前纵隔原发性肿瘤78例CT分析

摘 要：［目的］ 探讨前纵隔原发性肿瘤的CT影像学特征,以提高鉴别诊断的准确性。［方法］ 回顾性分析经病理证实的78例前纵隔肿瘤的CT影像学特征,所有病例均行CT平扫及增强检查。［结果］45例胸腺瘤中40岁以上的有38例（84%）,17例（38%）伴重症肌无力的症状,29例（64%）表现为偏侧性生长。17例非侵袭性胸腺瘤中14例表现为密度均匀、边界清、轻中度强化的软组织密度肿物,28例侵袭性胸腺瘤多表现为边界不清、密度不均匀及明显不均匀强化的肿物;8例胸腺囊肿表现为边界清的液性密度肿物,增强检查无强化;14例淋巴瘤表现为肿块周围多发或孤立的结节,10例向纵隔两侧生长且伴有纵隔淋巴结肿大;11例生殖细胞肿瘤患者平均年龄为30岁,10例成熟型囊性畸胎瘤中表现为囊实性混杂密度的肿物,9例（90%）病变内可见脂肪密度。1例精原细胞瘤表现为右前纵隔类圆形的软组织密度肿物,增强检查为中度强化。不同前纵隔肿瘤有其特征性影像学表现。［结论］ CT检查是诊断前纵隔肿瘤类型的重要方法,结合临床特征,对于鉴别诊断有很大价值。     

甲状腺恶性肿瘤的CT诊断价值

目的：评价CT对甲状腺恶性肿瘤的诊断价值。方法：回顾分析了30例经手术和病理证实的甲状腺恶性肿瘤的CT征像。结果30例甲状腺恶性肿瘤的主要CT表现为形态不规则或类圆形的低密度区,密度均匀或不均匀,瘤壁不规则和不光滑,伴有坏死（例）、囊性变（2例）、钙化（6例）和淋巴结肿大（18例）。CT能正确地显示肿瘤向四周侵犯的程度。比如,气管受压（6例）,气管食管沟浸润和纵隔延伸（8例）局部皮肤浸润（21例）颈动脉鞘受侵犯（12例）、颈癌部淋巴结和颈部肌肉融合不清（8例）,结论CT对甲状遥恶性肿瘤诊断具有很大的价值。术前应用CT扫描可以明确病变的范围及临床术前评价和分期。     

30例原发性肺肉瘤样癌的CT表现

  目的  探讨原发性肺肉瘤样癌（PSC）的CT表现,提高对该疾病的认识。  方法  回顾性分析30例经病理证实的原发性肺肉瘤样癌患者的临床病理资料及CT表现。  结果  周围型27例,中央型3例;肿瘤直径3.0~9.2 cm,平均5.7 cm;右肺上叶10例、中叶1例、下叶8例,左肺上叶7例、下叶4例;肿瘤形态不规则13例,类圆形或卵圆形12例,浅或深分叶5例;边缘较光整者15例,毛糙者14例,1例因中央型肿块伴阻塞性肺不张致边界欠清。CT平扫密度均匀者9例,密度不均匀者21例,瘤内可见空洞者6例,伴钙化者4例;18例行CT增强扫描,中心呈不明显强化或不规则斑片状强化,周边呈厚薄不均环形强化。17例伴肺门和（或）纵隔淋巴结肿大,12例侵犯胸膜或胸壁组织,其中5例伴有邻近骨质破坏,5例出现转移。  结论  原发性肺肉瘤样癌的增强CT表现具有一定特征性,但确诊仍需依靠病理。      

前纵隔肿瘤的影像学与临床病理对照分析

目的:探讨前纵隔常见肿瘤的临床、病理及影像学特点。方法:回顾性分析经手术、穿刺活检病理证实的前纵隔肿瘤48例,并结合临床、病理资料进行对照分析。结果:胸腺瘤占50%(24例),均位于前上纵膈,11例可见囊变、坏死区或钙化,增强后呈轻度或不均匀强化。畸胎瘤占23%(11例),前上纵隔5例,前下纵隔6例,以囊实性为主,其内可见软组织、液体、脂肪及钙化成份,囊壁及实性成份呈轻中度强化。淋巴瘤17%(8例),可见多个结节样软组织融合呈团块状肿物,2例见坏死、囊变区,呈轻度强化或无强化。胸内甲状腺肿6%（3例）。心包囊肿4%（2例）。结论:依据前纵隔常见肿瘤发病部位、密度以及周围组织结构的关系,结合患者年龄伴随体征等特征,可提高前纵隔肿瘤的诊断与鉴别诊断准确率。     

