 都司影像專欄  病例回顧 1 患者病史 · 一般信息：女，69歲，因上腹部間歇性疼痛2月入院 · 現(xiàn)病史：患者2月前無(wú)明顯誘因出現(xiàn)上腹部間歇性疼痛,，無(wú)惡心,、嘔吐，無(wú)胸悶,、心悸,，自服“健胃消食片”，無(wú)明顯療效,。為求進(jìn)一步診治,，來(lái)我院就診。起病以來(lái),，一般情況好,，神清，大小便正常,，飲食無(wú)明顯變化 · 既往史：患者既往體健,，否認(rèn)高血壓、糖尿病史,；否認(rèn)乙肝,、結(jié)核等傳染病史；否認(rèn)食物,、藥物過(guò)敏史,；5歲時(shí)行“右側(cè)結(jié)腸”手術(shù) · 本院彩超：肝左葉外側(cè)實(shí)性占位 2 影像檢查          上下滑動(dòng)查看共12張影片 上下滑動(dòng)查看共12張影片 上下滑動(dòng)查看共8張影片 3 手術(shù)記錄 …探查可見胃及橫結(jié)腸之間胰腺頸、體部可見一凸起性腫塊,，大小約18.0cm × 12.0cm,，將胃及橫結(jié)腸壓向兩邊，向后方擠壓左側(cè)腎臟及腎血管,，向左側(cè)推擠脾臟,，邊界較清，活動(dòng)度好,。肝臟色質(zhì)尚可,，表面尚光滑，未觸及明顯結(jié)節(jié)和包塊,。胃漿膜面尚光滑,，未觸及明顯腫塊；大網(wǎng)膜,、盆腔未及明顯腫塊…解剖標(biāo)本,，腫塊呈菜花樣，質(zhì)硬,，切面呈魚肉樣… 4 光鏡所見 5 病理診斷 胰腺腫瘤+脾切除標(biāo)本： 1、（胰腺腫瘤）結(jié)合免疫組化，符合平滑肌肉瘤,。 2,、胰腺切緣組織未見腫瘤。 3,、脾臟局灶海綿狀血管瘤形成,。 4、胰周淋巴結(jié)（0/3）枚均未見腫瘤,。 5,、免疫組化結(jié)果顯示：CD117(-), CD34(血管+), Desmin(+), DOG-1(-), Ki67(+, 約30%), S-100(-), SDHB(+), SMA(+), P16(+), P53(-), ER(弱+), PCK(-), PR(-), EMA(+), CDK4(-), MDM2(-), H3K27ME3(+), H-Caldesmon(+)。 原位雜交：EBER(-),。 最終診斷：原發(fā)性胰腺平滑肌肉瘤 (Primary Pancreatic Leiomyosarcomas) 一 疾病介紹 (Introduction) 全文疾病介紹模塊均為中英文對(duì)照版本 English introduction is attached · 平滑肌肉瘤是一種罕見的起源于平滑肌的惡性腫瘤,，占所有惡性腫瘤的1%以下，占肉瘤的2%-9% ,。它們主要發(fā)生在胃腸道,、腹膜后、泌尿系,、子宮和軟組織中 · 原發(fā)性胰腺平滑肌肉瘤是一種罕見的惡性腫瘤,，占所有胰腺惡性腫瘤的 0.1% · 腫瘤源自胰腺中的血管或?qū)Ч艿钠交?/p> · 常見癥狀包括上腹痛、體重減輕,、黃疸,、惡心和嘔吐，偶爾為意外發(fā)現(xiàn),。平均年齡為 45.3 歲（14-83 歲） · 胰腺平滑肌肉瘤可能位于胰頭,、胰體或胰尾，腫瘤在發(fā)現(xiàn)時(shí)通常很大 · 增強(qiáng)CT 表現(xiàn)為伴/不伴壞死或鈣化的腫塊,，密度不均,，可能有鄰近器官（如十二指腸）的受壓和侵襲 · Leiomyosarcomas are rare malignant tumors of smooth muscle origin. They comprise less than 1% of all cancers and 2%-9% of sarcomas. They appear mostly in the gastrointestinal tract, retroperitoneum, urinary tract, uterus, and soft tissue · Primary pancreatic leiomyosarcoma is a rare malignant neoplasm. It accounts for 0.1% of all malignant pancreatic cancers · The tumor is considered to derive from the vasculature or ductal smooth muscle in the pancreas · Usual symptoms are epigastric pain, weight loss, jaundice, nausea and vomiting. Incidentally detected cases have also been reported. The mean age is 45.3 (14-83 years) · Pancreatic leiomyosarcomas may be located in the pancreatic head, body, or tail. These tumors are usually large in size at presentation · CECT characteristics of leiomyosarcomas include a heterogeneous mass with or without necrosis or calcification. Due to the large size of the lesion, compression and invasion of neighboring organs (such as the duodenum) can be present · Pancreatic leiomyosarcomas appear isointense with skeletal muscle on T1-weighted images, hyperintense on T2-weighted images, and exhibit heterogeneous enhancement following intravenous gadolinium like most leiomyosarcomas · About 25% of pancreatic leiomyosarcomas may already have distant metastases at the time of diagnosis, and 19% of patients present with local infiltration. Lymph node metastasis is an extremely rare finding in patients with pancreatic leiomyosarcoma Scroll down for more 向下滑動(dòng)查看更多 CASE 1：胰頭平滑肌肉瘤 71歲，女性,，胰頭平滑肌肉瘤 CASE 2：胰尾平滑肌肉瘤 55 歲男性,，胰尾平滑肌肉瘤，CT及T1WI增強(qiáng)示胰尾 17mm× 14 mm 不均勻增強(qiáng)病灶 CASE 3：胰腺體尾部平滑肌肉瘤 56 歲女性,，胰腺體尾部平滑肌肉瘤,，并囊變、壞死及鈣化 CASE 4：胰腺體尾部平滑肌肉瘤 69 歲男性,，胰腺體尾部平滑肌肉瘤,，并囊變、壞死 二 鑒別診斷 胰腺間質(zhì)腫瘤 Pancreatic MesenchymalTumors · 胰腺間質(zhì)腫瘤相對(duì)罕見,，包括結(jié)締組織,、淋巴組織、血管組織和神經(jīng)組織等來(lái)源 · 根據(jù)其組織學(xué)來(lái)源可分為脂肪瘤、神經(jīng)鞘瘤,、孤立纖維瘤,、血管周圍上皮細(xì)胞瘤、肉瘤,、促纖維增生的小圓細(xì)胞瘤和淋巴管瘤 · Although the great majority of pancreatic neoplasms arise from pancreatic epithelial cells, mesenchymal tumors, while rare, can be derived from non-epithelial cells, including the connective, lymphatic, vascular, and neuronal tissue of the pancreas · Mesenchymal tumors are classified according to their histologic origin, including lipoma, schwannoma, solitary fibrous tumor, perivascular epithelial-cell neoplasm, sarcoma, desmoplastic small-round-cell tumors , and lymphangioma CASE 1：胰腺脂肪瘤 CASE 2：胰腺神經(jīng)鞘瘤 胰腺神經(jīng)內(nèi)分泌腫瘤 PancreaticNeuroendocrine Neoplasms (PNETs) · PNETs是一類起源于神經(jīng)內(nèi)分泌細(xì)胞的異質(zhì)性腫瘤,，具有顯著的行為差異 · 發(fā)病率無(wú)性別差異，胰腺各部均可發(fā)病 · 根據(jù)相關(guān)的臨床癥狀將其分為功能性和非功能性,，其中胰島素瘤,、胃泌素瘤和胰高血糖素瘤是最常見的功能性PNETs · 影像學(xué)檢查，PNETs常表現(xiàn)為邊界清晰的富血供腫塊,，提示其有豐富的毛細(xì)血管網(wǎng) · 腫瘤較大時(shí)常見囊變,、鈣化和壞死，且預(yù)后相對(duì)較差,，易發(fā)生局部血管侵犯和轉(zhuǎn)移 · 磁共振成像，腫瘤在T1WI呈低信號(hào),、T2WI呈等或高信號(hào) · PNETs血供極其豐富,，因此在動(dòng)脈期呈顯著強(qiáng)化，而且較正常胰腺組織強(qiáng)化更為迅速和明顯 · 小的腫瘤往往呈均勻強(qiáng)化,，大的腫瘤強(qiáng)化不均,，可呈環(huán)形強(qiáng)化 · 在門脈期，腫瘤強(qiáng)化信號(hào)多變,，有些可表現(xiàn)為非典型的延遲強(qiáng)化 · 腫瘤轉(zhuǎn)移到淋巴結(jié)和實(shí)性器官（如肝臟）,，可有類似于原發(fā)腫瘤的強(qiáng)化方式 · PNETs constitute a heterogeneous group of tumors that originate from neuroendocrine cells and which have significant behavioral differences · PNETs occur with an equal frequency in men and women. PNETs are found with equal frequency throughout the pancreas · They are classified as functioning or nonfunctioning according to their associated clinical symptoms, and insulinoma, gastrinoma, and glucagonoma are the most common functioning PNETs · On imaging, PNETs typically appear as well-defined, hypervascular masses, a finding indicative of their rich capillary network · Cystic change, calcification, and necrosis are common in large tumors which are associated with a poorer prognosis and a higher prevalence of local and vascular invasion and metastases than smaller tumors · On MR imaging, PNETs generally have low signal intensity on T1-weighted images and intermediate to high signal intensity on T2-weighted images · PNETs have a rich vascular supply and therefore enhance substantially during the arterial phase, thus enhancing more rapidly and intensely than the normal pancreas · Homogeneous enhancement is typical for small tumors, whereas larger lesions tend to show heterogeneous enhancement which can be ringlike · During the portal phase, tumors may be either hyper-, iso-, or hypoenhancing relative to the normal pancreas. Some tumors demonstrate atypical delayed enhancement and are best seen on portal phase imaging · Metastases to lymph nodes and solid organs, such as the liver, may have an enhancement pattern similar to that of the primary tumor Scroll down for more 向下滑動(dòng)查看更多 CASE：胰腺神經(jīng)內(nèi)分泌腫瘤 - 胰島素瘤 胰腺轉(zhuǎn)移瘤 Metastasisto the Pancreas · 胰腺轉(zhuǎn)移瘤少見,。最常見的是腎細(xì)胞癌(RCC)和肺癌，其次是乳腺癌,、結(jié)直腸癌和黑色素瘤，多數(shù)患者無(wú)癥狀 · 胰腺轉(zhuǎn)移瘤一般比胰腺癌的預(yù)后好 · 有單發(fā),、多發(fā)和彌漫型三種 · 單發(fā)型通常邊界清晰,，在CT平掃上呈低/等密度，在T1WI上呈低信號(hào),，T2WI呈高信號(hào) · 增強(qiáng)掃描,，胰腺轉(zhuǎn)移瘤與原發(fā)腫瘤表現(xiàn)非常相似 · Metastasis to the pancreas is an uncommon occurrence accounting. Metastases to the pancreas are most frequently from renal cell carcinoma (RCC) and lung carcinoma , followed by breast carcinoma, colorectal