Radiation-Induced Undifferentiated Pleomorphic Sarcoma after Breast Conserving Therapy: Case Report / Sarcoma Pleomórfico de Mama Radioinduzido Pós-Tratamento Conservador de Câncer de Mama Inicial: Relato de Caso / Sarcoma Pleomórfico de Mama Radioinducido tras Tratamiento Conservador de Cáncer de Mama Inicial: Informe de Caso

Introduction: Breast sarcoma is a rare form of malignancy that arises from connective tissue, comprising less than 5% of all sarcomas. Undifferentiated pleomorphic sarcoma (UPS) of the breast is a rare and aggressive subtype of radiation-induced sarcoma that can occur in treated breast cancer patients. The diagnosis is challenging and often missed due to the low incidence, long latency period, unspecific imaging finding, and difficulties in clinical and histological detection. Case report: A 56-year-old woman was diagnosed with early-stage triple-negative breast cancer in 2013 and underwent breast-conserving therapy (BCT). After five years follow-up, she developed mastalgia and breast induration, and after mammography and ultrasound without suspicious lesions, a magnetic resonance imaging (MRI) was performed and showed a highly suggestive malignancy mass measuring 8.0 cm and invading the chest wall. The core biopsy revealed a spindle cells malignant tumor, negative for pan cytokeratin and most of immuno-histochemical markers, suggesting sarcoma, but requiring investigation of surgical specimen to exclude metaplastic carcinoma. She underwent Halsted radical mastectomy, full-thickness left anterior chest wall resection contemplating segments of the 4th and 5th ribs and reconstruction with synthetic mesh. The surgical specimen evidenced a UPS with clear margins. The patient had good postoperative recovery and remains in follow-up with the mastology team. Conclusion: This report shows that radiation-induced sarcomas of the breast can be difficult to diagnose, and how later treatment can demand a major surgery with higher morbidity. Prognosis may improve if detected early

Introdução: O sarcoma de mama é uma forma rara de câncer que surge do tecido conjuntivo, compreendendo menos de 5% de todos os sarcomas. O sarcoma pleomórfico indiferenciado (SPI) da mama é um subtipo raro e agressivo de sarcoma induzido por radiação que pode surgir em pacientes pós-tratamento conservador de câncer de mama. O diagnóstico é desafiador e, muitas vezes, tardio, em virtude da baixa incidência, longo período de latência, achados de imagem inespecíficos e dificuldades na detecção clínica e histológica. Relato do caso: Paciente do sexo feminino, 56 anos, foi diagnosticada com câncer de mama triplo-negativo estadiamento inicial em 2013 e submetida a tratamento conservador de câncer de mama. Após cinco anos de seguimento, evoluiu com mastalgia e endurecimento mamário e, após mamografia e ultrassonografia sem lesões suspeitas, foi realizada ressonância magnética que evidenciou massa altamente sugestiva de malignidade medindo 8,0 cm com invasão da parede torácica. O resultado da core biopsy revelou um tumor maligno de células fusiformes, negativo para pancitoqueratina e para a maioria dos marcadores imuno-histoquímicos, sugerindo sarcoma, mas exigindo estudo de peça cirúrgica para excluir carcinoma metaplásico. A paciente foi submetida à mastectomia radical de Halsted, ressecção da parede torácica anterior esquerda contemplando segmentos das 4ª e 5ª costelas e reconstrução com tela sintética. O laudo histopatológico da peça cirúrgica evidenciou SPI com margens livres. Evoluiu com boa recuperação pós-operatória, permanecendo em acompanhamento com a equipe de mastologia. Conclusão: O relato de caso ilustra como os sarcomas de mama induzidos por radiação podem ser de difícil diagnóstico e como o tratamento postergado pode exigir uma cirurgia de grande porte com maior morbidade. Estar ciente dessa condição pode melhorar o prognóstico do paciente

