Extraskeletal myxoid chondrosarcoma is a malignant cartilage tumor that rarely presents in the joint space. [ncbi.nlm.nih.gov]

• Fishing

  The third case had intact SMARCB1 expression and showed instead a rearrangement of the EWSR1 gene detected by FISH, with an EWSR1-CREB1 gene fusion on NGS. None of the cases showed rearrangement of the NR4A3 gene, neither by FISH nor by NGS. [zora.uzh.ch]

  There was no evidence of an EWSR1 rearrangement by dual-color break-apart fluorescence in situ hybridization (FISH). Dual-color FISH probes revealed fusion of NR4A3 and FUS, a member of the TET family of genes, which includes EWSR1 and TAF15. [ncbi.nlm.nih.gov]

  Methods: We characterized the cytogenetic rearrangements of 25 comprehensive EMC tumors by RT-PCR and/or fluorescence in situ hybridization (FISH). [cancerres.aacrjournals.org]

• Pathologist

  Histologic diagnosis is difficult and close co-operation among clinician, radiologist and pathologist is recommended. [ncbi.nlm.nih.gov]

  The pathologist evaluated the specimen in the hospital where the procedure took place and then sent out the specimen for further evaluation to the Johns Hopkins pathology department. [podiatrytoday.com]

  None of these tumors display the distinctive cytological features of the cells of EMC; this aids the pathologist in confidently making a diagnosis of EMC. [jcytol.org]

• Asymptomatic

  A 40-year-old man presented with an asymptomatic mass at the left ischial fossa. Magnetic resonance imaging showed a 9x6-cm mass with variable signal intensity and cystic formations. [ncbi.nlm.nih.gov]

  Following surgery, the patient remained asymptomatic and reported that his pre-operative symptoms had abated. No adjuvant therapy was recommended. He remained asymptomatic 12 months after his initial surgery. [pagepress.org]

  Extraskeletal Myxoid Chondrosarcoma signs and symptoms include: In the initial growing phase of the tumors, they are normally asymptomatic The thigh, knee, buttock, and upper extremities, account for up to 80% of all EMC occurrences. [dovemed.com]

• Surgical Procedure

  […] from the symptoms and reduce blood loss, during a surgical procedure In case the tumor has metastasized into the lymph nodes, a surgery may be then followed by chemotherapy When EMC is at an inaccessible location, or is unsafe for surgical intervention [dovemed.com]

  Specialized imaging techniques may be used to help evaluate the size, placement, and extension of the tumor and to serve as an aid for future surgical procedures, among individuals with mesenchymal chondrosarcoma. [rarediseases.org]

• Vomiting

  Overall, toxicity was as expected, with ≥G2 neutropenia in 60% of patients, and nausea, vomiting, asthenia, fever and mucositis as the main non-haematologic toxicities. [clinicalsarcomaresearch.biomedcentral.com]

  We have reviewed literature and have summarized diagnosis and treatment options. 2 Case report In March 2015, a 41-year-old woman presented with a sudden headache associated with vomiting of gastric contents in the afternoon and was immediately sent to [journals.lww.com]

• Nausea

  Overall, toxicity was as expected, with ≥G2 neutropenia in 60% of patients, and nausea, vomiting, asthenia, fever and mucositis as the main non-haematologic toxicities. [clinicalsarcomaresearch.biomedcentral.com]

• Aggressive Behavior

  The primary extraskeletal myxoid chondrosarcoma of bone seems to have a more aggressive behavior than the soft tissue counterpart. [moh-it.pure.elsevier.com]

  These indicate more aggressive behavior, requiring a closer follow-up of the patient [10]. In summary, ESMC is an uncommon but distinct soft tissue sarcoma, clearly different from conventional skeletal chondrosarcoma. [jbstjournal.com]

  The presence of any of these adverse prognostic factors can indicate the possibility of a more aggressive behavior in extraskeletal myxoid chondrosarcoma, and a closer follow-up is suggested. [nature.com]

• Confusion

  EMC has rarely been reported in the bone and may be confused with conventional chondrosarcoma with myxoid features or various small round cell sarcomas. We present 5 cases of molecularly confirmed EMC arising primarily in the bone. [ncbi.nlm.nih.gov]

  The low cellularity and abundant myxoid matrix associated with EMC may cause diagnostic confusion with certain benign lesions such as myxoma and nodular fasciitis. [pagepress.org]

  Chondrosarcoma is a rare cartilaginous neoplasm, accounting for [17] Although the name implies that its origin is from the cartilage, there is no clear evidence for the chondroid lineage of the malignant cells. [18] There is still confusion about the [journals.lww.com]

  Chondrosarcoma should not be confused with osteosarcoma, also called osteogenic sarcoma. Osteosarcoma is a malignant tumor arising from bone cells, (not from cartilage) and is the most common primary bone cancer. [sarcomahelp.org]

