Surgery with HIPEC

A single-institution phase II study showed HIPEC to be a potentially promising addition to complete surgical resection. Fourteen patients with desmoplastic small round cell tumor and five patients with other sarcomas were enrolled. These highly selected patients had tumor limited to the abdominal cavity. They demonstrated a partial response to neoadjuvant Ewing-type chemotherapy, had complete surgical resections and received HIPEC using cisplatin. They also received adjuvant total-abdominal radiation therapy followed by adjuvant chemotherapy.[119]

A single-institution phase II study showed HIPEC to be a potentially promising addition to complete surgical resection. Fourteen patients with desmoplastic small round cell tumor and five patients with other sarcomas were enrolled. These highly selected patients had tumor limited to the abdominal cavity. They demonstrated a partial response to neoadjuvant Ewing-type chemotherapy, had complete surgical resections and received HIPEC using cisplatin. They also received adjuvant total-abdominal radiation therapy followed by adjuvant chemotherapy.[119]

With this standardized approach, patients with desmoplastic small round cell tumors had an OS rate of 80% at 30 months and 40% at 50 months.

A single-institution phase II study showed HIPEC to be a potentially promising addition to complete surgical resection. Fourteen patients with desmoplastic small round cell tumor and five patients with other sarcomas were enrolled. These highly selected patients had tumor limited to the abdominal cavity. They demonstrated a partial response to neoadjuvant Ewing-type chemotherapy, had complete surgical resections and received HIPEC using cisplatin. They also received adjuvant total-abdominal radiation therapy followed by adjuvant chemotherapy.[119]

Patients with desmoplastic small round cell tumors without liver metastasis had no intra-abdominal recurrences, whereas 87% of patients with liver metastasis or portal disease had a recurrence.

In a retrospective study from centers in France, patients were treated with cytoreductive surgery and HIPEC. Twenty-two patients were selected, and the median age at diagnosis was 14.8 years (range, 4.2–17.6 years). Seven patients had peritoneal mesotheliomas, seven patients had desmoplastic small round cells tumors, and eight patients had other histological tumor types. A complete macroscopic resection (CC-0, where CC is completeness of cytoreduction) was achieved in 16 cases (73%). Four of the seven patients with desmoplastic small round cell tumors had complete resections.[120][Level of evidence C1]

In a retrospective study from centers in France, patients were treated with cytoreductive surgery and HIPEC. Twenty-two patients were selected, and the median age at diagnosis was 14.8 years (range, 4.2–17.6 years). Seven patients had peritoneal mesotheliomas, seven patients had desmoplastic small round cells tumors, and eight patients had other histological tumor types. A complete macroscopic resection (CC-0, where CC is completeness of cytoreduction) was achieved in 16 cases (73%). Four of the seven patients with desmoplastic small round cell tumors had complete resections.[120][Level of evidence C1]

Sixteen patients (72%) experienced relapses after a median time of 9.6 months (range, 1.4–86.4 months).

In a retrospective study from centers in France, patients were treated with cytoreductive surgery and HIPEC. Twenty-two patients were selected, and the median age at diagnosis was 14.8 years (range, 4.2–17.6 years). Seven patients had peritoneal mesotheliomas, seven patients had desmoplastic small round cells tumors, and eight patients had other histological tumor types. A complete macroscopic resection (CC-0, where CC is completeness of cytoreduction) was achieved in 16 cases (73%). Four of the seven patients with desmoplastic small round cell tumors had complete resections.[120][Level of evidence C1]

Nine patients (41%) died of relapsed disease after a median time of 5.3 months (range, 0.1–36.1 months).

Another study from France reviewed the use of cytoreductive surgery and HIPEC for the treatment of patients with desmoplastic small round cell tumors who had disease limited to the abdomen. In 107 patients with desmoplastic small round cell tumors, 48 had no extraperitoneal metastasis and underwent cytoreductive surgery. Of 48 patients (mean age, 22 years), 38 (79%) received preoperative and/or postoperative chemotherapy, and 23 (48%) received postoperative whole-abdominopelvic radiation therapy. Intraperitoneal chemotherapy was administered to 11 patients (23%), 2 of whom received early postoperative intraperitoneal chemotherapy (EPIC) and 9 of whom received HIPEC.[121]

Another study from France reviewed the use of cytoreductive surgery and HIPEC for the treatment of patients with desmoplastic small round cell tumors who had disease limited to the abdomen. In 107 patients with desmoplastic small round cell tumors, 48 had no extraperitoneal metastasis and underwent cytoreductive surgery. Of 48 patients (mean age, 22 years), 38 (79%) received preoperative and/or postoperative chemotherapy, and 23 (48%) received postoperative whole-abdominopelvic radiation therapy. Intraperitoneal chemotherapy was administered to 11 patients (23%), 2 of whom received early postoperative intraperitoneal chemotherapy (EPIC) and 9 of whom received HIPEC.[121]

After a median follow-up of 30 months, the median OS of the entire cohort was 42 months.

Another study from France reviewed the use of cytoreductive surgery and HIPEC for the treatment of patients with desmoplastic small round cell tumors who had disease limited to the abdomen. In 107 patients with desmoplastic small round cell tumors, 48 had no extraperitoneal metastasis and underwent cytoreductive surgery. Of 48 patients (mean age, 22 years), 38 (79%) received preoperative and/or postoperative chemotherapy, and 23 (48%) received postoperative whole-abdominopelvic radiation therapy. Intraperitoneal chemotherapy was administered to 11 patients (23%), 2 of whom received early postoperative intraperitoneal chemotherapy (EPIC) and 9 of whom received HIPEC.[121]

The 2-year OS rate was 72%, and the 5-year OS rate was 19%.

