Case Report: Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy Application in Intraperitoneally Disseminated Inflammatory Myofibroblastic Tumor and in the Youngest Patient in the World: New Indication and Modification of Technique

Abstract

Introduction: Peritoneal metastases occur in cancers that spread to the peritoneal cavity and indicate the advanced stage of the disease. In children they are mainly seen in sarcomas, Gastrointestinal Stromal Tumors and primary disseminated ovarian tumors. Inflammatory Myofibroblastic Tumor (IMT) is a very rare lesion, characterized by an unpredictable clinical course. The absorption of chemotherapeutic agents through the peritoneal-plasma barrier (PPB) is minimized, thus HIPEC procedure limits the systemic exposure to chemotherapy and permits the administration of its higher doses. The main purpose of HIPEC is to remove the visible macroscopic disease in order to achieve complete cytoreduction (CRS).HIPEC Procedure in Children: Several papers deal with the CRS and HIPEC in children and adolescents, however pediatric experience is still limited. Thus far, the HIPEC procedure has been carried out on patients over 2 years old. The most common indication for the surgery and the best outcome was experienced by patients with desmoplastic small round cell tumor (DSRCT). Most patients received intraperitoneal cisplatin.HIPEC Modification: A 5-month-old infant was admitted to the Department of Pediatric Oncology due to the abdominal distention and blood in the stool. The Computed Tomography (CT) revealed a solid-cystic mass in the right abdominal area. The primary tumor and numerous peritoneal metastasis were removed and the Inflammatory Myofibroblastic Tumor (IMT) was diagnosed. The patient underwent subsequently CRS and modified HIPEC procedure. To avoid overheating of the infant, the intraperitoneal normothermic chemoperfusion was performed. Due to the low body weight a modified dosage of intraperitoneal doxorubicin was used. The child underwent standard postoperative chemotherapy and received crizotinib therapy. At 12 months follow-up since treatment completion the patient remains in complete remission. To our knowledge this is the youngest patient, the only infant and the first pediatric patient with IMT who underwent the modified HIPEC procedure in the world.Conclusions: CRS and HIPEC is technically possible also in infants. For its safe course patients selection and technique modification are necessary. Use of HIPEC should be also considered in intraperitoneally disseminated IMT. A complete cytoreductive surgery as the first HIPEC step seems to be the key factor in survival.

Changing incidence and survival of desmoplastic small round cell tumor in the USA

“…5,6,[13][14][15] Several trials involving hyperthermic intraperitoneal chemotherapy, immunotherapy, and radioimmunotherapy are underway. [16][17][18] Since there were no cases of treatment with surgery and radiotherapy, Cox regression was not conducted to avoid confounding. According to the Kaplan-Meier curves, surgery alone showed more prolonged survival than surgery with adjuvant therapy; a possible hypothesis is the aggressive presentation of those cases.…”

A Review of the Use of Hyperthermic Intraperitoneal Chemotherapy for Peritoneal Malignancy in Pediatric Patients

“…The decision was made after two weeks to pursue complete CRS and infuse doxorubicin NIPEC followed by adjuvant chemotherapy and crizotinib. At 12 months follow-up since treatment completion, the patient remains in complete remission [43].…”

Latest Advances in the Management of Pediatric Gastrointestinal Stromal Tumors

“…As peritoneal metastases may be present in pediatric GISTs, one of the methods used to remove the visible macroscopic disease is HIPEC (hyperthermic intraperitoneal chemotherapy) with complete cytoreduction (CRS) that can be proceeded even in infant treatment [ 95 ]. There is still little data concerning the use of HIPEC not only in pediatrics, but also adult patients, especially in respect to GIST, though its use in peritoneal sarcomatosis is gaining more attention.…”

“…Importantly, HIPEC procedure in pediatric patients was linked with a smaller mortality and mortality as in adult population [ 96 , 97 , 98 ]. However, owing to the safety of the procedure patient selection and technique modification is necessary, and all important considerations were summarized by Garnier et al CRS preceding HIPEC makes up the key factor in survival [ 95 ]. It is unknown, however, which cytostatic agent should be used in HIPEC treatment of GIST—there are reports on the use of mitomycin C.…”