Involvement Of Rosai-Dorfman Disease Research Articles

Hairpin vessels in purely cutaneous Rosai-Dorfman disease of the face: A case report

Rosai-Dorfman disease (RDD) is a benign non-Langerhansian histiocytosis mainly affecting lymph node areas. Although cutaneous involvement in RDD is common, purely cutaneous disease is rare and poorly documented. We report the case of a 25-year-old female patient presenting with a 6-month history of a slow-growing asymptomatic lesion of the right cheek mimicking a cutaneous sarcoidosis. The aim of this article is to describe this rare entity with the presentation of a new dermoscopic finding: the hairpin vessels. We also highlight the differential diagnosis with sarcoidosis.

Sciatic Nerve Involvement in Rosai-Dorfman Disease.

Sciatic Nerve Involvement in Rosai-Dorfman Disease.

An unusual diagnosis of a dural based intracranial lesion

An unusual diagnosis of a dural based intracranial lesion

Thyroid Rosai-Dorfman disease with infiltration of IgG4-bearing plasma cells associated with multiple small pulmonary cysts

BackgroundRosai-Dorfman disease (RDD) is a rare histiocytosis which involves principally lymph nodes. Thyroid involvement in RDD is a very rare situation, and lung involvement is even rarer.Case presentationWe report the case of a 46-year-old woman presenting a painless mass in the right side of the neck and subacute dyspnoea. Computerised tomography (CT) scans of the neck and thorax showed a large thyroid mass causing tracheal stenosis and multiple cystic lesions in both lungs. Subtotal thyroidectomy with a tracheal segment resection and histological analysis confirmed the diagnosis of nodal and extranodal (thyroid, tracheal and probably lung) Rosai-Dorfman disease (RDD) with the presence of increased numbers of IgG4-bearing plasma cells. Clinical, functional and radiological follow up 4 years after surgery without medical treatment did not show any disease progression.ConclusionsThis case report indicates a benign course of nodal RDD with thyroid and tracheal infiltration following surgical resection, association of typical histological signs of RDD (emperipolesis) with IgG4-related disease features, and that lung cysts might be a manifestation of RDD.

Bilateral Breast Involvement in Rosai-Dorfman Disease: Report of a Case in a Middle Aged Lady

Bilateral Breast Involvement in Rosai-Dorfman Disease: Report of a Case in a Middle Aged Lady

Bronchial Involvement in Rosai Dorfman Disease

Bronchial Involvement in Rosai Dorfman Disease

Rosai-Dorfman Disease Involving Multiple Organs: An Unusual Case with Poor Prognosis.

Rosai-Dorfman disease is a rare, benign histiocytic proliferative disorder that usually affects the lymph nodes. Although extranodal involvement has been reported in diverse sites, manifestation in the cardiovascular system is extremely rare. Specifically, cardiac involvement in Rosai-Dorfman disease is an extraordinarily infrequent event. We describe a case of a 36-year-old female who presented Rosai-Dorfman disease of multiple organs including the heart, with poor prognosis.

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.

A Rare Cause of Massive Lymphadenopathy

Rosai Dorfman Disease is a rare disease presenting mainly as painless enlargement of cervical lymph nodes. It is a benign disorder showing unique histological features characterized by reactive proliferation of sinus histiocytes. In 40% of cases, extranodal sites may be involved. The condition has to be differentiated from certain types of B cell lymphoma showing similar features. We report here a 17year old male presenting with nodal and extranodal involvement of Rosai Dorfman Disease.

Rosai–Dorfman disease: A case report with extranodal thyroid involvement

Rosai-Dorfman Disease (RDD) or Sinus Histiocytosis with Massive Lymphadenopathy (SHML) is a rare disorder typically manifesting as enlarged lymph nodes with or without systemic involvement. These cases are often clinically misdiagnosed as lymphoma. Recognising this entity to distinguish it from lymphoma and other causes of histiocytosis is important because of different treatment modalities for these disorders. Extranodal involvement is also common, often with a particular predilection for the head and neck region. We report a rare case of Rosai-Dorfman Disease with both nodal and extranodal involvement in a 33-year-old woman. The patient had bilateral cervical lymphadenopathy and diffuse thyroid enlargement. Thyroid gland involvement in RDD diagnosed on fine needle aspiration cytology (FNAC) has rarely been reported in literature. FNAC is a useful and reliable tool for the diagnosis of RDD and the biopsy can be avoided in these patients, thus reducing inconvenience to patients.

