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Annals of Palliative Medicine Nov 2021Intracranial mesenchymal chondrosarcoma (IMC) is a rare primary malignant tumor in the skull, but mostly originates from the abnormal residual chondrocytes in the... (Review)
Review
Intracranial mesenchymal chondrosarcoma (IMC) is a rare primary malignant tumor in the skull, but mostly originates from the abnormal residual chondrocytes in the embryonic period, which grow slowly, and primarily occurs at the junction of the cartilage of the skull base. IMC is difficult to diagnose by preoperative imaging and is easily misdiagnosed. It needs to be differentiated from meningiomas, gliomas, hemangioma, fibroids, etc.; this article introduces a case of primary IMC in a 38-year-old female teacher, and reviews the literature on the diagnosis and treatment of symptoms. The patient suffered from persistent severe headaches without nausea and vomiting. There was no obvious abnormality in the physical examination. Magnetic resonance imaging (MRI) of the head showed a circular space-occupying lesion on the right frontal bone and forehead; the mass was approximately 5.9 cm × 5.2 cm × 5.5 cm, and there was a large edema band surrounding it. The space-occupying effect was obvious; bilateral ventricles were compressed, and on the right side, the midline structure was shifted to the left. The patient was initially diagnosed with simple meningioma. After admission, the right frontal lobe meningioma was resected under general anesthesia, and the tumor tissue was completely removed in blocks. The postoperative pathology report stated: malignant tumor of the right frontal lobe; consider mesenchymal chondrosarcoma. Following a difficult pathological consultation at the Provincial Medical Association, the tumor was found to be consistent with mesenchymal chondrosarcoma. Intracranial chondrosarcoma is a very rare malignant tumor. Other intracranial masses, such as meningioma and glioma, should be distinguished through differential diagnosis. At present, more attention is paid to surgical intervention and combined radiotherapy and chemotherapy for the treatment of IMC, which should also be the future treatment option.
Topics: Adult; Bone Neoplasms; Chondrosarcoma; Chondrosarcoma, Mesenchymal; Female; Humans; Magnetic Resonance Imaging; Skull
PubMed: 34872324
DOI: 10.21037/apm-21-2290 -
The Annals of Thoracic Surgery Jun 2002Mesenchymal chondrosarcoma has been well documented in the somatic soft tissue and bone. It is a rare subtype of chondrosarcoma characterized by the presence of islands... (Review)
Review
Mesenchymal chondrosarcoma has been well documented in the somatic soft tissue and bone. It is a rare subtype of chondrosarcoma characterized by the presence of islands of chondroid or by less osteoid tissue enmeshed within dense sheets of primitive small blue mesenchymal cells with hemangiopericytoma-like vessels, or by both. The vast majority of previously published pulmonary mesenchymal chondrosarcoma was metastatic. To the best of our knowledge, only one case of primary pulmonary mesenchymal chondrosarcoma has been described in the literature. Herein, we report the second case of primary mesenchymal chondrosarcoma of the lung and emphasize that biopsy may yield only nonspecific small blue cells, whereas a detailed evaluation of the resected specimen allows definite diagnosis of this rare lung tumor.
Topics: Adult; Chondrosarcoma, Mesenchymal; Female; Humans; Lung Neoplasms
PubMed: 12078803
DOI: 10.1016/s0003-4975(01)03462-2 -
BMC Neurology Mar 2022Mesenchymal chondrosarcoma (MCS) is an ultra-rare, high-grade subtype of chondrosarcoma affecting both bone and soft tissues. Extra-skeletal MCS rarely occurs in intra-... (Review)
Review
BACKGROUND
Mesenchymal chondrosarcoma (MCS) is an ultra-rare, high-grade subtype of chondrosarcoma affecting both bone and soft tissues. Extra-skeletal MCS rarely occurs in intra- and extradural regions.
CASE PRESENTATION
We presented a case of intraspinal dumbbell-shaped MCS at the T12-L2 level with isolated punctate calcification in a 19-year-old male complaining of progressive lower back pain. Surgical treatment for removal of the tumor was performed. The postoperative pathological result confirmed MCS. The patient showed symptomatic improvement and follow-up MRI showed no evidence of recurrence or metastasis for nearly 1 year after surgery.
