Angiomyofibroblastoma of the vulva: A mitotically active variant?

A case of angbmyofibrobiastoma in a 48-yearold woman is reported. The tumor occurred as a left vulval mass and was treated by simple excision. It was located in the subcutaneous tissue of the left vuiva and was well circumscribed, measuring 2.8 × 2.7 × 2.5 cm. Microscop Ically, the tumor was composed of hypocellular and cellular arees with well-developed small vessels. Spindle or polygonal cells were arranged with perlvascular accentuation In an edematous or fibrocollagenous background. Some spindle-shaped or polygonal stromal cells were also arranged in epithelioid nests. In some areas, mitosas were frequent (maximum 3/10 high-power field). lmmunohistochemicaily, the stromal cells were positive for vimentln and desmin, but negative for α-smooth muscle actin, S-100, neurofilament, estrogen receptor, progesterone remptor, CD31 and CD34. The average labeling index of Kl-67 In stromal cells was 3.1%. Ultrastructural analysis demonstrated that the stromal cells adhered with primitive Junctions and contained lntermediate filaments with no focal density In the cytoplasm. These findings were consistent with anglomyofibrobiastoma, although previously reported cases did not show so many mitoses. Therefore, this case was suggested to be a mitotically active variant.     

血管肌纤维母细胞瘤16例临床病理分析

目的探讨血管肌纤维母细胞瘤（angiomyofibroblastoma,AMF）的临床病理、免疫组化特征、组织来源以及鉴别诊断。方法通过16例AMF的临床表现、病理形态和免疫组化研究,并结合文献复习,总结AMF的临床病理、免疫组化特征及鉴别诊断。结果16例AMF,大体境界清楚,无包膜,可见一层假纤维膜包绕;组织学上呈疏松水肿样,丰富薄壁海绵样血管,肿瘤细胞多样性,梭形及上皮样细胞多见,常围绕血管排列呈疏密交替分布特征。免疫组化结果：瘤细胞desmin及vimentin呈弥漫强阳性表达,actin、CD34、ER和PR染色稍弱、呈灶状分布,而S-100蛋白、NF和CK均阴性。结论AMF临床上多无明显症状;发病部位以女性外阴及盆腔最多见,但全身多处部位也可见;其生物学行为及组织来源尚无定论;术前很难确定诊断AMF,需与侵袭性血管黏液瘤、富于细胞性血管纤维瘤等相鉴别。     

血管肌纤维母细胞瘤2例报道及文献复习

目的 探讨血管肌纤维母细胞瘤(AMF)的临床病理特征及诊断和鉴别诊断。方法 对2例AMF进行组织学观察和免疫组化S—P法标记，抗体为vimentin、desmin、SMA、S—100蛋白、CD34、ER、PR、CK等。结果 1例为30岁女性，表现为外阴囊肿；1例为51岁男性，表现为腹股沟区精索肿块。眼观：肿瘤境界均清楚；镜检：肿瘤均由相互交错分布的细胞密集区和细胞稀疏区组成，其间血管丰富，多为毛细血管至中等大薄壁血管。瘤细胞成巢或束状围绕血管周围排列。可见红细胞漏出。其中1例尚可见成群分布的脂肪细胞存在。免疫表型：瘤细胞vimentin(2／2)、ER(2／2)、PR(2／2)、SMA(1／2)、CD34(2／2)阳性，SMA(1／2)、S—100蛋白(1／2)灶性阳性，desmin、CK阴性。结论 AMF是一种少见的好发于外阴生殖道的间质肿瘤，可能来源于血管周具有多向分化潜能的干细胞，可向肌纤维母细胞分化。在组织形态上AMF应与侵袭性血管黏液瘤、富细胞性血管纤维瘤、浅表性血管黏液瘤、纤维上皮性间质息肉、梭形细胞脂肪瘤相鉴别。     

