Pulmonary neuroendocrine (carcinoid) tumors: European Neuroendocrine Tumor Society expert consensus and recommendations for best practice for typical and atypical pulmonary carcinoids.

BACKGROUND: Pulmonary carcinoids (PCs) are rare tumors. As there is a paucity of randomized studies, this expert consensus document represents an initiative by the European Neuroendocrine Tumor Society to provide guidance on their management. PATIENTS AND METHODS: Bibliographical searches were carried out in PubMed for the terms 'pulmonary neuroendocrine tumors', 'bronchial neuroendocrine tumors', 'bronchial carcinoid tumors', 'pulmonary carcinoid', 'pulmonary typical/atypical carcinoid', and 'pulmonary carcinoid and diagnosis/treatment/epidemiology/prognosis'. A systematic review of the relevant literature was carried out, followed by expert review. RESULTS: PCs are well-differentiated neuroendocrine tumors and include low- and intermediate-grade malignant tumors, i.e. typical (TC) and atypical carcinoid (AC), respectively. Contrast CT scan is the diagnostic gold standard for PCs, but pathology examination is mandatory for their correct classification. Somatostatin receptor imaging may visualize nearly 80% of the primary tumors and is most sensitive for metastatic disease. Plasma chromogranin A can be increased in PCs. Surgery is the treatment of choice for PCs with the aim of removing the tumor and preserving as much lung tissue as possible. Resection of metastases should be considered whenever possible with curative intent. Somatostatin analogs are the first-line treatment of carcinoid syndrome and may be considered as first-line systemic antiproliferative treatment in unresectable PCs, particularly of low-grade TC and AC. Locoregional or radiotargeted therapies should be considered for metastatic disease. Systemic chemotherapy is used for progressive PCs, although cytotoxic regimens have demonstrated limited effects with etoposide and platinum combination the most commonly used, however, temozolomide has shown most clinical benefit. CONCLUSIONS: PCs are complex tumors which require a multidisciplinary approach and long-term follow-up.

Atypical carcinoid tumours of the lung: prognostic factors and patterns of recurrence.

BACKGROUND: Atypical carcinoids (AC) of the lung are rare intermediate-grade neuroendocrine neoplasms. Prognostic factors for these tumours are undefined. METHODS: Our cooperative group retrieved data on 127 patients operated between 1980 and 2009 because of an AC. Several clinical and pathological features were studied. RESULTS: In a univariable analysis, T-status (p=0.005), N-status (p=0.021), preoperative M-status (previously treated) (p=0.04), and distant recurrence developed during the outcome (p<0.001) presented statistically significant differences related to survival of these patients. In a multivariable analysis, only distant recurrence was demonstrated to be an independent risk factor for survival (p<0.001; HR: 13.1). During the monitoring, 25.2% of the patients presented some kind of recurrence. When we studied recurrence factors in a univariable manner, sublobar resections presented significant relationship with locoregional recurrence (p<0.001). In the case of distant recurrence, T and N status presented significant differences. Patients with preoperative M1 status presented higher frequencies of locoregional and distant recurrence (p=0.004 and p<0.001, respectively). In a multivariable analysis, sublobar resection was an independent prognostic factor to predict locoregional recurrence (p=0.002; HR: 18.1). CONCLUSIONS: Complete standard surgical resection with radical lymphadenectomy is essential for AC. Sublobar resections are related to locoregional recurrence, so they should be avoided except for carefully selected patients. Nodal status is an important prognostic factor to predict survival and recurrence. Distant recurrence is related to poor outcome.

Hernia diafragmática traumática tardía complicada en vólvulo de colon intratorácico / Traumatic diaphragmatic hernia complicated with colon volvulus

La hernia diafragmática traumática es una complicación que puede aparecer en traumatismos torácicos cerrados. Son más frecuentes en el lado izquierdo y en varones. Su diagnóstico suele realizarse de forma precoz. Describimos un caso de hernia postraumática derecha tardía, cuyo síntoma inicial fue un cuadro de oclusión intestinal, producida por volvulación de colon en hemitórax derecho, después de 20 meses de producirse el traumatismo torácico cerrado (AU)

Traumatic diaphragmatic hernia is an uncommon complication that may occur in blunt thoracoabdominal trauma. They are more frequent in males and left side. The diagnosis can be missed in the initial evaluation. We present a case with a delayed right diaphragmatic hernia, which first symptom was an intestinal obstruction, due to colon volvulus in right hemithorax twenty months after blunt thoracoabdominal trauma (AU)

Hydatid cyst of the lung: diagnosis and treatment.

