[Bilateral pneumothorax complicating cavitary pulmonary metastases in angiosarcoma]. / Neumotórax bilateral como complicación de metástasis pulmonar cavitaria de un angiosarcoma.

Metastatic pulmonary angiosarcomas are a common complication of a rare condition. Generally, they are presented as peripheral solid nodules, infiltrates, and pleural effusions. We report the case of a 65 year-old man with bilateral recurrent pneumothorax secondary to metastatic cavitary lesions from angiosarcoma of the scalp. In this case, videothoracoscopy allowed tumor resection, inspection, and pleurodesis. There weren't complications or tumor recurrence at six months follow up.

Neumotórax bilateral como complicación de metástasis pulmonar cavitaria de un angiosarcoma / Bilateral pneumothorax complicating cavitary pulmonary metastases in angiosarcoma

Las metástasis pulmonares de angiosarcoma constituyen una complicación común de una neoplasia maligna poco frecuente. Habitualmente se presentan como nódulos solidos periféricos y derrame pleural. Presentamos el caso de un hombre de 65 años de edad con neumotórax bilateral recurrente, secundario a metástasis cavitadas de un angiosarcoma primitivo de cuero cabelludo. La videotoracoscopia permitió la inspección, la resección de las metástasis y la pleurodesis. No ocurrieron complicaciones ni recurrencia tumoral a los seis meses de seguimiento.

Metastatic pulmonary angiosarcomas are a common complication of a rare condition. Generally, they are presented as peripheral solid nodules, infiltrates, and pleural effusions. We report the case of a 65 year-old man with bilateral recurrent pneumothorax secondary to metastatic cavitary lesions from angiosarcoma of the scalp. In this case, videothoracoscopy allowed tumor resection, inspection, and pleurodesis. There weren't complications or tumor recurrence at six months follow up.

Neumotórax bilateral como complicación de metástasis pulmonar cavitaria de un angiosarcoma / Bilateral pneumothorax complicating cavitary pulmonary metastases in angiosarcoma

Las metástasis pulmonares de angiosarcoma constituyen una complicación común de una neoplasia maligna poco frecuente. Habitualmente se presentan como nódulos solidos periféricos y derrame pleural. Presentamos el caso de un hombre de 65 años de edad con neumotórax bilateral recurrente, secundario a metástasis cavitadas de un angiosarcoma primitivo de cuero cabelludo. La videotoracoscopia permitió la inspección, la resección de las metástasis y la pleurodesis. No ocurrieron complicaciones ni recurrencia tumoral a los seis meses de seguimiento.(AU)

Metastatic pulmonary angiosarcomas are a common complication of a rare condition. Generally, they are presented as peripheral solid nodules, infiltrates, and pleural effusions. We report the case of a 65 year-old man with bilateral recurrent pneumothorax secondary to metastatic cavitary lesions from angiosarcoma of the scalp. In this case, videothoracoscopy allowed tumor resection, inspection, and pleurodesis. There werent complications or tumor recurrence at six months follow up.(AU)

Neumotórax bilateral como complicación de metástasis pulmonar cavitaria de un angiosarcoma. / [Bilateral pneumothorax complicating cavitary pulmonary metastases in angiosarcoma].

Metastatic pulmonary angiosarcomas are a common complication of a rare condition. Generally, they are presented as peripheral solid nodules, infiltrates, and pleural effusions. We report the case of a 65 year-old man with bilateral recurrent pneumothorax secondary to metastatic cavitary lesions from angiosarcoma of the scalp. In this case, videothoracoscopy allowed tumor resection, inspection, and pleurodesis. There werent complications or tumor recurrence at six months follow up.

Síndrome de Erasmus: a propósito de un caso / Erasmus syndrome: report of a case

En 1957, L. D. Erasmus comunicó la asociación entre sílice y esclerosis sistémica, destacando la importancia de la exposición a sílice como un factor de riesgo para el desarrollo de esclerodermia ocupacional. Si bien existen reportes de la interacción entre sílice y el sistema inmune, continúa siendo actualmente una asociación infrecuente. El objetivo es presentar un paciente varón de 41 años con esclerosis sistémica e historia de exposición a gran cantidad de polvo de sílice, que desarrolló síndrome de Erasmus. Realizamos además una revisión de la literatura.

