복막에 발생한 결합조직형성 소원형세포종양 1예
A case of peritoneal desmoplastic small round cell tumor
1
조회
0
다운로드
0
인용
0
참고문헌
키워드
초록
결합조직성 소원형세포종양은 주로 젊은 연령층의 남자에게 호발하는 질환으로 드물고 예후가 좋지 않은 악성종양이다. 흔히 복부 및 골반 내 악성종양으로 나타나며 뚜렷하고 특징적인 조직학적 세포유전학적 특징을 보인다. 현재까지 다양한 치료 방법들이 시도되어 왔으나 불행히도 난치성이며 장기 생존율에서 매우 불량한 결과를 보이고 있다. 그러나 복합적인 치료 방법을 이용한 적극적인 치료를 할 경우 일부에서는 일시적인 생존율 향상을 보인다. 최근에 본원에서 매우 불량한 예후를 보인 31세 여성의 복막에서 기원한 결합조직성 소원형세포종양 1예를 경험하였기에 간단한 문헌고찰과 함께 보고하는 바이다.
Abstract
Desmoplastic small round cell tumor is a rare and aggressive tumor that affects young males. It is usually an abdominopelvic malignancy that demonstrates distinct histological appearances and a unique cytogenetic profile. There have been many different approaches to the treatment of desmoplastic small round cell tumor, but unfortunately it remains incurable and has poor long-term survival rates. However, with an aggressive approach to the treatment using multiple modalities, some temporary benefit to survival can be achieved. There has not yet been a case in which treatment has led to a curative outcome. Recently, we also experienced a case of very poor outcome of 31-year-old female with small round cell tumor in peritoneum. Here, we report the case with a brief review of literatures.
참고문헌 (15)
- Sesterhenn I, "Undifferentiated malignant epithelial tumors involving serosal surfaces of scrotum and abdomen in young males", J Urol, 137, pp.241A~ (1987)
- Zaloudek C, "Desmoplastic small cell tumor of the ovary: A unique polyphenotypic tumor with an unfavorable prognosis", Int J Gynecol Pathol, 14, pp.260~265 (1995)
- 김준경, "양측 난소를 침범한 복막의 결합조직형성 소원형세포종양 1예", Korean Journal of Obstetrics and Gynecology, 49(1), pp.224~229 (2006), 대한산부인과학회
- Amato RJ, "Intraabdominal desmoplastic small cell tumor. Report and discussion of five cases", Cancer, 78, pp.845~851 (1996)
- Gerald WL, "Clinical, pathologic, and molecular spectrum of tumors associated with t(11;22)(p13;q12): desmoplastic small round cell tumor and its variants", J Clin Oncol, 16, pp.3028~3036 (1998)
- Kretschmar CS, "Crombleholme TM. Desmoplastic small cell tumor: A report of three cases and a review of the literature", J Pediatr Hematol Oncol, 18, pp.293~298 (1996)
- Sawyer JR, "A novel reciprocal chromosome translocation t(11;22)(p23;q12) in an intra-abdominal desmoplastic small round-cell tumor", Am J Surg Pathol, 16, pp.411~416 (1992)
- Wolf AN, "The expanding clinical spectrum of desmoplastic small round-cell tumor: A report of two cases with molecular confirmation", Hum Pathol, 30, pp.430~435 (1999)
- Yoshizawa J, "A case of intra-abdominal desmoplastic small-round-cell tumor with elevated serum CA125", Pediatr Surg Int, 18, pp.238~240 (2002)
- Gerald WL, "Intra-abdominal desmoplastic samll round-cell tumor: Report of 19 cases of a distinctive type of high-grade polyphenotypic malignancy affecting young individuals", Am J Surg Pathol, 15, pp.499~513 (1991)
- Slomovitz BM, "Desmoplastic small round cell tumor with primary ovarian involvement: case report and review", Gynecol Oncol, 79, pp.124~128 (2000)
- Pickhardt PJ, "Desmoplastic small round cell tumor of the abdomen: radiologic-histopathologic correlation", Radiology, 210, pp.633~638 (1999)
- Varma DG, "Primary malignant small round cell tumor of the abdomen: CT findings in five cases", AJR Am J Roentgenol, 158, pp.1031~1034 (1992)
- Kushner BH, "Desmoplastic small round-cell tumor: Prolonged progression-free survival with aggressive multimodality therapy", J Clin Oncol, 14, pp.1526~1531 (1996)
- Akhtar M, "Small round cell tumor wkth divergent differentiation: cytologic, histologic, and ultrastructural findings", Diagn Cytopathol, 11, pp.159~164 (1994)