Survival analysis in patients with metastatic spinal disease: the influence of surgery, histology, clinical and neurologic status / Análise de sobrevida em pacientes com doença metastática espinhal: influência da cirurgia, histologia, status clínico e neurológico

Spine is the most common site for skeletal metastasis in patients with malignancy. Vertebral involvement quantification, neurological status, general health status and primary tumor histology are factors to set surgical planning and therapeutic targets. We evaluated the impact of general clinical and neurological status, histologic type and surgery in survival. Method : The study sample consisted of consecutive patients admitted from July 2010 to January 2013 for treatment. Results : Sixty eight patients were evaluated. 23 were female and 45 were male. Main primary neoplasic sites were: breast, prostate, lung/pleura and linfoproliferative. Thirty three out of 68 received surgical treatment, 2 received percutaneous biopsy and 33 had nonsurgical treatment. Survival : Log Rank curves revealed no statistical significant difference according to histological type, surgical approach and Frankel Score. Karnofsky Score was statistically different. Conclusion : Histological type and clinical status were statistically associated with life expectancy in vertebral metastatic disease. .

A coluna vertebral é o sítio mais comum de metastases ósseas. A quantificação do acometimento vertebral, o status neurológico, status clínico e histologia do tumor primário são fatores importantes para planejamento cirúrgico e metas terapêuticas. Nós avaliamos o impacto do status clinico geral e neurológico, tipo histológico e cirurgia na sobrevida de pacientes com metástases espinhais. Método : A amostra consistiu de pacientes consecutivamente admitidos de Julho de 2010 a Janeiro de 2013. Resultados : Sessenta e oito pacientes foram avaliados. 23 eram mulheres e 45 eram homens. Os principais sítios primários foram mama, próstata, pulmão e linfoproliferativos. Trinta e três realizaram tratamento cirúrgico, 2 realizaram biópsia percutânea e 33 tiveram tratamento conservador e radioterapia. Conclusão As curvas Log Rank não revelaram significância quanto à cirurgia e escore de Frankel, mas revelaram associação com Karnofsky e tipo histológico. .

Askin tumour: A rare thoracopulmonary tumour in adults.

Askin tumour, a primitive neuroectodermal tumour of the thoracopulmonary region, is a rare tumour presenting in childhood. Its presentation in adults is rare. We report a case of an Askin tumour in an adult patient who presented to us with worsening breathlessness and vague chest pain. Investigations including immunohistochemistry confirmed the diagnosis of Askin tumour.

Enfermedad pulmonar por amianto en trabajadores de acería / Pulmonary disease due to asbestos in steel industry workers

Las enfermedades relacionadas al amianto se producen por la inhalación de fibras de asbestos en su variedad crisotilo o amianto blanco. A pesar de que en la Argentina la prohibición data del año 2003, existen numerosas industrias donde se sigue trabajando con este mineral, entre ellas las metalúrgicas y acerías. Actualmente se conoce la alta patogenicidad de este material, por lo que en muchos países existen programas de seguimiento de los trabajadores expuestos. Se describen las características generales y manifestaciones clínicas pulmonares de 27 pacientes que trabajaron en una gran acería de América del Sur. El diagnóstico de amiantopatías se realizó mediante historia clínica laboral, antecedente de exposición al amianto, estudios complementarios de función pulmonar e imágenes del tórax. Se analizaron la fuente de exposición (laboral, doméstica y ambiental), tiempo de exposición y período de latencia en los pacientes de los cuales se detectó enfermedad relacionada. Los antecedentes de tabaquismo fueron tenidos en cuenta para el análisis. En 22 pacientes se presentaron patologías benignas (81.4%), 16 de ellos tenían lesiones exclusivamente pleurales y otros 6 asbestosis. Las patologías malignas se presentaron en 5 pacientes (18.5%), en 4 fueron mesoteliomas y en uno carcinoma pulmonar. El problema de la exposición al amianto tiene vigencia actual. De ahí la necesidad de un programa de vigilancia en trabajadores expuestos al amianto actualmente o en el pasado, para detectar, notificar, registrar e investigar las características de estas patologías.

