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Small cell carcinoma of the lung (SCLC) represents about 25% of all lung cancers. Extrapulmonary small cell carcinoma is a very uncommon feature, mostly described in the oesophagus. We report here a case of small cell carcinoma of the pleura with histopathological findings, clinical course and response to therapy typical of SCLC.
Non-neoplastic diseases of the bronchi, pulmonary parenchyma, mediastinum, and pleura that are amenable to surgical management represent a wide range of unrelated etiopathogenic conditions that usually have a focal distribution. The author discusses the presurgical clinical, radiographic, and laboratory assessment and prognoses, and addresses therapeutic recommendations.
The histogenesis of localized fibrous tumor of the pleura (LFTP) is controversial. We studied 12 LFTP's by light microscopy; by immunohistochemical staining for cytokeratin (CK), vimentin, muscle-specific actin, desmin, S-100 protein, epithelial membrane antigen (EMA) and factor VIII; by electron microscopy in 6 tumors; and by lung digestion for asbestos bodies in 4 cases. Three histologic patterns occurred in combination: 1) collagenous, 2) cellular and 3) hypocellular/myxoid. Hemangiopericytoma-like foci were prominent in the cellular areas of 9 tumors. Unusual features included diffuse small cells in 3 tumors, microcystic foci in 2, macrocystic areas in 5 and tumor giant cells in 4 tumors. Neoplastic cells in all patterns stained positively for vimentin and actin in 9 and 4 tumors, respectively, and were negative for all other markers. CK and EMA were identified in mesothelial and epithelial invaginations only. Ultrastructurally, neoplastic cells demonstrated intercellular junctions, intermediate or thin filaments, dense bodies and rough endoplasmic reticulum. Basal lamina was focally present in 5 tumors, while tonofilaments, desmosomes and short microvilli were observed in one case. Our results support the conclusion that LFTP is a neoplasm of the multipotential subserosal cell, and usually expresses mesenchymal (fibroblastic/myofibroblastic) differentiation. Coexpression of mesothelial features is rare. Lung asbestos body quantitation in 4 patients suggests that there is no association between LFTP and asbestos exposure.
The visceral pleura of 8 lung tissue specimens with non-tumorous pleural lesions and of 10 specimens with secondary pleural infiltration of different primary tumours were tested by avidin-biotin-method with the following antibodies: anti-keratin KL1, anti-vimentin V9, anti-CEA (BMA 130c), HEA 125, Leu M1, HMFG 2 and anti-collagen type IV. In all cases anti-keratin positive subserosal cells could be proved. Activated mesothelial cells expressed vimentin additionally to keratin. The antibodies Leu M1, HEA 125 and BMA 130c (against CEA) showed no reaction in subserosal and mesothelial cells. With the antibody HMFG 2, however, a weak reaction could be observed. A distinction between reactive and neoplastic pleural lesions is not possible by using these antibodies. The antibodies LeuM1, HEA 125 and BMA 130c can be helpful for differential diagnosis in single cases with pleural carcinosis. The antibody against collagen type IV demonstrates newly developed basal membrane structures in areas with proliferating subserosal cells. Considering our results the entity of mesothelium and submesothelium is discussed with regard to the histogenetical aspect of mesothelioma.
We report a patient with recurrent hypoglycemia most likely caused by a solitary fibrous tumor of the pleura. After removal of the tumor, hypoglycemia resolved. The bland histologic picture of this tumor is emphasized. Electron microscopic and immunohistochemical observations support its non-mesothelial derivation. In vitro studies demonstrated that the tumor tissue produced insulin-like growth factor I (IGF-I), and its role as the cause of hypoglycemia is discussed.
PURPOSE: To determine the factors influencing the response to palliative radiotherapy (RT) in malignant mesothelioma of the pleura (MM). METHODS AND MATERIALS: A retrospective review was conducted of the records of all patients with mesothelioma who were referred to our institution between 1979 and 1996. A total of 227 RT series were administered to 189 patients with MM. Of these, 21 patients with chest wall nodules also received concomitant local hyperthermia. RESULTS: The median survival was 5 months from the start of RT and only 17% of patients were alive at 1 year after treatment. Chest pain and painful chest wall metastases were the main indications for RT. A higher local response rate was seen for patients treated with a 4-Gy per fraction scheme, vs. those receiving fractions of less than 4 Gy (50% vs. 39%). Pain recurrence occurred predominantly within the previous RT field, and pain recurred after a median of 69 days (range 32-363) in the group treated using 4-Gy fractions. When compared with a matched group, patients treated with combined RT and hyperthermia had higher response rates and fewer in-field recurrences. CONCLUSIONS: RT provides local palliation in at least 50% of patients with MM who were treated using a 4-Gy/fraction scheme to a median dose of 36 Gy. The low response rates with RT alone suggest that combined RT and local hyperthermia should be further evaluated in MM.
