第28章 軟組織和骨（Soft Tissue and Bone）Tumors of soft tissue are among the most challenging in surgical pathology. There are several reasons for this: they are rare, so you see few in training; they are overlapping in morphology; they do not always obey the principles that help you to identify malignant potential in carcinomas; and each entity has at least three names, four variants, and seven mimickers. However, we will cover some of the names you will hear most commonly. The tumors are broken down into lines of differentiation, with the caveats that there are some tumors that do not differentiate along any known lineage (grouped separately) and that many soft tissue tumors dedifferentiate into the same final common malignant pathway, the entity formerly known as malignant fibrous histiocytoma (MFH). In many instances, MFH is simply a generic dedifferentiated sarcoma, the high-grade form of any one of a number of precursor lesions. The good news is, once it is that high grade, the origin becomes sort of academic.在外科病理學(xué)中，軟組織腫瘤具有挑戰(zhàn)性。原因：（1）少見，所以你在培訓(xùn)時(shí)看得少；（2）形態(tài)學(xué)重疊；（3）識(shí)別癌的惡性潛能的原則，在此不一定適用；（4）每個(gè)疾病實(shí)體都有多個(gè)同義詞、變異型和類似病變。我們將介紹一些最常見的腫瘤名字，按分化細(xì)胞系進(jìn)行分類。注意：有些腫瘤的分化細(xì)胞系仍然未知（單獨(dú)分組），許多惡性軟組織腫瘤會(huì)發(fā)生去分化，都會(huì)變成最終共同惡性途徑，以前稱為惡性纖維組織細(xì)胞瘤（MFH）。許多情況下，MFH只是去分化肉瘤的一個(gè)總稱，是由多種前體病變進(jìn)展而來(lái)的一種高級(jí)別形式。好消息是，一旦達(dá)到如此高的級(jí)別，它的起源就變得只是學(xué)術(shù)性探討了。When diagnosing a soft tissue lesion, especially in its initial presentation, you must always walk yourself through the mental game of, “what else could this be?” It is a good habit for any organ system but especially in the field of sarcomas and spindle cell lesions. For lesions that do not look clearly malignant (by which we mean they lack nuclear atypia and necrosis), you must always consider that it might be a reactive lesion. For lesions with bizarre and huge nuclei, despite the malignant look, you must consider benign entities with degenerative atypia (such as an ancient schwannoma). For lesions in or near an organ, such as in visceral sites, you must always ask yourself if it could be a carcinoma masquerading as a sarcoma. For spindle cell lesions anywhere, you must ask yourself if it could be melanoma. Some of these questions require immunostains to answer, some just a skeptical eye.診斷軟組織病變時(shí)，尤其是在觀察其最初表現(xiàn)時(shí)，你必須始終進(jìn)行“這還能是什么？”的智力游戲，這種診斷思路對(duì)任何器官系統(tǒng)都是一個(gè)好習(xí)慣，尤其是在肉瘤和梭形細(xì)胞病變領(lǐng)域?？雌饋?lái)不是明顯惡性的病變（意思是缺乏核異型性和壞死），你必須始終認(rèn)為它可能是反應(yīng)性損傷。對(duì)于具有奇異和巨大核的病變，盡管惡性外觀，你必須考慮良性實(shí)體與退化非典型性（如退變性神經(jīng)鞘瘤）。對(duì)于器官內(nèi)或器官附近的病變，如內(nèi)臟部位，你必須始終捫心自問(wèn)是否可能是偽裝成肉瘤的癌。對(duì)于任何地方的梭形細(xì)胞病變，你必須問(wèn)問(wèn)自己是否可能是黑色素瘤。有些問(wèn)題需要免疫染色來(lái)回答，有些問(wèn)題只是懷疑的眼光。（譯注：梭形細(xì)胞病變：簡(jiǎn)化的診斷思路（假的：①無(wú)異型，無(wú)壞死--良性，反應(yīng)性？②巨核，怪異核—退變，老化？）（假的：①臟器周圍—癌？②任何部位--黑色素瘤？）（免疫組化；即使做了免疫組化也可能沒(méi)有答案））The second question to ask, once you have ordered the cytokeratins and melanoma markers, is “what family of soft tissue does it belong in?” Table 28.1 lists some stereotypical features of different tumor families, seen best in low-grade (well-differentiated) lesions.一旦你做細(xì)胞角蛋白和黑色素瘤標(biāo)記物的免疫組化，就產(chǎn)生第二個(gè)問(wèn)題：“它屬于哪個(gè)軟組織家族？”表28.1列出了不同腫瘤家族的一些固有特征，在低級(jí)別（高分化）病變中最容易看到。Table 28.1. Characteristics of tumor families.高級(jí)別肉瘤（High-Grade Sarcomas）As mentioned, once sarcomas turn high grade, they may take on any number of appearances, regardless of differentiation. Some classic visual patterns are shown in Figure 28.1 and described in Table 28.2.如前所述，一旦肉瘤變?yōu)楦呒?jí)別，無(wú)論分化程度如何，都可能出現(xiàn)任意數(shù)量上的表現(xiàn)。一些經(jīng)典的視覺(jué)模式如圖28.1所示，如表28.2所述。Figure 28.1. High grade sarcomas. (A) Fibrosarcoma, with densely packed hyperchromatic s pindle cells. (B) Pleomorphic MFH, with very large and bizarre cells (arrowhead). (C) Myxoid MFH or myxofibrosarcoma, showing pleomorphic cells in a myxoid background. (D) Leiomyosarcoma, with perpendicular fascicles.
