ContextRosai-Dorfman disease is an uncommon histiocytic disorder most frequently presenting as bilateral cervical lymphadenopathy in children and young adults. Extranodal disease occurs in a significant proportion of patients. It has been recently classified as part of the ‘R group’ of histiocytoses by the Histiocyte Society in 2016. Cutaneous Rosai-Dorfman disease is regarded as a separate disease entity that falls into the ‘C group’ of histiocytoses according to this classification system. The pathogenesis was previously poorly understood; however, recent evidence demonstrating clonality in a subset of cases raises the possibility of a neoplastic process. A possible association with IgG4-related disease remains controversial.ObjectivesTo provide a comprehensive review of Rosai-Dorfman disease, including nodal, extranodal and cutaneous forms, with a particular emphasis on new insights into the possible clonal nature of the disease; to discuss the recently revised classification of the histiocytoses by the Histiocyte Society; and to summarise the findings from the literature regarding the controversial association with IgG4-related disease.Data sourcesThis review is based on published peer-reviewed English literature.ConclusionsClassic Rosai-Dorfman disease, which may be sporadic or familial, is considered a separate entity from cutaneous disease, which is reflected in the revised classification of histiocytoses. An increase in IgG4-positive plasma cells may be seen in Rosai-Dorfman disease. This finding in isolation is of limited significance and should be interpreted with caution. Studies investigating the molecular profile of the disease show that in at least a subset of cases the disease is a clonal process. The classification of Rosai-Dorfman disease is therefore likely to change as our understanding of the aetiopathogenesis evolves.

Background: Transthoracic ultrasound (US) has gained popularity as a tool for visualizing pleural effusions and assisting thoracentesis or chest drain placement. In the absence of effusion, US just as well demonstrates solid masses involving or abutting the pleura, yet biopsy of such lesions is not widely performed by chest physicians. Objective: To assess the feasibility and the safety of US-assisted cutting needle biopsy performed by chest physicians in routine practice. Methods: Lesions involving or abutting the pleura ≧20 mm in diameter on US were sampled with a 14-gauge cutting needle under local anesthesia. Biopsy site, needle direction and depth of penetration were determined with US. The procedure was performed without direct US guidance in ‘free-hand’ technique. Results: Ninety-one patients underwent 96 cutting-needle biopsies for suspected peripheral lung tumors (n = 44, 46%), pleural-based (n = 39, 41%), mediastinal (n = 10, 10%), or chest wall lesions (n = 3, 3%), which were single in 71%, multiple in 6% and diffuse in 23%. Sensitivity for malignant neoplasms (n = 65) was 85.5% and 100% for mesothelioma (n = 10). Pneumothorax occurred in 4%. Conclusions: US-assisted cutting-needle biopsy of lesions ≧20 mm in diameter is safe in the hands of pulmonologists. The yield for neoplastic disease including mesothelioma is high.

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The present study compared the diagnostic yield of ultrasound-assisted cuttingneedle biopsy (CNB) and fine-needle aspiration biopsy (FNAB) in chest lesions.A physician performed ultrasound and FNAB with a 22-G spinal needle in all patients, directly followed by a 14-G CNB in patients without contraindication.A total of 155 consecutive lesions arising from the lung (74%), pleura (12%), mediastinum (11%) or chest wall (3%) in patients with a final diagnosis of lung carcinoma (74%), other malignant tumours (12%), non-neoplastic disease (9%) or unknown (5%) were prospectively included. The overall diagnostic yield was 87%. Combined specimens were obtained in 123 lesions (79%). In these, yields of FNAB, CNB and both methods combined were 82, 76 and 89%, respectively. FNAB was significantly better than CNB in lung carcinoma (95 versus 81%) but CNB was superior in noncarcinomatous tumours and in benign lesions. On-site cytology was 90% sensitive and 100% specific for predicting a positive FNAB. One patient required drainage for pneumothorax (0.6%).Ultrasound-assisted fine-needle aspiration biopsy performed by chest physicians is an accurate and safe initial diagnostic procedure in patients with a high clinical probability of lung carcinoma. All other patients should undergo concurrent fine-needle aspiration biopsy and cutting-needle biopsy.