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。     

Cellular angiofibroma. A rare vulvar tumor. Report of a case

Cellular angiofibroma is a rare tumor. We report a vulvar case in a 37 year old woman. This nodular, well circonscribed tumor consists of bland spindle cells, numerous thin or thick often hyalinized vessels and adipocytes. The stromal cells are positive for vimentin and negative for CD34, protein S100, smooth muscle actin, desmin, epithelial membrane antigen and cytokeratin. Cellular angiofibroma is a benign tumor that has to be differentiated from aggressive angiomyxoma, angiomyofibroblastoma, glomangiopericytoma, spindle cell lipoma, solitary fibrous tumor and perineurioma.     

Angiomyofibroblastoma of the male inguinal region

We present a case of benign angiomyxoid tumor arising in the inguinal region of a 27-year-old man. The tumor was a gelatinous mass completely encapsulated by a thin fibrous capsule with no hemorrhage or necrosis. Histologically, a proliferation of spindle cells as well as occasional pleomorphic cells was observed within the myxofibrous stroma, intermingled with abundant capillary-sized blood vessels. Immunohistochemical staining of the tumor demonstrated spindle, oval, and pleomorphic cells equally positive for vimentin, desmin, and CD34, but not for alpha-smooth muscle actin. Based on these histologic and immunophenotypic features, we conclude that this angiomyxoid tumor of the male inguinal region is indistinguishable from the female angiomyofibroblastoma of the pelvic and perineal regions.     

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     

A subcutaneous pleomorphic hyalinizing angiectatic tumor of soft parts of the right chest wall: report of a rare case

Pleomorphic hyalinizing angiectatic tumor of the soft parts is an extremely rare mesenchymal tumor consisting of spindled and pleomorphic tumor cells and clusters of ectatic, fibrin-lined vessels. It typically occurs in the subcutaneous tissues of the distal extremities, usually the ankles and feet. Here we present a case of pleomorphic hyalinizing angiectatic tumor of the soft parts of the right chest wall in a 51-year old female. The tumor was subcutaneous, nonencapsulated, and about 2.0 cm×1.0 cm. Microscopically, the tumor was composed of numerous ectatic, fibrin-filled, thin-walled blood vessels, surrounded by spindled or pleomorphic tumor cells arranged in sheet-like or fascicular architecture, or randomly. Mitotic activity of the tumor cells was low. Immunohistochemical analysis shows that the tumor cells were positive for CD34 and vimentin, but negative for CD31, CK, desmin, EMA, HMB45, Myo D1, P63 and S-100. Ki67 index was about 1%.     

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与侵袭性血管粘液瘤临床病理分析

目的：探讨血管肌纤维母细胞瘤（ＡＭＦＢ）的临床病理特点及与侵袭性血管粘液瘤（ＡＡ）的鉴别。方法：对５例ＡＭＦＢ和５例ＡＡ进行临床病理和免疫组化研究,对３例ＡＭＦＢ进行电镜观察。结果：ＡＭＦＢ位于外阴或腹股沟我,肿瘤边界清楚,大小０．８～４ｃｍ。光镜：肿瘤细胞呈梭形上皮样、束头及巢状排列,常围绕小至中等大小的薄壁血管周围。肿瘤有细胞密集区和细胞分散区。免疫组化：肿瘤细胞表达ｖｉｍｅｎｔｉｎ,ｄｅｓｍ     

Polypoid angiomyofibroblastoma-like tumor of the oral cavity: a hitherto unreported soft tissue tumor mimicking embryonal rhabdomyosarcoma

We report on a previously unrecognized fibro-myofibroblastic tumor in the oral cavity of a 15-year-old girl. Morphologically, the tumor mimicked a rhabdomyosarcoma, botryoid variant. It was composed of mitotically active small- to medium-sized, vimentin+/desmin+, round- to oval- to epithelioid-shaped cells embedded in an alternating fibrous to myxoid/edematous stroma. These cells were separated from the overlying squamous epithelium by a rim of fibrous stroma. The tumor contained abundant small- to medium-sized, thin-walled blood vessels without hyalinization. Frequently, neoplastic cells condensed around these vessels. An unusual and striking feature was the presence of numerous hyalinized collagen mats, including "amianthoid-like fibers", similar to those observed in myofibroblastomas. The presence of these collagen mats and the expression of desmin, in association with no immunoreactivity to myogenin and MyoD1, were in keeping with the fibro-myofibroblastic nature of the tumor, excluding the diagnosis of embryonal rhabdomyosarcoma. Regarding fibro-myofibroblastic tumors, we believe that the present case falls within the wide spectrum of benign stromal tumors, originally described in the lower female genital tract, but potentially occurring also at extragenital sites. As morphological and immunohistochemical features were reminiscent of, but not identical with, angiomyofibroblastoma, the term "polypoid angiomyofibroblastoma-like tumor" is proposed. Awareness and recognition of this tumor is crucial to avoid a diagnosis of malignancy.     

