鞍区原发性间叶组织来源恶性肿瘤2例及文献复习

目的总结鞍区原发性间叶组织来源恶性肿瘤的临床特点。方法回顾性分析2例鞍区原发性肉瘤的临床和病理资料,结合文献,对这一罕见疾病的临床表现、影像学、治疗及预后等进行复习。结果术中所见和术后病理证实2例病人分别为纤维肉瘤和软骨肉瘤,临床特点与侵袭性垂体腺瘤差异较大。结论鞍区原发性肉瘤十分罕见,预后较差。     

A rare tentorial mesenchymal chondrosarcoma in posterior cranial fossa: Case report

Intracranial extraskeletal mesenchymal chondrosarcoma is a very rare malignant tumor with predilection site of frontoparietal falx cerebri. Only few cases of mesenchymal chondrosarcoma in posterior cranial fossa are reported. Here, we report a 23-year-old young man with a dura-attached mass in left posterior cranial fossa misdiagnosed as a tentorial meningioma preoperatively. According to the following operation, the lesion was confirmed as mesenchymal chondrosarcoma surgically and pathologically. On MRI, the tumor was characterized by lobulated soft-tissue mass with dura-attached base, patchy calcifications and heterogeneous signal intensities. On contrast-enhanced MRI, it was well-defined, with marked enhancement. We consider that these imaging features above might remind us to consider the diagnosis of mesenchymal chondrosarcoma in posterior cranial fossa. The postoperative treatment of radiotherapy is still controversial. As for our case, according to the 24 months follow-up after postoperative γ-knife, our patient shows an optimistic prognosis so far.     

Diagnostic pitfall in the diagnosis of mesenchymal chondrosarcoma arising in the central nervous system

Mesenchymal chondrosarcoma is a rare aggressive neoplasm typically affecting the bones of young adults. It may also arise in somatic soft tissue, the CNS and other organs. It has a characteristic biphasic histological pattern composed of highly undifferentiated small round cells and islands of well‐differentiated hyaline cartilage. We report a case of mesenchymal chondrosarcoma arising from the right tentorium cerebelli in a 21‐year‐old woman with symptoms relating to mass effect. Histological examination demonstrated a purely small round cell appearance in a specimen obtained during partial resection at an outside institution, leading to an erroneous diagnosis of Ewing sarcoma/primitive neuroectodermal tumor (PNET). The diagnosis of mesenchymal chondrosarcoma was made only after tissue obtained during a definitive complete macroscopic removal involving the regional tentorium cerebelli, transverse and sigmoid dural venous sinuses which showed a prominent cartilaginous component. We discuss the features of mesenchymal chondrosarcoma arising in the CNS, the important differential diagnoses of small round‐cell tumors within the CNS, and the differentiating features of mesenchymal chondrosarcoma from Ewing sarcoma/PNET, medulloblastoma, hemangiopericytoma, monophasic synovial sarcoma and atypical teratoid/rhabdoid tumour.     

Chondroma of the lumbar spinal canal: a case report]

Chondroma is a benign cartilaginous tumor and fairly rare in the spine. A case of chondroma in the lumbar spine is presented. A male at age 66 was suffered from progressive low back pain associated with hypesthesia in his right leg. Radiographic examination showed an extradural mass in the dorsal part of spinal canal at L 5 level. No osteolytic change was noted by CT scan. MRI showed iso-intensity with marginal enhancement on T 1-weighted images and heterogeneous intensity on T 2-weighted image. The mass was totally removed by laminectomy and pathohistologically diagnosed as chondroma. Postoperative course was uneventful and the symptoms disappeared completely. One of the origin of chondroma is thought to be metaplasia of the connective tissue in contact with spine. This is why no pathological change was found in bone adjacent to the tumor. Careful preoperative diagnosis and total removal of the tumor is important since malignant transformation may happen in chondroma.     

Atypical location of a solitary intracranial chondroma without meningeal attachment

The case of a 19-year-old female patient with a history of severe headache for several months is presented. Computed tomography (CT) as well as magnetic resonance imaging (MRI) revealed an intracranial, space-occupying mass with no meningeal attachment, located in the left frontal lobe. The entire tumour was removed, the pathological examination revealed a chondroma. The origin of this tumour is analysed, the clinical and histological findings are described and the literature is reviewed.     

