Supratentorial Primitive Neuroectodermal Tumors in Adults

A retrospective clinical analysis was made of 12 patients with supratentorial primitive neuroectodermal tumor (PNET) who ranged in age from 20 to 62 years (median 24) and were managed at Seoul National University Hospital between January 1987 and December 1997. Six patients were male and six were female. Most presented with symptoms of increased intracranial pressure and mean duration of symptoms was four months (range: 1–12 months). The tumors were located in the posterior parieto-occipital area in six cases and the mean diameter of mass of these tumors was 5.3cm. The characteristic magnetic resonance image finding was a large well-demarcated lobulating mass with intratumoral cyst, necrosis, and/or hemorrhage. Calcification was seen in five out of six patients who underwent computed tomography scan. All patients underwent craniotomy and three of them received subsequent operations due to local recurrence. Ten patients received postoperative whole neuraxis radiation therapy and five patients received additional chemotherapy. Mean survival after diagnosis was 86 months. The patients having intratumoral calcifications are all alive and two out of three showing a Ki-67 labelling index greater than 30% died at eight and 20 months after operation, respectively. In conclusion, supratentorial PNET must be included, even in adults, in the differential diagnoses if a tumor has characteristic radiological features. The adult supratentorial PNET seemed similar to that of children in the clinical features and the prognosis. Intratumoral calcifications and the Ki-67 labelling index might be prognostic factors, however, it should be considered that the sample size is too small and not all patients were evaluated.     

外周型原始神经外胚叶瘤临床特征及治疗分析

目的 探讨外周型原始神经外胚叶瘤（peripheral primitive neuroectodermal tumors, pPNET）的临床特征及治疗方法。方法 回顾性分析重庆医科大学附属第一医院2005年11月—2014年4月收治的10例pPNET患者的临床病理特征、治疗方案等资料,并用直接计算法计算其生存率。结果 10例患者中以青少年男性居多,免疫组织化学CD99、NSE表达率高。6例行手术治疗,其中5例行术后放疗+化疗。2例行放疗+化疗,1例行化疗+HIFU治疗,1例行中药治疗。治疗后1、2、5年生存率分别为80.0%（8/10）、71.4%（5/7）、40.0%（2/5）。结论 pPNET的临床表现缺乏特异性,以细胞形态学联合免疫组织化学确诊,采取手术、放疗、化疗为主的综合治疗。     

中枢性原始神经外胚层肿瘤16例临床病理分析

[目的]探讨中枢性原始神经外胚层肿瘤(PNETs)的病理诊断、鉴别诊断及意义。[方法]采用光镜、免疫组织化学、特染及电镜对16例PNETs病例进行观察。[结果]肿瘤细胞排列成巢状、团块状或散在分布,细胞呈小圆形,大小一致,有菊形团形成;免疫组化显示有两项以上神经源性抗体呈阳性表达,以CD99最为显著;特殊染色显示瘤组织间网状纤维明显减少,部分瘤细胞胞浆内有糖原颗粒;电镜下部分瘤细胞胞浆内有神经内分泌颗粒。[结论]中枢性原始神经外胚层肿瘤归于神经上皮组织的胚胎性肿瘤,属高度恶性,诊断时须与髓母细胞瘤等相鉴别。     

外周原始神经外胚层肿瘤形态学免疫表型及临床预后研究

目的：探讨外周原始神经外胚层肿瘤(PNET)的临床表现，病理及免疫组织化学特点及预后。方法：对15例外周原始神经外胚层肿瘤进行临床资料及预后，病理组织形态学和超微结构特点及免疫组化表型研究。结果：患者以男性为主，男女之比约为6．5：1，年龄6～35岁，年龄中位数为16，发生部位较多，可发生在直肠，腹膜后，腹股沟，淋巴结，胸壁，睾丸，鼻腔及骨组织等；免疫组化：肿瘤均弥漫表达CD99，并不同程度地表达NSE，SYN，CgA等，但不表达CK，Desmin，LCA等，其中1例表达组织化学染色PAS，随访最长时间为24月。结论：PNET是一种发生在年轻男性，进展迅速，预后非常差的恶性小圆细胞肿瘤，认识其临床病理特点及免疫组化表型对于该恶性肿瘤的诊断及临床治疗意义重大。     

Intense P53 Staining is a Valuable Prognostic Indicator for Poor Prognosis in Medulloblastoma/central Nervous System Primitive Neuroectodermal Tumors

