I came across an article recently discussing the use of hyperthermic intraperitoneal chemotherapy (HIPEC) in patients with desmoplastic small round cell tumor (DSRCT). Since pediatrics as a specialty is not my area of expertise, I wanted to learn more about this rare type of cancer.
DSRCT is classified as a soft tissue sarcoma that primarily presents as masses in the abdomen and pelvis. Considered a childhood cancer, it predominantly affects boys and young adults and occurs rarely in females. But as you can see in the article above, it can occur in females -- oftentimes mistaken for ovarian cancer.
Because this is a rare and aggressive tumor type, it is frequently misdiagnosed, and the risk of metastasis is high.
Though the origin of this cancer is currently not known, there appears to be a relationship between DSRCT, Wilms' tumor, and Ewing's sarcoma, along with a unique chromosomal translocation. Diagnosis is confirmed via a biopsy.
Symptoms may resemble those of other abdominal cancers: abdominal distention/ascites, palpable mass, pain, poor appetite, blood clotting, and urological complications.
Chemotherapy, tumor debulking, and radiation have been the standard treatment options for this kind of tumor, but smaller tumors may be difficult to detect and treat. This is where HIPEC treatment comes into play.
During HIPEC treatment, surgeons, oncologists, and perfusionists deliver heated chemotherapy directly into the patient's abdominal (peritoneal) cavity via a catheter in the hopes of eradicating any small tumor deposits. In addition, since the chemotherapy is heated, this may induce changes in blood flow to the tumor cells.
Those patients with disease spread to the peritoneum are especially good candidates for HIPEC treatment. Patients with metastatic colon cancer, certain types of sarcoma, and malignant ovarian germ cell tumors may also be treated with HIPEC.
With DSRCT being such a rare type of cancer -- affecting approximately 300 patients each year in the US -- it is aggressive and requires a specialist in this area.
Have you treated patients with this diagnosis or with HIPEC treatment? If so, what was the outcome?