ICHTHYOSIS LINEARIS CIRCUMFLEXA: NOT ALWAYS A PART OF NETHERTON SYNDROME.

Ichthyosis Linearis Circumfiexa (ILC) is a rarely seen autosomal recessive keratinization disorder and is characterized by erythematous, polycystic, plaques with 'double-edged' scales. Its histological features resemble psoriasis. A triad of ichthyosis (usually Ichthyosis Linearis Circumfiexa), trichorrhexis invaginata (a distinctive hair shaft disorder) and atopy is named as Netherton syndrome. Herein, we report a 12 year-old girl presenting with ILC not accompanied with typical atopy findings and hair shaft disorder.

Preoperative chemotherapy for T2 breast cancer is associated with improved surgical outcome.

BACKGROUND: The aim of this study is to compare the clinical outcome in T2 breast cancer patients who underwent preoperative chemotherapy (PC) and who did not. The study also tried to define a subgroup of patients, who are more beneficial after PC in terms of lower re-excision rates, better cosmetic results and local recurrence free survival. MATERIALS AND METHODS: 251 consecutive patients treated for nonmetastatic T2 invasive breast cancer were analyzed retrospectively. Of those; 141 underwent primary surgery (PS) followed by chemotherapy, whereas 110 were treated with combination of PC and surgery. RESULTS: The patients who were treated with PC had a significantly higher incidence of negative margins and lower rate of re-excision (5% vs. 16%, p = 0.02). Of all patients attempted breast conserving surgery (BCS), patients in the PC group were more likely to undergo BCS as their definitive operation compared to patients with PS group (BCS rates; PC group: 99% vs. PS group: 92%, p = 0.05). Multifocal disease (OR: 7, 95% Cl, 2.7-18.4, p = 0.0001) and PC (OR = 0.2; 95% CI, 0.06-0.72, p = 0.01) were factors associated with margin positivity in patients treated with BCS. There was no statistically significant difference in 5 year local-recurrence free survival rates between 2 groups. CONCLUSIONS: Our study shows that PC significantly decreases the re-excision in patients undergoing BCS with primary T2 breast tumors. This data suggests that any patient with a tumor greater than 2 cm might be considered for PC to increase BCS success with final negative margins.

Management of a Benign Phyllodes Tumor in a 13-Year-Old Girl with Trans-position of the Nipple Areola Complex and Breast Reconstruction.

Phyllodes tumor is a rare primary tumor of the breast. In children and adolescents, it is even rarer with only 20 cases, treatment of which vary in the literature. Herein we report the case of a 13-year-old female patient with a giant benign phyllodes tumor eroding the bottom of the breast skin and causing nipple retraction. We performed breast conservative surgery by mobilizing the areola, using skin flaps and inserting an implant. Breast malignancy, including phyllodes tumor (PT), is very rare in adolescents. PT, previously called cystosarcoma phylloides, consists of leaf-like fronds, from which the tumor gets its name (1, 2). Although PT is most often seen in the fourth decade of life, almost 20 cases have been reported in the adolescent period, most of which are benign. The histologic types are benign, borderline, and malignant, depending on the mitotic rate of the tumor (3, 4).

Guidelines for the Cytopathologic Diagnosis of Epithelioid and Mixed-Type Malignant Mesothelioma: a secondary publication.

OBJECTIVE: To provide practical guidelines for the cytopathologic diagnosis of malignant mesothelioma. DATA SOURCES: Cytopathologists with an interest in the field involved in the International Mesothelioma Interest Group (IMIG) and the International Academy of Cytology (IAC) contributed to this update. Reference material includes peer-reviewed publications and textbooks. RATIONALE: This article is the result of discussions during and after the IMIG 2012 conference in Boston, followed by thorough discussions during the 2013 IAC meeting in Paris. Additional contributions have been obtained from cytopathologists and scientists who could not attend these meetings, with final discussions and input during the IMIG 2014 conference in Cape Town.

First Report of Campylocarpon fasciculare Causing Black Foot Disease of Grapevine in Turkey.

