A giant cranial aneurysmal bone cyst associated with fibrous dysplasia

An aneurysmal bone cyst (ABC) is a rare, benign fibro-osseous lesion, considered a vascular phenomenon secondary to fibrous dysplasia or a giant-cell tumour, and occurs mainly in long bones and vertebrae. In this case report a 16-year-old male presented with massive epistaxis. He was admitted with a 3-year history of chronic rhinitis, headaches, right ocular pain and recurrent epistaxis. CT scans showed a predominantly cystic, expansive mass obstructing both nasal cavities, extending to all paranasal sinuses and both orbits, with evidence of anterior cranial fossa skull base destruction. The patient underwent a craniofacial resection of the tumour performed with an external approach and an immediate reconstruction of the dural defect. Histology confirmed the lesion was an ABC associated with fibrous dysplasia. The patient's recovery was complete. A large facial aneurysmal bone cyst can damage the facial skeleton and skull base, and requires excision by a combined external approach.     

Aneurysmal bone cyst of the cranial base treated by partial resection and calcitonin injection. A case report

STUDY DESIGN: First published report of a cranial aneurysmal bone cyst (ABC) treated successfully with intralesional injection of calcitonin. OBJECTIVES: To describe a safe and effective treatment method for ABCs of the cranial base. Summary of background data: ABC is a rare form of dystrophic pseudotumor. Less than 100 cases involving the skull have been reported in the literature, most of them localised in the cranial vault. Cranial base locations are rare and difficult to treat. We selected this treatment after a very rapid recurrence of the lesion following a partial resection. Method. - After a partial resection of a 10 cm petro-occipital ABC that encased the vertebral artery and the lower cranial nerves, an Ommaya reservoir was implanted with a catheter tip inside the ABC. Repeated intralesional injections of calcitonin were performed through the reservoir. RESULTS: Shrinkage of the cyst occurred with disappearance of its heterogeneous cystic content and ossification of its walls. There was no complication and the lesion remains quiescent at a 3 year follow-up. CONCLUSION: We reviewed the pertinent literature concerning percutaneous treatment of ABC. The percutaneous intralesionnal injection of calcitonin was reported in the literature only in 3 publications reporting 9 cases that did not involve the skull. This treatment seems safe and effective, worthy in cranial base ABCs that are difficult to resect completely.     

Monostotic fibrous dysplasia of the cranial vault: a case report

Fibrous dysplasia, when it occurs in the craniofacial region, mostly involves the skull base and is rarely localized in the cranial vault. Although there have been several reports on magnetic resonance imaging (MRI) findings of fibrous dysplasia involving the skull base, cases occurring in the cranial vault have seldom been reported. We describe here a rare case of monostotic fibrous dysplasia that occurred in the parietal bone and discuss the characteristics of the MRI findings. A 47-year-old female was admitted to our hospital with a complaint of vertigo. A computed tomography (CT) scan did not reveal any intraparenchymal lesions in either the infra- or supratentorium, and her vertigo improved immediately without any treatments. However, a solitary osteolytic lesion was found incidentally in the left parietal bone. MRI showed that the lesion demonstrated hypointensity on T1-weighted images and hyperintensity on T2-weighted images, and was enhanced heterogeneously following injection of Gadolinium-DTPA. Removal of the parietal bone containing the lesion was performed according to the patient's wishes. The histopathological findings of the removed tissues corresponded to fibrous dysplasia. Although it is well known that craniofacial fibrous dysplasia demonstrates iso- or hypointensity on T1- as well as T2-weighted images, in the present case, the lesion showed apparent hyperintensity on T2-weighted images. These findings suggest that fibrous dysplasia can display various MR intensities depending on its origin.     

Pigmented villonodular synovitis of the temporomandibular joint: a rare tumor of the temporal skull base

Musculoskeletal tumors are not usually encountered at the skull base. Benign lesions such as cholesterol granuloma, cholesteatoma, fibrous dysplasia, and Paget disease are common examples of such tumors arising from the bone of the cranial base. The authors report a case involving an adult male patient with the rare finding of pigmented villonodular synovitis of the temporomandibular joint. This case is reported as an example of another lesion to consider in the differential diagnosis. Due to the complexity of the anatomy at the cranial base, radical resection as recommended for the usual sites of occurrence in major joints is problematic. Because of the reported high recurrence rates at other sites, as radical excision as possible with vigilance for recurrence is recommended.     

