原发性十二指肠恶性肿瘤临床影像病理学研究

目的探讨原发性十二指肠恶性肿瘤的临床表现、病理学特征影像学诊断及治疗方法.方法对82例原发性十二指肠恶性肿瘤患者的临床资料进行回顾性分析.结果本组患者发病部位乳头区53例、降段18例.临床表现依次为上腹疼痛57例、黄疸53例、消化道出血41例.乳头区肿瘤多表现为腹痛、黄疸,乳头区以外肿瘤常则多表现为腹痛、黑便及腹部包块.病理学类型以腺癌多见(72例), 平滑肌肉瘤(5例)、恶性淋巴瘤(3例)次之.结论原发性十二指肠恶性肿瘤以上腹痛、黄疸、消化道出血为主要表现,但缺乏特征性.病理类型以腺癌为主,好发于十二指肠乳头区及降段.CT、B超和内镜检查是主要诊断手段.手术切除是原发性十二指肠恶性肿瘤的主要治疗方法,早期诊断是改善患者预后的关键.     

原发性十二指肠癌 46 例内镜诊断及病理组织分析简

目的提高原发性十二指肠癌的诊断水平。方法选择46例原发性十二指肠癌患者,其中男性26例．女性20例：年龄31-71岁,平均年龄62_3岁。行内镜诊断和病理活组织检查或术后病理组织检查。回顾性分析其临床、内镜及病理学关系。结果46例十二指肠癌发生于十二指肠球部9例,水平部5例．乳头部30例．乳头下段2例。病理活组织检查或术后病理学诊断示中分化腺癌8例,低分化腺癌26例．印戒细胞癌10例．未分型2例。结论临床应提高对原发性十二指肠癌的警惕性;原发性十二指肠癌以十二指肠乳头部好发,病理分型以腺癌为主,诊断首选上消化道内镜及活组织检查,内镜操作时应尽可能观察到十二指肠降部以下,特别应注意观察乳头部。对十二指肠球部溃疡反复治疗效果不佳者,应提高警惕,及时进行活组织检查。手术切除尤其是胰十二指肠切除术是最常用、最可靠的治疗方法。     

原发性十二指肠癌46例内镜诊断及病理组织分析

目的提高原发性十二指肠癌的诊断水平。方法选择46例原发性十二指肠癌患者,其中男性26例,女性20例;年龄31~71岁,平均年龄62.3岁。行内镜诊断和病理活组织检查或术后病理组织检查。回顾性分析其临床、内镜及病理学关系。结果46例十二指肠癌发生于十二指肠球部9例,水平部5例,乳头部30例,乳头下段2例。病理活组织检查或术后病理学诊断示中分化腺癌8例,低分化腺癌26例,印戒细胞癌10例,未分型2例。结论临床应提高对原发性十二指肠癌的警惕性;原发性十二指肠癌以十二指肠乳头部好发,病理分型以腺癌为主,诊断首选上消化道内镜及活组织检查,内镜操作时应尽可能观察到十二指肠降部以下,特别应注意观察乳头部。对十二指肠球部溃疡反复治疗效果不佳者,应提高警惕,及时进行活组织检查。手术切除尤其是胰十二指肠切除术是最常用、最可靠的治疗方法。     

116例原发性小肠肿瘤临床分析

目的分析原发性小肠肿瘤的临床病理类型及诊断手段。方法回顾性分析经病理证实的116例原发性小肠肿瘤的临床病理资料。结果术前明确诊断率20.68%(24/116),病理类型恶性占76.72%(89/116),以腺癌、平滑肌肉瘤,恶性淋巴瘤为主;良性以腺瘤、平滑肌瘤为主。主要临床表现为腹痛、腹块、贫血、出血等。结论原发性小肠肿瘤病理类型呈多样性,恶性肿瘤多见。临床表现缺乏特异性,检查手段相对不足,易导致延误诊断。     

