Extraskeletal myxoid chondrosarcoma: A high incidence of metastatic disease to lymph nodes

Abstract

Background: Extraskeletal myxoid chondrosarcoma (ESMC) is a rare type of soft-tissue sarcoma with limited series reporting outcome of treatment. Currently there is limited data on the incidence and impact on patient outcome in those with metastatic disease to lymph nodes in ESMC. Methods: Thirty (21 males, 9 females) patients, mean age 50 ± 16 years, with ESMC were reviewed. The tumors were most commonly located in the lower extremity (n = 23, 77%) and the mean tumor size and volume were 9 ± 5 cm and 490 ± 833 cm 3. Mean follow up was 7 ± 4 years. Results: Six (20%) patients either presented (n = 3, 10%) or developed (n = 3, 10%) lymph node metastatic disease. When comparing patients without, with lymph node metastasis and metastasis elsewhere, patients with lymph nodes metastasis had worse survival than those without metastasis, however better 10-year disease specific survival than those with metastasis elsewhere (100% vs 62% vs 0%; P < .001). Conclusion: There is a high incidence of lymph node metastatic disease in patients with ESMC. Although survival in these patients is worse compared to those without metastasis, their survival is better than those with metastasis elsewhere. Due to the high incidence of lymph node metastatic disease, preoperative staging of the lymph node should be considered.

Extraskeletal myxoid chondrosarcoma: A case series and review of the literature

“…This trend resembles other studies demonstrating the lungs as the primary metastatic site for EMC. 4 , 13 Contrary to previous studies, we appreciated only two patients with episodes of recurrence. One patient presented less than 50 days after incomplete excision at an outside hospital and underwent wide re-excision with free flap coverage and adjuvant radiation therapy.…”

“…These findings are consistent with the current literature which demonstrates an average age at time of presentation in the 5 th and 6 th decade and a 2:1 of males to females ( Table 1 ). 2 , 4 , 6 , 13 …”

“…The average tumor size in our study at largest dimension was 7.14 cm (range, 2.4–18.7), which resembles prior studies demonstrating mean tumor sizes ranging from 7 to 9 cm. 1 , 9 , 13 The average size of STSs at time of presentation is 10.2 cm and studies have shown large size to be a poor prognostic factor with a 3.5-fold greater risk of mortality in tumors greater than 15 cm compared to those less than 5 cm. 1 , 6 , 20 With regard to EMC specifically, Drilon et al demonstrated that size greater than 10 cm is a poor prognostic indicator for OS; however, we did not appreciate this in our cohort.…”

Metastatic extraskeletal myxoid chondrosarcoma presenting as a forehead mass

“… 4 The most common site of distant metastasis is the lungs; however, other sites include lymph nodes, bony skeleton, soft tissue, and the brain. 8 , 9 …”

“…4 The most common site of distant metastasis is the lungs; however, other sites include lymph nodes, bony skeleton, soft tissue, and the brain. 8,9 Our patient underwent surveillance with yearly chest computed tomography and nuclear bone scans after the initial diagnosis of EMC of the foot and was considered disease-free for 9 years before presenting to the dermatology clinic for a forehead mass presumed to be a lipoma. Other differential considerations for a mass on the forehead included dermoid cyst, meningocele/meningocephalocele, osteoma, fibrous dysplasia, osteoid osteoma, ossifying fibroma, giant cell tumor, aneurysmal bone cyst, neuroblastoma, lymphoma, Ewing sarcoma, osteogenic sarcoma, calcified cephalohematoma, and growing skull fracture (leptomeningeal cyst).…”