[Two patients with a rare manifestation of amyloidosis in the respiratory system]. / Twee patiënten met een zeldzame manifestatie van amyloïdose in het respiratoire systeem.

Amyloidosis of the respiratory tract was diagnosed in 2 patients. The patients were men, 62 and 55 years ofage. The first patient presented with dyspnoea and diffuse parenchymal lung abnormalities. There was a rapidly progressive obstructive lung function disorder and a severe diffusion impairment. The second patient had haemoptysis due to tracheobronchial amyloidosis. Amyloidosis of the respiratory system is rarely diagnosed. Nearly all cases ofclinically relevant respiratory amyloidosis are due to light chain amyloid (AL amyloidosis). The described diffuse lung parenchymal abnormalities are a manifestation of systemic AL amyloidosis. On the other hand, tracheobronchial amyloidosis is a disorder which usually remains localised in the airways. Systemic AL amyloidosis may be treated with chemotherapy or stem cell transplantation. It is unknown whether this treatment leads to a decrease of pulmonary function abnormalities. Tracheobronchial amyloidosis can be treated by endobronchial therapy. Often this treatment is required repeatedly. The first patient died 2 months after diagnosis due to pneumonia. The second patient was treated with endobronchial argon plasma coagulation and diathermy and has been symptom-free for 3 years since.

Therapeutic bronchoscopy with immediate effect: laser, electrocautery, argon plasma coagulation and stents.

Minimally invasive diagnostic and therapeutic approaches in medicine have been applied for a more selective and tailored approach to reduce patients' morbidity and mortality. The efficacy of interventional pulmonology for palliation of patients with central airways obstruction has been established and its curative potential for intralesional treatment of early cancer has raised great interest in current screening programmes. This is due to the fact that surgical resection and systemic nodal dissection as the gold standard is relatively morbid and risky, especially when dealing with individuals with limited functional reserves due to smoking-related comorbidities, such as chronic obstructive pulmonary disease. Furthermore, such comorbidities have been proven to harbour early stage lesions of several millimetres in size without involvement of nodal disease that may be amenable to local bronchoscopic treatment. Therefore, the success of minimally invasive strategies for palliation and treatment with curative intent strongly depends on the diligent identification of the various factors in lung cancer management, including full comprehension of the limits and potential of each particular technique. Maximal preservation of quality of life is a prerequisite in successfully dealing with individuals at risk of harbouring asymptomatic early lung cancer, to prevent aggressive surgical diagnostic and therapeutic strategies since overdiagnosis remains an issue that is heavily debated. In the palliative setting of alleviating central airway obstruction, laser resection, electrocautery, argon plasma coagulation and stenting are techniques that can provide immediate relief, in contrast to cryotherapy, brachytherapy and photodynamic therapy with delayed effects. With curative intent, intraluminal techniques that easily coagulate early stage cancer lesions will increase the implementation of interventional pulmonology for benign and relatively benign diseases, as well as early cancer lesions and its precursors at their earliest stage of disease.

Granular cell tumors of the tracheobronchial tree.

OBJECTIVE: To describe the population-based incidence and clinical characteristics of granular cell tumors of the tracheobronchial tree. METHODS: All newly registered tracheobronchial granular cell tumors in the Dutch Network and National Database for Pathology for 10 consecutive years (1990-1999) were identified. The histologic diagnosis was confirmed and patient demographics, management, and follow-up data were analyzed. RESULTS: Thirty-one tumors were registered in 30 patients (12 male patients and 18 female patients; mean age 51 years; range 11-84) in a population of approximately 15 million. Tracheal tumors were identified in 11 patients and bronchial tumors in 19 patients (1 patient had 2 tumors). About half of the patients were asymptomatic. In the majority of the patients (61%) the granular cell tumor was an incidental finding during workup for lung carcinoma. Tracheal granular cell tumors were more frequent in women, whereas bronchial granular cell tumors showed no sex predilection. In the lung these tumors occurred more often in the upper (10 tumors) than in the lower lobes (3 tumors). There was no preference for either side. Four patients with tracheal and 4 with bronchial granular cell tumors were treated with surgery and remain in complete remission. Four bronchial granular cell tumor patients were treated locally with neodymium/yttrium-aluminum-garnet laser or electrocautery and are in complete remission or have stable residual disease. In 3 patients no residual disease was found after biopsy. Of all granular cell tumor patients 17 received no treatment for a variety of reasons, but none of these patients died in the follow-up period because of the granular cell tumors. CONCLUSION: Tracheobronchial granular cell tumor is a benign tumor with a good prognosis. In symptomatic patients surgical intervention is the first choice of treatment, but local treatment is a reasonable option and gives successful results.