Soil-borne fungal diseases have become an important problem in grapevine nurseries of the Aegean region (western Turkey) in recent years. Reduced vigor, black vascular streaking in basal ends, blackish-sunken necrotic root lesions, and young vine death were observed in 15 grapevine nurseries of Manisa city in May 2011 and 2012. To determine the causal agents, symptomatic young grapevine (Vitis vinifera cv. Sultana 7) plants (grafted on 1103 Paulsen) were collected from nurseries (8 to 10 plants from each). Symptomatic basal end tissues were surface disinfested with 95% ethanol and flame sterilized. The internal tissues were plated onto potato dextrose agar amended with tetracycline (0.01%). Campylocarpon-like fungi were isolated (with 37.9% isolation frequency) from only one nursery (corresponding to 6.7% of all surveyed nurseries). Fungal colonies were incubated for 21 days in the dark to induce sporulation. Fungal colonies produced cottony aerial mycelium and turned chocolate-brown to dark brown on PDA. Abundant macroconidia were observed at branched conidiophores on long and cylindrical phialides. Microconidia were not observed. Macroconidia were generally 2 to 4 septate, cylindrical and slightly curved, with the following dimensions: 2 septate: 33.5 to 40.7 × 6.1 to 7.6 µm (mean: 35.9 × 6.8 µm), 3 septate: 36.2 to 43.4 × 6.6 to 8.3 µm (mean: 37.3 × 7.6 µm), and 4 septate: 48.9 to 53.5 × 7.6 to 8.3 µm (mean: 50.7 × 8.0 µm). Fifty macroconidia were measured. Morphologically, the isolates resembled the published description of Campylocarpon fasciculare Schroers, Halleen & Crous (2,4). For molecular identification, fungal DNA was extracted from mycelium and ribosomal DNA fragments (ITS1, 5.8S ITS2 rDNA), ß-tubulin, and histone H3 genes, amplified with ITS 4-5, Bt 2a-2b, and H3 1a-1b primers (3,5), and sequenced. Sequences were compared with those deposited in GenBank. The isolate (MBAi45CL) showed 99% similarity with Campylocarpon fasciculare isolates AY677303 (ITS), AY377225 (ß-tubulin), and JF735502 (histone H3). The DNA sequences were deposited into GenBank under accessions KJ573392, KJ573393, and KJ573394 for ITS, ß-tubulin, and Histone H3 genes, respectively. To fulfill Koch's postulates, pathogenicity tests were conducted under greenhouse conditions on own-rooted grapevines (Vitis vinifera) cv. Sultana 7. Plants were removed from the rooting bench and the roots were slightly trimmed and submerged in a 107 ml-1 conidial suspension of the isolate for 60 min (5). After inoculation, the rooted cuttings were planted in 1-liter bags containing a mixture of soil, peat, and sand (2:1:1, v/v/v), and maintained in the greenhouse (24°C. 16/8-h day/night, 75% RH). Ten plants were inoculated with the isolate and five plants were submerged in sterile distilled water (control). After 4 months, young vines were examined for vascular discoloration, reduced root biomass, blackish lesions, and recovery of fungal isolates. The experiment was repeated twice. Blackish-brown discoloration of xylem vessels and necrosis in the basal ends was visible in the inoculated plants but not in the control plants. The pathogen was successfully re-isolated from 69.1% of the inoculated plants. This report is important for the new studies aiming at black foot disease control in Turkey viticulture. References: (1) A. Cabral et al. Phytopathol. Mediterr. 51:340, 2012. (2) P. Chaverri et al. Stud. Mycol. 68:67, 2011. (3) N. L. Glass and G. C. Donaldson. Appl. Environ. Microbiol. 61:1323, 1995. (4) F. Halleen et al. Stud. Mycol. 50:431, 2004. (5) T. J. White et al. PCR Protocols: A Guide to Methods and Applications. Academic Press, San Diego, CA, 1990.

The Bethesda system for reporting thyroid cytopathology: an institutional experience of the outcome of indeterminate categories.

OBJECTIVE: In this study, we investigated the efficacy of the Bethesda system (TBS) for reporting thyroid cytopathology in determining the risk of malignancy for indeterminate cases. These cases comprised atypia or follicular lesion of undetermined significance (AUS/FLUS), follicular neoplasia or suspicious for follicular neoplasia (FN/SFN) and suspicious for malignancy (SM) categories. AUS/FLUS cases were further subcategorized according to their patterns, and the malignancy rate for each subcategory was calculated. METHODS: A total of 6310 thyroid fine needle aspiration (FNA) cases were analysed and 655 indeterminate cases were enrolled in the study. AUS/FLUS cases were further subcategorized into four patterns as follows: papillary thyroid carcinoma (PTC) pattern, microfollicle pattern, atypical cell pattern and Hürthle cell pattern. RESULTS: Malignancy rates for AUS/FLUS, FN/SFN and SM were 18.9%, 45.7% and 71%, respectively. Malignancy rates for AUS/FLUS subcategories were as follows: PTC pattern, 28%; microfollicle pattern, 6.9%; atypical cell pattern, 22.2%; and Hürthle cell pattern, 0%. CONCLUSION: The Bethesda system categorizes thyroid FNAs with increasing risk of malignancy as advocated. However, our findings suggest that the risk of malignancy for AUS/FLUS might be higher than estimated. Particularly, AUS/FLUS cases with focal features suggestive of PTC seem to be associated with a much higher risk of malignancy than other patterns.

