Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease)

A child of 11 years with a solitary involvement of the right radius by sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) is presented. The literature of bone involvement in this condition is reviewed.     

Fine-needle aspiration cytology of sinus histiocytosis with massive lymphadenopathy: A case report

Cytological findings of sinus histiocytosis with massive lymphadenopathy (SHML), involving bilateral cervical lymph nodes of a 12-yr-old boy, are presented here. A striking feature was the presence of large histiocytes with abundant pale cytoplasm containing well-preserved lymphocytes, plasma cells, and occasional neutrophils, scattered in a background of reactive lymphoid cells. Subsequent open biopsy of the lymph node mass confirmed the cytologic findings. Cytologic features of SHML are virtually diagnostic and can negate the need for an open biopsy in most cases. Diagn Cytopathol 1996;15:221–223. © 1996 Wiley-Liss, Inc.     

Sinus histiocytosis with massive lymphadenopathy (Rosai–Dorfman disease): report of a case with widespread nodal and extra nodal dissemination

A fatal case of sinus histiocytosis with massive lymphadenopathy (SHML) is described in a male patient who presented at 11 years-of-age with swelling of the malar region and nasal obstruction due to the disease and who died aged 28 in uraemic coma following renal involvement. At autopsy SHML tissue extended into the hilum of both lungs and from the retroperitoneal tissue into the pancreas and kidneys. The appearances suggest that the extension of the disease into these organs may have been due to retrograde lymphatic spread from involved lymph nodes. Replacement of the atypical sinus histiocyte by fibrous tissue was seen in the cervical lymph nodes and this may be the usual mode of resolution of the lesion. The nature of SHML is discussed in relation to a possible infectious aetiology and defects in the host immune system. Finally, it is noted that the term 'massive lymphadenopathy' is not applicable to all cases and that sinus histiocytosis is not appropriate for the now frequently recognized extranodal manifestations of the disease. It is proposed that the term 'lymphophagocytic histiocytosis syndrome' should be adopted or that the eponymous title 'Rosai-Dorfman disease' should be used.     

Diagnosis of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) by fine needle aspiration cytology

Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) is a rare benign disease of unknown etiology presenting with massive lymphadenopathy. We report four cases of this disease retrieved from the archives of cytopathology laboratory, BPKIHS, Dharan, Nepal, during the period of January 2002 to October 2007. The cytologic morphology revealed lymphocytes, plasma cells, and single to multinucleated histiocytes with well preserved lymphocytes in their cytoplasm (emperipolesis or lymphophagocytosis). It is concluded that FNAC is a useful and reliable tool for the diagnosis of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) and biopsy can be avoided in these patients, thereby reducing patient inconvenience and health care cost.     

Sinus histiocytosis with massive lymphadenopathy Rosai-Dorfman: three unusual manifestations

We report on three exceptional courses of sinus histiocytosis Rosai-Dorfman. Patient one developed regional lymph-node disease subsequent to two independent malignancies in the right head and neck region. Patient two suffered from extensive extranodal disease with more than 100 mucocutaneous lesions over 17 years, which spontaneously resolved. Patient three showed exclusively extranodal disease, including bilateral conjunctival/scleral lesions, before he developed lung cancer. Our cases are unique for three reasons: the association of the disease with solid malignancies in two cases, the extent and persistence of exclusively extranodal disease in one patient and the appearance of thus far undescribed conjunctival/scleral lesions.     

Sinus histiocytosis with massive lymphadenopathy (Rosai Dorfman disease) in an HIV-positive patient

Summary We report a case of a 31-year-old HIV infected black female, who presented with asymptomatic generalized lymphadenopathy. Three particularly enlarged lymph nodes were biopsied (2 cervical and 1 axillary). The histological picture was consistent with a diagnosis of sinus histiocytosis with massive lymphadenopathy (SHML) or Rosai-Dorfman disease. Large histiocytes, positive for a variety of macrophage markers and for the S-100 protein, were observed in the distended sinuses. A few hyperplastic follicles, such as usually seen in HIV-infection-associated lymphadenopathy, were present at the periphery of one lymph node. No infections agent besides HIV could be detected by histological or microbiological analysis or by in situ hybridization. This is the first reported case of SHML associated with HIV infection. The possible relationship between the two diseases is discussed.     

