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M N Koss

Publications and source records attributed to M N Koss.

At least 19 recordsLinked to original sources
PubMed1998

Reproducibility of neuroendocrine lung tumor classification.

For a tumor classification scheme to be useful, it must be reproducible and it must show clinical significance. Classification of neuroendocrine lung tumors is a difficult problem with little information about interobserver reproducibility. We sought to evaluate the classification of typical carcinoid (TC), atypical carcinoid (AC), large-cell neuroendocrine carcinoma (LCNEC), and small-cell carcinoma (SCC) tumors as proposed by W.D. Travis et al (Am J Surg Pathol 15:529, 1991). Forty neuroendocrine tumors were retrieved from the Armed Forces Institute of Pathology (AFIP) files and independently evaluated by five lung pathologists and classified as TC, AC, LCNEC, or SCC (pure SCC, mixed small cell/large cell, and combined SCC). A single hematoxylin and eosin-stained slide from each case was reviewed. Each participant was provided a set of tables summarizing the criteria for separation of the four major categories. Agreement was regarded as unanimous if all five pathologists agreed, a majority if four agreed, and a consensus if three or more pathologists agreed. The kappa statistic was calculated to measure the degree of agreement between two observers. A consensus diagnosis was achieved in all 40 cases (100%), a majority agreement in 31 of 40 (78%), and unanimous agreement in 22 of 40 (55%) of cases. Unanimous agreement occurred in seven of SCC (70%), seven of TC (58%), four of AC (50%), and four of LCNEC (40%). A majority diagnosis was achieved in 11 of 12 (92%) of TC, 9 of 10 (90%) of SCC, 6 of 8 (75%) of AC, and 5 of 10 (50%) of LCNEC. Most of the kappa values were 0.70 or greater, falling into the substantial agreement category. The most common disagreements fell between LCNEC and SCC, followed by TC and AC, and AC and LCNEC. The highest reproducibility occurred for SCC and TC, with disagreement in 8% and 10% of the diagnoses, respectively. For TC, 10% of the diagnoses rendered were AC. For AC, 15% of the diagnoses were rendered as TC, with 2.5% called LCNEC and 2.5% called SCC. For LCNEC, 18% and 4% of the diagnoses were called SCC and AC, respectively. For SCC, 4% of the diagnoses were called AC and 4% were called LCNEC. Thus, using the classification scheme tested, a consensus diagnosis can be achieved for virtually all neuroendocrine lung tumors with substantial agreement between experienced lung pathologists. Classification of NE tumors is most reproducible for classification of TC and SCC but less reproducible for AC and LCNEC. These results indicate a need for more careful definition and application of criteria for TC versus AC and SCC versus LCNEC.

Carcinoid Tumor↗
PubMed1998

Primary pulmonary ganglioneuroblastoma: a clinicopathologic and immunohistochemical study of two cases.

We present two patients with primary ganglioneuroblastoma involving the bronchial wall. The first, a 38-year-old woman, presented with signs and symptoms suggestive of multiple endocrine neoplasia, including gastric ulceration and hypercalcemia. Chest radiographic studies revealed a 3-cm nodule in the hilus of the right lung and two less-pronounced lesions in the periphery of the right lung. The second, a 20-year-old asymptomatic woman, was evaluated for a solitary mass in the upper lobe of the left lung that was peribronchial and that impinged on the lumen of a bronchus. Grossly, both neoplasms extended from bronchi, were well-circumscribed, firm, tan or white, and homogeneous, and measured 5 x 5 cm and 3 x 3 cm, respectively. Histologically, both tumors were characterized by neuroblastoma with areas of neuropil and multifocal areas of ganglion cells. Immunohistochemical studies performed in one case showed focal staining for neurofilament protein and S-100 protein and diffuse staining for neuron-specific enolase. Follow-up information showed that one patient died a few days after admission to the hospital; the second patient has remained well and without evidence of recurrence or metastases 1 year after initial diagnosis. These two cases confirm that ganglioneuroblastoma can occur as a primary pulmonary tumor in adults, presumably arising from sympathetic ganglia of the bronchus.

Adult↗
PubMed1998

Constrictive (obliterative) bronchiolitis: diagnosis, etiology, and a critical review of the literature.

