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This is an aggressive, rare, fast spreading tumor and both pediatric and adult patients should be treated at a sarcoma center.There is no standard protocol for the disease; however, recent journals and studies have reported that some patients respond to high-dose (P6 Protocol) chemotherapy, maintenance chemotherapy, debulking operation, cytoreductive surgery, and radiation therapy. Because the disease can be misdiagnosed or remain undetected, tumors frequently grow large within the abdomen and metastasize or seed to other parts of the body. DSRCT can metastasize through lymph nodes or the blood stream.The abdominal masses can grow to enormous size before being noticed by the patient.The tumors can be felt as hard, round masses by palpating the abdomen.Other areas affected may include the lymph nodes, the lining of the abdomen, diaphragm, spleen, liver, chest wall, skull, spinal cord, large intestine, small intestine, bladder, brain, lungs, testicles, ovaries, and the pelvis.Reported sites of metastatic spread include the liver, lungs, lymph nodes, brain, skull, and bones. It is considered a childhood cancer that predominantly strikes boys and young adults.In recent studies, patients who were in wheelchairs were able to walk again thanks to stem cell therapy. There are 4 different types of stem cells, but the ones we’re interested in are adult stem cells, which can be harvested from bone marrow.
A multi-modality approach of high-dose chemotherapy, aggressive surgical resection, radiation, and stem cell rescue improves survival for some patients.
DSRCT in young patients can be mistaken for other abdominal tumors including rhabdomyosarcoma, neuroblastoma, and mesenteric carcinoid.
In older patients DSRCT can resemble lymphoma, peritoneal mesothelioma, and peritoneal carcinomatosis.
First symptoms of the disease often include abdominal distention, abdominal mass, abdominal or back pain, gastrointestinal obstruction, lack of appetite, ascites, anemia, and/or cachexia.
Other reported symptoms include unknown lumps, thyroid conditions, hormonal conditions, blood clotting, kidney or urological problems, testicle, breast, uterine, vaginal, or ovarian masses.