黏液炎性纤维母细胞肉瘤与结节性腱鞘炎的相关意义

目的 探讨黏液炎性纤维母细胞肉瘤与结节性腱鞘炎的相关意义.方法 对1例复发于结节性腱鞘炎的黏液炎性纤维母细胞肉瘤进行光镜和免疫组织化学检查.结果 证实黏液炎性纤维母细胞肉瘤具有特征性异型细胞,包括假脂肪母细胞、印戒样细胞及相似于病毒细胞、神经节细胞和R-S细胞.组织结构由明显黏液样区、纤维透明变性和炎症性病变混杂组成.肿瘤细胞表达vimentin弥漫阳性,CD34和CD68部分阳性,大多数淋巴细胞表达CD3和CD45RO阳性.结节性腱鞘炎不出现特征性异型细胞和明显黏液样区.结论黏液炎性纤维母细胞肉瘤是一种低度恶性肿瘤,好发于肢端,生长缓慢,复发率高,与肢端结节性腱鞘炎的临床病理和免疫表型有明显相似处,惟没有特征性异型细胞和明显黏液,MIFS和结节性腱鞘炎相互关系有待进一步研究.     

肢端黏液样炎性纤维母细胞肉瘤2例及文献复习

目的探讨肢端黏液样炎性纤维母细胞肉瘤的临床病理学特征及鉴别诊断。方法对2例发生在下肢末端的黏液样炎性纤维母细胞肉瘤进行光镜观察和免疫组化标记，并复习文献。结果2例发生在下肢末端的病程较长的渐进性肿块，术后局部复发。镜检：病变呈多结节状，边界不清；黏液样基质中见数量不等的各类炎细胞浸润，散在或灶性分布梭形、奇异形和多空泡状脂肪母细胞样3种形态的瘤细胞。免疫表型：肿瘤细胞Vim弥漫阳性，CD68和CD34灶性阳性，CK、SMA、HHF-35、S-100蛋白、CD45、CD45R0、CD15、CD30均阴性。结论此病病程较长，术后易局部复发，是一种低度恶性的肿瘤。鉴于病变黏液样基质及各类炎细胞浸润的背景较为突出，而特征性的瘤细胞稀疏，应注意与炎症性病变或具有相似组织形态的良性或恶性肿瘤鉴别。     

骨内侵袭性血管黏液瘤的诊断及与其他黏液性骨肿瘤的鉴别

目的探讨骨内侵袭性血管黏液瘤(aggressive angio-myxoma,AAM)的临床病理特征、诊断及鉴别诊断。方法对1例骨内AAM的临床、影像学和病理特征进行观察,并通过HE、免疫组化染色将其与1例软组织AAM、1例黏液样软骨肉瘤(myxofibrosarcoma,MFS)、1例黏液纤维肉瘤(myxofi-brosarcoma,MFS)、2例黏液性脂肪肉瘤(myxoid/round cellliposarcoma,ML/RCL)及4例肌内黏液瘤(intramuscularmyxoma,IM)进行对比分析。结果镜下骨内AAM由稀疏排列的细胞及富含黏液的水肿性间质组成,细胞呈星形或梭形,部分细胞呈肌纤维母细胞样,细胞核小,梭形,细胞间可见疏松排列的纤细红染的胶原成分,胶原间可见大小不等扩张的血管,局部可见肿瘤浸润骨皮质进入周围肌肉组织。免疫表型:所有肿瘤组织均表达vimentin,骨内AAM与软组织AAM尚表达SMA、actin,未检测到ER、PR的表达,软组织AAM表达PR及CD34,两者均未检测到desmin的表达。黏液样软骨肉瘤及黏液性脂肪肉瘤尚表达S-100,黏液纤维肉瘤及肌内黏液瘤尚表达CD68。结论骨内AAM罕见,诊断时应结合组织学及免疫组化特点并与其他含黏液的肿瘤相鉴别。     