Hydatid infestation of the lung can be primary or secondary. In three of four cases the cyst is a single one. Hydatidosis of a different location, particularly the liver, may be associated. The period of initial growth of primary hydatidosis is frequently asymptomatic. Bronchial fistulization is an important event in the evolution of the cyst. Intrapleural rupture constitutes a rare eventuality, but it is often as characteristic as it is severe. Secondary, metastatic hydatidosis, due to breaking of a primary visceral cyst in a vein or heart, is rare. A special form is so-called multiple malignant pulmonary hydatidosis, which causes progressive respiratory deficiency and right ventricular failure. There are a variety of radiographic images. Ultrasonography, computed tomography, and magnetic resonance imaging can recognize certain details of the lesions and discover others that are not visible by conventional radiography. For a specific serologic diagnosis, our experience favors the immunoglobulin G enzyme-linked immunosorbent assay and immunoelectrophoresis. Treatment is essentially surgical. In general, chemotherapy is used as a complement to operative treatment to avoid recurrence. Surgery has two objectives: to remove the parasite and to treat the bronchipericyst pathology and other associated lesions. The prognosis has changed during the last few years, and results are now commonly satisfactory. The most frequent complications are pleural infection and prolonged air leakage. Operative mortality does not exceed 1% to 2%. Despite the low mortality and the limited recurrence rate, it is necessary to remember the invading character of pulmonary hydatid disease, which sometimes makes therapy difficult and questionable. Prophylaxis is essential to eradicate the disease completely.

[Assessment of mediastinal tumor involvement (T factor) in lung cancer with transesophageal ultrasonography]. / Evaluación de la afectación mediastínica tumoral (factor T) en el cáncer de pulmón mediante ecografía transesofágica.

UNLABELLED: Transesophageal sonography was originally used to assess esophageal-gastric neoplasm. The technique may also be useful in the evaluation of pulmonary neoplasms for possible mediastinal involvement, with regard to both T and N factors. OBJECTIVE: To consider the validity of this minimally-invasive technique for assessing possible mediastinal involvement by direct tumoral invasion (T4) and to help obtain the most accurate staging of lung cancer. PATIENTS AND METHODS: Sixteen patients with confirmed histopathological diagnoses of pulmonary neoplasm were examined by transesophageal sonography in order to evaluate possible mediastinal involvement. They had previously been classified by computed tomography as T4 (12 patients) or possible T4 (4 patients). Mediastinal involvement was also assessed by mediastinotomy or thoracotomy in 15 of the 16 patients. RESULTS: Transesophageal sonography revealed mediastinal involvement in eight of the 16 patients; the rest had no such involvement. Surgical exploration of the mediastinum confirmed involvement in seven of those who had been so classified by sonography (with the remaining patient not having been assessed surgically). Among the eight patients who were considered free of mediastinal involvement, there was in fact none, although we found previously undetected infiltration of the posterior surface of the right pulmonary artery in one patient. Sensitivity was 87.5%, specificity 100% and accuracy 93.3%. CONCLUSION: Transesophageal ultrasound is a diagnostic tool that can provide additional information to complement other diagnostic strategies.