Asbestos-related diseases are caused by the inhalation of asbestos fibers in their variety chrysotile or white asbestos. Although the ban in Argentina dates from 2003, there are numerous industries where work continues with this mineral, including iron and steel industries. It is currently known the high pathogenicity of this material, so that in many countries there are programs to monitoring the exposed workers. Here we describe the general characteristics and pulmonary manifestations in 27 patients who had worked in a very huge steel factory in South America. The diagnosis of asbestos-related diseases was made by a medical-occupational record, history of asbestos exposure, additional studies of lung function and chest images. Then the sources of exposure (occupational, domestic and environmental), exposure time and latency period were analyzed, in those patients in whom a related disease was detected. Smoking history was also taken into account. Twenty-two patients had benigns pathologies (81.4%), sixteen of them with lesions localyzed in pleura, and other six pulmonary asbestosis. The malignant pathologies occurred in five patients (18.5%), in four of them mesothelioma and in other one lung cancer. The problem of asbestos exposure has contemporary relevance. Hence the need for a surveillance program in workers exposed to asbestos in the past or currently, to detect, report, record and investigate the characteristics of these pathologies.

Metastatic pleural effusion: A rare presentation of salivary gland adenoid cystic carcinoma.

Adenoid cystic carcinoma, an uncommon malignant salivary gland neoplasm, is known for its long clinical course, indolent growth, local recurrence and late distant metastasis. We report an unusual case of adenoid cystic carcinoma of the palate in a 64-year-old woman, undiagnosed for more than 15 years, who presented as malignant pleural effusion.

Tumor fibroso solitario gigante de la pleura / Giant solitary fibrous tumor of the pleura

Introducción: El tumor fibroso solitario es el segundo tumor primario de la pleura y puede alcanzar hasta 39 cm de diámetro; para tener la denominación de “gigante” debe ocupar al menos 40 % del hemitórax afectado. Por lo general su comportamiento es benigno, pero existen criterios de malignidad. El objetivo de esta investigación fue efectuar una revisión de la evaluación inicial, diagnóstico, manejo quirúrgico, resultado del tratamiento y pronóstico. Material y métodos: Estudio descriptivo, observacional, longitudinal y retrospectivo, realizado de 2002 a 2006, en pacientes operados con diagnóstico de tumor fibroso solitario gigante de la pleura. Resultados: Se incluyeron seis pacientes, 83.3 % del sexo femenino, con edad promedio de 48 años; todos sintomáticos con predominio de disnea, tos y dolor; en 66.7 % se encontró del lado izquierdo; a 83.3 % se realizó angiografía y embolización preoperatorias, logrando resección completa en todos; predominó aporte arterial de la arteria mamaria interna. Se encontró una tasa de complicaciones transoperatorias de 17 %. En 66.7 % se identificó un pedículo ascular; el tumor mayor midió 40 cm de diámetro con peso de 4500 g; solo uno presentó actividad mitótica elevada. El seguimiento promedio fue de 14 meses. Conclusiones: La sintomatología encontrada fue acorde con informes previos, aunque en porcentajes mayores. El diagnóstico correcto es de vital importancia, ya que con la resección quirúrgica el tumor fibroso solitario es potencialmente curable, sin embargo, requiere seguimiento a largo plazo. Dado el tamaño de este tipo de tumores es aconsejable llevar a cabo embolización preoperatoria.

