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Low-grade Bone Lesions in Survivors of Childhood Medulloblastoma/Primitive Neuroectodermal Tumor

Posted Dec 31, 2011 Updated Apr 10, 2023
By Korgun Koral MD , Dave Roy MD , Charles F. Timmons MD PhD , Lynn Gargan PhD , Daniel C. Bowers MD
5 min read

Objectives and Rationale

Medulloblastoma/primitive neuroectodermal tumor (MB/PNET) is the most common malignant tumor of the central nervous system (CNS) in children. MB/PNET survivors are at an increased risk for developing second malignancies. Little has been reported on development of low-grade lesions of the calvarium in the radiation field in MB/PNET survivors. The purpose of this study was to assess the frequency of the low-grade bone lesion development in the radiotherapy field in pediatric MB/PNET survivors and describe the imaging characteristics of these lesions.

Materials and Methods

Institutional review board approval was obtained for this retrospective review which was compliant with Health Insurance Portability and Accountability Act. Forty-one MB/PNET patients (29 male) who survived for at least 2 years after initiation of radiation therapy were included. The medical records were reviewed. The most recent available brain magnetic resonance imaging studies were evaluated.

Results

Three patients (7.3%) developed low-grade calvarial lesions and underwent resection and/or biopsy of the lesions. There were one Langerhans cell histiocytosis, one benign spindle cell lesion with myxoid change, and one fibrous dysplasia.

Conclusion

Development of low-grade bone lesions of calvarium is not very rare in pediatric PNET/MB survivors. Bones in the radiation therapy field need to be carefully examined for assessment of secondary lesions.

Medulloblastoma/primitive neuroectodermal tumor (MB/PNET) is the most common malignant brain tumor of childhood . With contemporary therapy, including surgical debulking, radiation therapy, and multiagent chemotherapy, current progression-free survival rates are up to 81% at 5 years . However, it is known that this impressive survival outcome is accompanied by many late therapy effects, including delay in cognitive functions, hormone deficiencies, growth impairment, second neoplasms, and strokes .

In addition to the late recurrence of the original neoplasm, the common second neoplasms reported among survivors of childhood MB/PNET include secondary leukemia, high-grade gliomas, meningiomas, and basal cell skin cancer . There are few data on the development of the low-grade skull lesions in the radiation therapy fields of pediatric MB/PNET survivors . The purpose of this study was to identify the frequency of low-grade skull lesions in the MB/PNET survivors at our institution and describe the imaging findings of these lesions.

Materials and methods

Patient Population

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Data Review

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Results

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Case 1

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Figure 1, (a) Axial computed tomography image in bone window shows multiple well-defined, lytic lesions ( arrows ) of the calvarium. There is no bony matrix or expansile soft-tissue component. (b) The lesion is composed of sheets of histiocytes with occasional nuclear grooves and abundant cytoplasm consistent with Langerhans cells, accompanied by an infiltrate of eosinophils and neutrophils. (hematoxylin & eosin, original magnification 40×).

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Case 2

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Figure 2, (a) Axial T2-weighted image displays a hyperintense lesion ( arrow ) of the parietal bones at vertex. (b) Sagittal T1-weighted image demonstrates that the lesion ( arrows ) is hypointense. (c) After intravenous gadolinium administration, there is intense enhancement ( arrows ). (d) Computed tomography shows a nonspecific lytic lesion without a bony matrix or expansile soft-tissue component ( arrows ). (e) The lesion is composed of a sparsely cellular proliferation of bland, fusiform cells in a loose connective tissue matrix and labeled as “a benign spindle cell lesion with myxoid change.” (hematoxylin & eosin, original magnification 40×).

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Case 3

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Figure 3, (a) Axial fluid attenuated inversion recovery (FLAIR) image shows a hyperintense left occipital bone lesion ( arrow ). (b,c) Sagittal precontrast and postcontrast images display that the T1-weighted hypointense lesion ( arrows ) enhances intensely with intravenous gadolinium. (d) Axial computed tomography image in bone window shows the characteristic “ground glass” appearance ( arrows ) of fibrous dysplasia. (e) The lesion exhibits biphasic histology, with irregularly shaped spicules and spherules of woven bone generally lacking a rim of osteoblasts and embedded in a stroma of bland fusiform cells without cytologic atypia or mitotic activity. (hematoxylin & eosin, original magnification 40×).

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Discussion

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Academic Radiology, Volume 19, Issue 1
Journals General Radiology
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