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.     

Vulvar hypertrophy with lymphedema. A mimicker of aggressive angiomyxoma

We report the case of a 43-year-old quadriplegic woman with bilateral vulvar enlargement. The clinical impression was labial hypertrophy, but the microscopic features mimicked aggressive angiomyxoma because of the location, hypocellular proliferation of fibroblastic cells in an edematous-myxoid stroma, and vessels with perivascular collagen deposition, which simulated the thick-walled vessels of aggressive angiomyxoma. Since the lesion lacked true thick-walled vessels and contained ectatic tortuous lymphatics, the pathologic interpretation was lymphedema. This vulvar lesion should be recognized to prevent the misdiagnosis of aggressive angiomyxoma.     

Recurrent aggressive angiomyxoma of vagina--a case report

Aggressive angiomyxoma is a rare tumour, which presents as a painless expanding mass in the vulvo-vaginal region. It usually occurs in 2nd to 3rd decades of life. It behaves like a low-grade sarcoma with high propensity for local spread and recurrence and can involve vulva, perineum, vagina and urinary bladder. It is difficult to differentiate clinically this tumour from other mesenchymal tumours occurring in this region. Microscopically it must be differentiated from malignant tumours with myxoid change, like liposarcoma and myxoid leiomyosarcoma. Hence, histopathologic examination has a central role in diagnosis of this tumour. We are presenting a case of young woman, who came with history of swelling in vulva and perineum. Imaging studies in the perineal region revealed a large pelvic mass. Fine needle aspiration cytology was inconclusive due to scanty material. Enucleation of mass was attempted in first surgery but complete extirpation could not be performed. The swelling recurred within few weeks after surgery and required irradiation. A second surgery, however, was successful in complete removal of the tumour.     

Multifocal aggressive angiomyxoma: a case report

A case of aggressive angiomyxoma in a 25 year old woman is presented. The patient was admitted to hospital with a history of hesitancy of micturation and pain in the right iliac fossa. She was found to have a left labial mass, which was clinically diagnosed to be a Bartholin gland cyst. A pelvic ultrasound revealed an additional mass in the right paravesical region. At surgery, two distinct masses were removed, one from the right perivesical space and the other from the left labium. Both masses were rubbery, white, and gelatinous and showed similar histopathology findings of thick and thin walled vascular channels set in a loose myxoid stroma. A diagnosis of multifocal aggressive angiomyxoma was made. This is the first reported case of aggressive angiomyxoma occurring as two distinct masses in one patient.     

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.     

Angiomyxoid tumour in the renal peripelvic tissues with features of aggressive angiomyxoma.

A case of angiomyxoid tumour in the renal peripelvic soft tissue of a 45 year old woman is reported. The encapsulated tumour, measuring 12 x 9 x 6 cm, was solid and firm. The cut surface revealed a yellowish-white, gelatinous, and glistening tumour. Histologically, the tumour was composed of loosely textured spindle and stellate cells with prominent blood vessels of variable calibre. The tumour cells stained strongly for vimentin. These findings strongly resemble those associated with aggressive angiomyxoma in the vulva and perineum.     

Angiomyofibroblastoma and aggressive angiomyxoma: two benign mesenchymal neoplasms of the female genital tract. An immunohistochemical study

We describe a rare case of angiomyofibroblastoma (AMF) of the vulva and one case of aggressive angiomyxoma (AAM) of the pelvic region and, with the help of an extensive revision of the literature, we attempt to define their histogenesis and peculiar biological behaviour by an immunohistological evaluation. Our results indicate that AAM, which is characterized by the presence of a high content of glycosaminoglycans in the stroma, expresses uniformly vimentin and hyaluronate receptor CD44, and heterogeneously muscle specific actin (MSA) and desmin, while AMF displays a positive reaction for vimentin, desmin and laminin, and only a weak and heterogeneous positivity for CD44. Both AMF and AAM showed no immunohistochemical reactivity for alpha-smooth muscle actin (ASMA), myoglobin, cytokeratin, collagen type IV, CD68 and S-100. The stromal cells of AAM were negative for laminin. These findings support the suggestion of an origin of the two entities by a common myofibroblastic progenitor, which normally occurs in the lower female genital tract and subsequently undergoes a neoplastic transformation. The expression of CD44 by AAM, which has never been reported before, could be responsible for its more aggressive behaviour, because this receptor is able to mediate migration of neoplastic cells on a hyaluronate rich extracellular matrix. It is speculated that the neoplastic cell of the AAM and AMF of the vulva is a specific myofibroblast which probably arises from undifferentiated mesenchymal cells normally occurring in the lower female genital tract.     

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.     

Aggressive angiomyxoma: an ultrastructural study of four cases

The histogenesis of the aggressive angiomyxoma of the vulvo-vaginal region was studied. Four cases of aggressive angiomyxoma were examined by using light microscopy, electron microscopy, and immunohistochemistry. Myofibroblastic from smooth muscle cell differentiation was distinguished by paying close attention to the structures of the neoplastic cell membrane. Aggressive angiomyxomas exhibit subtle features of smooth muscle differentiation, suggesting that the neoplastic cells differentiate from a multipotent perivascular cell.     

