Myxoid Variant of Primary Cutaneous Anaplastic Large Cell Lymphoma Mimicking Soft Tissue Sarcoma: Diagnostic Pitfalls and Literature Review.
Ihar I Haiduk, Oxana A Podyacheva, Maria S Vladimirkina et al.
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Case in brief
A 40-year-old man presented with a slowly enlarging ulcerated mass on his left thigh, interpreted radiologically as a superficial soft tissue sarcoma. Excision showed a dermal and subcutaneous pleomorphic tumor with abundant myxoid stroma. Tumor cells expressed CD30, CD2, and CD4, while lacking ALK and markers of several other lineages, supporting ALK-negative myxoid primary cutaneous anaplastic large cell lymphoma. PET-CT and bone marrow biopsy found no extracutaneous disease. After 6 cycles of CHOP-E chemotherapy, he achieved complete metabolic remission and remained disease-free for 40 months after diagnosis.
What made this case unusual
The abundant myxoid stroma, varied pleomorphic cell forms, and delicate curvilinear vessels made this cutaneous lymphoma resemble a myxoid soft tissue sarcoma on imaging and histology.
Diagnostic / clinical pearl
In pleomorphic myxoid soft tissue tumors, particularly on the extremities, include CD30 and T-cell markers in the immunohistochemical evaluation to avoid overlooking myxoid PCALCL.