In 1957, L. D. Erasmus reported the association between silica andsystemic sclerosis, highlighting the importance of the silica exposureas a risk factor for developing scleroderma occupational. While thereare reports of the interaction between silica and the immune systemcurrently remains an infrequent association.The objective is to report a 41 year old male patient with systemicsclerosis and history of high quantity of silica dust exposure whodeveloped an Erasmus syndrome. A review of the literature has alsobeen performed.

[Hemophagocytic syndrome. Report of four cases]. / Síndrome hematofagocítico. Reporte de cuatro casos y revisión de la literatura.

Hemophagocytic syndrome (HS) is a severe hyper inflammatory condition whose cardinal symptoms are prolonged fever, cytopenia, hepatosplenomegaly, and hemophagocytosis by activated, morphologically benign macrophages. The clinical course resembles sepsis, sharing similar physiopathological features. We report four patients with the syndrome. A 61-year-old female presenting with fever and pleuritic pain. During the course of the disease, a pancytopenia was detected and a bone marrow aspiration was suggestive of HS. The patient was treated with cyclosporine and steroids with a good response. A 61-year-old male with fever and pancytopenia and a bone marrow aspirate suggestive of HS. The patient did not respond to treatment and died. A 23-year-old male with fever, pancytopenia and positive Hanta virus antibodies. A bone marrow aspirate was suggestive of HS. The patient recovered without any treatment. A 72-year-old male admitted with the diagnosis of pneumonia, that developed a progressive pancytopenia and bone marrow aspirate was suggestive of HS. A bronchoalveolar lavage showed the presence of Acinetobacter baumanii. Despite treatment with methylprednisolone and gammaglobulin, the patient died. Awareness of the clinical symptoms and of the diagnostic criteria of HS is important to start life-saving therapy in time.

Síndrome hematofagocítico: Reporte de cuatro casos y revisión de la literatura / Hemophagocytic syndrome: Report of four cases

Hemophagocytic syndrome (HS) is a severe hyper inflammatory condition whose cardinal symptoms are prolonged fever, cytopenia, hepatosplenomegaly, and hemophagocytosis by activated, morphologically benign macrophages. The clinical course resembles sepsis, sharing similar physiopathological features. We report four patients with the syndrome. A 61-year-old female presenting with fever and pleuritic pain. During the course of the disease, a pancytopenia was detected and a bone marrow aspiration was suggestive of HS. The patient was treated with cyclosporine and steroids with a good response. A 61-year-old male with fever and pancytopenia and a bone marrow aspirate suggestive of HS. The patient did not respond to treatment and died. A 23-year-old male with fever, pancytopenia and positive Hanta virus antibodies. A bone marrow aspirate was suggestive of HS. The patient recovered without any treatment. A 72-year-old male admitted with the diagnosis of pneumonia, that developed a progressive pancytopenia and bone marrow aspirate was suggestive of HS. A bronchoalveolar lavage showed the presence of Acinetobacter baumanii. Despite treatment with methylprednisolone and gammaglobulin, the patient died. Awareness of the clinical symptoms and of the diagnostic criteria of HS is important to start life-saving therapy in time.

Multiplexed methylation profiles of tumor suppressor genes and clinical outcome in lung cancer.