Tracheobronchial stenting in the terminal care of cancer patients with central airways obstruction.

STUDY OBJECTIVES: To evaluate the palliative benefit of stent insertion in a group of patients with central airways obstruction due to terminal cancer. DESIGN: Retrospective analysis of the symptomatic score of patients immediately after stent insertion, and questionnaires completed by the general practitioner (GP) after the patients died at home. SETTING: Academic hospital, tertiary referral center for interventional bronchoscopy. PATIENTS AND METHODS: Fourteen patients with imminent suffocation due to major obstruction of the central airways, caused by end-stage esophageal cancer (n = 5) and non-small cell lung cancer (n = 9), were referred for stent insertion. All prostheses were placed within 24 h after hospital admittance. Patients were then asked whether their symptoms had improved. After the patients died, a questionnaire was sent to each patient's GP. RESULTS: All patients expressed immediate benefit after stenting. The average length of survival after stent insertion was 11 weeks (range, 0.5 to 34 weeks). Two patients died within 1 week in our hospital after stent placement. In the remaining 12 patients, the GP considered stent insertion in 7 patients to be worthwhile, no judgment was made in 4 patients, and stent insertion in 1 patient was regarded as futile. CONCLUSION: Despite terminal disease and the fact that, in our country, patients may legally refuse any treatment and formally ask for euthanasia, the palliative benefit of stent placement should always be considered. All patients had immediate symptomatic relief afterwards. Retrospectively, the GPs responsible for terminal care at home still considered stent insertion worthwhile in 58% (7 of 12 patients) of cases. Stent placement should always be considered as part of the treatment of terminal cancer patients with imminent suffocation.

Autofluorescence bronchoscopy improves staging of radiographically occult lung cancer and has an impact on therapeutic strategy.

BACKGROUND: The ability of conventional CT scans and fiberoptic bronchoscopy to localize and properly stage radiographically occult lung cancer (ROLC) in the major airways is limited. High-resolution CT (HRCT) scanning and autofluorescence bronchoscopy (AFB) may improve the assessment of ROLC before the most appropriate therapy can be considered. PATIENTS AND METHODS: We prospectively studied 23 patients with ROLC, who were referred for intraluminal bronchoscopic treatment (IBT) with curative intent. Additional staging with HRCT and AFB was performed prior to treatment. Twenty patients were men, 9 patients had first primary cancers, and 14 patients had second primary cancers or synchronous cancers. RESULTS: HRCT scanning showed that 19 patients (83%) had no visible tumor or enlarged lymph nodes. With AFB, only 6 of the 19 patients (32%) proved to have tumors < or = 1 cm(2) with visible distal margins. They were treated with IBT. In the remaining 13 patients, abnormal fluorescence indicated more extensive tumor infiltration than could be seen with conventional bronchoscopy alone. Six patients underwent radical surgery for stage T1-2N0 (n = 5) and stage T2N1 (n = 1) tumors. Specimens showed that tumors were indeed more invasive than initially expected. The remaining seven patients technically did not have operable conditions, so they were treated with external irradiation (n = 4) and IBT (n = 3). The range for the time of follow-up for all patients has been 4 to 58 months (median, 40 months). The follow-up data underscore the correlation between accurate tumor staging and survival. CONCLUSIONS: Our data showed that 70% of patients presenting with ROLC had a more advanced cancer than that initially diagnosed, which precludes IBT with curative intent. Additional staging with HRCT and AFB enabled better classification of true occult cancers. Our approach enabled the choice of the most appropriate therapy for each individual patient with ROLC.

