颈动脉体副神经节瘤肝骨多发转移一例并文献复习

目的探讨颈动脉体恶性副神经节瘤的临床特点、诊断、治疗方法及预后。方法报道一例颈动脉体恶性副神经节瘤肝脏及骨多发转移患者的临床资料、病理特点、免疫组织化学及治疗方法,并对该病变相关文献资料进行复习研究。结果该例患者病史清楚,临床症状及体征典型,原发肿瘤及转移病变明显,经彩色超声、CT、肝穿、病理、免疫组织化学及骨扫描等检查后确诊为左颈动脉体恶性副神经节瘤,肝脏及骨多发转移,经相关治疗后随访2年病情无进展。结论颈动脉体瘤（CBP）也称颈动脉体化学感受器瘤或非嗜铬性副神经节瘤,来源于颈动脉鞘内颈总动脉分叉处后侧的化学感受器,内含大量类似于肾上腺髓质的副神经节细胞,女性多见,少数肿瘤为恶性,可发生远处转移。该病发病率较低,好发于30～40岁人群,恶变率为5％．10％,临床极为罕见。部分影像学检查可显示肿瘤部位及形态,彩色超声、螺旋CT、MRI及颈动脉血管造影（DSA）在检查中各有特点。DSA检查对CBP的确诊率最高达100％。CBP临床极少见,正确诊断非常重要,手术切除仍是目前首选的治疗方法。     

颈动脉体瘤诊断与治疗进展

颈动脉体瘤属于化学感受器肿瘤，具有家族遗传倾向颈动脉体瘤的发生与SDH基因的突变有关，多数为良性，恶性率不超过10％，诊断恶性的标准为局部淋巴结或远处转移应根据仔细的临床检查和特征性影像学结果做出诊断，影像学检查包括B超（多普勒）、CT、MRI／MRA及DSADSA检查可作为诊断颈动脉体瘤的金标准，一旦诊断颈动脉体瘤，应积极采取手术治疗目前，放射治疗亦可作为治疗颈动咏体瘤的有效手段，本文对颈动咏体瘤的诊断及治疗进展作一简要综述     

恶性颈动脉体瘤病理诊断(附五例报告)

颈动脉体瘤绝大多数是良性 ,手术后可治愈 ,极少发生复发或转移。恶性颈动脉体瘤十分罕见 ,对其诊断标准说法不一 ,本文报告 5例恶性颈动脉体瘤的临床病理资料 ,结合文献进行讨论。1　材料与方法复习天津肿瘤医院 196 2年～ 2 0 0 0年 6 3例颈动脉体瘤病理档案材料 ,其中 5例为恶性 ,全部为术后切除标本。每例取材 3～ 8块 ,石蜡包埋 ,4～ 6 μｍ切片 ,ＨＥ染色后 ,光镜下观察。2　结果见附表。附表　临床资料及病理情况性别 年龄(岁 ) 病史手术日期 肿瘤大小(ｃｍ) 大体所见镜下特点术后存活女 44 右颈肿物 2 0年 ,伴血压不稳 1 962 7 2 3…     

10例恶性颈动脉体瘤之生物学行为分析

目的:观察恶性颈动脉体瘤在临床过程中的表现。方法:观察10例组织学诊断为恶性颈动脉体瘤的临床经过,了解是否有转移发生。结果:10例中有 2例发生转移(淋巴结、骨、肺),表现为恶性,而其余8例经手术切除后未见发展。结论:长期临床经验提示组织学上恶性表现,不一定能完全反映其生物学行为。     

恶性颈动脉体瘤合并广泛转移一例

颈动脉体瘤罕见,多为良性。本文报告一例颈动脉体瘤次全切除术11个月后发生广泛转移而死亡。患者女性,27岁,家庭妇女,颈右侧肿物3年。于1974年8月发现肿物,1975年1月就医。肿物硬不痛,行抗痨治疗不见缩小,于1976年3月19日切检。镜下见瘤组织呈腺泡状排列,血管丰富,瘤细     

10例恶生颈动脉体瘤之生物学行为分析

目的：观察恶生颈动脉体瘤在临床过程中的表现。方法：观察10例组织学诊断为恶性颈动脉体瘤的临床经过,了解是否有转移发生。结果：10例中有2例发生转移（淋巴结、骨、肺）,表现为恶性,而其余8例经手术切除后未见发展。结论：长期临床经验提示组织学上恶性表现,不一定能完全反映其生物学行为。     

