Benign osteoblastoma of the parietal bone

A 5-year-old girl presented with a 1-year history of a subcutaneous swelling in the right parietal region. There were no cerebral symptoms or signs. Plain X-ray of the skull showed a lytic bony lesion with sclerotic margin. Computed tomography showed a hypodense, osteolytic lesion with thinned and bulged inner and outer skull tables with intact continuity. There was no intracranial lesion. At operation, the mass was found to be pink and granular, and was totally enucleated. Histology revealed it to be a benign osteoblastoma.     

Pediatric cranial osteoblastoma: Technical note of surgical treatment and review of the literature

Osteoblastoma of the skull is a rare entity, and they account only for 2-4% of all the cases of osteoblastoma. We perform a comprehensive review of the pertinent literature on the subject and we report a case of a 3-year-old girl presenting with a 6-month history of a supraorbital mass and exophthalmos due to an osteoblastoma of the frontal and ethmoid bones involving the orbit and anterior skull base. A 3D printed model of the patient's skull was used for the preoperative planning and reconstruction strategy. Total en-bloc resection of the tumor followed by immediate reconstruction was achieved. No recurrence was detected 3 years after the surgery. Gross total resection is strongly advised with skull osteoblastoma, especially in young age, because of the risk of the recurrence and malignant transformation. 3D printing is proven to be a valuable tool to enhance surgical performance by avoiding complications while achieving total resection with accurate reconstruction. Long-term follow-up is important to detect recurrences and improve the management of these young patients.     

脊柱骨母细胞瘤的CT诊断(附3例报告)

目的:探讨脊柱骨母细胞瘤的CT表现。方法:3例做CT扫描,均经手术病理证实为脊柱骨母细胞瘤,1例局部恶变为侵袭性骨母细胞瘤。结果:脊柱骨母细胞瘤以椎板和椎弓根好发,呈囊状膨胀性骨质破坏,边缘有轻度硬化,内见小点片状、斑点状钙化、骨化影,部分有软组织肿块。结论:CT扫描能显示肿瘤的内部结构,有利于诊断和鉴别诊断。     

Osteogenic tumors of bone

In this paper we provide an overview of benign and malignant osteogenic bone tumors. We describe the diagnostic features, radiographic findings, and pertinent ancillary studies needed to diagnose these bone-forming lesions. We begin with osteoid osteoma and osteoblastoma, which are histologically bland and eminently benign with rare possible exceptions. On the other end of the behavioral spectrum is osteosarcoma, which encompasses many subtypes ranging from high-grade osteogenic osteosarcoma to less overtly osteogenic lesions such as telangiectatic and small cell osteosarcoma. While classic osteogenic osteosarcoma can be easily recognized by its high grade morphology and formation of extracellular lace-like osteoid, its variants may pose diagnostic dilemmas as their differential diagnoses can include benign, fibrous, and vascular lesions, among others. Recognition of these variants is essential to avoid diagnostic pitfalls. In equivocal cases, some forms of osteosarcoma have shown molecular alterations that may prove diagnostically useful.     

侵袭性骨母细胞瘤的影像诊断与鉴别诊断

目的分析侵袭性骨母细胞瘤（aggressive osteoblastoma,AO）的影像学表现,总结AO的影像学特点及鉴别诊断要点。 方法回顾性分析经病理证实的7例AO的X线、CT和MRI,分析影像学表现包括：发病部位、骨质破坏情况、病变内的密度、病变区的MR信号特点及周围侵袭性特点。 结果7例AO患者中,男性4例,女性3例,年龄为13~56岁,中位年龄32岁。本组7例AO的发病部位是椎体5例,肱骨及肩胛骨各1例。7例AO均表现为膨胀性骨质破坏、骨皮质不完整,6例AO病变内有钙化或骨化,5例AO有厚薄不均匀的肿块影,3例AO病变累及邻骨,2例AO具有较少软组织水肿,1例AO可见骨膜反应。MR表现为T1WI呈不均匀等或低信号,T2WI呈不均匀低、等或高信号,钙化及骨化在T1WI和T2WI均为低信号,增强检查呈不均匀强化,2例病变区可见液液面（合并动脉瘤样骨囊肿）。 结论AO是一种罕见的骨肿瘤,影像学表现有一定的特点:呈膨胀性骨质破坏,病变范围大,皮质不完整,病灶内钙化或骨化。AO应与骨巨细胞瘤、软骨母细胞瘤、骨肉瘤及转移瘤相鉴别。     