CONCLUSIONS
CT and MRI play an important role in differential diagnosis for intraspinal MCS. MCS should be added to the differential diagnosis of intraspinal dumbbell-shaped tumors, especially when radiological examinations reveal punctate calcification in a homogeneous enhanced tumor without dural tail sign. However, the final diagnosis depends on histopathological results. Despite the good prognosis of intraspinal MCS, close follow-up after operation is still necessary.
Topics: Adult; Calcinosis; Chondrosarcoma, Mesenchymal; Humans; Magnetic Resonance Imaging; Male; Radiography; Spinal Neoplasms; Young Adult
PubMed: 35321663
DOI: 10.1186/s12883-022-02645-x -
The Neuroradiology Journal Feb 2021Parapharyngeal space (PPS) masses are relatively rare lesions of the head and neck, and account for 0.5-1.5% of head and neck lesions. The most common lesion to occur in... (Review)
Review
Parapharyngeal space (PPS) masses are relatively rare lesions of the head and neck, and account for 0.5-1.5% of head and neck lesions. The most common lesion to occur in the PPS is a benign salivary neoplasm, typically pleomorphic adenoma either from the deep parotid or from ectopic parotid tissue rests within the PPS. A calcified or ossified mass in this location is exceedingly rare, but a calcified variant of pleomorphic adenoma has been reported. In this study, we present a patient with a heavily calcified PPS mesenchymal chondrosarcoma with an unusual presentation. We discuss the imaging and pathologic findings followed by a review of the current literature.
Topics: Adult; Calcinosis; Chondrosarcoma, Mesenchymal; Diagnosis, Differential; Humans; Male; Parapharyngeal Space
PubMed: 32998632
DOI: 10.1177/1971400920962821 -
Annals of Diagnostic Pathology Feb 2021Mesenchymal chondrosarcoma is a rare subtype of chondrosarcoma. The tumor has a characteristic bimorphic pattern with areas of poorly differentiated small round cell...
INTRODUCTION
Mesenchymal chondrosarcoma is a rare subtype of chondrosarcoma. The tumor has a characteristic bimorphic pattern with areas of poorly differentiated small round cell component and interspersed islands of well differentiated hyaline cartilage. Histological diagnosis of mesenchymal chondrosarcoma is very challenging especially in small biopsies when tumor presents with little cartilaginous component. In such cases, it is very difficult to distinguish mesenchymal chondrosarcoma from other round blue cell tumors like Ewing's sarcoma, rhabdomyosarcoma, small cell osteosarcoma and desmoplastic round blue cell tumor. Immunohistochemically, mesenchymal chondrosarcoma stains positive for NKX2.2, CD99, S100 and SOX9. This immunoprofile is non-specific and overlaps with other round blue cell tumors. Till recently, there was no reliable immunohistochemical marker to differentiate mesenchymal chondrosarcoma from other round blue cell tumors. NKX3.1, though widely used as a diagnostic biomarker for prostatic adenocarcinoma, has been recently proposed by Yoshida et al. (2020) as a unique marker of mesenchymal chondrosarcoma and EWSR1-NFATC2 sarcoma.
OBJECTIVE
The aim of our study was to further explore utility of NKX3.1 as a diagnostic marker of mesenchymal chondrosarcoma.
MATERIAL & METHODS
We applied NKX3.1 immunohistochemistry to 21 cases of mesenchymal chondrosarcoma and 32 cases of other round blue cell tumors.
RESULTS
14 out of 21 cases (66.7%) of mesenchymal chondrosarcoma stained positive for NKX3.1 with nuclear expression in small round component. Cartilaginous component was predominantly negative. All other round blue cell tumors showed negative results.
CONCLUSION
Based on our study results we suggest that NKX3.1 is a useful immunohistochemical marker in differentiating mesenchymal chondrosarcoma from its histological mimics.