Fluorescence bronchoscopy for early detection of lung cancer: a clinical perspective.

The conventional method of bronchoscopy has only a 30% sensitivity to detect early stage cancer in the central airways. For patients with positive sputum cytology who clearly harbor early cancers, repeat and lengthy sessions of bronchoscopies are required for accurate localization of these lesions. This leads to a significant delay in obtaining the diagnosis, postponing an appropriate treatment and reduces the chance for cure. There are valid reasons for improving the detection rate of early stage lung cancers. The number of individuals at risk forms a large population, the outcome of patients treated with early stage cancer has been shown to be better and bronchoscopic treatments, e.g. photodynamic therapy and electrocautery, are currently alternatives for surgical resection. Finding more early stage cancers by screening the population at risk and accurate staging to enable treatment at the earliest stage feasible, may improve the dismal prognosis of many patients. This article deals with the clinical background and current problems in early detection of lung cancer and discusses our expectations regarding new developments in bronchoscopy for early detection, accurate staging and treatment of lung cancer.

Bronchial intraepithelial neoplastic lesions in head and neck cancer patients: results of autofluorescence bronchoscopy.

To determine the efficacy of autofluorescence bronchoscopy for detection of bronchial intraepithelial neoplastic lesions in head and neck cancer patients, we analyzed data from head and neck cancer patients who underwent both white light bronchoscopy and autofluorescence bronchoscopy for the rates of detection of intraepithelial neoplastic lesions. The results of the histopathologic examination were compared with the bronchoscopic findings. The sensitivity for detection of intraepithelial neoplastic lesions was calculated. Eleven moderate dysplasias and 3 severe dysplasias were detected during 8 of the 42 bronchoscopic examinations (19%) in 6 of the 24 patients (25%). The sensitivities for white light bronchoscopy alone and for white light bronchoscopy combined with autofluorescence bronchoscopy for detection of intraepithelial neoplastic lesions were, respectively, 21% (3 of 14) and 57% (8 of 14). In short, bronchial intraepithelial neoplastic lesions were found in a considerable percentage of head and neck cancer patients. Use of autofluorescence bronchoscopy improved the detection of these lesions.

The curative potential of intraluminal bronchoscopic treatment for early-stage non-small-cell lung cancer.

Bronchoscopic treatment modalities such as lasers, electrocautery, cryotherapy, photodynamic therapy, and brachytherapy are potentially curative for patients with very-early-stage non-small-cell lung cancer (NSCLC) in the central airways. Previously, studies had primarily focused on the effectiveness of surgery, surgical bronchoplasty, and photodynamic therapy. The cure rate of intraluminal bronchoscopic treatment is strongly related to the patient's functional status and tumor stage. Intraluminal tumors are curable bronchoscopically when they are accessible to the fiberoptic bronchoscope, strictly intraluminal, and superficial with visible proximal and distal tumor margins. Early-stage cancer infiltrating deeper into the bronchial wall may already harbor metastases to the regional lymph nodes; hence, curative intraluminal treatment is not feasible. The use of new diagnostic tools (eg, high-resolution computed tomography, autofluorescence bronchoscopy, and endobronchial ultrasound) may improve staging to select the category of patients in whom intraluminal bronchoscopic therapy with curative intent is appropriate. An accurate intraluminal tumor staging will improve our ability to exploit the curative potential of many bronchoscopic techniques for complete tumor eradication in patients with very-early-stage intraluminal NSCLC in their central airways. The use of bronchoscopic treatment as a less morbid alternative than surgical resection will benefit patients most when tumor is detected at the earliest stage possible.

