Chondromyxoid fibroma of the seventh cervical vertebra

Chondromyxoid fibroma is a rare benign bone tumor representing less than 0.5% of all bone tumors. It commonly involves the long tubular bones. Involvement of the spine is rare. A 35-year-old man presented with history of neck pain, restriction of neck movements, pain and numbness along the medial aspect of the left forearm and weakness with wasting of the left hand. A presumptive diagnosis of a bony tumor such as an aneurysmal bone cyst or a giant cell tumor involving the seventh vertebral body was made on plain X-rays, MRI and bone scan. He underwent C7 central corpectomy, incomplete intralesional curettage with iliac bone grafting and C6 to T1 interspinous wiring. The histological diagnosis was chondromyxoid fibroma. On eight years' follow-up, CT scan showed no progression of the tumor with good alignment and fusion of the graft at the site of the corpectomy. The authors conclude that corpectomy and iliac bone grafting for chondromyxoid fibroma has a good outcome on long-term follow-up.     

Treatment strategy for chondromyxoid fibroma of the sacrum

We present a 59-year-old woman with a rare sacral chondromyxoid fibroma discovered incidentally and treated with curettage, bone grafting, and lumbopelvic fixation. At 1 year following surgery she remains symptom-free, has successfully fused her lumbopelvic construct and has no signs of tumor progression. The known literature contains only six previous reports of sacral chondromyxoid fibroma and discussion is presented on the diagnosis and treatment of this rare lesion.     

Intravascular Papillary Endothelial Hyperplasia (Masson tumor) of the Skull : Case Report and Literature Review

A 10-year-old female patient presented with a rapidly growing nodular mass lesion on her right frontal area. On skull radiography and computed tomography (CT) imaging, this mass had a well-demarcated punch-out lesion with a transdiploic, exophytic soft tissue mass nodule on the frontal scalp. Magnetic resonance (MR) imaging revealed the presence of a 1.5×1.2×1 cm sized calvarial lesion. This lesion was hypointense on T1 and heterogenous hyperintense on T2 weighted MR images, and exhibited heterogeneous enhancement of the soft tissue filling the punch-out lesion after intravenous administration of gadolinium. En block removal of the tumor with resection of the rim of the normal bone was performed. The pathological diagnosis was intravascular papillary endothelial hyperplasia (IPEH). After surgery, no recurrence was found for 8 months. IPEH is a rare and benign reactive lesion usually found in thrombosed subcutaneous blood vessels. Involvement of skull bone is rare. In this article, we present a case of IPEH involving the calvarium, in a 10-year-old woman.     

A rare case of chondromyxoid fibroma of the parietal bone

The authors present a very rare case of the chondromyxoid fibroma (CMF) of the parietal bone. This is an uncommon chondroid tumour which constitutes less than 1% of primary bone tumours and which is usually localised in the metaphysis of the long bones, often in the knee region. There are 23 cases of cranial localisation of the chondromyxoid fibroma reported in several papers, 14 cases involved the cranial base and 9 the calvaria. A different ossification process of the skull-base and calvaria is probably responsible for this distribution. In our case we found the focal, lytic lesion in the parietal bone and fibro-greasy tumour mass. The dura was spared. The tumour was removed totally. Curettage of these kind of lesions should be avoided because it may lead to tumour recurrence. Histologically this tumour may cause problems in a differential diagnosis with other chondroid tumours like chondrosarcoma or chondroblastoma.     

Chondromyxoid fibroma of the anterior skull base invading the orbit in a pediatric patient: case report and review of the literature

Chondromyxoid fibroma (CMF) is a rare bone tumor of cartilaginous derivation representing less than 1% of all benign bone neoplasms. It is a slow-growing, locally destructive tumor that usually affects the metaphyseal region of long bones of patients in their second or third decade of life. Involvement of the skull base and orbit is extremely rare. We present the first case of histopathology-proven CMF originating in the greater sphenoid wing and extending into the orbit in a 14-year-old boy. He complained of pain and swelling of his left lateral orbit for 4 months and had 3-mm nonaxial proptosis. Magnetic resonance imaging of the orbit revealed a well-defined lesion in the lateral wall of the left orbit, hypointense on T1-weighted images, and heterogenous hyperintense on T2-weighted images and enhanced after contrast media injection. Through a left semicoronal skin incision, the mass was removed totally and the lateral orbital wall was reconstructed. The clinical manifestations, imaging findings, and surgical treatment of the lesion are discussed with a review of the literature.     