BACKGROUND: Changes in DNA methylation of crucial cancer genes including tumor suppressors can occur early in carcinogenesis, being potentially important early indicators of cancer. The objective of this study was to examine a multiplexed approach to assess the methylation of tumor suppressor genes as tumor stratification and clinical outcome prognostic biomarkers for lung cancer. METHODS: A multicandidate probe panel interrogated DNA for aberrant methylation status in 18 tumor suppressor genes in lung cancer using a methylation-specific multiplex ligation-dependent probe amplification assay (MS-MLPA). Lung cancer cell lines (n = 7), and primary lung tumors (n = 54) were examined using MS-MLPA. RESULTS: Genes frequently methylated in lung cancer cell lines including SCGB3A1, ID4, CCND2 were found among the most commonly methylated in the lung tumors analyzed. HLTF, BNIP3, H2AFX, CACNA1G, TGIF, ID4 and CACNA1A were identified as novel tumor suppressor candidates methylated in lung tumors. The most frequently methylated genes in lung tumors were SCGB3A1 and DLC1 (both 50.0%). Methylation rates for ID4, DCL1, BNIP3, H2AFX, CACNA1G and TIMP3 were significantly different between squamous and adenocarcinomas. Methylation of RUNX3, SCGB3A1, SFRP4, and DLC1 was significantly associated with the extent of the disease when comparing localized versus metastatic tumors. Moreover, methylation of HTLF, SFRP5 and TIMP3 were significantly associated with overall survival. CONCLUSIONS: MS-MLPA can be used for classification of certain types of lung tumors and clinical outcome prediction. This latter is clinically relevant by offering an adjunct strategy for the clinical management of lung cancer patients.

Embolismo pulmonar tumoral: Reporte de dos casos / Pulmonary tumor embolism: Report of two cases

Dyspnea and hypoxemia in a patient with cancer may have several causes, including infections, thromboembolism, metastases, and pulmonary injuries by drugs. We report a 47-year-old female with breast cancer and a 70-year-old male with urinary bladder cancer who were admitted for dyspnea, hypoxemia and pulmonary hypertension. Chest X rays and CT scans were normal. The ventilation-perfusion scintigram was highly suspicions of thromboembolism in the female. The male died 16 hours after admission and the post mortem examination revealed the presence of tumor cells in the pulmonary vasculature.

[Pulmonary tumor embolism: Report of two cases]. / Embolismo pulmonar tumoral: Reporte de dos casos.

Dyspnea and hypoxemia in a patient with cancer may have several causes, including infections, thromboembolism, metastases, and pulmonary injuries by drugs. We report a 47-year-old female with breast cancer and a 70-year-old male with urinary bladder cancer who were admitted for dyspnea, hypoxemia and pulmonary hypertension. Chest X rays and CT scans were normal. The ventilation-perfusion scintigram was highly suspicions of thromboembolism in the female. The male died 16 hours after admission and the post mortem examination revealed the presence of tumor cells in the pulmonary vasculature.

Analysis of survival in 400 surgically resected non-small cell lung carcinomas: towards a redefinition of the T factor.

INTRODUCTION: The tumor, node, metastasis (TNM) system has been recognized internationally as the standard for staging disease extension, but despite the improvements of the 1997/2002 international staging system, there may be marked differences in postoperative 5-year survival rates within each stage. There is controversy about the impact of tumor size itself as a variable unrelated to stage.The objective of this study was to analyze the influence of tumor size on the survival in patients with surgically resected non-small cell lung carcinoma (NSCLC). METHODS: Between August 1985 and January 2006, 400 patients underwent pulmonary resection with a curative intention for non-small cell lung carcinoma. Patients were excluded if they had received neoadjuvant chemotherapy. The clinicopathological records of each patient were examined for prognostic factors such as age, sex, right or left side cancer, histology, tumor location, tumor size, clinical nodal stage number, and distribution of metastatic nodes. RESULTS: Operative mortality was 2.2% for lobectomy and 18% for pneumonectomy (p < 0.05). Adenocarcinoma was the most common type (n = 245, 61.2%). Surgery was considered a complete resection in 341 patients (85.2%). When only patients without neoplastic hilar or mediastinal metastases (pN0) were included, the difference in survival was significantly different in terms of tumor size (log rank 28.46, p < 0.0001). Univariate analysis for the group of pN0 patients showed survival was not significantly affected by age, sex, side, or adenocarcinoma histology. In the multivariate analysis, tumor size and the T factor were found to have maintained its independent prognostic effects on overall survival. Among patients with pN0 tumors smaller that 15 mm in diameter, 5-year survival was 95% whereas patients with tumors bigger than 16 mm in diameter had a 5-year survival of 65% (p < 0.0001). CONCLUSION: In conclusion, our data suggest that tumors over 15 mm are associated with shorter 5-year survival in all TNM stages. Current TNM categories are not sufficiently discriminatory and the T factor requires to be reevaluated in further revisions of the TNM classification.

