Extrapulmonary Localization Research Articles

Aspects cliniques et thérapeutiques de la tuberculose chez l'enfant et l'adulte

Tuberculosis is a chronic bacterial infection caused by Mycobacterium tuberculosis. It is an important cause of morbidity and mortality worldwide. Pulmonary tuberculosis is the leading focus of infection and also the mode of transmission. But whatever pulmonary or extra-pulmonary localization, diagnosis and treatment follow common principles. Although infection can be suspected on clinical signs, tuberculin skin test, radiological examination or histology, demonstration of bacilli in bacteriological samples is the keystone of the disease diagnosis. In children, tuberculosis is always due to recent contamination. The risk of developing severe disease is higher in infants, and teenagers may have a rapidly progressive form. The treatment of tuberculosis is well defined for drug-susceptible bacilli. Standardized antituberculosis chemotherapy consists in a 6-month regimen based on combination of effective drugs. Its success depends on total compliance. Defaults lead to treatment failure and the emergence of drug-resistant bacilli. Completely supervised regimens have been developed with success. A number of medical conditions may alter immune responsiveness and increase tuberculosis susceptibility. Management of treatment for multi-drug resistant tuberculosis or immunodeficiency-related tuberculosis is complex. Drug toxicities should be monitored, with the greatest concern for hepatitis. Prophylactic measures are essential for decreasing the spread of infection.

Tuberculosis in Turkish patients with systemic lupus erythematosus: increased frequency of extrapulmonary localization.

The objective was to investigate the frequency and characteristics of tuberculosis (TB) in patients with systemic lupus erythematosus (SLE). We reviewed the charts of 556 patients with SLE who were followed up between 1978 and 2001 in our lupus clinic. Patients who developed TB after the diagnosis of SLE were identified (SLE/TB+). Ninety-six consecutive patients with SLE who did not develop TB during the follow-up were evaluated as a control group (SLE/TB-). Clinical, laboratory and management characteristics of these two groups of patients were recorded according to a predefined protocol, and compared. Of the 556 patients evaluated, 20 patients (3.6%) with TB were identified. Nine of the 20 patients (45%) had extrapulmonary TB (vertebral involvement in three patients, meningeal in two, and joint and soft tissue in four). Arthritis and renal involvement were significantly high in the SLE/TB+ group (P = 0.045, P = 0.009, respectively). The mean daily dose of prednisolone before the diagnosis of TB and the cumulative dose of prednisolone were significantly higher in the SLE/TB+ group compared to the SLE/TB- group (27 +/- 22 g versus 16 +/- 16 g, 24 +/- 45 mg versus 11 +/- 8.5 mg, respectively). In conclusion, we found an increased frequency of TB infection and a high prevalence of extrapulmonary TB in a large cohort of SLE patients. The mean daily dose of prednisolone before the diagnosis of TB and the cumulative dose of prednisolone, which possibly related to disease severity, were important determinants for the increased risk of TB in these patients with SLE.

Gastrointestinal tuberculosis.

Gastrointestinal tuberculosis is defined as infection of the peritoneum, hollow or solid abdominal organs, and abdominal lymphatics with Mycobacterium tuberculosis organisms. Gastrointestinal tuberculosis is relatively rare in the United States and is the sixth most common extrapulmonary location. Populations at risk include immigrants to the United States, the homeless, prisoners, residents of long-term care facilities, and the immunocompromised. The peritoneum and the ileocecal region are the most likely sites of infection and are involved in the majority of cases by hematogenous spread or through swallowing of infected sputum from primary pulmonary tuberculosis. Pulmonary tuberculosis is apparent in less than half of patients. Patients usually present with abdominal pain, weight loss, fever, anorexia, change in bowel habits, nausea, and vomiting. The diagnosis is often delayed and is usually made through a combination of radiologic, endoscopic, microbiologic, histologic, and molecular techniques. Antimicrobial treatment is the same as for pulmonary tuberculosis. Surgery is occasionally required.

Giant Intrathoracic Extrapulmonary Hydatid Cyst Manifested As Unilateral Pectus Carinatum

Liver and lung are the most common sites of hydatid disease, but it can also be seen elsewhere in the body. Extrapulmonary intrathoracic location of the disease is rare. This case of giant intrathoracic extrapulmonary hydatid cyst manifested as unilateral pectus carinatum serves to illustrate that hydatid disease can produce various symptoms and that it may also exist in locations apart from lung and liver.

