肺原发性腺泡状软组织肉瘤1例

患者女性,48岁,体检发现右下肺包块,无咳嗽和胸痛病史.胸部X线、CT示:右下肺见大小为8 cm×7 cm×5 cm的肿块,边界清楚,考虑肺癌.四肢未见明显包块,无手术史.行肿块切除术,肿块送病理检查.     

肺原发性腺泡状软组织肉瘤1例

<正>患者女性,30岁,因反复咳嗽、咯血1个月,症状加重3天入院。既往无特殊。胸部CT示右肺上叶见一结节密度增高影,大小2. 3 cm×1. 6 cm,边界较清楚(图1),影像学诊断:右肺上叶结节影,考虑良性血管性病变可能性大,肺动静脉畸形?临床诊断:右上肺结节:肺动静脉畸形?完善相关检查后行右上肺叶切除+胸膜粘连烙断+隔神经麻痹术。术中见肿物位于右上肺,靠近中肺叶裂交界处,肿物质软,边界不清,大小2. 5 cm×2 cm,肺门纵隔未见肿大淋巴结。     

纵隔腺泡状软组织肉瘤1例

纵隔腺泡状软组织肉瘤１例关键词纵隔肿瘤，腺泡状软组织肉瘤中国图书分类号Ｒ７３４．５０２李玉军患者；女，２１岁。胸痛、胸闷半年。胸片及ＣＴ发现前纵隔肿物，大小约１０ｃｍ×７，５ｃｍ×６．２ｃｍ，密度较高，边界欠清。临床诊断恶性胸腺瘤，于１９９２年７月６...     

腺泡状软组织肉瘤1例

腺泡状软组织肉瘤１例屈传贵张丽△刘艹倩患者女，３０岁。因左大腿肿块复发２个月，于１９９５年４月１８日门诊手术切除。６年前发现局部枣状大小肿块，１年半前在当地予以切除，肿块鸡蛋大小。本次手术见肿瘤位于股四头肌内，中等硬度，与周围组织粘连。术后随访１２个...     

口腔腺泡状软组织肉瘤2例

口腔腺泡状软组织肉瘤２例宋晓俊王三锡例１：患者女性，２６岁。舌根部肿物１月余。初起时为一蚕豆大小的无痛性包块，不影响进食吞咽，在当地医院做肿物切除活检，病理报告“舌根颗粒细胞肌母细胞瘤”。一月后复发，且生长加快，吞咽时微痛。局部检查：左舌根部可见２．...     

下颌骨腺泡状软组织肉瘤一例

患者男 ,2 1岁。左下颌骨无痛性肿块渐进性增大 2年 ,近 3个月肿块生长加速、左下后牙松动并在当地医院拔除 ,病情无好转并出现下唇麻木 ,于 1998年 1月 14日入院。体检 :左下颌骨 |3至下颌角区可触及一约 5ｃｍ× 4ｃｍ大小肿块 ,粘膜正常 ,张口受限 ,|4 8区颊侧骨性膨隆、骨质变薄、触之有乒乓感、无压痛 ,|7缺失。未发现其他异常。Ｘ线检查 :左下颌体见 5ｃｍ× 4ｃｍ密度减低影 ,其内可见骨间隔。 |6根尖吸收。临床诊断 :左下颌骨成釉细胞瘤。行左下颌骨切除加髂骨移植术。病理检查 :送检为 |2至同侧升支之下颌骨切除标本 ,于|3至同侧…     

