Angiomyofibroblastoma-like tumor (cellular angiofibroma) in the male inguinal region.

Angiomyofibroblastoma-like tumor is a rare mesenchymal tumor involving the male genital tract. We report a case of an angiomyofibroblastoma-like tumor that arose in the subcutaneous tissue of the left inguinal region in a 50-year-old man. Ultrasonography of the region demonstrated a well-circumscribed subcutaneous mass. Intralesional fat was revealed on magnetic resonance images. Although these imaging features are nonspecific, radiological findings enable considering the diagnosis of angiomyofibroblastoma-like tumor.

A study of four cases of extra-orbital giant cell angiofibroma with documentation of some unusual features.

AIMS: To document the clinical, light microscopic, immunohistochemical and ultrastructural features of four cases of extra-orbital giant cell angiofibromas. METHODS AND RESULTS: Sections of formalin-fixed paraffin-embedded specimens were studied by haematoxylin and eosin, reticulin and immunohistochemical stains. Electron microscopy was carried out in two cases on tissue fixed in formalin. The age of the patients ranged from 30 to 41 years. Two patients presented with a soft tissue swelling in the left groin, one patient had a left axillary soft tissue lump and one patient presented with a parotid lump. All lesions were well circumscribed and contained variably cellular and vascularized tissue composed of round to spindle cells with a patternless arrangement, scattered multinucleate giant cells and pseudovascular spaces conforming to the description of giant cell angiofibroma. Mononuclear and multinucleate tumour cells were both positive for vimentin and CD34; one tumour exhibited focal S100 protein and GFAP positivity. Both of the tumours examined by electron microscopy showed fibroblastic features, but in addition one contained cells having Schwannian features. All four patients were well without recurrent disease on follow-up (average 25 months). CONCLUSION: Giant cell angiofibroma shares many features with solitary fibrous tumour and giant cell fibroblastoma and shows a wider distribution than initially recognized. Rarely, Schwannian differentiation may be observed in these tumours.

Tumores de las fosas nasales y senos paranasales. Tumores no epiteliales / Tumors of the nasal fossae and paranasal sinuses. Non epithelial tumors

Los tumores no epiteliales de las fosas nasales incluyen tumores de origen mesectodérmicos y neuroectodérmicos. Las neoplasias mesectodérmicas mas frecuentes son los vasculares y de estos los angiofibromas son los mas frecuentes y le siguen los hemangiomas, los angiosarcomas son excepcionales, Los linfomas son raros no obstante el anillo de Waldeyer. Los mixomas son frecuentes y por su vecindad con la órbita y la cavidad craneana aunque su comportamiento es benigno son muy destructivos y pueden llevar a la muerte. Dentro de los sarcomas lo rabdomiosarcomas son los mas frecuentes

Angiofibroblastoma of the skin: a histological, immunohistochemical and ultrastructural report of two cases of an undescribed fibrous tumour.

AIMS: The aim of this report is to present two cases of a distinct mesenchymal tumour of the skin that does not fit into one of the established entities. METHODS AND RESULTS: All cases of fibromyxoid tumours from the files of two dermatopathology centres, together with clinical data and histopathological records, were reviewed. Two cases of a lesion composed of stellate and spindle-shaped cells with the phenotype of fibroblasts embedded in a fibromyxoid to dense fibrous stroma were identified. Because of the large number of capillary-sized blood vessels and their peculiar distribution within the stroma, the name angiofibroblastoma of the skin is proposed for this peculiar neoplasm. In both cases, the tumour appeared as an indolent and slowly growing nodule on the extremities of adults. Immunohistochemical and, in one case, ultrastructural studies were performed. CONCLUSIONS: The lack of recurrences during a follow-up period of 9 and 3 years, respectively, after complete excision together with the bland histopathological appearance supports the benign character of the tumour.

Giant cell angiofibroma of the orbit and eyelid.

PURPOSE: To report the clinicopathologic features of a newly recognized tumor, giant cell angiofibroma. DESIGN: Observational case series. MAIN OUTCOME MEASURES: Clinical and histopathologic features of giant cell angiofibroma. METHODS: Light and electron microscopy and immunohistochemistry of five cases of giant cell angiofibroma. RESULTS: A total of five patients (4 women and 1 man) are described: two presented with a painless mass in the eyelid, two with a mass in the orbit, and one presented with a conjunctival lesion. All lesions were well demarcated with no capsule and were composed of blood vessels, a patternless spindle-shaped cell proliferation with a solid and pseudovascular appearance, and multinucleated giant cells. Both spindle-shaped and giant tumor cells were intensely positive for CD34 and vimentin. CONCLUSION: Giant cell angiofibroma resembles solitary fibrous tumor and giant cell fibroblastoma and should be considered in the differential diagnosis of spindle-cell tumors in the eyelid, orbit, and conjunctiva.

