I. De Wever

Lesions of 13q may occur independently of deletion of 16q in spindle cell/pleomorphic lipomas.

Very recent multidisciplinary investigations have allowed for the definition among lipomas of a clinical and histological subtype called spindle cell and/or pleomorphic lipoma, possibly associated with partial monosomy 16 and anomalies of chromosome 13. In order to get nearer to the underlying critical molecular changes further multidisciplinary pathological and genetic research is indicated, to identify which chromosome(s) anomalies are crucial in the development of these tumours.

Changing phenotype of gastrointestinal stromal tumours under imatinib mesylate treatment: a potential diagnostic pitfall

Aims : The diagnosis of gastrointesinal stromal tumours (GISTs) is widely based on morphological features and KIT (CD117) immunoreactivity. Most patients with advanced GISTs show a major clinical response after treatment with imatinib mesylate. The histopathological features of GISTs in patients on prolonged imatinib treatment have, thus far, not been addressed in detail. In this report, we present three patients with metastatic GISTs, who received more than 1 year of therapy with imatinib, and whose tumours changed their morphological and immunohistochemical characteristics during continued treatment with the drug.

Treatment of VX2 liver tumor in rabbits with "wet" electrode mediated radio-frequency ablation.

Abstract. Radio-frequency ablation (RFA) has been considered as an alternative therapy for liver tumors. A “wet” electrode with interstitial infusion of hypertonic saline was tested for the RFA of liver tumor in rabbits. Seventy-eight liver tumors (? 1.5 to 3.0 cm) were induced in 41 rabbits by VX2 carcinoma implantation. Fifty-one tumors in 27 rabbits were treated with RFA. Under laparotomy, the RF energy was delivered while 5 % saline was infused through the electrode into the tumor at 1 ml/min. Six rabbits with 12 tumors were treated with only intratumoral 5 % saline infusion without RFA. Another 8 rabbits with 15 tumors received sham operation as untreated controls. The efficacy of the therapy was evaluated with survival rate, MRI, microangiography, and histopathology. In the RFA group, 6 rabbits survived longer than 6 months (absolute eradication rate 22.2 %); 12 rabbits were found free of viable tumor at the moment when they were sacrificed (relative eradication rate 44.4 %); 9 rabbits showed local tumor relapse and/or lung metastasis 2–10 weeks after ablation (recurrent rate 33.3 %). In control groups of saline infusion and sham operation, all 14 rabbits died within 3 months (mortality rate 100 %). Three-month survival rates between RFA group and control groups were significantly different (p < 0.05). Findings of MRI, microangiography, and histology supported these outcomes. Radical treatment of liver malignancy in rabbits is possible with the present modified RFA technique. Its clinical usefulness has to be further proven.

Cytogenetic evidence of clonality in cutaneous benign fibrous histiocytomas: a report of the CHAMP Study Group

Aims

Proliferative myositis in a child

Abstract A case of proliferative myositis in the lumbar paraspinal muscles in a 14-year-old boy is presented. Imaging investigations including plain radiograph, ultrasound, computed tomography (CT), magnetic resonance imaging (MRI), bone scan and positron emission tomography (PET) were suggestive of an inflammatory process such as myositis ossificans. The diagnosis was made by incisional biopsy. More pronounced edema, more muscle fiber necrosis and a higher cellularity were found compared to adult cases. The karyotype of the lesion was normal. Clinically, the mass disappeared spontaneously. After 24 months, asymptomatic bridging ossification between the third and fourth lumbar vertebrae was noted.

PLEUROPULMONARY BLASTOMA (PULMONARY BLASTOMA OF CHILDHOOD): GENETIC LINK WITH OTHER EMBRYONAL MALIGNANCIES?

A case of pleuropulmonary blastoma (childhood variant of pulmonary blastoma) was examined using histological, immunohistochemical, ultrastructural and cytogenetic methods. The tumour consisted of undifferentiated ‘blastematous’ areas admixed with zones of rhabdomyoblastic and chondroid differentiation and fascicular areas. Desmin and S‐100 protein immunoreactivity confirmed the myogenic and cartilaginous differentiation. Ultrastructurally only undifferentiated mesenchymal cells were present. The cytogenetic analysis revealed abnormalities of 2q. Involvement of 2q has also been described in hepatoblastoma and embryonal rhabdomyosarcoma. Although further confirmation is needed, our cytogenetic findings in pleuropulmonary blastoma suggest common genetic mechanisms in some paediatric embryonal malignancies.

V-Y BILATERAL GLUTEUS MAXIMUS MYOCUTANEOUS ADVANCEMENT FLAP IN THE RECONSTRUCTION OF LARGE PERINEAL DEFECTS AFTER RESECTION OF PELVIC MALIGNANCIES

Objective  To evaluate the role of the V‐Y bilateral gluteus maximus myocutaneous flap (GLM) in the reconstruction of large perineal defects after wide surgical resections for pelvic malignancies.

Cytogenetic characterization of congenital or infantile fibrosarcoma

Chromosome analysis of a congenital or infantile fibrosarcoma from the lower left leg of a 3-week-old baby girl showed only numerical changes involving chromosomes 11, 17 and 20. As three more cases with similar combinations of trisomies of the same chromosomes have been described, this report confirms that adult and congenital fibrosarcoma are cytogenetically different and trisomy 11 may be the key-event.

Fibrolipomatous hamartoma in the foot: atypical MR imaging findings

Lipomatosis of a nerve is a well-known but uncommon entity mostly seen in the median nerve. Magnetic resonance imaging (MRI) has been shown to provide pathognomonic features, obviating the need for diagnostic biopsy. We present a case of lipomatosis of a branch of the medial plantar nerve with an atypical appearance on magnetic resonance imaging.

Ecteinascidin-743: Evidence of Activity in Advanced, Pretreated Soft Tissue and Bone Sarcoma Patients

Purpose. To evaluate the activity and safety of ecteinascidin (ET-743) in pretreated patients with advanced or metastatic soft tissue and bone sarcoma. Patients or subjects. Eighty-nine patients received ET-743 as a 24-hour continuous infusion at a dose of 900–1500 μg/m2 every 3 weeks. Results. We observed one complete remission, 5 partial remissions, one minimal response, and 16 patients with a disease stabilization of 6 months or more. The objective response rate was 6.7% and the clinical benefit rate at 3 and 6 months was 37.7% and 23.4%, respectively. Responses were noted in patients with lipo-, leiomyo-, osteo-, and myogenic sarcoma, with a median duration of 9.85 months. Toxicity mainly involved an asymptomatic elevation of transaminases and neutropenia. Estimated 1- and 2-year survival rates were 39.4% and 15.8%. Median overall survival was 8.25 months. Discussion. This retrospective analysis confirms that ET-743 induces objective responses and progression arrest in a clinically relevant proportion of patients.