Fibrous tumors of bone

Benign and malignant fibrous tumors of bone are some of the most common tumors encountered by the orthopedic surgeon. Fibrous dysplasia, fibrous cortical defect, non-ossifying fibroma, benign fibrous histiocytoma and osteofibrous dysplasia are benign tumors best treated conservatively or by intralesional/marginal excision. Desmoplastic fibromas are locally aggressive and require a wide margin for surgical cure. Malignant fibrous histiocytoma and fibrosarcoma of bone are malignant bone tumors and must be treated with wide/radical margins and for malignant fibrous histiocytoma adjunctive chemotherapy.     

Fibrous dysplasia of the temporal bone: the use of computerized tomography

Fibrous dysplasia of the temporal bone is a rare condition characterized histologically by proliferation of fibrous tissue with scattered trabeculae of immature bone. Eighteen cases of monostotic fibrous dysplasia of the temporal bone have been reported in the literature. The clinical course of temporal bone fibrous dysplasia is unpredictable. Potential complications include cholesteatoma, recurrence, and malignant transformation. Surgery has been the recommended treatment, but the indications, approach, and extent have not been clearly established. The introduction of computerized tomography with high-resolution bone reconstruction is a significant advance in the therapeutic approach to temporal bone fibrous dysplasia. It accurately defines the extent of the disease within the temporal bone, and periodic scanning will reveal any progression. This information can be used to resolve many surgical dilemmas and to minimize secondary complications. This article includes a comprehensive review of the literature on temporal bone fibrous dysplasia and summarizes a case in which computerized tomography was used.     

病灶清除、植骨联合内固定治疗下肢长管状骨纤维结构不良的疗效和并发症观察

背景：骨纤维结构不良临床上可表现为单发或多发的骨骼畸形、疼痛和病理性骨折。目前该病的治疗方法以手术清除病灶、植骨内固定为主。目的：探讨病灶清除、植骨联合髓外固定治疗下肢长管状骨纤维结构不良的疗效和可行性。方法方法：选取2010年2月至2012年11月我院收治的15例骨纤维结构不良患者的病例资料进行回顾性分析,男8例,女7例;年龄26-48岁,平均（32.1±1.2）岁;股骨9例,胫骨6例。均采用病灶清除,取髂骨植骨,动力髋螺钉或锁定接骨板固定。围手术期予抗炎、补液对症处理,术后石膏固定,指导患者功能锻炼。髋关节功能按Harris评分系统评定。膝关节功能按Merchan评分系统评定。术后定期摄X线片,观察肿瘤复发情况,植骨融合情况和不良反应发生情况。结果：所有伤口均甲级愈合,按期拆线。所有患者均完成18个月随访,无1例复发,植骨均融合,术后部分负重下地时间（4.3±0.6）个月,X线片未见骨溶解,术后（4.0±0.8）个月可见新生骨长入,2例患者残留手术区疼痛,对症治疗后缓解。术后髋关节功能Harris评分为97.2分,膝关节功能Merchan评分为95.8分,均较术前显著提高（P〈0.05）。结论：病灶清除、植骨联合髓外固定在下肢长管状骨纤维结构不良患者中固定可靠,预后佳,是一种可行的治疗方法。     

经眶外侧颅内外联合径路治疗颅眶骨纤维异常增殖症

目的 探讨颅眶骨纤维异常增殖症手术治疗的径路. 方法 对累及额骨、眼眶、蝶骨、颞骨的复杂颅面骨纤维异常增殖症患者,应用经眶外侧颅内外联合径路进行手术治疗,包括眶外侧及颞部开窗,病灶的部分或大部切除,视神经减压,颅眶骨外形重建等. 结果 自2005年以来,于临床治疗8例患者,术后随访9个月至3年,无明显并发症发生,复杂颅眶部畸形得以矫正,视力障碍有所改善.头颅CT示骨瓣愈合良好,未见病变复发. 结论 经眶外侧颅内外联合径路可作为治疗颅眶部骨纤维异常增殖症的首选方法.     

