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Bone and soft tissue sarcomas, tumors of the skin

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No 3 (2010)

EDITORIAL

3-9 84
Abstract
Metastatic disease of the spine is one of the most actual problems in modern oncology, because of moderately increasing number of these patients and opportunities in different treatment modalities. This problem is on the edge of different specialties such as: neurology, orthopedic surgery, oncology. So the decision making team has to be multidisciplinary to suggest an adequate treatment.

BONE TUMORS

10-15 67
Abstract
Objective. To demonstrate surgical treatment outcomes for combined modality treatment in patients with metastases in long bones. Materials and methods. Over the periods 2006-2010 surgery for metastases in long bones has been performed in 37 patients. Solitary metastases occurred in 10 (28%) cases and multiple metastases were diagnosed in 27 (72%) patients. The danger of pathological fracture was in 17 (46%) patients and established fractures were diagnosed in 20 (54%) cases. Segmental resection with endoprosthetic replacement was done in 14 (38%) patients. External (intramedullary nailing and external fixation) osteosynthesis was done in 23 (62%) patients. Results. There were no intraoperative complications. Patients which received orthopedics care for metastases in long bones were activated on the 2-7 day. 33 (89%) patients had no or mild pain syndrome in postoperative period. Improvement of general condition after surgery occurred in 35 (94%) patients. Improvement of orthopedic status was noticed in 32 (86%) patients. Oncological criteria included assessment of survival by Kaplan Meier survival analysis. The follow-up ranged from 2 to 55 months. The highest mean follow-up period of 21 months was in patients with metastases ofbreast cancer. Conclusion. Appropriate recovery of functional activity using organ preserving surgery (endoprosthetic replacement, external osteosynthesis) in orthopedics in patients with bone metastases improves quality of their life and is very important for social and psychological adaptation.
16-29 297
Abstract
Aims. Sarcomas of the skull base is a rare pathology. Head and neck involved only in 15-20% of all sarcomas cases. Local relapse is the main reason of cure fails in patients (pts) with sarcomas of the skull base. In this link forced local control is the corner stone of successful treatment. Materials and methods. 81 pts with different types of sarcomas of the skull base treated at our clinic during the last 30 years. Pts age varied between 13-80 years. The majority of soft tissue sarcomas (n=44) consisted of tumors located in infratemporal fossa - 50% (n=22) and paranasal sinuses - 34,1% (n=15). Of bone sarcomas it were maxilla - 38,5% (n=15), ethmoid bone - 20,5% (n=8) and mandibula with second involvement of infratemporal fossa - 12,8% (n=5). The main morphological types of soft tissue sarcomas were - rabdomyosarcoma (29,5%, n=13), malignant shwannoma (22,7%, n=10) and malignant fibrous hystiocytoma (18,2%, n=8). In a group of bone sarcomas they were - osteosarcoma (29,7%, n=ll) and chondrosarcoma (24 ,3%, n=9). Previously untreated cases diagnosed in 68 pts and 13 pts had recurrence sarcomas. The majority of cases consisted of advanced tumors in the limits of the scull base. Cure tactic in a study group depends on several factors: tumor morphology, tumor volume and chemoradio sensitivity. The main treatment choice was surgery (70%, n=56) alone or in combination with chemoradiotherapy (32,1%). 22(27,5%) pts had conservative treatment course. Different types of surgical approaches were used for tumor removal: transfacial resections (n=32), combined craniofacial resections (n=6), resections of the lateral skull base (n=16), posterior skull base approaches (n=2). Results. The most favorable 5-year overall survival rate was pointed out for a soft tissue sarcomas - 33,2%. 5-year overall survival rate for embryonal type of rabdomyosarcoma was 24,3% (median 13,5 months), chondrosarcoma - 23,5% (median 23 months). The worse results were pictured for malignant shwannoma, osteosarcoma and Ewing/PNET sarcomas where none of the pts overlive 5 year period. Pts with chemoradiotherapy had little improvement in a group of soft tissue sarcomas. Chemoradiotherapy had not any benefits in treatment of bone sarcomas, so 5-year overall survival rate was most favorable in a group of surgical cure - 23% (median 16,5 months) in comparison with conservative (6,3%, median 16 months) and complex treatment (0%, median 12 months) (p=0,l). Total tumor resection was the main prognostic factor associated with better outcome. Local recurrence rate was significantly higher in a group of unradical operations - 73,3% vs 51,2% (radical surgery) (p=0,02). Among factors associated with negative prognosis were - recurrence tumors, primary regional node involvement, tumor spread into the cavernous sinus, primary tumor involvement of the posterior skull base or combined destruction of anterior and lateral skull base. Conclusions. Embryonal rabdomyosarcoma and chondrosarcoma associated with the most favorable follow-up. Chemoradiotherapy doesn’t improve outcome for bone sarcomas and malignant shwannoma of the skull base. On the other hand chemoradiotherapy can potentially decrease local recurrence rate in a group of soft tissue sarcomas. Surgery appears to be a method of choice in a treatment of the majority of skull base sarcomas, especially of bone sarcomas, residual and chemoradioresistant tumors.
30-33 78
Abstract
We have analyzed the long-term results of treatment in 70 patients with localized forms of osteosarcoma who received therapy at MRRC RAMS in the years 1990-2008. In IORT groups, the five-year recurrence-free survival rates for osteosarcoma (no recurrence) were significantly higher than those in control groups. IORT did not appear to increase the complication rate and hamper the single-stage reconstruction of osteoarticular defects.
34-38 59
Abstract
Expression of VEGF and its receptors and proliferative activities were studied in cells of giant cell tumor of the bone. Immuno-histochemical analysis was performed on 11 formalin-fixed, formalin-embedded samples of giant cell tumor of the bone. VEGF and its receptors VEGFR-1 (Flt-1) and VEGFR-2 (Flk-l/KDR) was detected in all specimens and all cellular components such as reactive osteoclast-like giant cells and stromal cells. Ki-67 expression was limited to the mononuclear stromal cell component of the tumors. The high degree of expression Flk-1 and Flt-1 significantly correlated with high proliferative activity of the tumor (≥5 %) (p=0,031 for Flk-1 and p=0,048 for Flt-1). These results suggest that VEGF receptor-ligand system might play a role in the pathogenesis of giant cell tumor of the bone. The further research should help to explain a unique nature and cell interaction mechanisms of this tumor.

