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Mesenchymal Chondrosarcoma

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Latest Research Publications

This list of publications is regularly updated (Source: PubMed).

De Amorim Bernstein K, Liebsch N, Chen YL, et al.
Clinical outcomes for patients after surgery and radiation therapy for mesenchymal chondrosarcomas.
J Surg Oncol. 2016; 114(8):982-986 [PubMed] Related Publications
INTRODUCTION: We report the outcome of 23 patients with mesenchymal chondrosarcomas treated with surgery and radiation therapy +/- chemotherapy. The intent of the project was to review the impact of patient and treatment variables on treatment outcome, in particular with regard to extent of surgery and radiation dose.
PATIENTS AND METHODS: Twenty-three patients with mesenchymal chondrosarcomas were treated with surgery and radiation therapy (min. dose 44 Gy; max. dose 78 Gy; median dose 60 Gy; mean dose 61 Gy).
RESULTS: The median survival for the entire cohort of patients was 21.65 years (95% confidence interval ± 4.25). The 5- and 10-year OS was 78.6%. Median disease-free survival for the 23 patients was 7.2 years. Disease-free survival (DFS) at 3 and 5 years was 70.7% and 57.8%, respectively. The local control rate at 5 and 10 years was 89.5% (95%CI 64.1-97.3%). Only three patients experienced local failure, three patients had regional failure, and eight developed distant metastases.
CONCLUSIONS: In this cohort of patients local tumor control was high when using a combination of surgery and radiation. There was not a clear relationship between radiation dose and local tumor control. J. Surg. Oncol. 2016;114:982-986. © 2016 Wiley Periodicals, Inc.

Uppaluri SA, Yin LH, Goh GH
Maxillary mesenchymal chondrosarcoma presenting with epistaxis in a child.
J Radiol Case Rep. 2015; 9(8):33-8 [PubMed] Free Access to Full Article Related Publications
Mesenchymal chondrosarcomas are a rare variant of primary chondrosarcomas and can pose a diagnostic dilemma, especially when the features on conventional imaging are equivocal for an aggressive lesion. There is very little PET-CT experience in mesenchymal chondrosarcomas as per the literature and to the best of our knowledge, we are the first to describe a maxillary mesenchymal chondrosarcoma on PET-CT imaging. We report a case where PET-CT not only complemented conventional imaging in suspecting a malignant osseous lesion, but also was indicative of the grade of the tumor.

Kabra RS, Patel SB, Shanbhag SS
Orbital Chondroma: A rare mesenchymal tumor of orbit.
Indian J Ophthalmol. 2015; 63(6):551-4 [PubMed] Free Access to Full Article Related Publications
While relatively common in the skeletal system, cartilaginous tumors are rarely seen originating from the orbit. Here, we report a rare case of an orbital chondroma. A 27-year-old male patient presented with a painless hard mass in the superonasal quadrant (SNQ) of left orbit since 3 months. On examination, best-corrected visual acuity of both eyes was 20/20, with normal anterior and posterior segment with full movements of eyeballs and normal intraocular pressure. Computerized tomography scan revealed well defined soft tissue density lesion in SNQ of left orbit. Patient was operated for anteromedial orbitotomy under general anesthesia. Mass was excised intact and sent for histopathological examination (HPE). HPE report showed lobular aggregates of benign cartilaginous cells with mild atypia suggesting of benign cartilaginous tumor - chondroma. Very few cases of orbital chondroma have been reported in literature so far.

Guo J, Gu Y, Guo L, et al.
A Case of Mesenchymal Chondrosarcoma Arising from the Femoral Vein with 8 Years of Follow-up.
Ann Vasc Surg. 2015; 29(7):1455.e1-5 [PubMed] Related Publications
Mesenchymal chondrosarcoma (MCS) is an infrequent malignancy of bone and soft tissue that is characterized by a peculiar bimorphic histologic pattern with areas of undifferentiated malignant small cells surrounding well-differentiated cartilaginous islands. Involvement of the large vessels is a rare occurrence. Here, we report a case of MCS arising from the femoral vein that was treated by wide-margin resection combined with autogenous vein revascularization and then followed up for 8 years. The long-term postoperative results showed distant metastasis to the pancreas and both lobes of the lung, without recurrence at the primary site. This case indicates that for MCS arising from the femoral vein, although wide-margin resection combined with autogenous vein revascularization may avoid recurrence at the primary site, this treatment strategy has no obvious benefit for controlling long-term distant metastases.

