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Background: The present article is an English-language version of the French National Diagnostic and Care Protocol, a pragmatic tool to optimize and harmonize the diagnosis, care pathway, management and follow-up of lymphangioleiomyomatosis in France., Methods: Practical recommendations were developed in accordance with the method for developing a National Diagnosis and Care Protocol for rare diseases of the Haute Autorité de Santé and following international guidelines and literature on lymphangioleiomyomatosis. It was developed by a multidisciplinary group, with the help of patient representatives and of RespiFIL, the rare disease network on respiratory diseases., Results: Lymphangioleiomyomatosis is a rare lung disease characterised by a proliferation of smooth muscle cells that leads to the formation of multiple lung cysts. It occurs sporadically or as part of a genetic disease called tuberous sclerosis complex (TSC). The document addresses multiple aspects of the disease, to guide the clinicians regarding when to suspect a diagnosis of lymphangioleiomyomatosis, what to do in case of recurrent pneumothorax or angiomyolipomas, what investigations are needed to make the diagnosis of lymphangioleiomyomatosis, what the diagnostic criteria are for lymphangioleiomyomatosis, what the principles of management are, and how follow-up can be organised. Recommendations are made regarding the use of pharmaceutical specialties and treatment other than medications., Conclusion: These recommendations are intended to guide the diagnosis and practical management of pulmonary lymphangioleiomyomatosis., Competing Interests: Declaration of Competing Interest Vincent COTTIN has no conflict of interest to disclose in relation with the content of the manuscript. Elodie BLANCHARD has no conflict of interest to disclose in relation with the content of the manuscript. Mallorie KERJOUAN declares research fees paid by Pfizer and Novartis to her institution. Romain LAZOR has no conflict of interest to disclose in relation with the content of the manuscript. Martine REYNAUD-GAUBERT has no conflict of interest to disclose in relation with the content of the manuscript. Romain LAZOR has no conflict of interest to disclose in relation with the content of the manuscript. Camille TAILLE declares consulting fees paid to her by Novartis. Yurdagül UZUNHAN has no conflict of interest to disclose in relation with the content of the manuscript. Lidwine WEMEAU has no conflict of interest to disclose in relation with the content of the manuscript. Claire ANDREJAK has no conflict of interest to disclose in relation with the content of the manuscript. Dany BAUD has no conflict of interest to disclose in relation with the content of the manuscript. Philippe BONNIAUD has no conflict of interest to disclose in relation with the content of the manuscript. Pierre-Yves BRILLET has no conflict of interest to disclose in relation with the content of the manuscript. Alain CALENDER has no conflict of interest to disclose in relation with the content of the manuscript. Lara CHALABREYSSE has no conflict of interest to disclose in relation with the content of the manuscript. Isabelle COURT-FORTUNE has no conflict of interest to disclose in relation with the content of the manuscript. Nicolas DESBAILLETS has no conflict of interest to disclose in relation with the content of the manuscript. Gilbert FERRETTI has no conflict of interest to disclose in relation with the content of the manuscript. Anne GUILLEMOT has no conflict of interest to disclose in relation with the content of the manuscript. Laurane HARDELIN has no conflict of interest to disclose in relation with the content of the manuscript. Marianne KAMBOUCHNER has no conflict of interest to disclose in relation with the content of the manuscript. Violette LECLERC (deceased). Mathieu LEDERLIN has no conflict of interest to disclose in relation with the content of the manuscript. Marie-Claire MALINGE has no conflict of interest to disclose in relation with the content of the manuscript. Alain MANCEL has no conflict of interest to disclose in relation with the content of the manuscript. Sylvain MARCHAND-ADAM declares consulting fees paid by Novartis. Jean-Michel MAURY has no conflict of interest to disclose in relation with the content of the manuscript. Jean-Marc NACCACHE has no conflict of interest to disclose in relation with the content of the manuscript. Mouhamad NASSER has no conflict of interest to disclose in relation with the content of the manuscript. Hilario NUNES has no conflict of interest to disclose in relation with the content of the manuscript. Gaële PAGNOUX has no conflict of interest to disclose in relation with the content of the manuscript. Grégoire PRÉVOT has no conflict of interest to disclose in relation with the content of the manuscript. Christine ROUSSET-JABLONSKI has no conflict of interest to disclose in relation with the content of the manuscript. Olivier ROUVIERE has no conflict of interest to disclose in relation with the content of the manuscript. Salim SI-MOHAMED has no conflict of interest to disclose in relation with the content of the manuscript. Renaud TOURAINE has no conflict of interest to disclose in relation with the content of the manuscript. Julie TRACLET has no conflict of interest to disclose in relation with the content of the manuscript. Ségolène TURQUIER has no conflict of interest to disclose in relation with the content of the manuscript. Stéphane VAGNARELLI has no conflict of interest to disclose in relation with the content of the manuscript. Kaïs AHMAD has no conflict of interest to disclose in relation with the content of the manuscript., (Copyright © 2023 SPLF and Elsevier Masson SAS. 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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.