BACKGROUND: Solitary fibrous tumor is the second primary malignancy of the pleura and can reach up to 39 cm in diameter; however, to be referred to as 'giant' it must occupy at least 40% of the affected hemithorax. Although this tumor usually shows a benign behavior, malignancy criteria have been described. The aim of the study was to assess the initial evaluation, diagnostic procedures, surgical management, treatment outcome, and prognosis. METHODS: We performed a descriptive, observational, longitudinal, and retrospective study from 2002 to 2006 on patients who underwent surgery with a diagnosis of giant solitary fibrous tumor of the pleura. RESULTS: Six patients were included; 83.3% were females. Mean age was 48 years. All patients were symptomatic, mainly dyspnea, cough and chest pain; 66.7% were left-sided. Preoperative angiography and embolization were performed in 83.3% cases with successful surgical resection. The predominant blood supply was derived from the internal mammalian artery. Intraoperative complication rate was 17%. A vascular pedicle was found in 66.7%. The largest lesion was 40 cm in diameter and weighed 4500 g. Only one case showed high mitotic activity. Mean follow-up to date is 14 months. CONCLUSIONS: Symptomatology found was consistent with previous reports but in higher percentages. Accurate diagnosis is critical because surgical resection involves a potential cure; however, long-term follow-up is mandatory. Preoperative embolization is recommended due to tumor size.

Morphological study of pathological lesions seen in pleural biopsies

To determine the frequency and types of different pathological lesions seen in pleural biopsies in our patients. This is a prospective study carried out in the Department of Pathology, Basic Medical Sciences Institute, Jinnah Postgraduate Medical Centre, Karachi. A total of 155 pleural biopsies over a period from 01st January 2007 to 30th June 2008 were reviewed. Haemotoxylin and Eosin [H and E] stained slides were examined to determine the various non- neoplastic and neoplastic lesions. Special stains were performed whenever required. Out of 155 cases of pleural biopsies, 110 cases were diagnosed as non-neoplastic lesions and 29 as neoplastic lesions. The remaining 16 cases were found inconclusive. The non-neoplastic lesions included chronic granulomatous inflammation consistent with tuberculosis 47 cases, chronic non-specific inflammation 42 cases and empyema 21 cases. Chronic granulomatous inflammation consistent with tuberculosis was found to be commonest non-neoplastic lesion with male predominance and maximum number of cases was seen in 2nd decade of life. Neoplastic lesions included adenocarcinoma 26 cases followed by undifferentiated small cell carcinoma, squamous cell carcinoma and malignant lymphoma each comprising single case. Adenocarcinoma was found to be commonest neoplastic lesion with slight female predominance and maximum number of cases was seen in 5th decade of life. The commonest non-neoplastic pleural biopsy lesion was found to be tuberculosis and the commonest neoplastic lesion was adenocarcinoma in pleural biopsy

Carcinosarcoma of the pleura and lung metastasized from a poorly differentiated squamous cell carcinoma of the esophagus

Transformation of a poorly differentiated squamous cell carcinoma [SCC] of the esophagus into a [carcinosarcoma] of the pleura and lung has never been reported and its histogenetic origin is still debated. A 48-year-old man was admitted due to progressive dysphagia and a weight loss of 5 kilograms within 2 months. Upper gastrointestinal panendoscopic biopsy revealed poorly differentiated SCC of thoracic esophagus, upper third, T4N1M1 a, stage IVa. He received concurrent chemoradiotherapy [CCRT]. About 9 months later, rapid progression of lung metastases and pleural effusion were found. According to the histopathological and immunohistochemical stain results, carcinosarcoma was diagnosed. Palliative therapy was given and the patient eventually died of the disease 9 months after SCC of the thoracic esophagus was diagnosed and one month after carcinosarcoma of the pleura and lung were confirmed

Pleuropulmonary blastoma in an infant.

Pleuropulmonary blastomas (PPB) are rare and highly aggressive tumors. Herein, we report an infantile case of type III PPB. A 9-mth-old boy presented to our unit with a history of cough and tachypnea for 2 days. Chest computed tomography revealed a mass in the left upper lobe, emphysema in the left upper lung, and mediastinum and heart shifted towards the right. The mass was removed completely by left upper lobectomy and histology confirmed diagnosis of type III PPB. The immature blastematous tissue was positive for vimentin while benign epithelium was positive for epithelial membrane antigen and cytokeratin. No lymph nodule metastasis was found in the 7 lymph nodules obtained from the hilum of the lung near the tumor. Currently, the patient is under close follow-up and is doing well.