原发性下消化道上皮样血管肉瘤3例临床病理分析

目的探讨原发性下消化道上皮样血管肉瘤(epithelioid angiosarcoma, EAS)的临床病理学特征、免疫表型及鉴别诊断。方法回顾性分析3例原发性下消化道EAS的临床病理资料,对根治性切除标本进行常规HE和免疫组化EnVision法染色,并结合相关文献复习,分析其临床病理学特征。结果 3例患者年龄68～75岁,男性2例,女性1例;发生于回肠2例,结肠1例;临床均以腹部疼痛就诊。镜下肿瘤均呈侵袭性生长,排列呈蜂窝状、海绵状、脾窦样和裂隙样,裂隙内可见乳头簇形成,瘤细胞呈上皮样,具有显著的异型性,表现为核大空泡状,核仁明显,可见多少不等的核分裂象。免疫表型:瘤细胞CD31、CD34、ERG和Fli-1阳性,Ki-67增殖指数50%～70%。结论原发性下消化道EAS临床罕见,以消化道出血、腹痛和贫血为主要表现,易与消化道癌、胃肠道间质瘤混淆,确诊需结合组织学形态及免疫表型,预后较差。     

十二指肠畸胎瘤1例报道并文献复习

目的探讨十二指肠畸胎瘤的临床、影像及病理学特征, 提高临床医生对该肿瘤罕见发病部位的认识。方法回顾性分析2021年3月山东省烟台市烟台山医院收治的1例十二指肠实性成熟性畸胎瘤患者的临床、影像及病理资料。在中国知网、万方数据库、维普数据库及PubMed中以"十二指肠/duodenal"和"畸胎瘤/teratoma"为关键词, 检索2020年12月之前有关十二指肠畸胎瘤的文献, 共检出英文文献报道3篇3例和中文文献报道2篇2例;结合本文报道的1例, 总结其临床影像及病理学特征。结果本例为40岁男性, 临床表现为上腹部胀痛不适半年, 影像学表现为十二指肠降段不规则混杂密度影, 内见不规则钙化样高密度影及脂肪密度影;临床诊断十二指肠降部占位, 行十二指肠肿物切除术, 术后病理诊断为十二指肠实性成熟性畸胎瘤, 恢复良好。结合文献报道的5例, 共计6例十二指肠畸胎瘤, 其中男4例、女2例, 年龄7 d～40岁。临床均表现为肠梗阻或肠梗阻症状, 其中2例伴有贫血、2例有腹部包块。发病部位均位于十二指肠降部及水平部。影像学检查:5例行CT和/或B超检查, 均提示肿瘤或占位, 显示囊性肿瘤1例、囊实性肿...     

阴道原发性子宫内膜样腺癌

阴道的原发性腺癌约占阴道所有原发性癌15％。除了透明细胞腺癌认识较清楚外，其他类型的腺癌尚未得到足够的重视。作者报道了18例阴道原发性子宫内膜样腺癌，患昔年龄45—81岁，平均60岁。临床症状主要表现为阴道出血或不适。16例患者曾行子宫切除术，9例有子宫内膜异位症病史，5例有长期服用雌激素史，其中1例患者在诊断时还已服用三苯氧胺5年，1例患者在诊断腺癌之前9年曾被诊断为阴道腺病。肿瘤大小为1．4～7．0cm。     

肉瘤样肝细胞癌4例临床病理分析

目的探讨肉瘤样肝细胞癌(sarcomatoid hepatocellular carcinoma,SHC)的临床病理学特征、诊断、鉴别诊断及预后。方法回顾性分析4例SHC的临床病理资料、免疫表型,并复习相关文献。结果 4例患者中男性3例,均有肝炎病毒感染,3例伴肝硬化,临床表现为程度不等的腹痛、腹胀、恶心、呕吐、消瘦等。肿瘤单发或多发,切面灰白色、多伴出血、坏死。镜下见肿瘤由肝细胞癌及肉瘤样成分构成。免疫表型:肝细胞癌AFP、GPC-3和Hep Par1均(+),肉瘤样成分:vimentin、CK8/18和CK19均(+)。4例患者随访2、5、8个月,均死亡。结论 SHC在肝恶性肿瘤中预后最差。     

