Sarcoma de Kaposi anoperineal: uma rara apresentação / Perineal Kaposi's Sarcoma: a rare presentation

O sarcoma de Kaposi é uma neoplasia maligna associada à infecção pelo herpes vírus humano 8 em doentes imunossupressos. O sarcoma de Kaposi Epidêmico é o tipo epidemiológico mais frequente e afeta indivíduos VIH-positivos. A região anoperineal é raramente envolvida e as lesões suspeitas devem ser biopsiadas para confirmação histológica. A base do tratamento é a restauração imune do doente. Relatamos o caso de um jovem, com diagnóstico recente de infeção pelo VIH, sem tratamento, que foi admitido no serviço de infectologia apresentando sintomas constitucionais, adenomegalias inguinais e extensa lesão verrucosa e ulcerada na região anoperineal. As biópsias confirmaram o diagnóstico de sarcoma de Kaposi e o doente iniciou terapia antirretroviral e quimioterapia. Houve recuperação clínica, regressão das lesões e desaparecimento das adenomegalias. Este relato objetiva alertar as equipes médicas no sentido de se incluir o sarcoma de Kaposi no diagnóstico diferencial das lesões que afetam a região anoperineal.

Kaposi's sarcoma is a malignant neoplasm associated with human herpesvirus 8 infection in immunocompromised patients. Epidemic Kaposi's sarcoma is the most common epidemiological type and affects HIV-positive patients. Perineal involvement is rare, and suspicious lesions should be biopsied to confirm histological diagnosis. Treatment consists of restoring the patient's immune system. We report the case of a young patient recently diagnosed with HIV, without treatment, who was admitted to the Department of Infectious Diseases with nonspecific symptoms, inguinal lymphadenopathy, and an extensive verrucous ulcerated lesion in the perineal region. Biopsy confirmed the diagnosis of Kaposi's sarcoma, and the patient was started on antiretroviral therapy and chemotherapy. Clinical recovery was achieved, with lesion reduction and inguinal adenopathy resolution. This case report aims to encourage physicians to include Kaposi's sarcoma in the differential diagnosis of perineal lesions.

Quistes subglóticos adquiridos: Reporte de tres casos y revisión de la literatura / Acquired subglottic cysts: Report of three cases and review of the literature

Los quistes subglóticos adquiridos son una causa rara de estridor en la infancia, cuyo reporte ha ido en aumento en las últimas décadas. Su aparición se relaciona con la prematurez y la intubación en el periodo neonatal. Histológicamente, se observa una obstrucción de las glándulas mucosas de la subglotis debido una metaplasia escamosa del epitelio respiratorio. Esta es una condición que usualmente requiere de un alto índice de sospecha para llegar al diagnóstico, ya que pueden confundirse con otras patologías como laringitis aguda (croup), laringomalacia o asma. La nasofibroscopía permite sospechar su presencia, pero el gold standard diagnóstico lo constituye la laringo-tra-queo-broncoscopía directa en pabellón. Existen diversas técnicas para su manejo, siendo las más frecuentemente utilizadas la marsupialización con instrumental frío y láser. La recurrencia es frecuente, por lo que algunos autores han utilizado mitomicina C y la terapia antirreflujo para intentar disminuirla. Sin embargo, hasta la fecha falta evidencia de calidad que permita llegar a un consenso respecto al manejo ideal de esta patología. En este trabajo, presentamos tres casos clínico de pacientes con antecedentes de prematurez que fueron diagnosticados con quistes subglóticos adquiridos y manejados en un hospital pediátrico de alta complejidad.

Acquired subglottic cysts are an infrequent cause of stridor in infants, which has been increasingly reported in the last decades. Its appearance is related to prematurity and intubation in the neonatal period. Histologically, findings are characterized by an obstruction of the mucosal glands, due to squamous metaplasia of the respiratory epithelium. This condition usually requires a high index of suspicion to be diagnosed, as it can be misdiagnosed as croup, laryngomalacia or asthma. Flexible nasendoscopy allows an initial exploration of the larynx, but direct laryngo-tracheo-bronchoscopy in the operating room is the diagnostic gold standard. There are several techniques for its management, but the most frequently used are cold-steel marsupialization and laser. Recurrence is common, and some authors have used mitomycin C and antireflux therapy to try to decrease it. However, up to date, there is a lack of high-quality evidence, regarding the ideal management of this pathology, which prevents reaching a consensus. In this article, we present three clinical cases of premature patients who were diagnosed with subglottic cysts, treated in a tertiary pediatric hospital.

Atresia de coana bilateral en adulto con malformación craneofacial asociada: un diagnóstico poco habitual / Bilateral choanal atresia in adult with associated craneofacial malformation: an unusual diagnosis

La atresia de coana es una rara malformación congénita improbable de encontrar de forma bilateral en un adolescente o adulto. Hasta la fecha, no se ha descrito ningún caso de atresia bilateral en un adulto con una malformación asociada de cabeza y cuello que haya requerido tratamiento conjunto. El tratamiento de elección de la atresia de coana bilateral continúa siendo la cirugía endoscópica, con controversia en el uso intraoperatorio de mitomicina o la colocación de stents para evitar estenosis. Lo que no está claro es el orden de tratamiento y la simultaneidad del procedimiento si se asocian otras posibles patologías que tengan una indicación quirúrgica. Presentamos un caso clínico que cumple con todos estos requisitos.