骨母细胞瘤的MRI表现

目的探讨骨母细胞瘤的MRI表现及MRI的诊断价值.方法经病理证实的骨母细胞瘤9例,良性5例,恶性4例,全部行MRI检查,3例同时行CT检查.结果MRI主要表现:T1WI呈等或稍低信号,T2WI呈以高信号为主的混杂信号;瘤内有钙化和骨化(5/9),囊变(6/9)及少量出血(2/9),线样低信号间隔(7/9)并可强化(3/7);瘤周有低信号的骨性包壳(9例);增强扫描肿瘤多明显强化.当肿瘤形态不规则,瘤内散在坏死和出血灶,不均匀强化,伴有骨膜反应、软组织肿块时,提示恶性可能.结论MRI有助于骨母细胞瘤的定位及定性诊断,并对肿瘤的良恶性鉴别有一定提示作用.     

A huge occipital osteoblastoma accompanied with aneurysmal bone cyst in the posterior cranial fossa

Osteoblastoma is an infrequent benign tumor and skull involvement is extremely rare. The occipital bone is much less frequently involved. We report an unusual case of a huge occipital osteoblastoma with aneurysmal bone cyst in the posterior cranial fossa of a 20-year-old young man. MRI scan and CT three-dimensional reconstruction revealed that the tumor (approximately 8.5 cm x 6 cm x 5 cm) occupied nearly half volume of the posterior cranial fossa. The cerebellum, the forth ventricle and brain stem were compressed remarkably and hydrocephalus was noted. The tumor mass was multiloculated cystic and highly vascular. Excessive bleeding occurred during operation and total removal was achieved. The patient was symptom-free and MRI scan revealed no evidence of recrudescence after a follow-up period of 18 months. Pathological examination revealed that the lesion was a benign osteoblastoma with secondary aneurysmal bone cyst (ABC). ABC constitutes a highly vascular lesion while complete surgical resection is often impeded by excessive intraoperative bleeding. Preoperative embolization may reduce the risk. Despite the benign nature of osteoblastoma, local recurrence after incomplete resection is not rare and malignant transformation during recurrence may occur even after adequate surgery. Our case confirms the findings of previous investigators that if osteoblastoma is removed completely, no recurrence is expected even without adjuvant irradiation and chemotherapy.     

多中心成骨肉瘤的治疗

目的探讨多中心成骨肉瘤的治疗方法。方法1998年1月至2005年6月治疗多中心成骨肉瘤患者9例，男7例，女2例。9例患者的临床特征、影像学表现、病理学检查均证实多中心成骨肉瘤的诊断。其中6例患者初诊时即存在多发骨肉瘤病灶；3例初诊时为单一骨肉瘤病灶，而后分别在确诊6、8、11个月出现第二处骨肉瘤病灶。1例患者因经济原因放弃治疗，其余8例患者接受阿霉素60mg／m^2,顺铂100mg／m^2,氨甲蝶呤8-12g／m^2长春新碱1．4mg／m^2、异环磷酰胺12．5g／m^2化疗1周期。化疗后根据病灶数目、部位以及对化疗的反应等决定包括手术、化疗在内的进一步治疗方案。对病灶数目较少、病灶可广泛切除的患者，如果化疗反应好，则在化疗的基础上广泛切除肿瘤，手术原则同单发性骨肉瘤；如果全身病灶数目多、部分病灶位于中轴骨等不易广泛切除的部位，则根据病情采取适当的姑息手术。对上述化疗反应差的患者，应用三氧化二砷、紫杉醇、VP-16化疗。结果放弃治疗的1例患者于确诊5个月后死于肿瘤引起的全身衰竭，5例初诊存在多发骨肉瘤病灶的患者平均生存时间9．8个月，其中2例对化疗反应好且完成4周期以上化疗的患者平均生存时间为15个月，对化疗反应差以及未能完成化疗的3例患者平均生存时间仅为7．1个月。3例病灶不同时出现的多中心成骨肉瘤患者平均随访2年，2例带瘤生存，无肺转移；1例无瘤生存。结论多中心成骨肉瘤预后较差，经积极的化疗，能够延长患者生命，改善预后。