Intracranial Mesenchymal Chondrosarcoma Research Articles

Extra-skeletal intracranial mesenchymal chondrosarcoma: systematic-literature review.

Intracranial mesenchymal chondrosarcoma (IMC) is a rare malignant tumor in pediatric population. IMC can present as extra- or intra-axial lesion in pediatric patients, though the former is commoner causing raised intracranial pressure (ICP). Radiological diagnosis is a challenge in these cases, as is it difficult to differentiate these from other extra-axial neoplasms due to the wide differential diagnosis in pediatric population. We aim to systematically review the literature and present a rare case of extraskeletal intracranial mesenchymal chondrosarcoma treated with safe maximal resection. A systematic review of literature was conducted in accordance with PRISMA guidelines. PubMed and Scopus databases were queried using the search terms, "primary intracranial chondrosarcoma", "extraskeletal mesenchymal chondrosarcoma", "mesenchymal chondrosarcoma" and "pediatric". Presentation, surgical management and outcome of a 15-year-old male with an extraskeletal IMC are also described. The search yielded 25 articles which met the inclusion criteria. These published records consisted of 33 IMC cases with mean age at presentation of 9.81 ± 5.2years (range 2months to 18years). Frontal region was the commonest locations (11, 33.3%). Most common presentation was headache (14, 42.4%). All patients underwent surgical intervention: gross total resection (20, 60.6%), subtotal resection (9, 27.3%) and no extent mentioned (4, 12.1%). No adjuvant therapy was received in 15 patients (45.5%). On latest follow-up, 11 patients (33.3%) are on remission, 5 patients (15.2%) are symptom free, 3 patients (9.1%) had recurrence, 2 patients (6.1%) had metastasis and 9 patients (27.3%) expired. IMC is a rare entity in pediatric population with imaging findings which are non-characteristic leading to its diagnostic challenge. It can masquerade as other extra-axial intracranial neoplasm (meningioma or hemangiopericytoma). Combination of clinico-radiological and pathological examination can help in accurate diagnosis. SafeMaximal resection followed by radiotherapy is the preferred treatment strategy.

Primary Intracranial Extraskeletal Mesenchymal Chondrosarcoma of the Brain in a Pediatric Patient: A Case Report and Review of Literature

AbstractIntracranial extraskeletal mesenchymal chondrosarcoma, which is characterized by undifferentiated mesenchymal cells in the presence of occasional pockets of mature hyaline cartilage, is rare in our clinical practice and commonly seen in young adults. In the pediatric population, only a few cases have been reported. In this article, we describe a case of primary recurrent intracranial mesenchymal chondrosarcoma in an 11-year-old boy well treated by surgery, radiation, and chemotherapy. We also reviewed all previously published reports on pediatric patients on the basis of their manifestations and management.

Hypofractionated Gamma Knife Stereotactic Radiosurgery as a Tool for Intracranial Mesenchymal Chondrosarcoma Local Treatment: Case Report and Literature Review

Mesenchymal chondrosarcoma is a rare, aggressive cartilaginous forming tumor that is rarely encountered in neurosurgical practice...

Intracranial extraskeletal mesenchymal chondrosarcoma: A case report

Background: Mesenchymal chondrosarcoma (MCS) is a rare and aggressive malignant tumor, a subtype of chondrosarcoma assumed to arise from remnants of the embryonic cartilage or metaplasia of meningeal fibroblasts. Intracranial extraskeletal MCSs are rare tumors, accounting for <0.16% of intracranial tumors. They usually occur in the age group of 20–30 years and are commonly found in the frontoparietal region arising from the falx and surrounding dura. Case Description: A 17-year-old girl presented with weakness of the right lower limb. Magnetic resonance imaging showed a large lobulated extra-axial mass lesion in the left parasagittal frontal region. She underwent a left frontoparietal craniotomy and excision of the tumor. She received adjuvant radiotherapy 60Gy/30 fractions. Conclusion: Intracranial MCSs are rare malignant tumors with a poor prognosis. The prognosis depends upon surgical resection and postoperative radiotherapy.

