Yavuz Koksal

Pediatric and young adult nasopharyngeal carcinoma patients treated with preradiation Cisplatin and docetaxel chemotherapy.

PURPOSE To evaluate treatment results for pediatric and young adult (aged <21 years) patients with nonmetastatic nasopharyngeal carcinoma treated with neoadjuvant cisplatin + docetaxel and radiotherapy. METHODS AND MATERIALS Ten patients with nasopharyngeal carcinoma who received diagnoses between 2004 and 2007 were treated with four cycles of cisplatin 100 mg/m(2) + docetaxel 75 mg/m(2) on Day 1 with premedication every 3 weeks. All patients were treated with fractionated external beam radiotherapy after chemotherapy to a median dose of 59.4 Gy (range, 54-59.4 Gy) to the primary disease and 40 Gy to the supraclavicular field with the clavicles shielded. Five children were monitored with serum EBV DNA quantification at diagnosis, after each cycle of chemotherapy, before radiotherapy, and at follow-up. RESULTS The median age of the patients was 14 years (range, 9-20 years), with a male:female ratio of 6:4. Stage distribution was as follows: 2 patients had Stage IIb disease, 2 had Stage III, 4 had Stage IVa, and 2 had Stage IVb disease. After cisplatin+docetaxel chemotherapy 1 patient had a complete response, 5 had a partial response, 3 had stable disease, and 1 had disease progression. The 2-year overall survival rate in our series was 90% and the event-free survival rate was 70%. No major chemotherapy toxicity was observed. The EBV DNA titers were higher in 2 of the 5 monitored patients at the time of diagnosis. CONCLUSION As neoadjuvant chemotherapy before radiotherapy, the cisplatin+docetaxel combination is safe for use in the treatment of childhood nasopharyngeal carcinoma.

CARDIAC FUNCTIONS EVALUATED WITH TISSUE DOPPLER IMAGING IN CHILDHOOD CANCERS TREATED WITH ANTHRACYCLINES

Aim: The aim of this study was to assess the cardiac functions using conventional echocardiography and tissue Doppler imaging in childhood cancers treated with anthracyclines. Methods: The study group was selected from the patients admitted to the pediatric oncology department for a treatment protocol that included doxorubicin. Body surface area was calculated and complete 2-dimensional, M-mode, pulse wave Doppler and pulse wave tissue Doppler echocardiographic examinations were performed just before the first treatment and at least 6 months after the last treatment. Results: This study included 20 patients (12 males and 8 females). Mean cumulative antracycline dose was 189 ± 102.90 mg/m2. There were no significant differences between the pre- and post-treatment groups regarding systolic and diastolic blood pressures, heart rates, left ventricular ejection fraction and fractional shortening, right and left ventricular conventional and tissue Doppler diastolic parameters (E and A waves, E/A ratio, E′ and A′ waves, E′/A′ ratio), but there were significant differences between the pre- and post-treatment groups regarding body surface area, right and left ventricular myocardial performance index observed by conventional pulse wave and pulse wave tissue Doppler methods. Conclusion: Tissue Doppler imaging provided additional information on cardiac functions. While systolic and diastolic functions were in normal range, myocardial performance index observed by tissue Doopler method was impaired in children who were treated with anthracyclines.

Giant Mature Adrenal Cystic Teratoma in an Infant

CONFLICT OF INTEREST: NONE DECLARED Introduction Teratomas are derived from embryonic tissues that are typically found in the gonadal and sacrococcygeal regions of adults and children. Primary teratomas in the retroperitoneum are very rare in infant and primary adrenal teratomas are extremely rare. Early diagnosis and surgical resection are important for effective treatment. Case report We report here the case of a histologically unusual adrenal teratomas detected on computed tomography during the workup of abdominal distension 3-mounth-old male infant. The evaluation and treatment of this condition and a review of the literature are included in this paper.

Desmoplastic non-infantile astrocytic tumor with BRAF V600E mutation

Desmoplastic infantile astrocytomas (DIA) are rare neoplasms of infancy which are defined by a combination of distinctive clinicopathologic features. DIA was originally defined in 1982 by Taratuto et al. as meningocerebral astrocytoma attached to dura with desmoplastic reaction. In 1993, it was included in the WHO classification under the term ‘desmoplastic cerebral astrocytoma of infancy’ [1]. DIA accounts for 1.25 % of pediatric brain tumors [2]. The large majority of cases presents within the first 24 months of life [1, 3]. Non-infantile cases are rarely encountered with only eight cases reported before [3–10]. However, in two of these cases, clinical symptoms ensued within the first year of life [4, 5]. Less is known about the molecular etiology of DIA [1, 11]. Recent studies suggest that certain types of mostly low grade and pediatric brain tumors may have higher rates of BRAF alterations. BRAF V600E mutations have been detected in small series of pediatric gangliogliomas, pleomorphic xanthoastrocytomas, desmoplastic infantile gangliogliomas and atypical teratoid/ rhabdoid tumors as well as pediatric glioblastomas, anaplastic astrocytomas and diffuse astrocytomas [11–15].