Giant Intrathoracic Extrapulmonary Hydatid Cyst Manifested As Unilateral Pectus Carinatum

Liver and lung are the most common sites of hydatid disease, but it can also be seen elsewhere in the body. Extrapulmonary intrathoracic location of the disease is rare. This case of giant intrathoracic extrapulmonary hydatid cyst manifested as unilateral pectus carinatum serves to illustrate that hydatid disease can produce various symptoms and that it may also exist in locations apart from lung and liver.

Resection of a branchiomeric paraganglioma at a rare extrapulmonary location

Thoracic paragangliomas are a rare cause of hypertension. We report the occurrence of a sporadic benign norepinephrine-producing branchiomeric paraganglioma in a 32-year-old man with paroxysms of hypertension. After localization by iodine 123–metaiodobenzylguanidine scintigraphy and magnetic resonance imaging, the paraganglioma was resected successfully below the right pulmonary artery through a right-sided posterolateral thoracotomy. The particular location was consistent with a branchiomeric paraganglioma in an extremely rare extrapulmonary location.

Outcome of multidrug-resistant tuberculosis in human immunodeficiency virus-infected patients.

Among 324 cases of culture-proven tuberculosis from 1988 to 1996 in a hospital in Milan, Italy, 90 (27.8%) were due to Mycobacterium tuberculosis strains resistant to isoniazid and rifampin. Sixty-one of 69 isolates tested had identical restriction fragment length polymorphism patterns. The prevalent strain tested susceptible only to ethionamide and was also resistant to ethambutol, streptomycin, cycloserine, amikacin, kanamycin, terizodone, ofloxacin, rifabutin, rifapentin, and KRM 1648. The median survival time was 94 days. Multivariate analysis showed a trend toward better outcome in the period 1994-1996 (hazard ratio, 4.16; P<.001), and extrapulmonary localization of tuberculosis was the only other independent predictor of a negative outcome (hazard ratio, 2.1; P = .019). The delay from symptoms to beginning of therapy did not seem to be a determining factor in survival time. Standard antituberculosis therapy with four drugs (isoniazid, rifampin, ethambutol, and pyrazinamide) had a higher efficacy than did other regimens with fewer drugs but without a statistically significant difference.

Réémergence de la tuberculose et précarité socioéconomique

Infectious diseases are the main cause of morbidity and mortality among socially deprived patients. In 1988-89 the secular decrease in tuberculosis stopped in France as in other industrial countries, and new emergence of the disease has been observed since then, especially among socially deprived patients. Clinical characteristics of tuberculosis remain classical among these patients, but advanced, even historical forms of the disease, are often observed, with frequent extra-pulmonary localizations. Diagnostic and therapeutical procedures must be adapted to the patients' living conditions, including their education and social support. Directly observed therapy is rarely used in France, but the development of adequate strategies for effective primary care management with adapted cost/efficiency ratios in the respect of human rights is essential. Early tracking down of patients lost from follow-up and detailed evaluation of treatment results and social care remain priorities.

Cutaneous Bronchogenic Cyst: Delineation of a Poorly Recognized Lesion

Abstract: Bronchogenic cyst is a benign congenital developmental abnormality of the embryonic foregut. The most common extrapulmonary location of these lesions is the mediastinum. Over 50 cases of cutaneous bronchogenic cyst were reported in the English literature. Nevertheless, it is poorly recognized by clinicians and is sometimes confused with branchial cyst. In almost all cases the diagnosis is established by histopathologic examination. We report an unusual clinical presentation of bronchogenic cyst in a 4‐year‐old girl and review the literature. This lesion is four times more common in males than in females. The most common location is the suprasternal notch, followed by the presternal area, neck, and scapula. The characteristic histologic findings are a ciliated pseudostratified epithelial lining with the presence of smooth muscle cells, goblet cells and, less frequently, cartilage. Our review of the clinical and histologic features of all reported cases further delineates this entity and may facilitate its clinical diagnosis. Bronchogenic cyst should be included in the differential diagnosis of congenital cystic and nodular skin lesions on the upper chest, upper back, and neck.

