scholarly journals Extraskeletal Chondrosarcoma of Labium Majus

2011 ◽  
Vol 2011 ◽  
pp. 1-3 ◽  
Author(s):  
Arshad S. Khan ◽  
Girish D. Bakhshi ◽  
Aftab Shaikh ◽  
Ashraf A. Khan ◽  
Adil A. Khan ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Adjuvant Therapy ◽  
Indian Subcontinent ◽  
Rare Tumor ◽  
Extraskeletal Myxoid Chondrosarcoma ◽  
Postoperative Course ◽  
The Third ◽  
Myxoid Chondrosarcoma ◽  
Soft Tissue Swelling ◽  
Labium Majus

Extraskeletal myxoid chondrosarcoma (ESMC) is a rare tumor seen more often in men. It is seen to arise from soft tissue of lower extremity or buttocks. We report a case of soft tissue swelling of left labium majus in a 66-year-old female. Patient underwent wide excision with uneventful postoperative course. Histopathology of specimen confirmed it to be ESMC. Patient refused adjuvant therapy. Followup of 1 year has shown her to be disease- and symptom- free. Only two cases arising from vulva have been reported in literature . This is the third case and first from Indian subcontinent. A brief review of clinical features, diagnosis, treatment, and outcome of patients with extraskeletal chondrosarcoma is presented.

scholarly journals Primary Extraskeletal Myxoid Chondrosarcoma: A Case Report

2021 ◽  
Author(s):  
Renuka Bangalore Nagaraj ◽  
Arshiya Sultana
Keyword(s):  
Case Report ◽  
Soft Tissue ◽  
Histopathological Examination ◽  
Matrix Material ◽  
Needle Aspiration ◽  
Extraskeletal Myxoid Chondrosarcoma ◽  
Soft Tissue Neoplasm ◽  
Rare Tumour ◽  
Myxoid Chondrosarcoma ◽  
Malignant Soft Tissue

Extraskeletal Myxoid Chondrosarcoma (EMC) is a rare malignant soft tissue sarcoma with uncertain differentiation, most often seen in males. The incidence is 3% of all soft tissue tumours with limited literature available on its cytological features. EMC may arise from lower extremities, upper extremities, retroperitoneum, pelvis, and buttocks. This case report was an effort to understand the role of Fine Needle Aspiration Cytology (FNAC), histopathology and immunochemistry in the diagnosis of EMC. Authors hereby report a case of a 70-year-old male patient with slow-growing soft tissue swelling on the back just behind the right shoulder who was referred for FNAC. Patient complete history and clinical findings were recorded. Radiological images were suggestive of malignant soft tissue neoplasm with no involvement of underlining bone. FNAC reveled tumour cells which appeared monotonous and they were seen in a myxoid stroma background. Subsequently, the excised lesion was sent for histopathological examination and the report revealed the presence of abundant chondromyxoid matrix material within which were found numerous elongated spindly shaped cells. These cells had moderately pleomorphic elongated nuclei with focal solid fibrocollagenous areas along intersecting fascicles of the moderately pleomorphic spindly cell. Few of these cells had multilobulated bizarre nuclei with nuclear inclusions. Immunohistochemical stains showed diffuse positivity for S-100, vimentin, and focally positive for Epithelial Membrane Antigen (EMA). The FNAC, histopathology and immunohistochemical features confirm the diagnosis of EMC on right shoulder. It’s a rare tumour whose diagnosis is made depending on history, clinical location, growth pattern, histopathology, and immunohistochemistry.

scholarly journals Extraskeletal Myxoid Chondrosarcoma: State of the Art and Current Research on Biology and Clinical Management

Cancers ◽  
2020 ◽  
Vol 12 (9) ◽  
pp. 2703 ◽  
Author(s):  
Silvia Stacchiotti ◽  
Giacomo Giulio Baldi ◽  
Carlo Morosi ◽  
Alessandro Gronchi ◽  
Roberta Maestro
Keyword(s):  
Soft Tissue ◽  
State Of The Art ◽  
Chimeric Proteins ◽  
Antiangiogenic Agents ◽  
Extraskeletal Myxoid Chondrosarcoma ◽  
Pharmacological Treatments ◽  
Myxoid Chondrosarcoma ◽  
Mesenchymal Neoplasm ◽  
Localized Disease ◽  
Specific Contribution

Extraskeletal myxoid chondrosarcoma (EMC) is an ultra-rare mesenchymal neoplasm with uncertain differentiation, which arises mostly in the deep soft tissue of proximal extremities and limb girdles. EMC is marked by a translocation involving the NR4A3 gene, which can be fused in-frame with different partners, most often EWSR1 or TAF1. Although EMC biology is still poorly defined, recent studies have started shedding light on the specific contribution of NR4A3 chimeric proteins to EMC pathogenesis and clinical outcome. Standard treatment for localized disease is surgery, plus or minus radiation therapy with an expected prolonged survival even though the risk of relapse is about 50%. In advanced cases, besides the standard chemotherapy currently used for soft tissue sarcoma, antiangiogenic agents have recently shown promising activity. The aim of this review is to provide the state of the art of treatment for localized and advanced disease, with a focus on pharmacological treatments available for EMC. The biological basis of current research and future perspectives will be also discussed.

