Spontaneous Regression of Rosai–Dorfman Disease Presenting as a Thigh Mass With Vascular Involvement: A Case Report

El tratamiento fiscal de las operaciones de permuta es uno de los asuntos mas controvertidos en los ultimos tiempos. En materia de IVA hemos asistido a pronunciamientos oficiales contradictorios. En el presente trabajo se analiza el tratamiento fiscal de las permutas, especialmente en el IVA y respecto a los intercambios de suelo por construcciones futuras. La Comision Europea tiene abierto un procedimiento de infraccion contra Espana sobre este particular. A raiz de este procedimiento y de reciente jurisprudencia del Tribunal Supremo, la Direccion General de Tributos ha cambiado su criterio. La argumentacion del Tribunal Supremo y de la Comision Europea sostiene que el recalculo de la base imponible en las operaciones de permuta no debe realizarse. La razon es que cuando la contraprestacion se abona en dinero las eventuales fluctuaciones desde la compra hasta la entrega de la edificacion no tienen efecto alguno respecto de la base imponible del Impuesto, por lo que el hecho de abonar la contraprestacion en especie no debe dar lugar a un resultado diferente.

Sinus histiocytosis with massive cervical lymphadenopathy (SHML) was originally described in 1969 as a benign clinicopathologic entity characterized by massive bilateral cervical lymphadenopathy, fever, leukocytosis, elevated ESR, and hypergammaglobulinemia, usually occurring within the first two decades of life. We present an illustrated case of an elderly patient with polyclonal hypergammaglobulinemia and a 2-year history of multilobulated cervical and submandibular lymphadenopathy. The etiology and pathogenesis of SHML are not known. Diagnosis requires lymph node biopsy to exclude other causes of cervical lymphadenopathy such as malignant lymphoma, malignant histiocytosis, metastatic carcinoma, and tuberculous lymphadenitis. Histologic examination shows marked dilatation of subcapsular and medullary lymph node sinuses containing large, foamy or vacuolated histiocytes. Although no curative treatment is known, corticosteroids, radiation therapy, vinblastine and oral cyclophosphamide, and surgery have been used to palliate constitutional symptoms and mechanical obstruction from massive lymphadenopathy. Since one third of SHML patients have evidence of disease for 5 years, and a mortality rate of 7% exists with benign histologic disease, all patients with SHML should be carefully screened for evidence of immunodeficiencies that may precipitate a fatal outcome.

Rosai Dorfman disease (RDD) also referred to as sinus histiocytosis with massive lymphadenopathy is a rare non-neoplastic lymphoproliferative disease with unknown etiology and pathogenesis first described by Rosai and Dorfman in 1969. It is characterized by histiocytic proliferation of lymphatic sinuses, which primarily involves the lymph nodes, but has an extranodal tendency. Frequently encountered extranodal sites in the head-and-neck region is paranasal sinuses and nasal cavity, orbit, parotid gland, and middle ear. We present a case of RDD occurring in the nasal cavity of a 28-year-old female from Nigeria. RDD should be considered a differential diagnosis in any patient with benign sinonasal mass especially arising from the nasal septum with massive cervical lymphadenopathy.

We report an unusual presentation of sinus histiocytosis with massive lymphadenopathy (SHML), also called Rosai Dorfman disease. The child presented with bilateral proptosis with massive cervical lymphadenopathy. The patient had fair response to steroid therapy but relapsed during the course of the disease.

Redefining the Prevalence of Dural Involvement in Rosai-Dorfman Disease of the Central Nervous System

