells, but is weak to intermediate in intensity.
Mentions: Microscopic features of a glioblastoma were present at examination of the tumor that was resected at recurrence in 2012 (Fig.2). Tumor cells had a stellate or elongated morphology, with a brisk mitotic activity, necrosis and vascular festoons. They were labeled by anti-Olig2 (70%) but not with anti-IDH1 (R132H) antibodies, and the Ki67 index reached 30% of the tumor cell nuclei. In addition, p53 immunoreactivity was strong, and 20% of the cell tumors were EGFR-immunoreactive. CD34 labeling remained negative. Immunofluorescence staining revealed a reduction in H3K27 methylation.

Bottom Line: We report the first case of a child with a H3F3A K27M mutated pilocytic astrocytoma, who presented with a 10 years survival, and underwent spontaneous malignant transformation.H3F3A K27M mutations are rarely observed in benign neoplasms and may be associated with an adverse outcome.This mutation might not be the major driver that led to the onset of tumorigenesis, and we could consider that the associated TP53 mutation, would be required for malignant transformation.

View Article: PubMed Central - PubMed

Affiliation: Pediatric Oncology Unit, Oscar Lambret Center Lille, France.

ABSTRACT
We report the first case of a child with a H3F3A K27M mutated pilocytic astrocytoma, who presented with a 10 years survival, and underwent spontaneous malignant transformation. The complex tumoral chromosomal rearrangements were consistent for genomic instability and for the histopathological features of malignant transformation into glioblastoma. H3F3A K27M mutations are rarely observed in benign neoplasms and may be associated with an adverse outcome. This mutation might not be the major driver that led to the onset of tumorigenesis, and we could consider that the associated TP53 mutation, would be required for malignant transformation.

No MeSH data available.


Related in: MedlinePlus