圖28.1 高級(jí)別肉瘤。（A）纖維肉瘤，有密集的深染的梭形細(xì)胞。（B）多形性MFH，有非常大的奇異的細(xì)胞（箭頭）。（C）黏液樣MFH或黏液纖維肉瘤，黏液樣背景中的多形性細(xì)胞。（D）平滑肌肉瘤，交錯(cuò)成角的束狀。Table 28.2. Features of high-grade sarcoma patterns.In high-grade sarcomas, the subtype may be clarified through immunostains, history, or the low-grade remnants of the tumor found at the periphery. Identifying the line of differentiation can be helpful in determining prognosis. Solving this puzzle is one of the more interesting games pathologists can play and breaks up the monotony of routine biopsies. However, from a clinical perspective, the oncologist is more concerned about the grade than the type, and, fortunately for all of us, high-grade sarcomas are hard to miss.高級(jí)別肉瘤的亞型，可通過(guò)免疫染色、病史或在周圍發(fā)現(xiàn)的低級(jí)別腫瘤殘留來(lái)闡明。識(shí)別分化細(xì)胞系有助于判斷預(yù)后。病理醫(yī)生有興趣解決這個(gè)難題，活躍了常規(guī)活檢的氛圍。然而，從臨床角度來(lái)看，腫瘤科醫(yī)生更關(guān)心級(jí)別而不是類型，幸運(yùn)的是，對(duì)我們所有人來(lái)說(shuō)，高級(jí)別肉瘤很難漏診。A reliable clue to a high-grade sarcoma is the presence of malignant nuclei. A sarcoma nucleus has some reproducible features across many tumor types. The nucleus has an irregularly shaped border and has dark, dense, granular chromatin that is fairly evenly distributed throughout the nucleus (Figure 28.2). Unlike carcinoma nuclei, prominent nucleoli and nuclear membranes are not a usual feature. Learning to recognize this sort of atypia is critical in identifying some of the subtle sarcomas.高級(jí)別肉瘤的可靠線索之一是惡性細(xì)胞核。肉瘤的細(xì)胞核在許多腫瘤類型中具有一些可重復(fù)的特征。核外形不規(guī)則，有深染、致密、顆粒狀染色質(zhì)，染色質(zhì)在整個(gè)核內(nèi)均勻分布（圖28.2）。與癌細(xì)胞核不同，明顯的核仁和核膜不是肉瘤的常見特征。學(xué)會(huì)識(shí)別這種異型性對(duì)于鑒別某些細(xì)微的肉瘤至關(guān)重要。Figure 28.2. The sarcoma cell vs. the reactive cell. (A) Malignant cells in a MFH or other high-grade sarcoma show large nuclei with irregular shapes and very dark chromatin with a coarse texture (arrowheads). It is as though (in fact, it is likely) the nucleus has way too many chromosomes, and the nucleus is swollen and dark with the extra chromatin (truly hyperchromatic). Nucleoli are not usually prominent. (B) Reactive fibroblasts in nodular fasciitis have large nuclei and prominent nucleoli that stand out against a pale nucleus (arrow). The nuclear membrane is smooth and oval.圖28.2 肉瘤細(xì)胞與反應(yīng)性細(xì)胞對(duì)比。（A）MFH或其他高級(jí)別肉瘤中的惡性細(xì)胞，大核，形狀不規(guī)則，染色質(zhì)非常深染，質(zhì)地粗糙（箭頭）。這就好像（事實(shí)上，很可能）細(xì)胞核有太多的染色體，而細(xì)胞核因額外的染色質(zhì)（真正的深染）而腫脹和變暗。核仁通常不明顯。（B）結(jié)節(jié)性筋膜炎中的反應(yīng)性纖維母細(xì)胞，有大核和明顯核仁，與淡染核形成明顯反差（箭頭）。核膜光滑，呈橢圓形。Throughout this chapter, you will find tables listing some of the more common entities, grouped by clinical behavior. Table 28.3 lists some of the common tumors of fat.本章會(huì)列表顯示一些較常見的疾病實(shí)體，按臨床行為分組。表28.3列舉一些常見的脂肪腫瘤。Table 28.3. Common neoplasms of fat.The most common soft tissue tumor is the lipoma. A lipoma is defined as a neoplasm of mature fat. It is histologically indistinguishable from ordinary fat; to tell the difference you must know it appeared clinically as a discrete lobular mass. There are many histologic variants of lipoma, classified based on what additional soft tissue component is present, such as the angiolipoma, fibrolipoma, angiomyolipoma, and so forth. The hibernoma is a lipoma of brown fat, in which the fat cells are full of innumerable tiny vacuoles. The lipoblastoma, despite the alarming name, is a benign pediatric tumor of mature fat and benign lipoblasts.最常見的軟組織腫瘤是脂肪瘤。脂肪瘤是指成熟脂肪的腫瘤。組織學(xué)上，它與普通脂肪無(wú)法區(qū)分；真要區(qū)分，必須知道它在臨床上表現(xiàn)為一個(gè)離散的分葉狀腫塊。脂肪瘤有許多組織學(xué)變異型，根據(jù)存在的其他軟組織成分分類，如血管脂肪瘤、纖維脂肪瘤、血管平滑肌脂肪瘤等。冬眠瘤是棕色脂肪的脂肪瘤，其中脂肪細(xì)胞充滿無(wú)數(shù)微小的空泡。脂肪母細(xì)胞瘤由成熟脂肪和良性脂肪母細(xì)胞組成，為兒科良性腫瘤，盡管其名稱令人震驚。Figure 28.3. Lipoblast. Small fat vacuoles indent the nucleus of this lipoblast (arrow), seen in a welldifferentiated liposarcoma. Other cells within the fibrous septa (arrowhead) have the look of sarcoma cells, with irregular, large, dark nuclei.圖28.3 脂肪母細(xì)胞。脂肪母細(xì)胞有小的脂肪空泡，擠壓細(xì)胞核（箭），見于高分化脂肪肉瘤。纖維間隔內(nèi)的其他細(xì)胞（箭頭）呈現(xiàn)肉瘤細(xì)胞的外觀，具有不規(guī)則的深染的大核。There is a lot of fuss about lipoblasts. They are immature fat cells in which the nucleus is star shaped, due to being indented on multiple sides by small bubbles of fat (Figure 28.3). They are often associated with liposarcomas, but they can also appear in the benign lipoblastoma, and they are not necessary for a diagnosis of liposarcoma (more on this later). Note that normal adipocytes are not mitotically active cells, so prominent mitoses are generally seen only in high-grade liposarcomas.關(guān)于脂肪母細(xì)胞，可能過(guò)分關(guān)注了。它是未成熟的脂肪細(xì)胞，核呈星形，因?yàn)樵诙鄠€(gè)側(cè)面被小的脂肪空泡擠壓變形（圖28.3）。它們通常與脂肪肉瘤相關(guān)，但也可出現(xiàn)在良性脂肪母細(xì)胞瘤中，并且對(duì)于脂肪肉瘤的診斷不是必需的（稍后詳述）。注意，正常脂肪細(xì)胞不會(huì)核分裂活躍，因此明顯的核分裂一般僅見于高級(jí)別脂肪肉瘤。