Hemangioendotelioma epitelioide de pleura: presentación de un caso con hemotórax / Epithelioid hemangioendothelioma of the pleura: presentation of a case with hemotorax

Los hemangioendoteliomas son tumores vasculares que pueden afectar pulmón. Abarcan desde lesiones benignas o de baja malignidad hasta lesiones de malignidad intermedia, como el hemangioendotelioma epitelioide o el polimorfo. El hemangioendotelioma epitelioide es un tumor muy raro que afecta principalmente a mujeres menores de 40 años, ha sido asociado al uso de anticonceptivos orales y a la inhalación de cloruro de vinilo. Sólo se describen en la literatura unos cincuenta casos de hemangioendotelioma epitelioide pulmonar y muy pocos pleurales. Inmunomarcadores permiten caracterizar este tipo de neoplasias. El diagnóstico diferencial incluye tumores benignos y malignos y el pronóstico es poco predecible. No hay consenso en cuanto al tratamiento, el cual ha incluído cirugía, carboplatino más etopósido e interferón. Se presenta una paciente de 37 años, se discuten los hallazgos clínicos su tratamiento y evolución.

The hemangioendotheliomas are vascular tumors that may involve lungs. The malignancy of these tumours can be benign, low or intermediate, such as the epithelioid or the polymorph hemangioendothelioma. The epithelioid hemangioendothelioma is an unusual tumour that appears more frequently in females, younger than 40 years of age. It has been associated to the use of oral contraceptives and the inhalation of vinile chloride. Only some 50 cases of pulmonary epithelioid hemangioendothelioma and few of pleural origin have been described in the literature. Immunomarkers may help to characterize this kind of tumours. The differential diagnosis includes malignant and benign tumours and its prognosis is hard to predict. Its therapy remains controversial, surgery, chemotherapy with carboplatin plus etoposide and interferon were used. The case of a 37 year old female is presented; clinical findings, therapy and outcome are discussed.

An autopsy report of a rare pediatric lung tumor: pleuropulmonary blastoma.

An autopsy report of pleuropulmonary blastoma (PPB) is described in a two-and-a-half-year-old male child who died within a few days of starting chemotherapy. Autopsy revealed a large tumor almost occupying the whole of left hemithorax with widespread extension to pleura. The diagnosis was confirmed to be PPB, type III on autopsy.

Mesotelioma pleural maligno: experiência multidisciplinar em hospital público terciário / Malignant pleural mesothelioma: multidisciplinary experience in a public tertiary hospital

OBJETIVOS: Avaliar a experiência com o diagnóstico e a terapêutica do mesotelioma pleural maligno (MPM) acumulada durante 5 anos em um hospital público terciário. MÉTODOS: Avaliação retrospectiva dos prontuários dos pacientes com diagnóstico de MPM entre janeiro de 2000 e fevereiro de 2005. RESULTADOS: Foram analisados 17 pacientes, 14 homens e 3 mulheres, com idade média de 54,1 (13-75) anos. Os espécimes de biópsia para exame histopatológico foram obtidos por meio de pleuroscopia em 9 pacientes (53 por cento), agulha de Cope em 5 (29,5 por cento) e biópsia pleural aberta em 3 (17,5 por cento). Os tipos histológicos foram: epitelial em 14 pacientes (82 por cento), sarcomatóide em 1 (6 por cento) e bifásico em 2 (12 por cento). As terapêuticas instituídas foram: multimodal (pleuropneumonectomia com radioterapia e quimioterapia adjuvante) em 6 pacientes (35 por cento), quimioterapia e radioterapia em 6 (35 por cento), radioterapia exclusiva em 3 (17,5 por cento) e quimioterapia exclusiva em 2 (12 por cento). A sobrevida média foi de 11 (1-26) meses. CONCLUSÕES: Na presente experiência foi empregada a abordagem multidisciplinar integrada, e contou-se com uma estrutura hospitalar de alta complexidade para o diagnóstico e tratamento do MPM, como preconizado na literatura. Apesar disso, a sobrevida média observada foi de apenas 11 meses, refletindo a agressividade da doença.

