Aydin Isisag

Primary orbital neuroblastoma in a neonate.

Neuroblastoma is an undifferentiated malignant tumor of the primitive neuroblasts. Orbital neuroblastoma is typically a metastatic tumor. We describe a two-days-old girl, who presented with a large tumor in her left orbit. Magnetic resonance imaging revealed that the tumor originated from the retrobulbar area, extending into the upper and lateral orbit. She was operated on the fifth day of life. A histopathologic diagnosis of neuroblastoma was made. Medical evaluation including chest roentgenogram, ultrasonography of the abdomen, whole body computerized tomogram and bone scintigraphy showed no evidence of systemic involvement or metastasis. Neuroblastoma should be considered in the differential diagnosis of neonatal orbital tumors.

Does asthma control as assessed by the asthma control test reflect airway inflammation

Background and aimsThe treatment of asthmatic patients is particularly focused on the control of symptoms as well as functional and inflammatory parameters. In our study, we investigated the relationship between the asthma control test (ACT) which evaluates symptoms and airway inflammation and functional parameters.Materials and methodsStable asthmatic patients admitted to our pulmonary outpatient clinic were enrolled in the study consecutively and underwent the ACT, pulmonary function tests and methacholine bronchial provocation test (MBPT). Additionally, fractional exhaled nitric oxide level (FeNO) and induced sputum cell distribution were assessed. All these parameters were re-evaluated at the third month after adjusting medications of the patients according to baseline ACT scores.ResultsOf the 101 patients screened, we analyzed 83 who proceeded to the follow up visit. At the baseline visit, 8 were totally controlled, 36 partially controlled and 39 uncontrolled according to ACT. At the follow up visit, 10 were totally controlled, 39 partially controlled and 34 uncontrolled. Comparison of the two visits in terms of all parameters revealed significant reductions only in the percentages of patients with MBPT positivity (p = 0.029) and FeNO levels > 20 ppb (p = 0.025) at follow up. The percentages of patients with FeNO > 20 ppb, MBPT positivity, induced sputum eosinophilia or induced sputum neutrophilia did not show significant differences between totally controlled, partially controlled and uncontrolled groups at both baseline and follow up visits.ConclusionAlthough the ACT scores did not show significant correlations with the airway inflammation parameters tested in this study, a marked reduction in the percentage of patients with MBPT positivity and FeNO > 20 ppb at follow up may suggest the importance of the control concept in the management of asthma.RiassuntoRazionale e scopoIl trattamento dei pazienti asmatici si concentra in modo particolare sul controllo dei sintomi, parallelamente ai sui parametri funzionali e infiammatori. Nel nostro studio abbiamo indagato i rapporti fra l’“asthma control test” (ACT), che valuta la sintomatologia, con l’infiammazione delle vie aeree e i parametri funzionali.Materiali e metodiSono stati arruolati pazienti asmatici consecutivi in fase stabile afferenti al nostro ambulatorio di pneumologia, e sottoposti a compilazione dell’ACT, test di funzionalità respiratoria, test di provocazione bronchiale con metacolina (MBPT), misurazione della frazione espiratoria di ossido nitrico (FeNO) e citologia dell’espettorato indotto. Tutti questi parametri sono stati rivalutati a tre mesi dopo aggiustamento per il trattamento farmacologico sulla base dei punteggi ACT di base.RisultatiSu 101 pazienti sottoposti a screening, ne sono stati valutati 83 che si sono ripresentati alla visita di follow up. Alla prima visita 8 pazienti erano totalmente controllati, 36 parzialmente controllati e 39 non controllati in base all’l’ACT. Nella visita di follow up, 10 pazienti erano completamente controllati, 39 parzialmente controllati e 34 non controllati. La comparazione fra le due visite ha evidenziato, fra tutti i parametri, una riduzione significativa solo nella percentuale di pazienti con positività a MBPT (p = 0,029) e livelli di FeNO > 20 ppb (p = 0,025) al follow up. La percentuale di pazienti con FeNO > 20 ppb, positività al MBPT, eosinofilia neutrofilia nell’espettorato indotto non hanno mostrato significative differenze tra i gruppi di pazienti ben controllati, parzialmente controllati e non controllati sia alla prima visita che al follow up.ConclusioniAnche se i punteggi dell’ACT non hanno mostrato correlazioni significative con i parametri di infiammazione delle vie aeree valutati in questo studio, la marcata riduzione della percentuale di pazienti con positività al MBPT e FeNO > 20 ppb alla visita di follow up può implicare l’importanza del concetto di controllo nella gestione dell’asma.

[Sinonasal-type haemangiopericytoma: a case report].

