• Less aggressive than similar bone tumor
• Usually adult extremities; also children and trunk
• Metastases to lung

Thigh tumor

Fallopian tube tumor

• Usually myxoid
• Cords of small cells with acidophilic cytoplasm, occasional vacuoles, usually in myxoid stroma
• Usually no obvious chondrocytes

Fallopian tube tumor

• S100, Leu7/CD57, lysozyme, glycogen, acid mucins

• Keratin

• Well developed endoplasmic reticulum, cytoplasmic filaments, glycogen

• t(9;22)(q22-31;q11-12): CHN-EWS fusion gene, is consistent marker for extraskeletal myxoid chondrosarcoma (Mod Pathol 1995;8:765)

• Rare
• Primitive appearance

• Orbit, dura, trunk, retroperitoneum, extremities, kidney
• Poor prognosis

• 30 year old man with dizziness, headache and imbalance (Neurol India 2012;60:121)
• 34 year old man with expansile, erosive tumor of mandible (J Cancer Res Ther 2011;7:192)
• 52 year old man with progressive proptosis of eye (Case Rep Med 2012;2012:292147)
• 64 year old man with renal mass (Diagn Pathol 2012;7:125)

Mandible tumor

Primary orbital tumor

Kidney tumor

• Clusters of undifferentiated small blue cells often with hemangiopericytoma-like appearance mixed with islands of mature-appearing hyaline cartilage

Renal tumor

Ureteric tumor

Abdominal mass

Primary orbital tumor

Pleural tumor

Mandible tumor

• Carilagenous areas: S100, mesenchymal cells CD99

• Robertsonian translocation der(13;21)(q10;q10) found in skeletal and extraskeletal cases (Mod Pathol 2002;15:572)

G-banded karyotype #1

#2

SKY karyotype

• Rare, first described in 1972
• 50-65% males, median age 50-52 years, range 6-89 years
• 80% in proximal extremities or limb girdles, 20% in trunk
• Local recurrence in 48%, metastases in 46%
• Survival of 90% at 5 years, 70-78% at 10 years (Mod Pathol 2000;13:900)

• Adverse prognostic factors: older age, larger tumor size (>10 cm), proximal extremity / limb girdle location, anaplastic cytology (Am J Surg Pathol 1999;23:636)

• 27 year old woman with tumor of broad ligament and fallopian tube (Indian J Pathol Microbiol 2012;55:80)
• 41 year old man with thigh mass (Oncol Lett 2012;3:621)
• 51 year old woman with lung mass (Diagn Pathol 2012;7:112)
• 51 year old man with swelling in thigh (J Cytol 2009;26:36)
• Tumor with neuroendocrine features and t(9;17)(q22;q11.2), representing fusion of CHN and RBP56 genes (Am J Surg Pathol 2000;24:1020)

• Median 7-10 cm (range 1-25 cm) in deep subcutaneous or deeper soft tissue

Fallopian tube nodule

Thigh tumor

• Anastamosing cords of neoplastic cells in a myxoid matrix
• Most have low cellularity
• May have focal hypercellular areas and > 2 mitotic figures / 10 HPF
• May have rhabdoid features if INI1 negative

Thigh tumor

Uterine adnexa

Various images

Asymptomatic lung mass with severe anemia

Thigh tumor

• Vimentin (90%), S100 (17-50%), synaptophysin (22-72%), EMA (0-28%)

• AE1/AE3, CAM5.2, EMA
• Often INI1 (Am J Surg Pathol 2008;32:1168)

• EWSR1 translocation in 46% (Am J Surg Pathol 2008;32:8)
• Often involves t(9;22)(q31;q12) EWSR1-NR4A3; EWS-CHN or TAF2N-CHN fusion gene transcripts (Hum Pathol 2001;32:1116)

This information is intended for physicians and related personnel, who understand that medical information is often imperfect, and must be interpreted in the context of a patient's clinical data using reasonable medical judgment. This website should not be used as a substitute for the advice of a licensed physician.

All information on this website is protected by copyright of PathologyOutlines.com, Inc. Information from third parties may also be protected by copyright. Please contact us at copyrightPathOut@gmail.com with any questions (click here for other contact information).