Charles A. Rohrmann, Jr, MD: 2000-2001 Armed Forces Institute of Pathology Distinguished Scientist.
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Academies and Institutes↗
Biomedical subjects
M L Rosado-de-Christenson
Publications and source records attributed to M L Rosado-de-Christenson.
Pulmonary pathology of Erdheim-Chester disease.
Erdheim-Chester disease (ECD) is a rare non-Langerhans' cell histiocytosis that may present with pulmonary symptoms. The condition seems to be nonfamilial and typically affects middle-aged adults. Radiographic and pathologic changes in the long bones are diagnostic, but patients often present with extraskeletal manifestations. Advanced pulmonary lesions are associated with extensive fibrosis that may lead to cardiorespiratory failure. The clinical, radiologic, and pathologic features of six patients with ECD with lung involvement are presented. The patients were three men and three women (mean age, 57). Five presented with progressive dyspnea, and one presented with diabetes insipidus. Open-lung biopsies showed histiocytic infiltrates in a lymphangitic pattern with associated fibrosis and lymphoplasmacytic inflammatory infiltrates. The histiocytes did not stain with periodic acid-Schiff. Immunoperoxidase studies performed on specimens from five of six patients showed that the histiocytes were positive for CD68 and Factor XIIIa and negative for CD1a. Specimens from two patients exhibited immunoreactivity for S-100 protein. Electron microscopy studies performed on specimens from two patients showed phagocytic lysosomes but no Birbeck granules. Clinical follow-up of up to 16 years was available. At the end of that time, five patients were dead of complications related to their disease; one patient remains alive 4 years after diagnosis but with severe respiratory compromise. ECD is a rare non-Langerhans' cell histiocytosis that may present as interstitial lung disease and resemble other pulmonary conditions, particularly usual interstitial pneumonitis and pulmonary Langerhans' cell histiocytosis. Recognition of this entity will allow better assessment of its true incidence, therapeutic options, and prognosis.
Adult↗
Charles A. Rohrmann, Jr, MD Armed Forces Institute of Pathology 2000-2001 Distinguished Scientist.
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History, 20th Century↗
Bronchogenic cyst: imaging features with clinical and histopathologic correlation.
PURPOSE: To characterize the imaging features of bronchogenic cysts. MATERIALS AND METHODS: The computed tomographic (CT) and/or magnetic resonance (MR) or ultrasonographic images in 68 histopathologically proved cases of bronchogenic cyst in 38 male and 30 female patients, aged newborn to 72 years (mean, 22 years), were retrospectively reviewed. RESULTS: There were 58 mediastinal and 10 extramediastinal cysts. At CT (n = 62), 60 cysts were sharply marginated with smooth (n = 35) or lobulated (n = 25) borders. Twenty-five cysts were of water attenuation, 25 were of soft-tissue attenuation, two were air filled, two had an air-fluid level, and two had dependent milk of calcium. On T1-weighted MR images (n = 23), 18 cysts were hyperintense and five were isointense to cerebrospinal fluid. On T2-weighted MR images (n = 18), 17 cysts were isointense or hyperintense to cerebrospinal fluid. Of the 25 soft-tissue-attenuation lesions at CT, 11 appeared cystic because of internal homogeneity, lack of internal enhancement, mural enhancement, and characteristic location. Fourteen appeared solid based on morphology and attenuation. MR imaging of nine of the latter showed marked hyperintensity on T2-weighted images. CONCLUSION: CT of bronchogenic cysts typically shows sharply marginated mediastinal masses of soft-tissue or water attenuation. Most appear cystic. A minority appear solid and can be confused with other lesions; MR imaging can be useful for elucidating the cystic nature of these lesions.
Adolescent↗
Opportunistic fungal pneumonia.
Opportunistic fungal infection is a common cause of serious morbidity and mortality in immunocompromised patients. These infections occur primarily in patients with chemotherapy-induced neutropenia, acquired immunodeficiency syndrome. or immunosuppression after solid organ or bone marrow transplantation. The most important opportunistic fungal pathogens include Cryptococcus neoformans, Candida and Aspergillus species, and the fungi that cause mucormycosis. Opportunistic pneumonia caused by previously unrecognized pathogens, such as Fusarium, Penicillium, and the dematiaceous fungi, are increasingly reported. The clinical and radiologic features of opportunistic fungal pneumonia are highly variable and often nonspecific. Diagnosis requires knowledge of the various modes of presentation, radiologic manifestations, and epidemiology of these infections. Because many of these organisms can colonize the upper airway, sputum cultures are considered diagnostically unreliable. Instead, definitive diagnosis requires culture of the fungus from infected tissue or demonstration of the organism on microscopic examination.
