Extraskeletal Myxoid Chondrosarcoma

Summary
Description

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.

People and Age
This tumor is more common in males by 2:1 and average age is in the 4th decade.
Symptoms and Presentation
This tumor may have a indolent presentation. The tumor may be palpable as a mass.
Brief description of the xray
Standard xrays do not show this tumor. MRI is needed.
Brief desc of tx
Wide surgical removal is the best treatment.
Tumor Name
Extraskeletal myxoid chondrosarcoma
Tumor Type
Tumors of Cartilage
Benign or Malignant
Malignant
Body region
pelvis - entire
knee, thigh, distal femur, proximal tibia
elbow and forearm, distal humerus
ankle and foot, leg
Most Common Bones
soft tissue
Tumor behavior
aggressive
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.