非典型褥疮性纤维组织增生

目的：探讨非典型褥疮性纤维组织增生的病理形态学特点。方法：对2例非典型褥疮性纤维组织增生进行临床病理学分析和免疫组化研究,并复习文献。结果：2例均为行动不便的老年患者,临床上分别表现为臀部和肩部深部软组织内的无痛性肿块。镜下,病变界限不清,呈多结节状,并大致呈现区带性,即由位于中央的成片纤维素性坏死区和位于周边的肉芽肿样组织组成,间质呈明显的黏液样变性。肉芽肿样区内的增生性纤维母细胞形态各异,有一定的异型性,并常见节细胞样细胞。似增生性筋膜炎。免疫组化显示强阳性表达vimentin,部分弱阳性表达α-SMA或MSA。除增生的纤维母细胞外,周边还可见增生的薄壁小血管,其内皮多肿胀,部分区域内似与周围的增生性纤维母细胞有移行。部分血管壁伴有透明样变性或可见纤维素性沉着。结论：非典型褥疮性纤维组织增生属于一种少见类型的假肉瘤性纤维母细胞性增生,由局部软组织长期间歇性受压引起局部缺血所致,有别于褥疮性溃疡。因增生的纤维母细胞常显示一定异型性,容易被误诊为肉瘤性病变,诊断时应特别加以注意。另一方面也需注意与增生性筋膜炎等其他类型的假肉瘤性纤维母细胞增生鉴别。     

浅表肢端纤维黏液瘤的临床病理特征

目的 探讨浅表肢端纤维黏液瘤(SAF)的临床病理学特点、免疫表型和鉴别诊断.方法 对1例发生于左手中指末端SAF的临床表现、组织形态和免疫学表型进行回顾性分析,并复习文献.结果 患者男,62岁.因左手中指背侧末端肿块伴疼痛就诊,曾有外伤史.术中见肿块近甲床,并深达骨膜.大体观察,肿块周界不清,直径约2 cm,切面呈灰白色,实性,质韧.镜下观察,肿瘤位于真皮层内,略呈分叶状.瘤细胞由梭形至星形纤维母细胞样细胞组成,呈杂乱状分布于黏液样基质内,局部区域可呈条束状或疏松的席纹状排列.黏液样基质内含有丰富的纤细血管,并可见较多散在的肥大细胞.瘤细胞异型性不明显或仅显示轻度的异型性,核分裂象罕见.肿瘤内也未见坏死.免疫组织化学标记显示,梭形和星形细胞表达波形蛋白、CD34和CD99,灶性表达CD10,不表达上皮细胞膜抗原、肌动蛋白、结蛋白和S-100蛋白.结论 SAF好发于成年人指趾末端.熟悉其临床病理特点则有助于与其他发生于指趾的软组织黏液性肿瘤相鉴别.临床上宜将SAF作完整性切除,以预防局部复发.     

低度恶性肌纤维母细胞性肉瘤

目的 探讨低度恶性肌纤维母细胞性肉瘤的临床病理学特征、免疫学表型和超做结构特点。方法 对1例发生于左上颌窦的低度恶性肌纤维母细胞性肉瘤进行临床资料复习、光镜观察、免疫组化标记和电镜检测。结果 患者因“左上颌窦囊肿”2次行切除活检，病理诊断分别为“纤维组织增生”和“鳞状细胞癌Ⅰ级”。4个月后因左上颌骨隐痛行左上颌骨部分切除术，术后病理检查未发现肿瘤组织。5个月后左上后磨牙区肿块复发，再行左上颌、颊、颈联合根治术，诊断为“侵袭性纤维瘤病”。9个月后左侧颧部出现包块，术后病理为“左上颌纤维肉瘤，部分伴平滑肌分化”复片显示，第1次活检标本中的“纤维组织增生”实际上是梭形细胞肿瘤组织，而第2次活检中的“鳞状细胞癌1级”实为鳞状上皮假上皮瘤样增生。第3次术后标本中肿瘤组织不明显，而第4次术后标本则由成束的梭形细胞组成，弥漫浸润至邻近的软组织内，类似侵袭性纤维瘤病，但部分区域内可见鱼骨样排列结构，类似低度恶性纤维肉瘤。第5次术后标本中瘤细胞显示轻～中度的异型性，可见核分裂象(2个／10HPF)，并弥漫浸润横纹肌组织，在部分区域内，瘤细胞穿插在肌束之间形成类似增生性肌炎中的棋盘样结构，另一些区域则在形态上类似经典的纤维肉瘤。免疫表型：瘤细胞表达Vim、α—SMA和Ki—67，不表达MSA、Des、h—caldesmon和S—100蛋白。电镜下瘤细胞胞质内可见到平行肌丝。结论 低度恶性肌纤维母细胞性肉瘤是一种少见的软组织肉瘤，组织学形态、免疫表型及超微结构均显示瘤细胞具肌纤维母细胞性分化。     