原发性子宫内膜神经内分泌小细胞癌7例临床病理分析

目的探讨原发性子宫神经内分泌小细胞癌的临床病理特征、诊断、鉴别诊断、治疗及预后。方法回顾性分析7例原发性子宫神经内分泌小细胞癌的临床资料,对其形态学、免疫表型进行观察。结果 7例患者平均年龄50岁,临床表现以阴道不规则出血为主,肿瘤呈菜花状或结节状,直径1.5~4.0 cm,切面灰黄、灰红色相间。光镜下肿瘤由大小较一致的小圆或卵圆形细胞构成,胞质少,核分裂象多见,排列呈片状、梁状、岛状或菊形团状,部分伴腺癌成分。免疫表型:肿瘤细胞Syn、CgA、CD56、CK(AE1/AE3)、EMA均阳性,NSE、CD45、p40、p63、CD10均阴性。术后随访患者1~5年,3例死亡,3例健在,1例失访。结论原发性子宫神经内分泌小细胞癌少见,临床无特异性表现,主要症状为阴道不规则出血,易误诊为其他低分化恶性肿瘤,需根据形态学及免疫表型综合判断,预后较差。     

结直肠原发性腺鳞癌的临床病理观察

目的 探讨结直肠原发性腺鳞癌的临床病理学特征、组织发生、生物学行为和人类乳头状瘤病毒(HPV)在其发病中的作用.方法 应用光镜、免疫组化标记及HPV原位杂交技术对5例发生于结肠的腺鳞癌进行临床组织病理学分析,并复习相关文献.结果 患者男3例,女2例,平均51岁.肿瘤位于结肠肝曲、脾曲、横结肠和乙状结肠距肛门12 cm及14 cm处.主要症状为便血、腹痛、腹泻和消瘦.4例患者分别死于术后12、21、22和33个月,1例术后3个月生存.镜下癌组织有腺癌和鳞癌成分共同组成,可见两种成分的移行.鳞癌细胞表达CK5/6、34βE12和p63,而腺癌细胞均阴性表达.腺癌细胞均表达CDX-2,鳞癌细胞弱阳性或阴性表达,二者均不表达TTF-1.原位杂交显示HPV6/11和HPV16/18均阴性.结论 结直肠原发性腺鳞癌是肠道恶性肿瘤的罕见类型,组织起源尚无定论,临床预后较单纯腺癌差.HPV6/11和HPV16/18感染与结直肠腺鳞癌的发生关系还难以明确.     

Duodenal somatostatinoma: a case report and review of 31 cases with special reference to the relationship between tumor size and metastasis

Somatostatinomas are rare functioning neoplasms usually arising in the pancreas and duodenum. We report a case of somatostatinoma in a 42-year-old male with neither neurofibromatosis nor somatostatinoma syndrome. A large tumor in the descending duodenum had given rise to multiple lymph node metastases. An additional 31 duodenal somatostatinoma cases were also reviewed. Most originated in the descending part of the duodenum, with the ampulla and peri-ampullary area as the most common sites (60%). Frequent manifestations were abdominal pain (25%), jaundice (25%), or cholelithiasis (19%), the latter two reflecting obstruction of the bile duct by tumors. Only two cases showed a possible somatostatinoma syndrome (6%). The tumors with metastases, lymph nodes (10) and liver (2), were significantly larger than average than those without (2.91 +/- 1.49 cm vs 1.36 +/- 0.71 cm, P < 0.05). With a cut-off point of 2.0 cm, diagnostic accuracy for metastasis was 77.78% with 87.50% specificity and 63.64% sensitivity. The smallest tumor with metastases was 0.8 cm and the largest without metastases was 3.0 cm. These results indicate that duodenal somatostatinomas are malignant by nature and the risk of metastasis significantly increases with tumors larger than 2.0 cm.     

