DSRCT is een soort kanker die in 1991 voor het eerst is beschreven door Dr. Rosai van het Memorial Sloan-Kettering Cancer Center in New York. Het vertoont overeenkomsten met Ewing Sarcoma en Helms syndrome. Het komt met name voor bij jonge jongens (12-20 jaar). Slechts een paar honderd mensen op de wereld hebben de ziekte of hebben haar gehad. De meeste zijn overleden, een paar overleven. Het is een 'onzichtbare' vorm van kanker omdat aan het bloed niets is af te lezen (geen tumormarkers).
De kanker genereert tumoren, zonder duidelijke oorsprong, in de buikholte, aanvankelijk zich verspreidend over het buikvlies, daarna naar de lever, lymfklieren en/of longen.
Bij Ineke is het bij toeval ontdekt (vriend voelde iets hards in buik).
What is DSRCT?
DSRCT is a malignancy first described in the literature by Dr. Rosai of Memorial Sloan-Kettering Cancer Center in New York in 1991.
There are distinctive clinical and histological characteristics that differentiate DSRCT from other small cell tumors. The malignancy most commonly occurs in young males of adolescent age and is characterized as a large intra-abdominal mass with no apparent origin first spreading to the peritoneal surfaces, then to the liver, lymph nodes and/or lungs. Since this lesion is normally not attached to an organ, it usually grows to a very large size and spreads to other parts of the body before the person experiences any ill effects of the disease.
Typical symptoms include intestinal blockage, swelling and pain in the abdomen and/or fever. Since DSRCT is so rare, probably less than 200 cases reported, statistics are unreliable. It appears that approximately 3/4 of cases are male with an average age in the late teens or early twenties and that over 95% of the cases had tumors located in the abdomen/pelvic area. However, cases have been reported in all age groups of both males and females and with tumors present in other parts of the body. It would be unrealistic to guess at statistics on prognosis and survival rates at this time.
Early reports describe a dismal prognosis for DSRCT due to its aggressive behavior and general non-responsiveness to known treatments of similar malignancies. However, in the past several years, there have been treatments developed that show promising results with many patients experiencing complete remissions sustained for longer than two years.
BACKGROUND: Intraabdominal desmoplastic small round cell tumor (DSRCT) is a recently recognized type of primitive sarcoma characterized by a predilection for young males, a usually very aggressive course and generally unsuccessful therapy. A primitive histologic appearance with prominent desmoplasia and striking divergent multilineage differentiation are well-described morphologic features of this tumor, along with a consistent fusion of the EWS and WT1 genes at the molecular level. The cytologic literature contains only scattered references to this type of neoplasm. Detailed information on the clinical and fine needle aspiration (FNA) biopsy and the immunocytochemical and ultrastructural findings in a patient with DSRCT is presented. CASE REPORT: A 23-year-old male had a firm abdominal mass with multiple secondary lesions of the liver. An FNA biopsy was performed under ultrasonographic guidance. CONCLUSION: FNA of the liver nodules showed cohesive groups of small cells with hyperchromatic nuclei and inconspicuous nucleoli; immunocytochemically vimentin and desmin showed characteristic perinuclear globular positivity. FNA cytology is an effective means of diagnosing deeply located lesions. The cytologic features of DSRCT need to become familiar to pathologists and must be considered in the differential diagnosis of liver metastasis.
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