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We present a retrospective series of 27 nasopharyngeal carcinomas, selected from those attended at Ramón y Cajal Hospital between 1977 and 1996, with the aim of review the role of the study of Epstein-Barr virus in the diagnostic process of nasopharyngeal carcinoma. Twenty-seven patients, ranging from 14 to 81 years, with an average age of 50 years were selected. Male/female ratio was 1,7. All but one case were Caucasian. A neck mass was the first symptom in 40% of cases, with a mean diagnostic delay of 17 months. Only 8 cases (23%) did not exhibit neck nodes at the moment of diagnosis. CT and MRI were essential to establish staging: 5 stage I, 7 stage II and 15 stage IV, due to regional extension and/or bone erosion. Radiotherapy was employed in all cases, helped by chemotherapy in 20% of them. With a mean follow-up of 62 months, 5-years survival was 32% (IC 14,06-52,09). Of 27 cases of nasopharyngeal carcinoma 4 were differentiated (type I), 2 moderately differentiated (type II) and 22 undifferentiated (type III). While LMP-1 was only expressed by 41% of cases, PCR detected Epstein-Barr virus genome in 26 cases (96%) and in situ hybridization for EBERs was positive in all cases. Thus, all nasopharyngeal carcinomas were related to Epstein-Barr virus. Expression of LMP-1 seemed to worse the prognosis of nasopharyngeal carcinoma.

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The aim of this study was to define the number of pleural biopsy samples necessary for optimum diagnostic performance and determine to what extent they are complementary. Eighty-four closed pleural biopsies were performed in our department between June 1996 and January 1998 on 55 males and 29 females with an average age of 64.4 +/- 16.7 years. The study of the pleural fluid included: pH, biochemical testing of pleura/serum (proteins, lactate dehydrogenase, glucose, cholesterol, triglycerides, albumin and adenosine deaminase), haemogram, cytology and microbiological testing (Gram-staining, aerobes, anaerobes and mycobacteriae cultures). The biopsies were performed using a Cope needle, with a total of five biopsies for each patient: four for pathological examination (taken numerically in the order in which they were performed: D1, D2, D3 and D4) and one for microbiological testing. In those cases in which the diagnosis was uncertain or effusion persisted, a thoracoscopy or thoracotomy was performed. There were no significant differences in the diagnostic yield of each individual sample (D1, D2, D3 and D4), but there were differences in the sum of the samples, depending on the number of biopsies performed.This was true for total group and the group with carcinomas, but not for the group with tuberculosis. The increase in diagnostic yield with the number of biopsies was more remarkable in the carcinoma cases, where it increased by 35% when four biopsies were performed (54% with one biopsy versus 89% with four biopsies, P < 0.002). In conclusion, the diagnostic yield increased with the number of biopsy samples in the total group and the group with malignancy but not in the group with tuberculous effusions. The best diagnostic performance for malignant pathology was obtained with four samples. In pleural tuberculosis, the diagnostic yield did not increase with more biopsy samples. One high quality sample should be enough to obtain a diagnosis.

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The association of bronchiolitis obliterans organizing pneumonia (BOOP) with insulin-dependent diabetes mellitus (IDDM) and Evans syndrome (autoimmune pancytopenia) has not been reported previously. We describe the case of a 4-year-old child diagnosed with IDDM and Evans syndrome who presented malaise, fever and nonproductive cough for several months. The chest radiograph revealed several patchy alveolar opacities with peripheral and bilateral distribution and multiple hilar and mediastinal adenopathies. An open lung biopsy established the diagnosis of BOOP. During the follow-up over the next 7 years, the patient had chronic relapses in spite of corticosteroid treatment and developed restrictive lung disease.

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We present a retrospective series of 16 cases of undifferentiated squamous carcinoma in cervical metastatic nodes of unknown primary. We review their clinical and radiological evaluation in search of a possible primary tumor, their response to treatment and the eventual development of a primary tumor. Furthermore, we study the presence of Epstein-Barr virus in the metastases as a predictor of their nasopharyngeal origin, in relation to the finding of primary lesions in the nasopharynx during further follow-up of the cases.

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We report a patient with cirrhosis and hepatopulmonary syndrome. This syndrome is an entity characterized by anomalies in the arterial oxygenation in patients with chronic hepatic disease and/or portal hypertension and demonstration of pulmonary vasodilatation (PV) in absence of primary cardiac or pulmonary disease. We show that the finding of PV with transthoracic contrast enhanced echocardiography (TCEE) in the diagnosis of PV is real and corresponds to direct measurement of capillary diameter by morphometry.

