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.     

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.     

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.     

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     

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.     

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     

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.     

Cytokeratin immunolocalization and lectin binding studies in oesophageal squamous dysplasia

Aggressive angiomyxoma is a distinctive soft tissue tumour associated with a high risk of local recurrence but lacks metastatic potential. This tumour occurs nearly exclusively in the soft tissues of the pelvis and perineum of adult women. The line of differentiation is not firmly established, but a fibroblastic/myofibroblastic origin has been proposed. We report 16 new cases of aggressive angiomyxoma of the pelvic soft tissue in women. In all cases bundles of cells, most often adjacent to vessels, with histological features of smooth muscle cells were identified. In 11 of 14 cases the myoid bundles were immunoreactive for desmin; they were also postive for smooth muscle actin in 10 of 11 cases. In 13 of 14 cases lesional stromal cells showed immunoreactivity for desmin. Three cases showed areas with histological features similar to those of angiomyofibroblastoma of the vulva, thus representing previously undescribed morphological overlap between these two entities. We conclude that aggressive angiomyxoma and angiomyofibroblastoma are related neoplasms in a spectrum of tumours showing myofibroblastic origin. Furthermore, the demonstration of immunoreactivity for desmin in aggressive angiomyxomas implies that this antibody is not helpful in discriminating between these two tumours, and the principal means of distinction remains histomorphological analysis.     

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.     

Vulvovaginal soft tissue tumours: update and review

Differences in biological behaviour make familiarity with (and accurate diagnosis of) vulvovaginal soft tissue tumours essential. Since ancillary studies such as immunoperoxidase staining and electron microscopy may not always be helpful in their distinction, one must often rely on morphological features to distinguish between different tumour types. This is particularly pertinent with regard to the relatively site-specific stromal tumours of this region. The purpose of this review article is to reacquaint the reader with these specific types of vulvovaginal soft tissue tumour, particularly focusing upon the salient morphological features that help in their distinction, as well as to review their clinical aspects and pathogenesis. The following soft tissue lesions are described: fibroepithelial stromal polyp, cellular angiofibroma, angiomyofibroblastoma, superficial angiomyxoma and aggressive angiomyxoma. Because of continued difficulty in predicting their behaviour, a discussion of vulvar smooth muscle tumours is also included, with a particular focus upon a practical approach to their diagnosis.     

Myxoid leiomyosarcoma of the uterus

Burch D M & Tavassoli F A
(2011) Histopathology  59 , 1144–1155
Myxoid leiomyosarcoma of the uterus Aims: Recent guidelines have suggested the presence of tumour cell necrosis (TCN), atypia and mitotic index as major features in the distinction of myxoid leiomyosarcomas (MLMSs) from myxoid leiomyomas. The aim of this study was to focus on an invasive growth pattern as a significant feature in this distinction in the absence of TCN. Methods and results: Twelve uterine smooth muscle tumours with myxoid change in ≥60% of the lesion were interpreted as MLMS on the basis of the presence of focal mild atypia as well as one or more of the following features: (i) infiltrative growth pattern; (ii) vascular invasion; (iii) mitotic index of ≥5 mitotic figures (m.f.)/50 high‐power fields (HPFs); and (iv) a combination of at least focal severe atypia and at least 2–4 m.f./50 HPFs. Unequivocal TCN was not evident in any of these tumours. The various morphological features were correlated with outcome. With follow‐ups ranging from 19 to 113 months (mean 60 months), five of the 12 women developed recurrences, and two of them died. Nine of the 12 tumours had an infiltrative growth pattern, and all five recurrent tumours were from this group. Conclusion: In the absence of TCN, an infiltrative margin is a major factor related to the potential for aggressive behaviour of MLMS.     

Epithelioid leiomyoma of the larynx

We describe the pathological features of a case of laryngeal epithelioid leiomyoma (leiomyoblastoma) which, to our knowledge, is the second case to be reported in the world literature. A review of the literature confirmed that leiomyoma as such is a very rare neoplasm in the larynx, and only 33 cases have been previously reported. The neoplasm was located in the left vocal cord and consisted of mainly epithelioid, round or spindle-shaped cells, often with clear cytoplasm, which were arranged predominantly in solid nests and sheets. The tumour cells showed positive immunoreactivity for smooth muscle actin and desmin. The tumour showed low mitotic activity and immunostaining with MIB 1 (Ki-67) accordingly revealed the occasional cell only to be positive, confirming a low proliferative activity in agreement with a benign neoplasm. Epithelioid leiomyomas located in other sites have been shown to act occasionally in a malignant fashion, and the necessity for careful long-term follow-up of the patient is therefore emphasized.     

