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British Journal of Neurosurgery Dec 2012Mesenchymal chondrosarcoma is a very rare malignant cartilaginous forming tumour in central nervous system (CNS), which is rarely encountered in clinical practice and... (Review)
Review
BACKGROUND
Mesenchymal chondrosarcoma is a very rare malignant cartilaginous forming tumour in central nervous system (CNS), which is rarely encountered in clinical practice and generally occurs in young adults. This article describes a case of primary intracranial mesenchymal chondrosarcoma in a 31-year-old woman and reviews the literature on its manifestations and management.
CASE REPORT
This patient had suffered from severe headache, intermittent nausea and vomiting for 1 week. Systemic examination was unremarkable. Magnetic resonance imaging (MRI) demonstrated a giant, heterogeneous, intensely enhancing mass of 6 × 5 × 4 cm, occupying the bilateral frontal and based on the anterior falx cerebri, which was initially thought to be a simply meningioma. The patient underwent a bicoronal craniotomy and gross total resection of the tumour. Pathologic examination revealed the mesenchymal chondrosarcoma.
CONCLUSION
Intracranial mesenchymal chondrosarcoma is an extreme rare neoplasm, which should be considered in the differential diagnosis of intracranial mass like a meningioma. We emphasize the importance of surgical intervention and combination of microsurgical resection and radiotherapy, it should be the therapeutical choice of the future.
Topics: Adult; Bone Neoplasms; Chondrosarcoma, Mesenchymal; Dura Mater; Female; Frontal Lobe; Humans
PubMed: 22731866
DOI: 10.3109/02688697.2012.697219 -
Medical and Pediatric Oncology Feb 1996We present a case of primary mesenchymal chondrosarcoma of the proximal phalanx of the first toe. The bones of the foot represent an infrequent primary site for this... (Review)
Review
We present a case of primary mesenchymal chondrosarcoma of the proximal phalanx of the first toe. The bones of the foot represent an infrequent primary site for this neoplasm. The tumour consisted of layers of undifferentiated round cells with scanty cytoplasm and hyperchromatic nuclei. The presence of brain, lung, and left auricle metastasis was demonstrated, and the patient died due to brain edema 18 days after admission. Mesenchymal chondrosarcoma is a rare tumor that more frequently involves the pelvic bones, the femur, and the humerus. To our knowledge, only nine cases of primary mesenchymal chondrosarcoma arising from the bones of the foot have been previously reported, with none involving the phalanx of the toe.
Topics: Adult; Bone Neoplasms; Chondrosarcoma, Mesenchymal; Foot Diseases; Hallux; Humans; Lung Neoplasms; Male
PubMed: 8531853
DOI: 10.1002/(SICI)1096-911X(199602)26:2<139::AID-MPO13>3.0.CO;2-D -
Journal of Vascular and Interventional... Dec 2014
Topics: Adult; Chondrosarcoma, Mesenchymal; Diagnosis, Differential; Fatal Outcome; Female; Humans; Iliac Vein; Magnetic Resonance Imaging; Tomography, X-Ray Computed; Vascular Neoplasms
PubMed: 25457141
DOI: 10.1016/j.jvir.2014.07.030 -
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 -
BMJ Case Reports Mar 2019Chondrosarcoma (CS) is a malignant tumour of long and flat bone characterised by the formation of cartilage. Mesenchymal chondrosarcoma (MCS) is a rare subtype of CS...
Chondrosarcoma (CS) is a malignant tumour of long and flat bone characterised by the formation of cartilage. Mesenchymal chondrosarcoma (MCS) is a rare subtype of CS that is more aggressive and may lead to erroneous diagnosis in a limited biopsy. The diagnosis is mainly based on the histopathological appearance of biphasic pattern of undifferentiated small round cells separated by islands of well-differentiated hyaline cartilage. We report a case of 13-year-old boy who initially presented with gum swelling and the biopsy result suggested a benign fibrous lesion. Following an extensive lesion shown in radiologic findings, the tumour excision was done and finally was diagnosed as an MCS of the maxilla. The patient was given postoperative chemotherapy (EURO-EWING 99 regimen), and now on regular follow-up for monitoring of local recurrence or tumour metastasis.
Topics: Adolescent; Aftercare; Biopsy; Chondrosarcoma, Mesenchymal; Diagnosis, Differential; Drug Therapy; Humans; Male; Maxilla; Neoplasm, Residual; Rare Diseases; Reoperation; Tomography, X-Ray Computed; Treatment Outcome
PubMed: 30852518
DOI: 10.1136/bcr-2018-228969 -
Cancer May 1996Mesenchymal chondrosarcomas arising in the central nervous system are extremely rare. Morphologic features have not been found to correlate reliably with prognosis. (Review)
Review
BACKGROUND
Mesenchymal chondrosarcomas arising in the central nervous system are extremely rare. Morphologic features have not been found to correlate reliably with prognosis.
METHODS
Eight intracranial and five intraspinal mesenchymal chondrosarcomas were reviewed with regard to location, treatment, and long term follow-up data. The histopathologic and immunohistochemical results, including Ki-67 nuclear staining frequency, were critically reviewed, and deoxyribonucleic acid content was analyzed by flow cytometry.