Choanal atresia is a rare congenital malformation that is unlikely to be found bilaterally and is seldom diagnosed in adulthood. To date, no clinical case of bilateral atresia has been described with a head and neck malformation that requires surgical treatment in an adult. The preferred treatment is still endoscopic sinonasal surgery with discrepancies of the use or not of intraoperative topical mitomycin or the placement of stents to avoid restenosis. What is not clear is the order of treatment and simultaneity of the procedure with other possible associated pathologies that have a surgical indication. We present a clinical case that meets all these requirements.

Evaluación del tratamiento de linfangiomas con bleomicina en población pediátrica de 0 a 18 años / Evaluation of the treatment of lymphangiomas with bleomycin in a pediatric population aged 0 to 18 years

INTRODUCCIÓN. Los linfangiomas son una malformación infrecuente a escala mundial y constituye una preocupación para los padres del infante; está asociado a problemas estéticos y a posibles efectos deletéreos debido a la obstrucción o compresión de órganos vitales. Se ha descrito a la escleroterapia como la mejor opción de tratamiento. OBJETIVO. Determinar la eficiencia del tratamiento con bleomicina en linfangiomas en la población pediátrica de 0 a 18 años. MATERIALES Y MÉTODOS. Estudio transversal analítico retrospectivo. Población y muestra conocida de 20 datos de Historias Clínicas electrónicas de pacientes diagnosticados con linfangiomas y tratados con bleomicina en el Hospital de Especialidades Carlos Andrade Marín, desde enero 2015 a enero 2018. Criterios de inclusión: pacientes de 0 a 18 años de edad con diagnóstico de linfangioma mediante ecografía y angiotomografía computarizada. Criterios de exclusión: pacientes mayores de 18 años de edad o sospecha diagnóstica de linfangioma sin estudios de imagen, y/o que no acudieron a la cita de control, pacientes diagnosticados de linfangioma que no recibieron bleomicina para su tratamiento, niños con otras malformaciones vasculares. El análisis de datos se realizó en el programa estadístico International Business Machines Statistical Package for the Social Sciences. RESULTADOS. La mediana de edad en mujeres fue de 6,25 años y 3,8 años en hombres. 10 pacientes fueron hombres. El promedio de seguimiento fue de 26,86 +/- 16,78 meses. El tamaño promedio de los linfangiomas fue de 5,77 +/- 3,73 cm. La localización más frecuente fue cervical con un 52,38%. La mayoría fueron macronodulares con un 85,71%. La respuesta fue buena o excelente en el 81,00% de los casos con la primera infiltración y subió al 95,00% con la segunda y tercera, según requerimiento. CONCLUSIÓN. El tratamiento de los linfangiomas con bleomicina fue muy efectivo en la población estudiada.

INTRODUCTION. Lymphatic malformation is a rare malformation worldwide and is a concern for the parents of the infant; it is associated with aesthetic problems and possible deleterious effects due to obstruction or compression of vital organs. Sclerotherapy has been described as the best treatment option. OBJECTIVE. To determine the efficiency of bleomycin treatment in lymphangiomas in the pediatric population aged 0 to 18 years. MATERIALS AND METHODS. Retrospective analytical cross-sectional study. Population and known sample of 20 data from Electronic Medical Records of patients diagnosed with lymphangiomas and treated with bleomycin at the Carlos Andrade Marín Specialties Hospital, from January 2015 to January 2018. Inclusion criteria: patients aged 0 to 18 years with diagnosis of lymphangioma by ultrasound and computed angiotomography. Exclusion criteria: patients older than 18 years of age or diagnostic suspicion of lymphangioma without imaging studies, and/or who did not attend the control appointment, patients diagnosed with lymphangioma who did not receive bleomycin for treatment, children with other vascular malformations. Data analysis was performed in the statistical program International Business Machines Statistical Package for the Social Sciences. RESULTS. The median age in women was 6,25 years and 3,8 years in men. Ten patients were men. The average follow-up was 26,86 +/- 16,78 months. The average size of the lymphatic malformations was 5,77 +/- 3,73 cm. The most frequent location was cervical with 52,38%. Most were macronodular with 85,71%. The response was good or excellent in 81,00% of cases with the first infiltration and rose to 95,00% with the second and third, as required. CONCLUSION. The treatment of lymphangiomas with bleomycin was very effective in the population studied.

Enfermedad inflamatoria orbitaria idiopática. Presentación de un caso / Idiopathic orbital inflammatory disease. Case presentation

RESUMEN El pseudotumor orbitario es la tercera causa de oftalmoplejía dolorosa; constituye un desafío diagnóstico pues obliga a descartar enfermedades de etiología muy diversa que lo provocan. Se presenta el caso de una paciente femenina, de color de piel blanca, de procedencia urbana, ama de casa, fumadora, que no practica ejercicios ni lleva dieta, con antecedentes de hipertensión arterial y artritis reumatoidea, que acudió al Servicio de Oftalmología por presentar síntomas inflamatorios anexiales: edema palpebral, quemosis conjuntival, vasos conjuntivales dilatados, que se acompañaban de proptosis, oftalmoplejía dolorosa con diplopía y de una masa tumoral palpable a nivel de la porción supero-externa de la órbita. Se ingresó y se le realizaron estudios imagenológicos e histológicos, que solo aportaron signos inflamatorios y una pansinusitis. Se concluyó como un pseudotumor orbitario en su forma aguda de aparición, asociada a la artritis reumatoidea, cuyo diagnóstico se realizó por exclusión sobre la base de los resultados negativos de los estudios imagenológicos y de la biopsia. Llevó tratamiento con antibióticos parenterales y altas dosis de esteroides orales con regresión del cuadro y mejoría clínica. Se decidió la presentación del caso porque en la provincia no existen reportes sobre esta entidad, y por el énfasis en cómo llegar al diagnóstico y su correcto manejo, evitando con ello secuelas en el órgano visual.