肾占位性病变的CT定性诊断(英文)

目的分析肾占位性病变的 CT 表现,探讨 CT 对该类疾病的定性诊断价值。方法对手术/病例证实的28例肾占位性病变进行回顾性分析,所有病例均行平扫和增强扫描。结果肾恶性肿瘤21例(75%),其中肾癌(RCC)16例,CT 表现为肾实质内形态不规则的低密度软组织肿块,增强后不强化或轻度强化;肾盂癌2例,CT 表现为肾盂内软组织肿块影,增强后不强化或轻度强化。肾母细胞瘤(Wilms 瘤)3例,CT 表现为较大不规则的分叶肿块,增强后不强化;肾良性肿瘤3例(11%),均为肾血管平滑肌脂肪瘤(ALL),两例 CT 表现为多种不同成分的混杂密度肿块,一例表现为均匀等密度肿块,增强后软组织部分强化,而脂肪成分不强化。其它4例(14%),3例肾囊肿,CT 表现为典型的边界清楚的囊性病灶,增强后未见强化;1例肾脓肿,CT 表现为实质性与囊性混杂密度的肿块,增强后实质性部分有轻度强化,囊性部分不强化。结论 CT 平扫及增强扫描,以及诊断过程中密切结合病史,对肾占位性病变有重要的鉴别诊断价值。     

腹盆腔及腹膜后富血供肿瘤的CT诊断及误诊分析

摘 要：［目的］ 探讨腹盆腔及腹膜后间隙来源富血供肿瘤的CT征象。［方法］ 回顾性分析29例病理证实的腹盆腔及腹膜后富血供肿瘤的CT资料,结合文献进行CT征象探讨和误诊分析。［结果］ ①29例中,胃肠间质瘤（GIST）9例,副神经节瘤8例,Castleman病（CD）3例,孤立性纤维性肿瘤（SFT）3例,平滑肌瘤、平滑肌肉瘤、炎性肌纤维母细胞瘤（IMT）各2例。定位准确率96.6%（28/29）,定性准确率82.8%（24/29）,定病准确率69.0%（20/29）。② 增强后实性成分呈“快进慢出”的是副神经节瘤、间质瘤、IMT和CD;呈“持续渐进性强化”的是SFT、平滑肌瘤和平滑肌肉瘤;病灶内低密度区渐进性强化的是SFT、IMT、CD和平滑肌瘤;③ GIST好发于腹腔,分叶状,坏死范围大且多位于中心,易与肠腔相通形成“气-液”平面,邻近肠系膜血管常增多、增粗;副神经节瘤好发于腹膜后中线区,边界清楚,囊变、坏死常见且散在分布,部分呈“星芒状”改变,肿瘤以内部血管增多为主;CD好发于腹膜后肾门水平,呈圆形或类圆形,密度较均匀,边缘渗出,周围可见多发“卫星灶”,边缘可见增粗血管影;SFT好发于肠系膜区或髂血管旁,边界清楚,包膜完整,呈分叶状或匍匐状,密度不均,内可见索条状低密度区及增粗血管影;IMT好发于肠系膜区,呈圆形或分叶状,边界清楚或不清楚,边缘可见渗出和增粗血管影,密度不均,内可见索条状低密度影或片状粘液变性区;平滑肌瘤好发于腹膜后或盆腔内,边界清楚,容易粘液变性,增强后低密度区和实性成分均渐进性强化,与子宫肌层密度相仿;平滑肌肉瘤好发于腹膜后间隙或下腔静脉旁,分叶状,坏死明显,可见肿瘤血管生成,易侵犯下腔静脉。［结论］ 腹盆腔及腹膜后间隙起源的富血供肿瘤具有一定的影像学特点,CT对肿瘤的定位、定性及定病诊断有重要价值。     