Background Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy (SHML), is a non-neoplastic histiocytic proliferation predominantly affecting lymph nodes, and usually presents with massive painless cervical lymphadenopathy accompanied by fever and weight loss. Extranodal involvement occurs in over 40% of patients; however, isolated extranodal disease without lymph node involvement is unusual. Although extranodal involvement has been reported in diverse sites, central nervous system manifestation, particularly in spinal cord is distinctly rare. It is a diagnostic challenge for radiologists and histopathologists to differentiate RDD from other spinal lesions because of its similarities in radiological and histological findings. Herein we describe a case of unusual isolated RDD in spinal cord. The radiology and clinicopathology of this lesion, as well as its differential diagnosis are discussed. Methods The clinical manifestation of a patient with intraspinal primary RDD occurring C 3-6 level was presented retrospectively. Gross resected mass was routinely paraffin-embedded and stained with hematoxylin and eosin. Dako EnVision immunohistochemical staining system was used to detect the tumor antigen expressions, including S-100 protein (S-100), CD1a and CD68 (KP-1). Results A 25-year-old female patient presented with 3-month history of numbness and weakness in both upper limbs associated with an increasing neck back pain. There was no fever and lymphadenopathy found in the patient. MRI of the whole spine revealed a subdural mass extending from C 3 to C 6 level of cervical spinal cord with homogeneous enhancement after contrast administration. Laminectomy and midline opening of the dura were performed. The subdural lesion appeared to have no capsule and attach the dura mater. The lesion was removed totally. Under the microscopic examination, dense fibrosis and intense chronic inflammation with focal neutrophilic infiltrates were noted. The clusters of large histiocytes with eosinophilic, finely granular cytoplasm and multinucleate giant cells were also observed in the lesion. Emperipolesis with intact lymphocytes within the cytoplasm of the large histiocytes were present. Mitotic activity and necrotic area were not observed. Special stains for organisms were negative. By immunohistochemical analysis, the characteristic histiocytes were positive for S-100 and CD68 and negative for CD1a. Based on clinical presentations and histological findings, a final histological diagnosis of primary RDD in spinal cord was made according to the criteria of WHO classification. The patient did not receive chemotherapy and radiotherapy, and attended follow-up for 12 months, without any neurological deficit or signs of recurrence. Conclusions Isolated intraspinal RDD is rare. The definite diagnosis of this lesion should be made under the microscopical examination because the preoperative radiological appearance of the lesion does not differ from other lesions occurring in spinal cord, such as inflammatory pseudotumor, meningioma and lymphoma. Although good prognosis is obtained from gross total resection in most of reported patients with this lesion, recurrence could be found in individual cases, and chemotherapy and (or) radiotherapy had been applied for a few cases with multiple organs involvement to control the progression of lesion. Due to the rarity of its site, the strictly differential diagnosis should be made when the isolated RDD is encountered in spinal cord. doi: 10.3969/j.issn.1672-6731.2014.04.011

Retropharyngeal phlegmon in Rosai Dorfman disease

Rosai-Dorfman disease (RDD), originally described as sinus histiocytosis with massive lymphadenopathy, is a rare histiocytic proliferative disorder with a distinctive microscopic appearance. Patients usually present with massive cervical lymphadenopathy. However other nodal sites (mediastinal and inguinal) are also frequently involved. The retro-peritoneum is an infrequent site of involvement. This uncommon entity should also be included in the list of differential diagnoses of diffuse and massive lymphadenopathy, which includes includes infectious/granulomatous conditions, lymphomatous and metastatic disease.

Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive lymphadenopathy, is a rare, benign clinical entity of unknown cause. RDD is characterised by the overproduction and accumulation of histiocytes, primarily in the lymph nodes, although it may affect every organ and system. It predominantly affects children and young adults. Typically, patients are in good general condition, with massive cervical lymphadenopathy and fever. In about 40% of cases extranodal localisation of RDD is diagnosed. In laboratory tests the most common abnormalities are increased erythrocyte sedimentation rate (ESR), leukocytosis with neutrophilia, normocytic anaemia, and hypergammaglobulinaemia. Histopathological examination remains the mainstay of diagnosis – lymph nodes have massive sinusoidal dilation, containing histiocytes positive for S-100 and CD68, and negative for CD1a. Most patients do not require treatment as spontaneous remissions are observed. We present a brief review of the literature and the case of a six-year-old boy with cervical lymphadenopathy diagnosed with RDD. So far, the patient has not required systemic treatment and has been kept under observation.

Long-Term Outcome of Spinal Extranodal Rosai-Dorfman Disease: A Report of Two Cases and Systematic Review

