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1/16. Expression of vascular endothelial growth factor in sera and lymph nodes of the plasma cell type of Castleman's disease.

    To evaluate the possible involvement of vascular endothelial growth factor (VEGF) in the pathogenesis of Castleman's disease, we studied VEGF levels in sera and supernatants of cultured lymph nodes from two patients with the plasma cell type of Castleman's disease, and analysed the expression of VEGF immunohistochemically in the lymph nodes. Clinically, one patient was classified as the localized type and the other as the multicentric type. Histologically, mature plasma cells and hyalinized vessels were prominent in the interfollicular region. The VEGF levels of the sera and the supernatants of cultured lymph nodes of both patients were higher than those of normal controls. VEGF was strongly expressed in plasma cells in the interfollicular region of the lymph nodes of both patients, but rarely in normal lymph nodes. Our results suggest that VEGF may be involved in the marked vascular proliferation in the interfollicular region of the lymph nodes of the plasma cell type of Castleman's disease.
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2/16. Angiofollicular lymph node hyperplasia of the lung simulating carcinoma: pseudolymphoma with inflammatory changes.

    Angiofollicular lymph node hyperplasia of the lung developed in a 45-year-old man who was a heavy smoker. Slow growth of the lesion 3 years after biopsy and the finding of atypical cells in the sputum and bronchial washings strongly suggested a malignant lesion. There were no systemic manifestations other than dyspnea, which may have been related to allergy. The lesion was angiofollicular lymph node hyperplasia of the hyaline-vascular type. Invasion by lymphoid cells of both blood vessels and bronchioles suggested that the pathogenesis was probably infectious or inflammatory; also, the nature of the infiltrate indicated an immune mechanism. The course of the disease, as in other cases reported, has been benign, though a patchy density has developed in contralateral lung 4 years after lobectomy.
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3/16. Preoperative embolization as an adjunct to the operative management of mediastinal Castleman disease.

    Castleman disease usually presents in children as a localized mass with prominent feeding vessels. The mainstay of treatment of Castleman disease is surgical resection; historically, resection is associated with excessive blood loss. These tumors are well known to have large feeding vessels and, thus, are amenable to preoperative arteriography with embolization. The authors present a case of Castleman disease treated with preoperative embolization as an adjunct to operative management.
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4/16. Localized Castleman's disease presenting as a vascular right iliac fossa mass.

    Castleman's disease is a rare lymphoproliferative disorder of unknown aetiology. The presentation is varied, diagnosis is difficult, and optimum management is still unknown. We report our experience with a case of Castleman's disease in a 34-year old woman who presented with pallor, hepatosplenomegaly, and a right iliac fossa mass that was 5 cm in diameter. this was initially diagnosed as a soft tissue sarcoma and preoperative tumour embolization was planned before excision. Mesenteric arteriogram revealed that the feeder arteries arose from the superior mesenteric artery and embolization was aborted for fear of causing bowel ischaemia. On laparotomy, lymphoid enlargement was found between the leaves of the jejunal mesentery. The tumour was relatively avascular and the overlying mesenteric vessels contributed to teh duplex ultrasound and computerized tomography appearance of hypervascularity. The tumour with the mesentery and the overlying segment of jejunum was excised completely. Histopathology confirmed Castleman's disease. The purpose of this report is to present this rare case that caused a diagnostic dilemma and to review the management of this disorder.
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5/16. A case of Castleman's disease arising from the lesser omentum.

    A 29-year-old female presented with upper abdominal pain. An upper gastrointestinal radiograph and endoscopy revealed an extra compression in the lesser curvature of the body of the stomach. A computed tomography scan and magnetic resonance imaging revealed a tumor located between the left lobe of the liver and the lesser omentum of the stomach. F-18 fluorodeoxyglucose positron emission tomography revealed high uptake at the tumor in the upper abdomen. In an angiogram, a large hypervascular mass had a prominent vascular supply from the left gastric artery; venous pooling and an enlarged feeding vessel were also apparent. From these results, we suspected that the patient had Castleman's disease arising from the lesser omentum. The patient underwent hand-assisted laparoscopic tumor resection. The resected tumor was an encapsulated mass, the surface of which was smooth and the dimensions of which were 77 x 51 x 43 mm. Based on microscopic findings, we diagnosed hyaline vascular type Castleman's disease. Since surgical intervention, the patient has remained asymptomatic, with no pathologic clinical or laboratory findings. Castleman's disease that occurs in the lesser omentum is extremely rare, and the preoperative diagnosis is very difficult. For the localized type of Castleman's disease, clinical findings are usually improved by complete surgical resection.
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6/16. Progressive transformation of germinal center presenting with histological features of hyaline-vascular type of Castleman's disease.