ÖZ Tüm sinonazal neoplazmların % 0,5’inden azını oluşturan sinonazal tip hemanjioperisitom, genellikle 3 cm çapta olup, nazal kavitede unilateral polipoid doku oluşturur. Sunulan 34 yaşındaki kadın hasta burun tıkanıklığı ve kanama şikayeti ile başvurmuştur. Yapılan nazal endoskopide; sağ alt konka ve lateral nazal duvar kaynaklı, üzeri düzgün mukoza ile kaplı, nazofarinksten orofarenkse uzanım gösteren, 3,5x3x2 cm ölçülerde polipoid kitle saptanmıştır. Olgumuz histopatolojik ve klinik bulgularıyla karakteristik özellikler taşımasına karşın, farklılıklar gösteren immünohistokimyasal bulgularıyla, bu tümörlerin doğası hakkında yeni bakış açıları sağlamaktadır. ABSTRACT Sinonasal-type hemangiopericytomas, which comprise less than 0.5% of all sinonasal neoplasms, arise unilaterally in the nasal cavity as polypoid masses with a mean diameter of about 3 cm. A 34-year-old female patient was admitted due to nasal obstruction and epistaxis. A polypoid mass covered with intact mucosa that originated both from the right inferior concha and lateral nasal wall was detected by nasal endoscopy. The tumor, extending from the oropharynx to the nasopharynx, was measured as 3,5x3x2 cm. Although exhibiting characteristic histopathological features and typical clinical symptoms, this case with unexpected immunohistochemical findings can provide a viewpoint on the nature of this kind of tumors

Sonic hedgehog, TBX18, and TSHZ3 proteins involved in pyeloureteral motility development are overexpressed in ureteropelvic junction obstruction. An immunohistochemical, histopathological, and clinical comparative study

Objectives: To compare pathological samples obtained from cases that underwent surgery for ureteropelvic junction (UPJ) obstruction with samples obtained during autopsies of subjects. Methods: Retrospectively, 42 patients who had undergone surgery due to UPJ obstruction (group 1) were included in the study. Histopathological and immunohistochemical features for sonic hedgehog (SHH), TBX18, and TSHZ3 of UPJ were evaluated and findings were compared with 20 autopsy cases (group 2). Results: In group 1, the scores were statistically significantly higher in terms of cytoplasmic SHH, nuclear TBX18, cytoplasmic and nuclear TSHZ3 staining. Statistically, no correlation was found between age and the staining scores belonging to these 3 antibodies in group 1 and group 2. Intense inflammation was found to be related with nuclear staining for TBX18. Conclusion: Gene product expressions of SHH, TBX18 and TSHZ3 are statistically higher in patients with UPJ obstruction, when compared with control group. The explanation may be the reactivation of the processes, which had shown their effects in the embryological period, due to the chronic inflammation and long-term micro-trauma created by the disease.

Square IgM crystals in bone marrow in Waldenström macroglobulinaemia.

A 60-year-old male was referred to our hospital with fatigue, palpitations and postural dizziness. Physical examination showed tachycardia and pallor. At presentation, a full blood count showed haemoglobin concentration 89 g/l, white blood cell count 6 6 9 10/l and platelet count 161 9 10/l. There was albumin discordance and protein electrophoresis showed an immunoglobulin (Ig) M monoclonal gammopathy (IgM 3 685 g/l). Excisional biopsy of a cervical lymph node and bone marrow biopsy were performed and a diagnosis of Waldenstr€ om macroglobulinaemia was made. Chemoimmunotherapy (vincristine, cyclophosphamide, methylprednisolone, rituximab and bortezomib) was administered. During this period, the patient also underwent five plasmapheresis procedures. The disease was refractory. At this stage a bone marrow biopsy (top) and immunohistochemistry staining for mu and kappa (bottom) showed square immunoglobulin crystals. Waldenstr€ om macroglobulinaemia is a lymphoma characterized by an IgM paraprotein and bone marrow infiltration by lymphoplasmacytic cells. In this patient there was a diffuse infiltrate of small lymphocytes, plasmacytoid lymphocytes and plasma cells. The surface membrane immunophenotype was CD20-, CD38+ , CD138+ , kappa+, lambda-, IgM+ and IgGwith the unusual observation of square immunoglobulin crystals within the cytoplasm

The Role of Endobronchial Biopsy in the Diagnosis of Pulmonary Sarcoidosis

Bronchofiberscopy (BFS) with endobronchial biopsy (EBB) was performed in 66 patients with pulmonary sarcoidosis (PS). Transbronchial biopsy (TBB) was made in 49 patients from this group to compare the diagnostic value of EBB and TBB. The normal endoscopic picture was observed in 19.6% of patients. Bronchial mucosal hypervascularization and thickening were found in 25.7% of cases; the signs of enlarged bronchopulmonary lymph nodes were detected in 13.6%; catarrhal endobronchitis in 10.6%, and tuberosity changes in 4.5%. Epithelioid granulomas in the bronchial mucosa were identified in 37.8% of cases (30.75 and 39.6% in the intact and abnormal mucosa, respectively). The diagnostic value of TBB was 65.3%. EBB could reveal epithelioid-cell granulomas in 4 of the 17 patients with negative TBB. The findings suggest that BFS with EBB should be included as a necessary diagnostic method for the examination of patients with PS.