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11/154. Castleman's disease: atypical manifestation in an 11-year-old girl.

    Castleman's disease (angiofollicular lymphoid hyperplasia) is a rare lymphoproliferative disorder that can be found in both nodal and extranodal sites. It is classified histologically as the more common hyaline vascular variant, a plasma cell variant, or a mixed form. The hyaline vascular variant is typically characterized by a benign clinical course with no constitutional symptoms other than localized pressure from the mass. We report an atypical case of the hyaline vascular variant with constitutional symptoms that have been clinically associated with the aggressive plasma cell variant. diagnosis was confirmed histopathologically after surgical resection, which was curative and resulted in resolution of all symptoms. This case broadens our understanding of Castleman's disease as a part of the spectrum of lymphoproliferative and inflammatory diseases. Furthermore, it supports recent studies suggesting that systemic pathogenicity is related to associated cytokine dysregulation and highlights the need to include Castleman's disease in the differential diagnosis of pediatric lymphoproliferative disorders.
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12/154. giant lymph node hyperplasia a diagnostic dilemma in the neck.

    giant lymph node hyperplasia (GLNH) or Castleman's disease is a rare pathological entity commonly presenting as a solitary mediastinal tumour (unifocal variant), although other anatomical sites have been reported including the head and neck, abdomen and axilla. Though the head and neck is the second commonest site for this lesion, only 60 cases [1,2] have been reported in the literature. Pre-operative diagnosis of the unifocal variant can be difficult as routine investigations carried out in the clinic setting are often inconclusive. A multicentric form of this disease with a poorer prognosis in comparison to the unifocal lesion has been described by Gaba et al. in 1978 [3]. We present a case of an unusually large, slow growing, asymptomatic, unifocal variant of this tumour restricted to the neck which underwent successful surgical excision. A review of the literature, histopathological characteristics and differential diagnosis is also presented.
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13/154. Castleman's disease (giant lymph node hyperplasia) of the neck: a case report.

    Castleman's disease (giant lymph node hyperplasia) is an uncommon cause of neck mass. Its cause and pathogenesis are still unknown. giant lymph node hyperplasia (GLH) usually presents as an asymptomatic solitary mass and can occur anywhere in the head and neck. Diagnostic test results are always inconclusive. Excision and histopathological evaluation are the only ways to make a definitive diagnosis. The disease is curable by surgical excision. A case of GLH presenting as a solitary neck mass in a 68-year-old man is reported. There has been no recurrence during about 7 months.
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14/154. CD56-Positive cutaneous lymphoma with multicentric Castleman's disease-like systemic manifestations.

    We report a 55-year-old Japanese male with CD56 cutaneous lymphoma. The patient had multiple cervical lymphadenopathy, a red nodule on his neck, and parotid gland nodularity. Histologic features of the biopsied cervical lymph node showed follicular hyperplasia with numerous plasma cells. A biopsied skin specimen of the nodule on his neck demonstrated dense infiltration of atypical large lymphocytes into the dermis. Immunohistochemical study of this specimen revealed CD3 , CD4 , and CD56 expression in the majority of neoplastic cells. Polymerase chain reaction assays for the detection of Epstein-Barr virus sequences were positive for lymph node and skin dna. Laboratory examinations showed polyclonal gammopathy, pancytopenia, and high serum interleukin-6 levels. These clinical and histological findings resembled those of multicentric Castleman's disease.
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15/154. A case of Castleman's disease with "downhill" varices in the absence of superior vena cava obstruction.

    Downhill esophageal varices", classically defined as those that develop in the upper region of the esophagus, are less common than the "uphill" type, which is usually produced by portal hypertension. Various causes of downhill varices have been reported, but mediastinal tumor is the most common responsible lesion. Castleman's disease, or angiofollicular lymph node hyperplasia, is a rare pathological process of unknown etiology that usually develops in the mediastinum. We report the case of a 60-year-old woman whose large esophageal varices were detected incidentally. The cause was a mediastinal mass which was diagnosed as Castleman's disease on histopathological examination of a surgical specimen. This patient's varices most likely formed as a result of copious blood drainage from the tumor into the esophageal veins. Evidence for this was the lack of the classic downhill pattern, the absence of superior vena cava obstruction, and the fact that the varices resolved after the tumor was removed. It is our opinion that this type of varices should be renamed, and we suggest that "overflow varices" would be an appropriate term.
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16/154. Castleman's disease: unusual case of inflammatory spinal dorsal epidural mass: case report.