scholarly journals NR4A3 rearrangement reliably distinguishes between the clinicopathologically overlapping entities myoepithelial carcinoma of soft tissue and cellular extraskeletal myxoid chondrosarcoma

2012 ◽  
Vol 460 (6) ◽  
pp. 621-628 ◽  
Author(s):  
Uta Flucke ◽  
Bastiaan B. J. Tops ◽  
Marian A. J. Verdijk ◽  
Patricia J. H. van Cleef ◽  
Peter H. van Zwam ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Myoepithelial Carcinoma ◽  
Extraskeletal Myxoid Chondrosarcoma ◽  
Myxoid Chondrosarcoma

scholarly journals Extraskeletal myxoid chondrosarcoma of thigh: a rare case report

2021 ◽  
Vol 9 (6) ◽  
pp. 1778
Author(s):  
Pinal C. Shah ◽  
R. N. Hathilal ◽  
Sheetal Sheth ◽  
Smita S. Gavit
Keyword(s):  
Soft Tissue ◽  
Needle Aspiration ◽  
Soft Tissue Tumors ◽  
Extraskeletal Myxoid Chondrosarcoma ◽  
Morphological Examination ◽  
Soft Tissue Neoplasm ◽  
Myxoid Chondrosarcoma ◽  
Neoplastic Lesion ◽  
Rare Case Report ◽  
Needle Aspiration Cytology

Extraskeletal myxoid chondrosarcoma (EMC) are a rare entity of soft tissue tumors that occur predominantly in soft tissue of lower extremities. Here we present a case of 45-year-old female presented with left thigh swelling. MRI finding suggested primary neoplastic lesion. Fine needle aspiration cytology (FNAC) suggested myxoid soft tissue neoplasm/sarcoma. Morphological examination revealed typical extraskeletal myxoid chondrosarcoma with strong immunoreactivity for vimentin and focal immunoreactivity for epithelial membrane antigen (EMA).

scholarly journals Locally recurrent extraskeletal myxoid chondrosarcoma of the shoulder: a case of complete neoadjuvant radiotherapy response

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Luca Improta ◽  
Sergio Valeri ◽  
Rossana Alloni ◽  
Chiara Pagnoni ◽  
Francesco Mallozzi Santa Maria ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Local Recurrence ◽  
Distant Metastasis ◽  
Surgical Excision ◽  
Lower Limbs ◽  
Extraskeletal Myxoid Chondrosarcoma ◽  
Combined Treatments ◽  
Referral Center ◽  
Myxoid Chondrosarcoma

Abstract Background Extraskeletal myxoid chondrosarcoma (EMC) is a rare soft tissue tumor that typically affects the lower limbs of men between the ages of 50 and 60. EMC of the shoulder is rare with a high risk of local recurrence and distant metastasis. A planned surgical excision in sarcoma referral centers (SRCs) is mandatory to obtain the best outcome. The role of chemotherapy (CHT) and Radiotherapy (RT) on soft tissue chondrosarcoma is still controversial. Case presentation A 47-year-old man presented to our referral center with a history of EMC in the right shoulder excised with microscopic positive surgical margins in a non-referral center. Staging imaging exams did not reveal distant metastasis or residual disease, but during follow-up a local recurrence was detected. After a multidisciplinary discussion, preoperative radiotherapy was administered with a total dose of 50 Gy, and then the patient underwent wide surgical excision. Histological examination was negative for viable tumor cells. No relapse occurred in a 24-months post-operative follow up. Conclusions The case here described suggests the importance of patient’s management in SRCs. A planned combined treatments with both surgery and RT seems to be the best choice to improve local control. RT seems to be promising within this specific histotype. Further studies are needed to confirm if the observed efficacy of combined treatments reflects in a consistent survival benefit for EMC patients.

scholarly journals A LOW GRADE TUMOR, EXTRA SKELETAL MYXOID CHONDROSARCOMA, WITH METASTATIC POTENTIAL: A CASE REPORT

2019 ◽  
Vol 3 (7) ◽  
Author(s):  
Sushil Kumar Shukla ◽  
Viney Kumar ◽  
Smita Chandra
Keyword(s):  
Soft Tissue ◽  
Soft Tissues ◽  
Histopathological Examination ◽  
Specific Treatment ◽  
Metastatic Potential ◽  
Low Grade ◽  
Extraskeletal Myxoid Chondrosarcoma ◽  
Soft Tissue Neoplasm ◽  
Myxoid Chondrosarcoma ◽  
Myxoid Matrix

Extraskeletal Myxoid Chondrosarcoma (EMC) is a rare soft tissue sarcoma of uncertain differentiation characterized by abundant myxoid matrix located in the soft tissues. It affects mainly the soft tissues of the proximal end of long bones. EMC has a male preference, and this occurs in soft tissue area in patients who are more than 40 years old. The present case was 63 year old female diagnosed as EMC on histopathological examination with immuno–histochemical confirmation and after eight months presented with pulmonary and inguinal metastasis. EMC is a rare tumor should be considered in the differential diagnosis of myxoid soft tissue neoplasm. Therefore, a multi-modal approach, having distinct clinical, cytological, histo-pathological, immunohistochemical features and cytogenetics analysis, must be necessary in establishing a more definitive diagnosis, which may finally lead to a more targeted and specific treatment for patients.         Keywords: Extraskeletal myxoid chondrosarcoma, Inguinal metastasis, low grade tumor, IHC

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