Primary aneurysmal bone cyst of the proximal tibia crossing the open physis

An aneurysmal bone cyst (ABC) is a hyperplastic reactive lesion of unknown etiology consisting of cystic cavities containing blood lined by mesenchymal reactive tissue. It occurs more often in females (2:1), usually before the age of 20 years, and most commonly in long bones [1]. The extension of the primary ABC to the epiphysis after crossing the viable physis is an exceptional finding, and very few such cases have been reported in the literature [2–6]. Although uncommon, ABC can cause severe destruction of long bones in children and young adults. The suggested treatment for such aggressive cases ranges from simple curettage to wide resection and structural reconstruction [7, 8]. Here, we report the follow-up for a 10-year-old girl with large, aggressive ABC of the proximal tibia with transphyseal extension to the epiphysis, which was treated with intralesional excision, curettage and bone grafting.     

Aneurysmal bone cyst of the zygomatic arch

Background: Aneurysmal bone cysts are rare vascular lesions that are most commonly found in the long bones. They are rare in the head and neck. Only two prior cases of aneurysmal bone cysts of the zygoma have been reported in the world literature. Results: We report a case of aneurysmal bone cyst arising in the zygomatic arch with intracranial extension treated with selective arterial embolization and complete excision via an infratemporal fossa approach. This is the first such case reported in the head and neck surgery literature. Conclusions: Selected cases of aneurysmal bone cyst may be safely treated with selective arterial embolization and complete resection.     

Activating mutations of Gs protein in monostotic fibrous lesions of bone

Activating mutations of the alpha chain of the heterotrimeric signal transducer Gs disrupt the inherent guanosine triphosphatase activity of the alpha chain, stimulate adenylyl cyclase, and can result in independent cell proliferation. Such mutations are identified in a number of endocrine disorders, including McCune-Albright syndrome, which is a triad of endocrinopathy, café au lait spots, and polyostotic fibrous dysplasia. The mutation in this syndrome is a missense point mutation in exon 8 that results in the substitution of either histidine or cysteine for arginine at position 201. Monostotic fibrous dysplasia is a nonhereditary isolated bone lesion. Other isolated bone lesions that share some cytologic and clinical similarities to fibrous dysplasia are osteofibrous dysplasia and aggressive fibromatosis involving bone. Four cases of monostotic fibrous dysplasia, four cases of aggressive fibromatosis involving bone, and one case of osteofibrous dysplasia were studied to determine if a mutation was present in exon 8 of the alpha chain of Gs. A missense mutation was present in all of the fibrous dysplasias. The other fibrous lesions and uninvolved tissue did not contain a mutation. Somatic activating mutations of Gs differentiate fibrous dysplasia from the other lesions and may be responsible for the loss of control of local proliferation and growth factor expression.     

Monostotic fibrous dysplasia in the parietal bone: a case report

In this paper a case of monostotic fibrous dysplasia in the left parietal bone is described. This 51-year-old female was admitted to our hospital for a head injury on January 12, 1983, and a flat, painless hump (7 X 7cm) was incidentally found in the left parietal region. Plain x-ray film of the skull showed a multicystic lesion with slightly sclerotic margin in the left parietal bone, and outward bulging of the outer table without destruction of the inner table in tangential view. CT scan at the level of bone window clearly demonstrated the same abnormality and intradiploic mass separated by some bony septums. Angiography revealed no positive findings, but RI bone scan (99m Tc) showed an abnormal uptake in this lesion. On February 1, 1983, operation was performed to verify the nature of the lesion and for relief of the left-sided headache. Post operative course was uneventful. Histological findings of this mass were of inactive fibrous dysplasia with fibrous tissue replacing the normal bone and surrounded by island or scorlled edge of bones. Monostotic fibrous dysplasia in the cranial vault is rare. It is often possible to make diagnosis in this disease on the clinical course, plain x-ray film and CT scan showing cystic findings and outer table bulging, and RI scan demonstrating abnormal uptake.(ABSTRACT TRUNCATED AT 250 WORDS)     

Magnetic resonance imaging and [11C]methyl-L-methionine positron emission tomography of fibrous dysplasia--two case reports

Two cases of fibrous dysplasia in the skull base bone appeared hypointense on T1- and T2-weighted magnetic resonance imaging, with accumulation of [11C]methyl-L-methionine ([11C]Met) on positron emission tomography (PET). Fibrous dysplasia is a benign bone disorder which is identified by its distinctive radiography, computed tomography, and bone scintigraphy findings. [11C]Met PET may indicate the presence of viable tumor-like cells in fibrous dysplasia.     

