Poor outcome of two children with relapsed stage IV
alveolar rhabdomyosarcoma after allogeneic stem cell transplantation.
Response to "Metastatic
alveolar rhabdomyosarcoma with increased serum creatinine kinase MB and cardiac Troponin T and normal cardiac troponin I".
Of note, with available data, the prognosis of BSNS is significantly better than
alveolar rhabdomyosarcomas with the same PAX3-FOXO1 gene fusion.
Rosenberger et al., "Activation of the hedgehog pathway confers a poor prognosis in embryonal and fusion gene-negative
alveolar rhabdomyosarcoma," Oncogene, vol.
Brody has Stage 4
alveolar rhabdomyosarcoma, which is a soft tissue cancer in children and occurs most often in the arms, legs and abdomen.
In 1995, the Intergroup Rhabdomyosarcoma Study proposed the "International Classification of Rhabdomyosarcoma," in which 4 main subtypes of RMS were established: 1) botryoid and spindle cell variants of embryonal rhabdomyosarcoma (ERMS) with a favorable prognosis; 2) conventional ERMS with an intermediate prognosis; 3)
alveolar rhabdomyosarcoma (ARMS) with a poor prognosis; and 4) undifferentiated sarcomas, also with a poor prognosis5.
Reciprocal translocations typical of
alveolar rhabdomyosarcoma, either t(1;13)(p36;q14) or t(2;13)(q35;q14), have not been present.
As well as getting chemotherapy for the cancer, metastatic
alveolar rhabdomyosarcoma, Hamish had surgery to remove a large tumour above his left knee.
In March 2002, rare facial cancer
Alveolar Rhabdomyosarcoma was diagnosed by doctors and managed by a six-month course of chemotherapy and radiotherapy.
The cells invariably express skeletal muscle markers including desmin and myogenin, may show positivity for keratin and EMA, and lack PAX3/7-FOXO1 transcripts characteristic of
alveolar rhabdomyosarcoma. (54,55)
On microscopic examination it revealed
alveolar rhabdomyosarcoma (figure 1 and 2).
And mum Susan said: "It's going to take a long time for him to get used to it but we're heading in the right direction." Within a week, he was diagnosed with metastatic
alveolar rhabdomyosarcoma, a cancer that attacks muscle cells.