Underlying chronic granulomatous disease in a patient with bronchocentric granulomatosis.

We present a case of bronchocentric granulomatosis in a woman with no history of asthma who was colonised with Aspergillusfumigatus. A family history of chronic granulomatous disease prompted further testing that demonstrated severely depressed neutrophil oxidant production and gp91(phox) deficiency compatible with the X linked carrier state of chronic granulomatous disease. Only one report of the association of these two rare diseases has previously appeared in the literature. We postulate that an ineffective immune response led to the prolonged colonisation of Afumigatus resulting in a hypersensitivity reaction that was manifest clinically as bronchocentric granulomatosis.

Successful treatment of juvenile laryngeal papillomatosis-related multicystic lung disease with cidofovir: case report and review of the literature.

Cidofovir, a nucleoside analog antiviral agent, has been used with moderate success in the treatment of juvenile laryngeal papillomatosis (JLP) by direct intralesional injection. We report the first case where IV cidofovir was used successfully to treat a rare but lethal multicystic lung disease complicating JLP. A 35-year-old woman with a history of JLP requiring multiple laser ablations of laryngeal papillomata each year presented with hemoptysis and was found on CT scan to have bilateral, multiple pulmonary nodules and cysts. The results of BAL fluid analysis demonstrated no evidence of malignancy, and cultures were negative for fungi and mycobacteria. Molecular DNA typing of a biopsy specimen obtained from a laryngeal papilloma confirmed infection with human papilloma virus type 11. She received 12 months of treatment with IV cidofovir followed by 9 months of combined treatmentwith IV cidofovir and subcutaneous interferon-alpha-2A. This therapeutic regime resulted in a markedly decreased requirement for surgical removal of laryngeal papillomata, and CT scanning documented the regression of the lesions in the lung parenchyma that persisted after the discontinuation of therapy. The results of this case demonstrate that cidofovir may be used successfully to treat JLP-related lung disease and suggest that further studies are warranted.

Pathological examination and the reporting of lung cancer specimens.

The Lung Cancer Disease Site Group (DSG) of the Cancer Care Ontario Practice Guidelines Initiative first met in January of 1994. Included in the membership were three pathologists who, with the other members of the DSG, felt that a useful contribution to the work of the group would be a recommendation on standardized examination and reporting of lung cancer specimens. This review summarizes the consensus of the Lung Cancer DSG pathologists based on their review of the literature and proposes a standard synoptic report, the Primary Lung Cancer Check-Off Sheet. If generally adopted, this standard would improve the quality of reporting of clinical and pathological stage information. Such high-quality staging information is essential to define patient populations for clinical trials and for outcome analyses.

Lymphoproliferative disorders after lung transplantation: imaging features.

PURPOSE: To define the imaging features of lymphoproliferative disorders that occur after lung transplantation. MATERIALS AND METHODS: In a retrospective review of the cases of 246 patients who had undergone lung transplantation (62 single- and 184 double-lung transplants) between 1987 and 1997, the authors found nine patients (seven men and two women, aged 16-59 years [mean, 42.2 years]) with posttransplantation lymphoproliferative disorders. All imaging, clinical, and pathologic findings pertaining to these disorders were reviewed. RESULTS: Eight of the nine patients had isolated intrathoracic disease. The most common abnormality (six patients) was the presence of multiple, well-defined pulmonary nodules. These nodules, when multiple, had basilar and peripheral predominance. Other abnormal features included hilar or mediastinal adenopathy (two patients), a consolidated upper lobe (one patient), a pleural mass (one patient), and bulky abdominal lymphadenopathy (one patient with pathologically proved Hodgkin lymphoma). Three patients had nodules with a surrounding area of ground-glass opacity (halo sign), and one patient had multiple ill-defined areas of centrilobular opacity. Air bronchograms were not often seen, and pleural effusions were absent. CONCLUSION: The presence of well-defined pulmonary nodules in a patient who has undergone lung transplantation should raise concern about the possibility of posttransplantation lymphoproliferative disorder. In such cases, performance of pathologic studies should be expedited.

Mediastinal parathyroid cysts.

BACKGROUND: Mediastinal parathyroid cysts are a relatively rare clinical entity. The clinical presentation can be quite varied, although most are found incidentally during investigations for esophageal or respiratory symptoms. METHODS: We present a review of the literature and describe two instructive cases showing specific clinical findings. The clinical presentation, radiologic and pathologic findings, and treatment of mediastinal parathyroid cysts are discussed. RESULTS: In the first patient, the presenting symptom was increasing hoarseness resulting from paresis of the right recurrent laryngeal nerve. This case illustrates the rare association of a benign mediastinal parathyroid cyst with unilateral vocal cord palsy. The second patient presented with the more classic findings of progressive dyspnea and stridor related to tracheal compression. CONCLUSIONS: Although mediastinal parathyroid cysts are rare and can have varied presentations, thorough investigation can reveal the underlying cyst. Surgical excision is the treatment of choice and can be expected to produce excellent results.