RESULTS
Microscopically, all 13 cases were remarkably similar. Immunoreactivity in the small cell component included vimentin in 100% and cytokeratin and glial fibrillary acidic protein in 25% of cases. S-100 immunoreactivity was noted in the cartilaginous component of 100% of cases, and in rare cells in the small cell component along the interface. Flow cytometry of the eight tumors studied revealed a diploid pattern in six, aneuploidy in two, and a wide range of S-phase fractions (0-36.5%).
CONCLUSIONS
Review of the literature and the findings of the current series indicates that mesenchymal chondrosarcomas presenting in the brain and spinal cord pursue a progressive course that correlates most reliably with extent of surgical resection. This limited retrospective study also suggests that survival may be shorter for those patients with a high S-phase fraction and a high Ki-67 staining frequency.
Topics: Adolescent; Adult; Aged; Aneuploidy; Antigens, Neoplasm; Brain Neoplasms; Child; Chondrosarcoma, Mesenchymal; DNA, Neoplasm; Diploidy; Female; Flow Cytometry; Follow-Up Studies; Glial Fibrillary Acidic Protein; Humans; Immunohistochemistry; Keratins; Ki-67 Antigen; Male; Middle Aged; Neoplasm Proteins; Nuclear Proteins; Prognosis; Retrospective Studies; S Phase; S100 Proteins; Spinal Cord Neoplasms; Survival Rate; Vimentin
PubMed: 8646689
DOI: 10.1002/(SICI)1097-0142(19960501)77:9<1884::AID-CNCR19>3.0.CO;2-W -
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 -
The Journal of Laryngology and Otology Jul 2000Mesenchymal chondrosarcoma (MC) is a rare tumour, with a predilection for the head and neck region. We describe a case of mesenchymal chondrosarcoma arising in the right... (Review)
Review
Mesenchymal chondrosarcoma (MC) is a rare tumour, with a predilection for the head and neck region. We describe a case of mesenchymal chondrosarcoma arising in the right maxilla extending to the basi-sphenoid. Its computed tomography (CT) and magnetic resonance imaging (MRI) and histopathological features and the management are presented. We also reviewed the literature of reported cases involving the maxilla.
Topics: Adolescent; Bone Neoplasms; Chondrosarcoma, Mesenchymal; Female; Humans; Magnetic Resonance Imaging; Maxillary Sinus Neoplasms; Tomography, X-Ray Computed
PubMed: 10992938
DOI: 10.1258/0022215001906084 -
Skeletal Radiology May 2018
Topics: Bone Neoplasms; Chondrosarcoma; Chondrosarcoma, Mesenchymal; Humans; Tibia
PubMed: 29397433
DOI: 10.1007/s00256-018-2885-9 -
The Laryngoscope May 2003Mesenchymal chondrosarcoma of the sinonasal tract is a rare, malignant tumor of extraskeletal origin. Isolated cases have been reported in the English literature, with... (Review)
Review
OBJECTIVES/HYPOTHESIS
Mesenchymal chondrosarcoma of the sinonasal tract is a rare, malignant tumor of extraskeletal origin. Isolated cases have been reported in the English literature, with no large series evaluating the clinicopathological aspects of these tumors.
STUDY DESIGN
Retrospective review.
METHODS
Thirteen patients with sinonasal mesenchymal chondrosarcoma were retrieved from the Otorhinolaryngologic-Head and Neck Registry of the Armed Forces Institute of Pathology.
RESULTS
Nine women and 4 men (age range, 11 to 83 y; mean age, 38.8 y) presented with nasal obstruction (n = 8), epistaxis (n = 7), or mass effect (n = 4), or a combination of these. No patients reported prior head and neck irradiation. The maxillary sinus was the most common site of involvement (n = 9), followed by the ethmoid sinuses (n = 7) and the nasal cavity (n = 5). Tumors had an overall mean size of 5.1 cm. Microscopically, the tumors displayed a small, blue, round cell morphology appearance arranged in a hemangiopericytoma-like pattern with foci of cartilaginous matrix. All cases were managed by surgery with adjuvant radiation therapy (n = 4) and/or chemotherapy (n = 3). The overall mean survival was 12.1 years, although five of six patients who developed local recurrences died of disease (mean survival, 6.5 y). Six patients were alive and disease free (mean survival, 17.3 y), and two patients were lost to follow-up.
CONCLUSIONS
Mesenchymal chondrosarcoma of the sinonasal tract is an aggressive tumor with a predilection for young women. The pattern of growth and scarcity of cartilaginous matrix result in frequent misdiagnosis. Recurrence develops in approximately one-third of patients and seems to predict a poor prognosis. Aggressive, exenterative surgery combined with adjuvant therapy appears to yield the best clinical outcome.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Chondrosarcoma, Mesenchymal; Disease-Free Survival; Female; Humans; Infant, Newborn; Male; Middle Aged; Neoplasm Staging; Paranasal Sinus Neoplasms; Retrospective Studies; Tomography, X-Ray Computed
PubMed: 12792311
DOI: 10.1097/00005537-200305000-00004