Circumscribed astrocytic gliomas: Contribution of molecular analyses to histopathology diagnosis in the WHO CNS5 classification

The newest revision of the WHO classification of tumors of the central nervous system, also known as WHO 5th edition, introduces substantial changes, especially within the glial tumor category and separates adult-type and pediatric-type glial tumors into different categories for the first time. In addition, another category of glial tumors, “Circumscribed Astrocytic Gliomas” were also created. This group includes pilocytic astrocytoma, pleomorphic xanthoastrocytoma, subependymal giant cell astrocytoma, chordoid glioma, astroblastoma, and the highly nebulous novel entity high-grade astrocytoma with piloid features. We present a brief and critical review of the pathological and molecular characteristics of these often well-demarcated tumors that can occur in adults as well as in the pediatric population.

Pitiriasis rubra pilaris en niños:informe de 2 casos / Pityriasis Rubra Pilaris in children: 2 case reports

RESUMEN Antecedentes: La pitiriasis rubra pilaris es una enfermedad infrecuente pápulo-escamosa crónica en la que existe un trastorno de la queratinización de la epidermis, caracterizada por pápulas foliculares hiperqueratósicas con tendencia a formar placas de coloración asalmonada, descamativas, con islas de piel sana asociadas a queratodermia palmo plantar. Presenta una distribución bimodal en la primera y sexta década de la vida, afectando a ambos sexos por igual. Su etiopatogenia es desconocida, se ha postulado una respuesta inmune anormal ante diferentes estímulos antigénicos, así como alteración del metabolismo de la vitamina A. Se ha clasificado en 6 tipos en base a su presentación, edad de inicio, curso y pronóstico, tratándose en forma tópica o sistémica. Casos clínicos: Se presentan dos casos en pacientes de 10 y 2 años de edad, con manifestaciones clínicas correspondientes al tipo juvenil circunscrito, que es el más frecuente en edad pediátrica y juvenil clásico respectivamente, con histopatología compatible y excelente respuesta al tratamiento tópico. Conclusiones: Aunque la pitiriasis rubra pilaris es una patología rara, deberá ser sospechada si el cuadro clínico es sugestivo, y si es compatible su histopatología. Debe considerarse que el tratamientotópico puede ser suficiente para lograr la resolución del cuadro.

ABSTRACT Background: Pityriasis rubra pilaris is an infrequent chronic papulosquamous disease in which there is a disorder of keratinization of the epidermis, characterized by hyperkeratotic follicular papules with a tendency to form salmon-colored, scaly plaques, with islands of healthy skin associated with palmoplantar keratoderma. It presents a bimodal distribution in the first and sixth decades of life, affecting both sexes equally. Its etiopathogenesis is unknown, an abnormal immune response has been postulated to different antigenic stimuli, as well as alteration of the metabolism of vitamin A. It has been classified into 6 types based on its presentation, age of onset, course and prognosis, being treated appropriately topical or systemic. Clinical cases: Two cases of 10 and 2 years of age are presented, with clinical manifestations corresponding to the circumscribed juvenile type, which is the most frequent in pediatric and classic juvenile age respectively, with compatible histopathology and excellent response to topical treatment. Conclusions: Although pityriasis rubra pilaris is a rare pathology, it should be suspected if the clinical picture is suggestive and its histopathology is compatible. It should be considered that topical treatment may be sufficient to achieve resolution of the condition.

Understanding the structural changes following photodynamic and transpupillary thermotherapy for choroidal hemangioma using optical coherence tomography and optical coherence tomography angiography