Constrictive bronchiolitis (CB) (or obliterative bronchiolitis) designates inflammation and fibrosis occurring predominantly in the walls and contiguous tissues of membranous and respiratory bronchioles, with resultant narrowing of their lumens. It differs from bronchiolitis obliterans-organizing pneumonia in its histopathology and clinical course. Most cases of CB occur in the setting of organ transplants, particularly lung and heart-lung transplants, but also in bone marrow transplants. Other bona fide cases are rare: infection, particularly viral infection, appears to be a well-documented precursor to CB in children, but not in immunocompetent adults. Constrictive bronchiolitis also has been reported in the course of rheumatoid arthritis, in certain other autoimmune diseases such as pemphigus vulgaris, after inhalation of toxic gases such as nitrogen oxide, after ingestion of certain drugs or medicinal agents such as Sauropus androgynous, and as a cryptogenic illness. Recent reports suggest that CB, as defined by clinical criteria (that is, bronchiolitis obliterans syndrome), is very common in lung allograft recipients who survive more than 5 years and, although it is associated with significant mortality, it also can be clinically stable. Furthermore, with the current practice of close monitoring of these patients, it appears that CB may now be diagnosed at an earlier stage, at which resolution, or at least stabilization of progression, is possible. A histopathologic diagnosis of CB in lung transplant and other patients may be difficult to make due to the patchy distribution of lesions, the technical difficulty in obtaining tissue in late lesions with extensive fibrosis, and the failure to recognize lesions. With regard to the last of these, in early stages of disease, CB may be subtle and easily missed in routine hematoxylin-eosin-stained specimens, while in advanced stages the disease may be equally difficult to diagnose if the patchy scarring in the lung is interpreted as nonspecific. The relative loss of bronchioles and the relationship of the scars to contiguous arteries should signal the need for elastic stains to look for the residual elastica of the bronchioles amidst the foci of fibrosis. Increasingly, clinical grounds, including pulmonary functions studies and high-resolution computed tomography findings, are proving to be relatively sensitive methods of detecting CB. Finally, the progressive airway destruction in chronic transplantation rejection appears to be a T-cell-mediated process. The "active" form of constrictive bronchiolitis, with attendant lymphocytic inflammation of the airways, likely precedes the "inactive" or scarred form of constrictive bronchiolitis.

Adult↗
PubMed1998

Pulmonary plasmacytomas: a clinicopathologic and immunohistochemical study of five cases.

Five cases of primary plasmacytoma of the lung are presented. The patients were four men and one woman between the ages of 50 and 79 years (mean age, 57 years; median age, 54 years). Two patients presented with symptoms related to their tumor; these included cough, dyspnea, and hemoptysis. In two patients, the tumor presented as a hilar mass, whereas in the remaining three patients, the tumor was located intraparenchymally. Clinically, only one patient had a reported monoclonal gammopathy (IgG, kappa). Because of the proximal location of these tumors, three patients underwent pneumonectomy; one other underwent a lobectomy, and one had a segmental resection. Grossly, the tumors ranged from 2.5 to 8 cm in maximum diameter (mean, 4.4 cm); they were either peribronchial or involved a major bronchus. Histologically, they were characterized by sheets of plasma cells that were well differentiated in two cases and moderately differentiated in three. Amyloid was present in two cases. In four tumors, there was a monoclonal population of lambda light chain-bearing plasma cells, whereas in one, the plasma cells expressed a monoclonal kappa light chain. The tumor cells predominantly expressed IgG heavy chains in two cases. Peribronchial and mediastinal lymph nodes were involved in three cases. Follow-up information ranged from 4 days to 262 months (average, 115 months; median, 36 months). Two patients survived more than 20 years before dying of non-tumor-related causes. Two patients died 28 months and 4 days after surgery with concurrent tumor in liver and mediastinal and para-aortic lymph nodes. Comparing the present cases and those reported in the literature, we noted that the patients herein presented are, on average, older than those published previously. Combining our cases with 14 other verifiable, previously published cases, the overall 2- and 5-year survivals of pulmonary plasmacytomas are 66% and 40%, respectively. Patients with pulmonary plasmacytomas can have a long-term survival, as evidenced by two of our patients who survived 20 or more years.

Aged↗
PubMed1998

Adenocarcinoma simulating mesothelioma: a clinicopathologic and immunohistochemical study of 29 cases.

