Aggressive angiomyxoma [corrected] of the female genital tract and pelvis--clinicopathologic features with immunohistochemical analysis.

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.

Paget's osteosarcoma and post-radiation osteosarcoma: secondary osteosarcoma at Middlemore Hospital, New Zealand.

AIMS: To review the clinical and histological features of our cases of Paget's osteosarcoma and post-radiation osteosarcoma. METHODS: A search through the files of the New Zealand Bone and Soft Tissue Tumour Registry was performed, patients were identified and the relevant details were collated. RESULTS: Thirty-one cases of Paget's osteosarcoma and eight cases of post-radiation osteosarcoma were identified. Patients with Paget's osteosarcoma were aged between 48 and 67 years, predominantly female, and axial and appendicular skeleton were equally affected. The outcome was known in 29 cases, with a median survival of 7.25 months and 5 year overall survival of 10%. Patients with post-radiation osteosarcoma were aged between 17 and 68 years, equally male or female, and axial and appendicular skeleton were equally affected. Index lesions included benign or malignant osseous and non-osseous conditions. The average age at diagnosis of the index lesion was 30.1 years and the average latent period was 13.5 years. The outcome was known in all eight cases, with a median survival of 33 months and 5 year overall survival of 38%. CONCLUSIONS: Paget's osteosarcoma and post-radiation osteosarcoma are examples of secondary osteosarcoma. The former affects elderly patients, and has a poor prognosis and response to treatment. The later affects a wide age group, and has a prognosis and response to treatment comparable with primary osteosarcoma.