Biomedical subjects

Teri J Franks

Publications and source records attributed to Teri J Franks.

At least 19 recordsLinked to original sources

PubMed2006

Desmoglein 3 as a prognostic factor in lung cancer.

Desmoglein 3 is a desmosomal protein of the cadherin family. Our cDNA expression profile demonstrated that desmoglein 3 was highly expressed in squamous cell carcinoma of the lung but not detected in pulmonary adenocarcinoma or normal lung. To investigate the clinical significance of desmoglein 3 in lung cancer, we surveyed its expression in primary non-small-cell lung cancers and neuroendocrine tumors. We used immunohistochemical analysis to examine the expression of desmoglein 3 by using tissue microarrays containing samples from 300 surgical non-small-cell lung cancer and 183 lung neuroendocrine tumor. Staining status was determined based on the sum of the distribution score (0, 1, or 2) and the intensity score (0, 1, 2, or 3) of the staining signal. Follow-up was available for 346 cases (median follow-up of 2.8 years). We determined the survival statistical significance of desmoglein 3 by using the log-rank test, and we plotted Kaplan-Meier curves. Negative immunohistochemical staining with desmoglein 3 was associated with shorter survival for all lung cancer patients regardless of the histologic subtype (5-year survival of 20.9% versus 49.5%, P < .001) in our series. In patients with atypical carcinoid tumors, lacking desmoglein 3 expression showed a 5-year survival of 0% compared with 36.8% for desmoglein 3-positive cases (P < .001). Desmoglein 3 status indicated a poor prognosis in lung cancers and portends a more aggressive behavior for atypical carcinoid tumors.

Adenocarcinoma↗

PubMed2006

Telomere length, telomerase activity, and expressions of human telomerase mRNA component (hTERC) and human telomerase reverse transcriptase (hTERT) mRNA in pulmonary neuroendocrine tumors.

BACKGROUND: Telomeres are important for chromosome structure and function, protecting them against degradation. However, few studies have examined telomeres in pulmonary neuroendocrine (NE) tumors. METHODS: We investigated deparaffinized sections obtained from 70 primary NE lung tumors [34 typical carcinoids (TCs), 10 atypical carcinoids (ACs), 16 large cell neuroendocrine carcinoma (LCNECs) and 10 small cell lung carcinomas (SCLCs)]. RESULTS: Positive expressions of human telomerase mRNA component (hTERC) and human telomerase reverse transcriptase (hTERT) mRNA were recognized, respectively, in 58% and 74% of TCs, and in 100% and 100% of ACs, LCNECs and SCLCs. Alteration of telomere length was greater in both LCNECs and SCLCs than in TCs. Telomerase activity was detected in LCNECs, but not in TCs. By the reverse-transcriptase polymerase chain reaction (RT-PCR), hTERC mRNA was detected in 100% of LCNECs and TCs examined, while hTERT mRNA was detected in 67% of LCNECs, but not at all in TCs. CONCLUSIONS: These results suggest that alterations in telomere length, telomerase activity, and the expression of hTERT mRNA may (i) play roles in pathogenesis in pulmonary neuroendocrine tumors, and (ii) be a useful tool for differential diagnosis between TCs and LCNECs.

Carcinoma, Neuroendocrine↗

PubMed2006

Pulmonary microcystic fibromyxoma: Report of 3 cases.

Three cases of pulmonary myxoid tumors showing a variable degree of microcystic change are described. They are presented as incidental solitary peripheral lung nodules on routine radiologic studies. The patients included 2 women, 45 and 65 years old, and 1 man, 33 years old. Clinical work-up showed no evidence of neoplasia in other sites. Following surgical resection, no evidence of disease was observed after a mean follow-up of 48 months. The tumors ranged in size from 1 to 2.3 cm (mean 1.4 cm). Microscopically, the lesions were well circumscribed with conspicuous cystic change and myxoid stroma. Innocuous, widely-spaced, spindled to stellate tumor cells showed minimal nuclear pleomorphism and absence of mitotic activity. The myxoid stroma contained acid mucopolysaccharides, sensitive to hyaluronidase digestion. No epithelial, chondroid, neural, myofibroblastic, lipomatous or vascular differentiation was evident on immunohistochemical studies. Although these cases display cytologic features, myxoid stroma and benign clinical course characteristic of pulmonary myxomas; the presence of microcystic architecture is unique to the current series, and thus a descriptive designation "microcystic fibromyxoma" is suggested.