血管肌纤维母细胞瘤的临床病理和鉴别诊断

目的　探讨血管肌纤维母细胞瘤 (AMF)的临床病理特征和鉴别诊断。方法　通过 4例AMF的病理形态和免疫组化研究 ,结合复习文献总结AMF的临床病理特征和鉴别诊断。结果　肿瘤境界清楚 ,直径 <5cm ,瘤细胞梭形或卵圆形 ,常围绕血管排列 ,呈疏密交替分布特征。免疫表型 :desmin、SMA、vimentin、CD34、ER和PR阳性或部分阳性 ,而MSA、S 10 0蛋白、Mac387和CK均阴性。结论　AMF是一种主要发生于生育期女性外阴的良性软组织肿瘤 ,需与侵袭性血管黏液瘤、富细胞性血管纤维瘤等相鉴别     

Myxoid leiomyoma of the vulva mimicking aggressive angiomyxoma

A case of vulvar leiomyoma with extensive myxoid change in a 40 year old female is described. The tumor had a unique connection with a non-degenerative leiomyoma that compressed the rectum and the bladder. Scattered smooth muscle cells in a loose myxoid stroma were immunoreactive for desmin. Fibroblast-like spindle cells were immunoreactive for vimentin but not for desmin. The initial, although incorrect, pathological diagnosis of the tumor was aggressive angiomyxoma based on the similarity in both clinical and pathological aspects with this more invasive tumor. Myxoid vulvar leiomyoma should also be differentiated from angio-myoflbroblastoma. The key to the differential diagnosis is the presence of interlacing smooth muscle cells and an awareness of tendency toward myxoid change in vulvar leiomyomas.     

Angiomyofibroblastoma of the vulva: Case report with immunohistochemical, ultrastructural and DNA ploidy studies and a review of the literature

A case of anglomyofibroblastoma of the vulva in a 49-year-old woman was examined. The tumor measured 3×2.5×2 cm and appeared light gray to tan In color on the cut surface. Light microscopic examinations revealed that spindle or oval shaped tumor cells were arranged in loose edematous stroma with numerous thin-walled vessels. Ultrastructurally, cell organellae were not well developed but intracytoplasmic filaments of intermediate size were abundant in the tumor cells. Desmin, CD34 and vimentin immunoreactivity were detected in almost all of the tumor cells. Both estrogen and progesterone receptors were diffusely expressed In the tumor, suggestive of the sex sterold-dependency of this tumor. The Ki-67 labeling index was less than 1% and the DNA content of the tumor cells, which was examined by image cytometry, demonstrated diploidy (DNA index=0.97). These findings may reflect the quiescent or slow growing features of angiomyofibroblastoma.     

Anglomyofibroblastoma of the vulva: Case report

Anglomyxomas are a group of relatively rare mesenchymal myxold tumors. Three types of angiomyxomas have been Identltled: superflciat anglomyxoma, aggresslve anglomyx-oma and anglomyoflbroblastoma. These tumors have a predllection for occurring in the perineum of middie-aged femaies and are often clinically diagnosed as Bartholin's cyst A case of a 42-year-old female with a perineal angiomy-ofibrobtastoma mimicking a Bartholin's cyst is presented. The pathological and clinical characteristics of this tumor are discussed.     

Vulvar angiomyxoma, aggressive angiomyxoma, and angiomyofibroblastoma: an immunohistochemical and ultrastructural study

To investigate the histogenetical unifying theory of a single, pluripotential primitive cell for vulvar angiomyxoma, aggresive angiomyxoma, and angiomyofibroblastoma, an optical, immunohistochemical and ultrastructural study of a superficial angiomyxoma, aggressive angiomyxoma, and angiomyofibroblastoma was performed. These three tumors showed immunohistochemical and ultrastructural overlapping features. The results of the study suggest that these three tumor entities probably arise on a common pluripotential primitive cell located around the vessels of connective tissue, which could show the capacity for modulating its penotype toward similar but distinct mature cell types.     

