Int J Clin Exp Med 2015;8(11):21724-21731 www.ijcem.com /ISSN:1940-5901/IJCEM0015463

Case Report Extensive epidural extension growth of a glioblastoma: a case report and literature review Jinlu Yu1, Limei Qu2, Xu Zheng1, Hongfa Yang1 Departments of 1Neurosurgery, 2Pathology, The First Hospital of Jilin University, Changchun 130021, P. R. China Received September 1, 2015; Accepted November 1, 2015; Epub November 15, 2015; Published November 30, 2015 Abstract: It is extremely rare for a glioblastoma to mainly have epidural growth after its invasion of the dura. The present paper reports such a rare case. The patient was a 37-year-old male. After the onset of headache, computed tomography (CT) examination revealed an extradural convex-shaped lesion on the frontal region that was mainly epidural. During the first surgery, the extradural lesion mainly consisted of bloody viscous liquid; therefore, it was misdiagnosed as simple epidural bleeding, and the best diagnosis and treatment opportunity were missed. One month later, a relapse of the disease occurred, with a symptom of exophthalmos. Imaging examination revealed extensive extradural growth of the lesion along the forehead and invasion downwards into the frontal sinus, sphenoidal sinus, ethmoidal sinus and orbit. The second surgery could not completely remove the lesion, and pathological diagnosis of the lesion was a glioblastoma. One month later, the glioblastoma continued to grow, the third operation was performed, this time most of tumor was removed, the treatment was assisted with radiotherapy and chemotherapy, the tumor was controlled effectively, and the patient did well after six months. While reporting this rare case, we also review the cases of glioblastoma invasion of the dura and their growth and the classification of dura invasions, aiming at improving our understanding in cases of glioblastoma invasion of the dura so that we can make timely, accurate diagnoses. Keywords: Glioblastoma, epidural growth, treatment

Introduction A glioblastoma is a malignant tumor that occurs in the brain tissue and has invasive growth characteristics [1]. However, due to the barrier function of the dura, it is extremely rare for a glioblastoma to penetrate the dura. Even when a malignant glioblastoma achieves extracranial metastasis, it is still uncommon for a glioblastoma to invade the dura [2]. Even if there are such reports, most glioblastomas penetrate the skull through the nerve and blood vessel foramina and fissures at the skull base, growing epidurally and entering the space at the skull base, such as the nasal cavity, the oral cavity and the infratemporal fossa [3, 4]. However, it is rare to encounter a tumor that directly penetrates the cerebral convexity dura and continues to grow. The present study reports a case of a rare frontal lobe glioblastoma that penetrated the frontal dura to enter the extradural space with extensive growth that also involved the orbit and frontal sinus. Beca-

use the tumor growth pattern was rare and was not well-understood, the optimal treatment opportunity was missed. Now, we report this case with the aim of improving the diagnosis and treatment of such disease. Case report The patient, male, 37 years old, was admitted into the hospital due to a “headache for a month”. No other neurological positive signs were revealed upon physical examination. Head computed tomography (CT) showed two convex shape lesions with slightly higher densities on the left frontal region, and a calcification shadow could be observed on the medial side of the larger lesion. The adjacent brain tissue was slightly compressed, and an edema image was visible (Figure 1). The lesions were considered epidural, and lesion removal was planned. The surgery used a left frontal approach. After the skull was opened, the lesions were visible. The lesions consisted of bloody viscous fluid, which

Epidural extension growth of a glioblastoma

Figure 1. CT images before and after the first surgery. A. Preoperative CT showed two convex-shaped lesions with slightly higher densities in the left frontal region, and a calcification shadow (white arrow) could be observed on the inner side of the larger lesion in the front. The adjacent brain tissue was slightly compressed, and edema was visible in the image. B. The lesion in the frontal region showed a typical epidural lesion manifestation. C, D. Postoperative CT revealed that the epidural lesion had been removed but that the calcification inside of the brain was still present (see D, white arrow).