Another study from France reviewed the use of cytoreductive surgery and HIPEC for the treatment of patients with desmoplastic small round cell tumors who had disease limited to the abdomen. In 107 patients with desmoplastic small round cell tumors, 48 had no extraperitoneal metastasis and underwent cytoreductive surgery. Of 48 patients (mean age, 22 years), 38 (79%) received preoperative and/or postoperative chemotherapy, and 23 (48%) received postoperative whole-abdominopelvic radiation therapy. Intraperitoneal chemotherapy was administered to 11 patients (23%), 2 of whom received early postoperative intraperitoneal chemotherapy (EPIC) and 9 of whom received HIPEC.[121]

The 2-year disease-free survival (DFS) rate was 30%, and the 5-year DFS rate was 12%.

Another study from France reviewed the use of cytoreductive surgery and HIPEC for the treatment of patients with desmoplastic small round cell tumors who had disease limited to the abdomen. In 107 patients with desmoplastic small round cell tumors, 48 had no extraperitoneal metastasis and underwent cytoreductive surgery. Of 48 patients (mean age, 22 years), 38 (79%) received preoperative and/or postoperative chemotherapy, and 23 (48%) received postoperative whole-abdominopelvic radiation therapy. Intraperitoneal chemotherapy was administered to 11 patients (23%), 2 of whom received early postoperative intraperitoneal chemotherapy (EPIC) and 9 of whom received HIPEC.[121]

Whole-abdominopelvic radiation therapy was the only variable associated with longer peritoneal recurrence-free survival and DFS after cytoreductive surgery.

Another study from France reviewed the use of cytoreductive surgery and HIPEC for the treatment of patients with desmoplastic small round cell tumors who had disease limited to the abdomen. In 107 patients with desmoplastic small round cell tumors, 48 had no extraperitoneal metastasis and underwent cytoreductive surgery. Of 48 patients (mean age, 22 years), 38 (79%) received preoperative and/or postoperative chemotherapy, and 23 (48%) received postoperative whole-abdominopelvic radiation therapy. Intraperitoneal chemotherapy was administered to 11 patients (23%), 2 of whom received early postoperative intraperitoneal chemotherapy (EPIC) and 9 of whom received HIPEC.[121]

Of 11 patients who received intraperitoneal chemotherapy (HIPEC or EPIC), six different chemotherapy regimens were used. The survival or outcome of this group is not reported in the manuscript.

Another study from France reviewed the use of cytoreductive surgery and HIPEC for the treatment of patients with desmoplastic small round cell tumors who had disease limited to the abdomen. In 107 patients with desmoplastic small round cell tumors, 48 had no extraperitoneal metastasis and underwent cytoreductive surgery. Of 48 patients (mean age, 22 years), 38 (79%) received preoperative and/or postoperative chemotherapy, and 23 (48%) received postoperative whole-abdominopelvic radiation therapy. Intraperitoneal chemotherapy was administered to 11 patients (23%), 2 of whom received early postoperative intraperitoneal chemotherapy (EPIC) and 9 of whom received HIPEC.[121]

The influence of HIPEC/EPIC on OS and DFS was not statistically significant, but standardized regimens were not used in all patients, making results difficult to determine.

A single-institutional retrospective study reported on nine patients (median age, 19 years) with desmoplastic small round cell tumor. Most patients had widespread disease, including four patients with extra-abdominal disease and five patients with liver involvement. These nine patients underwent ten cytoreductive and HIPEC treatments. Additionally, seven patients also received radiation therapy, and three patients underwent stem cell transplant.[122]

A single-institutional retrospective study reported on nine patients (median age, 19 years) with desmoplastic small round cell tumor. Most patients had widespread disease, including four patients with extra-abdominal disease and five patients with liver involvement. These nine patients underwent ten cytoreductive and HIPEC treatments. Additionally, seven patients also received radiation therapy, and three patients underwent stem cell transplant.[122]

The 3-year relapse-free survival rate was 13%, and the OS rate was 55%.

A single-institutional retrospective study reported on nine patients (median age, 19 years) with desmoplastic small round cell tumor. Most patients had widespread disease, including four patients with extra-abdominal disease and five patients with liver involvement. These nine patients underwent ten cytoreductive and HIPEC treatments. Additionally, seven patients also received radiation therapy, and three patients underwent stem cell transplant.[122]

Therapy was often associated with prolonged hospitalizations.

A single-institutional retrospective study reported on nine patients (median age, 19 years) with desmoplastic small round cell tumor. Most patients had widespread disease, including four patients with extra-abdominal disease and five patients with liver involvement. These nine patients underwent ten cytoreductive and HIPEC treatments. Additionally, seven patients also received radiation therapy, and three patients underwent stem cell transplant.[122]

Long-term parenteral nutrition was required in eight patients for a median of 261 days.

A single-institutional retrospective study reported on nine patients (median age, 19 years) with desmoplastic small round cell tumor. Most patients had widespread disease, including four patients with extra-abdominal disease and five patients with liver involvement. These nine patients underwent ten cytoreductive and HIPEC treatments. Additionally, seven patients also received radiation therapy, and three patients underwent stem cell transplant.[122]

Other long-term complications included gastroparesis (n = 1), small bowel obstruction (n = 3), and hemorrhagic cystitis (n = 2).