Pleural fibroma, or fibrous tumor of the pleura, is an uncommon entity which is characterized by slow proliferation of undifferentiated, intermediary or mature fibroblasts associated with collagen fibers forming a tumor stroma. We report a case in a 49-Year-old man who developed exercise-induced dyspnea and right chest pain. The thoracic CT scan revealed the presence of a mass in the right lung base composed of heterogeneous encapsulated tIssue. Tumor resection was performed leading to the histological diagnosis of pleural fibroma. Immunohistochemistry tests revealed positive vimetin and CD34, and negative cytokeratin uptake. These immunohistochemistry data contributed to the differential diagnosis with malignant pleural mesothelium. Pleural fibroma is a benign tumor in 80% of the cases. Prognosis is excellent. Local recurrence is exceptional and generally occurs after incomplete resection. Radial surgical treatment determines the prognosis and is required to prevent local recurrence. Other criteria of malignancy are not correlated with the clinical course of this type of tumor.
Diagnosis of pleural plaques depends on adequate knowledge of the normal anatomy of the pleura and intercostals spaces which is indispensable for understanding the normal computed tomographic images of the thorax and anatomic variants.
Five percent of pleural tumors are benign. Solitary fibrous tumors are the most frequent. We recall the clinical and pathological features and present the radiographic, computed tomographic and magenetic resonance imaging results obtained for the main types of benign tumors of the pleura.
Synovial sarcoma most commonly occurs in the peri-articular regions of the extremities. The present report describes a rare case of primary biphasic synovial sarcoma of the pleura in an 18-year-old female. The diagnosis was made on the basis of light microscopy, immunohistochemistry, electron microscopy and the characteristic translocation found on cytogenetic analysis. Synovial sarcoma should be included in the differential diagnoses of pleural tumors.
We report the interesting case of an elderly woman who presented with hypoglycaemic episodes and weight loss. She was found to have a solitary fibrous tumour weighing more than 1.7 kg arising from the diaphragmatic pleura, which had been producing insulin-like growth factor II. After surgical removal of this well-encapsulated, pedunculated tumour her hypoglycaemia resolved and she returned to normal both clinically and biochemically.
Solitary fibrous tumors of the pleura are an uncommon but increasingly recognized neoplasm derived from mesenchymal cells located in the submesothelial lining of the pleural space. They may be differentiated from mesotheliomas and other pleural tumors due to unique immunohistochemical characteristics. The vast majority of these tumors are benign, and complete surgical resection remains the mainstay of therapy for both the benign and malignant variants. In this review, the clinical presentation, radiologic features, histologic findings, and treatment options for this tumor are described.
The geographical distribution of mortality from mesothelioma of the pleura during the years 1968-78 in England and Wales has been studied using extracts from the death records held by the Office of Population Censuses and Surveys. Using the national death rate as standard, Local Authority areas with raised mortality are identified. The patterns are somewhat different for each sex. In men the high-mortality areas are mainly the major ports where shipbuilding and repairing have been concentrated, whereas in women areas where gas masks are manufactured are predominant. In both sexes there are also high death rates on the eastern side of London. Nearly all the areas of high mortality are known to have had a major asbestos-using industry in the past. Over the 11-year period the annual number of deaths from pleural mesothelioma rose by approximately 75%. This marked increase was virtually confined to men, in whom the number of deaths had reached almost 200 per annum by 1978. The indications are that the effect of past high exposures, in particular to amphibole asbestos, have not yet reached a peak in terms of mortality. On the other hand imports and usage of amphiboles, particularly crocidolite, have decreased rapidly since the mid-1960s, and dust levels in the working environment have improved even more radically.
From 1984 to 1989, 63 patients with diffuse, malignant mesothelioma of the pleura were treated with 4-8 courses of high-dose methotrexate (HDMTX, 3 g total dose) and citrovorum factor rescue. There were 61 male and two female patients of median age 60 years. CT scan was performed before and after treatment and used for response evaluation. Of 60 patients evaluable for response, 37% showed partial or complete remission, 32% showed no change and 32% showed progressive disease. Median survival from start of treatment for all patients was 11 months, for 42 patients with the epithelial type 12 months, and for 20 patients with sarcomatous or mixed types only 5 months. Toxicity was acceptable, with only five patients (8%) terminating therapy due to toxicity. One toxic death occurred. We conclude that HDMTX is an active regimen in malignant pleural mesothelioma. The significantly shorter survival for patients with the sarcomatous or mixed subtypes indicates that further investigations on the activity of HDMTX in mesothelioma should be limited to patients with the epithelial subtype.