Introducción: El sarcoma de mama es una forma rara de cáncer que surge del tejido conectivo y comprende menos del 5% de todos los sarcomas. El sarcoma pleomórfico indiferenciado (SPI) de la mama es un subtipo raro y agresivo de sarcoma inducido por radiación que puede surgir en pacientes después de un tratamiento conservador del cáncer de mama. El diagnóstico es un desafío y, a menudo, se retrasa debido a la baja incidencia, el largo período de latencia, los hallazgos de imagen inespecíficos y las dificultades en la detección clínica e histológica. Informe del caso: Una paciente de 56 años fue diagnosticada de cáncer de mama triple negativo en estadio inicial en 2013 y se sometió a un tratamiento conservador para el cáncer de mama. A los cinco años de seguimiento desarrolló mastalgia y endurecimiento mamario, y tras mamografía y ecografía sin lesiones sospechosas, se realizó una resonancia magnética, que mostró una masa altamente sugestiva de malignidad de 8,0 cm. con invasión de la pared torácica. El estudio anatomopatológico de la lesión mostró un tumor de células fusiformes maligno, negativo para pancitoqueratina y para la mayoría de los marcadores inmuno-histoquímicos, lo que sugería sarcoma, pero requirió un estudio de la pieza quirúrgica para descartar un carcinoma metaplásico. La paciente fue intervenida de mastectomía radical de Halsted, resección de la pared torácica anterior izquierda cubriendo segmentos de las 4ª y 5ª costillas y reconstrucción con malla sintética. El informe histopatológico de la pieza quirúrgica mostró UPS, con márgenes libres. Evolucionó con buena recuperación postoperatoria, permaneciendo en seguimiento con el equipo de mastología. Conclusión: El relato de caso ilustra cómo los sarcomas de mama inducidos por radiación pueden ser difíciles de diagnosticar y cómo el tratamiento tardío puede requerir una cirugía mayor con mayor morbilidad. Ser consciente de esta condición puede mejorar el pronóstico del paciente.Palabras clave: sarcoma; neoplasias de la mama; neoplasias inducidas por radiación; neoplasias primarias secundarias; informes de casos

Diagnóstico de cuatro neoplasias primarias sincrónicas en un adulto / Diagnosis of four synchronous primary neoplasms in an adult

Paciente femenina de 72 años. Mediante estudios de imagen (ultrasonido y tomografía), se le identificó lesión en parénquima hepático, anexo derecho, peritoneo y apéndice cecal, y mediante estudio histopatológico se determinó la presencia concomitante de carcinoma hepatocelular de células claras, tumor mucinoso limítrofe de bajo potencial maligno o borderline, pseudomixoma peritoneal y neoplasia mucinosa de bajo grado del apéndice cecal, respectivamente. Debido a que las neoplasias reportadas no guardan relación con el mismo órgano ni con el sistema, se considera que son neoplasias aparecidas al azar y de tipo sincrónico por ser diagnosticadas en el mismo espacio temporal. Se practicó laparotomía exploradora con exéresis de lesión anexial y de apéndice cecal. La lesión hepática recibió quimioembilización transarte rial por radiología intervencionista. Posterior a la intervención quirúrgica, la paciente presenta buen estado general. En seguimiento con resonancia magnética se cataloga con persistencia de lesión hepática ya tratada, por lo tanto, con enfermedad estable; se refiere a oncología clínica para valoración de quimioterapia en el manejo del pseudomixoma peritoneal. Dieciocho meses después de los diagnósticos iniciales, se documenta carcinoma basocelular y se cataloga como neoplasia metacrónica por la diferencia de tiempo entre los diagnósticos

A 72-year-old female patient with a one-year history of abdominal pain in the right upper quadrant, colicky, radiating to the back, accompanied by adynamia and weight loss. Abdominal distension and a painful mass on palpation in the right hypochondrium were evidenced. Imaging studies identified different lesions in the hepatic parenchyma, right adnexa, peritoneum and cecal appendix. The histopathological study described the presence of clear cell hepatocellular carcinoma, borderline mucinous tumor of low malignant potential or borderline, peritoneal pseudomyxoma and low-grade mucinous neoplasm of the cecal appendix respectively, as synchronous neoplasms. An exploratory laparotomy was performed with excision of the adnexal lesion and the cecal appendix. The hepatic lesion received transarterial chemoembilization by interventional radiology. Follow-up with conservative management by clinical oncology was indicated. The patient evolved with good general condition, in the follow-up with magnetic resonance imaging was classified with persistence of stable hepatic lesion. Eighteen months after the diagnosis of synchronous neoplasms, basal cell carcinoma was identified, due to the difference in the time of diagnosis this is considered a metachronous neoplasm

Segundas resecciones anatómicas en pacientes con resección anatómica previa / Second pulmonary anatomical lung resections in patients with previous anatomical resections