Resultados del tratamiento quirúrgico del carcinoma broncopulmonar no microcítico en estadio III-A

To analyze results and survival after surgical treatment of various stage III-a subgroups. We reviewed 748 case histories of patients with non-small cell bronchopulmonary carcinoma between January 1970 and December 1991, selecting 121 patients who underwent resection of stage III-a tumors, according to the pathologist's report. Overall survival at 3, 5 and 10 years was 26, 16 and 12%, respectively. The subgroup with better response to treatment was that of patients with T-3 tumors (peripheral), N-0, with survival of 21% at five years. If peribronchial and/or hilar (N-1) nodes were affected, survival was slightly less (16%). If mediastinal adenopathy (N-2) was present, there were no survivors after 3 years. In this same T-3 group, but with neoplastic invasion of the pericardium, mediastinal pleura or principal bronchi, survival after 3 years was nil. The survival rates of peripheral T-3 patients undergoing block resections were 27, 20 and 17% at 3, 5 and 10 years, respectively, 16% at 2 years and 0% at 5, for those with extrapleural resection. Patients with the poorest response to treatment were those with mediastinal nodes (N-2), in whom survival was 17, 12 and 8% at 3, 5 and 10 years, respectively. In the 34 patients received postoperative irradiation of the mediastinum, survival was 16% higher. The survival rates after 5 years were similar for epidermoid carcinoma (18%) and adenocarcinoma (14%). The results of surgical treatment are variable and depend on several factors: extension and extrapulmonary location of lesions, surgical technique used for T-3 tumors and histological type.

Pregnancy and tuberculosis: influence of treatment on perinatal outcome.

The objective of this article is to evaluate the impact of tuberculosis (TB) on perinatal outcome in a cohort of 25 pregnant women with TB treated at the National Institute of Perinatology (Mexico, City) from March 1990 to September 1995. They were compared with a cohort of normal pregnant women; both cohorts were matched by age, gestational age, and socioeconomic status. For purposes of analysis, patients with TB were further stratified into two groups: one included 9 women who started treatment either before or at the beginning of pregnancy, and the other constituted 16 women who started treatment in either the second or third trimester of gestation. Thirteen women (52%) had pulmonary TB, 7 (28%) had renal infection, and the rest of patients had diverse extrapulmonary localization of the infection. Obstetrical morbidity and neonatal mortality were significantly higher in pregnant women with TB who started treatment late in pregnancy. Perinatal morbidity was similar in pregnant women receiving antituberculous drugs early during pregnancy to that in uninfected women. We conclude that TB represents a risk factor for pregnancy. Early treatment of the disease during gestation reverts its negative impact on perinatal outcome.

Unusually located hydatid cysts: Intrathoracic but extrapulmonary

Hydatid cyst disease is still a problem in Turkey, as well as in many other places in the world. Extrapulmonary location of the disease in the thorax is very rare, and surgical procedures can be considered that differ from those used for pulmonary hydatid cysts. We reviewed retrospectively our experience in the surgical treatment of 22 patients with intrathoracic, extrapulmonary hydatid cysts. In our department, 297 patients with thoracic hydatid cysts were managed surgically in the last 14 years, in 22 (7.4%) of whom the cysts were localized extrapulmonarily in the thorax. The locations of these hydatid cysts were a fissure, the pleural cavity, chest wall, mediastinum, myocardium, and diaphragm. Total resection was chosen as the surgical procedure in all patients except 4 (18.2%), 1 of whom had cystectomy and capitonnage for cardiac hydatid cyst and 3 of whom had cystectomy and local curettage for cysts located in the chest wall. Empyema developed postoperatively in 1 case (4.5%) with a cyst in the fissure. The follow-up period was 1 year, and there were no deaths. Hydatid cyst may be found in many different sites, including extrapulmonarily in the thorax, and bearing this in mind will facilitate planning of the operation.