浅表肢端纤维黏液瘤1例临床病理学观察

目的 探讨浅表肢端纤维黏液瘤的临床病理学特征、诊断及鉴别诊断。方法 对1例发生于右手食指末端的浅表肢端纤维黏液瘤的临床表现、组织学形态及免疫表型进行回顾性分析,并文献复习。结果 患者男性,78岁,因右手食指末端肿块伴疼痛就诊。术中见肿块累及甲床,深至骨膜。大体可见肿块界限不清,直径约2 cm,切面灰白色,实性,质韧。镜下肿瘤位于真皮层内,无包膜。肿瘤实质由星形及梭形纤维母细胞样细胞组成,肿瘤细胞杂乱排列于间质中,部分区域呈席纹状及束状排列,间质呈黏液样及黏液胶原样。黏液样基质内见较丰富的纤细血管,并见散在的肥大细胞。肿瘤细胞温和,轻度异型。肿瘤无坏死,未见核分裂象。免疫表型:肿瘤细胞vimentin、CD34、CD99均呈弥漫阳性,EMA灶阳性,S-100、HMB-45、SMA、MSA、desmin、GFAP和CK均呈阴性。术后随访10个月,未见复发。结论 浅表肢端黏液瘤是一好发于指趾末端的软组织肿瘤,熟悉其临床病理特征,有助于与其他发生于指趾的软组织黏液性肿瘤鉴别。     

伴有肌样/肌纤维母细胞性分化的隆突性皮纤维肉瘤的临床病理分析

目的 探讨隆突性皮纤维肉瘤（ＤＦＳＰ）中肌样／肌纤维母细胞性分化的本质及其临床病理学意义。方法 采用常规ＨＥ切片对１２４例ＤＦＳＰ进行筛选,对6例伴有肌样／肌纤维母细胞性分化的ＤＦＳＰ病例进行免疫组织化学标记,其中２例加做电镜检测。结果 肌样／肌纤维母细胞性分化多出现在纤维肉瘤型ＤＦＳＰ（ＦＳ－ＤＦＳＰ）中,表现为肿瘤周边部或肿瘤内散在性分布的深嗜伊红色小结节或短要束,由梭形细胞组成,细胞多无异型性,核分裂象也罕见,形态上似平滑肌细胞或肌纤维母细胞。免疫组织化学标记显示肌样区域细胞表达α－平滑肌肌动蛋白和肌物质特异性肌动抗原,不表达ＣＤ３４;电镜观察证实细胞含有质膜下微丝束、局灶性致密体及微胸饮囊泡样结构,与肌纤维母细胞相一致,结论 ＤＦＳＰ中的肌样／肌纤维母细胞性分化可能是间质中肌纤维母细胞增生的结果,并非代表了瘤细胞的真性肌纤维母细胞性分化。     