We report 29 cases of adenocarcinomas whose clinical, gross, and microscopic appearance resembled diffuse malignant pleural mesothelioma. Initial criteria for inclusion in the study included availability of an open pleural biopsy or decortication specimen and microscopic evidence of neutral (periodic acid-Schiff positive) mucin in the tumor. The median age of the patients was 63 years (range, 31 to 78 years), with a peak age in the seventh decade. There were 24 men and five women. Thirteen of them had a history of smoking; six (21%) had possible or definite occupational exposure to asbestos. Three (21%) of 14 lung specimens showed ferruginous bodies and two (14%) showed microscopic evidence of asbestosis. At least 25 patients had pleural effusion, most typically unilateral. Needle biopsy of pleura showed malignancy in 10 (77%) of 13 cases. Most (20 of 29) patients underwent pleural stripping. Radiotherapy and chemotherapy was each given to three patients without effect. Median survival by Kaplan-Meier estimate was 8 months, with an 18-month survival of 13%. Histologically, glands (23 cases), nests (13 cases), tubulopapillary arrays (12 cases), or sheets (eight cases) of tumor cells were found. Spindling of neoplastic cells was seen in 10% of cases. Three (21%) of 14 lung specimens showed a subpleural adenocarcinoma. Antibodies to polyclonal CEA, Ber-EP4, Leu-M1, and B72.3 were positive in 94%, 56%, 50%, and 44% of cases, respectively. All but one of the cases stained with two or more of the antibodies CEA, Ber-EP4, Leu-M1, or B72.3. This study indicates that adenocarcinomas simulating mesothelioma are aggressive variants of peripheral adenocarcinomas with a poor prognosis, that they can show pathological evidence of asbestos exposure in a subset of cases, and that immunohistochemical and histochemical stains are useful in their differential diagnosis with diffuse malignant mesotheliomas.

Adenocarcinoma↗
PubMed1998

Survival analysis of 200 pulmonary neuroendocrine tumors with clarification of criteria for atypical carcinoid and its separation from typical carcinoid.

Neuroendocrine tumors of the lung embrace a spectrum from low-grade typical carcinoid (TC), intermediate-grade atypical carcinoid (AC), and high-grade categories of large cell neuroendocrine carcinoma (LCNEC) and small cell carcinoma (SCLC). We studied 200 neuroendocrine lung tumors to critically evaluate the Arrigoni histologic criteria for AC using statistical analysis to delimit more rigorously an intermediate survival for AC between TC and the high-grade tumors of LCNEC and SCLC. Histologic features that might predict prognosis were used for Kaplan-Meier and Cox proportional hazards survival analysis, and an optimal mitotic range for AC was calculated. The optimal mitotic range for AC was 2 to 10 mitoses per 2 mm2 of viable tumor (10 high-power fields). Based on this finding, we collapsed mitoses into three categories (< 2; 2-10; > or = 11) and performed Cox multivariate analysis for all 200 neuroendocrine tumors. Mitotic counts were the only independent predictor of prognosis. Based on this analysis, we propose that AC be defined as a tumor with neuroendocrine morphology, mitotic counts between 2-10 per 2 mm2 of viable tumor (10 high-power fields), or coagulative necrosis. Using these criteria, the 200 neuroendocrine tumors were classified as 51 TC, 62 AC, 37 LCNEC, and 50 SCLC. The 5- and 10-year survival was 87% and 87% for TC, 56% and 35% for AC, 27% and 9% for LCNEC, and 9% and 5% for SCLC, respectively. After stratification for stage, survival for AC was significantly worse than for TC (p < 0.001); for LCNEC and SCLC it was significantly worse than for AC; but the survival for LCNEC was no different than that for SCLC.

Carcinoid Tumor↗
PubMed1998

Proliferation and cellular phenotype in lymphomatoid granulomatosis: implications of a higher proliferation index in B cells.