Sajjad EA, Sikora K, Paciejewski T, et al.
Intraparenchymal mesenchymal chondrosarcoma of the frontal lobe--a case report and molecular detection of specific gene fusions from archival FFPE sample.
Clin Neuropathol. 2015 Sep-Oct; 34(5):288-93 [PubMed] Related Publications
Mesenchymal chondrosarcoma is a rare tumor of cartilaginous origin characterized by its bimorphic pattern composed of highly undifferentiated small round cells separated by islands of well-differentiated hyaline cartilage. It exhibits higher malignancy and earlier occurrence in comparison to classic chondrosarcomas. Recently identified HEY1-NCOA2 and IRF2BP2-CDX1 gene fusions confirm their distinct molecular origin and pose a promising diagnostic marker. The majority of cases arise from craniofacial bones. In this study, we present a rare case of mesenchymal chondrosarcoma encompassed within the brain parenchyma of the frontal lobe without any dural or bone attachment. We demonstrate histopathological findings and confirm the HEY1-NCOA2 gene fusion in a formalin-fixed paraffin-embedded archival sample using simple reverse transcription polymerase chain reaction (RT-PCR) method. IRF2BP2-CDX1 gene fusion was absent in the analyzed sample. The clinical follow-up is also presented with a review of treatment modalities for this entity.

Xu J, Li D, Xie L, et al.
Mesenchymal chondrosarcoma of bone and soft tissue: a systematic review of 107 patients in the past 20 years.
PLoS One. 2015; 10(4):e0122216 [PubMed] Free Access to Full Article Related Publications
BACKGROUND: Mesenchymal chondrosarcoma(MCS) is a rare high-grade variant of chondrosarcoma. Consensus has not been reached on its optimal management. Resection with wide margins is usually recommended, but the effect of margins has been demonstrated by little positive evidence. Moreover, the effectiveness of adjuvant chemo- and/or radiotherapy remains controversial.
OBJECTIVES: To describe the clinical characteristics and outcomes of MCS of bone and soft tissue, to assess the efficacies of surgery, chemotherapy and radiation, and finally to deliver a more appropriate therapy.
MATERIALS AND METHODS: We reviewed EMBASE-, MEDLINE-, Cochrane-, Ovid- and PubMed-based to find out all cases of MCS of bone and soft tissue described between April 1994 and April 2014. Description of treatment and regular follow-up was required for each study. Language was restricted to English and Chinese. Issues of age, gender, location, metastasis, and treatment were all evaluated for each case. Kaplan-Meier Method and Cox Proportional Hazard Regression Model were used in the survival analysis.
RESULTS: From the 630 identified publications, 18 meeting the inclusion criteria were selected, involving a total of 107 patients. Based on these data, the 5-, 10-and 20-year overall survival are 55.0%, 43.5% and 15.7% respectively. The 5-, 10-, 20- year event-free survival rates are 45.0%, 27.2% and 8.1%, respectively. Treatment without surgery is associated with poorer overall survival and event-free survival. Negative surgical margins could significantly bring down the local-recurrence rate and are associated with a higher event-free survival rate. Chemotherapy regime based on anthracyclines does not benefit the overall survival. The addition of radiation therapy is not significantly associated with the overall or event-free survival. However, we recommend radiation as the salvage therapy for patients with positive margin so as to achieve better local control.
CONCLUSIONS: This review shows that surgery is essential in the management of MCS of bone and soft tissue. Appropriate adjuvant therapy may reduce local recurrence, but cannot benefit the overall survival.

Kim WS, Jittreetat T, Nam W, et al.
Reconstruction of the segmental mandibular defect using a retroauricular or modified face-lift incision with an intraoral approach in head and neck cancer.
Acta Otolaryngol. 2015; 135(5):500-6 [PubMed] Related Publications
CONCLUSIONS: This is the first report of mandibular reconstruction using the retroauricular (RA) or the modified face-lift (MFL) approach in head and neck cancer. This approach may have advantages over the conventional approach, especially in its superior aesthetic results.
OBJECTIVE: The fibular osseous or osteocutaneous free flap is a widely accepted option for the reconstruction of mandibular defects. Recently, we devised an RA or an MFL approach for neck dissection (ND) using an endoscopic or robotic surgical system. Here, we performed the reconstruction of a segmental mandibular defect with a fibular free flap using the RA or the MFL approach.
METHODS: A total of five patients underwent mandibular reconstruction with the RA or MFL approach for mandibular discontinuity, which developed after the surgical extirpation of head and neck cancer. We performed ND, segmental mandibulectomy, and the reconstruction of the mandibular defect via RA or MFL incisions.
RESULTS: An osseous free flap was used for the reconstruction in two patients and the osteocutaneous free flap was used in three patients. The mean operation times for mandibulectomy and ND were 82 (range 45-120) min and 156 (range 140-180) min, respectively. No significant complications were noted. All flaps survived successfully.