Topics: 12E7 Antigen; Adult; Aged; Biomarkers, Tumor; Biopsy; Bone Neoplasms; Cell Differentiation; Cell Nucleus; Child; Chondrosarcoma, Mesenchymal; Desmoplastic Small Round Cell Tumor; Diagnosis, Differential; Female; Homeobox Protein Nkx-2.2; Homeodomain Proteins; Humans; Hyaline Cartilage; Immunohistochemistry; Male; Middle Aged; NFATC Transcription Factors; Nuclear Proteins; RNA-Binding Protein EWS; Rhabdomyosarcoma; S100 Proteins; SOX9 Transcription Factor; Sarcoma, Ewing; Sarcoma, Small Cell; Transcription Factors
PubMed: 33302222
DOI: 10.1016/j.anndiagpath.2020.151660 -
Medicina Oral, Patologia Oral Y Cirugia... Jul 2011Mesenchymal chondrosarcoma (MC) is a rare variant of chondrosarcoma (CS) that accounts for upto 3-9% of all CS and has high predilection for the head and neck region. It... (Review)
Review
Mesenchymal chondrosarcoma (MC) is a rare variant of chondrosarcoma (CS) that accounts for upto 3-9% of all CS and has high predilection for the head and neck region. It is usually seen in younger age group compared to conventional CS and maxillary anterior alveolus is the most common site. The tumor is most unusual as it has been described as a particularly aggressive neoplasm with a high tendency for late recurrence and delayed metastasis. It is a biphasic tumor with areas comprising of spindle cell mesenchyme interspread with areas of chondroid differentiation. A 75 year old male presented to us as a painless mass in maxilla. Contrast enhanced computed tomography (CECT) revealed a lytic expansile lesion in the left maxillary bone with foci of calcification within soft tissue lesion. Fine needle aspiration cytology (FNAC) and incisional biopsy was performed which confirmed the diagnosis of maxillary MC. The patient underwent right and left subtotal maxillectomy with 2 cm margins. The review of literature shows that very few cases of maxillary MC have been reported so far. Thus an attempt is made to add this rare case of MC of maxillary alveolus in the English literature.
Topics: Aged; Chondrosarcoma, Mesenchymal; Humans; Male; Maxillary Neoplasms
PubMed: 20711141
DOI: 10.4317/medoral.16.e493 -
Orbit (Amsterdam, Netherlands) 2008Mesenchymal chondrosarcoma, an uncommon lesion in bone and extraskeletal tissue, is extremely rare in the orbit. Two cases of orbital mesenchymal chondrosarcoma in young... (Review)
Review
Mesenchymal chondrosarcoma, an uncommon lesion in bone and extraskeletal tissue, is extremely rare in the orbit. Two cases of orbital mesenchymal chondrosarcoma in young adults presenting with proptosis and diminution of vision are reported. The diagnosis was established by histopathological examination in both cases, which showed undifferentiated mesenchymal cells with islands of cartilage. Both patients underwent exenteration followed by chemotherapy and radiation therapy and are alive with healthy orbits after two years of follow-up.
Topics: Adult; Antineoplastic Combined Chemotherapy Protocols; Chondrosarcoma, Mesenchymal; Cyclophosphamide; Dactinomycin; Female; Humans; Male; Neoplasm Recurrence, Local; Orbit Evisceration; Orbital Neoplasms; Radiotherapy, Adjuvant; Reoperation; Vincristine
PubMed: 18307151
DOI: 10.1080/01676830601169007 -
Genes, Chromosomes & Cancer Nov 2022Mesenchymal chondrosarcoma (MCS) is a rare translocation-associated sarcoma, driven by a canonical HEY1::NCOA2 fusion. The tumors typically have a biphasic phenotype of...
BACKGROUND
Mesenchymal chondrosarcoma (MCS) is a rare translocation-associated sarcoma, driven by a canonical HEY1::NCOA2 fusion. The tumors typically have a biphasic phenotype of primitive small blue round cells intermixed with hyaline cartilage. The head and neck (HN) region is a common site for MCS, accounting for 12-45% of all cases reported.
