PubMed Health⌕ Search

Biomedical subjects

Inci Ayan

Publications and source records attributed to Inci Ayan.

14 recordsLinked to original sources

Adult clear cell sarcoma of the kidney: a case report.

BACKGROUND: Clear cell sarcoma of the kidney (CCSK) in adults is extremely rare. Optimal treatment of adult patients with CCSK remains unclear. CASE PRESENTATION: A 22-year-old man presented with a 2-month history of left flank pain. A color duplex sonography revealed a hypervascular, heterogeneous renal mass. Abdominal and pelvic computerized tomography showed a heterogeneous mass originating from the lower pole of the left kidney and infiltrating to the psoas muscle. Further evaluation including bone scan did not demonstrate any evidence of metastases. A left radical nephrectomy with hilar lymphadenectomy through an intraperitoneal approach with an anterior subcostal incision was performed. The histopathological diagnosis of the mass was a clear cell sarcoma of the kidney. No lymph node metastases were found. Concomitant chemo-radiotherapy was performed. Therapy-related serious side effects were not observed. There was no evidence of local recurrence or metastases during the following twenty-four months after therapy. CONCLUSION: We believe that the combination therapy is efficacious for preventing the local recurrence and distant metastases. Accurate diagnosis is very important and therapy must also include doxorubicin regardless of the disease stage in adult patients with CCSK.

Adult↗

Are serum levels of CD44 relevant in children with pediatric sarcomas?

BACKGROUND: Variation in serum levels of CD44, which acts as an adhesion receptor involved in lymphocyte migration and binding, have been reported in some malignancies. The aim of this study is to compare serum levels of CD44 in children with sarcomas with those in healthy children. PROCEDURE: CD44 levels were measured by enzyme-linked immunosorbent assay (ELISA) in serum samples taken at diagnosis from 55 children with sarcomas and from 27 healthy children of similar age, sex, and socioeconomic status. RESULTS: There was no statistically significant difference between CD44 serum levels of children with sarcomas and those of healthy children. No significant difference was observed between CD44 serum levels of each patient group and those of control group (P > 0.05). There was no significant difference among CD44 serum levels of patient groups according to stage or outcome. CONCLUSIONS: In this study, serum CD44 levels were not found to be of value in the diagnosis or prognosis in children with sarcomas.

Adolescent↗

The prognostic significance of angiogenesis and the effect of vascular endothelial growth factor on angiogenic process in Wilms' tumour.

AIMS: There is a subgroup of patients with Wilms' tumour (WT) having favourable clinicopathological features but adverse outcome. We aimed to investigate the prognostic significance of angiogenesis and whether it can be used for predicting which patients will fall into this category, and the possible role of vascular endothelial growth factor (VEGF) on angiogenesis in WT. METHODS: Tumours in nephrectomy specimens from 63 WT patients were investigated for neovascularisation and VEGF expression by immunohistochemistry. The endothelial cells were highlighted by anti-CD34 and anti-CD31, and the microvessels in the hot-spots were counted. Correlations between the microvessel density (MVD), VEGF expression, clinicopathological features and prognosis were studied. RESULTS: Among 21 patients with follow-up data, favourable histology was detected in 17, seven of which died of disease. Patients with highly vascular tumours showed significantly poorer prognosis than those with low vascular tumours. There was no significant relationship between angiogenesis and VEGF expression. VEGF immunostaining revealed various patterns in different components of WT. CONCLUSIONS: We suggest that high MVD can be used as an indicator of poor prognosis with WT patients displaying favourable histology and there might be some additional growth factors other than VEGF which may also be responsible for angiogenesis in WTs.

Antigens, CD34↗

[Surgical treatment of Langerhans cell histiocytosis with bone and soft tissue involvement in pediatric patients].