Extranodal Rosai-Dorfman Disease Involving Colon A Case Report with Review of the Literature

Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare, idiopathic and non-neoplastic histiocytic disorder. The most common presentation of RDD is painless cervical lymphadenopathy in a young patient. 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 other extranodal sites (extranodal type). This can pose a diagnostic challenge if RDD is not considered in differential diagnoses. Gastrointestinal (GI) involvement of RDD is extremely rare; only 15 cases had been reported. We report a case of extra-nodal RDD involving the GI tract of a 24-year-old African-American woman who presented with chronic lower abdominal pain, constipation and an 8 cm mass involving her colonic wall. Microscopically, the mass was well demarcated, partially covered with serosa and extended into the muscularis propria and submucosa of the colon. The lesional cells consisted of spindle-shaped and epithelioid histiocytes intermixed with plasma cells and lymphocytes. Rare histiocytes showed emperipolesis. The histiocytes were positive for S100 (strong) and CD68, and negative for ckit and CD1a. The above morphological and immunohistochemical features are diagnostic for RDD, extra-nodal type. The related literature was reviewed and compared with this case.

P934 Breast involvement in Rosai-Dorfman disease – Related case

P934 Breast involvement in Rosai-Dorfman disease – Related case

Extranodal Rosai–Dorfman disease involving bilateral ovaries in a patient with a ventriculoperitoneal shunt

Rosai-Dorfman disease (RDD) is a rare condition of unknown etiology, and female genital tract involvement in RDD is uncommon. We describe the first case of RDD with bilateral ovarian involvement in a patient implanted with a ventriculoperitoneal (VP) shunt. The patient was a 17-year-old Japanese woman who had undergone radiotherapy, surgery for extranodal RDD involving the brain, and VP shunt insertion at age 12. Bilateral pelvic masses were incidentally detected on a computed tomography scan. She underwent laparotomy for lesion extirpation. On abdominal washing cytology, histiocytes showing emperipolesis were identified. Bilateral salpingo-oophorectomy was performed instead of extirpation, as it was difficult to identify the lesion margins. At 24 months after surgery, the patient is well and has not developed local recurrence. Thus, RDD can recur because of implantation of lesion cells into the abdominal cavity through a VP shunt, as is observed in the case of cerebral neoplasms.

Extranodal Rosai–Dorfman disease with multilevel lumbar spinal lesions

The authors describe the case of a 44-year-old man with multilevel lumbar spinal Rosai-Dorfman disease (RDD), a rare clinical entity. To the authors' knowledge, there have been only 2 cases of lumbar spinal involvement of RDD (epidural) reported in the literature, and the current case is the third but the only one showing lumbar spinal intradural involvement of RDD. This case of RDD mimicked a meningioma both clinically and radiologically. The patient underwent a procedure in which the tumor was excised, and postoperatively the patient made a clinically acceptable recovery. Vertebral canal involvement of RDD should be considered in the differential diagnosis of vertebral canal tumors. Resection is an acceptable treatment option.

Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): a clinicoradiological profile of three cases including two with skeletal disease

Originally described as sinus histiocytosis with massive lymphadenopathy, Rosai-Dorfman disease (RDD) is a rare histiocytic proliferative disorder with a distinctive microscopic appearance. A rare entity, RDD is often under-diagnosed because of a low index of suspicion by both radiologist and pathologist. Through this article, we wish to apprise radiologists of the spectrum of disease that can be encountered in this disorder. RDD can mimic other common childhood skeletal diseases including benign Langerhans cell histiocytosis and lymphoma. The clinical and radiological manifestations of RDD vary depending upon organ involvement, and its imaging features are often confused with those of other disorders. RDD should be considered in the differential diagnosis of unifocal and multifocal skeletal involvement caused by granulomatous diseases, infections, pseudogranulomatous lesions and malignancy. As long-term outcome is usually good, a conservative approach is justified in most cases. Contrasted with its typical appearance, presenting with bilateral symmetrical cervical adenopathy (as shown in one patient), we also report extranodal involvement of bone in two patients. Extranodal disease occurs along with concomitant nodal disease in about 43% of patients. In 23% of patients, isolated extranodal RDD can be seen, most commonly in the head and neck. In two of our patients, we observed extranodal involvement with skeletal involvement away from the head and neck not associated with lymphadenopathy. Skeletal involvement in RDD without lymphadenopathy is rare, occurring only in 2% of all the patients reported to date.