OBJECTIVE: To evaluate the experience in diagnosing and treating malignant pleural mesothelioma (MPM) accumulated over 5 years in a tertiary public hospital. METHODS: The medical charts of the patients diagnosed with MPM between January of 2000 and February of 2005 were evaluated retrospectively. RESULTS: Of the 17 patients analyzed, 14 were male and 3 were female. The mean age was 54.1 years (range, 13-75 years). The biopsy specimens for histopathological examination were obtained through thoracoscopy in 9 patients (53 percent), Cope needle in 5 (29.5 percent), and open pleural biopsy in 3 (17.5 percent). The following histological types were identified: epithelial, in 14 patients (82 percent); sarcomatoid, in 1 (6 percent); and biphasic, in 2 (12 percent). The therapeutic approaches used were as follows: multimodal (pleuropneumonectomy and adjuvant radiotherapy and chemotherapy) in 6 patients (35 percent); chemotherapy and radiotherapy in 6 (35 percent); radiotherapy alone in 3 (17.5 percent); and chemotherapy alone in 2 (12 percent). The mean survival was 11 months (range, 1-26 months). CONCLUSIONS: In the cases studied, an integrated multidisciplinary approach was used, and a highly complex hospital infrastructure was available for the diagnosis and treatment of MPM, as recommended in the literature. However, the mean survival was only 11 months, reflecting the aggressiveness of the disease.

Marcadores morfológicos de prognóstico no mesotelioma maligno: um estudo de 58 casos / Morphological aspects as prognostic factors in malignant mesothelioma: a study of 58 cases

OBJETIVO: Diversos marcadores têm se mostrados promissórios como preditores do diagnóstico e prognóstico do mesotelioma maligno (MM). MÉTODO: Mediante estudo morfométrico e inmunomarcação de componentes estromais (calretinina, CEA, Leu-M1 e trombomodulina) e nucleares (p53 e Ki-67), avaliamos a sobrevida após o diagnóstico de 58 pacientes com tumores malignos de pleura. RESULTADOS: O padrão histológico típico do mesotelioma maligno foi encontrado em 50 casos e o padrão atípico em 8 casos. Imunohistoquimicamente foram confirmados 40 casos como sendo mesoteliomas, 11 como adenocarcimonas e 7 casos do padrão atípico não puderam ser classificados. A análise multivariavel do Cox demonstrou a coexistência de um maior fator de risco de morte (476.2), nos pacientes com idade avançada, subtipo histológico bifásico e componentes de expressão nuclear. CONCLUSÃO: A calretinina foi o marcador inmunohistoquímico (IHQ) mais útil para o diagnóstico do mesotelioma e o CEA para o de adenocarcinoma. A quantificação por IHQ da trombomodulina foi fundamental na diferenciação do mesotelioma quando este foi positivo tanto para calretinina e como para o CEA. A informação prognostica mais valiosa foi a fornecida pela análise rotineira histopatológica do tipo histológico tumoral. Um ponto importante, divisor natural, foi a idade com uma media de 55 anos e 30.5 por cento de componentes nucleares de marcação IHQ, separando os pacientes em dois grupos: pacientes com uma sobrevivência curta contra pacientes com uma sobrevivência mais longa que a esperada. Assim, a análise histopatológica oferece uma arma poderosa e de elevado potencial para guiar no tratamento adjuvante de quimioterápicos após a retirada cirúrgica do mesotelioma.

OBJECTIVE: Various markers have shown promise as diagnostic markers and prognostic predictors in malignant mesothelioma (MM). METHODS: Through morphometric and immunological studies of markers in stromal components (calretinin, CEA, Leu-M1 and thrombomodulin) and nuclear components (p53 and Ki-67), we evaluated post-diagnosis survival in 58 patients with MM. RESULTS: The histologic pattern of the MM was typical in 50 cases and atypical in 8. Through immunohistochemistry, we confirmed 40 cases of mesothelioma and 11 cases of adenocarcinoma, although we were unable to classify 7 of the 8 cases presenting atypical histologic patterns. Cox multivariate analysis revealed that the risk factor for death was higher (476.2) among patients of advanced age, presenting the biphasic subtype and testing positive for components expressed at the nuclear level. CONCLUSION: The most useful immunohistochemical markers were was calretinin (for mesothelioma) and CEA (for adenocarcinoma). Immunohistochemical quantification of thrombomodulin facilitated the diagnosis of mesothelioma in patients testing positive for both calretinin and CEA. The most useful prognostic information was that provided by the routine histopathological analysis of the tumor type. It is of note that the combination of a mean age of 55 years and 30.5 percent immunohistochemical markers in nuclear components created a natural dividing point between patients in which survival was shorter than expected and those in which it was longer than expected. Therefore, histopathological analysis offers a powerful weapon with great potential to inform decisions regarding the use of adjuvant chemotherapy after surgical excision of a mesothelioma.