ABSTRACT Orbital pseudotumor is the third cause of painful ophthalmoplegia; it constitutes a diagnostic challenge as it forces us to rule out diseases of very diverse etiology that cause it. A female patient, of white skin color, of urban origin, housewife, smoker, who does not practice exercises or is not on a diet, with a history of high blood pressure and rheumatoid arthritis, who attended the Ophthalmology service for presenting Adnexal inflammatory symptoms: eyelid edema, conjunctival chemosis, dilated conjunctival vessels, which were accompanied by proptosis, painful ophthalmoplegia with diplopia and a palpable tumor mass at the level of the superior-external portion of the orbit was presented. She was admitted and imaging and histological studies were performed, which only revealed inflammatory signs and pansinusitis. It was concluded as an orbital pseudotumor in its acute onset form, associated with rheumatoid arthritis, the diagnosis of which was made by exclusion on the basis of the negative results of imaging studies and biopsy. She was treated with parenteral antibiotics and high doses of oral steroids with regression of the symptoms and clinical improvement. The presentation of the case was decided because in the province there are no reports on this entity, and because of the emphasis on how to reach the diagnosis and its correct management, thereby avoiding effects in the visual organ.

Síndrome de CLOVES: tratamiento con rapamicina oral: reporte de dos casos / CLOVES syndrome: treatment with oral rapamycin: report of two cases

INTRODUCCIÓN: El síndrome de CLOVES se caracteriza por sobrecrecimiento lipomatoso asociado a malformaciones vasculares, representando un desafío diagnóstico y terapéutico. La rapamicina, un inhibidor de la vía mTOR, ha demostrado ser una buena alternativa terapéutica en un grupo de anomalías vasculares. Reportamos dos casos de síndrome de CLOVES con buena respuesta al tratamiento con rapamicina oral. OBJETIVO: Reportar la experiencia del uso de rapamicina oral en el tratamiento de dos pacientes con síndrome de CLOVES. CASOS CLÍNICOS: Caso 1: preescolar femenino de tres años de edad con sín drome de CLOVES e historia de hospitalizaciones reiteradas por infección severa de malformaciones linfáticas macroquísticas y episodios trombóticos. Evoluciona con mala calidad de vida, múltiples hospitalizaciones, riesgo quirúrgico y progresión de las lesiones, por lo que se indicó rapamicina oral. A los 6 meses de tratamiento se evidenció reducción clínica y radiológica del tamaño de las masas lipomatosas y linfáticas, ausencia de linforrea cutánea y mejoría significativa de la calidad de vida, sin requerir nuevas hospitalizaciones. Caso 2: escolar femenino de diez años de edad, portadora de síndrome de CLOVES, que desarrolló escoliosis y deterioro de su capacidad motora, haciéndose dependiente del uso de silla de ruedas. Se indicó rapamicina oral, evidenciándose a los cuatro meses de tratamiento mejoría en su capacidad física, independencia y autovalencia, con desaparición de la linforrea. CONCLUSIÓN: Proponemos la rapamicina oral para el tratamiento de pacientes con sín drome de CLOVES que presenten complicaciones y deterioro de la calidad de vida producto de su enfermedad.

INTRODUCTION: CLOVES syndrome is characterized by lipomatous overgrowth associated with vascular malforma tions, representing a diagnostic and a therapeutic challenge. Rapamycin, an mTOR inhibitor, has proved to be a good therapeutic option in some vascular anomalies. In this article, we report two ca ses of CLOVES syndrome with good response to oral rapamycin treatment. OBJECTIVE: To report the outcome of two patients with CLOVES syndrome treated with oral rapamycin. CLINICAL CASES: Case 1: A three-year-old female preschooler with CLOVES syndrome and history of repeated hospita lizations due to severe infections resulting from macrocystic lymphatic malformations and due to thrombotic episodes. The patient evolved with poor quality of life, multiple hospitalizations, surgical risk and progression of the lesions, therefore, oral rapamycin was indicated. After six months of treatment, clinical and radiological reduction in the size of the lipomatous and lymphatic masses, cutaneous lymphorrhea absence and a significant improvement of her quality of life were observed, without requiring new hospitalizations. Case 2: a ten-year-old female schooler with CLOVES syndro me, who developed scoliosis and deterioration of her motor skills, becoming wheelchair-dependent. Oral rapamycin was indicated, showing improvement in her physical capacity, independence and au tonomy, and absence of lymphorrhea after four months of treatment. CONCLUSION: We propose oral rapamycin for the treatment of patients with CLOVES syndrome who present with complications and deterioration in the quality of life as a result of the disease.