Sinus histiocytosis with massive lymphadenopathy: evidence for its relationship to macrophages and for a cytokine-related disorder

AIMS: Sinus histiocytosis with massive lymphadenopathy (SHML) or Rosai-Dorfman disease is a rare histiocytic disorder of unknown origin. Immunophenotypically the histiocytes of SHML express intensively the S100 protein and in addition a panel of macrophage-associated antigens. Their exact relationship to either monocytes/macrophages or immune accessory dendritic cells is, however, still controversial. METHODS AND RESULTS: In this report recurrent nodal and extranodal manifestations of SHML of a 70-year-old patient were analysed by differential phenotyping using a panel of monoclonal and polyclonal antibodies to macrophage and immune accessory dendritic cell related antigens and by applying nonradioactive in-situ hybridization. CONCLUSIONS: We conclude that stimulation of monocytes/macrophages via macrophage colony stimulating factor (M-CSF) leading to immune suppressive macrophages represents a main mechanism for the pathogenesis of SHML. The study further provides evidence for the monocyte/macrophage but not dendritic cell differentiation of SHML histiocytes.     

Hodgkin''s disease and sinus histiocytosis with massive lymphadenopathy-like changes

Sinus histiocytosis with massive lymphadenopathy (SHML) is a disorder of unknown origin which is only infrequently associated with lymphoid neoplasms. We report the first two cases of Hodgkin's disease with simultaneous SHML-like changes in the same lymph node.     

Salivary gland manifestations of sinus histiocytosis with massive lymphadenopathy: fine-needle aspiration cytology findings. A case report

Sinus histiocytosis with massive lymphadenopathy (SHML or Rosai-Dorfman disease) is a nonneoplastic, usually self-limiting disease. Alhough it affects all age groups, it is more commonly seen in young males in their first or second decades. The disease primarily manifests as painless lymphadenopathy of cervical region, but other nodal groups and extranodal sites may also be affected. Seldom SHML may involve the salivary glands. We present the cytologic features and differential diagnoses of one such case.A 45-yr-old woman presented with an enlarged submandibular gland on the left side with ipsilateral cervical lymphadenopathy that had been persisting for 1 mo. The gland and the two enlarged nodes measured 2.5 x 2.5 cm(2) each and were firm in consistency. Clinically, tumor of the salivary glands was suspected. Fine-needle aspiration (FNA) smears showed moderate cellularity, with large histiocytes dispersed in the background of intense lymphoplasmacytic infiltrate. These histiocytes showed lymphophagocytosis. Isolated stromal fragments consisting of fibrocytes were seen separate from salivary acinar clusters on repeat aspirations. The diagnosis of SHML involving left submandibular gland and ipsilateral lymph nodes was returned.When analyzed in the context of clinical findings (laboratory data), the cytologic features of SHML involving salivary gland could be differentiated from those of malignancies and other benign lesions, especially Kuttner's tumor of the submandibular gland, which mimics neoplasm clinically.     

Focal changes of sinus histiocytosis with massive lymphadenopathy (RosaiDorfman disease) associated with nodular lymphocyte predominant hodgkin's disease

Sinus histiocytosis with massive lymphadenopathy (SHML/RosaiDorfman disease) has, on rare occasions been identified as an isolated phenomenon in lymph nodes affected by malignant lymphomas. The Registry includes four cases of SHML in patients with non-Hodgkin's lymphomas and one with multiple myeloma. SHML has more recently been recorded as a focal finding in lymph nodes involved by Hodgkin's disease of the mixed cellularity type. We report two patients presenting with lymphadenopathy caused by involvement by nodular lymphocyte predominant Hodgkin's disease with focal changes of SHML, an association not previously recorded in the literature. Responsiveness of the histiocytic cells of SHML to B-cell derived cytokines is postulated as a mechanism for this phenomenon, an hypothesis previously raised in regard to the association of focal Langerhans cell histiocytosis with Hodgkin's disease and with non-Hodgkin's lymphomas.     

