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.     

Cytogenic and molecular analysis of an aggressive angiomyxoma.

Aggressive angiomyxoma is a rare mesenchymal tumor occurring mainly in the vulvar region extending into the paravaginal and perirectal region. Histologically, this tumor is rich in vascular structures and in collagen fibers and is of myxoid appearance. Cytogenetic and molecular analysis was performed on a case of an aggressive angiomyxoma and revealed clonal karyotypic abnormalities. All 50 metaphases analyzed showed a translocation involving the chromosomal region 12q14-15. Chromosomal aberrations involving the breakpoint region 12q14-15 are frequently seen in a variety of other mesenchymal tumors as uterine leiomyomas, lipomas, hamartomas of the lung, liposarcomas, or hemangiopericytomas. Therefore, this breakpoint region seems to be the most frequent chromosomal abnormality associated with the initiation of human mesenchymal neoplasms. To narrow down the breakpoint region on a molecular level in the cells of the angiomyxoma we performed FISH analysis with different cosmid clones originating from a yeast artificial chromosome and cosmid contig overspanning parts of the region 12q14-15. We were able to narrow down the region to approximately 70-80 kb belonging to an area designated a multiple aberration region, because it also includes the breakpoints of leiomyomas, lipomas, and pleomorphic adenomas with 12q13-15 abnormalities. Our molecular and cytogenic data suggest that angiomyxomas or at least a subset of them molecularly belong to the benign group of mesenchymal tumors showing multiple aberration region involvement.     

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.     

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.     

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.     

An aggressive lipoblastic tumour in the orbit of a child

The case is described of a locally aggressive lipoblastic tumour presenting in the orbit of an 8-year-old boy. There do not appear to be any previous reports of a comparable tumour in this situation.     

Prognostic impact of peripelvic fat invasion in pT3 renal pelvic transitional cell carcinoma

Renal pelvic transitional cell carcinoma (TCC), which invades beyond muscularis into peripelvic fat or the renal parenchyma, is diagnosed as stage pT3 despite its structural complexity. We evaluated the prognostic impact of peripelvic fat invasion in pT3 renal pelvic TCC. Between 1986 and 2004, the medical records on 128 patients who were surgically treated for renal pelvic TCC were retrospectively reviewed. Sixty patients with pT3 disease were eligible for the main analysis. The prognostic impact of various clinicopathological factors was analyzed using univariate and multivariate analyses. On univariate analysis, sex, age, concomitant bladder tumors, concomitant ureter tumors, lymphadenectomy, adjuvant chemotherapy, tumor grade, multiplicity, renal parenchymal invasion, and carcinoma in situ did not influence the disease-specific survival (p>0.05). By contrast, peripelvic fat invasion, lymph node invasion, and lymphovascular invasion were each significantly associated with disease-specific survival (p<0.05). Multivariate analysis showed that peripelvic fat invasion (p=0.012) and lymph node invasion (p=0.004) were independent prognostic factors. In conclusion, peripelvic fat invasion is a strong prognostic factor in pT3 renal pelvic TCC. Thus, systemic adjuvant therapy should be considered in the presence of peripelvic fat invasion, even if the lymph nodes are not involved.     

Rhabdoid features in leiomyosarcoma of soft tissue: with special reference to aggressive behavior.

The presence of rhabdoid cells has been reported in various types of malignant neoplasms and has been determined to be a predictor of aggressive behavior of neoplasms regardless of tumor histogenesis. One hundred and thirteen cases of leiomyosarcoma, selected from 1800 soft tissue sarcomas, were reviewed on hematoxylin and eosin sections, and immunohistochemical staining when available, and seven cases with rhabdoid features were retrieved. Clinicopathologic differences were analyzed to compare between cases with rhabdoid features and those without rhabdoid features. In the seven cases with rhabdoid features, two were intra-abdominal, and the others arose in external soft tissues including muscle, subcutis, and cutis. Patient age ranged from 33 to 84 years, three were female, and four were male. Tumor size ranged from 3 to 22 cm. Clinical evidence showed no differences from those cases without rhabdoid features. Histologically, one of the abdominal cases was epithelioid leiomyosarcoma. Two of the 7 cases were better subclassified as pleomorphic leiomyosarcoma, in which rhabdoid cells are diffusely scattered. In cases other than those with pleomorphic leiomyosarcomas, foci of anaplastic areas were observed, and collections of rhabdoid cells were present in those areas. Immunohistochemical examination of the cases confirmed myogenic differentiation, and showed rhabdoid cells being positive for vimentin and desmin in the inclusion bodies, and diffusely so for muscle actin in the cytoplasm. After dividing all the cases of leiomyosarcoma by their location, prognostic analysis was performed. Leiomyosarcoma of external soft tissue with rhabdoid cells showed a tendency for poorer prognoses than cases without rhabdoid features. On the contrary, retroperitoneal cases did not. This study indicates that rhabdoid features are associated with aggressive biological behavior in leiomyosarcoma of the external soft tissue.     

