Primary Kaposi’s sarcoma of the nasal cavity not associated with AIDS

Kaposi’s sarcoma (KS) is an unusual vascular tumor characterized by multiple reddish blue nodules, which usually present on the skin of the lower and upper extremities. KS may also involve mucosal sites, lymph nodes and visceral organs. During the last two decades, with the large increase in the incidence of this tumor associated with the acquired immune deficiency syndrome (AIDS), there have been increasing number of cases with KS presenting on the skin or mucosa of the head and neck. A review of the literature revealed that only six cases of primary KS of the nasal cavity have previously been published and only one of them presented in a patient not associated with AIDS. We report the case of a 59-year-old woman who presented 4 years ago with nasal obstruction and intermittent minor epistaxes. Physical examination revealed the presence of a fleshy tumor arising from the left nasal septum, which was excised. Histological examination of the tumor showed morphological and immunohistochemical features of KS. A complete physical and laboratory examination revealed no other pathological findings. The patient received no further treatment and 4 years later, she is in excellent condition. In the present study, we report the second case where the primary manifestation of the KS was in the nasal cavity in a patient with an adequate immune system.     

Granulocytic sarcoma of the nasal cavity

Granulocytic sarcoma (GS) is a rare localised tumour of malignant myeloid precursor cells occurring at an extramedullary site. It is usually associated with a myeloproliferative disorder but may be seen preceding the onset of leukemia. Extracranial GS may occur virtually anywhere in the body and may be easily confused with large cell and lymphoblastic lymphoma. This paper reports an unusual case of primary GS of the nasal cavity and paranasal sinuses, which presented as a nasal obstruction. Multidrug combination chemotherapies and bone marrow transplantation were performed. Awareness of the potential location of GS in the nasal cavity and paranasal sinuses should contribute to better define the prognostic significance of this uncommon entity.     

A case with sinonasal teratocarcinosarcoma in the nasal cavity and ethmoid sinus

Sinonasal teratocarcinosarcoma (SNTCS) is a rare and aggressive malignant neoplasm histologically characterized by the combination of one or more epithelial elements and mesenchymal components. We report a 61-year-old man with SNTCS involving left nasal cavity and ethmoid sinus. He complained of left epistaxis for 1 week. Computed tomography and magnetic resonance imaging revealed a soft tissue filling the left middle meatus and ethmoid sinus, and effusion in the left sphenoid sinus but no invasion to the orbit or skull base. Tumor was completely removed with lateral rhinotomy, and post-operative radiation therapy (Liniac 60 Gy) was performed. Follow-up examination for 3 years and 5 months after the radiation therapy has shown no evidence of recurrence or metastasis. This report describes his clinical course, etiology, diagnosis and management of SNTCS with a review of literature.     

鼻腔鼻窦低度恶性肌纤维母细胞肉瘤一例

正1病例资料患者,女,51岁。因"反复鼻阻5月余,加重2月余"于2017年2月3日入院。既往史:2007年行鼻中隔矫正术,2012年行左鼻息肉手术,余无特殊。入院专科情况:外鼻无畸形,双侧鼻腔见大量样新生物堵塞(图1),鼻中隔后份骨质破坏,右侧鼻腔新生物来源于左侧鼻腔,表面血供丰富,鼻腔未见明显分     

Phosphaturic mesenchymal tumor of the nasal cavity and paranasal sinuses: A clinical curiosity presenting a diagnostic challenge

Phosphaturic mesenchymal tumor (PMT) is a rare mesenchymal neoplasm associated with tumor-induced osteomalacia (TIO) and elevated serum FGF-23. Common in extremities, PMT rarely occurs in sinonasal region. We report a series of sinonasal PMT diagnosed at our institute over a 6-year period.Six cases of sinonasal PMT were identified during this period, of which five presented with features of TIO. Median age of patients was 45.5 years. All six tumors were composed of stellate to spindled cells, with prominent staghorn vasculature in four cases. Typical smudgy matrix was seen in all cases, but only focally; grungy calcification was absent.Accurate diagnosis of PMTs is imperative, as complete excision leads to dramatic resolution of TIO symptoms. Lack of knowledge of this entity prevents clinicians from ordering relevant investigations. Absence of specific morphological features, like grungy calcification, and presentation at atypical locations makes the diagnosis challenging. Awareness of this entity is essential in order to suspect PMT in patients presenting with a soft tissue mass and features of TIO, however unusual the location may be.     

