黏液纤维肉瘤和低级别纤维黏液样肉瘤的诊断和鉴别诊断

黏液纤维肉瘤（myxofibmsarcoma,MFS）,尤其是其亚型,低级别黏液纤维肉瘤（low-grademyxofibmsarcoma,LGMFS）,与低级别纤维黏液样肉瘤（low-gradefibromyxoidsarcoma,LGFMS）不仅好发部位,而且命名相似,颇易相互混淆,但二者的生物学行为不同,鉴别十分必要。MFS是真性肉瘤,依其分化程度分为3级,其中LGMFS最需要与LGFMS相鉴别。LGFMS亦称为Evans瘤,曾被认为有较高的复发、转移及病死率,但现有研究表明这三方面均较过去的报道少得多。因此,LGFMS与隆突性皮肤纤维肉瘤一样,属低级别肉瘤,即一种中间型软组织肿瘤。同时,需与软组织的其他黏液性肿瘤,如肌内黏液瘤、富于细胞的黏液瘤、关节旁黏液瘤、侵袭性血管黏液瘤、浅表性血管黏液瘤、神经鞘黏液瘤以及黏液样脂肪肉瘤和黏液样隆突性皮肤纤维肉瘤等进行鉴别。在日常诊断病理学工作中,易将MFS和LGFMS误诊为软组织的其他黏液性肿瘤。因此,弄清二者的诊断标准和鉴别诊断,在实际工作中十分重要。     

CD163在皮肤梭形细胞肿瘤中的诊断价值

皮肤恶性梭形细胞肿瘤种类较多,如非典型纤维黄色瘤(AFX)、梭形细胞鳞状细胞癌(SCC)、梭形细胞/促纤维增生性恶性黑色素瘤、皮肤隆突性纤维肉瘤(DFSP)和平滑肌肉瘤等,这些恶性梭形细胞肿瘤的形态学诊断有时比较困难.一般来说,AFX是一个排除性诊断,是根据免疫标记CK和S-100蛋白表达阴性,而组织细胞标记物CD68表达阳性而做出的诊断.     

纤维肉瘤少见亚型的诊断及鉴别诊断

在20世纪50-70年代，纤维肉瘤是日常工作中最常诊断的一种软组织梭形细胞肉瘤，但随着诊断技术的进步，特别是免疫组织化学的普及以及细胞和分子遗传学检测的开展，发现过去所诊断的纤维肉瘤实际上是其他类型的梭形细胞肉瘤，如单相纤维型滑膜肉瘤和恶性周围神经鞘膜瘤等，故真正的纤维肉瘤并不多见，在目前的实际工作中，往往是在排除其他类型的梭形细胞肉瘤后才诊断。     

隆突性皮肤纤维肉瘤的形态转化及其恶性潜能

目的：探讨隆突性皮肤纤维肉瘤的形态转化及其恶性潜能。方法：采用ＨＥ和组织化学染色,对４８例隆突性皮肤纤维肉瘤作临床病理分析。结果：４８例肿瘤被分为普通型、粘液型和纤维肉瘤变３个亚型。结论：普通型隆突性皮肤纤维肉瘤是一种低度恶性肿瘤,纤维肉瘤变型的恶性度更高,粘液型的恶性度与其它两型的差异尚待观察。     

婴儿型纤维肉瘤的临床病理分析

目的探讨婴儿型纤维肉瘤的临床病理学特征、诊断、鉴别诊断和预后。方法收集12例手术切除的婴儿型纤维肉瘤的临床资料进行肉眼观察、光镜和免疫组化检查,并进行随访。结果12例中,男性患儿5例,女性7例;平均年龄（4.7±4.4）个月,术前病程（18.7±30.5）个月。肿块以胸背部和头颈部好发,平均直径（5.8±3.2）cm,无包膜。镜下梭形细胞形态较一致,致密,排列成鲱鱼骨样,核分裂显著,胶原纤维较少或缺乏。瘤细胞免疫组化染色表达vim entin,SMA、MSA染色结果不明确。手术切除后未见局部复发及转移的病例。结论婴儿型纤维肉瘤大多发生于1岁之内,染色体的检查可帮助诊断,治疗以广泛局部手术切除为主,可辅以化疗。需与婴幼儿纤维瘤病、恶性纤维组织细胞瘤、横纹肌肉瘤、良性或恶性神经鞘瘤、平滑肌肉瘤、滑膜肉瘤、血管外皮瘤及成人型纤维肉瘤相鉴别。     