CT在嗜铬细胞瘤中的诊断价值

[目的]探讨CT对嗜铬细胞瘤的诊断价值。[方法]回顾性分析20例经手术病理证实的嗜铬细胞瘤的CT表现，包括肿瘤部位、大小、形态、密度及强化特征。[结果]良性16例．恶性4例：肿瘤他于肾上腺者18例，其中1例为双侧，其余为单发，共21个病灶。良性肿瘤表现为边缘光整的圆形或类圆形肿块，其中密度均匀2例，12例密度不均匀，内见单个或多个结节状低密度区，2例表现为厚壁囊肿样病灶；恶性表现为边缘呈分叶状肿块、侵袭性生长、中心不规则坏此．增强扫描动脉期14个病灶实质部分明显强化，其巾4个病灶内呢粗大扭曲的血管断面、坏死和囊变部分不强化．6个病灶呈轻中度强化，1个病灶无明显强化。门脉期仍有12个病灶持续强化，8个病灶强化程度下降，1个病灶始终不强化。[结论]嗜铬细胞瘤多为富血供的肿瘤．CT表现具有一定的特征性，CT具有重要的定位和定性诊断价值．     

孤立性纤维性肿瘤的影像学表现及病理分析

目的探讨孤立性纤维性肿瘤(SFT)的影像表现及病理特征,进一步提高对本病的认识。方法回顾性分析17例经过手术或穿刺活检病理证实的SFT患者临床资料。结果17例SFT均为单发,位于胸腔5例,肺内、腹膜后及颅脑各2例,纵隔、肋骨、颈部、肾脏、腹壁及骶管各1例。病灶形状多样,边界清晰。16例行CT平扫,呈等、稍低密度,密度均匀或不均匀,4例伴有钙化。13例行CT动态增强扫描,2例轻度均匀强化,11例强化不均匀,其中“慢进慢出”强化表现(n=2),“快进慢出”强化表现(n=9);动脉期8例瘤内或瘤周可见迂曲粗大血管影。3例行MRI平扫及增强扫描,1例呈囊实性,实性部分T1WI呈等信号,T2WI呈等低信号,增强后实性部分明显强化,囊内分隔强化;另2例平扫T1WI、T2WI呈等信号,T2WI内见小斑片状、小囊状高信号影,增强后呈不均匀明显强化。光镜下肿瘤组织主要由不同比例的梭形细胞及胶原纤维构成,部分病灶内富含分支状或"鹿角状"薄壁血管。免疫组化示大部分肿瘤CD34(13/17)、CD99(10/17)、bcl-2(9/17)阳性,其中9例行STAT6检测,阳性率为100%。结论SFT影像表现具有一定特征,尤其是病灶T2WI低信号区增强后强化及CT动态增强具有延迟强化。免疫组化CD34及STAT6阳性有重要诊断价值。     

30例小细胞肺癌的CT表现

目的分析小细胞肺癌的CT表现特点,以提高对该病的认识和诊断能力。方法回顾性分析30例经病理证实的小细胞肺癌的螺旋CT表现。结果中央型肺癌24例,CT表现为肺门分叶状结节或肿块,伴阻塞性肺炎5例（20.8%）,阻塞性不张3例（12.5%）,21例（84.5%）纵隔淋巴结肿大。25例行CT增强,其中23例肿块和淋巴结均匀强化,11例（45.8%）纵隔大血管被包埋,周围型6例,表现为边缘规整的结节状或葡萄状。结论小细胞肺癌以中心型为主,呈实体性生长,CT上肿块密度较均匀,易侵犯支气管及纵隔大血管,伴肺门、纵隔淋巴结转移。     

胰腺实性假乳头状瘤的影像学诊断与病理对照分析

[目的]分析胰腺实性假乳头状瘤（SPTP）的CT和MRI表现,并与病理结果对照分析。[方法]回顾性分析12例经手术和病理证实的S门P的I临床及CT和MRI表现．分析肿瘤的部位、大小、形态、密度、信号以及强化方式,并将CT与MRI表现与病理对照。[结果]SPTP好发于胰头,影像学表现为境界清楚的圆形或类圆形胰腺肿块,瘤体通常比较大。CT主要表现为囊实性混杂密度影,部分实性结构呈乳头状或壁结节样突起,增强后实性部分呈渐进性强化;MRI表现为肿块在T1WI、T2WI上呈不均匀混杂信号,可识别肿瘤内部的坏死囊变及出血等特异性征象,实性部分增强呈渐进性强化。[结论]胰腺实性假乳头状瘤影像学表现具有一定特征性,对其诊断具有重要指导意义。     

Primary carcinoid tumor of the testis: case report, ultrastructure and review of the literature.