The Differences Between Intracranial Mesenchymal Chondrosarcoma and Conventional Chondrosarcoma in Clinical Features and Outcomes

To report differences in clinical features and outcomes between intracranial mesenchymal chondrosarcoma (MCS) and conventional chondrosarcoma (CCS). Clinical data of patients with primary intracranial MCS and CCS were retrospectively extracted and analyzed to compare differences between MCS and CCS. Seventy-four patients with intracranial chondrosarcoma (61 cases with MCS and 13 cases with CCS) were included. Compared with patients with CCS, patients with MCS presented at a younger mean age (21.1 years vs. 34.5 years, P < 0.001) and had a poor mean preoperative Karnofsky performance scale score (64.6 vs. 77.8, P= 0.014). Compared with CCS, MCS was less often located in the skull base (38.5% vs. 96.7%, P < 0.001) and had a larger tumor volume (87.8 cm3 vs. 26.7 cm3, P < 0.001). Rates of gross total resection in MCS and CCS subgroups were 41.1% (n= 25) and 46.2% (n= 6), respectively; rates of adjuvant radiotherapy postoperatively were 44.2% (n= 27) and 46.2% (n= 6), respectively. After mean follow-up of 41.7 months, 1-year, 3-year, and 5-year overall survival and progression-free survival of MCS were significantly shorter than overall survival and progression-free survival of CCS. Multivariate analysis revealed that tumor pathology and extent of surgery were independent predictors for tumor recurrence. Clinical features of MCS are quite different from CCS. Treatment strategies used for CCS do not yield satisfactory outcomes for MCS. Treatment of MCS should be aggressive and individualized.

Intracranial Mesenchymal Chondrosarcoma: Report of 16 Cases

Limited data regarding intracranial mesenchymal chondrosarcoma (MCS) are available. The goal of this study was to report the clinical characteristics, challenges in management, and poor outcomes of intracranial MCS. Clinical data for 16 patients with MCS were reviewed retrospectively to evaluate their clinical characteristics, management, and outcomes. This study included 11 male and 5 female patients with a mean age of 22.9 ± 14.4 years. The most common presentations were headache (n= 10; 62.5%), followed by cranial deficits (n= 7; 43.6%). The radiologic spectrum for MCS was broad, and only 18.8% (3/16) of MCSs were correctly diagnosed preoperatively. Aggressive resection (including subtotal resection and gross total resection) and partial resection was performed in 62.5% (10/16) and 37.50% (6/16) of patients. With a median follow-up of 34 months (range, 10-78 months), 5 patients (31.3%) died and 8 patients (50%) developed tumor recurrence. The 1-, 3-, and 5-year rates of progression-free survival and overall survival were 86%, 53%, and 42% and 93%, 70%, and 56%, respectively. Although the differences were not significantly different, aggressive resection and the use of radiotherapy tended to improve the prognosis of the patients. Clinical characteristics of MCS are variable. The current management of intracranial MCS referring to conventional chondrosarcoma could not yield satisfactory outcomes. Further study is needed to identify the optimal treatments.

Primary Intracranial Mesenchymal Chondrosarcoma: A Case Report

Mesenchymal chondrosarcoma is a rare malignancy characterized by a biphasic pattern of small undifferentiated round cells intermixed with island of well-differentiated cartilaginous matrix. Although this type of tumor exhibits widespread distribution in the body, intracranial mesenchymal chondrosarcoma is rare. Radical excision of intracranial mesenchymal chondrosarcoma is important for obtaining a positive prognosis but the effectiveness of chemotherapy and radiotherapy remains uncertain. An 18-year-old patient presented with generalized seizure with magnetic resonance imaging showing a large dura-attached solid mass in the frontal convexity, which was presumptively diagnosed as a meningioma. Gross total resection of tumor was achieved without postoperative deficit. The final histological diagnosis was extra-skeletal mesenchymal chondrosarcoma, grade I. There was no adjuvant treatment after surgery. Five years after surgery, the prognosis was good after gross total resection only; however, long-term follow-up is necessary in the future. J Med Cases. 2018;9(5):135-138 doi: https://doi.org/10.14740/jmc3040w

