Spinal Melorheostosis: A Rare Presentation.

CASE: A 22-year-old female patient was referred to the orthopaedic department for further examination after a radiopaque area was observed in the T6 vertebra in her chest radiograph. Computed Tomography (CT) showed a sclerotic mass with smooth borders, involving the entire body of the T6 vertebra, left posterior elements, posterior of the rib past the left zygapophyseal joint, and a "flowing candle wax" image toward the T7 vertebra. Spinal melorheostosis was considered radiologically in the patient, but malignancy could not be completely excluded. Thereupon, open biopsy was performed under general anesthesia. CONCLUSION: Spinal melorheostosis is a rare condition. Histological examination should be considered in cases where the diagnosis remains suspicious after clinical and radiological evaluations.

Pseudomyogenic (epithelioid sarcoma-like) hemangioendothelioma of bone: Clinicopathologic features of 5 cases.

Pseudomyogenic hemangioendothelioma (PHE) is an uncommon mesenchymal tumor of intermediate malignant potential with characteristic clinicopathologic and genetic features. Although bone involvement accompanies nearly one-fourth of reported cases of soft tissue PHEs, primary intraosseous PHE is rare. Herein, we report five cases of primary intraosseous PHEs. Male to female ratio was 4:1, with an average age of 28 years (age range, 5-44 years). Radiologically, tumors presented as lytic lesions in the proximal femur (two), diaphysis of the tibia (one), distal radius (one) and vertebrae (one). Multifocal lesions were observed in four cases. Histopathologic examination revealed plump spindle cells and prominent nucleoli. New bone formation was noted in three cases. Immunohistochemically, all tumors were positive for CD31 and negative for CD34. Pan Cytokeratin (CK) (AE1/3) was positively expressed in all, except a single tumor, in which CK7 and Cam5.2 were expressed. INI1/SMARCB1 was completely retained in all tumors. A single patient underwent surgical resection. During follow-up, two cases showed no evidence of disease within two and five years, respectively. Differential diagnosis of a PHE of bone includes osteoblastoma, epithelioid angiosarcoma, metastatic carcinoma, metastatic rhabdomyosarcoma, and epithelioid sarcoma. Caution must be exercised as pan CK (AE1/3) might not be expressed; therefore, the use of other cytokeratins, such as Cam5.2 is recommended. Awareness of such an entity in bone is the key to the diagnosis.

Histopathology of giant cell tumors of the bone: With special emphasis on fibrohistiocytic and aneurysmal bone cyst like components.

OBJECTIVE: The aim of this study was to define histopathological features of giant cell tumor of bone, especially accompanying fibrohistiocytic or aneurysmal bone cyst like components, in the light of our institutions experience. METHODS: A total of 120 cases (64 females and 56 males; mean age: 36.2 (12-80)) with 'GCT' diagnosed between the years 1996-2016 were included in this retrospective analysis. Cases were evaluated according to clinical features such as age, gender, localization, recurrence, metastasis and histopathological features. RESULTS: Tumors were localized most frequently at proximal tibia and distal femur, respectively. In 11 cases areas rich in fibrohistiocytic component and in 20 cases aneurysmal bone cyst like component were observed. In 2 cases both components were present. Twenty three cases recurred. In 1 case which was primarily located at calcaneus, tumor metastasized to lung 4 years later during follow-up. CONCLUSION: GCT can be confused with other tumor or tumor-like lesions involving giant cells. Secondary changes such as fibrohistiocytic or aneurysmal bone cyst-like components and coagulation necrosis were frequently seen in conventional giant cell tumor of bone. For tumors having prominent fibrohistiocytic and/or aneurysmal bone cyst-like components, in order to detect characteristic areas representing GCT, additional sampling is essential. Although secondary histopathological changes do not appear to affect clinical outcome, these features are important in differential diagnosis. Approximately one fifth of GCT cases show recurrence and sacrum and foot bones were the most frequent sites for recurrence. LEVEL OF EVIDENCE: Level IV, diagnostic study.