    OBJECTIVE AND IMPORTANCE: Castleman's disease (giant lymph node hyperplasia) is a rare, heterogeneous, lymphoproliferative disorder of unknown cause and pathogenesis. Most cases occur as mediastinal masses, although extrathoracic involvement, including nodal and extranodal locations, has been reported. The localized variants (solitary lesions) respond well to surgical excision. CLINICAL PRESENTATION: We report the case of a 34-year-old man with headache, intermittent febrile sense, progressive weakness of the legs, and urinary incontinence. magnetic resonance imaging disclosed an enhancing dorsal extradural mass with impingement on the spinal cord at the T2-T3 level. Other abnormal laboratory findings were increased erythrocyte sedimentation rate and mild leukocytosis. TECHNIQUE: The mass lesion was removed; the histopathological findings included diffuse lymphoid cell infiltration and vascular proliferation, which are compatible with Castleman's disease. CONCLUSION: Although the dorsal epidural site of this case is very unusual, Castleman's disease was considered the most appropriate diagnosis on the basis of the associated systemic findings. This patient with dorsal epidural Castleman's disease may be the first reported case in the literature.
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17/154. Castleman's disease masquerading as sigmoid colon tumor and Hodgkin lymphoma.

    Castleman's disease is a benign lymphoid neoplasm first reported as hyperplasia of mediastinal lymph nodes. Some authors referred to the lesions as isolated tumors, described as a variant of Hodgkin's disease with a possibility of a malignant potential and others proposed that the lymphoid masses were of a hamartomatous nature. Three histologic variants and two clinical types of the disease have been described. The disease may occur in almost any area in which lymph nodes are normally found. The most common locations are thorax (63%), abdomen (11%) and axilla (4%). We report two separate histologic types of Castleman's disease which were rare in the literature, mimicking sigmoid colon tumor and Hodgkin lymphoma. The diagnostic and therapeutic aspects of this rare entity is discussed.
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18/154. Unicentric Castleman's disease complicated by systemic AA amyloidosis: a curable disease.

    BACKGROUND: Castleman's disease (angiofollicular lymph node hyperplasia) is a group of rare lymphoproliferative disorders sharing characteristic clinical and histological features, and usually accompanied by a marked systemic inflammatory response. All types may be complicated by acquired systemic amyloidosis, usually of AA type, but occasionally of AL type associated with monoclonal gammopathy. DESIGN: Descriptive study of five patients with unicentric Castleman's disease complicated by systemic AA amyloidosis. methods: A diagnosis of amyloidosis was confirmed by microscopy and immunohistochemical staining. Serum concentrations of c-reactive protein (CRP) and serum amyloid a protein (SAA) were measured by immunoassays. Radiolabelled serum amyloid P component scintigraphy was used to monitor the progress of amyloid deposition. RESULTS: In four patients the primary diagnosis was made only after years of investigation of systemic symptoms. The tumours were resected in all cases, leading to remission of the systemic inflammatory state. Long-term follow-up in four patients, including scintigraphy, showed regression of amyloid deposits. DISCUSSION: This rare but usually fatal condition can be cured surgically even in advanced cases. awareness of the diagnosis and its correct management are important in investigation of patients with unexplained systemic symptoms, especially associated with systemic amyloidosis.
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19/154. Linear IgA disease in a patient with Castleman's disease.

    Linear IgA disease (LAD) is a well recognized subepidermal blistering disorder characterized by linear deposits of IgA at the basement membrane zone. The aetiology is unknown but there is a recognized association with lymphoproliferative malignancies. We report a case of LAD occurring in a patient with multicentric Castleman's disease (angiofollicular lymph node hyperplasia), an association not previously recorded in the literature.
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20/154. Castleman's disease: an unusual cause of mediastinal mass and anemia.

    We reported a case of thoracic localized, mixed variant, Castleman's disease (angiofollicular lymph node hyperplasia) with a favourable clinical course presenting with mediastinal mass, microcytic anemia, mild thrombocytosis, polyclonal hyper-gammaglobulinaemia and without symptoms. Castleman's disease must be always considered in the differential diagnosis of solitary mediastinal masses. Transthoracic fine needle aspiration cytology is usually nondiagnostic; in fact, cytologically, Castleman's disease can be confused with other mediastinal lymphoproliferative disorders (such as thymomas or lymphomas). The optimal therapeutic approach is unknown but a complete surgical resection is treatment of choice for localized disease.
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