carcinoma , and melanoma , and most of these patients are asymptomatic · The prognosis is generally more favorable than that for pancreatic adenocarcinoma · There are three, morphologic patterns of involvement: solitary, multifocal, and diffuse · The solitary form is generally well marginated. Masses may be hypo- or isoattenuating on unenhanced CT. Metastases typically have low signal intensity on T1-weighted MR images and high signal intensity on T2-weighted images. On contrastenhanced CT and MR imaging, the appearance of pancreatic metastases closely resemble that of primary carcinoma CASE：腎癌胰腺轉(zhuǎn)移瘤 作者 醫(yī)院名稱：武漢大學(xué)人民醫(yī)院放射科 主治醫(yī)師：陳思涵 來(lái)源 優(yōu)維顯?370 Ultravist?370 簡(jiǎn)明處方 在使用前應(yīng)該對(duì)對(duì)比劑進(jìn)行目視檢查，不得使用變色,、存在顆粒物質(zhì)（包括結(jié)晶）或容器存在破損的對(duì)比劑,。 【藥品名稱】 通用名稱: 碘普羅胺注射液 商品名稱: 優(yōu)維顯? Ultravist? 英文名稱: Iopromide Injection 【成份】本品活性成份為碘普羅胺 【適應(yīng)癥】用于診斷用藥。碘普羅胺注射液300/370 用于計(jì)算機(jī)X線體層掃描(CT)增強(qiáng),，動(dòng)脈造影和靜脈造影,，動(dòng)脈法/靜脈法數(shù)字減影血管造影(DSA)，靜脈尿路造影,，內(nèi)窺鏡逆行胰膽管造影(ERCP),，關(guān)節(jié)腔造影和其他體腔檢查。碘普羅胺注射液370 特別適用于心血管造影,。 【用法用量】碘普羅胺注射液300/370 用于血管內(nèi)和體腔內(nèi),，不能在鞘內(nèi)使用。劑量應(yīng)依據(jù)年齡,，體重,，臨床情況和檢查技術(shù)來(lái)進(jìn)行調(diào)整。詳見說(shuō)明書,。 【不良反應(yīng)】最常見頭痛,、惡心和血管擴(kuò)張。使用本品的患者中發(fā)生的最嚴(yán)重的藥物不良反應(yīng)（在臨床試驗(yàn)或上市后監(jiān)測(cè)過(guò)程中已有威脅生命和/或致死性病例報(bào)告的）包括：類過(guò)敏性休克,、呼吸停止,、支氣管痙攣、喉水腫,、咽部水腫,、哮喘,、昏迷、大腦梗死,、卒中,、腦水腫、驚厥,、心律失常,、心臟停搏、心肌缺血,、心肌梗死,、心力衰竭、心動(dòng)過(guò)緩,、發(fā)紺,、低血壓、休克,、呼吸困難,、肺水腫、呼吸功能不全和誤吸,。除了上面列出的不良反應(yīng)以外,，鞘內(nèi)使用時(shí)曾報(bào)告：化學(xué)性腦膜炎和假性腦膜炎（頻率不明）；用于ERCP檢查時(shí)報(bào)告：胰酶水平升高和胰腺炎（頻率不明）,；脊髓或體腔造影后發(fā)生的不良反應(yīng)大部分出現(xiàn)在給藥后數(shù)小時(shí)內(nèi),。詳見說(shuō)明書。 【禁忌】對(duì)含碘對(duì)比劑過(guò)敏及明顯的甲狀腺功能亢進(jìn)的患者禁用,。妊娠及急性盆腔炎患者禁行子宮輸卵管造影,。急性胰腺炎時(shí), 禁行ERCP (內(nèi)窺鏡逆行性胰膽管造影)。 禁用于鞘內(nèi)注射,。鞘內(nèi)給藥也許會(huì)導(dǎo)致死亡,、驚厥、腦出血,、昏迷,、癱瘓、蛛網(wǎng)膜炎,、急性腎功能衰竭,、心臟驟停、抽搐,、橫紋肌溶解癥,、高熱和腦水腫、化學(xué)性腦膜炎,、假性腦膜炎,。 【注意事項(xiàng)】對(duì)于所有的適應(yīng)癥,，血管內(nèi)使用時(shí)危險(xiǎn)性較高。 對(duì)于已知對(duì)碘普羅胺注射液或其輔料過(guò)敏或先前對(duì)任何其他含碘對(duì)比劑過(guò)敏的患者,，甲狀腺功能不全的患者,，老年人和身體狀況很差的患者需進(jìn)行特別謹(jǐn)慎的風(fēng)險(xiǎn)/收益評(píng)估。在先前對(duì)對(duì)比劑有過(guò)敏反應(yīng),，有支氣管哮喘史或其他過(guò)敏史的患者中危險(xiǎn)性更高,。建議給藥后對(duì)病人進(jìn)行觀察，緊急措施的建立對(duì)所有患者都是必要的,。 