<%server.execute "isdev.asp"%> Radiation-induced supratentorial osteosarcoma following curative treatment of infratentorial ependymoma in a child Vankipuram S, Jaiswal M, Singh SK, Qayoo S, Ojha BK - J Pediatr Neurosci
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Year : 2020  |  Volume : 15  |  Issue : 3  |  Page : 304-307

Radiation-induced supratentorial osteosarcoma following curative treatment of infratentorial ependymoma in a child

1 Department of Neurosurgery, Shatabdi Hospital Phase 2, King George Medical University, Lucknow, Uttar Pradesh, India
2 Department of Neurosurgery, Apollo Hospital, Lucknow, Uttar Pradesh, India
3 Department of Pathology, King George Medical University, Lucknow, Uttar Pradesh, India

Date of Submission09-Jan-2020
Date of Decision19-Mar-2020
Date of Acceptance03-Apr-2020
Date of Web Publication06-Nov-2020

Correspondence Address:
Dr. Siddharth Vankipuram
Department of Neurosurgery, Shatabdi Hospital Phase 2, King George Medical University, Lucknow, Uttar Pradesh.
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jpn.JPN_8_20

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The development of secondary neoplasms following therapeutic cranial irradiation is rare and quite often lethal. Meningiomas, sarcomas, and high-grade gliomas are the most common tumors that manifest as a result of radiation therapy. We report the case of an 11-year-old child who presented with symptoms of supratentorial space-occupying lesion 7 years after curative surgery and cranial irradiation for a posterior fossa ependymoma. Magnetic resonance imaging of the brain revealed a right-sided temporoparietal dural-based contrast-enhancing lesion with evidence of overlying bone and skin involvement. The histological report of ependymoma from the previous surgery led us to suspect that we were dealing with a recurrence until the histopathology of the second surgery revealed highly malignant osteosarcoma. The child recovered fully and underwent chemotherapy, but ultimately succumbed to the disease. We report this case to highlight the importance of recognizing these neoplasms and to review its management.

Keywords: Pediatric brain tumor, posterior fossa ependymoma, postradiation sarcoma, radiation-induced osteosarcoma

How to cite this article:
Vankipuram S, Jaiswal M, Singh SK, Qayoo S, Ojha BK. Radiation-induced supratentorial osteosarcoma following curative treatment of infratentorial ependymoma in a child. J Pediatr Neurosci 2020;15:304-7

How to cite this URL:
Vankipuram S, Jaiswal M, Singh SK, Qayoo S, Ojha BK. Radiation-induced supratentorial osteosarcoma following curative treatment of infratentorial ependymoma in a child. J Pediatr Neurosci [serial online] 2020 [cited 2022 Nov 29];15:304-7. Available from: https://www.pediatricneurosciences.com/text.asp?2020/15/3/304/300066

   Introduction Top

The first evidence of causal relationship between ionizing radiation and brain tumors came from a survey of 11,000 children undergoing radiotherapy for tinea capitis.[1] These tumors are being increasingly reported and include sarcomas (especially fibrosarcoma), meningiomas, and high-grade gliomas.[1],[2] Cahan described the criteria for these tumors as: (1) microscopic or radiological evidence that the involved area was initially normal, (2) tumor occurring in an area that has been irradiated, (3) latent period of at least 5 years following irradiation, and (4) histological confirmation of different tumor type.[3] We report a case of supratentorial osteosarcoma following cranial irradiation for posterior fossa ependymoma.