Brunner's gland hyperplasia: treatment of severe diffuse nodular hyperplasia mimicking a malignancy on pancreatic-duodenal area

Brunner's gland hyperplasia is a benign tumor of the duodenum and it is rarely associated with clinical symptoms. We report on a 64-yr-old man with Brunner's gland hyperplasia who had undergone a duodenocephalo-pancreatectomy. The reason is that he presented upper gastrointestinal obstructive symptoms and the esophagogastroduodenoscopic finding revealed the lesion to be an infiltrating type mass on the second portion of the duodenum with luminal narrowing. An abdominal computed tomography showed a 2.5 cm-sized mass in the duodenal second portion with a suspicious pancreatic invasion and 7 mm-sized lymph node around the duodenum. Duodenocephalopancreatectomy was successfully performed. Histological examination revealed a Brunner's gland hyperplasia. The final diagnosis was the coexistence of Brunner's gland hyperplasia and pancreatic heterotopia with a pancreatic head invasion. The literature on Brunner's gland hyperplasia is reviewed.     

Endometriosis-associated intestinal tumors: a clinical and pathological study of 6 cases with a review of the literature

This clinicopathologic study of primary Mullerian tumors of the bowel arising in foci of endometriosis is based on six new cases and an analysis of 17 previously reported cases. Varieties of Mullerian tumors occur in the bowel; the most common types are endometrioid carcinoma, followed by various mixed Mullerian tumors and stromal sarcomas. Seventy-eight percent develop in the rectosigmoid colon, the remaining in the cecum or ileum. Those in the latter area tend to be sarcomas or mixed Mullerian tumors. Certain architectural growth characteristics, derived from precursor endometriosis, are common to most endometriosis-associated intestinal tumors (EAITs). Seventy percent of EAITs occur in the outer bowel wall. Transmural tumors tend to form luminal polyps and assume an hourglass shape. Metachronous or synchronous Mullerian tumors occur in 39% of cases. Seventy percent of women with EAITs are in their mid 30s to early 50s. Common presenting symptoms are abdominal or pelvic pain, melena, and an abdominal or pelvic mass. Documented in 26% of patients is a history of prolonged unopposed estrogen therapy. Only 28.5% of cases die of their tumors, but follow-up is less than 5 years in all but 2 patients.     

Primary adenocarcinoma of the duodenum in the elderly: clinicopathological and immunohistochemical study of 17 cases

Seventeen cases of primary duodenal adenocarcinoma occurring in the elderly (older than 65 years) were examined to clarify their clinicopathological features and biological behavior. The mean age was 77.4 years (range, 66-104), and there was no appreciable difference in the incidence between the sexes (female: male ratio, 8: 9). Thirteen patients had tumors located in the first portion of the duodenum, three in the second portion and one in the third portion. Grossly, there were three varieties of lesions: six polypoid, four flat-elevated and seven ulcerative-invasive. Sixteen cases showed well-differentiated adenocarcinoma, three of which were difficult to distinguish from adenoma, and one was poorly differentiated adenocarcinoma. Three of 16 tumors had poor differentiation in the invasive area, whereas mucosal lesions were well differentiated. Eight tumors had invaded the duodenal wall with occasional involvement of the pancreas. Immunohistochemistry demonstrated p53 protein overexpression in two intramucosal (22.2%) and five invasive (62.5%) cancers. In the intramucosal area the mean Ki-67-positive rate (PR) of the tumors with distant metastasis was significantly higher than that of the tumors without metastasis (46.0 vs 31.6%; P < 0.05), while there was no significant difference in the association between PR and gross feature or depth of the tumors. Clinical follow-up showed three of the five patients with invasive cancer died of carcinoma within 28 months. Compared with published data from other investigators, the results of the present study indicate a proximal shift of duodenal carcinoma in the elderly. Furthermore, it is concluded that invasive duodenal adenocarcinomas with high PR should be considered as potentially aggressive tumors, although their histology may indicate a high degree of differentiation.     