Resumen Introducción: Las segundas resecciones anatómicas son cada vez más frecuentes en el tratamiento de carcinomas pulmonares sincrónicos, metacrónicos y de metástasis pulmonares de origen extrapulmonar. Objetivo: Determinar si las segundas resecciones anatómicas pulmonares se asocian con un mayor riesgo de complicaciones posoperatorias comparadas con la primera intervención. Materiales y Método: Hemos analizado todos los pacientes sometidos a una segunda resección anatómica en nuestro centro entre octubre de 2000 y febrero de 2019. Las complicaciones fueron clasificadas en mayores y menores según la clasificación estandarizada de morbilidad posoperatoria de Clavien-Dindo. Se compararon las características clínicas y demográficas de los pacientes y la ocurrencia de complicaciones mayores tras la primera y la segunda intervención quirúrgica mediante la prueba T para muestras relacionadas y la prueba exacta de McNemar para las variables cuantitativas y categóricas, respectivamente. Resultados: Setenta y cinco pacientes fueron sometidos a una segunda resección anatómica. La prevalencia de complicaciones globales y mayores tras la primera intervención fue del 26,7% y el 4% frente al 34,7% y al 6,7% tras la segunda intervención (p = 0,362 y p = 0,727, respectivamente). Las segundas resecciones pulmonares ipsilaterales se asociaron con un 16,7% de complicaciones mayores y los procedimientos consistentes en completar la neumonectomía con un 25%. Conclusión: Las segundas resecciones anatómicas pulmonares no se asocian con un mayor riesgo de complicaciones posoperatorias comparadas con la primera intervención. Sin embargo, las segundas resecciones ipsilaterales y las resecciones que impliquen completar la neumonectomía se asocian con riesgo significativamente superior de complicaciones mayores posoperatorias.

Introduction: Second anatomical resections are becoming more frequent in the treatment of synchronous, metachronous and pulmonary metastases of extrapulmonary origin. Aim: The objective of this study is to determine whether second pulmonary anatomical resections are associated with an increased risk of postoperative complications compared to the first intervention. Materials and Method: We have analyzed all patients undergoing a second anatomical resection in our center between October 2000 and February 2019. Complications were classified in major and minor according to the standardized Clavien-Dindo postoperative morbidity classification. The clinical and demographic characteristics of the patients and the occurrence of major complications after the first and second surgical intervention were compared using the T test for related samples and the McNemar exact test for quantitative and categorical variables, respectively. Results: Seventy-five patients underwent a second anatomic resection. The prevalence of global and major complications after the first intervention was 26.7% and 4% compared to 34.7% and 6.7% after the second intervention (p = 0.362 and p = 0.727, respectively). Second ipsilateral lung resections were associated with 16.7% of major complications and procedures consisting of completing pneumonectomy with 25%. Conclusion: Second lung anatomical resections are not associated with an increased risk of postoperative complications compared to the first intervention. However, second ipsilateral resections and resections that involve completing pneumonectomy are associated with a significantly higher risk of major postoperative complications.

Radiation therapy with elective lymph node irradiation for breast cancer: dosimetric study and impact on cardiovascular risk and second neoplasms

SUMMARY OBJECTIVE: The aim of this study was to perform dosimetric analysis of radiotherapy (RT) plans with or without elective nodal irradiation (ENI) and estimate whether the increase in mean doses (MDs) in the heart and lungs with ENI may lead to late side effects that may surpass the benefits of treatment. METHODS: The dosimetric analysis of 30 treatment plans was done with or without ENI. The planning and dose-volume histograms were analyzed, and the impact on the mortality of cardiovascular and lung cancer was estimated based on the correlation of the dosimetric data with data from population studies. RESULTS: RT with ENI increased the doses in the lungs and heterogeneity in the plans compared to breast-exclusive RT. When the increase in MDs is correlated with the increase of late side-effect risks, the most important effect of ENI is the increased risk of lung cancer, especially in patients who smoke (average increase in absolute risk=1.38%). The increase in the absolute risk of cardiovascular diseases was below 0.1% in the all the situations analyzed. CONCLUSIONS: ENI increases the heterogeneity and the doses at the lungs. When recommending ENI, the risks and benefits must be taken into account, considering the oncology factors and the plan of each patient. Special attention must be given to patients who smoke as ENI may lead to a significant increase in MD in the lung and the increased risk of radiation-induced lung cancer may surpass the benefits from this treatment.