Plexiform fibrohistiocytic tumour with novel phenotypic features

Plexiform fibrohistiocytic tumour is a recently described type of fibrohistiocytic tumour, the differentiation pattern of which is poorly understood. A case representing the predominantly fibrohistiocytic type of the tumour is reported. Immunohistochemical analysis supports the histiocytic origin of this entity. The expression of EBM/11 and tissue transglutaminase both in fibroblast-like and histiocyte-like cells indicate that a single cell line produces the dual morphological features. It is proposed that a non-phagocytic epithelioid pattern of histiocytic differentiation characterizes this tumour.     

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.     

Bone marrow necrosis and extramedullary myeloid tumour necrosis in aggressive chronic myeloid leukaemia.

A patient with chronic myeloid leukaemia who developed widespread bone marrow necrosis associated wtih evolution of a new cell clone and with aggressive behaviour of the myeloid tumour in extramedullary sites is described. Cell necrosis in extramedullary tumours was also present; the sharp demarcation between zones of necrotic cells and intact cells and the histological evidence of vascular invasion by tumour cells suggest a vascular basis for cell necrosis.     

Anaplastic Wilms' tumor and other clinically aggressive childhood renal neoplasms: ultrastructural and immunocytochemical features

The ultrastructural and immunocytochemical findings in a series of 32 rarely encountered childhood renal malignancies are reviewed. The discussion includes anaplastic Wilms' tumors, renal clear cell sarcomas, rhabdoid tumors, carcinomas, sarcomas, neuroendocrine tumors, and lymphomas. An attempt is made to assess the relative merits of these two techniques in the differential diagnosis of these often troublesome lesions.     

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.     

Renal cell carcinoma with rhabdoid features: an aggressive neoplasm

AIMS: Only a few reports on renal cell carcinoma with rhabdoid features have been published. This study was performed to investigate the clinicopathological characteristics of renal cell carcinomas with rhabdoid features. METHODS AND RESULTS: Among 253 cases of renal cell carcinoma in adults, eight cases with rhabdoid features were detected. Rhabdoid areas ranged from 10% to 90% of each of the cases. Seven of the eight cases were TNM stage III or IV, and four of the eight cases died within 8 months of surgery. Immunohistochemically, the rhabdoid areas were positive for CAM 5.2 (4/8), AE1/AE3 (6/8), epithelial membrane antigen (6/8) and vimentin (8/8), and negative for myogenetic markers (0/8). The mean MIB-1 labelling index in the rhabdoid areas was higher than that in the definite carcinomatous areas. Ultrastructurally, perinuclear whorls of intermediate filaments were demonstrated in three of the eight cases using paraffin-embedded blocks. CONCLUSIONS: The rhabdoid areas in renal cell carcinoma have histological, immunohistochemical and ultrastructural similarities to malignant rhabdoid tumours. Renal cell carcinoma with rhabdoid features is a highly aggressive neoplasm and its malignant behaviour may be due to the high cell-proliferative activity of the rhabdoid areas. Rhabdoid features in renal cell carcinoma may represent the endpoint of clonal evolution of renal cell carcinoma (especially in clear cell type cases).     

Pathological features of lymphoid tissues in cats with natural feline immunodeficiency virus infection.

A range of tissues from a total of 17 cats naturally infected with the feline immunodeficiency virus was examined histologically. In 11 cases, chronic inflammatory lesions were present in various tissues including, most commonly, the intestine, brain and lung. Extensive inflammation in the intestinal wall was present in seven of the cats. No particular bacterial organisms were demonstrated in these inflammatory lesions. A range of changes was present in the lymph nodes, including hyperplasia, atrophy or a mixed pattern. Erythrophagocytosis was a consistent feature. The changes resembled those reported in human acquired immunodeficiency syndrome as a result of infection with human immunodeficiency virus.     

A case of retiform-hemangioendothelioma with unusual presentation and aggressive clinical features

Retiform hemangioendothelioma (RH) is an extremely rare low-grade angiosarcoma mainly involving the skin and subcutaneous tissue. Clinically patients often present with an asymptomatic slow-growing solitary nodular or plaque-like lesion. RH is characterized by frequent local recurrences but a very low metastatic rate. Here we reported a case of RH in a 61-year-old Chinese woman who presented with a rapid growing cutaneous plaque-like lesion on her right scalp, followed by another lesion behind the right ear. The lesions were associated with paroxysmal sharp needle-stabbing like headache. She underwent wide excision and skin engraftment. Three months post surgery, she experienced tumor recurrence, and died 9 months after the initial diagnosis.