不血管重建的肿瘤合并颈动脉切除术

目的:总结天津医科大学附属肿瘤医院头颈科1979年～2002年间对颈部肿瘤固定于颈动脉的患者施行不血管重建的颈动脉切除术31例的经验.方法:对颈部肿瘤固定于颈动脉的患者自1979年施行首例不血管重建的颈动脉切除术成功以来,至2002年4月共行此术31例,其中男17例,女14例,年龄14～58岁,以21～40岁最多,共21例,占67.8%.31例中包括颈动脉体瘤23例(恶性8例),迷走神经体瘤2例(恶性1例),颈动脉瘤4例,颈部转移癌累及颈动脉2例.31例中,曾接受肿瘤不全切除术22例.术前行颈动脉压迫锻炼,准确检测脑代偿供血合格后.行肿瘤合并颈动脉切除术.结果:31例患者无手术死亡,无术后脑血管并发症.死于肿瘤未控制3例:其中1例恶性颈动脉体瘤术后1年因远处转移死亡,2例转移癌术后2～5年死于癌复发及远处转移.28例术后6～29年均健在,5年生存率为90.3%.健在28例痛例中未出现脑供血不全表现,预后良好.结论:不血管重建的肿瘤合并颈动脉切除术是安全可行的.另外,较合并血管重建术具以下优点:①因不考虑血管重建,肿瘤切除应更较彻底;②术后甚少产生血栓、感染或致命性血管破裂出血等并发症;③如需术后放疗,可无顾虑;④术式更加简化.     

彩色多普勒超声在颈动脉体瘤诊断治疗中的应用价值--附12例报告

目的探讨彩色多普勒超声（ＣＤＦＩ）对颈动脉体瘤的诊断及术式选择的应用价值。方法通过ＣＤＦＩ对颈部肿物和颈部大血管进行常规及血流检查。结果颈动脉体瘤为颈动脉分叉部低回声不均质光团,内部血流极为丰富。行颈动脉压迫试验,健侧颈动脉血流流速、流量有不同程度增加。本组１２例中,仅１例误诊,确诊率９１．７％。结论ＣＤＦＩ对于颈动脉体瘤的诊断和术式选择能起很大的指导作用。     

口腔化学感受器瘤的恶性生物学行为及临床处理

目的:探讨口腔化学感受器瘤的恶性生物学行为。方法:追踪分析1例口腔舌部化学感受器瘤患者临床资料及组织病理特点,总结其恶性生物学行为。结果:发生于口腔化学感受器瘤较发生于身体其它部位(颈动脉体)更容易表现出恶性生物学特性,恶性生物学行为主要表现为肿瘤浸润,包膜不清楚,手术后出现原位肿瘤复发及远处(肺部)转移。结论:对于口腔的化学感受器瘤应根据其临床特点结合细胞学特征确定生物学行为并做相应临床处理。     

胰腺实性假乳头状瘤的临床诊治策略:附13例报告

目的 探讨胰腺实性假乳头状瘤的临床病理特点与治疗方法.方法 回顾性分析我院2000年5月至2011年3月诊治的13例胰腺实性假乳头状瘤病例的临床资料.结果 13例胰腺实性假乳头状瘤患者中女性12例,男性1例.发病年龄15-42岁,平均年龄29岁.患者血糖正常,无外伤和胰腺炎病史.肿瘤位于胰头3例,位于胰颈3例,位于胰体、尾7例.肿瘤直径3~9 cm不等.13例患者均行手术治疗,术中探查均未发现腹腔或肝脏转移.其中肿物位于胰头部的2例行胰头、十二指肠切除术,位于胰、体尾部的4例行常规开腹胰体、尾切除术,1例行胰体尾联合脾脏切除术,1例行腔镜下胰体、尾切除术,其余5例行完整肿物切除术.术后均未行放、化疗等综合治疗.随访时间从2个月到60个月不等,未发现复发、转移,无死亡.结论 胰腺实性假乳头状瘤术前诊断困难.最终诊断依赖于病理检查结果.其恶性度很低.很少发生周围浸润和远处转移.治疗首选在彻底切除肿瘤的前提下.最大可能保留患者脏器功能的手术方式.     