Evaluación de la afectación mediastínica tumoral (factor T) en el cáncer de pulmón mediante ecografía transesofágica / Transesophageal ultrasound assessment of mediastinal tumor involvement (T factor) in lung cancer

La ecografía transesofágica es una técnica inicialmente empleada en la valoración de las neoplasias esofagogástricas. Su utilización en la evaluación de las neoplasias pulmonares puede ser una ayuda valiosa a la hora de considerar la posible afectación mediastínica tanto en el factor T como en el N. Objetivo: Considerar la validez de esta exploración incruenta para determinar la posible afectación mediastínica por invasión directa del tumor (T4) como medio para conseguir una más correcta estadificación del cáncer de pulmón. Pacientes y métodos: Dieciséis pacientes con neoplasia de pulmón confirmada histopatológicamente han sido explorados mediante ecografía transesofágica para evaluar una posible afectación mediastínica. Previamente habían sido etiquetados por tomografía computarizada como T4 (12) o dudosos T4 (4). En 15 de los 16 pacientes se evaluó la afectación del mediastino con mediastinotomía o toractomía. Resultados: De los 16 pacientes, ocho presentaban afectación mediastínica mediante ecografía transesofágica y en el resto no. La evaluación quirúrgica del mediastino confirmó la afectación de éste en siete de los detectados por la ecografía (uno no se evaluó quirúrgicamente). De los 8 pacientes considerados como sin afectación mediastínica en siete no existía y en uno no se detectó una infiltración de la cara posterior de la arteria pulmonar derecha. La sensibilidad fue del 87,5 por ciento, la especificidad, del 100 por ciento y la exactitud del 93,3 por ciento. Conclusión: La ecografía transesofágica es un método diagnóstico que puede aportar información adicional y complementaria a otros métodos diagnósticos. (AU)

[Surgical treatment of lung cancer: comparative assessment of the staging systems of 1986 and 1997. Results in 500 consecutive patients]. / Tratamiento quirúrgico del cáncer de pulmón: evaluación comparativa de los sistemas de estadificación de 1986 y 1997. Resultados en 500 pacientes consecutivos.

OBJECTIVE: To evaluate the influence of different variables on survival in relation to the staging guidelines of 1986 and 1997. PATIENTS AND METHODS: Five hundred patients (473 men and 27 women) with non-small cell lung cancer were treated surgically from 1980 to 1997. Resections performed: 184 lobectomies, 16 bi-lobectomies, 244 pneumonectomies, 2 bronchoplastic lobectomies, and 54 segmentectomies. HISTOLOGY: 338 epidermoid, 86 adenocarcinoma, 40 giant cell, 36 mixed tumor. Differentiation: 216 N1, 91 N2, 193 N3. Stages according to 1986 guidelines were I: 246 (49.2%) (T1: 32, T2: 214); II: 27 (5.4%); IIIa: 197 (39.4%) (N0: 84; N1: 2; N2: 111); IIIb: 23 (4.6%) (N0: 12; N2: 11); and IV: 7 (1.4%) (N0: 4; N2: 3). Stages according to the 1997 guidelines were used for comparison of survival between patients with Ia and Ib tumors and with IIb and IIIa tumors. RESULTS: With follow-up periods ranging from 2 to 17 years, 141 patients (28%) were alive, 26 (5%) were lost to follow-up and 333 had died. Two patients (0.4%) died during surgery and 36 (7.2%) died during the postoperative period. Among the remaining 462 patients, 295 deaths were related to the following causes: metastasis in 130 cases (44%), recurrence in 81 cases (27%), functional causes in 17 (6%), independent causes in 54 (18%) and unknown causes in 13 (4%). Overall survival rates at 5 and 10 years were 36 and 26%, respectively; survival rates by histological type: epidermoid 36 and 26%, adenocarcinoma 35 and 26%; stage I, 51 and 41% (Ia, 81 and 75%; Ib, 44 and 33%); IIIa 24 and 15% (IIb of 1997: 27 and 17%; IIIa of 1997: 20 and 13%). Survival by N factor: N0, 44 and 34%; N2, 17 and 8% (1986) and 17 and 11% (1997). CONCLUSIONS: Survival agrees with other studies. The 1997 staging guidelines are useful for differentiating survival between stages Ia and Ib and between IIb and IIIa. N and T factors, histology and stage influence the appearance of metastasis; T factor influences recurrence.

Prognostic factors in neuroendocrine lung tumors: a Spanish Multicenter Study. Spanish Multicenter Study of Neuroendocrine Tumors of the Lung of the Spanish Society of Pneumonology and Thoracic Surgery (EMETNE-SEPAR).