Endobronchial lipoma: a series of three cases and the role of electrocautery.

Lipomas are rare endobronchial tumors that may cause severe parenchymal damage due to bronchus obstruction and subsequent pneumonia. Therefore, accurate diagnosis and radical treatment are essential. We describe three cases of endobronchial lipoma. One patient presented with hemoptysis, two patients were initially diagnosed as COPD. They were all treated by electrocautery which achieved complete removal. We recommend electrocautery as an easy and cost-effective alternative for removal of intraluminal tumors including lipoma.

Outcome of bronchial carcinoma in situ.

INTRODUCTION: The proportion of patients with carcinoma in situ in whom invasive cancer will develop is not known. It is important for clinical decision making to know the outcome of these lesions. The same applies for studies assessing the effectiveness of chemoprevention treatment or endobronchial therapy. METHODS: The records of patients with a bronchial carcinoma in situ who had undergone autofluorescence bronchoscopic examinations at regular intervals during a follow-up period for at least 6 months were reviewed. Data were examined for the outcome of carcinoma in situ, and for the detection, course, and bronchoscopic findings of neoplastic lesions at other bronchial sites. RESULTS: Progression to carcinoma occurred in five of nine patients (56%) with a carcinoma in situ. Eight neoplastic lesions were detected at other sites in four of the nine patients (44%). In earlier biopsy specimens of two sites that later showed a severe dysplasia and a carcinoma, only normal epithelium was found. Biopsies had been performed at these sites because they were assessed as suspicious during autofluorescence bronchoscopy. CONCLUSION: The majority of sites showing a carcinoma in situ progressed to invasive carcinoma. A considerable portion of the patients had neoplastic lesions at other bronchial sites. The fluorescence pattern of the bronchial mucosa may reflect early changes that are not found at histopathologic examination, but which may progress to neoplastic growth.

Tissue effects of bronchoscopic electrocautery: bronchoscopic appearance and histologic changes of bronchial wall after electrocautery.

STUDY OBJECTIVES: To study tissue effects of bronchoscopic electrocautery (BE). DESIGN: In six patients with non-small cell lung cancer, a BE procedure was performed immediately before surgery. After patients were placed on ventilation, normal mucosa on different carinae was treated with a cautery probe (2-mm(2) surface area) at a power setting of 30 W with a variable time of application of 1 to 5 s. Bronchoscopic appearance of the treated area was documented photographically, and histologic changes of the bronchial wall were examined. SETTING: Bronchoscopy unit of a university hospital. MEASUREMENTS AND RESULTS: BE resulted bronchoscopically in whitening of the bronchial mucosa with crater-shaped lesions. After longer duration of BE application, deeper craters with more profound charring were seen. Histologic changes of the lesions showed craters containing a variable amount of necrotic tissue. In one case, thin subsegmental carinae were coagulated and measurements could not be performed. In the remaining five cases, microscopic findings revealed 0.2 +/- 0.1-mm necrosis after 1 s; 0.4 +/- 0.2-mm necrosis after 2 s; 0.9 +/- 0.5-mm necrosis after 3 s; and 1.9 +/- 0.8-mm necrosis after 5 s. A variable degree of tissue damage surrounding the necrotic tissue area was found. In one case, cartilage damage appeared after 3 s of coagulation, and extensive damage of the underlying cartilage was seen in four cases after 5 s of application. CONCLUSIONS: Superficial damage was obtained by short duration of BE (< or = 2 s), and longer duration of coagulation (3 s or 5 s) caused damage to the underlying cartilage. Bronchoscopic appearance after endobronchial electrocautery corresponded with the histologic changes.

High-resolution CT in patients with intraluminal typical bronchial carcinoid tumors treated with bronchoscopic therapy.

