Calvarial Ewing s Sarcoma Presented with Increased Intracranial Pressure Signs in an 11-year-old Child

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1 台灣癌症醫誌 (J. Cancer Res. Pract.) 26(2),72-76, 2010 Case Report journal homepage: Calvarial Ewing s Sarcoma Presented with Increased Intracranial Pressure Signs in an 11-year-old Child Shu-Mei Chen 1,2, Tai-Ngar Lui 2, Chi-Cheng Chuang, Wen-Ching Tzaan, Hsien-Chih Chen * 1 Graduate Institute of Clinical Medical Sciences, College of Medicine, Chang Gung University, Taoyuan, Taiwan 2 Department of Neurosurgery, Taipei Medical University-Wan Fang Hospital, Taipei Medical University, Taipei, Taiwan Department of Neurosurgery, Chang Gung Memorial Hospital at Keelung, Chang Gung University, Taiwan Abstract. Dr. James Ewing first described Ewing s sarcoma (ES) in ES is the second most common primary malignant bone tumor in children and adolescents. ES generally develops in long bones or pelvis and rarely occurs in the calvarial skull. In this article, we describe a female child who suffered from a mild head injury, with signs of increased intracranial pressure developing one month later. ES was found in the parietal skull, and an emergency craniectomy was performed to remove the lesion. Postoperative examinations confirmed the diagnosis of multiple metastatic ES. Finally, the patient died of the progression of multiple metastases. Calvarial ES is rarely reported in the literature. Signs of IICP may become more prominent after a head injury in the case of ES, often leading to an emergent surgical procedure. 病例報告 Keywords : Ewing s sarcoma, skull tumor, emergency craniectomy, signs of increased intracranial pressure 顱骨 Ewing 氏肉瘤的 11 歲孩童表現出顱內壓升高之症候 陳淑美 1,2 雷大雅 2 莊 政 昝文清 陳先志 * 1 長庚大學醫學院臨床醫學研究所 2 臺北醫學大學市立萬芳醫院神經外科 長庚大學基隆長庚紀念醫院腦神經外科 中文摘要 Ewing 氏肉瘤 (Ewing s sarcoma) 最早是在 1920 年代由 Dr James Ewing 所命名 它是兒童和青少年第二常見的惡性骨頭腫瘤, 但它通常發生在長的骨頭或骨盆骨中, 極少發生在頭顱骨 本文中, 我們報告一個少見的扁平頭顱骨 Ewing 氏肉瘤病例, 十一歲的小女孩在受過頭部輕微撞擊後, 發現後頭頂部有一腫塊, 初期誤以為是頭皮下血腫, 一個月後, 病人以顱內壓升高症候為表現, 電腦斷層顯示出一個頭頂部顱骨腫瘤併對腦造成壓迫, 因此我們安排緊急的顱骨移除外科手術, 術後診斷為扁平頭顱骨 Ewing 氏肉瘤並已肝 肺轉移, 雖然經過化學治療最後仍死於多處轉移 頭顱骨 Ewing 氏肉瘤病例在文獻報告上屬於少見, 在經過頭部撞擊後, 可能加速腦壓過高的表現, 而需要緊急開顱手術

2 S. M. Chen et al./jcrp 26(2010) 關鍵字 : Ewing 氏肉瘤 顱骨腫瘤 緊急顱骨移除外科 顱內壓升高症候 INTRODUCTION Ewing s sarcoma (ES) was first described by James Ewing in 1921 as a bone tumor originating from the endothelial cells of blood vessels in the bone [1]. Today it is certain that Ewing sarcoma is originated from primitive neuroectodermal tissue. The tumor is part of the Ewing family of tumors, and can be seen both in bones and soft tissues. It accounts for approximately 10% of primary malignant bone tumors [2]. Primary ES usually develops in a long bone or pelvis, and rarely occur in the skull bone. The most frequent neurological signs in patients with intracranial ES are headache and papilledema []. Herein is described a rare case of calvarial skull ES that initially presenting with signs of increased intracranial pressure (IICP) one month after a mild head injury and eventually required emergent craniectomy surgery. CASE REPORT An 11-year-old female child suffered a mild head injury after another child slapped her on the back of the head during play. Initially, she felt no discomfort and paid no attention to the injury. However, one week later, she began to have headaches while jumping and lost her appetite. Her parents noted a small soft mass on the right side of her head. The girl visited our pediatric neurological out-patient department, where no neurological deficit was found and the initial impression was a small subgaleal hematoma. One month after the head injury, the headache had worsened suddenly and projectile vomiting was noted. The patient was sent to our emergency department, where brady- *Corresponding author: Dr. Hsien-Chih Chen * 通訊作者 : 陳先志醫師 Tel: ext.2670 Fax: samchen@cgmh.org.tw cardia with elevated blood pressure was found. The emergent brain computerized tomography (CT) revealed a large bony destructive calvarial lesion in the right parietal region with compression of the underlying brain tissue (Figure 1). Because of obvious signs of IICP, emergent craniectomy was performed to remove the lesion. Lifting the scalp flap revealed a bony mass of slightly soft consistency (Figure 2), and lifting the skull flap revealed that the tumor was predominantly located in the epidural space and compressed the underlying dura and brain. Following gross total removal of the skull lesion, cranioplasty with methyl methacrylate was performed immediately to fix the skull defect. Initially, the hematoxylin eosin (H&E) stain revealed malignant small blue cell tumor. The differential diagnosis of small blue round cell tumor include lymphoma, leukemia, Ewing's sarcoma, rhabdomyosarcoma, neuroblastoma, and metastatic carcinoma of small cell lung cancer. Unlike most other small blue cell tumors of childhood, ES strongly expresses the MIC2 (CD99) antigen. Then, the specific immunohistochemical pathological findings (O-1[+], vimentin[+], NSE[+], and chromogranin[-] malignant small blue round cell tumor) confirmed the diagnosis of Ewing s sarcoma family of tumors (Figure ). O-1 is the antibody to MIC2 (CD99) gene product. Due to symptoms of multiple trunk pain and cough, post-operative surveys (including bone scan, chest CT and abdominal CT) revealed metastases in the T5 and T10 spinal vertebrae, liver, and lung. The girl was treated for multiple systemic metastases. Endoxan and prednisolone (the first course) were administered under the initial impression of malignant small blue cell tumor (by H&E stain) and then changed to etoposide and ifosfamide after the final diagnosis of ES was confirmed by the specific immunohistochemical stains. The patient received three one-month courses of the combination therapy of etoposide and ifosfamide.

