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Indian Journal of Nuclear Medicine :... 2023Mesenchymal chondrosarcoma (MC) is a rare malignant tumor that represents <3% of all chondrosarcomas. Herein, we describe extraskeletal MC involving the mediastinum in a...
Mesenchymal chondrosarcoma (MC) is a rare malignant tumor that represents <3% of all chondrosarcomas. Herein, we describe extraskeletal MC involving the mediastinum in a 24-year-old gentleman with a rare phenomenon of adrenal metastasis.
PubMed: 37180190
DOI: 10.4103/ijnm.ijnm_180_21 -
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 -
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 -
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 -
Indian Journal of Otolaryngology and... Dec 2022Liposarcomas of the larynx is an extremely rare entity, and less than 50 cases have been published in English language literature. It is a malignant mesenchymal tumour...
Liposarcomas of the larynx is an extremely rare entity, and less than 50 cases have been published in English language literature. It is a malignant mesenchymal tumour arising from adipose tissue with a very high propensity for local recurrence. Well-differentiated liposarcomas are the most common variety but are challenging to diagnose because of their resemblance with benign tumors like lipoma and other malignant soft tissue sarcomas like myxoid chondrosarcoma. Therefore, immunohistochemistry (IHC) should be considered for confirmation. Wide local excision is the treatment of choice, and post-operative radiotherapy can be considered in cases of positive resection margins not amenable for revision surgery, high tumour grade, and myxoid variant. We are reporting a case of well-differentiated liposarcoma of the left aryepiglottic fold (AEF) in a 66-year-old man who was diagnosed to have a benign lipomatous lesion in the same location 3 years back. Based on the reports of the published cases, we are presenting a management algorithm for this entity.
PubMed: 36742670
DOI: 10.1007/s12070-021-02466-3 -
Indian Journal of Otolaryngology and... Dec 2022Mass lesions of the larynx are one of the most common clinical entity which we come across in routine otorhinolaryngology and head neck practice with varied...
Mass lesions of the larynx are one of the most common clinical entity which we come across in routine otorhinolaryngology and head neck practice with varied symptomatology. Among all the mass lesions of the larynx, Epithelial neoplasms constitute up to 97%. Mesenchymal tumours of the larynx constitute only 0.3-1.0% of all the laryngeal tumors. Abundance of cartilage structures in the larynx made it a spot for mesenchymal tumors [chondromas and chandrosacrcomas]. The spectrum of mesenchymal neoplasms can vary from chondromas, chondroblastoma to chondrosarcoma. Here we want to share our experience of a mesenchymal tumour of the larynx. This case is reported for the rarity and ambiguity in diagnosis. Though these are slow-growing tumours with an early presentation, in our case, the patient had a supportive tracheostomy without definitive treatment for more than 2 years. We managed this patient by excising the mass by lateral pharyngotomy with the preservation of larynx followed by successful Decannulation in 20 days.
PubMed: 36742506
DOI: 10.1007/s12070-020-02308-8 -
Cureus Dec 2022We present an extremely rare case where the sarcomatoid urothelial carcinoma of the urinary bladder was present with chondrosarcomatous and squamous cell...
We present an extremely rare case where the sarcomatoid urothelial carcinoma of the urinary bladder was present with chondrosarcomatous and squamous cell differentiation. A 74-year-old male smoker presented with intermittent hematuria with the passage of clots. On imaging, an irregular polypoidal lesion was diagnosed near the right vesicoureteric junction, and transurethral resection of the bladder tumor was performed. Histopathological examination showed sarcomatoid urothelial carcinoma with chondrosarcoma and squamous cell differentiation. He refused the surgical treatment of radical cystectomy and underwent Gemcitabine and Cisplatin chemotherapy. He died nine months after the diagnosis. Sarcomatoid urothelial carcinoma is a high-grade biphasic neoplasm with malignant epithelial and mesenchymal components. Its association with squamous cell carcinoma is infrequent. It is very aggressive, and there is no standard treatment for this disease. The radical surgical option appears to be the main form of treatment. It is scarce with a grave prognosis.