A case of primary pure carcinoid tumor of the testis which occurred in a 71-year-old male is reported. The patient was treated by radical orchiectomy and remains well and symptom free 10 months after operation. Histologically as well as ultrastructurally the tumor showed typical appearances of carcinoid tumor of midgut derivation. 23 cases of carcinoid tumors of the testis were discovered in the literature. Of these 17 were primary testicular carcinoids, and 6 were metastatic to the testis. Of the 17 cases of primary carcinoid tumors, 14 were pure carcinoids and only 3 were associated with teratoma. None of the primary testicular carcinoids were associated with metastases and the prognosis after orchiectomy was excellent, thus indicating that no further therapy is necessary. The prognosis of patients with carcinoid metastatic to the testis is poor. In view of this it is very important to determine whether the tumor is primary or metastatic.     

胸腺上皮源性肿瘤的WHO组织学分型和CT征象的相关性

背景与目的:胸腺上皮肿瘤的生物学和大体形态学的特征多样化,本文旨在探讨根据1999年WHO组织学分型的各亚型胸腺上皮源性肿瘤的CT征象。方法:对经病理检查证实的94例胸腺上皮源性肿瘤患者螺旋CT征象进行回顾性分析,其中包括A型7例,AB型24例,B1型16例,B2型13例,B3型16例,C型18例。结果:在本组94例患者病灶中,A、AB型的长短径明显小于C型(P<0.05);所有A型肿瘤均表现为轮廓光滑,A→C型出现率逐渐下降,A、AB、B1型更常呈轮廓光滑(P<0.05),B3、C型更常呈轮廓不规则(P<0.05);A型瘤内的坏死灶较其他亚型少见(P<0.001);B2、B3、C型肿瘤较A、AB、B1型的瘤灶内更常见多发钙化灶(P<0.05);瘤灶均匀强化更常见于A、AB、B1、B2型(P<0.001);B3、C型瘤灶浸润纵隔脂肪的发生率明显高于其他亚型(P<0.05)。结论:尽管各亚型肿瘤的CT征象存在一定的重叠,小肿瘤、轮廓光滑、类圆形、密度均匀(无钙化、坏死灶)、均匀强化高度提示A型肿瘤,大肿瘤、轮廓不规则、瘤内出现坏死灶、多发钙化、不均匀强化、浸润纵隔脂肪、浸润大血管等高度提示B3、C型肿瘤。     

肾脏肌样型血管平滑肌脂肪瘤的CT特征

背景与目的:肾脏占位性病变的检出日益增多,肾脏的肌样型血管平滑肌脂肪瘤(myomatous angiomyolipoma,mAML)与恶性病变的鉴别对诊疗有重要意义.该研究旨在探讨肾脏mAML的CT特征,以提高其诊断及鉴别的准确性.方法:回顾性分析手术病理证实的肾脏mAML10例.其中男性4例,女性6例,年龄38~64岁,平均53.2岁.分析其CT平扫、增强和临床资料,记录病灶的一般形态特征,有无可见脂肪、钙化、假包膜、瘤内囊变或出血,劈裂征是否阳性;观察其平扫及动态增强CT表现,并与邻近正常肾脏实质和同侧腰背部骨骼肌对照.结果:10例病例中,9例为单发,1例伴混合型血管平滑肌脂肪瘤.病灶位置位于左肾6例,右肾4例;5例位于肾外,5例位于肾内.所有病例均边界清楚,9例形态规则.肿瘤最大径15~80 mm,平均(43±19)mm.2例病灶含少量可见脂肪,3例含增粗的瘤内血管,2例伴囊变,所有病灶均无钙化或出血.CT平扫结果显示,7例病灶呈稍高密度,3例为等密度,5例病灶均质,3例为轻度不均,2例为不均.病灶平均CT值为(48.60±5.50)HU,显著高于邻近正常肾脏实质[(39.70±6.67)HU](P<0.05),与骨骼肌平均CT值[(48.90±2.28)HU]差异无统计学意义(P>0.05).增强扫描结果显示,4例呈轻度强化,5例中度强化,1例明显增强;强化均匀、轻度不均匀和不均匀者分别为3例和4例、3例.持续性强化者为4例,渐进性强化者为4例,快进快出者为2例.结论:mAML在CT上主要表现为边界清楚的结节,大多不含可见脂肪,平扫呈稍高密度,均质或轻度不均,增强后呈轻度至中度强化,强化均匀或轻度不均,渐进性强化或持续性强化.对于影像表现的综合分析有利于其诊断和鉴别诊断.     

