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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

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
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).
case continued
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
slide6
EMC

4/26/00

case continued8
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
patient history conclusion
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
emc extraskeletal myxoid chondrosarcoma
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
slide13
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

extraskeletal myxoid chondrosarcoma14
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

3 typical microscopic views of emc
3 typical microscopic views of EMC

Meis-Kindblom JM et al Am J Surg Path 1999; 23:636

differential pathologic diagnosis
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
local and metastatic risk overall survival
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

therapy
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

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