AIDS-Related Opportunistic Infections↗
Tumors of the thymus.
Thymic neoplasms are a common cause of an anterior mediastinal mass and may be benign or malignant. Thymic cysts are congenital or acquired and may be associated with a thymic malignancy. True thymic hyperplasia and thymic lymphoid hyperplasia may enlarge the thymus and simulate a neoplasm. Thymoma and thymic carcinoma are epithelial malignancies with distinct clinicopathologic features. Thymic carcinoid is a rare aggressive neuroendocrine malignancy associated with multiple endocrine neoplasia 1. Thymolipoma is a benign neoplasm. Hodgkin and non-Hodgkin lymphoma may primarily or secondarily involve the thymus. Primary mediastinal germ cell tumors may arise primarily within the thymus and include mature teratoma, seminoma, and non-seminomatous malignant germ cell tumors.
Cysts↗
Pulmonary inflammatory pseudotumor: radiologic features.
PURPOSE: To define the radiologic features of pulmonary inflammatory pseudotumor. MATERIALS AND METHODS: Between 1966 and 1994, 61 cases of pulmonary inflammatory pseudotumor involved 36 male and 25 female patients (age range, 17 months to 61 years; mean, 28 years). Clinical presentation, pathologic features, and radiologic findings were noted. RESULTS: At radiography, 52 patients had solitary peripheral nodules or masses, and extraparenchymal involvement--including hilar, mediastinal, and airway invasion--was found in 11. At computed tomography, 12 lesions were of heterogeneous attenuation and five, homogeneous. At T1-weighted magnetic resonance imaging, five lesions had intermediate signal intensity; of two lesions studied with T2-weighted imaging, two had high signal intensity; and the one lesion studied with gadolinium-enhanced imaging had diffuse enhancement. CONCLUSION: Pulmonary inflammatory pseudotumor was typically a solitary, peripheral, sharply circumscribed mass with an anatomic bias for the lower lobes. Local invasion and primary involvement of the mediastinum and hilar structures were unusual manifestations.
Adult↗
Diane S. Babcock, MD, and Carol M. Rumack, MD Armed Forces Institute of Pathology 1998-1999 distinguished scientists.
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Academies and Institutes↗
Diffuse panbronchiolitis: histologic diagnosis in unsuspected cases involving North American residents of Asian descent.
OBJECTIVE: To study and report two cases of diffuse panbronchiolitis in patients of Asian ancestry residing in the United States and to review the literature pertaining to this disease. DESIGN: Diffuse panbronchiolitis is a progressive interstitial pneumonitis occurring primarily in Japan. Rare cases are now being identified in Europe and North America. Patients often have a history of sinusitis, present with dyspnea on exertion, and show a restrictive pattern on pulmonary function tests. The clinical, radiologic, and pathologic features of two cases of the disease received for consultation at the Armed Forces Institute of Pathology, Washington, DC, are reported with a review of the literature. RESULTS: Chest radiographs revealed bilateral small nodular opacities with ill-defined borders. High-resolution computed tomography demonstrated the abnormalities to have a centrilobular distribution. Histologically, there was transmural chronic inflammation centered on the terminal bronchioles and an interstitial infiltrate of foamy macrophages. CONCLUSION: Diffuse panbronchiolitis may be mistaken for other more common small airway diseases and may be underrecognized in Western nations. The immigration of Asians and sporadic case reports involving non-Asians make recognition of this disease entity important, as the implications for therapy are different than that of other small airway diseases.
Adult↗
Primary mediastinal tumors: part II. Tumors of the middle and posterior mediastinum.
Lymphoma, mediastinal cysts, and neurogenic neoplasms are the most common primary middle and posterior mediastinal tumors. Lymphoma may involve the anterior, middle and/or posterior mediastinum, frequently as lymphadenopathy or as a discrete mass. Foregut cysts are common congenital mediastinal cysts and frequently arise in the middle mediastinum. Pericardial cysts are rare. Schwannoma and neurofibroma are benign peripheral nerve neoplasms, represent the most common mediastinal neurogenic tumors, and rarely degenerate into malignant tumors of nerve sheath origin. Sympathetic ganglia tumors include benign ganglioneuroma and malignant ganglioneuroblastoma and neuroblastoma. Lateral thoracic meningocele is a rare cause of a posterior mediastinal mass.
Combined Modality Therapy↗
Mediastinal mature teratoma: imaging features.