Purpose: To study optical coherence tomography (OCT) and optical coherence tomography angiography (OCT-A) features of circumscribed choroidal hemangioma (CCH) following treatment with photodynamic therapy (PDT) and transpupillary thermotherapy (TTT). Methods: A retrospective chart review of consecutive patients treated for CCH over 2 years (May 2016�April 2018). The investigations, in addition to comprehensive eye examination, included color fundus photography, B-scan ultrasonography, OCT, and OCT-A. Results: The study included 16 eyes of 16 patients (9 males and 7 females). The mean age at presentation was 43.5 � 9 years (range 33�62 years). Macula (n = 6) and superior arcade (n = 5) were the common tumor locations. Twelve eyes received multiple treatment sessions: TTT (seven eyes; mean 2.4 sessions) and PDT (five eyes; mean 2 sessions). Four eyes were observed because vision was not threatened. Pretreatment OCT features were Bruch's membrane atrophy (15 eyes), retinal pigment epithelial atrophy (13 eyes), outer retinal abnormalities (12 eyes), and macular subretinal fluid (12 eyes). Pretreatment OCT-A features were complete loss of choriocapillaris (16 eyes), irregularly arranged fine arborizing vessels (11 eyes), and more than 50% signal void hyporeflective areas (12 eyes). Posttreatment OCT-A showed persistence of choriocapillaris loss, flat scar with fibrosis and thinning of choroid in all eyes treated with TTT, and persistence of deeper choroidal vessels and no loss of choriocapillaris in eyes treated with PDT. Conclusion: OCT and OCT-A help understand the structural outcome following PDT and TTT in circumscribed choroidal hemangioma.

Evaluation of photodynamic therapy based on indocyanine green angiography for circumscribed choroidal hemangioma / 中华实验眼科杂志

Objective To observe the efficacy of photodynamic therapy (PDT) based on indocyanine green angiography (ICGA) for circumscribed choroidal hemangioma (CCH).Methods A retrospective observational case series study was performed.Seventeen eyes of 17 patients were diagnosed with CCH and then underwent PDT based on ICGA image in Xuzhou Municipal Hospital from August,2010 to May,2018.The size of the treatment spot was decided according to ICGA images.The period of mean follow-up visit ranged from 6 to 36 months,with an average of (23.3± 1 1.8) months.Best corrected visual acuity (BCVA),sub-retinal fluid (SRF) and central retinal thickness (CRT) based on optical coherence tomography (OCT),the tumor diameter based on ICGA and the tumor thickness based on coloured doppler imaging (CDI) before and after treatment were analyzed.Results Sixteen cases received only one PDT session,one case had ever received PDT and another case had ever received focal laser photocoagulation.The mean BCVA (LogMAR) was 0.99±0.52,1.09±0.50,0.97±0.53,0.81±0.66,0.79±0.69,and the mean CRT was (440.76±281.34),(329.18± 175.02),(274.24± 169.55),(271.53 ± 150.00),(291.06 ± 201.41)μm before PDT,at 1 week,1 month,3 months and the last visit after treatment,with significant differences among the follow-up time (F =6.965,P =0.006;F =8.784,P =0.002).The decimal visual acuity was increased by at least 2 lines in 9 eyes (52.9％),fluctuated within 2 lines in 7 eyes (41.2％),while decreased by at least 2 lines in one eye (5.9％).SRF of all patients was involved in macular area.The absolute absorption of SRF was found in 7 eyes (41.2％) and partial absorption in 8 eyes (47.1％) at 3 months follow-up visit.In the last follow-up visit,the absolute absorption of SRF was found in 12 eyes (70.6％),partial absorption was found in 3 eyes (17.6％) and 2 eyes (11.8％) underwent no change.On FFA and ICGA,the decrease of tumor size was found in 15 patients,while the others underwent no change.All patients underwent less enhanced hyperfluorescent after treatment.The mean tumor height was (3.80± 1.13)mm before PDT and (3.42± 1.14)mm on the last visit,with a significant difference between them (t=4.101,P=0.010).Conclusions PDT with verteporfin for CCH can promote the improvement of visual acuity,the absorption of SRF,the anatomical reposition of the retina and the decrease of tumor size.The majority of patients can get therapeutic effect after one PDT session.

Circumscribed Palmar Hypokeratosis Showing Bowenoid Change Secondary to Human Papilloma Virus / 대한피부과학회지

Circumscribed palmar or plantar hypokeratosis is a rare condition characterized by the occurrence of an erythematous, well-circumscribed, and depressed macule or patch on the palms or soles. Histopathologically, it is characterized by sharp, stair-like abrupt thinning of the horny layer between the affected and unaffected skin. The pathogenesis of this condition remains unclear. Recently, the human papilloma virus has been implicated as a possible etiological contributor. Circumscribed palmar or plantar hypokeratosis usually shows a benign course. Previous reports have not described malignant changes in these lesions. However, its association with actinic keratosis has been reported in a previous case. We report a case of circumscribed palmar hypokeratosis showing bowenoid epidermal change with expression of the human papilloma virus types 6 and 16 in a patient who was successfully treated with ingenol mebutate gel and cryotherapy.

