1/14. Rosai-Dorfman disease presenting as a pituitary tumour.A 45-year-old woman had pyrexia, headaches, collapse and hyponatraemia. Intracerebral abscess, bacterial meningitis and subarachnoid haemorrhage were excluded. She was given intravenous antibiotics and gradually recovered. One month later she was readmitted with diplopia, headache and vomiting. serum sodium was low (107 mmol/l) and a diagnosis of inappropriate ADH secretion was made. MRI scan showed a suprasellar tumour arising from the posterior pituitary gland. A skin rash gradually faded. serum cortisol, prolactin, gonadotrophins and thyroid hormone levels were low. A pituitary tumour was removed trans-sphenoidally, she had external pituitary radiotherapy, and replacement hydrocortisone and thyroxine. She was well for 12 months when she developed progressive weakness and numbness of both legs. Examination suggested spinal cord compression at the level of T2 where MRI scanning showed an intradural enhancing mass. This spinal tumour was removed and her neurological symptoms disappeared. Nine months after this she developed facial pain and nasal obstruction. CT scan showed tumour growth into the sphenoid sinus and nasal cavities. A right Cauldwell-Luc operation was done and residual tumour in the nasal passages was treated by fractionated external radiotherapy and prednisolone. Histological examination of the specimens from pituitary, spinal mass, and nasal sinuses showed Rosai-Dorfman disease, a rare entity characterized by histiocytic proliferation, emperipolesis (lymphophagocytosis) and lymphadenopathy. aged 48 she developed cranial diabetes insipidus. Although Rosai-Dorfman syndrome is rare, it is being reported with increasing frequency, and should be borne in mind as a possible cause of a pituitary tumour.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
2/14. Cold thyroid nodule as the sole manifestation of Rosai-Dorfman disease with mild lymphadenopathy, coexisting with chronic autoimmune thyroiditis.A case of thyroid Rosai-Dorfman disease (RDD) without apparent lymphadenopathy in a 49-year-old woman with underlying euthyroid chronic autoimmune thyroiditis, as indicated by high thyroid autoantibodies titers, is presented. The initial presentation was that of a cold, hypoechogenic nodule of left thyroid lobe which increased in size during the two years of follow up, together with new ultrasonographic findings of the right lobe. No biochemical abnormalities were found apart from mild hypercalcemia. A near total thyroidectomy was performed. Histologically, the left lobe nodule as well as the right lobe lesions consisted of typical RDD cellular population, with the pathognomonic phenomenon of emperipolesis. Infiltration to the periphery of the gland was observed and three adjacent lymph nodes were also involved. The uninvolved thyroid parenchyma showed changes compatible with chronic autoimmune thyroiditis. No other localizations or systemic manifestations of RDD were revealed. Normocalcemia was restored promptly and the patient remains free of clinically overt disease one year post-operatively.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
3/14. Rosai-Dorfman disease presenting as bilateral lacrimal gland enlargement.PURPOSE: To report a patient with bilateral lacrimal gland enlargement as the initial manifestation of Rosai--Dorfman disease. methods: Case report. RESULTS: A 14-year-old female presented with left lacrimal gland enlargement followed by right lacrimal gland enlargement 11 weeks later. Bilateral lacrimal gland biopsies were performed, and histopathologic examination revealed the diagnosis of Rosai--Dorfman disease. CONCLUSION: patients with Rosai--Dorfman disease may present with bilateral lacrimal gland swelling in the absence of lymphadenopathy. Rosai--Dorfman disease should be considered in the differential diagnosis of bilateral lacrimal gland enlargement.- - - - - - - - - - ranking = 10keywords = gland (Clic here for more details about this article) |
4/14. Rosai-Dorfman disease of the parotid gland: cytologic and histopathologic findings with immunohistochemical correlation.Rosai-Dorfman disease is a rare histiocytic proliferative disorder of unknown origin and a distinct clinicopathologic entity also known as sinus histiocytosis with massive lymphadenopathy. The disease can involve extranodal tissues and rarely can present as salivary gland enlargement without significant lymphadenopathy. Involvement of the extranodal head and neck sites appears to be more common in patients with immunologic abnormalities. The disease was first described in 4 patients in 1969, and with later descriptions of more patients, the disease was established as a well-defined clinicopathologic entity. The characteristic pathologic feature of this disease is proliferation of distinctive histiocytic cells that demonstrate emperipolesis in the background of a mixed inflammatory infiltrate, consisting of moderately abundant plasma cells and lymphocytes. Fine-needle aspiration biopsy can be helpful in establishing the correct diagnosis, since surgical treatment is not necessary other than obtaining tissue for definitive diagnosis. We describe cytologic, histopathologic, and immunohistochemical features of a case of Rosai-Dorfman disease that involved a major salivary gland without significant lymphadenopathy in a 48-year-old patient with systemic lupus erythematosus. We also briefly discuss possible causes and pathogenesis and review the literature.- - - - - - - - - - ranking = 6keywords = gland (Clic here for more details about this article) |