Intense p53 immunostaining may predict for a poor prognosis in central nervous system primitive neuroectodermal tumor of childhood. Background: Medulloblastoma is a common childhood primary brain tumor. Potential prognostic indicators for patients with local disease are age, extent of resection, and gender. However, none of these are well established. Immunohistologic staining is a potentially useful means to identify high-risk patients. The purpose of this clinical pathologic study was to investigate the prognostic significance of GFAP, synaptophysin, Ki-67, and p53 immunostaining in medulloblastoma/central nervous system primitive neuroectodermal tumors (CNS PNETs.) Materials and methods: The records of 40 patients with CNS PNETs were reviewed. Their surgical specimens were immunostained for p53, glial fibrillary acidic protein (GFAP), synaptophysin, and Ki-67. The p53 specimens were scored blindly for the intensity of staining of nuclei (intense vs weak) and the quantity of cells stained. The Ki-67, GFAP, and synaptophysin specimens were analyzed for quantity of cells stained. Results: Ten patients' specimens stained intensely for the p53 protein. Eleven had weakly staining nuclei. Nineteen specimens had no staining. The patients with specimens that stained intensely had a statistically significant decreased disease free survival (P = 0.03). Mere presence or quantity of p53 nuclear staining did not correlate with disease free survival. Immunohistochemical staining for Ki-67, GFAP, and synaptophysin did not correlate with disease free survival. Clinical parameters of age, gender, and extent of resection also did not approach statistical break significance for disease free survival. Conclusion: Intense nuclear staining for p53 was the only variable in this clinical pathologic study that reached statistical significance for disease free survival. This suggests that intense staining for p53 may be the most important prognostic indicator for non-metastatic CNS PNETs. p53 Immunostaining with antibodies against p53 in CNS PNETs should be studied in a multi-institutional setting with larger numbers of patients.     

High-dose Chemotherapy with Autologous Stem Cell Rescue as First Line of Treatment in Young Children with Medulloblastoma and Supratentorial Primitive Neuroectodermal Tumors

In order to improve the dismal prognosis of patients younger than 4 years old with medulloblastoma and supratentorial primitive neuroectodermal tumors (stPNET) seven young children were treated with high-dose chemotherapy (HDCT) and autologous stem cell rescue in our center. All patients underwent surgical debulking and standard chemotherapy. None of them received irradiation. The HDCT included busulfan 16 mg/kg, orally over 4 days (from days -5 to -2) in 6 hourly divided doses, and melphalan at a dose of 140 mg/m2 given by intravenous infusion over 5 min on day -1. Three patients additionally received thiotepa 250 mg/m2 given by intravenous infusion daily over 2 days (from day -2 to -1) and two patients additionally received topotecan 2 mg/m2 given by intravenous infusion daily over 30 min for 5 days (from day -11 to -7). Patients' stem cells were mobilized with granulocyte colony-stimulating factor at a dose of 12 microg/kg twice daily subcutaneously for four consecutive days. Cryopreserved peripheral blood progenitor cells were reinfused 48 h after completion of chemotherapy. With a median follow-up of 21 months (range 5-64) five complete responses were observed; one patient had partial response and one had stable disease. There was no treatment-related mortality. The 2 year event-free survival was 71.43 +/- 17%. Therefore we conclude that HDCT as consolidation regimen may improve the cure rates in very young children with medulloblastoma/stPNET avoiding long-term sequelae of radiotherapy.     

Surgical treatment of pancreatic cancer: the role of adjuvant and multimodal therapies.

Surgical treatment in specialized referral centers has improved the prognosis of resectable pancreatic cancer considerably despite the generally aggressive behavior of this malignancy. At the same time, adjuvant therapy for pancreatic cancer has been shown to be effective in providing a survival benefit. However, some controversy remains over whether to use chemotherapy alone or combined chemoradiation. Few prospective randomized controlled clinical trials (RCTs) on the use of adjuvant chemotherapy and chemoradiation have demonstrated a distinct survival advantage of systemic chemotherapy (5-FU/FA or gemcitabine) following surgical resection. The most notable published trial is the European Study Group for Pancreatic Cancer (ESPAC)-1 trial. In addition, there are several retrospective analyses and two randomized studies on adjuvant radiation and chemoradiation. Some of these suggested increased survival rates using chemoradiation, which was subsequently widely introduced in clinical routine, especially in the United States. RCTs and a recent meta-analysis of these RCTs confirm, however, the superiority of chemotherapy over chemoradiation, except for a subgroup of patients with positive resection margins. Thus, curative surgery followed by adjuvant systemic chemotherapy should be the standard treatment for patients with resectable, locally confined pancreatic cancer. Further RCTs may clarify potential benefits of chemoradiation in the adjuvant treatment setting. Moreover, the best chemotherapy, or a combination thereof, remains to be determined in large-scale randomized trials.     