Intracranial Mesenchymal Chondrosarcoma: Case Report and Review of Literature

Background: Chondrosarcoma is a very rare brain tumor. Patients with mesenchymal chondrosarcoma have a worse prognosis and survival than other subtypes of brain chondrosarcoma. Case Report: A 29-year-old man underwent craniotomy because of a headache and blurred vision in his left eye lasting for 15 days. Pathologic examination revealed mesenchymal chondrosarcoma. Conclusion: Chondrosarcoma should be considered in the differential diagnoses of intracranial extra-axial hypo- and hypervascular tumors, especially in young patients. Surgical resection and radiotherapy are the preferred treatment for these patients.[GMJ.2016;5(4):219-24]

Intracranial Mesenchymal Chondrosarcoma: A Case Report

Primary intracranial mesenchymal chondrosarcoma is an extremely rare tumor. We present a case of primary intracranial mesenchymal chondrosarcoma in a young female who presented with headache, loss of vision, and one episode of convulsions associated with nausea and vomiting. MRI brain revealed a large, well-marginated, lobulated, partially calcified, intensely enhancing lesion with a broad dural base in bilateral frontal regions with marked mass effect suggestive of meningioma. Bilateral frontal craniotomy with gross tumor excision was performed. Histopathology revealed mesenchymal chondrosarcoma. Adjuvant radiotherapy was delivered and patient is currently on follow-up. We emphasize that intracranial mesenchymal chondrosarcoma is a rare neoplasm and should be considered in the differential diagnoses of intracranial mass along with meningioma. Treatment of choice is surgery followed by adjuvant radiotherapy. We review the relevant literature and discuss the characteristics of this rare tumor.

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.

Giant intracranial mesenchymal chondrosarcoma with uncal herniation

Summary Mesenchymal chondrosarcomas are very rare central nervous system (CNS) tumors consisting of undifferentiated mesenchymal cells plus islets of cartilage. We report a case of giant intracranial mesenchymal chondrosarcomas presenting with acute neurologic deterioration. A 22-year-old woman presented with right facial and hand numbness and ocular torsion for about 2 weeks. Magnetic resonance imaging (MRI) disclosed a mass in the right middle cranial fossa with a mass effect. Two days before scheduled surgery, the patient suddenly lost consciousness and was found to have uncal herniation. At emergency surgery, the tumor was totally excised. Pathologically, there was a dimorphic pattern of undifferentiated cells and islets of cartilage, consistent with mesenchymal chondrosarcoma. Although the patient survived surgery, she remained in a vegetative state. The tumor recurred 3 years after surgery. Intracranial mesenchymal chondrosarcoma is rare and should be considered when evaluating a tumor with dural involvement, particularly in younger adults. Differential diagnoses include intracranial meningioma and schwannoma. An aggressive surgical resection is highly recommended because of the high risk of recurrence.

Intracranial mesenchymal chondrosarcoma: Report of two pediatric cases

Intracranial mesenchymal chondrosarcoma: Report of two pediatric cases

Congenital Intracranial Mesenchymal Chondrosarcoma: Case Report and Review of the Literature in Pediatric Patients

In this paper we report the 1st case of a congenital intracranial mesenchymal chondrosarcoma in a 2-month-old infant, apparently present at birth. A magnetic resonance image showed a large left parietal solid mass, while microscopy revealed a mixture of undifferentiated small cells and mature hyaline cartilage islands, positive for vimentin, S-100, and CD99. A surgical excision was performed but the patient died after a few weeks as a result of a rapid relapse of the tumor. We also review the pediatric cases (in patients less than 20 years old) of extraskeletal (intracranial) mesenchymal chondrosarcomas of the literature, with a focus on the most recent cytogenetic and immunohistochemical studies.