关节旁黏液瘤临床病理特点

目的探讨关节旁黏液瘤(Juxta-articular myxoma,JAM)临床病理特点和鉴别诊断。方法对1例JAM进行组织形态学观察、免疫组化标记并复习文献。结果肿块位于左胫骨内侧髌骨下缘,不规则组织7cm×3cm×2cm,境界不清,切面周围为淡黄色脂肪组织,中央大部分区域呈黏液胶冻状。镜检:梭形、星芒状纤维母细胞样瘤细胞稀疏散在分布于丰富的黏液样基质中,细胞形态良善,间质血管稀少。部分区域血管丰富。散在有形状、大小不同的囊性腱鞘囊肿样腔隙。肿瘤界限不清,内有脂肪组织陷入。特殊染色:黏液样基质阿辛蓝弥漫(+)。免疫表型:瘤细胞Vim(+),部分瘤细胞α-SMA、CD34(+)。随访8个月未见复发。结论 JAM为良性病变,组织形态和免疫表型类似于肌内黏液瘤,见于膝、肩、肘、踝、髋等大关节旁。约有1/3病例复发,故长期随访是必要的。需与黏液性脂肪肉瘤、黏液纤维肉瘤、骨外黏液性软骨肉瘤、低度恶性纤维黏液样肉瘤等鉴别,避免过度治疗。     

去分化脂肪肉瘤28例临床病理分析

目的 探讨去分化脂肪肉瘤(dedifferentiated liposarcoma,DL)中去分化成分的形态学特征.方法 用常规HE染色和免疫组织化学方法,对28例DL进行观察分析.结果 在28例DL中,25例由非典型脂肪瘤样肿瘤/高分化脂肪肉瘤和非脂肪性梭形细胞肉瘤组成;1例为黏液样脂肪肉瘤和非脂肪性梭形细胞肉瘤组成;2例复发性病例未见到高分化脂肪肉瘤成分,均为非脂肪性梭形细胞肉瘤成分.免疫组化:28例中有14例行免疫组化染色,脂肪肉瘤区域脂母细胞S-100蛋白(+),部分脂肪肉瘤中的梭形细胞CD34(+).14例DL中去分化成分3例SMA和HHF35(+),1例desmin和HHF35(+),CD34、CD117、S-100、CD99、AACT、HMB-45、CK、CR均(-),CD68部分病例散在(+).通过对DL的形态学观察发现,去分化区域可以单独或混合呈现以下形态结构:(1)多形性恶性纤维组织细胞瘤样,(2)纤维肉瘤样,(3)低度恶性黏液纤维肉瘤样,(4)纤维瘤病样,(5)平滑肌肉瘤样,(6)脑膜瘤样漩涡结构,(7)横纹肌肉瘤分化,(8)骨/软骨分化,(9)炎性肌纤维母细胞瘤样,(10)血管外皮瘤样等.其中炎性肌纤维母细胞瘤样和血管外皮瘤样结构文献中尚未见报道.结论 DL中去分化成分最常见的结构是高级别肉瘤形态,但也可以是低度恶性黏液纤维肉瘤样、纤维瘤病样、炎性肌纤维母细胞瘤样、血管外皮瘤样等低级别肉瘤形态.可以是单一分化,也可以向平滑肌、横纹肌、骨/软骨等异源性分化.     

Acral myxoinflammatory fibroblastic sarcoma fine needle aspiration: a case report

Cytological diagnosis of low grade sarcomas can be a daunting task, owing to the varied cytomorphological appearances possible. We report a case of acral myxoinflammatory fibroblastic sarcoma (AMIFS) in a woman who presented with a longstanding mass on the dorsum of her left foot. The diagnosis was suggested by fine needle aspiration cytology and established by wide excision. Microscopic examination showed that fine needle aspirate smears of this lesion contained the characteristic features seen in the surgical excision of this AMIFS: myxoid material, spindled to epithelioid cells with variably prominent nucleoli, nuclear pseudoinclusions, bipolar cytoplasmic extensions, globules of extracellular material, and bizarre virocyte or ganglion-like giant cells.     

Acral myxoinflammatory fibroblastic sarcoma with unique clonal chromosomal changes

Acral myxoinflammatory fibroblastic sarcoma is a rare tumor of the distal extremities. We present the hitherto unreported karyotypic abnormalities of this new entity. The tumor presented as a mass in the dorsum of the foot in a 53-year-old woman and showed the typical virocyte-like and lipoblast-like cells in a myxoid and inflammatory background. Cytogenetic analysis revealed a complex karyotype with a reciprocal translocation t(1;10) (p22;q24) in addition to the loss of chromosomes 3 and 13. Fluorescence in situ hybridization with the 769E11YAC and BAC 31L5 and 2H23 probes showed the breakpoint to be located proximally to BCL10 and distally to GOT1 genes on chromosomes 1p22 and 10q24, respectively. The presence of these clonal chromosomal changes supports the neoplastic nature of acral myxoinflammatory fibroblastic sarcoma and underscores that it represents a separate entity.     