鼻腔、咽部非霍奇金氏淋巴瘤

目的 :探讨鼻腔、咽部非霍奇金氏淋巴瘤 ( NHL)的临床特征、诊断要点及误诊原因。方法 :对1 986年～ 1 996年住院的 2 4例鼻腔、咽部非霍奇金氏淋巴瘤的患者作一回顾性分析。结果 :2 4例临床表现差异较大 ,缺乏特征性的表现。首次诊断 NHL6例 ,其余 1 8例初诊时诊断 :慢性鼻炎 8例 ,恶性肉芽肿3例 ,鼻息肉 2例 ,鼻中隔粘膜肥厚 2例 ,扁桃体炎 2例 ,慢性咽炎 1例。 2 4例均经病理切片及免疫组化确诊。结论 :鼻腔、咽部非霍奇金氏淋巴瘤临床表现复杂 ,缺乏特征性 ,早期易误诊。提高临床医师对本病的认识 ,反复活检及免疫组化等方法有利于本病的确诊。     

鼻腔尤文肉瘤一例

患者女,40岁.因右侧鼻塞伴右眼流泪2个月于2006年9月8日入院.无涕中带血、视力模糊、头痛、面部麻木、牙齿疼痛及嗅觉减退症状.体检:鼻外形无明显畸形,鼻中隔稍偏左,右侧鼻腔总鼻道及下鼻道充满红色新生物,中鼻道少量黏性分泌物,左侧下鼻甲、中鼻道未见明显异常.鼻窦CT示右侧鼻腔可见大小约2.8 cm×2.5 cm的肿块,右侧下鼻甲及卜颌窦内侧壁骨质部分吸收(图1).     

原发性鼻腔鼻窦骨外尤文肉瘤/外周原始神经外胚层肿瘤2例并文献复习

目的 分析原发性鼻腔鼻窦骨外尤文肉瘤/外周原始神经外胚层肿瘤的发病特点,为该病的诊疗提供参考。 方法 回顾性分析2014年3月至2019年7月收治入院的2例鼻腔鼻窦骨外尤文肉瘤/外周原始神经外胚层肿瘤患者的临床资料,并检索2006年至2021年国内外相关文献,共48例(含本文2例)患者纳入研究,总结临床表现、治疗方法及预后。 结果 本组患者7~83岁、中位数26岁,男女比例1∶1.5,以鼻部症状为主,鼻塞16/28(57.1%)、鼻出血/涕中带血12/28(42.9%)、头痛3/28(10.7%)、嗅觉减退3/28(10.7%)。病变侵犯部位:鼻腔32/48(66.7%)、上颌窦24/48(50.0%)、筛窦27/48(56.3%)、蝶窦8/48(16.7%)、额窦3/48(6.3%),侵袭眼眶17/48(35.4%)、颅底6/48(12.5%)。单因素分析:综合治疗(P=0.009)、放疗(P=0.014)可明显改善患者预后,病变侵犯蝶窦(P=0.027)、颅底(P=0.002)提示患者预后不良,多因素COX回归分析示侵犯颅底为预后的独立危险因素(P=0.008),与未侵犯颅底的患者相比,侵犯颅底的患者死亡风险更高,HR=8.940(95%CI:1.756~45.506)。 结论 鼻腔鼻窦骨外尤文肉瘤(Extra-skeletal Ewing Sarcoma, EES)好发于青年女性,症状以鼻塞、鼻出血/涕中带血多见,病变范围主要累及鼻腔、上颌窦、筛窦,可侵及眼眶、颅底,病变侵犯颅底为死亡的独立危险因素,治疗以化疗联合放疗的综合治疗为主。     

鼻腔原发性粒细胞肉瘤一例

患者女,57岁,2007年4月因双侧鼻塞反复发作1年余入院.查体:鼻中隔左偏,双侧下鼻甲充血、肿胀,双侧中鼻道未见息肉样物及分泌物.行鼻窦CT检查示:左侧上颌窦、双侧筛窦炎症,双侧下鼻道见软组织密度影,阻塞右侧后鼻孔(图1a).入院诊断:"慢性鼻-鼻窦炎(Ⅰ型2期)、慢性肥厚性鼻炎".     