was considered bleeding and was removed by aspiration. The fluid was not sent for pathological examination. After the surgery, the bone flaps were returned, and headache symptoms were relieved. The patient was discharged after recovery. One month after surgery, the patient’s headaches gradually exacerbated, and ghosting of images and exophthalmos of the left eye occurred. The patient returned to the hospital for a reexamination. Physical examination revealed diplopia, exophthalmos of the left eyeball, 21725

and no other neurological positive signs. Head CT revealed relapse of the lesion, which had extended extradurally to the front and top of the left temple and also involved the sphenoidal sinus, the ethmoidal sinus and the frontal sinus. The lesion had also involved the left orbit to cause exophthalmos. The lesion mainly consisted of epidural growth, and cystic degeneration and calcification were visible within it (Figure 2). Lesion resection was planned. The surgery used the incision of the previous surgery. Int J Clin Exp Med 2015;8(11):21724-21731

Epidural extension growth of a glioblastoma

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Int J Clin Exp Med 2015;8(11):21724-21731

Epidural extension growth of a glioblastoma Figure 2. CT Images before and after the second surgery, after the tumor relapsed. A-D. Preoperative CT showed that the lesion was mainly in the left frontal region, and had grown mainly epidurally; cystic degeneration and calcification were also visible. Furthermore, the lesion extended extradurally to the front and top of the temple and also involved the orbit, causing exophthalmos (white arrow). E, F. Postoperative CT revealed that the frontal lobe lesion had been removed, while the distant epidural extension was untreated (white arrow).

Figure 3. Pathological examination results (× 100). A. HE staining showed that neoplastic astrocytes had poor differentiation, high density, apparent nuclear atypia and active nuclear division. The hyperplasia of small blood vessels, vascular endothelial cells and vascular smooth muscle cells was obvious in the tumor interstitium, and local bleeding and calcification were also observed. The cells were highly consistent and appeared as small cells, mainly round or short spindle-shaped. The cells were densely arranged, the nuclei were stained slightly darker, the nucleus-to-cytoplasm ratio was increased, and the cells were anaplastic. B. GFAP expression was positive. C. Vimentin expression was positive. D. The Ki-67-positive rate was over 40%.

When the skull was opened at the surgical region, old extradural bloody fluid was visible, and approximately 30 ml of the fluid was removed. A yellow-whitish secretion could then be observed on the surface of the dura. After the secretion was removed, thickened dura could be observed. The dura mater was cut open to investigate the surface of the brain, where it was found that a dark-colored blood clot covered its surface. After the blood clot was removed, brain lesions were grayish brown in color, with a soft texture without an envelope. The section was grayish-red and grayish-white

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in color, and the texture was delicate, with some local hyaline regions and cystic degeneration. After the diseased tissues were completely removed, they were sent for pathological examination. However, some of the diseased epidural tissues that extended into the distant region could not be treated. The lesion was considered malignant during the surgery, so the bone flaps were removed. After the surgery, the patient’s headache was significantly relieved. Pathological examination revealed neoplastic astrocytes with poor differ-

Int J Clin Exp Med 2015;8(11):21724-21731

Epidural extension growth of a glioblastoma

Figure 4. Enhanced MRI examination 30 days after surgery. A. The tumor invaded the sphenoidal sinus, the ethmoidal sinus and the orbit (white arrow). B. The tumor involved the sagittal sinus and the contralateral epidural space, and a cystic lesion convex in shape (white arrow) was visible in the epidural space in the frontal region. C. The tumor involved the frontal sinus and extensive epidural space (white arrow).

entiation, high density, apparent nuclear atypia and active nuclear division. Significant hyperplasia of small blood vessels, vascular endothelial cells and vascular smooth muscle cells were visible in the tumor interstitium. Local bleeding and calcification were also observed. Tumor cells were highly consistent; the cells were smaller in size and round or short spindleshaped. The cells were densely arranged, the nuclei were stained slightly darker, the nucleusto-cytoplasm ratio was increased, and the cells were anaplastic. Immunohistochemical results revealed glial fibrillary acidic protein (GFAP) (+), Vimentin (+), Ki-67 (> 40%), O-6-methylguanine DNA methyltransferase (MGMT) (> 60%), S100 (+), and Nestin (+) (Figure 3). The pathological