使用對(duì)比劑后可能發(fā)生腎功能的暫時(shí)性損傷,，在一些病例中可能發(fā)生急性腎功能衰竭。危險(xiǎn)因素包括,，先前存在的腎功能不全，脫水,，糖尿病,，多發(fā)性骨髓瘤/病變蛋白血癥，反復(fù)和/或大劑量使用本品,。血管內(nèi)使用對(duì)比劑前后必須確保所有患者處于充分的水化狀態(tài)【大多數(shù)患者可以根據(jù)需要通過(guò)口服補(bǔ)液來(lái)達(dá)到充分的水化狀態(tài),。關(guān)于哪些患者需要預(yù)防性靜脈水化的決定應(yīng)基于最新的循證臨床指南的建議以及個(gè)體獲益-風(fēng)險(xiǎn)比。這應(yīng)包括考慮使用的劑量（例如高劑量）,、給藥途徑（“首過(guò)”暴露）和腎功能（嚴(yán)重腎功能衰竭的存在）】,。推遲新的對(duì)比劑檢查，直至腎功能恢復(fù)到檢查前的水平,。 對(duì)于患有嚴(yán)重的心臟疾病和重度的冠狀動(dòng)脈疾病的患者,，臨床相關(guān)的血液動(dòng)力學(xué)改變和心律失常的危險(xiǎn)性增加。 血管內(nèi)使用 有中樞神經(jīng)系統(tǒng)（CNS）異常的患者發(fā)生并發(fā)癥的風(fēng)險(xiǎn)可能增加,。嗜鉻細(xì)胞瘤患者有發(fā)生高血壓危象的風(fēng)險(xiǎn),。其他如在自身免疫疾病的患者、重癥肌無(wú)力,、酒精中毒患者中使用風(fēng)險(xiǎn)性增加,。 體腔內(nèi)的使用 進(jìn)行子宮輸卵管造影前，必須除外妊娠的可能性,。膽管或輸卵管炎癥可以增加檢查后發(fā)生不良反應(yīng)的危險(xiǎn)性,。 詳見說(shuō)明書。 【藥物分類】處方藥 【藥品上市許可持有人】Bayer Vital GmbH 【生產(chǎn)企業(yè)】 企業(yè)名稱：Bayer AG 生產(chǎn)地址：Müllerstrasse 178, 13353 Berlin, Germany 【分包裝企業(yè)】 企業(yè)名稱：拜耳醫(yī)藥保健有限公司廣州分公司 生產(chǎn)地址：中國(guó)廣州市經(jīng)濟(jì)技術(shù)開發(fā)區(qū)友誼路103號(hào) 【說(shuō)明書版本】 核準(zhǔn)日期：2006年10月13日 修改日期：2022 年 09 月 22 日 關(guān)于本產(chǎn)品完整的處方信息請(qǐng)參閱產(chǎn)品說(shuō)明書,。 參考文獻(xiàn) 1. Makimoto S, Hatano K, Kataoka N, et al. A case report of primary pancreatic leiomyosarcoma requiring six additional resections for recurrences. Int J Surg Case Rep 2017;41:272–6. 2. J.A. Soreide, E.S. Undersrud, M.S.S. Al-Saiddi, T. Tholfsen, K. Soreide, PrimaryLeiomyosarcoma of the pancreas – a case report and a comprehensive review,J. Gastrointest. Cancer 47 (2016) 358–365. 3. E. Kocakoc, N. Havan, M. Bilgin, M. Atay, Primary pancreatic leiomyosarcoma,Iran. J. Radiol 11 (2014) e4880. 4. Barral M, Faraoun SA, Fishman EK, et al. Imaging features of rare pancreatic tumors. Diagn Interv Imaging 2016;97:1259–73. 5. Zhang H, Yu S, Wang W, Cheng Y, Xiao Y, Lu Z, Chen J: Primary mesenchymal tumors of the pancreas in a single center over 15 years. Oncol Lett. 2016, 12:4027-34. 6. Kim JY, Song JS, Park H, Byun JH, Song KB, Kim KP, Kim SC, Hong SM. Primary mesenchymal tumors of the pancreas: single center experience over 16 years. Pancreas. 2014;43:959–68. 7. Byung Ihn Choi. Radiology Illustrated: Hepatobiliary and Pancreatic Radiology. 2014. 668-701. |
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