• Asthenia

  Overall, toxicity was as expected, with ≥G2 neutropenia in 60% of patients, and nausea, vomiting, asthenia, fever and mucositis as the main non-haematologic toxicities. [clinicalsarcomaresearch.biomedcentral.com]

This case shows the importance of searching for antineural antibodies in oncologic patients with new neurologic deficits, and of having a judicious workup for occult malignancies in patients with known antineural antibodies. [ncbi.nlm.nih.gov]

A thorough workup should be done to rule out other metastatic foci, including imaging of chest given the frequency of lung metastasis. Although EMC is a rare tumor, it should be included in the differential for extra skeletal soft tissue masses. [lungindia.com]

[…] of the lumbosacral plexus or the sciatic or femoral nerves, with pelvic lesions near a neurovascular bundle Limitation of joint range of motion and disturbance of joint function, with chondrosarcomas close to a joint Pathologic fracture Diagnosis The workup [emedicine.medscape.com]

• Multiple Pulmonary Nodules

  The patient was a 25-year-old woman who presented with multiple pulmonary nodules on her bilateral lungs on the annual chest roentgenograph. Simultaneously, a swelling mass on her subcutaneous inguinal region was observed. [ncbi.nlm.nih.gov]

  A computed tomography scan of the chest showed multiple pulmonary nodules. A computed tomography–guided core needle biopsy was consistent with low-grade extraskeletal myxoid chondrosarcoma. [healio.com]

Spontaneous regression of tumors is very unusual and is defined as a partial or complete disappearance of metastatic tumors without any treatment. This phenomenon has been reported in almost all types of cancer. [ncbi.nlm.nih.gov]

The tumour has a better prognosis than myxoid chondrosarcoma of the bone but surgical resection may be difficult due to its gelatinous nature. [ncbi.nlm.nih.gov]

Prognosis Frequent local recurrence; late metastases; 5-year, 10-year, 15-year survival, 90%, 70%, 60% respectively; continued follow-up is necessary. [medical-dictionary.thefreedictionary.com]

(Etiology) The exact cause and mechanism of Extraskeletal Myxoid Chondrosarcoma formation is unknown They are thought to occur as a consequence of genetic defects that take place due to certain irregular chromosomal exchanges What are the Signs and Symptoms [dovemed.com]

Epidemiology Male:female ratio, 2:1. DiffDx Intramuscular myxoma, myxoid liposarcoma, fibromyxoid sarcoma, myxoid embryonal rhabdomyosarcoma. Differentiation Cartilaginous, neuroendocrine, neural. [medical-dictionary.thefreedictionary.com]

Epidemiology Extraskeletal myxoid chondrosarcoma is a rare tumor, encompassing 2.3% of soft tissue sarcomas in a Japanese series. Mean ages reported in various series range from 46 to 57 years. Males are affected about twice as often as females. [atlasgeneticsoncology.org]

NR4A3 rearrangement Terminology NR4A3 rearranged myxoid sarcoma (provisional) ICD coding ICD-O: 9231/3 - Extraskeletal myxoid chondrosarcoma ICD-11: 2B5F.2 & XH9344 - Sarcoma, not elsewhere classified of other specific sites and myxoid chondrosarcoma Epidemiology [pathologyoutlines.com]

[2] Grade and stage are independent predictors of survival. [2] Clinical/epidemiologic features: [3] Usually arise in a (benign) abnormality of cartilage (e.g. osteochondroma, enchondroma). [librepathology.org]

[…] classified of other specific sites and myxoid chondrosarcoma Epidemiology Rare, accounting for < 1% of soft tissue sarcomas Usually occurs in adults, mainly in the fourth to seventh decades (Mod Pathol 2018;31:744) M:F = ~2:1 (Mod Pathol 2018;31:744) Pathophysiology [pathologyoutlines.com]

Pathophysiology Chondrosarcomas may be divided into primary and secondary lesions on the basis of their origins. [5] Primary chondrosarcomas arise de novo, whereas secondary chondrosarcomas arise from preexisting lesions of the cartilage. [emedicine.medscape.com]

Current medical research has not established a way of preventing Extraskeletal Myxoid Chondrosarcoma Genetic testing of the expecting parents (and related family members) and prenatal diagnosis (molecular testing of the fetus during pregnancy) may help [dovemed.com]

Clinical Trial Resources ESMO Clinical Trial Resources are designed to support you with designing, participating in and reviewing clinical trials Educational Slides Educational slides on Cancer Prevention, Clinical Trials, Colorectal Cancer and Molecular [oncologypro.esmo.org]

Postoperative therapy is also quite important to prevent recurrence and progression of the intracranial EMC. [journals.lww.com]