Extranodal Rosai-Dorfman Disease Research Articles

Extranodal Rosai-Dorfman Disease Involving Maxilla

Sinus histiocytosis with massive lymphadenopathy, described in 1969 by Rosai and Dorfman, is a benign and self-limited disease, characterized by a painless cervical lymphadenopathy, usually bilateral, accompanied of fever, neutrophilic leukocytosis, and polyclonal hypergammaglobulinemia. It has a predilection for young adult men. We report an atypical case of Rosai-Dorfman disease with isolated involvement of the maxilla. The diagnosis was established by correlating detailed histopathological, immunohistochemical, and clinical data. Several lesions can be included in the differential diagnosis of Rosai-Dorfman disease, such as Langerhans cell histiocytosis, lymphoma, metastatic tumors, and various infections, including leprosy and histoplasmosis. Although the disease is rare, recognizing extranodal Rosai-Dorfman disease involving the jaws is essential to avoid misdiagnosis as a malignant neoplasm or infectious disease and to provide appropriate treatment.

Rosai dorfman syndrome with extranodal presentation

Rosai-Dorfman disease (RDD) also known as sinus histiocytosis with massive lymphadenopathy is a rare idiopathic and non-neoplastic disorder of histiocytes, resembling the sinus histiocytes of lymph nodes. The most common presentation of RDD is painless cervical lymphadenopathy in a young patient. The typical clinical features of this disease include bilateral painless lymphadenopathy, fever, and polyclonal hyperglobulinemia. However, in about 25% of cases there is no lymph node involvement and the disease presents as a mass in skin, soft-tissue, nasal cavity, eye, bone, or in other extranodal sites (extranodal type). The condition may present with extranodal involvement in 43% of cases, and cutaneous lesions are the most common form of extranodal disease. This can pose a diagnostic challenge if RDD is not considered in differential diagnoses. Isolated extranodal RDD is relatively uncommon. We report this rare case of extranodal RDD in a 32-year-old female who presented with erythematous tender subcutaneous plaques on lower extremities with left periorbital swelling. Histopathological examination demonstrated lymphophagocytosis (emperipolesis) consistent with a diagnosis of RDD.

Benign scalp lump as an unusual presentation of extranodal Rosai-Dorfman Disease

Background:Rosai–Dorfman disease (RDD) is a rare benign histioproliferative disease. It is typically characterized by benign histiocyte proliferation with lymphadenopathy, fever, and leukocytosis and was first described in 1969 by Rosai and Dorfman. Extranodal involvement has been reported in approximately up to 43% of the cases with isolated central nervous system (CNS) manifestations being even rarer.Case Description:We report our management of a 41-year-old female with extranodalpurely CNS RDD presenting as a benign scalp lump. Her lump progressed from an asymptomatic benign lesion to one causing localized cerebral edema. Treatment was surgical excision of both the cervical and CNS lesions achieving complete removal of the lesions and resolution of her symptoms.Conclusion:RDD is a rare condition and isolated CNS RDD is even less common. Benign scalp lumps have a myriad of differential diagnoses, but RDD should be a consideration in the presence of preexisting RDD lesions at other sites given its potential to progress and result in morbidity. It is imperative to be aware that symptoms may be especially deceiving as the absence of lymphadenopathy may point away from RDD as the diagnosis.

