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E J Mark

Publications and source records attributed to E J Mark.

At least 37 records · Page 2Linked to original sources
PubMed1999

Primary pulmonary sarcoma: a clinicopathologic study of 26 cases.

The clinical and pathologic features of 26 primary pulmonary sarcomas were analyzed. Fourteen patients were male and 12 were female; ranging in age from 18 to 75 years (mean, 48 yr). The tumors measured from 0.9 cm in greatest diameter to filling the entire hemithorax. Thirteen tumors were in the left lung and nine in the right lung; one was bilateral, two were in the pulmonary artery, and the location of one tumor was not available. The histologic diagnoses were malignant fibrous histiocytoma (7), synovial sarcoma (6), malignant peripheral-nerve sheath tumor (3), leiomyosarcoma (3), angiosarcoma (2), intimal sarcoma (2), fibrosarcoma (2), and one case of epithelioid hemangioendothelioma. Immunohistochemical and ultrastructural examination supported these diagnoses. Morphologically, the differential diagnosis often included sarcomatoid carcinoma or desmoplastic malignant mesothelioma Patients were treated with surgery, chemotherapy, radiation therapy, or a combination of these. Follow-up was available for 22 patients and ranged from 2 to 183 months (mean, 45 mo). Fourteen patients are free of disease, four died of disease, three are alive with disease, and one died of surgical complications. A variety of sarcomas, especially malignant fibrous histiocytoma and synovial sarcoma, arise within the pulmonary parenchyma. These tumors have the potential to behave aggressively but can be cured by resection, with or without adjuvant therapy. Immunohistochemistry and electron microscopy can be helpful in distinguishing primary pulmonary sarcoma from other tumors in the differential diagnosis.

Adolescent↗
PubMed1998

Pleuro-pulmonary endometriosis and pulmonary ectopic deciduosis: a clinicopathologic and immunohistochemical study of 10 cases with emphasis on diagnostic pitfalls.

The clinical and pathological features of nine cases of pleuro-pulmonary endometriosis and the first case of pulmonary ectopic deciduosis are presented. The patients were all women between the ages of 27 and 74 years (median, 36 years) who presented with symptoms of catamenial pleural pain, shortness of breath, hemoptysis, or radiographically detected lung masses. Clinically, six patients were multiparous, one patient had pelvic endometriosis, and four patients had undergone prior pelvic surgical procedures, including dilatation and curettage. Radiographically, eight patients had pulmonary infiltrates or nodules, and four patients had pneumothorax. Three cases involved the visceral pleura and one case the parietal pleura. The other six cases, including the single case of ectopic deciduosis, involved the lung parenchyma. Histologically, the single or multifocal lesions were well circumscribed or infiltrative, nodular, cystic, or nodulo-cystic, and showed the characteristic features of proliferative or secretory endometrium with numerous mullerian metaplastic changes. Mucin stains were negative in five cases of endometriosis and in the single case of ectopic deciduosis. Immunohistochemical studies were performed in these same six cases using antibodies to epithelial, mesenchymal, vascular, and neuroendocrine markers. The glandular epithelium was decorated with antibodies to pan-cytokeratin, CK7, BER-EP4, ER, and PR, whereas the stromal cells showed positive staining for vimentin, actin, smooth muscle actin, desmin, ER, and PR. Follow-up information obtained in seven patients showed all women without recurrences after 1 to 20 years. The current study highlights the importance of recognizing intrathoracic endometriosis and ectopic deciduosis and properly assessing small biopsy specimens to avoid a misdiagnosis of malignancy.

Adult↗
PubMed1998

Pulmonary adenocarcinomas of the fetal lung type: a clinicopathologic study indicating differences in histology, epidemiology, and natural history of low-grade and high-grade forms.