Pulmonary involvement by lymphomatoid granulomatosis (LYG) is characterized by nodules of a polymorphous lymphoreticular infiltrate with necrosis, angioinvasion, and variable numbers of large, atypical cells. Using combined immunohistochemistry, the authors compared the expression of a marker of proliferation (DNA topoisomerase IIalpha) between B cells, T cells, and histiocytes. Sixteen cases of LYG were stained by combined immunohistochemistry for DNA topoisomerase IIalpha and CD-20, CD-3, CD-68, and CD-57. A proliferation index was determined for B cells, T cells, histiocytes, and natural killer cells by dividing the number of cells with coexpression of DNA topoisomerase IIalpha and CD-20, CD-3, CD-68, or CD-57 by the total number of CD-20+, CD-3+, CD-68+, or CD-57+ cells, respectively. A significantly higher proliferation index was present in B cells compared to T cells, histiocytes, or natural killer cells (p < 0.002). The average proliferation index for B cells was 0.25+/-0.24 (range, 0.00-0.76), for T cells was 0.02+/-0.01 (range, 0.00-0.04), for histiocytes was 0.00+/-0.01 (range, 0-0.02), and for natural killer cells was 0.00+/-0.00 (range, 0.0-0.02). The average proliferation index of CD-20+ cells was greater in grade III LYG (0.36) than in grade II LYG (0.17) or the single case of grade I LYG (0.00). The authors conclude that (1) there is a spectrum of B-cell proliferation in LYG that roughly correlates with histologic grade, (2) T cells, histiocytes, and natural killer cells do not proliferate but are recruited, and (3) the average B-cell proliferation index in grade III LYG is similar to that observed in large cell non-Hodgkin's B-cell lymphomas. These observations provide a possible rationale for the use of chemotherapy for grade III LYG and observation or immunologic adjuvants for LYG with grade I or grade II histology.

Adult↗
PubMed1998

Solitary pulmonary papillomas in adults: a clinicopathologic and in situ hybridization study of 14 cases combined with 27 cases in the literature.

Solitary endobronchial papillomas in adults are rare neoplasms. Only sporadic cases have been documented. The histologic classification of these tumors remains problematic, and little is known about their clinical behavior. The clinical and pathologic features of 13 endobronchial papillomas and a single endobronchiolar papilloma were reviewed. In situ hybridization for human papillomavirus (HPV) types 6/11, 16/18, and 31/33/51 was performed on seven cases. Twenty-seven additional well-documented cases were identified in a literature review. Human papillomavirus studies were performed in four of the previously reported cases. The 41 neoplasms combined from the Armed Forces Institute of Pathology and literature review were divided into three groups according to their histologic features. Thirty-one of 41 (76%) patients were men. The ages of the patients ranged from 26 to 74 years (median, 57 years). Three morphologically distinct histologic types were recognized; 27 squamous cell papillomas, 7 glandular papillomas, and 7 mixed squamous and glandular papillomas. Squamous papillomas: 23 of 27 (85%) patients were men, and the median age was 54 years. Six of eleven (55%) of these patients smoked. Twenty-six lesions were exophytic and a single lesion had an inverted pattern. Seven of 24 (29%) lesions featured cytologic atypia and 5 of 24 (14%) had viral cytopathic effect. Five of seven (71%) cases examined for HPV DNA were positive. Three of 18 (17%) recurred. Glandular papillomas: Four of seven (57%) patients were women. The mean age was 67 years. One of five (20%) patients smoked. Five lesions were central, and two were peripheral. Four lesions had columnar epithelium, and three had ciliated epithelium. One of six (17%) lesions recurred. Mixed papillomas: five of seven (71%) patients were men. The median age was 64 years. Three of five (60%) patients smoked. Three of seven (43%) lesions featured cytologic atypia. Four of five lesions were examined for HPV DNA and all were negative. No lesions recurred. This study demonstrates that solitary endobronchial papillomas can be separated into three distinct morphologic categories. Squamous cell and mixed papillomas are predominantly lesions of male smokers in their 6th decade. Although cytologic atypia is observed in many cases, the rarity of these tumors and difficulty in separating papillomas from endobronchial papillary squamous carcinomas make generalizations regarding the risk of progression to carcinoma tenuous at best. Human papillomavirus appears to play a pathogenetic role in some squamous cell papillomas, but not in mixed papillomas, yet its presence in the squamous lesions does not correlate with recurrence or malignancy. The first report of an inverted squamous cell papilloma indicates clinical features similar to the more common exophytic squamous cell papillomas. Glandular papillomas, the rarest of all endobronchial papillomas, are found in an older age group than squamous and mixed papillomas, and most-patients are nonsmokers. Based on these findings, all endobronchial papillomas should be completely excised.

Aged↗
PubMed1998

Primary pulmonary glomus tumor: a clinicopathologic and immunohistochemical study of two cases.

