Massive Cervical Lymphadenopathy Research Articles

Rosai‐dorfman disease with extranodal involvement

Rosai-Dorfman disease is a rare condition of marrow hematopoietic stem-cell origin. Patients can show extranodal involvement as well as lymphatic involvement, but only about 5% of extranodal cases involve intracranial lesions. A 53-year-old male was admitted to our hospital with bilateral cervical lymphadenopathy. Intracranial tumors and bone lesions were also detected. Cervical lymph node biopsy and intracranial tumor resection were performed, and histopathological examination revealed Rosai-Dorfman disease. The patient showed good clinical course without significant enlargement of the tumor. This study describes the case of a patient with Rosai-Dorfman disease presenting with massive cervical lymphadenopathy mimicking malignant neoplasm.

Orbital Rosai–Dorfman Disease in a fifty-eight years old woman

Rosai-Dorfman disease (RDD) is rare and characterized by histiocytic proliferation and massive cervical lymphadenopathy. About 40% of patients have extra-nodal involvement. Opthalmic involvement is seen in 10% of cases. A case of orbital Rosai Dorfman disease in a 58 years old woman is presented here, who was misdiagnosed as orbital inflammatory disease initially. The patient did not respond to a course of oral prednisolone. Then complete surgical excision of the mass was performed and the histopathological examination was consistent with a diagnosis of RDD.

Rosai-Dorfman Disease with Isolated Laryngeal Involvement

Rosai-Dorfman disease is a rare histiocyte disorder that is typically characterized by massive cervical lymphadenopathy. Isolated extranodal involvement is uncommon, and isolated laryngeal involvement is extremely rare. We report an unusual case of Rosai-Dorfman disease with isolated laryngeal involvement that led to recurrent dysphonia and airway obstruction. We discuss the challenges we faced in reaching a correct pathologic diagnosis and in deciding on an appropriate treatment regimen. Based on our experience, we believe that Rosai-Dorfman disease should be considered as a differential diagnosis in patients who present with a recurrent inflammatory (histiocytic) mass lesion of the larynx.

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.

Nasopharyngeal Tuberculosis Presenting as Massive Cervical Lymphadenopathy and Hearing Loss

Lymphadenitis is the most common form of tuberculosis in the head and neck region, but it can be seen in the other areas of the head and neck. Nasopharyngeal tuberculosis is a rare condition without pulmonary and systemic involvement. The majority of patients present with neck mass. A 17-year-old female patient admitted to our outpatient clinic with the complaints of swelling on both sides of the neck and hearing loss. The endoscopic examination revealed a nasopharyngeal mass, and biopsies were taken from the mass. The result of pathologic examination was reported as caseating granulomatous inflammation compatible with tuberculosis. In this report, a nasopharyngeal tuberculosis case associated with massive cervical lymphadenopathy was reported, and etiopathogenesis and treatment were also discussed.

Sonographic Manifestation of Thyroid's Rosai-Dorfman Disease

Rosai-Dorfman disease (RDD) is a rare and benign histiocytic proliferative disorder of unknown etiology. It can affect all age groups, particularly young adults. The typical clinical manifestation is painless, bilateral, massive cervical lymphadenopathy with or without extra nodal involvement, along with fever, weight loss, and night sweats. However, thyroid involvement is very uncommon. The ultrasonographic manifestations of RDD are rarely reported. A case of RDD involving the thyroid that was recorded in our hospital PACS system during 2002–2010 is reported here, which was initially misdiagnosed as non-Hodgkin’s lymphoma (NHL). In our case, ultrasonography revealed diffuse enlargement of whole thyroid glands with heterogeneous hypoechogenicity. Areas of linear hyperechogenicity were noted on the sonograms with multiple enlarged nodes over the cervical, supraclavicular, and submandibular areas. Though not specific, in order to avoid unnecessary surgery or a total thyroidectomy, ultrasonography and ultrasonography-guided core needle biopsy should be performed to establish an accurate diagnosis.

Multicentric sinus histicytosis (Rosai-Dorfman Disease): Computed tomography, magnetic resonance imaging findings

Sinus histicytosis (Rosai–Dorfman disease) is a rare, benign granulomatous disease that typically presents with massive cervical lymphadenopathy. Extranodal involvement along with concomitant nodal disease occurs in about 43% of cases. We present a unique case of a 30-year-old male with cervical, retroperitoneal lymphadenopathy, associated with renal and pituitary sellar masses.

Bilateral Hilar and Mediastinal Lymphadenopathy

A 41-year-old man presented to the ER with a 9-month history of cough, exertional dyspnea, nocturnal diaphoresis, and weight loss. The physical examination revealed painless, massive cervical and supraclavicular lymphadenopathy.