钙化性纤维性肿瘤的临床病理学分析

目的探讨钙化性纤维性肿瘤(calcifying fibrous tumor,CVF)的临床病理学特征、免疫表型和鉴别诊断。方法回顾性分析7例CFT的临床资料、病理学形态和免疫组化标记结果。结果患者中1例为少年,6例均为成年人,年龄14—50岁,平均37岁。5例临床表现为局部缓慢性生长的无痛性肿块,2例为术中偶然发现。肿瘤分别位于腹腔／盆腔(3例)、颈部(2例)、左腹股沟(1例)和左小腿(1例)。眼观：肿块境界清楚,卵圆形或结节状,质地坚韧。镜检：肿瘤由大量胶原化的纤维结缔组织组成,其间夹杂少量梭形细胞。特征性形态学表现为在胶原化的纤维组织间可见散在的钙化灶或砂砾小体,间质内伴有多少不等的淋巴细胞和浆细胞浸润灶,部分病例中可见生发中心形成。免疫组化标记显示,梭形细胞主要表达vimentin,不表达CD34、S-100蛋白、actin、desmin、h-caldesmon和ALK1等标记。随访6例,均无复发。结论CFT是一种不同于炎性肌纤维母细胞瘤的良性纤维母细胞性肿瘤。CFT不仅好发于儿童和青少年,也可发生于成年人。组织学上应与伴有钙化的纤维母细胞／肌纤维母细胞性病变相鉴别。     

Primary liposarcoma of the orbit: a clinicopathologic study of seven cases

Liposarcoma, the most common soft tissue sarcoma in adults, will rarely involve the orbit, either primarily or as a metastasis. We describe seven primary orbital liposarcomas, representing the largest documented series of primary orbital liposarcoma to date. Affected patients were three males and four females ranging in age from 28 to 69 years (median, 51 years). Five patients presented with painless proptosis, one patient had painful proptosis, and no details of presenting symptoms are available in one case. The site distribution was retrobulbar (3 cases), lateral orbital wall (2 cases), medial wall (1 case), and unspecified (1 case). Radiologic impression included hemangioma, lipoma, and an inflammatory process. Lesional size ranged from 2.8 to 4 cm. Five liposarcomas were purely well-differentiated, one was dedifferentiated, and one was pleomorphic in type. The well-differentiated cases comprised the following subtypes: spindle cell (2 cases), adipocytic (2 cases), and combined adipocytic/sclerosing/inflammatory (1 case). Five patients underwent orbital exenteration (one followed by radiation) and two had marginal/partial excision of their tumors. Follow-up was available for five patients, ranging from 13 to 204 months (median 65 months). Four patients showed no evidence of recurrence, including the patient with pleomorphic liposarcoma who had a long, disease-free survival (65 months) following marginal excision. One patient has had multiple recurrences following initial partial excision. One patient died of an unrelated cause with no clinical evidence of recurrence. Despite the difficulty in obtaining wide surgical margins, the small tumor size at presentation and the apparent predominance of the well-differentiated type means that the prognosis for orbital liposarcoma is generally good. In view of the various morphologic patterns that may occur, liposarcoma should be considered in the differential diagnosis of any histologically unusual mesenchymal lesion in the orbit.     

Fine-needle aspiration cytology findings in a case of aggressive angiomyxoma: A case report and review of the literature

