Rosai-dorfman disease of the pleura: a rare extranodal presentation

doi:10.1016/S0046-8177(03)00402-7

Human Pathology

Volume 34, Issue 11, November 2003, Pages 1210-1211

https://doi.org/10.1016/S0046-8177(03)00402-7 Get rights and content

Abstract

We present a case of Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) manifesting primarily as a pleural disease without involvement of the lung parenchyma. The patient is an 81-year-old man who presented with increasing shortness of breath. Radiographic studies revealed a large loculated left-sided pleural effusion and parietal pleural thickening. Video-assisted thoracoscopic biopsy of the pleura showed the characteristic S100-positive histiocytes exhibiting emperipolesis. In this rare case of pleural Rosai-Dorfman disease, the extensive involvement of the pleural lymphatics by the characteristic histiocytes appears to have been responsible for the pleural effusion.

Section snippets

Case report

The patient was an 81-year-old man of Mediterranean extract who presented with complaints of increasing shortness of breath. Computed tomography scan of the chest revealed a large loculated left-sided pleural effusion, parietal pleural thickening, complete collapse of the left lower lobe, and partial left upper-lobe atelectasis. There was no thickening involving the left visceral pleura, right pleura, or pericardium. The lungs contained multiple calcified nodules bilaterally, but no

Pathologic findings

The pleural biopsy specimen displayed thickening with fibrosis and diffuse infiltration by histiocytes, lymphocytes, and occasional neutrophils. These inflammatory cells dissected through the pleural collagen fibers and extended into the adipose tissue. The histiocytes were plump, with lightly eosinophilic cytoplasm and exhibited emperipolesis (Fig 1). Immunohistochemical analysis demonstrated positive reactivity of the characteristic histiocytes with S100 and CD68 antibodies (Fig 2).

Discussion

Rosai-Dorfman disease involving the thorax is rare and most often manifests as pulmonary disease, which accounts for only 2% of Rosai-Dorfman disease cases.2 The patients who develop pulmonary Rosai-Dorfman disease are young (average age, 14 years), have involvement of lymph nodes and other extranodal sites (nasal cavity, paranasal sinuses). The tracheobronchial tree is infiltrated most commonly, and intraluminal polypoid growth of Rosai-Dorfman disease may produce airway obstruction.2, 3, 4, 5

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There are more references available in the full text version of this article.