Maxillary aggressive angiomyxoma showing ineffective to radiotherapy: a rare case report and review of literature

Aggressive angiomyxoma, mostly originating in the female pelvis and peritoneum or in the male analogous sites, is a rare mesenchymal neoplasm characterized with infiltrative growth to adjacent tissue and local recurrence after primary excision. Herein, we report a case of aggressive angiomyxoma of maxilla in a 60-year-old male patient for its rarity. The patient presented with a one-year history of progressively enlarging maxillary mass on left side. Before referred to our hospital, he was given a biopsy and diagnosed as aggressive angiomyxoma by immunohistochemical staining. After that, he underwent 60Gy radiotherapy. Unfortunately, CT scan showed bigger mass infiltrated to adjacent facial soft tissues and bones compared with that of before radiotherapy. Besides that, he began to suffer with ingravescent headache. The mass was surgically removed and the diagnosis was confirmed by immunohistology in our hospital. As a case of aggressive angiomyxoma occurred in a rare site and experienced an ongoing growth in spite of radiotherapy, its characteristics was discussed with a brief literature review, which may aid further understanding of aggressive angiomyoma.     

Myxoid Liposarcoma: Study of Ten Cases

The ultrastructure of 10 myxoid liposarcomas from 9 patients is presented. Features closely resembling those of developing fetal adipose tissue were seen in all tumors. The various cell types identified ranged from undifferentiated cells to typical multivacuolated and univacuolated lipoblasts. In addition, a secretory-type mesenchymal cell with distended cisternae of rough endoplasmic reticulum was found in every case as well as intermediate cells with secretory product and lipid accumulation. Relative proportions of these types varied from case to case.

A prominent feature in all cases was the particular spatial relationship of the abundant capillaries to the various cell types. The undifferentiated cells had perivascular processes with a tendency to envelop the primitive capillaries. These cells progressively enlarged and acquired features of the differentiated cell types as they separated from the lamina. Mitotic activity, when detectable, was prominent in the capillary wall and its vicinity. Lipoblasts resembling those of brown adipose tissue frequently coexisted in the same tumor with others analogous to those of white adipose tissue.

Our findings suggest that the tumor cells in lipo-sarcoma originate from a dividing population of perivascular mesenchymal cells, in a manner akin to that of developing white adipose tissue.     

Myxoid variant of primary cutaneous malignant melanoma

We report a rare case of primary cutaneous myxoid melanoma. Histologically, the tumour was composed of spindle and stellate-shaped cells, embedded in a myxoid stroma. Positivity for S-100 protein and the presence of melanosomes were demonstrated in the tumour. Primary cutaneous myxoid melanoma is rare. This is the second report of such a case.     

浅表性血管黏液瘤3例临床病理分析

目的探讨浅表性血管黏液瘤(superficial angiomyxoma,SA)的临床病理特征、免疫组化及鉴别诊断要点。方法对3例SA进行临床病理学及免疫组化染色观察,结合文献对该肿瘤的临床表现、病理形态学特征以及鉴别诊断要点进行讨论。结果 SA主要位于皮肤真皮及皮下,镜下肿瘤略呈分叶状,黏液性基质中可见散在无异型的梭形和星形瘤细胞及较多的中、小薄壁血管,散在有多少不等的中性粒细胞浸润。免疫组化染色显示肿瘤细胞表达vimentin。结论 SA是罕见的黏液性肿瘤,位于皮肤表浅部位,可局部复发但不转移,诊断时应注意和其它黏液性软组织肿瘤鉴别。     

Myxoid Liposarcoma: Study of Ten Cases

The ultrastructure of 10 myxoid liposarcomas from 9 patients is presented. Features closely resembling those of developing fetal adipose tissue were seen in all tumors. The various cell types identified ranged from undifferentiated cells to typical multivacuolated and univacuolated lipoblasts. In addition, a secretory-type mesenchymal cell with distended cisternae of rough endoplasmic reticulum was found in every case as well as intermediate cells with secretory product and lipid accumulation. Relative proportions of these types varied from case to case.

A prominent feature in all cases was the particular spatial relationship of the abundant capillaries to the various cell types. The undifferentiated cells had perivascular processes with a tendency to envelop the primitive capillaries. These cells progressively enlarged and acquired features of the differentiated cell types as they separated from the lamina. Mitotic activity, when detectable, was prominent in the capillary wall and its vicinity. Lipoblasts resembling those of brown adipose tissue frequently coexisted in the same tumor with others analogous to those of white adipose tissue.

Our findings suggest that the tumor cells in lipo-sarcoma originate from a dividing population of perivascular mesenchymal cells, in a manner akin to that of developing white adipose tissue.     

Myxoid change within cellular blue naevi: a diagnostic pitfall

We describe four cases of a distinctive myxoid change located in the centre of cellular blue naevi. Because all the reported lesions were large and were present on pressure-exposed locations, the myxoid change is thought to be caused by pressure together with direct repeated minor tissue trauma.