副神经节瘤伴腰椎转移1例并文献复习

Paraganglioma and phaeochromocytoma are neoplasms of chromaffin and non-chromaffin tissues found within the autonomic nervous system-associated paraganglia, which could occur as benign or malignant tumor ubiquitously. Paraganglioma often occurs in the retroperitoneal space, which accounts for 1.88 percent of the primary retroperitoneal tumor[1]. We report a case of paraganglioma which was incorrectly diagnosed as adrenal carcinoma preoperatively, but the frozen section analysis and clinical manifestation revealed a malignant paraganglioma.     

马尾区副神经节瘤

马尾区副神经节瘤(Paragangiioma of the cauda equina region)于1970年首先由Miller和Torack以“分泌性室管膜瘤”一名报道,1972年Lerman等首次以神经节细胞神经瘤—副神经节瘤命名。近10年此瘤才逐渐被人们所认识。迄今,国外文献报道近70例,其中除一组为31例报道外,余均为个案或几例报道,足见此瘤罕见。另外,病理诊断上此瘤易误诊为室管膜瘤,此瘤生物     

副脊索瘤3例报道并文献复习

目的探讨副脊索瘤临床病理学特点及其诊断与鉴别诊断。方法对报道的3例副脊索瘤进行临床资料分析、组织学形态和免疫表型观察并复习相关文献。结果副脊索瘤好发于四肢深部软组织。大体呈分叶状或多结节状,瘤细胞圆形或多边形,呈条索状或小巢状排列,胞质内含有空泡,间质富有黏液基质。免疫组化标记:CAM 5.2、EMA、S-100、SMA、ColⅣ阳性表达,CEA、CK19、Des阴性。结论副脊索瘤是一种少见的软组织肿瘤,组织学形态特点是其诊断的主要依据,免疫组化标记有助于诊断和鉴别。     

肋缘肌间恶性血管球瘤1例报道并文献复习

目的 探讨恶性血管球瘤(malignant glomus tumor,MGT)的临床病理特点、诊断和鉴别诊断.方法 对1例发生在右侧第11肋弓处的MGT进行组织学和免疫组化观察,结合文献复习.结果 右侧肋缘下肿物大小6 cm×7 cm,质韧,可活动,边界清晰,无压痛,放射痛.上腹部增强CT:右侧第11肋弓处可见一软组织肿块影,边界光滑,其内密度不均匀,中心可见斑片状低密度影,大小5.8 cm×4.17 cm,局部肋骨可见斑片状骨质破坏.光镜下肿瘤细胞围绕裂隙状血管弥漫性生长并浸润周围脂肪组织.瘤细胞大小较一致,圆形或多角形,胞质丰富,淡红染,细胞界限清晰.核圆形或卵圆形,染色质粗糙,核仁明显,有异型,核分裂9个/10 HPF,可见病理性核分裂.肿瘤组织间纤维间质少,有丰富扩张的小血管,可见多灶坏死和出血.免疫表型:肿瘤细胞表达SMA、h-caldesmon、vimentin、p53、BCL-2、CD99、Collagen IV和Ki-67,p63、HMB-45、CK、EMA、desmin、S-100、CD34、TTF-1、calretinin、CD68、Lysozyme、CD3、CD20、CD30为阴性.结论 MGT是罕见的软组织恶性肿瘤.确诊必须依赖于临床特点、组织形态及免疫组化结果,且需要和具有血管周上皮样细胞分化特点的肿瘤及血管外皮细胞瘤等其他肿瘤予以鉴别.     

副神经节瘤

副神经节瘤是发生在副神经节的肿瘤,一般分布与副神经节的分布相当。肾上腺髓质是一种特殊的副神经节,故一般将肾上腺髓质发生的肿瘤称为嗜铬细胞瘤,而发生在肾上腺外的副神经节瘤称为肾上腺外副神经节瘤。副神经节瘤可分为交感神经副神经节瘤和副交感神经副神经节瘤两大类。     