Angiofibromas juveniles nasofaríngeos / Youthful rhinofaringeal angiofibromas

Los angiofibromas juveniles nasofaríngeos son los tumores nasofaríngeos benignos más frecuentes. Se presentan predominantemente en varones púberes. Los síntomas más frecuentes son obstrucción nasal y epistaxis recurrentes. A pesar de que su comportamiento biológico es benigno, tiende a crecer destruyendo las estructuras óseas vecinas a la nasofaringe hasta extenderse a la base del cráneo. Recurren con una frecuencia que va del 35 al 57 por ciento después de la extirpación quirúrgica incompleta. El diagnóstico diferencial debe hacerse con hemangiopericitoma, tumor fibroso solitario, hemangiomas, schwannoma e histiocitoma fibroso

Cirugía de angiofibroma nasofaringeo juvenil / Surgery of of juvenile nasopharyngeal angiofibroma

En el presente trabajo, analizamos los distintos tratamientos quirúrgicos que se llevaron a cabo en 6 casos de angiofibroma nasofaríngeo juvenil (ANJ). Las vías quirúrgicas que se utilizaron fueron la nasal paralateral, la transpalatina, de Rouge-Denker, y la vía natural oral. Analizamos el criterio actual sobre la preparación prequirúrgica, la utilización de las distintas técnicas actuales, haciendo mención especial del "degloving". Se hace referencia a la relación entre los diferentes estadios del tumor: tamaño y localización, y consecuentemente las distintas técnicas a emplear

Angiomyofibroblastoma of the vulva: a mitotically active variant?

A case of angiomyofibroblastoma in a 48-year-old 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 vulva and was well circumscribed, measuring 2.8 x 2.7 x 2.5 cm. Microscopically, the tumor was composed of hypocellular and cellular areas with well-developed small vessels. Spindle or polygonal cells were arranged with perivascular accentuation in an edematous or fibrocollagenous background. Some spindle-shaped or polygonal stromal cells were also arranged in epithelioid nests. In some areas, mitoses were frequent (maximum 3/10 high-power field). Immunohistochemically, the stromal cells were positive for vimentin and desmin, but negative for alpha-smooth muscle actin, S-100, neurofilament, estrogen receptor, progesterone receptor, CD31 and CD34. The average labeling index of Ki-67 in stromal cells was 3.1%. Ultrastructural analysis demonstrated that the stromal cells adhered with primitive junctions and contained intermediate filaments with no focal density in the cytoplasm. These findings were consistent with angiomyofibroblastoma, although previously reported cases did not show so many mitoses. Therefore, this case was suggested to be a mitotically active variant.

Angiomyofibroblastoma of the vulva.

Angiomyofibroblastoma is a rare, myxoid tumor of the vulva. To date only 12 cases have been reported in the world literature. Patients are usually premenopausal and present with a vulval mass initially diagnosed as a Bartholin's cyst. The lesions are well circumscribed and range from 0.5 to 12 cm in size. Microscopically the tumors are characterized by high cellularity, numerous blood vessels, and plump stromal cells. Treatment is by surgical excision. There are currently no published reports of local recurrence or metastatic disease. Angiomyofibroblastoma should be differentiated from other neoplasms of the vulva where radical surgical treatment is indicated. A Case Report of angiomyofibroblastoma of the periclitoral region diagnosed in a postmenopausal woman is presented.

[Angio-fibrolipoma in epidural space of the vertebral canal]. / Naczyniako-wlókniako-tluszczak w przestrzeni zewnatrzoponowej kanalu kregowego.

55-yera old patient with angio-fibro-lipoma of thoracic part of the vertebral canal was described. This rare, benign tumour was totally removed. Subsequently the neurological state of the patient improved.

[Nasal angiofibroma in an adult]. / Angiofibroma nasal. A propósito de un caso de presentación en la edad adulta.

We have studied a nasal Angiofibroma in a 71-years-old male, who had not undergone any male-hormonal-therapy. The age of occurrence and the clinical course of this case made the tumor very unlikely to be a silent puberal nasal angiofibroma. After morphologic exam and exclusion of other bleeding entities of the nasal cavity we arrived at the diagnosis of nasal angiofibroma. We believe that this tumor might be arisen "ex novo" and that the nasal angiofibroma of elderly people might be a new entity.