Two types of lipoma are notable for their unusual cytologic features. They are both usually found on the back or neck of elderly men and are noticeably fibrous and nonfatty on low power. These are the pleomorphic lipoma and spindle cell lipoma. The spindle cell lipoma has areas of nondescript spindle cells and collagen and may remind you of a nerve sheath tumor if there is not much fat in the lesion. The pleomorphic lipoma is similar, with the addition of large giant cells and floret cells (wreath-shaped nuclei). These giant cells (Figure 28.4) are an example of a benign lesion simulating malignant atypia; clinical information is helpful in not mistaking these for liposarcomas.兩種脂肪瘤具有不尋常的細(xì)胞學(xué)特征，即，多形性脂肪瘤和梭形細(xì)胞脂肪瘤。它們通常出現(xiàn)在老年男性的背部或頸部，在低倍鏡下明顯為纖維狀和非脂肪狀。梭形細(xì)胞脂肪瘤有一些難以描述的梭形細(xì)胞和膠原區(qū)域，如果病變中沒(méi)有太多脂肪，你可能會(huì)想到神經(jīng)鞘腫瘤。多形性脂肪瘤與之相似，還有大的巨細(xì)胞和小花細(xì)胞（環(huán)狀核）。這些巨細(xì)胞（圖28.4）是典型的假惡性（良性假冒惡性）案例；臨床信息有助于避免誤認(rèn)為脂肪肉瘤。Figure 28.4. Pleomorphic lipoma. This type of benign lipoma is known for having very bizarre stromal cells that mimic sarcoma. The classic cell is the floret cell, with a circular wreath of nuclear lobes (arrows). Their presence suggests the diagnosis of pleomorphic lipoma.圖28.4 多形性脂肪瘤。這種良性脂肪瘤很著名，因?yàn)榫哂蟹浅Ｆ娈惖拿菜迫饬龅拈g質(zhì)細(xì)胞。典型的細(xì)胞是小花樣多核巨細(xì)胞，多個(gè)核葉圍成圓環(huán)的花環(huán)（箭）。其存在提示多形性脂肪瘤的診斷。The well-differentiated liposarcoma (WDLS) is a tumor of adults. It looks similar to a lipoma on low power except for an increase in fibrous “interstitium” between fat cells and/or fibrous bands (Figure 28.5). A close examination of the fibrous areas reveals hyperchromatic, irregularly shaped nuclei; these are usually large and dark enough to be visible at 4×. Finding a lipoblast is a bonus. A softer feature is an assortment of differently sized fat cells, unlike the monomorphic benign lipoma. The WDLS is so named when it occurs in a nonresectable location, such as the retroperitoneum. By definition, when it occurs on an extremity, it is called an atypical lipoma, as the prognosis in these sites is excellent.高分化脂肪肉瘤（WDLS）是一種成人腫瘤。低倍鏡下，除了脂肪細(xì)胞的纖維“間質(zhì)”增多和/或纖維帶增多之外，它看似脂肪瘤（圖28.5）。仔細(xì)檢查纖維區(qū)，發(fā)現(xiàn)核深染、形狀不規(guī)則；它們通常大而深染，足以在4倍物鏡看到。找到脂肪母細(xì)胞是一個(gè)額外的收獲。較軟的特征是各種各樣的不同大小的脂肪細(xì)胞，不同于形態(tài)單一的良性脂肪瘤。發(fā)生在不可切除的部位時(shí)稱為WDLS，如腹膜后。根據(jù)定義，發(fā)生在四肢時(shí)，稱為非典型性脂肪瘤，因?yàn)檫@些部位的預(yù)后非常好。Figure 28.5. Well-differentiated liposarcoma. There is an increased amount of fibrous interstitium between fat cells, and atypical cells stand out at low power (arrowheads).圖28.5 高分化脂肪肉瘤。脂肪細(xì)胞之間的纖維間質(zhì)增多，非典型細(xì)胞在低倍鏡下明顯可見（箭頭）。When the WDLS has been around for a while, especially in a recurrent or occult retroperitoneal lesion, there is a risk of the tumor transforming into a high-grade pleomorphic sarcoma. When this happens, you will see a tumor with well-differentiated lipomatous areas and an abrupt transition to a high-grade tumor (storiform/spindled, MFH-like, or even rhabdo- or leiomyosarcomatous). Regardless of morphology, this is called a dedifferentiated liposarcoma, and the key to diagnosis is recognizing the adjacent WDLS. Because dedifferentiated liposarcoma is the most likely diagnosis in a retroperitoneal pleomorphic sarcoma, if you are grossing such a tumor, be sure to sample anything near the tumor that looks like fat: it may be the well-differentiated component.WDLS出現(xiàn)一段時(shí)間后，尤其是在復(fù)發(fā)性或隱匿性腹膜后病變中，腫瘤有轉(zhuǎn)化為高級(jí)別多形性肉瘤的風(fēng)險(xiǎn)。這種情況發(fā)生時(shí)，你會(huì)看到腫瘤有一個(gè)高分化的脂肪瘤樣區(qū)域，突然轉(zhuǎn)變?yōu)楦呒?jí)別腫瘤（席紋狀/梭形、MFH樣，甚至橫紋肌肉瘤樣或平滑肌肉瘤樣）。不管形態(tài)學(xué)如何，都稱為去分化脂肪肉瘤，診斷的關(guān)鍵是識(shí)別相鄰的WDLS。因?yàn)槿シ只救饬鍪歉鼓ず蠖嘈涡匀饬鲋凶钣锌赡艿脑\斷，如果你正在大體檢查，在腫瘤附近看似脂肪的東西一定要取材：它可能是高分化成分。Myxoid liposarcoma is a different type of low-grade liposarcoma. It is not as clearly fatty as the WDLS, and the low-power impression is that of a gelatinous tumor with scattered fat cells and a stereotypical capillary network that has been compared to chicken-wire (Figure 28.6). These vessels are very delicate, and, unlike normal capillaries, they have little substance to their walls; they appear as a naked sleeve of endothelium stretched through the tumor. The tumor cells themselves are small spindled or rounded cells and lipoblasts, without the large atypical cells of the WDLS.黏液樣脂肪肉瘤是另一種的低級(jí)別脂肪肉瘤。它不像WDLS那樣有明顯脂肪，低倍鏡下的印象是黏液樣腫瘤，有散在的脂肪細(xì)胞和特征性毛細(xì)血管網(wǎng)，后者像雞籠（圖28.6）。這些血管非常纖細(xì)，與正常的毛細(xì)血管不同，它們的血管壁幾乎沒(méi)有物質(zhì)；就像裸露的內(nèi)皮細(xì)胞袖套，穿過(guò)腫瘤。腫瘤細(xì)胞本身是小的梭形或圓形細(xì)胞和脂肪母細(xì)胞，沒(méi)有WDLS那樣的非典型大細(xì)胞。