Intracranial mesenchymal chondrosarcoma: a case report and literature review

Intracranial mesenchymal chondrosarcoma is a rare entity. The case of a 13-year-old girl with mesenchymal chondrosarcoma which seemed to arise from the dura mater is presented here together with a review of the literature. In the present case, the tumor involved the superior sagittal sinus. It recurred 21 months after gross total removal of the mass and coagulation of the involved wall of the superior sagittal sinus. Reoperation was performed followed by radiation therapy.     

Chondroid tumors arising from the meninges--report of 2 cases and review of the literature

Chondroid tumors are rare intracranial tumors usually arising from the base of the skull. We present 2 cases of intracranial cartilaginous tumors with unusual location. In case 1, a 19-year-old woman, a chondroma of the falx cerebri with extensive secondary ossification was diagnosed. In case 2, a 30-year-old woman, a low-grade chondrosarcoma was resected from the right frontal lobe. Both patients showed an uneventful clinical course without evidence of disease 4.5 and 6 years after total extirpation. Our cases show that chondromas and low-grade chondrosarcomas of the dura and meninges usually occur in young adults with a good prognosis after complete extirpation.     

颅内软骨肉瘤的综合治疗(附八例报告)

目的 探讨颅内软骨肉瘤的诊断及显微手术+放射治疗的综合治疗效果.方法 对8例颅内软骨肉瘤患者的临床资料、术后病理及显微手术+放射治疗的结果进行分析总结.结果 高分化软骨肉瘤5例,间叶件软骨肉瘤2例,黏液件软骨肉痛1例.手术全切除5例,次全切除2例,人部分切除1例.随访6个月-3年,肿瘤全切除的5例患者中有2例分别于术后9个月和12个月时复发,行放射治疗,生存良好;1例于术后18个月时复发,末行任何治疗,于术后24个月时死亡;另2例患者术后即行预防性放射治疗,随访至今分别随访28个月、33个月,未见肿瘤确切复发.肿瘤未全切除的3例患者术后早期1个月内即行放射治疗,肿瘤体积未见明确增大,随访至今,2例患者生存良好,1例患者失访.结论 颅内软骨肉瘤虽属颅内恶性肿瘤,但经过积极的显微外科手术切除+放射治疗的综合治疗,患者可获得较良好的预后.     

Microsurgical treatment of intracranial chondroma

Intracranial chondromas are rare benign tumors. Little data are available on the diagnosis and treatment of these tumors. Thus, we aimed to describe a large series of patients with intracranial chondroma and to analyze and discuss the clinical features and microsurgical treatment of these patients. We retrospectively analyzed the clinical data of 30 patients (12 males and 18 females; mean age 35.4 years; age range 16-60 years) who had pathologically confirmed intracranial chondroma treated at our hospital from September 1996 to June 2008. Surgery was performed on all 30 patients: five patients underwent postoperative radiotherapy; 26 patients were followed up postoperatively for a mean duration of 45.8 months. The surgical approach was selected according to tumor location. Total resection was achieved in 11 patients, subtotal resection in 13, and partial resection in nine (three patients had recurrent chondroma). Follow-up showed that 21 patients recovered without recurrences, three had recurrence, and two patients died. The clinical manifestations included headache and multiple cranial nerve lesions. Imaging usually showed a well-demarcated extramedullary tumor, centrally located, without surrounding brain edema, partially calcified (73.3%) and with minimal vascularity, often accompanied by erosion and destruction of surrounding bone (56.7%). It is difficult to totally remove an intracranial chondroma, and it is not possible to differentiate a chondroma from a myxoma or chordoma at the cranial base on the basis of clinical manifestations and neuroradiological findings. Selection of the appropriate surgical approach is important for resection of the tumor.     

