Während das Watch & Wait-Konzept bei weitgehend asymptomatischen Patienten mit follikulärem Lymphom noch immer als Standard gilt, gibt es beim Mantelzell-Lymphom deutliche Fortschritte. Durch die Behandlung mit Ibrutinib, einem Inhibitor der Bruton-Tyrosinkinase (BTK), kann beim Mantelzell-Lymphom ein verbessertes progressionsfreies Überleben und ein numerisch besseres Gesamtüberleben gegenüber dem Standard einer Immunchemotherapie mit anschließender Stammzelltransplantation erzielt werden.

Mantle cell lymphoma

Progress of exosomes in the diagnosis and treatment of lung cancer.

Bronchoalveolar lavage (BAL), a noninvasive, well-tolerated procedure is an important diagnostic tool that can facilitate the diagnosis of various lung diseases. This procedure allows the assessmen...

Bronchoalveolar lavage: role in the evaluation of pulmonary interstitial disease.

Immune-checkpoint inhibitors (ICI) present a new treatment for malignancies by boosting the immune system. This has led to a variety of immune-related adverse events, including ICI-associated pneumonitis (ICIaP). Diagnosis thereof is often challenging, and its pathogenesis has not yet been fully understood. The aim of this cross-sectional case–control study was to investigate cytokines in serum and bronchoalveolar lavage fluid (BALF) expressed in patients with ICIaP compared to controls consisting of healthy individuals, patients with lung cancer and patients with interstitial lung diseases (ILD) other than ICIaP. From January 2018 until June 2019, 401 adult patients with various lung diseases were prospectively enrolled in a BALF- and serum biobank, called BALOTHEK. Of these, 12 patients were diagnosed with ICIaP (Pembrolizumab, Ipilimumab, or both, and Durvalumab) serving as case group. Subjects with one of three diagnosis groups from BALOTHEK, including lung cancer, ILD other than ICIaP, and healthy individuals, served as matched controls. The following 11 cytokines were simultaneously analyzed in BALF and serum of each study participant: interferon gamma, tumor necrosis factor alpha, interleukin (IL) 1b, IL-2, IL-4, IL-5, IL-6, IL-8, IL-12p70, IL-13 and IL-17A. This study was approved by the local ethic review committee (BASEC-ID 2017-02,307 and 2018-01,724). Absolute number and percentage of lymphocytes in BALF of patients with ICIaP were significantly higher compared to control groups. For the investigated cytokines in BALF, a significant increase of IL-6 level was shown for patients with ICIaP compared to control groups (p = 0.031, adjusted for multiple comparisons). Cytokine profile assessed in BALF shows promising potential for facilitating diagnosis and understanding of pathophysiology of ICIaP. IL-6 may not only contribute to better understanding of pathophysiology but also herald therapeutic implications for Tocilizumab.

/pdf/analysis-of-cytokines-in-serum-and-bronchoalveolar-lavage-2csz5846s0.pdf

Analysis of cytokines in serum and bronchoalveolar lavage fluid in patients with immune-checkpoint inhibitor-associated pneumonitis: a cross-sectional case-control study.

AIMS Pulmonary adenofibromas are rare benign fibroepithelial tumours of the lung with unknown histogenesis and an indolent clinical behaviour. Their stroma resembles that of solitary fibrous tumours, whereas the glands are composed of respiratory epithelium organized in a phyllodes-like architecture. Differentiation of pulmonary adenofibromas from other more aggressive intrathoracic tumours is clinically relevant. However, their biology is unknown. Here, we sought to characterize pulmonary adenofibromas at a clinicopathological level and to define whether they could be underpinned by a highly recurrent somatic genetic alteration akin to tumours with similar morphology. METHODS AND RESULTS Seven pulmonary adenofibromas were subjected to immunohistochemical analysis for thyroid transcription factor 1 (TTF1), napsin A, cytokeratin 7, E-cadherin, CD99, CD34, CD31, STAT6, oestrogen receptor (ER), progesterone receptor, androgen receptor, bcl-2, and vimentin, as well as electron microscopy and capillary sequencing on microdissected samples to evaluate the presence of NAB2-STAT6 fusion genes and MED12 exon 2 mutations in their discrete components. A control group comprising pulmonary solitary fibrous tumours, pulmonary hamartomas and breast fibroadenomas was also analysed. We confirmed that the stromal elements of pulmonary adenofibromas pertain to the fibroblastic lineage, and show ER overexpression in 71% of cases, whereas the epithelium consists of TTF1-positive, E-cadherin positive bronchiolar elements. A highly recurrent NAB2-STAT6 fusion variant (exon 4-exon 2) was detected in the stroma but not in the epithelium. No MED12 mutations were identified. CONCLUSIONS Here, we demonstrate that pulmonary adenofibromas are neoplastic lesions harbouring the molecular hallmark of solitary fibrous tumours.