Síndrome POEMS: um diagnóstico desafiador / POEMS Syndrome: a challenging diagnosis

A síndrome POEMS é um distúrbio multissistêmico. Sua patogênese não está totalmente estabelecida, mas sabe-se que tem relação com fator de crescimento vascular endotelial, interleucinas e fator de necrose tumoral alfa. A idade média de incidência é 50 anos, com maior prevalência em homens. Neuropatia periférica e gamopatia monoclonal estão presentes em todos os pacientes e são consideradas critérios maiores; quando associadas a pelo menos um critério menor, estabelecem diagnóstico da síndrome. As opções de tratamento são radioterapia, corticosteroides e quimioterapia, além de transplante autólogo de células-tronco hematopoiéticas. (AU)

POEMS syndrome is a multisystem disorder. Its pathogenesis isn't fully established, but it is known to be related to endothelial vascular growth factor, interleukins, and tumoral necrosis factor alpha (TNF-α). The mean age at incidence is 50 years, with a higher prevalence in men. Peripheral neuropathy and monoclonal gammopathy are present in all patients, and are considered major criteria; when associated with at least one minor criterium, they establish the diagnosis of the syndrome. Treatment options are radiotherapy, corticosteroids, chemotherapy, as well as autologous hematopoietic stem cell transplantation. (AU)

Mycophenolate mofetil as an alternative treatment for autoimmune hepatitis

Autoimmune hepatitis (AIH) is an immune-mediated chronic liver disease characterized by hepatocellular inflammation, necrosis, and fibrosis, which can progress to cirrhosis and fulminant hepatic failure. The standard treatment for AIH includes corticosteroids alone or in combination with azathioprine. Although most patients achieve remission using the standard regimen, some patients do not respond due to either drug intolerance or refractory disease; in such cases alternative immunosuppressive agents should be explored. The second-line therapies are cyclophilin inhibitors such as cyclosporine A or tacrolimus, and nowadays mycophenolate mofetil (MMF) is widely used if azathioprine-based therapies are not tolerated. Although these are recommended as an alternative to the first-line regimen, there is insufficient evidence for the efficacy of second-line therapies, with the evidence based mainly on expert opinion. Therefore, we report an AIH patient receiving the standard regimen in whom remission did not occur due to side effects to azathioprine, but was successfully treated with MMF in combination with corticosteroids as an alternative to the standard regimen.

Corneal melanosis successfully treated using topical mitomycin-C and alcohol corneal epitheliectomy: a 3-year follow-up case report / Tratamento com êxito da melanose de córnea utilizando mitomicina-C tópica e epiteliectomia auxiliada por álcool: relato de caso com três anos de seguimento

ABSTRACTWe report a case of primary acquired corneal melanosis without atypia associated with corneal haze in a patient with a history of limbal malignant melanoma and the effect of mitomycin-C. A 75-year-old woman with a history of limbal malignant melanoma presented with loss of vision in right eye. Corneal examination showed a patchy melanotic pigmentation with a central haze. Topical mitomycin-C improved visual acuity and corneal haze. However, the pigmented lesions persisted, and they were removed with alcohol corneal epitheliectomy. Histopathological examination demonstrated primary acquired melanosis without atypia. The lesions were successfully removed, and there were no recurrences during the follow-up period of 36 months. The association of conjunctival and corneal melanosis without atypia is a rare condition. In addition, co-existence of central corneal haze and melanosis may decrease visual acuity. Topical mitomycin-C and alcohol corneal epitheliectomy can be useful treatments in this condition.

RESUMORelatar um caso de melanose adquirida primária de córnea sem atipia, associado a haze corneano em um paciente com história de melanoma maligno de limbo e o efeito da mitomicina-C. Uma mulher de 75 anos de idade, com história de melanoma maligno do limbo apresentado com diminuição de visão no olho direito. O exame de córnea mostrou uma pigmentação melânica irregular com um haze central. O uso de mitomicina-C tópica levou à melhora da acuidade visual e da opacidade corneana. No entanto, as lesões pigmentadas persistiram e foram removidas com epiteliectomia associada ao álcool. O exame histopatológico demonstrou melanose adquirida primária sem atipia. As lesões foram removidas com êxito, e não houve recidiva durante o período de acompanhamento de 36 meses. A associação melanose sem atipia da conjuntiva e da córnea é uma condição rara. Além disso, a coexistência de haze corneano central e melanose pode diminuir a acuidade visual. O uso de mitomicina-C tópica e epiteliectomia corneana auxiliada pelo álcool podem ser tratamentos úteis nessa situação.

CIAPIN1 gene silencing enhances chemosensitivity in a drug-resistant animal model in vivo

Overexpression of cytokine-induced apoptosis inhibitor 1 (CIAPIN1) contributes to multidrug resistance (MDR) in breast cancer. This study aimed to evaluate the potential of CIAPIN1 gene silencing by RNA interference (RNAi) as a treatment for drug-resistant breast cancer and to investigate the effect of CIAPIN1 on the drug resistance of breast cancer in vivo. We used lentivirus-vector-based RNAi to knock down CIAPIN1 in nude mice bearing MDR breast cancer tumors and found that lentivirus-vector-mediated silencing of CIAPIN1 could efficiently and significantly inhibit tumor growth when combined with chemotherapy in vivo. Furthermore, Western blot analysis showed that both CIAPIN1 and P-glycoprotein expression were efficiently downregulated, and P53 was upregulated, after RNAi. Therefore, we concluded that lentivirus-vector-mediated RNAi targeting of CIAPIN1 is a potential approach to reverse MDR of breast cancer. In addition, CIAPIN1 may participate in MDR of breast cancer by regulating P-glycoprotein and P53 expression.