La pneumocystose pulmonaire au cours du sida: analyse rétrospective de 80 cas documentés (1985–1993)

Eighty initial episodes of HIV-associated Pneumocystis carinii pneumonia (PCP) diagnosed at Bordeaux hospital between 1985 and 1993 are reported (57 were men and 23 women). PCP revealed HIV infection in 29 patients (36%). Others cases were patients with poor medical follow up (10%), with a CD4+ lymphocyte count above 200/mm3 at last follow-up (9%), non compliant with PCP prophylaxis (9%), or using aerolized pentamidine (AP+) (20%). The main clinical symptoms were fever (90%), dyspnea (68%), non productive (63%) and productive (17%) cough. Radiographic infiltrates were purely interstitial (59%), acinar and interstitial (25%), purely acinar (5%) and absent (11%). Thirty-eight percent of AP+ had upper lobe preferential involvement and 13% a pleural effusion. In all cases, Pneumocystis carinii was detected in bronchoalveolar lavage. Extrapulmonary localizations of pneumocystosis were noticed (eye, liver, spleen, ascitis) in two AP+. Mean CD4+ count was 54/mm3 in patients not having received aerolized pentamidine (AP-) and 22/mm3 in AP+. P24 antigenemia was positive in 53% (AP-) and 88% (AP+). PaO2 LDH and albuminemia were similar in both groups. Antimicrobial therapy (Cotrimoxazole in 91% of the cases) was combined with corticosteroids in 45% and mechanic ventilation in 19%. After 30 days of follow-up, 17 deaths were observed (21%) and 14 attributed to PCP: mortality was worse in AP+ (31%) than in AP- (19%). The main conclusions of our study are the followings: HIV related PCP is still in 1995 frequent and severe; atypical features should not rule out diagnosis; preventive measures are neither sufficient nor efficient. PCP remains in 1995 a priority in HIV related public health and therapeutical research.

Le nouveau visage de la tuberculose dans le contexte de l'association Tuberculose-VIH à Abidjan, Côte d'Ivoire

This article reviews the main clinical aspects and progression of HIV-related tuberculosis in Abidjan. HIV prevalence in tuberculosis patients is high, estimated at 46.2% in 1992, with a clear predominance of HIV-1 over HIV-2. More than 61% of co-infected tuberculous patients meet the WHO's clinical definition of AIDS (the Bangui definition) at the time of diagnosis of tuberculosis. This rates falls to 46–51% when cough is excluded from the definition. On X-ray, the signs of pulmonary tuberculosis in co-infected tuberculous patients are atypical in the advanced stages of HIV infection, when extra-pulmonary localization, mainly mediastinal adenopathy, is frequent. Short-course chemotherapy consisting of 2 months' unsupervised daily treatment with rifampicin/isoniazid/ pyrazinamide, followed by 4 months of a daily combination of rifampicin/isoniazid, applied in the Ivory Coast since 15 July 1985, has proved successful in HIV-associated tuberculosis, with treatment effectiveness rates of more than 90%.

Tuberculosis in the autopsy material: analysis of 1500 autopsies performed between 1972 and 1991 in the institute of tuberculosis and chest diseases, Warsaw, Poland

The present study is based on 1500 autopsies done in the Institute of Tuberculosis and Chest Diseases Warsaw, Poland during the years 1972-81 and 1982-91. To assess the correctness of tuberculosis diagnosis before death in the above mentioned time periods. The autopsy reports were examined for the diagnosis of active tuberculosis proved by microscopy. The form and localisation of tuberculosis was assessed. The postmortem diagnosis was compared with clinical diagnosis. In those cases in which tuberculosis was not recognised before death the possible causes of this failure were analyzed. Active tuberculosis was found in 119 cases, 7.9% of all autopsies. It was localised in the lungs exclusively in 72 cases, in lungs and in extrapulmonary sites in 45 and in extrapulmonary sites only in 2. The frequency of active tuberculosis was the same for the two periods under evaluation. Tuberculosis was however not recognised before death in a much higher proportion of cases in 1982-92 (54%) than in 1972-81 (24%). The main cause of diagnostic failure was connected with atypical localisation of lesions on chest X-ray and with dissemination outside the lungs. Previous tuberculosis was a factor which facilitated the diagnosis of tuberculosis. In parallel with the decline of tuberculosis incidence in Poland, many doctors lack experience in diagnosing this disease, especially in cases with atypical X-ray presentation and with extrapulmonary localisation. This may lead to late or even very late diagnosis and have a significant impact on the epidemiological situation.

"The sarcoidosis map": a joint survey of clinical and immunogenetic findings in two European countries.