Beschwerdefreie derbe, rotbraune Papeln

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.

Destombes–Rosai Dorfman Disease: A Rare Case Report

Destombes Rosai Dorfman disease and sinus histocytosis with massive lymphadenopathy is a rare benign idiopathic proliferative disease of phagocytic histiocytes. Approximately 80% of patient present with painless massive cervical lymphadenopathy. It rarely involves other sites for example CNS, eyes, upper respiratory tract and skin. A case of Destombes Rosai Dorfman disease is reported with history of gradual protrusion of left eye since many years. The histological features were consistent with the diagnosis.

Epitheloid Haemangioendothelioma Liver Coexisting with Retroperitoneal Rosai Dorfman Disease– A Rare Case Report

Epitheloid hemangioendothelioma is a rare tumor of vascular origin which occurs at sites such as soft tissues liver or lung and has a highly unpredictable malignant potential. It is an intermediate entity between well-differentiated hemangioma and angiosarcoma. Neoplastic cells express factor VIII related antigen CD31 and CD34. Hepatic epitheloid hemangioendothelioma is a rare vascular tumor. The clinical course is unpredictable and different treatment modalities are offered depending on the patientrsquos conditions. Rosai-Dorfman disease 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.We report a case of a 41 year old man presented with recurrent pain abdomen diagnosed as epitheloid hemangioendothelioma liver coexisting with retroperitoneal Rosai Dorfman disease. He was managed symptomatically without any modalities of treatment with regular follow up every month since one year. This case highlights the diagnostic dilemma of retroperitoneal Rosai Dorfman disease which mimicked metastasis from hepatic neoplasm and its rare association with epitheloid hemangioendothelioma liver

Orbital Rosai-Dorfman Disease in a five year-old boy

Rosai–Dorfman disease (RDD) is characterized by histiocytic proliferation and massive cervical lymphadenopathy, although some patients have extra-nodal involvement. We report a case of extranodal RDD in a five-year-old child, initially misdiagnosed as orbital inflammatory disease and treated with oral steroids. A subsequent orbital biopsy three years later confirmed the diagnosis of Rosai Dorfman disease.

Cutaneous Colesional Acquired Immunodeficiency Syndrome Associated Kaposi Sarcoma and Cryptococcosis

The clinicopathologic features of 4 AIDS patients with cutaneous colesional Kaposi sarcoma (KS) and cryptococcosis, a rare phenomenon, are described. Biopsies from 3 patients who were highly active antiretroviral therapy (HAART)-naive demonstrated predominant KS with a conspicuous spindle cell component and small aggregates of cryptococcal yeasts in 2 biopsies and predominant gelatinous cryptococcosis with attenuated KS spindle cells in 1 biopsy. One patient was HAART exposed. He had childhood pulmonary tuberculosis, was treated for disseminated cutaneous cryptococcosis 18 months earlier and presented with cutaneous lesions, odynophagia and massive cervical lymphadenopathy in the eighth week of HAART, after achieving viral suppression and a CD4 cell increase from 28 to 184 cells/μL. His skin biopsy demonstrated a dense lymphoplasmacytic infiltrate, neutrophils, and granulomas with admixed aggregates and single Cryptococcus neoformans and focal aggregation of human herpes virus 8-immunopositive spindle cells. Acid fast bacilli were not identified and mycobacterial molecular studies were negative. The features were compatible with cutaneous cryptococcal immune reconstitution inflammatory syndrome. His nodal and oropharyngeal biopsies demonstrated dense mixed, including granulomatous, inflammation with few cryptococcal yeasts and acid fast bacilli, confirmed to be Mycobacterium tuberculosis on polymerase chain reaction testing, without KS. These features were also compatible with immune reconstitution inflammatory syndrome, but the exact role of each infection in the extracutaneous sites was unconfirmed. Colesional KS and cryptococcosis served as the sentinel lesion of AIDS in 3 patients and of immune reconstitution inflammatory syndrome in 1 patient.