Adult↗

PubMed2006

Inhalational anthrax.

Inhalational anthrax is a lethal infection acquired from the inhalation of Bacillus anthracis, a pathogen classified as a Category A bioterrorist agent by the Centers for Disease Control and Prevention. The recent 2001 attack in which weaponized spores were delivered by mail to several US cities exposed our vulnerability to bioterrorism, and taught us important lessons in the timely diagnosis of this devastating disease. It is clear that patient mortality is significantly decreased by early recognition and immediate administration of antibiotic therapy. Unfortunately, the nonspecific clinical presentation is often misinterpreted as a flu-like illness and confirmatory microbiologic tests may take up to 24 hours. Radiologic manifestations, however, are distinctive and may prove essential in directing appropriate clinical care in the critical early hours of inhalational anthrax.

Anthrax↗

PubMed2005

Pulmonary sequelae in convalescent patients after severe acute respiratory syndrome: evaluation with thin-section CT.

PURPOSE: To prospectively evaluate lung parenchyma on paired inspiration-expiration thin-section computed tomographic (CT) scans in patients recovering from severe acute respiratory syndrome (SARS). MATERIALS AND METHODS: After the institutional review board approved the study and written consent was obtained from patients, 40 patients (25 female, 15 male; mean age, 42.8 years +/- 12.3 [standard deviation]) underwent thin-section CT at 51.8 days +/- 20.2 after onset of SARS symptoms. Twenty of the 40 patients underwent follow-up thin-section CT at 140.7 days +/- 26.7 after symptom onset. Lung findings were scored according to extent and then grouped in three categories (ground-glass opacity, interstitial opacity, and air trapping) for analysis. Mean CT scores for each finding in the various patient subgroups were compared by using the Mann-Whitney test. Clinical parameters and scores were evaluated for correlation by using Spearman rank correlation analysis. Mean scores for each finding were compared between the two serial examinations by using the Wilcoxon matched-pairs signed rank test. RESULTS: Air trapping, ground-glass opacity, and reticulation were found in 37 (92%), 36 (90%), and 28 (70%) of 40 patients, respectively, at initial thin-section CT examination and in 16 (80%), 14 (70%), and 10 (50%) of 20 patients, respectively, at follow-up examination. Scans from patients with adult respiratory distress syndrome (ARDS) had a significantly higher score for ground-glass opacity than did those from patients without ARDS (P = .009). A comparison of scores for the serial thin-section CT examinations indicated a significant reduction in the extent of ground-glass opacity (P < .001) and interstitial opacity (P < .001) but not in that of air trapping (P = .38) at follow-up examination. At initial thin-section CT, scores for ground-glass opacity, interstitial opacity, and air trapping correlated with age; those for ground-glass opacity and air trapping, with peak C-reactive protein level. At the second examination, scores for ground-glass opacity and interstitial opacity correlated with peak lactate dehydrogenase level; that for air trapping, with age and peak C-reactive protein level. CONCLUSION: Thin-section CT scores correlated with clinical and laboratory parameters in patients after SARS. Although ground-glass opacity and interstitial opacity resolve over time, air trapping persists.

Adult↗

PubMed2005

Evolving concepts in the pathology and computed tomography imaging of lung adenocarcinoma and bronchioloalveolar carcinoma.

