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Oral Surgery, Oral Medicine, Oral... Aug 2019Low-grade chondrosarcoma presents with features similar to those of benign lesions, such as chondroma and synovial chondromatosis, increasing the difficulty in reaching...
OBJECTIVES
Low-grade chondrosarcoma presents with features similar to those of benign lesions, such as chondroma and synovial chondromatosis, increasing the difficulty in reaching an accurate diagnosis preoperatively. In this study, we retrospectively reviewed 10 chondrosarcoma cases and evaluated the diagnostic approaches and management modalities.
STUDY DESIGN
Ten cases were included in the present study. We evaluated the clinical features, initial diagnosis, histopathology subtype, immunohistologic markers, final diagnosis, and treatment modalities.
RESULTS
Most of the lesions were found in the mandible. Two cases were followed up for 1 month and 4 years, respectively as benign lesions before malignant changes were detected. With regard to chondrosarcoma histopathology subtypes, 6 cases were identified as conventional chondrosarcoma, whereas 4 cases were diagnosed as mesenchymal chondrosarcoma with aggressive behavior; of these, 3 were associated with local recurrence and metastasis. The immunohistologic markers showed no specificity for chondrosarcoma.
CONCLUSIONS
Distinguishing low-grade chondrosarcoma, particularly in the temporomandibular joint, from benign lesions, such as chondroma or synovial chondromatosis, remains difficult. Currently, the correlation between clinical, radiographic, and histologic features accompanied by close follow-up is extremely important for patients diagnosed with chondrogenic lesions. Postoperative radiotherapy seems to be beneficial in patients with positive surgical margins.
Topics: Bone Neoplasms; Chondromatosis, Synovial; Chondrosarcoma; Humans; Neoplasm Recurrence, Local; Retrospective Studies
PubMed: 31204208
DOI: 10.1016/j.oooo.2019.05.008 -
Journal of Oral and Maxillofacial... Aug 2021Mesenchymal chondrosarcoma is a rare high-grade variant of chondrosarcoma distinguished by its aggressive nature. Molecular studies aid in establishing the diagnosis. We... (Review)
Review
Mesenchymal chondrosarcoma is a rare high-grade variant of chondrosarcoma distinguished by its aggressive nature. Molecular studies aid in establishing the diagnosis. We present a case report of mesenchymal chondrosarcoma in the maxilla of a 39-year-old male patient and a literature review of 42 gnathic cases of mesenchymal chondrosarcoma with a discussion of clinical, imaging, microscopic, immunohistochemical, and molecular features.
Topics: Adult; Aggression; Chondrosarcoma, Mesenchymal; Diagnostic Imaging; Humans; Male; Maxilla
PubMed: 33773967
DOI: 10.1016/j.joms.2021.02.020 -
Clinical Neurology and Neurosurgery Nov 2020Chondrosarcomas are extremely rare, locally invasive, and potentially mortal malignant cartilaginous tumors. In this study, we aimed to evaluate the incidence and...
INTRODUCTION
Chondrosarcomas are extremely rare, locally invasive, and potentially mortal malignant cartilaginous tumors. In this study, we aimed to evaluate the incidence and survival rates and trends of skull base chondrosarcomas (SBC).
METHODS
Data from SBC patients between 1975 and 2017 were extracted from the Surveillance, Epidemiology, and End Results (SEER) database. The age-adjusted incidence rates (AAR) were calculated for the overall cases and based on gender, age, race, and histology. Furthermore, the relative survival rates for one, three, and five years, and the rates stratified to the aforementioned selected variables were computed. Besides, we conducted a joint point regression analysis to calculate the annual percent change (APC) and its associated standard error (SE) for AAR and mortality.
RESULTS
The AAR rate of SBC was 0.019 per 100,000. Higher AAR rates were observed in patients who were in the 65-74-year-age-group, females, Caucasians, and had none mesenchymal subtype. The relative one-year, three-year and five-year-survival rates were 99.58 %, 93.67 %, and 89.10 %, respectively. Lower survival rates were noted in patients who were males, African Americans, and had a mesenchymal subtype. The trend analysis has shown a significant yearly increase (P < 0.001) in AAR of SBC (APC ± SE = 0.0005 %±0.0001), along with a significant yearly decline in mortality rates (APC ± SE= -0.0202 %±0.0029).