Esclerodermia localizada juvenil: ¿es una enfermedad benigna? / Juvenile localized scleroderma: is it a benign disease?

Resumen Introducción: La esclerodermia localizada juvenil es una enfermedad polimórfica que ocurre con mayor frecuencia en niñas. Se acompaña de morbilidad importante. El compromiso extradérmico es frecuente y se reportan tasas de poliautoinmunidad de hasta 7%. Al momento, se desconocen las características clínicas de los pacientes colombianos con esta enfermedad. Objetivo: Describir las características clínicas, morbilidades y secuelas en pacientes con diagnóstico de esclerodermia localizada juvenil, en múltiples centros de reumatología pediátrica en Colombia. Materiales y métodos: Estudio descriptivo, retrospectivo y multicéntrico. Pacientes con diagnóstico de esclerodermia localizada juvenil con un mínimo de 1 ario de evolución y 6 meses de seguimiento, en 10 centros de reumatología pediátrica mediante revisión de historias clínicas. Resultados: El n = 88. La distribución por género fue: femenino 2,1; masculino 1. Edad promedio al inicio de la enfermedad 7,1 años (0-14). Promedio de duración de la enfermedad al diagnóstico 16,5 meses (1-96). La distribución por subtipos fue morfea circunscrita (32,9%), mixta (31,8%), linear (21,5%, asciende a 55% al incluir formas mixtas con lesiones lineares) generalizada (11,4%) y panesclerótica (2,3%). Se detectaron alteraciones estéticas en el 91%, alteraciones del crecimiento en 41% y compromiso funcional de articulaciones vecinas en 32%. Se presentó compromiso extradérmico en 22,7% y poliautoinmunidad en 12,5%. Conclusiones: La esclerodermia localizada juvenil es una enfermedad polimórfica e impredecible. En la mayoría de los casos el diagnóstico es tardío. La tasa de compromiso extradérmico sugiere que no es una enfermedad limitada a la piel. Un diagnóstico temprano, tratamiento dinámico y seguimiento cercano permiten prevenir y detectar tempranamente complicaciones derivadas de la enfermedad.

Abstract Introduction: Juvenile localized scleroderma is a polymorphic disease. It is more prevalent in girls and has a significant morbidity. Extra-cutaneous involvement is common, and polyautoimmunity can reach 7%. The clinical characteristics of this disease in Colombian patients are currently unknown. Objective: To describe the clinical characteristics, morbidity and outcomes in patients with juvenile localized scleroderma in different paediatric rheumatology clinics in Colombia. Materials and methods: A descriptive, retrospective, and multicentre study was conducted on patients with juvenile localized scleroderma with a minimum of 1 year of disease onset, and 6 months of follow-up in 10 paediatric rheumatology clinics. Results: The study included 88 patients, with a gender distribution of female 2.1: male 1. Mean age at disease onset was 7.1 years (0-14). Mean disease duration at diagnosis was 16.5 months (1-96). Sub-type distribution was, circumscribed (32.9%), mixed (31.8%), and linear (21.5%, that increased to 55% if linear lesions of the mixed subtype are included), generalised (11.4%), and pan-sclerotic morphea (2.3%). Aesthetic compromise was detected in 91%, with growth disturbances in 41%, and joint functional compromise in 32%. Extra-cutaneous involvement occurred in 22.7% and polyautoimmunity in 12.5%. Conclusions: Juvenile localized scleroderma is a polymorphic and unpredictable disease. It diagnosed late in most of the cases. Extra-cutaneous involvement suggests that is not a disease limited to skin. An early diagnosis, a dynamic treatment and a close follow-up helps to prevent, and detect, complications arising from the disease.

Effect of photodynamic therapy combined with intravitreal ranibizumab injection on circumscribed choroidal hemangioma / 国际眼科杂志(Guoji Yanke Zazhi)

