Errol U. Hutagalung

Lipoblastoma and Lipoblastomatosis of the Lower Leg

Lipoblastoma is a benign lesion of immature fat cells that is found almost exclusively in pediatric population. This tumor is a rare tumor that occurs in infancy and early childhood, accounting for less than 1% of all childhood neoplasm. It is more common in male than in female and often presents as an asymptomatic, rapidly enlarging, soft lobular mass on the extremity. Although benign, it gives great difficulty in its management, due to its extensions into different facial planes, especially in lipoblastomatosis. Thus, complete surgical excision is the treatment of choice.

Wide resection versus curettage with adjuvant therapy for giant cell tumour of bone.

Purpose To determine the association between type of surgery (wide resection versus curettage with adjuvant therapy) and outcome in patients with giant cell tumour (GCT) of bone. Methods Records of 30 male and 52 female consecutive patients aged 10 to 62 years who underwent wide resection (n=57) or curettage with adjuvant therapy (n=25) for primary GCT of bone were reviewed. The surgical decision was based on patient age, tumour location, functional demand, and patient preference. The median tumour size was 8.5 cm. Tumours were classified as stage 1 (n=4), stage 2 (n=60), and stage 3 (n=18), and 25%, 68.3%, and 83.3% of them were treated with wide resection, respectively. Functional outcome was assessed using the Musculoskeletal Tumor Society (MSTS) score; the maximum score was 30. Results The wide resection and curettage with adjuvant therapy groups were comparable in terms of patient age, gender, tumour size, location, symptoms, tumour stage, type of biopsy, and MSTS score. The MSTS score was excellent in 50.2% of patients, good in 38.7% of patients, and fair and poor in the remaining patients. The MSTS score was not associated with tumour stage or type of surgery. Four patients in the wide resection group had metastasis to the lung. They also had lower haemoglobin level (10.6 vs. 12.7 g/dl, p=0.020) and higher percentage of stage-3 tumour (100% vs. 17.9%, p=0.001) but had no recurrence (0% vs. 6.4%, p=0.774), compared with those without metastatsis. All died from massive haemoptysis and respiratory failure. Eight patients died; their haemoglobin level was lower than that of patients who were still living (11.2 vs. 12.7 g/dl, p=0.032). Mortality was associated with metastasis (100% vs 5.2%, p<0.001) but not recurrence or complication. Two patients in each group had recurrence; recurrence was not associated with type of surgery. Conclusion There was no association between type of surgery and tumour recurrence, metastasis, or outcome. Curettage with adjuvant therapy was more commonly performed for stage 1 and 2 tumours, whereas wide resection was more for stage 3 tumours. Metastasis was associated with stage 3 tumour and mortality but not recurrence.

Correlation between Survival and Tumour Characteristics in Patients with Chondrosarcoma

Purpose. To evaluate the correlation between survival and tumour characteristics in 23 patients with chondrosarcoma. Methods. Records of 15 men and 8 women aged 14 to 66 (mean, 37) years who were diagnosed with primary (n=19) or secondary (n=4) chondrosarcoma of the axial skeleton (n=8), proximal extremity (n=9), or distal extremity (n=6) were reviewed. The tumour diameter was <10 cm in 4 patients, 10–19 cm in 12, and 20–30 cm in 7. The tumour involved the intramedullary in 17 and the periosteum in 6 patients; tumour extension was intracompartmental in 5 and extracompartmental in 18 patients. The Evans histological grade for the tumours was grade 1 in 6 patients, grade 2 in 10, and grade 3 in 7. The mean tumour size was 12.3 cm for grade 1 tumours, 18.2 cm for grade 2 tumours, and 18.3 cm for grade 3 tumours. 13 patients had no metastasis and 3 of 10 patients with grade 2 tumours and all 7 patients with grade 3 tumours had metastasis to the lung at presentation. 17 patients underwent surgery, one underwent adjuvant treatment only, and 5 declined treatment. Results. The mean follow-up period for the 23 patients was 3.1 years (range, 3 weeks to 9 years). The 5-year survival rate was 43% overall, 83.3% for grade 1 tumours, 50% for grade 2 tumours, and 0% for grade 3 tumours. The median survival duration was 20 (95% confidence interval, 11–29) months. Two patients had local recurrence and 16 did not, and the 5 patients who declined treatment died. Survival correlated with Evans histological grading (p=0.004), the presence of metastasis at presentation (p=0.026) and local recurrence (p=0.004). Conclusion. The survival rate was lower in patients with higher Evan grading, metastasis, or local recurrence.

Primary malignant mesenchymoma of bone

Pimary malignant mesenchymoma of bone (PMMB) is an exceedingly rare neoplasm consisting of two or more unrelated malignant mesenchymal components other than fibrosarcoma or malignant fibrous histiocytoma. Review of the literature reports only 16 cases, most of which were composed of osteosarcoma and liposarcoma. We report a case af PMMB composed of liposarcoma, rhabdomyosarcoma and high grade chondrosarcoma arising within the left distal femur in a 52-year-old male, resulting in the patients death 3 months after presentation. (Med J Indones 2001;10: 235-41) Keywords: Bone tumor-malignancy, liposarcoma, dedifferentiated chondrosarcoma, rhabdomyosarcoma