The company's New Drug Application submission is based mainly on data from the 62 patient
epithelioid sarcoma cohort of its ongoing Phase two study of tazemetostat.
Epizyme has proposed an indication of metastatic or locally advanced
epithelioid sarcoma not eligible for curative surgery.
Superficial lesions may present as ulcerated nodules, (1) similar to
epithelioid sarcoma (ES).
It was diagnosed on recognition of three patterns as described by Das DK et al [4] which were Type 1--Epithelioid cell granulomas without caseous necrosis, Type 2--Epithelioid cell granulomas with caseous necrosis and Type 3--Caseous necrosis with or without polymorphonuclear (PMN) cells and occasional
epithelioid cells.
Fifteen (55.5%) tumors were spindle cell tumors, 7 (25.9%) were
epithelioid tumors, and 5 (18.5%) were mixed form tumors (Figure 6).
It was composed of a fascicular proliferation of plump spindle cells and
epithelioid cells with prominent nucleoli and brightly eosinophilic cytoplasm, mimicking rhabdomyoblasts (Figs.
Dissecting in-between benign prostatic ducts and acini were solid clusters of atypical
epithelioid cells with ample eosinophilic cytoplasm and limited lumen formation.
In this article we report 2 patients with
epithelioid sarcoma of the hand for whom the coverage was made by a dermal regeneration template (Integra[R]).
This variant only occurs in the stomach and are commonly multi-focal with predominant
epithelioid morphology5.
The morphology and immunophenotype provided no support for a reactive process, melanoma, lymphoma, carcinoma,
epithelioid sarcoma,
epithelioid malignant peripheral nerve sheath tumor, rhabdoid tumor, or alveolar soft part sarcoma.
Among adolescents the most common identified form was "superficial spreading," in 32%, with nodular in 6%, mixed
epithelioid and spindle cell in 2%, and "not otherwise specified" in 54%.
(1) The
epithelioid subtype is more rare and represents a well-recognized entity comprising of about 5 percent of these tumors.