Reconstruction of a Patient With Rosai-Dorfman Disease Using Ramus Graft and Osseointegrated Implants: A Case Report

R osai-Dorfman disease (RDD), or sinus histiocytosis with massive lymphadenopathy, is a rare and benign, frequently self-limited histiocytic disorder of unknown etiology. The most common clinical findings are broad painless lymphadenopathy accompanied by fever, weight loss, tonsillitis, rhinorrhea, nasal obstruction, and sometimes hepatosplenomegaly. Laboratory findings in patients with RDD include an elevated erythrocyte sedimentation rate, anemia, leukocytosis and polyclonal hypergammaglobulinemia. RDD classically presents with painless massive cervical lymphadenopathy, which is caused by accumulation of lymphocytes and histiocytes distending the lymph node sinuses. However, the disease may occur at extranodal sites. In most instances, the extranodal sites include the eyes, head and neck, skin, or bone. Mandibular cortical grafts are the gold standard in the restoration of intraoral osseous defects. These grafts do not produce immune reactions and are incorporated by osteoclastic resorption with a short healing period when compared with other methods of osseous repair. Intraoral donor sites include the mandibular ramus, angle of the mandible, mandibular symphysis, and mandibular exostoses. The mandibular ramus provides primarily a dense cortical graft. Other advantages of a mandibular ramus transplant are minimal resorption, maintenance of osseous density, intraoral access, and proximity to the recipient site. Cortical grafts, including the ramus, are reliable donor sites for the reconstruction of the alveolar ridge deformities before dental implant placement. The highest concentrations of promoter proteins (eg, bone morphogenetic proteins) are found in the mandibular cortical grafts. This report presents a case of an isolated RDD involving the mandible as well as its surgical management, preprosthetic reconstruction, and restoration with dental implants.

ROSAI-DORFMAN DESEASE WITH INFRA­TENTORIAL LOCALIZATION: A CASE REPORT

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.

Extranodal Rosai-Dorfman disease of the kidney

Extranodal Rosai-Dorfman disease of the kidney

Rosai-Dorfman Disease Presenting as an Isolated Extranodal Mass of the Carotid Sheath: A Case Report

Rosai-Dorfman disease is a rare, benign granulomatous disease that typically presents with massive cervical lymphadenopathy. In less than 50% of cases, other soft-tissue manifestations may also be found in the head and neck. Rosai-Dorfman disease can be difficult to diagnose because of its rarity and its ability to mimic, both clinically and radiologically, more common diseases such as lymphoma. The histopathologic diagnosis can also be difficult to make, particularly when the disease exhibits extranodal manifestations. We present a case of isolated extranodal Rosai-Dorfman disease involving the carotid sheath, without the typical massive adenopathy.

Massive Cervical and Abdominal Lymphadenopathy Caused by Localized Amyloidosis

Massive Cervical and Abdominal Lymphadenopathy Caused by Localized Amyloidosis

INFANT ANAPLASTIC LYMPHOMA: Case Report and Review of the Literature

Anaplastic large cell lymphoma (ALCL) is a well-known entity, but there are no data on prognosis according to the age of the patient, especially in infants. A 2-month-old girl was admitted with a 2-week history of coughing, fever, and lymphadenopathy. Physical examination revealed mild respiratory distress, an erythematous macular rash on her trunk, massive cervical lymphadenopathy, splenomegaly, and very mild ascites. Chest radiograph showed bilateral pulmonary infiltrates, pleural effusion, and a mediastinal mass. CBC count showed WBC: 172,000/μL (PMN 40%, lymphocytes 47%, monocytes 3%); hemoglobin concentration: 8.7 g/dL; platelets: 390,000/μL. Cervical lymph node biopsy revealed anaplastic lymphoma with positive staining to ALK 1 and TIA 1. Immunophenotypic analysis of peripheral and bone marrow lymphoid cells showed an aberrant T-cell immunophenotype, including expression of CD3, CD45R0+, CD43+, and CD30+. Cytogenetic analysis performed on blood and bone marrow samples demontrated the translocation t(2;5) (p23;q35), and trisomy 47. After leucophoresis, the child received chemotherapy according to the ALCL-99-EICNHL protocol, and was started on corticosteroids and cyclophosphamide, which resulted in marked improvement. After the second course, WBC decreased to 6000/μL without tumor lysis syndrome, but the child developed bacterial and fungal disseminated infections and died of septic shock with multiorgan failure. This report is of a rare case of infant anaplastic lymphoma and excellent response to treatment. Unfortunately, she did succomb to overwhelming infection. More reports of similar cases may determine the cause and prognosis of such children, helping to tailor therapy according to the age of the child and other prognostic factors, especially bone marrow involvement.

Systemic Metastasis Following Hyphema Drainage in an Unsuspected Retinoblastoma

A 6-year-old girl had total hyphema and elevated left intraocular pressure following trivial trauma. B-scan with vector A-scan revealed vitreous opacities consistent with hemorrhage. The drained hyphema did not recur. A left vascular conjunctival mass and massive cervical lymphadenopathy occurred 7 months later. Biopsy revealed extraocular retinoblastoma and lymph node metastasis. Computed tomography showed an intraocular mass with intracranial extension. She died of metastatic disease despite intensive chemotherapy. Retinoblastoma should be suspected in a child with hyphema following trivial trauma.