Aggressive angiomyxomas are uncommon but distinct soft-tissue neoplasms occurring predominantly in the pelvis and peritoneum of females, but they have occasionally been reported in association with inguinal hernias in males. Histologically, these neoplasms are characterized by a proliferation of spindle- or stellate-shaped cells widely separated by loose myxoid stroma in which is dispersed a prominent vascular component. The vascular component is comprised of large, thick-walled vessels that generally do not show an arborizing pattern. Mitotic activity has been exceedingly low in the cases reported. Because of their occurrence within the groin, these lesions may undergo fine-needle aspiration (FNA). Cytologic examination of this material will reveal hypocellular smears containing scattered spindle cells with bipolar cytoplasmic processes, as well as bland stellate cells. The nuclei are fusiform to oval with a bland chromatin pattern. The stromal cells lie in a background of watery myxoid material. While specific diagnosis by FNA is not possible, the recognition of this cytologic appearance should exclude lymphoproliferative processes as well as metastatic disease from the differential diagnosis. Careful attention to cytologic detail should also help exclude certain other myxoid neoplasms, especially myxoid liposarcoma. Once the myxoid stromal nature of the proliferation is recognized, a differential diagnosis of myxoid lesions can be considered along with a recommendation for open biopsy to establish the definitive diagnosis. Diagn. Cytopathol. 16:425–429, 1997. © 1997 Wiley-Liss, Inc.     

Pleomorphic adenoma of the breast: A clinicopathologic and immunohistochemical study of 10 cases

Pleomorphic adenomas of the breast (PAB) are uncommon tumors. We studied the clinicopathologic features of 10 cases of PAB, seven of which were assessed immunohistochemically. Nine patients were women, with a median age at diagnosis of 65 years. Eight patients presented with a palpable mass; two had a nipple discharge. Nine of the tumors were periareolar. The latter epidemiologic findings, coupled with histologic observations, appeared to indicate a preferential origin for PAB within large intramammary ducts. These neoplasms histologically resemble their analogues in salivary glands. Moreover, a gradual histologic transition between different morphologic areas in PAB, especially between mesenchymal and epithelial regions, supported the contention that the neoplasm arises from a single cell type capable of divergent differentiation and thus should not be considered a "mixed" tumor. This contention was further substantiated by immunohistochemical findings, in which three intermediate filaments (cytokeratin, vimentin, and glial fibrillary acidic protein) and muscle-specific actin were expressed conjointly in tumor cells with a variety of morphologic appearances. In addition, cells differentiating along mesenchymal lines by conventional microscopy were found to express epithelial membrane antigen or gross cystic disease fluid protein-15 in five cases. The benign nature of PAB was supported by a lack of metastases in this series during a median follow-up period of 4.9 years. However, one lesion recurred locally. Regarding therapy, we believe that PAB can be excised successfully with only a narrow circumferential margin of uninvolved breast.     

Angiomyofibroblastoma of the vulva: a large pedunculated mass formation

Angiomyofibroblastoma is a rare, usually small benign mesenchymal tumor that occurs in vulvar lesions of premenopausal women. A case of angiomyofibroblastoma that arose as a unique pedunculated and particularly large mass in the left vulva of a 48-year-old woman is presented herein. The patient had been aware of a gradually enlarged mass of 7 years duration without any other gynecological symptoms or signs. The maximum dimension of the tumor measured 11 cm. The resected tumor was well circumscribed with a bulging and glistening cut surface. Histological examination revealed an admixture of irregularly distributed hypercellular and hypocellular areas with spindled, plump spindled, or plasmacytoid stromal cells and abundant venular or capillary-sized vessels. Stromal cells characteristically cluster around delicate vessels within an edematous to collagenous matrix. In the present case, intralesional adipose tissue was present throughout the tumor. There was no significant nuclear atypia, and mitotic figures were very sparse. There was little stromal mucin throughout the tumor. Immunohistochemically, the stromal cells were characterized by strong reactivity for vimentin and CD34, with focal reactivity for desmin and alpha smooth muscle actin. Both estrogen and progesterone receptors were diffusely expressed in the stromal cells. These histological findings are consistent with angiomyofibroblastoma and support the hypothesis that angiomyofibroblastoma originates from perivascular stem cells with a capacity for myofibroblastic and fatty differentiation.     