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.     

Superficial angiomyxoma: a c linicopathologic analysis of three cases

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.     

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。     

Cytogenic investigations in the assessment of response to treatment in neuroblastoma.

In a patient with stage IV disseminated neuroblastoma treated by chemotherapy extensive cytogenetic investigations were performed on the residual primary tumour and bone marrow immediately before myeloablative treatment and autologous marrow rescue. Two abnormal clones both showing lp+, a characteristic abnormality of neuroblastoma, were detected in cells from the residual primary tumour, providing direct evidence of persisting viable tumour. Such investigations should be a routine part of the assessment of response to treatment in patients with neuroblastoma, and could be extended to others in whom "second look" surgery is performed.     

Urothelial carcinoma following augmentation cystoplasty: an aggressive variant with distinct clinicopathological characteristics and molecular genetic alterations

Aims:  Patients who have undergone intestinal augmentation cystoplasty are at risk for developing latent vesicle malignancy. The aim was to evaluate the histological and immunohistochemical characteristics and molecular genetic alterations in these neoplasms.
Methods and results:  Four patients developing urothelial neoplasms after augmentation cystoplasty were included in the current study. The mean age of the patients, including two men and two women, was 37 years. The latency from bladder augmentation to developing malignancy ranged from 17 to 21 years (mean 19 years). All patients died of cancer shortly after diagnosis (mean 5 months). In the morphological evaluation, all tumours were high-grade (grade 3) invasive urothelial carcinoma comprising various architectural patterns with brisk mitoses and tumour necrosis. Three harboured glandular differentiation and the remaining one showed squamous differentiation. All cases revealed abnormal decreasing β-catenin expression. Two tumours showed nuclear expression of CDX2. On UroVysion fluorescence in situ hybridization (FISH) analysis, all tumours displayed characteristic chromosomal abnormalities. Point mutations of both FGFR3 and p53 genes were identified in one case.
Conclusions:  Urothelial carcinomas developed after augmentation cystoplasty are extremely aggressive and exhibit distinct morphological, immunohistochemical and genetic characteristics. UroVysion FISH analysis may offer a surveillance strategy in patients who undergo augmentation cystoplasty.     

Aggressive angiomyxoma of the vulva. Report of a case

A case of aggressive angiomyxoma of the vulva is reported. A 43-year-old woman presented initially in 1987 with a left vulvar mass which clinically was thought to be a lipoma or a Bartholin gland cyst. Local excision was performed after a 27-month follow-up without any change in size. The resected tumor measured 2.7 x 2.0 x 2.0 cm and had a smooth, glistening and myxoid cut surface. Histologically, the lesion was composed of spindle-shaped or stellate neoplastic cells, which were loosely textured in the fibromyxoid matrix, and a prominent vascular component characterized by randomly distributed vascular channels of variable caliber. Immunohistochemically, the tumor cells were positive for vimentin, but not for desmin and myosin. The fine structure of the neoplastic cells was compatible with that of fibroblasts rather than myofibroblasts. These findings suggest the fibroblastic differentiation of this tumor. The patient is currently well with no evidence of recurrence, 7 months after excision of the tumor.     

Aggressive angiomyxoma of the lung

Aggressive angiomyxoma (AAM) is a rare mesenchymal neoplasm that usually occurs in the pelvic-perineal region. Only two cases of AAM occurring outside this region have been reported. The case of AAM reported here originated from lung. A 70-year-old woman was admitted for evaluation of an incidentally detected pulmonary mass on chest radiography. Tumour resection under the thoracoscopy was performed. Pathological examination revealed microscopical features that were characteristic of AAM. The features were oval- to spindle-shaped tumour cells in a myxoid stroma, hyalinised thick vessels, and characteristic immunophenotype. The differential diagnosis of AAM and other mesenchymal neoplasms of lung is also discussed.     

Linkage disequilibrium analysis of case-control data: an application to generalized aggressive periodontitis

Several studies have shown a role for the involvement of interleukin (IL)-1 gene cluster polymorphisms in the risk of periodontal diseases. In the present study, we tested polymorphisms, derived from genes of the IL-1 cluster, for association with generalized aggressive periodontitis (GAP) through both allelic association and by constructing a linkage disequilibrium (LD) map of the 2q13-14 disease candidate region. The IL-1RN (VNTR) genotype distribution observed was significantly different in GAP and control subjects (P=0.019). We also observed some evidence for an association between GAP and the IL-1B(+3953) polymorphism (P=0.039). The pattern of association in the region, represented as an LD map, identifies a recombination hot area between the IL-1B(+3953) and IL-1B(-511) polymorphisms. Multilocus modelling of association with disease gives a location for the peak association at the IL-1B(+3953) marker, although support for the peak is not significant. Haplotype analysis identifies a IL-1B(+3953)-IL-1B(-511) haplotype as having the lowest P-value in the region. Recognition of the presence of a recombination hot area between the IL-1B(+3953) and IL-1B(-511) polymorphisms will have an important bearing on future efforts to develop higher resolution SNP analysis in this region for both this and other diseases for which this cluster is implicated.