Kyoo-Ho Shin

Alveolar soft part sarcoma: MR and angiographic findings

Abstract Objective. To present the MR and angiographic findings of alveolar soft part sarcoma (ASPS). Design and patients. MR examinations (12 tumors of 10 patients) of ASPS performed at multiple hospitals were retrospectively reviewed. The tumors were found in the thigh (n=4), lower leg (n=4), femur (n=2, local metastasis), scalp (n=1) and arm (n=1). The MR signal characteristics including signal intensity, homogeneity and signal void of lesions and bony invasion including direct invasion or local metastasis were evaluated. Angiographic findings (n=4) and post-embolotherapy follow-up MR imaging (n=2) findings were also assessed. Results. Local bony metastasis was found in two cases. Seven tumors showed heterogeneous high signal intensity on T1- and T2-weighted images with good enhancement. One tumor had a very high signal on T1-weighted images. Eight tumors (67%) showed numerous signal voids in or near the tumors. All four angiographic studies showed numerous enlarged vessels, arteriovenous shunts and delayed washout. Two cases mimicked arteriovenous malformations on angiographic studies but MR images demonstrated solid soft tissue components as well as tortuous vessels. Conclusions. High signal on T1-weighted image and numerous signal voids are highly suggestive of ASPS, although they are not universal as has been suggested and arteriovenous malformation should be included in the differential diagnosis. Local bony metastases in ASPS were seen in two cases and should be carefully investigated.

Tumor volume change as a predictor of chemotherapeutic response in osteosarcoma

The change in osteosarcoma tumor volume after preoperative adjuvant chemotherapy and its relationship to the histopathologic response was investigated using various reproducible volumetric methods. Tumor volume was measured before and after chemotherapy in 41 patients with osteosarcoma using an ellipsoid formula in plain radiography and magnetic resonance imaging and three-dimensional magnetic resonance imaging measurement. Based on intraobserver and interobserver variability for the volumetric measurements of each method, three-dimensional magnetic resonance imaging measurement was the most reproducible. In three-dimensional magnetic resonance measurements, the correlation of the histopathologic response with absolute and relative total tumor volume changes and extraosseous volume change were significant. The good responder group showed a greater reduction in tumor volume after chemotherapy, although there was no significant difference in pretreatment tumor volume between the good and poor responder groups. The group with a decreased or stable tumor volume represented a good histopathologic response with a sensitivity of 85%, specificity of 76%, and positive predictive value of 88%. The change in tumor volume of osteosarcoma measured by three-dimensional magnetic resonance imaging could predict histopathologic response after three cycles of neoadjuvant chemotherapy.

Venous malformations: Sclerotherapy with a mixture of ethanol and lipiodol

PurposeTo evaluate the usefulness of a mixture of absolute ethanol and lipiodol in the management of venous malformations.MethodsPercutaneous sclerotherapy was performed with a mixture of absolute ethanol and lipiodol (9∶1) in 17 patients with venous malformations, once in 12 patients, twice in 5. The therapeutic efficacy was evaluated by pain reduction. Conventional radiographs (n=15) and posttreatment magnetic resonance imaging (n=5) were obtained for the follow-up evaluation.ResultsSclerotherapy was successful in all but two patients. The therapeutic effect was excellent in two patients, good in seven, fair in five, and poor in one. Radiopacity of lipiodol was beneficial for monitoring the procedure rather than for follow-up evaluations. Areas with low signal-intensity strands were increased on T2-weighted images obtained after the sclerotherapy.ConclusionSclerotherapy with a mixture of ethanol and lipiodol is effective in treating venous malformations.

MR appearance of distal femoral cortical irregularity (cortical desmoid)

PURPOSE Our goal was to describe the MR appearance of distal femoral cortical irregularity (DFCI). METHOD With plain radiographs and MR images of 100 knees, the presence of DFCIs was determined, and the shapes of DFCIs were classified into three subgroups: concave, convex, and divergent cortical shapes. Radiographic and MR shapes of DFCIs were compared. RESULTS DFCIs were shown in various shapes on both the radiographs and the MR images. Forty-four DFCIs were found both on radiograph and by MR image. An additional 14 DFCIs were identifiable only on MR images. However, the majority of DFCIs showed an association between radiographic and MR shapes. MRI revealed that all 58 DFCIs were located at the attachment site of the medial gastrocnemius muscle. DFCIs were enhanced in three of the four patients who underwent postcontrast MR study. CONCLUSION A good understanding of radiographic and MR findings of the DFCI may be of great help in the differential diagnosis of distal femoral lesions.

Limb salvage using original low heat-treated tumor-bearing bone.

Limb salvage, using original low heat-treated tumor-bearing bone and a conventional joint prosthesis, was done in six patients with malignant tumors of the proximal humerus (one patient with chondrosarcoma and five patients with osteosarcoma) and in six patients with tumor of the proximal femur (two patients with malignant spindle cell sarcoma and four patients with osteosarcoma). Wide excision of the lesion was done and the tumor and surrounding soft tissues were removed. The excised bone was treated with heat and the prosthesis was inserted into the treated bone and fixed with cement. This construct was reinserted into the original site and anchored to the host bone with a plate. The overall union rate of the low heat-treated bone with normal host bone was 91.7%, and the mean union time was 4.6 months (range, 3–7 months) after surgery. The functional result of the proximal femur and proximal humerus were 76.7% and 56.8%, respectively, using the Musculoskeletal Tumor Society functional evaluation system. Complications included hip dislocation in one patient, fracture of the low heat-treated bone in two patients, and absorption of the low heat-treated bone of the humerus in four of six patients. The 5-year survival rate of the low heat-treated tumor-bearing bone was 83.3% using Kaplan-Meier survival analysis. Based on the results of this study, limb salvage using original low heat-treated tumor-bearing bone seems to be effective in treating primary bone sarcoma with high survival and acceptable complication rates, circumventing the complications of allograft bone.