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diagnosis was small cell glioblastoma (World Health Organization [WHO] grade IV). Thirty days after the surgery, magnetic resonance imaging (MRI) was performed and yielded evidence that the tumor had continued to grow and extend into epidural region, invading the sphenoidal sinus, the ethmoidal sinus and the orbit, involving the sagittal sinus and the contralateral epidural region. Cystic lesion formation, which was convex in shape, could be observed in the frontal epidural region. The tumor also involved the frontal sinus and extensive epidural space (Figure 4). The third operation was performed, during the operation the bone flap was removed and the bone window

Int J Clin Exp Med 2015;8(11):21724-21731

Epidural extension growth of a glioblastoma

Figure 5. CT examination after the third surgery. A, B. Most of the tumors invaded the orbit and tempora were removed. C, D. The tumor involved in the epidural space in the frontal region was removed, the tumor involved the extensive parietal epidural space was left.

was enlarged to left middle skull base to explore the tumors, meanwhile the orbit was opened, most of recurrent tumors were resected (Figure 5). Postoperative adjuvant radiotherapy and chemotherapy were used. Radiation therapy was conducted with the tumor bed as the center, and radiotherapy was also conducted on the residual extradural tumor, which was not removed surgically. The course of treatment was six weeks, 5 times each week, with 64 Gy each treatment. Meanwhile, oral administration of temozolomide was used for chemotherapy for 5 treatment courses, 1 course every 28

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days, with the drug taken during the first five days of a course. The dose for the first course was 250 mg per day, and the doses for the second to the 5th courses were 350 mg per day. The tumor was controlled effectively, and the patient did well after six months. Discussion Gliomas are tumors that grow in brain tissue. During their growth, they are mainly confined to the brain tissue, and extracranial metastases rarely occur. However, with increasing grades of

Int J Clin Exp Med 2015;8(11):21724-21731

Epidural extension growth of a glioblastoma glioma, such as the WHO grades III and IV tumors, invasiveness also gradually increases [5]. Glioblastoma is a common malignant glioma with a very strong invasive growth capacity. It can grow across different cerebral lobes, such as involving frontal and temporal lobes, and can grow contralaterally along the corpus callosum [6]. Extracranial metastases of glioblastoma have also been observed. For example, if a glioblastoma invades the soft membrane, after shedding, it will fall into the subarachnoid space, and it will spread and metastasize with the cerebrospinal fluid. Alternatively, it can metastasize and grow through the surgical region of a craniotomy, a puncture biopsy or a shunt. It can even metastasize through the lymphatic and blood circulation systems [7]. For example, in 2013, Grah et al. reported a 59-year-old female case who, after having multiple surgeries, developed leptomeningeal and intramedullary metastases [8]. In another example, in 2010, Zhen et al. reported a young patient who had a grade II astrocytoma removed. One year later, it had transformed into a glioblastoma, and cervical lymph node and multiple bone metastases occurred [9]. Moreover, glioblastomas may also relapse and grow along the surgical path. For example, in 2015, Forsyth et al. reported a case of glioblastoma multiforme. The relapse of the tumor occurred six months after the surgery. The tumor grew in the surgical region and invaded the skull [10]. Minimally invasive biopsies may also lead to the growth of glioblastomas along the puncture tract. For example, in 2001, Aichholzer et al. reported a case of a 42-year-old male patient who had a puncture biopsy of a glioblastoma at the basal ganglia. The patient died of cerebral edema and heart disease, and the autopsy showed that the tumor had grown epidurally along the puncture tract in a nodular shape. Hence, metastasized growths of glioblastoma can be diversified. Even though the invasiveness of a glioblastoma is very strong, it is still extremely rare for the tumor to directly invade the dura mater and to develop epidural extensions and growth; such cases are limited to only a few reports. For example, Horiuchi reported a case of temporoparietal glioblastoma in 1996. When tumor relapse occurred, the tumor directly breached the middle cranial fossa base and entered the orbit, oral cavity and nasal cavity [11]. Orita et

