A Rare Coexistence of Retroperitoneal Pararenal Castleman's Disease with Focal Nodular Hyperplasia

Castleman's disease is a distinct form of lymph node hyperplasia divided into a solitary and a multicentric type. The solitary type occurs most commonly in the mediastinum and is usually asymptomatic. We present a patient with Castleman's disease of the hyaline-vascular solitary type located in the retroperitoneum. The patient was a 38-year-old male, who presented to our hospital with fever. The imaging workup revealed a retroperitoneal mass, measuring 4 × 6 cm, located lateral to the aorta, inferior to the left renal artery and vein, and posterior to the left testicular vein. At workup, a solid hepatic lesion, 3 cm in diameter, located in the left lobe of the liver, segment IV, was also identified. Both lesions were surgically excised. The retroperitoneal tumor had the features of angiofollicular hyperplasia (Castleman's disease), hyaline-vascular type, whereas a diagnosis of focal nodular hyperplasia was made for the hepatic lesion. The patient is well at fourty months followup postoperatively. Surgical excision is the treatment of choice for unifocal Castleman's disease.

An Extra Ordinary Respone of Castleman’s Disease to Rituximab.

Castleman’s disease is a very rare non-neoplastic lymphoproliferative disorder of unknown cause that is diagnosed on a pathological basis. It is often associated with autoimmune diseases, and diagnosed in the context of human immunodeficiency virus (HIV) positive patients. No standard treatment has been developed for this rare disease. CASE REPORT: This 54-year-old male presented with nausea and vomiting. He has a past medical history of hypertension and chronic glomerulonephritis. His laboratory results showed acute renal failure. Hemodialysis was started prior to further work up. A chest x-ray showed bilateral hilar lymphadenopathy that was later confirmed by computerized tomography (CT) of the chest. A mediastinoscopy and mediastinotomy were performed. Mediastinal lymph node biopsy showed findings consistent with Castleman’s disease including atrophic follicles with hyalinized vessels. Remission was obtained for one year by prednisone and cyclophosphamide, but the patient relapsed with multiple lymphadenopathy. Biopsy confirmed the recurrence of Castleman’s disease. Rituximab was added to cyclophosphamide as a treatment regimen and for more than two years the patient has done extremely well. His kidney function returned to his baseline, hemodialysis was stopped, and the patient has long-term remission on CT scan and postron emission tomography (PET). DISCUSSION: Castleman’s disease is lymphadenopathy of unknown cause. About 400 patients have been reported to date. It is diagnosed microscopically by lymphadenopathy with angiofollicular hyperplasia and plasma cell infiltration. It has a rapid progressive and often fatal course. Traditional treatment is chemotherapy. A new trend in the treatment of Castleman’s disease is Rituximab which is a monoclonal antibody that showed success in HIV positive patients with Castleman’s disease. We believe that this is the first reported case of long-term remission of Castleman’s disease in an HIV-negative patient treated with Rituximab and chemotherapy.

Transitional type of Castleman's disease manifested as the POEMS syndrome

Background. Castleman?s disease is an atypical lymphoproliferative disorder characterized by angiofollicular hyperplasia of lymph nodes. Histologically it can be classified into a hyaline-vascular type, plasma-cell type, and transitional (mixed-cell) type, while clinically localized type has been classified as unicentric, or generalized (multicentric) form of the disease. Case report. This paper presents a 21 years old male patient with multicentric Castleman?s disease, a transitional (mixed-cell) type. The disease was manifested by fever, generalized enlargement of peripherial lymph nodes, breast glands enlargement, hyperemia of the face, and weakness of the lower extremities. We found mediastinal lymphadenopathy, pleural and pericardial effusions, sensorimotor peripherial neuropathy and polyclonal hypergammaglobulinemia. The simultaneous presence of these manifestations of the disease (sensomotor peripheral neuropathy, lymphadenopathy, effusions endocrinopathy, polyclonal gammaglobulinemia and skin changes) is indentified as POEMS syndrome. The diagnosis of Castleman?s disease was based on the results of histopathologic analysis of mediastinal lymph node biopsies after thoracotomy. The patient was treated with corticosteroids (prednisone 80 mg daily for 2 weeks followed by 60 mg daily). A partial response was achieved after 4 months of treatment. Conclusion. A transitional type of multicentric Castleman?s disease may be present itself as POEMS syndrome. The effect of corticosteroid therapy in this form of the disease is unpredictable.

Angiofollicular Lymph Node Hyperplasia

A case of angiofollicular hyperplasia of a lymph node on the right pulmonary hilus is described. The mass 5 cm large was strictly adherent to the pulmonary arteria; its cure required pneumonectomy.