PURPOSE: To review recent advances in pathology and computed tomography (CT) of lung adenocarcinoma and bronchioloalveolar carcinoma (BAC). METHODS: A pathology/CT review panel of pathologists and radiologists met during a November 2004 International Association for the Study of Lung Cancer/American Society of Clinical Oncology consensus workshop in New York. The purpose was to determine if existing data was sufficient to propose modification of criteria for adenocarcinoma and BAC as newly published in the 2004 WHO Classification of Lung Tumors, and to address the pathologic/radiologic concept of diffuse/multicentric BAC. RESULTS: Solitary small, peripheral BACs have an excellent prognosis. Most lung adenocarcinomas with a BAC pattern are not pure BAC, but rather adenocarcinoma, mixed subtype with invasive patterns. This applies to tumors presenting with a diffuse/multinodular as well as solitary nodule pattern. The percent of BAC versus invasive components in lung adenocarcinomas appears to be prognostically important. However, a consensus definition of "minimally invasive" BAC with a favorable prognosis could not be achieved. While recognition of a BAC component is possible, the diagnosis of BAC with exclusion of invasive adenocarcinoma cannot be made by small biopsy or cytology specimens. CONCLUSION: There is a need to work toward a mutual understanding and consensus between pathologists, clinicians, and researchers with the use of the term BAC versus adenocarcinoma. Future studies should make some attempt to quantitate these components and/or other features such as size of scar, size of invasive component, or pattern of invasion. Hopefully, this work will allow definition of a category of adenocarcinoma, mixed subtype with predominant BAC/minimal invasion and a favorable prognosis.

Adenocarcinoma↗

PubMed2005

Discordance between general and pulmonary pathologists in the diagnosis of interstitial lung disease.

BACKGROUND: Interstitial lung diseases (ILDs) often present diagnostic challenges to both the clinician and pathologist. Surgical lung biopsy (SLB) is often pursued in the evaluation of ILD and the clinician uses the histopathologic conclusions to guide management. However, the agreement between general and pulmonary pathologists in histopathologic diagnosis of ILD has not been established. OBJECTIVE: To determine the agreement between general and pulmonary pathologists in the histopathologic interpretation of ILDs and whether disagreements result in changes in clinical management. METHODS: We retrospectively reviewed all patients who underwent SLB for ILD at our institution, between 1996 and 2002. We compared the interpretations of general pathologists to those of pulmonary pathologists to evaluate the degree of inter-rater agreement. We assumed the specialist pathologist represented the "gold standard." We further determined if changes in the histopathologic diagnosis altered clinical management. RESULTS: Of 83 subjects who underwent SLB, 44 (mean age 58.5 +/- 14.2, 47.7% male) were examined by both general and specialty pathologists. There was poor agreement between the two sets of reviewers. The histopathologic interpretation by the specialist pathologist differed from the generalist in 52.3% of cases (kappa 0.21, P < 0.0001). This high rate of discordance led to frequent (60.0%) changes in clinical management. As a screening test for usual interstitial pneumonia, the observations of the general pathologist had moderate sensitivity and specificity (76.5% and 66.7%, respectively). CONCLUSIONS: General and pulmonary pathologists often differ in their interpretation of the histopathology in ILD. This significant discordance may have important clinical implications for patient care.

Adult↗

PubMed2005

Needs and opportunities for research in hypersensitivity pneumonitis.

Hypersensitivity pneumonitis (HP) develops after inhalation of many different environmental antigens, causing variable clinical symptoms that often make diagnosis uncertain. The prevalence of HP is higher than recognized, especially its chronic form. Mechanisms of disease are still incompletely known. Strategies to improve detection and diagnosis are needed, and treatment options, principally avoidance, are limited. A workshop recommended: a population-based study to more accurately document the incidence and prevalence of HP; better classification of disease stages, including natural history; evaluation of diagnostic tests and biomarkers used to detect disease; better correlation of computerized tomography lung imaging and pathologic changes; more study of inflammatory and immune mechanisms; and improvement of animal models that are more relevant for human disease.

Adult↗

PubMed2005

Pulmonary mucoepidermoid carcinoma with prominent tumor-associated lymphoid proliferation.

We report 6 cases of low-grade pulmonary mucoepidermoid carcinoma displaying a striking lymphoplasmacytic infiltrate. All six tumors had a typical pulmonary mucoepidermoid carcinoma presentation as a polypoid endobronchial mass involving the proximal bronchi. The patients were 3 females and 3 males with a mean age of 33 years (range, 5-61 years). Half of the patients were asymptomatic, while half experienced mild symptoms of pneumonia, asthma-like symptoms, or hemoptysis. No tumor-related deaths were observed, with a mean follow-up of 51 months. The tumor size ranged from 2.1 to 3.4 cm (mean, 2.9 cm). The tumors characteristically displayed an elaborate tubulocystic epithelial component composed of intermediate, epidermoid, and mucus-producing cells, and variable numbers of clear cells, multinucleated giant cells, columnar cells, and oncocytic cells. The tumors' lymphoplasmacytic infiltrate with occasional Russell bodies was sufficiently intense to raise concern of a low-grade lymphoma. All tested tumors were immunoreactive with CK7 while nonreactive with TTF-1 and CK20. Recognition of this histologic variant is important for a correct diagnosis of low-grade pulmonary mucoepidermoid carcinoma. The dense lymphoplasmacytic infiltrate is similar to that previously described in salivary glands as tumor-associated lymphoid proliferation.