Figure 28.6. Myxoid liposarcoma. The fatty component may be very subtle in myxoid liposarcoma; the vessels are more often the tip off. The vasculature is composed of a delicate network of very thin capillaries with three- and four-way branch points, similar to chicken-wire (arrow). The cell population is composed of small cells, which may have fat vacuoles in them, and a myxoid background. Large atypical cells should not be present. Inset: Areas of closely packed small cells are indicative of round cell differentiation.圖28.6 黏液樣脂肪肉瘤。脂肪成分可能非常細(xì)微；血管通常是診斷線索。血管由非常細(xì)的毛細(xì)血管組成，并形成精致的網(wǎng)。毛細(xì)血管具有三向和四向分支點(diǎn)，就像雞籠（箭）。細(xì)胞群由小細(xì)胞組成，可能有脂肪空泡，位于黏液樣背景中。不應(yīng)出現(xiàn)非典型大細(xì)胞。插圖：緊密排列的小細(xì)胞區(qū)域提示圓形細(xì)胞分化。The myxoid liposarcoma can also transform into a higher grade lesion. When the small uniform cells become very densely packed and obscure the vascular pattern, it is indicative of round cell differentiation. The presence of more than 5% round cell differentiation is worth noting; a tumor with more than 75% is called a round cell liposarcoma.黏液樣脂肪肉瘤也可轉(zhuǎn)化為更高級(jí)別病變。當(dāng)小而均勻的細(xì)胞變得非常密集，使血管模式變得模糊時(shí)，表明細(xì)胞分化為圓形。圓形細(xì)胞分化超過(guò)5%沒(méi)有意義；超過(guò)75%的腫瘤稱為圓形細(xì)胞脂肪肉瘤。The rare pleomorphic liposarcoma describes a high-grade tumor with extremely bizarre pleomorphic lipoblasts. It differs from the dedifferentiated liposarcoma in that it is still recognizable as a lipomatous tumor. It does not arise from, or have any relation to, the pleomorphic lipoma.多形性脂肪肉瘤是一種罕見的高級(jí)別腫瘤，具有極其奇異的多形性脂肪母細(xì)胞。它不同于去分化脂肪肉瘤，它仍然可以識(shí)別為脂肪性腫瘤。它不是由多形性脂肪瘤引起的，也與多形性脂肪瘤無(wú)關(guān)。纖維腫瘤和黏液樣腫瘤（Fibrous Tumors and Myxoid Tumors）The fibroblast and the myofibroblast are ubiquitous cells, in charge of the reparative changes that take place in every part of the body. In resting state, they are fusiform to stellate cells with oblong pale nuclei, and they lay down a collagen matrix. Their job is to proliferate, and therefore mitotic activity is not unusual in reactive lesions. Although myofibroblasts may stain for actin (and are occasionally mistaken for smooth muscle), in general immunostains are not helpful in this tumor family.纖維母細(xì)胞和肌纖維母細(xì)胞是普遍存在的細(xì)胞，負(fù)責(zé)身體各個(gè)部位發(fā)生的修復(fù)性變化。在靜息狀態(tài)下，它們呈梭形至星芒狀，核呈淡染的長(zhǎng)方形。它們產(chǎn)生膠原基質(zhì)。它們的工作是增殖，因此核分裂活性在反應(yīng)性病變中并不罕見。雖然肌纖維母細(xì)胞可以染actin（偶爾被誤認(rèn)為平滑肌），但一般來(lái)說(shuō)，免疫染色對(duì)該腫瘤家族沒(méi)有幫助。反應(yīng)性病變（Reactive lesions）Before we discuss the true neoplasms, we will review the collection of reactive (inflammatory or posttraumatic) lesions that present as tumors (lumps). Keloid is a common fibrous lesion, occurring at a site of trauma. It is similar to the normal fibroblastic proliferation of a dermal scar, except for its large size and characteristic thick cords of collagen called keloidal collagen. It is clinically recognizable and not usually a diagnostic dilemma.在討論真正的腫瘤之前，我們先回顧一組表現(xiàn)為腫瘤（腫塊）形式的反應(yīng)性（炎癥或創(chuàng)傷后）病變。瘢痕疙瘩是一種常見的纖維病變，發(fā)生在創(chuàng)傷部位。它類似于真皮瘢痕的正常纖維母細(xì)胞增殖，除了它的尺寸大和特征性厚膠原索（稱為瘢痕疙瘩膠原）。它在臨床上可以識(shí)別，通常不是診斷難題。Nodular fasciitis, on the other hand, may simulate a neoplasm clinically and is therefore more challenging for the pathologist. It is classically a rapidly growing lesion, sometimes associated with known trauma, sometimes not. On low power, it is a fairly circumscribed lesion with a hypercellular periphery, and it has a heterogeneous, as opposed to monomorphic, look. A microcystic appearance is classic. On high power the fibroblasts show a “tissue culture” appearance (fusiform to stellate with distinct cytoplasmic processes), and they float in a myxoid background with surrounding red blood cells and lymphocytes (Figure 28.7). Older lesions may become more dense, collagenized, and pink and may resemble a fibromatosis (see later) with chronic inflammation. There should be no nuclear atypia, but you will see mitotic activity.另一方面，結(jié)節(jié)性筋膜炎在臨床上可能假冒腫瘤，因此對(duì)病理醫(yī)生來(lái)說(shuō)更具挑戰(zhàn)性。它是典型的快速增長(zhǎng)的病變，有時(shí)與已知的創(chuàng)傷有關(guān)，有時(shí)不相關(guān)。在低倍鏡下，它具有相當(dāng)清楚邊界，外周細(xì)胞豐富，并且它具有異質(zhì)性，而不是單一形態(tài)的外觀。微囊表現(xiàn)是典型的。在高倍鏡下，纖維母細(xì)胞呈現(xiàn)“組織培養(yǎng)”外觀（梭形到星芒狀，具有清楚的細(xì)胞質(zhì)突起），它們漂浮在黏液樣背景中，周圍有紅細(xì)胞和淋巴細(xì)胞（圖28.7）。較老的病變可能變得更致密、膠原化和粉紅色，可能類似于伴有慢性炎癥的纖維瘤病（見下文）。應(yīng)該沒(méi)有核異型性，但你會(huì)看到核分裂活性。The biggest pitfall in nodular fasciitis is misinterpreting the patchy high cellularity and mitotic activity for a sarcoma. The clinical history is helpful, as is recognizing the reactive versus malignant nuclear features (something that takes practice).