非典型性脑膜瘤的临床病理学

目的 探讨非典型性脑膜瘤(AM)的病理组织学特点和诊断指标。方法 光镜观察26例AM组织学改变、计数异型核、大核仁、核分裂和瘤巨细胞；图像分析胞核胞质面积比；免疫组化S-P法标记Ki-67单克隆抗体，求标记指数；统计患者生存时间、肿瘤复发和长期生存率。同时以35例良性脑膜瘤(BM)和13例恶性脑膜瘤(MM)作对照。结果 26例AM中梭形细胞为主10例，上皮样细胞为主14例，透明细胞型和囊性各1例，占脑膜瘤总数的16．45％。12例术后复发，2例复发后转为恶性，3例有脑组织微浸润。AM瘤细胞核质面积比为29．84％，每10个高倍视野大核仁数255．4、异型核27．03、瘤巨细胞5．41、核分裂象数4．30，Ki-67标记指数是10．15%，比BM高，较MM低，与两者相比差异都有显著性。标记指数和核分裂数呈正相关。AM复发率为46．15％，生存时间84．07个月，5年、10年生存率分别为53．61％和23．08％，介于MM和BM之间，与两型差异均有显著性。结论 AM是复发率高、潜在恶性的交界性肿瘤。瘤组织不形成明显的漩涡状脑膜瘤结构，瘤细胞周界不清呈合体状；瘤细胞密集排列，核质比例增大；核中度异型性和多形性；有明显的大核仁；核分裂象≥4个／10HPF；以上是诊断AM的必要指标。散在瘤巨细胞、局灶坏死、脑组织微浸润也是重要指标。     

Aggressive angiomyxoma: a second case of metastasis with patient's death

Aggressive angiomyxoma is a rare tumor that predominates in the female genital tract. Multiple relapses may occur in adjacent organs and tissues, but metastases have not been reported. We present a case of aggressive angiomyxoma in a young woman with multiple local recurrences that metastasized to the lungs, killing the patient. We document this case and report a similar one, found in the literature, of a postmenopausal woman with pulmonary and mediastinic metastases. These cases may expand the current concepts of potential behavior of aggressive angiomyxoma.     

Elastofibroma: a clinicopathologic and immunohistochemical study of seven cases and literature review

Elastofibroma is a rare fibrous lesion characterized by accumulated abnormal elastic fibers whose etiology remains largely unknown. In this study, we analyzed seven cases of elastofibroma to further explore the characteristics of its cellular composition. Immunohistochemistry was performed for mast cell tryptase, S-100 protein, vimentin, CD34, smooth muscle actin, desmin and collagen type IV. Histochemical staining methods for Gomori's trichrome and Verhoeff elastica-van Gieson were also evaluated. Histopathologically, a haphazard array of collagen, eosinophilic amorphous fibers, and globules in a fibrous tissue was seen. The elastic nature of the fibers was confirmed by elastic stain, and with Gomori's trichrome collagen fibers were also demonstrated. The interspersed spindle or stellate cells were almost consistently positive for vimentin and frequently positive for CD34. Mast cell tryptase-positive cells were present in five of the cases. Collagen type IV immunoreactivity was seen in two cases. No staining was observed with smooth muscle actin, desmin or S-100 protein. Our findings suggest that CD34-positive mesenchymal cells are an integral component of elastofibroma.     