AIMS: Synovial sarcomas are rare tumours occasionally arising in the pleural cavity, a site where their histological characteristics may be mistaken for those of malignant mesothelioma. We examined three cases of primary pleural synovial sarcoma in order to look for clinicopathological features that may help in distinguishing them from both mesotheliomas and other sarcomas that may arise in the pleura. METHODS AND RESULTS: All three patients were male, aged 42, 28 and 42, respectively, and had no known exposure to asbestos. One biphasic tumour contained neutral mucin in focal epithelial elements that also stained positively for BerEP4 and AUA1. All three tumours showed focal positivity for either keratin or EMA in the sarcomatous elements, and they also stained positively for bcl-2 protein and MIC2 gene product (CD99). CONCLUSIONS: Our results emphasize the importance of being aware of synovial sarcoma as a possible primary pleural malignancy, especially in a young patient with a localized mass. In addition, the presence of bcl-2 protein perhaps represents a useful marker in distinguishing synovial sarcoma, especially monophasic variants, from mesothelioma within a panel of antibodies.
AIMS: To present two rare cases of malignant mesotheliomas with deciduoid features arising in the pleura, both with long survival. METHODS AND RESULTS: These two cases of deciduoid mesotheliomas were observed in adult patients (one 73-year-old male and one 23-year-old female). Only the male had a history of occupational asbestos exposure, whereas the woman had a history of familial mesothelioma. A deciduoid morphology was predominant and focal areas with tubular-papillary features were noted. The tumour cells were positive for cytokeratins, HMBE-1, calretinin, EMA and mitochondrion antibodies. The follow-up data did not suggest a particularly poor prognosis; the mean survival observed was 23 months (17 and 39 months, respectively). CONCLUSIONS: This deciduoid mesothelioma histological subtype does not appear to represent an unfavourable prognostic category.
AIMS: To undertake a clinicopathological study of diffuse serosal neoplasms of epithelial histogenesis which clinically and pathologically mimic malignant pleural mesothelioma. METHODS AND RESULTS: Over a 10-year (1990-2000) study period 53 carcinomas mimicking diffuse pleural mesothelioma ('pseudomesotheliomatous' carcinoma) were identified. The study group comprised 50 men and three females, age range 33-77 (median 68) years. In 46 (87%) cases there was a history of smoking and in 40 (76%) cases a history of asbestos exposure. Histologically the pleural 'pseudomesotheliomatous' carcinomas could be divided into two broad groups: primary pulmonary carcinomas with florid pleurotropic growth (n = 47), of which 34 (70%) were adenocarcinomas; and diffuse carcinomatous involvement of the pleura by metastatic tumour (n = 6). This latter group comprised two transitional cell carcinomas of bladder, one renal (clear) cell carcinoma, one ductal pancreatic adenocarcinoma, one prostatic adenocarcinoma and one squamous cell carcinoma of parotid gland origin. Follow-up data were available in 35 cases. Regardless of tumour type, survival was poor (median 8 months) and comparable to diffuse pleural mesothelioma. CONCLUSIONS: Pleural 'pseudomesotheliomatous' carcinomas are uncommon (comprising 6% of referrals), pathologically heterogeneous tumours with poor prognosis. Tissue diagnosis should be obtained in all cases of suspected diffuse pleural neoplasia. By light microscopy and immunophenotype many of the tumours mimicked malignant mesothelioma. In particular, an awareness that all neoplasms exhibiting squamous differentiation may express cytokeratin 5/6 and thrombomodulin is important to prevent misinterpretation. In this respect, calretinin is regarded as the most specific and sensitive mesothelial marker. Misdiagnosis may have medico-legal implications in asbestos-related compensation claims.
We describe a case of malignant pleural mesothelioma appearing as a solitary pleural tumor in a 56-year-old Japanese man with no history of exposure to asbestos. A chest radiograph revealed an isolated extrapulmonary mass in the left hemithorax. The patient underwent tumor resection, but the tumor later recurred on the contralateral pleura. The patient developed cerebral metastases and died 16 months after the initial surgery. The resected tumor was sessile with broad-based pleural attachment. Microscopically, the tumor was composed of interlacing fascicles of plump spindle cells intermixed with few polygonal cells. Most of the tumor cells showed positive immunoreactivity for cytokeratins (AE1 and AE3) and vimentin. Many of the tumor cells were positive for epithelial membrane antigen, and a few were positive for desmin. In contrast, the tumor cells were consistently negative for carcinoembryonic antigen, epithelial antigen BerEP4, calretinin, S-100 protein, neuron-specific enolase, muscle actin antigen HHF35, alpha-smooth muscle actin antigen and CD34. Ultrastructurally, the tumor cells had diffusely distributed cytoplasmic intermediate filaments, desmosome-like junctions, and a few microvilli. Some tumor cells contained cytoplasmic tonofilaments. Immunohistochemical and ultrastructural findings supported the mesothelial nature of the tumor, and led us to diagnose this tumor as a sarcomatoid localized malignant mesothelioma.