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Surgical Neurology International 2023Mesenchymal chondrosarcoma is an uncommon malignant variant of chondrosarcoma that mainly affects the bones and cartilaginous tissues, but may rarely involve the spine....
BACKGROUND
Mesenchymal chondrosarcoma is an uncommon malignant variant of chondrosarcoma that mainly affects the bones and cartilaginous tissues, but may rarely involve the spine. Careful preoperative planning for surgical tumor removal and spine reconstruction is mandatory and must be based on oncologic and surgical staging.
CASE DESCRIPTION
Over 1 month, a 16-year-old female became paraplegic with a T9 sensory level and urinary dysfunction. The magnetic resonance imaging revealed an intraspinal extradural T7-T9 mass that was isointense in T1W1 and markedly enhanced with gadolinium. The patient underwent gross-total tumor resection followed by an osteoplastic laminectomy with fusion. The histological examination was consistent with a mesenchymal chondrosarcoma. She had received radiation and chemotherapy. One year later, she was readmitted for tumor recurrence with multiple metastases involving L1, the lung, and peritoneum. Despite full course of radiotherapy and chemotherapy, she died after 6 months of the second surgery.
CONCLUSION
Total resection of mesenchymal chondrosarcomas is the gold standard for treatment and is typically followed by radiation and/or chemotherapy. However, the status of residual tumor, local extension, and or metastases best determine the overall survival which may prove extremely limited.
PubMed: 37151474
DOI: 10.25259/SNI_206_2023 -
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 -
International Journal of Surgical... Feb 2024A 50-year-old man presented for evaluation of a periscapular mass. Biopsy suggested a low-grade chondrosarcoma; however, the resected specimen revealed a grade 2...
A 50-year-old man presented for evaluation of a periscapular mass. Biopsy suggested a low-grade chondrosarcoma; however, the resected specimen revealed a grade 2 chondrosarcoma with a low-grade dedifferentiated mesenchymal component. The mass multiply recurred as chondrosarcoma without a dedifferentiated component before the patient developed lung metastases of chondrosarcoma without a dedifferentiated component 11 years after the initial diagnosis and died of disease. This is one of the first reported cases of a dedifferentiated chondrosarcoma with low-grade dedifferentiated component. While overall prognosis may be better than in typical dedifferentiated chondrosarcoma, this tumor demonstrated numerous local recurrences as well as metastasis.
Topics: Male; Humans; Middle Aged; Bone Neoplasms; Prognosis; Biopsy; Chronic Disease; Chondrosarcoma; Recurrence
PubMed: 37143346
DOI: 10.1177/10668969231173379 -
Revista Espanola de Enfermedades... Mar 2024A 47-year-old man with a history of ESMC resection of the left chest wall seven years ago was admitted to our hospital due to mid-upper abdominal pain and jaundice for...
A 47-year-old man with a history of ESMC resection of the left chest wall seven years ago was admitted to our hospital due to mid-upper abdominal pain and jaundice for more than 10 days. Laboratory tests showed elevated direct bilirubin, alanine aminotransferase, gamma-glutamyltranspeptidase, and alkaline phosphatase. Computed tomography (CT) of the abdomen revealed soft tissue mass in the head and body of the pancreas with irregularly shaped calcifications, and an enhanced scan showed heterogeneous enhancement. Combined with the patient's past medical history, the possibility of pancreatic metastasis of ESMC was considered. After anti-inflammatory, hepatoprotective, and cholagogical treatment jaundice improved, and ultrasound endoscopy-guided fine-needle aspiration (EUS-FNA) was performed to clarify the nature of the mass, which showed a 4.1*4.2 cm mixed echogenic area with internal calcification in the head of the pancreas. Aspiration pathology showed proliferation of short spindle and round cells into nests, the immunohistochemistry stain showed CD99 (+); CD34, CD117, Dog-1, and S-100 were negative. Pancreatic metastasis of ESMC was diagnosed. Four months later, endoscopic biliary metal stent drainage (EMBD) was performed when the patient developed obstructive jaundice again due to lesion progression. PET/CT at a 2-year follow-up showed multiple high-density calcifications and abnormally increased FDG metabolism throughout the body.