Small cell carcinoma of the kidney: a clinicopathologic study of 14 cases

Small cell carcinoma of the kidney is distinctively rare. We searched pathology files in 2 institutions and found 14 cases of renal small cell carcinoma. The patients' mean age at diagnosis was 59 years (range, 22-75 years); 8 were women, and 6 were men. Patients usually presented with hematuria (n = 6) and abdominal pain (n = 5). The mean tumor size was 7.1 cm (range, 3.5-14.0 cm). The small cell carcinoma was pure in 9 cases and mixed with high-grade urothelial carcinoma in 5 cases. None was associated with any type of renal cell carcinoma. Tumor necrosis was present in all cases, and lymphovascular invasion was identified in 6 cases. The tumor invaded the perinephric adipose tissue in 13 cases and was confined to the kidney in only 1 case. Lymph node metastases were identified in all patients who underwent lymph node dissection (5/5). On immunostains, the small cell carcinoma cells were positive for pancytokeratin (11/12), chromogranin (6/9), and synaptophysin (8/9). Follow-up data were available for 13 patients, and 11 died of small cell carcinoma at a mean of 15 months (range, 4-31 months) after diagnosis. Of the 2 surviving patients, 1 was alive at 5 months after diagnosis, and the other, whose disease was confined to the kidney, was alive with no evidence of disease at 137 months. In summary, renal small cell carcinoma is a highly aggressive disease that often presents at an advanced stage with widespread metastases. Patients usually have a poor clinical outcome despite multimodal therapy. The frequent coexistence of small cell carcinoma with urothelial carcinoma suggests that renal small cell carcinomas may evolve from a preexisting urothelial carcinoma.     

Myxoid solitary fibrous tumor: a clinicopathologic study of three cases

While focal myxoid areas are occasionally observed in solitary fibrous tumors, neoplasms of this type exhibiting extensive myxoid change are considered exceedingly uncommon. Due to their rarity, the biologic behavior of myxoid solitary fibrous tumor has not been determined. Three cases of myxoid solitary fibrous tumor are described in order to better characterize the clinical and pathologic features of this uncommon variant of solitary fibrous tumor. The tumors occurred in one man and two women, with ages of 37, 47, and 58 years, respectively. Sites of involvement included the retroperitoneum, pelvis, and soft tissue of the neck. Histologically, all cases were characterized predominantly by the presence of myxoid stroma comprising 70% to 100% of the tumor. The tumor cells were predominantly spindled in all cases, and arranged randomly, in loose fascicles, or in anastomosing strands imparting a microcystic/reticular appearance. The lesional cells had a bland cytologic appearance and low mitotic count. All tumors lacked necrosis and areas of increased cellularity. By immunohistochemistry, all cases were positive for CD34, CD99, and bcl-2, and negative for keratin, epithelial membrane antigen, desmin, actin, smooth muscle actin, and S-100 protein. To date, all cases have followed a benign course without evidence of recurrence or metastasis with a follow-up duration ranging from 50 to 87 months. The data suggest that myxoid solitary fibrous tumors are associated with an indolent clinical course and favorable prognosis.     

Composite ganglioglioma/dysembryoplastic neuroepithelial tumor: a clinicopathologic study of 8 cases

Ganglioglioma and dysembryoplastic neuroepithelial tumor are both low-grade glioneuronal neoplasms that most commonly occur in association with chronic epilepsy. Rare cases of tumors with composite features of ganglioglioma and dysembryoplastic neuroepithelial tumor have been reported. We retrospectively reviewed the clinicopathologic features of 8 composite tumors (7 were female; median age, 20 years). All patients had chronic epilepsy and had tumors in either the temporal or the frontal lobe. Six patients are currently seizure-free (follow-up: median, 90 months). All tumors were multinodular. Some nodules had distinct features of each tumor type (range, 5%-85% of the tumor). Seven tumors contained nodules with mixed features of both tumor types. Five of 7 evaluable tumors demonstrated adjacent focal cortical dysplasia (Palmini type I). Mitotic activity, vascular proliferation, or necrosis was not observed in any of the tumors. Three tumors demonstrated focal meningeal extension. Composite tumors commonly arise in the temporal lobe in young patients with chronic epilepsy; they appear to behave as a World Health Organization grade I neoplasm. Histologically, these multinodular tumors appear to maintain distinct areas with features of each tumor and foci where the 2 patterns are merged. A subset of composite tumors are associated with adjacent focal cortical dysplasia.