Desmoplastik küçük yuvarlak hücreli tümör (Olgu sunumu)

Amaç: Dezmoplastik küçük yuvarlak hücreli tümör Introduction: (DKYHT), genellikle çocukluk çağında görülen, abdominal veya peritoneal yüzeylerde ortaya çıkan, ektodermal ve mezenkimal immünfenotipik özellik gösteren bir tümördür. Amacımız ender görülen ve ayırıcı tanısı sorun yaratabilen bu tümörün özelliklerini irdelemektir

Anahtar Kelimeler:

Dezmoplastik küçük yuvarlak hücreli tümör, çocukluk çağı tümörleri, abdominal tümörler

Desmoplastic small round cell tumor (Case report)

(DSRCT) is an aggressive neoplasia that occurs mainly in childhood and involves abdominal or peritoneal sites, coexpressing ectodermal and mesenchimal immunophenotypic markers. Since it is a rare tumor, we aimed to report an additional case and discuss the differential diagnosis. Case: In this study, a 3- year- old boy with a large irregular abdominal and retroperitoneal infiltrating mass is presented. A large biopsy from the abdominal mass and a nodule from the liver were excised during explorative surgery. The surgical specimens are irregular solid fragile tissues with a grey-white cut surface. Uniform malignant round cells are observed in sharply demarcated nests and sheets in a desmoplastic stroma in microscopic examination. The nuclei are hyperchromatic, nucleoli are inconspicuous and cytoplasm is scant and eosinophilic. Immunohistochemistry showed perinucleer positivity for desmin, diffuse positivity for vimentin, cytokeratin, chromogranin, plasental alkaline phosphatase and negativity for alpha fetoprotein and CD99 in tumor cells. Conclusion: DSRCT demonstrates histopathologic and phenotypic overlap with some other malignancies. Differential diagnosis includes problems especially on tru-cut biopsies. Its aggressive course and necessity of complicated therapy requires a definite differential diagnosis

Keywords:

Desmoplastic small round cell tumor, childhood cancer, abdominal tumor,

PDF

___

Amato RJ, Ellerhorst JA, Ayala Ag. Intraabdominal desmoplastic small cell tumor. Report and discussion of five cases. Cancer 1996; 78: 845-851.
Desmoplastic round cell tumor. In: Rosai J,ed. Atlas of tumor Pathology: Tumors of soft tissues. Washington, Armed Forces Instıtute of Pathology; 2001: 452-458.
Bisagno G, Roganovich J, Sotti G, Ninfo V et al. Desmoplastic small round cell tumour in children and adolescents. Med Pediatr Oncol 2000; 34: 338-342.
Reich O, Justus J, Tamussino KF. Intraabdominal desmoplastic small round cell tumor in a 68 year old female. Eur J Gynaecol Oncol 2000; 21: 126-127.
Barnoud R, Sabourin JC, Pasquier D, Ranchere D et al. Immunohistochemical expression of WT1 by desmoplastic small round cell tumor : a comparative study with other small round cell tumors. Am J Surg Pathol 2000; 24: 830-836.
Sapi Z, Szentirmay Z, Orosz Z. Desmoplastic small round cell tumor of the pleura: a case report with further cytogenetic and ultrastuructural evidence of mesothelioblastemic origin. Eur J Surg Oncol 1999; 25: 633-634.
Liu J, Nau MM, Yeh JC, Allegra CJ wt al. Molecular heterogenity and function of EWS-WT1 fusion transcripts in desmoplastic small round cell tumors. Clin Cancer 2000; 6: 3522-3529.
Takekawa Y, Ugajin W, Koide H, Nishio S et al. Pathologic, cytologic and immunohistochemical findings of an intraabdo- minal desmoplastic small round cell tumor in a 15 year old male. Pathol Int 2000; 50: 417-420.
Reynolds PA, Smolen GA, Palmer RE, Sgroi D, Yajnik V, Gerald WL, Haber DA. Identification of a DNA-binding site and transcriptional target for the EWS-WT1(+KTS) oncoprotein. Genes Dev 2003; 17(17): 2094-107.
Su MC, Jeng YM, Chu YC. Desmoplastic small round cell tumor of the kidney. Am J Surg Pathol. 2004; 28(10): 1379-83.
Chiu LL, Koay ES, Chan NH, Salto-Tellez M. Sequence confirmation of the EWS-WT1 fusion gene transcript in the peritoneal effusion of a patient with desmoplastic small round cell tumor. Diagn Cytopathol 2003; 29(6): 341-3.
Kawano N, Inayama Y, Nagashima Y, Miyagi Y et al. Desmoplastic small round cell tumor of the paratesticular region : report of an adult case with demonstration of EWS and WT1 gene fusion using paraffin embedded tissue. Mod Pathol 1999; 12: 729-734.
Takahira K, Ohi S, Fujii N, Matsumra Y et al. Intra abdominal desmoplastic small round cell tumor (IDSRCT). J Gastroenterol 2000; 35: 712-716.
Natan JD, Gingalewski C, Salem RR. Intra-abdominal desmo- plastic small round cell tumor. Yale J Biol Med 2001; 74: 13-20.
Bosman C, Boldrini R. Unusual aspects of desmoplastic small round cell tumor. Ultrastruct Pathol 2004; 28(2): 83-96.
Kurre P, Felgenhauer JL, Miser JS, Patterson K, Hawkins DS. Successful dose-intensive treatment of desmoplastic small round cell tumor in three children. J Pediatr Hematol Oncol 2000; 22: 446-4
Bertuzzi A, Castagna L, Quagliuolo V, Ginanni V, Compasso S, Magagnoli M, Balzarotti M, Nozza A, Siracusano L, Timofeeva I, Sarina B, Parra HS, Santoro A. Prospective study of high-dose chemotherapy and autologous peripheral stem cell transplanta- tion in adult patients with advanced desmoplastic small round- cell tumour. Br J Cancer 2003; 89(7): 1159-61.