We present two cases of glomus tumors arising within the lung parenchyma. The patients are a 40-year-old man and a 51-year-old man. Clinically, the two men were asymptomatic, and the pulmonary tumor was detected during a routine chest roentgenographic examination. Complete surgical resection of the pulmonary tumors was performed. Grossly, the tumors measured 1.1 and 1.5 cm. in greatest dimension; they were well circumscribed and subpleural. Neither tumor showed evidence of invasion of lung or pleura. Histologically, both tumors had pseudocapsules, lacked invasion of surrounding lung structures, and demonstrated the appearance of the solid/mucohyaline, or "glomus tumor proper" type of neoplasm. This included oval-to-round cells, with central uniform nuclei; variably eosinophilic-to-clear cytoplasm; and well-demarcated cell borders in close proximity to a rich vascular supply showing perivascular fibrosis. Immunohistochemically, both tumors showed diffuse, moderate-to-strong staining for vimentin, muscle-specific actin, and smooth muscle actin. One tumor also showed diffuse strong staining for desmin, whereas the other was negative. Follow-up information obtained from one of the patients revealed that he was alive and well 47 months after surgical resection. Our cases highlight the ubiquitous distribution of glomus tumor and its similar histologic appearance and immunohistochemical profile to soft tissue glomus tumors.

Actins↗
PubMed1997

Primary germ cell tumors of the mediastinum: II. Mediastinal seminomas--a clinicopathologic and immunohistochemical study of 120 cases.

BACKGROUND: Primary seminomas of the mediastinum are unusual neoplasms that are morphologically indistinguishable from their gonadal counterparts but may have different biologic behavior because they arise at this particular location. METHODS: The clinical and pathologic features in 120 cases of primary mediastinal seminoma were reviewed, and the immunohistochemical staining patterns in 50 of these tumors were also analyzed. RESULTS: The patients were all men between the ages of 14 and 79 years (mean age, 46.5 years). Their clinical symptoms included cough, chest pain, and dyspnea. In some patients, the lesions were asymptomatic and discovered incidentally on routine chest radiographs. None of the patients had a previous history of testicular neoplasm or tumor elsewhere. Macroscopically, the tumors were described as soft and tan, with a slightly lobulated cut surface, and measured up to 16 cm in greatest dimension. Histologically, the morphologic features were similar to those of tumors occurring in the gonads, namely, a neoplastic proliferation of round-to-polygonal cells with indistinct cell borders, clear-to-lightly-eosinophilic cytoplasm with round-to-oval nuclei and prominent nucleoli, associated with a prominent inflammatory background composed mainly of mature lymphocytes. Necrosis, hemorrhage, multinucleated giant cells, granulomatous reaction, and remnants of thymic tissue were observed in a variable number of cases; mitoses were rare. Immunohistochemical studies in 50 cases showed cytoplasmic staining with placental alkaline phosphatase in 80% of the tumors, focal dotlike positivity for CAM 5.2 low-molecular-weight keratins in 75%, focal cytoplasmic staining for wide-spectrum keratin in 70%, focal positive reaction with vimentin in 70%, and focal positivity with HCG in singly scattered cells in 5%. Immunostains for carcinoembryonic antigen, epithelial membrane antigen, and alpha-fetoprotein were negative in all the cases studied. Fifty patients were Stage I, 3 patients were Stage II, and 12 patients were Stage III. Clinical follow-up information was obtained for 65 cases (54%). Forty-nine patients were alive and disease free after a period ranging from 1 to 19 years (mean follow-up, 10 years). Sixteen patients died within the same period and were found to have metastases to distant organs. Of the 16 patients who died, 6 showed extension of the tumor outside of the mediastinal compartment at the time of initial diagnosis (Stage III lesions). Aside from clinical staging, the authors' findings also suggest that patients >37 years have worse outcomes than younger individuals. The authors were unable to find any correlation between histopathologic features and clinical behavior in any of these cases. CONCLUSIONS: Clinical and pathologic staging of mediastinal seminomas are important parameters that can be useful in determining the clinical outcomes of patients with these tumors. Tumor invasion into adjacent organs represents a marker of increased morbidity and mortality. The authors' findings suggest that patients with mediastinal seminomas may have a very good prognosis when the diagnosis is made early; patients with more advanced lesions may require more aggressive therapy for improved local control and prevention of distant metastases.

