PMC2430011_01
Peritoneal nodule of DSRCT at diagnosis. Nodules are composed of fibrocollagenous
tissue and irregular islands of malignant undifferentiated small round
cells. Immunoperoxidase stains (not
shown) in this case were strongly positive for EMA, vimentin, and desmin,
partially positive for CD99 and negative for synaptophysin, chromogranin,
myogenin, SMA, Bcl-2, CD3, and CD20. 
Cytogenetic analysis demonstrated t(11;22)(p15;q12). Magnification: (a) x 10, (b) x 40, (c) high
power.
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fig1
Pathology of tumor biopsies (see Figure 1) showed features of a highly anaplastic and somewhat pleomorphic small blue cell malignancy.
Outpatient neoadjuvant and adjuvant chemotherapy plan for DSRCT. After initial diagnosis and treatment of peritoneal, abdominal and
pelvic disease, systemic chemotherapy
agents were administered on an outpatient basis followed by local control with
cytoreductive surgery + CHPP with cisplatin, then whole abdomen radiation with
temozolomide as a radiation sensitizer, followed by temozolomide and
irinotecan. Outpatient therapy was 15
months.
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fig2
 Figure 2 shows a sequence of Neoadjuvant and adjuvant chemotherapy.
Continuous hyperthermic peritoneal perfusion (CHPP). The CHPP procedure involves placing
temperature probes, inflow and outflow cannulas, and continuous perfusion
of plasmalyte followed by cisplatin
containing chemotherapy as the perfusate at 41-42 C. To achieve this, the inflow was usually about
45 C. Manual agitation of the closed abdomen was done for 1.5 hours
to ensure that all peritoneal surfaces were exposed to the cisplatin.
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fig3
PET-CT fusion imaging. (a) Residual uptake
in the peritoneal implants after 2 cycles of therapy, SUV 3. (b) No evidence of
FDG avid disease after cytoreductive therapy. (c) Relapse in the perihepatic
space including dome of the liver (SUV 2.8) and retroperitoneal nodes (SUV 5).
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fig4
Although the patient continued to be healthy, attend school, and had no symptoms, routine PET-CT done 2 months after completion of therapy showed five FDG avid areas (Figure 4).
Radio frequency ablation and cryoablation procedure. Under direct CT fluoroscopic guidance, an RFA electrode was advanced
into the perihepatic lesions. These were heated from 42 C to 43 C for 30 minutes (a) and (b) then a cryoablation probe was advanced and 2 cycles of
freeze and thaw for 8-10 minutes (c).
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fig5
To destroy cells in the perihepatic nodules and increase chance of mounting an immune response, both radio frequency ablation and cryoablation were used (Figure 5).
Schema for local control
of relapse foci and continuation adjuvant therapy. Radiation in combination with
bevacizumab was used to pericaval disease. 
Localized perihepatic lesions were treated with a combination of RFA and
cryoablation. These measures were followed with a chronic adjuvant regimen.
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fig6
The patient received a regimen of valproate (250 mg BID), rapamycin (2 mg daily), and sorafenib (200 mg BID) and chemotherapy similar to that reported by Casanova et al., vinorelbine (25 mg/m2 IV monthly) and oral cyclophosphamide (25 mg daily) (see Figure 6). Sorafenib and vinorelbine were discontinued because of significant diarrhea (see treatment schema in Figure 6).