Adult↗

PubMed2005

Peribronchiolar metaplasia: a common histologic lesion in diffuse lung disease and a rare cause of interstitial lung disease: clinicopathologic features of 15 cases.

Peribronchiolar metaplasia (PBM) is a histologic lesion consisting of peribronchiolar metaplasia (PBM) of bronchiolar-type epithelium. Although widely recognized, PBM has received little attention in the pathologic literature and is not known to have clinical significance. We identified 15 cases in which PBM was the only major histologic finding in surgical lung biopsies from patients with interstitial lung disease (PBM-ILD), and we reviewed the clinical, imaging, and pathologic findings. The mean age was 57 years (range, 44-74 years) with 13 females and 2 males. One patient had been a welder with fume and asbestos exposure; another had pigeon exposure. Smoking history was available for 13 patients: three current smokers, one cocaine user, two former smokers, and seven never smokers. Three patients had collagen vascular disease. One had elevated serum antinuclear antibody titers. Pulmonary function data were available for 10 patients: one obstructive, five restrictive, two mixed obstructive and restrictive, and two normal. Computerized tomography in 7 patients showed mosaic attenuation in 3 patients and air trapping in 1 patient; no bronchiectasis, septal lines, or honeycombing were seen in any cases. All 11 patients with available follow-up are alive; 4 of them have experienced symptomatic improvement (follow-up, 0.6-6.9 years; mean, 2.4 years). PBM was found focally in other interstitial lung diseases, which were assessed for this lesion: 59% of usual interstitial pneumonia (17 of 29), 50% of nonspecific interstitial pneumonia (10 of 20), desquamative interstitial pneumonia (3 of 6), hypersensitivity pneumonitis (9 of 18), and 11% of respiratory bronchiolitis (2 of 18). In summary, PBM is a common histologic finding in various interstitial lung disorders. It is rarely the sole major lung biopsy finding in patients presenting with interstitial lung disease (PBM-ILD). Patients are mostly older women, with mild symptoms and CT findings. Survival appears to be favorable.

Adult↗

PubMed2004

Clinical issues and research in respiratory failure from severe acute respiratory syndrome.

The National Heart, Lung, and Blood Institute, along with the Centers for Disease Control and Prevention and the National Institute of Allergy and Infectious Diseases, convened a panel to develop recommendations for treatment, prevention, and research for respiratory failure from severe acute respiratory syndrome (SARS) and other newly emerging infections. The clinical and pathological features of acute lung injury (ALI) from SARS appear indistinguishable from ALI from other causes. The mainstay of treatments for ALI remains supportive. Patients with ALI from SARS who require mechanical ventilation should receive a lung protective, low tidal volume strategy. Adjuvant treatments recommended include prevention of venous thromboembolism, stress ulcer prophylaxis, and semirecumbent positioning during ventilation. Based on previous experience in Canada, infection control resources and protocols were recommended. Leadership structure, communication, training, and morale are an essential aspect of SARS management. A multicenter, placebo-controlled trial of corticosteroids for late SARS is justified because of widespread clinical use and uncertainties about relative risks and benefits. Studies of combined pathophysiologic endpoints were recommended, with mortality as a secondary endpoint. The group recommended preparation for studies, including protocols, ethical considerations, Web-based registries, and data entry systems.

Adrenal Cortex Hormones↗

PubMed2004

Analysis of deaths during the severe acute respiratory syndrome (SARS) epidemic in Singapore: challenges in determining a SARS diagnosis.