Metástasis pancreática metacrónica de carcinoma renal / Metachronous pancreatic metastasis from renal cell carcinoma

RESUMEN Las metástasis pancreáticas por carcinoma renal de células claras son excepcionales; en general, se presentan como tumores asintomáticos de diagnóstico incidental. Caso clínico: hombre de 61 años, nefrectomizado hace 10 años; durante el seguimiento se detecta una masa pancreática distal de 3 cm, hipervascular. Se realiza pancreatectomía corporocaudal laparoscópica, confirmándose la presencia de una metástasis de carcinoma renal. Las metástasis pancreáticas del carcinoma renal suelen apare cer en forma asintomática y metacrónica. La resección quirúrgica logra buenos resultados oncológicos y debe intentarse siempre que sea posible. En este caso destacamos que se pudo realizar una exéresis radical mediante abordaje mínimamente invasivo.

ABSTRACT Pancreatic metastases from clear cell renal carcinoma are very rare, and generally present as incidental asymptomatic tumors. Case report: a 61-year-old male patient with a history of nephrectomy 10 years before presented a 3-cm hypervascular mass in the distal pancreas during follow-up. A laparoscopic distal pancreatectomy was performed, confirming the presence of metastasis from a renal cell carci noma. Pancreatic metastases from renal cell carcinoma are usually metachronous and asymptomatic. Surgical resection is associated with favorable oncological results and, if possible, should be attempted. In our case, a successful oncological resection could be performed through a minimally invasive ap proach.

Cáncer escamoso asentado sobre sinus pilonidal recurrente: la evolución de una patología benigna y frecuente a una causa de muerte infrecuente / Squamous cancer settled on recurrent pilonidal sinus: the evolution of a benign and frequent pathology to an infrequent cause of death

El sinus pilonidal es una patología frecuente cuya malignización es infrecuente aunque su pronóstico puede ser fatal. El objetivo de esta publicación es presentar un caso de un paciente intervenido en múltiples ocasiones de escisiones de sinus pilonidal con degeneración maligna del mismo y evolución fatal, con el fin de recalcar la importancia del examen anatomopatológico sistemático de todas las muestras de escisión quirúrgica. (AU)

The pilonidal sinus is a frequent pathology whose malignization is uncommon although its prognosis can be fatal. The objective of this publication is to present a case of a patient intervened on multiple occasions of pilonidal sinus excisions with malignant degeneration and fatal evolution, in order to emphasize the importance of the systematic pathological examination of all surgical excision samples. (AU)

Distribución Topográfica de las Metástasis de Origen Desconocido / Topographic Distribution of Metastases of Unknown Origin

Introducción: La metástasis de origen desconocido es una entidad clínica relativamente común, que representa del 5% de todos los cánceres invasivos. La búsqueda del origen primario puede resultar desafiante por un patrón atípico metastásico, no obstante el adenocarcinoma y carcinomas indiferenciados representan un 75% siendo el adenocarcinoma el más complicado de determinar su sitio primario ya que sus características citológicas/histológicas generalmente no son específicas. Por esta razón surge la necesidad identificar el origen primario de las lesiones metastásica de origen desconocido en pacientes con y sin antecedentes oncológicos personales, el sexo y edad de mayor prevalencia. Métodos: Investigación observacional descriptiva, retrospectiva tomándose como universo de 100 pacientes con diagnóstico histopatológico de Carcinoma/Adenocarcinoma metastásico en el departamento de Anatomía patológica del Instituto Oncológico Nacional "Dr. Juan Tanca Marengo"­ Solca-Guayaquil en el periodo 2013-2015, con y sin antecedentes oncológicos personales. Resultados: Se obtuvo una muestra de 91 pacientes, donde la localización metastásica más frecuente fue en los ganglios cervicales (27%), seguida del hígado (13%), hueso y epiplón (9%). Además, en 69 de ellos pudo ser posible la identificación del origen primario de la lesión metastásica. Conclusión: La topografía metastásica de neoplasia primaria desconocida tales como los ganglios cervicales y el hígado son los lugares de notable predominio, siendo el ganglio cervical el lugar de biopsia por excelencia debido a su mayor accesibilidad. La metástasis de origen desconocido a pesar de ser más frecuente en el sexo femenino, tiene una gran incidencia en el grupo etario entre 61-70 años.

