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Desmoplastic small round cell tumor
Classification and external resources
ICD-O:8806/3
Display of small round blue cells characteristic of desmoplastic small round cell tumor.
Display of small round blue cells characteristic of desmoplastic small round cell tumor. The International Classification of Diseases for Oncology (ICD-O is a domain specific extension of the International Statistical Classification of Diseases and Related Health Problems
Cell exhibiting blue oval and round shapes of desmoplastic small round blue cell tumor
Cell exhibiting blue oval and round shapes of desmoplastic small round blue cell tumor

Desmoplastic small round cell tumor is classified as a soft tissue sarcoma. In Medicine, desmoplasia refers to the formation of Adhesions or Fibrosis (fibrosis refers to scar tissue in the stroma of a Neoplasm. A soft tissue sarcoma is a rare form of Cancer that develops in mesenchymal tissue the Muscle, Connective tissues and Bones of the body It is an aggressive and rare tumor that primarily occurs as multiple masses in the abdomen. In Vertebrates such as Mammals the abdomen (belly constitutes the part of the body between the Thorax (chest and Pelvis. 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. A Lymph node ( lɪmf noʊd is an organ consisting of many types of cells and is a part of the Lymphatic system. In Vertebrates such as Mammals the abdomen (belly constitutes the part of the body between the Thorax (chest and Pelvis. For other types of diaphragm see Diaphragm. In the Anatomy of Mammals the thoracic diaphragm is a sheet of Muscle The pelvis (pl pelvises or pelves) or pelvic girdle is the irregular bony structure located at the base of the spine (properly known Reported sites of metatastic spread include the liver, lungs, lymph nodes, brain, skull, and bones. The liver is a vital organ in the human body and is present in Vertebrates and some other animals lung is the essential Respiration organ in air-breathing Animals including most Tetrapods a few Fish and a few Snails The most primitive A Lymph node ( lɪmf noʊd is an organ consisting of many types of cells and is a part of the Lymphatic system. The brain is the center of the Nervous system in animals All Vertebrates and the majority of Invertebrates have a brain Bones are rigid organs that form part of the Endoskeleton of Vertebrates They function to move support and protect the various organs of the body produce

The tumor is considered a childhood cancer that predominantly strikes boys and young adults. See also Cancer A tumor or tumour is the name for a swelling or lesion formed by an abnormal growth of cells (termed neoplastic

The disease rarely occurs in females, but when it does the tumors can be mistaken for ovarian cancer. Ovarian cancer is a cancerous growth arising from an Ovary. The cancer most commonly forms in the lining of the ovary (resulting in epithelial [1]

Contents

Causes

There are no known risk factors that have been identified specific to the disease. The tumor appears to arise from the primitive cells of childhood, and is considered a childhood cancer.

Research has indicated that there is a chimeric relationship between desmoplastic small round cell tumor and Wilm's tumor and Ewing's sarcoma. Wilms' tumor or nephroblastoma is a Tumor of the Kidneys that typically occurs in Children rarely in Adults Its common name is Ewing sarcoma is a malignant round-cell tumor It is a rare disease in which Cancer cells are found in the Bone or in soft tissue.

DSRCT is associated with a unique chromosomal translocation (t11;22)(p13:q12) resulting in a EWS/WTI transcript that is diagnostic of this tumor. [2] This transcript codes for a protein that acts as a transcriptional activator that fails to suppress tumor growth.

Symptoms

There are few early warning signs that a patient has a desmoplastic small round cell tumor. Patients are often young and healthy as the tumors grow and spread uninhibited within the abdominal cavity. These are rare tumors and symptoms are often misdiagnosed by family physicians. 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.

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. In Medicine ( Gastroenterology) ascites (also known as peritoneal cavity fluid, peritoneal fluid excess, hydroperitoneum or more Cachexia (kəˈkɛksiə is loss of weight, Muscle atrophy, fatigue weakness and significant loss of appetite in someone who is not actively trying to

Other reported symptoms include unknown lumps, thyroid conditions, hormonal conditions, blood clotting, kidney or urological problems, testicle, breast, uterine, vaginal, or ovarian masses.

Differentials

Because this is a rare tumor not many family physicians or oncologists are familiar with this disease. DSRCT in young patients can be mistaken for other abdominal tumors including rhabdomyosarcoma, neuroblastoma, and mesenteric carcinoid. A rhabdomyosarcoma is a type of Cancer, specifically a Sarcoma (cancer of Connective tissues, in which the cancer cells are thought to arise from Neuroblastoma is the most common extracranial solid Cancer in childhood and the most common cancer in infancy with an annual incidence of about 650 new cases per year in the Carcinoid (also carcinoid tumour or carcinoid tumor) is a slow-growing but Malignant type of Neuroendocrine tumour, originating in the cells In older patients DSRCT can resemble lymphoma, peritoneal mesothelioma, and peritoneal carcinomatosis. Lymphoma a type of Neoplasm that originates in Lymphocytes (a type of White blood cell in the vertebrate Immune system) In males DSRCT may be mistaken for germ cell or testicular cancer. Testicular cancer is Cancer that develops in the Testicles a part of the Male reproductive system In females DSRCT can be mistaken for Ovarian cancer. Desmoplastic small round cell tumor shares characteristics with other small round cell cancers including Ewing's sarcoma, acute leukemia, small cell mesothelioma, neuroblastoma, primitive neuroectodermal tumor, rhabdomyosarcoma, and Wilm's tumor. Primitive neuroectodermal tumor (PNET is a rare Tumor, usually occurring in children under 10 years old A rhabdomyosarcoma is a type of Cancer, specifically a Sarcoma (cancer of Connective tissues, in which the cancer cells are thought to arise from

Pathology

Pathology reveals well circumscribed solid tumor nodules within a dense desmoplastic stroma. Often areas of central necrosis are present. Tumor cells have hyperchromatic nuclei with increased nuclear/cytoplasmic ratio.