BACKGROUND: This study examines the experience of the Spanish Multicenter Study of Neuroendocrine Tumors of the Lung through the clinical data and behavior of patients treated for this pathologic process. METHODS: From 1980 to 1997, 361 cases of neuroendocrine carcinomas (NEC) were treated surgically. Patients were enrolled in a protocol using the pathologic and follow-up reports. According to Dreslers' criteria, the cases were segregated into grade 1 (typical carcinoid), grade 2 (atypical carcinoid), grade 3 large cell type, and grade 3 small cell type. Several variables were reviewed in all patients. Statistical analysis was performed to determine whether clinical characteristics and differentiation were associated with significant differences in the prognosis. RESULTS: A total of 261 cases of NEC were identified with grade 1, 43 with grade 2, and with grade 3: 22 of large and 35 of small cells. Five-year survival for different grades was as follows: grade 1, 96%; 2, 72%; 3 large cell type, 21%; and 3 small cell type, 14%. When a comparative analysis between typical and atypical carcinoids was performed a significant difference for mean age, tumor size, nodal metastases, and recurrence was observed. However, female sex, nodal metastases, and recurrence rate differed between atypical carcinoids and grade 3 NEC of large cells. A difference in recurrence rate was found between patients with both types of grade 3 NEC. CONCLUSIONS: The progressive deterioration of tumor organization highlights that neuroendocrine tumors constitute a continuous spectrum. A careful observation of pathologic findings is necessary to individualize their prognostic factors.

[Mediastinal hydatid cysts: 8 cases]. / Quistes hidatídicos de mediastino: 8 casos.

Hydatidosis in humans is a parasitic disease produced by cystic growth of the larvae of the tapeworm Echinococcus granulosus, affecting mainly the liver and lung. Larvae are rarely present in the mediastinum, although approximately 100 cases have been described in the literature. We report eight cases of hydatid cysts of the mediastinum (HCM) treated surgically over a period of 21 years. The incidence was similar in males and females and ages ranged from 10 to 74 years. Symptoms depend on size, location and involvement of neighboring structures in HCM. The most serious complication is cyst rupture with consequent transfer of hydatid material to the blood, possibly causing anaphylactic shock and even death. Currently, sonography, computed tomography and magnetic resonance images facilitate diagnosis. Treatment involves excision of the cyst and peri-cystic tissue. CHM should be suspected when mediastinal cysts are found in countries where the incidence of hydatidosis is high.

Tratamiento quirúrgico del cáncer de pulmón: evaluación comparativa de los sistemas de estadificación de 1986 y 1997. resultados en 500 pacientes consecutivos / Surgical treatment of lung cancer: comparative evaluation of the 1986 and 1997 staging systems. Results for 500 consecutive patients