Mammary myofibroblastoma with extensive myxoedematous stromal changes: A potential diagnostic pitfall

Myofibroblastoma (MFB) of the breast is a relatively rare, benign stromal tumor arising in the breast of both males and females. Several morphological variants have been recognized in the last two decades, including infiltrating, cellular, fibrous/collagenized, epithelioid/deciduoid-like cell, lipomatous and myxoid variants. Myxoid MFB is an extremely rare variant which can represent a potential diagnostic pitfall, posing differential diagnostic problems with other myxoid lesions occurring primarily in the breast parenchyma. We first report an unusual case of mammary MFB showing so extensive myxoedematous stromal changes that obscured the underlying neoplastic cells, rendering difficult its recognition as MFB. Tumor was composed of a few bland-looking spindled, stellate cells embedded in an abundant myxoedematous stroma containing thin and thick collagen fibers and numerous blood vessels with fibrin and foamy histiocytes deposits in their walls. Identification of a small-sized cellular area, with morphological and immunohistochemical features consistent with mammary-type MFB, was crucial for a correct diagnostic interpretation of the lesion. These unusual stromal and vascular features are likely morphological changes to which undergo long-standing mammary MFBs in response to ischemic, traumatic or inflammatory stimuli. Differential diagnostic problems with benign and malignant myxoid lesions of the breast parenchyma are discussed. The present case, for which the term “mammary MFB with extensive myxoedematous stromal changes” is proposed, contributes to widen the morphological spectrum of this unusual mammary tumor, suggesting that when pathologist is dealing with a hypocellular myxoedematous lesion of the breast, a MFB should be ruled out.     

Myxoid endometrial stromal sarcoma of the uterus

A rare case of a myxoid type of endometrial stromal sarcoma of the uterus in a 41-year-old woman is reported. A tumor was found in the myometrium and was well circumscribed, measuring 9 x 7 x 7 cm in size. The tumor was mainly composed of a hypocellular area with tumor cells separated by prominent myxoid stroma. The tumor cells were spindle-shaped and resembled endometrial stromal cells. Numerous small thin-walled vessels were seen throughout the tumor. Immunohistochemically, the tumor cells were diffusely stained for estrogen and progesterone receptors and CD10, and focally and weakly for HHF35, alpha-smooth muscle actin and desmin, but not stained for h-caldesmon. These results indicated that the tumor originated from endometrial stromal cells. The tumor had an increased mitotic activity (MIB-1 labeling index: 1-10%), and focally showed nuclear pleomorphism. Thus, this tumor had a malignant potential and was diagnosed as a myxoid type of low-grade endometrial stromal sarcoma. The patient is currently well with no evidence of local recurrence or metastasis 21 months after the operation. This case indicates a wide morphological spectrum of endometrial stromal tumor. A myxoid endometrial stromal sarcoma should be considered in the different diagnosis of the intramural myxoid tumors in the uterus.     

Gastrointestinal stromal tumor presenting as a recurrent vaginal mass

Gastrointestinal stromal tumors are CD117 (c-Kit)-positive mesenchymal neoplasms with histologic and ultrastructural features of the interstitial cell of Cajal. While tumors outside of the gastrointestinal tract have been described, to our knowledge the case we present is the first such case in the vagina. We describe a 75-year-old woman with a recurrent vaginal gastrointestinal stromal tumor without apparent rectal involvement. This tumor was characterized by short intersecting fascicles of spindled cells, focal necrosis, and 12 to 15 mitoses per 50 high-power fields. Immunohistochemistry revealed diffuse cytoplasmic positivity for CD117 (c-Kit), CD34, vimentin, and h-caldesmon. Tumor cells were negative for S100, desmin, actin, and CAM 5.2. The differential diagnosis in this case included a vaginal smooth muscle tumor. While histologically similar to a smooth muscle neoplasm, the immunohistochemical profile ruled out smooth muscle differentiation. Gastrointestinal stromal tumor should be considered in the differential diagnosis of vaginal mesenchymal neoplasms.     