Transition to adulthood with a bladder augmentation: histopathologic concerns

ABSTRACT Aim: To investigate the histopathologic changes in native bladder and gastrointestinal segment, the relation between histopathologic changes, type of operation and the period passed over operation in patients with bladder augmentation. Materials and methods: Twenty consecutive patients were enrolled in this study. Histopathologic evaluation of the cystoscopic mucosal biopsies from native bladder and enteric augment was performed in all patients. Results: Active or chronic non-specific inflammation of various degrees was found in all specimens except two. Metaplastic changes were detected in 3 patients. Two patients had squamous metaplasia (one focal, one extensive) and one patient had intestinal metaplasia. All metaplastic changes were found in native bladder specimens. The type of augmentation in patients with metaplastic changes were ileocystoplasty and sigmoidocystoplasty. No signs of malignancy were detected in any patient. Conclusion: The complexity of the disorders requiring bladder augmentation does not let the surgeons to draw a clear line between different groups of complications including malignancy formation. However, due to challenging course of the augmentation procedure itself, surgeons should be well aware of the possibility of malignancy development.

Transition to adulthood with a bladder augmentation: histopathologic concerns.

AIM: To investigate the histopathologic changes in native bladder and gastrointestinal segment, the relation between histopathologic changes, type of operation and the period passed over operation in patients with bladder augmentation. MATERIALS AND METHODS: Twenty consecutive patients were enrolled in this study. Histopathologic evaluation of the cystoscopic mucosal biopsies from native bladder and enteric augment was performed in all patients. RESULTS: Active or chronic non-specific inflammation of various degrees was found in all specimens except two. Metaplastic changes were detected in 3 patients. Two patients had squamous metaplasia (one focal, one extensive) and one patient had intestinal metaplasia. All metaplastic changes were found in native bladder specimens. The type of augmentation in patients with metaplastic changes were ileocystoplasty and sigmoidocystoplasty. No signs of malignancy were detected in any patient. CONCLUSION: The complexity of the disorders requiring bladder augmentation does not let the surgeons to draw a clear line between different groups of complications including malignancy formation. However, due to challenging course of the augmentation procedure itself, surgeons should be well aware of the possibility of malignancy development.

Clinical and pathological characteristics of gastrointestinal stromal tumor (GIST) metastatic to bone.

Our aim in this study was to describe the clinical, morphological, and molecular profile of gastrointestinal stromal tumor (GIST) metastatic to bone. We analyzed the morphological, phenotypic, and molecular characteristics of seven cases, and in addition reviewed 17 cases from literature. Sequence analysis of KIT and PDGFRA genes was possible for six cases. For the GIST cases with bone metastasis, the most common primaries were small intestine (29%), stomach (25%), and rectum (21%). Sites of bone metastases were vertebrae (11), pelvis (8), femur (8), ribs (6), humerus (5), skull (3), scapula (1), and mandible (1). The size ranged from 1.5 to 13 cm (median, 3.8 cm). Bone metastases without involvement of any other organ were seen in 17% of the cases and were solitary in 14 (58%). Adjacent soft tissue involvement was present in nearly half of the patients. Bone metastasis was either manifest at the time of diagnosis (28%) or occurred after a mean period of 4.7 years (3 months-20 years). Morphologically, neoplastic cells were spindle in 67%, epithelioid in 13%, and mixed epithelioid and spindle in 20%. CD117, DOG1, and CD34 were positive in 88, 86, and 85% of the cases, respectively. KIT Exon 11 mutations were the most frequent gene alteration (78%), followed by KIT Exon 13 mutations. Of 17 of the cases with available follow-up information, 7 (41%) patients developed bone metastasis under imatinib therapy. Five patients (29%) died of disease within a mean of 17 months. Bone metastases from GIST are usually found in patients with advanced disease and typically present as lytic masses with occasional soft tissue involvement. We could not identify any KIT or PDGFRA alterations predisposing to bone metastasis.

Clinicopathologic features and immunohistochemical spectrum of 11 cases of epithelioid malignant peripheral nerve sheath tumors, including INI1/SMARCB1 results and BRAF V600E analysis.