Objetivo: Evaluar la influencia de diferentes variables en la supervivencia con referencia a la estadificación de 1986 y 1997. Pacientes y métodos: Se incluyeron en el estudio 500 pacientes afectados de cáncer de pulmón no de células pequeñas, tratados quirúrgicamente de 1980 a 1997; 473 eran varones y 27 mujeres. Resecciones practicadas: 184 lobectomías, 16 bilobectomías, 244 neumonectomías, 2 lobectomías broncoplásticas y 54 segmentectomías. Histología: 338 carcinomas epidermoides, 86 adenocarcinomas, 40 carcinomas de células grandes, 36 tumores mixtos. Diferenciación: 216 G1, 91 G2, 193 G3. Estadios de 1986: I: 246 (49,2 por ciento) (T1: 32; T2: 214); II: 27 (5,4 por ciento); IIIa: 197 (39,4 por ciento) (N0: 84; N1: 2; N2: 111); IIIb 23 (4,6 por ciento) (N0: 12; N2: 11), y IV: 7 (1,4 por ciento) (N0: 4; N2: 3). Se utilizó la estadificación de 1997 para la comparación de la supervivencia entre los tumores Ia y Ib, y IIb y IIIa. Resultados: Transcurridos entre 2 y 17 años, 141 pacientes (28 por ciento) permanecen vivos, 26 (5 por ciento) perdidos y 333 han muerto. Mortalidad intraoperatoria: 2 (0,4 por ciento); postoperatoria: 36 (7,2 por ciento). De los 462 pacientes restantes, 295 fallecieron por diversas causas: metástasis 130 (44 por ciento), recidiva 81 (27 por ciento), causas funcionales 17 (6 por ciento), causas independientes 54 (18 por ciento), causas desconocidas 13 (4 por ciento). Supervivencia a 5 y 10 años: global 36 y 26 por ciento; histología: carcinomas epidermoides 36 y 26 por ciento; adenocarcinomas 35 y 26 por ciento; estadios: I: 51 y 41 por ciento (Ia, 81 y 75 por ciento; Ib, 44 y 33 por ciento); IIIa: 24 y 15 (IIb de 1997: 27 y 17 por ciento; IIIa de 1997: 20 y 13 por ciento); de acuerdo con factor N: N0: 44 y 34 por ciento; N2: 17 y 8 por ciento (1986) y 17 y 11 por ciento (1997). Conclusiones: En este trabajo, la supervivencia hallada es concordante con otros estudios. La utilidad de la estadificación de 1997 se puede establecer en función de las diferencias de supervivencia hallada entre los estadios Ia y Ib y IIb y IIIa. Se aprecia una influencia de factores N y T, la histología y el estadio de la aparición de metástasis, así como el factor T en la de recidivas. (AU)

Quistes hidatídicos de mediastino: 8 casos / Hydatid cysts of the mediastinum: 8 cases

La hidatidosis en el ser humano es una enfermedad parasitaria producida por el crecimiento quístico de la larva de la tenia Echinococcus granulosus. Los órganos más afectados son el hígado y el pulmón. La localización mediastínica es muy rara y se han descrito aproximadamente 100 casos en la bibliografía. Presentamos 8 casos de quistes hidatídicos de mediastino (QHM) intervenidos en un período de 21 años. La distribución fue similar en ambos sexos. La edad osciló entre 10 y 74 años. En los QHM la sintomatología depende del tamaño, localización y afectación de estructuras próximas. La complicación más grave es la rotura del quiste y el paso del material hidatídico a la sangre, lo que puede provocar shock anafiláctico e incluso la muerte del paciente. Actualmente, la ecografía, la tomografía computarizada (TAC) y la resonancia magnética (RM) facilitan el diagnóstico. El tratamiento es la exéresis del quiste y de la periquística. Se debe sospechar QHM en las lesiones quísticas mediastínicas en países de alta incidencia de hidatidosis (AU)

Evaluation of six serological tests in diagnosis and postoperative control of pulmonary hydatid disease patients.

Latex agglutination (LA), passive hemagglutination (PHA), immunoelectrophoresis (IEP) and specific IgE, IgM, IgG enzyme-linked immunosorbent assay (ELISA) tests for diagnosis and postoperative follow-up of 79 patients with surgically confirmed pulmonary hydatidosis were evaluated. Specific IgG ELISA was the most sensitive test (83.5%) and the least sensitive tests were specific IgE ELISA (44.3%) and IEP (50.6%). The specificity obtained for all the serologic test was above 97% in all cases. The greatest number of false positives in all tests (except IEP) occurred in patients with Taenia saginata and Taenia solium cysticerci infestations and in patients with lymphoma and leukemia. Specific IgG ELISA demonstrated the highest negative predictive value (93.8%). No statistically significant differences (p > 0.050) were found in the sensitivity of the tests when patients with only one cyst and patients with various cysts were compared. Considering only the patients without relapse, the percentage of seropositive patients increased in all tests at 1 and 3 months after surgery. After that time the percentage of seropositive patients decreased. At 48 months after surgery all patients without relapse became negative in IEP, specific IgE ELISA, and specific IgM ELISA. The antibody titers in all seropositive patients increased during the 3 months after surgery. From these 3 months onward, antibody levels decreased in all serologic tests studied in the group of patients without relapse. The patients who had relapses during the first year after surgery presented persistently elevated antibody titers in all postoperative sera. The antibody titers of the patients who relapsed between the third and fourth years after surgery decreased progressively the third month after surgery, and increased in the serum obtained at the moment of relapse diagnosis. Our results show that persistence of elevated antibody titers in patients with pulmonary hydatidosis in the year after surgery or titer increase after a progressive decrease are indicative of relapse or reinfection.