Clinical Features,Treatments, and Outcomes of Patients with Thymic Carcinoids and Multiple Endocrine Neoplasia Type 1 Syndrome at MD Anderson Cancer Center

Thymic carcinoids are rare neuroendocrine tumors that occur in 1–5 % of patients with multiple endocrine neoplasia type 1 (MEN1) and are a major cause of morbidity and mortality. The few published reports associate these tumors with male sex and smoking. Our objective was to describe cases of these tumors treated at our institution. We performed a retrospective chart review of all patients diagnosed with MEN1 at our tertiary referral center from 1980 to 2014. Patients with a histopathologic, fine-needle aspiration, or clinical diagnosis of a thymic carcinoid were included. Two hundred ninety-one patients fulfilled the criteria for a diagnosis of MEN1. Clinicopathologic characteristics, MEN1 genetic testing results, treatments, and survival rates were analyzed. Nine patients had a thymic carcinoid, six men (67 %) and three women (33 %). Six patients were non-smokers (67 %). Two patients had synchronous (22 %) and eight patients (89 %) had metachronous distant metastasis. The 10-year overall survival rate was 45 % (lower 95 % upper 95 % CI 20–100 %). The 10-year disease-free survival rate was 42 % (lower 95 % upper 95 % CI 15–100 %). Five patients had MEN1 genetic testing, and the genotypes of affected individuals were p.W341X, c.275_286delGCTTCACCGCCC, p.R98X, c.1350+(1_11)del11, and partial duplication of exons 9 and 10. A higher percentage of MEN1-related thymic carcinoids can occur in women and in non-smokers than previously reported. Both novel and known mutations were present in our cohort. Eighty nine percent of patients developed a metachronous metastasis from the thymic carcinoid. Patients with MEN1 and thymic carcinoids should be followed closely.     

Paraneoplastic Cushing's syndrome associated to locally advanced thymic carcinoid tumor

BACKGROUND: Thymic carcinoid is a frequent cause of Cushing's syndrome due to ectopic adrenocorticotropin secretion. Histology and immunohistochemistry allow differential diagnosis from other epithelial thymic tumors, such as thymomas and thymic carcinomas. The term used to name this tumor is confusing, since it is a malignant neuroendocrine neoplasm, and therapeutic approaches need to bear that in mind. CASE REPORT: Unlike most cases of thymic carcinoid associated to Cushing's syndrome that had distant metastases at diagnosis, we report a 50-year-old male who presented with Cushing's syndrome and was diagnosed with thymic carcinoid without distant metastases. Multimodal treatment with surgery, radiotherapy and chemotherapy (cisplatin plus etoposide) induced a complete clinical and biochemical remission lasting for 46 months.     

Tryptophan hydroxylase and 5-hydroxytryptamine in carcinoids of the gastrointestinal tract

BACKGROUND: Tryptophan hydroxylase is the enzyme that catalyzes the formation of 5-hydroxy-L-tryptophan which is the initial and rate-limiting enzyme in the biosynthesis of 5-hydroxytryptamine (serotonin). The aim of this study was to analyze the differential expression of tryptophan hydroxylase and 5-hydroxytryptamine in human gastrointestinal carcinoids. MATERIAL AND METHODS: Primary carcinoids and hepatic carcinoid metastases (n = 8) were investigated for the presence of tryptophan hydroxylase, 5-hydroxytryptamine and chromogranin A. RESULTS: Only one of the tumors examined was immunoreactive for tryptophan hydroxylase, as determined by immunohistochemical techniques and Western blot analysis. All carcinoids expressed 5-hydroxytryptamine and chromogranin A. CONCLUSIONS: Carcinoids appear to be a heterogeneous group with respect to the expression of this monooxygenase. Based on these results it is proposed that other cells of the intestine are involved in the biosynthesis of 5-hydroxytryptamine precursors taken up by carcinoid tumor cells.     