Disappearance of Intrahepatic Bile Duct Hepatocellular Carcinoma after Endoscopic Retrograde Cholangiopancreatography and Transarterial Chemoinfusion: A Case Report / 대한소화기학회지

Invasion of the bile duct by hepatocellular carcinoma (HCC), which is called intrahepatic bile duct HCC, is rare and has a poor prognosis. Early diagnosis and surgical resection is important for treatment. A 58-year-old man who underwent hepatic resection for HCC 4 years ago and received transarterial chemoembolization (TACE) 2 years after the operation for recurred HCC presented with jaundice. CT scan revealed a tumor in the common bile duct without intrahepatic lesion. Therefore, ERCP was done to perform biopsy and biliary drainage. Histological examination was compatible with hepatocellular carcinoma. However, the tumor could not be visualized at angiography and thus, only transarterial chemoinfusion was performed without embolization. The tumor had disappeared on follow-up CT scan, and the patient has been disease free for 23 months without evidence of recurrence. Herein, we report a case of intrahepatic bile duct HCC which disappeared after ERCP.

Sirolimus (rapamicina) en pacientes con hemangioendotelioma kaposiforme: caso clínico / Sirolimus (rapamycin) in patients with kaposiform hemangioendothelioma: clinical case

Introducción: El hemangioendotelioma kaposiforme (HEK) es un tumor vascular poco frecuente, que se asocia a coagulopatía grave, conocida como fenómeno de Kasabach-Merritt (FKM). Puede ser letal en 20 por ciento de los casos. Se han descrito múltiples tratamientos, pero a pesar de las complicaciones potencialmente severas que pueden presentar estos pacientes, no hay hasta ahora un protocolo único. Existen experiencias internacionales, en modelos experimentales, con el uso de Sirolimus (SRL), macrólido, inhibidor de mTOR, con actividad antiangiogénica. Sin embargo, la experiencia de su uso en niños en el tratamiento de anomalías vasculares como el HEK es limitada. Objetivo: Reportar la evolución de dos pacientes con HEK y FKM tratados con SRL en centros oncológicos, después de múltiples fallas con otras terapias. Casos clínicos: Reportamos dos recién nacidos con HEK profundo asociado a FKM, refractarios a terapias habituales y que evolucionaron con complicaciones amenazantes de la vida, en quienes se inicio tratamiento con SRL. El uso de SRL permitió que los dos pacientes mostraran mejoría en su estado clínico, con normalización del recuento de plaquetas y de las pruebas de coagulación, disminución del tamaño de la lesión, del dolor asociado, desaparición del riesgo vital y sin efectos adversos. Conclusión: SRL parece ser efectivo y seguro en el tratamiento de HEK, representando una nueva opción terapéutica, disponible en nuestro medio.

Introduction: Kaposiform Hemangioendothelioma (KHE) is a rare vascular tumor, which is associated with the severe coagulopathy known as Kasabach-Merritt phenomenon (KMP). This condition has a mortality rate around 20 percent. Many treatments have been described, but despite the potentially severe complications that these patients may present, so far there is not a single protocol to follow. International experimental models have used Sirolimus (SRL), a macrolide mTOR inhibitor with antiangiogenic effect. However, its use in children against vascular anomalies, such as KHE, is limited. Objective: To report the development of two patients with KHE and KMP treated with SRL in cancer centers, after multiple failures with other therapies. Case reports: Two infants with severe KHE associated to FKM, resistant to standard therapies and who overcame life-threatening complications after treatment with SRL are presented. After SRL, the two patients showed normal platelet counts and coagulation tests, reduced lesion size and associated pain, no presence of life threatening conditions or side effects. Conclusion: SRL appears to be effective and safe in treating KHE, representing a new available therapeutic option.

Embryonal Rhabdomyosarcoma Arising from a Mediastinal Teratoma: An Unusual Case Report

We report an unusual case of 9.5-cm-sized embryonal rhabdomyosarcoma arose from a mediastinal mature teratoma in a 46-yr-old man. A man presented with chest trauma as a result of an accident at 10 September 2011. On chest X-ray, an anterior mediastinal mass was detected. To obtain further information, chest computed tomography (CT) with contrast enhancement was performed, revealing an anterior mediastinal mass. Complete surgical excision was performed and entire specimen was evaluated. Pathologic diagnosis was embryonal rhabdomyosarcoma arising in mature cystic teratoma. After surgical excision, two cycles of dactinomycin-based chemotherapy were performed. Lung metastasis was detected on follow up CT in September 2012, and wedge resection was performed. Pathological finding of the lung lesion showed same feature with that of primary rhabdomyosarcoma.

Melanose primária adquirida associada a erosões epiteliais recorrentes da córnea / Primary acquired melanosis associated with recurrent corneal erosions

Relato de caso de melanose primária adquirida unilateral em paciente do sexo feminino, de 59 anos, com cerca de quatro anos de evolução, associada a episódios de erosões recorrentes do epitélio corneano. A paciente foi submetida à exerese cirúrgica da conjuntiva alterada, ceratectomia superficial e uso de mitomicina-C tópica no pós-operatório. O exame anatomopatológico confirmou o diagnóstico de melanose primária adquirida, com atipias nucleares discretas a moderadas. Um ano após a cirurgia, a paciente não apresentou novos episódios de erosões recorrentes do epitélio corneano, sendo mantida apenas com o uso noturno de pomada lubrificante.

A case report of a 59 years old woman who has had ocular primary melanosis for four years, associated with episodes of corneal epithelial recurrent erosions. The patient underwent resection of the affected conjunctiva, superficial keratectomy and topical mitomicin C after the surgery. The pathologic examination confirmed the diagnosis of primary acquired melanosis, with mild to moderate nuclear atypia. One year after the treatment, she had only a small area of pigmented epithelium located in the lower corneal surface, showing no tendency to progression. She used lubricant ointment at night and did not have other epithelial erosions during the follow-up period.

