Sergülen Dervişoğlu

Extraskeletal Ewing's Sarcoma Family of Tumors in Adults: Prognostic Factors and Clinical Outcome

OBJECTIVE The aim of this study was to evaluate prognostic factors, survival rate and the efficacy of the treatment modalities used in patients with extraskeletal Ewings sarcoma. METHODS Data of patients with extraskeletal Ewings sarcoma followed up at our center between 1997 and 2010 were retrospectively analyzed. RESULTS The median age of 27 patients was 24 years (range, 16-54 years). The median follow-up was 31.8 months (range, 6-144 months). Tumor size was between 1.5 and 14 cm (median: 8 cm). Eighty-five percent of patients had localized disease at presentation and 15% had metastatic disease. Local therapy was surgery alone in 16% of patients, surgery combined with radiotherapy in 42% and radiotherapy alone in 27%. All patients were treated with vincristine, doxorubicin, cyclophosphamide and actinomycin-D, alternating with ifosfamide and etoposide every 3 weeks. In patients with localized disease at presentation, the 5-year event-free survival and overall survival were 59.7 and 64.5%, respectively. At univariate analysis, patients with tumor size ≥ 8 cm, high serum lactate dehydrogenase, metastasis at presentation, poor histological response to chemotherapy and positive surgical margin had significantly worse event-free survival. The significant predictors of worse overall survival at univariate analysis were tumor size 8 ≥ cm, high lactate dehydrogenase, metastasis at presentation, poor histological response to chemotherapy, radiotherapy only as local treatment and positive surgical margin. CONCLUSIONS Prognostic factors were similar to primary osseous Ewings sarcomas. Adequate surgical resection, aggressive chemotherapy (vincristine, doxorubicin, cyclophosphamide and actinomycin-D alternating with ifosfamide and etoposide) and radiotherapy if indicated are the recommended therapy for patients with extraskeletal Ewings sarcoma.

Localized pigmented villonodular synovitis of the knee.

Eight patients with localized pigmented villonodular synovitis (LPVNS) of the knee were treated with arthroscopic and open techniques, with diagnosis confirmed by histological examination. Average patient age was 29 years (range: 13-50 years). At arthroscopy, all lesions except one were in the anterior compartment of the involved knee. Treatment consisted of complete local excision with partial synovectomy. This procedure was completed arthroscopically in seven patients. No recurrence was reported at average 24-month follow-up (range: 12-33 months). Arthroscopy is a valuable tool in the diagnosis and treatment of LPVNS.

Immunohistochemical detection of p53 protein in rhabdomyosarcoma: association with clinicopathological features and outcome.

PURPOSE Alteration in the p53 tumor suppressor gene is the most common tumor specific genetic change identified in most major cancer types including rhabdomyosarcomas. To investigate the overexpression of p53 and its relation to clinical features and outcome in patients with rhabdomyosarcoma (RMS), an immunocytochemical study was performed. METHODS Formalin-fixed paraffin embedded tissue sections obtained from 42 cases of RMS were immunostained with a mouse monoclonal antibody p53-D07. Staining was assessed by evaluating the percentage of p53 immunopositive cancer cell nuclei. RESULTS Nuclear accumulation of p53 protein was detected in 8 of 42 (19%) samples. Clinical analyses of patients demonstrated no correlation between positive staining and age, sex, histological subtype, stage and overall survival. This analysis, however, was limited by the small number of patients who demonstrated p53 immunostaining. Nonetheless, a statistically significant association was observed between p53 expression and adverse outcome. Nuclear p53 expression was associated with disease progression or recurrence (p <0.001) and with a worse event free survival (p = 0.0015). CONCLUSION The nuclear p53 immunoreaction rate is low in RMS, but p53 expression appears to correlate with poor prognosis.

An unusual long-term complication of burn injury : malignant fibrous histiocytoma developed in chronic burn scar

Development of malignant tumors in chronic burn wounds is a well-known complication. These tumors are almost always squamous cell carcinomas, although other types of malignancies such as basal cell carcinoma, malignant melanoma and sarcomas can be seen rarely. There are only three previously reported cases of malignant fibrous histiocytoma developed in chronic burn scar in the literature. Two cases with malignant fibrous histiocytoma developed in chronic, badly treated burn wounds are presented. One of the tumors was multifocal and overexpression of the p53 gene was present. Both tumors were excised widely and skin grafted. Regional lymph node dissection was performed in one case. One of the patients died due to tumor recurrence and lymphatic metastases. These cases represent a very uncommon complication of burn injury and indicate the importance of the appropriate primary treatment of the burn wound.

Duplication of appendix vermiformis: a case in a child

Abstract Appendiceal anomalies are extremely rare malformations that are usually found in adult population as an incidental finding during laparotomy due to another reason. When appendiceal duplications are detected in childhood, almost all the patients have serious associated intestinal, genito-urinary or vertebral malformations. Presented herein are a case of a huge (15×13 cm mass) appendiceal duplication in a child causing acute abdomen without an accompanying pathology, its differentiation from appendiceal and caecal diverticulum, and classification of appendiceal duplications with the review of literature.