OBJECTIVES: We evaluated pediatric patients who underwent surgery for Langerhans cell histiocytosis with mono- or polyostatic involvement and an extensive soft tissue component and/or a high risk for pathologic fractures. METHODS: The study included 13 children (6 boys, 7 girls; mean age 6 years; range 2 to 11 years) who were treated surgically for bone and soft tissue involvement of Langerhans cell histiocytosis with a high risk for fractures of the long bones and the pelvis. Eleven patients had only skeletal involvement while three patients had multisystem involvement. There were 17 bone lesions in the following localizations: acetabulum (n=7), femur neck and subtrochanteric region-proximal femur (n=5), and proximal (n=2) and distal (n=2) humerus. One patient had multifocal bone involvement. The risk for pathologic fractures was assessed using the scoring system of Mirels. Treatment consisted of surgery followed by chemotherapy. The results of treatment were evaluated according to the criteria by the Study Group of Langerhans Cell Histiocytosis. The mean follow-up after orthopedic surgery was 67 months (range 34 to 172 months). RESULTS: Only one patient developed recurrence, in which case bone marrow involvement responded well to chemotherapy. Radiographically, graft consolidation was observed in all the patients in the third postoperative month. No bone sequelae such as shortness and deformity were detected throughout the follow-up period. CONCLUSION: Surgical treatment combined with chemotherapy is effective in the treatment of aggressive histiocytic bone lesions associated with extensive soft tissue involvement and high risk for fractures.

Bone Diseases↗

Hypertrophic osteoarthropathy and intrathoracic Hodgkin's disease in children.

BACKGROUND: Hypertrophic osteoarthropathy (HOA) is a syndrome characterized by clubbing of the fingers and toes, periosteal new bone formation of the long bones and polyarthritis. CASE REPORT: In this report, two children with intrathoracic Hodgkin's disease and HOA are presented. CONCLUSIONS: Intrathoracic neoplasms are one of the major causes of HOA in adults; however HOA is rarely associated with intrathoracic malignancies in children. HOA associated with intrathoracic Hodgkin's disease is even more rare, but should be kept in mind.

Adolescent↗

Ependymal tumors in childhood.

BACKGROUND: Ependymal tumors are classified as ependymoma (benign or low grade) versus anaplastic ependymoma (malignant or high grade). Ependymomas represent 5-10% of intracranial neoplasm in children. In this study, demographic data and the treatment results of pediatric patients with ependymal tumors, treated in a single institute, is reported. PATIENTS AND METHODS: Between 1989 and 2001, 40 (22 M/18 F) previously untreated patients with a median age of 5.5 years (3 months-15 years), of histologically proven ependymal tumors (except ependymoblastomas) were referred to the Institute of Oncology, University of Istanbul. The localization was supratentorial in 18, infratentorial in 20, both supra and infratentorial in two patients. Histologic subgroups were 18 ependymomas (43.6%), and 22 anaplastic ependymomas (56.4%). Total tumor resection was performed in 20 patients (50%), subtotal in 18 patients (45%), and biopsy only in 2 patients (5%). Postoperative treatment consisted of regional (8 patients) or craniospinal (CSI) (9 patients) radiotherapy (RT) in patients with ependymoma; regional (7 patients) or CSI RT (14 patients) with chemotherapy (ChT) in patients with anaplastic ependymoma; ChT only (1 patient) in patients less than 3 years of age. The standard technique for posterior fossa irradiation was parallel-opposed lateral fields and total dose was 45-54 Gy. Between September 1989 and May 1991 patients received regimen A, which consisted of RT followed by eight-in-one ChT, given every 4 weeks for eight courses. Patients who were treated between June 1991 and July 1994, received regimen B, which included two courses of postoperative "VEC" (vincristine, etoposide, cisplatin) ChT, administered every 3 weeks, followed by RT applied with low dose concomitant cisplatin used as a radiosensitizer. Patients with objective response to postoperative "VEC" continued to have "VEC" after completion of RT for six more courses. From August 1994 on, patients received regimen C, consisting of RT and concomitant infusion of cisplatin followed by "VCPCU" (vincristine, cyclophosphamide, procarbazine, lomustine) administered every 4 weeks for eight courses. RESULTS: A total of 40 patients were included in the outcome and survival data. The 5-year overall survival (OS) rate was 64.9%, and the 5-year progression-free survival rate was 50.8% for the whole series. Median time for progression or relapse was 24.3 months and there were 19 patients (43.6%) with relapse or progression. Non-metastatic patients (P = 0.0008, 5-year OS rate was 82% vs. 29%), and totally resected patients (P = 0.01, 5-year OS rate was 80% vs. 55%), and > or =3 years of age (P = 0.04, 5-year OS rate was 75% vs. 38%) had significantly better outcome. CONCLUSIONS: The majority of complete responders were patients who had total tumor removal. Treatment failure occurred mainly within the first 2 years, and outcome was dismal for patients who relapsed or had progressive disease. The median age at diagnosis is 6 years in our patient group; younger children (less than 3 years old) have less favorable outcome. There was no significant difference in survival or progression-free survival between the two histologic subtypes.