Effects of dorsal root ganglion destruction by adriamycin in patients with postherpetic neuralgia / Efeitos da destruição da raiz dorsal ganglionar pela adriamicina em pacientes com neuralgia pós-herpética

PURPOSE: To investigate the effects of dorsal root ganglion destruction in patients with postherpetic neuralgia (PHN). METHODS: Seventy-two patients with PHN selected were randomly divided into two groups (n=36). Group A was the control group (treated by injection) and group B was the group of dorsal root ganglion destruction by adriamycin. Visual analog scale scores (VAS), SAS, SF-MPQ scores. Clinical effects and therapy safety were evaluated before therapy, one week, three and six months after therapy. Forty-four patients were available for intention-to-treat analysis. RESULTS: The average pain scores on the Likert scale were significantly reduced at each point in group B. Patients in group B reported clinical effectiveness at six months as excellent response, good response, improved but unsatisfactory or unchanged 16, 12 and 8.VAS scores at each time point after the operation were lower than that before operation and in group A, there was significant difference. Patients showed significant improvement in sleep scores in group B. There was significant difference at T2 in group A than T1. There was no significant difference in group A at T3, T4 after the operation than that before operation. Between group comparison: there was significant difference between group A and group B at each time point after the operation. CONCLUSIONS: Dorsal root ganglion destruction by adriamycin under guidance of C-arm perspective, the puncture operation was accurate without any adverse reaction or serious complications, which could effectively relieve pain of patients with postherpetic neuralgia, but the long-term effects needed further study.

OBJETIVO: Investigar os efeitos da destruição da raiz dorsal ganglionar em pacientes com neuralgia pós-herpética. MÉTODOS: Setenta e dois pacientes selecionados com neuralgia pós-herpética foram randomicamente distribuídos em dois grupos (n=36). Grupo A foi o grupo controle (tratado por injeção) e o grupo B foi o grupo com destruição da raiz dorsal do gânglio pela adriamicina. Os escores da Escala Analógica Visual (VAS), SAS, SF-MPQ escores, efeitos clínicos e segurança terapêutica foram avaliados as antes da terapia, uma semana, três e seis meses após a terapia. Quarenta e quatro pacientes foram avaliados pela análise de intenção-em-tratar. RESULTADOS: A média dos escores de dor na escala de Likert foi significativamente reduzida em cada ponto no grupo B. Pacientes no grupo B relataram efetividade clínica aos seis meses com excelente resposta (16), boa resposta (12), melhora mais insatisfatória ou sem modificações (8). Escores VAS a cada tempo após o procedimento foram melhores em comparação ao pré-operatório. No grupo A não foi observada diferença significativa. Pacientes mostraram melhora nos escores de dormir no grupo B. Houve diferença significante no T2 no grupo A que T1. Não houve diferença significante no grupo A nos tempos T3 e T4 após a cirurgia em relação a antes. Comparação entre os grupos: houve diferença significante entre os grupos A e B a cada tempo após a cirurgia. CONCLUSÕES: A destruição da raiz dorsal ganglionar pela adriamicina sob perspectiva guiada pelo C-arm, a cirurgia pontual foi acurada sem qualquer reação adversa ou complicação séria, que pode efetivamente aliviar a dor em pacientes com neuralgia pós-herpética, mas os efeitos de longo prazo necessitam mais estudos.

Eficácia do uso intraoperatório de mitomicina C na cirurgia do pterígio: análise de 102 casos / Eficacy of intraoperative use of mitomycin C pterygium surgery: analysis of 102 cases

OBJETIVO: Avaliar a eficácia e a segurança do uso intraoperatório da mitomicina C (MMC) no tratamento cirúrgico do pterígio. MÉTODOS: Estudo de 102 pacientes (204 olhos) divididos em dois grupos, e submetidos a exérese do pterígio com rotação de retalho conjuntival e aplicação intra-operatória de MMC 0,02% por 2 minutos e a mesma técnica utilizando solução salina a 0,9% (grupo controle). Os pacientes foram avaliados no 1º, 7 º, 21º, 30º, 60º e 90º dia de pós-operatório (PO). RESULTADOS: Houve um maior acometimento do pterígio no sexo feminino (59,9%). A idade variou entre 33 a 69 anos. As recidivas (9) no no grupo MMC foram identificadas no 90º PO. No grupo controle foi identificado 18 recidivas em 60 dias de pós-operatório. As complicações identificadas no grupo da MMC foram: ceratite, edema de pálpebra, deiscência de sutura, isquemia, granuloma, corneal dellen e simbléfaro. CONCLUSÃO: O uso da MMC foi útil na redução da taxa de recidiva do pterígio, comparada ao uso de solução salina, porém seus efeitos deletérios devem sempre ser avaliados.

PURPOSE: Measure of efficacy and the security of intraoperative of mitomycin C (MMC) in the pterygium surgery. METHODS: Realized prospective analysis of 102 patients (204 eyes) divided in two groups submitted intraoperative MMC 0.02 % for 2 minutes or saline solution 0.9%. The patients were available in 1º, 7 º, 21º, 30º, 60º and 90º days of postoperative. RESULTS: The pterygium was more prevalence in females (58,33%). The age various 33 to 69 years. The recidive (9) was in the 90º PO in the group of MMC. In the control group was identificated two recidives in 60 days. The complications found in MMC group were: ceratitis, lips edema, dehiscence of suture, ischemia, granuloma, corneal dellen and simblefaro. CONCLUSION: The use of MMC was useful in reducing the rate of recurrence in pterygium compared to the use of saline solution, but its harmful effects must always be evaluated.