SUMMARY Rosai-Dorfman disease (RDD), or sinus histiocytosis with massive cervical lymphadenopathy, occurs mostly in children and young adults. RDD extremely rarely affects the infratentorial region, without involvement of other anatomical structures. We report a case of a 36-year old woman with isolated RDD in the cerebellum. Clinical features were presented with a neocerebellar syndrome. Brain MRI showed a lesion with varying density, located in the left cerebellar hemisphere. No extracranial lesions were detected, The patient was operated on, and recovery was full. Microscopically, the process presented with lymphoplasmatic and multinucleate histiocytic infiltration. The prognosis is good. Rosai-Dorfman disease (RDD), also known as sinus histiocytosis with massive cervical lymphadenopathy is a very rare condiction (probably less than 1000 cases reported in the literature) of unknown etiology. It commonly presents as massive, painless, bilateral lymph node enlargement in the neck with fever. Most cases occur in the first or second decade of life and have a predilection for blacks. It was first described by Rosai and Dorfman in 1969. They reported 4 cases of a disorder diagnosed as „malignant reticuloendotheliosis”. The disease can involve lymph nodes (nodular form) or the respiratory system , nasal sinuses, eye-orbits, bones skin (extranodular form). Nodular cases have been reported, with extranodular localizations . Intracranial localization of the disease without involvement of other sites is a very rare. Clinical presentation in 90% of the cases is lymphadenopathy, but there are cases where it can be asymptomatic, painful, anemia (normo- or hypochromic microcytosis) with antibodies against erythrocytes (polyclonal hypergamaglobulinemia) was registred in 90% of the cases. Diagnosis is based on histological investigations. Lymphoplasmatic cells are differentiated, though not atypical in shape. The cytoplasm of some histiocytes is pale and eosinophylic, or yeasty. Electron microscopy reveals phylopods protruding from membranes of all histiocytes. The abovementioned facts prove that diagnosis based on clinical features alone is impossible.

Sinus histiocytosis with massive lymphadenopathy (SHML) is generally characterized by severe illness and massive cervical lymphadenopathy. Sinus histiocytosis limited to the skin is now recognized as a rare distinct entity called cutaneous Rosai-Dorfman disease (CRD). We report CRD in a 57-year-old male patient presenting with disseminated firm red-brown papules. Histology revealed a dermal infiltrate with CD1a-, CD68+, S100+ multinuclear giant cells. Some of these histiocytes showed hemophagocytosis (emperipolesis). We achieved a good therapeutic result with topical clobetasol propionate.

ObjectivesTo compare neuroimaging characteristics of three types of histiocytoses, namely Langerhans cell histiocytosis (LCH), Erdheim-Chester disease (ECD), and Rosai-Dorfman disease (RDD), with central nervous system (CNS) involvement.MethodsA total of 121 adult patients with histiocytoses (77 LCH, 37 ECD, and 7 RDD) and CNS involvement were retrospectively included. Histiocytoses were diagnosed based on histopathological findings combined with suggestive clinical and imaging features. Brain and dedicated pituitary MRIs were systematically analyzed for tumorous, vascular, degenerative lesions, sinus, and orbital involvement and for hypothalamic pituitary axis involvement.ResultsEndocrine disorders, including diabetes insipidus and central hypogonadism, were more common in LCH patients than in ECD and RDD patients (p < 0.001). In LCH, tumorous lesions were mostly solitary (85.7%), located in the hypothalamic pituitary region (92.9%), and without peritumoral edema (92.9%), while in ECD and RDD, tumorous lesions were often multiple (ECD: 81.3%, RDD: 85.7%), their distribution was more widespread with meninges mostly involved (ECD: 75%, RDD: 71.4%), and they most likely presented with peritumoral edema (ECD: 50%, RDD: 57.1%; all p ≤ 0.020). Vascular involvement was an exclusive imaging characteristic of ECD (17.2%), which was not observed in LCH or RDD; this was also associated with a higher risk of death (p = 0.013, hazard ratio = 11.09).ConclusionThe typical characteristic of adult CNS-LCH was endocrine disorders with radiological findings limited to the hypothalamic pituitary axis. The pattern of multiple tumorous lesions with predominant involvement of meninges was the main manifestation of CNS-ECD and CNS-RDD, while vascular involvement was pathognomonic for ECD and associated with poor prognosis.Clinical relevance statementInvolvement of the hypothalamic-pituitary axis is the typical imaging characteristic of Langerhans cell histiocytosis. Multiple tumorous lesions, predominantly involving but not limited to meninges, occur in most Erdheim-Chester disease and Rosai-Dorfman disease patients. Vascular involvement occurs only in Erdheim-Chester disease patients.Key Points• The different distribution patterns of brain tumorous lesions can help differentiate among LCH, ECD, and RDD.• Vascular involvement was an exclusive imaging finding of ECD and was associated with high mortality.• Some cases with atypical imaging manifestations were reported to further expand the knowledge on these diseases.

Rosai-Dorfman disease (RDD), sometimes known as sinus histiocytosis with massive lymphadenopathy, is a rare histiocytic disorder that most commonly presents as painless, massive cervical lymphadenopathy in young adults. Extranodal disease can occur in up to 40% of patients but primary involvement of bone is rare. We present two cases of primary RDD of bone: one case of multifocal osseous RDD presenting as a painful lesion in the elbow, and one case of a solitary osseous lesion presenting as a painful lesion in the wrist.

An unusual diagnosis of a dural based intracranial lesion