Adolescent↗
PubMed1997

Primary germ cell tumors of the mediastinum: III. Yolk sac tumor, embryonal carcinoma, choriocarcinoma, and combined nonteratomatous germ cell tumors of the mediastinum--a clinicopathologic and immunohistochemical study of 64 cases.

BACKGROUND: Yolk sac tumor (YST), embryonal carcinoma (EC), choriocarcinoma (CC), and combined germ cell tumors (CGCTs) of the mediastinum are uncommon neoplasms. Only sporadic cases have been documented in the literature; therefore, the clinical behavior of these tumors when located in the mediastinum remains relatively unknown. METHODS: The clinical and pathologic features of 64 cases of primary YST, EC, CC, and CGCTs without teratomatous components were reviewed. The immunohistochemical findings in 29 cases were also analyzed using a panel of monoclonal and polyclonal antibodies in formalin fixed, paraffin embedded tissues. RESULTS: The patients were all men between the ages of 14 and 63 years (mean, 38.5 years). Their clinical symptoms included chest pain, shortness of breath, chills, fever, and superior vena cava syndrome. None of the patients had a previous history of testicular neoplasm or tumor elsewhere. Macroscopically, the lesions in 27 patients were described as large, soft, hemorrhagic, and in some cases necrotic, and varied in greatest dimension from 6 to 20 cm. Histologically, the tumors displayed morphologic features similar to those of their gonadal counterparts. Pure YSTs accounted for the majority of cases in this series (38 of 64, 60%), followed by pure CCs (8 of 64, 12%) and pure ECs (6 of 64, 9%). CGCTs accounted for only 18% of the total cases (12 of 64). YSTs showed a variety of growth patterns; however, the reticular pattern was the most commonly observed. ECs showed a more solid growth pattern with marked pleomorphism of the tumor cells and abundant areas of necrosis. CCs were characterized by the presence of cytotrophoblastic and syncytiotrophoblastic elements with frequent areas of necrosis and hemorrhage. The cases of nonteratomatous CGCT consisted of 5 cases of EC + YST, 2 cases of EC + seminoma, 4 cases of YST + seminoma, and 1 case of EC + CC. In clinical staging, 14 patients were Stage I, 6 were Stage II, and 19 were Stage III. Information on follow-up ranging from 1 month to 13 years was available for 40 patients. Seventeen patients with YST died of their tumors. Of these, 10 who presented with Stage III disease died within the first 2 years. Unfortunately, no clinical staging was obtained for the other 7 patients in this group, and they died within 6-36 months. It is noteworthy that 4 patients with YST have survived for more than 2 years; these patients presented in Stages I and II, and 2 of them received aggressive therapy with chemotherapeutic agents and radiation. Two patients with Stage III EC died within 2 years. The remaining 4 patients with EC were lost to follow-up. Eight patients with CC died within 6 months after initial diagnosis; 7 of them presented Stage III disease and only 1 presented with Stage I. In the nonteratomatous CGCT category, 2 patients with EC + YST died within 2 years after initial diagnosis, whereas 3 patients with YST + seminoma were alive after 4-9 years. CONCLUSIONS: The results of this study confirm the aggressive nature of primary nonseminomatous germ cell tumor of the mediastinum; 72% of the patients with adequate follow-up died of their tumors within 6-36 months after diagnosis, despite aggressive therapy. Clinical and pathologic staging of mediastinal YST, EC, and CGCT are important parameters that may be helpful in predicting the clinical outcomes of patients with these tumors. The authors' findings suggest that the majority of tumors that are not limited to the mediastinum at the time of diagnosis have more aggressive behavior. On the other hand, mediastinal choriocarcinomas appeared to follow a very aggressive clinical course, regardless of treatment modality or clinical tumor stage.

Adolescent↗
PubMed1997

Primary malignant 'triton' tumour of the lung.