   Case Report Top

An 11-year-old female child presented to us with complaints of holocranial headache, intermittent episodes of vomiting, left eye ptosis, and left-sided weakness for the last 4 months. Seven years back, she had undergone endoscopic third ventriculostomy followed by midline suboccipital craniotomy for the excision of a posterior fossa mass lesion with hydrocephalus [Figure 1]A. The histological diagnosis revealed Grade II ependymoma (ki-67, 20%), for which she underwent radiotherapy. She received 54 Gray (Gy) in 27 fractions cranial irradiation and 5 Gy to tumor bed over 6 weeks. She was on regular follow-up with no evidence of recurrent disease for 7 years till her current illness [Figure 1]B.
Figure 1: (A) Initial T2W axial MRI showing 4th ventricle ependymoma. (B) Postoperative CT scan with no residual lesion. (C–F) Preoperative MRI showing T1W isointense lesion with significant midline shift (black arrow), no recurrence in posterior fossa (blue arrow) and perilesional edema on T2W sequence. Contrast images show dural-based enhancing lesion with bony and galeal involvement (green arrow). Orange arrow in 1F is to indicate heterogenous hyperintense lesion with perilesional edema on T2W sequence. (G, H) Intraoperative photographs revealing bony involvement and dural-based yellowish-white hard tumor. Black arrow in 1G indicates bony involvement while 1H indicates dural-based lesion. (I) Postoperative CT scan showing complete excision

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On examination, she was alert and conscious with a Glasgow Coma Scale (GCS) score of E4V5M6. Neurological examination revealed left-sided third nerve palsy, left hemiparesis (3/5 in both upper and lower limb), with residual left sixth and seventh nerve paresis from the previous surgery. Fundoscopy showed Grade II papilledema, and visual acuity was 6/24 in both eyes. Gadolinium-enhanced magnetic resonance imaging (MRI) revealed a dural-based right temporoparietal heterogeneously contrast-enhancing lesion with irregular margins and areas of necrosis [Figure 1D] and E] The lesion was predominantly isointense on T1-weighted (T1W) and predominantly hyperintense on T2-weighted (T2W) imaging [Figure 1C and F]. Further, the tumor was extending up to the midline with the effacement of ipsilateral lateral ventricles and midline shift to the left [Figure 1C and F]. There were also areas of abnormal enhancement of the overlying bone and galea [Figure 1C–E]. There was no recurrence in the posterior fossa, and screening of whole spine revealed no drop metastasis. After reviewing the MRI, we considered a differential diagnosis of supratentorial anaplastic ependymoma, sarcoma with bony involvement, and atypical/anaplastic meningioma.

As there was a significant mass effect, we proceeded for surgery using a fronto-temporo-parietal skin flap. Raising the skin flap confirmed bony and galeal involvement, and attachment of the tumor to the dura was seen after craniotomy [Figure 1G and H]. The involved galea and involved portion of the bone were excised, and the tumor was completely resected. The patient made a full recovery with improvement in hemiparesis to 4/5 in both upper and lower limbs [Figure 1I]. Histopathological examination reported a highly malignant tumor with bizarre pleomorphic spindle cells and areas of malignant osteoid. Immunohistochemistry was positive for vimentin and osteopontin, whereas it was negative for epithelial membrane antigen and glial fibrillary acidic protein [Figure 2]A–G. On the basis of this, a final diagnosis of postradiotherapy osteosarcoma was formulated, and the patient was administered adjuvant chemotherapy with cisplatin and doxorubicin. The child ultimately succumbed to the disease 3 months after surgery.
Figure 2: (A) Malignant tumor composed of bizarre pleomorphic spindle cells (H&E, ×4). (B) Areas of necrosis (H&E, ×10). (C) Presence of malignant osteoid. (D) Highly pleomorphic spindle cells with hyperchromatic nuclei. (E, F) Immunohistochemistry (IHC) negative for glial fibrillary acidic protein and epithelial membrane antigen, respectively. (G) IHC positive for osteopontin

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   Discussion Top

Improved adjuvant treatment for patients with brain tumors has led to an increase in the number of reported cases of radiation-induced brain neoplasms.[2 The development of these tumors is influenced by factors such as tissue vulnerability],[ cumulative radiation dosage],[ and age of initial irradiation.[2],[4] Malignant gliomas and sarcomas tend to occur with higher dose radiotherapy (15–56 Gy) and present earlier compared to benign tumors, such as meningiomas, which manifest later, with doses less than 15 Gy.[1],[2],[4]

The largest series on radiation-induced tumors has been described by Yamanaka et al.[3] in three separate reviews in which they reported 296 gliomas, 251 meningiomas, and 180 sarcomas. These three groups of tumors account for the majority of cases seen following radiation. Postradiotherapy osteosarcoma is much rarer, accounting for 0.01%–0.03% of all such tumors and develops after a mean latent period of 9 years,[1],[3],[4] though they are still more common than primary intracranial osteosarcomas, which have less than 20 cases reported worldwide.[5]

The incidence of these tumors also varies with the type of radiation administered. Though the exact frequency is not known, the relative risk of stereotactic radiosurgery (SRS) is lower than that of whole brain radiation (WBRT).[6] Reasons include smaller volume of irradiated brain and single dose of radiation that will lead to cytotoxicity but not mutagenicity.[6] However, it would be prudent to counsel such patients, receiving radiosurgery, of the risks of second tumor formation.