5例子宫Mullerian腺肉瘤形态学观察

通过光镜、电镜、组化及免疫组化等方法观察5例子宫Müllerian腺肉瘤之形态学特点。其腺体衬以Müllerian管多型良性上皮,而其肉瘤部分既可是高分化,亦可为低分化之多形性。特染及免疫组化染色证明其肉瘤部分是内膜间质肉瘤、纤维肉瘤、少量平滑肌及粘液肉瘤,未见确切横纹肌及软骨成分。据光、电镜特点支持本瘤起源于多潜能的内膜间充质细胞,对瘤巨细胞和透明球的性质进行简要讨论。     

色素型隆突性皮肤纤维肉瘤8例光镜、免疫组织化学及电镜观察

目的 探讨色素型隆突性皮肤纤维肉瘤(DFSP)临床病理特征和鉴别诊断。方法 对8例色素型DFSP进行了光镜和免疫组化染色观察，其中4例做电镜观察。结果 色素型DFSP患者的年龄、性别、肿瘤部位和肿瘤生长方式与普通型DFSP相同。随访结果3例发生复发，无1例发生转移。4例肿瘤切面见浅黑色或黑色斑。所有病例既可见典型的DFSP组织形态学特征，同时又见散在性分布、数量多少不等的黑色素细胞。本组6例瘤组织显示普通型DFSP图像，2例部分区域显示纤维肉瘤型DFSP图像，偶可见血管壁平滑肌增生形成的肌样结节和黏液样变性区。Fontana染色黑色素细胞呈阳性，Perls染色则为阴性。梭形瘤细胞呈vim和CD34阳性，CD34阳性具有一定辅助诊断价值，S-100蛋白和NSE阴性；黑色素细胞呈S-100蛋白和Vim阳性；纤维肉瘤型DFSP区Ki—67阳性表达率高于普通型DFSP区。电镜观察：可见梭形纤维母细胞样细胞和含有许多成熟黑色素小体黑色素细胞。结论 色素型DFSP是一种少见的DFSP亚型，临床病理特征与普通型DFSP对比有许多共同之处，需与动脉瘤样型纤维组织细胞瘤、弥漫性神经纤维瘤、细胞性蓝痣和促结缔组织增生性黑色素瘤鉴别。     

肢端黏液炎性纤维母细胞性肉瘤

目的 探讨肢端黏液炎性纤维母细胞性肉瘤的临床病理学特征、免疫学表型及其鉴别诊断。方法 对1例发生于足背和右小腿远端的肢端黏液炎性纤维母细胞性肉瘤进行光镜观察和免疫组化标记。结果 患者因足背皮下“结节性筋膜炎”局部切除术后复发就诊。体检发现足背至右小腿远端前外侧皮下多发性结节,直径1～4cm,影像学检查提示肿瘤累及深部骨膜。镜下肿瘤由比例不等的黏液样区、透明变性区及炎症性区域混合组成。黏液样区域主要由交织条柬状排列的梭形瘤细胞组成,核显示轻至中度异型性,核分裂象罕见,间质疏松、黏液样,局部区域可见黏液湖形成。其内可见单空泡印戒样或多空泡状假脂肪母细胞,形态类似黏液纤维肉瘤。透明样区域由散在的胖梭形至卵圆形的瘤细胞和透明样间质混和组成。炎症性区域由成簇的淋巴细胞组成,与黏液样区域和透明变性区相混杂。病变内可见体积较大类似节细胞或R-S细胞的畸形细胞。免疫组化标记显示瘤细胞弥漫表达Vim,个别细胞表达p53,而CD68、actin、Des、CD34、CD30和S-100蛋白等标记均为阴性,多数淋巴细胞表达CD45RO。结论 肢端黏液炎性纤维母细胞性肉瘤是一种罕见的低度恶性软组织肉瘤,瘤细胞由变异的纤维母细胞衍化而来,熟悉其形态学特征对避免误诊为其它良性或恶性病变具有重要意义。该瘤常在局部呈侵袭性生长,具有较高的复发率,临床上应予以完整切除。     

梭形细胞型癌光镜与电镜观察

本文报告3例梭形细胞型癌(口腔,颈和手指)光镜与电镜观察。病人年龄26～73岁,男性2例,女性1例。主要症状是无痛性包块和溃疡。组织病理学特征是由成片梭形细胞构成,类似肉瘤。电镜检查梭形瘤细胞显示张力原纤维和无数发育的桥粒。因此,梭形细胞癌应与纤维肉瘤和癌肉瘤相区别。本文强调超微结构诊断梭形细胞癌的重要意义。     