Pseudopapillary pattern in intra-operative squash smear preparations of central nervous system germinomas.

BACKGROUND: Although the morphology of central nervous system (CNS) germ cell tumours is very similar to that of gonadal germ cell tumours, some architectural changes may dominate the microscopic appearance of CNS germinomas leading to misdiagnosis at low-power magnification. METHODS: We report five cases of CNS germinoma demonstrating delicate pseudopapillary fronds on squash smear preparations. RESULTS: The age of the patients ranged from 5 to 21 years (mean 14). Three were female and two male. Three patients presented with symptoms of diabetes insipidus, including polydipsia and polyuria, while absence seizures, meaningless speech, hemiparesia, weight loss, insufficient breast development, amenorrhoea and symptoms of raised intracranial pressure were also encountered depending on the location of the tumours. Tumours were located in the hypophysis in two cases and in the suprasellar region in three. During the intra-operative pathological consultation, evenly distributed pseudopapillary or papillary structures formed the dominant pattern in the squash preparations of all cases. The neoplastic cells were characterized by pale variably vacuolated cytoplasm, pleomorphic nuclei with irregular membranes, and several prominent nucleoli. Variable numbers of small lymphocytes were also found. CONCLUSION: Intracranial germinomas may commonly exhibit a pseudopapillary pattern on squash smears that may cause misdiagnosis as neoplasms with papillary morphology. Careful examination of cellular details is essential in order to reach the correct diagnosis.

Maxillofacial osteosarcoma successfully treated with surgery and adjuvant chemotherapy in a child.

Maxillofacial osteosarcoma constitutes a minor percentage of all the head and neck tumors. We describe a 10 year-old girl presenting with swelling and pain in left maxillary region and diagnosed as low grade osteosarcoma. The patient was operated and given a chemotherapy protocol consisted of Cisplatin and Doxorubicin. After six courses of chemotherapy the patient was in complete remission and she is well with no evidence of disease for five years. Since high local recurrence rates have been reported in craniofacial osteoarcoma and we know the deleterious side effects of radiation therapy in children, we believe that best management strategy for osteosarcomas in maxillofacial region in children is radical surgical excision and postoperative chemotherapy (Fig. 3, Ref. 11).

A rare histological presentation of a common lung tumor: adenoleiomyomatous hamartoma.

Hamartomas are the most common benign pulmonary tumors. They most often contain either chondromatous or lipomatous tissues. Thus, they show typical radiological findings which help to make a preoperative diagnosis of this benign tumor. We present a very rare case of a pulmonary hamartoma with atypical radiologic and histologic findings. Histological examination revealed the lesion to be an adenoleiomyomatous hamartoma.

Cystic lesions of the head and neck: cytohistological correlation in 63 cases.

OBJECTIVE: To investigate the accuracy of fine needle aspiration cytology (FNAC) in the diagnosis of cystic masses of the head and neck (H&N), excluding thyroid lesions. METHODS: A total of 198 cases, 63 of whom had consequent surgical specimens, were retrieved from the files of two university hospitals and reviewed. RESULTS: FNAC correctly diagnosed 25 of 36 neoplasms with a cystic component. Five Warthin's tumours, two squamous cell carcinomas, two mucoepidermoid carcinomas and two schwannomas yielded non-representative aspirates. Four of the missed Warthin's tumours and two mucoepidermoid carcinomas which were misdiagnosed as benign cysts were aspirated by their clinician. One branchial cleft cyst was cytologically interpreted as highly suspicious for carcinoma. CONCLUSIONS: Disparate entities may present with similar cytological findings in the H&N region. A detailed description of differential diagnosis should be given in the cytology report in suspicious cases. Repeated aspirations from different sites of the lesion may reduce the false-negative rate.

Histopathological alterations in ciliary ganglions in meningitis: an experimental study.

