Surgical Pathology Criteria

Desmoplastic Small Cell Tumor

Differential Diagnosis

Extrarenal Rhabdoid Tumor Desmoplastic Small Cell Tumor
No desmoplastic stroma Prominent desmoplastic stroma
Desmin negative Desmin positive
INI1 loss 85% INI1 expressed 100%
Median age 8-9 years Mean age 25 years
Various deletions, translocations involving 22q11 t(11;22)(p13;q12)
Both may be rhabdoid and express keratin

 

The small cell pattern of DSCT may be confused with rhabdomyosarcoma. Both are desmin positive.
Embryonal Rhabdomyosarcoma Desmoplastic Small Cell Tumor
No desmoplastic stroma Prominent desmoplastic stroma
Keratin variable, faint and focal Keratin 90% positive, prominent
EMA negative EMA 90% positive
WT1 rare, focal WT1 positive
Muscle actin positive 95% Muscle actin rare
Median age 7 years Mean age 25 years
No consistent genetic abnormality t(11;22)(p13;q12)

 

Sclerosing Rhabdomyosarcoma Desmoplastic Small Cell Tumor
Keratin 5-50% positive, focal, weak Keratin 90% positive, prominent
EMA negative EMA 90% positive
WT1(C) negative WT1(C) positive
Any age group Mean age 25 years
No consistent genetic abnormality t(11;22)(p13;q12)
Both are desmin positive with a prominent sclerotic/desmoplastic stroma

 

The small cell pattern of DSCT may be confused with PPNET
Peripheral Primitive Neuroectodermal Tumor Desmoplastic Small Cell Tumor
CD99 positive CD99 35%
WT1 negative WT1 positive
Desmin only in ectomesenchymoma variant Desmin positive
Keratin 5-15% Keratin positive
EMA negative EMA positive
No desmoplastic stroma Prominent desmoplastic stroma
Various translocations involving 22q12 (no translocations involving 11p13) t(11;22)(p13;q12)

 

Carcinoma may simulate DSCT but is desmin negative

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