Intracranial mesenchymal chondrosarcoma with osteoid formation: report of a pediatric case

CASE REPORT: We present a case of a 14-year-old girl with a 3-week history of severe progressive headache and intermittent vomiting. Magnetic resonance imaging (MRI) revealed a large intensely enhancing mass, which seemed to arise from the right side of the cerebral fossa with implant base in the inferior face of the tentorium and significant mass effect on the nearby structures. A presumptive preoperative diagnosis of meningioma was made. Subtotal surgical resection was performed using the occipital approach. Histologically the neoplasms had the classic features of a mesenchymal chondrosarcoma associated with the focal presence of osteoid matrix. DISCUSSION: Clinical features, therapeutic approaches and prognosis of this rare tumour are discussed with regard to the known 30 cases in the literature.     

A 71-year-old woman with an intracranial dural-based mesenchymal chondrosarcoma

Intracranial mesenchymal chondrosarcoma is a rare, high-grade malignancy with the highest prevalence in young adults. Because of its rarity, most data regarding survival are limited to case studies and small series. We present a 71-year-old woman with an intracranial dural based mesenchymal chondrosarcoma located in the anterior skull base, to our knowledge the oldest patient reported with this tumor.     

Infantile gliosarcoma: a case and a review of the literature

INTRODUCTION: Gliosarcoma in infant is a very rare entity. CASE REPORT: The authors present a case of gliosarcoma in a 23-month-old boy. The patient was admitted to our hospital with persistent headache and frequent vomiting. MR imaging demonstrated a large frontal well-circumscribed lesion with a heterogeneous gadolinium enhancement. Although macroscopically the tumor was totally extirpated, the boy died of rapid tumor regrowth 2 months after surgery. The surgical specimens obtained from the tumor showed an admixture of two distinctive neoplastic tissues. One was a malignant mesenchymal feature. Its fibrosarcomatous nature was characterized by spindle-shaped cells with fine fibers that were deeply stained in silver preparations for reticulin. The other was gliomatous tissue forming islands surrounded by the sarcomatous tissues. Its glioblastomatous nature was obvious, as it was characterized by endothelial proliferation and perinecrotic pseudopalisading. Both tissues were histologically malignant, as evidenced by mitotic figure, high cellularity, atypical features, and variability. DISCUSSION: The literature concerning gliosarcomatous tumors is reviewed, and pathological and clinical features of the tumor are briefly discussed.     

原发性颅底软骨肉瘤

原发于颅底的软骨肉瘤罕见，其临床诊断较困难，与颅底脊索瘤，脑膜瘤等其他颅底肿瘤不易区分。原发性颅底软骨肉瘤主要分为普通型和间叶型，两型的临床特点不同，冶疗预后亦不同。     

Solitary intradural fibrochondroma in a 16-year-old boy

The following case report is that of a 16-year-old boy who had a solitary benign intracranial parietal fibrochondroma. He presented with a 2 month history of headache. A cranial CT demonstrated an isodense, non-contrast enhancing, extra-axial lesion with decreased central opacity and flecks of hyperdensity consistent with calcification. MRI revealed a heterogeneous well circumscribed mass which had an increased signal on T2-weighted images; there was no enhancement with gadolinium. This lesion was totally removed and had histopathological features of a benign chondroma with a fibrocartilaginous character. The literature of this rare turnout is summarized.     

Gliomyosarcoma: an immunohistochemical analysis.

Tumours of mixed glial and sarcomatous elements occurring in intracranial neoplasms are well recognised and have been termed gliosarcomas. These tumours account for up to 8% of all glioblastomas. The sarcomatous elements are thought to derive from the neoplastic transformation of mesenchymal cells in or adjacent to the tumour. This transformation usually has the appearance of a fibrosarcoma or angiosarcoma. Alternative mesenchymal neoplastic differentiation may occur, however, giving rise to the appearances of chondrosarcoma and osteosarcoma. In 1969 Goldman described a case in which the sarcomatous elements of a mixed gliosarcoma appeared, on the basis of light microscopy alone, to differentiate towards skeletal muscle having the features of a rhabdomyosarcoma. He coined the term gliomyosarcoma. In 1986 Barnard et al reported a second case and demonstrated the features of rhabdomyosarcoma using the electron microscope. A further case characterised with both light microscopic and immunohistochemical techniques is reported.     