Miliaria-rash after neutropenic fever and induction chemotherapy for acute myelogenous leukemia / Miliária 'rash' após neutropenia febril e quimioterapia de indução para a leucemia mielóide aguda

Miliaria is a disorder of the eccrine sweat glands which occurs in conditions of increased heat and humidity. It can be associated with persistent febrile states as well as with certain drugs. We presented a 40 year-old female with myelodysplastic syndrome and progression to acute myelogenous leukemia who was admitted to the hospital for chemotherapy induction. The patient was treated with idarubicin and cytarabine. She became pancytopenic and developed neutropenic fever and was started on vancomycin and cefepime, but was persistently febrile with night sweats. Five days into her fevers, she developed diffuse, nonpruritic and fragile vesicles together with drenching nightsweats. The patient's exanthem was diagnosed as Miliaria crystallina, most probably induced by neutropenic fever and idarubucin exposure.

Miliária é uma desordem das glândulas sudoríparas écrinas, que ocorre em condições de aumento de calor e umidade. Miliária pode ser associada com estados febris persistentes bem como com certos medicamentos. Apresentamos o caso de uma mulher de 40 anos com síndrome mielodisplásica e progressão para leucemia mielóide aguda que foi admitida no hospital para quimioterapia de indução. A paciente foi tratada com idarrubicina e citarabina. Ela se tornou pancitopênica e desenvolveu neutropenia febril. Iniciou tratamento com vancomicina e cefepime, mas a febre com sudorese noturna continou. Cinco dias depois a paciente desenvolveu vesículas difusas, não pruríticas e frágeis juntamente com a persistência de sudorese noturna. O exantema do paciente foi diagnosticado como Miliária cristalina, provavelmente induzida por neutropenia febril e exposição a idarubucin.

Uso profilático de Mitomicina C para inibir a formação de 'haze' após ceratectomia fotorrefrativa em miopias altas resultados após dois anos / Prophylactic use of Mitomycin C to inhibit haze formation after photorefractive keratectomy in high myopia results after two years

OBJETIVO: Avaliar os resultados a longo prazo da aplicação profilática da Mitomicina C para inibir a formação de opacidade corneana em ceratectomia fotorrefrativa (PRK) em pacientes com miopia alta. MÉTODOS: Estudo retrospectivo, descritivo, longitudinal que compreendeu 59 olhos (30 pacientes) que realizaram cirurgia de PRK com aplicação de Mitomicina C entre janeiro e março de 2008. Os critérios de inclusão dos pacientes foram: equivalente esférico maior do que -4,00 dioptrias, espessura corneana maior do que 480 µm, ausência de cirurgia prévia, de trauma ocular e de enfermidades oculares e sistêmicas. Após realização do PRK, os olhos foram tratados com micro esponja embebida com MMC 0.02 por cento por 20 segundos sobre a área da ablação. Foram realizadas medidas de refração, acuidade visual sem correção, acuidade visual corrigida e observação da opacidade corneana através da lâmpada de fenda nos dois anos de seguimento. RESULTADOS: No pós-operatório, não foram observados efeitos adversos imediatos ou tardios da MMC. Não foi observada opacidade corneana em nenhum olho após dois anos do ato cirúrgico. Em um olho foi observada opacidade grau 2,0 do terceiro até o sexto mês de seguimento. Nos demais olhos, não houve opacidade maior do que 1,0. A acuidade visual não corrigida foi de 20/40 ou melhor em 96,91 por cento dos olhos e de 20/20 em 81,35 por cento dos olhos. A acuidade visual corrigida manteve-se em 57 olhos e houve ganho de uma linha em 2 olhos. CONCLUSÃO: O uso profilático da Mitomicina C a 0,02 por cento após PRK pareceu mostrar-se eficaz na redução de formação de opacidade ocular.

PURPOSE: to evaluate the long-term results of the prophylactic use of Mitomycin C in inhibiting haze formation after photorefractive keratectomy (PRK) for high myopia. METHODS: This retrospective, descriptive and longitudinal study comprised 59 eyes (30 patients). The inclusion criteria were a spherical equivalent correction over -4,00 diopters, corneal thickness greater than 480 µm, no prior surgery, ocular trauma and ocular and systemic diseases. After PRK, the eyes were treated with a single intraoperative dose of MMC 0.02 percent applied topically with a soaked micro sponge placed over the ablated area for two minutes. Refraction, uncorrected visual acuity, best corrected visual acuity and slit lamp evidence of corneal opacity (haze) were performed on the two years of follow up. RESULTS: No toxic or side effects, either immediate or delayed, were encountered postoperatively. Haze was not observed in any eye after two years of surgery. In one eye haze rate 2,00 was observed from the third until the sixth month of follow-up. In the other eyes, no haze rate higher than 1.0 was observed. The uncorrected visual acuity was 20/40 or better in 96.91 percent of the eyes and 20/20 in 81.35 percent of the eyes. Best corrected visual acuity was unchanged in 57 eyes and improved one line in 2 eyes. CONCLUSION: The prophylactic use of mitomycin C 0.02 percent after PRK suggested effectiveness in reducing haze formation.