原发性指骨骨髓腔内血管球瘤1例并文献复习

目的 探讨血管球瘤(glomus tumor,GT)的临床病理特点、诊断和鉴别诊断.方法 对1例发生在指骨骨髓腔内的GT进行组织学和免疫组化结果观察,结合文献复习,分析GT的临床表现、影像学特征、病理学形态特点和免疫学表型.结果 左拇指末节肿胀隆起,约4.5 cm×3.5 cm,X线提示左拇指远节远端骨髓腔内低密度影.光镜下肿瘤组织由小血管及周围的肿瘤细胞组成,瘤细胞丰富,圆形、卵圆形,形态较一致,胞质丰富红染或透亮,无明显异型性和核分裂象,间质明显黏液变性、水肿.免疫表型:肿瘤细胞表达SMA、MSA、vimentin、Ⅳ型胶原,EMA、CK、S-100、HMB-45、desmin、calponin、CgA、Syn均阴性.结论 血管球瘤是少见的软组织肿瘤,一般位于正常血管球细胞所在的部位,如甲床下或皮下浅表软组织.发生于骨髓腔内的血管球瘤非常罕见.确诊必须依赖于临床特征、组织形态及免疫组化结果相结合,且需要和其他肿瘤予以鉴别.     

气管血管球瘤2例并文献复习

目的 探讨气管血管球瘤(glomus tumor,GT)的临床病理特征、诊断及鉴别诊断.方法 对2例罕见的气管GT进行临床、病理组织学形态及免疫组织化学染色观察,并结合文献进行探讨.结果 气管GT临床缺乏特征性,纤维支气管镜及CT检查示气管内新生物.镜下细胞形态具有特征性,细胞大小一致,细胞圆形或卵圆形,细胞质嗜酸性,细胞核稍大,位于细胞中央,未见核分裂,其间见大量扩张的薄壁血管,肿瘤细胞围绕血管周围排列.免疫表型:vimentin、actin、SMA均(+).结论 发生于气管的GT极其罕见,由于其缺乏特异性的术前诊断依据极易误诊,确诊需依赖于病理学检查,免疫组织化学染色有助于诊断及鉴别诊断.     

颈静脉孔区神经鞘瘤（附1例报告及文献复习）

颈静脉孔区神经鞘瘤为颅底少见肿瘤,手术难度较大。Maniglia等在1979年复习文献资料报道了56例,Kaye等在1984年报道了经手术治疗的13例,还有些证实为颈静脉孔区神经鞘瘤的零星报道。我科于1998年3月份收治一例颈静脉孔区巨大神经鞘瘤,现就其临床诊断、手术及术后处理结合文献报道如下。     

13例副神经节瘤病理形态及免疫组化研究

副神经节瘤(Paraganglioma)起源于神经嵴细胞,主要分布于头、颈、胸和腹膜后等有副神经节分布的部位。1969年Szijjct等首先提出了APUD瘤概念后,不少学者发现Paraganglioma具有与之相似的组织化学和超微结构特征,因此将此瘤归属APUD瘤。由于Paraganglioma较为少见,文献中对它的命名、分型不一,以致概念模糊。为此我们收     

Glomus tumor of the trachea: a rare case report

A tracheal glomus tumor is extremely rare. There were approximately 18 reported cases before in China. Here we report a 48-year-old male with glomus tumor of the trachea. The computed tomography (CT) scan of the chest showed a nodulein the basalsegmentof right lower lobe, focal uplift in the left-posterior wall of the trachea, measured 1.5×1.2×1.0 cm. Microscopically, the tumor tissue was rich with vessels, shaped flake-nest, and the tumor consisted of a sheet of uniform cells surrounding the vascular spaces. Immumohistochemical staining were positive for SMA, vimentin, collagen IV, CD34, the Ki-67 proliferation activity was low (<1%), and were negative for Syn, CgA, S-100, AE1/AE3 and EMA. PSA staining showing clear cell borders. With a review of the literature, the clinical, pathological characteristics and treatment modalities of this rare tumor are discussed.     

Glomus tumor of renal pelvis: a case report and review of the literature

Glomus tumors are uncommon benign perivascular neoplasms that have rarely been described outside of their usual peripheral soft tissue sites. We report a unique case of glomus tumor of the renal pelvis in a 53-year-old woman who presented with microscopic hematuria associated with obstruction of the ureteropelvic junction and marked hydronephrosis. At initial gross examination, the tumor mimicked a urothelial carcinoma.     