5/14. A case of Rosai-Dorfman disease involving the lacrimal gland in an elderly patient.Rosai-Dorfman disease (sinus histiocytosis with massive lymphadenopathy) is a rare disease characterised by benign proliferation of histiocytes, with painless lymph node enlargement and frequent extranodal involvement. Orbital involvement occurs in 11% of cases and onset of the disease is typically in the first two decades of life. A benign course is usual, but in some cases blindness or fatality may result. We present a case with late onset at age 78 years and lacrimal gland involvement, and review the literature on this disease.- - - - - - - - - - ranking = 5keywords = gland (Clic here for more details about this article) |
6/14. Orbital sinus histiocytosis (Rosai-Dorfman disease): a lacrimal gland involvement.We report the clinical and histopathological features of 2 cases of orbital Rosai-Dorfman disease with lacrimal gland involvement but no lymphadenopathy or cutaneous involvement. The first case was a 7-year-old boy, who was referred to the ophthalmology department with a proptosis in the left eye. The second case involved a 57-year-old African man, who developed oedema of the right upper eyelid over a 4-month period. Ocular involvement in Rosai-Dorfman disease is rare (10%). The diagnosis is histological in combination with immunohistology and is often delicate, especially in extranodal localizations. Histopathologically, normal gland morphology was altered by fibrosis and inflammatory cells. To our knowledge, this is the first report on lacrimal involvement in incomplete, thus uncommon sinus histiocytosis with massive lymphadenopathy.- - - - - - - - - - ranking = 6keywords = gland (Clic here for more details about this article) |
7/14. Lacrimal location of sinus histiocytosis (Rosai-Dorfman-Destombes disease).A 7-year-old West African male patient presented with recurrence of multiple cervical lymphadenopathy. Cervical node biopsy disclosed a sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman-Destombes disease). During follow-up, the patient developed a left orbital mass corresponding to an enlarged lacrimal gland shown by CT. The histologic features of the gland were consistent with the diagnosis, and with steroid treatment, the gland decreased in size. Although rare, the diagnosis of sinus histiocytosis with massive lymphadenopathy has to be considered in cases of lacrimal gland enlargement.- - - - - - - - - - ranking = 4keywords = gland (Clic here for more details about this article) |
8/14. Rosai-Dorfman disease in a patient with systemic lupus erythematosus.Rosai-Dorfman disease (RDD), also known as sinus histocytosis with massive lymphadenopathy, is a clinically benign, frequently chronic, painless lymphadenopathy. It can also involve extranodal sites. We describe a 37-year-old man with a recent diagnosis of systemic lupus erythematosus and antiphospholipid antibody syndrome who had lacrimal gland and orbital involvement and nodal and extranodal sites with RDD.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
9/14. 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.- - - - - - - - - - ranking = 11keywords = gland (Clic here for more details about this article) |
10/14. Sinus histiocytosis with massive lymphadenopathy and subglottic stenosis: a case report.An 18 year-old woman presented with massive cervical lymphadenopathy. She also had bilateral parotid gland enlargement, and later upper airway obstruction from subglottic mass compression. Surgical excision of lymph nodes and bilateral total parotidectomy with facial nerve preservation were selected. Direct laryngoscopy and CO2 laser excision were used to remove the subglottic mass. Airway was secured by tracheostomy and after that it was removed. Histologic finding was Sinus histiocytosis with massive lymphadenopathy (SHML) that involved the subglottic. SHML is very rare, probably less than 1000 cases reported in the literature and this is the 6th case in the literature that involved the subglottic. There are no standard treatment modalities. The treatment is comprised of surgery, steroid, chemotherapy and local control. In the present case the authors chose surgery, steroid and local control of disease in the subglottic by using CO2 laser excision. Now, the disease is under control.- - - - - - - - - - ranking = 1keywords = gland (Clic here for more details about this article) |
| | Next -> |