Primary amelanotic melanoma of the esophagus.

Primary melanoma of the esophagus (PME) is an uncommon malignancy with less than 250 cases reported in the literature. Amelanotic PME is exceedingly rare and accounts for 10-25% of melanomas of the esophagus. A 59-year-old male with a history of mild dysphagia, heartburn, moderate anorexia and weight loss for 1 month is described. Barium swallow examination and videogastroscopy showed a polypoid, ulcerated mass located 30-38 cm from the incisors. No skin or eye melanoma lesions were found. Five biopsy samples were obtained. Histological examinations revealed proliferation of large, loosely cohesive cells of variable shapes and prominent central nucleoli in the deep mucosa. Immunohistochemical findings included positive vimentin, protein S-100, Melan A, and HMB-45, and negative AE1/AE3, CD 17, and desmin. A total transhiatal esophagectomy with high cervical esophagogastric anastomosis was performed. Peritumoral lymph nodes revealed malignant invasion. A diagnosis of primary amelanotic melanoma of the esophagus was made. Fourteen months after diagnosis the patient developed disseminated PME.

[Simultaneous multiple myeloma and giant cell arteritis without systemic amyloidosis]. / Mieloma multiple y arteritis de celulas gigantes sin amiloidosis.

Primary systemic amyloidosis with clinical and histopathologic features of giant cell arteritis has already been described. The association of multiple myeloma (with primary amyloidosis) and giant cell arteritis is also known. We present the first case in the literature of a patient with multiple myeloma and giant cell arteritis without systemic amyloidosis, suggesting a pathogenic relationship between the two diseases.

Mieloma múltiple y arteritis de células gigantes sin amiloidosis / Simultaneous multiple myeloma and giant cell arteritis without systemic amyloidosis

La amiloidosis sistémica primaria y el mieloma múltiple con amiloidosis primaria se han presentado con características clínicas e histopatológicas que simulan una arteritis de células gigantes. Hasta el momento la asociación se basaba en el rol antigénico del depósito de amiloide sobre las arterias, desencadenando la respuesta inmune que finaliza con una arteritis. Presentamos el primer caso en la literatura de un paciente con mieloma múltiple y arteritis de células gigantes sin amiloidosis sistémica, sugiriendo una relación patogénica entre estas dos entidades. En el caso de nuestro paciente se descartó la presencia de amiloide en la pared arterial, por lo que proponemos que el estímulo para el desarrollo de la arteritis podría ser una excesiva producción de interleuquina 6 fabricada por las células mielomatosas

Primary systemic amyloidosis with clinical and histopathologic features of giant cell arteritis has already been described. The association of multiple myeloma (with primary amyloidosis) and giant cell arteritis is also known. We present the first case in the literature of a patient with multiple myeloma and giant cell arteritis without systemic amyloidosis, suggesting a pathogenic relationship between the two diseases

Mieloma múltiple y arteritis de células gigantes sin amiloidosis / Simultaneous multiple myeloma and giant cell arteritis without systemic amyloidosis

La amiloidosis sistémica primaria y el mieloma múltiple con amiloidosis primaria se han presentado con características clínicas e histopatológicas que simulan una arteritis de células gigantes. Hasta el momento la asociación se basaba en el rol antigénico del depósito de amiloide sobre las arterias, desencadenando la respuesta inmune que finaliza con una arteritis. Presentamos el primer caso en la literatura de un paciente con mieloma múltiple y arteritis de células gigantes sin amiloidosis sistémica, sugiriendo una relación patogénica entre estas dos entidades. En el caso de nuestro paciente se descartó la presencia de amiloide en la pared arterial, por lo que proponemos que el estímulo para el desarrollo de la arteritis podría ser una excesiva producción de interleuquina 6 fabricada por las células mielomatosas (AU)

Primary systemic amyloidosis with clinical and histopathologic features of giant cell arteritis has already been described. The association of multiple myeloma (with primary amyloidosis) and giant cell arteritis is also known. We present the first case in the literature of a patient with multiple myeloma and giant cell arteritis without systemic amyloidosis, suggesting a pathogenic relationship between the two diseases (AU)