Metastatic lung cancer presenting with jugular foramen syndrome in a case of von Recklinghausens disease

Metastatic carcinomas from a distant primary malignancy involving the temporal bone particularly the jugular foramen are rare tumors. A 57-year-old gentleman had multiple gradually increasing swellings over the body since many years. For last two years, he noticed enlargement of the swelling in left calf and rapidly enlarging painful new swelling over the left chest wall in back for last one year. He had recent involvement of left seventh, eighth and lower cranial nerves. CT scan showed an extensive lesion in left jugular foramen region with bone destruction. The patient underwent decompression of the left jugular foramen mass lesion. The tumor was extremely vascular and a partial decompression could only be performed. Although there was relief in the headache but the neurological deficits were persisting. Histopathology of the tumor showed features of metastatic small ling cancer. In the present case, there was presence of long standing multiple swelling all over the body and the patient had painful enlargement that he perceived as an ongoing process of the von Recklinghausen's disease and made a delay in seeking the medical advice resulting in a well advanced disease and with poor prognosis.     

A case of successful surgical resection followed by S-1 administration for hepatocellular carcinoma with lung metastases and a tumor thrombus into right atrium

A case of a 68-year-old man with hepatocellular carcinoma (HCC) is presented. He underwent partial liver resection for three times and transcatheter arterial chemoembolization (TACE) for three times. Follow-up CT revealed a recurrent hepatic surface mass with malignant extended into the inferior vena cava (IVC) and right atrium (RA). CT scan also revealed multiple metastatic nodules in bilateral lungs. The tumor thrombus into the RA and the hepatic surface mass were successfully treated with surgical resection. Pathological specimen allowed the diagnosis of poorly-differentiated HCC. Adjuvant chemotherapy with S-1 resulted in complete remission of lung metastases. Tumor markers showed a significant improvement after S-1 administration. This case report suggests that a surgical resection followed by S-1 administration would be effective for a patient with lung metastases and a tumor thrombus into IVC or RA.     

A case of complete response to interferon-α and S-1 combination therapy for multiple pulmonary recurrences of hepatocellular carcinoma after hepatic resection

A 77-year-old man was diagnosed as hepatocellular carcinoma, and was referred to our hospital. After he was treated by transcatheter chemoembolization, he underwent a left hepatic lobectomy of the liver and cholecystectomy. Serum AFP and PIVKA-II remarkably elevated 7 months after surgery, and CT scan revealed multiple metastatic nodules in bilateral lungs. The nodules were diagnosed as lung metastasis of HCC. Because the lesions grew larger, S-1/IFN was administered. Diagnostic imaging and tumor markers showed a marked improvement after 4 courses of S-1/IFN therapy, and he is still alive with good condition without recurrence and progression of tumors.     

An autopsy case of sudden death by obstruction of the pulmonary artery in cardiac rhabdomyosarcoma

The patient was a 50-year-old man. Chest X-ray films showed a tumor shadow near the heart, which was diagnosed as carcinoma of the left lung. The patient's heart suddenly and unexpectedly stopped and he consequently died when his body was set in the right lateral position for operation on the left pulmonary tumor. A tumor measuring 6 X 3 X 3 cm was found in the region from the right ventricle to the pulmonary artery at autopsy. It was postulated that the tumor might have obstructed the pulmonary artery when the position of the patient's body was changed for surgery. A metastatic nodule was found in the left lung. The tumor was a rhabdomyosarcoma, the pleomorphic type.     

A case of malignant neuroepithelioma in the retroperitoneum

Reported is a case of a malignant neuroepithelioma in the retroperitoneum. The patient was a 37-year-old male suffering from lumbago and edema of the lower extremities. After ultrasonography and a CT scan, a diagnosis of a retroperitoneal tumor was made and a surgical resection of the tumor was performed in October, 1985. The subsequent pathological diagnosis was malignant neuroepithelioma which showed epithelioid cell clusters that stained positively to NSE. Eleven months later, metastatic tumors were seen present in the lungs and the liver though they regressed markedly after adjuvant chemotherapy with ADM, CPM, and VCR. Forty-one months after his first operation, the patient continues to work. In this case, adjuvant chemotherapy has proved effective.     

Dedifferentiated chondrosarcoma of the lung: case report and review of the literature

Primary malignant chondromatoid tumors of the lungs are rare. We report on a case of a 49-year-old woman who presented with a round focus in the upper lobe of the left lung. The performed biopsy showed features of a leiomyosarcoma. After chemotherapy and consecutive tumor resection, the histologic investigation of the entire tumor mass revealed a dedifferentiated chondrosarcoma. Careful clinical and radiologic examinations showed no evidence of further pulmonary and extra-pulmonary tumor lesions. The final diagnosis of a primary dedifferentiated chondrosarcoma of the lung was based on the morphologic criteria and review of the literature. The differential diagnosis of malignant hamartoma was discussed. Follow-up investigation after 2.5 years showed no evidence of another primary tumor site.     