Rothberg MB, Bhalodi AA, Reda EF, et al.
Primary renal mesenchymal chondrosarcoma: a case report.
Urology. 2015; 85(3):676-8 [PubMed] Related Publications
A 16-year-old female patient presented to our emergency department with a left upper extremity radiculopathy prompting several imaging studies. Magnetic resonance imaging of the neck revealed multiple cervical vertebrae lesions and computed tomography imaging demonstrated a 15-cm calcified mass originating from the left kidney and extending into the surrounding structures. Pathologic assessment of the open left radical nephrectomy specimen revealed a primary renal mesenchymal chondrosarcoma, a tumor that has only been documented in 5 prior case reports in the literature.

Frezza AM, Cesari M, Baumhoer D, et al.
Mesenchymal chondrosarcoma: prognostic factors and outcome in 113 patients. A European Musculoskeletal Oncology Society study.
Eur J Cancer. 2015; 51(3):374-81 [PubMed] Related Publications
BACKGROUND: Mesenchymal chondrosarcoma (MCS) is a distinct, very rare sarcoma with little evidence supporting treatment recommendations.
PATIENTS AND METHODS: Specialist centres collaborated to report prognostic factors and outcome for 113 patients.
RESULTS: Median age was 30 years (range: 11-80), male/female ratio 1.1. Primary sites were extremities (40%), trunk (47%) and head and neck (13%), 41 arising primarily in soft tissue. Seventeen patients had metastases at diagnosis. Mean follow-up was 14.9 years (range: 1-34), median overall survival (OS) 17 years (95% confidence interval (CI): 10.3-28.6). Ninety-five of 96 patients with localised disease underwent surgery, 54 additionally received combination chemotherapy. Sixty-five of 95 patients are alive and 45 progression-free (5 local recurrence, 34 distant metastases, 11 combined). Median progression-free survival (PFS) and OS were 7 (95% CI: 3.03-10.96) and 20 (95% CI: 12.63-27.36) years respectively. Chemotherapy administration in patients with localised disease was associated with reduced risk of recurrence (P=0.046; hazard ratio (HR)=0.482 95% CI: 0.213-0.996) and death (P=0.004; HR=0.445 95% CI: 0.256-0.774). Clear resection margins predicted less frequent local recurrence (2% versus 27%; P=0.002). Primary site and origin did not influence survival. The absence of metastases at diagnosis was associated with a significantly better outcome (P<0.0001). Data on radiotherapy indications, dose and fractionation were insufficiently complete, to allow comment of its impact on outcomes. Median OS for patients with metastases at presentation was 3 years (95% CI: 0-4.25).
CONCLUSIONS: Prognosis in MCS varies considerably. Metastatic disease at diagnosis has the strongest impact on survival. Complete resection and adjuvant chemotherapy should be considered as standard of care for localised disease.

Gherman V, Tomuleasa C, Bungardean C, et al.
Management of renal extraskeletal mesenchymal chondrosarcoma.
BMC Surg. 2014; 14:107 [PubMed] Free Access to Full Article Related Publications
BACKGROUND: Primary mesenchymal chondrosarcoma of the kidney is an extremely rare malignant tumor. To our best knowledge, only 9 such cases have been reported so far.
CASE PRESENTATION: In the current paper, we present the case of a 67 year-old patient with recurrent left lumbar pain, increased fatigability and intermittent macroscopic hematuria. He underwent a surgical resection of the left kidney and left hemicolon.The pathological diagnosis was primary extraskeletal renal mesenchymal chondrosarcoma. Overall survival was 9 months, with pulmonary metastasis and local recurrence at 6 months. The management of the patient is described, from the initial differential diagnosis, after the first clinical examination to the surgical resection, with a special emphasis on the surgical procedures that were carried out.
CONCLUSION: Extraskeletal chondrosarcoma of primary origin in the kidney are extremely rare tumors with a highly malignant potential and very poor prognosis. Because the role of chemotherapy or radiation therapy has not been evaluated properly yet, we underline the importance of surgery in the management of such cases as the main and best approach to achieve clinical remission and long-term survival, provided the patient is referred to a surgical consult in time.