Recurrent NAB2-STAT6 gene fusions and oestrogen receptor-α expression in pulmonary adenofibromas.

Glomus tumors of the trachea are extremely rare and generally benign, with very few cases of malignant transformation reported in literature. We present the case of a 74-year-old man explored for cough and dyspnea. Bronchoscopy showed a polypoid mass arising from the posterolateral tracheal wall. Computed tomography demonstrated a mid-tracheal tissular mass obliterating the tracheal lumen. Resection and anastomosis of the trachea were performed. The histological and immunohistochemical findings were consistent with malignant glomus tumor.

Malignant glomus tumor of trachea: a case report with literature review.

Background and Aims

Coexistence of mantle cell lymphoma and lung adenocarcinoma is extremely rare. The aim of this study is to present the first case of incidental discovery of primary lung adenocarcinoma associated to lymph node mantle cell lymphoma.



Methods

A 45 year-old man, admitted for inguinal mass appeared since three months. Physical examination showed a 1 cm axillary and 3 cm inguinal adenopathies which were hard and fixed. A biopsy of inguinal mass revealed mantle cell lymphoma. Assessment of disease extension found a right superior lobe tumor with mediastinal lymphadenopathies. Fibreoptic bronchoscopy was normal. A fine needle biopsy of lung tumor was performed to determinate the nature of the pulmonary lesion. Histopathological findings were consistent with primary poor differentiated pulmonary adenocarcinoma with TTF-1 expression. A first mediastinoscopy was realized to verify mediastinal lymphadenopathies; frozen section showed a mantle cell lymphoma. A right lobectomy was then performed confirming the diagnosis of lung adenocarcinoma.



Then, the patient was treated for the mantle cell lymphoma by 6 cures of alternating RCHOP and RDHAP (dexamethasone, high-dose Ara-Cytarabine and cisplatin) regimens followed by autologous stem cell transplantation.



Results

The patient died because of treatment complications 3 years after diagnosis.



Conclusion

Progresses are necessary to understand the pathogenesis of of synchronous occurrence of both diseases.

Synchronous mantle cell lymph node lymphoma and pulmonary adenocarcinoma: a case report with literature review

BackgroundNeurogenic tumors of the mediastinum are rare tumors located mainly in the posterior mediastinum. The aim of this study was to report a single-institution experience of these tumors.Metho...

A single-institution experience of neurogenic tumors of the mediastinum.

We report a case of a primary lung tumor characterized by complex gland-like spaces lined by simple cuboidal to columnar epithelium surrounded by a hyalinized spindle-cell fibroblastic proliferation reminiscent of adenofibroma of the female genital tract. The lesion occurred in a 56 year-old male with a history of left thoracic pain and hemoptysis. The tumor presented clinically as 1.5 cm solitary lesion and was discovered on chest X-rays. The lesion was treated by lobectomy. The main importance lies in distinguishing this lesion histologically from other types of pulmonary hamartoma, pulmonary blastoma, intrapulmonary solitary fibrous tumor and metastases from soft tissues and visceral sarcomas.

/pdf/pulmonary-adenofibroma-report-of-a-rare-case-with-amyloid-1aibnc5828.pdf

Pulmonary Adenofibroma: Report of a Rare Case with Amyloid Deposit

BackgroundThymic carcinomas are rare tumors with a challenging diagnosis. Our aim was to report our 17-year experience of these tumors and to highlight the challenges encountered and the main differential diagnoses ruled out.MethodsWe studied 12 (92%) men and 1 (7.7%) woman with a mean age of 37 years (range 15–60 years). All patients were symptomatic, with chest pain representing the most frequent symptom. Radiology revealed anterior mediastinal masses in all cases, with either infiltration of the adjacent organs or pulmonary parenchymal metastases.ResultsThe diagnosis was made on surgical biopsies in 12 cases and a lymph node biopsy in one. Microscopic examination revealed squamous carcinoma in 3 cases, synovial sarcoma in 1, mucinous adenocarcinoma in 1, undifferentiated carcinoma in 2, clear cell carcinoma in 1, lymphoepithelioma-like carcinoma in 2, atypical carcinoid tumor in 2, and sarcomatoid carcinoma in 1. Total surgical resection was possible in one patient after neoadjuvant chemotherapy and ra...

About thymic carcinomas: challenges in diagnosis and management.

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