HMGA2 is a Sensitive But Not Specific Immunohistochemical Marker of Vulvovaginal Aggressive Angiomyxoma
Department of Pathology, Belfast Health and Social Care Trust, Belfast, Northern Ireland.The American journal of surgical pathology (Impact Factor: 5.15). 07/2010; 34(7):1037-42. DOI: 10.1097/PAS.0b013e3181e32a11
Aggressive angiomyxoma is a rare mesenchymal neoplasm, which almost always occurs in the vulvovaginal region and which exhibits a marked tendency for local recurrence after excision. A wide range of other mesenchymal lesions occur in this region, which potentially mimic aggressive angiomyxoma to a variable extent. Because rearrangement of the HMGA2 gene has been shown in a significant percentage of aggressive angiomyxomas, we examined the expression of HMGA2 protein in this neoplasm and in a large number of other mesenchymal lesions occurring at this site, including many which were referred with a possible diagnosis of aggressive angiomyxoma. There was positive staining of tumor cell nuclei, mostly with a diffuse distribution, in all but 2 aggressive angiomyxomas (n=12). Blood vessel endothelial cells within the neoplasms, entrapped tissues, and surrounding normal tissues were negative. Ten of 23 leiomyomas were positive, as were occasional other neoplasms. Based on our study, we conclude that HMGA2 is positive in most aggressive angiomyxomas and is useful in diagnosis as most mesenchymal lesions which closely mimic this are negative. However, caution should be exercised as some other vulvovaginal mesenchymal lesions, especially, but not exclusively leiomyomas can be HMGA2-positive. As well as being of value in primary diagnosis, HMGA2 is useful in evaluating margins and in reexcision specimens of aggressive angiomyxoma in identifying foci of residual or recurrent tumor.
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ABSTRACT: Perineal nodules occurring in male cyclists are reported in the literature, although the histologic features are not extensively documented. There has been little description of similar lesions in the female population. We describe 4 cases in which a vulval nodule or swelling developed in competitive female cyclists aged 15 to 45 years. The lesions were unilateral and occurred on the right or left labium majus (2 cases each). The histologic features were similar in all cases and consisted of a haphazard admixture of adipose tissue, variably cellular hyalinized tissue containing bland spindle-shaped fibroblasts, blood vessels, and nerve fibers. In some areas, thick cords of fibrous tissue imparted a keloid-like appearance. Other histologic features included plump mesenchymal cells with round or ovoid nuclei and abundant eosinophilic cytoplasm resulting in an epithelioid, plasmacytoid, or ganglion-like appearance (2 cases), a lymphocytic infiltrate around blood vessels (3 cases), foci of fat necrosis (1 case), and collections of elastic fibers (2 cases). One case recurred, the histologic features of the recurrent lesion being identical to the original. The overall morphologic appearances, especially in the cases with plump mesenchymal cells, bore some resemblance to proliferative fasciitis. Immunohistochemically, the cells were estrogen receptor positive and the plump mesenchymal cells were smooth muscle actin positive, in keeping with myofibroblasts. Desmin, S100, CD34, and HMGA2 were negative. Pathologists should be aware of this pseudoneoplastic lesion occurring on the vulva, which arises in a specific clinical setting and has the potential to be misdiagnosed as a variety of other mesenchymal lesions. We term this lesion as reactive fibroblastic and myofibroblastic proliferation of the vulva or "cyclist's nodule."The American journal of surgical pathology 01/2011; 35(1):110-4. DOI:10.1097/PAS.0b013e3181ffd8ab · 5.15 Impact Factor
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ABSTRACT: Aggressive angiomyxoma (AA) is a benign, slow-growing tumor that characteristically occurs in women of reproductive age. Local recurrence is cited in 30% to 40% of cases. Wide local excision is the treatment of choice. However, recent reports suggest a role for hormone manipulation in the management of these tumors. The morphology and immunophenotype of AA overlap with that of other, mainly benign vulvovaginal mesenchymal tumors. Diagnosis rests primarily on hematoxylin and eosin staining features, and distinction is important in determining appropriate treatment and follow-up. Rearrangement of HMGA2 has been shown in AA, and reports suggest that HMGA2 immunohistochemistry may have a role in the routine diagnosis of AA, its distinction from mimics, and in the evaluation of margins. Furthermore, CDK4 immunopositivity has been described in AA. We describe a series of 9 cases of AA with typical histology and long-term follow-up, and evaluate the role of HMGA2, CDK4, estrogen, and progesterone immunohistochemistry. One of 9 women (11%) experienced recurrence, with the second at 17 years, which is the longest recorded in the English literature. HMGA2 immunohistochemistry was positive in 37.5% of cases, consistent with the reported frequency of HMGA2 gene rearrangement, and negative in all benign mimics. CDK4 immunoreactivity was weak, diagnostically not helpful, and of uncertain significance. Immunohistochemistry for estrogen and progesterone were positive in 87.5% of AAs, and were widely positive in control groups.International journal of gynecological pathology: official journal of the International Society of Gynecological Pathologists 09/2011; 30(5):505-13. DOI:10.1097/PGP.0b013e318211d56c · 1.67 Impact Factor
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ABSTRACT: Se presenta el caso clínico de un angiomixoma agresivo, de presentación en un paciente de sexo masculino, con localización presacra, el cual fue diagnosticado e intervenido en el Hospital Militar Central de Bogotá. Se realizó un abordaje tipo Kraske con la escisión completa de la lesión y mínimo impacto funcional y estético para el paciente. Esta clase de tumores son extremadamente infrecuentes y predominan en mujeres, con una localización pélvica. La presentación en hombres es aún más exótica, con pocos casos reportados en la literatura. Su manejo obliga a la extirpación completa de la lesión y al seguimiento clínico e imagenológico periódico, dada su alta tasa de recidiva.Revista Colombiana de Gastroenterologia 12/2011; 26(4):304-310.
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