Epithelioid malignant peripheral nerve sheath tumor (MPNST) is a rare, relatively less chemosensitive sarcoma. We report clinicopathologic features of 11 epithelioid MPNSTs, including rare forms, along with INI1 immunostaining and BRAF V600E mutation results. BRAF V600E mutation was tested by Real-time polymerase chain reaction (PCR) technique. Eleven tumors occurred in six men and five women (M:F ratio = 0.85:1) within an age range of 5-73 years (average = 44), mostly in lower limbs (five), followed by upper limbs (four). Tumor size (n = 6), varied from 3.1 to 15 cm (average = 8.3). Histopathologically, most tumors were multilobular, characterized by epithelioid to round-shaped, malignant cells, along with spindle cells (three cases), "rhabdoid-like" cells (seven cases) and pleomorphic giant cells (single case). By immunohistochemistry, tumor cells were positive for S100 protein (11/11) (100%), EMA (3/7) (42.8%), pan CK(2/7) (28.5%), and HMB45 (1/11) (9%), while these were negative for Melan A (0/11) and INI1 (3/11), including a single tumor, displaying HMB45 positivity. BRAF V600E mutation was positive in 1/8 cases, that lacked melanocytic marker expression. All patients (n = 5) were treated by surgical resection. During follow-up (n = 8, median duration = 23 months), four patients developed tumor recurrences and four developed metastasis, mostly to lymph nodes (3). Finally, four patients were alive with disease, two were alive with no evidence of disease, and two patients died of disease. Epithelioid MPNSTs have a diverse histopathologic spectrum. Loss of INI1 is useful, including in identifying rare forms of epithelioid MPNST, displaying melanocytic differentiation. Most tumors are treated by surgical resection. Loss of INI1 and the presence of BRAF V600E mutation in some cases raises future possibility of exploring targeted therapy in those, rare epithelioid MPNSTs.

A Translocation Renal Cell Carcinoma with Skeletal Muscle Metastasis in a Child.

Renal cell carcinoma is a tumor that is well known for a high rate of metastasis to several locations like the lung, liver and bones. Skeletal muscle is a rare location for dissemination of the disease. Herein, we describe a 7-year-old boy who presented with flank pain. On physical examination, an abdominal mass located on the left kidney as well as a solid palpable lesion on the left upper arm were detected. Total nephrectomy with subsequent excision of the arm mass was performed. Pathology examination revealed presence of translocation renal cell carcinoma. The patient received α-interferon followed by multikinase inhibitor (Sorafenib) treatment but was lost due to progressive disease. This is the first description of a pediatric patient with skeletal muscle metastases of translocation renal cell carcinoma in the literature.

A review of intussusception cases involving failed pneumatic reduction and re-intussusception.

BACKGROUND: The aim of the present study was to evaluate cases in which intussusception was unsuccessfully treated with pneumatic reduction (PR), and intussusception recurred following PR. METHODS: The medical records of 401 patients who presented with intussusception between 2003 and 2014 were retrospectively analyzed. Included were 61 patients, 20 of whom underwent unsuccessful PR (Group 1), and 41 of whom experienced intussusception recurrence following PR (Group 2). Treatment and outcome were summarized. RESULTS: In Group 1 (mean age: 14.2 months; range: 2.5 months-6 years) surgery was indicated due to PR failure in 15 patients, and perforation occurred during PR in 5. In these 5 patients, age was under 1 year (range 6-9 months) and mean time to presentation was 3 days (range 2-4). During laparotomy, pathologies were noted: mesenteric lymphadenopathy (LAP) and/or Peyer's patch hyperplasia was observed in 15 cases, Meckel's diverticulum in 5 cases. In Group 2 (mean age: 20 months; range: 3 months-6 years), intussusception developed after successful PR in 41 patients, most frequently within the first 24 hours (21.51%). Of the 41 patients, recurrent intussusception (RI) was treated with PR in 36, and laparotomy in 5. Operative findings were mesenteric LAP in 4 and polyp in 1. CONCLUSION: PR is effective for the treatment of intussusception and recurrences. Delayed presentation reduces the success rate. In the event of failure, a lead point is usually encountered at laparotomy.

Osteoid osteoma of the pisiform: A case report.

BACKGROUND: Osteoid osteoma is an uncommon benign bone-forming tumor of the musculoskeletal system but it is the most common primary bone tumor of the carpal region. However, only seven cases of its pisiform involvement have been described. CASE: We present a 19 year-old male patient with osteoid osteoma of the pisiform which initially could not be detected in any diagnostic study for long standing wrist pain. The patient was treated with excision and curettage. In 1 year of follow-up, the patient was asymptomatic without recurrence. CONCLUSION: In cases with long standing ulnar wrist pain, one should consider osteoid osteoma of the pisiform in differential diagnosis. If an OO is suspected, thin slice CT scan in initial evaluation would be valuable in preventing the patient from misdiagnosis or delay in diagnosis.

Risk continues: Very late manifestation of helicobacter pylori at gastric augmented bladder.

Helicobacter Pylori was previously demonstrated at gastric patch after gastrocystoplasty and a possible relationship with acid-haematuria syndrome was established after symptomatic relief by medical treatment. We present the long term outcome of a male bladder exstrophy patient after gastrocystoplasty. There was past history of bladder perforation, acid haematuria syndrome and treatment of HP and recurrent urinary tract infections, noncompliance on regular follow-up and cadaveric renal transplantation. At the preoperative evaluation for renal transplantation HP was present in the biopsy samples collected during gastroscopy and cystoscopy. The significance of persistant HP after gastrocystoplasty in the long term follow-up was discussed.