Angiomyofibroblastoma of the Male Perineum: An Unusual Location for a Rare Lesion

We report a case of angiomyofibroblastoma (AMF)-like tumor of the perineum in a 54-year-old man. The asymptomatic lesion measured 3 cm and appeared as a tan homogeneous mass. Histologically, it appeared as a circumscribed nodular spindle cell proliferation with alternating cellular and hypocellular areas. The spindle cells exhibited minimal nuclear pleomorphism and scanty mitotic activity. Focally, some cells were epithelioid. Large blood vessels were present, with perivascular fibrosis. The spindle cells expressed vimentin, but not desmin, actin, S100, or CD34. These features were similar to those of AMF as initially reported in the vulva. A perineal localion of this lesion in a male has not been reported in the literature, to the best of our knowledge. Int J Surg Pathol 8(1):79-82, 2000     

Angiomyofibroblastoma of the vulva: A clinicopathologic study of seven cases

A clinicopathologic and immunohistochemical review was made of seven cases of angiomyofibroblastoma. The patients were middle-aged women who had a slowly growing mass, measuring 1.5–6 cm in maximum dimension, located sub-cutaneously in the vulva. The tumors were well-demarcated and characterized by well-vascularized, alternating hyper-cellular and hypocellular edematous areas composed of bland, plump spindle- or oval-shaped stromal cells frequently aggregated around small blood vessels. An epithelioid appearance of the stromal cells was seen in two cases. Immunohistochemically, the stromal cells were consistently positive for vimentin and desmin, but negative for muscle specific actin, a-smooth muscle actin, myosin, cytokeratins, S-100 protein or von Willebrand factor. Ultrastructurally, the plump stromal cells had a small amount of peripherally located rough endoplasmic reticulum, numerous pinocytotic vesicles and abundant intermediate filaments, on which immunogold probes for desmin were localized, whereas fine filaments were few and there were no electron dense plaques. Thus, while the proliferating stromal cells expressed an immunohistochemical profile of peculiar myoid differentiation, ultrastructural findings differed from those of smooth muscle cells or those seen in typical myofibroblasts. At 1–4 years after surgery, there was no evidence of recurrence.     

Fine‐needle aspiration of brown tumor of bone: Cytologic features with radiologic and histologic correlation

We report the case of a 40‐year‐old man with tertiary hyperparathyroidism due to end stage renal disease who initially presented with acute‐onset paraplegia, elevated serum parathyroid hormone, and multiple bone abnormalities, including a large extradural intraspinal mass seen by magnetic resonance imaging. In contrast with imaging features, fine‐needle aspiration cytology showed numerous benign‐appearing multinucleated osteoclast‐type giant cells that are the characteristics of either brown tumor or benign giant cell tumor of bone. Sheets of mononuclear spindled stromal cells were also noted. A core‐needle biopsy confirmed the diagnostic features of brown tumor of hyperparathyroidism. Diagn. Cytopathol. 2009. © 2008 Wiley‐Liss, Inc.     

血管肌纤维母细胞瘤临床病理特征

目的：探讨血管肌纤维母细胞瘤的临床病理形态特征及鉴别诊断。方法：对2例血管肌纤维母细胞进行组织病理学、免疫组织化学研究,结合文献资料分析本病的临床表现、病理形态特点及鉴别诊断。结果：血管肌纤维母细胞瘤呈大片黏液背景、丰富薄壁海绵样血管和梭形上皮样细胞即肌纤维母细胞,后者胞质丰富,呈嗜酸性,核卵圆或杆状,两端对称变细或稍钝,瘤细胞分布有明显的疏密区,密集区聚于血管周围,成束或链状排列、疏松区弥散于黏液背景中,且瘤细胞常与胶原纤维伴行。结论：血管肌纤维母细胞瘤为良性肿瘤,好发于女性外生殖器,起源与肌纤维母细胞相关,既向纤维,又向平滑肌分化,且伴有丰富的海绵样薄壁血压和间质黏液变性。     

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.     

