[A 37-year-old woman with cough and bilateral nodular picture on a chest x-ray (clinico-pathological conference)].
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Adult↗
Biomedical subjects
E J Mark
Publications and source records attributed to E J Mark.
The pulmonary biopsy in the early diagnosis of Wegener's (pathergic) granulomatosis: a study based on 35 open lung biopsies.
We reviewed open lung biopsies from 35 patients with Wegener's (pathergic) granulomatosis in order to study the histogenesis of the pulmonary lesions and to identify the early lesions. The process of pathergic necrosis is fundamental in the production of extravascular and vascular lesions and was divided into micronecrotic and macronecrotic types. Micronecrosis, usually with neutrophils (microabscesses), constitutes the early phase in the development of the pathognomonic organized palisading granuloma. The palisading granuloma differs from the compact granuloma of tuberculoid type, which occurs in infections and sarcoidosis but not in Wegener's (pathergic) granulomatosis. There is a progression of disease from micronecrosis to macronecrosis (widespread necrosis) and then to fibrosis. Macronecrosis surrounded by palisading histiocytes or diffuse granulomatous tissue indicates active disease, whereas necrosis surrounded by fibrous tissue indicates previously active disease. Most cases have a combination of micronecrosis, and fibrosis. We established the relative diagnostic value of various histologic features. Arteritis and phlebitis as classically described in Wegener's granulomatosis were present in most but not all cases. We believe that Wegener's granulomatosis primarily affects both vascular and extravascular collagen and reticulum and that vasculitis represents a primary necrosis of walls of blood vessels. We believe that the concept of Wegener's granulomatosis as a vasculitis is too restrictive and does not include many cases with only extravascular histologic changes.
Adult↗
Inflammatory pseudotumors of the lung: progression from organizing pneumonia to fibrous histiocytoma or to plasma cell granuloma in 32 cases.
Clinicopathologic findings in 32 cases of inflammatory pseudotumor of the lung are described. Depending on the major histopathologic features, the cases are divided into three groups: Organizing pneumonia type (44%), fibrous histiocytoma type (44%), and lymphoplasmacytic type (12%). Organizing pneumonia type has intraalveolar lymphohistiocytic inflammation which converts to intraalveolar fibrosis peripherally and interstitial fibrosis centrally because of a proliferation of fibroblasts. Fibrous histiocytoma type has a predominant proliferation of spindle cells and histiocytes in storiform pattern, with loss of alveolar architecture. Lymphoplasmacytic type has a predominance of lymphocytes and plasma cells with little fibrosis. There is considerable histologic overlap among the three types. All cases have regions of organizing pneumonia. Most or all cases of inflammatory pseudotumor are believed to originate as organizing pneumonia.
Adolescent↗
Epithelioid sarcoma with diffuse bone marrow metastases and associated leukemoid reaction. A case report and brief literature review.
A patient with a recurrent epithelioid sarcoma of the hand was observed for five years with a diagnosis of nodular fasciitis. Eventually, the patient had a pulmonary metastasis and diffuse bone marrow spread, which was associated with a leukemoid reaction. Bone marrow dissemination by sarcoma is unusual, and its production of a leukemoid reaction very rare; this is its first reported occurrence in epithelioid sarcoma that the authors have been able to identify in the literature.
Adult↗
Thoracic hemangiomatosis masquerading as interstitial lung disease.
Pulmonary capillary hemangiomatosis is an extremely rare condition, first described by Wagenvoort. In this condition, sheets of thin-walled blood vessels infiltrate the lung parenchyma, bronchioles, and pleura. Three previous cases have been described in the literature. We describe a fourth. It probably represents a congenital abnormality.
Adult↗
Mesenchymal cystic hamartoma of the lung.
Mesenchymal hamartomatous nodules and cysts in the lungs caused hemoptysis, pneumothorax, hemothorax, pleuritic chest pain, dyspnea of slight or moderate degree, or a combination of these signs and symptoms in five patients. In four cases the disease was multifocal and bilateral. The nodules were composed of primitive mesenchymal cells subdivided into papillae by a plexus of small airways lined with respiratory epithelium. The nodules grew slowly in number and size over the years and apparently became cystic when they reached a diameter of about 1 cm. The cysts had a cambium layer of mesenchymal cells and were lined with normal or metaplastic respiratory epithelium. In general, the disease had an indolent course. The most serious complications were sudden hemorrhage into a cyst from large systemic arteries supplying the walls of the cysts, pneumothorax or hemothorax from rupture of a subpleural cyst, and malignant transformation in one case. This disease appears to represent a distinct clinicopathological entity, which I term mesenchymal cystic hamartoma of the lung.
Adult↗
Chronic Epstein-Barr virus infection associated with fever and interstitial pneumonitis. Clinical and serologic features and response to antiviral chemotherapy.
