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Background: Chest physicians have a limited experience of primary pulmonary sarcomas, which represent a particular entity among rare intrathoracic neoplasms., Design: Retrospective review of medical records., Purpose: To study patients with primary sarcomas of the lung diagnosed in our pathology department in order to define their clinical characteristics, treatment, and prognosis., Patients: The study group consisted of 12 patients, with a mean age of 53 years., Results: The main symptoms were chest pain, and cough. Imaging findings consisted of: eight single peripheral opacities, three single parahilar opacities, and one lobar actelectasis. The histologic diagnoses confirmed in all cases by detailed immunohistochemical study were leiomyosarcoma (7), monophasic synovial sarcoma (2), one case each of malignant peripheral nerve sheath tumor (MPNST), epithelioid sarcoma, and malignant fibrous histiocytoma. Thoracic surgery done in nine cases consisted of six lobectomies with further parietal resection in two cases, and three pneumonectomies. Four patients received chemotherapy and two patients had radiation therapy postoperatively. Follow up available on 12 patients ranged from 3 to 144 (mean 42) months. Long term survival up to 3 years was observed in five patients. Median overall survival was 48 months. Overall 5-year survival rate was 38%., Conclusions: Primary sarcomas of the lung are a rare and aggressive malignancy. Treatment and prognosis do not differ from other soft tissue sarcomas.

Four cases of lymphomatoid granulomatosis are reported, three of them involving the lung. Histological features included a true angiocentric and angiodestructive polymorphic cellular proliferation. This included histiocytes, plasma cells, many reactive T-cells and rare large, atypical cells which were of the B phenotype. Epstein-Barr virus was detected in the atypical cells by in situ hybridization in three cases, with expression of both latent membrane proteins (LMP)-1 and Epstein-Barr nuclear antigen-2 in two cases and expression of only LMP-1 in the third case. Expression of both of these proteins suggests a defect in the T-cell-mediated immunity and that Epstein-Barr virus is not only a silent passenger but may also be involved in the pathogenesis of the disease. This could have implications for therapy.

Cure after 8 years of a non-small-cell lung cancer (NSCLC), inoperable because of locoregional spread, is reported. The authors believe this case represents the longest survival after monochemotherapy reported to date and provides the opportunity to review studies of prognostic factors concerning long-term survival in NSCLC.

We report a retrospective study of the pathological features in 69 primary pulmonary non-Hodgkin's lymphomas which have previously been clinically reviewed. The tumours consisted of 61 (88%) low-grade and eight (12%) high-grade malignant lymphomas. Fifty-four of the low-grade malignant lymphomas were MALT lymphomas. Lymphoepithelial lesions were observed in bronchial, bronchiolar and alveolar lining. All tumours were composed of nodules, forming a lymphangitic pattern at the periphery and a confluent central mass. Invasion of pleura and vessels was often seen but this without any consequence on survival. Granulomas were found in 20% of cases. Six of the eight high-grade tumours were centroblastic and another two were B-cell lymphomas of undetermined type. In four cases, associated areas of low-grade malignant lymphoma with lympho-epithelial lesions indicated a preexisting MALT lymphoma. Clinical data suggest that limited surgery or non-aggressive chemotherapy can provide long-term survival in patients with such slowly developing neoplasms. However, non-invasive diagnostic methods need to be developed.

We studied 70 patients with biopsy-proven pulmonary non-Hodgkin's lymphomas without extrathoracic involvement or mediastinal adenopathy to determine the clinical, imaging, and endoscopic features of this condition in a homogeneous series. In low-grade (LG) lymphomas, symptoms were cough, dyspnea, chest pain, hemoptysis. Imaging features consisted of localized alveolar opacities, infiltrative diffuse opacities, atelectasis, and pleural effusions. Inflammatory changes of the mucosa were present in some patients, leading to bronchial stenosis in 7; biopsies showed lymphomatous infiltration in 12. Prognosis of LG lymphomas was excellent, with 93.6 percent survival at five years. High-grade lymphomas differed from LG lymphomas principally by a more aggressive course and a worse survival. Inflammatory changes occurred in seven of nine cases leading to stenosis in two, and biopsies showed lymphomatous involvement in five. The profile of primary pulmonary lymphomas in this study could help clinicians consider this condition and prompt them to evaluate new diagnostic tools.

