Unicentric Castlemans disease involving central nervous system – plasma cell type: A rare entity.

Castleman’s disease (CD) is a rare disease primarily involving lymph nodes and lymphatic system. Other synonyms used for the Casleman's disease are giant lymph node hyperplasia, and angio- follicular lymph node hyperplasia (AFH). CD is not a true cancer and is a lympho-proliferative disorder which means there is an abnormal overgrowth of cells of the lymphatic system that is similar in many ways to lymphomas (cancers of lymph nodes). Even though CD is not officially a true cancer, one form of this disease (known as multi-centric Castleman’s disease) acts very much like lymphoma. In fact we can call it as premalignant condition as many people with this disease eventually develop lymphomas. Multi-centric CD is more common in people infected with HIV. In recent years, it’s become clear that another virus, known as human herpesvirus-8 (HHV-8) or Kaposi sarcoma herpes virus (KSHV), is often found in the lymph node cells of people with multi-centric CD. In fact, HHV-8 is found in the lymph nodes of nearly all CD patients who are HIV positive. We present a case of unicentric plasma cell type of Castleman' s disease involving central nervous system ( CNS) or brain perenchyam in a young male patient which is a rare occurrence.

Plasma Cell Type of Castleman's Disease Involving Renal Parenchyma and Sinus with Cardiac Tamponade: Case Report and Literature Review

Castleman's disease is an uncommon disorder characterized by benign proliferation of the lymphoid tissue that occurs most commonly in the mediastinum. Although unusual locations and manifestations have been reported, involvement of the renal parenchyma and sinus, and moreover, manifestations as cardiac tamponade are extremely rare. Here, we present a rare case of Castleman's disease in the renal parenchyma and sinus that also accompanied cardiac tamponade.

Castleman's Disease of the Renal Sinus Presenting as a Urothelial Malignancy: A Brief Case Report

Castleman's disease is a rare benign lymphoproliferative disorder that frequently affects lymph nodes of the mediastinal thorax and the neck. It very rarely affects the renal sinus. We report a case of Castleman's disease arising in the renal sinus in a 64-year-old man. The patient visited the hospital with the chief complaint of hematuria. Abdominal computed tomography revealed a homogeneous mass in the sinus of the left kidney, radiologically interpreted as a malignant urothelial tumor. Subsequently, nephroureterectomy was performed, after which microscopic examination of the specimen revealed a diffuse lymphoproliferative lesion with reactive lymphoid follicles of various sizes and prominent plasma cell infiltration of interfollicular spaces, highlighted by immunohistochemical staining for CD138. The lesion was diagnosed as Castleman's disease of the plasma cell type. Although preoperative diagnosis of Castleman's disease is difficult and the incidence is exceedingly rare, it should be considered in the differential diagnosis of renal sinus tumors.

Ileal Mesenteric Castleman's Disease

Castleman's disease is a rare disorder characterized by tumorous masses that may develop in the lymph node tissue throughout the body. Most common location is mediastinum, but it can also affect retroperitoneum, neck, pelvis, and/or axilla. It may exceptionally affect extranodal sites like striated muscle, thoracic wall, lungs, skull, larynx, and/or vulva. The presentation is varied and diagnosis is difficult. There are two main types of Castleman's disease: hyaline-vascular type and plasma cell type. The hyaline vascular type accounts for approximately 90% of the cases. Most individuals exhibit no symptoms of this form of the disorder or they may develop non-cancerous growths in the lymph nodes. The plasma cell type is often associated with fever, weight loss, skin rash, early destruction of red blood cells, leading to unusually low levels of circulating red blood cells (hemolytic anemia), and/ or abnormally increased amounts of certain immune factors in the blood (hypergammaglobulinemia). We here report a case of Castleman's disease of ileal mesentery in 30-years old female patient. Abdominal mass, 4.7x3.6 cm in size, was completely removed from ileal mesentery without complication, and confirmed histologically mesenteric Castleman's disease of the mixed type.