Chondromyxoid fibroma in the sella turcica region

Chondromyxoid fibroma (CMF) is an uncommon tumor that primarily develops in the long bones of young males. It is rarely seen in the skull and involvement of the skull base is rare. We report a patient with CMF arising in the region of the sella turcica. A literature review regarding the clinical and histological features of CMF, as well as recommended modalities of treatment, is presented. A 55-year-old male was admitted with polyuria and headache. A CT scan showed a well-defined expansive lesion with a sclerotic margin measuring approximately 2 cm in diameter in the sellar region. T1-weighted MRI revealed a well-circumscribed, lobulated and strongly enhancing lesion. On the T2-weighted MRI, the lesion showed high heterogeneous signal intensity. Using the trans sphenoidal approach, surgical exploration revealed a well-defined tumor underneath the optic chiasm. The piecemeal tumor removal was considered complete. We conclude that sellar region CMF can cause severe disabilities due to tumor compression. CMF should be taken into consideration in the differential diagnosis of a solitary tumor mass in the sellar area. As much as possible, surgical resection of the tumor is the cornerstone of treatment. Although CMF are generally regarded as benign neoplasms, they may show an infiltrative pattern and may recur; particularly when they are in locations where complete surgical excision may be difficult or impossible.     

Benign osteoblastoma of the occipital bone: Case report and literature review

We present a case of benign osteoblastoma of the occipital bone. Benign osteoblastoma is an uncommon primary bone tumor, which usually involves the vertebrae and the long bones. This tumor rarely develops in the calvaria, showing a preference for the temporal and frontal bones when it does. To the best of our knowledge, this case is only the eighth reported case of benign osteoblastoma confined to the occipital bone. A 20‐year‐old male presented with a mild tender mass lesion of the occipital area, just below the lambda. Plain X‐ray films and CT scans demonstrated an osteolytic mass surrounded by the sclerotic rim within the diploic space. MRI proved to be effective for the evaluation of the intracranial and intraosseous extensions of the tumor. However, it was very difficult to formulate a differential diagnosis against other osteoblastic tumors, or osteoid osteoma, in view of its radiological appearance. The final diagnosis was obtained by careful consideration of the histopathological characteristics of the tumor combined with its clinical and radiological features. Although generally regarded as benign, a complete resection is preferred over conventional curettage as this can guard against possible recurrence and malignant transformation.     

颅骨硬纤维瘤一例报告并文献复习

目的探讨颅骨硬纤维瘤(desmoplastic fibroma,DF)的临床、病理、放射学特征及治疗方法。方法报告1例颅骨硬纤维瘤病例,结合文献探讨该疾病的临床特点、放射学特征、病理表现、治疗及预后。结果颅骨硬纤维瘤在临床上极为罕见。X线和CT表现为溶骨性破坏,形成均匀一致或泡沫状的软组织包块,MRI T1加权像表现为中等信号或低信号,有明显增强效应,T2加权表现为低信号,局部区域为高信号。病理学特点:胶原纤维构成病灶的主要成分,其中分布有长梭形或圆胖形的纤维细胞,无异形性,没有有丝分裂像,可与其他恶性肿瘤相鉴别。本病有复发和转移倾向,手术应彻底,可配合放射治疗和内分泌治疗。结论颅骨硬纤维瘤是一种局部侵袭性较强的病变,应结合CT、MRI和病理学特点诊断本病,最好的治疗措施为手术全切,根据激素受体水平考虑内分泌治疗,可减少复发。     

Chondromyxoid fibroma of the parietal bone

We report the case of an 11‐year old girl who presented with an intracranial chondromyxoid fibroma arising from parietal bone. Chondromyxoid fibroma is a benign cartilaginous neoplasm, which can be easily mistaken for chondrosarcoma, especially in small biopsies. It is more common in long bones and is least common in cranial bones. Published work describes about 33 cases occurring in cranial bones with almost equal incidence in the bones of the base of the skull and cranial vault. To our knowledge, this is the second case of chondromyxoid fibroma reported to occur in parietal bone and is the first case reported in an English‐language journal. We present this case for its rare anatomical, clinical, radiological and histological presentations.     

Desmoplastic fibroma of the frontal bone

Desmoplastic fibroma is a benign but locally aggressive tumor arising usually from the mandible, pelvis and long bones with a potential for recurrence. We report a case of desmoplastic fibroma of the frontal bone in a young male.     

室管膜下巨细胞型星形细胞瘤六例分析

目的 探讨室管膜下巨细胞型星形细胞瘤(SEGA)患者的临床、影像学、病理学特点以及诊断和治疗方法.方法 对自2000年2月至2007年8月收治的6例SEGA患者的临床和影像学表现、病理学特点、治疗及随访结果进行回顾分析.结果 6例SEGA的临床表现以高颅压和视力下降最为常见.CT显示肿瘤实体部分呈等或稍高密度,边界清楚,部分可见瘤内钙化.MRI扫描肿瘤实体部分T1WI多呈等信号,少数可呈稍低信号;T2WI呈等或稍高信号:增强扫描肿瘤实体部分呈不均匀强化.4例经胼胝体入路,2例经额叶皮质入路,肿瘤全切除均成功,无手术死亡.随访10月～8年,肿瘤无复发.结论 室管膜下病变如位于室间孔周围伴有结节性硬化,引起脑积水或呈现逐渐生长趋势者,应考虑SEGA的诊断,尽早手术治疗.SEGA为良性肿瘤,手术全切者预后良好.定期影像学检查对于跟踪室管膜下结节以及监测肿瘤复发是必要的.     