BACKGROUND: Although it is well known that meningitis frequently results in optic nerve (ON) and oculomotor nerve (OMN) dysfunctions, the effects of meningitis on the ciliary ganglion (CG) have not been studied. It is expected that the CG may be affected due to the involvement of these cranial nerves in meningitis. In this study, we aimed to investigate the effect of meningitis on the CG. METHODS: This study was conducted on thirteen rabbits. Experimental meningitis has been achieved with Streptococcus pneumonias inoculation into the cisterna magna of the animals. After follow-up of two months, all animals were sacrificed. CGs of all animals were examined histopathologically. Neuron numbers and morphological changes of the CGs were examined. RESULTS: Arachnoiditis and axonal degeneration at the cisternal segments of both oculomotor and optic nerves were observed. Neuronal irregularity, cellular angulation, shrinkage, nuclear irregularity and cytoplasmic condensation were observed in neurons of the CG. The mean number of live neurons in a CG was 3200 in healthy rabbits, whereas it was 2800 in animals with meningitis. CONCLUSION: Cisternal segments of the ON and OMN have a meningeal sleeve and a rich vascular supply. Meningitis may cause vasculitis or vasospasm at these arteries and may result in infectious neuropathy of the OMN and ON, and also afferent and efferent loops of the light reflex were structurally interrupted. Consequently, parasympathetic preganglionic denervation of the CG may occur and may result in degeneration in the neurons of the CG.

Ocular surface epithelia contain ABCG2-dependent side population cells exhibiting features associated with stem cells.

When cell populations are incubated with the DNA-binding dye Hoechst 33342 and subjected to flow cytometry analysis for Hoechst 33342 emissions, active efflux of the dye by the ABCG2/BCRP1 transporter causes certain cells to appear as a segregated cohort, known as a side population (SP). Stem cells from several tissues have been shown to possess the SP phenotype. As the lack of specific surface markers has hindered the isolation and subsequent biochemical characterization of epithelial stem cells this study sought to determine the existence of SP cells and expression of ABCG2 in the epithelia of the ocular surface and evaluate whether such SP cells had features associated with epithelial stem cells. Human and rabbit limbal-corneal and conjunctival epithelial cells were incubated with Hoechst 33342, and analyzed and sorted by flow cytometry. Sorted cells were subjected to several tests to determine whether the isolated SP cells displayed features consistent with the stem cell phenotype. Side populations amounting to <1% of total cells, which were sensitive to the ABCG2-inhibitor fumitremorgin C, were found in the conjunctival and limbal epithelia, but were absent from the stem cell-free corneal epithelium. Immunohistochemistry was used to establish the spatial expression pattern of ABCG2. The antigen was detected in clusters of conjunctival and limbal epithelia basal cells but was not present in the corneal epithelium. SP cells were characterized by extremely low light side scattering and contained a high percentage of cells that: showed slow cycling prior to tissue collection; exhibited an initial delay in proliferation after culturing; and displayed clonogenic capacity and resistance to phorbol-induced differentiation; all features that are consistent with a stem cell phenotype.

Expression of KIR and C-type lectin receptors in Behcet's disease.

OBJECTIVE: Behçet's disease (BD) is a multisystemic disorder with a possible underlying pathology of immune-mediated vasculitis. Genetic susceptibility associated with HLA-B*51 and B*2702 has been implicated in its pathogenesis. Considering the recently defined regulatory mechanisms of NK cells through HLA class I binding receptors, we hypothesized that interactions of NK and T cells through the NK receptors may be important in the pathogenesis of BD. METHODS: The impact of different expression patterns of HLA-recognizing receptors on NK or T cells was analysed in 51 patients with BD and 32 healthy controls. We used flow cytometry to investigate the expression of KIR3DL1 from the polymorphic killer immunoglobulin-like receptor (KIR) family, which binds a shared HLA-Bw4 motif on HLA-B51 and *2702 alleles, and CD94 from the conserved C-type lectin receptor family, which binds HLA-E. Thirty-three of the BD patients and 19 of the controls carried the same HLA-Bw4 motif. RESULTS: CD3(+) T cells were increased in patients with BD compared with controls (81 vs 75%, P = 0.001), whereas the NK cells did not show any difference between the two groups. Increased expression of CD94 in BD was observed on CD16(+)CD56(+) cells (66 vs 57, P = 0.04) and on CD3(+) (7.7 vs 4.0, P < 0.001) and CD3(+)CD56(+) (44 vs 35, P = 0.02) T cells. KIR3DL1 expression on the NK and T cells was not statistically different between the two groups. No effect of HLA-Bw4 motif was observed on the expression of CD94 and KIR3DL1 in both the patients and the controls. CONCLUSION: The absence of a correlation between KIR3DL1 expression and HLA-Bw4 motif confirms previous work reporting that the expression of these molecules is regulated separately. Increased expression of CD94 may suggest that NK receptors play a pathogenic or regulatory role in BD.