STUDY OBJECTIVE: To evaluate the extent to which high-resolution CT (HRCT) can predict the clinical outcome of bronchoscopic treatment with curative intent in patients with intraluminal typical bronchial carcinoid tumors. DESIGN: An observational study. SETTING: Bronchoscopy unit and radiology department of a university hospital. PATIENTS AND INTERVENTIONS: Eighteen patients with intraluminal typical bronchial carcinoid tumors in the absence of nodal and distant disease were treated with bronchoscopic electrocautery or Nd-YAG laser as an alternative to surgical resection. Prior to treatment, HRCT was performed. RESULTS: In 10 patients, HRCT showed no peribronchial tumor extension, and 9 of these patients were found to be tumor free after bronchoscopic treatment. So far during follow-up, none of these patients has had a recurrence of the tumor. The median duration of follow-up was 33 months (range, 13 to 68 months). In five patients, HRCT showed signs of peribronchial tumor extension. In three of these patients, specimens taken from biopsies performed after bronchoscopic treatment showed residual tumors, and salvage surgery was carried out. In three patients, HRCT was unable to assess peribronchial tumor extension: in two because of insufficient connective tissue contrast between the hilar structures and in one patient because of suboptimal scan technique. CONCLUSION: HRCT findings were complementary but not conclusive in patients with intraluminal typical bronchial carcinoid tumors treated with bronchoscopic therapy. However, in a category of patients in whom HRCT showed strictly intraluminal tumors, bronchoscopic resection as an alternative for surgical resection seems justified.

Atmospheric pressure changes and outdoor temperature changes in relation to spontaneous pneumothorax.

STUDY AIMS: To examine the influence of atmospheric pressure (AP) and temperature changes on the incidence of idiopathic spontaneous pneumothorax (SP). METHODS: From December 1991 through November 1993, 115 consecutive SP cases were selected. Patients were included after being in Amsterdam at least 1 full day before contracting the SP. Differences in air temperature and AP (provided hourly by the national weather bureau) for the days of the SP occurrence and the days previous to it were recorded to measure influences of air temperature and AP. The correlation between days with lightning and SP and clustering of SP was evaluated. RESULTS: SP occurred on 14.7% of the days in the 2-year period. There was no relationship between SP and a rise or fall in AP (Poisson regression). There was an average temperature rise of 0.57 degrees C from the day prior to the day of the SP, compared with a 0.08 degrees C fall on the days without SP. This difference is statistically significant and was consistent over the four seasons and both years. Seventy-three percent of the SP cases were clustered. A relationship between SP and thunderstorms was found. CONCLUSIONS: AP differences do not seem to influence the chance of developing SP. SP occurs in clusters, and more often 1 to 2 days after thunderstorms. Whether the identified temperature rise prior to the SP is a causative factor is unlikely; coexisting weather phenomena might explain this unexpected finding and should be studied in the future.

Results of two years expenience with fluorescence bronchoscopy in detection of preinvasive bronchial neoplasia.

The aim of the study was to determine whether use of fluorescence bronchoscopy improves the detection of preinvasive neoplastic bronchial lesions. The data of all patients who underwent fluorescence bronchoscopy and in whom bronchial biopsies were taken, were analyzed. Most patients were at risk for preinvasive lesions. A total of 174 bronchoscopies were performed in 95 patients. Of the 681 representative biopsies, 31 were found to be moderate dysplastic, 39 were found to be severe dysplastic and 9 exhibited carcinoma in situ. These 79 preinvasive lesions were found in 34 patients. The respective results of fluorescence bronchoscopy in addition to conventional bronchoscopy and of conventional bronchoscopy alone for detection of preinvasive lesions were: sensitivity 85% (67/79) and 59% (47/79); specificity 60% (351/581) and 85% (493/581); positive predictive values of 23% (67/297) and 35% (47/135); negative predictive values of 97% (351/363) and 94% (493/525). A separate analysis of only the first bronchoscopy of each patient showed similar results. Results of fluoresence bronchoscopy were better in the second part of the patient group. We conclude that after a learning period fluorescence bronchoscopy can increase the yield of finding preinvasive neoplastic lesions when used in addition to conventional bronchoscopy.