Atypical presentation of currarino syndrome: A case report.

INTRODUCTION: Currarino syndrome is a rare congenital disorder characterized by a triad of anorectal malformation, a sacral bone defect, and a presacral mass. It results of an abnormal separation of the ectoderm from the endoderm caused by HLXB9 mutation in chromosome 7q36 in 50% of cases. The disorder is mostly hereditary as it can also be sporadic with a variable expression spectrum. PRESENTATION OF CASE: The case of a previously healthy 3-month-old girl with abdominal distension, post-prandial vomiting, obstipation, and anuria of 5 days' history is presented in this article. Abdomino-pelvic magnetic resonance imaging (MRI) showed a large cystic multilobulated mass in the sacrococcygeal region with a dural communication evident of an anterior sacral meningocele. 1 year later, the child came back with constipation and was found to a have a malignant mixed germ cell tumor in the presacral area, a very rare presentation in Currarino syndrome. DISCUSSION: In a child presenting with at least one of the features of Currarino syndrome's triad, a diagnosis should be suspected. After reviewing the literature, the syndrome is usually missed and hence is under diagnosed. MRI is the best imaging modality for diagnostics and follow-up for any mass, benign or malignant, can bring life saving measures. Most masses are benign but can undergo malignant transformation even after resection. De novo malignancy is very rare and is described in our case. CONCLUSION: Physicians treating patients with spinal dysraphism should suspect a diagnosis of Currarino syndrome by follow up imaging for any new benign or malignant growth.

Selective intra-arterial chemoembolization of pelvic and spine bone metastases.

The purpose of this study was to determine the effect of interventional palliative therapy by using chemoembolization on metastatic bone pain and tumor bulk in inoperable metastases where chemotherapy and radiotherapy had failed. Twenty-five patients (mean age: 59 years) underwent chemoembolization of symptomatic lytic lesions involving the spinal column (n=10), iliac bone and sacrum (n=15). The study design consisted of at least three procedures based on combined chemoembolization performed under analog-sedation. Therapeutic agents were carboplatin for selective chemotherapy and pirarubicin mixed with polyvinyl alcohol foam for chemoembolization. Fifteen of 18 (83%) patients had significant pain relief, as shown by the decrease of analgesic drug use. Mean clinical response duration was 12 months. Radiologically, ten patients were stable. A partial response was observed in four patients, while a complete response was seen in two others. Selective intra-arterial chemoembolization gives longer pain relief than embolization, compared to the literature data, probably because of partial response with local anti-cancer drugs.

In vitro polyvinylformaldehyde particle compatibility with chemotherapeutic drugs used for chemoembolization therapy.

PURPOSE: Because the effects of pirarubicin and carboplatin on the physical structure of particles made from polyvinylformaldehyde are not well known, we describe an experiment to test the in vitro polyvinylformaldehyde particle compatibility with these drugs used for chemoembolization of bone metastases. MATERIALS AND METHODS: Polyvinylformaldehyde particles (Ultra-Drivalon) were mixed in vitro with either pirarubicin or carboplatin as experimental samples, and with distilled water as control samples, and left for 24 h at 37 degrees C. The particles used measured 150-250 microm and 600-1000 microm in diameter. Particle morphology, including appearance, overall shape, and surface characteristics were examined using a microscope equipped with a videocamera. Particle size was measured by granulometry. Qualitative and quantitative variables were analyzed using, respectively, the two-sided Fisher's exact test and the Wilcoxon signed rank test for paired values, with a significance level of 0.05. RESULTS: No broken particles or microscopic degradations in the appearance, overall shape, or surface characteristics of any particles were observed. The particle size distribution was not significantly different between the experimental samples containing pirarubicin or carboplatin and the control sample of particles with diameters in the same range. CONCLUSION: Particles made from polyvinylformaldehyde can be mixed with pirarubicin or carboplatin without any risk of damaging their physical properties.

In vitro study of the compatibility of tris-acryl gelatin microspheres with various chemotherapeutic agents.

PURPOSE: To evaluate the in-vitro effects of chemotherapeutic agents on the physical structure of tris-acryl gelatin microspheres. MATERIALS AND METHODS: Microspheres (Embospheres) were mixed in vitro with carboplatin, mitomycin C, 5-fluorouracil, or pirarubicin as experimental samples and with distilled water as control samples and kept for 24 hours at 37 degrees C. The microspheres used measured 100-300 micro m and 700-900 micro m in diameter. Microsphere morphology, including appearance, overall shape, smoothness of surface, and thickness of gelatin coating, was examined with use of a microscope equipped with a cold light. Microsphere dimensions including larger diameter, smaller diameter, and surface area were measured by granulometry. Microsphere geometry was evaluated by calculating a sphericity index and surface regularity index. Qualitative and quantitative variables, respectively, were analyzed with the two-sided Fisher exact test and Wilcoxon signed-rank test for paired values, with a significance level of 0.05. RESULTS: No broken microspheres or microscopic degradations in the appearance, overall shape, smoothness of surface, or thickness of gelatin coating of any microspheres were observed. The respective distributions of large and small diameters, surface area, sphericity index, or surface regularity index of the microspheres were not significantly different between the experimental samples containing carboplatin, mitomycin C, 5-fluorouracil, or pirarubicin and the control sample of microspheres with similar diameters. CONCLUSION: Embosphere microspheres can be mixed with carboplatin, mitomycin C, 5-fluorouracil, or pirarubicin for chemoembolization therapy without any risk of damaging their morphology, dimensions, or geometric characteristics.

Interventional Radiology for Treating Soft Tissue Sarcomas.

The role of interventional radiology for soft tissue sarcomas is only occasionally addressed in the literature. However, different techniques such as embolization, selective chemotherapy, chemoembolization, and acrylic cement osteoplasty can be helpful with the primary tumor, recurrences, and metastases. This article discusses these techniques and their complications in treatment of soft tissue sarcomas.