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Table 2 Clinical data of 8 cases of DSRCT

From: Clinicopathological features of desmoplastic small round cell tumors: clinical series and literature review

No

Gender/age

Symptoms

Preoperative chemotherapy

Surgical approach

Ascites (ml)

PCI/CC (Score)

Postoperative chemotherapy

Follow-up (Month)

Survival Status

1

Male/33

Altered bowel habits, abdominal distension and pain

AI solution × 2 cycles

CRS+HIPEC

CRS: hepatic round ligament, greater omentum, lesser omentum, abdominal wall tumors, sub mesenteric vein root tumors, transverse colon tumors, left popliteal tumors, ascending colon surface tumors, transverse colon bowel lip, rectum + sigmoid colon, partial ileum + colon

HIPEC: doxorubicin 40 mg + ifosfamide 3 g, 43 °C, 60 min

200

30/3

None

8.4

Live

2

Male/41

Stomachache, abdominal distension and pain

None

CRS+HIPEC;

CRS: right colon, omentum;

HIPEC: cisplatin 120 mg + docetaxel 120 mg, 43 °C, 90 min

None

7/0

ICE × 8 cycles

20.0

Live

3

Male/24

Abdominal distension, right lower quadrant pain, altered bowel habits

VAC × 4 cycles, IE × 3 cycles, irinotecan + recombinant human endostatin + anlotinib × 10 cycles

CRS+HIPEC

CRS: greater omentum, right diaphragm, hepatic hilum tumor, right lobe tumor, rectum + pelvic tumor, mesenteric tumor

HIPEC: doxorubicin 120 mg + ifosfamide 3 g, 43 °C, 60 min

1000

24/1

AIM plan + PD1 (details unknown)

20.3

Live

4

Male/29

Abdominal pain and distension, constipation

TC plan × 1 cycle

AI solution × 2 cycles

CRS+HIPEC

CRS: tumors of the upper abdominal wall, tumors of the left lower abdomen, colonic spleen flexure peritoneum, omental tumors, descending colon peritoneal tumors, hepatic ligament

HIPEC: docetaxel 120 mg + ifosfamide 3 g, 43 °C, 60 min

2200

39/3

AI × 6 cycles

IE × 6 cycles

14.4

Dead

5

Male/27

Abdominal pain and distension, constipation

AI Plan×7 cycles

CRS+HIPEC

CRS: hepatic round ligament, greater omentum, rectal pelvic floor tumor, liver and kidney crypt tumor, small intestinal mesenteric tumor, colon mesenteric tumor, right diaphragm muscle tumor, ileocecal + appendix, left diaphragm muscle tumor

HIPEC: docetaxel 120 mg + ifosfamide 3 g, 43 °C, 60 min

100

25/1

AI solution × 6 cycles +

Retroperitoneal lymph node radiotherapy

28.5

Dead

6

Male/45

Abdominal pain and distension, exhaustion, fever

None

Laparotomy: partial abdominal tumor resection

Unknown

38/2

Unknown

22.6

Live

7

Male/26

Abdominal distension with nausea and vomiting

None

CRS+HIPEC

CRS: omental tumor, liver curvature tumor, splenic curvature tumor

HIPEC: docetaxel 120 mg, 43 °C, 30 min

1900

39/2

Unknown

8.9

Dead

8

Male/

31

Abdominal pain and distension

IE×1cycles

Laparotomy: partial abdominal tumor resection

None

31/2

VAC solution × 5 cycles

7.5

Live

  1. CRS, cytoreductive surgery; HIPEC, hyperthermic intraperitoneal chemotherapy; AI, doxorubicin + ifosfamide; ICE, cis-platinum + ifosfamide + etoposide; VAC, vincristine + pharmorubicin + cyclophosphamide; IE: cis-platinum + etoposide; TC, Taxol + carboplatin; DOX, doxorubicin; DOC, docetaxel; IFO, ifosfamide; CDDP, cis-platin; PD-1, Programmed cell death-1