Acral myxoinflammatory fibroblastic sarcoma: a report of five cases and review of the literature

Five cases of acral myxoinflammatory fibroblastic sarcoma that occurred in the distal extremities within the subcutaneous tissue are described. In one case, recurrence and metastases were recognized rather rapidly, only 3 months after the first excision. There have been no reports of early recurrence or metastases, especially the latter. The predominant type of constituent cells, cellularity of the neoplastic cells and density of inflammatory cells varied microscopically among cases. However, characteristic ganglion-like cells, Reed-Sternberg-like cells, round mononuclear cells and myxoid stroma, sometimes only seen focally, were found in all cases. Positive immunoreaction for vimentin was present in all cases. There was no correlation between positivity of MIB-1 or p53 for the primary tumor and presence of recurrence or metastases. In conclusion, we should be more cautious about the possibility of recurrence or metastases in earlier phases of acral myxoinflammatory fibroblastic sarcoma. Identification of the atypical bizarre fibroblastic component as the manifestation of the malignant nature of this lesion is vital to correct diagnosis, and it is important to attend to the myxoid and hyalinized zones, the inflammatory infiltrate, the presence of ganglion-like cells and acral location as features of acral myxoinflammatory fibroblastic sarcoma.     

黏液炎性纤维母细胞性肉瘤的临床病理学观察

目的 探讨黏液炎性纤维母细胞性肉瘤(MIFS)的临床病理学特征、诊断和鉴别诊断.方法 对6例MIFS的临床资料、光镜形态和免疫表型进行回顾性分析,并复习文献.结果 6例均发生于成年人,其中男性2例,女性4例,中位和平均年龄分别为47岁和50岁.肿瘤位于下肢3例,上肢2例,腋窝1例.临床上,患者多表现为肢体局部肿胀或缓慢性生长的肿块,伴有轻微疼痛或胀痛感.大体上,肿瘤呈灰白色结节状,直径2.5～4.6 cm(平均3.4 cm).镜下,肿瘤由黏液样区域、玻璃样变区域和炎性区域混杂组成.除梭形细胞外,在3种区域内均可见到呈单个散在分布或小簇状分布的异型大细胞,核大、核仁明显,形态上类似病毒细胞、R-S细胞或神经节细胞,核分裂象罕见.在黏液样区域内还可见到黏液湖形成及漂浮的单泡状或多泡状脂母样细胞.免疫组织化学标记显示,畸形大细胞主要表达波形蛋白,其他标记包括白细胞共同抗原、CD30、CD68、CD34、S-100蛋白、HMB45、细胞角蛋白和肌动蛋白等均为阴性.随访4例,2例于局部切除后复发.结论 MIFS是一种低度恶性的纤维母细胞性肉瘤,易被误诊为良性病变,熟悉其临床病理学特点有助于诊断和鉴别诊断.

Abstract：

Objective To study the clinicopathologic features, immunophenotypes and differential diagnosis of myxoinflammatory fibroblastic sarcoma (MIFS). Methods The clinical and pathologic features of 6 cases of MIFS were analyzed. lmmunohistochemical study was performed using the standard EnVision method. Results There were altogether 2 adult males and 4 adult females ( median age =47 years and mean age = 50 years). Three cases were located in the lower extremities, 2 in the upper limbs and 1 in the axillary region. Common presentation included slowly growing mass or swelling in the extremities, accompanied by mild pain or tenderness. Grossly, the tumor appeared multinodular and ranged from 2. 5 cm to 4. 6 cm in diameter ( mean = 3.4 cm). Microscopically, there was a dense inflannatory infiltrate merging with hyaline and myxoid zones in various proportions. Spindle-shaped tumor cells were seen admixed with large atypical cells which distributed singly or in small clusters, amongst an inflammatory, hyaline or a myxoid background. These atypical cells had large nuclei and prominent nucleoli, resembling virocytes, Reed-Sternberg cells or ganglion cells. Mitotic figures were rarely identified. Extracellular mucin associated with scattered monovacuolated or multivacuolated lipeblast-like cells was noted. Immunohistochemically, these bizarre cells were consistently positive for vimentin, but negative for a panel of antibodies including LCA,CD15, CD30, CD34, CD68, S-100, HMB45, AE1/AE3, smooth muscle actin and desmin. Follow-upresult was available in 4 cases; and 2 of them showed local recurrence after an incomplete excision. There was no evidence of distant metastasis. Conclusions MISF is a low-grade sarcoma of fibroblastic differentiation. Awareness of the clinical and pathologic characteristics is helpful in arriving at the correct diagnosis and distinction from benign inflammatory fibromyxoid lesions.     