DYSGERMINOMA IN A CHILD WITH ATAXIA–TELANGIECTASIA

Ataxia–telangiectasia is an autosomal recessive disease characterized by progressive cerebellar ataxia, oculocutaneous telangiectasia, immunodeficiency, high incidence of cancer, and increased sensitivity to ionizing radiation. The authors report a case of dysgerminoma in a child with high α-fetoprotein, CA125 and β-human chorionic gonadotropin, who has been followed-up for ataxia–telangiectasia for 2 years.

Neurofibroma adjacent to the thyroid gland and a thyroid papillary carcinoma in a patient with neurofibromatosis type 1: Report of a case

Neurofibromatosis (NF) type 1, also known as von Recklinghausen’s disease, is an autosomal-dominant inherited disorder. Some tumors may develop in these patients, including optic pathway gliomas, astrocytomas, brainstem gliomas, chronic myeloid leukemia, and rhabdomyosarcoma. Patients with neurofibromatosis type 1 show also an increased risk of endocrine tumors, especially pheochromocytomas, whereas thyroid carcinoma is very rare. It is also rare for a neurofibroma to arise in the tissue neighboring the thyroid gland, and mimicking a nonfunctional thyroid nodule. This report presents a case of a neurofibroma adherent to the thyroid gland with thyroid papillary carcinoma in a 26-year-old woman with NF type 1.

Thyroid Medullary Carcinoma in a Teenager With Cowden Syndrome

Cowden syndrome is a multisystemic cancer predisposition disorder, in which the mucocutaneous lesions are the most constant features. We present a case of Cowden syndrome in a teenage boy with hyperkeratotic papules of the facial skin, especially on the eyelids, with episcleritis, proptosis, and papillomatous lesions on the tip of the tongue and lip mucosa. In addition, two nodules in the thyroid gland were detected. On laboratory examination, the carcinoembryonic antigen (CEA) was 34 ng/mL (normal limits: 0–3 ng/mL). Total thyroidectomy was performed. Histopathologic examination of the specimen revealed thyroid medullary carcinoma. After operation, levels of CEA decreased to normal limits.

Synovial sarcoma in a premature newborn

Synovial sarcoma is rarely detected in infants, with an annual incidence of 0.5 per million. Synovial sarcoma occurs more frequently in adolescents and young adults, with the majority of patients presenting between 15 and 40 years of age. It is extremely rare, however, in pediatric patients under 2 years of age. In the present study we examined a 3‐day‐old male infant born at 32 weeks who had a mass on his left arm. Synovial sarcoma was identified on histopathological and immunohistochemical analysis of biopsy material acquired from the mass. On whole body magnetic resonance imaging, diffuse metastases were detected in the bilateral lungs in the retroperitoneal zone, in bilateral suprarenal glands, the right liver lobe, the right kidney, and the brain. To our knowledge this is the youngest patient to be diagnosed with synovial sarcoma in the literature.

KLUVER-BUCY SYNDROME IN A BOY WITH NON-HODGKIN LYMPHOMA

Kluver-Bucy syndrome is a rare neurobehavioral condition characterized by visual agnosia, excessive oral tendency, hypermetamorphosis, placidity, altered sexual behaviors, and changes in dietary habits. The authors report a case of Kluver-Bucy syndrome in a 10-year-old boy with non-Hodgkin lymphoma after intratechal methotrexate administration. He was treated by risperidone without any sequels.

A giant gastroenteric cyst associated with pectus excavatum and compression of the thoracic duct: a case report.

Gastroenteric cysts are unusual developmental cysts encountered in the posterior mediastinum of patients. They are usually lined by alimentary (squamous or enteric) epithelium and contain gastric mucosa and, rarely, pancreatic tissue. Gastroenteric cysts are usually associated with vertebral abnormalities and less often with complete situs inversus. We present a case of a gastroenteric cyst with pectus excavatum and compression of the thoracic duct.