48例鼻腔鼻窦肉瘤的临床分析

目的：探讨鼻腔鼻窦肉瘤的临床表现、诊断、治疗及预后，以提高对鼻腔鼻窦肉瘤的诊疗水平。方法：回顾性分析48例鼻腔鼻窦肉瘤患者的临床资料。结果：鼻腔鼻窦纤维肉瘤4例，恶性纤维性组织细胞瘤3例，脂肪肉瘤4例，平滑肌肉瘤5例，横纹肌肉瘤5例，骨肉瘤2例，软骨肉瘤3例，恶性黑色素瘤4例，非霍奇金淋巴瘤4例，浆细胞肉瘤1例，恶性神经鞘膜瘤5例，嗅神经母细胞瘤5例，血管肉瘤3例，综合治疗后随访，1，3，5年生存率分别为62．5％，46．7％，35．7％。结论：鼻腔鼻窦肉瘤不易明确诊断，结合免疫组织化学有助于诊断，对肿瘤易复发及全身转移者，综合治疗可提高生存率。     

Primary synovial sarcoma arising in the parotid region diagnosed by fluorescence in situ hybridization

Synovial sarcomas account for 5% of pediatric soft tissue sarcomas, and primarily arise in the extremities. We report a case of synovial sarcoma in the parotid region. A 29-year-old woman presented with a 3-year history of a slow-growing, slight painful swelling in the preauricular region. The patient underwent right deep parotidectomy preserved with facial nerve. In immunohistochemistry, the epithelial cells were positive for cytokeratin AE1/3 and epithelial membrane antigen. The stromal cells were immunoreactive for vimentin and BCL2. The both types of cells were positive for CD99. The SYT-SSX fusion gene from chromosomal translocation was detected by fluorescence in situ hybridization in this case.     

儿童筛窦尤文氏肉瘤1例并文献复习

目的 探讨儿童鼻窦尤文肉瘤(ES)的临床特征、诊疗方法及预后。方法 回顾性分析1例筛窦ES的临床资料,并结合相关文献进行讨论。结果 该患者为原发性筛窦ES,术前诊断缺乏特异性临床表现和影像学特征。最终通过组织病理及免疫组织化学确诊。患者接受手术联合化疗、放疗的综合治疗,随访8个月无复发和转移。结论 儿童筛窦ES极罕见,临床表现无特异性,病理特征与鼻腔其他小圆细胞肿瘤相似,建议采用免疫标记物CD99+NKX2.2的方法诊断。鼻窦ES多采用综合治疗方案,预后较好。     

Alveolar soft part sarcoma of the tongue

Alveolar soft part sarcoma is a rare malignancy. To our knowledge, 13 cases in the head and neck have been reported previously. It occurs more commonly in the extremities, where it is associated with a poor prognosis. Its clinical course resembles that of adenoid cystic adenocarcinoma in that late metastases often cause death in 10 to 15 years. A 5-year-old girl with alveolar soft part sarcoma was successfully treated with partial glossectomy and is free of disease at two-year follow-up.     

Schwannoma of the nasal septum: Rare presentation and literature review

Schwannomas are benign primary tumors of the Schwann cells of the nerve sheath known to occur throughout the body. We present a unique case of a schwannoma arising from the nasal septum in a healthy 16-year-old adolescent boy. The patient had approximately two months of left-sided nasal obstruction after sustaining a lacrosse injury to the nose. Imaging revealed a nonspecific soft tissue mass, which after nasal endoscopy and excision was revealed to be a schwannoma. We discuss our results as well as present a review of the existing literature.