腺泡状软组织肉瘤1例并文献复习

目的阐明高血糖诱导对阿尔兹海默症 (Alzheimer''s Disease,AD) 样病理改变的潜在分子机制。方法选取18 周龄的 Sprague Dawley (SD) 大鼠 30 只,将其随机分为对照组 (Control)、模型组 (STZ+HFD) 以及模型+AMPK 激动剂 AICAR (5-Aminoimidazole-4-carboxamide1-β-D-ribofuranoside) 组 (STZ+HFD+AICAR),每组均为 10只大鼠。模型组在高脂饮食 (High-Fat Diet,HFD) 喂养的基础上,经腹腔注射的方式给予大鼠 50 mg/kg 链脲佐菌素 (Streptozotocin,STZ) 建立 2 型糖尿病 (Type Ⅱ Diabetes Mellitus,T2MD) /高血糖模型,STZ+HFD+AICAR 组另给予大鼠 100 mg/kg 的 AICAR, Control组给予常规饲料喂养并给予等量的柠檬酸钠缓冲液腹腔注射作为对照。8周内连续测量大鼠空腹血糖,8周后对各组大鼠进行糖耐量检测。通过Morris水迷宫实验分析各组大鼠认知功能的变化。采用Western Blot方法检测三个实验组大鼠脑组织AMPK/SIRT1通路分子、CDK5和H3acK9的表达变化。脑组织免疫组织化学染色检测AD特征性分子病理蛋白MAPT/ Tau的磷酸化活性。结果与Control组相比,8周高脂喂养联合STZ注射3天后,大鼠空腹血糖于第2周开始升高直至第8周 (P＜0.05),且 8周后糖耐量显著受损 (P＜0.05),血清胰岛素水平显著升高 (P＜0.01)。STZ+HFD大鼠的水迷宫定位航行试验逃避潜伏时间明显延长 (P＜0.05),而水迷宫空间探索时间显著降低 (P＜0.05)。Western Blot 结果显示,与 Control 组相比,STZ+HFD 大鼠脑组织磷酸化的 AMPK 蛋白以及 SIRT1蛋白的表达水平显著降低,而 H3acK9和 CDK5蛋白表达水平显著升高。脑组织免疫组织化学结果显示,Tau蛋白磷酸化水平显著升高。AICAR可部分改善脑组织分子病理改变,如：增加磷酸化 AMPK 蛋白以及 SIRT1 蛋白的表达,减少 H3acK9 和 CDK5 蛋白表达,进而降低 Tau 蛋白磷酸化水平。结论 AMPK/ SIRT1通路失活促进去乙酰化蛋白H3acK9及CDK5的表达,进而引起Tau蛋白活性增加,该机制是高血糖引起脑组织AD样分子病理改变的潜在机制之一 。     

腺泡状软组织肉瘤的临床病理分析

目的：观察腺泡状软组织肉瘤的临床及病理形态学特点,并探讨其组织发生方法：对12例腺泡状软组织肉瘤进行光镜观察,并对全部病例做组化及免疫组化染色。结果：12例腺泡状软组织肉瘤,男性4例,女性8例,发病年龄16－38岁（平均24岁）。发生部位主要位于四肢尤其是下肢的深部软组织,与骨髓肌关系密切。镜下瘤细胞呈胞泡状排列,周围为富含血窦的间质,瘤细胞质内含有丰富的嗜酸性颗粒,PAS全部阳性,免疫组化：SMA阳性率75％,desmin阳性率66．6％,myoglobin阳性率25％。结论：支持腺泡状软组织肉瘤的组织发生为横纹肌来源或向横纹肌分化。     

右大腿腺泡状软组织肉瘤1例

患者女性,36岁,因右大腿内侧包块伴疼痛数月余入院.术中见内收肌内有一3 cm×2 cm×1.5 cm大小包块,质韧,边界清楚.临床行"右大腿肌纤维瘤"切除术,包块切除后送病理检查.     