Laser biostimulation of wound healing: bioimpedance measurements support histology.

Laser biostimulation in medicine has become widespread supporting the idea of therapeutic effects of photobiomodulation in biological tissues. The aim of this study was to investigate the biostimulation effect of laser irradiation on healing of cutaneous skin wounds, in vivo, by means of bioimpedance measurements and histological examinations. Cutaneous skin wounds on rats were subjected to 635 nm diode laser irradiations at two energy densities of 1 and 3 J/cm2 separately. Changes in the electrical properties of the wound sites were examined with multi-frequency electrical impedance measurements performed on the 3rd, 7th, 10th, and 14th days following the wounding. Tissue samples were both morphologically and histologically examined to determine the relationship between electrical properties and structure of tissues during healing. Laser irradiations of both energy densities stimulated the wound healing process. In particular, laser irradiation of lower energy density had more evidence especially for the first days of healing process. On the 7th day of healing, 3 J/cm2 laser-irradiated tissues had significantly smaller wound areas compared to non-irradiated wounds (p < 0.05). The electrical impedance results supported the idea of laser biostimulation on healing of cutaneous skin wounds. Thus, bioimpedance measurements may be considered as a non-invasive supplementary method for following the healing process of laser-irradiated tissues.

A Comparison of the Effects of Neuronal Nitric Oxide Synthase and Inducible Nitric Oxide Synthase Inhibition on Cartilage Damage.

The objective of this study was to investigate the effects of selective inducible nitric oxide synthase and neuronal nitric oxide synthase inhibitors on cartilage regeneration. The study involved 27 Wistar rats that were divided into five groups. On Day 1, both knees of 3 rats were resected and placed in a formalin solution as a control group. The remaining 24 rats were separated into 4 groups, and their right knees were surgically damaged. Depending on the groups, the rats were injected with intra-articular normal saline solution, neuronal nitric oxide synthase inhibitor 7-nitroindazole (50 mg/kg), inducible nitric oxide synthase inhibitor amino-guanidine (30 mg/kg), or nitric oxide precursor L-arginine (200 mg/kg). After 21 days, the right and left knees of the rats were resected and placed in formalin solution. The samples were histopathologically examined by a blinded evaluator and scored on 8 parameters. Although selective neuronal nitric oxide synthase inhibition exhibited significant (P = 0.044) positive effects on cartilage regeneration following cartilage damage, it was determined that inducible nitric oxide synthase inhibition had no statistically significant effect on cartilage regeneration. It was observed that the nitric oxide synthase activation triggered advanced arthrosis symptoms, such as osteophyte formation. The fact that selective neuronal nitric oxide synthase inhibitors were observed to have mitigating effects on the severity of the damage may, in the future, influence the development of new agents to be used in the treatment of cartilage disorders.

The Role of Radiotherapy in the Treatment of Primary or Recurrent Desmoid Tumors and Long-Term Results.

BACKGROUND: Desmoid tumors are uncommon and benign mesenchymal neoplasms. The optimal treatment of patients with desmoid tumors is still controversial. Surgery is the primary treatment for locally invasive or recurrent desmoid tumors. Also, radiotherapy is a treatment option for patients at high risk for local failure such as those with positive margins or recurrent and unresectable tumors. AIMS: To report our institutional experience and long-term results of patients with desmoid tumors who received radiotherapy. STUDY DESIGN: Retrospective cross-sectional study. METHODS: Between 1980 and 2009, 20 patients who received radiotherapy (RT) in our institution were analyzed. The majority of patients (80%) were referred with a recurrent tumor after previous surgery. Thirteen patients underwent marginal resection, 4 had wide local excision and 3 patients had only biopsy. Resection margin was positive in 15 (75%) patients. All patients received radiation therapy. The median prescribed dose was 60 Gy. Five patients received less than 54 Gy. RESULTS: The median follow-up time was 77.5 months (28-283 months). Nine patients developed local recurrence after RT. Seven local failures (78%) were in field. Time to local recurrence ranged from 3-165 months (median 33 months). The 2-5 year local control (LC) rates were 80% and 69%, respectively. On univariate analysis, the 5 year local control rate was significantly better in the patients treated with ≥54 Gy than in patients who received <54 Gy (p=0.023). The most common acute side effect was grade 1-2 skin toxicity. As a late side effect of radiotherapy, soft tissue fibrosis was detected in 10 patients and lymphangitis was seen in 1 patient. One patient developed radiation-induced sarcoma. CONCLUSION: According to our results, radiotherapy is especially effective in recurrent disease and provides a high local control rate in the patients received more than 54 Gy.

