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Extraskeletal Myxoid Chondrosarcoma

Extraskeletal Myxoid Chondrosarcoma. An overview from a clinical and basic science perspective 1. Clinical case and overview. Robert Maki, Memorial Sloan-Kettering Cancer Center, New York, NY. Case presentation.

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Extraskeletal Myxoid Chondrosarcoma

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  1. Extraskeletal Myxoid Chondrosarcoma An overview from a clinical and basic science perspective 1. Clinical case and overview Robert Maki, Memorial Sloan-Kettering Cancer Center, New York, NY

  2. Case presentation • 64 M with a two-year history of a >10 cm para-testicular perineal mass causing discomfort when sitting • Hypertension only pertinent medical history, no significant family, social history. • Exam, ultrasound → Complex cystic and solid mass. • 01 / 2000: Resected with radical orchiectomy with portions of left corpus cavernosum and pubis (15 cm tumor, 1300 gram specimen).

  3. Case, continued. • Dx: EMC, t(9;22) positive, margins grossly positive, with lung metastases noted • 04/2000: Local tumor recurred, complicated by cutaneous fistula to urethra that slowly healed • Mass grew further, mostly in perineum

  4. CT 1/19/2000

  5. 4/12/00 CT of recurrence site

  6. EMC 4/26/00

  7. CT 8/16/00

  8. Case, continued. • 9/00-11/00: doxorubicin 75 mg/m2 x 3 cycles – no change • Declined further systemic therapy • Later agreed to thalidomide up to 300 mg a day but he had no benefit • Asymptomatic despite increasing size of lung metastases for the remainder of his life

  9. 2/20/01

  10. 5/31/02

  11. Patient history, conclusion • 07/02: Motor vehicle accident: found with CNS lesions, (largest 4.1 cm parietal mass): biopsy = glioblastoma multiforme • Therapy: whole brain XRT, glucocorticoids; patient declined an operation • Died of GBM 03/03

  12. EMC: Extraskeletal Myxoid Chondrosarcoma • Rare, perhaps 1-2% of sarcomas • First described in 1953 by Stout and Verner, more clearly defined by Enzinger and Shiraki in 1972 • M : F ~ 2 : 1, peak incidence ~ age 50 • Thigh and popliteal fossa most common sites (like myxoid liposarcoma) • Somewhat similar to myxoid liposarcoma microscopically • Prolonged course with local recurrences and late metastases

  13. EMC • Largest study 117 pts, 6-89 yrs old (median 52), median size 7 cm (1-25 cm) • 80% proximal extremities, limb girdles • 99 cases with follow up, median 9 yrs • 48 NED, 23 AWD, 18 DOD • Local recurrence in 48% (40/83), mets in 46% (35/76 patients) • Grading not of prognostic value in this series • Microscopically identical tumor appears in bone, but in bone it has no specific translocation and lower risk of metastasis Meis-Kindblom JM et al. Am J Surg Path 1999; 23:636 Antonescu C et al. Cancer 1998; 83:1504

  14. Extraskeletal myxoid (chondrosarcoma)? • Not chondroid at all: • Cells most frequently do not show chondroid differentiation • Cells are S100 negative, typically • EM: cells not particularly chondrocytic, but more primitive • Collagen expression • Little collagen II, no collagen X, like chondrocytes have • More typically collagens I, III, VI positive, like fibroblasts • May represent a primitive mesenchymal precursor • Rules out relationship to mesenchymal chondrosarcoma, standard chondrosarcoma, and chordoma Aigner T et al. Mod Pathol 2004; 17: 214

  15. 3 typical microscopic views of EMC Meis-Kindblom JM et al Am J Surg Path 1999; 23:636

  16. Differential pathologic diagnosis • mixed myoepithelial tumors • chondroid lipoma • myxoid liposarcoma • myxofibrosarcoma • fibromyxoid sarcoma • myxoid variants of ossifying fibromyxoid tumor • of soft parts • myxoid sclerosing epithelioid fibrosarcoma

  17. Local and metastatic risk, overall survival Local recurrence risk Risk of metastases 60% Overall Survival at 15 years, ?disease specific Overall survival Meis-Kindblom JM et al. Am J Surg Path 1999; 23:636

  18. Therapy • Surgery, ?radiation • Chemotherapy appears entirely ineffective • MD Anderson experience: 11 cases, 30 yrs • 10 cm median size, 10/11 received chemotherapy, median 4 cycles • Doxorubicin-based • Dacarbazine-based • Three received ifosfamide, no response • One patient in Winnipeg, MB received IFN-alfa-2b with a response Patel SR et al. Am J Clin Oncol 1995; 18:161 Rubinger M et al. Chest 1995; 108:281

  19. EMC typical translocation t(9;22)

  20. EMC translocation • t(9;22) EWS-TEC • TEC also called NR4A3, NOR1, MINOR, CHN • Seen in 75% of EMC • Fusion protein links trans-activation domain of EWS to entire TEC protein, an orphan nuclear steroid/thyroid receptor gene superfamily member • Less common: t(9;17)(q22;q11) TAF2N-TEC (15%), rare t(9;15) TCF12-TEC.

  21. EMC Therapy • Given the lack of viable options, one hopes that understanding of the molecular events associated with EMC will lead to better therapy

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