結(jié)節(jié)性筋膜炎的最大診斷陷阱，就是把肉瘤的斑片狀高度富于細(xì)胞的和核分裂活躍的區(qū)域誤認(rèn)為肉瘤。臨床病史很有幫助，認(rèn)識(shí)反應(yīng)性與惡性核特征的區(qū)別也有助于診斷（需要實(shí)踐經(jīng)驗(yàn)）。Figure 28.7. Nodular fasciitis. In this field the inflammatory component is not prominent, but the “tissue culture” pattern is seen clearly, with fusiform and stellate fibroblasts stretching delicate processes through the myxoid background (arrow).圖28.7 結(jié)節(jié)性筋膜炎。在這個(gè)視野，炎癥成分不明顯，但“組織培養(yǎng)”模式清晰可見，梭形和星芒狀纖維母細(xì)胞在黏液樣背景中伸展出精細(xì)的突起（箭）。Proliferative fasciitis is similar to nodular fasciitis except with the addition of large pink ganglionlike cells. Proliferative myositis is essentially the same lesion but in an intramuscular location.增生性筋膜炎與結(jié)節(jié)性筋膜炎相似，只是還有大的粉紅色神經(jīng)節(jié)樣細(xì)胞。增生性肌炎本質(zhì)上是相同的病變，但位于肌肉內(nèi)。Myositis ossificans is a variant of proliferative myositis that shows reactive bone formation.骨化性肌炎是增生性肌炎的一種變異型，表現(xiàn)為反應(yīng)性骨形成。Inflammatory myofibroblastic tumor has gone by many names (inflammatory pseudotumor, inflammatory fibrosarcoma, plasma cell granuloma, others), but in this chapter it will be shortened to IMT. While long considered a reactive lesion, occasional cases have spread aggressively and even metastasized. Therefore, it is beginning to be regarded as a neoplasm, and will be included below, despite its histologic similarity to nodular fasciitis.炎性肌纖維母細(xì)胞瘤有許多名稱（炎性假瘤、炎性纖維肉瘤、漿細(xì)胞肉芽腫等），但在本章中簡(jiǎn)稱為IMT。雖然長(zhǎng)期以來(lái)認(rèn)為它是反應(yīng)性病變，但偶爾也會(huì)發(fā)生侵襲性擴(kuò)散甚至轉(zhuǎn)移。因此，盡管其組織學(xué)與結(jié)節(jié)性筋膜炎相似，但它已開始被視為腫瘤，并將在下文中予以介紹。The prototypical benign fibroblastic lesion is the fibromatosis (Table 28.4). This is a bland and indistinct tumor composed of normal-looking fibroblasts: fascicles of pink cells with pale tapering nuclei in a collagenous background (Figure 28.8). The very pale nuclei make the capillaries stand out and appear dark in comparison. It is very infiltrative around the edges, much like a normal scar. Superficial fibromatoses can occur on the palm (palmar fibromatosis, Dupuytren’s contracture), sole (plantar, Ledderhose disease), or penis (Peyronie’s disease), where they are benign but can recur. Axial or deep fibromatoses, such as on the chest wall or mesentery, are typically more aggressive in their expansion and are called desmoid tumors. The desmoid tumors are characterized by a specific immunohistochemical trait, the accumulation of β-catenin in nuclei.良性纖維母細(xì)胞病變的原型是纖維瘤?。ū?8.4）。這是一種形態(tài)學(xué)溫和、模糊不清的腫瘤，由看起來(lái)正常的纖維母細(xì)胞組成：粉紅色細(xì)胞束，淡染的錐形核，位于膠原背景中（圖28.8）。核非常淡染，使得毛細(xì)血管明顯和深染。邊緣呈明顯的侵襲性，很像正常的疤痕。淺表性纖維瘤病可發(fā)生在手掌（掌纖維瘤病、Dupuytren攣縮）、腳底（跖纖維瘤病、Ledderhose病）或陰莖（Peyronie?。┑炔课唬@些都是良性但可能復(fù)發(fā)（譯注：2020年WHO分類中ICD-O編碼8813/1）。軀干或深部纖維瘤，如胸壁或腸系膜上的纖維瘤，通常在擴(kuò)散時(shí)更具侵襲性，稱為硬纖維瘤。硬纖維瘤具有特殊的免疫組化表達(dá)，即β-catenin在細(xì)胞核中的積累。Table 28.4. Fibrous and myxoid neoplasms.Low-grade fibromyxoid sarcoma is one of those most troublesome entities; it simulates a benign lesion (fibromatosis) yet has metastatic potential. It may appear hypocellular, myxoid, or vaguely storiform, much like a fibromatosis.低級(jí)別纖維黏液樣肉瘤是最麻煩的實(shí)體之一；它貌似良性病變（纖維瘤?。?，但具有轉(zhuǎn)移潛能。它可能表現(xiàn)為細(xì)胞少、黏液樣或隱約的席紋狀，很像纖維瘤病。Figure 28.8. Fibromatosis. The cells in this lesion are pale and indistinct, with the small wavy nuclei (arrow) noticeably hypochromatic relative to the endothelial cells of the nearby capillary (a good internal control; asterisk). There is abundant collagen in the stroma (arrowhead).
圖28.8 纖維瘤病。病變中的細(xì)胞淡染、不清楚，與附近毛細(xì)血管（良好的內(nèi)部控制；星號(hào)）的內(nèi)皮細(xì)胞相比，小的波紋狀核（箭）明顯淡染?；|(zhì)中有豐富的膠原（箭頭）。Fibrosarcoma is the high-grade endpoint of this spectrum of lesions. It is the classic pure “herringbone” lesion, with fascicles alternating in a zigzag pattern. There is no significant collagen or inflammation to speak of. It has a high mitotic rate, but the cells are not particularly atypical: the nuclei tend to be monomorphic, oval, and euchromatic (Figure 28.9). It is mainly the cellular density and mitotic activity that set this lesion apart as malignant.纖維肉瘤是這個(gè)病變譜系中的高級(jí)別終點(diǎn)。這是典型的純“鯡魚骨”病變，細(xì)胞束以之字形交替排列。沒(méi)有明顯的膠原或炎癥。核分裂率高，但細(xì)胞異型性并不特別明顯：核往往形態(tài)單一、橢圓形和正常染色（圖28.9）。主要根據(jù)細(xì)胞密集和核分裂活性判定為惡性。Figure 28.9. Fibrosarcoma. This field shows the typical herringbone pattern of fibrosarcoma, with zigzagging bands of spindle cells. Many other tumors can have this pattern.圖28.9 纖維肉瘤。