Radiological confirmation can be tricky when the tumor develops in the same area as initial pathology. In these cases, recurrence and radiation necrosis have to be considered, and additional imaging modalities such as 201Tl-SPECT (single-photon emission computerized tomography) and FDG-PET (fludeoxyglucose-positron emission tomography) scans are required.[2] In our case, we initially considered recurrent ependymoma keeping in mind the previous histology. Although retrograde supratentorial spread of a posterior fossa ependymoma has been reported, the involvement of galea and bone is rare. Sarcoma fits the clinical diagnosis accurately, given the relatively quick progression of disease and extensive bony invasion.

Surgical resections with tumor-free margins followed by adjuvant chemotherapy are the best indicators for long-term survival. Kellie et al.[7] reported a case of radiation-induced osteosarcoma of the temporal bone, treated by partial resection and followed by ifosfamide, cisplatin, and doxorubicin to achieve a 3-year tumor-free survival period. Carpentier et al.[8] also reported a postradiation sarcoma of the skull, treated successfully with chemotherapy. They suggested a regimen including ifosfamide, vepeside, and adriamycin/cisplatin (ifosfamide, vepeside, adriamycin/ifosfamide, vepeside, cisplatin [IVA/IVP] regimen).

Alternative treatments, such as SRS, are suggested, though outcomes are poor due to the locally aggressive nature of the tumor and the possibility of extraneural metastases.[2]

   Conclusion Top

The need to recognize radiation-induced tumors and establish a clinical diagnosis is vital in prognostication. The risk of tumor development after SRS is lower than that after WBRT. Treatment includes surgical resection followed by adjuvant chemotherapy.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Salvati M, Artico M, Caruso R, Rocchi G, Orlando ER, Nucci F A report on radiation-induced gliomas. Cancer 1991;67:392-7.  Back to cited text no. 1
Nishio S, Morioka T, Inamura T, Takeshita I, Fukui M, Sasaki M, et al. Radiation-induced brain tumours: potential late complications of radiation therapy for brain tumours. Acta Neurochir (Wien) 1998;140:763-70.  Back to cited text no. 2
Yamanaka R, Abe E, Sato T, Hayano A, Takashima Y Secondary intracranial tumors following radiotherapy for pituitary adenomas: a systematic review. Cancers (Basel) 2017;9:103.  Back to cited text no. 3
Hong N, Yoo H, Shin SH, Gwak HS, Lee SH A case of radiation-induced osteosarcoma after the treatment of pineoblastoma. Brain Tumor Res Treat 2015;3:156-9.  Back to cited text no. 4
Pourrashidi Boshrabadi A, Surakiazad M, Yarandi KK, Amirjamshidi A Primary intraventricular osteosarcoma in a 3-year-old boy: report of a case and review of literature. Childs Nerv Syst 2017;33:1389-94.  Back to cited text no. 5
Loeffler JS, Niemierko A, Chapman PH Second tumors after radiosurgery: tip of the iceberg or a bump in the road? Neurosurgery 2003;52:1436-40; discussion 1440-2.  Back to cited text no. 6
Kellie SJ, Hutchison RE, Robertson JT, Pratt CB Successful treatment of a radiation-associated extradural osteosarcoma with chemotherapy in an adolescent girl. Med Pediatr Oncol 1989;17:514-9.  Back to cited text no. 7
Carpentier AF, Chantelard JV, Henin D, Poisson M Osteosarcoma following radiation treatment for meningioma: report of a case and effective treatment with chemotherapy. J Neurooncol 1994;21:249-53.  Back to cited text no. 8


  [Figure 1], [Figure 2]


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