Primary extraskeletal myxoid chondrosarcoma in cerebellum

Qin, You; Zhang, Haibo; Ke, Chao; Huang, Jing; Wu, Bing-Fei; Chen, Wan; Yang, Chen-Su; Yang, Kunyu

doi:10.1097/md.0000000000008684

Cited by 10 publications

(11 citation statements)

References 30 publications

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“…Our demographic analysis of locoregional myxoid leiomyosarcomas showed that most patients were older than 50 compared with locoregional myxoid liposarcomas, and locoregional myxoid chondrosarcoma patients were showing no big difference between age variables according to data reported by SEER, and these results are similar to that reported in literature revealing that myxoid leiomyosarcomas occurred mostly in the elderly [ 44 – 46 ]. Myxoid liposarcoma and myxoid chondrosarcoma tumors with locoregional metastasis have mostly male predilection [ 44 , 47 , 48 ], and the locoregional myxoid leiomyosarcoma tumor has commonly female predilection [ 49 ]. Our obtained data indicated that cases of myxoid liposarcomas and myxoid chondrosarcomas with locoregional metastasis were mostly male patients.…”

Section: Discussionmentioning

confidence: 99%

Surgery Treatment Improved the Overall Survival Rate in Locoregional Myxoid Leiomyosarcoma than Other Myxosarcomas in the United States

Almoiliqy

Al-Danakh

Safi

et al. 2021

Oxidative Medicine and Cellular Longevity

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Myxosarcomas are rare malignant tumors of soft connective tissues, classified into various subtypes, including myxoid liposarcoma, myxoid chondrosarcoma, and myxoid leiomyosarcoma. In this study, we proposed to study the demographic, tumor characteristics, and overall survival rate and compared the treatment modalities between these cancers. Patient data collected based on locoregional metastasis presentation of the abovementioned tumors with a cutoff study of survival duration up to 10 years were obtained from the SEER database during 1975-2016. Our results indicated that elderly patients and females were more in locoregional myxoid leiomyosarcoma than myxoid liposarcoma and myxoid chondrosarcoma with locoregional metastasis. The white race represented the most patients who suffered from these cancers than other races. The heart is the primary site for the abovementioned cancers, in addition to the female genitals to the myxoid leiomyosarcoma. Myxoid liposarcoma and myxoid chondrosarcoma patients with locoregional metastasis were suffering from grade II, while locoregional myxoid leiomyosarcoma patients with blank grading were due to missed data. Surgery was the most common treatment modality in this study compared with radiotherapy and chemotherapy. Kaplan-Meier analysis showed a significant difference in survival time between the three subtypes by using histology, and myxoid leiomyosarcoma showed prolonged survival than others. Elderly, female, white, unknown grade, surgery, no radiation, and no chemotherapy variables were independent factors associated with overall survival among these cancers. Multivariate analysis also showed significant differences in overall survival between the three tumors by histology, and myxoid leiomyosarcoma was with a better prognosis than others. Multivariate analysis of locoregional myxoid leiomyosarcoma showed the statistical significance of black race, grade, and radiotherapy, indicating them as independent prognostic factors of locoregional myxoid leiomyosarcoma. We conclude that surgery was the primary treatment modality against these cancers than radiotherapy and chemotherapy. And the locoregional myxoid leiomyosarcomas showed a better prognosis and higher survival rate than locoregional myxoid liposarcoma and locoregional myxoid chondrosarcoma.

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Section: Discussionmentioning

confidence: 99%

Surgery Treatment Improved the Overall Survival Rate in Locoregional Myxoid Leiomyosarcoma than Other Myxosarcomas in the United States

Almoiliqy

Al-Danakh

Safi

et al. 2021

Oxidative Medicine and Cellular Longevity

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“…IHC panel of markers such as vimentin, cytokeratin, S100 and GFAP play a vital role in histopathological diagnosis. Vimentin positivity and cytokeratin negativity were reported in most cases of primary intracranial EMC in literature [14,17,18,21]. S100 negativity helps to rule out chordoma [34].…”

Section: Discussionmentioning

confidence: 97%

“…Over past 28 years, since the first description of myxoid chondrosarcoma in 1972, there are only 17 cases of intracranial extra-skeletal myxoid chondrosarcoma (EMC) reported in literature. The intracranial sites of origin were cerebellopontine angle, cerebellum, dura, fourth ventricle, pineal gland, falx cerebri and brain parenchyma [7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23]. Among the reported cases, there was only one case of intraventricular origin, which originated from fourth ventricle [7].…”

