The diagnosis and treatment analysis for primitive neuroectodermal tumor of the adrenal gland:2 cases report and literature review

Abstract

Objective To explore the diagnosis and treatment for primitive neuroectodermal tumor of the adrenal gland.Methods The clinical data of 2 patients with primitive neuroectodermal tumor of the adrenal gland were retrospectively analyzed.Literatures about diagnosis and treatment of primitive neuroectodermal tumor arising from the adrenal gland were also reviewed.Results B type ultrasonography and computer tomography scans showed a cystic and solid soft tissue masses about 3 cm (patient 1) and 15 cm (patient 2) in maximum diameter with rough border.Surgeries were performed successfully in 2 patients.The diagnosis of primitive neuroectodermal tumor arising from the adrenal gland was confirmed by histopathology and immunohistochemistry.During follow up,evidences of tumor recurrence were not found in the first patient.However,the second patient had diffuse retroperitoneal lymph node metastasis 3 months after surgery.Then,the patient died 6 months after surgery.Conclusion The primitive neuroectodermal tumor of the adrenal gland is extremely rare with highly malignant and poor prognosis.Its diagnosis is based on histopathology and immunohistochemistry.Multimodal therapeutic regimens including surgery,combination with chemotherapy and radiotherapy were recommended. Key words: Adrenal; Neural, ectodermal, tumor; Diagnosis ; Treatment