@#AIM:To investigate the effect of photodynamic therapy(PDT)combined with intravitreal injection of ranibizumab on circumscribed choroidal hemangioma(CCH). <p>METHODS:A retrospective study was performed for 6 eyes(6 cases)diagnosed as CCH. Before treatment, OCT examination showed macular cystoid edema and retinal neurepithelium layer detachment in all patients. All patients underwent photodynamic therapy, then intravitreal injection of ranibizumab 0.5mg(0.05mL)were administered at 48h after PDT. The best corrected visual acuity(BCVA), examination of the ocular fundus, fundus photography, fluorescence fundus angiography(FFA), indocyanine green angiography(ICGA), eye B ultrasonic and optical coherence tomography(OCT)were performed respectively at 1, 3 and 6mo after treatment. <p>RESULTS:The patients were followed up for 4 to 10mo, the final vision of follow-up increased than before, it was raised 7 lines. The images of ICGA revealed hypofluorescence or no leakage in focal area. Eye B ultrasonic showed that hemangioma shrunk or faded. The images of ICGA revealed macular region retinal reattached well and edema disappeared completely. Mean flow-up was 6mo postoperative. There had no evidence of recurrence. <p>CONCLUSION:For CCH patients, hemangioma got smaller obviously by PDT. Intravitreal ranibizumab injection promote effusion absorption under the retina. Combining use of the two therapies could improve visual acuity in a short-term.

Treatment Outcome of Serous Macular Detachment in Circumscribed Choroidal Hemangioma

PURPOSE: To determine the clinical outcome of anti-vascular endothelial growth factor (anti-VEGF) and photodynamic therapy (PDT) for circumscribed choroidal hemangioma (CCH) with serous retinal detachment. METHODS: The medical records of patients having CCH with serous retinal detachment in macula were retrospectively reviewed. CCH and serous retinal detachment were evaluated via fundus photography, optical coherence tomography, indocyanine green angiography, and ultrasonography. RESULTS: A total of 9 eyes were enrolled in this study. The average follow-up period was 19.2 months. The mean visual acuity was 0.51 ± 0.52 (logMAR) and the mean maximum diameter and thickness of the tumor were 6,154.4 ± 2,019.9 µm and 2,224.4 ± 862.1 µm, respectively. Of the 6 eyes receiving anti-VEGF (mean number of injections: 3.16) as the first treatment for serous retinal detachment, 5 had sustained or recurred intraretinal/subretinal fluid (IRF/SRF) and needed additional PDT and transpupillary thermotherapy. In the 3 eyes that received PDT (mean number of treatments: 1.3) as an initial treatment, IRF/SRF was completely resolved. Finally, 8 eyes achieved complete resolution of SRF and IRF; however visual recovery was limited. CONCLUSIONS: PDT, even with a small number of treatments, can alleviate IRF/SRF in CCH, while anti-VEGF did not.

Choroidal neovascular membrane in a treated choroidal hemangioma.

Association of choroidal neovascular (CNV) membrane with circumscribed choroidal hemangioma is rare, and the CNV development after photodynamic therapy (PDT) is also rare. Etiopathogenesis of these associations is poorly understood. We noted the development of CNV over choroidal hemangioma after PDT therapy in a young female patient in our hospital. Temporal association of CNV development after PDT treatment points toward the possible side effects of PDT. Repeat injections of antivascular endothelial growth factor (ranibizumab) regressed the CNV resulting in a favorable visual outcome.

Cysts within Otherwise Probably Benign Solid Breast Masses and the Risk of Malignancy / Cistos no interior de nódulo mamários benignos: risco de malignidade

Abstract Objective The objective of this study is to assess whether the largest cyst diameter is useful for BI-RADS ultrasonography classification of predominantly solid breast masses with an oval shape, circumscribed margins, and largest axis parallel to the skin, which, except for the cystic component, would be likely classified as benign. Methods This study received approval from the local institutional review board. From March 2009 to August 2014, we prospectively biopsied 170 breast masses from 164 women. We grouped the largest cyst and mass diameters according to histopathological diagnoses. We used Student's t-test, linear regression, and the area under the receiver operating characteristic curve (AUC) for statistical assessment. Results Histopathological examination revealed 143 (84%) benign and 27 (16%) malignant masses. The mean largest mass diameter was larger among malignant (mean standard deviation, 34.1 16.6 mm) than benign masses (24.7 16.7 mm) (P < 0.008). The mean largest cyst diameter was also larger among malignant (9.9 7.1 mm) than benign masses (4.6 3.6 mm) (P < 0.001). Agreement between measurements of the largest mass and cyst diameters was low (R2 = 0.26). AUC for the largest cyst diameter (0.78) was similar to the AUC for the largest mass diameter (0.69) ( p = 0.2). A largest cyst diameter < 3, 3 to < 11, and 11 mm had a positive predictive value of 0, 15, and 52%, respectively. Conclusion A largest cystic component < 3 mm identified within breast masses that show favorable characteristics may be considered clinically inconsequential in ultrasonography characterization. Conversely, masses with a largest cystic component 3 mm should be classified as BI-RADS-US category 4.

