Castleman's disease is a rare disease characterized by lymph node hyperplasia. Although Castleman's disease can occur wherever lymphoid tissue is found, it rarely appears in the abdominal cavity, and is especially rare adjacent to the liver. Here, we report a rare case of Castleman's disease in the portal area that mimicked a hepatocellular carcinoma (HCC) in a chronic hepatitis B patient. A 40 year-old woman with chronic hepatitis B presented with right upper quadrant discomfort. Computed tomography and magnetic resonance imaging results showed a 2.2 cm-sized, exophytic hypervascular mass in the portal area. HCC was suspected. However, histologic examination revealed Castleman's disease. We suggest that Castleman's disease should be included as a rare differential diagnosis of a hypervascular mass in the portal area, even in patients with chronic hepatitis B.
Adult ; Carcinoma, Hepatocellular/diagnosis ; Diagnosis, Differential ; Female ; Giant Lymph Node Hyperplasia/complications/*diagnosis/pathology ; Hepatitis B, Chronic/complications/diagnosis ; Humans ; Immunohistochemistry ; Liver Neoplasms/diagnosis ; Magnetic Resonance Imaging ; Receptors, Complement 3d/metabolism ; Tomography, X-Ray Computed

Kaposi sarcoma herpes virus (KSHV), also known as human herpesvirus-8, plays an important role in the pathogenesis of Kaposi sarcoma (KS), multicentric Castleman disease (MCD) of the plasma cell type, and primary effusion lymphoma. KSHV is rarely associated with the hemophagocytic syndrome (HPS), but when it does occur, it most occurs in immunocompromised patients. We report herein an unusual case of KSHV-associated HPS in an immunocompetent patient. A previously healthy 62-yr-old male was referred for evaluation of leukocytopenia and multiple lymphadenopathies. After a lymph node biopsy, he was diagnosed with MCD of the plasma cell type. KSHV DNA was detected in the lymph node tissue by polymerase chain reaction. Following a short-term response of the leukocytopenia to prednisolone, mental change, left side weakness, fever, thrombocytopenia, hemolytic anemia, and renal failure developed. Despite intravenous immunoglobulin therapy and plasmapheresis, he expired. The lymph nodes were infiltrated by hemophagocytic histiocytes in the sinuses. Pulmonary nodules and gastric erosions were shown to be KS. KSHV DNA was detected in the stomach, lung, and liver. This is the first case of multiple KSHV associated diseases including MCD and KS with KSHV-associated hemophagocytic syndrome in an HIV-negative, non-transplant, immunocompetent patient.
Autopsy ; Giant Lymph Node Hyperplasia/complications/*diagnosis/pathology ; HIV Seronegativity ; Herpesviridae Infections/*diagnosis ; Herpesvirus 8, Human/*isolation & purification ; Humans ; Immunocompetence ; Lymphohistiocytosis, Hemophagocytic/*diagnosis/etiology ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Polymerase Chain Reaction ; Sarcoma, Kaposi/complications/*diagnosis

Follicular dendritic cell (FDC) sarcoma is an extremely rare malignant neoplasm arising from FDCs. The exact origin of FDCs remains unclear; both a hematopoietic lineage origin and a stromal cell derivation have been proposed. Proliferation of FDCs can lead to benign reactive lesions or generate neoplastic conditions. The lesions are most commonly found in lymph nodes and usually involve the head and neck area. Castleman's disease is a rare non-neoplasitic lymphoproliferative disorder. Rare cases of hyaline-vascular Castleman's disease have been associated with FDC sarcoma, but a clonal relationship has not been convincingly demonstrated. A pathway toward tumor evolution, beginning with hyperplasia and dysplasia of FDCs, has been proposed. Despite this known association between Castleman's disease and FDC sarcoma, there have only been few reported cases of sarcoma arising as a complication of pre-existing Castleman's disease, especially in abdominal lesions. We describe here a 51-year-old female with an FDC sarcoma arising from unicentric, hyaline-vascular type Castleman's disease in an intra-abdominal mass. Pathologically, the lesion showed a series of changes during the process of transformation from Castleman's disease to FDC sarcoma.
Abdomen/ultrasonography ; Abdominal Neoplasms/*diagnosis/etiology/pathology ; Dendritic Cell Sarcoma, Follicular/*diagnosis/etiology/pathology ; Female ; Giant Lymph Node Hyperplasia/complications/*diagnosis ; Humans ; Middle Aged ; Positron-Emission Tomography ; Tomography, X-Ray Computed

Castleman disease (CD) is a rare lymphoproliferative disorder of unknown etiology with different clinical manifestations. A previous healthy 50 year-old man was hospitalized for right upper quadrant (RUQ) abdominal pain. He had jaundice and a 1 cm-sized lymph node in the right supraclavicular area. Pancreas and biliary computed tomography (CT) showed masses at the right renal hilum and peripancreatic areas. Positron emission tomography (PET) showed widespread systemic lymphadenopathy. Excisional biopsy of the right supraclavicular node revealed a hyaline vascular variant of CD. Corticosteroid therapy was started and the extent of disease decreased. We here report a case of multicentric CD, the hyaline vascular variant, presenting with jaundice, diagnosed by excisional biopsy and successfully treated with corticosteroids.
Abdominal Pain/etiology ; Adrenal Cortex Hormones/therapeutic use ; Biopsy ; Giant Lymph Node Hyperplasia/*diagnosis/drug therapy/pathology ; Humans ; Jaundice/*complications ; Male ; Middle Aged ; Positron-Emission Tomography ; Tomography, X-Ray Computed ; Vascular Diseases/*diagnosis/pathology