软组织肌纤维母细胞肉瘤的组织学特征及鉴别诊断

软组织肌纤维母细胞肉瘤是一种独立的新型肿瘤，较罕见。光镜下主要由梭形肿瘤细胞组成。组织化学及免疫组化染色具有辅助诊断价值。电镜下，瘤细胞胞质中含有丰富的粗面内质网、大量肌丝束及多少不等的密体和密斑，这是诊断的重要依据。鉴别诊断主要包括平滑肌肉瘤和纤维肉瘤     

Tenascin expression in human dermis is related to epidermal proliferation.

The extracellular matrix glycoprotein tenascin is sparsely distributed in normal human dermis. The authors have shown that in a number of skin diseases (psoriasis, skin tumors), tenascin expression is strongly increased. In this immunohistochemical study, using polyclonal and monoclonal antisera, we have tested the hypothesis that tenascin expression in vivo is linked to epidermal proliferation. Using the sellotape stripping model in normal human skin, which causes a rapid recruitment of keratinocytes into the cell cycle, induction of tenascin expression was found in the upper dermis within 24 hours after stripping. In contrast, in normoproliferative monogenic disorders of keratinization (X-linked recessive ichthyosis, autosomal dominant ichthyosis vulgaris, non-erythrodermic lamellar ichthyosis), no increase in tenascin expression was found compared with normal skin. These findings demonstrate a relationship between epidermal proliferation and metabolic alterations in the dermal compartment.     

Cutis rhomboidalis protects skin from malignant epithelial tumors

Cutis rhomboidalis nuchae is a skin alteration which comes from chronic sun exposure and it integrates the solar elastosis group, acquiring a coriaceous aspect, with a yellowish and grooved surface. There is the occurrence of elastic and collagen fibers degeneration found in the dermis caused by ultraviolet radiation [1]. Another group of skin diseases which has solar exposure as a determining factor is the group of actinic keratoses, the non-melanoma malignant epithelial tumors {basal cell carcinoma (CBC) and squamous cell carcinoma (CEC)} [2].However, the occurrence of actinic keratoses, CBCs or CECs on the area of cutis rhomboidalis is infrequent in dermatology clinical practice. The authors do not know why people with neoplasias and pre neoplastic lesions in some areas with chronic photo damage amendments (face and upper limbs), do not present the same pre and neoplastic lesions in areas with similar appearance of chronic sun damage (nape). The authors seek to understand why the nape is protected for pre and neoplastic lesions. We suggest that cutis rhomboidalis protects skin from malignant epithelial tumors in nuchae.     

Syringomatous tumor of the nipple

The authors describe two women with locally infiltrative neoplasms of the nipple that exhibited sweat duct differentiation. The findings are compared with those previously recorded for syringomatous adenoma of the nipple. The patients presented with firm, subareolar nodules. Nests, cords, and ducts composed of cytologically uniform squamous cells were dispersed throughout the dermis and also involved subcutaneous tissue and adjacent breast parenchyma. Small keratocysts formed a minor component of the lesions. Additional observations in the present study included connections between tumor and epidermis; distinctive fibrous stroma; sparse mitotic figures; perineural invasion; carcinoembryomic antigen in luminal content and periluminal cytoplasm; tumor cells that contained S-100 protein; and Langerhans' cells within neoplastic cellular aggregates. Neither of the lesions had recurred after follow-up of 9 and 12 months. The foregoing neoplasms of the nipple appear to be part of a family of microscopically similar tumors that have a predilection for the face, breast and possibly the axilla and salivary tissue. Although capable of local recurrence, syringomatous tumors of the nipple and related lesions at other sites have not been reported to metastasize.     

Tumors of the skin composed of large cells with abundant eosinophilic cytoplasm.

Tumors of the skin composed of large epithelioid cells displaying abundant smooth or granular eosinophilic cytoplasm are relatively rare, yet may constitute a source of diagnostic difficulties for pathologists. Lesions derived from various skin elements are represented in this group, including tumors originating from adnexal structures and connective tissue elements of the dermis. The large, pink-cell or epithelioid appearance of the tumor cells may be the result of attempts to recapitulate the phenotype of a cell line normally characterized by such morphological appearance, such as apocrine epithelium, or may result from secondary changes in the cytoplasm of the tumor cells, such as accumulation of autophagosomes in granular cell tumors. The characteristic pathological features of this group of tumors is the subject of this review, with special emphasis on their differential diagnosis and the morphological features that may be of help for practicing pathologists to arrive at a correct diagnosis.     