Clinicopathologic Features of Two Rare Cases of Mesenchymal Metastatic Tumors in the Pancreas

A clinicopathologic presentation of 2 unusual cases of metastatic mesenchymal neoplasms in the pancreas. The first case concerns a 26-year-old man with a history of intracranial mesenchymal chondrosarcoma (since the age of 17), 2 left lung operations, and 3 right thigh operations. Distal pancreatectomy and splenectomy was performed because of suspicious mass in the pancreas. The second case concerns a 66-year-old woman with a history of uterus leiomyosarcoma (10 years ago) with left axillary and right femoral metastases. She underwent distal pancreatectomy and splenectomy because of suspicious mass measuring 4 x 4 cm, in the pancreatic body. In the first case, the pathological examination revealed a tumor measuring 3.8 x 3.5 cm and histologically compatible with mesenchymal chondrosarcoma, developing in a vessel lumen and invading into the pancreatic parenchyma. In the second case, the pathological examination showed metastatic leiomyosarcoma of high-grade malignancy. The incidence of metastatic pancreatic tumors has been reported to be only 1.6% to 3%. Most of these tumors were of epithelial origin, and the most common sites of the primary lesions were the lung, kidney, and gastrointestinal tract. The cases of metastatic uterus leiomyosarcoma and the metastatic intracranial chondrosarcoma are, to our knowledge, the first to be described.

Successful treatment of a chemoresistant tumor with temozolomide in an adult patient: report of a recurrent intracranial mesenchymal chondrosarcoma

Successful treatment of a chemoresistant tumor with temozolomide in an adult patient: report of a recurrent intracranial mesenchymal chondrosarcoma

Extraskeletal Intracranial Mesenchymal Chondrosarcoma: Case Report and Literature Review

We present the case of a 13-year-old girl with a huge intracranial mesenchymal chondrosarcoma. She had suffered from severe headache, diplopia, intermittent nausea and vomiting for 1 month. Neurologic examination revealed bilateral blurred optic disc margins and abducens paresis. Magnetic resonance imaging demonstrated a giant, heterogeneous, intensely enhancing mass of 7 x 8 x 6 cm, occupying the bilateral frontal and left high parietal regions and based on the anterior cerebral falx. A presumptive diagnosis of aggressive meningioma was made. The patient underwent bicoronal craniotomy and gross total resection of the tumor. Pathologic examination revealed an extraskeletal mesenchymal chondrosarcoma. She was under regular follow-up and remained free of recurrence after surgery. In addition to the current case, we review previously reported cases of extraskeletal intracranial mesenchymal chondrosarcoma and discuss treatment strategies and outcomes.

Primary intracerebral mesenchymal chondrosarcoma with rhabdomyosarcomatous differentiation: case report and literature review

Intracranial chondrosarcoma (Ch-S) is a slow-growing, locally recurrent, malignant cartilaginous tumour of the skull base. Intracranial mesenchymal chondrosarcoma (MsCh-S) is a rarer, more malignant variant associated with the supratentorial meninges. Only seven cases of Ch-S, and six of MsCh-S, that were primarily intraparenchymal in origin have been reported. Moreover, no case of intracranial Ch-S or MsCh-S has been reported in which rhabdomyosarcomatous differentiation was prominent. A 17-year-old Asian girl presented with a 4-week history of occipital headache, vomiting and paraesthesia in the left hand. She was drowsy with a left hemiparesis and had a dilated right pupil with bilateral papilloedema. CT demonstrated a large, partly calcified, contrast-enhancing mass in the right temporo-parietal region with oedema and midline shift. Through a large craniotomy, a tense brain was encountered with no apparent cortical abnormality. Despite a radical tumour excision, with excellent initial clinical recovery, a local recurrence rapidly occurred within weeks prior to the administration of any radiotherapy. Initial histopathological examination revealed a primary MsCh-S with osseous and rhabdomyosarcomatous differentiation, with an indistinct margin. After a second radical excision, a second recurrence rapidly occurred; however, this proved excessively vascular and inoperable. Radiotherapy was declined and death followed within 3 weeks. This is the seventh case of primary intracerebral MsCh-S to be reported and the first to demonstrate rhabdomyosarcomatous differentiation. It was characterized clinically by rapid, local recurrence with increased vascularity.

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.

Primary Intracranial Mesenchymal Chondrosarcoma

A case of a large intracranial mesenchymal chondrosarcoma with probable distant metastases to the humerus and the lung is presented. The pertinent literature is reviewed with respect to the presentation, treatment, and prognosis of this tumor. Total removal of the tumor is the treatment of choice. The prognosis is poor because of frequent recurrence and metastasis.

Primary Intracranial Mesenchymal Chondrosarcoma

Primary Intracranial Mesenchymal Chondrosarcoma