Sinonasal Rosai-Dorfman Disease

ABSTRACTAimRosai-dorfman disease (RDD) is a rare histiocytic proliferative disorder characterized by painless massive lymphadenopathy. Extranodal manifestations may occur in the head and neck area. This is a case report of a patient with sinonasal RDD and its management.Materials and methodsThis is a case report of a 36-year-old patient with sinonasal RDD. The clinical features, imaging and histological features are described. The patient underwent endoscopic surgery. Recurrence (8 months later) was managed by laserization of the nasal mass with KTP/532 laser followed by oral corticosteroid therapy.ResultsA 36-year-old lady presented with complaints of left sided nasal obstruction, nasal discharge and left facial discomfort of 6 months duration. There was history of occasional blood stained nasal discharge. Diagnostic nasal endoscopy revealed a polypoidal mass in the left nasal cavity. CT scans revealed soft tissue opacity in the left maxillary antrum, left ostiomeatal complex and extending into the left orbit. There was erosion of the posterolateral wall of the left maxilla. The patient underwent endoscopic surgery and had an uneventful postoperative recovery. Biopsy was reported as Rosai-Dorfman disease. Subsequently, 8 months later, the patient reported with a complaint of nasal obstruction and discharge. A recurrent lesion was noted in the left nasal cavity and maxillary antrum. The mass was managed by laserization followed by oral corticosteroid therapy. One year later, the patient is free from disease and has been advised regarding the necessity of periodic follow-ups.ConclusionRDD is a rare disease encountered by the otolaryngologist. The manifestation of extranodal Rosai-Dorfman disease is most commonly seen in the nasal cavity and paranasal sinuses. The clinical findings and imaging characteristics are variable and the diagnosis can be easily missed. Otolaryngologists and pathologists must be aware of the clinical presentation, imaging characteristics and histologic features of Rosai-Dorfman disease.How to cite this articleNatarajan K, Devarasetty A, Murali S, Senthilvadivu A, Sudhamaheswari, Kameswaran M. Sinonasal Rosai-Dorfman Disease. Clin Rhinol An Int J 2014;7(3):142-146.

Rosai-dorfman disease in thoracic spine: a rare case of compression fracture.

Sinus histiocytosis with massive lymphadenopathy known as Rosai-Dorfman disease is characterized by painless bilateral cervical lymphadenopathy. Extranodal manifestations are uncommon and spinal involvement is rare. A 15-year-old man presented with intermittent midthoracic back pain only. He had no specific findings on neurologic examinations, hematologic and biochemical laboratory tests. Radiological examination of thoracic spine revealed collapse of T6 vertebrae with thoracic kyphosis and osteolytic lesion of T12 vertebra body. He underwent a removal of bone tumor, anterior reconstruction with mesh and pedicle screw fixation via posterior approach for pathologic confirmation and stabilization. Histopathologic study of the lesion revealed focal infiltration of large histiocytes showing emperipolesis. Immunochemistry stain of histiocytes was positive for CD68 and S-100 but negative for CD1a. This report presents a rare case and literature review of extranodal Rosai-dorfman disease in thoracic spine.

Rosai-Dorfraan disease in nasal cavity：two case reports and literature review

Objective To study the clinical manifestations, pathological features and differential diagnosis of extranodal Rosai-Dorfman disease （RDD） in nasal cavity. Methods The clinical, pathologic, immunohistochemical and special staining features of 2 cases of RDD were evaluated and related literatures were reviewed. Results Extranodal RDD in nasal cavity and various degrees of stromal fibrosis were seen association with mixed inflammatory cells （especially plasma cells）.The large polygonal histiocytes varied in number and were distributed in clusters or patches. Conclusion Extranodal RDD of nasal cavity is relatively rare and pathologic diagnosis may be difficult. It can easily mimic rhinoscleroma, hyperplasia of langerhans cells, granulomatous inflammation and fibrous tumors. Immunohistochemical and histochemical study is helpful in the differential diagnosis. Key words: Rosai-Dorfman disease; Nasal cavity; Histiocytosis; Immunohistochemistry; Differentialdiagnosis

Relationship Between Rosai-Dorfman Disease and IgG4-Related Disease

To assess the association between Rosai-Dorfman disease (RDD) and IgG4-related disease (IgG4-RD). We studied the number of IgG4-positive plasma cells and the IgG4/IgG ratio in 32 biopsy specimens (13 nodal, 19 extranodal) from 29 patients with RDD and compared the findings with those in IgG4-RD of the pancreas and reactive lymph nodes. We also assessed the number of FOXP3-positive regulatory T cells (Tregs) since they were reported to be increased in IgG4-RD. We found that RDD cases had much lower numbers of IgG4-positive plasma cells and lower IgG4/IgG ratios compared with IgG4-RD but were similar to reactive lymph nodes. Furthermore, RDD had lower numbers of FOXP3-positive Tregs than did IgG4-RD. There were no significant differences in the number of IgG4-positive plasma cells and the IgG4/IgG ratio between the nodal and extranodal RDD cases. Our study suggests that RDD does not belong in the spectrum of IgG4-RD.