AIMS
We assembled a relatively large cohort of 13 molecularly confirmed HN MCS for a detailed clinicopathologic analysis. The underlying fusion events were determined using fluorescence in situ hybridization and/or targeted RNA sequencing.
RESULTS
The median age of presentation was 19 years. Five MCSs (39%) had an intraosseous presentation (skull, maxilla, palate, and mandible), while the remaining eight cases occurred in the brain/meninges, orbit, and nasal cavity. Microscopically, HN MCSs were characterized by primitive round cells arranged in a distinctive nested architecture and a rich staghorn vasculature. A cartilaginous component of hyaline cartilage islands and/or single chondrocytes were present in 69% cases. A combined immunoprofile of CD99(+)/SATB2(+)/CD34(-)/STAT6(-) was typically noted. As this immunoprofile is non-specific, the referral diagnoses in cases lacking a cartilaginous component included Ewing sarcoma family and osteosarcoma. Among the seven patients with follow-up data, three developed distant metastasis and one died of disease.
CONCLUSION
HN MCS may arise at intra- or extra-osseous sites. The HN MCS appears to have a more prolonged survival compared other MCS sites. Testing for HEY1::NCOA2 fusion is recommended in HN tumors with nested round cell morphology and staghorn vasculature that lack a distinctive cartilaginous component.
Topics: Adult; Basic Helix-Loop-Helix Transcription Factors; Cell Cycle Proteins; Child; Chondrosarcoma, Mesenchymal; Female; Gene Fusion; Head and Neck Neoplasms; Humans; In Situ Hybridization, Fluorescence; Male; Nuclear Receptor Coactivator 2; Young Adult
PubMed: 35672279
DOI: 10.1002/gcc.23075 -
Radiologic Clinics of North America Jan 1999Although an extremely rare neoplasm, orbital mesenchymal chondrosarcoma has a characteristic radiologic appearance. Suspicion of this lesion preoperatively can be of... (Review)
Review
Although an extremely rare neoplasm, orbital mesenchymal chondrosarcoma has a characteristic radiologic appearance. Suspicion of this lesion preoperatively can be of great value to the ophthalmologist because it tends to produce profuse bleeding at biopsy. This article reviews the clinical presentation, pathologic features, and radiologic findings of patients with orbital mesenchymal chondrosarcoma.
Topics: Biopsy; Chondrosarcoma, Mesenchymal; Diagnosis, Differential; Humans; Magnetic Resonance Imaging; Orbital Neoplasms; Postoperative Hemorrhage; Tomography, X-Ray Computed
PubMed: 10026739
DOI: 10.1016/s0033-8389(05)70088-9 -
International Journal of Surgery Case... Feb 2023Chondrosarcoma is the most common primary malignant bone tumor of the hand, Mesenchymal chondrosarcoma (MCS) is a rare subtype of chondrosarcoma, extraosseous...
INTRODUCTION AND IMPORTANCE
Chondrosarcoma is the most common primary malignant bone tumor of the hand, Mesenchymal chondrosarcoma (MCS) is a rare subtype of chondrosarcoma, extraosseous mesenchymal chondrosarcoma (EMCS) is even rarer.
CASE PRESENTATION
We presented a case of primary EMCS involving the distal thumb in a 59-year-old man. The patient had abnormally grown nails and a higher skin temperature than the contralateral finger. After admission to our hospital, we found that the tumor has the possibility of malignant transformation, but imageological examinations did not reveal invasive bone lesions and calcification. Finally, due to the patient's strong desire to preserve the thumb, a conservative surgical approach was selected to preserve the patient's finger function, and histological examination indicated MCS.
CONCLUSION
EMCS is a highly malignant tumor with strong tendency of invasion and metastasis. Imaging is sometimes difficult to diagnose, and pathology is a necessary part of distinguishing between benign and malignant tumors. Operation is the major treatment to EMCS, meanwhile, adjuvant radiotherapy and chemotherapy also be effective. The overall prognosis is poor.
PubMed: 36652820
DOI: 10.1016/j.ijscr.2023.107903