Peripheral Neuroectodermal Tumor (PNET) of the breast - A 6-year follow-up

Abstract

Background: Peripheral primitive neuroectodermal tumors (PNET) are usually located in bone or soft tissue in children or young adults but can also appear at a more advanced age. Case Report: A 60-year-old woman was admitted to our hospital presenting with a tumor of the right breast. Imaging studies led to the diagnosis of suspected benign lesion. Cytologic examination showed atypical cells. The biopsy showed a tumor composed of medium-sized tumor cells with round and ovoid vesicular nuclei and ill-defined cellular margins. The growth pattern was mainly solid in cell clusters. Focally sinusoidal, blood-filled spaces were present. Cytogenetic examination of 20 metaphases showed the karyotype 46,XX/t(11;22)(q24,q12)/46,XX. Mastectomy was performed, and adjuvant chemotherapy and radiotherapy were given. After a follow-up of 72 month, the patient is alive without evidence of disease. Conclusion: The adjuvant therapy (VACA regime) seems to lead, independently from tumor localization and patient age, to longer disease-free survival. © 2006 S. Karger GmbH.