Role of open lung biopsy for diagnosis in lung transplant recipients: ten-year experience.

Between November 1983 and August 1993, The Toronto Lung Transplant Program performed 153 transplantations in 144 recipients: 53 single-lung transplantations (SLT) and 100 double-lung transplantations (DLT). Thirty-eight open lung biopsies (OLBs) were done in 32 (22% of all recipients): 19 in SLT (36% of SLT) 12 in DLT (12% of DLT), and 1 in a patient who had a SLT and then a double retransplantation. Six recipients underwent OLB twice: 1 DLT, 3 SLT, and 2 who had OLB both before and after retransplantation. Indication for 11 early OLBs (< or = 45 days postoperative) was persistent parenchymal infiltrates. Indications for 27 late OLBs (> 45 days postoperative) included progressive radiologic disease with clinical findings or progressive loss of pulmonary function (18), persistent poor graft function (3), mass or nodules (3), persistent infiltrates without functional loss (2), and persistent lymphocytosis in bronchoalveolar lavage (1). Open lung biopsy confirmed a previous clinical or pathologic diagnosis in 11, suggested a diagnosis in 2, yielded nonspecific information in 16, and provided different diagnosis in 9. New diagnosis that changed therapy was made in 1 of 11 early OLBs and in 8 of 27 late OLBs. These 9 diagnoses included in SLTs: bronchiolitis obliterans (2), bronchiolitis obliterans organizing pneumonia (1), malignant lymphoma (1), and chronic vascular rejection (1) in SLT, and bronchiolitis obliterans organizing pneumonia (3) and Burkholderia cepacia infection (1) in DLT. We conclude that OLB is of little value in the perioperative period but yields useful information in approximately 30% of patients when performed late.

Thin-walled cystic lesions in bronchioalveolar carcinoma.

In a retrospective study, thin-walled cystic lesions were identified in four patients with proved bronchioalveolar carcinoma (BAC). The radiographic appearances are described, and possible mechanisms of formation of the lesions are discussed. Although such appearances due to BAC have rarely been described in the literature, the authors believe that the differential diagnosis of thin-walled cystic lesions associated with consolidation should include BAC.

Role of basic fibroblast growth factor in revascularization of rabbit tracheal autografts.

Despite omentopexy of the bronchial anastomosis, donor airway ischemia remains a problem after lung transplantation. This study examined the hypothesis that surface abrasion and topical application of basic fibroblast growth factor (bFGF) would enhance omental revascularization of trachea in a rabbit heterotopic autograft model. Tracheal segments were excised, primary tracheal anastomoses performed, and the segments placed in the peritoneal cavity wrapped in omentum. Animals were randomized to one of six groups according to tracheal segment treatment: control, surgical abrasion, Surgicel wrap with topical bFGF, Surgicel wrap with bFGF vehicle, Gelfoam wrap with bFGF, and topical bFGF alone. One week later, animals were heparinized, perfused with Aquablak dye, and killed. Tracheal segments were excised and sectioned for light microscopic quantitative assessment of viability and dye perfusion. There was no significant improvement in viability or perfusion between abraded tracheal segments or segments treated with bFGF/Gelfoam or bFGF alone when compared with control segments. Airways wrapped in Surgicel had significantly greater ischemic injury compared with the control group, regardless of bFGF application. Neither surgical abrasion nor topical bFGF increased omental revascularization of transplanted tracheal segments after 7 days.

Anterior mediastinal masses: utility of transthoracic needle biopsy.

The results of 143 transthoracic needle biopsies (TNBs) in 126 patients with anterior mediastinal masses were compared with the final diagnosis, which was proved with pathologic study (n = 95) or clinicoradiologic methods (n = 31). In the 26 patients with lymphoma, the sensitivity of TNB was 42%; the specificity, 96%. In the 15 patients with Hodgkin disease, the sensitivity was 20%. In the 28 patients with thymoma, the sensitivity was 71%; the specificity, 94%. In the 11 patients with germ cell tumors, the sensitivity was 91%; specificity, 98%. The sensitivity in the 33 patients with metastatic disease was 70%; specificity, 100%. The cytologic specimens were examined with light microscopy and the Papanicolaou method only, a limitation that explains the difficulty in differentiating lymphoma from thymoma and that can now be overcome with immunohistochemical study. TNB of anterior mediastinal masses is useful in metastatic disease and germ cell tumors. Lymphoma and thymoma are less reliably diagnosed unless immunohistochemical cytologic methods are applied.

Bronchiolitis obliterans after lung transplantation: findings at chest radiography and high-resolution CT. The Toronto Lung Transplant Group.