卵巢Brenner瘤的CT表现

目的 分析卵巢Brenner瘤的CT表现,帮助临床医生提高对卵巢Brenner瘤术前诊断的准确率.方法 收集1999年1月至2009年1月间经术后病理证实、有完整CT资料的卵巢Brenner瘤9例,其中良性8例,交界性1例.分析卵巢Brenner瘤的CT表现等.结果 9例卵巢Brenner瘤均为单侧发病,呈类球形、浅分叶状或不规则形,平均直径为7.8 cm,边界清楚.9例卵巢Brenner瘤中,单纯良性Brenner瘤5例,良性Brenner瘤伴有其他成分3例,交界性Brenner瘤1例.5例单纯良性Brenner瘤的CT表现均为实性或伴有小囊变,密度低于肌肉,其中2例伴有钙化,2例增强扫描后呈轻度不均匀强化.3例良性Brenner瘤伴有其他病理类型者的CT表现呈囊实性或囊性,囊实性成分分界清晰,实性成分密度亦低于肌肉,呈轻度强化,其中1例伴有钙化.1例交界性Brenner瘤的CT表现为不均匀实性肿块,密度高于肌肉,伴有大片低密度区及大块状钙化,增强扫描后呈明显不均匀强化.9例患者均无转移和胸腹水.结论 卵巢Brenner瘤常为单侧发病,合并其他肿瘤成分者多见.当肿瘤成分单一时,在影像学上常表现为实性,密度均匀或不均匀;当合并其他肿瘤成分时,可表现为囊实性或囊性,部分可伴钙化.在强化形式上,良性肿瘤呈轻度强化,交界性肿瘤呈中高度强化.     

Carcinoid tumors of the extrahepatic bile duct. A rare cause of malignant biliary obstruction

BACKGROUND: Carcinoid tumors of the extrahepatic bile duct are rare and account for 0.2-2% of all gastrointestinal carcinoids. Similar to other tumors of the bile duct, these lesions are difficult to diagnose preoperatively and nearly impossible to distinguish from cholangiocarcinoma. METHODS: The authors retrospectively analyzed all reported cases of carcinoid tumor of the bile duct and report on two additional cases. RESULTS: Thirty cases of carcinoid tumor of the bile duct have been reported. Among this group were 20 women and 9 men (female-to-male ratio, 2.2:1) with an overall mean age of 47 years (range, 19-79 years). The most common anatomic sites for extrahepatic carcinoid tumors were the common bile duct (58%), perihilar region (28%), cystic duct (11%), and common hepatic duct (3%). Jaundice was the most common presenting complaint (55%). Sixty-nine percent of patients with extrahepatic biliary carcinoids had disease confined to the bile duct, whereas 31% had evidence of distant metastases. All patients who presented with localized disease remain disease free with a mean follow-up of 32 months (range, 3 months to 20 years). CONCLUSIONS: Carcinoid tumor of the bile duct is a rare form of malignant biliary obstruction. Unlike cholangiocarcinoma, biliary carcinoids occur more commonly in younger patients and in women. Aggressive local invasion by the primary tumor is rare, and metastases occur in less than one-third of patients. All patients who underwent a curative surgical resection were alive and disease free at time of published report, implying a more favorable prognosis. Aggressive surgical resection is recommended.     

Detection of somatostatin receptors in surgical and percutaneous needle biopsy samples of carcinoids and islet cell carcinomas