PubMed: 36721592
DOI: 10.7759/cureus.33107 -
International Journal of Molecular... Jan 2023This review provides an overview of histopathology, clinical presentation, molecular pathways, and potential new systemic treatments of high-grade chondrosarcomas (CS),... (Review)
Review
This review provides an overview of histopathology, clinical presentation, molecular pathways, and potential new systemic treatments of high-grade chondrosarcomas (CS), including grade 2−3 conventional, dedifferentiated, and mesenchymal CS. The diagnosis of CS combines radiological and histological data in conjunction with patient clinical presentations. Conventional CS is the most frequent subtype of CS (85%) and represents about 25% of primary bone tumors in adults; they can be categorized according to their bone location into central, peripheral, and periosteal chondrosarcomas. Central and peripheral CS differ at the molecular level with either IDH1/2 mutations or EXT1/2 mutations, respectively. CDKN2A/B deletions are also frequent in conventional CS, as well as COL2A1 mutations. Dedifferentiated CS develops when low-grade conventional CS transforms into a high-grade sarcoma and most frequently exhibits features of osteosarcoma, fibrosarcoma, or undifferentiated pleomorphic sarcoma. Their molecular characteristics are similar to conventional CS. Mesenchymal CS is a totally different pathological entity exhibiting recurrent translocations. Their clinical presentation and management are different too. The standard treatment of CSs is wide en-bloc resection. CS are relatively radiotherapy resistant; therefore, doses >60 Gy are needed in an attempt to achieve local control in unresectable tumors. Chemotherapy is possibly effective in mesenchymal chondrosarcoma and is of uncertain value in dedifferentiated chondrosarcoma. Due to resistance to standard anticancer agents, the prognosis is poor in patients with metastatic or unresectable chondrosarcomas. Recently, the refined characterization of the molecular profile, as well as the development of new treatments, allow new therapeutic options for these rare tumors. The efficiency of IDH1 inhibitors in other malignancies suggests that these inhibitors will be part of IDH1/2 mutated conventional CS management soon. Other treatment approaches, such as PIK3-AKT-mTOR inhibitors, cell cycle inhibitors, and epigenetic or immune modulators based on improving our understanding of CS molecular biology, are emerging.
Topics: Adult; Humans; Chondrosarcoma; Bone Neoplasms; Radiography; Osteosarcoma; Biology
PubMed: 36674874
DOI: 10.3390/ijms24021361 -
Indian Journal of Pathology &... 2023Ewing sarcoma (ES) are malignant small round cell tumors (MSRCT) characterized by rearrangements of EWSR1 gene. Although gold standard for diagnosis is detection of...
Correlation NKX2.2 IHC and break-apart FISH in the diagnosis of Ewing sarcoma: Can combined NKX2.2 and CD99 immunoexpression obviate or minimize the need of FISH testing? First assessment study from Indian tertiary cancer care center.
CONTEXT
Ewing sarcoma (ES) are malignant small round cell tumors (MSRCT) characterized by rearrangements of EWSR1 gene. Although gold standard for diagnosis is detection of specific fusion genes by molecular testing, these ancillary tests are costly and only available in limited number of settings. There is a persuasive evidence for reliability of NKX2.2 immunohistochemistry (IHC) as a surrogate marker for EWSR1 gene rearrangement in ES.
AIMS
The aim of this study is to correlate the NKX2.2 immuno-expression with genetically confirmed ES cases and also to assess the reliability and accuracy of NKX2.2 along with combined positivity of NXX2.2 and CD99 in diagnosing ES and differentiating it from other relevant histological mimics.
SETTINGS AND DESIGN
The present study is a retrospective study conducted over a period of 6-year duration in a tertiary cancer care center.
METHODS AND MATERIAL
We evaluated NKX2.2 immunoexpression in 35 genetically confirmed cases of ES and also in pertaining differential entities (n = 58) of ES including rhabdomyosarcoma (n = 20), lymphoblastic lymphoma (n = 14), Wilms tumor (n = 10), poorly differentiated synovial sarcoma (n = 4), small-cell osteosarcoma (n = 4), neuroblastoma (n = 5), and mesenchymal chondrosarcoma (n = 1). CD99 was performed in the category of MSRCTs showing NKX2.2 positivity to evaluate combined specificity for the diagnosis of ES.
RESULTS
Of the 35 genetically confirmed cases of ES, 29 cases (83%) showed NKX2.2-positive expression (83% sensitivity). Compared to ES, NKX2.2 was positive in only 05% cases (3/58 cases) of non-ES MSRCT. Only two of five cases of neuroblastomas and one case of mesenchymal chondrosarcoma showed NKX2.2 positivity. CD99 positivity was seen in 100% of ES and in the single case of mesenchymal chondrosarcoma. All five cases (100%) of neuroblastoma were negative for CD99.
CONCLUSIONS
The presented study, which is the first from an Indian oncology center, showed NKX2.2 IHC is quite reliable in diagnosis of ES in the right clinicopathological context. With remarkable sensitivity and specificity of NKX2.2 IHC for diagnosis of ES, we propose that combined positivity of CD99 and NKX2.2 IHC can obviate or minimize the need of EWSR1 gene rearrangement molecular testing for diagnosis of ES.
Topics: Humans; 12E7 Antigen; Biomarkers, Tumor; Chondrosarcoma, Mesenchymal; Immunohistochemistry; Neuroblastoma; Neuroectodermal Tumors, Primitive, Peripheral; Reproducibility of Results; Retrospective Studies; RNA-Binding Protein EWS; Sarcoma; Sarcoma, Ewing; Transcription Factors; Homeobox Protein Nkx-2.2
PubMed: 36656211
DOI: 10.4103/ijpm.ijpm_535_21