Inmunocitoquímica para líquidos de pleura y peritoneo: estandarización de la técnica en el Hospital de San Jose / Immunocytochemistry for pleural and peritoneal fluids: standardization of the technique at the San Jose Hospital

El objetivo de esta investigación fue estandarizar y crear un protocolo para una técnica de inmunocitoquímica en líquidos pleural y peritoneal, procedimiento de gran utilidad como ayuda diagnóstica cuando no se sabe con certeza donde se encuentra el tumor primario. Incluye la obtención del líquido para determinar mediante el procedimiento tradicional si es positivo para malignidad, el método de procesamiento y el desarrollo de la técnica inmunocitoquímica.

Pleuropulmonary blastoma--a rare pathology.

Pleuropulmonary blastoma is a rare and highly aggressive pulmonary malignancy in children. The clinical features, radiologic, cytologic, gross and histopathological features of one such case in a 9 year old girl is discussed along with a short review of literature.

Hemangioendotelioma epitelióide de pleura / Epithelioid hemangioendothelioma of the pleura

Relata-se o caso de um paciente exposto profissionalmente a asbesto por dez anos e portador de um tumor pleural muito raro, o hemangioendotelioma epitelióde. O paciente apresentava derrame pleural serohemorrágico, sem evidência de células neoplásicas e com predomínio de linfócitos. A biópsia pleural por agulha revelou processo inflamatório crônico inespecífico, com áreas de tecido mixóide. A videotoracoscopia mostrou nódulos difusos nas pleuras parietal e visceral. A biópsia revelou neoplasia mesenquimal e eram semelhantes às áreas focais observadas na primeira biópsia. O estudo imunohistoquímico demonstrou a presença dos marcadores vasculares CD31, CD34 e Fator VIII, caracterizando a origem vascular do tumor. O paciente foi tratado com cisplatina e ectoposide, tendo o óbito ocorrido três meses após o diagnóstico.

Variáveis clínicas e laboratoriais no diagnóstico de derrame pleural por tuberculose e neoplasia / Clinical and laboratorial variables in diagnosis of tuberculosis and malignant pleural effusions

Modelos diagnósticos (MD) de baixo custo e complexidade com variáveis clínicas e laboratoriais que diferenciem, com eficiência, derrame pleural neoplásico (DPN) e tuberculoso (DPT). MÉTODO. Variáveis clínicas, exames bioquímicos, citologia quantitativa e oncótica (ONC) e histologia do tecido pleural de 403 casos de DPT (200) ou DPN (203) foram analisados. / Diagnoses Models (DM) of low cost and complexity with clinical and laboratorial variables that differentiate, with efficiency, malignance (MPE) and tuberculous (TPE) pleural effusion. METHOD. Clinical variables, biochemical tests, quantitative cytology and oncotic (ONC) and histology of pleural tissue of 403 cases of DPE (200) or MPE (203) were analyzed...

Primary pleuro-pulmonary malignant germ cell tumours.

Lungs and pleura are rare sites for malignant germ-cell tumours. Two cases, pure yolk-sac tumour and yolk sac-sac tumour/embryonal carcinoma are described in young males who presented with rapid progression of respiratory symptoms. The malignant mixed germ cell tumour occurred in the right lung, while the yolk-sac tumour had a pseudomesotheliomatous growth pattern suggesting a pleural origin. Alpha-foetoprotein was immunohistochemically demonstrated in both.