Analysis on the Characteristics and Prognosis of Pulmonary Neuroendocrine Tumors

Objective: To retrospectively review the clinical characteristics and analyze the prognostic factors of Chinesepatients with pulmonary neuroendocrine tumors. Materials and Methods: The clinical data of 176 patientswith pulmonary neuroendocrine tumors in Chinese PLA General Hospital from Mar., 2000 to Oct., 2012 wereretrospectively analyzed. The parameters were evaluated by univariate and multivariate analysis, including thegender, age, smoking history, family history, TNM staging, localization (central or peripheral), tumor size, nodalstatus, histological subtype and treatment (operation or non-operation). Results: There were 23 patients withtypical carcinoids (TC) (13.1%), 41 with atypical carcinoids (AC) (23.3%), 10 with large cell neuroendocrinecarcinoma (LCNEC) (5.7%) and 102 with small cell lung cancer (SCLC) (57.9%). The median follow-up time was64.5 months for AC, 38 months for LCNEC and 27 months for SCLC. The typical carcinoid censored data was 18(more than 50% of the patients), so the median follow-up time was not obtained, and actuarial 5-year survivalsfor TC, AC, LCNEC and SCLC were 75.1%, 51.7%, 26.7% and 38.8%, respectively. COX univariate analysisrevealed that the age (P=0.001), histological subtype (P=0.005), nodal status (P=0.000), treatment (P=0.000) andTNM staging (P=0.000) were the prognostic factors of the patients with pulmonary neuroendocrine tumors,whereas its multivariate analysis showed that only the age(P=0.001), TNM staging (P=0.002) and treatment(P=0.000) were independent prognostic factors. Conclusions: Radical surgery remains the treatment of choice,and is the only curative option. The age, TNM staging and treatment are confirmed to be the independentprognostic factors in multivariable models for pulmonary neuroendocrine tumors.     

EGFR基因突变对靶向治疗非小细胞肺癌患者的疗效影响及生存分析

目的 探讨表皮生长因子受体(EGFR)基因不同染色体上外显子突变对靶向治疗非小细胞肺癌患者的疗效影响及生存分析.方法 选取76例EGFR基因敏感突变非小细胞肺癌患者,根据EGFR基因检测结果分为四组:A组,EGFR基因18号染色体上外显子突变型组(18例);B组,EGFR基因19号染色体上外显子突变型组(20例);C组,EGFR基因20号染色体上外显子突变型组(19例);D组,EGFR基因21号染色体上外显子突变型组(19例).同时选取同期住院治疗的20例EGFR基因野生型(EGFR基因未突变)非小细胞肺癌患者作为E组(对照组).各组患者均给予吉非替尼(250 mg/d)治疗.结果 与E组比较,A、B、D组的总有效率和疾病控制率均升高,差异均有统计学意义(P均<0.05);与E组比较,C组的总有效率和疾病控制率均降低,差异均有统计学意义(P均<0.05).与E组比较,A、B、D组PFS、OS和QOL均升高,差异均有统计学意义(P均<0.05);与E组比较,C组的PFS、OS和QOL均降低,差异均有统计学意义(P均<0.05).与E组比较,A、B、D组总不良反应发生率均降低,差异均有统计学意义(P均<0.05);与E组比较,C组的总不良反应发生率升高,差异有统计学意义(P<0.05).结论 采用靶向药物吉非替尼治疗EGFR基因18、19和21号染色体上外显子突变的非小细胞肺癌患者效果较好,同时延长患者生存时间,降低不良反应;相反,采用靶向药物吉非替尼治疗EGFR基因20号染色体上外显子突变的非小细胞肺癌患者因出现耐药而效果不佳.     