Extranodal Rosai-Dorfman Disease Associated With Increased Numbers of Immunoglobulin G4 Plasma Cells Involving the Colon: Case Report With Literature Review

A 49-year-old woman presented with fever, weight loss, night sweats, hematochezia, and acid reflux symptoms. Two large, firm cecal lesions were seen at colonoscopy, but multiple biopsies were inconclusive. The patient underwent a right hemicolectomy for a clinical diagnosis of colon cancer. Noncaseating granulomatous inflammation with background lymphocytes, plasma cells, and histiocytes exhibiting emperipolesis were identified. With these histologic features and immunoreactivity for S-100 protein and CD68, a diagnosis of Rosai-Dorfman disease was rendered. Other areas had storiform fibrosis admixed with numerous immunoglobulin G4 (IgG4)-positive plasma cells. Although a few preliminary reports have noted an increased number of IgG4-positive plasma cells in Rosai-Dorfman disease, the relationship between these 2 conditions is unclear. To our knowledge, this is the first case report of a possible association of colonic Rosai-Dorfman disease with an increased number of IgG4-positive plasma cells. Reviews of colonic Rosai-Dorfman disease and IgG4-related sclerosis are presented to heighten awareness of this rare presentation.

A Subset of Rosai-Dorfman Disease Exhibits Features of IgG4-Related Disease

In this study we investigated the distribution of IgG4+ plasma cells and regulatory T (T(REG)) cells, a major regulator of IgG4 production, in nodal and extranodal Rosai-Dorfman disease (RDD). Twenty-six specimens (15 nodal, 11 extranodal) were examined, with reactive lymph nodes and site-matched extranodal specimens as controls. Overall, 84.6% (22/26) of the specimens showed various degrees of sclerosis (7 mild, 8 moderate, and 7 severe). Nineteen cases (73.1%) exhibited more than 10 IgG4+ cells/0.060 mm(2) (photographed area at ×40), and 8 cases (30.8%) showed more than 40% of IgG+ cells being IgG4+. Only 1 control case exhibited more than 10 IgG4+ cells/0.060 mm(2) (P < .05). The number of T(REG) cells was comparable between nodal RDD and controls, whereas extranodal RDD exhibited significantly higher numbers of T(REG) cells than controls. These findings demonstrate that a subset of RDD shows features of IgG4-related disease and indicate an overlap between certain aspects of the 2 diseases.

Extranodal Rosai-Dorfman disease involving the pulmonary artery†

Extranodal Rosai-Dorfman disease involving the pulmonary artery†

Extranodal Rosai–Dorfman disease: a rare soft tissue neoplasm masquerading as a sarcoma

IntroductionRosai–Dorfman disease (RDD) is a rare proliferative histiocytic disorder of unknown etiology. RDD typically presents with generalized lymphadenopathy and polymorphic histiocytic infiltration of the lymph node sinuses; however, occurrences of extranodal soft tissue RDD may rarely occur when masquerading as a soft tissue sarcoma.Materials and methodsA comprehensive search of all published cases of soft tissue RDD without associated lymphadenopathy was conducted using PubMed and Google Scholar for the years 1988 to 2011. Ophthalmic RDD was excluded.ResultsThirty-six cases of extranodal soft tissue RDD, including the current one, have been reported since 1988. Anatomical distribution varied among patients. Four (11.1%) patients presented with bilateral lesions in the same anatomic region. Pain was the most common symptom in six (16.8%) patients. Sixteen (41.6%) patients were managed surgically, of which one (2.8%) case experienced recurrence of disease.ConclusionRDD is a rare inflammatory non-neoplastic process that should be considered in the differential diagnosis of a soft tissue tumor. Thus, differentiation of extranodal RDD from more common soft tissue tumors such as soft tissue sarcoma or inflammatory myofibroblastic tumor is often difficult and typically requires definitive surgical excision with histopathological examination. While the optimal treatment for extranodal RDD remains ill-defined and controversial, surgical excision is typically curative.