A case of plasma cell type castleman's disease that transformed into a malignant lymphoma / 대한내과학회지

Castleman's disease is a benign disorder characterized by hypervascular lymphoid hyperplasia. The etiology of the disease is still unknown. Although it may occur at the various sites such as peritoneum, retroperitoneum, pelvic lymph node, muscle and lung, it occurs most commonly at the mediastinum. Even though specific pathophysiologic relations are still not clearly determined between two different histologic types -hyaline vascular type and plasma cell type-, there were several previous reports dealing with the plasma cell type transformed or progressed into different types of malignancies such as malignant lymphoma, Kaposi's sarcoma and Hodgkin's disease. We experienced a case of plasma cell type Castleman's disease that transformed into a malignant lymphoma and report this case with review of literatures.

A Case of Plasma Cell Type Giant Lymph Node Hyperplasia (Castleman's Disease) / 대한소아혈액종양학회지

Castleman's disease was first described in 1956 in a group of patients with localized mediastinal lymph node enlargement characterized by hyperplasia of lymphoid follicles and marked capillary proliferation with endothelial hyperplasia. They have been divided into 2 histologic types: the hyaline-vascular type, which was more common and usually asymptomatic, was characterized by small hyaline-vascular follicles and interfollicular capillary proliferation; the plasma-cell type was characterized by large follicles with intervening sheets of plasma cells. Systemic manifestations, such as fever, anemia and hyperglobulinemia, were frequently associated with the plasma cell type. Localized lesions have behaved in a benign fashion, and complete surgical excision has been curative. But recent years, reports have described a multicentric variety with severe systemic manifestations, exorable clinical course and poor outcome. Although Castleman's disease has been described at all ages, the disease is rare in childhood. This paper describes a case of plasma cell type Castleman's disease in a 12-year-old boy and review of the literature. We conclude that the Castleman's disease must be considered in childhood lymphadenopathy and the clinicians should be mindful of the malignant potential of the disease and their possible multicentricity. Appropriate treatment plan, close follow-up and periodic surveillance are necessary.

Giatn Lymph Node Hyperplasia : Analysis of 17 Cases with Special Reference to 5 Cases of Plasma Cell Type

This report describes the pathologic features of 17 cases of Castleman's disease, examined at the Department of Pathology, Seoul National University Hospital during a period from 1973 to 1989. The lesions in 12 cases were hyaline-vascular type and the remainders plasma cell type. The pathologic features favoring the plasma cell type over the hyaline vascular type included a sufficient number to large-sized follicles. However, a histologic overlapping between two types was present. In the hyaline vascular type the age of the patients ranged from 7 to 76 years and they appeared to be no particular sex predominence. The majority of the lesions occurred in the neck and within the chest. Almost all cases presented with a solitary mass except three cases. Neither conventional symptoms nor systemic manifestations were associated. The plasma cell type was characterized by presentation of constitutional symptoms, involvement of intra abdominal and inguinal lymphnodes, in association with unusual clinicopathologic features including IgA nephropathy, diabetes mellitus, systemic progressive sclerosis, peripheral neuropathy, and anemia. Immunohistochemical study was performed in three cases of the plasma cell type. Two cases revealed poly-clonal plasma cell infiltration. In a patient with IgA nephropathy, however, serum IgA was increase and a strong immunoreactivity to IgA heavy chain was found. Another case, associated with systemic progressive sclerosis and neuropathy, revealed monoclonal plasma cell infiltration (IgG and lambda light chain). The above results support a possibility that in some of the plasma cell type an altered immune mechanism is involved in its pathogenesis.

Angiofollicular Lymph Node Hyperplasia(=Castleman's Disease): Report of A Case / 대한치료방사선과학회지

Angiofollicular lymph node hyperplasia (AFLNH) with well marginated lymphoid masses, is a rare benign disease of unknown etiology. The majority of the disease develop intrathoracically. Histologically this disease can be divided into the hyaline-vascular and the plasma cell types with the hyaline-vascular type prevailing. The plasma cell variant has been associated with nephritic syndrome, anemia, growth failure, fever, hyperglobulinemia, peripheral neuropathy, and hypoalbuminemia. Surgical resection is known to be treatment of choice in most cases, and radiotherapy is reserved for advanced, unresectable lesions. We report a complete remission of AFLNH in a case treated by surgical excision followed by irradiation.