Long-term treatment results in soft tissue sarcomas of the thoracic wall treated with pre-or-postoperative radiotherapy--a single institution experience.

OBJECTIVE: To evaluate the long term results among patients with soft tissue sarcoma of the thoracic wall. MATERIALS AND METHODS: Twenty-six patients who were treated with pre-or postoperative radiotherapy between December 1980-December 2007, with a diagnosis of soft tissue sarcoma of the thoracic wall were retrospectively evaluated. RESULTS: The median age was 44 years (14-85 years) and 15 of them were male. A total of 50% of patients were grade 3. The most common histologic type of tumor was undifferentiated pleomorphic sarcoma (26.9%). Tumor size varied between 2-25 cm (median 6.5 cm). Seventeen of the cases had marginal and 9 had wide local resection. Four cases received preoperative radiotherapy and 22 postoperative radiotherapy. Six of the patients with large and high grade tumors received chemotherapy. Median follow-up time was 82 months (9-309 months). Local recurrence and metastasis was detected in 34.6% and 42.3% of patients, respectively. Five- year local control (LC), disease-free survival (DFS), overall survival (OS), and disease-specific survival (DSS) were 62%, 38%, 69%, and 76% respectively. On univariate analysis, the patients with positive surgical margins had a markedly lower 5-year LC rate than patients with negative surgical margin, but the difference was not significant (43% vs 78%, p=0.1). Five-year DFS (66% vs 17%) and DSS (92% vs 60%) rates were significantly worse for the patients who had high grade tumors (p=0.01, p=0.008 respectively). CONCLUSIONS: Tumor grade and surgical margin are essential parameters for determining the prognosis of thoracic wall soft tissue sarcoma both in our series and the literature.

Histopathologic changes in the middle ear mucosa after exposure to pepsin and unconjugated bile acid.

OBJECTIVE: An increasing number of studies indicate that pepsin and bile acid cause damage to the ear, nose, and throat structures as a result of extraesophageal reflux. The aim of this study was to evaluate and compare the damaging effect of bile acids and pepsin on the middle ear mucosa. MATERIAL AND METHODS: Twenty-nine healthy rats were included in this study. The animals were divided into 5 groups. A single daily dose of 40 µmol/L chenodeoxycholic acid, 40 µg/mL pepsin, and saline were injected separately into the right middle ear of the rats. On day 30, all rats were decapitated, and formalin-fixed, paraffin-embedded samples of the middle ear both from the control and experimental rats were prepared. A semiquantitative analysis was performed. RESULTS: Inflammatory response was seen in all middle ear mucosa of rats except control group 1. The degree of inflammatory response was higher in the bile acid group when compared with the other groups. Epithelial metaplastic changes with varying number of goblet cells were observed in both the bile acid- and pepsin-injected groups. These metaplastic changes were also higher in the bile acid-induced group than in the pepsin-injected group. CONCLUSIONS: This is the first study on the middle ear mucosal damage of both pepsin and bile acid. Our results demonstrate that bile acids were associated with more extensive mucosal injury at pH 7 in comparison to pepsin in a rat animal model. Inflammatory response and metaplastic changes may play an important role in the etiology of middle ear pathologies.

Non-metastatic Ewing's sarcoma family of tumors of bone in adolescents and adults: prognostic factors and clinical outcome-single institution results.

AIMS AND BACKGROUND: There is limited data regarding outcomes of Ewing's sarcoma family of tumors in adolescents and adults compared with the same tumors in childhood. The aim of the study was to analyze prognostic factors and treatment results in a cohort of adolescents and adults with non-metastatic skeletal Ewing's sarcoma family of tumors. METHODS AND STUDY DESIGN: From 1992-2008, 90 adolescents and adults with Ewing's sarcoma family of tumors of the bone were referred to our institution. Sixty-five (72%) non-metastatic patients with analyzable data and treated in our institution were retrospectively evaluated. All patients were treated with alternated chemotherapy regimens administered every 3 weeks. The local treatment modality was selected according to tumor and patient characteristics. RESULTS: The median age was 21 years (range, 13-50). Most patients (74%) were >17 years of age. Forty-six percent of the tumors were located in the extremities. Local therapy was surgery in 45 patients and radiotherapy alone in 19 patients. Twenty-one patients received preoperative and 13 patients postoperative radiotherapy. Median follow-up was 43 months (range, 7-167). The 5-year event-free and overall survival rates for all patients were 44% and 49%, respectively. On univariate survival analysis, event-free and overall survival were worse for patients >17 years of age, tumor size >8 cm in diameter, an axial location, positive surgical margins, and poor histopathological response (<90% necrosis). Age, tumor site and tumor size on event-free and overall survival remained significant on multivariate analysis. CONCLUSIONS: We identified age, tumor size, and tumor site as independent prognostic factors, in accord with the Western literature. These patients require novel treatment modalities.