Aneurysmal bone cyst of the temporal bone: case report

BACKGROUND: Aneurysmal bone cysts (ABCs) are uncommon lesions of the temporal bone and their occurrence in the calvarium is rare. CASE DESCRIPTION: A case of a right temporal ABC is reported in a 14-year-old boy who presented swelling of the right temporal region. Magnetic resonance imaging showed a destructive and expansile bone lesion on the right anterior temporal and orbital bone. The lesion was removed in total by the right temporal craniotomy and orbitozygomatic osteotomy. ABC was diagnosed in the pathologic examination. The patient had good recovery during the postoperative course. CONCLUSIONS: This report presents the diagnosis and imaging of an ABC in the temporal bone. This localization is very rare for ABC. Total excision, if feasible, is the ideal treatment.     

Surgical reconstruction of fibrous dysplasia of bone in long-term follow-up

PURPOSE: To examine how different operative measures influence the surgical outcome in patients with fibrous dysplasia of bone. METHODS: 118 dysplastic fibrous lesions of bone were surgically treated and reviewed in 70 patients between 1983 to 1993 (eleven years) with a median follow-up of six and a half years. Surgery consisted of intralesional curettage in 93 and marginal en bloc resection in 25 lesions. Bony defects were reconstructed with autogenous iliac crest graft in 55 lesions, with autogenous fibula graft in 9, with homologous bone chips in 28, and 5 times with a homologous fibula graft from the bone bank. In 33 lesions the entire defect was filled with polymethylmethacrylate. Osteosynthesis was performed in 41 patients. RESULTS: Recurrences requiring surgical revision were observed in 26 of 74 primary lesions (= 35% overall recurrence rate) at a mean 123.6 weeks postoperatively. The most frequent primary and recurrence location was the proximal femur (85% revision rate). 69% of all recurrences occurred under the age of 20. After intralesional curettage the reoperation rate was 32% and after marginal resection 8%. After reconstruction with autogenous iliac crest graft recurrence rate was 36%, after autogenous fibula graft 55%, after homologous bone chips 18%, after polymethylmethacrylate 9% and allograft fibula reconstruction showed no recurrences. A combined stable osteosynthesis bridging the fibrous osseous defect significantly reduced the revision rate to 3% (p = 0.01). CONCLUSION: Intralesional curettage and reconstruction with autogenous iliac crest graft in fibrous dysplasia of bone leads to a high recurrence rate. Reconstruction with cortical grafts or bone chips from the bone bank, if necessary in combination with a durable osteosynthesis in mechanically demanding locations, or solely bone cement in mechanically less demanding areas, reduces the revision rate in patients with monoostotic and polyostotic fibrous dysplasia.     

Aneurysmal bone cyst

Summary Authors report on the results of treatment of 52 primary and 16 secondary aneurysmal bone cysts (ABC). ABC grow rapidly; 84% of them have already destroyed more than the half of the bone width at recognition. En bloc resection is preferred when the ABC is growing superficially and eccentrically and more than half of the bone width is intact. Careful curettage and bone grafting still remains the surgical method of choice in the majority of cases, when the ABC is more destructive and affects the subchondral bone of the joints. Segmental resection is only indicated when removal of the affected bone does not influence the function of the extremity. Superselective embolization of the cyst was performed in seven cases with excellent results. This method is suggested for ABC in certain locations inaccessible to surgical intervention, e.g., the pelvis, or to avoid excessive bleeding in hypervascularized tumors. In one case, however, an incomplete rebuilding of the ABC could only be achieved by the administration of calcitonin. The 16 cases of secondary ABC were observed mostly in association with osteoblastomas, giant-cell tumors, and osteosarcomas. The incidence of the secondary ABC was 23% in the whole ABC group but not more than 2–4% among the osteosarcomas and giant-cell tumors. Secondary ABC may confuse the histological and clinical diagnoses and that, especially in cases of osteosarcoma, may have fatal consequences.     