Rosai-Dorfman Disease: A Retrospective Analysis of 13 Cases

Rosai-Dorfman disease (RDD) is a rare, idiopathic, histiocytic proliferative disorder, the infrequent occurrence of which limits in-depth studies. Consequently, many characteristics of this disease remain unknown, restricting early diagnosis and proper treatment. In this study, the literature was reviewed and a retrospective analysis of the medical records of 13 patients with RDD conducted to investigate the demographic data, clinical data, laboratory and imaging results, treatment, and prognosis of this disease. Of the 13 cases in our sample, 10 (77%) were purely extranodal RDD, 2 (15%) were both nodal and extranodal, and 1 (8%) was purely nodal. The locations of the 10 purely extranodal RDD lesions included the central nervous system (n = 6, 60%), nasal cavity and paranasal sinuses (n = 3, 30%), and the cutis (n = 1, 10%). The locations of the central nervous system-related RDD lesions included the cerebral subdura (n = 2, 29%), the sellar region (n = 3, 14%), the cerebral parenchyma (n = 1, 14%) and the spinal subdura (n = 1, 14%). Ten patients (77%) had stable conditions, 3 (23%) experienced recurrence, and 2 (15%) experienced recurrence and lesion metastasis. RDD is rare, requiring knowledge of its clinical manifestations for a rapid and correct diagnosis. In light of the possibility of recurrence and lesion metastasis, long-term follow-up is needed. Treatment is still controversial. Future efforts should be directed at investigating the etiology and postoperative treatment for relapsing cases or those with subresected lesions.

Steroid-Resistant Extranodal Rosai-Dorfman Disease of Cheek Mass and Ptosis Treated with Radiation Therapy

Background. Rosai-Dorfman Disease (RDD) is rare benign hematologic disorder of histiocytes, which usually manifests as painless lymphadenopathy, fever, leukocytosis and hypergammaglobulinemia. Extranodal RDD has been reported in 43% of cases, with skin as commonly involved site followed by head and neck region. However, soft tissue cheek mass is rare presentation of extra-nodal RDD. Case Presentation. A 26-year-old Saudi man presented with a six-month history of right cheek swelling and left upper eyelid swelling. Physical examination revealed right cheek mass of size 3 × 3 cm and left upper eyelid mass of size 1 × 2 cm and no palpable cervical lymphadenopathy. Incisional biopsy of cheek mass showed positivity for S100 and negativity for CD1a, consistent with extra-nodal RDD. Patient did not respond to systemic steroids and was given radiation therapy to left orbit with minimal response. Then, he was started on chemotherapy Rituximab, cyclophosphamide, vincristine, and prednisolone (RCVP) 8 cycles followed by reirradiation 10 Gy in 10 fractions with 6 MeV electron with complete response. After 12 months of followup, patient had recurrence in right cheek and was started on radiotherapy to the cheek mass. Conclusion. Extra-nodal RDD with involvement of cheek is a rare presentation. Incorporation of S100 and CD1a is helpful in diagnoses of RDD and differentiating it from other benign histiocytosis. Treatment consists of surgery, steroids, chemotherapy, and radiation therapy.

Extranodal Rosai-Dorfman disease of the nose: a clinicopathologic study of 10 cases with review of literature

Rosai-Dorfman病(RDD)是少见的良性组织细胞增生性疾病,患者常以无痛性淋巴结肿大为主要症状.该疾病在亚洲地区发病率较低,且鼻的结外RDD组织学表现随病程进展变异性增大,故易误诊或漏诊.我们对10例鼻的结外RDD病例回顾性研究,讨论其病理诊断与鉴别诊断。

Isolated Cardiac Involvement of Rosai-Dorfman Disease

Rosai-Dorfman disease is a rare and multisystem disorder of unknown etiology. It commonly presents as cervical lymph node enlargement, but extranodal involvement may be presented in one-third of the cases. Usually, the clinical course of Rosai-Dorfman disease is benign but it can be malignant, both clinically and pathologically. Herein, we present an isolated cardiac case of extranodal Rosai-Dorfman disease without lymphadenopathy that involves the left ventricle in a symptomatic adult patient and a description of cardiac magnetic resonance imaging findings of this disease.