Adolescent↗

Brain metastasis in pediatric extracranial solid tumors: survey and literature review.

OBJECTIVES: Brain is a rare site of metastasis in most extracranial pediatric solid tumors. The aim of this study is to investigate the incidence, treatment, prognosis of brain metastasis in extracranial pediatric malignant tumors in a single institution and to review the literature. METHODS: From September 1989 to December 2002, 1100 children <or=16 years of age with extracranial solid tumors including lymphomas were diagnosed and treated in the Division of Pediatric Oncology, Oncology Institute, Istanbul University. Patients with parenchymal metastases in the brain were assessed. RESULTS: Sixteen (10 female, 6 male) of 1100 patients (1.45%) with extracranial solid tumors developed brain metastases. The median age of the patients was 10.5 (1-16) years. The diagnosis was sarcomas in 12 patients: 5 osteosarcomas, 4 Ewing's sarcoma family tumors, 1 rhabdomyosarcoma, 1 clear cell sarcoma of the soft tissue, 1 alveolar soft part sarcoma. Two patients had Wilms' tumor and two had germ cell tumors. Four patients (25%) had brain metastasis at diagnosis. Twelve (75%) developed brain metastasis during therapy or relapse at a median duration of 16 (1-70) months from initial diagnosis. All patients had metastases to various sites, mostly lung, at the time the brain metastases were detected. Treatment included surgery, followed by postoperative radiotherapy (RT) and chemotherapy (CT) in 1, S and RT in 1, S in 1, RT and CT in 6, RT in 1, CT in 1 and no treatment in 5. Only one patient with alveolar soft part sarcoma is alive with disease 20 months from diagnosis of brain metastasis. All other patients died at a median time of 2 months (2 days-6 months) from the time of brain metastasis. CONCLUSIONS: Children with metastatic cancer who develop headaches or any other neurologic symptom should be investigated for possible brain metastasis. Although, the outcome for these patients is dismal in this series and in the literature; reports of long term survival in a few cases with Wilms' tumor, osteosarcoma and alveolar soft part sarcoma who had isolated brain metastasis, suggest that a subset of patients may benefit from therapy.

Adolescent↗

Oral etoposide for recurrent/progressive sarcomas of childhood.

BACKGROUND: Etoposide (VP-16) is a topoisomerase II inhibitor that is effective in a broad spectrum of pediatric and adult malignancies. Chronic, low-dose, oral VP-16 has also been shown to be active in some recurrent malignancies mostly in adults. The aim of this prospective, single institution study is to assess the efficacy and toxicity of oral VP-16 in children with progressive or recurrent (P/R) sarcomas. PROCEDURE: Twenty-one children (10 girls and 11 boys) with R/P sarcomas and a median age of 11 years (range 3-16 years) were enrolled in this study. The diagnosis was Ewing sarcoma family tumor (ESFT) in seven, osteosarcoma in eight, rhabdomyosarcoma in four, clear cell sarcoma of soft tissue in one, fibrosarcoma in one patient. Oral VP-16 was administered at a dose of 50 mg/m(2)/daily for 20 days. The next course was initiated after a 10 day rest. Response to oral VP-16 was assessed after two courses. RESULTS: There was an objective response (one complete response [CR], two partial responses [PR]) in three patients (14%) by two courses of oral VP-16 alone. One of these patients with PR achieved CR by the use of radiotherapy (RT) and further oral VP-16. Two more patients (9.5%) achieved CR by RT and oral VP-16. Eight (38%) patients had disease stabilization for 2-15 months. Two patients (9.5%) are long-term survivors. They are alive with no evidence of disease (NED) 79 and 94 months from time of relapse/progressive disease (PD). A patient developed acute myeloid leukemia and died. There was no major acute toxicity related to oral VP-16 in a total of 126 courses. CONCLUSIONS: Oral VP-16 therapy is simple, relatively nontoxic, and does not necessitate hospitalization. The cure rate is small. Given the risk of second malignancy, especially in children with previous exposure to topoisomerase II inhibitors and alkylating agents, this regimen may be used as a palliative treatment or in patients with poor prognosis.

Administration, Oral↗

Congenital mesenchymal chondrosarcoma of the orbit: case report and review of the literature.

