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1/10. Chordoid meningioma.

    Chordoid meningioma is a relatively rare variant that is often associated with peritumoral lymphoplasmacellular infiltration causing Castleman syndrome (CS). We present a 44-year-old woman with chordoid meningioma not associated with CS. The patient presented with epilepsy and right hemiparesis (Todd's palsy) on admission. The radiological findings revealed an extraaxial mass lesion in the premotor cortex. They were compatible with a preoperative diagnosis of meningioma. No physical abnormalities related to CS were detected. A left frontal craniotomy was performed. The tumor surface was gelatinous, and it was totally resected with the attached dura mater (Simpson grade I). The patient had an uneventful recovery, and her seizures subsided. The pathological findings of the specimens revealed nests and cords of spindle and epithelioid cells with abundant myxoid matrix, mimicking the features of chordoma. On the basis of radiological, immunohistochemical, and electron microscopic findings, chordoid meningioma was verified, and a review of the literature was performed.
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2/10. Secondary amyloidosis in Castleman's disease: review of the literature and report of a case.

    It is quite rare to diagnose secondary amyloidosis during the course of Castleman's disease (CD). A 51-year-old female who complained of fatigue, weight loss, and fever was diagnosed with CD -- plasma cell type -- in our hospital in 1993. One year after diagnosis, she developed nephrotic syndrome, the etiology of which was found to be secondary amyloidosis based on renal biopsy. As the patient rejected therapy, she was discharged after only symptomatic treatment. At her last follow-up in March 2001, she had no complaints; physical examination, blood chemistries, and urinalysis were normal. Abdominopelvic tomography revealed no lymphadenopathy in the abdomen, which had been previously present. We could identify 17 other cases of CD with secondary amyloidosis in the literature. Ours is the 18th such case and the 2nd case of multicentric CD leading to amyloidosis. This case also shows that CD might sometimes run a relatively benign course being cured with no therapy, whereas it might have a rapidly fatal downhill course -- even with therapy -- in others. Still, effective treatment strategies need to be developed.
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keywords = physical examination, physical
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3/10. Castleman's disease in a child presenting with a partly mineralized solitary meningeal mass.

    We report a case of solitary intracranial childhood Castleman's disease (CD) presenting with a sudden onset of partial seizures due to a meningeal and cortical mass lesion. The patient was a previously healthy 8-year-old girl who developed a new onset of simple partial seizures with motor signs. On physical examination, she was neurologically intact. Other findings included low-grade fever, mild microcytic anemia and lymphopenia. magnetic resonance imaging (MRI) of the brain revealed a left posterior parietal, partly mineralized, contrast-enhancing meningeal mass with cortical invasion and adjacent white matter edema. A complete surgical resection of the dural-based component and a subtotal resection of the adherent, invasive cortical lesion were performed. Pathohistology and flow cytometry of the dural-based lesion disclosed a hyaline-vascular type of CD with striking proliferation of polyclonal B lymphocytes, scattered plasma cells and extensive multifocal cortical mineralization. At the 6-month follow-up, the patient was seizure free on antiepileptics and had returned to normal daily activities. MRI showed no residual lesion, and a workup for systemic disease was negative.
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keywords = physical examination, physical
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4/10. New observations in a child with angiofollicular lymph node hyperplasia (Castleman's disease) originated from the mesenteric root.

    Localized plasma cell type Castleman's disease (CD) is an unusual pathologic entity. It is frequently associated with clinical and laboratory characteristics and rarely occurs in children. Total surgical excision results in cure in all aspects. To make early diagnosis of mesenteric CD is not easy, especially for children. An 11-year-old Taiwanese boy was recently evaluated for anemia and delayed growth. His clinical findings included a syndrome of severe hypochromic microcytic anemia, neutropenia, thrombocytosis, hypoferremia, hypergammaglobulinemia, and growth failure. Radiological examinations (abdominal ultrasound, small intestinal series, and computerized tomography) identified hepatosplenomegaly, nephromegaly, and huge masses in the middle abdomen with precaval, celiac, and paraaortic lymph nodal enlargement. However, detailed physical examination failed to detect a mass. At laparotomy a double-fist-sized confluent mass was found arising from the mesenteric root. Most masses were discrete and were excised individually. The pathologic diagnosis was plasma-cell type angiofollicular lymph node hyperplasia (Castleman's disease). Seven weeks after surgery, he had an episode of acute hepatitis b. Postoperatively, he exhibited a dramatic growth spurt; the hemoglobin, red blood cell indices, serum iron, and immunoglobulins returned to normal in 2 months. neutropenia, which has not been previously related to mesenteric CD, was an unexpected finding in our case; however, it resolved spontaneously 3 months after the surgery, suggesting its causal relationship with the tumor.
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keywords = physical examination, physical
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5/10. Peripancreatic Castleman disease.