Mieloma múltiple y arteritis de células gigantes sin amiloidosis / Simultaneous multiple myeloma and giant cell arteritis without systemic amyloidosis

La amiloidosis sistémica primaria y el mieloma múltiple con amiloidosis primaria se han presentado con características clínicas e histopatológicas que simulan una arteritis de células gigantes. Hasta el momento la asociación se basaba en el rol antigénico del depósito de amiloide sobre las arterias, desencadenando la respuesta inmune que finaliza con una arteritis. Presentamos el primer caso en la literatura de un paciente con mieloma múltiple y arteritis de células gigantes sin amiloidosis sistémica, sugiriendo una relación patogénica entre estas dos entidades. En el caso de nuestro paciente se descartó la presencia de amiloide en la pared arterial, por lo que proponemos que el estímulo para el desarrollo de la arteritis podría ser una excesiva producción de interleuquina 6 fabricada por las células mielomatosas (AU)

Primary systemic amyloidosis with clinical and histopathologic features of giant cell arteritis has already been described. The association of multiple myeloma (with primary amyloidosis) and giant cell arteritis is also known. We present the first case in the literature of a patient with multiple myeloma and giant cell arteritis without systemic amyloidosis, suggesting a pathogenic relationship between the two diseases (AU)

Biopsia del ganglio linfático centinela con colorantes vitales en pacientes con cáncer de mama: experiencia inicial del Hospital Británico de Buenos Aires / Implementation of the sentinel lymph node detection technique in patients with breast cancer

Objetivo: Implementación de la técnica de detección del ganglio linfático centinela en pacientes portadoras de cáncer de mama. Material y Métodos: Entre septiembre de 1999 y mayo de 2001 se realizaron 100 biopsias de ganglio centinela en un número igual de pacientes, mediante la inyección de azul patente al 1 por ciento peritumoral o en la periferia del área de resección previa. Luego de 5 minutos de masaje, se abordó quirúrgicamente la axila. El estudio histopatológico del ganglio centinela se realizó con E&H en todos los casos, y con inmunohistoquímica en aquellos negativos en E&H. Resultados: Se detectó el ganglio centinela en 72 casos (72 por ciento), con un promedio de 1,3 ganglios por verdaderos negativos. La sensibilidad del método fue de 92 por ciento, especificidad de 100 por ciento, tasa de falsos negativos del 8 por ciento y precisión diagnóstica del 97,2 por ciento. Comparando las distintas variables que podrían influir en los resultados, no se obtuvieron diferencias significativas en la localización del tumor, la palpabilidad de la lesión, el sitio de inyección y el intervalo entre la inyección y la cirugía. En las mayores de 70 años la identificación fue significativamente menor (50 por ciento). Si bien la identificación global fue del 72 por ciento, en los últimos 34 casos se obtuvo una identificación del 85,3 por ciento. Conclusión: La detección del ganglio linfático centinela axilar en pacientes portadoras de cáncer de mama puede ser realizada con la inyección de colorantes vitales, obteniendo índices de identificación, sensibilidad y tasa de falsos negativos aceptables. El no contar con sonda para detección intraoperatoria de radioisótopos no debería ser una limitante para que cada equipo quirúrgico involucrado en el manejo del cáncer de mama empiece su experiencia inicial y así superar la curva de aprendizaje

Biopsia del ganglio linfático centinela con colorantes vitales en pacientes con cáncer de mama: experiencia inicial del Hospital Británico de Buenos Aires / Implementation of the sentinel lymph node detection technique in patients with breast cancer