Two cases of malignant 'triton' tumour arising within lung parenchyma are described. The patients were a three-year-old child and a 53-year-old man. Both patients presented with shortness of breath and a large intrapulmonary mass on chest X-ray. Neither patient had a history of von Recklinhausen's neurofibromatosis. The lesions were treated by pneumonectomy. Grossly, both tumours presented as large, soft and gelatinous intraparenchymatous masses measuring 130 mm and 80 mm, respectively. Histologically, they were characterized by an atypical spindle cell proliferation embedded in an abundant myxoid stroma. Focal areas of rhabdomyoblastic differentiation characterized by large cells with abundant eosinophilic cytoplasm and occasional cytoplasmic cross-striations could be seen admixed with the atypical spindle cell elements. Immunohistochemical studies showed a focal positive reaction for S-100 protein in the atypical spindle cells embedded within the myxoid stroma, and a strong positive reaction for desmin and myoglobin in the rhabdomyoblastic areas. The child died three months after diagnosis with extension of the tumour into the thoracic cavity. The second patient has been lost to follow-up. Although rare, malignant 'triton' tumour should be considered in the differential diagnosis of primary spindle cell sarcomas of the lung.

Child, Preschool↗
PubMed1997

Role of electron microscopy in interstitial lung disease.

Although electron microscopy no longer enjoys the important role in the diagnosis of interstitial lung diseases that it had in the 1960s and 1970s, it remains an important adjunct in the differential diagnosis of certain pulmonary diseases. Examples include various manifestations of systemic lupus erythematous pneumonitis, in which the presence of tubuloreticular structures and electron-dense deposits are useful for diagnosis; immotile cilia disorders, in which qualitative and now quantitative studies of the cilia of respiratory epithelial cells can help to establish the diagnosis; infections by viruses and other subcellular microorganisms as shown by the role played by electron microscopy in the initial diagnosis of the Hantavirus pulmonary syndrome; pneumoconioses, in which, in conjunction with elemental analysis probes, scanning electron microscopy is of critical importance in establishing the presence of offending foreign compounds in lung tissue or fluids; pulmonary fibrilloses, such as amyloidosis, light chain disease, and fibrillary glomerulonephritis, affecting the lung; and cases of alveolar proteinosis or Langerhans cell granulomatosis diagnosed from fluids such as bronchoalveolar lavages or small tissue samples. As important, electron microscopy remains of enormous usefulness in the study of early structural events leading to the pathogenesis of diseases. For example, recent uses of the technique have focused on the alveolar-capillary wall damage induced by alveolitis in hypersensitivity pneumonitis and sarcoidosis. In summary, electron microscopy remains a useful method in the study and diagnosis of some interstitial lung diseases, but because of its expense it is incumbent on the clinician to use good judgment in the selection of cases and diseases for study by this method.

Diagnosis, Differential↗
PubMed1997

Primary leiomyosarcomas of the lung: a clinicopathologic and immunohistochemical study of 18 cases.

We studied 18 patients with primary malignant smooth muscle tumors of the lung (7 women and 11 men, 5-76 yr old, with a mean age of 50 yr). Lesions varied from 1.7 to 10 cm in greatest diameter. The tumors were classified as low (4 cases), intermediate (2), or high grade (12). Low-grade lesions were characterized by an orderly proliferation of fascicles of spindle cells that intersected at right angles and showed oval-to-spindle cells with cigar-shaped nuclei, minimal pleomorphism, and low mitotic activity, without hemorrhage or necrosis. Intermediate-grade lesions retained the fascicular configuration but showed increased cellularity with atypia and dense chromatin pattern, occasional pleomorphism, and mild increase in mitotic activity. High-grade lesions showed high cellularity, marked pleomorphism and atypia, frequent areas of hemorrhage and necrosis, and high mitotic activity. Immunohistochemical studies in 16 cases showed positive staining of tumor cells with smooth muscle actin in 12, desmin in 5, and coexpression of actin and/or desmin and keratin in 3. Six patients with low- and intermediate-grade lesions were alive and well from 2 to 12 years after diagnosis (mean follow-up time, 6 yr); 8 with high-grade lesions died of their tumors with widespread metastases from 1 to 24 months after diagnosis (median survival time, 5 mo). One patient whose tumor showed features of high-grade leiomyosarcoma was alive and well 12 years after surgery. Three patients with high-grade tumors were lost to follow-up. Our findings suggest that histologic grade may be the most reliable prognostic parameter for predicting clinical behavior in primary leiomyosarcoma of the lung and that smooth muscle actin is the most sensitive immunohistochemical marker for establishing the diagnosis in these tumors. Primary leiomyosarcoma should be considered in the differential diagnosis of pulmonary spindle cell neoplasms; histologic grading may be of value in the planning of therapy and assessment of prognosis for these lesions.