CONTEXT: An outbreak of severe acute respiratory syndrome (SARS), an infectious disease attributed to a novel coronavirus, occurred in Singapore during the first quarter of 2003 and led to 204 patients with diagnosed illnesses and 26 deaths by May 2, 2003. Twenty-one percent of these patients required admission to the medical intensive care unit. During this period, the Center for Forensic Medicine, Health Sciences Authority, Singapore, performed a total of 14 postmortem examinations for probable and suspected SARS. Of these, a total of 8 were later confirmed as SARS infections. OBJECTIVE: Our series documents the difficulties encountered at autopsy during the initial phases of the SARS epidemic, when the pattern of infection and definitive diagnostic laboratory criteria were yet to be established. DESIGN: Autopsies were performed by pathologists affiliated with the Center for Forensic Medicine, Health Sciences Authority, Singapore. Tissue was accessed and read at the Tan Tock Seng Hospital, Singapore, and at the Armed Forces Institute of Pathology, Washington, DC. Autopsy tissue was submitted to the Virology Department, Singapore General Hospital, for analysis, and in situ hybridization for the SARS coronavirus was carried out at the National Institute of Infectious Diseases, Tokyo, Japan. RESULTS: Thirteen of 14 patients showed features of diffuse alveolar damage. In 8 patients, no precipitating etiology was identified, and in all of these patients, we now have laboratory confirmation of coronavirus infection. Two of the 8 patients presented at autopsy as sudden unexpected deaths, while the remaining 6 patients had been hospitalized with varying lengths of stay in the intensive care unit. In 3 patients, including the 2 sudden unexpected deaths, in situ hybridization showed the presence of virally infected cells within the lung. In 4 of the 8 SARS patients, pulmonary thromboemboli were also recognized on gross examination, while one patient had marantic cardiac valvular vegetations. CONCLUSIONS: It is unfortunate that the term atypical pneumonia has been used in conjunction with SARS. Although nonspecific by itself, the term does not accurately reflect the underlying dangers of viral pneumonia, which may progress rapidly to acute respiratory distress syndrome. We observed that the clinical spectrum of disease as seen in our autopsy series included sudden deaths. This is a worrisome finding that illustrates that viral diseases will have a spectrum of clinical presentations and that the diagnoses made for such patients must incorporate laboratory as well as clinical data.

Adult↗

PubMed2003

Lung pathology of severe acute respiratory syndrome (SARS): a study of 8 autopsy cases from Singapore.

Severe acute respiratory syndrome (SARS) is an infectious condition caused by the SARS-associated coronavirus (SARS-CoV), a new member in the family Coronaviridae. To evaluate the lung pathology in this life-threatening respiratory illness, we studied postmortem lung sections from 8 patients who died from SARS during the spring 2003 Singapore outbreak. The predominant pattern of lung injury in all 8 cases was diffuse alveolar damage. The histology varied according to the duration of illness. Cases of 10 or fewer days' duration demonstrated acute-phase diffuse alveolar damage (DAD), airspace edema, and bronchiolar fibrin. Cases of more than 10 days' duration exhibited organizing-phase DAD, type II pneumocyte hyperplasia, squamous metaplasia, multinucleated giant cells, and acute bronchopneumonia. In acute-phase DAD, pancytokeratin staining was positive in hyaline membranes along alveolar walls and highlighted the absence of pneumocytes. Multinucleated cells were shown to be both type II pneumocytes and macrophages by pancytokeratin, thyroid transcription factor-1, and CD68 staining. SARS-CoV RNA was identified by reverse transcriptase-polymerase chain reaction in 7 of 8 cases in fresh autopsy tissue and in 8 of 8 cases in formalin-fixed, paraffin-embedded lung tissue, including the 1 negative case in fresh tissue. Understanding the pathology of DAD in SARS patients may provide the basis for therapeutic strategies. Further studies of the pathogenesis of SARS may reveal new insight into the mechanisms of DAD.

Adult↗

PubMed2003

Reclassification of cystic bronchioloalveolar carcinomas to adenocarcinomas based on the revised World Health Organization Classification of Lung and Pleural Tumours.

