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Archives of Pathology & Laboratory... Nov 2018Extraskeletal mesenchymal chondrosarcoma is a rare soft tissue sarcoma arising from soft tissues, mainly of the lower extremities, meninges, and orbits. It usually... (Review)
Review
Extraskeletal mesenchymal chondrosarcoma is a rare soft tissue sarcoma arising from soft tissues, mainly of the lower extremities, meninges, and orbits. It usually presents during the second to third decades of life, and has a slight predominance in females. Histologically, it has a typical biphasic pattern comprising small cells and islands of hyaline cartilage. It can pose a diagnostic challenge in small biopsy specimens where 1 of the 2 components can be absent. The prognosis is extremely variable; survival varies depending on the location of the tumor.
Topics: Chondrosarcoma, Mesenchymal; Humans; Sarcoma
PubMed: 30407856
DOI: 10.5858/arpa.2017-0109-RS -
Current Oncology Reports Mar 2018Mesenchymal chondrosarcoma is a rare but deadly form of chondrosarcoma that typically affects adolescents and young adults. While curative intent is possible for... (Review)
Review
Mesenchymal chondrosarcoma is a rare but deadly form of chondrosarcoma that typically affects adolescents and young adults. While curative intent is possible for patients with localized disease, few options exist for patients in the unresectable/metastatic setting. Thus, it is imperative to understand the fusion-driven biology of this rare malignant neoplasm so as to lead to the future development of better therapeutics for this disease. This manuscript will briefly review the clinical and pathologic features of mesenchymal chondrosarcoma followed by an appraisal of existing data linked to the fusions, HEY1-NCOA2 and IRF2BP2-CDX1, and the associated downstream pathways.
Topics: Bone Neoplasms; Chondrosarcoma, Mesenchymal; Humans; Oncogene Proteins, Fusion; Prognosis
PubMed: 29582189
DOI: 10.1007/s11912-018-0668-z -
The Gulf Journal of Oncology Jan 2021Mesenchymal chondrosarcoma is a rare high grade malignant neoplasm that accounts for 3-10% of all chondrosarcomas. Histopathologically, it shows biphasic population...
INTRODUCTION
Mesenchymal chondrosarcoma is a rare high grade malignant neoplasm that accounts for 3-10% of all chondrosarcomas. Histopathologically, it shows biphasic population composed of small round to ovoid with occasional spindle cells and islands of well differentiated cartilage. The study aimed at retrospectively analysing the clinical, pathological, radiological features of these cases in our institution.
MATERIALS AND METHODS
This is a retrospective descriptional study. All the cases of mesenchymal chondrosarcomas were retrieved from our archives of pathology over a period of 10 years .The demographic details including the age, clinical presentation including skeletal/extraskeletal along with radiology were noted for all these cases. The treatment details along with the follow up of the patients were archived from the medical records.
RESULTS
A total of 13 cases of mesenchymal chondrosarcoma were retrieved for our study. The mean age of presentation was 33 years with a slight male predilection. Extra skeletal soft tissue origin was noted in 3 of our cases (3/13), one case in forearm, another in pelvis. The third case was intracranial origin which presented as a dural based parieto-occipital mass and rest all had bony origin .The radiological and clinical correlation was done for all these cases.
CONCLUSION
Mesenchymal chondrosarcoma presents multiple diagnostic challenges, most common include inadequate biopsy samples which may result in errors in diagnosis, namely with small blue round cell tumours .A better understanding of this entity may help the pathologists in conferring an accurate diagnosis to the clinicians.
Topics: Adolescent; Adult; Aged; Child; Chondrosarcoma, Mesenchymal; Female; Humans; Male; Middle Aged; Retrospective Studies; Young Adult
PubMed: 33716213
DOI: No ID Found -
Diagnostics (Basel, Switzerland) Mar 2022Mesenchymal chondrosarcoma is an uncommon malignant mesenchymal tumor with an aggressive behavior. Diagnoses of mesenchymal chondrosarcoma are established based on...
