Andrew tragically died aged just 26 after being diagnosed with small round cell sarcoma
The others were diagnosed as angiosarcoma, undifferentiated pleomorphic sarcoma, solitary fibrous tumor, low-grade fibromyxoid sarcoma, malignant peripheral nerve sheath tumor, breast phyllodes tumor, round cell sarcoma
with CIC rearrangement, neuroblastoma, malignant extrarenal rhabdoid tumor, and myxofibrosarcoma.
Hence a diagnosis of malignant round cell sarcoma
was made.12 The differential diag- nosis included PNET, Lymphoma and Rhabdomyosa- rcoma.
Chloe Bigmore, of Laleston, near Bridgend, died aged 14 in 2007 after contracting a rare cancer called desmoplastic round cell sarcoma
Behavior is largely determined by the amount of round cell sarcoma
(13,14) Type II collagen stains the extraskeletal matrix of the small cell regions in EMCS, and has been shown to be useful in distinguishing EMCS from other round cell sarcomas
in a small series of limited tumor types.
Moreover, small round cell sarcomas
such as Ewing sarcomas are known to be relatively sensitive to chemotherapy and irradiation .
They concluded that renal sarcomas with BCOR-CCNB3 gene fusion overlap with CCSK and are in keeping with a "BCOR alteration family" of renal and extrarenal neoplasms which includes CCSK and undifferentiated round cell sarcomas
of soft tissue and bone/soft tissue sarcomas with BCOR-CCNB3 gene fusion, all of which are driven by BCOR overexpression and have overlapping clinicopathologic features.
In the present series, round cell sarcomas
were more commonly found on lower extremities, which is comparable to that found by Roy et al.
High prevalence of CIC fusion with double-homeobox (DUX4) transcription factors in EWSR1-negative undifferentiated small blue round cell sarcomas
. Genes Chromosomes Cancer.
Round cell sarcomas
beyond Ewing: emerging entities.
Round cell sarcomas
such as Ewing and rhabdomyosarcoma and malignant peripheral nerve sheath tumor were excluded to fulfill the FNCLCC criteria.