21例腺泡状软组织肉瘤的临床病理分析

目的探讨腺泡状软组织肉瘤的临床病理学特征及其鉴别诊断。方法对21例ASPS的临床资料进行回顾性分析,对标本进行组织病理学观察及免疫组织化学研究。结果21例腺泡状软组织肉瘤,男性11例,女性10例,发病年龄4～56岁,平均25.9岁,发病部位主要位于下肢深部软组织内,镜下肿瘤细胞排列成腺泡状或实性,细胞巢之间可见窦状血管分隔,瘤细胞胞质内含丰富的嗜酸性颗粒。PAS染色,瘤细胞胞质内可见棒状结晶体,免疫组化:MyoD110例阳性,desmin4例阳性,S-1009例阳性,NSE11例阳性,Vim11例阳性,AE1/AE3、CK、EMA、SMA、MSA、Syn全部为阴性。结论ASPS是多见于青少年和青年的罕见肿瘤,但多数早期出现血液转移,切除后易复发,最终预后欠佳,结合临床病理学特征及免疫组化,可作出正确诊断。     

Primary alveolar soft part sarcoma of the lung

Alveolar soft part sarcoma (ASPS) is a rare epithelial-like soft tissue sarcoma. The two main sites of its occurrence are the lower extremities in adults and the head and neck in children. Primary pulmonary involvement of this sarcoma, without evidence of soft tissue tumor elsewhere, is very exceptional. We present a case of primary ASPS of the lung in a 42-yr-old woman. A computed tomographic scan of the thorax demonstrated a well-circumscribed, solid tumor located in the right upper lobe. The mass was resected by right upper lobectomy. After 5 months, three metastatic lesions, involving lumbar vertebrae and occipital scalp, were found. Histologically, the tumor consisted of alveolar nests of large polygonal tumor cells, the cytoplasm of which frequently revealed periodic acid-Schiff-positive, diastase-resistant intracytoplasmic rod-like structures. On immunohistochemical staining, the tumor cells were positive only for vimentin and alpha-smooth muscle actin. Ultrastructural study using electron microscopy revealed characteristic electron-dense, rhomboid intracytoplasmic crystals.     

Primary intraosseous alveolar soft part sarcoma: Report of two cases with radiologic-pathologic correlation

Alveolar soft part sarcoma (ASPS) accounts for less than 1 % of all soft tissue sarcomas. ASPS presents a poor prognosis and develops frequent metastases, especially in the lungs, brain and bones. Current therapies, such as surgery, radiotherapy and chemotherapy, are not fully effective and other alternative treatments are currently being studied. ASPS is predominantly found in the deep soft tissues of the lower extremities. To our knowledge, only thirteen primary intraosseous ASPS have been reported in the literature. In this study, we report two new cases of this exceedingly rare entity. Both cases already had multiple metastases since diagnosis; one of them represents the first case of a primary bone ASPS in the ulna and is also the primary intraosseous ASPS with the longest reported case of survival, after having maintained long periods of stabilization despite not having received any systemic treatment.     

Primary alveolar soft part sarcoma of the stomach: a case report and review

Alveolar soft part sarcoma (ASPS) is a rare tumor typically located in skeletal muscles and muscolofascial planes. Isolated cases of ASPS have been described as arising in the viscera. We report a mesenchymal tumor of the stomach in a 54-year-old Italian woman without evidence of primary neoplasm elsewhere ten years following the initial diagnosis. The histologic, histochemical, immunohistochemical, and electron microscopic findings were all consistent with the diagnosis of ASPS and allowed differentiating it from morphologically similar and more common tumors, such as metastatic renal cell carcinoma and paraganglioma. The patient is alive and well ten years following the initial presentation.     

Cytologic features of alveolar soft part sarcoma: report of three cases

Alveolar soft part sarcoma (ASPS) is a rare, high-grade, epithelial-like sarcoma that shows characteristic histopathologic findings. Although a chromosomal anomaly that seems specific has been recently described, its diagnosis is based on histologic and ultrastructural features. The tumor shows no specific immunohistologic findings. Cytologic features of three cases of ASPS are presented. Preoperative fine-needle aspiration (FNA) of the primary soft tissue tumor was performed in two cases. In another two, mediastinal and pulmonary and subcutaneous metastatic lesions were aspirated. In all cases the cytologic image was identical with numerous, dissociated, large neoplastic cells with round-to-plasmocytoid morphology. Cytoplasmic fragility and granularity with abundant, atypical, naked nuclei were present. In one case, FNA material was available for ultrastructural studies. It disclosed the characteristic cytoplasmic crystalline structures. A specific cytologic diagnosis of ASPS was given in all cases. In conclusion, ASPS is a rare neoplastic entity that shows a characteristic cytologic image. When accompanied by an adequate clinical context it permits specific preoperative recognition. While immunocytologic studies are helpful to exclude other neoplasms, ultrastructure may result in an exact diagnosis.     