CONCLUSION
Despite the increase in AAR over time, there has been a significant decline in mortality rates over time, which might have been due to the advancement of treatment modalities, improvement in diagnostic imaging, and modification in disease grading.
Topics: Adolescent; Adult; Aged; Chondrosarcoma; Female; Humans; Incidence; Male; Middle Aged; Retrospective Studies; Skull Base Neoplasms; Young Adult
PubMed: 32818757
DOI: 10.1016/j.clineuro.2020.106153 -
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 -
World Neurosurgery Jan 2021Among chondrosarcomas arising from bones and soft tissues, mesenchymal chondrosarcoma (MCS), especially primary spinal intradural extramedullary MCS, is extremely rare,...
BACKGROUND
Among chondrosarcomas arising from bones and soft tissues, mesenchymal chondrosarcoma (MCS), especially primary spinal intradural extramedullary MCS, is extremely rare, and only 18 cases have been reported to date. We report an adult case of this rare condition mimicking meningioma.
CASE DESCRIPTION
A 42-year-old woman presented with paraplegia and sensory disturbance associated with right-sided Brown-Séquard syndrome following back pain. Magnetic resonance imaging showed an intradural mass in the right dorsal spinal canal with homogeneous enhancement and dural tail sign at the T8 level. Computed tomography demonstrated a calcified portion in the mass. Following T7-8 laminectomies, an intradural extramedullary tumor was completely removed after detaching the tumor from the dura mater. The histopathologic diagnosis was MCS, and positron emission tomography showed no metastatic lesions at other sites. The patient did not receive adjuvant therapy, and magnetic resonance imaging revealed no evidence of recurrence during 2-year follow-up.
CONCLUSIONS
Primary spinal intradural extramedullary MCS has been reported to have a better prognosis than MCS occurring in other regions. In a case with early complete surgical resection, adjuvant therapy should be considered at the time of recurrence.
Topics: Adult; Chondrosarcoma, Mesenchymal; Female; Humans; Spinal Cord Neoplasms
PubMed: 33049384
DOI: 10.1016/j.wneu.2020.10.007 -
BMC Musculoskeletal Disorders Sep 2019Mesenchymal chondrosarcoma (MCS) is a rare malignant variant of chondrosarcoma with a high tendency of recurrence and metastasis. Intradural extramedullary spinal MCS is... (Review)
Review
BACKGROUND
Mesenchymal chondrosarcoma (MCS) is a rare malignant variant of chondrosarcoma with a high tendency of recurrence and metastasis. Intradural extramedullary spinal MCS is exceedingly rare and usually found in pediatric patients. Herein, we present an elderly patient with primary intradural extramedullary spinal MCS. Relevant literatures are reviewed to disclose characteristics of intradural extramedullary spinal MCS.
CASE PRESENTATION
A 64-year-old female presented with urinary difficulty and tightness of upper back preceding progressive weakness of right lower extremity. Magnetic resonance imaging revealed an intradural extramedullary tumor at the level of 3rd thoracic vertebra. This patient underwent total tumor resection and then received adjuvant radiotherapy. Histopathological examination showed that the tumor composed of spindle and round cells with high nucleocytoplasmic ratio accompanied by scattered eosinophilic chondroid matrix. Along with immunohistochemical findings and the existence of HEY1-NCOA2 fusion transcript, the diagnosis of MCS was confirmed. Neurologic deficit recovered nearly completely after surgery. No evidence of local recurrence or distant metastasis was found 5 years after treatments. Including the current case, a total of 18 cases have been reported in the literature with only one case with local recurrence and one case of mortality. The current case was the eldest patient diagnosed with primary intraspinal MCS in the literature.
CONCLUSIONS
MCS rarely appears in the intradural space of the spine. In contrast to classic MCS, treatment outcome of primary intradural extramedullary spinal MCS is usually excellent as total tumor resection is commonly achievable. Adjuvant radiotherapy may reduce local recurrence and chemotherapy may be associated with fewer recurrences especially for unresectable tumors.