Ortiz S, Tortosa F, Sobrinho Simões M
An extraordinary case of mesenchymal chondrosarcoma metastasis in the thyroid.
Endocr Pathol. 2015; 26(1):33-6 [PubMed] Related Publications
Secondary tumours of the thyroid gland account for 1.25 to 3 % in clinical series and reach 24 % in autopsy series. Chondrosarcoma is a rare malignant mesenchymal tumour of chondrogenic nature; the mesenchymal variant represents less than 3 % of all chondrosarcomas, being therefore extremely rare. A mesenchymal chondrosarcoma metastasis in the thyroid is exceptional; to our knowledge, only three previous cases of chondrosarcoma metastasis in the thyroid have been reported to date but none of such cases corresponded to a mesenchymal chondrosarcoma. We present the first of such a case in a 27-year-old woman with a 4-year history of mesenchymal chondrosarcoma of the sacrum that was treated by surgery and chemotherapy. At the present admission, head and neck computed tomography revealed a well-defined nodule in the thyroid gland. The diagnosis of metastasis from the mesenchymal chondrosarcoma was made in the right lobectomy specimen.

Moriya K, Katayama S, Onuma M, et al.
Mesenchymal chondrosarcoma diagnosed on FISH for HEY1-NCOA2 fusion gene.
Pediatr Int. 2014; 56(5):e55-7 [PubMed] Related Publications
Mesenchymal chondrosarcoma (MC) is an extremely rare subtype of chondrosarcoma that has a small round-cell sarcoma with focal cartilaginous differentiation, often with a pericytomatous vascular pattern. The non-cartilaginous components are usually dominant, and such lesions might be confused with other small round-cell tumors. Recently, a tumor-specific HEY1-NCOA2 fusion gene was identified in MC. Here we report the case of a 9-year-old boy who was diagnosed with MC by detection of HEY1-NCOA2 fusion signals in almost 50% of tumor cells in tissue sections on fluorescence in situ hybridization (FISH). In this way, the tumor was definitively diagnosed as MC. This case suggests that the detection of the HEY1-NCOA2 fusion gene on FISH is of diagnostic value for MC.

Singh RK, Varshney S, Bist SS, et al.
Mesenchymal chondrosarcoma of the mandible: a rare malignant tumor.
Ear Nose Throat J. 2014; 93(9):E18-21 [PubMed] Related Publications
Mesenchymal chondrosarcoma (MCHS) is a highly aggressive, malignant, cartilaginous tumor that represents about 1% of all chondrosarcomas. Only about 50 cases of MCHS of the jaw have been reported in the English-language literature; maxillary tumors are much more common than mandibular tumors. We present a new case of MCHS of the mandible that arose in a 22-year-old woman whose initial two biopsies did not reveal the features of the tumor. The diagnosis was made only after an incisional biopsy.

Escobar E, Nguyen BD, Colvin OC
PET/CT and MRI of chondroid lipoma of the deltoid muscle.
Clin Nucl Med. 2014; 39(11):984-7 [PubMed] Related Publications
Chondroid lipoma is an uncommon benign tumor of mesenchymal origin characterized by the presence of adipose tissue and chondroid matrix. Its imaging features have been reported with radiography, ultrasonography, CT, and MRI. Because this lesion may be mistaken histologically for myxoid liposarcoma and myxoid chondrosarcoma, it is of value, through this presentation, to raise the awareness of this benign tumor, which may demonstrate prominent 18F-FDG avidity on PET/CT imaging. The authors present a case of a 52-year-old male patient with a 6-month history of painless chondroid lipoma of the left shoulder.

Xiao A, Li Z, He X, You C
A rare tentorial mesenchymal chondrosarcoma in posterior cranial fossa: case report.
Neurol Neurochir Pol. 2014; 48(4):287-91 [PubMed] Related Publications
Intracranial extraskeletal mesenchymal chondrosarcoma is a very rare malignant tumor with predilection site of frontoparietal falx cerebri. Only few cases of mesenchymal chondrosarcoma in posterior cranial fossa are reported. Here, we report a 23-year-old young man with a dura-attached mass in left posterior cranial fossa misdiagnosed as a tentorial meningioma preoperatively. According to the following operation, the lesion was confirmed as mesenchymal chondrosarcoma surgically and pathologically. On MRI, the tumor was characterized by lobulated soft-tissue mass with dura-attached base, patchy calcifications and heterogeneous signal intensities. On contrast-enhanced MRI, it was well-defined, with marked enhancement. We consider that these imaging features above might remind us to consider the diagnosis of mesenchymal chondrosarcoma in posterior cranial fossa. The postoperative treatment of radiotherapy is still controversial. As for our case, according to the 24 months follow-up after postoperative γ-knife, our patient shows an optimistic prognosis so far.