Pulmonary gangliocytic paraganglioma: a case report and review of the literature

Gangliocytic paraganglioma (GP) is a rare histologic type of neuroendocrine tumors. We report a case of pulmonary GP in a 29-year-old male presenting with an asymptomatic endobronchial nodule. Grossly, the tumor showed a 4.0x3.8x3.5 cm well-defined nodule with yellowish cut surface. Microscopically, the tumor was composed of three distinct cellular types: epithelioid cells, ganglion-like cells and spindle cells. Meanwhile, transitional cells, having morphologic features between ganglion-like and epithelioid cells, were also presented. The epithelioid cells arranged in various morphologic architectures, including Zellballen, papillary, cystic and microcystic pattern. The epithelioid cells were positive for AE1/AE3, CAM 5.2, chromogranin A and synaptophysin. Ganglion-like cells showed immunoreactivity for chromogranin A and synaptophysin. A few ganglion-like cells were also positive for AE1/AE3 and/or CAM 5.2. The spindle cells were positive for S-100 protein and neurofilament. The transitional cells showed a similar immunohistochemical profile to the epithelioid cells. The authors believe stem cell theory is a reasonable explanation for the origin of GP. GP probably originate from some kind of mucosa associated stem cell which can differentiate into diverse cellular lineages.     

肾素瘤1例临床病理及文献复习

目的：阐述肾素瘤的病因，病理特征及鉴别诊断要点。方法：对１例肾素瘤进行大体、光交易、免疫组化镜观察，并复习有关文献。结果：肾素瘤包膜完整光下酪似血管外皮瘤；免疫线化，部分细胞Ｖｉｍｅｎｔｉｎ呈阳性反应，瘤组织的腺样及乳头样结构区瘤细胞ｃｙｔｏｋｅｒａｔｉｎ呈阳性反应。电下瘤细胞胞浆内可见多量圆形分泌颗粒及棱莆和梯形结晶样物。结论：肾素瘤是发生于变更的肾小球入球小动脉血管平滑肌细胞的罕见衣性肿瘤，明     

Glomus tumor of uncertain malignant potential of the lung: a case report and review of literature

Glomus tumor is an uncommon tumor usually presenting in the dermis. Rarely, it occurred in visceral organs including stomach, liver and long. The majority of glomus tumors were benign. Herein, we present a case of glomus tumor located in the left lobe of the lung in a 49 year-old Chinese male. An irregular mass measuring 3 cm was detected by imaging examination because of his suffering from cough, dyspnea and chest pain. Histologically, the tumor is composed predominantly of sheets of ovoid to round cells with clear border, pale cytoplasm and fine granular chromatin. The mitotic count was less than 5 per 50 HPF. The tumor focally invaded the surrounding normal bronchial and alveolar tissue. Immunohistochemical staining showed that the cells were diffusely positive for SMA, caldesmon, and vimentin. The Ki-67 proliferation index was approximately 20%. Based on morphologic features and the immunohistochemical profile, the tumor was consistent with glomus tumor of uncertain malignant potential.     

Malignant rhabdoid tumor of the liver: case report and literature review

A case of malignant rhabdoid tumor (MRT) occurring as a primary hepatic neoplasm in a 12-month-old Japanese female infant is presented. The patient had a slight fever for 2 weeks and presented with a palpable mass in her left hypochondrial region. After admission, the hepatic artery was embolized due to intra-abdominal hemorrhage arising from the tumor. The patient received chemotherapy with cisplatin, cyclophosphamide and adriacin. Despite treatment, the patient developed dyspnea, pancytopenia and disseminated intravascular coagulation. Rupture of the tumor resulted in death within 3 weeks. A limited abdominal autopsy revealed that the liver weighed 1240 g and was occupied by multiple hemorrhagic and/or necrotic tumor nodules. Histologically, neoplastic cells had an abundant eosinophilic cytoplasm containing paranuclear inclusions, and vesicular nuclei with a centrally located prominent nucleolus. Ultrastructurally, the cytoplasmic inclusions were composed of whorled filaments measuring 10 nm. Immunohistochemically, almost all of the neoplastic cells were positive for vimentin and cytokeratins (CK) 8 and 18, some were positive for CK 7 and 19, while none were positive for CK 1, 10, 13-17 and 20. The tumor cells did not express desmin, myoglobin, and alpha-fetoprotein. We found 18 cases of MRT of the liver published in English language literature and then, adding the present case, we summarized the 19 cases. Hepatic MRT is an uncommon neoplasm. However, it should be considered in the differential diagnosis of an aggressive liver neoplasm in childhood.     