Isolated enchondroma of atlas

Compression at the craniovertebral junction because of tumors is not a very common entity. The commonest tumors present here are neurofibroma and meningioma. Any vertebral tumour can be present at this location. Benign bony tumors are very uncommon at this location and amongst these enchondromas are exceptionally rare. Enchondromas are rare bony tumours of chondrogenic origin. These are benign tumours with a propensity for malignant transformation. There are four histological types: osteochondromas, enchondromas, chondroblastoma and chondromyxoid fibroma. Enchondromas are often asymptomatic because of their slow growth but may have varied presentation. An enchondroma may occur as an individual tumor or as several tumors together. We here report a case of enchondroma arising from the atlas and causing myelopathy. The best treatment is complete excision which we could achieve in our case.     

Co‐existing fibrous dysplasia and atypical lymphoplasmacyte‐rich meningioma

We report an unusual and extremely rare case of coexisting fibrous dysplasia of the sphenoid sinus with atypical lymphoplasmacyte rich meningioma (World Health Organization Grade II), right frontal lobe in a 25‐year‐old male. The patient presented with history of generalized tonic clonic seizures with a loss of vision in the right eye. Radiologically it was seen that the sphenoid sinus lesion was sclerotic and was extending and causing compression of the optic nerve. Magnetic resonance imaging revealed mass lesion in the right frontal lobe, iso to hypointense on T₁ weighted images and hyperintense on T2 weighted images with uniform contrast enhancement. Clinically and radiologically the possibility of fibrous dysplasia of the sphenoid bone and sinus with metastases in the frontal lobe from a different primary was considered. On histopathology the frontal lobe lesion showed a tumor composed of cells with vesicular nuclei, focal nuclear pleomorphism, a mitotic rate of = 4/10 high power field and a single focus of necrosis. The cells were arranged in fascicles and had a prominent lymphoplasmacytic infiltrate. In contrast, the sphenoid sinus lesion was composed of narrow, curved bony trabeculae with a characteristic fishhook configuration without osteoblastic rimming and interspersed with fibrous tissue of variable cellularity. Although no definite causative factors are described for the coexistence of these tumors, however, a common yet undetermined denominator in the origin has been proposed in the literature.     

Intraventricular malignant meningioma: one case report.

We report a case of primary intraventricular malignant meningioma in a 74-year-old man who presented with progressive weakness of the left limbs and dizziness, The preoperative magnetic resonance (MR) imaging of the head initially suggested that a glioblastoma multiforme or a metastatic lesion might be present. But later, the excised tumor was histologically diagnosed as a primary malignant meningioma. So far, only a few cases of intraventricular malignant meningioma have been reported. The MR imaging of this tumor has rarely been described in the literature. In the present case, the mass lesion showed hyperintensity on the T1-weighted image (T1WI) and hypointensity on the T2-weighted image (T2WI) which was not commonly seen in meningioma. The unusual MR imaging of this case may be attributed to the intratumoral hemorrhage. Although intraventricular malignant meningioma is difficult to diagnose based on MR or computed tomographic images alone, the characteristics of the MR images in the present case may offer an option for preoperative differential diagnosis of intraventricular tumors.     

Intraosseous meningioma mimicking osteosarcoma in an adolescent: a case report

Intaosseous meningiomas (IM) are the one of the less frequent benign tumors of the skull. The etiology of IM has not been cleared yet. The frontoparietal and orbital regions are the most common locations for IM. The average age for IM diagnosis is 50.5. A 16-year-old girl with a right frontoparietal mass was referred to our outpatient clinic. Cranial CT revealed a mass lesion which resulted in expansion in the right parietal and posterior frontal bone, having lytic and sclerotic regions inside with accompanying irregular cortex in inner and outer tables of the calvarium. Prediagnosis was osteosarcoma according to the imaging studies and after the performed biopsy and consecutive surgery, the lesion was diagnosed as IM. Though CT with bone windows is often useful, it is not always diagnostic. Biopsy plays an important role in calvarial vault lesions for planning the treatment. In our case, malignant criteria in radiology did not match the benign histology revealed. Radiological preoperative misdiagnosis of meningioma is possible.     