Panagopoulos I, Gorunova L, Bjerkehagen B, et al.
Chromosome aberrations and HEY1-NCOA2 fusion gene in a mesenchymal chondrosarcoma.
Oncol Rep. 2014; 32(1):40-4 [PubMed] Free Access to Full Article Related Publications
Mesenchymal chondrosarcomas are fast-growing tumors that account for 2-10% of primary chondrosarcomas. Cytogenetic information is restricted to 12 cases that did not show a specific aberration pattern. Recently, two fusion genes were described in mesenchymal chondrosarcomas: a recurrent HEY1-NCOA2 found in tumors that had not been cytogenetically characterized and an IRF2BP2-CDX1 found in a tumor carrying a t(1;5)(q42;q32) translocation as the sole chromosomal abnormality. Here, we present the cytogenetic and molecular genetic analysis of a mesenchymal chondrosarcoma in which the patient had two histologically indistinguishable tumor lesions, one in the neck and one in the thigh. An abnormal clone with the G-banding karyotype 46,XX,add(6)(q23),add(8)(p23),del(10)(p11),+12,-15[6] was found in the neck tumor whereas a normal karyotype, 46,XX, was found in the tumor of the thigh. RT-PCR and Sanger sequencing showed that exon 4 of HEY1 was fused to exon 13 of NCOA2 in the sample from the thigh lesion; we did not have spare material to perform a similar analysis of the neck tumor. Examining the published karyotypes we observed numerical or structural aberrations of chromosome 8 in the majority of the karyotyped mesenchymal chondrosarcomas. Chromosome 8 was also structurally affected in the present study. The pathogenetic mechanisms behind this nonrandom involvement are unknown, but the presence on 8q of two genes, HEY1 and NCOA2, now known to be involved in mesenchymal chondrosarcoma tumorigenesis is, of course, suggestive.

Namini FT, Raisolsadat SM, Ghafarzadegan K, Ashkezari A
Congenital sacral mesenchymal chondrosarcoma in a neonate: a case report and review of literature.
Afr J Paediatr Surg. 2014 Jan-Mar; 11(1):87-90 [PubMed] Related Publications
Mesenchymal chondrosarcomas are rare malignant tumours in children, especially, in neonates. The authors present a case of congenital mesenchymal chondrosarcoma in a 1-day neonate located in sacrum. According to the authors' literature searches, this case is the first congenital sacral mesenchymal chondrosarcoma. We also reviewed the papers published in English literatures.

Fritchie KJ, Jin L, Ruano A, et al.
Are meningeal hemangiopericytoma and mesenchymal chondrosarcoma the same?: a study of HEY1-NCOA2 fusion.
Am J Clin Pathol. 2013; 140(5):670-4 [PubMed] Related Publications
OBJECTIVES: Meningeal hemangiopericytoma (HPC) and mesenchymal chondrosarcoma are aggressive neoplasms with a propensity to involve the meninges and dura. In addition to similar clinical presentations, both meningeal HPC and mesenchymal chondrosarcoma share overlapping morphologic features, including ovoid cells, variable collagen deposition, and a branching vascular pattern. Recently, a novel HEY1-NCOA2 fusion was reported as a recurrent event in mesenchymal chondrosarcomas.
METHODS: Thirteen mesenchymal chondrosarcomas and 18 meningeal HPCs were identified from surgical pathology archives, and the tumors were evaluated for HEY1-NCOA2 fusion with reverse transcriptase-polymerase chain reaction (RT-PCR).
RESULTS: HEY1-NCOA2 fusion transcript was detected in all six cases of mesenchymal chondrosarcoma but in none of the meningeal HPC cases (0/11) that were evaluable with RT-PCR.
CONCLUSIONS: These results show that (1) meningeal HPC and mesenchymal chondrosarcoma are distinct at the molecular level, and (2) the identification of HEY1-NCOA2 can be used as an auxiliary diagnostic tool to differentiate these entities.

