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Extraskeletal Myxoid Chondrosarcoma

Summary

Extraskeletal myxoid chondrosarcoma is an intermediate-grade neoplasm with a tendency toward recurrence and metastasis. This rare variant of chondrosarcoma occurs in the soft tissues.

This tumor is more common in males by 2:1 and average age is in the 4th decade.
This tumor may have a indolent presentation. The tumor may be palpable as a mass.
Standard xrays do not show this tumor. MRI is needed.
Wide surgical removal is the best treatment.
Complete Information on this Tumor
Introduction and Definition: 

Extraskeletal myxoid chondrosarcoma is an intermediate-grade neoplasm with a tendency toward recurrence and metastasis. This rare variant of chondrosarcoma occurs in the deep soft tissues, usually of the limb girdles or extremities, but may occur in the trunk wall, thorax, abdomen, breast, or elsewhere. An identical tumor also arises in bone. The tumor has distinct molecular features. More than 75% of cases demonstrate the EWS-CHN gene fusion. The tumor is aggressive and can metastasize.

Incidence and Demographics: 
Median age is in the 40's or 50's. Males are affected twice as often as females.
Symptoms and Presentation: 

Approximately 80% of these tumors occur in the extremities, and 20% in the trunk. The lower extremity is the most common site. Patients present with swelling, and the tumor can be quite bulky.

X-Ray Appearance and Advanced Imaging Findings: 
A large heterogeneous mass in the soft tissues without bone involvement or minimal bone involvement is seen.
Differential Diagnosis: 
Soft tissue sarcoma, periosteal chondrosarcoma
Preferred Biopsy Technique for this Tumor: 
Incisional
Histopathology findings: 
The tumor is a multilobulated mass of uniform cells that are rounded or elongated, with small nuclei and a small amount of eosinophilic cytoplasm, similar to chondroblasts.
Outcomes of Treatment and Prognosis: 
The 5-year, 10-year, and 15-year overall survival rates were 82%, 65%, and 58%.
Suggested Reading and Reference: 
Cancer. 2008 Dec 15;113(12):3364-71. Extraskeletal myxoid chondrosarcoma: a retrospective review from 2 referral centers emphasizing long-term outcomes with surgery and chemotherapy. Drilon AD, Popat S, Bhuchar G, D'Adamo DR, Keohan ML, Fisher C, Antonescu CR, Singer S, Brennan MF, Judson I, Maki RG.