A Case of Rosai-Dorfman Disease Presenting as an Isolated Pleural Plaque

Our plain-English summary of A Case of Rosai-Dorfman Disease Presenting as an Isolated Pleural Plaque, by A. Rathinam, S. Gupta, R. Khanam, et al (Curēus, 2023).

The study

• Authors: A. Rathinam, S. Gupta, R. Khanam, T. Sahai
• Journal: Curēus
• Published: 2023;15(7):e41334
• Type of article: Case report
• Cited by: 1 article, as counted by NIH iCite
• Read it: PubMed · Publisher · Free full text

Abstract

Rosai-Dorfman disease (RDD) is a rare non-Langerhans histiocytic disorder primarily involving lymph nodes. Extranodal RDD has a heterogenous presentation, and isolated pulmonary involvement is rare. We report the only case of RDD presenting as an isolated pleural mass. Our patient was a 55-year-old female with multiple comorbidities who presented with chest pain. Imaging revealed an enlarging pleural-based lesion. She underwent resection of the pleural mass, showing an atypical histiocytic infiltrate in a prominent background of collagenous fibrosis. Immunohistochemistry shows CD1a-negative and S100-positive atypical histiocytic cells demonstrating emperipolesis, confirming the diagnosis of RDD. She is currently on six-month CT surveillance with no recurrence of the disease. This case highlights the unique pulmonary presentation of RDD. It also underscores that observations may be appropriate in isolated asymptomatic pleural involvement cases.

Copyright © 2023, Rathinam et al.

In plain English

This was a case report of one patient, a 55-year-old Caucasian woman. The patient was admitted with two days of constant left-sided chest pain. A chest X-ray and CT scan showed a growing lesion on the lining of the left lung (the pleura). A CT scan two years earlier had shown nonspecific pleural thickening in the same area. Surgeons removed the mass. Tissue examination, confirmed by a second opinion at Mayo Clinic, showed Rosai-Dorfman disease, a rare disorder of immune cells called histiocytes. These cells stained positive for S100 and negative for CD1a. PET-CT and brain MRI found no disease elsewhere in the body. After surgery, follow-up CT scans showed no return of the disease. The authors described this as, to their knowledge, the only reported case of this disease appearing as an isolated pleural mass without pleural effusion. They concluded that isolated pleural thickening from this disease, when it causes no symptoms, may not need systemic treatment or surgery and can be managed with regular surveillance. The article does not mention asbestos.

We wrote this plain-English summary from the full article with the help of AI, and a second AI model checked each sentence against the article. It is not the authors' wording; the abstract and the article are their own account.

What the study did and found

• Design: Case report (one patient)
• Who was studied: One 55-year-old Caucasian woman with type 2 diabetes, hypertension, peripheral arterial disease, high cholesterol and a past pulmonary embolism. The article does not state where she was treated. The authors are affiliated with Carle Foundation Hospital, Urbana, USA.
• Exposure: No exposure was studied. The only exposure history reported is cigarette smoking: half a pack a day until 2013, a total of 20 pack-years. No asbestos exposure is mentioned.
• Main results:
  • A contrast CT scan during the admission showed that the pleural-based lesion in the left chest had grown compared with a scan two years earlier
  • After the mass was surgically removed, the abnormal histiocytes stained positive for S100 and negative for CD1a. This confirmed Rosai-Dorfman disease.
  • The plasma cells in the tissue did not show an increased IgG4 to IgG ratio
  • PET-CT and brain MRI found no disease elsewhere in the body
  • A follow-up CT scan after surgery showed no recurrence of the disease
• The authors' conclusion: The authors concluded that isolated asymptomatic pleural thickening in Rosai-Dorfman disease may not need systemic treatment or surgery, and that such patients can be managed conservatively with regular surveillance

Topics

Medical terms in this study, explained in our Lexicon: Pleural Plaques, Fibrosis, Immunohistochemistry.

Related studies

• Rosai-Dorfman disease presenting as a solitary mediastinal mass — A.I. Hida, S. Yagi, et al. (2009)
• Pleural Rosai-Dorfman disease complicated with renal clear cell carcinoma: a case report and literature review — S. Tang, X. Yang, et al. (2024)
• Coronavirus Disease 2019 Presenting as Abdominal Distension in a Mesothelioma Patient: Do We Need to Think Outside the Box? — M. Irfan, T. Almas, et al. (2020)
• Asbestos-related pleural diseases: dimensional criteria are not appropriate to differentiate diffuse pleural thickening from pleural plaques — J. Ameille, M. Matrat, et al. (2004)
• Carboxyterminal propeptide of type I procollagen in ELF: elevation in asbestosis, but not in pleural plaque disease — L. Lammi, L. Ryhänen, et al. (1999)
• Yellow Nail Syndrome with Bilateral Pleural Plaques and Diffuse Pleural Thickening: A Mimic of Asbestos Related Disease — A. Dallmann, R.L. Attanoos (2018)

Browse more research in Curēus, or return to medical research.