Lee E, Lee HY, Choe G, et al.
Extraskeletal intraspinal mesenchymal chondrosarcoma; 18F-FDG PET/CT finding.
Clin Nucl Med. 2014; 39(1):e64-6 [PubMed] Related Publications
Mesenchymal chondrosarcoma is a rare and aggressive form of chondrosarcoma. The extraskeletal intraspinal type is even rare among the mesenchymal chondrosarcoma cases. We presented a case of a 17-year-old boy pathologically diagnosed with intraspinal mesenchymal chondrosarcoma. MRI showed multiple intradural extramedullary masses with contrast enhancement, without the evidence of brain lesion. On F-FDG PET/CT, hypermetabolism was observed in the lesions matched with enhancement on spine MRI. The lesions were well differentiated from spinal cord. In the case of hypermetabolic lesion in intradural and extramedullary lesion of the spinal cord, mesenchymal chondrosarcoma should be considered for the differential diagnosis.

Kawaguchi S, Weiss I, Lin PP, et al.
Radiation therapy is associated with fewer recurrences in mesenchymal chondrosarcoma.
Clin Orthop Relat Res. 2014; 472(3):856-64 [PubMed] Free Access to Full Article Related Publications
BACKGROUND: Mesenchymal chondrosarcoma (MSC) is a rare variant of chondrosarcoma. Because of the rarity of the disease, most studies only contain a small number of patients and thus the prognostic variables and role of adjuvant therapies remain controversial.
QUESTIONS/PURPOSES: We therefore asked (1) what the overall and disease-free survival were for patients with this diagnosis at 5 and 10 years; (2) whether there were significant prognostic factors associated with survival; and (3) whether use of adjuvant chemotherapy or radiotherapy was associated with survival in patients with MSC.
METHODS: We retrospectively reviewed the cases of MSC diagnosed from 1979 to 2010 at one referral center. Forty-three cases were identified. Thirty-seven cases were analyzed for demographics, treatments, and outcomes. Thirty patients with localized disease were analyzed for prognostic factors. The minimum followup was 1 month (mean, 6 years; range, 1 month to 17 years). There were 17 females and 20 males. The mean age at diagnosis was 33 years (range, 11-65 years). Nineteen cases were skeletal and 18 cases were extraskeletal. Seventy-six percent of the tumors were located in the trunk.
RESULTS: Five- and 10-year overall survival was 51% and 37%, respectively. Five- and 10-year disease-free survival was 23% and 5%, respectively. Age (< 30 years) and male sex were associated with poorer overall and disease-free survival in patients presenting with a localized tumor, respectively. Patients who did not receive radiotherapy were more likely to have a local recurrence. Adjuvant chemotherapy failed to show a significant association with overall, disease-free, metastasis-free, or local recurrence-free survival.
CONCLUSIONS: The present study reinforced the role of adjuvant radiotherapy for local tumor control.
LEVEL OF EVIDENCE: Level IV, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence.

Thakar S, Dadlani R, Furtado SV, et al.
Extensive anterior skull-base mesenchymal chondrosarcoma: unusual cause of multiple indolent masses on the forehead.
Clin Neuropathol. 2013 Sep-Oct; 32(5):393-8 [PubMed] Related Publications
Primary intracranial occurrence of an extraskeletal mesenchymal chondrosarcoma (MC) is unusual. The commonly involved sites are the orbit, clivus and temporo-occipital junction. Occurrence of the lesion in the anterior skull-base (ASB) in an infiltrative manner and with extra-calvarial involvement, is anecdotal. We report the case of a 35-year-old woman who presented with two indolent swellings on the forehead for a duration of 1 year. Examination revealed impaired visual acuity and complete external ophthalmoplegia in the right eye and 5 × 6 cm and 2 × 3 cm sized hard masses on the forehead. CT and MRI revealed a large, intensely enhancing ASB mass with extensions into the right orbit, ethmoid sinus, nasal cavity, and anteriorly, into the subcutaneous tissue of the frontal scalp through erosions in the bone. At surgery via a right frontal recraniotomy, the lesion was found to be firm and very vascular. Owing to its extreme vascularity, decompression was limited to excision of the intracranial and extra-calvarial components of the lesion. Histopathology was consistent with the diagnosis of MC. The patient was advised a second stage surgery for excision of the residual lesion. She, however, opted for radiation therapy and was lost to follow-up. This report, with one of the most extensive ASB MCs described to date, adds to the list of rare differentials of indolent forehead masses in the diagnostic armamentarium of the neuropathologist.