Somatostatin (SS) receptor status was investigated in the tumor tissues from 62 patients with carcinoid tumors and 15 patients with islet cell carcinomas using receptor autoradiography techniques with two different iodinated somatostatin analogues as radioligands, a [Leu8, DTrp22, Tyr25]somatostatin-28 and a somatostatin octapeptide, Tyr3-octreotide. The carcinoid tumors were either primaries (n = 32) or metastases (n = 43), sampled as surgical specimens or as small needle liver biopsies. Fifty-four of 62 carcinoid patients had SS receptor-positive tumors (87%). All 15 islet cell carcinoma patients had positive tumors (4 primaries, 11 metastases), i.e., 3 vipomas, 3 insulinomas, 2 glucagonomas, 1 gastrinoma, 2 polyfunctional tumors, and 4 nonfunctioning tumors. Saturation and competition experiments on tissue sections revealed saturable, high affinity binding sites pharmacologically specific for bioactive SS analogues. In a majority of the tumors, the receptors were densely distributed and were always homogeneously found in the whole tumor. All except two tumors were labeled with both radioligands. Multiple liver metastases (n = 16) from three different patients were all shown to contain a comparable amount of receptors. SS receptors could be demonstrated even in very small tissue samples of liver metastases obtained by percutaneous liver biopsies (mean weight, 6.8 mg). The majority of the eight SS receptor-negative carcinoids were mainly bronchial carcinoids (n = 5), usually poorly differentiated. On the contrary, SS receptor-positive cases were never found to be anaplastic. All tumors except one from patients pretreated with octreotide (3 days to 3.8 years) were SS receptor positive. In the majority of carcinoids or islet cell carcinomas, the SS receptor status correlated with the in vivo biochemical response (hormone inhibition) to octreotide. These data demonstrate (a) the high prevalence of SS receptors in the primary tumors of both carcinoids and islet cell carcinomas, (b) their presence in metastases as well, (c) their continuous expression even during long term octreotide therapy, (d) the possibility of measuring SS receptors in percutaneous needle liver biopsies, and (e) the evidence of their functionality. This study therefore suggests that tumoral SS receptors may be the likely molecular basis for octreotide action and may be an important parameter for predicting the therapeutic efficacy of SS analogues in carcinoids and islet cell carcinomas.     

A 5-decade analysis of 13,715 carcinoid tumors

BACKGROUND: Carcinoid tumors represent an unusual and complex disease spectrum with protean clinical manifestations. This compilation of several large United States-based databases comprising patients from 1950 to 1999 examines 13,715 carcinoid tumors and provides epidemiologic information regarding the natural history and evolution of the detection and diagnosis of this entity. METHODS: The authors evaluated 10,878 carcinoid tumors that were identified by the Surveillance, Epidemiology, and End Results (SEER) Program of the National Cancer Institute (NCI) from 1973 to 1999 in addition to 2837 carcinoid tumors that were registered previously by two earlier NCI programs. To the authors' knowledge, this represents the largest current epidemiology series addressing carcinoid tumors to date. RESULTS: Specific trends in incidence for carcinoid tumors of certain sites were identified. Among the most recently collected subset of data, sites that demonstrated the greatest incidence of carcinoids were the gastrointestinal tract (67.5%) and the bronchopulmonary system (25.3%). Within the gastrointestinal tract, most carcinoid tumors occurred in the small intestine (41.8%), rectum (27.4%), and stomach (8.7%). For all sites, age-adjusted incidence rates were highest in black males (4.48 per 100,000 population per year). Associated noncarcinoid tumors were frequent in conjunction with small intestinal (29.0%), gastric (20.5%), colonic (20.0%), and appendiceal (18.2%) carcinoids. The highest percentages of nonlocalized lesions were noted for cecal (81.5-83.2%) and pancreatic (71.9-81.3%) carcinoids, whereas the highest percentage of localized disease was found among rectal (81.7%), gastric (67.5%), and bronchopulmonary (65.4%) carcinoids. The best 5-year survival rates were recorded for patients with rectal (88.3%), bronchopulmonary (73.5%), and appendiceal (71.0%) carcinoids; these tumors exhibited invasive growth or metastatic spread in 3.9%, 27.5%, and 38.8% of patients, respectively. CONCLUSIONS: Carcinoids appear to have increased in overall incidence over the past 30 years; for some sites, this trend has been evident for nearly half a century. Recent marked increases in gastric and rectal carcinoids and a concomitant decrease in appendiceal carcinoid incidence may be due in part to varying rules of registration among the compiled databases examined in this report or to improvements in diagnostic technology; increased awareness of and about carcinoid tumors also may play a significant role. In 12.9% of all patients with carcinoid, distant metastases already were evident at the time of diagnosis; the overall 5-year survival rate for all carcinoid tumors, regardless of site, was 67.2%. These findings bring into question the widely promulgated relative benignity of carcinoid disease. Certain carcinoid tumors, such as those of the rectum, appear to be over-represented among the black and Asian populations within the United States, suggesting the role of genetics in the development of this intriguing disease.