Topics: Basic Helix-Loop-Helix Transcription Factors; Cell Cycle Proteins; Chondrosarcoma, Mesenchymal; Dura Mater; Female; Humans; Laminectomy; Magnetic Resonance Imaging; Middle Aged; Nuclear Receptor Coactivator 2; Radiotherapy, Adjuvant; Spinal Cord Neoplasms; Spinal Fusion; Treatment Outcome
PubMed: 31484514
DOI: 10.1186/s12891-019-2799-2 -
Translational Cancer Research Sep 2022Intracranial extraskeletal mesenchymal chondrosarcoma (EMCS) is a rare neoplasm and often misdiagnosed before histopathological examination due to its rarity. There were...
BACKGROUND
Intracranial extraskeletal mesenchymal chondrosarcoma (EMCS) is a rare neoplasm and often misdiagnosed before histopathological examination due to its rarity. There were few reports previously on the radiological features of intracranial EMCS. We described a 20-year-old male patient with intracranial EMCS focusing on the imaging characteristics.
CASE DESCRIPTION
The patient was admitted to our hospital due to headache and dizziness for two months, without nausea, vomiting, limb convulsions and loss of consciousness during the illness. Pre-contrast computed tomography (CT) revealed a large slightly hyperdense mass with irregularly lobulated margins in the right parietal and occipital region and multiple patchy calcifications in peripheral of the lesion. The inner table of right parietal bone adjacent to the mass was compressed, thickened, and eroded. Magnetic resonance imaging (MRI) exhibited intermediate and hypo-intensity on T1-weighted images (TWI) and slight hyper-intensity on T2-weighted images (TWI) with extremely high intensity rim of cerebral spinal fluid (CSF) and low intensity flow-void vessel. The mass demonstrated heterogeneous remarkable enhancement and "dural tail" sign also was noted. The important imaging signs of this case are irregular calcifications of soft tissue on CT and "dural tail" sign on MRI. The patient underwent tumor resection and was followed up postoperatively with serial MRI every three months. He was alive without obvious clinical symptoms and evidence of recurrence for 9 months. EMCS is a highly invasive tumor and it is difficult to differentiate EMCS from the other intracranial malignant tumors only by clinical characteristics or findings of CT and conventional MR imaging. Radiotherapy and chemotherapy after radical resection are the best treatment choice. Therefore, postoperative patients should be reviewed routinely.
CONCLUSIONS
A knowledge of the imaging features could facilitate differentiation of intracranial EMCS, but the final diagnosis depends on pathological examinations. This paper focuses on the imaging characteristics of EMCS and fully describes the details of lesions in order to provide clinicians with effective differential diagnosis information and improve clinical decision-making.
PubMed: 36237268
DOI: 10.21037/tcr-21-2547 -
Journal of Neuro-oncology Dec 2020Skull base primary malignancies represent a heterogeneous group of histologic diagnoses and sarcomas of the skull base are specific malignant tumors that arise from... (Review)
Review
Skull base primary malignancies represent a heterogeneous group of histologic diagnoses and sarcomas of the skull base are specific malignant tumors that arise from mesenchymal cells and can be classified by site of origin into bony and soft tissue sarcomas. The most common bony sarcomas include: chondrosarcoma, osteosarcoma, chordoma, Ewing's sarcoma. Given the relative rarity of each histologic diagnosis, especially in the skull base, there is limited published data to guide the management of patients with skull base sarcomas. An electronic search of the literature was performed to obtain key publications in the management of bony sarcomas of the skull base published within the last decade. This article is thus a review of the multi-disciplinary management principles of primary bony sarcomas of the skull base. Of note, there have been several recent advancements in the realm of skull base sarcoma management that have resulted in improved survival. These include advances in: imaging and diagnostic techniques, surgical techniques that incorporate oncologic surgical principles, conformal radiation paradigms and targeted systemic therapies. Early access to coordinated multi-disciplinary subspecialty care immediately at suspicion of diagnosis has further improved outcomes. There are several ongoing trials in the realms of radiation therapy and systemic therapy that will hopefully provide further insight about the optimal management of bony sarcomas of the skull base.