這個(gè)視野顯示纖維肉瘤典型的鯡魚骨模式，梭形細(xì)胞呈之字形帶狀排列。許多其他腫瘤也有這種模式。However, what looks like fibrosarcoma is not usually fibrosarcoma. True fibrosarcoma is quite rare, while its imitators, especially malignant peripheral nerve sheath tumor and synovial sarcoma, are more common. Therefore, fibrosarcoma is a diagnosis of exclusion.然而，看似纖維肉瘤的，通常不是纖維肉瘤。真正的纖維肉瘤非常罕見，其假冒病變更為常見，尤其是惡性周圍神經(jīng)鞘瘤和滑膜肉瘤。因此，纖維肉瘤是一種診斷排除。The solitary fibrous tumor is included here because of its resemblance to fibroblastic tumors, but in truth the type of differentiation is not known. The solitary fibrous tumor has a unique staining pattern (CD34, CD99, and bcl-2) and typically arises from serosal surfaces. Because of its association with the pleura, it was once called the “benign mesothelioma.” On low power, the tumor is described as having a patternless pattern, which evidently means nonstoriform-nonherringbone-nonfascicular. The swirling mass of uniform cells is reminiscent of ovarian stroma, but appears more pink due to abundant collagen (Figure 28.10). Collagen is laid down in parallel bundles, and the cellularity varies from one field to the next. The vessels are of the “staghorn” type, meaning they are gaping, branching vessels without an appreciable wall thickness: the tumor appears to extend right up to the endothelium. A mitotic rate of more than 4 per 10 high-power fields (hpf) suggests a malignant solitary fibrous tumor.由于與纖維母細(xì)胞瘤相似，在此討論孤立性纖維性腫瘤（SFT），但實(shí)際上分化類型尚不清楚。SFT具有獨(dú)特的染色模式（CD34、CD99和bcl-2），通常發(fā)生在漿膜表面。由于它與胸膜有關(guān)，曾被稱為“良性間皮瘤”。在低倍鏡下，腫瘤被描述為“無(wú)模式的模式'，這顯然意味著非席紋狀非鯡魚骨非束狀結(jié)構(gòu)。形態(tài)均勻的細(xì)胞形成漩渦狀細(xì)胞團(tuán)，就像卵巢間質(zhì)，但由于富含膠原而顯得更紅（圖28.10）。膠原以平行束狀排列，各個(gè)視野的細(xì)胞密度不相同。血管為“鹿角”型，即，開放的分支血管，沒(méi)有明顯的厚壁血管：腫瘤似乎一直延伸到內(nèi)皮細(xì)胞。核分裂率超過(guò)4/10HPF提示為惡性SFT。（譯注：2020年WHO分類中，SFT歸入纖維母細(xì)胞腫瘤，臨床行為有良性、中間性和惡性，編碼分別為8815/0、8815/1和8815/3）Figure 28.10. Solitary fibrous tumor. The most noticeable features at low power are the staghorn vessels (v), which this tumor shares with the related hemangiopericytoma. The tumor is composed of areas of small nondescript spindle cells (arrowhead) and collagenous stroma (arrow). The pattern of the spindle cells is described as “patternless,” meaning somewhat chaotic.圖28.10 孤立性纖維性腫瘤。低倍鏡下最顯著的特征是鹿角狀血管（v），這與相關(guān)的血管外皮細(xì)胞瘤相同。腫瘤由無(wú)法描述的小梭形細(xì)胞區(qū)域（箭頭）和膠原基質(zhì)區(qū)域（箭）組成。梭形細(xì)胞的模式被描述為“無(wú)模式”，這意味著有些混亂。皮膚的纖維性腫瘤(Fibrous Tumors of the Skin)The benign fibrous/fibrohistiocytic tumor of the dermis is the dermatofibroma or benign fibrous histiocytoma. It appears clinically as a little nodule, and at low power you see a diffuse, hazy, indistinct “blueness” occupying and expanding the dermis. On higher power, the dermatofibroma shows a textbook storiform pattern, with spindly cells arranged in little radial sunbursts, like spokes on a wheel (see Figure 27.23 in Chapter 27). There is also usually accompanying inflammation, including lymphocytes, plasma cells, and foamy macrophages.真皮的良性纖維/纖維組織細(xì)胞腫瘤是皮膚纖維瘤或良性纖維組織細(xì)胞瘤。臨床上表現(xiàn)為小結(jié)節(jié)，低倍鏡下可見彌漫、云霧狀、模糊的“藍(lán)色”占據(jù)并擴(kuò)張真皮。在高倍鏡下，皮膚纖維瘤呈教科書式的席紋狀模式，梭形細(xì)胞排列成小的放射狀，就像車輪上的輻條（參見第27章圖27.23）。通常伴有炎癥，包括淋巴細(xì)胞、漿細(xì)胞和泡沫狀巨噬細(xì)胞。The malignant, albeit indolent, counterpart is the dermatofibrosarcoma protuberans (DFSP). This lesion is also distinctly storiform, and the tumor cells, as in the dermatofibroma, are monomorphic, fusiform, and just slightly hyperchromatic. However, the DFSP penetrates more deeply and classically infiltrates the subcutaneous fat, wrapping around fat cells in a distinctive pattern (see Chapter 27). In contrast to the dermatofibroma, the DFSP is ironically more uniform in cytology and lacks the associated inflammatory cells.惡性但惰性的對(duì)應(yīng)物是隆突性皮膚纖維肉瘤（DFSP）。這種病變也是明顯的席紋狀，腫瘤細(xì)胞正如皮膚纖維瘤，是單形的梭形細(xì)胞，只是稍微深染。然而，DFSP浸潤(rùn)更深，通常浸潤(rùn)到皮下脂肪，以獨(dú)特的模式包裹脂肪細(xì)胞（見第27章）。與皮膚纖維瘤相比，矛盾的是DFSP在細(xì)胞學(xué)上更為一致，并且缺少相關(guān)的炎性細(xì)胞。The skin also has its own MFH variant, called an atypical fibroxanthoma. Despite being histologically equivalent to a pleomorphic MFH, this superficial tumor is easily resected and therefore has a good prognosis.皮膚也有自己的MFH變異型，稱為非典型纖維黃色瘤。盡管組織學(xué)相當(dāng)于多形性MFH，但這種淺表腫瘤很容易切除，因此預(yù)后良好。