Topics: Male; Humans; Animals; Dogs; Middle Aged; Chondrosarcoma, Mesenchymal; Positron Emission Tomography Computed Tomography; Pancreatic Neoplasms; Pancreas; Endoscopic Ultrasound-Guided Fine Needle Aspiration; Jaundice; Endoscopy, Gastrointestinal
PubMed: 37114405
DOI: 10.17235/reed.2023.9586/2023 -
The American Journal of Surgical... Jun 2023Calcified chondroid mesenchymal neoplasm is a term proposed for tumors with a spectrum of morphologic features, including cartilage/chondroid matrix formation, that...
Calcified chondroid mesenchymal neoplasm is a term proposed for tumors with a spectrum of morphologic features, including cartilage/chondroid matrix formation, that frequently harbor FN1 gene fusions. We report a series of 33 cases of putative calcified chondroid mesenchymal neoplasms, mostly referred for expert consultation out of concern for malignancy. Patients included 17 males and 16 females, with a mean age of 51.3 years. Anatomic locations include the hands and fingers, feet and toes, head and neck, and temporomandibular joint; 1 patient presented with multifocal disease. Radiologic review showed soft tissue masses with variable internal calcification, which occasionally scalloped bone but in all cases appeared indolent/benign. Tumors had a mean gross size of 2.1 cm and a homogenous rubbery to fibrous/gritty tan-white cut surface. Histology demonstrated multinodular architecture with a prominent chondroid matrix and increased cellularity towards the periphery of the nodules. The tumor cells were polygonal with eccentric nuclei and bland cytologic features and showed a variable amount of increased spindled / fibroblastic forms in the perinodular septa. The majority of cases had notable grungy and/or lacy calcifications. A subset of cases demonstrated at least focal areas of increased cellularity and osteoclast-like giant cells. Herein, we confirm the distinct morphologic and clinicopathologic features associated with this entity with the largest series to date, with a focus on practical diagnostic separation from similar chondroid neoplasms. Awareness of these features is critical in avoiding pitfalls, including a malignant diagnosis of chondrosarcoma.
Topics: Male; Female; Humans; Middle Aged; Neoplasms, Connective and Soft Tissue; Chondrosarcoma; Cartilage; Toes; Bone Neoplasms
PubMed: 37102574
DOI: 10.1097/PAS.0000000000002044 -
The Journal of Craniofacial SurgeryChondrosarcomas (CHS) are malignant mesenchymal tumors arising from cartilage. Chondrosarcomas arising in the temporomandibular joint are especially rare. Herein, the...
Chondrosarcomas (CHS) are malignant mesenchymal tumors arising from cartilage. Chondrosarcomas arising in the temporomandibular joint are especially rare. Herein, the authors report an unusual case of a 50-year-old female who presented with a progressive swelling in the left preauricular region. Computed tomography and magnetic resonance imaging revealed an osteolytic and expansive lesion in the left condyle. The diagnosis tended to be a malignant tumor. A resection guide and 3-dimensional printed titanium prosthesis for condyle reconstruction were designed. Extended resection of the left condyle and reconstruction were performed. The pathologic diagnosis was consistent with CHS (grade II). There was no tumor recurrence or distant metastasis at the 6-year follow-up, and good occlusion and satisfactory function of the temporomandibular joint were regained.
Topics: Female; Humans; Middle Aged; Mandibular Condyle; Titanium; Follow-Up Studies; Neoplasm Recurrence, Local; Dental Implants; Chondrosarcoma; Bone Neoplasms
PubMed: 37062856
DOI: 10.1097/SCS.0000000000009331 -
Revista Espanola de Medicina Nuclear E... 2023Radioguided surgery uses radioactive substances to identify and remove hard-to-locate lesions. Mesenchymal tumors constitute a heterogeneous group of neoplasms derived... (Observational Study)
Observational Study
INTRODUCTION
Radioguided surgery uses radioactive substances to identify and remove hard-to-locate lesions. Mesenchymal tumors constitute a heterogeneous group of neoplasms derived from the mesoderm, including benign lesions and malignant sarcomas. The aim of this study was to evaluate the ability of I radioactive seeds to guide intraoperative localization of mesenchymal tumors, analyzing the complication rates and evaluating the margins of the surgical specimens retrieved.
METHODS
Retrospective observational study of all consecutive patients undergoing radioguided surgery of a mesenchymal tumor with a I radioactive seed from January 2012 to January 2020 at a tertiary referral center in Spain. The seed was inserted percutaneously guided by ultrasound or computed tomography in an outpatient setting.