Primary testicular sarcoma: a report of two cases

The clinical and pathologic features of two primary pure testicular sarcomas are reported. One tumor was an osteosarcoma and was found in a 30-year-old man; the other was a fibrosarcoma from an 86-year-old man. The patients were treated with orchiectomy and high ligation of the spermatic cord and received no postoperative therapy. Both of them were alive and well with no evidence of disease 6 months and 5 1/2 years, respectively, after orchiectomy. Most testicular sarcomas arise from a teratoma or, less commonly, originate from a spermatocytic seminoma, but our two cases and rare previously reported cases illustrate that pure sarcomas occur occasionally and may be associated with a favorable prognosis.     

Primary alveolar soft part sarcoma of bone

AIMS: Alveolar soft part sarcoma is a distinct, rare soft tissue tumour occurring primarily within the skeletal muscles or musculofascial planes in young adults. Primary involvement of bone is extremely rare. We report on six patients with alveolar soft part sarcoma occurring primarily in bone. METHODS AND RESULTS: Thorough clinical and radiographic examinations were done to rule out any other primary site. The patients were four women and two men aged 17-35 years (mean, 24.5 years). The primary site of the tumour was the femur in three patients, the ilium in one and the fibula in two. In one of the patients with fibular involvement, the tibia was also involved by direct extension. Of the long bone lesions, three were centred in the metaphysis and one in the diaphysis. Radiographically, all of the lesions demonstrated an osteolytic pattern of bone destruction with ill-defined margins and a wide zone of transition between the lesion and adjacent normal bone. Microscopically, all tumours showed the typical histological pattern of alveolar soft part sarcoma. Diastase-resistant, periodic acid-Schiff-positive crystalline structures were identified within the cytoplasm and confirmed ultrastructurally. Immunohistochemically, a keratin stain was negative in all cases; there was positive staining for MyoD1 in the cytoplasm but not the nuclei. Distant metastasis developed in four patients; one died. CONCLUSION: Alveolar soft part sarcoma arising in bone is extraordinarily rare but should be considered in the differential diagnosis of metastatic hypernephroma in a young patient.     

脑膜腺泡状软组织肉瘤误诊为脑膜瘤一例

患者男,30岁.患者于7个月前在外院诊断为左侧蝶骨嵴外侧及颞下窝脑膜瘤,行手术治疗.4个月前胸片见"两肺散在结节影";头颅CT见"左侧中颅凹底颢下窝脑膜瘤复发".2个月前,胸CT示"双肺多发转移瘤";1个月前,患者自觉左侧面部麻木,左侧眼球突出,左侧视力下降明显,左眼球活动受限,左眼睑下垂,左侧结膜充血水肿,并伴有张口受限.为进一步治疗,于2007年1月22日入住本院.头颅MRI:左侧蝶骨嵴外侧及颞下窝可见一不规则异常信号影,病变向前方累及眼眶内,视神经受压明显,向内侧突入蝶窦,增强明显强化(图1).     

Primary alveolar soft part sarcoma of the uterine cervix: a case report and literature review

Alveolar soft part sarcoma (ASPS) is a tumor of unknown histogenesis, composed of large, epithelioid cells with eosinophilic cytoplasm, having an alveolar pattern. Primary ASPS of uterine cervix is very rare. In this report, we present a 21-aged-old female with primary ASPS in the uterine cervix and discuss the clinicopathological characteristics, immunophenotype, molecular genetic feature and differential diagnosis of ASPS of cervix.