Secondary amyloidosis and gastrointestinal stromal tumors. A case report and discussion of pathogenesis

A 69-year-old man presented with a malignant gastrointestinal stromal tumor associated with secondary amyloidosis. The tumor had classic features of a malignant gastrointestinal stromal tumor with interlacing fascicles and whorls of spindled cells, numerous and conspicuous mitotic figures, and extensive coagulative necrosis. The cells stained diffusely for CD117 (c-Kit), confirming the diagnosis of gastrointestinal stromal tumor. The spleen, 1 adrenal gland, and part of the pancreas were removed en block with the stomach. By microscopy, the spleen and adrenal gland were partially replaced with amyloid deposits confirmed by Congo red staining, electron microscopy, and immunohistochemistry. In contrast, neither the tumor nor the surrounding vasculature showed amyloid deposition. To our knowledge, this represents only the second case of systemic amyloidosis associated with a gastrointestinal stromal tumor. This case is unique in that extensive, diffuse amyloid deposits were observed in the spleen, adrenal gland, and liver.     

Pleomorphic hyalinizing angiectatic tumor with a sarcomatous component recurring as high-grade myxofibrosarcoma

Pleomorphic hyalinizing angiectatic tumor (PHAT) of the soft tissue is a rare distinctive tumor listed as a benign neoplasm in the new World Health Organization classification. It may recur and most reported recurrent tumors retained the typical morphological appearance of PHAT; rare tumors recurred with the appearance of a sarcoma. Reported herein is an additional example of recurrent PHAT, but in contrast to the previously described cases the present tumor morphologically qualified as a sarcoma from the very beginning; it recurred as a high-grade myxofibrosarcoma. A 76-year-old woman presented with a solitary subcutaneous tumor in the axilla that was surgically removed. Seven months later, the patient experienced a local recurrence. Microscopically, the typical features of PHAT were identified in the initial lesion, namely hyalinized, fibrin-containing vessels and pleomorphic stromal cells; there were areas of hemorrhage and necrosis. Additionally, peripherally located areas of the tumor manifested highly pleomorphic cells with frequent atypical mitoses, producing a sarcomatous appearance. The mitotic index in the sarcomatous part was 1/10 high-power fields (HPF). Hyalinized, fibrin-containing vessels were absent in these sarcomatous areas, and the stroma was myxoid. The recurrent lesion was composed of large highly pleomorphic oval, round, spindled or bizarre cells with a high mitotic rate, ranging from 3/10 HPF to 7/10 HPF. The neoplastic cells were arranged haphazardly in a myxoid matrix. Hyalinized, fibrin-containing vessels typical for PHAT were absent. PHAT may be more aggressive than previously thought, and PHAT may encompass a morphological spectrum of the lesion ranging from benign to malignant.     

Hepatic angiomyolipoma resembling an inflammatory pseudotumor of the liver. A case report

Hepatic angiomyolipoma (AML) may demonstrate a marked histologic diversity and is frequently misdiagnosed. HMB45 is a promising marker for this tumor and is expected to facilitate the recognition of some AMLs with unusual morphology. We report on a case of hepatic AML exhibiting histologic features that were similar to inflammatory pseudotumor (IPT) or to IPT-like follicular dendritic cell (FDC) tumor of the liver. The patient was a 21-year-old Japanese woman with a mass in the left lobe of the liver (70 x 73 mm). There were no clinical features of tuberous sclerosis. Histologically, numerous inflammatory cells, including small lymphocytes, plasma cells, and histiocytes, showed diffuse infiltration throughout the lesion. However, the present case also shared some of the morphologic findings of hepatic AML, including clusters of smooth muscle cells with clear cytoplasm, a few scattered adipose cells, and thick-walled blood vessels. Moreover, the smooth muscle cells consisted of spindle-shaped cells or larger, more rounded cells with either clear cytoplasm or eosinophilic epithelioid cell features positive for vimentin, muscle-specific actin, and smooth muscle actin. HMB45 immunostaining confirmed the diagnosis of AML. The present case indicates that IPT or IPT-like FDC tumor should be added to the list of differential diagnoses for AML of the liver.     

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.