Resumo Objetivo Avaliar se o maior diâmetro do cisto é útil para a classificação ultrassonográfica BI-RADS de nódulos mamários predominantemente sólidos, com forma oval, margens circunscritas e maior eixo paralelo à pele que, exceto pela presença do componente cístico, seriam classificados como provavelmente benignos. Métodos Este estudo foi aprovado pelo Comitê de Ética local. De março de 2009 a agosto de 2014, 170 nódulos mamários de 164 mulheres foram prospectivamente biópsiados. As medidas do maior diâmetro do maior cisto e do maior diâmetro do nódulo foram agrupados de acordo com os diagnósticos histopatológicos. O teste t de Student, a regressão linear e a área sob a curva ROC (AUC) foram utilizados para a avaliação estatística. Resultados O exame histopatológico revelou 143 (84%) nódulos benignos e 27 (16%) nódulos malignos. A média da medida do maior diâmetro dos nódulos foi maior entre os nódulos malignos (média desvio padrão, 34,1 16,6 mm) do que nos nódulos benignos (24,7 16,7 mm) (p < 0,008). A média do maior diâmetro do maior cisto também foi maior entre os nódulos malignos (9,9 7,1 mm) do que nos nódulos benignos (4,6 3,6 mm) (p < 0,001). A concordância entre as medidas dos maiores diâmetros dos nódulos e do maior diâmetro do maior cisto foi baixa (R2 = 0,26). A AUC do maior diâmetro do maior cisto (0,78) foi semelhante à AUC do maior diâmetro do nódulo (0,69) (p = 0,2). Os maiores diâmetros dos maiores cistos medindo < 3; 3 e < 11; e 11 mm tiveram um valor preditivo positivo de 0, 15 e 52%, respectivamente. Conclusão Componentes císticos < 3 mm identificados dentro de nódulos mamários que apresentam as demais características provavelmente benignas podem ser considerados clinicamente irrelevantes na caracterização ultrassonográfica. Por outro lado, nódulos que apresentam um componente cístico medindo 3 mm devem ser classificadas na categoria BI-RADS-US 4.

Hemangioma coroideo / Choroidal hemangioma

El hemangioma coroideo circunscrito es un tumor vascular hamartomatoso benigno de baja incidencia. Se presenta este caso debido a su evolución lenta y la pobre respuesta a tratamiento con terapia fotodinámica. El paciente, de sexo masculino, 49 años de edad, con disminución de la visión del ojo derecho de ocho meses de evolución y sin otra sintomatología asociada. Las exploraciones complementarias realizadas (retinografia, OCT, Ecografía y Angiografía flurosceínica) evidencian un tumor yuxtapapilar nasal que es diagnosticado como hemangioma circunscrito de coroides. Se decide tratarlo con terapia fotodinámica. En este caso, aunque el resultado en la ganancia visual no es el esperado, en los exámenes complementarios realizados se observa una discreta mejoría. La lenta evolución de este paciente responde, a criterio de los autores, a varios factores como la edad, el deterioro de la visión en el momento del diagnóstico, el número de aplicaciones de tratamiento con la TFD realizadas, el tiempo de evolución del tumor y el tamaño de la lesión(AU)

Circumscribed choroidal hemangioma is a benign hamartomatous vascular tumor of low incidence. This case is presented for its slow natural history and the poor response to treatment with photodynamic therapy. The 49-year-old male patient with right-eye visual reduction of eight month of evolution and without any other associated symptomatology. Complementary explorations carried out (retinography, OCT, sonogram, fluorescein angiography) evinces a juxtapapillary nasal tumor diagnosed as circumscribed choroidal hemangioma. The decision is to treat it with photodynamic therapy. In this case, despite the fact the visual gain outcome is not the desired, the complementary tests performed revealed a fair improvement. According to the authors, the slow evolution of this patient is due to several factors such as age, visual decay at the time of the diagnosis, the TFD number of applications, the tumor´s time of evolution and the lesion seize(AU)

Circumscribed Palmoplantar Hypokeratosis Treated with Complete Excision / 대한피부과학회지

No abstract available.