Introduction: The metastasis of unknown origin is clinical entity relatively common, which represents 5% of all invasive cancer. The research of the primary origin could be difficult because of its atypical pattern, instead that, the undifferentiated adenocarcinoma and carcinoma represents 75% , being adenocarcinoma the most complicated to diagnoses the primary origin because of their unspecific characteristic cytological/histological. For this reason is necessary to identify the primary origin of the metastatic lesion with unknown origin in patients with or without personal oncological background, sex and age with higher prevalence. Methods: Observational, descriptive- retrospective investigation that used the collection 100 patients with histopathologic diagnoses carcinoma/adenocarcinoma metastatic in the Anatomy Pathologic Department of ION-SOLCA period 2013-2015, with and without personal oncological background. Results: Sample of 91 patients, in 69 of them were the most frequently metastatic location was superior cervical ganglion (27%), liver (13%), bone and omentum (9%). Furthermore, 69 patients were possible to identify the primary origin of the metastatic lesion. Conclusion: The metastatic location of the neoplasm unknown primary such as superior cervical ganglion and liver are the places more common, being superior cervical ganglion with most accessibility for biopsies. The neoplasm unknown primary is more frequently in female sex and has a high incidence at the ages of 61-70 years.

Bronchial carcinoid tumors: second primary neoplasms and outcomes of surgical treatment / Tumores carcinoides brônquicos: segunda neoplasia primária e desfechos do tratamento cirúrgico

ABSTRACT Objective: To analyze determinants of prognosis in patients with bronchial carcinoid tumors treated surgically and the potential concomitance of such tumors with second primary neoplasms. Methods: This was a retrospective analysis of 51 bronchial carcinoid tumors treated surgically between 2007 and 2016. Disease-free survival (DFS) was calculated by the Kaplan-Meier method, and determinants of prognosis were evaluated. Primary neoplasms that were concomitant with the bronchial carcinoid tumors were identified by reviewing patient charts. Results: The median age was 51.2 years, 58.8% of the patients were female, and 52.9% were asymptomatic. The most common histology was typical carcinoid (in 80.4%). Five-year DFS was 89.8%. Ki-67 expression was determined in 27 patients, and five-year DFS was better among the patients in whom Ki-67 expression was ≤ 5% than among those in whom it was > 5% (100% vs. 47.6%; p = 0.01). Concomitant primary neoplasms were observed in 14 (27.4%) of the 51 cases. Among the concomitant primary neoplasms that were malignant, the most common was lung adenocarcinoma, which was observed in 3 cases. Concomitant primary neoplasms were more common in patients who were asymptomatic and in those with small tumors. Conclusions: Surgical resection is the mainstay treatment of bronchopulmonary carcinoid tumors and confers a good prognosis. Bronchial carcinoid tumors are likely to be accompanied by second primary neoplasms.

RESUMO Objetivo: Analisar os determinantes do prognóstico em pacientes com tumores carcinoides brônquicos tratados cirurgicamente e possível segunda neoplasia primária concomitante. Métodos: Trata-se de uma análise retrospectiva de 51 tumores carcinoides brônquicos tratados cirurgicamente entre 2007 e 2016. A sobrevida livre de doença (SLD) foi calculada pelo método de Kaplan-Meier, e os determinantes do prognóstico foram avaliados. As neoplasias primárias concomitantes aos tumores carcinoides brônquicos foram identificadas por meio da análise dos prontuários dos pacientes. Resultados: A mediana de idade foi de 51,2 anos, 58,8% dos pacientes eram do sexo feminino e 52,9% eram assintomáticos. A classificação histológica mais comum foi carcinoide típico (em 80,4%). A SLD em cinco anos foi de 89,8%. A expressão de Ki-67 foi determinada em 27 pacientes, e a SLD em cinco anos foi melhor nos pacientes nos quais a expressão de Ki-67 foi ≤ 5% do que naqueles nos quais a expressão de Ki-67 foi > 5% (100% vs. 47,6%; p = 0,01). Neoplasias primárias concomitantes foram observadas em 14 (27,4%) dos 51 casos. Entre as neoplasias primárias malignas concomitantes, a mais comum foi o adenocarcinoma pulmonar, observado em 3 casos. Neoplasias primárias concomitantes foram mais comuns em pacientes assintomáticos e naqueles com tumores pequenos. Conclusões: A resseção cirúrgica é o principal tratamento de tumores carcinoides broncopulmonares e propicia um bom prognóstico. É provável que tumores carcinoides brônquicos se relacionem com segunda neoplasia primária.

Metástase hepática colorretal metacrônica tem melhor prognóstico - é verdade? / Metachronous colorectal liver metastases has better prognosis - is it true?