Tsai SY, Chang KC, Tsai HW, D D S YT
Ectomesenchymal chondromyxoid tumor of tongue.
Indian J Pathol Microbiol. 2012 Oct-Dec; 55(4):519-20 [PubMed] Related Publications
Ectomesenchymal chondromyxoid tumor (ECMT) is a rare entity of the dorsal tongue first described in 1995. Herein, we report a rare case of lingual ECMT in a 41-year-old man. Patient presented with an asymptomatic, small nodule (0.5 cm in diameter) in the anterior tongue. The pathological findings showed uni-lobular proliferation of fusiform cells, arranged in net-like sheets or swirls, in a chondromyxoid background. The tumor cells were immunoreactive for S-100 and glial fibrillary acidic protein (GFAP), but negative for epithelial markers. Familiarity with this entity helps pathologists make a correct diagnosis.

van Oosterwijk JG, Meijer D, van Ruler MA, et al.
Screening for potential targets for therapy in mesenchymal, clear cell, and dedifferentiated chondrosarcoma reveals Bcl-2 family members and TGFβ as potential targets.
Am J Pathol. 2013; 182(4):1347-56 [PubMed] Related Publications
The mesenchymal, clear cell, and dedifferentiated chondrosarcoma subtypes are extremely rare, together constituting 10% to 15% of all chondrosarcomas. Their poor prognosis and lack of efficacious treatment emphasizes the need to elucidate the pathways playing a pivotal role in these tumors. We constructed tissue microarrays containing 42 dedifferentiated, 23 clear cell, and 23 mesenchymal chondrosarcomas and performed immunohistochemistry to study the expression of growth plate-signaling molecules and molecules shown to be involved in conventional chondrosarcoma. We observed high expression of SOX-9 and FGFR-3, as well as aberrant cellular localization of heparan sulfate proteoglycans, in all subtypes. TGFβ signaling through p-SMAD2 and PAI-1 was highly active in all chondrosarcoma subtypes, which suggests that TGFβ inhibitors as a possible therapeutic strategy in rare chondrosarcoma subtypes. As in conventional chondrosarcoma, antiapoptotic proteins (Bcl-2, and/or Bcl-xl) were highly expressed in all subtypes. Inhibition with the BH-3 mimetic ABT-737 rendered dedifferentiated chondrosarcoma cell lines sensitive to doxorubicin or cisplatin. Our data indicate that antiapoptotic proteins may play an important role in chemoresistance, suggesting a promising role for targeting Bcl-2 family members in chondrosarcoma treatment, irrespective of the subtype.

Nakayama R, Miura Y, Ogino J, et al.
Detection of HEY1-NCOA2 fusion by fluorescence in-situ hybridization in formalin-fixed paraffin-embedded tissues as a possible diagnostic tool for mesenchymal chondrosarcoma.
Pathol Int. 2012; 62(12):823-6 [PubMed] Related Publications
Mesenchymal chondrosarcoma (MC) is an extremely rare subtype of chondrosarcoma. A tumor specific fusion gene, HEY1-NCOA2 fusion, was recently identified in this tumor. The finding raises the possibility that the diagnosis of MC can be improved by examining the fusion gene. In the present study, we aimed to evaluate the efficacy of fluorescence in situ hybridization (FISH) in detecting HEY1-NCOA2 fusion for the diagnosis of MC. Specimens from 10 patients diagnosed with MC were used for the study. Dual-color FISH was performed using two different probes that specifically hybridize to HEY1 and NCOA2, respectively. Fusion signals were identified in all but two specimens, in which no signal was detected, presumably because of inadequate sample preparation. In accordance with results of a previous study, FISH analysis was highly sensitive in detecting HEY1-NCOA2 fusion in adequately prepared MC samples. The current study adds further support for the use of HEY1-NCOA2 fusion as a valid diagnostic marker for MC.

Mei B, Lai YL, He GJ, et al.
Giant primary mesenchymal chondrosarcoma of the lung: case report and review of literature.
Ann Thorac Cardiovasc Surg. 2013; 19(6):481-4 [PubMed] Related Publications
Mesenchymal chondrosarcoma, a rare malignant tumor, was predominantly occurring in the bone and may involve somatic soft tissue but it is extremely rare in the lung.We report the case of a 20-year-old female who presented with a 2-month history of irritant nonproductive cough and chest pain. The histopathologic examination revealed the tumor composed of atypical undifferentiated small cells and islands of matured chondroid matrix typically presented as bimorphic appearances. Immunohistochemical examination revealed that the tumor cells were positive for vimentin and CD99 for all components, and to S-100 limited to the areas of cartilage. In addition, previously reported cases of primary lung mesenchymal chondrosarcoma were reviewed, and the relevant clinical knowledge regarding its clinical manifestations, diagnosis, and treatment were discussed.