Mesenchymal chondrosarcoma is an uncommon malignant mesenchymal tumor with an aggressive behavior. Diagnoses of mesenchymal chondrosarcoma are established based on histomorphological, immunohistochemical, and molecular findings. Only one case of extraskeletal mesenchymal chondrosarcoma (EMC) of the uterus has been reported. This article presents the second case of primary uterine EMC, occurring in a 33-year-old woman. We describe the histological and immunophenotypical features of EMC. Our observations will help pathologists and clinicians perform accurate histological diagnoses of uterine EMC and plan appropriate treatment strategies for this rare tumor.
PubMed: 35328196
DOI: 10.3390/diagnostics12030643 -
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 -
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 -
Pathology International Apr 2023
Topics: Humans; Chondrosarcoma, Mesenchymal; Oncogene Proteins, Fusion; Bone Neoplasms; Lung
PubMed: 36752330
DOI: 10.1111/pin.13313 -
Cancer Medicine Jan 2023Mesenchymal chondrosarcoma (MCS) is an ultra-rare sarcoma that follows a more aggressive course than conventional chondrosarcoma. This study evaluates prognostic...
BACKGROUND
Mesenchymal chondrosarcoma (MCS) is an ultra-rare sarcoma that follows a more aggressive course than conventional chondrosarcoma. This study evaluates prognostic factors, treatments (surgery, chemotherapy, and radiation), and outcomes in an Australian setting.
METHODS
We collected demographics, clinicopathological variables, treatment characteristics, and survival status from patients with MCS registered on the national ACCORD sarcoma database. Outcomes include overall survival (OS) and progression-free survival (PFS).
RESULTS
We identified 22 patients with MCS between 2001-2022. Median age was 28 (range 10-59) years, 19 (86%) had localised disease at diagnosis of whom 16 had surgery (84%), 11 received radiation (58%), and 10 chemotherapy (53%). Ten (52%) developed recurrence and/or metastases on follow-up and three patients with initial metastatic disease received surgery, radiation, and chemotherapy. At a median follow-up of 50.9 (range 0.4-210) months nine patients had died. The median OS was 104.1 months (95% CI 25.8-182.3). There was improved OS for patients with localised disease who had surgical resection of the primary (p = 0.003) and those with ECOG 0-1 compared to 2-3 (p = 0.023) on univariate analysis.
CONCLUSIONS
This study demonstrates contemporary Australian treatment patterns of MCS. The role of chemotherapy for localised disease remains uncertain. Understanding treatment patterns and outcomes help support treatment decisions and design of trials for novel therapeutic strategies.
Topics: Humans; Child; Adolescent; Young Adult; Adult; Middle Aged; Chondrosarcoma, Mesenchymal; Bone Neoplasms; Australia; Soft Tissue Neoplasms; Sarcoma; Cohort Studies; Retrospective Studies
PubMed: 35603739
DOI: 10.1002/cam4.4849 -
International Journal of Surgery Case... Feb 2022Chondrosarcomas of the head and neck are extremely rare, accounting for approximatively 0.1% of all head and neck malignant tumors. Mesenchymal chondrosarcomas are...
INTRODUCTION
Chondrosarcomas of the head and neck are extremely rare, accounting for approximatively 0.1% of all head and neck malignant tumors. Mesenchymal chondrosarcomas are particularly aggressive, with a high propensity for recurrence.
CASE REPORT
We report the case of a 25 years old man with no prior medical history, who presented to our facility with an aggressive tumor of the left maxilla. Upon radiologic and histopathological examinations, the tumor was revealed to be a mesenchymal chondrosarcoma. The patient was treated using surgical excision of the tumor, neoadjuvant chemotherapy and adjuvant radiotherapy.
CONCLUSION
Due to the high risk of recurrence and the possibility of metastasis occurring long after the initial diagnosis, it is important that patients with mesenchymal chondrosarcomas receive close and regular follow-ups after treatment.
PubMed: 35074656
DOI: 10.1016/j.ijscr.2021.106696 -
The Journal of Bone and Joint Surgery.... Jun 1964
Topics: Bone Neoplasms; Brain Neoplasms; Chondrosarcoma; Chondrosarcoma, Mesenchymal; Neoplasm Metastasis; Pathology; Skull Neoplasms; Surgical Procedures, Operative
PubMed: 14161087
DOI: No ID Found