ABSTRACT BACKGROUND: Liver metastases from colorectal cancer are an important public health problem due to the increasing incidence of colorectal cancer worldwide. Synchronous colorectal liver metastasis has been associated with worse survival, but this prognosis is controversial. OBJECTIVE: The objective of this study was to evaluate the recurrence-free survival and overall survival between groups of patients with metachronous and synchronous colorectal hepatic metastasis. METHODS: This was a retrospective analysis of medical records of patients with colorectal liver metastases seen from 2013 to 2016, divided into a metachronous and a synchronous group. The Cox regression model and the Kaplan-Meier method with log-rank test were used to compare survival between groups. RESULTS: The mean recurrence-free survival was 9.75 months and 50% at 1 year in the metachronous group and 19.73 months and 63.3% at 1 year in the synchronous group. The mean overall survival was 20.00 months and 6.2% at 3 years in the metachronous group and 30.39 months and 31.6% at 3 years in the synchronous group. Patients with metachronous hepatic metastasis presented worse overall survival in multivariate analysis. The use of biological drugs combined with chemotherapy was related to the best overall survival prognosis. CONCLUSION: Metachronous colorectal hepatic metastasis was associated with a worse prognosis for overall survival. There was no difference in recurrence-free survival between metachronous and synchronous metastases.

RESUMO CONTEXTO: As metástases hepáticas de câncer colorretal representam um importante problema de saúde pública devido à incidência crescente de câncer colorretal pelo mundo. A metástase hepática colorretal sincrônica está associada a pior sobrevida, no entanto, o pior prognóstico é assunto controverso. OBJETIVO: O objetivo do estudo foi avaliar a sobrevida livre de recorrência e a sobrevida global entre os grupos de pacientes com metástase hepática colorretal metacrônica e sincrônica. MÉTODO: Análise retrospectiva através de revisão de prontuários de pacientes com metástase hepática colorretal atendidos no período de 2013 a 2016, divididos em grupos metacrônico e sincrônico. Foram utilizados o modelo de regressão de Cox e o método de Kaplan-Meier com teste de Log-rank para comparação de sobrevida entre os grupos. RESULTADOS: A média de sobrevida livre de recorrência no grupo metacrônico foi de 9,75 meses e 50% em 1 ano, e no grupo sincrônico 19,73 meses e 63,3% em 1 ano. A média de sobrevida global no grupo metacrônico foi de 20,00 meses e 6,2% em 3 anos, e no grupo sincrônico 30,39 meses e 31,6% em 3 anos. Os pacientes com metástase hepática metacrônica apresentaram pior sobrevida global em análise multivariada. O uso de drogas biológicas associadas ao tratamento quimioterápico foi relacionado ao melhor prognóstico em sobrevida global. CONCLUSÃO: A metástase hepática colorretal metacrônica foi associada a pior prognóstico na sobrevida global. Não houve diferença na sobrevida livre de recorrência entre as metástases metacrônica e sincrônica.

Pleomorphic undifferentiated soft tissue sarcoma in patient with long standing inflammatory bowel disease

Inflammatory bowel disease (IBD) has been associated with the development of both gastrointestinal and extraintestinal malignancy. The role of therapy in the development of malignancy in IBD has been controversial. We present the case of a 40-year-old female patient with long-standing mild IBD, who developed an undifferentiated pleomorphic sarcoma of the inguinal region and provide a brief review of the relevant literature. While our case likely represents a coincidence of two unrelated pathological entities, clinicians should keep in mind the possibility of soft tissue sarcomas in patients chronically treated with anti-inflammatory agents.

Vaginal cuff recurrence after radical cystectomy: an under - studied site of bladder cancer relapse