Complicações pós-operatórias precoces de trabeculectomia com mitomicina, em pacientes portadores de glaucoma primário de ângulo aberto / Precocious postoperative complications of mitomicina trabeculectomy, in carrying patients with primary open angle glaucoma

OBJETIVO: Avaliar as complicações precoces encontradas em cirurgias de trabeculectomia com uso de mitomicina, realizadas em serviço-escola de residência médica, visando melhor aperfeiçoamento na técnica e acompanhamento pós-operatório dos pacientes. MÉTODOS: Estudo retrospectivo por meio de análise de prontuários. Os pacientes foram avaliados quanto ao sexo, idade e raça. Foram incluídos todos os pacientes portadores de glaucoma primário de ângulo aberto e submetidos à trabeculectomia com mitomicina. Um total de 43 pacientes foram analisados, 25 eram do sexo masculino, 18 do sexo feminino; a idade variou de 51 a 82 anos (média 68 anos) e 19 eram negros. Os pacientes eram avaliados no 1º e 7º dia de pós-operatórios, ou se necessário antes da 1ª semana. As complicações foram registradas e constituíam de: vazamento da bolha; câmara anterior rasa; atalamia; h; Descolamento de coróide. RESULTADOS: A média de idade foi de 68 anos, 58,1 por cento dos pacientes (25 no total) eram do sexo masculino, 44,1 por cento eram negros. O vazamento da bolha foi a intercorrência mais frequente, representando 25,56 por cento das cirurgias e 36,66 por cento de todas as complicações (11 pacientes). Câmara anterior rasa foi observada em 17,5 por cento das cirurgias e 22,5 por cento das complicações (sete pacientes). Descolamento de coróide esteve presente em 14,6 por cento dos pacientes e 19,3 por cento das complicações na 1ª semana (sete pacientes). Atalamia foi encontrada em três pacientes, representou de 6,97 por cento dos pacientes e 9,67 por cento das complicações. Hifema também foi observado em três pacientes, sendo todos eles encontrados no pós-operatório imediato. CONCLUSÃO: A frequência de complicações encontrados neste trabalho está dentro dos limites observados na literatura. Quantificar o percentual de complicações pós-operatórias durante a primeira semana é de fundamental importância para melhorias na técnica intraoperatória e padronização das condutas...

OBJECTIVE: To evaluate the joined precocious complications in surgeries of mitomycin trabeculectomy, carried through in residence service-school, aiming at better perfectioning in the technique and postoperative accompaniment of the patients. METHODS: Retrospective study by means of record analysis. The patients evaluated amount sex, age and race. Was included patients with primary of angle open glaucoma and submitted the mitomycin trabeculectomy. A total of 43 patients had been analyzed, 25 were male and 18 female; 19 were black, and the age ranged of 51 the 82 years (average 68 years). The patients were evaluated in first and seventh postoperative days, or if necessary before first week. The complications in had been registered it and were: bleb leaking, shallow anterior chamber, atalamy; hifema, choroidal detachment. RESULTS: The age average was of 68 years, 58.1 percent of the patients (25 in the total) was male and 44.1 percent were black. The Bleb leaking was the most frequent complication, representing 25.56 percent of surgeries and 36.66 percent of all the complications (11 patients). Shallow anterior chamber was observed in 17.5 percent of surgeries and 22.5 percent of complications (seven patients). Choroidal detachment was present in 14.6 percent of patients and 19.3 percent of the complications in 1a week ( seven patients). Atalamy was found in three patients, represented of 6.97 percent of patients and 9.67 percent of the complications. Hifema also was observed in three patients, being all found they in the Immediate Postoperative CONCLUSION: The frequency of complications found in this work this inside of the limits observed in literature. Quantify the percentage of postoperative complications during the first week is of fundamental importance for improvements in intra operative and standardization of the behaviors front to each complication, standardization of behavior in response to each complication, thus reducing the...

A Case of Therapy-related ALL with MLL Gene Rearrangement Following Treatment of Breast Cancer / 대한진단검사의학회지

ALL with MLL gene rearrangement secondary to chemotherapy has been rarely reported. We report a case of therapy-related ALL (t-ALL) with MLL gene rearrangement in a patient who had undergone treatment for breast cancer. A 60-yr-old woman with breast cancer underwent breast-conserving surgery followed by 6 cycles of adjuvant chemotherapy (cyclophosphamide, epirubicin, and fluorouracil) and radiation therapy (dose, 5,040 cGy to the left breast and a 1,000 cGy boost to the tumor bed). A follow-up examination performed 14 months after the chemotherapy revealed no evidence of breast malignancy. However, the patient's complete blood cell count indicated acute leukemia: white blood cell count, 174.1x10(9)/L with 88% blasts; Hb level, 12.5 g/dL; and platelet count, 103.0x10(9)/L. Examination of the bone marrow aspirate smear revealed a high percentage of blasts (85.1% of all nucleated cells); the blasts showed a pro-B immunophenotype and were positive for CD19, CD79a, HLA-DR, CD34, and terminal deoxynucleotidyl transferase (TdT). Cytogenetic and FISH analyses revealed t(4;11)(q21;q23) and MLL gene rearrangement, respectively. The patient received induction chemotherapy with cyclophosphamide, vincristine, doxorubicin, and dexamethasone and achieved complete remission. Following consolidation chemotherapy, she underwent allogenic peripheral blood stem cell transplantation and has been clinically stable. To our knowledge, this is the first reported case of t-ALL with MLL gene rearrangement following treatment of breast cancer in Korea.