Although bronchiolitis obliterans (BO) is seen commonly after heart-lung transplantation, its occurrence after lung transplantation appears to be relatively infrequent. In the 55 single- and double-lung transplantations performed at Toronto General Hospital, 41 patients have survived longer than 3 months and four (10%) have developed pathologically proved BO. Chest radiographic findings in these four patients included slight to moderate decreased peripheral vascular markings (n = 3), slight to moderate volume loss (n = 2), subsegmental atelectasis (n = 2), and new, thin, linear, irregular areas of increased opacity (n = 2). High-resolution computed tomography (HRCT) demonstrated mild peripheral bronchiectasis (n = 4) and decreased peripheral vascular markings (n = 3). Although the radiographic and HRCT findings did not appear to be specific for posttransplantation BO, they may be of value in suggesting the diagnosis of BO within this patient population.

Pulmonary lymphangioleiomyomatosis: high-resolution CT findings in four cases.

Lymphangioleiomyomatosis is a rare disease of unknown cause that affects women of reproductive age. It is characterized by progressive proliferation of smooth muscle in the lung. The patients present with progressive shortness of breath, pneumothorax, chylous effusion, and hemoptysis. Four patients with biopsy-proved lymphangioleiomyomatosis of the lung were evaluated using high-resolution CT. In all patients, the scan showed well-defined cystic air spaces, surrounded by uniformly thin walls, distributed diffusely throughout both lungs. The cystic air spaces ranged in size from a few millimeters to 5 cm. Pathologically, these cysts were predominantly bounded by normal-looking parenchymal components, with occasional patchy involvement by a smooth-muscle proliferative process. The CT appearance of lymphangioleiomyomatosis differs quite distinctly from that of other diseases that can cause cystic air spaces, such as fibrosing alveolitis, neurofibromatosis, and bronchiectasis, and less distinctly from pulmonary emphysema and eosinophilic granuloma. Our experience in these few cases suggests that the high-resolution CT findings in lymphangioleiomyomatosis are characteristic of the disease.

Airway hyperreactivity in patients undergoing lung and heart/lung transplantation.

To assess airway reactivity in lung transplant patients, three heart/lung, three double lung, and eight single lung transplant patients underwent airway challenge tests. All patients were assessed by methacholine aerosol challenge and thirteen were also assessed by histamine aerosol challenge at least three months after transplant surgery. The airways of patients with bilaterally denervated lungs (heart/lung and double lung transplants) were significantly more reactive to both methacholine and histamine (p less than 0.01) than were the airways of patients with unilaterally denervated (single lung transplants) lungs. Inflammatory changes in the airway mucosal biopsies were minimal in three patients and absent in all others. These studies raise questions about the role of central innervation in the maintenance of normal airway function as well as the mechanisms of action of both methacholine and histamine in causing bronchoconstriction.

Pulmonary blastoma, report of three cases and a review of the literature.

Pulmonary blastoma is one of the least common primary malignant tumors of the lung. There is considerable controversy as to its exact histogenesis; it is uncertain if it arises from a single cell or if it is a mixed tumor arising from two different cell lines. We report three patients with pulmonary blastomas, describing in detail their radiological, cytological, and histopathological features.

A critical evaluation of bronchoalveolar lavage. Criteria for identifying unsatisfactory specimens.

In a multicenter, city-wide study of the use of bronchoalveolar lavage for the evaluation of diffuse interstitial lung diseases, the occurrence of specimens unsuitable for analysis was evaluated. Using a standardized bronchoscopy technique, 26 physicians obtained 1,588 lavage specimens from 787 patients over a 52-month period. After transport to and processing in one laboratory using standardized procedures, all specimens were interpreted by one pathologist. Specimens were considered unsatisfactory if they contained: (1) a paucity of alveolar macrophages (i.e., less than ten alveolar macrophages/high-power field), (2) excessive numbers of airway-derived cells (i.e., more than the alveolar macrophages present), (3) a mucopurulent exudate, (4) cells altered by degeneration or (5) laboratory artifacts. Using these criteria, 30.4% of the specimens were considered unsuitable for analysis. There were no significant differences in the frequency of unsatisfactory specimens among participating physicians and institutions or between smoking and nonsmoking patients. Appraisal of alveolar inflammatory and immune effector cells in bronchoalveolar lavage specimens from patients with interstitial lung disease should include an assessment for contamination from airways proximal to the terminal bronchioles before conclusions are drawn about the activity of alveolar inflammation.

Small cell anaplastic carcinoma: cytological-histological correlations from percutaneous fine-needle aspiration biopsy.

Small cell anaplastic carcinoma (SCAC) is usually treated non-surgically and, therefore, diagnosis is best done by the least invasive means. Percutaneous fine-needle aspiration biopsy is very accurate in diagnosing malignancy and reasonably accurate in diagnosing malignant cell type. An unequivocal cytological diagnosis of SCAC has a positive predictive value of 0.90 at our institution. The accuracy is lowered by uncertain or suggestive cytological diagnoses.