On immunohistochemistry, these cells have trilinear coexpression including the epithelial marker cytokeratin, the mesenchymal markers desmin and vimentin, and the neuronal marker neuron-specific enolase. Thus, although initially thought to be of mesothelial origin due to sites of presentation, it is now hypothesized to arise from a progenitor cell with multiphenotypic differentiation.

Treatment

Desmoplastic small round cell tumor is frequently misdiagnosed. Adult patients should always be referred to a sarcoma specialist. 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. [3] 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. Chemotherapy, in its most general sense refers to treatment of disease by chemicals that kill cells specifically those of micro-organisms or Cancer. Debulking is the surgical removal of part of a malignant Tumour which cannot be completely excised so as to enhance the effectiveness of radiation or Radiation therapy (or radiotherapy) is the medical use of Ionizing radiation as part of Cancer treatment to control Malignant Other treatment options include: hematopoietic stem cell transplantation, intensity-modulated radiation Therapy, radiofrequency ablation, stereotactic body radiation therapy, intraperitoneal hyperthermic chemoperfusion, and clinical trials. Radio Frequency Ablation ( RFA) of Lung kidney breast bone and Liver tumors RFA is performed to cure tumors in lung liver kidney bone and rarely in other Intraperitoneal hyperthermic chemoperfusion is a procedure in which a warmed Solution containing Anti-cancer drugs is infused and circulated in the In health care clinical trials are conducted to allow safety and Efficacy data to be collected for new drugs or devices

Prognosis

The prognosis for desmoplastic small round cell tumor remains poor with less than 20% surviving beyond two to three years. Prognosis often depends upon the stage of the cancer and the grade of the tumor. Because the disease can be misdiagnosed or remain undetected tumors frequently grow large within the abdomen and metastasized or seed to other parts of the body. Metastasis ( Greek: displacement μετά=next + στάσις=placement, plural metastases) sometimes abbreviated mets,

There is no known organ or area of origin. Desmoplastic small round cell tumor can metastasize through lymph nodes or the blood stream. Sites of metastatis include the spleen, diaphragm, liver, large and small intestine, lungs, central nervous system, bones, uterus, bladder, genitals, abdominal cavity, and the brain.

A multi-modality approach of high dose chemotherapy, 90-100% surgical resection, radiation, and stem cell rescue improves survival for some patients. Reports have indicated that patients will initially respond to first line chemotherapy and treatment but that relapse is common.

Some patients in remission or with inoperable tumor seem to benefit from long term low dose chemotherapy, turning desmoplastic small round cell tumor into a chronic disease.

Research

The Stehlin Foundation currently offers DSRCT patients the opportunity to send samples of their tumors free of charge for testing. Research scientists are growing the samples on nude mice and testing various chemical agents to find which are most effective against the individual's tumor.

Patients with advanced desmoplastic small round cell tumor may qualify to participate in clinical trials that are researching new drugs to treat the disease. Because of the similarities with Ewing's sarcoma patients with desmoplastic small round cell tumor are often eligible for the same clinical trials.

Alternative names

This disease is also known as: desmoplastic small round blue cell tumor; intraabdominal desmoplastic small round blue cell tumor; desmoplastic small cell tumor; desmoplastic cancer; desmoplastic sarcoma; DSRCT. In Medicine, desmoplasia refers to the formation of Adhesions or Fibrosis (fibrosis refers to scar tissue in the stroma of a Neoplasm.

There is no connection to mesothelioma which is another disease sometimes described as desmoplastic. Mesothelioma is a form of Cancer that is almost always caused by previous exposure to Asbestos.

References

  1. ^ Bland AE, Shah AA, Piscitelli JT, Bentley RC, Secord AA (2007). "Desmoplastic small round cell tumor masquerading as advanced ovarian cancer". Int J Gynecol Cancer. doi:10.1111/j.1525-1438.2007.01110.x. A digital object identifier ( DOI) is a permanent identifier given to an Electronic document. PMID 18081791.  
  2. ^ Lee YS, Hsiao CH (2007). "Desmoplastic small round cell tumor: a clinicopathologic, immunohistochemical and molecular study of four patients". J. Formos. Med. Assoc. 106 (10): 854–60. PMID 17964965.  
  3. ^ Talarico F, Iusco D, Negri L, Belinelli D (2007). "Combined resection and multi-agent adjuvant chemotherapy for intra-abdominal desmoplastic small round cell tumour: case report and review of the literature". G Chir 28 (10): 367–70. PMID 17915050.  

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