ABSTRACT Introduction: Vaginal cuff recurrence of tumor following radical cystectomy is a rare site of disease recurrence, however it has never been specifically studied. The aim of the study is to evaluate incidence, risk factors, and long-term oncologic outcomes of vaginal cuff recur- rence in a cohort of female patients treated with radical cystectomy for invasive urothelial carcinoma of the bladder. Materials and Methods: From 1985 to 2012, a prospectively maintained institutional blad- der cancer registry was queried for vaginal cuff recurrence post radical cystectomy. Over- all mortality and cancer-specific mortality were reported using the Kaplan-Meier method for patients with vaginal cuff recurrence, recurrence at another local or distant site, and those without evidence of recurrence. Comparisons were performed using the log-rank test. Cox proportional hazards regression model was performed to assess predictors of vaginal cuff recurrence. Results: From 469 women treated with radical cystectomy for bladder cancer, 34 patients (7.3%) developed vaginal cuff recurrence, 130 patients (27.7%) had recurrence involving ei- ther a local or distant site, and 305 patients (65%) had no evidence of recurrence. The 5-year overall mortality-free survival rate was 32.4% for vaginal cuff recurrence, but 25.0% for other sites of recurrence. Cancer-specific mortality-free survival rate was 32.4% for vaginal cuff recurrence, and 30.3% for the other sites of recurrence. Multivariate Cox proportional hazards regression analysis demonstrated that the presence of tumor in posterior location at radical cystectomy (Hazard Ratio [HR], 0.353 [95% CI, 0.159-0.783]) and anterior vaginec- tomy, compared to no vaginectomy (HR, 2.595 [95% CI, 1.077-6.249]) were independently associated with vaginal cuff recurrence. Conclusion: Anterior vaginectomy, despite our best attempts, is perhaps not sufficient to prevent vaginal cuff recurrence. Therefore, follow-up evaluation is essential, and further studies are necessary to address the optimal approach for initial management. Patient Summary: Although vaginal cuff recurrence is an unusual site of recurrence, careful evaluation is needed before cystectomy and during follow-up to identify patients at risk.

Incidental Discovery of Acinic Cell Carcinoma of the Oral Mucosa with Metachronous Adenoid Cystic Carcinoma of the Upper Lip / Hallazgo Incidental de Carcinoma de Células Acinares de la Mucosa Oral con Carcinoma Adenoide Quístico Metacrónico del Labio Superior

ABSTRACT: Multiple salivary gland tumors represent an unusual event characterized by the development of composite lesions originated from minor or major salivary glands. These neoplasms can be categorized into three perspectives: Histologic type, time of appearance and topographic distribution. We report an unusual case of a 73-year-old black man with an acinic cell carcinoma (ACC) of the oral mucosa discovered incidentally during surgical removal of an adjacent mucocele. Approximately one year after the first consultation, the patient was seen at the local cancer reference center with a third lesion that was diagnosed as an adenoid cystic carcinoma (AdCC) of the upper lip. The patient underwent surgical reconstruction of the treated areas and has been free of the disease for the past year. To our knowledge, the combination of ACC and AdCC in intraoral sites has not been reported in the literature.

RESUMEN: Los tumores de glándulas salivales múltiples representan un evento inusual caracterizado por el desarrollo de lesiones compuestas, originadas en glándulas salivales menores o mayores. Estos neoplasmas se pueden categorizar en tres perspectivas: tipo histológico, tiempo de aparición y distribución topográfica. Reportamos un caso inusual de un hombre negro de 73 años con un carcinoma de célula acínica (ACC) de la mucosa oral descubierta incidentalmente durante la extirpación quirúrgica de un mucocele adyacente. Aproximadamente un año después de la primera consulta, el paciente se presentó en el centro de referencia del cáncer local con una tercera lesión que fue diagnosticada como carcinoma adenoide quístico (AdCC) del labio superior. El paciente se sometió a la reconstrucción quirúrgica de las áreas tratadas y durante el último año no ha presentado recurrencia de la enfermedad. De acuerdo a nuestro conocimiento la combinación de ACC y AdCC en sitios intraorales no se ha informado en la literatura.

Radioterapia e cardiotoxicidade / Radiotherapy and cardiotoxicity

Os avanços da radioterapia aumentaram a taxa de sobrevida dos pacientes em tratamento oncológico em até 90% em alguns casos. A prevalência acumulada de doença cardíaca relacionada com a radioterapia é estimada em 10% a 30% de 5 a 10 anos após o tratamento e é dose-dependente. A dose acumulada e seu fracionamento podem determinar os efeitos cardíacos agudos e crônicos da radioterapia. Além disso, a progressão da doença cardíaca induzida pela radiação a curto e longo prazo em cada paciente ainda não é completamente compreendida. Sabe-se que o dano cardíaco está relacionado com o dano endotelial que acomete a micro e a macrovasculatura, e o acompanhamento dos pacientes expostos deve seguir recomendações.

Advances in radiotherapy have increased the survival rate of cancer patients by up to 90% in some cases. The cumulative prevalence of radiotherapy-related heart disease is estimated at 10% to 30% five to ten years after treatment, and is dose-dependent. The accumulated dose and its fractionation may result in acute and chronic cardiac effects of radiotherapy. In addition, the progression of short- and long-term radiation-induced heart disease in each patient is still not fully understood. It is known that heart damage is related to endothelial damage, which affects the micro and macro vasculature, and the follow-up to these exposed patients should follow the recommendations

Asociación de tumorlet pulmonar con segundas neoplasias / Pulmonary tumorlet associated with second neoplasia

Los tumorlets son tumores neuroendocrinos pulmonares menores a 0.5 cm, de evolución benigna y habitualmente asintomáticos. Su diagnóstico es importante para realizar la diferenciación con otras afecciones neuroendocrinas y enfermedad metastásica de otro origen, que requerirán una intervención terapéutica. Se presenta un caso de dicha entidad asociada a otros tumores.