Benign fibrous histiocytoma of the skin with potential for local recurrence: a tumor to be distinguished from dermatofibroma

We studied eight patients with benign fibrous histiocytoma (BFH) of the skin that differed, morphologically and biologically, from ordinary dermatofibroma (DF) and compared their tumors with a consecutive series of 141 DF. The patients ranged in age from 13 to 49 yr. Their lesions consisted of cutaneous nodules, 2 cm or less in size, that had been present for less than 1 yr. Seven were located on an extremity. Three of six patients (50%) who could be followed had recurrences 2, 5, and 8 mo following excision of their tumors. Microscopically, seven of the eight cutaneous BFH (CBFH) involved both dermis and subcutis. Five were distinctly multinodular. Subcutaneous involvement and/or multinodularity were present in each case. Fibroblastic and histiocytic cells were arranged in a monotonous storiform pattern throughout most or all of each lesion. There was moderate nuclear variability, but pleomorphism was absent. Several of the tumors contained sparse inflammatory, multinucleate, and foam cells and hemosiderin deposits. Vascularity was prominent. We believe that the multinodular architecture of CBFH and its involvement of both dermis and subcutis usually allow distinction from DF. The latter is uninodular, centered in the dermis, and exhibits only minor subcutaneous extension. Most DF lack the diffuse, well developed storiform appearance of CBFH. Local recurrence further distinguishes CBFH from DF, which rarely recurs. Rather than representing an aberrant form of DF, the clinical and microscopic features of CBFH suggest that it is closely related to, if not identical with, BFH of other tissues and organs.     

Giant cell tumor of the skin: a morphologic and immunohistochemical study of five cases

Giant cell tumor (GCT) of the skin is a rare entity that possesses similar gross and histologic features to GCT of bone. When located predominantly in the dermis GCT has been mistaken for benign fibrous histiocytoma and atypical fibroxanthoma. We report the clinical, morphologic, and immunohistochemical features of five cases of GCT of the skin. With one exception, all tumors are confined to the dermis. Patients' ages range from 6 to 78 years (median, 73 years) with a male to female ratio of 3:2. Gross and histologic features of the lesions are similar to those of GCT of bone (eg, brown fleshy tumor and a biphasic population of mononuclear cells admixed with osteoclast-like giant cells, respectively). The nuclei of the giant cells are similar to those of the mononuclear cells. A fascicular pattern with focal storiform arrangement of spindle neoplastic cells is noted in two cases. The osteoclast-like giant cells and some of the mononuclear cells are strongly positive for CD68, alpha-1-antitrypsin, and alpha-1-antichymotrypsin. Only the mononuclear cells express smooth muscle actin focally in one case. Both the osteoclast-like giant cells and the mononuclear cells are negative for cytokeratins (AE1/AE3 and CAM5.2) and S-100 protein in all cases. One patient developed lung metastases at presentation and local recurrence 4 months status post surgery. All patients are without evidence of disease 1 month to 12 years status post surgery. Cutaneous GCTs are low-grade sarcomas that can recur locally and infrequently metastasize. These tumors should be distinguished from a variety of cutaneous neoplasms that contain multinucleated giant cells.     

Fine-needle aspiration biopsy: Dermatofibrosarcoma protuberans

Dermatofibrosarcoma protuberans (DFSP) represents a spectrum of mesenchymal spindle cell tumors that typically involve both dermis and subcutis. Presented herein are six cases of DFSP, four of which were initially diagnosed by FNAB. The cytologic features useful in the identification of this lesion on Papanicolaou- and Diff-Quik-stained smears are discussed. Chief among these are the storiform stromal fragments, presence of entrapped adipose tissue and the recognition of fibrohistiocytic spindle cells. The potential pitfalls and the differential diagnostic possibilities of spindle-cell lesions, particularly those of fibrohistiocytic origin are discussed.     

Alterations in proteoglycan synthesis common to healing wounds and tumors.

