扁桃体滤泡树突细胞肉瘤1例

目的 探讨扁桃体滤泡树突状肉瘤（FDCS）的临床表现、体征、诊断、病理特征、治疗方法，提高对该病的认识，减少误诊率。方法 回顾性分析1例扁桃体FDCS疾病特征，该患者因右侧口咽肿胀入院，无明显临床症状。入院后行颌面部及颈部CT检查，结果示右侧口咽部占位性病变，病理及免疫组化检查结果为FDCS，遂对该患者行右侧扁桃体及肿物扩大切除术。结果 完全切除右侧扁桃体及肿物，术后无出血、感染等并发症。术后予以放疗，随访3个月，未见复发。结论 FDCS是一种罕见的恶性肿瘤，临床症状表现无特异性，在临床工作中易被误诊，病理学及免疫组化检查是诊断该疾病的主要方法，治疗以完全性手术切除为主，术后可辅以放疗和（或）化疗。     

Follicular dendritic cell sarcoma of the tonsil: a case report

Follicular dendritic cell sarcomas of the tonsil are very rare tumors. Only 10 cases have been published in the English-language medical literature. We present an additional case that occurred in a 27-year-old woman. She presented with a tumor of the right tonsil. Histological sections of tonsillar biopsies and tonsillectomy suggested that this tumor was a nerve sheath tumor or a malignant melanoma. Histological examination and immunohistochemical study were reviewed. The final diagnosis was follicular dendritic cell sarcoma. She underwent a transoral resection of the right oropharynx with ipsilateral selective neck dissection and postoperative irradiation. We emphasize the difficulties in diagnosing this type of tumor. The use of monoclonal specific markers is required to avoid misdiagnosis. We discuss therapeutic methods for this rare tumor for which optimal treatment has not yet been defined.     

非霍奇金淋巴瘤愈后继发扁桃体滤泡树突状肉瘤1例

1病例报告 患者,男,59岁,因“口咽异物感伴咯血3年,症状加重伴发现左颈部包块4个月余”于2012年5月1日于我科就诊。入院时无发热、吞咽疼痛、吞咽梗阻、呼吸困难等,体检见：左侧扁桃体肿大,表面暗红溃烂,有血痂附着（图1）;     

Interdigitating dendritic cell sarcoma of the parotid gland: case report and literature review

Interdigitating dendritic cell sarcoma (IDCS) is an exceedingly rare neoplasm arising from the antigen-presenting cells of the immune system. We report a case of IDCS occurring in a 69-year-old man who presented to an outside institution with a painless mass in his right parotid gland for several months. He presented to our institution after undergoing a superficial parotidectomy. A diagnosis of undifferentiated neoplasm, favoring poorly differentiated carcinoma, was made at that time. He underwent a total parotidectomy and neck dissection at our institution. Microscopically, the tumor was composed of atypical spindle cells involving the parotid gland and an ipsilateral level III lymph node. Immunophenotypic analysis demonstrated positive staining for S100, fascin, vimentin, and HLA-II. Follicular dendritic cell, lymphoid, epithelial, myoepithelial, and melanoma markers were negative. Taken together, the above features were consistent with IDCS. An IDCS of the parotid gland is extremely rare, with only 2 cases reported in the literature. The unusual location and morphological similarity to follicular dendritic sarcoma and other types of soft tissue sarcomas can be a diagnostic challenge. Awareness of this tumor and the use of appropriate markers are crucial in making the diagnosis. The patient did well postoperatively, and he underwent a complete course of postoperative irradiation to the right parotid and neck.     

Follicular dendritic cell sarcoma of a cervical lymph node: case report and review of the literature

Follicular dendritic cell sarcoma is a rare tumor that occurs mainly in lymph nodes. We report a case of follicular dendritic cell sarcoma in a cervical lymph node that was initially diagnosed as a B-cell lymphoma by Tru-Cut biopsy. The correct diagnosis was established by excision biopsy and immunohistochemistry. This tumor is of interest to head and neck surgeons because its recurrence rate is significant and its metastatic potential has been underestimated because of its rarity and the difficulty in making the diagnosis. We discuss the salient clinical and pathologic features of this tumor, as well as its management protocol, and we review the literature.     

Bilateral tonsillar and esophageal Kaposi sarcoma in an HIV-negative patient

Tonsillar involvement in Kaposi sarcoma is extremely rare, as only a few such cases have been reported; all but 1 of these previously reported cases occurred in patients with human immunodeficiency virus (HIV) infection. We describe what to the best of our knowledge is the first reported case of concurrent bilateral tonsillar and esophageal Kaposi sarcoma in an HIV-negative patient. A 68-year-old man presented with sore throat and dysphagia. Clinical examination revealed the presence of bilateral and asymmetrical tonsillar masses, as well as generalized lymphadenopathy in the cervical chain. The masses were resected, and findings on histopathologic analysis were consistent with Kaposi sarcoma. In addition, human herpesvirus 8 was demonstrated on a tonsil specimen by polymerase chain reaction, and microinvasive squamous cell carcinoma was also detected. Later, another Kaposi sarcoma lesion was detected in the lower third of the esophagus. We recommend that clinicians not discount the possibility of oral classic Kaposi sarcoma in the workup of an immunocompetent patient with oral vascular lesions.     

