Clinical and pathological analysis of primitive neuroectodermal tumor of adrenal

Abstract

Objective To analyze the clinical and pathological features of the adrenal primitive neuroectodermal tumors(PNET). Methods Four cases of PNET were analyzed.Of them,2 were males and 2 females,aged from 21 to 30 years old with a mean age of 24.No significant abnormal data was found in routine laboratory and endocrine examinations.The CT scan showed a soft tissue mass with unclear boundary and cystic changes in adrenal area.The tumor diameter was 8-17 cm. Results One patient refused treatment alter diagnosed by biopsy and died 6 months later.Another one received palliative operationand died after 8 months.The third patient was found distant metastasis 1 month after operation and had radiotherapy and chemotherapy.The fourth patient was found local tumor recurrence 1 month after operation,and started chemotherapy.All patients were diagnosed by pathology.At HE staining,tumor was consisted of even,uniform small round cells;the cells distributed diffusively or formed lobulated structures (Homer-Wright rosette).Immunohistochemical staining showed CD99 positive in all 4 patients. Canclusion The adrenal PNET is a rare disease originated from primitive neuroectodermal,mostly occurs between 20 and 30 years old,and has non-specific clinical and imaging findings.Histopathologieal examination is the key point for diagnosis.Rapid progression,highly malignant,poor prognosis are the characteristics of this disease. Key words: Adrenal gland neoplasms; Neuroectodermal tumors,primitive,peripheral