Section: Introductionmentioning

confidence: 99%

Primary intracranial extra-skeletal myxoid chondrosarcoma of right lateral ventricle with EWSR1 gene fusion: a case report and review of literature

Selvaraj

Gudipudi

Khera

et al. 2021

ecancer

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Background:Primary intracranial malignancies with extra-skeletal myxoid chondrosarcoma (EMC) features are extremely rare. EMC constitutes a distinct genomic entity characterised by reciprocal translocation of fusion genes, most commonly EWS RNA Binding Protein 1 (EWSR1) in 22q12 with Nuclear Receptor Subfamily 4 Group A Member 3 (NR4A3) in 9q2-q31.1. It is reported to have a high propensity for local recurrence and has potential for metastasis. So far in 28 years since its first description, only 17 cases of primary intracranial EMC were reported in literature. This would be the second case of intraventricular origin and first case from lateral ventricle.Case presentation: A 27-year-old male presenting with complaints of headache, seizures and pain in neck was diagnosed to have a mass lesion in right lateral ventricle in Magnetic Resonance Imaging of brain. He underwent right parieto-occipital craniotomy with total excision of the lesion. Initial histopathological examination was reported as Ependymoma, WHO grade II. However, blocks and slides review with immunohistochemistry (IHC) markers revealed neoplastic aetiology with extensive myxoid changes. Hence, fluorescent in-situ hybridisation (FISH) testing was done with EWSR1 break apart probe, which demonstrated EWSR1 break apart signals. Therefore, correlating the clinical findings with morphology, IHC and FISH, the diagnosis of primary intracranial EMC was rendered. Patient received adjuvant external beam radiation of 54 Gy in 30 fractions to the post-op region. At 29-month follow-up, there was no evidence of disease recurrence. Conclusions:Owing to the rarity of the condition, there are no standard treatment guidelines available for primary intracranial EMC. A combined treatment approach with surgery followed by adjuvant radiotherapy provides good local control with less morbidity.

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“…Therefore the current case can rule out chordoma. EMC is an extremely rare malignant tumor showing malignant chondroblast-like cells in a background of myxoid matrix 20. It is characterized by hypocellular myxoid nodules separated by fibrous septa of variable thicknesses;21 immunohistochemistry is positive for vimentin and synaptophysin and negative for S-100 and EMA.…”

Section: Discussionmentioning

confidence: 99%

<p>Low-Grade Chondrosarcoma In The Sellar Area: Case Report And Literature Review</p>

Zhang

Pang

Wang

et al. 2019

OTT

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Low-grade chondrosarcoma (LGC) is a very rare intracranial tumor, particularly in the sellar area. Herein, we describe an unusual case of LGC occurring in the sellar area. A 52-year-old man presented with diminution of vision for more than 3 months, but did not exhibit headaches reported in previous cases. MRI showed that the maximum size of the tumor was 7 cm on the left side of the saddle. We characterized the specific pathological characteristics. Histologically, the tumor had polypoid areas and a lobulated growth pattern under low-power examination. At high magnification, the tumor consisted of small cells with hyperchromatic nuclei in the cartilage matrix, with an alternating loose hypocellular zone and rich myxoid area. In our case, LGC needed to be distinguished from chordoma. Immunohistochemically, the tumor cells showed diffuse positivity for S-100 and vimentin, IDH1 was weakly cytoplasm positive. The Ki-67 labeling index was less than 5%. Additionally, AE1/3, EMA, and CK19 were negative, which could be used to exclude chordoma. This case report expands the literature on LGC and will help clinicians and pathologists better understand this entity.

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Primary extraskeletal myxoid chondrosarcoma in cerebellum

Cited by 10 publications

References 30 publications

Surgery Treatment Improved the Overall Survival Rate in Locoregional Myxoid Leiomyosarcoma than Other Myxosarcomas in the United States

Surgery Treatment Improved the Overall Survival Rate in Locoregional Myxoid Leiomyosarcoma than Other Myxosarcomas in the United States

Primary intracranial extra-skeletal myxoid chondrosarcoma of right lateral ventricle with EWSR1 gene fusion: a case report and review of literature

<p>Low-Grade Chondrosarcoma In The Sellar Area: Case Report And Literature Review</p>

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