Follicular dendritic cell sarcoma of the tonsil: a case report and literature review

We describe a case of follicular dendritic cell sarcoma (FDCS) of the tonsil in a 59-year-old woman. She was successfully treated with excision of the mass and postoperative radiation therapy. According to our review of the literature, only 25 cases of extranodal FDCS in the head and neck have been previously reported, including only 10 cases that involved a tonsil. We briefly review these earlier reports, and we discuss the diagnosis and management of FDCS.     

Cervical follicular dendritic-cell sarcoma

Follicular dendritic-cell sarcoma is a rare neoplasm occurring mostly in the head and neck region. Originating in reticular dendritic cells, it is classified as nodal or extra-nodal depending on the organ affected. The clinical behaviour, pathology diagnosis, treatment, and evolution are not well known. We present the case of a patient with a cervical tumour of lymphatic origin that was diagnosed as follicular dendritic-cell sarcoma. We analyze diagnosis and treatment, and discuss the reviews of published cases.     

鼻及咽部树突细胞肿瘤4例临床分析并文献复习

目的：探讨树突细胞肿瘤的临床表现、病理特点、治疗和预后。方法：4例鼻及咽部树突细胞肿瘤患者中，滤泡状树突细胞肉瘤（FDCS）2例，朗格汉斯组织细胞增多症（LCH）1例，朗格汉斯细胞肉瘤（LCS）1例。2例FDCS中有1例行综合治疗（手术加放化疗），1例仅行放化疗；1例LCH及1例LCS患儿均行手术治疗。结果：2例FDCS患者，l例术后行4个疗程CHOP方案及同步放疗（50Gy）后达完全缓解，1例仅行放化疗者随访7个月仍带瘤生存；1例LCH患儿随访2年余，肿瘤无复发及转移；1例LCS患儿，术后未行放化疗，随访10个月后死亡。结论：树突细胞肿瘤罕见，临床易误诊，确诊需病理组织形态、免疫组织化学及电镜相结合，FDCS、LCH及LCS具有独特的病理表现、免疫表型、诊治及预后。     

Nasopharyngeal dendritic cell sarcoma, not otherwise specified, in a 34-year-old man

Dendritic cell sarcoma, not otherwise specified (NOS), is an entity that is poorly defined because of its rarity and poorly understood pathogenesis. It is characterized by positive immunohistochemical staining for S-100 and CD1a, along with an absence of cytoplasmic Birbeck granules on electron microscopy. We report the case of a surgically inaccessible nasopharyngeal dendritic cell sarcoma, NOS, in a 34-year-old man. Treatment with chemotherapy along with adjuvant radiation therapy was successful in decreasing the size of the nasopharyngeal mass, and the patient remained free of any evidence of recurrence nearly 5 years after treatment.     

Extrathoracic castleman’S disease — presenting as a neck mass

Castleman’s disease, also known as angiofollicular hyperplasia, is a disease of unsettled complexity and morbidity, usually occurs in the mediastinum and less frequently in the neck as a solitary mass and is often associated with other illnesses. Two variants have been recognized, the hyaline vascular type which is mare common and the less common plasma cell type with a third hybrid or intermediate type that shares features with both. It has been described with AIDS and Kaposi’s sarcoma as well as follicular dendritic cell tumours. We report a case of Castleman’s disease of the hyaline vascular type arising in the left supra-clavicular region in 41 year old male without any associated systemic illness. The neck is an uncommon location for this rare disease.     