Open-window thoracostomy and thoracomyoplasty to manage chronic pleural empyema.

BACKGROUND: The purpose of this study is to report our 15-year experience treating chronic empyemas after pulmonary resection and tuberculosis. METHODS: Open-window thoracostomy and thoracomyoplasty were used to treat 40 patients with chronic pleural empyema characterized by residual empyematic cavity, bronchopleural fistula, and persistent pleural infections that were secondary to tuberculosis (n = 22) or pulmonary resection (n = 18). Between 2 and 7 months after thoracostomy, thoracomyoplasty was performed to eliminate a persistent pleural cavity. In 2 patients with postpulmonary resection empyema and a large bronchopleural fistula, intrathoracic transposition of the latissimus dorsi flap and open-window thoracostomy were performed simultaneously to close the fistula. RESULTS: The pleural space was eliminated per primam intentionem in 21 of 22 patients with tuberculosis and in 14 of 18 with a postpulmonary resection empyema. Another myoplasty was performed in an additional 3 patients to eliminate the pleural space. During open-window thoracostomy, the latissimus dorsi muscle was preserved with minimal injury to the anterior serratus muscle. One patient died postoperatively. CONCLUSIONS: Successful treatment of chronic pleural empyema requires adequate timing of surgical procedures. Our two-procedure technique is relatively simple and safe.

Hemodynamic and cerebral repercussions arising from surgical interruption of the superior vena cava. Experimental model.

This study was designed to analyze the hemodynamic and cerebral repercussions arising from the surgical interruption of the superior vena cava. The experiments were carried out in 12 mongrel dogs under two different conditions: with shunt (group A, n = 6) and without the installation of a shunt (group B, n = 6). The period of occlusion was 35 minutes. The right atrium pressure, pulmonary arterial pressure, and aortic pressure are not significantly modified in the two groups. The intracranial pressure had an important correlation with the central venous pressure (r2 = 0.8572). In group B, the intracranial pressure had a sharp increase between the basal period (6.9 +/- 1.47 mm Hg) and the clamping superior vena cava (17.2 +/- 1.05 mm Hg), accentuated with the interruption of the azygous vein (32.2 +/- 0.7 mm Hg). In group A, the use of a shunt avoided this alteration during clamping of the superior vena cava (6.8 +/- 2.0 mm Hg) and the azygous vein (8.0 +/- 2.24 mm Hg). However, after removal of the clamps in group B, an elevated residual intracranial pressure was observed (21.1 +/- 3.33 mm Hg) in contrast to the central venous pressure, which returned to the basal values (4.4 +/- 0.7 mm Hg). The biomechanic findings of the volume-pressure curves (with Miller and Marmarou-Shapiro tests) and the cerebral necropsy showed brain damage in group B, without the shunt. Three animals had areas of hemorrhagic infarction. Histologic study demonstrated signs the incipient vasogenic edema. In group A, all findings were compatible with the normal. In conclusion, these results suggest the importance of shunting the blood in those cases of a nonobstructed superior vena cava because the clamping and reconstruction produce hemodynamic compromise and brain damage.

[Etiologic considerations regarding superior vena cava syndrome]. / Consideraciones etiológicas sobre el síndrome de vena cava superior.

Literature review indicates a change in the etiological trends of the Superior Cava Syndrome. While during the first half of the twentieth century, aortic aneurysms, malignant tumors and mediastinitis were its main causes, nowadays talking about obstruction of the superior cava is the same than talking about obstruction secondary to a malignant cause (85-90%). Benign affections account for 10-15% and, among them, intravenous iatrogenic foreign bodies constitute a new chapter as cause of thrombosis. Given that small cells simplex carcinoma is the most common cause of SCS and given that benign processes or other chemosensitive tumors can be present, it is currently necessary to secure the histologic diagnosis before starting the treatment.