Circumscribed Acral Hypokeratosis with Expression of Human Papillomavirus High-risk Type 16, 18 / 대한피부과학회지

Circumscribed acral hypokeratosis (CAH) is a rare disorder characterized by a well-circumscribed and erythematous depressed patch on the palm or sole. It usually presents as an asymptomatic, solitary, long-standing lesion in middle-aged to elderly women. Histologically, it has a characteristic stair-like configuration between normal and involved skin with an abrupt thinning of the horny layer and a decreased granular layer. Although the pathogenesis of CAH is not well established, several hypotheses were proposed. To our knowledge, there have been only two cases to document HPV in a single lesion of CAH. Herein, we report the third case of circumscribed acral hypokeratosis with expression of human papillomavirus, high-risk type 16, 18.

Intravitreal bevacizumab (avastin) for circumscribed choroidal hemangioma.

Circumscribed choroidal hemangiomas are rare ophthalmic entities that cause diminution in vision due to accumulation of subretinal and/or intraretinal fluid in the macular area. Various treatment options ranging from conventional laser to photodynamic therapy have been employed to destroy the tumor and reduce the exudation; however, either the inability to penetrate through the exudative fluid or the collateral retinal damage induced by these treatment modalities make them unsuitable for lesions within the macula. We evaluated the role of intravitreal bevacizumab, a pan-vascular endothelial growth factor (VEGF) inhibitor, in reducing the sub- and intraretinal fluid in three patients with circumscribed choroidal hemangiomas. All the patients had complete resolution of the serous retinal detachment that was maintained till at least 12 months after the first injection. Intravitreal bevacizumab may be used in combination with thermal laser or photodynamic therapy in treating circumscribed choroidal hemangiomas with subretinal fluid.

Circumscr ibed Palmar Hypokeratosis Treated with Cryotherapy / 대한피부과학회지

Circumscribed palmar or plantar hypokeratosis is a rare dermatosis, and it consists of asymptomatic erythematous, well-circumscribed and depressed patch with scaly borders on the palm or sole. It usually affects middle-aged or elderly women. Histopathologically, it has a sharp stair between the normal and involved skin, and a demarcated area with an abrupt decrease in the horny layer thickness. We report herein a case of circumscribed palmar hypokeratosis that was successfully treated with cryotherapy.

Circumscr ibed Palmar Hypokeratosis Treated with Cryotherapy / 대한피부과학회지

Circumscribed palmar or plantar hypokeratosis is a rare dermatosis, and it consists of asymptomatic erythematous, well-circumscribed and depressed patch with scaly borders on the palm or sole. It usually affects middle-aged or elderly women. Histopathologically, it has a sharp stair between the normal and involved skin, and a demarcated area with an abrupt decrease in the horny layer thickness. We report herein a case of circumscribed palmar hypokeratosis that was successfully treated with cryotherapy.

Treatment review of sight threatening circumscribed choroidal haemangioma / 国际眼科杂志(Guoji Yanke Zazhi)

AIM:To describe our clinical experience in treating circumscribed choroidal haemangioma(CCH)in a tertiary referral centre over a fifteen year period prior to photodynamic therapy.METHODS:The departmental database and photographic records of a tertiary referral center were used to identify patients who were treated for CCH between 1992 and 2007.Their case records were reviewed.RESULTS:Visual acuity improved(＞2 Snellen lines)in eleven patients(69%)remained stable in one patient (6%)and deteriorated in four patients(25%).Six of the seven treated with brachytherapy and three of the four treated with transpupillary thermotherapy achieved better visual acuity after treatment.86% of patients treated within six months of onset of symptoms and 50% of patients treated after six months of onset of symptoms noted an improvement in visual acuity.Only one patient in our series had a final VA of 6/60 or worse.Mean follow-up was thirty-five months.CONCLUSION:Visual outcome is better when treatment is performed within 6 months of symptoms.The majority of patients achieved an improvement in visual acuity without any adverse effect following treatment.