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al. also reported a case of a 38-year-old male with frontal lobe oligodendroglioma in 1989. Ten years after surgery, the tumor relapsed as glioblastoma, and the tumor entered the nasal cavity and orbit along the olfactory nerve [2]. In another example, Kwak et al. reported a case of a 53-year-old male with a glioblastoma. When the glioblastoma relapsed, it penetrated the hole of the middle cranial fossa base and entered the masticator space [12]. The transdural outgrowth pattern of glioblastomas was categorized by Kawano et al. in 1977. The first type of outgrowth that the tumor uses is the perivascular or dural slit, especially at the middle cranial fossa. The second type of outgrowth that the tumor uses is crossing of the dura mater by way of the cranial or spinal nerves. The third type of outgrowth involves the tumor directly destroying the dura, though this type is rare [13]. The case reported in the present paper belongs to the rare type 3 transdural growth type, but what is even more special is that the tumor invaded the dura mater over the convexity of the frontal region of the brain to enter the epidural space without destroying the skull, instead extending and growing as an epidural hematoma. This growth pattern may occur because the adhesion between the dura mater over the brain convexity and the skull is not close, and growing in this manner is not as difficult as separating the dura mater from the bone at the skull base. The glioblastoma in the present case invaded the dura mater over the brain convexity and formed an image feature that was similar to an epidural hematoma; in addition, the lesion appeared as bloody viscous fluid during surgery. Thus, the condition was misdiagnosed as simple bleeding, and the optimal diagnosis and treatment opportunity was missed. This occurrence caused the tumor to quickly relapse and to extend extensively into the extradural space, and the opportunity to remove it completely was then lost. The pathological diagnosis was small cell glioblastoma. Therefore, we should improve our understanding of the third type of transdural growth pattern of a glioblastoma. Because it is rare for a glioblastoma to invade the dura and grow, at present, there is no mechanism for the molecular biological evaluation of these tumor cases. However, from the literature, we have learned of the biological features of extracranial metas-

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Epidural extension growth of a glioblastoma tases of glioblastomas. For example, Ueda et al. studied a case of recurrent extracranially metastasized glioblastoma in 2003 and found that the extracranial metastasis occurred through the blood and was spread by directly invading blood vessels. After studying the gene expression of this tumor case, they found that insulin-like growth factor-binding protein 2 and DNA-dependent protein kinase were overexpressed; therefore, they speculated that these two factors may be associated with the extracranial metastasis of glioblastoma [14]. Therefore, a glioblastoma that invades the dura mater and grows may also express certain special growth factors, but multiple cases and multiple specimen screening studies are needed to confirm this hypothesis. Relying on the specimen from a single case to investigate the expression of a certain factor does not carry much significance. Glioblastoma is a WHO grade IV highly malignant brain tumor. Currently, there is no effective treatment for this condition. Rather, comprehensive treatment is emphasized. Early complete surgical resection and postoperative adjuvant radiotherapy and chemotherapy are the keys to treatment. Even so, the prognosis is still very poor, and the survival duration is very short. Therefore, our understanding of the third type of invasion of the dura mater by a glioblastoma should be improved. Apart from the invasion of the dura mater at the skull base, the glioblastoma may also invade the dura over the brain convexity and grow extensively in the extradural space. Early diagnosis and treatment of this type of invasion are key. Disclosure of conflict of interest None. Address correspondence to: Hongfa Yang, Department of Neurosurgery, First Hospital of Jilin University, 71 Xinmin Avenue, Changchun 130021, Jilin, P. R. China. E-mail: [email protected]

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Int J Clin Exp Med 2015;8(11):21724-21731

Extensive epidural extension growth of a glioblastoma: a case report and literature review.

It is extremely rare for a glioblastoma to mainly have epidural growth after its invasion of the dura. The present paper reports such a rare case. The...
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