Isolation of FDC-lymphocyte clusters from human tonsillar tissues

Follicular B cells and follicular dendritic cells (FDCs) form FDC-lymphocyte clusters and play a central role in events related to humoral immunity in the lymphoid follicle (LF). The secondary LF is divided into five zones, each of which exhibits functional differences. However, the distribution of the clusters across the five follicular zones remains unclear. We here report a procedure for isolating FDC-lymphocyte clusters from fixed tonsillar tissues and compare of the structure of clusters isolated from three follicular zones: the mantle, light and dark zones. First, the germinal centers (GCs) of the secondary LFs were removed under a stereoscope, and the GCs were enzymatically digested for 20, 30, 40 and 50 minutes at 37 degrees C. The FDC-lymphocyte clusters were then isolated using a discontinuous density gradient and a Magnetic Particle Concentrator, followed by microbeads. The number of isolated medium sized clusters composed of 6-25 cells was greatest when the samples were incubated for 40 minutes. To detect the mantle, light and dark zones, and GCs, isolated FDC-lymphocyte clusters from each zone were immunostained. Their cell structures were then compared. The clusters were composed mainly of B cells (comprising about 80% of the cells in each cluster, on average), T cells, natural killer/T cells and macrophages were also observed, but less frequently. The proportions of CD 45 RO-positive cells and CD4-positive cells were clearly different for each zone, with CD4-positive cells in the majority. No clear differences in isolated clusters from fixed and unfixed tonsillar tissues were observed. Our data indicate that this procedure is suitable for isolating FDC-lymphocyte clusters from fixed lymphoid tissues and that the proportions of cells composing the clusters differ in the three follicular zones.     

Follicular dendritic cell tumor of the cervical lymph node

Follicular dendritic cell (FDC) tumor is an uncommon sarcoma, which occurs in lymph nodes or in extranodal sites. A 61-year-old female was referred to our hospital because of a nontender mass on her neck. The mass was surgically removed and initially diagnosed as a metastasis of malignant fibrous histiocytoma (MFH). Because the subsequent progress was inconsistent with the common prognosis of a patient with MFH, the possibility of FDC tumor was taken into consideration. Immunohistological studies for FDC antigens, such as CD21 and CD35, led to final diagnosis. It is suggested that FDC tumor should be included in the diagnostic considerations when a spindle cell tumor is present in a lymph node.     

Extranodales follikuläres dendritisches Sarkom der Tonsille

Follicular dendritic cell (FDC) sarcoma is a rare tumor deriving from dendritic cells of the lymphoid follicle. Only a few cases of extranodal manifestation of FDC sarcoma of the head and neck have been reported. We describe the case of a 47-year-old woman with a FDC sarcoma of the left tonsil that first manifested 11 years ago. She has suffered three recurrences since this time, at continually reducing intervals. Histologically, the neoplasm consisted of an increased number of spindle-shaped cells with partly hyperchromatic nuclei. The patient underwent local tumor excisions, and then received an adjuvant chemotherapy with doxorubicin and ifosfamide when she presented with the third recurrence. The 11 year follow-up bears out a more aggressive behaviour of FDC sarcoma than first assumed. Therefore, a wide local excision, eventually combined with neck dissection in the case of recurrences, seems to be necessary.     

Extranodal follicular dendritic cell tumor of the tonsil: report of a diagnostic pitfall and literature review

Follicular dendritic cell tumor (FDCT) usually arises in the dendritic reticulum cells of the lymph nodes. Extranodal cases are rare; only 24 cases have been reported in the head and neck region, and most are in the oropharynx. Nine cases of primary FDCT of the tonsil have been reported in English-language literature, to which we add the 10th case. This 77-year-old white woman presented with a tonsillar mass that was misdiagnosed as squamous cell carcinoma. She underwent neoadjuvant radiotherapy plus combined oropharyngeal and mandibular resection with radical neck dissection. Eight years later, she presented with dyspnea and was found to have a lung mass with hilar lymphadenopathy. A biopsy specimen was taken from the hilar lymph nodes, and histologic analysis yielded results similar to those found in the original tumor. Immunohistochemical analysis confirmed that FDCT was present at both sites. We wish to highlight this potential diagnostic pitfall because the treatment and prognosis for FDCT are vastly different from the treatment and prognosis for squamous cell carcinoma of the tonsil.     

Follicular dendritic cell sarcoma of the head and neck: case report and literature review

Objectives/Hypothesis: This article presents a case of a patient with follicular dendritic cell sarcoma (FDS), a rare neoplasm usually of the head and neck, and reviews the literature. Study Design: Literature review. Methods: A MEDLINE literature search was performed and the literature was reviewed. Results: Our patient presented with an FDS that had been excised from the upper neck and recurred in a level V node. He was treated with neck dissection and postoperative irradiation and remains disease free 5.25 years after salvage treatment. The literature search yielded 67 case reports on FDS of the head and/or neck. Most patients were treated with surgery (94%). Twenty-eight percent of patients received adjuvant radiotherapy; 18% received adjuvant chemotherapy. Fifty percent of patients were alive with no evidence of disease at last follow-up; 9% died from disease. Conclusions: We currently treat head and neck FDS with wide resection and postoperative radiotherapy.     

