Extraskeletal myxoid chondrosarcoma

Kawaguchi, Satoshi; Wada, Takuro; Nagoya, Satoshi; Ikeda, Tatsuru; Isu, Kazuo; Yamashiro, Katsushige; Kawai, Akira; Ishii, Takeshi; Araki, Nobuhito; Myoui, Akira; Matsumoto, Seiichi; Umeda, T; Yoshikawa, Hideki; Hasegawa, Tadashi

doi:10.1002/cncr.11162

Cited by 99 publications

(49 citation statements)

References 27 publications

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“…Patients with EMC follow a protracted clinical course with a relatively high incidence of distant spread, typically to the lung,[15] and poor response rates to most conventional chemotherapeutic agents. [16] In one of the larger clinicopathologic studies to date including 42 patients with EMC, the overall survival was 100% at 5 years and 69% at 10 years.…”

Section: Discussionmentioning

confidence: 99%

“…[16] In one of the larger clinicopathologic studies to date including 42 patients with EMC, the overall survival was 100% at 5 years and 69% at 10 years. [15] The correlation between histologic grade in EMC and behavior has been controversial. Most cases of EMC are predominantly myxoid, with low cellularity and classified as low grade tumors.…”

Section: Discussionmentioning

confidence: 99%

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Extraskeletal myxoid chondrosarcoma with non–EWSR1-NR4A3 variant fusions correlate with rhabdoid phenotype and high-grade morphology

et al. 2014

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Extraskeletal myxoid chondrosarcomas (EMC) are rare soft tissue sarcomas with distinctive histology and uncertain histogenesis, characterized by EWSR1-NR4A3 fusion in 75% of the cases. A smaller proportion of cases show NR4A3 fused to other gene partners including TAF15, TCF12 and TFG. The impact of various gene fusions on morphology and outcome has not been previously evaluated. We investigated 26 consecutive EMCs and correlated the genetic findings with morphology and clinical outcome. There were 5 females and 21 males (median age of 49.5 years). Mean size of the tumors was 11 cm. FISH analysis showed EWSR1-NR4A3 gene fusion in 16 (62%) cases; TAF15-NR4A3 gene fusion in 7 (27%) cases and TCF12-NR4A3 gene fusion in one (4%) case. Two cases showed only NR4A3 gene rearrangements. Morphologically, most EWSR1-rearranged tumors (10 of 16) showed low cellularity, minimal cytologic atypia and low mitotic counts. In contrast, 80% of EMCs with variant (non-EWSR1) NR4A3 gene fusions (TAF15, TCF12) had high grade morphology with increased cellularity, proliferation and cytologic atypia, showing a plasmacytoid / rhabdoid morphology in half the cases. Follow-up showed that only 1 of 16 patients with EWSR1-rearranged tumors died of disease, in contrast to 3 of 7 (43%) TAF15–rearranged tumors. In conclusion, EMCs with variant NR4A3 gene fusions show a higher incidence of rhabdoid phenotype, high grade morphology and, a more aggressive outcome compared to the EWSR1-NR4A3 positive tumors. Furthermore, FISH assay for NR4A3, along with EWSR1, may be an additional ancillary test to confirm diagnosis of EMCs.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Extraskeletal myxoid chondrosarcoma with non–EWSR1-NR4A3 variant fusions correlate with rhabdoid phenotype and high-grade morphology

et al. 2014

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“…[6] Kawaguchi et al present data to support the role of a wide local excision due to a high rate of local recurrence. [7] Chondrosarcomas are notoriously resistant to conventional chemotherapy and radiation treatments, making curative resection the only definitive cure. In advanced or metastatic tumors, recent studies with novel chemotherapy regimens have not demonstrated increased survival.…”

Section: Discussionmentioning

confidence: 99%

A rare case of extraskeletal pulmonary myxoid chondrosarcoma

Endicott

Barak

Mortman

2015

JST

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Primary malignant cartilaginous tumors of the lung are rare with limited reports in the literature. Here we describe the case of a 69-year-old male who presented with a lobulated left upper lobe mass. Preoperative percutaneous biopsy was consistent with a mucinous adenocarcinoma. Final pathology demonstrated a primary extraskeletal pulmonary myxoid chrondrosarcoma. The clinical presentation and histologic characteristics are described.

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“…Despite its high tendency to local recurrences and distant metastases, it has a relatively slow growth and a lazy clinical course [12, 13]. …”

Section: Discussionmentioning

confidence: 99%

Recurrence of Pelvic Chondrosarcoma through Fascial Defect into Abdominal Cavity

Gökkuş

Akin

Sağtaş

et al. 2014

Case Reports in Oncological Medicine

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Our patient was a 76-year-old female who has been operated on 2 times in 8 years for pelvic chondrosarcoma. The patient came to our clinic with a large mass in left iliac region which extended into the paravertebral area. Physical examination and preoperative imagining studies revealed a mass at the left iliac area that infiltrated sciatic notch and extended from posterior iliac region towards the anterior side of iliac bone through the sciatic notch and an incisional hernia including descending colon. The mass was also penetrating the abdominal cavity through the hernia. Surgical intervention was planned. Since the tumor infiltrated the sciatic nerve, hemipelvectomy was indicated. Patient refused hemipelvectomy. Therefore, palliative debulking surgery was considered. We treated the case with marginal excision and abdominal wall reconstruction employing prolene and vicryl suture materials in order to prevent a postoperative visceral herniation and local invasion. At the latest follow-up appointment in 2 years, the patient still had no signs of tumor recurrence. This case showed us that an incisional hernia can serve as a pathway for the recurrence invasion of the chondrosarcoma.

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Extraskeletal myxoid chondrosarcoma

Cited by 99 publications

References 27 publications

Extraskeletal myxoid chondrosarcoma with non–EWSR1-NR4A3 variant fusions correlate with rhabdoid phenotype and high-grade morphology

Extraskeletal myxoid chondrosarcoma with non–EWSR1-NR4A3 variant fusions correlate with rhabdoid phenotype and high-grade morphology

A rare case of extraskeletal pulmonary myxoid chondrosarcoma

Recurrence of Pelvic Chondrosarcoma through Fascial Defect into Abdominal Cavity

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