The myxoid lesions included here are those that are not myxoid variants of other tumor types (such as myxoid liposarcoma). Many different lesions may converge on the myxoid phenotype, however. What we call myxoid is really the accumulation of hyaluronic acid, a gel-like substance that is essentially a form of solid water in the body (as seen in tissue edema). It may appear clear to a very pale blue on routine stains. A myxoid differential diagnosis would include myxoma, angiomyxoma, neurofibroma, and nodular fasciitis (all benign) and myxoid MFH (myxofibrosarcoma), myxoid liposarcoma, myxoid chondrosarcoma, myxoid leiomyosarcoma, and the low-grade fibromyxo- and myxofibro- entities (all malignant). You would also need to exclude tumors that may appear myxoid but are not, including chordoma, cartilaginous tumors, and epithelial mucinous tumors.這里討論的黏液樣病變不是其他腫瘤類型的黏液樣變異型（如黏液樣脂肪肉瘤）。然而，許多不同的病變可呈黏液樣表型。所謂的黏液樣物質(zhì)實(shí)際上是透明質(zhì)酸的積累，一種凝膠狀物質(zhì)，本質(zhì)上是身體內(nèi)固體水的一種形式（如組織水腫）。常規(guī)染色，它可能呈現(xiàn)非常淡的藍(lán)色。黏液樣腫瘤的鑒別診斷包括黏液瘤、血管黏液瘤、神經(jīng)纖維瘤、結(jié)節(jié)性筋膜炎（均為良性）和黏液樣MFH（黏液纖維肉瘤）、黏液樣脂肪肉瘤、黏液樣軟骨肉瘤、黏液樣平滑肌肉瘤，以及低級(jí)別黏液纖維肉瘤和黏液纖維肉瘤。你還需要排除可能呈黏液樣，但并非黏液樣腫瘤，包括脊索瘤、軟骨性腫瘤和上皮性黏液腫瘤。The intramuscular myxoma is a benign and nearly acellular lesion that presents as a gelatinous mass within a muscle, usually in women. There are rare small stellate cells without atypia. What separates the benign myxoma from other more worrisome lesions is its virtual lack of capillaries.肌內(nèi)黏液瘤是一種良性的幾乎無(wú)細(xì)胞的病變，通常表現(xiàn)為女性患者肌肉內(nèi)的凝膠狀腫塊。罕見的小星芒狀細(xì)胞沒(méi)有異型性。良性黏液瘤與其他更令人擔(dān)憂的病變的區(qū)別在于它實(shí)際上缺乏毛細(xì)血管。Myxofibrosarcoma is a high-grade tumor also known as myxoid MFH, and its low-grade counterpart is the low-grade myxofibrosarcoma (not to be confused with the fibromatosis-like low-grade fibromyxoid sarcoma!). The myxofibrosarcomas are tumors that are prominently myxoid but that have an increasing cellularity, nuclear pleomorphism, and mitotic rate compared to myxoma. Because of prominent vessels and bubbly cells (pseudolipoblasts), they may be mistaken for myxoid liposarcoma. However, the vessels are different. Myxofibrosarcoma vessels are “curvilinear,” which means they make short thick arcs in the tumor, and the tumor cells appear to drip off of them like wax from a candle (Figure 28.11). Compare these to the delicate branching capillaries of the myxoid liposarcoma (see Figure 28.6). The nuclei of myxofibrosarcoma also set them apart; they are hyperchromatic and pleomorphic, unlike the uniform nuclei of the myxoid liposarcoma.黏液纖維肉瘤是一種高級(jí)別腫瘤，也稱為黏液樣MFH，其低級(jí)別對(duì)應(yīng)物是低級(jí)別黏液纖維肉瘤（不要與纖維瘤病樣低級(jí)別纖維黏液樣肉瘤混淆?。ｐひ豪w維肉瘤是一種明顯的黏液樣腫瘤，但與黏液瘤相比，其細(xì)胞數(shù)量、核多形性和核分裂率增加。由于明顯的血管和空泡狀細(xì)胞（假脂母細(xì)胞），它們可能誤認(rèn)為是黏液樣脂肪肉瘤。然而，血管是不同的。黏液纖維肉瘤血管呈“曲線”，這意味著它們?cè)谀[瘤中形成短而粗的弧形，腫瘤細(xì)胞似乎像蠟燭上的蠟一樣從血管中滴下（圖28.11）。對(duì)比黏液樣脂肪肉瘤的精致的分支狀毛細(xì)血管（見圖28.6）。黏液纖維肉瘤的細(xì)胞核呈深染和多形性，也能鑒別黏液樣脂肪肉瘤的均勻細(xì)胞核。Figure 28.11. Myxofibrosarcoma. Although the cells here resemble those of pleomorphic malignant fibrous histiocytoma, the stroma is myxoid, and the vessels are unique (arrow). They appear as short arcs or segments, unlike the complex branching vessels of myxoid liposarcoma, and the tumor cells are intimately associated with the vessels, like wax dripping down the side of a candle.圖28.11 黏液纖維肉瘤。雖然這里的細(xì)胞類似于多形性MFH，但基質(zhì)是黏液樣的，血管是獨(dú)特的（箭）。與黏液樣脂肪肉瘤的復(fù)雜分支血管不同，它們呈短弧形或節(jié)段，腫瘤細(xì)胞與血管密切相關(guān)，就像蠟從蠟燭邊滴下一樣。Inflammatory myofibroblastic tumor (IMT) is a neoplasm of mainly young people, often arising in the abdominal cavity. It is a proliferation of plump fibroblasts with abundant associated inflammation, especially plasma cells. It is very similar in appearance to a nodular fasciitis in that there are tissue culture–like fibroblasts in a myxoid, granulation tissue–like background (Figure 28.12). It differs by its visceral location and prominence of plasma cells (not seen in nodular fasciitis). The hypercellularity may be very worrisome for a high-grade sarcoma. However, while the nuclei may be enlarged, with prominent nuclear membranes or large nucleoli, you should not see the irregularly shaped hyperchromatic nuclei of MFH. Many cases show immunoreactivity for ALK.炎性肌纖維母細(xì)胞瘤（IMT）是一種主要發(fā)生于年輕人的腫瘤，常發(fā)生于腹腔。它是一種豐滿的纖維母細(xì)胞增殖，伴有大量相關(guān)炎癥，尤其是漿細(xì)胞。它看起來(lái)很像結(jié)節(jié)性筋膜炎：組織培養(yǎng)樣纖維母細(xì)胞，位于黏液樣、肉芽組織樣背景中（圖28.12）。其不同之處在于內(nèi)臟位置和漿細(xì)胞的明顯（結(jié)節(jié)性筋膜炎中未見）。細(xì)胞豐富可能令人擔(dān)心高級(jí)別肉瘤來(lái)。然而，盡管核可能增大，有明顯核膜或大核仁，但不應(yīng)看到MFH那種形狀不規(guī)則的深染核。許多病例對(duì)ALK有免疫反應(yīng)。Figure 28.12. Inflammatory myofibroblastic tumor. The tumor is composed of a network of reactivelooking fibroblasts (arrow), capillaries, and inflammation, especially plasma cells (arrowhead).