胰腺内分泌肿瘤的外科诊断和治疗

  目的  探讨胰腺内分泌肿瘤的诊断及外科治疗方法。  方法  对收治的胰腺内分泌肿瘤16例患者的临床资料进行回顾性分析。  结果  本组16例患者中胰岛素瘤8例,胃泌素瘤4例,无功能性胰岛细胞瘤3例,生长抑素瘤1例。其中14例行根治切除术,2例因肿瘤无法切除而放弃手术治疗。术后发生胰瘘和炎性肠梗阻各2例,16例中3例失访,13例患者平均随访（4.5±2.3）年,2例仅行活检术的患者均在1年内死亡,2例恶性（胰岛素瘤和胃泌素瘤各1例）患者由于肿瘤复发转移在3年内死亡。随访期间良性病例全部生存。  结论  手术切除是胰腺内分泌肿瘤最为理想的治疗方法,选择合适的术式有助于避免术后并发症的发生和改善预后。      

单发脑转移瘤的外科治疗

本文报告了５１例单发脑转移瘤，全部为手术和病理检查所证实。５１例中，男性３２例，女性１９例，平均年龄为４６．８岁。这些患者的主要表现为颅内压增高。本研究结果表明年龄较小、身体条件好的单发脑转移瘤患者，如果他们的原发灶经过适当处理，或转移性脑瘤是可切除的，都应尽早地行手术治疗，并同时行减压术。术后患者应行全脑放疗和化疗，以便延长其生命，改善生活质量。     

Primitive Neuroectodermal Tumor of the Jejunum; A Case Report and Literature Review

Objective and Importance

The aim of this paper is to report an unusual presentation of extranodal follicular dendritic cell tumor of neck with spinal metastasis. Follicular dendritic cells are nonlymphoid immune accessory cells present in the germinal centers of lymphoid follicles and play a crucial role in the induction and maintenance of the humoral immune response. Tumors from these cells are rare and treatment modality poorly defined.

Clinical Presentation

A 37-year-old lady presented with recurrent neck swelling which was initially reported as malignant paraganglioma. The primary disease was treated with surgery and radiotherapy. Eleven years later, the patient presented with metastasis to the spinal cord. Subsequent immunohistochemical analysis of the primary site tumor and the metastatic deposits revealed it to be a follicular dendritic cell tumor.

Intervention

The patient was treated with surgery followed by radiotherapy to spine, and one and half year after treatment, the patient is doing well and has regained complete motor functions.

Conclusion

Metastasis to spinal cord for follicular dendritic cell tumor is very rare, and to the best of our knowledge, no such case has been previously reported in the scientific literature so far. In the present case, good local control was achieved with initial surgery and radiotherapy but resulted in distant failure after 11 years. This underlines the need for adjuvant systemic therapy, and understanding the biology of the tumor may help in formulating targeted therapy in the future for this rare disorder.     

肺癌侵犯胸壁的外科治疗

目的探讨肺癌侵犯胸壁的手术切除方式及影响患者生存的因素.方法对30例侵犯胸壁的肺癌患者的外科治疗结果进行综合分析.结果全组中肺叶切除26例,双肺叶切除2例,全肺切除2例;壁层胸膜外切除6例,胸壁肌肉和肋骨切除(整块切除)24例.根治性切除25例,根治切除率83.3%(25/30).手术并发症发生率6.7%(2/30),手术死亡率3.3%(1/30).鳞癌18例,腺鳞癌8例,腺癌3例,大细胞未分化癌1例.T3N0M0 20例,T3N1M0 5例,T3N2M0 5例.采用寿命表法(life table)计算生存率,用对数秩和检验(Logrank test)其显著性.1、3、5年生存率分别为40.2%、10.8%和10.8%.根治性切除患者5年生存率为13.2%,姑息性切除者中无5年生存者(P＞0.05).根治性切除无淋巴结转移者5年生存率为15.5%,有淋巴结转移者中无5年生存者(P＞0.05).不考虑淋巴结转移情况,根治性切除患者中,肿瘤侵犯胸壁局限于壁层胸膜者的5年生存率为15.0%,而侵犯胸壁肌肉和肋骨者中则无5年生存者(P＞0.05).结论胸膜外切除或胸壁整块切除是外科治疗肺癌侵犯胸壁的主要手段.能否根治切除、有无淋巴结转移以及胸壁受侵程度是影响患者术后生存的重要因素.     

乳腺原位癌的手术治疗和预后

目的探讨乳腺原位癌的手术方式和预后.方法对20年间获得随访的97例103个乳腺原位癌进行回顾性分析.结果乳腺原位癌5年生存率99.0%,10年生存率89.8%,15年生存率83.3%,20年生存率80.0%;导管内癌10年生存率偏低(P＜0.05);各种术式对预后的影响不明显(P＞0.05).结论原位癌的预后可能与乳腺癌的多原发灶有关,而与手术方式关系不明显;乳腺单纯切除术是乳腺原位癌的首选术式.