Nyquist KB, Panagopoulos I, Thorsen J, et al.
Whole-transcriptome sequencing identifies novel IRF2BP2-CDX1 fusion gene brought about by translocation t(1;5)(q42;q32) in mesenchymal chondrosarcoma.
PLoS One. 2012; 7(11):e49705 [PubMed] Free Access to Full Article Related Publications
Mesenchymal chondrosarcomas (MCs) account for 3-10% of primary chondrosarcomas. The cytogenetic literature includes only ten such tumours with karyotypic information and no specific aberrations have been identified. Using a purely molecular genetic approach a HEY1-NCOA2 fusion gene was recently detected in 10 of 15 investigated MCs. The fusion probably arises through intrachromosomal rearrangement of chromosome arm 8 q. We report a new case of MC showing a t(1;5)(q42;q32) as the sole karyotypic aberration. Through FISH and whole transcriptome sequencing analysis we found a novel fusion between the IRF2BP2 gene and the transcription factor CDX1 gene arising from the translocation. The IRF2BP2-CDX1 has not formerly been described in human neoplasia. In our hospital's archives three more cases of MC were found, and we examined them looking for the supposedly more common HEY1-NCOA2 fusion, finding it in all three tumours but not in the case showing t(1;5) and IRF2BP2-CDX1 gene fusion. This demonstrates that genetic heterogeneity exists in mesenchymal chondrosarcoma.

Hanakita S, Kawai K, Shibahara J, et al.
Mesenchymal chondrosarcoma of the orbit--case report.
Neurol Med Chir (Tokyo). 2012; 52(10):747-50 [PubMed] Related Publications
A 20-year-old woman presented with a rare case of intraorbital mesenchymal chondrosarcoma manifesting as a 6-month history of progressive ptosis and exophthalmos of her left eye. Computed tomography and magnetic resonance imaging revealed a partially calcified round mass occupying the postbulbar space. Partial removal of the tumor via a left fronto-orbital approach was performed. The histological diagnosis was mesenchymal chondrosarcoma, and additional intraorbital exenteration was performed. Neither chemotherapy nor radiotherapy was performed. She was free from tumor recurrence at the 6-year follow-up examination. Radical resection, including exenteration if possible, is recommended for intraorbital mesenchymal chondrosarcoma.

Xu H, Shao M, Sun H, Li S
Primary mesenchymal chondrosarcoma of the kidney with synchronous implant and infiltrating urothelial carcinoma of the ureter.
Diagn Pathol. 2012; 7:125 [PubMed] Free Access to Full Article Related Publications
UNLABELLED: Primary mesenchymal chondrosarcoma of the kidney is rare, and it shows distinct undifferentiated tumor cells and well differentiated cartilagenous components. Also assident infiltrating urothelial carcinoma of the ureter is an extremely rare cancer. We report a case of primary mesenchymal chondrosarcoma occurring in the left kidney with an ipsilateral and distinct distal ureteric implant, and a coexisting infiltrating urothelial carcinoma of the ureter in a 64-year-old man. Histopathological examination and immunohistochemical studuies showed the classic features of mesenchymal chondrosarcoma in kidney, as well as a few infiltrating urothelial in ureter. Multitarget fluorescence in situ hybridization (FISH) suggested that the development of the urothelial carcinoma in the ureter may be triggered or induced by the chondrosarcoma component. The patient died 2 month after left nephro-ureterectomy. This is the first reported case of a primary mesenchymal chondrosarcoma of the kidney with coexisting infiltrating urothelial carcinoma of the ureter.
VIRTUAL SLIDES: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1522835667751019.

Kan Z, Li H, Zhang J, You C
Intracranial mesenchymal chondrosarcoma: case report and literature review.
Br J Neurosurg. 2012; 26(6):912-4 [PubMed] Related Publications
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.

Chen Y, Wang X, Guo L, et al.
Radiological features and pathology of extraskeletal mesenchymal chondrosarcoma.
Clin Imaging. 2012 Jul-Aug; 36(4):365-70 [PubMed] Related Publications
OBJECTIVE: The objective of this study was to elucidate the imaging and pathological features of extraskeletal mesenchymal chondrosarcomas (EMCs).
METHODS: Imaging findings of eight EMC cases were retrospectively analyzed.
RESULTS: Soft tissue masses with different patterns of mineralization were found in five cases on computed tomographic scans. On magnetic resonance images, peripherally located EMCs demonstrated mixed signal intensity on T2-weighted images and heterogeneous enhancement with both calcified and noncalcified areas.
CONCLUSION: EMCs exhibited several characteristic imaging features, which when used in combination with the mineralization pattern, enhancement of the calcified area, and signal intensity feature might have diagnostic value for this rare tumor.

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