圖28.12 炎性肌纖維母細(xì)胞瘤。腫瘤由看似反應(yīng)性的纖維母細(xì)胞（箭）、毛細(xì)血管和炎癥，尤其是漿細(xì)胞（箭頭）組成。平滑肌腫瘤(Smooth Muscle Tumors )There are no reactive smooth muscle lesions, so we will go straight to neoplasms (Table 28.5). Smooth muscle neoplasms may be positively identified by immunoreactivity to actin and desmin but may sometimes show spurious cytokeratin or EMA staining.沒(méi)有反應(yīng)性平滑肌病變，因此我們將直接討論腫瘤（表28.5）。平滑肌腫瘤可通過(guò)actin和desmin的免疫反應(yīng)陽(yáng)性來(lái)識(shí)別，但有時(shí)可顯示細(xì)胞角蛋白或EMA染色的假陽(yáng)性。Table 28.5. Smooth muscle neoplasms.The leiomyoma should be familiar, as it is identical to the uterine tumor. It can occur as a primary neoplasm in cutaneous, gastrointestinal, and other sites. However, unlike in the uterus, in these body sites there is a very low threshold for bumping the lesion up to leiomyosarcoma. In general, greater than 1 mitosis per 10 hpf is worrisome.軟組織平滑肌瘤應(yīng)該很熟悉，因?yàn)樗c子宮平滑肌瘤相同。它可以發(fā)生于皮膚、胃腸道和其他部位。然而，與子宮不同，這些部位診斷為平滑肌肉瘤的閾值非常低。總的來(lái)說(shuō)，核分裂象超過(guò)1/10HPF是令人擔(dān)憂的。The leiomyoma is characterized by long parallel bundles of smooth muscle cells that intersect at right angles, such that some are seen longitudinally and some cut in cross section. The nuclei are often described as cigar or box-car shaped, with blunt ends. You may also see c orkscrew nuclei, which appear twisted about themselves and are associated with the contracted state. Paranuclear vacuoles are common (Figure 28.13).平滑肌瘤的特征是長(zhǎng)的平行的平滑肌細(xì)胞束，以直角相交，有的縱向可見，有的橫切面可見。細(xì)胞核通常被描述為雪茄或車廂形，末端鈍圓。你也可以看到螺旋核，它看起來(lái)是扭曲的，與收縮狀態(tài)有關(guān)。核旁空泡常見（圖28.13）。Figure 28.13. Leiomyoma of colon. As in the leiomyoma of the uterus, there are smooth muscle cells in bundles running parallel to and perpendicular to (asterisk) the slide. The features of benign smooth muscle include elongated pale nuclei with paranuclear vacuoles (arrowhead) and occasional corkscrew nuclei in which the nuclei appear twisted (arrow). Wavy pink muscle fibers are usually visible between the nuclei.圖28.13 結(jié)腸平滑肌瘤。正如子宮平滑肌瘤，平滑肌細(xì)胞呈束狀平行于或垂直于載玻片（星號(hào)）。良性平滑肌的特征包括帶有核旁空泡的細(xì)長(zhǎng)淡染細(xì)胞核（箭頭），偶見螺旋形細(xì)胞核，核扭曲（箭）。核之間常見波浪狀粉紅色肌纖維。Leiomyosarcomas range in appearance from something very similar to leiomyoma to a densely cellular and hyperchromatic tumor with scattered highly atypical nuclei (Figure 28.14). They can occur in the skin, where they are relatively indolent, or in the retroperitoneum, soft tissues, or any organ with smooth muscle, where they are more aggressive.平滑肌肉瘤的變化范圍很大，從很像平滑肌瘤，到細(xì)胞密集、深染的腫瘤伴散在的高度非典型核（圖28.14）。它們可以發(fā)生在皮膚，相對(duì)惰性；或者發(fā)生在腹膜后、軟組織或任何有平滑肌的器官中，侵襲性較強(qiáng)。Figure 28.14. Leiomyosarcoma. A malignant version of the leiomyoma, this tumor has the architectural pattern and nuclear morphology of its benign cousin but with much higher cellularity, hyperchromatic nuclei, frequent mitoses (arrow), and large atypical cells (arrowhead).圖28.14 平滑肌肉瘤。與平滑肌瘤對(duì)應(yīng)的惡性腫瘤，具有平滑肌瘤的結(jié)構(gòu)模式和核形態(tài)，但細(xì)胞密度更高，核深染，核分裂頻繁（箭）和非典型大細(xì)胞（箭頭）。In a smooth muscle–like lesion arising anywhere near the gastrointestinal tract, you should consider the gastrointestinal stromal tumor (GIST) in the differential diagnosis. Many of what were once called gastric leiomyomas are now identified as GISTS. The cells differentiate along the line of the interstitial cell of Cajal, the pacemaker cell of the stomach, and like this cell, the GIST stains for c-kit and CD34. The GIST may take a spindle cell morphology, overlapping with leiomyoma or schwannoma, or may be epithelioid with a wide range of morphology. Clinical behaviors range from benign to malignant, depending on site and histologic factors.在胃腸道附近的平滑肌樣病變中，應(yīng)考慮胃腸道間質(zhì)瘤（GIST）的鑒別診斷。許多曾經(jīng)被稱為胃平滑肌瘤的腫瘤現(xiàn)在被確認(rèn)為GIST。這些細(xì)胞沿著Cajal間質(zhì)細(xì)胞（胃的起搏細(xì)胞）的細(xì)胞線分化，與此細(xì)胞一樣，GIST免疫染色顯示c-kit和CD34。GIST可能呈梭形細(xì)胞形態(tài)，與平滑肌瘤或神經(jīng)鞘瘤重疊，也可能為上皮樣細(xì)胞，形態(tài)多樣。根據(jù)部位和組織學(xué)因素，臨床表現(xiàn)從良性到惡性不等。
The Practice of Surgical Pathology：A Beginner’s Guide to the Diagnostic Process外科病理學(xué)實(shí)踐：診斷過(guò)程的初學(xué)者指南Diana Weedman Molavi, MD, PhDSinai Hospital, Baltimore, MarylandISBN: 978-0-387-74485-8 e-ISBN: 978-0-387-74486-5Library of Congress Control Number: 2007932936? 2008 Springer Science Business Media, LLC僅供學(xué)習(xí)交流，不得用于其他任何途徑。如有侵權(quán)，請(qǐng)聯(lián)系刪除
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