Linfangioma capilar: Revisión de la literatura y reporte de un caso caso pediátrico

hamartomatoso o de displasia congénita. Afectan piel o mucosas, así como tejidos subcutáneos o submucosos, en cualquier localización. Se clasifican en superficiales (circunscritos) y profundos (cavernosos, higromas quísticos), en base a su asiento y al tamaño de las formaciones vasculares linfáticas. Presentación del caso. Niña adoptada, de 11 años de edad, de etnia hindú. Tenía varias pápulas granulosas en el dorso lingual, indoloras, de consistencia blanda, de entre 1 y varios milímetros de diámetro y coloración algo más rosada que el tejido circundante, por delante de la V lingual. Se barajaron los diagnósticos de sospecha de papilomas y de linfangioma. Se tomó una biopsia de la lesión más anterior. Histopatología. Mucosa escamosa, con engrosamiento del epitelio, sin desorden madurativo ni atipia y con marcada papilomatosis. La submucosa del eje de las papilas mostraba numerosos conductos vasculares de endotelio aplanado y muy ectásicos, en contacto directo con el epitelio, compatibles con el diagnóstico de linfangioma capilar. Tratamiento. Ante la extensión y localización poco comprometidas de la lesión y dada la edad de la paciente, se optó por mantener una conducta expectante. En el presente artículo se revisa la literatura y se presenta el caso clínico, resaltando la importancia del conocimiento de los aspectos clínicos de los linfangiomas en el ámbito odontológico y estomatológico, al tratarse de una patología de asiento preponderante en la región cervicofacial.

CAPILLAR LYMPHANGIOMA. Review of the literature and report of a pediatric case Lymphangiomas are congenital uncommon malformations of the lymphatic system, that different authors consider to have neoplasic, hamartomatous or congenital dysplasia characters. They can affect skin, mucosal, as well as subcutaneal or submucosal areas, in any location. According to their location and the size of the vascular lymphatic formation they are classified as superficial (circumscribed) and deep (cavernous, cystic hygromas). Case presentation: An eleven-years-old adopted girl of hindu ethnic group presented several granular papules on the lingual back, painless and soft consistent. They were located ahead of lingual V, with a diameter between one and several millimetres. Their colour were slightly more pink than the surrounding tissues. Under a suspection diagnosis of papillomas or lymphangiomas, a biopsy of the most anterior lesion was taken. Histopathology: Squamous mucosa, with enlarged epithelium, without maturative disorder nor atypia, and a marked papillomatosis was found. The submucosa of papilla axis showed numerous vascular ducts of flat endothelium with vascular ectasia. These ducts were in direct contact with the epithelium. The histopathologic diagnosis was of capillar lymphangioma. Treatment: Due to the extension and location of the lesion, without risk and taking into account the age of the patient, an expectant behaviour was chosen. In this paper the literature about lymphangioma is reviewed and a clinical case is presented, highlighting the importance of the knowledge of the clinical aspects of the lymphangioma in the odontologic and estomatologic area. This kind of pathology places with preponderance in the cervicofacial area.

A Case of Circumscribed Palmar Hypokeratosis / 대한피부과학회지

Circumscribed palmar or plantar hypokeratosis is a rare disease that is characterized by a well-circumscribed, erythematous depressed macule or patch on the palm or sole. It usually presents as an asymptomatic, solitary lesion in middle-aged or elderly women. The characteristic histopathological finding is a sharp stair-like alteration between involved and uninvolved skin with a markedly thinner horny layer and slightly decreased granular layer compared with adjacent normal skin. Herein we report a case of circumscribed palmar hypokeratosis and review the literature.

Circumscribed Choroidal Hemangioma: a Report of 7 Cases

PURPOSE: To report the clinical features and management of circumscribed choroidal hemangioma. METHODS: Seven patients with circumscribed choroidal hemangioma were reviewed retrospectively. One eye having a small tumor without subretinal fluid was observed. Four eyes with large tumors around the posterior pole were treated with laser photocoagulation or/and transpupillary thermotherapy. Stereotactic radiosurgery with a gamma knife was applied in 2 eyes with large tumors on the peripheral retina. RESULTS: In all cases, the retinal detachment showed complete resolution. Tumor thickness was decreased in 2 cases treated with stereotactic radiosurgery. Visual acuity was either improved or unchanged in 6 eyes and decreased in one eye after treatment. CONCLUSIONS: Circumscribed choroidal hemangioma is a rare intraocular tumor. These tumors can be treated with variable modalities based on their size, location, and subretinal fluid. They have a favorable prognosis. Stereotactic radiotherapy is a reasonable treatment alternative for large symptomatic choroidal hemangiomas.