Contrast enhanced fast fluid-attenuated inversion-recovery MR imaging for diagnosing cerebral venous angioma: report of two cases]

It has been reported that contrast-enhanced fluid-attenuated inversion-recovery (FLAIR) sequences were useful for detecting superficial abnormalities, such as meningeal disease, because they do not demonstrate contrast enhancement of cortical vessels with slow flow as do T1-weighted images. We reported the usefulness of contrast-enhanced FLAIR images to differentiate cerebral venous angioma from tumor in two patients. Case 1 was a 71-year-old man developed cortical hemorrhage. Post contrast-enhanced T1-weighted images showed an enhanced lesion around the hematoma, whereas contrast-enhanced FLAIR images showed no enhancement of the lesion, thus he was diagnosed as cortical hemorrhage from cerebral venous angioma. Case 2 was a 72-year-old woman, who was examined MR images because of the jugular foramen neurinoma. There was a T2-high-intensity lesion in the right frontal lobe, and post contrast-enhanced T1-weighted images showed an enhanced lesion in and around the T2-high-intensity lesion. Post-contrast FLAIR images showed no enhancement, and she was diagnosed as cerebral venous angioma. Contrast-enhanced fast FLAIR sequences was useful in differentiation between venous angiomas and tumors. Identification of these lesions was due to the flow-void phenomenon in vessels with slow-flowing blood such as venous angioma, which could not be differentiated from tumors on T1-weighted images.     

Ossifying fibroma of the lumbar spine: case report

Ossifying fibroma of the vertebra is a very rare condition. We report a 16-year-old boy with ossifying fibroma of the third lumbar vertebra. Neurological and laboratory examinations demonstrated normal findings. Low-back pain, in the absence of radicular pain, was the presenting symptom. Plain radiography and computerized tomography scanning revealed an osteolytic lesion. The tumor in the third lumbar vertebra was curetted and filled with bone allograft. The histopathological diagnosis was consistent with ossifying fibroma. There was no postoperative complication. The case is the second to be reported. The clinical and radiological aspects of this rare tumor are discussed with the pertinent literature.     

胚胎发育不良性神经上皮肿瘤(二例报告及文献复习)

目的探讨胚胎发育不良性神经上皮肿瘤的临床特点、诊断及治疗。方法报告2例表现为癫痫的患儿(男女各1例),介绍其临床特征、神经影像学特点、手术治疗及预后。结果2例均为儿童,男女各1例,癫痫发作5～6年,MRI显示额叶长T1长T2信号,无强化,无占位效应,局部颅骨受压变薄,肿瘤近全切除后效果良好。随诊3～6个月,无复发,无癫痫发作。结论胚胎发育不良性神经上皮肿瘤是手术切除肿瘤可治愈的良性病变,术后不需放疗及化疗。     

Destructive Radiologic Development of Intravascular Papillary Endothelial Hyperplasia on Skull Bone

Intravascular papillary endothelial hyperplasia (IPEH) is a rare vascular benign lesion that rarely involves the central nervous system with or without skull invasion. We report a rare case of IPEH on the skull bone, which displayed destructive radiologic development associated with hemorrhage. A 14-year-old male presented with an incidentally detected a small enhancing, left frontal osteolytic lesion. Previously, he underwent operation and received adjuvant chemoradiation therapy for cerebellar medulloblastoma. Follow-up magnetic resonance imaging revealed a left frontal bone lesion, which expanded to an approximately 2 cm-sized well-circumscribed osteolytic lesion associated with hemorrhage for 20 months. Frontal craniectomy and cranioplasty were performed. Destructive change was detected on the inner table and diploic space of the skull. The mass had a cystic feature with hemorrhagic content without dural attachment. Pathologic examination showed the capsule consisted of parallel collagen lamellae representing a vascular wall, vascular lumen, which was pathognomonic for IPEH. Immunohistochemical staining revealed that the capsule was positive for CD34 and factor VIII, which favor the final diagnosis of IPEH. This was the first case of intracalvarial IPEH.     

Multiple intracranial and intraspinal meningiomas successively discovered in the absence of neurofibromatosis: 2 cases

Multiple meningiomas in different neuroaxial compartments are quite rare. We describe the case of a 44-year-old woman who developed three intracranial meningiomas and 8 years later a T3 dorsal meningioma. Histologically, the frontal and dorsal tumors appeared as benign psammomatouss meningiomas. Both tumors were removed successfully. The second patient was a 31-year-old woman who developed right benign fronto-parietal transitional meningioma. She presented local and spheno-orbital recurrences, then a lombo-sacral lesion. The histological picture worsened from benign to malignant with multiple recurrences. Several mechanisms could account for multiple meningiomas. Such meningiomas could arise from a single primary tumor via subarachnoidal spread of a benign or malignant nature. Alternatively, they could be atypical forms of neurofibromatosis type 2 or tumors with a multifocal origin.