Two patients developed fever, interstitial pneumonitis, and pancytopenia associated with extremely high titers of antibody to replicative antigens of the Epstein-Barr virus. In contrast to most patients seropositive for Epstein-Barr virus, neither patient had an antibody response to the Epstein-Barr nuclear antigen K polypeptide. In addition, virus isolated from one patient had a deletion of the B95-8 type in the EcoRI C region of the genome. An etiologic relation between Epstein-Barr virus replication and the clinical manifestations of this syndrome is further shown by the response of each patient to acyclovir therapy. These patients have a new Epstein-Barr-virus-associated syndrome and provide additional evidence that acyclovir may play a role in therapy for selected patients with Epstein-Barr virus infection.
Acyclovir↗
Intrapulmonary lymph nodes. Clinical, radiologic, and pathologic features.
The clinical, radiographic, and pathologic material from ten patients with intrapulmonary lymph nodes was analyzed, along with information from all previously reported cases. In our series the median age was 56 years, and 80 percent (eight) of the patients were men. All patients are or were cigarette smokers. Sixty percent (six) had a history of exposure to either asbestos, nonfibrous silicates, or both. There were no characteristic symptoms attributable to the intrapulmonary lymph nodes. Forty percent (four) had an unexplained pulmonary nodule or nodules on the chest roentgenogram, which prompted further diagnostic studies, including thoracotomy, to rule out a malignant neoplasm. Radiographically, in our cases and in the literature, almost all cases of intrapulmonary lymph nodes are subpleural, inferior to the level of the carina, and less than 2.0 cm in diameter. Thirty-five percent are multiple. Pathologically, in our cases, all intrapulmonary lymph nodes were markedly anthracotic, and 60 percent were additionally silicotic. Although their histogenesis is uncertain, intrapulmonary lymph nodes are probably hyperplastic lymphoid nodules related to inhalation of irritant dusts and attendant distortion of local lymphatic vessels. Precise clinical and radiographic analysis correlated carefully with cytologic and histologic material obtained by percutaneous needle biopsy should obviate exploratory thoracotomy in most instances.
Adult↗
Congenital polycystic tumor of the atrioventricular node. Autopsy study with immunohistochemical findings suggesting endodermal derivation.
Atrioventricular node congenital polycystic tumor, a lesion that causes heart block and sudden unexpected death, has been postulated to arise from the lymphatics, foregut endoderm, cardiac mesoderm, and mesothelium since its initial description in 1911. No theory concerning the histogenesis of this tumor has been completely satisfactory, either from an embryologic or a clinical standpoint. Biologic studies including cell culture systems from the lesions as well as optimal ultrastructural examination have been compromised due to postmortem autolysis. We studied four examples of this entity by standard histochemical stains for mucopolysaccharides, and by modified immunohistochemical labeling for carcinoembryonic antigen, factor VIII, and keratin. Results suggest an origin from foregut endoderm. The lesion has not been reported to be outside the region of the atrioventricular junction, but was demonstrated in the thymus gland in one of our cases.
Adult↗
Peripheral small-cell carcinoma of the lung resembling carcinoid tumor. A clinical and pathologic study of 14 cases.
We studied 14 small-cell, epithelial tumors of the lung with histologic features intermediate between typical carcinoid tumor and undifferentiated small-cell carcinoma. All of the tumors arose in the periphery of the lung beyond a segmental bronchus and were excised. Histologically, three of the tumors were low grade and 11 were high grade. The low-grade tumors had an organoid pattern comprising more than half of the area examined histologically, less than five mitoses per 10 high-power fields, individual cell necrosis, and nuclear pleomorphism of less than half of the cells. The high-grade tumors had the opposite characteristics. Distinction of these peripheral tumors from typical carcinoid tumors and from undifferentiated small-cell carcinomas is established after excision and cannot be made reliably on the findings of a transbronchial or needle biopsy. Prognosis was worse than that for typical carcinoid tumors but better than that for undifferentiated small-cell carcinomas.
Carcinoid Tumor↗
Pulmonary capillaritis and hemorrhage in patients with systemic vasculitis.
Thirteen patients with prominent pulmonary signs and symptoms had pulmonary capillaritis and extensive hemorrhage demonstrated by open-lung biopsy or autopsy. Vasculitis was demonstrated in other organs before or after the lung biopsy or at autopsy. Although there were several suspected causes for the pulmonary capillaritis and different final clinicopathologic diagnoses, the histopathologic features in the lung were similar in all cases and distinctive enough to separate capillaritis from other causes of hemorrhagic lung. All patients were treated with prednisone or cyclophosphamide, or both. Six patients died of their vasculitis, five in respiratory failure and one in renal failure. None of the seven survivors had a clinical recrudescence of pulmonary hemorrhage. By extrapolation from these 13 cases, one may histopathologically recognize pulmonary capillaritis when it causes hemorrhagic lung in a patient without clinically evident extrapulmonary vasculitis. One can then proceed to investigate the patient and possibly determine the nature of the vasculitis.
