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Child's Nervous System : ChNS :... May 2024Intracranial mesenchymal chondrosarcoma (IMC) is a rare malignant tumor in pediatric population. IMC can present as extra- or intra-axial lesion in pediatric patients,... (Review)
Review
BACKGROUND
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.
METHODS
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.
RESULTS
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 ± 5.2 years (range 2 months to 18 years). 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.
CONCLUSION
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. Safe Maximal resection followed by radiotherapy is the preferred treatment strategy.
PubMed: 38762839
DOI: 10.1007/s00381-024-06452-2 -
PloS One 2015Mesenchymal chondrosarcoma(MCS) is a rare high-grade variant of chondrosarcoma. Consensus has not been reached on its optimal management. Resection with wide margins is... (Review)
Review
BACKGROUND
Mesenchymal chondrosarcoma(MCS) is a rare high-grade variant of chondrosarcoma. Consensus has not been reached on its optimal management. Resection with wide margins is usually recommended, but the effect of margins has been demonstrated by little positive evidence. Moreover, the effectiveness of adjuvant chemo- and/or radiotherapy remains controversial.
OBJECTIVES
To describe the clinical characteristics and outcomes of MCS of bone and soft tissue, to assess the efficacies of surgery, chemotherapy and radiation, and finally to deliver a more appropriate therapy.
MATERIALS AND METHODS
We reviewed EMBASE-, MEDLINE-, Cochrane-, Ovid- and PubMed-based to find out all cases of MCS of bone and soft tissue described between April 1994 and April 2014. Description of treatment and regular follow-up was required for each study. Language was restricted to English and Chinese. Issues of age, gender, location, metastasis, and treatment were all evaluated for each case. Kaplan-Meier Method and Cox Proportional Hazard Regression Model were used in the survival analysis.
RESULTS
From the 630 identified publications, 18 meeting the inclusion criteria were selected, involving a total of 107 patients. Based on these data, the 5-, 10-and 20-year overall survival are 55.0%, 43.5% and 15.7% respectively. The 5-, 10-, 20- year event-free survival rates are 45.0%, 27.2% and 8.1%, respectively. Treatment without surgery is associated with poorer overall survival and event-free survival. Negative surgical margins could significantly bring down the local-recurrence rate and are associated with a higher event-free survival rate. Chemotherapy regime based on anthracyclines does not benefit the overall survival. The addition of radiation therapy is not significantly associated with the overall or event-free survival. However, we recommend radiation as the salvage therapy for patients with positive margin so as to achieve better local control.
CONCLUSIONS
This review shows that surgery is essential in the management of MCS of bone and soft tissue. Appropriate adjuvant therapy may reduce local recurrence, but cannot benefit the overall survival.
Topics: Bone Neoplasms; Chondrosarcoma, Mesenchymal; Humans; Soft Tissue Neoplasms; Treatment Outcome
PubMed: 25849226
DOI: 10.1371/journal.pone.0122216 -
Brain Tumor Research and Treatment Apr 2023Cerebral chondrosarcoma metastases are rare and aggressive neoplasms. The rarity of presentation has precluded rigorous analysis of diagnosis, risk factors, treatment,...
BACKGROUND
Cerebral chondrosarcoma metastases are rare and aggressive neoplasms. The rarity of presentation has precluded rigorous analysis of diagnosis, risk factors, treatment, and survival. We analyzed every reported case through exhaustive literature review. We further present the first case with Maffucci syndrome.
METHODS
Three databases, PubMed, Embase, and Google Scholar, and crossed references were queried for cerebral chondrosarcoma metastases. Extracted variables included demographics, risk factors, tumor characteristics, interventions, and outcomes. Univariate and multivariate analyses were performed.
RESULTS
Fifty-six patients were included from 1,489 literature results. The average age at brain metastasis was 46.6±17.6 years and occurred at a median of 24±2.8 months from primary diagnosis. Primary tumor histology (dedifferentiated 5.0±1.5 months, mesenchymal 24±3.0 months, conventional 41±7.4 months, <0.05) and grade (low grade 54±16.7 months vs. high-grade 10±6.4 months, <0.001) correlated with time interval until brain metastasis. A multiple enchondromatosis syndrome occurred in 13.2% of cases. At time of brain metastases diagnosis, extracranial metastases were identified in 76.2% of cases. Median survival after the development of brain metastasis was 2.0±0.78 months with a 1-year survival of 10.0%. On regression analysis, surgery reduced brain metastasis mortality risk and radiation trended towards reduced mortality risk (surgery: hazard ratio [HR] 0.22, 95% confidence interval [CI] 0.064-0.763, =0.017; radiation: HR 0.31, 95% CI 0.091-1.072, =0.064).
CONCLUSION
We present a systematic review of cerebral chondrosarcoma metastases. Primary tumor histology and grade correlate with time until cerebral metastasis. Following cerebral metastasis, these tumors have poor prognosis and modestly benefit from surgery.
PubMed: 37151152
DOI: 10.14791/btrt.2023.0003 -
Clinical Neurology and Neurosurgery Mar 2022Primary central nervous system (CNS) sarcomas represent a heterogeneous group of rare neoplasms with unclear etiology. Available data on clinical characteristics,... (Review)
Review
BACKGROUND
Primary central nervous system (CNS) sarcomas represent a heterogeneous group of rare neoplasms with unclear etiology. Available data on clinical characteristics, treatment strategies, and survival are scarce. We comprehensively reviewed management strategies and outcomes of primary CNS sarcomas in adults.
METHODS
PubMed, Scopus, and Cochrane were search following the PRISMA guidelines to include studies on primary CNS sarcomas in adults. Clinical features, management strategies, and survival were analyzed.
RESULTS
We included 9 studies comprising 78 patients. Primary CNS sarcomas were mostly intracranial (87.2%), frequently located in the parietal (17.9%), frontal (14.1%), and temporal (14.1%) lobes. Spinal CNS sarcomas were found in 10 patients (12.8%). The most common tumor histology were fibrosarcoma (16.7%), intracranial synovial sarcoma (12.8%), extraosseous mesenchymal chondrosarcoma (11.5%), perivascular sarcoma (11.5%), reticulum cell sarcoma (11.5%), and myeloid sarcoma (9%). Partial resection (57.7%) was preferred over complete resection (42.3%), and 43 patients (55.1%) received adjuvant treatments: radiotherapy (51.3%) and/or systemic chemotherapy (20.5%). 21 patients experienced CNS sarcomas recurrences, with a median progression-free survival of 9 months (range, 4-48). At last follow-up, 60 patients (76.9%) were dead, with a median overall survival of 9 months (0.1-396). Overall survival was significantly longer in patients with fibrosarcoma (p = 0.001).
CONCLUSION
Surgical resection coupled with adjuvant chemotherapy or radiation has historically been the cornerstone treatment for CNS sarcoma but showed poor local control and dismal survival. A better understanding of the CNS sarcoma microenvironment may favor the development of tailored strategies aimed at improving survival.
Topics: Adult; Central Nervous System; Central Nervous System Neoplasms; Chemotherapy, Adjuvant; Fibrosarcoma; Humans; Retrospective Studies; Sarcoma; Tumor Microenvironment
PubMed: 35151057
DOI: 10.1016/j.clineuro.2022.107127