    CONTEXT: Castleman disease or giant lymph node hyperplasia is a rare disorder of the lymphoid tissue, usually occurring in the mediastinum. We report a case of localized peripancreatic plasma cell type Castleman disease, which is an unusual site. CASE REPORT: A 45-year-old woman with a history of mild epigastric pain radiating to the back for the previous year was admitted and her physical examination was normal. A raised erythrocyte sedimentation rate, c-reactive protein and hypergammaglobulinemia were found. Abdominal ultrasonography and magnetic resonance imaging demonstrated a homogeneously hypoechoic solid mass having a smooth-surface, 6 cm in diameter, located between the head of the pancreas, the lower margin of the antrum and the left lobe of the liver. The mass was totally excised surgically with repair of the pancreas capsule. Histopathological examination of the tumor showed plasma-cell type Castleman disease. CONCLUSION: Clinicians should be aware that Castleman disease may involve peripancreatic tissue which leads to difficulties in arriving at a differential diagnosis. Surgical excision is both a diagnostic and a curative method for management of the disease.
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keywords = physical examination, physical
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6/10. Severe polyneuropathy: initial manifestation of Castleman's disease associated with poems syndrome.

    Castleman's disease is a rare clinicopathological entity characterized by multicentric angiofollicular lymph node hyperplasia. Peripheral neuropathy has infrequently been described in patients with Castleman's disease. A patient is described who presented initially with severe painful sensorimotor polyneuropathy of his lower limbs diagnosed as a plasma cell variant of Castleman's disease associated with features of poems syndrome (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin change). The patient was treated with plasmapheresis, immunosuppressive agents, and intensive rehabilitation. His functional status improved from being wheelchair bound to ambulating independently with crutches. Clinicians should be alert to the relationship of Castleman's disease and mixed polyneuropathy because physical improvement is possible with treatment. Also, the evaluation of patients presenting with peripheral neuropathy of unknown etiology and lymphadenopathy should include lymph node biopsy to rule out Castleman's disease.
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7/10. Castleman's disease presenting as a pediatric surgical problem.

    A 9-year-old girl presented with anemia, fever, and poor growth over a 2 1/2-year period. On physical examination, a right lower quadrant mass was palpated. Her signs and symptoms were consistent with Castleman's disease of the plasma cell type. The mass, a giant hyperplastic lymph node, was excised, and the patient's symptoms resolved. Castleman's disease is a benign lymph node disorder that occurs very rarely in the pediatric population and is cured by operative excision of the lymphatic mass.
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ranking = 16.306119966558
keywords = physical examination, physical
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8/10. Castleman's disease presenting as an asymptomatic solitary pulmonary nodule.

    We report the case of a 51 year old white woman with an asymptomatic solitary pulmonary nodule, fortuitously discovered on chest radiography. Her physical examination and biochemical tests were unremarkable for pathological findings. Thoracic computed tomography (CT) scan and nuclear magnetic resonance (NMR) spectroscopy confirmed the presence of a dishomogeneous thick lesion of lobular shape, localized between the middle and lower lobe of the right lung. Fibreoptic bronchoscopic examination failed to produce histological diagnosis of the lesion. Surgical removal of the lesion demonstrated intraparenchymal localization of Castelman's disease (giant lymph node hyperplasia). Three years after surgery, no sign of disease recurrence has been recorded.
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keywords = physical examination, physical
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9/10. Castleman's disease of the mesentery in a child: a case of seven years' duration without typical X-ray findings.

    This report describes a 9-year-old boy with intermediate variant type of giant lymph node hyperplasia or Castleman's disease (CD) originating from the mesentery. He had symptoms and signs related to the disease for seven years before the final diagnosis. The patient's general condition remained good, except for periods of fever and abdominal pain. pallor and slow growth were the only abnormal findings on physical examination during the follow-up. Laboratory measurements showed worsening microcytic anemia, low serum iron level, and low iron stores in bone marrow samples. The erythrocyte sedimentation rate (ESR) increased to 110 mm/h, and the serum levels of c-reactive protein varied between 80 and 120 mg/l. The level of serum albumin was low, 25-28 g/l, and serum immunoglobulin g was somewhat elevated, varying between 17-13 g/l. The radiologic examination of intenstine gave pathological results suggesting a small bowel disease, but no tumor was detected. The abnormal laboratory values and symptoms of the patient resolved completely after surgical removal of the mass.
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ranking = 16.306119966558
keywords = physical examination, physical
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10/10. Localized mucosal involvement and severe pulmonary involvement in a young patient with paraneoplastic pemphigus associated with Castleman's tumour.

    We describe a 19-year-old female patient who developed recurrent ulcerations limited to the orogenital mucosa for the last 3 years. She also developed dyspnoea 5 months after the onset of the orogenital lesions. Castleman's tumour of the retroperitoneum was found incidentally during routine physical examination. The diagnosis of paraneoplastic pemphigus (PNP) was made by pathological and immunological studies. The orogenital ulceration responded well to corticosteroid therapy, but severe bronchiolitis obliterans progressed despite intensive care. The patient eventually died from respiratory failure. This case demonstrates the diversity of clinical features of paraneoplastic pemphigus.
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ranking = 16.306119966558
keywords = physical examination, physical
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