Adolescent↗
Primary Kaposi's sarcoma of the lung in an immunocompetent 32-year-old heterosexual white man.
Primary visceral involvement by Kaposi's sarcoma in the immunocompetent adult American population is extremely rare. Six cases of primary Kaposi's sarcoma of the lung have been previously reported; all four patients in whom immune function was evaluated had abnormalities. A case of primary Kaposi's sarcoma of the lung in a young adult heterosexual man with normal immunologic function is described. The patient had no response to either weekly vinblastine or doxorubicin (Adriamycin). A subsequent solitary right occipital metastasis responded to 3500 rad of radiotherapy. The patient died 3 months after diagnosis; postmortem examination revealed only two small nodules of extrathoracic Kaposi's sarcoma, one in the liver and the other in the right posterior occipital lobe of the brain. Bacterial, mycobacterial, fungal, and viral cultures were negative.
Adult↗
Benign lymphoid disorders of the lung, with a theory regarding their development.
The authors review the clinical, radiographic, and pathologic features of the benign pulmonary lymphoid disorders. The essential role of immunopathologic techniques in distinguishing pulmonary lymphoid hyperplasias from malignant lymphoma is stressed. A theory regarding the development of pulmonary lymphoid hyperplasia is proposed as a basis for understanding these disorders. The authors suggest that lymphoid hyperplasia is a basic inflammatory response of the lung.
Animals↗
Immunoblastic lymphoma arising in chronic lymphoid hyperplasia of the pulmonary interstitium.
Chronic pulmonary lymphoid hyperplasia occurs alone or is associated with a variety of disorders of the immune system. It may be a diffuse or localized process and has been referred to as lymphoid interstitial pneumonitis or pseudolymphoma, respectively. Chronic lymphoid hyperplasia in other organs has been followed by the development of malignant lymphoma, particularly when associated with disorders of the immune system. This report describes two patients with chronic hyperplasia of the pulmonary interstitial lymphoid tissue who had malignant lymphoma, immunoblastic type found at autopsy. Long-term steroid usage in one patient suggests the possibility that immunosuppressive therapy may increase the risk of developing malignant lymphoma in patients with chronic pulmonary lymphoid hyperplasia.
Aged↗
Pulmonary blastoma with argyrophil cells and lacking sarcomatous features (pulmonary endodermal tumor resembling fetal lung).
A tumor of the lung in a 35-year-old woman contained numerous complex, branching tubules resembling the developing fetal lung in its canalicular state. Some of the epithelial cells within the tubules were argyrophilic, and electron microscopy demonstrated the dense-core neurosecretory-type cytoplasmic granules. These characteristics support an endodermal origin for this neoplasm. Morphologically the tubules are similar to the epithelial component seen in pulmonary blastoma, but the tumor lacks the sarcomatous areas integral to the concept of blastoma. The designation pulmonary endodermal tumor resembling fetal lung may better describe this unusual neoplasm.
Adult↗
Arteriography in the localization of massive pleural tumors.
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Adult↗
Histopathologic diagnosis of pityriasis lichenoides et varioliformis acuta and its clinical correlation.
To assess the specificity of the histopathologic features in the diagnosis of pityriasis lichenoides et varioliformis acuta (PLEVA), we reviewed the clinical manifestations and courses of 42 patients for whom this diagnosis was suggested in the pathology report. The histologic diagnosis of PLEVA was clinically substantiated in 16 of these 42 cases. Of the 26 cases in which PLEVA was erroneously diagnosed histologically, the correct clinical diagnosis was suggested before biopsies were done in 21 instances. In the five remaining cases, both the prebiopsy clinical diagnosis and the pathologic diagnosis proved to be incorrect. Pityriasis rosea, insect bites, and eczematous dermatitis accounted for the majority of the cases that histologically mimicked PLEVA. The constellation of histologic findings described in PLEVA (presence of intraepidermal lymphocytes and erythrocytes, dermal hemorrhage, and so-called lymphocytic vasculitis) is not specific and may be seen in a variety of dermatologic disorders.
Biopsy↗
Papillary carcinoid tumor of the lung.
Carcinoid tumors of the lung have a wide histologic spectrum. The histologic differential diagnosis of papillary tumors in the lung generally does not include carcinoid. A carcinoid tumor that formed a discrete coin lesion on chest radiograph is presented in this report. On gross examination the center appeared and felt spongy. On microscopic examination delicate, compacted papillae were separated by a serpentine space continuous with air spaces at the periphery of the tumor. The papillae were each composed of a fibrovascular core, an undulating basement membrane separating stroma from epithelial cells, and cuboidal clear and dark cells that proved to be, respectively, healthy and degenerate cells of carcinoid tumor with neurosecretory granules. Upon the carcinoid cells, draped in the manner of an umbrella, were nonciliated, respiratory cells that proved to be Clara cells. The various histologic patterns of bronchopulmonary carcinoids and the differential diagnosis of papillary tumors within the lung are tabulated.
Aged↗