Lymphangiomyomatosis (LAM) consists of smooth muscle (SM) cell proliferation of unknown origin involving the lymph nodes and the lung interstitium. From morphological studies showing both SM differentiation of the proliferating cells and lymphatic hyperplasia, hypotheses were suggested concerning the origin of the proliferation. Two cases of LAM were investigated by electron microscopy and immunohistochemistry; tissues were obtained by lymph node and open lung biopsies. Cytoplasmic and matrix protein markers were used in order to clarify the pattern of differentiation of the proliferating cells and to characterize their connective tissue environment. The proliferating cells present ultrastructural characteristics of SM cells; they contain vimentin, desmin, and alpha-SM actin and are devoid of Factor VIII, favouring a parieto-arterial origin. The connective tissue matrix inside the infiltrate is composed of interstitial collagens and basement membrane components. At the late stage of the disease, remodelling of the interstitial matrix accompanies the infiltrate and remains perilesional.

We report a case illustrating the therapeutic consequences of an intravenous metastasis to the left superior pulmonary vein following resection of a voluminous primary lung carcinoma. Arterial spread of malignant cells occurred because the size of the tumor did not allow immediate clamping of the left superior pulmonary vein. The embolism was situated at the aortic bifurcation and lower limb ischemia persisted despite also emergency embolectomy. Distal (lower popliteal) embolectomy was also unsuccessful, and lower limb amputation was inevitable. This case illustrates the problems encountered in surgical treatment of pulmonary vein invasion by lung carcinoma and the role of adjuvant chemotherapy and radiotherapy.

We report four cases of primary pulmonary pseudolymphoma and lymphoma with the usual features of these disorders: lack of symptoms, radiological opacities sometimes multiple with soft wooly contours and air bronchograms, and slowly evolving pattern. The distinction between pseudolymphoma and lymphoma, prior to histological diagnosis is currently controversial. Immunological studies aim at defining whether the lymphoid proliferation is polyclonal or monoclonal (the benign process being considered polyclonal and the malign monoclonal). This distinction works in some cases, as in one of our cases of lymphoplasmacytic lymphoma with a monoclonal IgM gammopathy, a predominance of cells containing IgM on immunofluorescence, and an illness evolving over 9 years. Other cases raise discussion points such as one of our patients with classical histological characteristics of pseudolymphoma, an absence of monoclonal markers on immunofluorescence, but having a monoclonal gammopathy in the serum. This example raises the question as to the possible coexistence of, or a switch over from, a polyclonal to a monoclonal process. In this way the primary pulmonary lymphoproliferative process ought to be considered as one element in a large spectrum ranging from benign to malign disorders and not as distinct illnesses with definitive patterns.

Three cases of transformation of small cell carcinomas into squamous cell cancer after chemotherapy are reported. A review of the literature shows that about 7% of small cell carcinomas are associated to different histological types of cancer before treatment. Such an association is found in about 25% of the cases after chemotherapy. Recent biochemical and ultrastructural approaches have led to new concepts concerning the histogenesis of lung cancer. The unicyst theory suggested by some authors could explain the therapy-induced maturation of undifferentiated small cell carcinoma.