We pooled immunogenetic data obtained in independent studies in two European populations (Italian and Czech) of patients affected by sarcoidosis. Correspondence analysis was used to investigate the associations between clinical and immunogenetic data. Two hundred and thirty-three patients were enrolled in the study, of which 126 were from the Czech Republic and 107 from Italy. Using a common protocol, we examined each patient for sex, age of disease onset, roentgenologic stage, extrapulmonary spread, and clinical course. One thousand and ten healthy individuals, HLA typed for class I and II serologic polymorphisms, served as controls. Findings that were essentially in agreement in both populations were: (1) a positive association of sarcoidosis with HLA-A1, B8, and DR3 markers, and a negative association with HLA-B12 and DR4; (2) a prevalence of HLA-DR3 and DR4 among females and of DR5 among males; (3) a relationship of B13 and B35 with early onset and of A30, B8, DR3, and DR4 with late onset of disease; (4) an association of B27 with sarcoidosis restricted to the lungs; (5) a relationship of A1, B8, B27, and DR3 to roentgenologic stage I and of B12 and DR4 to stage III; and (6) an association of HLA-DR3 with a good outcome. Population-restricted findings essentially concerned the alleles HLA-B13 and B22, the former being associated with the disease, male sex, early onset, extrapulmonary localization and relapse only in Czechs, and the latter to disease spread only in Italians. Our results seem to support the concept that immunogenetic background may at least partly account for the clinical heterogeneity of sarcoidosis.

Primary Pulmonary Alveolar Soft-part Sarcoma

Alveolar soft-part sarcoma rarely arises in the lung. Only two patients with tumors that were apparently primary in the lung have been documented in the literature. We report an additional case in a 25-year-old woman. The tumor formed a solitary 5 cm, solid mass in the left perihilar region. Clinical examination, radiologic studies, and follow-up examination of more than 1 year have failed to reveal tumor in an extrapul monary location. The light microscopic appearance was typical for alveolar soft-part sarcoma, and crystalloids were identified in the tumor cells by the periodic acid-Schiff stain and by electron microscopy, confirming the diagnosis. Immunocytochemical studies did not show evidence of myogenic, epithelial, or neuroendocrine differentia tion. Int J Surg Pathol 2(1):57-62, 1994

HLA Class I, II, and III Polymorphism in Italian Patients With Sarcoidosis

We studied the HLA polymorphisms (class I, II, and III) in 107 Italian patients with biopsy specimen-proven sarcoidosis in order to investigate the immunogenetic background of this disease. The mean age of onset of the disease was 36.08 +/- 12.4 years. Four patients (3.73 percent) were in radiologic stage 0, 38 patients (35.51 percent) were in radiologic stage I, 40 patients (37.38 percent) were in stage II, and 25 (23.36 percent) were in stage III. Thirty-eight patients (35.51 percent) had one or more extrapulmonary localization(s) of the disease. Positive association between sarcoidosis and HLA-B8 (chi 2 = 6.07, p = 0.0127, RR = 1.91) was confirmed. Regarding the age of onset of the disease, HLA-B35 was more frequent (chi 2 = 7.34, p = 0.0056, pc < 0.05, RR = 4.62) in patients with early onset of symptoms and/or signs, before the mean age of 36 years. With reference to the radiologic stage of the disease, HLA class II marker DR3 was more frequent in patients with stage I (chi 2 = 7.22, p = 0.0061, pc < 0.05, RR = 7.08). No significant relationship was found between sarcoidosis and HLA class III markers. These results seem to confirm an association of sarcoidosis with HLA classic genes and can sustain the hypothesis of a genetic heterogeneity of this disease.

Extrapulmonary localization of gallium in sarcoidosis.

This paper describes the spectrum of extrapulmonary localization of gallium in patients with sarcoidosis. The usefulness of Ga-67 scintiscans in detecting clinically occult lesions, in directing clinicians to accessible sites for biopsy, and in following the course of extrapulmonary sites of involvement with therapy is emphasized.

Esophageal Reflux as a Trigger in Asthma

Hiatal hernia with reflux has been found in 30 per cent of all asthmatics studied recently. In 24 patients, the hernia was repaired and the valve at the esophagogastric junction was restored. Eighteen of these patients have been followed for more than six months. In all but one, correction of this mechanical defect resulted in improvement or complete relief. In the asthmatic patient, organic trigger mechanisms may exist. An intrapulmonary location is more common, but an extrapulmonary location should not be overlooked. Spillover of material from the gastrointestinal system to the bronchial system may be at fault.