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Histological demonstration of sarcoidosis affecting the paranasal sinus is rare in the clinical practice. In this paper, we present a case with a diagnosis of lupus pernio with extensive involvement of the nasal cavity and the right maxillary sinus. Long before being diagnosed as having lupus pernio, the patient had had a long history of nasal obstruction and had been treated surgically without success on 3 occasions. The literature on the topic is reviewed.

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Laryngeal tuberculosis, although the most common granulomatous disease of the larynx, is a rare form of extrapulmonary tuberculosis, never reported in immunosuppressed allograft recipients. We present two cases of laryngeal tuberculosis in renal transplant patients and a review of the literature. Two women, a 29-year-old and a 60-year-old, each more than 9 years after their cadaveric renal allograft, presented with a 2-week febrile illness with hoarseness and dysphagia, and both were found to have laryngeal tuberculosis by direct laryngoscopy. Although both radiographs were unremarkable, both patients had sputum positive for acid-fast bacilli that subsequently grew Mycobacterium tuberculosis. Clinical response promptly followed institution of isoniazid, rifampicin, and pyrazinamide in each case, although both required threefold increases in daily cyclosporin A dosage to maintain therapeutic levels.

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Bronchocentric granulomatosis (BG) is an uncommon pathologic finding characterized by necrotizing granulomas centered on bronchi and bronchioles. We report a nonasthmatic patient with a BG in whom no etiologic agent was identified. We discuss the different pathogenic mechanisms proposed in this group of patients.

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The case of a 70-year-old male with lymphoblastic leukemia is reviewed, who presented the rare and almost always fatal complication of pulmonary mucormycosis, but who was treated satisfactorily with amphotericin B and surgery. The risk of massive hemoptysis in the course of mucormycosis that invades the lung vessels, makes us believe that surgery is an essential part of the management of this disease. It is suggested that the patient be operated as soon as the diagnosis is obtained, as we did in our case, to avoid other risks in combined management with amphotericin B.

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We describe a 22-year-old man with a bone mass in the middle and lower third of the sternum with no signs of respiratory deterioration. After biopsy by incision established a diagnosis of hemangiosarcoma, appropriate resection and repair of the thoracic wall was scheduled. An adequate amount of tissue for analysis must be obtained to allow choice of a surgical technique that will assure the best prognosis in malignant tumors of the sternum and assignation of the most appropriate mechanical ventilation procedure.

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Fourteen cases are revised of bronchiolalveolar carcinoma (BAC) diagnosed in our institution from 1980 to 1992. The time interval from clinical manifestations to diagnosis was 5.13 +/- 6.01 months. The incidence of smokers in the population was 42.8%. Clinical findings were non-specific, except in three cases, consisting in bronchorrhea with more than 100 ml. The most frequent site of the tumor was bilateral and diffuse (71%), mainly in nonsmokers. Endoscopic findings suggested a likely malignancy in 21% of cases. Transbronchial biopsy was the most sensitive diagnostic method. Histological confirmation was obtained in 85% of cases. To note the possible association with Epstein-Barr virus (EBV), as demonstrated by the findings of EBV genome in the cases when this study was performed.

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We describe a case of Horner's syndrome secondary to a thymic cyst. Following successful surgical removal of the cyst, the patient's symptoms resolved. To the best of our knowledge, a similar case has not been reported.

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We present a case with gigantic carcinosarcoma (the largest described to date, diameter of 20.5 cm), located in the right upper lobe, in which the discordance between the brief clinical course with limited symptoms and its large size were remarkable. Preoperative diagnosis by bronchial biopsy, as used to be the case in this malignancy, was incorrect (epidermoid), and the define histological characterization was made by thoracotomy. The controversy about pathology, diagnosis and treatment of the carcinosarcoma is discussed.

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A 22-year-old man with a history of repeated whole blood transfusions since the age of 7, was admitted to the hospital for dyspnea and blood-stained sputum. A complete blood cell count, a test for reticulin antibodies, an x-ray film of the chest, a transbronchial biopsy and a biopsy of the small bowel were performed and the results confirmed the diagnosis of IPH, associated with CD. The patient has been asymptomatic for four years on a gluten-free diet. In cases of IPH, it may be important to investigate the coexistence of CD because treatment of the latter could lead to a better prognosis.

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A primary liposarcoma of the lung occurred in an 18 year old girl, the first to be reported in anyone under 40. Though rare, intrathoracic liposarcoma should be included in the differential diagnosis of pleural effusion in younger patients.

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