Intravascular papillary endothelial hyperplasia of the extremities: MR imaging findings with pathologic correlation

We report the MRI findings of three cases of intravascular papillary endothelial hyperplasia (IPEH) of the extremities with correlation of the pathologic findings. The IPEH is a non-neoplastic reactive lesion within the vessels and is commonly associated with thrombi. Signal intensity of the IPEH is complex due to the thrombi and the PEH itself. The thrombi are characterized by a slightly hyperintense signal on T1- and T2-weighted images compared with that of muscle when it comes at the medium stage of hemorrhage. Papillary endothelial hyperplastic tissue appears either as iso- or hyperintense to the muscle on T2- and T1-weighted images and shows variable enhancement on Gd-DTPA-enhanced images.

Potential of nucleofected human MSCs for insulin secretion.

The goal of this experiment was to generate insulin‐producing human mesenchymal stem cells (hMSCs) as a therapeutic source for type I diabetes mellitus, which is caused by insulin deficiency due to the destruction of islet β cells. In various trials for the treatment of type I diabetes, cell‐based therapy using adult stem cells is considered to be one of the most useful candidates for the treatment. In this experiment, a non‐viral method called nucleofection was used to transfect hMSCs with pEGFP‐C2 and furin‐cleavable human preproinsulin gene (hPPI) to produce insulin‐secreting cells as surrogate β cells. Transfection efficiency was determined using flow cytometry analysis. Expression and production of insulin were tested using RT–PCR and ELISA. The expression, production and maturation of insulin from the genetically engineered hMSCs showed an increase when compared with a non‐transfected control group. Insulin expression from hMSCs using nucleofection in this study has shown the potential for type I diabetes therapy. For further study, an evaluation for in vivo experiments and clinical applications must be supplemented. Copyright

Multiple neurilemomas. A case report.

Multiple neurilemomas in diverse locations of the body developed in a 53-year-old woman. The patient had multiple neurilemomas which occurred in the thoracic spine, lumbar spine, retroperitoneal sympathetic chain, sacral nerve root, femoral nerve, both sciatic nerves, radial nerve, and ulnar nerve without evidence of Von Recklinghausens disease. This is the first well documented report on multiple neurilemomas with whole body distribution.

Postoperative nomogram to predict the probability of metastasis in Enneking stage IIB extremity osteosarcoma

BackgroundMetastasis is the most crucial prognostic factor in osteosarcoma. The goal of this study was to develop a new nomogram to predict the probability of metastasis in Enneking stage IIB extremity osteosarcoma after neoadjuvant chemotherapy and limb salvage surgery.MethodsWe examined medical records of 91 patients who had undergone surgery between March 1994 and March 2007. A nomogram was developed using multivariate logistic regression. The nomogram was validated internally by bootstrapping-method (200 repetitions) and externally in independent validation set (n = 34). A Youden-derived cutoff value was assigned to the nomogram to predict dichotomous outcomes for metastasis.ResultsThe nomogram was built from four predictors of tumor site, serum alkaline phosphatase, intracapsular extension, and Huvos grade, and an additional clause that the cutoff value should be added to the total points in the cases of incomplete surgical resection. P-value of Hosmer and Lemshow Goodness-of-fit test of this model was 0.649. Area under receiver operating curve values of 0.83 (95% confidence interval [CI], 0.75 to 0.92) in the training set and 0.80 (95% CI, 0.63 to 0.96) in the validation set were obtained. The accuracy of dichotomous outcomes was 79.1% (95% CI, 0.69 to 0.86) and 82.4% (95% CI, 0.63 to 0.92) in the training and validation sets.ConclusionsWe have developed a new high-performance nomogram to predict the probability of metastasis in Enneking stage IIB extremity osteosarcoma after limb salvage surgery.

Tumor Volume Change after Chemotheraphy as a Predictive Factor of Disease Free Survival for Osteosarcoma

Change in tumor volume after chemotherapy appears to have a prognostic significance for the outcome of osteosarcoma. A newly developed volume measurement method based on three-dimensional summation with a proved reproducibility was utilized to measure osteosarcoma tumor volume. This retrospective analysis included 38 patients with biopsy-proven, nonsurface, skeletal high-grade osteosarcoma. The treatment was started by using three cycles of preoperative chemotherapy with cisplastin (100 mg/m2) and adriamycin (30 mg/m2). The tumor volume was measured before and after preoperative chemotherapy using three-dimensional magnetic resonance image measurement. The percentage of tumor necrosis was assessed by pathologic exam. After three cycle of postoperative chemotherapy, the patients were followed up at regular interval. For the 23 good responder patients, the mean survival time was 73.2 months (95% confidence interval 61.9 - 84.5 months), and for the 15 poor responder patients, the mean survival time was 50.8 months (95% confidence interval 38.6 - 63.1 months) (p<0.05). For the 14 patients with increased tumor volume after chemotherapy, the mean survival time was 47.5 months (range: 36.3 - 58.6 months) and for the 24 patients with stable or decreased tumor volume, the mean survival time was 74.3 months (range: 63.79 - 84.88 months) (p<0.05). Among the various factors, histopathologic response and tumor volume change after chemotherapy predicted disease free survival (p<0.05). Change in the tumor volume that was measured with a reproducible method and the histopathologic response after chemotherapy were the important predictors of disease free survival for osteosarcoma patients.