Continuous neural monitoring in lumbar spine surgery: experience with 101 patients.

The first report about lumbar spinal surgery under epidural anesthesia has been published in 1958. The aim of this study was assess to epidural anesthesia as a regional operative anesthesia in lower lumbar spine surgery and to use the opportunity of verbal interaction with the patient who is able to move her/his lower extremities voluntarily during surgery. We called this simultaneous clinical assessment process as continuous neural monitoring. Established pathologies in the patients were as follows; lumbar herniated intervertebral disc disease (n = 95), lumbar spondylolisthesis (n = 4), lumbar lamina fracture (n = 1) and lumbar dumbbell Ewing's sarcoma (n = 1). Preoperative findings, operative findings, operative complications, postoperative complications and short-term results were assessed by the surgeon-anesthesiologist team. The patients, 99 out of 101, were followed-up for more than 15 months (mean 21.2 months). Fair and failure results were assessed in 7.0 % of the patients. Even though epidural anesthesia is almost equal to general anesthesia in terms of anesthetic complications, surgical complications and surgical results in uncomplicated lumbar spinal surgery cases, it has the advantage of being able to simultaneously evaluate motor results of surgical maneuvers and/or manipulations on the neural structures. This advantage, which is not available at the same accuracy with other neurophysiologic monitoring modalities, would be valuable in complex lumbar spine surgery cases.

Effect of neocuproine, a selective Cu(I) chelator, on nitrergic relaxations in the mouse corpus cavernosum.

The effects of neocuproine and bathocuproine, Cu(I) and Cu(II) chelators, respectively, were studied on relaxations in response to electrical field stimulation, acetylcholine, S-nitrosoglutathione, acidified sodium nitrite and sodium nitroprusside in the mouse corpus cavernosum precontracted with phenylephrine. Neocuproine significantly inhibited relaxations induced by electrical field stimulation, acetylcholine and S-nitrosoglutathione, but not by acidified sodium nitrite and sodium nitroprusside. The pre-prepared neocuproine-Cu(I) complex was ineffective on the responses. The discrepancy between the shape of relaxations in response to electrical field stimulation or to acetylcholine and S-nitrosoglutathione was abolished by adding CuCl(2) into the bathing medium. The copper action was blocked by neocuproine but not by bathocuproine. However, the pre-prepared bathocuproine-Cu(II) complex did not accelerate the relaxations affected by CuCl(2). These findings suggest that a Cu(I)-dependent mechanism may play a role in the relaxation induced by the endogenous relaxant factor as well as by S-nitrosoglutathione in mouse cavernosal tissue.

Inhibitory actions of hydroxocobalamin, cyanocobalamin, and folic acid on the ultraviolet light-induced relaxation of the frog upper oesophageal strip.

The applications of ultraviolet (UV) light (336 nm) on the upper oesophageal strips of frog elicited relaxant responses in the presence of NaNO2 (50 microM). The tissues were mounted under the tension 0.5 g in an organ bath containing Ringer solution, maintained at 25 degrees C and gassed with 100% O2. The responses were recorded on a kymograph via an isotonic lever. Antimegaloblastic agents, including hydroxocobalamin (1, 10, and 100 microM), cyanocobalamin (1, 10, 25, and 100 microM), and folic acid (1, 10, 50, 100, and 200 microM), significantly attenuated the relaxation response to UV light. Folinic acid (1, 10, 25, and 100 microM), however, enhanced the relaxation. Pyrogallol (50 microM), hydroquinone (50 microM), and diethyldithiocarbamic acid (8 mM) were found ineffective for attenuation, though FeSO4 (200, 400, and 500 microM) and hemoglobin (50 microM), respectively, exerted significant inhibition. L-arginine methylester (500 microM) did not impair UV-induced relaxation. Based on these results, we concluded that a mechanism involving undefined action(s) of antimegaloblastic drugs may cause alterations in the UV light-induced relaxation of the tissue used.