J Korean Med Sci.  2013 Sep;28(9):1394-1398. 10.3346/jkms.2013.28.9.1394.

An Infant with Prenatally Diagnosed Congenital Anaplastic Astrocytoma Who Remains Disease-Free after Proton Therapy

• Shin HJ
• Kwon YJ
• Park HJ
• Park BK
• Shin SH
• Kim JY
• Lee SH
• Kim HS
• Kim DW

• KMID: 1793059
• DOI: http://doi.org/10.3346/jkms.2013.28.9.1394

Abstract

The authors present a rare of prenatally diagnosed congenital anaplastic astrocytoma. A 9-month-old boy had three recurrences despite two surgical resections and various chemotherapeutic regimens. He underwent the 3rd gross tumor removal at 11 months of age, followed by proton therapy, and now he remains disease-free for 3 yr without a significant neurocognitive dysfunction. This is the 1st case of a pediatric tumor treated by proton therapy in Korea, and proton therapy may be a treatment of choice for a congenital anaplastic astrocytoma in infants and young children, considering limitation of radiation therapy.

Keyword

Congenital Anaplastic Astrocytoma; Proton Therapy; Recurrence

MeSH Terms

Astrocytoma/*diagnosis/radiotherapy/surgery
Brain Neoplasms/*diagnosis/radiotherapy/surgery
Disease-Free Survival
Humans
Infant
Magnetic Resonance Imaging
Male
Prenatal Diagnosis
Proton Therapy
Tomography, X-Ray Computed

Figure

• 

  Fig. 1 Brain MRI at 1 week after birth showing a well-defined, purely solid mass occupying entire left occipital lobe, measuring about 70×42×50 mm. The mass shows homogeneous isointensity with gray matter on all sequences and subtle enhancement in the inferior aspect of the tumor mass. (A) Axial, T1 weighted image, (B) Axial, T2 weighted image, (C) Sagittal, T1 weighted image, (D) Sagittal, T2 weighted image.

• 

  Fig. 2 Photomicrograph showing diffuse proliferation of astrocytes with large nuclei and frequent mitoses (arrows) obtained in a biopsy from the initial surgery (H&E, original magnification×400).

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  Fig. 3 Proton planning showing 95% coverage of target volume with no radiation dose beyond the target. Proton therapy was administered after the third operation, and with a total dose 40 CGE/20 Fx for 4 weeks. (A) Axial image, (B) Dose volume histogram, (C) Coronal image, (D) Sagittal image.

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  Fig. 4 Last follow up brain MRI (3 yr post-proton therapy) showing grossly no interval change of large surgical defect, left parietooccipital operative bed. (A) Axial, T1 weighted image, (B) Sagittal, T1 weighted image.

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