Surgical Pathology Criteria

Clear Cell Sarcoma

Differential Diagnosis

The first five entities are either melanocytic or HMB45 positive and thus may be confused with clear cell sarcoma

Melanoma Clear Cell Sarcoma
Evidence of skin primary Deep origin
Nuclear pleomorphism frequent Nuclear pleomorphism infrequent
Mitoses frequently numerous Mitoses usually infrequent
t(11;12) not present t(11;12) frequent
CD117 65% CD117 negative


Cellular Blue Nevus Clear Cell Sarcoma
Usually on trunk Usually on extremities
Usually superficial Involves tendons and deep fascia


Malignant Blue Nevus Clear Cell Sarcoma
Usually on scalp Usually on extremeties
Superficial Deep
Frequently associated with benign blue nevus No association with blue nevus


PEComa Clear Cell Sarcoma
S100 30% S100 80%
Actin 90% Actin negative


Paraganglioma-like Dermal Melanocytic Tumor Clear Cell Sarcoma
Based in dermis Deep soft tissue and tendons
Nucleoli not prominent Prominent large nucleoli
Lacks necrosis Frequent necrosis


Epithelioid Leiomyosarcoma Clear Cell Sarcoma
No irregular nests Usually irregular nests
HMB45 negative HMB45 80% positive
Actin, desmin positive Actin, desmin negative


Malignant Peripiheral Nerve Sheath Tumor Clear Cell Sarcoma
Associated with nerve, neurofibroma or von Recklinghausen disease No such association
Melanin and HMB45 rare Melanin and HMB45 80% positive
Frequently pleomorphic Pleomorphism infrequent
Mitoses usually frequent Mitoses usually infrequent


Synovial Sarcoma Clear Cell Sarcoma
Dense nuclei with stippled chromatin Vesicular nuclei with prominent nucleoli
Keratin 50-80% positive Keratin negative (one report of 30% positive)
Melanin and HMB45 negative Melanin and HMB45 positive 80%
Calcification common Calcification uncommon
SYT-SSX gene fusion present t(11;12) frequent
TLE1 97% TLE1 14%


Paraganglioma Clear Cell Sarcoma
Salt and pepper chromatin Vesicular chromatin with nucleoli
Chromogranin, synaptophysin positive HMB45 positive 80%, MelanA positive 40%
S100+ sustentacular cells No sustentacular cells

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