Aneurysmal bone cyst of the hand: a report of four cases.

Aneurysmal bone cysts are benign lesions that rarely occur in the bones of the hand. Curettage and bone grafting are the most common treatment modalities performed considering the possible functional loss after total excision. Four cases of aneurysmal bone cyst of the hand were identified. Three out of four cases were treated initially with curettage and bone grafting alone without any other local therapy. One had total excision of the lesion. There were two recurrences in the curettage group. Both were treated with excision and followed up for a minimum of 12 months. Curettage alone was associated with high recurrence rates. Total excision of the lesion was the most successful procedure employed. In view of the high recurrence rates following curettage alone and non-neoplastic nature of the lesion, adjuvant treatment modalities such as cryosurgery, electric cauterisation should be considered for initial treatment of ABC. Resection should be preserved for recurrent cases.     

Fibromyxoma of bone: a case report and review of the literature

In this study, a case of fibromyxoma of the proximal femur in a 59-year old woman is reported. The classification of this rare bone tumour is still a matter of debate and some investigators have suggested that these lesions represent a degenerative form of fibrous dysplasia. Some authors make a further distinction between fibromyxoma and myxoma of bone. In a review of 23 cases of fibromyxoma and five cases of myxoma, no differences in clinical, radiographic and biologic behaviour between fibromyxoma and myxoma were found. Apart from the age at diagnosis, the most important difference between fibromyxoma and myxoma was the degree of myxoid matrix. Therefore, we suggest that extragnathic myxoma is a regressive variant of extragnathic fibromyxoma and should be termed as the same entity. In contrast to monostotic fibrous dysplasia fibromyxoma / myxoma often causes pain and presents as a Lodwick IC lesion with a soft tissue mass. Therefore, fibromyxoma / myxoma should be distinguished from fibrous dysplasia because of its different clinical and radiographic features.     

Fibrous dysplasia of the clivus with a second T8 bone lesion: case report

BACKGROUND: We present a rare case of a probably poliostotic fibrous dysplasia of the clivus in a young woman with a clival and vertebral involvement. We also compare our case with data reported in tables that include all cases of clival fibrous dysplasia present in literature. CASE DESCRIPTION: Thirty-year-old women presented with headache. We reviewed radiological and clinical literature before surgery. It was possible to perform a preoperative diagnosis of fibrous dysplasia of the clivus so it was possible to adopt a minimally invasive surgical approach on the clivus. The diagnosis of fibrous dysplasia of the clivus was confirmed after the operation. As the patient was asymptomatic, we did not operate on the vertebral lesion which was stable after 1 year. CONCLUSION: We draw the conclusion that it is possible to obtain a correct preoperative diagnosis of fibrous dysplasia based on magnetic resonance imaging and computed tomographic scans; this makes it possible to avoid major surgical operations. The reported case is particularly interesting because apart from the clival fibrous dysplasia, a vertebral involvement of the same type can be assumed; this association has never been reported in literature.     

Modern surgical treatment of primary aneurysmal bone cyst of the spine in children and adolescents

Aneurysmal bone cyst (ABC) is a benign but locally aggressive tumor-like condition. Treating children with spinal involvement poses special hazards due to the proximity of the lesion to the spinal cord and the need to preserve spinal stability and balance after surgery. Twelve children with ABC treated between 1990 and 2002 at a tertiary pediatric musculoskeletal tumor center using modern surgical techniques and technology were retrospectively reviewed. A surgical technique using a four-step approach of intralesional curettage, high-speed bur, electrocautery, and bone grafting was found to have a significantly reduced rate of recurrence (0/8 cases) compared with traditional intralesional curettage and bone grafting (4/4 cases) (P < 0.002). Short-segment spinal fusion with instrumentation was also done in five cases immediately after excision of the ABC under the same anesthesia and was a nonsignificant marker for reduced rate of recurrence (P < 0.08), likely secondary to wide exposure. Overall, at last follow-up, all 12 patients were alive and well with no evidence of disease and no significant spinal deformity. The four-step approach to treatment of ABC of the spine with appropriate spinal instrumentation and fusion is recommended for successful treatment of this aggressive lesion.