[Unfavorable sialoblastoma: a rare case]. / Kötü gidisli sialoblastom: Nadir bir olgu.

Sialoblastoma is an extremely rare tumor of childhood. It is commonly presented as a mass of parotid gland and submandibular gland. In this article, we report a recurrent unfavorable sialoblastoma in four-year-old boy.

The impact of share wave elastography in differentiation of hepatic hemangioma from malignant liver tumors in pediatric population.

OBJECTIVE: In children it is crucial to differentiate malignant liver tumors from the most common benign tumor, hepatic hemangiomas since the treatment strategies are quite different. We aimed to evaluate the efficiency of shear wave elastography (SWE) technique in differentiation of malignant hepatic tumors and hepatic hemangiomas. METHODS: Twenty patients with hepatic tumor were included in our study. Two radiologists performed SWE for 13 patients with malignant hepatic tumors including hepatoblastoma (n=7), hepatocellular carcinoma (n=3), metastasis (n=2), embryonal sarcoma (n=1) and 7 patients with hepatic hemangioma. All of our patients were between the age of 1 and 192 months (mean age: 56.88 months). Receiver operating characteristic analysis was achieved to evaluate the diagnostic accuracy of SWE and to determine the optimal cut-off value in differentiation hepatic hemangioma from malignant hepatic tumors. RESULTS: The mean SWE values (in kPa) for the first observer were 46.94 (13.8-145) and 22.38 (6.6-49.6) and those for the second observer were 57.91 (11-237) and 23.87 (6.4-57.5), respectively for malignant hepatic tumors and hepatic hemangiomas. The SWE values of malignant hepatic tumors were significantly higher than those of hepatic hemangioma (p=0.02). The inter-observer agreement was almost perfect (0.81). The area under the receiver operating characteristic curve of SWE for differentiating the hepatic hemangioma from malignant hepatic tumors was 0.77 with a sensitivity of 72.7% and a specificity of 66.7% at a cutoff value of 23.62 with 95% confidence interval. CONCLUSION: Shear wave elastography can be helpful in differentiation of malignant hepatic tumors and hepatic hemangioma.

Neoadjuvant treatment with preoperative radiotherapy for extremity soft tissue sarcomas: long-term results from a single institution in Turkey.

BACKGROUND: To assess the long term clinical outcome of preoperative radiotherapy with or without chemotherapy followed by limb sparing surgery in patients with non-metastatic soft tissue sarcomas (STS) of the extremities. MATERIALS AND METHODS: Sixty patients with locally advanced STS were retrospectively analyzed. The median tumor diameter was 12 cm. All patients were treated with preoperative radiotherapy delivered with two different fractionation schedules (35Gy/10fr or 46-50Gy/23-25fr). Neoadjuvant chemotherapy was added to 44 patients with large and/or high grade tumors. Surgery was performed 2-6 weeks after radiotherapy. Chemotherapy was completed up to 6 courses after surgery in patients who had good responses. RESULTS: Median follow-up time was 67 months (8-268 months). All of the patients had limb sparing surgery. The 5-year local control (LC), disease free (DFS) and overall survival (OSS) rates for all of the patients were 81%, 48.1% and 68.3% respectively. 5-year LC, DFS and cause specific survival (CSS) were 81.7%, 47%, 69.8%, and 80%, 60%, 60% in the chemoradiotherapy and radiotherapy groups, respectively. On univariate analysis, patients who were treated with hypofractionation experienced significantly superior LC, DFS and CSS rates with similar rates of late toxicity when compared with patients who were treated with conventional fractionation and statistical significance was retained on multivariate analysis. CONCLUSIONS: Treatment results are consistent with the literature. As neoadjuvant chemoradiotherapy provides effective LC and CSS with acceptable morbidity, it should be preferred for patients with large and borderline resectable STS.