Intracranial extraskeletal mesenchymal chondrosarcoma

Intracranial extraskeletal mesenchymal chondrosarcoma is a rare malignant variant of chondrosarcomas. We present a 5-year-old Saudi male child who was brought to the Emergency Department with the complaints of headache, irritability, vomiting, and unsteadiness of gait with right hemiparesis. Radiological studies confirmed the presence of a space-occupying lesion in the left cerebral hemisphere of the brain. Through a left temporoparietal craniotomy a total macroscopic excision of the tumor was carried out, and the tumor was found attached to the dura at the base of the temporal fossa. The tumor was well circumscribed, extra axial in location, and was easily dissected from the other part of the brain. The histopathological examination revealed the tumor to be a mesenchymal chondrosarcoma. This rare tumor of the pediatric age group should be included in the differential diagnosis of all intracranial tumors with aggressive characteristics.     

Glomus Tumor in the Femoral Nerve

The glomus tumor of the peripheral nerve is one of the mesenchymal tumors originating in the epineurium, and is extremely rare. A 56-year-old man presented complaining of lancinating pain on the left thigh, which was provoked by pressure or exercise. Subsequent image study revealed a mass in the femoral nerve. Total surgical excision with the aid of intraoperative ultrasonography was performed and the pain was successfully controlled. The authors report an unusual case of a patient diagnosed with glomus tumor in peripheral nerve, with a review of the clinical features, imaging, and pathological findings.     

Mesenchymal chondrosarcoma of the craniocervical junction

Mesenchymal chondrosarcomas are found with equal low frequency in bone and soft tissue. The CNS is the most commonly reported site of extraosseous origin of mesenchymal chondrosarcomas. This report concerns the CT, MRI, and pathologic findings as well as the operative management of a mesenchymal chondrosarcoma of the craniocervical junction.     

Primary falcine chondrosarcoma

Non-skull-base intracranial chondrosarcomas are extremely rare. We present a patient with a classic falcine chondrosarcoma and review the radiological features that may aid in a pre-operative diagnosis, as well as the adjuvant treatment options. A 32-year-old woman presented with a 5-year history of progressive weakness in her right leg. MRI scan demonstrated a 4.9 x 4.3 x 2.7 cm irregular parasaggital mass in the left frontoparietal region that was in contact with the falx. A left fronto-parietal craniotomy was performed and gross total excision was achieved. Intracranial chondrosarcomas that occur above the skull base tend to be of the mesenchymal variety. Classic chondrosarcomas of the falx are rare. The management of these tumours is surgery, with adjuvant radiotherapy for incompletely excised lesions.     

Intracranial chondrosarcoma: review of the literature and report of 15 cases

The available data in the literature (177 cases), two current clinical patients, and cases which occurred in The Netherlands (13) were reviewed concerning the clinical presentation, pathological features, radiological data, and treatment options of chondrosarcoma of the cranial base.
The mean age of patients was 37 years, the male/female ratio 1: 1.1. The most frequent complaints were diplopia with oculomotor disorders (51%), headache (31%), and decreased hearing, dizziness, and tinnitus with statoacusticus dysfunction (21%). The mean duration of symptoms before diagnosis was 27months.
The chondrosarcomas were located in the petrosal bone in 37% (47 cases), in the occipital bone and clivus in 23% (30 cases), in the sphenoïd bone in 20% (25 cases) and to a lesser extent in frontal, ethmoïdal, and parietal bones (14%). In 6% (eight cases) the primary location was in dural tissue. Radiological examinations showed bone destruction and variable calcification (CT), involvement of neuronal and vascular structures (MRI), and mostly hypovascularity on angiography. On histological examination 51% of tumours were classified as grade I, 11% grade II, 30% mesenchymal, and 8% myxoïd. The mesenchymal type was the most malignant as illustrated by a strong tendency to intradural and cerebral growth and possibly occurrence in younger age groups. The treatment of choice until recently was surgery because of the critical location and local aggressive nature. Regrowth of tumour after surgery occurred in 53% of the patients (average after 32 months). Charged particle irradiation gave a five year survival of 83-94% and a local control rate of 78%-91%. Both in surgery and radiotherapy there is treatment related morbidity and mortality that should be considered when offering these therapies.
Recent promising results imply that charged particle radiotherapy, in combination with surgery, may be the therapeutical choice of the future.