A retrospective study of 152 cases of lymphomatoid granulomatosis (LYG) was undertaken to expand current knowledge about clinical manifestations and therapy, and to identify prognostic indicators in this disease. The major clinical and radiographic features and extra-pulmonary sites of involvement noted in previous studies were essentially confirmed in our study. Adverse prognostic factors included neurologic manifestations, and large numbers of atypical lymphoreticular cells within the pulmonary infiltrate. Unilateral chest lesions and large numbers of small lymphocytes and histiocytes within the infiltrate were associated with a better prognosis. Malignant lymphoma involving lymph nodes developed in 12% of patients. Almost two-thirds of patients have died and the median survival was only 14 months. No particular mode of therapy was found to be satisfactory for LYG, although there was some suggestion that corticosteroids may be useful. Randomized prospective studies are necessary for definitive recommendations regarding therapy. Cancer 43:360-373, 1979. YMPHOMATOID GRANULOMATOSIS (LYG) is L a unique form of pulmonary angiitis and granulomatosis which was first described in 1972 by Liebow et al. 17319 Histologically it is a necrotizing angiocentric and angiodestructive infiltrative process composed of small lympho-cytes, plasma cells, histiocytes and atypical lympho-reticular cells. It usually presents in middle age with bilateral pulmonary infiltrates ; skin, nervous system, and kidney are commonly involved, and the mortality is high. Since the initial report which included 40 patients , only a few additional cases of LYG have been reported in the literature'~2~3~7~8~"2.'5.'6.-22,253-33.35 and many questions remain un-answered regarding therapy, prognosis and natural history. The purpose of this paper is to expand the current knowledge about clinical manifestations and response to therapy in a large number of patients, and to identify clinical and pathologic features which may be useful prognostic indicators in this disease. MATERIALS A N D METHODS One hundred and fifty-seven patients with LYG were identified in the consultation files of A. A. Liebow. All of the cases had been referred to Dr. Liebow for consultation; 36 were included in the first description of LYG,lS and 12 others were subsequently pub-hematoxylin and eosin stained microscopic slides of biopsy or autopsy material were available for review in each case; paraffin blocks and wet tissue were available in some and a variety of special stains were done in many. Five of the patients were excluded from the present study because they manifested only extrapulmonary involvement (skin, central nervous system, tongue, nasal cavity and naso-pharynx, liver and mesentery), without evidence of lung involvement. Clinical data and follow-up information were obtained from the referring physicians or the patients themselves. Since we did not have access to hospital charts, details of clinical and laboratory data were not always complete. lished e l s e w h e r e. ...

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The availability of targeted therapies has created a need for precise subtyping of non-small cell lung carcinomas (NSCLCs). The aim of this study was to assess the utility of immunohistochemical markers in subtyping poorly differentiated NSCLC and to compare the results of immunohistochemical staining on biopsies with the corresponding resections. Thirty-nine cases of NSCLC that could not be further classified on biopsy and had subsequent resection specimens were identified. Classification of the tumor was based on the resection specimen using the World Health Organization criteria. All biopsies and resections were stained with CK7, TTF-1, napsin A (novel aspartic proteinase of the pepsin family), p63, CK5/6, and 34βE12. The specimens included 20 adenocarcinomas (ACs), 15 squamous cell carcinomas (SCCs), and 4 large-cell carcinomas (LCCs). TTF-1 was positive in biopsies from 16 of 20 ACs, 2 of 4 LCCs, and none of the SCCs. p63 was positive in all 15 SCCs, 2 of 20 ACs (both were also positive for TTF-1 and napsin A), and none of the LCCs. CK5/6 was positive in 11 of 15 SCCs (all p63 positive) but none of the ACs or LCCs. Napsin A stained 11 of 19 ACs (all TTF-1 positive) but none of the other tumors. Staining for CK7 was present in 19 of 19 ACs and 9 of 15 SCCs. 34βE12 stained both SCCs (15 of 15) and ACs (12 of 20). The combination of TTF-1, napsin A, p63, and CK5/6 allowed an accurate classification of 30 of39 (77%) cases. Of 232 pairs of slides (biopsy and resection) stained with immunohistochemical markers, 12 (5%) showed discrepancies in immunohistochemical staining between biopsies and their corresponding resections. Immunohistochemical staining using a combination of TTF-1, napsin A, p63, and CK5/6 allows subclassification of poorly differentiated NSCLCs on small lung biopsies in most cases. Discrepancies in immunohistochemical staining between biopsies and resections are uncommon.

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