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1/34. poems syndrome: cicatricial alopecia as an unusual cutaneous manifestation associated with an underlying plasmacytoma.

    poems syndrome is a rare condition with cutaneous manifestations commonly including angiomas, hypertrichosis, hyperpigmentation, and thickening of the skin. We describe a male patient with a 2-year history of cervical lymphadenopathy, erythematous thickening of the skin on the neck, and progressive walking difficulties. The patient had an occipital erythema with scarring alopecia and sparse follicular pustules at the edge of the lesion. Further investigation revealed symmetric polyneuropathy, hepatosplenomegaly, monoclonal gammopathy, subclinical thyreopathy, and an osteolytic bone lesion of the skull. Histologically, a plasmacytoma with lambda cell restriction was found. The overlying skin showed marked fibrosis, with loss of hair follicles, and a plasma cell infiltrate of polyclonal origin. The cervical lymph nodes showed histologic characteristics of multicentric Castleman's disease, and the skin of the neck showed thickening and vasoproliferation. There was no evidence of further plamacytomas. After excision of the plasmacytoma and postoperative irradiation, the symptoms gradually resolved within a few months. A cicatricial lesion remained on the occiput without further folliculitis or hair loss on the rest of the scalp. This case illustrates the reactive character of poems syndrome as a paraneoplastic syndrome in myeloma patients.
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2/34. Follicular dendritic cell sarcoma of the neck: report of two cases complicated by pulmonary metastases.

    BACKGROUND: Follicular dendritic cell (FDC) sarcoma is an uncommon neoplasm occurring primarily in lymph nodes but also in extranodal sites. A correct diagnosis can be difficult to make, especially in the latter sites. methods: Two patients with FDC sarcoma of the cervical soft tissues that metastasized to the lungs are reported. Both were initially misdiagnosed as having CASTLE (carcinoma showing a thymus-like element). Additional immunohistochemical stains were performed. RESULTS: The primary tumors showed jigsaw puzzle-like lobulation resembling thymic epithelial tumor and consisted of spindly cells arranged in fascicles, whorls, and a storiform pattern. The spindly cells had indistinct cell borders, vesicular nuclei, and distinct nucleoli. Perivascular spaces were present. lymphocytes were sprinkled throughout the tumor in one case but were sparse in the other. The metastatic deposits in the lungs appeared 27 and 2 years, respectively, after the initial presentation and were histologically similar to the original tumors. The FDC nature of the primary and metastatic tumors was confirmed by positive staining with CD21/CD35 cocktail and CD23 and by negative staining for cytokeratin. In one case, in direct continuity with the main tumor, there was a lobulated lesion composed of small lymphocytes punctuated by large cells with vesicular nuclei, histologically reminiscent of thymoma. The large cells were shown by immunohistochemistry to represent FDCs forming complex interconnecting meshworks. It is unclear whether this contiguous mass represents a precursor lesion or an unusual-looking component of the neoplasm. CONCLUSIONS: FDC sarcoma can look deceptively like a thymic epithelial tumor histologically. A correct diagnosis requires a high index of suspicion and immunohistochemical evaluation. The tumor shows a propensity to metastasize to the lungs, which can be delayed until more than 20 years after initial presentation.
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3/34. Cervical Castleman's disease: imaging findings.

    Castleman's disease, a lymphoproliferative disease of unknown etiology, rarely causes a neck mass and may mimic granulomatous, inflammatory and metastatic lymph nodes, or lymphoma with its nonspecific imaging findings. Definitive diagnosis and treatment is possible with complete surgical resection. Imaging findings of a case with Castleman's disease detected during multinodular goiter surgery and later verified through excisional biopsy are presented. This report, as far as we know, is the first evaluation of the disease with sonography, Doppler sonography, CT, MR imaging, and digital subtraction angiography (DSA) findings.
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4/34. 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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keywords = neck
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5/34. 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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ranking = 3.5
keywords = neck
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6/34. Plasma cell variant of Castleman's disease occurring concurrently with Hodgkin's disease in the neck.

    BACKGROUND: Castleman's disease, a benign lymphoproliferative disorder, may be seen as a self-limited, curable unifocal process, or highly aggressive multicentric disease frequently resulting in death despite aggressive management. Non-Hodgkin's lymphoma has been known to arise within the context of Castleman's disease, usually when multicentric. Hodgkin's lymphoma, however, can also arise within the context of Castleman's disease, but this is a rare process. We report a case of unifocal Castleman's disease (plasma cell variant) occurring concurrently with Hodgkin's disease in the neck of a young woman. methods: The presentation, workup, pathologic evaluation, and management of a young woman diagnosed with Castleman's disease occurring concurrently with Hodgkin's disease in the neck is presented and discussed. RESULTS: A 32-year-old woman with a 5-year history of unifocal right cervicoparotid Castleman's disease (plasma cell variant) underwent right functional neck dissection and superficial parotidectomy for cosmetic and functional purposes. Pathologic and immunohistochemical analysis confirmed Hodgkin's lymphoma occurring in a background of the plasma cell variant of Castleman's disease. The patient subsequently underwent external beam radiation therapy as definitive management for her early-stage Hodgkin's lymphoma. CONCLUSIONS: Castleman's disease can occur as an isolated regional process in the head and neck. Furthermore, lymphoma (and specifically Hodgkin's lymphoma) can develop within regionally isolated cervical Castleman's disease. Although complete surgical excision of unifocal Castleman's disease is curative, the management of lymphoma occurring within the context of the Castleman's disease warrants a standard lymphoma workup and management strategy.
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ranking = 4
keywords = neck
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7/34. A rapidly expanding lesion in the neck: unusual presentation of Castleman's disease.

    We report a painless but rapidly enlarging 9 cm x 4 cm lobulated hard neck mass. CT scanning suggested lymphoid tissue that was not confirmed by cytopathology. Histopathological analysis of the excision specimen detected Castleman's disease, extending to the resection margin. Postoperative radiotherapy was administered. The patient remains disease-free after 44 months.
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ranking = 2.5
keywords = neck
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8/34. 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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keywords = neck
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9/34. Castleman's disease of the buccal mucosa: report of a case and review of the literature of head and neck cases.

    A case of Castleman's disease occurring in the buccal mucosa is described. An 84-year-old woman noticed that a mass in the left buccal mucosa that had been present for half a year. Computed tomography revealed a well-demarcated submucosal tumor, measuring 4.0 x 3.0 x 2.0 cm. The patient received no treatment at this time, and continued growth of the mass was observed. After incisional biopsy, the lesion was surgically removed. Histologically, the tumor consisted of an enlarged lymph node with conspicuous lymph follicles, in which vascular channels and deposits of eosinophilic material were noted. Laboratory examination showed an increase of serum antibody level of cytomegalovirus but of no other viruses. The patient was followed up for 1(1/2) years, with no clinical evidence of recurrence. This is the first report of Castleman's disease presenting in an oral site.
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ranking = 2
keywords = neck
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10/34. Castleman's disease presenting as a midline neck mass.

    We report the case of a 13-year-old girl who presented with a painless midline submental mass. Excision biopsy confirmed Castleman's disease of the hyaline-vascular type. This unusual condition needs to be considered in the differential diagnosis of masses arising in the neck.
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ranking = 2.5
keywords = neck
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