Seven cases of pulmonary hemangiopericytoma (presumed to be primary v.i.) are reported. Personal observations on these cases combined with 43 already described in the literature allow certain characteristics of the disorder to be accurately defined. Clinically, primary pulmonary hemangiopericytoma raises the aetiological problem of a peripheral solitary tumour beyond endoscopic vision. Until the present time the diagnosis has always been made by thoracotomy. Transpleural pulmonary biopsies may allow a preoperative diagnosis. Silver stains and electron microscopy enable an anatomo-pathological diagnosis. Histological studies can neither distinguish between benign and malignant forms nor differentiate between primary or metastatic hemangiopericytoma. For this reason there is always a long period of doubt whether the tumour is primary; only prolonged survival of the patients after excision will confirm whether the tumour was a primary or not. Treatment is essentially surgical. It seems that new techniques in radiotherapy (high energy) and new possibilities of chemotherapy (with Adriamycin) are capable of improving the prognosis of the malign form. However, such a therapeutic strategy remains to be defined as these tumours are so rare.

A part from the interest aroused by the exceptional nature of this case of sarcoma of the right superior pulmonary vein, it enabled definition of the angiographic appearances of the affection of the vein. Physiopathological examinations, including vascular redistribution, development of a systemic collateral circulation, and the reflux of hematosed blood into the pulmonary trunk, were able to establish the diagnosis in vivo.

A series of 49 patients with pulmonary hamartoma subjected to operation is described. 28 patients were male and 21 female, with a mean age of 49.8 years. Most cases were discovered on routine chest films. Six patients had radiologic changes produced by obstruction, while 43 had a rounded peripheral opacity. Fine calcifications were present in four cases. In no case was diagnosis achieved by bronchoscopy, whereas one of the more recent cases was diagnosed by percutaneous fine needle biopsy. Sinner has shown that this last technique can establish diagnosis in most cases. As such tumors are invariably benign and grow slowly, diagnosis by percutaneous biopsy renders operation unnecessary: periodic follow up only is required.

The detection of bone marrow involvement might be of prognostic value and may influence therapeutic decisions in small cell lung cancer. By unilateral bone marrow aspiration and biopsy, evidence of bone marrow metastases is seen in 15-30% of patients with this disease. Since magnetic resonance imaging of the lower body and immunostaining with monoclonal antibodies have recently been shown to be very sensitive detection methods, we investigated the value of these two techniques in detecting bone marrow involvement in 35 consecutive patients with small cell lung cancer. The results were compared to those obtained with conventional cytohistological analysis. In all cases when cytology and/or bone marrow biopsy were positive, monoclonal antibodies immunostaining and magnetic resonance imaging also detected malignant cells. Furthermore, evidence of bone marrow involvement was shown with magnetic resonance imaging and/or immunostaining in 10 of 26 cases (38%) where routine procedures were unable to detect malignant cells. In one of these 26 patients, magnetic resonance imaging and immunostaining provided the only evidence of metastatic disease. These data suggest that the rate of bone marrow metastases is underestimated by routine procedures. Further investigation is needed to determine whether or not these new non-invasive methods have prognostic value or affect therapeutic choices in small cell lung carcinoma.

We report a case, cytologically diagnosed, of broncho-alveolar cancer associated with localised interstitial pulmonary fibrosis. This association of fibrosis with bronchiolo-alveolar cancer is classical and a pathogenic affiliation has been deduced by the majority of authors. We review the pathogenesis in relation to recent datas concerning the activation of successive oncogenes: the first stage of carcinogenesis may correspond to the activation of an oncogene coding for a fibroblastic growth factor leading to fibrosis.

We studied the clinical and radiological features of pulmonary metastases present in 4 out of 22 patients with medullary carcinoma of thyroid. Two patients presented with common metastases: macronodules in one, and micronodules in the other one. The other two patients presented initially with reticulonodular perihilar lesions on chest X-ray, leading to the diagnosis of sarcoidosis in both of them. The observation of such pulmonary metastases is original: initially latent, they progress very slowly, and they correspond to a lymphangitic spread of the tumour with amyloid deposition in peribronchovascular structures without alveolar involvement, as shown in one of our cases with pathologic study.