Sinus histiocytosis with massive lymphadenopathy (rosai-dorfman disease): Diagnosis with fine-needle aspiration in a case with nodal and nasal involvement

Sinus histiocytosis with massive lymphadenopathy (SHML) is a rare entity characterized by the proliferation of cells belonging to the macrophage-histiocyte family, but whose exact origin is unknown. A case of SHML occurring in a 62-yr-old female is described in which the diagnosis is initially suggested by fine-needle aspiration (FNA) of a cervical node. The smears show a background of lymphocytes and plasma cells and large histiocytes with well-preserved lymphocytes in their cytoplasm (emperipolesis or lymphophagocytosis). Both nodal and extranodal (nasal mass) involvement are confirmed by surgical biopsy. The immunohisto-chemistry suggests that SHML cells are functionally activated macrophages. Eight months after diagnosis, the patient is clinically well, with partial improvement of lymphadenopathy. © 1995 Wiley-Liss, Inc.     

Sinus histiocytosis with massive lymphadenopathy in Rwanda: report of eight cases with immunohistochemical and ultrastructural studies

The clinico-pathological, immunohistochemical and ultrastructural features of eight cases of sinus histiocytosis with massive lymphadenopathy (SHML) recorded in Rwanda from 1975 to 1980 are reported.
The main histopathological features were a massive enlargement of the lymph node sinuses which were filled with large histiocytes, a great number of which phagocytosed blood cells, especially lymphocytes, and an important proliferation of plasma cells in the medullary cords. The pathogenesis of this syndrome is discussed, particularly the role of immunological disorders challenged by a chronic, but non-specific, infectious state. The ultrastructural study revealed no micro-organisms and no Langerhans' granules. Immunohistochemical staining revealed two interesting features: the presence of immunoglobulins in the cytoplasm of the histiocytes indicating the presence of an immune phagocytosis and the absence of lysozyme and α₁-antichymotrypsin (α₁-AC) from the histiocytes. The significance of the latter phenomenon is still unknown.     

Extranodal Rosai–Dorfman disease. Clinical and pathological characteristics in a patient with a pseudotumor of bone

We report the clinical and pathological findings of a 19-year-old Venezuelan patient with a proximal tibial lesion showing the histological and immunohistochemical characteristics of Rosai–Dorfman disease. The radiological studies showed a lithic bone lesion that was interpreted as a giant cell tumor.     

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

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.     

Iangerhans' cell histiocytosis of the lung in association with mediastjnal lymphadenopathy

A 17 year old female was seen because of a non-productive cough and one episode of blood-tinged sputum. A computerized tomography (CT) scan of the chest showed multiple small cystic lucencies in the upper lobes. The mediastinal CT window revealed concurrent mediastinal lymphadenopathy. An open lung biopsy showed Langerhans' cell histiocytosis (LCH). The significance of nodal involvement in LCH of the lung is not known, but raises the possibility of regional LCH and warrants a further search for bone or visceral involvement as well as closer follow-up and monitoring of patients.     

Rectal extranodal Rosai–Dorfman disease diagnosed by EUS‐FNA: A case report and review of the literature

Rosai–Dorfman disease (RDD), also known as “sinus histiocytosis with massive lymphadenopathy,” only rarely involves the gastrointestinal (GI) tract. Therefore, this unusual site of presentation can be challenging for the pathologist. We present a case of RDD manifesting as a rectal submucosal mass associated with rectal bleeding in a 54 year old woman. The diagnosis was made on cytologic preparations obtained through endoscopic ultrasound guided fine needle aspiration (EUS‐FNA) and subsequently confirmed by biopsy. To our knowledge, this is the first time extranodal RDD of the GI tract has been diagnosed by EUS‐FNA. A review of previously published cases of GI RDD is presented to increase awareness of this exceptional presentation. Diagn. Cytopathol. 2015;43:40–44. © 2014 Wiley Periodicals, Inc.     

The Electron Microsopic Features of Sinus Histiocytosis with Massive Lymphadenopathy: A Study of 11 Cases

Eleven cases of sinus histiocytosis with massive lymphadenopathy (SHML) involving lymph nodes were studied electron microscopically. Histiocytes were the most conspicuous element of the infiltrate. They could be divided into small and large forms, although transitions were apparent among them. Most of the small histiocytes were located in the medullary cords. The large histiocytes were predominantly seen within sinuses and were subdivided into two types on the basis of their appearance. The most distinctive feature of these histiocytes was the presence of lymphocytes, plasma cells, and neutrophils within their cytoplasm. Other cells present in the infiltrate were lymphocytes, plasma cells, and occasional neutrophils and mast cells. Blood vessels were prominent throughout. Virus particles, bacteria, and Langerhans granules were consistently absent. No morphologic clues were provided by this study as to the etiology of this disorder.