Akrales myxoinflammatorisches fibroblastisches Sarkom Sechs Fälle einer neuen Tumorentität

Acral myxoinflammatory fibroblastic sarcomas are low-grade sarcomas of the distal extremities, first described by Meis-Kindblom and Kindblom in 1998. In our series of six cases this sarcoma occurred in four men and two women aged 22-60 years (mean 42.8). The tumors measured 1-4 cm (mean 2.4) and were localized in the foot (two cases), finger (two), wrist (one), and upper arm (one). The patients had a short history of a painless mass (mean duration 3 months). The tumors were poorly circumscribed, and infiltrated the subcutaneous fat and in one case each also the dermis and musculature. Histological features of the tumors were a multinodular configuration with infiltrative margins, myxoid and fibrotic zones with hyalinized areas, moderately pleomorphic cells with large cells showing a prominent nucleolus and an inflammatory infiltrate consisting of lymphocytes and histiocytes. Two months after excision the tumor recurred locally in two cases; no metastases were observed. Differential diagnosis is especially necessary from inflammatory pseudotumors and more aggressive sarcomas.     

Acral myxoinflammatory fibroblastic sarcoma: a report of two cases

Acral myxoinflammatory fibroblastic sarcoma is a rare low-grade malignant soft tissue tumor, usually observed in the extremities of middle-aged adults. We report two cases which occurred in the thumb and knee of middle-aged women. Both tumors showed a multinodular architecture, with cellular areas, occasional foci of hyalinized fibrosis, and hypocellular areas with a myxoid background. Various neoplastic cells were identified including spindled or rounded epithelioid cells and occasional bizarre giant cells, morphologically mimicking Reed-Sternberg cells or ganglion cells. Tumor cells were strongly immunoreactive for vimentin, and variably positive for CD68 and CD34. Both tumors were completely resected and patients were free of disease without any further treatment after a mean follow-up of 14 months.     

Low-grade periductal stromal sarcoma of the breast with myxoid features: Immunohistochemistry

A 52-year-old woman was admitted with a painful right breast tumor measuring more than 20 cm in largest diameter, which ulcerated the overlying skin. The lesion had appeared 4 years previously but the patient hesitated to seek medical care due to 'fear of cancer'. Microscopically, the tumor was composed of spindle cells that formed cuffs around multiple open tubules and ducts set in an abundant, myxoid stroma. The spindle cells had significant atypia with nuclear pleomorphism, occasional cytoplasmic vacuolation and moderate mitotic activity. The ducts and lobules surrounded by the proliferating tumor cells had minimal distortion, with a pericanalicular growth pattern devoid of the phyllodes pattern. The tumor had a multinodular growth pattern with coalesced and individual tumor nodules, the latter being found mostly at the periphery of the lesion. On immunohistochemistry the tumor cells were positive for smooth muscle actin, CD34, and vimentin, and focally positive for CD10. A diagnosis of low-grade periductal stromal sarcoma (PDSS) with myxoid features was established. PDSS is a distinct low-grade breast sarcoma, the appropriate diagnosis of which requires extensive tumor sampling and additional broad immunohistochemistry. PDSS should not be confused with other spindle cell breast tumors because they require different treatment.