空回肠间质瘤的临床、影像和病理学研究

目的　探讨空回肠间质瘤诊断和治疗经验 .方法　对我经术后病理和免疫组化证实 13例空回肠间质瘤病例进行回顾性分析 .结果　本组患者主要临床表现为消化道出血、腹痛、头晕、乏力、黑便 ,发病至确诊时间 2月～ 7年 .术前选择性血管造影发现小肠出血 2例、核素扫描发现小肠出血 1例 .剖腹探查确诊 7例 ,腹腔镜探查确诊 4例 ,因其他疾病腹腔镜探查确诊 1例 ,术前确诊仅 1例 .随访 2月～ 9年 ,除 1例患者死于其它疾患外 ,余 12例患者均健在 ,未发现空回肠间质瘤复发 .结论　空回肠间质瘤预后甚好 ,但本病缺乏特征性的临床表现及有效的诊断手段 ,易致长期延误诊治 ;对长期不明原因的消化道出血患者及早行剖腹探查或腹腔镜探查是避免空回肠间质瘤长期延误诊治、改善患者预后的关键     

Duodenal somatostatinomas associated with von Recklinghausen's neurofibromatosis. Apropos of 2 cases

Somatostatinomas are rare neuroendocrine tumors; they are essentially located in the pancreas and in the duodenum. The association with a neurofibromatosis type I is especially observed when the tumor is located in the ampulla of Vater. These tumors are not associated with a "somatostatin syndrome", but often present with gastrointestinal bleeding, abdominal pain and obstructive jaundice. The diagnosis is confirmed by immunohistochemical studies. The aim of this study is to report 2 cases of metastazing duodenal periampullary somatostatinomas associated with von Recklinghausen's disease and to discuss the prognosis of these tumors. Future genetic research are necessary as point out the familial feature of this association in one of our cases.     

LEIOMYOSARCOMA OF THE HEART COLLIDING WITH ATRIAL MYXOMA

An autopsy case of a primary cardiac leiomyosarcoma of the left atrium colliding with a myxoma in the same chamber was presented. Cardiac signs and symptoms were remarkably silent. She died with multiple systemic metastasis of the leiomyosarcoma. As far as we are aware of, this is the first report of a case with double primary cardiac tumors in collision.     

Duodenal gangliocytic paraganglioma. Clinicopathologic and immunocytochemical study of 11 cases

The clinicopathologic features of 11 cases (8 in men) of duodenal gangliocytic paraganglioma are presented. The patients averaged 56 years of age; none showed evidence of phakomatosis. Ten tumors occurred in the second portion of the duodenum, and one arose in the third portion. All tumors were polypoid, and half presented with gastrointestinal bleeding. The neoplasms were composed of paraganglioma and carcinoid-like elements, neurons, and Schwann as well as sustentacular cells. All tumors behaved in a benign fashion after local resection or snare polypectomy; long-term follow-up (1-25 years; mean, 8.3 years) showed no recurrence in any case. Immunocytochemical examination demonstrated the presence of somatostatin, serotonin, and human pancreatic polypeptide within endocrine cells and neurons.     

Primary adenocarcinoma of the minor duodenal papilla

A 70-year-old man was admitted to our institution due to aggravation of blood-sugar level control and because an abdominal CT showed dilatation of the main pancreatic duct. Upper gastrointestinal endoscopy revealed a flat elevated tumor with central ulceration in the second portion of the duodenum. Subsequent duodenoscopy for a more detailed examination showed that the tumor had originated in the minor duodenal papilla. A biopsy specimen showed moderately differentiated adenocarcinoma. Endoscopic retrograde pancreatography via the major duodenal papilla revealed a slightly dilated main pancreatic duct and obstruction of the accessory pancreatic duct. Endoscopic ultrasonography showed a hypoechoic mass in the minor duodenal papilla with retention of the muscularis propria of the duodenum. These findings suggest that the tumor existed only to a limited extent in the minor duodenal papilla, and that the tumor did not infiltrate into the pancreas. For treatment, pylorus-preserving pancreatoduodenectomy was performed, and histological findings revealed a well-differentiated adenocarcinoma that originated in the minor duodenal papilla. Primary adenocarcinoma of the minor duodenal papilla is extremely rare. Our case is the first report of primary adenocarcinoma of the minor duodenal papilla at an early stage with no infiltration into muscularis propria of the duodenum and pancreas.