Topics: Animals; Bone Neoplasms; Combined Modality Therapy; Disease Management; Humans; Sarcoma; Skull Base Neoplasms
PubMed: 32306199
DOI: 10.1007/s11060-020-03497-6 -
The Journal of Pathology Aug 2022Mesenchymal chondrosarcoma is a rare, high-grade, primitive mesenchymal tumor. It accounts for around 2-10% of all chondrosarcomas and mainly affects adolescents and...
Mesenchymal chondrosarcoma is a rare, high-grade, primitive mesenchymal tumor. It accounts for around 2-10% of all chondrosarcomas and mainly affects adolescents and young adults. We previously described the HEY1-NCOA2 as a recurrent gene fusion in mesenchymal chondrosarcoma, an important breakthrough for characterizing this disease; however, little study had been done to characterize the fusion protein functionally, in large part due to a lack of suitable models for evaluating the impact of HEY1-NCOA2 expression in the appropriate cellular context. We used iPSC-derived mesenchymal stem cells (iPSC-MSCs), which can differentiate into chondrocytes, and generated stable transduced iPSC-MSCs with inducible expression of HEY1-NCOA2 fusion protein, wildtype HEY1 or wildtype NCOA2. We next comprehensively analyzed both the DNA binding properties and transcriptional impact of HEY1-NCOA2 expression by integrating genome-wide chromatin immunoprecipitation sequencing (ChIP-seq) and expression profiling (RNA-seq). We demonstrated that HEY1-NCOA2 fusion protein preferentially binds to promoter regions of canonical HEY1 targets, resulting in transactivation of HEY1 targets, and significantly enhances cell proliferation. Intriguingly, we identified that both PDGFB and PDGFRA were directly targeted and upregulated by HEY1-NCOA2; and the fusion protein, but not wildtype HEY1 or NCOA2, dramatically increased the level of phospho-AKT (Ser473). Our findings provide a rationale for exploring PDGF/PI3K/AKT inhibition in treating mesenchymal chondrosarcoma. © 2022 The Authors. The Journal of Pathology published by John Wiley & Sons Ltd on behalf of The Pathological Society of Great Britain and Ireland.
Topics: Adolescent; Basic Helix-Loop-Helix Transcription Factors; Bone Neoplasms; Carcinogenesis; Cell Cycle Proteins; Cell Transformation, Neoplastic; Chondrosarcoma, Mesenchymal; Gene Fusion; Genomics; Humans; Nuclear Receptor Coactivator 2; Phosphatidylinositol 3-Kinases; Proto-Oncogene Proteins c-akt; Young Adult
PubMed: 35342947
DOI: 10.1002/path.5899 -
Translational Cancer Research Sep 2022Primary mesenchymal chondrosarcoma (PMC) is a relatively rare malignancy that can occur in bone or soft tissue, but rarely in the lumbar spine; there is currently no...
BACKGROUND
Primary mesenchymal chondrosarcoma (PMC) is a relatively rare malignancy that can occur in bone or soft tissue, but rarely in the lumbar spine; there is currently no unified treatment. We report a case of mesenchymal chondrosarcoma originating from the L1 vertebra.
CASE DESCRIPTION
A 47-year-old female patient was admitted to the hospital with intermittent low back pain for 20 years, accompanied by intermittent headache and radiating pain in both lower limbs. After admission, magnetic resonance imaging (MRI) showed bone destruction of the L1 vertebral body and accessories and a surrounding soft tissue mass. Enhanced MRI revealed significant enhancement of the L1 vertebral body and soft tissue mass. Technetium 99 m-methylene diphosphonate (99 m Tc-MDP) bone scan showed abnormally high metabolism in the L1 vertebral body, which is highly suspicious of malignancy, and vertebral biopsy revealed a soft tissue malignancy originating from the mesenchymal tissue. Total vertebrectomy combined with postoperative adjuvant radiotherapy was planned, but the patient refused radiotherapy for financial reasons. Intraoperative frozen sections indicated mesenchymal chondrosarcoma, as confirmed by postoperative pathological examination. After 1 year of outpatient follow-up, the patient had no related symptoms, and normal motor and sensory function, and her condition had improved.
CONCLUSIONS
Total tumor resection is an effective treatment for PMC, and increased attention to this disease in the clinic is essential.
PubMed: 36237229
DOI: 10.21037/tcr-22-122