Seven cases of high-grade adenocarcinoma of fetal lung type (H-FLAC) are compared with nine cases of pulmonary endodermal tumor resembling fetal lung or low-grade adenocarcinoma of fetal lung type (L-FLAC). Of the seven patients with of H-FLAC, four were men and three were women. All of the patients but one were in their 60s or 70s. Five patients were smokers. After resection of the tumor, three patients died of metastases, two patients are alive with no evidence of disease, and two patients died of a postoperative complication. Histologically, H-FLAC and L-FLAC have both complex glandular structures resembling fetal lung and neuroendocrine differentiation. Two cases of H-FLAC had stromal proliferation typical of biphasic pulmonary blastoma. The H-FLAC was distinguished from L-FLAC by the presence of disorganized glands, large vesicular nuclei, prominent nucleoli, pronounced anisonucleosis, absence of morules, transition to conventional adenocarcinoma, broad areas of necrosis, desmoplastic stroma, overexpression of p53 protein, and production of alpha-fetoprotein. High and low grades of FLAC explain discrepancies in previously reported clinicopathologic features of FLAC. The H-FLAC needs to be distinguished from L-FLAC. Both forms may have stromal components, so both have been referred to as blastomas. The H-FLAC represents the prototype of so-called pulmonary blastoma predominantly seen in the elderly, whereas L-FLAC and its biphasic form predominate in the middle-aged population.

Adenocarcinoma↗
PubMed1997

Treated Wegener's granulomatosis: distinctive pathological findings in the lungs of 20 patients and what they tell us about the natural history of the disease.

Patients with an established diagnosis of Wegener's granulomatosis (WG) sometimes undergo lung biopsy when the disease does not behave in the expected manner. Treatment affects the tissue reaction. The microscopic recognition of partially treated disease is important, as the absence of expected lesions may lead to nonspecific diagnoses and inappropriate management. The appearance of treated disease over time may offer insight into its histogenesis and natural history. We correlated clinical features and pulmonary histology in 20 patients with WG after they had been treated with corticosteroids or cyclophosphamide or both. All patients had inflammatory or fibrotic pulmonary disease resulting from WG, but only 4 (20%) had macronodular necrosis typical of WG. Serum antineutrophil cytoplasmic antibody (ANCA) was elevated in all patients in whom it was measured. We divided the pathological findings into (1) vasculitis, (2) extravascular necrosis, (3) bronchiolitis, and (4) other lesions, and further divided them into (a) diagnostic for active disease, (b) suspicious for active disease, (c) suspicious for healing disease, (d) suspicious for residual disease, and (e) possible disease. Diagnostic or suspicious vascular lesions occurred in 15 patients (75%) and included granulomatous vasculitis, capillaritis or suspicious capillaritis, and neutrophilic vasculitis. Diagnostic or suspicious extravascular lesions occurred in 12 patients (60%) and included palisading granuloma, microabscess, macronodular pathergic necrosis, giant cell nodules, and micronodular scars. The giant cell nodules and nodular scars were an unusual healing pattern of palisading granulomas. Diagnostic bronchiolar lesions occurred in 1 patient (6%) and suspicious lesions in 13 patients (65%), including three novel patterns of bronchiolitis fibrosa (BF): (1) BF with giant cells, (2) BF with hemosiderin, and (3) BF with micronodular scars. Other features related to WG included diffuse alveolar damage, peculiar alveolar fibrin, interstitial fibrosis, pneumonitis resembling usual interstitial pneumonitis, and lipoid pneumonia. Classic necrotic nodules and vasculitis of WG should not be anticipated after therapy, but the diagnosis of pulmonary WG after treatment may be made if the effects of treatment on histology are considered. Changes in anticipated histology are found after therapy as short as 6 days. The histology typically has muted features. BF develops in most patients and may reflect a salutary effect of therapy. Palisading granuloma may convert to giant cell nodule or micronodular scar. Interstitial fibrosis is common, and pneumonitis resembling usual interstitial pneumonitis can develop. If only healing or residual disease is encountered, one should search further clinically and pathologically for active disease. Dampened inflammatory lesions represent smoldering disease that presumably needs additional therapy. Scarring presumably represents successfully treated but permanent disease.