RESULTS
Fifteen lesions were resected in 11 interventions in 11 patients, recovering all lesions marked (100%) with a I seed. The lesions included areas of benign fibrosis (26.7%), cellular angiofibroma (6.7%), desmoid tumor (20%), solitary fibrous tumor (13.3%), chondrosarcoma (6.7%), and pleomorphic sarcoma (26.7%), with a high rate of recurrent tumors (60%). There was only one complication (6.7%) due to the seed falling within the surgical bed. According to the UICC classification of residual tumors, 80% of the lesions resulted in an R0 resection, 6.7% were R1 resections, and 13.3% were R2 resections.
CONCLUSION
Radioguided surgery is an accurate technique for the resection of hard-to-locate mesenchymal tumors.
Topics: Humans; Neoplasm Recurrence, Local; Iodine Radioisotopes; Surgery, Computer-Assisted; Retrospective Studies
PubMed: 37062451
DOI: 10.1016/j.remnie.2023.04.004 -
Genes, Chromosomes & Cancer Oct 2023Extraskeletal myxoid chondrosarcoma (EMC) is a rare sarcoma of uncertain differentiation predominantly arising in deep soft tissue. Its conventional morphologic...
Extraskeletal myxoid chondrosarcoma (EMC) is a rare sarcoma of uncertain differentiation predominantly arising in deep soft tissue. Its conventional morphologic appearance manifests as a relatively well-circumscribed, multilobular tumor composed of uniform short spindle-to-ovoid primitive mesenchymal cells with deeply eosinophilic cytoplasm arranged in anastomosing cords within abundant myxoid matrix. The genetic hallmark of EMC has long been considered to be pathognomonic gene rearrangements involving NR4A3, which when fused to TAF15, often have high-grade morphology with increased cellularity, moderate to severe cytologic atypia, and rhabdoid cytomorphology. Herein, we describe two cases of EMC with TAF15::NR4A3 fusion that appear morphologically distinct from both conventional and high-grade EMC. Both cases had an unusual biphasic appearance and showed diffuse positivity for p63, mimicking myoepithelial tumors. DNA methylation profiling demonstrated that both cases clearly cluster with EMC, indicating that they most likely represent morphologically distinct variants of EMC. The clinical significance and prognostic impact of this morphologic variance remains to be determined. Molecular testing, including DNA methylation profiling, can help to confirm the diagnosis and avoid confusion with mimics; it adds another layer of data to support expanding the morphologic spectrum of EMC.
PubMed: 37057757
DOI: 10.1002/gcc.23144 -
Cancers Mar 2023Chondrosarcomas can be classified into various forms according to the presence or absence of a precursor lesion, location, and histological subtype. The new 2020 World... (Review)
Review
Chondrosarcomas can be classified into various forms according to the presence or absence of a precursor lesion, location, and histological subtype. The new 2020 World Health Organization (WHO) Classification of Tumors of Soft Tissue and Bone classifies chondrogenic bone tumors as benign, intermediate (locally aggressive), or malignant, and separates atypical cartilaginous tumors (ACTs) and chondrosarcoma grade 1 (CS1) as intermediate and malignant tumors. respectively. Furthermore, the classification categorizes chondrosarcomas (including ACT) into eight subtypes: central conventional (grade 1 vs. 2-3), secondary peripheral (grade 1 vs. 2-3), periosteal, dedifferentiated, mesenchymal, and clear cell chondrosarcoma. Most chondrosarcomas are the low-grade, primary central conventional type. The rarer subtypes include clear cell, mesenchymal, and dedifferentiated chondrosarcomas. Comprehensive analysis of the characteristic imaging findings can help differentiate various forms of chondrosarcomas. However, distinguishing low-grade chondrosarcomas from enchondromas or high-grade chondrosarcomas is radiologically and histopathologically challenging, even for experienced radiologists and pathologists.
PubMed: 36980590
DOI: 10.3390/cancers15061703 -
Pathology International Apr 2023
Topics: Humans; Chondrosarcoma, Mesenchymal; Oncogene Proteins, Fusion; Bone Neoplasms; Lung
PubMed: 36752330
DOI: 10.1111/pin.13313