Oropharyngeal kaposi sarcoma in related persons negative for human immunodeficiency virus

OBJECTIVES: Kaposi sarcoma (KS) is a vascular tumor that can affect the mucosa of the upper aerodigestive tract. Although KS is the most common malignancy in patients with acquired immunodeficiency syndrome, it is rare in immunocompetent persons. We describe an unusual presentation of KS in 2 related individuals and describe our attempts to determine whether oropharyngeal KS is associated with human herpesvirus 8 (HHV-8). METHODS: All relevant clinical and surgical information, including information on tumor histopathologic and human immunodeficiency virus (HIV) serologic tests, was abstracted from the patient charts and electronic databases. HHV-8 immunohistochemistry was performed on paraffin-fixed specimens. RESULTS: Both patient 1 and patient 2 (the nephew of patient 1) were referred for workup of a tonsillar mass that was pathologically confirmed to be KS. In each case, HIV serologic testing was negative, and a screening immunologic workup, including a quantitative natural killer cell count, a B- and T-lymphocyte count, and immunoglobulin analysis, also yielded findings that were within normal limits. Immunohistochemistry performed on 1 pathological specimen showed positive staining for the presence of HHV-8, the etiologic agent of KS. CONCLUSIONS: The presence of oropharyngeal KS in 2 related HIV-negative individuals supports a role for genetic factors in susceptibility to KS, a common exposure to an infectious agent such as HHV-8, or both. Whereas most KS cases in industrialized countries are associated with immunodeficiency, clinical and laboratory data do not suggest that either of the patients described in this report are immunodeficient. Their susceptibility to KS may be secondary to a subtle inherited defect in host resistance to HHV-8, or another unknown factor.     

Upper airway obstruction due to tonsillar lymphadenopathy in human immunodeficiency virus infection

Head and neck manifestations of human immunodeficiency virus (HIV) infection are common and include diffuse cervical lymphadenopathy, cutaneous and mucosal Kaposi's sarcoma, mucosal herpes simplex infection, upper aerodigestivetractcandidiasis, and parotidlymphadenopathy and cysts. Recurrent otitis media and chronic sinusitis have been noted in the pediatric HIV population. We describe a patient with HIV-associated tonsillar and adenoid lymphadenopathy and upper airway obstruction. Pathologic analysis of the tonsillar tissue revealed severe lymphofollicular hyperplasia similar to that of other lymphoid tissue in HIV infection. The importance of symptomatic treatment of the airway obstruction is stressed.     

滤泡树突细胞肉瘤临床分析

目的:报道扁桃体滤泡树突细胞肉瘤(FDCS)1例,探讨其临床病理特点、诊断和鉴别诊断方法。方法:运用组织病理学、免疫组织化学及电镜观察扁桃体FDCS的特征,总结临床特点和治疗情况,并复习相关文献加以分析。该例患者于全身麻醉下行右侧扁桃体肿物低温等离子射频消融切除术。结果:术后随访2年局部无复发。结论:FDCS诊断主要依赖其免疫组织化学及电镜观察,该病极易误诊,运用低温等离子射频消融术治疗此种肿瘤需积累更多的临床经验。     

Synovial sarcoma of the cervical area

Synovial sarcoma is a malignant tumor which usually arises from the extremities. Involvement of the cervical area has been quite rare and to date only 12 cases have been reported. Two additional cases are reported in this paper and their management discussed. Case 1 is a 32-year-old white female with a submucosal mass of the right retromolar area displacing the tonsillar fossa and soft palate anteriorly. Surgical exploration revealed a well encapsulated mass separate from the parotid gland. Surgical excision was carried out on August 30, 1972. Pathological report was a synovial sarcoma. There has been no recurrence to date. Case 2 is a 34-year-old male with a 4 to 5 centimeter submandibular mass. Sialography and carotid arteriography were non-contributory. Biopsy showed synovial sarcoma. On May 12, 1973, surgical excision of the mass was carried out with the ipsilateral attached greater corneal of the hyoid bone and right hypoglossal nerve which ran into the tumor. Postoperative X-ray therapy was given. There is no recurrence to date. Discussion: Synovial sarcoma represents a type of mesenchymal tumor whose differention can mimic non-neoplastic synovial membrane. Histologically the tumor contains two neoplastic cell types. These are described as the fibrosarcomatous element and the synovial element. The tumor usually has no true capsule and grows by expansion with adherance to surrounding structures. The rate of growth is slow but the long term prognosis is poor. Metastasis usually occurs via blood stream most commonly to the lungs. Regional lymph node metastasis occurs in areas outside of the head and neck but has not been recorded in synovial sarcoma of the head and neck to date. Surgical excision is agreed to be the mode of treatment. The value of X-ray therapy is uncertain but Pack and Ariel were able to show their best results with a combination of surgical resection followed by postoperative X-ray therapy. It has been suggested that synovial sarcoma of the head and neck may behave in a more benign manner than synovial sarcoma elsewhere. Long term follow-up is not available in enough of the cervical sarcoma cases to determine if this is true. Until a more benign behavior is proven the tumor should be treated as an aggressive malignancy. Wide surgical excision is the treatment of choice. Postoperative X-ray therapy should be considered.