Adult↗
PubMed1997

Pulmonary cholesterol crystal embolization.

BACKGROUND: Cholesterol crystal embolization (CCE) has been documented to affect nearly every organ system. However, CCE involving the lung is distinctly uncommon and has been documented only in the setting of an aortocaval fistula. DESIGN: A case at the Massachusetts General Hospital and a MEDLINE search of English-language medical articles published between 1966 and 1997 provide the basis for this report. RESULTS: The precipitants of CCE include invasive vascular procedures, anticoagulant therapy, and thrombolysis. The most common symptoms include claudication of the calf, gastrointestinal bleeding, and weight loss. The most common signs include livedo reticularis, gangrene, and ulcers. Azotemia, proteinuria, normocytic anemia, and eosinophilia often are found. Herein is described the first pathologically confirmed case of CCE to the lung in the absence of an arteriovenous fistula. CONCLUSION: Pulmonary hemorrhage should now be included in the diverse list of presenting signs of CCE. Moreover, CCE should be considered in the differential diagnosis of pulmonary-renal syndromes.

Aged↗
PubMed1997

Routine addition of an automated biopsy device to fine-needle aspiration of the lung: a prospective assessment.

OBJECTIVE: The purpose of this study was to prospectively assess the usefulness of the routine addition of an automated biopsy device (ABD) to fine-needle aspiration (FNA) of the lung and to examine the complication rate of this procedure. SUBJECTS AND METHODS: Fifty biopsies were performed under CT guidance using a coaxial technique with a 19-gauge introducer needle and a 22-gauge aspirating needle followed by a 20-gauge ABD. An average of 3.5 FNA specimens and 2.5 core specimens were obtained. Cytology and histology specimens were interpreted separately by two experienced pathologists who were unaware of the other's interpretation. Final diagnoses were based on surgery, microbiology, definitive biopsy diagnosis, and clinical follow-up. All complications were recorded. RESULTS: Of 34 malignant lesions, we achieved a diagnostic accuracy of 94% for FNA and 59% for core biopsy (p < .01). Combined accuracy was 94%. Of 16 benign lesions, an accurate definitive diagnosis was made in 31% of cases using FNA and in 69% of cases using core biopsy (p = .08). Combined accuracy was 69%. In the subset of benign lesions that were not acute infections (n = 8), an accurate definitive benign diagnosis was made in 12% of cases using FNA and in 75% of cases using core biopsy (p < .05). No false-positive diagnoses of malignancy occurred. Complications included pneumothorax, nine (18%) of 50 cases; chest tube, one (2%) of 50 cases minor pulmonary hemorrhage, seven (14%) of 50 cases; and minor hemoptysis, two (4%) of 50 cases. CONCLUSION: The complication rates of FNA with the addition of an ABD are similar to those reported in the literature for FNA alone. The addition of an ABD significantly increases the diagnostic accuracy only for the subset of benign lesions that are not acute infections.

Adolescent↗
PubMed1996

Barrett's esophagus: metaplastic cells with loss of heterozygosity at the APC gene locus are clonal precursors to invasive adenocarcinoma.

Adenocarcinoma in Barrett's esophagus is the second most rapidly increasing cancer in western society. The cause and pathogenesis are unknown. Although histological studies suggest that there is successive progression from metaplasia and dysplasia, with a high risk of subsequent invasive carcinoma, at present there is no direct evidence that metaplastic and dysplastic epithelia are clonal precursors of adenocarcinoma. We selected 12 esophagectomy specimens of Barrett's esophagus patients, which showed a spectrum of normal tissue, metaplasia, dysplasia, and invasive carcinoma in each individual biopsy. We applied the microdissection technique to selectively procure microscopic tissue samples from H&E-stained slides for genetic evaluation using polymorphic markers flanking the APC gene locus. Identical APC gene alterations were found in the dysplastic and adenocarcinoma foci of all informative cases. The same changes were observed even in some metaplastic foci adjacent to dysplasia. Furthermore, clonality analysis of X-chromosome inactivation in female cases verified the same X-chromosome inactivation pattern in carcinoma, dysplasia, and metaplasia adjacent to dysplasia. No APC gene alterations were found in the normal epithelium and metaplasia distant from dysplasia. These data show for the first time that a tissue field of genotypic changes precedes the histopathological phenotypic changes of carcinoma in Barrett's esophagus syndrome. Our findings, in conjunction with the applied tissue microdissection technique, may help identify genotypic changes in patients with Barrett's esophagus before phenotypic changes occur. Therefore, genotyping of Barrett's metaplastic epithelium may supplement the histopathological evaluation of Barrett's esophagus.