3 74 S. M. Chen et al./jcrp 26(2010) Figure 1. A large, bony-destructive heterogeneous-enhanced skull lesion of the right parietal region with a midline shift to the left Figure 2. The bone flap, removed by craniectomy, displayed a circlet of soft tumor protruding from it. The tumor compressed the underlying brain but it did not breach the dura Two months after the chemotherapy and five months after craniectomy, she was alive without any neurologic deficit or signs of IICP. The brain MRI revealed no local recurrence of the tumor. However, eight months after craniectomy, the girl died from multiple metastases and respiratory failure and related sepsis. DISCUSSION ES is the second most common bone cancer of childhood, accounting for 42% of cases [4]. The most common age at the presentation is the second decade. ES arising from the skull is excessively rare. Involvement of the skull bone occurs in less than 1% of ES cases [5-8]. Our subject case was an 11-year-old girl suffering a minor head injury who then developed IICP signs one month later. The pre-operative CT scan revealed destructive bony changes of the lesion on the bone window and confirmed the diagnosis to be a calvarial skull lesion (Figure 1). In comparison to patients with primary skull tumors, patients with skull metastases present less frequently with a neurologic deficit and are characterized as having higher age and shorter duration of symptoms [9]. Due to the young age of our case, the differential diagnosis should be more likely primary skull tumor, but metastasis from a child malignancy should also be considered. In contrast to the frequent metastasis seen in ES in general, distant metastasis is rare in patients with primary calvarial Ewing s sarcoma [10]. Besides primary ES of the skull, ES may metastasize to the skull from remote primary sites. If the skull lesion is the single site of ES, the prognosis is better. In opposition, if the ES has

4 S. M. Chen et al./jcrp 26(2010) become a multiple metastatic status, the prognosis is poorer, obviously. Therefore, workup for extracranial primary sites is indicated in all cases of ES of the skull. Aggressive adjuvant therapy such as chemotherapy and radiotherapy should be given after the establishing the diagnosis with definitive surgical treatment, particularly at the sites from which the lesion is difficult to remove totally, such as the skull base. Calvarial ES rarely presents with signs of IICP initially, and therefore needs emergent craniectomy. Among the examples of calvarial ES cases, a 2-year-old boy did not present any neurologic deficit or IICP signs and did not need emergent neurosurgery [11]. On the contrary, two cases needed emergent neurosurgery, an extradural ES in an 11-year-old girl who presented with sudden onset of headache and diplopia that lead to an acute surgical emergency [12] and a primary Ewing s sarcoma of the occipital bone in a boy aged 1 year and 4 months who presented with obstructive hydrocephalus [1]. The parents of the patient reported here would not have noticed the scalp mass if their daughter had not suffered from the head injury. This head injury, however, misled parents and doctors to suggest a scalp hematoma rather than a skull bone tumor until the IICP signs developed, calvarial ES was diagnosed and became an emergent indication for decompressive craniectomy. In conclusion, ES usually develops in a long bone and is infrequent in a skull bone. A calvarial ES can present as an emergent surgical condition. A survey of extracranial primary sites or metastases is necessary. Figure. Microscopically, the tumor consisted of sheets of small, round cells that were immunopositive for O-1 (A) and vimentin (B). The cells were immunonegative for chromogranin (C) (original magnification 200) REFERENCES 1. Ewing JE. Diffuse endothelioma of bone. Proc N Y Pathol Soc 21: 17-24, Park TS, Kaufman BA. Tumors of the skull and metastatic brain tumors. In: Cheek WR, Marlin AE, McLone DG, Reigel DH, Walker ML, eds. Pediatric Neurosurgery, rd ed. Philadelphia: Saunders, pp47-445, Falk S, Alpert M. The clinical and roentgen as-

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