OBJECTIVE: The purpose of this study was to characterize the cross-sectional imaging features of mediastinal mature teratomas. MATERIALS AND METHODS: Sixty-six cases of mediastinal mature teratoma were retrospectively reviewed, noting clinical, radiologic, surgical, and pathologic findings. The patient population consisted of 38 females and 28 males who were 1 week to 67 years old (mean age, 23 years 10 months). RESULTS: Forty-six patients presented with symptoms, predominantly chest pain, dyspnea, and cough. Twenty patients were asymptomatic. Fifty-four tumors were found in the anterior mediastinum, two in the posterior mediastinum, one in the middle mediastinum, and nine in multiple compartments. CT studies (n = 66) showed masses of heterogeneous attenuation with varying combinations of soft tissue, fluid, fat, and calcium. Soft-tissue attenuation was observed in 66 tumors (100%), fluid in 58 tumors (88%), fat in 50 tumors (76%), and calcification in 35 tumors (53%). The most frequent combination of attenuations was soft tissue, fluid, fat, and calcium, which was noted in 26 masses (39%). The combination of soft tissue, fluid, and fat was seen in 16 tumors (24%); and the combination of soft tissue and fluid was seen in 10 tumors (15%). Fat-fluid levels were seen in seven masses (11%). CONCLUSION: Mediastinal mature teratoma typically manifests on CT as a heterogeneous anterior mediastinal mass containing soft-tissue, fluid, fat, or calcium attenuation, or any combination of the four. Fluid-containing cystic areas, fat, and calcification occur frequently. Cystic lesions without fat or calcium were seen in 15% of tumors. Fat-fluid levels, considered highly specific for the diagnosis of mediastinal mature teratoma, are uncommon. CT is the imaging technique of choice in the evaluation of these lesions.
Adolescent↗
From the archives of the AFIP. Malignant pleural mesothelioma: radiologic-pathologic correlation.
Malignant pleural mesothelioma (MPM) is a rare malignant neoplasm that typically affects individuals occupationally exposed to asbestos through a variety of industries. The patients experience an insidious onset of symptoms, including dyspnea, chest pain, cough, malaise, and weight loss. The pathologic diagnosis of MPM is difficult, and special stains or immunohistochemical or ultrastructural analysis may be required to differentiate MPM from metastatic adenocarcinoma. The tumor affects both the parietal and visceral pleural surfaces and progresses to encase the lung and invade the lung, mediastinum, and chest wall. Radiologically, MPM manifests as unilateral pleural effusion, pleural nodules, or pleural masses. Imaging studies are useful for diagnosis and staging in patients who are potential surgical candidates. Although a variety of multimodality therapies are available and radical surgical procedures have been developed, the prognosis remains dismal.
Aged↗
The alphabet soup revisited: the chronic interstitial pneumonias in the 1990s.
Liebow classified the idiopathic interstitial pneumonias as usual (UIP), desquamative (DIP), bronchiolitis obliterans (BIP), lymphoid (LIP), and giant cell (GIP) interstitial pneumonias. This classification was modified to exclude LIP and GIP. UIP, the most common type, is characterized by synchronous foci of inflammation, collagen deposition, and fibrosis with interspersed normal lung. It usually affects men 40-60 years old and manifests radiologically with bilateral, basilar irregular opacities and volume loss. In most cases, a confident diagnosis can be made at high-resolution computed tomography because of characteristic subpleural irregular linear opacities, ground-glass opacities, honeycombing, and traction bronchiectasis. DIP affects younger patients and is characterized by diffuse intraalveolar macrophage aggregation. Typical radiologic features include bilateral, basilar ground-glass opacities and preserved lung volumes. BIP, renamed bronchiolitis obliterans with organizing pneumonia, affects middle-aged patients and manifests with multifocal plugs of immature fibroblasts in the air spaces. Typical radiologic features include bilateral consolidations and normal lung volumes. Recently described entities include acute (AIP) and nonspecific (NIP) interstitial pneumonias and respiratory bronchiolitis with interstitial lung disease (RB-ILD). AIP is a rapidly progressive, often fatal, illness characterized by diffuse alveolar damage and manifests with clinical and radiologic features of adult respiratory distress syndrome. NIP is a heterogeneous group of fibrosing disorders that cannot be otherwise classified. RB-ILD is a disease of smokers with a good prognosis.
Adult↗
Tumors of the trachea.
Tumors of the trachea are rare and create signs and symptoms that mimic common upper airway diseases. A tracheal mass is not usually considered in the clinical differential diagnosis of an affected patient and is often overlooked on chest radiographs. The purpose of this article is to prompt earlier diagnosis and improve long-term survival of patients with a tracheal tumor through the presentation of the salient clinical, pathological, and radiological features of a variety of benign and malignant tracheal diseases.