Wound healing and tumor stroma generation share several important properties, including hyperpermeable blood vessels, extravasation of fibrinogen, and extravascular clotting. In both, the deposits of fibrin gel serve initially as provisional stroma and later are replaced by granulation tissue. Proteoglycans (PG) are also important constituents of the extracellular matrix, but their composition and role in healing wounds and tumor stroma generation are poorly understood. The authors used immunohistochemical and biochemical methods to investigate the dermatan sulfate proteoglycan (DSPG) and chondroitin sulfate proteoglycan (CSPG) composition of healing skin wounds and solid tumors. By immunohistochemistry, the great majority of normal guinea pig and human dermis stained weakly for CSPG and strongly for decorin. In contrast, the granulation tissue of healing skin wounds and scars stained intensely for CSPG and weakly or not at all for decorin; however decorin staining was restored to normal intensity after digestion with chondroitin ABC lyase, suggesting that decorin antigenic sites had been masked by glycosaminoglycan (GAG) chains. Like wounds, the stroma of several carcinomas (line 1 guinea pig, human breast, colon, basal cell, and squamous) stained strongly for CSPG and weakly or not at all for decorin, but decorin staining developed after chondroitin ABC lyase digestion. Thus healing wounds and tumor stroma express a common pattern of altered PG staining, adding another to the properties these pathologic entities share. Proteoglycans extracted from healing wounds after in situ labelling with [35S] Na sulfate contained more CSPG than normal dermis with significantly longer GAG chains. Granulation tissue also synthesized more DSPG than normal skin, with greater heterogeneity and longer GAG chains. These alterations in PG synthesis correlate with the cell proliferation, migration, and collagen synthesis that accompany wound healing and may provide clues to the mechanisms responsible for both wound healing and tumor stroma generation.     

Cytologically interesting cases of primary skin tumors and tumor-like conditions identified by fine-needle aspiration biopsy

The aims of this study are to depict the special but constant cytomorphologic features which allow a distinction among grossly similar skin tumors and to show that cytological assessment of many uncommon skin lesions is possible. The fine-needle aspiration findings of 181 cytologically interesting cases of primary skin tumors selected from 2,301 primary skin lesions examined in our department from 1990–1995 are described. Seventy-five cases were cytologically reported as benign and 89 as malignant. In 17 cases the aspirated material was considered inconclusive, although some possible diagnoses could be supported. A subsequent histological examination followed the cytologic assessment in all cases. Fine-needle aspiration cytology identified uncommon cutaneous cystic lesions (bronchogenic, dermoid, myxoid), benign and malignant tumors of epidermal appendages such as chondroid syringomas, cylindromas, pilomatrixomas, sebaceous carcinoma, and primary adenoid cystic carcinoma, as well as tumors of the dermis and subcutaneous fat. Primary cutaneous lymphomas were also diagnosed and classified morphologically and immunocytochemically. In our diagnoses of uncommon skin tumors new cytologic criteria were created and utilized and are described in this report in detail. Diagn. Cytopathol. 1998;19:17–28. © 1998 Wiley-Liss, Inc.     

Collision tumor composed of mammary-type myofibroblastoma and eccrine adenocarcinoma of the vulva

Collision tumors of the vulva are uncommon. Mammary-type myofibroblastoma is a rare benign tumor arising at extramammary sites with morphological and immunophenotypic features similar to the breast counterpart. Eccrine adenocarcinoma of the vulva is rare. The authors report here a true collision tumor containing a mammary-type myofibroblastoma with intermingling components of eccrine adenocarcinoma occurring in the vulva. An 80-year-old woman presented with a subcutaneous nodule in the vulva. The tumor was well circumscribed but unencapsulated. The mammary-type myofibroblastoma and the adenocarcinoma measured 4 cm × 3.5 cm × 1.0 cm and 1.5 cm × 1.2 cm × 0.8 cm, respectively. In the overlying superficial dermis there was an eccrine adenocarcinoma. It was composed of uniform basophilic cells arranged in tubules, solid nests, and cribriform growth patterns. The eccrine adenocarcinoma infiltrated the mammary-type myofibroblastoma in the submucosa. The soft tissue lesion revealed a spindle cell proliferation composed of fascicles of myofibroblastic cells embedded in a collagenous stroma with a predominance of mast cells. Interspersed thick hyalinized collagen bundles separated the fascicles. Immunohistochemically, the spindle cell tumor strikingly stained for both CD34 and desmin. To the best of our knowledge, this is the first case report of a vulva collision tumor composed of mammary-type myofibroblastoma intermixed with primary eccrine adenocarcinoma in the English literature.