The objective of this study was to evaluate the radiologic-pathologic correlation of cystic change in cases of bronchioloalveolar carcinoma (BAC) using the 1999 revised World Health Organization Classification of Lung and Pleural Tumours. A total of 304 cases diagnosed as BAC before 1999 were reviewed retrospectively for radiologic findings of cystic change (n = 31). Of these, 20 had adequate clinical, pathologic, and radiologic material available for review. Patient demographics and history; the method, location, and size of specimen biopsy; and pathology reports were evaluated. A pulmonary pathologist reviewed the microscopic findings to confirm the diagnosis based on the 1999 World Health Organization criteria. Chest radiographs (n = 20) and computed tomographic scans (n = 11) were analyzed for location, size, and multiplicity of pulmonary nodules, masses, consolidations, or characterization of cystic change. Associated findings of lymphadenopathy, pleural effusion, atelectasis, and underlying lung disease were noted. There were 10 men and 10 women (mean age, 49.3 years) who participated in the study. Eleven were symptomatic. Pathologic review, based on the revised World Health Organization classification, yielded the diagnosis of adenocarcinoma (n = 14); adenocarcinoma, possible BAC (n = 4); and BAC (n = 2). Radiologically, adenocarcinoma (n = 14) manifested as a solitary pulmonary nodule/mass (n = 5) or consolidation (n = 6) and as multifocal mixed disease (n = 3) with solitary (n = 8) or multicystic (n = 6) change. Adenocarcinoma, possible BAC (n = 4) manifested as multifocal and multicystic consolidations (n = 2) or mixed disease (n = 1) and as a solitary cystic mass (n = 1). BAC (n = 2) manifested as a cystic mass (n = 1) and as multifocal multicystic mixed disease (n = 1). Most cases of BAC with radiologic cystic change were reclassified as adenocarcinoma. The radiologic features of BAC may need to be redefined in light of the current diagnostic criteria to help identify patients with limited and potentially curable disease.

Adenocarcinoma↗

PubMed2002

Extensive squamous metaplasia with cytologic atypia in diffuse alveolar damage mimicking squamous cell carcinoma: a report of 2 cases.

Squamous metaplasia in the setting of diffuse alveolar damage, a form of acute lung injury, in rare cases can be very extensive with cytologic atypia, mimicking squamous cell carcinoma. We describe 2 such cases that posed diagnostic difficulty in the evaluation of biopsy specimens. These cases demonstrate that histologic findings must be correlated with clinical and radiologic information to avoid a misdiagnosis of malignancy.

Adolescent↗

PubMed2002

Acute fibrinous and organizing pneumonia: a histological pattern of lung injury and possible variant of diffuse alveolar damage.

CONTEXT: The histologic patterns of diffuse alveolar damage (DAD), bronchiolitis obliterans with organizing pneumonia (BOOP), and eosinophilic pneumonia (EP) are well-recognized histologic patterns of lung injury associated with an acute or subacute clinical presentation. We have recognized acute fibrinous and organizing pneumonia (AFOP) as a histologic pattern, which also occurs in this clinical setting but does not meet the classic histologic criteria for DAD, BOOP, or EP and may represent an underreported variant. OBJECTIVE: To investigate the clinical significance of the AFOP histologic pattern and to explore its possible relationship to other disorders, including DAD and BOOP. DESIGN: Open lung biopsy specimens and autopsy specimens were selected from the consultation files of the Armed Forces Institute of Pathology, which showed a dominant histologic pattern of intra-alveolar fibrin and organizing pneumonia. Varying amounts of organizing pneumonia, type 2 pneumocyte hyperplasia, edema, acute and chronic inflammation, and interstitial widening were seen. Cases with histologic patterns of classic DAD, BOOP, abscess formation, or eosinophilic pneumonia were excluded. To determine the clinical behavior of patients with this histologic finding, clinical and radiographic information and follow-up information were obtained. Statistical analysis was performed using Kaplan-Meier and chi(2) analysis. RESULTS: Seventeen patients (10 men, 7 women) with a mean age of 62 years (range, 33-78 years) had acute-onset symptoms of dyspnea (11), fever (6), cough (3), and hemoptysis (2). Associations believed to be clinically related to the lung disease included definitive or probable collagen vascular disease (3), amiodarone (1), sputum culture positive for Haemophilus influenza (1), lung culture positive for Acinetobacter sp. (1), lymphoma (1), hairspray (1), construction work (1), coal mining (1), and zoological work (1). Six patients had no identifiable origin or association. Follow-up revealed 2 clinical patterns of disease progression: a fulminate illness with rapid progression to death (n = 9; mean survival, 0.1 year) and a more subacute illness, with recovery (n = 8). Histologic analysis and initial symptoms did not correlate with eventual outcome, but 5 of the 5 patients who required mechanical ventilation died (P =.007). CONCLUSIONS: Acute fibrinous and organizing pneumonia is a histologic pattern associated with a clinical picture of acute lung injury that differs from the classic histologic patterns of DAD, BOOP, or EP. Similar to these patterns of acute lung injury, the AFOP pattern can occur in an idiopathic setting or with a spectrum of clinical associations. The overall mortality rate is similar to DAD and therefore may represent a histologic variant; however, AFOP appears to have 2 distinct patterns of disease progression and outcome. The need for mechanical ventilation was the only parameter that correlated with prognosis. None of the patients with a subacute clinical course required mechanical ventilation.