Actins↗
PubMed1997

Pulmonary alveolar microlithiasis. A clinicopathologic and chemical analysis of seven cases.

OBJECTIVE: To determine the clinical features and outcome of patients with pulmonary alveolar microlithiasis and to determine the chemical composition of the microliths. CASE MATERIAL: We studied seven cases of pulmonary alveolar microlithiasis. The patients were six women and one man, aged 19 to 70 years (mean age 44.5 years). Clinically, five patients were known to have suffered from this condition for 5 to 41 years. One patient presented with shortness of breath, and another had a gradual decrease in exercise tolerance. None of the patients had a previous history of disturbances in metabolism or any other relevant medical condition. Reports on radiographic studies were available in six cases, and chest radiographs were available for review in the seventh case. They all showed diffuse bilateral pulmonary infiltrates. Open lung biopsies were performed in two patients, and autopsy lung material was reviewed in five patients. RESULTS: Histologically, the lung showed the typical features of pulmonary alveolar microlithiasis, that is, presence of numerous microliths filling the alveolar spaces with either a normal or thickened fibrotic interstitium. Chemical analysis performed on the lung tissue of six of these patients revealed that the microliths consisted principally of calcium and phosphorus salts. Five of these patients died of respiratory failure; however, their deaths occurred from 5 to 41 years following the initial diagnosis. No follow-up information was obtained in two patients. CONCLUSIONS: The findings of this study confirm that pulmonary alveolar microlithiasis can be seen in any age group and that the microliths are composed principally of salts of calcium and phosphorus. Additionally, these cases confirm that the disease typically follows a protracted course.

Adult↗
PubMed1997

Pulmonary lipomatosis: a variant of placental transmogrification.

An unusual pulmonary lesion is presented. The patient is a 55-year-old white man with a 2-week history of pleuritic chest pain, shortness of breath, and bronchopneumonia. The patient had also a history of smoking and chronic cough for 12 years. Chest radiographic studies showed a 20-cm bulla in the left upper lobe. A left upper lobectomy was performed obtaining an almost completely collapsed lobe with destruction of the normal architecture by a meshwork of yellowish tissue. Histologically, there were strikingly papillary structures composed almost exclusively of mature adipose tissue with small collections of inflammatory cells. Minimal emphysema in the adjacent lung parenchyma was observed. The case herein presented most likely represents part of the spectrum of placental transmogrification or placentoid bullous lesions of the lung.

Adipose Tissue↗
PubMed1996

Primary intrapulmonary meningiomas. A clinicopathologic and immunohistochemical study of ten cases.

BACKGROUND: Primary intrapulmonary meningiomas are rare, and their occurrence has been reported in the literature only sporadically. METHODS: The clinical, pathologic, and immunohistochemical features of ten cases of primary intrapulmonary meningioma were reviewed. RESULTS: The patients were 4 women and 6 men between the ages of 30 and 72 years (mean age: 51 years). In nine patients, the lung tumor was found during a routine chest radiographic study. The only patient with clinical symptomatology was a man who presented with a persistent cough of 45 days' duration. None of the patients had a previous history of central nervous system (CNS) meningioma or other tumor at the time of diagnosis. Macroscopically, the neoplasms were described as soft, white, well circumscribed lesions that ranged in size from 1.5 cm to 4 cm in greatest dimension. No predilection for any particular lobe or segment of lung was observed. Histologically, all the tumors showed the characteristic growth pattern of CNS meningiomas and were classified accordingly. Seven tumors were transitional meningiomas, and three were fibrous meningiomas. Psammoma bodies were observed in 50% of the tumors. Immunohistochemically, six cases showed positive staining for Epithelial Membrane Antigen (EMA) and vimentin, whereas two of these cases also showed focal positive staining for CD34. Clinical follow-up ranging from 1 month to 24 years was obtained for 6 patients. All the patients were alive and well except for 1 female who died 20 years after the initial diagnosis at age 92 of a cerebrovascular accident. CONCLUSIONS: The clinical follow-up in this study suggests that these tumors are amenable to surgical resection, and when the tumor is resected in its entirety, the patient is cured. In addition, the immunohistochemical results suggest that, as in CNS meningiomas, EMA and vimentin are the most reliable immunologic markers for these tumors.

Adult↗