A 60-year-old male patient with small carcinoma of the lung was treated with chemotherapy and radiotherapy. The tumour regressed, but two squamous cell carcinomas appeared in the contralateral lung. The first one was discovered after 3 months' treatment and developed slowly; the second one, diagnosed after 9 months' treatment, was rapidly progressive. The patient died 13 months after the beginning of treatment, and only the squamous cell carcinomas could be confirmed on autopsy. Such cases may reflect either selection of tumours that were present from the beginning of treatment, or development of other malignancies, or treatment-induced histological changes in the original tumour.

Two patients with small cell carcinoma of the lung treated by chemoradiotherapy developed, diffuse interstitial pneumonitis after 7 and 21 months of treatment while in complete remission of their malignant disease. One patient died after an acute respiratory distress syndrome, but the second improved after steroid therapy and discontinuation of cytotoxic therapy. Pulmonary toxicity appeared to be related to cyclophosphamide therapy, but radiation therapy could have potentiated cyclophosphamide toxicity as it has been shown in bleomycin lung disease.

The diagnosis in the same patient of the two diseases is very often delayed. Generally, tuberculosis is diagnosed first, and then cancer when the radiologic lesions extend despite of antituberculous treatment. In half of our cases, tuberculous lesions could not be individualized from cancer on the chest X-ray films. Antineoplastic chemotherapies may favour the development of tuberculosis, or atypical mycobacteriosis, in cancer patients, and it must be considered as an opportunistic infection in such patients. Clinicians should be aware of the possible coexistence of the two diseases and, in practice, ask for fiberoptic bronchoscopy and sputum cytology in tuberculous patients presenting atypical features, and for sputum examination in cancer patients.

Pulmonary metastases of a placental choriocarcinoma are common but they are rarely the presenting factor, and whenever there are pulmonary metastases in a young woman a systematic search is required. We report on 7 cases and emphasize several important points. The diagnosis rests on the level of Beta H.C.G. One should be cautioned about transparietal aspiration or fibreoptic biopsy in view of the haemorrhagic nature of the lesion. An extensive work-up should be performed before classifying patients as low, medium or high risk as on this will determine the type of chemotherapy, either monotherapy with Methotrexate or polychemotherapy. The duration of treatment will depend on the clinical, radiological and biological outcome.

We report an observation of pneumopericardium occurring, without iatrogenic trauma in the preceding days, during the course of an epidermoid bronchial carcinoma, treated for a year by radio- and chemotherapy. The pneumopericardium produced a very attenuated clinical picture and was resorbed without incidence. But the patient died a few weeks after an overwhelming haemoptysis. The radiological picture is very characteristic. The differential diagnosis is above all that of a pneumo-mediastinum. But in the latter case, the clear zone exceeds the level of the great vessels and subcutaneous cervical emphysema in generally very clear both clinically and radiologically.