Desmoplastic small round cell tumor: review of therapy including surgery followed by continuous hyperthermic peritoneal
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REVIEW
Desmoplastic small round cell tumor: review of therapy including surgery followed by continuous hyperthermic peritoneal perfusion of chemotherapy Andrea Hayes-Jordan Æ Peter Anderson
Received: 26 May 2009 / Accepted: 12 August 2009 / Published online: 12 September 2009 Ó Springer-Verlag 2009
Abstract Desmoplastic small round cell tumor (DSRCT) is a very rare disease of children, adolescents, and young adults and involves the abdominal cavity. DSRCT has characteristic fusion gene involving EWS1 and WT1 translocation, t(11;22)(p13;q12). Unlike Ewing’s sarcoma of bone, DSRCT usually presents with diffuse peritoneal implants that are prone to recur. The primary organ of origin of DSRCT is mesenchyme of the peritoneum. This makes it a very unique tumor that is difficult to treat because of the infiltrative and diffuse nature of the peritoneum. The challenge of local control is to remove dozens to hundreds of tumors studding the peritoneal cavity, and then eliminate microscopic disease. We review a sequential multimodality strategy to reduce macroscopic and microscopic disease including neoadjuvant chemotherapy, aggressive surgery including an emerging new therapy to use after surgery to treat microscopic residual disease: continuous hyperthermic peritoneal chemotherapy, then radiation and adjuvant chemotherapy. Keywords Desmoplastic small round cell tumor Ewing’s family of tumors Pediatric surgery Hyperthermia Intraperitoneal chemotherapy Peritoneal metastases
A. Hayes-Jordan (&) P. Anderson University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Box 444, Houston, TX 77030, USA e-mail: [email protected] P. Anderson e-mail: [email protected]
Introduction Desmoplastic small round cell tumor (DSRCT) is a rare disease of children, adolescents, and young adults, and involves peritoneal surfaces. Less than 200 cases are reported in the world literature. Despite multimodal treatment, including aggressive surgical excision, chemotherapy, and radiotherapy, multiple series have shown that approximately 75% of patients succumb to their disease within 3 years [1–7]. The general pattern of treatment failure is recurrent disease first in the peritoneal cavity, then in the liver, and finally in distant sites. Patients typically are young (ages 5–30 years) at presentation and 80–90% are males [3, 8–11]. The reason for male preponderance is unknown. In one study androgen receptors were present in 10/27 (37%; P = 0.0045) [10]. Young people with DSRCT usually present with diffuse abdominal metastatic disease very similar in gross appearance to peritoneal carcinomatosis [12–15]. DSRCT can also involve or metastasize to other serosal locations including the pleura, ovary or testicle, and soft tissue (e.g., liver, kidney) [2, 16]. DSRCT was relatively recently described pathologically in 1991 by Gerald et al. [17]. Histologically, DSRCT consists of small round blue cell nests separated by desmoplastic stroma (Fig. 1; ref. [4, 8, 11]). The presence of a specific recurring translocation, t(11;2
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