SOFT TISSUE SARCOMAS

39-43 76
Abstract
The patient described suffers from disseminated idiopathic Kaposi’s sarcoma and secondary systemic amyloidosis complicated with development of renal insufficiency. Complex therapy in combination with hemodialysis resulted in the regress of the tumor.
44-48 240
Abstract
A 4 and 8 month old boys and 1,5 year boy presented with a 6 week history of a skull mass of the anterior fontanelle. The mass was excised, and the histopathologic features were diagnostic for melanotic neuroectodermal tumor of infancy. The tumors consisted of a biphasic cell population in a fibrous stroma. The small, darkly staining cells comprising the first population stained positively for NSE, chromogranin and synaptophysin, showing them to be neuroblasts, and the large epithelioid cells comprising the second cell population stained positively with the antibodies CK7, HMB 45, Melan A and TTF 1, showing them to be melanocytes. Ultrastructural examination confirmed the biphasic nature of the tumor cells, with secretory granules and neurofilaments in the neuroblastic population, and melanosomes in the epithelioid population. It is the identification of the epithelioid cells in the smears which differentiates this tumor from other round cell tumors of infancy and helps in the correct diagnosis. These results add to the evidence supporting the neural crest origin of melanotic neuroectodermal tumor of infancy and confirm its formation from two distinct cell lines.

TUMORS OF THE SKIN

49-53 72
Abstract
20 patients were examined during studying of ultrasonic picture of different morphological types of skin cancer Specific features of ultrasonic picture of skin structures in lesion focus were discovered, and also their vascularization.

Concomitant and auxiliary therapy

54-62 109
Abstract
Neutropenia is one of the most often life-threatening adverse events after cytotoxic chemotherapy. During the past two decades myeloid growth factors are used for the reducing the risk of neutripenia. Current recommendations allow routine usage of growth factors when the risk of neutropenia is more than 20%. Myeloid growth factors reduce neutropenia duration and make the possibility to give dose-dense and dose-intensive regimens in a preplanned schedule. Pegfilgrastim is a pegilated recombinant granulocyte colonystimulating factor (GCSF) with a prolonged mechanism of action. Clinical trials data showed that pegfilgrastim is more effective in decreasing of febril neutropenia comparing to filgrastim. This article reviews neutropenia and its complications and pegfilgrastin clinical experience.

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ISSN 2219-4614 (Print)
ISSN 2782-3687 (Online)