Tumorlets are pulmonary neuroendocrine tumors smaller than 0.5 cm. They are benign and usually asymptomatic. Their diagnosis is important so as to differentiate them from other neuroendocrine pathologies that require therapeutic intervention. We report a case of such entity and a discussion on the subject that can contribute to highlight the importance of diagnosing this entity.

A second reported malignancy in a patient with Morquio syndrome

Morquio syndrome is a rare lysosomal storage disease that affects multiple organ systems. However, it is rarely associated with malignancy. We present the case of a 30-year old man with Morquio syndrome associated with gastric adenocarcinoma. This case also demonstrates two other findings that have not been previously described in patients with Morquio syndrome - malrotation of brainstem and cerebellum, without clinical neurologic deficit, and persistence of fetal lobulation in the kidneys.

Transición a Radioterapia Tridimensional con escalada de dosis: Impacto en los Gliomas de alto grado / Transition to three-dimensional radiotherapy with dose scale: Impact on high-grade Gliomas

Introducción: La radioterapia externa con dosis altas, ha incrementado el efecto terapéutico en Gliomas Anaplásicos. El propósito de nuestro estudio fue demostrar la eficacia de la radioterapia conformada tridimensional con altas dosis en estos pacientes. Métodos: Estudio retrospectivo-prospectivo y descriptivo realizado en 45 pacientes adultos con diagnóstico de Glioma de alto grado supratentorial, índice de Karnofsky ≥ 70, sin previa quimioterapia o inmunoterapia; con exéresis quirúrgica y radioterapia postoperatoria estándar bidimensional óconformada tridimensional en el Instituto de Oncología de Cuba del 2004-2007. Se excluyeron los casos no operados. Se utilizó Kaplan Meier y regresión de Cox entre las variables. Resultados: La edad promedio fue 55 años, relación sexo hombre/mujer fue 27/18 y el Glioblastoma resultóla histología más frecuente. Predominóla exéresis subtotal del tumor y radioterapia postoperatoria con dosis entre 60-70 Gy. La supervivencia fue mayor en pacientes con mejor pronóstico, resección tumoral total y radioterapia con altas dosis. La supervivencia global fue de 13 meses y la media (Kaplan-Meier) fue significativamente mejor en los pacientes con dosis altas de radioterapia tridimensional (16 vs 9 meses) P<0.001. La supervivencia a 1 y 2 años, fue del 51 % y 28 % respectivamente en el grupo de altas dosis, asícomo del 28% y 16% respectivamente en el grupo de dosis estándar, sin evidenciarse un incremento en las toxicidades. Conclusiones: La intensificación de la radioterapia local con escalada de dosis es recomendable para los pacientes con Gliomas de alto grado y factores pronósticos favorables

Introduction: High-dose with external beam radiotherapy has been shown to enhance the radiation effect in anaplastic gliomas. The aim of this trial is to report on the efficacy of 3D conformal radiotherapy (3D-CRT) for patients with intracranial malignant gliomas. Methods: A retrospective-prospective and descriptive study was made from 45 patients with supratentorial high-grade gliomas,Karnofsky performance scale ≥ 70, no previous chemotherapy/ immunotherapy treatment, who received postoperative 2D standard or 3D-CRTat the Oncology Institute of Cuba from 2004-2007. Results: The median age of patients was 55 years, gender (M/F:27/18). Glioblastoma histology, subtotal resection, and postoperative radiotherapy (60-70Gy) were the most frequently. The survival time was much better in patients who were better prognosis, gross total resection and high-dose 3D-CRT postoperatively .The overall survival was 13 months and the estimated median survival (Kaplan-Meier) was better in high-dose group (16 vs 9 months), and the 1 and 2 yearssurvival rates were 51% & 28%, respectively for 3D-CRT group; and 28% & 16% for 2D-RT. No significant treatment toxicities were observed. Conclusions: Intensification of local radiotherapy with dose escalation is feasible for patients with high-grade gliomas and good prognostic factors.