Epithelioid trophoblastic tumor after induced abortion with previous broad choriocarcinoma: a case report and review of literature

Epithelioid trophoblastic tumor (ETT) is a rare trophoblastic tumor originating from chorionic-type intermediate trophoblasts (ITs). It is usually associated with a prior gestational event. We present a 44-year-old woman who had unusual pregnancy related history. The patient received her second spontaneous abortion at the age of 25 years and had suffered from choriocarcinoma in left board ligament at the age of 29 years. She admitted no more treatment after 3 courses of multiagent chemotherapy when serum β-hCG returned to normal. Then she had Full-term delivery, induced abortion at the ages of 32, 33 years. The patient had high serum levels of beta-human chorionic gonadotropin (6587 IU/L). Microscopically, the tumor was composed of mainly mononuclear tumor cells, grew in cords, nests, and sheets within which were aggregates of hyaline material. Most were with distinct cell borders, eosinophilic cytoplasm. Immunohistochemical staining revealed strong diffuse reactivity for cytokeratins (AE1/AE3, CK18), P63, focal reactivity for beta-human chorionic gonadotropin, human placental lactogen, and inhibin-alpha. The Ki-67 index was 77%. The histological and immunohistochemical features were characteristic of epithelioid trophoblastic tumor. This is the first reported case of these two gestational trophoblastic tumor happened on one person with the intervening normal pregnancy.     

肾恶性孤立性纤维性肿瘤1例报道及文献复习

目的 探讨肾脏恶性孤立性纤维性肿瘤的临床病理特征、鉴别诊断及治疗和预后.方法 报道1例肾恶性孤立性纤维性肿瘤的临床病理资料并复习文献.结果 患者男性,56岁.临床主要表现为乏力、出汗、心慌、气短1年,以低血糖原因待查入院.CT示左肾上方及左肾前下方见3个类圆形软组织密度影,与肾分界不清,大小分别为9 cm×8 cm×7 cm、7 cm×7cm×7 cm和4 cm×4 cm ×4 cm.增强后边缘轻度强化,中心不均匀强化.临床、CT检查考虑为左.肾癌.行左肾癌根治术.眼观:左肾上、下极分别见一肿块,切面灰白色,部分区见有坏死,与肾界限不清.镜检:肿瘤由短梭形或卵圆形细胞组成,排列成束状、旋涡状或不规则状.部分区域血管丰富,细胞密集呈血管外皮瘤样结构;部分区域细胞稀疏伴纤维化、黏液变性.肿瘤超过60%的区域内(尤其是肾下极肿块)瘤细胞丰富密集,有异型性,核分裂象多见(>4个/10 HPF),边缘呈浸润性生长,部分区可见出血坏死.免疫表型:肿瘤细胞vimentin、CD34弥漫阳性,CD99、bcl-2阳性,Ki-67增殖指数>20%.病理诊断:左肾恶性孤立性纤维性肿瘤(低度恶性).术后随访24个月情况良好.结论 肾恶性孤立性纤维性肿瘤非常罕见,确诊主要依靠病理形态学及免疫组织化学标记,并应与肾的其他梭形细胞肿瘤进行鉴别.治疗以手术完全切除为主,并注意长期随访.     

婴儿色素性神经外胚瘤1例报道并文献复习

目的 探讨婴儿色素性神经外胚瘤的临床病理特征、免疫组化、诊断和鉴别诊断要点。方法 对1例婴儿色素性神经外胚瘤进行组织学和免疫组化观察和文献复习。结果 婴儿色素性神经外胚瘤好发于1岁以内的婴儿,肿瘤多见于上颌骨和颅骨,表现为浸润性和溶骨性破坏。组织学上显示大的并含不等量色素颗粒的上皮样细胞和小的神经母细胞样细胞。免疫组化显示CK、HMB-45、S-100蛋白、NSE在上皮样细胞呈阳性表达,小圆形瘤细胞S-100蛋白、NSE阳性或部分阳性。肿瘤彻底切除,随访3年未发现转移和复发。结论 婴儿色素性神经外胚瘤是一种少见的起源于神经嵴细胞的肿瘤,具有特征性的临床病理改变,需要和神经母细胞瘤、恶性黑色素瘤及其它小圆细胞肿瘤鉴别,生物学行为属于潜在恶性或低度恶性肿瘤,彻底切除预后良好。