Pilot Study of Hyperthermic Peritoneal Perfusion (HIPEC) for Adolescent and Young Adults With Desmoplastic Small Round Cell Tumor
Pilot Study of Hyperthermic Peritoneal Perfusion (HIPEC) for Adolescent and Young Adults With Desmoplastic Small Round Cell Tumor
The goal of this clinical research study is to learn if heated intra-abdominal cisplatin can
 help to control abdominal tumors in patients having surgery to remove the tumors. The safety
 of this drug will also be studied.
The Study Drugs:

 Cisplatin has a platinum atom at its center. The platinum is supposed to poison the cancer
 cells, which may cause them to die.

 Surgery and Study Drug Administration:

 If you are found to be eligible, the abdominal surgery will be performed to try to remove as
 many tumors as possible. The abdominal surgery is not being performed specifically for this
 research study and would be performed even if you didn't take part in this study. You will be
 given a separate consent form to sign that explains the details and risks of abdominal
 surgery in more detail.

 During the surgery you will receive sodium thiosulfate by vein. The abdomen will then be
 temporarily closed. Then the abdominal wash will begin. During the "abdominal wash," heated
 cisplatin will be delivered through plastic tubing that is connected to a pump into the
 abdomen. The pump pushes the heated cisplatin into the abdomen and then pulls it out and
 recirculates the cisplatin. The skin of the abdomen is temporarily closed during the
 abdominal wash. The surgeon will also "wash" over the closed area of the surgical site. A
 pump will be used to pump heated cisplatin in and out of the abdomen over 90 minutes while
 the surgeon gently presses on the abdominal wall so the cisplatin reaches all areas in the
 abdomen. After 90 minutes, the cisplatin is removed and the abdomen will be "washed" with
 saline and all fluid will be removed before the surgeon permanently closes the abdomen with 3
 layers of stitches.

 Two (2) pea-sized tumor samples will be sent to the laboratory of Dr. Dina Lev for tissue
 evaluation and extraction of RNA (genetic material). One (1) tumor sample will be removed
 before the procedure and the other sample will be removed at the end of the procedure. The
 samples will be examined under a microscope and DNA (the genetic material in cells) will be
 removed to look for any changes to the DNA. The results of these tests will not be shared
 with you, nor will they be used for any decisions regarding your treatment. The samples will
 be destroyed after these tests are complete.

 There is a chance that the surgeon may decide during the surgery that the abdominal wash will
 not be performed, for example if the disease has spread to or attached to certain organs. If
 this occurs, your doctor will discuss other treatment options with you.

 Study Visits:

 On Days 1-5, 11, and 14, (Day 1 being the day after surgery):

 -  Blood (about 1 teaspoon) will be drawn for routine tests.

 -  You will have a physical exam, including measurement of your weight and vital signs.

 Follow-Up Visits:

 About 1, 3, and 6 months after the surgery is complete, you will have follow-up visits and
 the following tests and procedures will be performed:

 -  You will have a physical exam, including measurement of your weight and vital signs.

 -  Blood (about 1 teaspoon) will be drawn at the 1 month visit only for routine tests.

 -  You will have a hearing test at the 1 month visit only.

 -  Blood (about 3 teaspoons) will be drawn for routine tests at the 3 and 6 month visits
 only.

 -  At the 3 and 6 month visits only, you will have a CT scan, positron emission tomography
 (PET)-CT scan, or MRI scan to check the status of the disease.

 Length of Study:

 You will remain on study for up to 6 months. You will be taken off study if the disease gets
 worse.

 If the study doctor learns that the disease has come back or gotten worse at the 6 month
 follow-up visit, you may be eligible for a re-perfusion, which is a repeat of the operation
 and study drug administration. If you are eligible for a re-perfusion, your participation on
 this study will end and then you will be given a new consent form to sign in order to be
 re-enrolled back onto this study.

 This is an investigational study. Cisplatin is FDA-approved and commercially available for
 the treatment of advanced bladder cancer, metastatic testicular cancer, metastatic ovarian
 cancer, hepatoblastoma, neuroblastoma, metastatic appendiceal cancer, and abdominal
 mesothelioma. The use of cisplatin in patients with abdominal sarcoma-type tumors is
 investigational.

 Up to 22 patients will take part in this study. All will be enrolled at MD Anderson.
Inclusion Criteria:

 1. Age greater than or equal to 1 years

 2. Histologically or genetically proven diffuse peritoneal or retroperitoneal tumor from
 desmoplastic round cell tumor, ovarian germ cell, sarcoma, Wilms' tumor, or other
 non-carcinoma tumors.

 3. Radiologic workup must demonstrate that the disease is confined to the abdominal
 cavity

 4. Radiologic workup or prior abdominal exploration must be consistent with disease which
 can be debulked to a residual size of less than or equal to 1 cm thickness per tumor
 deposit

 5. Patients must have a minimum expected duration of survival of greater than 6 weeks as
 determined and documented by the attending surgeon or medical oncologist.

 6. Patients must not have any systemic illness which precludes them from being an
 operative candidate as determined by anesthesia preoperative evaluation. This includes
 but is not limited to, sepsis, liver failure, pregnant or lactating females.

 7. Patients must have fully intact mental status and normal neurologic abilities. Intact
 mental status is defined by 'the capacity to identify and recall one's identity and
 place in time and space.' Assessment of mental status and documentation of fully
 intact mental status will be completed using physical and mental exam by the referring
 doctor or oncologist.

 8. Patients must have adequate renal function (serum creatinine </= 1.5 mg/dl without
 history dialysis or renal failure or creatinine clearance less than 50 mL/min/1.73M^2
 if less than 5 years of age)

 9. Patients will be eligible if the white blood cell count (WBC) is >/=2000/microliter or
 absolute neutrophil count (ANC) is >/=1,500 and platelets are >/= 100,000/mm^3

 10. Patients will be eligible if serum total bilirubin and liver enzymes are </=2 times
 the upper limit of normal

 11. Patients must be recovered from any toxicity from all prior chemotherapy,
 immunotherapy, or radiotherapy and be at least 14 days past the date of their last
 treatment

 Exclusion Criteria:

 1. Patients will be ineligible if they have any concomitant cardiopulmonary disease which
 would place them at unacceptable risk for a major surgical procedure

 2. Patients will be ineligible if they have disease outside of the abdominal cavity which
 is uncontrolled

 3. Patients will be ineligible if they have a baseline neurologic toxicity of Grade 3 or
 greater (because of the potential neurotoxicity associated with platinum)

 4. Patients who have failed previous intraperitoneal platinum therapy will be ineligible

 5. Patients with Retroperitoneal Liposarcoma will be ineligible.