    We report three cases showing progressive transformation of the germinal center (PTGC) with histological features reminiscent of the hyaline-vascular (HV) variant of Castleman's disease (CD). Each case contained a few small HV germinal centers as well as PTGC and hyperplastic germinal centers with or without follicular lysis. Moreover, some of the PTGC were penetrated by hyalinized small vessels. Our three cases also showed some of the characteristic histological findings of HV type of CD: (i) reactive lymphoid follicles with small hyaline-vascular germinal centers surrounded by small lymphocytes in a concentrated fashion; (ii) a few small foci of plasmacytoid monocytes; (iii) perivascular fibrosis; (iv) interfollicular vascularity; (v) tight/concentric pattern of the follicular dendritic cell network; and (vi) absence of CD57 T-cells in the HV follicles. The PTGC with coexistent HV type of CD may represent a certain form of reactive follicular hyperplasia. The possibility of PTGC should be considered and excluded before diagnosing CD.
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7/16. Mediastinal Castleman disease mimicking mediastinal pulmonary sequestration.

    We describe a 39-year-old woman who presented with anterior mediastinal Castleman disease that mimicked an anterior mediastinal pulmonary sequestration due to the presence of both prominent systemic arterial feeding vessels and a systemic draining vein as seen on computed tomography and magnetic resonance imaging.
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8/16. Development of vascular neoplasia in Castleman's disease. Report of seven cases.

    Seven cases of vascular neoplasia arising within lesions of hypervascular follicular hyperplasia (HFH) fulfilling the criteria of Castleman's disease are described. The patients did not have evidence of acquired immunodeficiency syndrome or other immunologic disorders. The masses were solitary and located in the retroperitoneum (five cases), mediastinum (one case), and axilla (one case). Grossly, they measured up to 20 cm and had a variegated appearance. In each case two morphologically distinct processes were present: a mesenchymal spindle-cell neoplasm with evidence of vascular differentiation and Castleman's disease of hyaline vascular type. The two processes blended with each other, with the neoplasm appearing to be continuous with the interfollicular proliferation of small vessels that is typical of Castleman's disease. The lesions behaved aggressively in two cases, both patients having died with metastatic disease. This remarkable association may be viewed as a pathologic manifestation of the intimate functional relationship that exists between the immune and the vascular systems. Other probable examples of this relationship are systemic Castleman's disease associated with Kaposi's sarcoma, localized Castleman's disease associated with vascular hamartoma, histiocytoid hemangioma/angiolymphoid hyperplasia with eosinophilia, and (possibly) angiomatoid malignant fibrous histiocytoma. Perhaps these associations are mediated by the production of angiogenic factors by the activated lymphoid cells.
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9/16. Histopathological study of a case of systemic angiofollicular hyperplasia (Castleman's disease).

    This report describes the nodal and hepatic lesions observed in a patient with generalized disorders that had been histologically diagnosed as a systemic angiofollicular hyperplasia. The diagnostic morphological findings were observed in the nodes of the axilla and were represented by diffuse marked plasmacytosis, prominence of the germinal centres, preservation of the architecture with a reactive proliferation of blood vessels and fibrous tissue in interfollicular areas.
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10/16. Angiomatoid malignant fibrous histiocytoma with extensive lymphadenopathy simulating Castleman's disease.

    We report the association in a 10-year-old boy of an angiomatoid malignant fibrous histiocytoma (AMFH) of the left thigh with ipsilateral inguinal, pelvic and extensive retroperitoneal lymphadenopathy, and severe systemic manifestations. These include growth retardation, fever, severe anemia, hypergammaglobinemia, and hypoalbuminemia. At ultrastructural level the tumor was characterized by an abundance of myofibroblasts, occasional histiocytes, and small vessels with marked reduplication of the basal lamina. Biopsies of the inguinal and abdominal lymph nodes showed follicular hyperplasia and massive plasmacytosis indistinguishable from Castleman's disease (giant lymph node hyperplasia) of plasma cell type. The radical surgical excision of the primary tumor in the thigh resulted in the disappearance of the abdominal lymphadenopathy and a marked reduction in size of the pelvic lymph nodes with marked decrease of the gammaglobulins, thus proving that the nodal lesions were the expression of a reactive process to the tumor rather than a coincidental independent lymphoproliferative disorder. Retroperitoneal and pelvic node dissection was performed 1 year after the radical excision of the thigh tumor because of persistent pelvic lymphadenopathy and failure of serum immunoglobulins M and A to return to normal level, with a recent peak of IgA to twofolds that of normal value. Metastatic AMFH was found in the three pelvic nodes. One month postoperatively IgA returned to near normal level whereas IgM remained slightly elevated.
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