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Destombes-Rosai-Dorfman disease (RDD) is a rare multisystemic histiocytosis. Pulmonary involvement during RDD has been poorly described. The goal of this study was to examine the clinical presentations, radiological features, and outcomes of 15 patients with RDD and lung involvement.
The cases of RDD with lung involvement were extracted from the French National Histiocytosis registry. Efficacy of the MEK inhibitor cobimetinib in treating lung disease was evaluated with an ¹⁸fluorodeoxyglucose PET scanner and chest CT scans.
Fifteen patients (six women; median age, 40 years at RDD diagnosis) were included. All patients had evidence of systemic disease with extrapulmonary localizations of the disease (lymphadenopathy [n = 12], skin [n = 9], bones [n = 6], retroperitoneal involvement [n = 3], sinuses [n = 3], parotid gland [n = 2], submandibular gland [n = 1], and breast [n = 1]). Presenting symptoms were dominated by dyspnea and dry cough in seven patients. Restrictive physiology was observed in two of five patients. BAL showed lymphocytosis in one of five cases. Eight patients received corticosteroids, all but one with variable immunosuppressive or immunomodulatory therapies. Two patients received cobimetinib for severe lung disease, with dramatic pulmonary metabolic and tumoral responses. Two patients died during follow-up: one of hemoptysis, and the other of an unrelated cerebral tumor.
Pulmonary involvement in RDD is rare, proteiform, and sometimes severe. The MEK inhibitor cobimetinib can lead to dramatic responses.
Diagnostic Pathology: Thoracic
2017, Diagnostic Pathology: Thoracic
A 38-year-old woman with an osteolytic rib lesion
2016, Chest
Citation Excerpt :
Involvement of the larynx, lung, and pleura has been reported in only a few cases.13 Authors of a case report documenting the rare isolated involvement of pleura reported involvement of the lower respiratory tract by RDD to be relatively rare, comprising 3% of extranodal cases.14 Pulmonary RDD typically involves the tracheobronchial tree, with intraluminal polypoid lesions sometimes associated with airway obstruction.
Rosai-Dorfman disease of multiple organs, including the epicardium: An unusual case with poor prognosis
2011, Heart and Lung: Journal of Acute and Critical Care
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Although the autopsy proved the involvement of pulmonary parenchyma, no obvious abnormalities were identified by CT scanning, possibly because the lesions were too tiny to be found. Ohori et al2 reported 1 case of RDD primarily limited in the pleura without pulmonary parenchymal involvement, which was confirmed by pleural biopsy;2 however, it is possible that there were also tiny lesions in the parenchyma that were missed during CT scanning. RDD involving the thorax is rare and often manifests as a pulmonary disease, which accounts for only 2% of RDD cases.6
Rosai–Dorfman disease (RDD) is a benign proliferative histiocytic disorder predominantly of the lymph nodes with extranodal involvement in some cases. However, serous membranes are seldom involved in the extranodal cases, and epicardial involvement is never reported. Most RDD cases show a self-limiting process with good prognosis and corticosteroid treatment in some patients exhibiting good effect. We recently observed a case of RDD primarily manifesting pericardial and bilateral pleural effusion that finally led to death, and corticosteroid treatment showed little effect. Autopsy showed the characteristic S100-positive and CD68-positive histiocytes exhibiting emperipolesis in the lungs, pleura, epicardium, mediastinal lymph nodes, and colon. This case illustrates the importance of RDD in the differential diagnosis of effusion in multiple serous cavities. The failure of corticosteroid therapy in this patient and her death showed the poor prognosis of some patients with RDD. Moreover, to our knowledge, epicardial involvement of RDD has not been reported.
Intrathoracic manifestations of Rosai-Dorfman disease
2010, Respiratory Medicine
Citation Excerpt :
When considering only individuals with lower respiratory tract involvement, 4 out of 21 patients were affected, representing a higher percentage and also a better prognosis than previously described. Case reports have been published on primary central airway involvement,9 and pleural involvement.7 In the current series, one patient had a pleural effusion.
Rosai–Dorfman Disease (RDD), also known as Sinus Histiocytosis with Massive Lymphadenopathy (SHML), is a rare monocyte/macrophage proliferative disorder of varied biological behavior. Although cutaneous and lymph node involvement are relatively well-described, intrathoracic manifestations of RDD have only occasionally been reported.
We conducted a retrospective computer-assisted search of the Mayo Clinic record from 1976 to 2005 for patients with histopathologic evidence of RDD on organ biopsy. Clinical characteristics were abstracted from charts and thoracic manifestations recorded. Survival was estimated using the national social security database.
A total of 21 patients were diagnosed with RDD over a period of 30 years; 9 had intrathoracic manifestations (43%). Main pulmonary symptoms included dyspnea and cough. Age at the time of diagnosis, gender, race, smoking history, mortality and time of survival after diagnosis were no different between RDD patients with and without intrathoracic manifestations. The most common radiographic thoracic manifestation was mediastinal lymphadenopathy (6 patients). Cystic change, interstitial lung disease, and airway disease were radiographically evident in 4 patients. Seven patients were treated at some point in the course of their disease, most commonly with oral corticosteroids. At the time of last follow-up 87% were alive, with a median (IQR) time interval since diagnosis of 8 years (4–9.7).
Intrathoracic manifestations of RDD are relatively common and include mediastinal lymphadenopathy, airway disease, pleural effusion, cystic and interstitial lung disease. Although limited in size, this series suggests the prognosis of patients with RDD and intrathoracic manifestations is relatively good.
Update on pulmonary and pleural lymphoproliferative disorders
2008, Diagnostic Histopathology
Citation Excerpt :
Parenchymal involvement is less common.210 Ohori et al. reported a single case of Rosai–Dorfman disease presenting within the pleura.211 Histological features are similar to nodal and other extranodal-based cases and consist of a distinctive proliferation of large histiocytic cells variably admixed with lymphocytes, plasma cells and neutrophils.
Pulmonary lymphoproliferative disorders consist of a spectrum of malignant and benign proliferations of lymphoid cells which primarily affect the lung. In recent years, the development of new molecular and immunohistochemical techniques has greatly enhanced our understanding of the classification and pathogenesis of these disorders. This review will highlight the pathological features of these entities and discuss the implications of recent discoveries regarding their pathogenesis.

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Original contributionRosai-dorfman disease of the pleura: a rare extranodal presentation

Abstract

Section snippets

Case report

Pathologic findings

Discussion

Immunologic abnormalities and their significance in sinus histiocytosis with massive lymphadenopathy

Am J Clin Pathol

Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease)Review of the entity

Semin Diagn Pathol

Non-neoplastic disorders of the lower respiratory tract

Disseminated sinus histiocytosis with massive lymphadenopathyIts pathologic aspects

Arch Pathol Lab Med

Original contribution
Rosai-dorfman disease of the pleura: a rare extranodal presentation