Bilateral intraventricular mass in a child

An 8-year-old boy presented with a history of holocranial headache for 4 weeks which was mild to moderate in intensity. There was history of intermittent vomiting for the same duration. The patient was drowsy for the last 10 days. Non-contrast CT scan of head showed a space-occupying lesion in lateral ventricles with hydrocephalus. MRI showed a large lobulated well-defined heterogeneous mass lesion filling up both lateral ventricles at the posterior and temporal horns, bilaterally reaching up to the level of the foramen magnum. The mass lesion was hypointense on T2-weighted images (T2W) and isointense to grey mater on T1W images. On contrast images there was intense homogeneous enhancement seen at the lesion (Fig. 1). Right fronto-temporo-parietal craniotomy with decompression of the tumor was performed. Perioperatively, the tumor was yellowish in color, firm, not able to be aspirated and minimally vascular. Clinical diagnosis of choroid plexus tumor was considered.

Cutaneous Rosai-Dorfman Disease Recurrence in Infraorbital Region

Rosai-Dorfman disease (RDD), also named sinus histiocytosis with massive lymphadenopathy, is a rare, idiopathic, and benign disorder that classically presents as a painless massive bilateral cervical lymphadenopathy. In cases of extranodal presentations, such as cutaneous RDD, it may not involve the classic manifestation. The diagnosis is usually made by histopathologic analysis. Surgery is suggested in the cases of significant cosmetic deformity or when there is fatal or functional obstruction. We reported a case of extranodal RDD that recurred in the infraorbital region postoperatively.

Poster 56: Oral Cavity Rosai-Dorfman Disease Without Lymphadenopathy: A Case Series From Two Institutions Within the Metropolitan New York Area

Rosai-Dorfman disease (RDD) is a rare benign non-Langerhans cell histiocytic disorder of unknown etiology. Classically associated with significant lymphadenopathy, it is often referred to as Sinus Histiocytosis with Massive Lymphadenopathy (SHML). Rare examples of RDD without clinical or radiographic evidence of concomitant lymph node involvement have been reported; however, only five cases thus far have been reported of extranodal RDD localized to the oral cavity. We report only the sixth and seventh cases, adding valuable information to this rare entity.

Extranodal Rosai–Dorfman disease in a carpal bone

We report a case of extranodal Rosai–Dorfman Disease (RDD) of the scophoid in a 52-year old female. The patient presented with pain, swelling, and tenderness on deep palpation of the left wrist. Clinicoradiological diagnosis was osteomyelitis or tenosynovitis and curettage was performed on the lytic lesion over scaphoid to procure tissue. Diagnosis was made by histomorphology supported by immunostaining. The patient was managed conservatively with resolution of the lesion.

Extranodal Rosai-Dorfman Disease Presenting as a Solitary Mass with Human Herpesvirus 6 Detection in a Pediatric Patient

In Rosai-Dorfman disease (RDD), exclusive extranodal involvement with lesions limited to the kidneys is very uncommon and has been described only in adult patients. Occasionally, human herpesvirus 6 (HHV-6) has also been detected in RDD tissue samples. We present the case of a 7-year-old boy referred to our center presenting a single solid mass in the right kidney measuring 3.4 cm, detected both on contrast computed tomography and magnetic resonance imaging. Surgical excision was successfully completed, and the pathology report informed characteristic histopathology and immmunohistochemistry features of RDD. Human herpesvirus 6 was detected and amplified by polymerase chain reaction, as well as by immunohistochemistry. We discuss imaging and histology-based differential diagnoses in the pediatric age group. Although RDD is a rare histiocytic disorder of unknown etiology and pathogenesis, the presence of HHV-6 observed in this case supports the possibility of an abnormal immunologic response linked to viral presence.