Adolescent↗
Thoracic mycoses from endemic fungi: radiologic-pathologic correlation.
The endemic fungi Histoplasma capsulatum, Blastomyces dermatitidis, and Coccidioides immitis are primary human pathogens whose major portal of entry is the respiratory tract. Their clinical manifestations are categorized as acute, chronic or chronic progressive, or disseminated fungal disease. Most acute pulmonary infections are self-limited, and many are asymptomatic. Chronic, progressive, or disseminated disease is much less common and most often occurs in immunocompromised patients. The radiologic manifestations of these disorders are protean. They include interstitial or air-space opacities, solitary or multiple pulmonary nodules, parenchymal masses, cavities, and hilar or mediastinal adenopathy. The diagnosis of a thoracic mycosis requires familiarity with the epidemiology of the fungus in question, the various modes of clinical presentation, and the full spectrum of radiologic manifestations. Although skin and serologic tests can be useful, definitive diagnosis requires culture of the fungus from infected tissue or demonstration of the organism at microscopic examination.
AIDS-Related Opportunistic Infections↗
Thoracic mycoses from opportunistic fungi: radiologic-pathologic correlation.
Fungi of the genera Aspergillus, Candida, and Cryptococcus and the class Zygomycetes are the most common causes of thoracic opportunistic mycoses in immunocompromised patients. Candidiasis and zygomycosis usually manifest as severe, often life-threatening, pneumonias. Aspergillus species are commonly implicated as the causative organisms in a broad spectrum of pulmonary disorders, ranging from hypersensitivity lung disease in atopic patients to invasive pneumonia in immunocompromised patients. Cryptococcus neoformans infects both immunologically normal and abnormal patients, with variable clinical and radiologic findings. The diagnosis of an opportunistic mycosis requires familiarity with the epidemiology of the disease, the various modes of clinical presentation, and the full spectrum of radiologic manifestations. Because many of these fungi may normally colonize in the upper respiratory tract, sputum cultures are considered diagnostically unreliable. Instead, definitive diagnosis hinges on either culture of the fungus from infected tissue or demonstration of the organism at microscopic examination.
Adult↗
Thoracic sarcoidosis: radiologic-pathologic correlation.
Sarcoidosis is a systemic disease of unknown etiology with variable presentation, prognosis, and progression. At diagnosis, about 50% of patients are asymptomatic, 25% complain of cough or dyspnea, and 25% have skin lesions (erythema nodosum, lupus pernio, or plaques or scars) or eye symptoms (or develop them during the course of the disease). Bilateral hilar adenopathy is the most common radiographic finding. Other characteristic findings include interstitial lung disease, occasional calcification of affected lymph nodes, and pleural effusions and thickening. Computed tomography is more sensitive than radiography in the detection of adenopathy and subtle parenchymal disease; gallium-67 scintigraphy is useful in identifying extrathoracic sites of involvement, detecting active disease, and assessing response to treatment. The diagnosis is established most securely when clinicoradiologic findings are supported by histologic evidence of widespread noncaseating granulomas. The disease ranges from a self-limited subclinical process to chronic debilitation and death, with the major complications being fibrosis, mycetoma formation, and cor pulmonale. Because the disease so often involves thoracic structures, chest radiography plays a crucial role in the diagnosis, staging, and follow-up of sarcoidosis.
Adult↗
Bronchogenic carcinoma: radiologic-pathologic correlation.
Bronchogenic carcinoma is the leading cause of death from cancer in men and women in the United States. Although the cause of this malignancy is probably multifactorial, approximately 85% of lung cancer deaths are attributable to cigarette smoking. Patients may present with symptoms of airway obstruction caused by central tumors, symptoms related to direct tumor invasion of surrounding structures, or symptoms produced by distant metastases. There are four major cell types: adenocarcinoma, squamous cell carcinoma, undifferentiated large cell carcinoma, and small cell carcinoma. Adenocarcinoma and undifferentiated large cell carcinoma are generally peripheral lesions manifesting as solitary nodules or masses, whereas squamous cell carcinoma and small cell carcinoma are typically central and may manifest as hilar masses, atelectasis, or pneumonia. The prognosis for patients with bronchogenic carcinoma is poor, with an overall 5-year survival of 10%-15%. In general, patients with squamous cell carcinoma have the best prognosis, those with adenocarcinoma and undifferentiated large cell carcinoma have an intermediate prognosis, and those with small cell carcinoma have the worst prognosis.
Adenocarcinoma↗