Adenocarcinoma↗
PubMed1996

Nasal biopsy in the early diagnosis of Wegener's (pathergic) granulomatosis. Significance of palisading granuloma and leukocytoclastic vasculitis.

The diagnostic value of the nasal biopsy in the early diagnosis of Wegener's granulomatosis and its value in prognosis were examined in 11 patients with a clinicopathological diagnosis of the disease. The vascular lesions found included microabscess in the vascular walls in 82%, leukocytoclastic capillaritis in 73%, fibrinoid necrosis of blood vessels in 45%, leukocytoclastic endovasculitis in 27%, and palisading granuloma in vascular wall in 9% of cases. The extravascular lesions included palisading granuloma in all cases, microabscess in 91%, and diffuse granulomatous tissues in 82%. Palisading microgranuloma (82%) was more frequent than palisading macrogranuloma (45%). After therapy, complete remission occurred in 8 patients, but 3 patients died of sepsis, diffuse pulmonary haemorrhage, and cerebral haemorrhage. Comparison of the frequency of each finding in the nasal biopsy specimens between patients who achieved remission and those who died showed that leukocytoclastic vasculitis was found more commonly in fatal cases, and leukocytoclastic endovasculitis was observed only in fatal cases. Palisading granuloma as a vascular or extravascular lesion is the primary and most important finding in a histopathological diagnosis of Wegener's granulomatosis, microabscess in vascular walls is a secondary but the next most important finding, and leukocytoclastic vasculitis heralds dissemination of the disease and poor prognosis. It requires aggressive therapy.

Adult↗
PubMed1995

Placentoid bullous lesion of the lung.

Four adult patients presented with unilateral multicystic lung disease. The cysts had been detected years previously on chest radiographs in three patients. Two patients had histories of repeated childhood pneumonias. Preoperative diagnosis of bullous emphysema with rupture was made in three patients who presented with pneumothorax. Lobectomy was done in two patients and pneumonectomy in two patients. Macroscopically, each lung was spongy with cysts that contained gelatinous vesicular or grape-like structures resembling normal or molar placental tissue. Bullous emphysema was evident in one lung and marked panacinar emphysema in another. In one patient who had a lobectomy, the ipsilateral lobe of compressed lung re-expanded after surgery and proved to be cystic as well on subsequent radiographs. The vesicular and grape-like structures histopathologically are papillary structures with central edematous cores and a covering of cuboidal epithelial cells. Degenerate villi became either hydatidiform, fibrotic, or calcified. At low magnification the histology was similar to that of placenta with hydatidiform change. Emphysematous lung in which villiform structures appeared at the edges of bullae was found histologically in two patients. Lymphangiectasia was present in all patients. The placentoid bullous lesion has distinct macroscopic and microscopic features and is clinically unusual in that the cysts are unilateral and appear in otherwise healthy young adults. We do not know whether the lesion is a malformation or a peculiar devolution of localized bullous emphysema. We favor the latter interpretation. Torsion of lung may cause the lymphangiectasia and contribute to the unusual histology. Excision is curative.

Adult↗
PubMed1995

Tumefactions as an extravascular manifestation of Wegener's granulomatosis.