Objetivo: Implementación de la técnica de detección del ganglio linfático centinela en pacientes portadoras de cáncer de mama. Material y Métodos: Entre septiembre de 1999 y mayo de 2001 se realizaron 100 biopsias de ganglio centinela en un número igual de pacientes, mediante la inyección de azul patente al 1 por ciento peritumoral o en la periferia del área de resección previa. Luego de 5 minutos de masaje, se abordó quirúrgicamente la axila. El estudio histopatológico del ganglio centinela se realizó con E&H en todos los casos, y con inmunohistoquímica en aquellos negativos en E&H. Resultados: Se detectó el ganglio centinela en 72 casos (72 por ciento), con un promedio de 1,3 ganglios por verdaderos negativos. La sensibilidad del método fue de 92 por ciento, especificidad de 100 por ciento, tasa de falsos negativos del 8 por ciento y precisión diagnóstica del 97,2 por ciento. Comparando las distintas variables que podrían influir en los resultados, no se obtuvieron diferencias significativas en la localización del tumor, la palpabilidad de la lesión, el sitio de inyección y el intervalo entre la inyección y la cirugía. En las mayores de 70 años la identificación fue significativamente menor (50 por ciento). Si bien la identificación global fue del 72 por ciento, en los últimos 34 casos se obtuvo una identificación del 85,3 por ciento. Conclusión: La detección del ganglio linfático centinela axilar en pacientes portadoras de cáncer de mama puede ser realizada con la inyección de colorantes vitales, obteniendo índices de identificación, sensibilidad y tasa de falsos negativos aceptables. El no contar con sonda para detección intraoperatoria de radioisótopos no debería ser una limitante para que cada equipo quirúrgico involucrado en el manejo del cáncer de mama empiece su experiencia inicial y así superar la curva de aprendizaje (AU)

Case report: peritoneal sarcoidosis in an unusual location.

A 48-year-old woman underwent surgery for a lesion seen on ultrasound and interpreted as a uterine myoma. The surgery revealed multiple nodules that had seeded on the omentum, peritoneum, and ovaries. The macroscopic interpretation was either metastasis or tuberculosis. The biopsy showed noncaseating granulomas, and a diagnosis of peritoneal sarcoidosis was reported. The AFB (acid fast bacillus) and Löwenstein-Jensen culture were negative. She was treated with methylprednisolone for 1 year for pulmonary sarcoidosis progression, with a resulting decrease in her DLCO (diffusing lung capacity for carbon monoxide). Computed tomography showed only a slight decrease in the multiple nodules and cysts. She is currently symptom-free.

Microcalcificaciones: una forma frecuente de aparición del carcinoma ductal in situ (DCIS) / Microcalcifications: a frequent pattern of breast ductal carcinoma in situ

Propósito: Describir las formas de presentación mamográfica del carcinoma ductal in situ (DCIS) y su correlación con las variantes histológicas. Material y métodos: Entre enero de 1996 y diciembre de 2000 se evaluaron retrospectivamente los estudios senográficos de 31 pacientes portadoras del DCIS puro confirmados histológicamente. Los estudios mamográficos consistieron en incidencias básicas que determinaron técnicas adicionales (localizadas y magnificadas) para una mejor categorización de los hallazgos detectados. Resultados: Sobre un total de 31 pacientes portadoras de DCIS, 24 (77 por ciento) presentaron microcalcificaciones categorizadas como Bi Rads 4 y 5; 2 pacientes (6 por ciento) distorsión tisular y 5 pacientes (16 por ciento) nódulos, los cuales se describieron en 3 pacientes (10 por ciento) de contornos poco precisos y en 2 (6 por ciento) definidos con microcalcificaciones en su interior. De las 24 pacientes (77 por ciento) con microcalcificaciones Bi Rads 4-5, 18 (58 por ciento) presentaron una morfología de tipo granular y 4 (13 por ciento) laminar; las primeras fueron de aparición frecuente en el DCIS pobremente diferenciado y las segundas en el bien diferenciado. De las 18 pacientes con morfología granular, en 4 (22 por ciento) se describieron microcalcificaciones lineales asociadas. Sólo 2 pacientes (6 por ciento) de las 24 portadoras de DCIS tuvieron microcalcificaciones que se interpretaron como de tipo Bi Rads 5 (heteromorfas y heterogéneas). Conclusión: De nuestro trabajo se deduce que las microcalcificaciones resultan una de las formas más frecuentes de presentación del DCIS, siendo de importancia su detección y caracterización (laminar o granular), para orientar el diagnóstico hacia una detección precoz del cáncer de mama en su mínima manifestación