Halit Pazarli

Retinoblastoma in Turkey: survival and clinical characteristics 1981 - 2004

Background: In this study, the authors aim to describe the survival and clinical characteristics of 141 retinoblastoma cases treated at Cerrahpasa Medical Faculty, University of Istanbul, Istanbul, Turkey, between 1981 and 2004.

Gamma knife stereotactic radiosurgery yields good long-term outcomes for low-volume uveal melanomas without intraocular complications.

We present the outcomes of 35 uveal melanoma patients treated with gamma knife stereotactic radiosurgery. All cases were previously untreated. During follow-up, regular MRI examinations were used to detect any changes in tumor size and estimate the local long-term tumor control rate. Treatment-related complications were also recorded. During follow-up, systemic dissemination was observed in two patients, one of whom died of metastases. The most frequent complication was retinal detachment (17.1%). Three patients required enucleation. Cumulative 1-year and 3-year local tumor growth control rates were 97% and 83%, respectively. The mean and median times to local tumor progression were 48.0 and 51.7 months, respectively. Gamma knife surgery may be a suitable alternative for the treatment of low-volume uveal tumors without intraocular complications, as the control rate and long-term outcomes compare favorably with those of surgical excision and brachytherapy.

Subconjunctival cyst extending into the orbit.

A fourteen year old girl who had developed a large subconjunctival mass extending into the orbit is presented. The mass had appeared after a strabismus operation, and it caused a consecutive exotropia and decreased adduction in the affected eye. She had had this cyst for six years. A cranial CT scan confirmed the presence of a massive orbital cyst. This cyst was removed surgically. Postoperatively, exotropia and adduction limitation improved. The pathological report stated that it was an epithelial cyst. This cyst and its differential diagnosis is the subject of this paper.

Malignant Rhabdoid Tumor of the Orbit / Orbital Malign Rabdoid Tümör

ÖZ Malign rabdoid tümör, çocukluk döneminin nadir rastlanan malign bir tümörüdür. En sık yerleşim yeri santral sinir sistemi ve böbrektir. Ancak nadir olarak orbitadan da kaynaklanabilir. Çocukluk çağının diğer yumuşak doku sarkomları ile ayırıcı tanısı yapılmalıdır. Bu yazıda, perinatal dönemde orbital malign rabdoid tümör tanısı ile cerrahi ve kemoterapi uygulanan 8 haft alık bir kız olgu sunulmuştur. Sekiz haft alık olgu, doğumdan itibaren fark edilen sağ göz kapağında şişlik şikayeti ile hastanemize getirildi. Sağ proptozisi dışında fizik muayene ve laboratuvar değerlendirmesi normaldi. Kitle cerrahi olarak çıkartıldı. Örnek, histopatolojik ve immünohistokimyasal inceleme sonucu malign rabdoid tümör olarak değerlendirildi ve kemoterapi verildi. Olgu klinik olarak remisyonda iken, febril nötropeni atağı ile kaybedildi. Malign rabdoid tümör, nadir de olsa gözden kaynaklanabilir. Göz kitlelerinin ayırıcı tanısında düşünülmeli ve kötü gidişli bir tümör olduğundan cerrahi, kemoterapi ve lokal radyoterapi gibi tedavi seçenekleri kombine olarak kullanılmalıdır. Abstract Malignant rhabdoid tumor is a rare and malignant tumor of childhood. Generally it originates from the kidney and central nervous system, but occasionally it may arise from the orbit. Differential diagnosis from other childhood soft tissue sarcomas should be done. We report here an 8-week-old female infant with malignant rhabdoid tumor of the orbit who was treated with chemotherapy and surgery. Th e 8 week-old girl was referred to our hospital with a history of right proptosis first noted at birth. Physical and laboratory evaluation of the patient was normal except for right proptosis. Th e mass was removed surgically. Histopathologic examination and immunohistochemical findings of the specimen were evaluated as malignant rhabdoid tumor. Chemotherapy was administered. While in clinical remission, she succumbed during a febrile episode. Malignant rhabdoid tumor can rarely originate from the orbit. Malignant rhabdoid tumor should be kept in mind in the differential diagnosis of orbital masses, and surgery, chemotherapy and local radiotherapy should be used as combined therapy due to the poor prognosis.

Gözde kitle nedeni ile başvuran hastalarımızın geriye dönük olarak dökümü: 1985 2004 Retrospective analysis of patients admitted to our clinic with eye mass: 1985 2004 Orijinal Araştırma

Objective: The objective of this study is to analyse the patients with eye mass who were admitted to our clinic in a period of 20 years to classify both benign and malignant orbital or ocular tumors and to inform the pediatricians about the outcome of our analysis Material and Method: The records of 315 patients applied or referred to our pediatric hematology oncology department with eye mass between January1985 and December 2004 were reviewed The sex distribution the involvement site and the age at dignosis were analysed Results: 156 49 5 patients were males while 159 50 5 were females 151 of the masses involved the left eye 47 9 while 147 of them 46 7 were at the right side and 17 5 4 involved both eyes The age at diagnosis range ed between 1 and 192 months The median age at diagnosis was 25 months 282 89 6 of the cases had orbital tumors 22 6 9 cases had ocular surface tumors and 11 3 5 had intraocular tumors Conclusions: Pediatricians should notice the signs and symptoms of pediatric ocular and orbital tumors while examining their patients We would like to emphasize the importance of noticing the signs and symptoms of pediatric ocular and orbital tumors and vitality of referring patients to ophtalmologist in time Key words: child eye mass orbital

Cerrahpaşa Tıp Fakültesi Retinoblastom deneyimi Orijinal Araştırma

Aim: In this study we aim to describe the survival and clinical characteristics of 141 retinoblastoma cases treated at Cerrahpasa Medical Faculty University of Istanbul between 1981 and 2004 Material and Method: We retrospectively analyzed the clinical recordos of 141 children 177 eyes nbsp; diagnosed with retinoblastoma and treated between 1981 2004 The survival rates were calculated by Kapla meier method Results: One hundred and five 74 5 cases were unilateral and 36 25 5 were bilateral The overall mean age at the time of diagnosis was 25 months; in unilateral cases 29 months; and in bilateral cases 16 months The most common presenting signs were leukocoria 116 cases 82 strabismus 14 cases 10 and proptosis 11 cases 8 Conclusions: The three year cumulative survival rate of the 141 patients was 89 68 unilateral 90 74 ; bilateral 87 35 p=0 9371 p gt;0 05 log rank test Key words: Prognosis retinoblastoma survival