RESUMO
Extramammary Paget's disease and Bowen's disease are histologically similar and immunohistochemistry is often required to make the diagnosis. We present a case of vulval Paget's disease with Bowen's disease in an elderly female. Strong positivity for cytokeratin 7, anti CAM 5.2, carcinoembryonic antigen (CEA) and periodic acid-Schiff (PAS) stain in clitoral, left labial and interface regions of the vulvectomy specimen confirmed the diagnosis of Paget's disease (PD) while positive staining for p63 in the right labial and interface regions helped in establishing the diagnosis of concurrent Bowen's disease (BD).
Assuntos
Doença de Bowen/patologia , Neoplasias Primárias Múltiplas/patologia , Doença de Paget Extramamária/patologia , Neoplasias Cutâneas/patologia , Neoplasias Vulvares/patologia , Biomarcadores/análise , Doença de Bowen/química , Doença de Bowen/cirurgia , Antígeno Carcinoembrionário/análise , Feminino , Humanos , Queratina-7/análise , Queratinas/análise , Proteínas de Membrana/análise , Pessoa de Meia-Idade , Neoplasias Primárias Múltiplas/química , Neoplasias Primárias Múltiplas/cirurgia , Doença de Paget Extramamária/química , Doença de Paget Extramamária/cirurgia , Neoplasias Cutâneas/química , Neoplasias Cutâneas/cirurgia , Neoplasias Vulvares/química , Neoplasias Vulvares/cirurgiaRESUMO
Aggressive angiomyxoma is a rare, slow-growing mesenchymal neoplasm with a tendency to recur. It mainly involves the pelvis, vulva, perineum, vagina, and urinary bladder in adult women of reproductive age group. We describe a 26-year-old female with large swellings of both labia majora which was histologically diagnosed as aggressive angiomyxoma. She also had systemic lupus erythematosus. The swelling was surgically removed and she had no recurrence at 1-year follow-up. Although it is a rare tumor, it must be considered as a differential diagnosis for any mass in the perineum or soft tissue of the pelvis. Long-term follow-up is necessary for early diagnosis of local recurrence.