Wegener's granulomatosis appeared as localized or infiltrative tumefactions in six patients. The tissue swellings affected the retroperitoneum, mediastinum (two), breast, retroorbital tissue (two), and gingiva. There was extensive fibroblastic proliferation in each case with a predominantly granulocytic inflammatory component, microabscess formation, and collagen necrobiosis. In one of the mediastinal lesions and in the gingiva, there was a granulomatous component as well. In the breast were scattered multinucleated histiocytes. Necrotizing vasculitis was absent or limited in all cases. All patients responded dramatically to cyclophosphamide and/or prednisone after the diagnosis was established. These observations indicate a role for tissue destruction in Wegener's granulomatosis mediated by granulocytes without a vasculitic component.

Adult↗
PubMed1995

Alveolar basement membrane breaks down in diffuse alveolar damage: an immunohistochemical study.

Sequential structural changes of the alveoli in diffuse alveolar damage (DAD) were examined by immunohistochemical methods. Lung specimens obtained at autopsy from 52 patients with DAD were stained with antibodies to laminin, 7S collagen (7S) and type IV collagen (type IV) for alveolar basement membrane, to von Willebrand factor, CD-31 and thrombomodulin (TM) for the alveolar capillary endothelial cell, and to epithelial membrane antigen and surfactant apo-protein (PE-10) for the alveolar epithelium. Forty-two of the patients had the exudative form of DAD; 10 of the patients had the proliferative form of DAD. The results were summarized as follows: (i) laminin was most easily impaired both in the epithelial and capillary basement membrane in the early exudative stage; (ii) following laminin, 7S and type IV in the capillary basement membrane were also injured in the early exudative stage, and recovered in the proliferative stage; (iii) subsequently, 7S and type IV in the epithelial basement membrane were also impaired in the late exudative stage, and remained impaired even in the proliferative stage; and (iv) alveolar epithelium regenerated almost completely in the late exudative stage, but staining for TM in the alveolar capillary recovered in the proliferative stage. Because the alveolar basement membrane must govern the homeostasis of alveolar tissue architecture, it was concluded that its preservation is necessary to avoid the abnormal remodeling of the alveoli in the reparative stage of DAD, if the patient survives the acute episodes of the disease.

Adolescent↗
PubMed1993

Diffuse malignant mesothelioma of the pleura: a clinicopathological study of six patients with a prolonged symptom-free interval or extended survival after biopsy and a review of the literature of long-term survival.

Diffuse malignant mesothelioma of the pleura generally proceeds rapidly with clinical deterioration soon after the initial histopathological diagnosis of the tumor. We encountered six patients in whom the symptom-free interval after biopsy was surprisingly prolonged. Histopathologically, the tumor in each case was epithelioid with prominent myxoid stroma and formation of tubules and micropapillae. Cytologically, the malignant cells had relatively regular nuclei with bland chromatin. As individual findings, these histological and cytological features are common in diffuse malignant mesothelioma. However, the combination of (1) bland cytology of epithelioid cells, (2) formation of tubular and micropapillary patterns, (3) a myxoid stroma and (4) an absence of sclerosing desmoplasia, as observed in these six cases, might indicate indolent disease at the outset in a few patients. It is possible that these features are related to early stage of disease rather than having discriminatory value in their own right. We do not conclude that initially restrained disease necessarily presages a longer mean survival because the follow-up in these patients has not been long enough. We present these observations because, to date, this malignancy has proven so refractory to clinical management.

Adult↗
PubMed1993

Idiopathic laryngotracheal stenosis and its management.

We describe idiopathic laryngotracheal and upper tracheal stenosis in 49 patients with no other cause for their stenosis. Traumatic, iatrogenic, infectious, and specific inflammatory processes were excluded. Histopathologically dense fibrosis of keloidal type thickened the lamina propria and choked the ducts of mucous glands but did not destroy cartilage. Thirty-five patients were treated by single-stage resection and reconstruction: 29 by laryngotracheal resection with laryngotracheoplasty and 6 by cricotracheal segmental resection. Thirty-two patients achieved good or excellent results in respiration and voice, 2 needed annual dilations, and 1 required permanent tracheostomy.

Adolescent↗