Extranodal Rosai-Dorfman Disease presenting as an isolated epibulbar mass

Rosai-Dorfman disease is a rare idiopathic disorder characterized by painless lymphadenopathy with cervical involvement in more than 80% cases. We report a case of Rosai-Dorfman disease presenting as an isolated epibulbar mass in a healthy young adult male. Epibulbar involvement in Rosai-Dorfman disease is a rare presentation as can be seen from a review of all literature. The presentation, differential diagnosis and treatment are discussed.

Thyroid Involvement in Rosai-Dorfman Disease

Rosai-Dorfman disease (RDD) with thyroid involvement is extremely rare; we herein present the sixth known case with a review of the previously reported five cases and discuss the clinicopathological characteristics of this entity. RDD with thyroid involvement has occurred only in females with a mean age of 56.3 years, which is significantly different from nodal RDD predominantly seen in young males. Besides this, autoimmune thyroiditis is frequently associated in RDD with thyroid involvement, which raises a suspicion of a common pathogenesis of both entities. RDD with thyroid involvement is commonly misdiagnosed preoperatively as thyroid malignancy with lymph node metastasis, which may lead to overtreatment and postoperative complication. Clinical presentations might be helpful for a preoperative differential diagnosis; while thyroid function, sonography, thyroid isotope scan, and fine needle aspiration have been less useful. All six cases were treated by surgery and complete remission was seen. However, two patients with a preoperative diagnosis of benign disease suffered from hypothyroidism due to total thyroidectomy.

Cutaneous rosai-dorfman disease is a distinct clinical entity.

Rosai-Dorfman disease (RDD) is a rare but distinctive clinicopathologic entity of unknown etiology affecting lymph nodes as well as extranodal sites. Although cutaneous involvement in RDD is common, purely cutaneous disease is rare and not well documented. We report 22 patients with cutaneous and superficial subcutaneous RDD. The lesions presented as papules and nodules, often with discoloration (9/22) and frequent multifocality (13/22), without predilection for a specific site of the body. Age distribution was wide and ranged from 15 to 68 years, with a median of 43.5 years. Of the 17 patients for whom information on racial background was available, 7 were Asian, 8 were white, and 2 were black, with a marked female predominance (2:1). The lesions resolved in 6 of 13 patients for whom follow-up data were available, regardless of the treatment given. Lesions persisted or recurred in 7 patients. Histologically, the lesions are invariably characterized by a proliferation of polygonal S100-positive histiocytes showing emperipolesis and a mixed inflammatory infiltrate. This study characterizes the histologic spectrum of cutaneous RDD in regard to variation in the numbers of typical S100-positive histiocytes and emperipolesis, variation in the quality and quantity of the inflammatory response, and the degree of stromal fibrosis, which resulted in a strikingly storiform growth pattern in six lesions and a lobulated pattern in two lesions. Whereas the clinical as well as histologic appearance of the cutaneous and subcutaneous lesions in the purely extranodal forms of RDD is indistinguishable from that of systemic RDD, this study emphasizes that purely cutaneous RDD is a distinct clinical entity in regard to its epidemiology and remains localized to the skin even with long-term follow-up. Patients with purely cutaneous RDD are of an older age at onset of disease (median = 43.5 years), with a reversed male/female ratio. There are no significant systemic extracutaneous or serologic manifestations. Whereas systemic RDD is commonly seen in blacks and only rarely reported in Orientals, the majority of the patients in this series with purely cutaneous RDD are Asians and whites.

Central nervous system involvement in Rosai-Dorfman disease: Report of a case with a review of the literature

Central nervous system involvement in Rosai-Dorfman disease: Report of a case with a review of the literature