Acute Disease↗

PubMedDate not supplied

From the archives of the AFIP: Localized fibrous tumor of the pleura.

Eighty-two localized fibrous tumors of the pleura (LFTP) were reviewed retrospectively for the clinical, pathologic, and radiologic findings. Forty-four women and 38 men ranged in age from 17 to 78 years (mean, 54.7 years). Sixty-four benign LFTP ranged in size from 2 to 30 cm (mean, 13.2 cm), and 18 malignant tumors ranged from 3 to 23 cm (mean, 14.4 cm). Forty-eight patients (60%) presented with symptoms. Radiographs of 76 patients demonstrated solitary masses occupying or extending into the inferior hemithorax (79%). Computed tomography (CT) of 78 lesions demonstrated lobular masses (83%) that formed at least one acute angle (96%) or only acute angles (65%) with the adjacent pleura. Heterogeneous lesion attenuation was documented in 88% of enhanced and in 68% of unenhanced CT scans. Contrast enhancement was common (62% of cases). Magnetic resonance (MR) imaging of 18 lesions demonstrated heterogeneous signal intensity on both T1- and T2-weighted images (78% and 83%, respectively). Multiplanar MR imaging allowed visualization of the diaphragm and documentation of an intrathoracic mass in all cases. LFTP are solitary lobular heterogeneous masses that occur in symptomatic adults and often affect the inferior hemithorax. Malignant lesions are radiologically indistinguishable from those with benign histologic characteristics. Radiographic and CT features characteristic of pleural location are typically absent.

Adolescent↗

PubMedDate not supplied

From the archives of the AFIP: pulmonary Langerhans cell histiocytosis.

Pulmonary Langerhans cell histiocytosis (PLCH) is an isolated form of Langerhans cell histiocytosis that primarily affects cigarette smokers. PLCH is characterized by peribronchiolar proliferation of Langerhans cell infiltrates that form stellate nodules. The nodular lesions frequently cavitate and form thick- and thin-walled cysts, which are thought to represent enlarged airway lumina. PLCH lesions display temporal microscopic heterogeneity, with progression from dense cellular nodules to apparently cavitary nodules to increasing degrees of fibrosis that may extend along alveolar walls. In advanced cases, fibrotic scars are surrounded by enlarged, distorted air spaces. Affected patients are typically young adults who often present with cough and dyspnea. The characteristic radiographic features of PLCH are bilateral nodular and reticulonodular areas of opacity that predominantly involve the upper and middle lung zones with relative sparing of the lung bases. High-resolution computed tomography (CT) shows nodules and cysts in the same distribution and allows a confident prospective diagnosis of PLCH in the appropriate clinical setting. In typical cases, a predominantly nodular pattern is seen on CT scans in early phases of the disease, whereas a cystic pattern predominates in later phases. The radiologic abnormalities may regress, resolve completely, become stable, or progress to advanced cystic changes. Treatment consists of smoking cessation, but corticosteroid therapy may be useful in selected patients. Chemotherapeutic agents and lung transplantation may be offered to patients with advanced disease. The prognosis of PLCH is variable with frequent regression, stabilization, or recurrence of disease that does not correlate with cessation or continuation of smoking.

Adolescent↗