A metastatic Merkel cell carcinoma successfully treated with chemotherapy

A 68-year-old man referred to the hospital because of a swelling of his bilateral cervical, left supraclavicular, and left axillary lymph nodes, and an ulcerated tumor in the right palatine tonsil. Three years previously, he had undergone a local excision in the left forearm of a skin tumor that had been pathologically diagnosed as a Merkel cell tumor. CT scan demonstrated an abdominal mass and his serum neurospecific-enolase (NSE) was found to be 20.3 ng/ml. Following biopsies of the lymph nodes and the tumor in the right tonsil, a metastatic Merkel cell carcinoma was diagnosed. Systemic chemotherapy thus was begun with doses of cyclophosphamide, doxorubicin and vincristine. After three cycles were completed, tumor were no longer present and his NSE returned to normal. Three months after the discontinuance of chemotherapy, he remains disease-free.     

An effective case of hepatic arterial infusion chemotherapy based on biochemical modulation for hepatic recurrence of non-functioning islet cell carcinoma of the pancreas

A 55-year-old man had a metastasis in segment 3 of the liver 5 months after surgery for non-functioning islet cell carcinoma of the pancreas. The metastatic lesion increased in size in a short period, and other liver micro-metastases that could not be detected by imaging may exist, so hepatic arterial infusion chemotherapy was scheduled for 3 months. The patient underwent hepatic arterial infusion chemotherapy of 5-fluorouracil (250 mg/day/body for 5 days/week) and adriamycin (10 mg/day/body for 2 days/week) and cisplatin (10 mg/day/body for 5 days/week) and he was put on Leucovorin 30 mg/day as a biochemical modulator of 5-FU and tamoxifen 40 mg/day as a biochemical modulator of ADM. A total 6,000 mg of 5-FU, 100 mg of ADM and 240 mg of CDDP had been administered, until hepatic arterial infusion chemotherapy was discontinued because of complicated gastric ulcer. Three months later, the size of the metastatic liver tumor was reduced remarkably and no other metastasis was detected on CT scan, so he underwent partial hepatectomy of the metastatic lesion. No recurrence was found and he has survived in good physical condition during the follow-up period of 5 months after the second operation.     

A case of huge submandibular malignant tumor treated successfully with UFT chemotherapy

We report a patient with a huge submandibular malignant tumor showing an excellent response to chemotherapy with UFT. A 76-year-old woman complaining of a submandibular mass was referred to us. The mass had an irregular margin and measured 11 x 7 cm on CT scan. A left submandibular lymph node was enlarged slightly. Fine needle aspiration cytology of the mass indicated undifferentiated malignant tumor. We diagnosed her with unresectable malignant tumor. She was treated with oral UFT (600 mg/day), as she refused chemoradiotherapy. The malignant tumor became dramatically smaller in 4 weeks, and clinically disappeared in 6 weeks. Oral UFT was discontinued due to liver dysfunction. There has been no evidence of recurrence for 5 years after discontinuation of chemotherapy. The patient remains under observation.     

Endobronquial metastasis of a ductal carcinoma in a male

Breast cancer in male is uncommon and 60% of the cases are presented in advanced stadiums of the disease. Malignant pleural effusion is a frequent form of presentation of breast cancer in women, being uncommon in males. We describe the case of a malignant pleural effusion and metastatic endobronchial of a ductal carcinoma of breast in male. A fifty five years old male, non-smoker, without other pathological recards, consulted our hospital because of a medical profile of 1 month of evolution of dyspnea of effort and dry cough. In the physical examination he only emphasized a hypophonesis in left side of chest and an exofitc injury in the right nipple. The blood analysis detected a mild normocitic anaemia and an ESR of 76 mm/h. The radiology of the chest showed a massive left pleural effusion, that was confirmed subsequently in a thoracic computed tomography scan. A bronchoscopy was practiced finding a tumor in the left principal bronchus which obstructed the light of the lumen. At the same time a mammography was accomplished, showing a nodule in the right breast suggestive of carcinoma whose aspirative puncture was positive for infiltrating ductal carcinoma moderately differentiated. Also, the result of the endobrochial biopsy was compatible with metastatic ductal carcinoma of the breast. No other metastasis were detected. The patient began chemoterapy during 6 cycles, appreciated a symptomatic improvement and a partial response in the thoracic computed tomography scan. After 6 months of follow-up the patient stays stable and without symptoms.