Aneurysmal Bone Cyst of the Sphenoid Bone

Aneurysmal bone cyst (ABC) is an uncommon benign lesion that rarely presents in the craniofacial region. Aneurysmal bone cysts represent nearly 1.4% of all bone tumors, and among those, only 3% are located in the cranium. In this study, we report on an ABC located in the sphenoid bone with superior nasal cavity and ethmoid extension. The presenting symptom of our patient was headache, followed by diplopia, loss of visual accuracy, and abduction restriction. We successfully resected the lesion by a combined subcranial-midfacial degloving approach without any complications or recurrence.

A well-differentiated giant liposarcoma originating from the buccal fat pad

Although liposarcoma is one of the most common soft-tissue sarcomas, facial localization is extremely rare. The buccal fat pad is an important anatomic structure located in the face that recently gained interest as a result of increasing research on facial anatomy. In this paper, we report a case of giant liposarcoma originating from the buccal fat pad. The precise localization of the tumor was determined preoperatively with computed tomography examination. The liposarcoma that invaded the body and the extensions of the buccal fat pad was resected completely. The pathological examination revealed a sclerosing, well-differentiated liposarcoma, which is known to be very rare in the head and neck region. Chemotherapy and radiotherapy were not necessary because of the favourable histological type of the tumor and the advanced age and poor general condition of the patient. Local recurrence and distant metastasis were not observed during the 1-year follow-up.

TREATMENT OF WILMS TUMOR: A Report from the Turkish Pediatric Oncology Group (TPOG)

Aim: To standardize diagnosis and treatment of childhood Wilms tumor (WT) in Turkey. Methods and patients: Between 1998 and 2006, WT patients were registered from 19 centers. Patients <16 years with unilateral WT whose treatment started in first postoperative 3 weeks were included. Treatments were stage I favorable (FH) and unfavorable histology (UH) patients, VCR + Act-D; stage IIA FH, VCR + Act-D; stage IIB FH, VCR + Act-D + radiotherapy (RT); stage III–IV FH, VCR + Act-D + adriamycin (ADR) + RT; stages II–IV UH tumors, VCR + Act-D + ADR + etoposide + RT. Results: 165/254 registered cases were eligible (bilateral, 5.9%) [median age 3.0 years; M/F: 0.99; 50/165 cases ≤2 years]. 9.7% cases had UH tumors. Disease stages were stage I 23.6%; IIA 36.4%; IIB 5.5%; III 22.4%; IV 12.1%. Cases >2 years had significantly more advanced disease. 1/11 cases with recurrent disease died; 2/165 had progressive disease, 2/165 had secondary cancers, and all 4 died. In all cases 4-year OS and EFS were 92.8 and 86.5%, respectively. Both OS and EFS were significantly worse in stage IV. Conclusions: Despite problems in patient management and follow-up, treatment results were encouraging in this first national experience with a multicentric study in pediatric oncology. Revisions and modifications are planned to further improve results and minimize short- and long-term side effects.

Head and neck hemangiopericytomas: diagnostic contradictions.

Purpose: To identify misdiagnostic points of hemangiopericytomas (HPs) of the head and neck. Patients and Methods: We reviewed our clinical records from 2000 to 2007 retrospectively and identified 5 patients with HP of the head and neck. The records of each patient with head and neck HP were evaluated for age, sex, location of primary, clinical course, treatment, and tumor embolization (if performed). Pathologic slides of all patients were reviewed to identify histologic features and correlation with the clinical course and outcome for each lesion. Results: Five patients with HP that arose from head and neck sites were identified. Five patients included 3 women and 2 men aged 9 to 52 years, with an average of 33.2 years. Each lesion of the patients derived from different parts of the head and neck. The most common complaints were painless mass (3 of 5) and nasal airway obstruction (3of 5). Magnetic resonance imaging of the 3 patients reflected hyperintense on T2-weighted imaging and hypointense on T1-weighted imaging with diffuse enhancement after intravenous administration of gadolinium. Angiography was performed to all these 3 patients, and only 1 patient with cranial HP involvement did not show significant vascularity. Two patients had preoperative histopathologic results. All patients were operated on with appropriate approach. Pathologic slides of all patients were investigated and graded according to the tumor diameter, cellularity, mitotic rate, and necrosis. Conclusion: Differential diagnosis of clinical features and radiologic and pathologic aspects must be managed more carefully. Diagnostic way of these tumors has different pitfalls for the clinician.

Patellar tendinopathy caused by a para-articular/extraskeletal osteochondroma in the lateral infrapatellar region of the knee: a case report

Patellar tendinopathy is characterized by activity-related anterior knee pain. It is most commonly related to sports activity, but has also been reported in the non-athletic population. Most injuries are caused by microtrauma, resulting in tendinitis or tendinosis. Extraskeletal paraarticular osteochondromas, which occur in the soft tissues near the joint, are rare. The infrapatellar fat pad and joint capsule are the most common sites of these tumors. Here, a case of patellar tendinitis caused by an extraskeletal paraarticular osteochondroma is reported. The symptoms included intensifying pain upon flexion and a palpable click that was located at the medial side of the mass. The patient was pain-free within 3 weeks after excision of the tumor and the clicking disappeared. To our best knowledge, no other case of patellar tendinitis caused by an extraskeletal paraarticular osteochondroma has been reported in the English literature.