PURPOSE: To report the first case of congenital extraskeletal mesenchymal chondrosarcoma arising in the orbit. DESIGN: Interventional case report and review of the literature. METHODS: Ophthalmologic examination and computed tomography scan of the orbit were performed. Histologic and histochemical examination and immunohistochemistry of the biopsy specimen were evaluated. MAIN OUTCOME MEASURES: Ocular and systemic disease control. RESULTS: A 5-month-old girl was seen with a papillomatous lesion in the right lower tarsal conjunctiva present from birth. Histologic examination demonstrated an admixture of undifferentiated mesenchymal cells and islands of mature hyaline cartilage, and immunohistochemistry studies revealing positivity for vimentin and S-100 were consistent with the diagnosis of mesenchymal chondrosarcoma. The patient was treated with combined chemotherapy (ifosfamide, epirubicin, and cisplatin), radiotherapy, and surgery (exenteration). She is alive with no evidence of disease after 4 years of follow-up. Facial asymmetry and dental malformations have developed as late effects. Further reconstructive surgery is planned for the malformations. CONCLUSIONS: Extraskeletal mesenchymal chondrosarcoma in the orbit is extremely rare. This study reports the first case of congenital extraskeletal mesenchymal chondrosarcoma arising in the orbit (also the youngest patient) both in skeletal and extraskeletal sites. Multimodality treatment (surgery, chemotherapy, and radiotherapy) may lead to long-term survival.

Biomarkers, Tumor↗

Childhood nasopharyngeal carcinoma: from biology to treatment.

Nasopharyngeal carcinoma is a rare disease in children with distinct epidemiological, histopathological, and clinical characteristics. Incidence varies widely around the world but bimodal incidence graphs show that in some populations a disproporionate number of cases occur in late childhood. Children with nasopharyngeal carcinoma almost always have the undifferentiated variant of the disease, which is associated with advanced locoregional spread and distant metastases. Both genetic and environmental factors contribute to the development of nasopharyngeal carcinoma, as evidenced by its risk factors which include: specific HLA subtypes; deletions of chromosomes 3p, 9p, 11q, 13q, and 14q; mutations of p53 and RB2/p130; polymorphism of the CYP2E1; and infection with Epstein-Barr virus. Traditional treatment consists of high-dose radiotherapy and cure rates range between 30% and 60%. The high incidence of failure due to systemic disease in children means that chemotherapy is preferable for first-line treatment in advanced-stage disease. Currently, cisplatin-based induction or adjuvant chemotherapy combinations are used along with high-dose radiotherapy. Although combined modality treatment has increased 5-year survival to 70-90%, late morbidity is a major concern.

Carcinoma↗

P53 overexpression in nasopharyngeal carcinoma.

Nasopharyngeal carcinoma (NPC) is a characteristic tumor displaying epidemiological, genetic and regional distribution properties and is unique by its natural behavior and therapy. Investigation of the molecular and biological changes, gene amplifications and activations that occur during carcinogenesis and progression can provide new insight into the pathology of the disease and may add biological factors that can be used as new prognostic markers. The p53 tumor suppressor gene is the most frequently mutated gene in human cancer. Although point mutations in the p53 gene are observed in nasopharyngeal cancer, the mutation rate is lower than in other tumors. Immunohistochemical studies have shown significant p53 overexpression in NPC material. In this study, p53 protein immunoreactivity was investigated in paraffin sections of primary nasopharyngeal tumors and metastatic cervical lymph nodes and association with clinical and histopathological characteristics was evaluated. Ninety-seven paraffin sections from 81 patients with NPC treated from 1990 to 1996 were examined by immunohistochemistry and were correlated with clinical features and treatment outcome. Among a total of 97 samples, positive staining for p53 protein was observed in 83 (85.5%) samples while no staining was found in 14 (14.5%) cases. Immunoreactivity was observed in 62 (81.5%) of the primary nasopharyngeal biopsy specimens. The correlation between p53 expression and histological type, stage, age and sex distributions was tested. After statistical analysis according to Chi-square test and Yates' correction, no significant difference was demonstrated (p>0.05). There was no statistically significant correlation with p53 immunoreactivity and overall and disease-free survival. Although the association between NPC and p53 is not clear, our study confirms that p53 overexpression is present in a considerable subset of patients with NPC.

Adolescent↗