Primitive neuroectodermal tumors of adrenal gland.
OBJECTIVE
To analyze the clinical and pathological characteristics of adrenal primitive neuroectodermal tumors for a better understanding of the disease.


METHODS
A retrospective analysis of four cases of adrenal primitive neuroectodermal tumors (two male, two female; age 21-30, average 24) was made. All patients went through necessary endocrinological exams, computer tomography scans (for site-specific diagnoses) and pathological tests.


RESULTS
No positive result was reported in routine laboratory tests and endocrinological exams. Computer tomography scans showed bulk soft tissue masses with rough borders. The masses were 8-17 cm in diameter with solid-cystic changes. Among the four patients, one refused to receive treatment after definitiver diagnosis through needle biopsy, three received surgical treatments and their post-operative pathological exams all confirmed the diagnoses of primitive neuroectodermal tumors. During the follow-ups, the untreated patient died 6 months afterwards, one patient died 8 months after palliative treatment; one patient showed distant metastasis 13 months after surgery and did not respond well to both radio- and chemotherapy; one patient had local recurrence 1 month after surgery and is presently undergoing chemotherapy.


CONCLUSIONS
Adrenal primitive neuroectodermal tumor is a very rare tumor. It originates in primitive neuroectoderma and is found mainly in 20-30-year-old young populations. It has non-specific clinical or imaging manifestation and its diagnosis is mostly based on pathological examinations. The tumor is fast-developing, highly malignant with poor prognosis.
Zhang
Yushi
Y
Department of Urology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Peking Union Medical College, Beijing 100730, China. zhangyushi@126.com
Li
Hanzhong
H
D000310:Adrenal Gland Neoplasms / Q000175:diagnosis* / Q000473:pathology* / Q000628:therapy; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D017024:Chemotherapy, Adjuvant; D017809:Fatal Outcome; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007813:Laparotomy; D008207:Lymphatic Metastasis; D008297:Male; D009360:Neoplastic Cells, Circulating / Q000473:pathology / Q000648:ultrastructure; D017599:Neuroectodermal Tumors / Q000175:diagnosis* / Q000473:pathology* / Q000556:secondary / Q000628:therapy; D011856:Radiographic Image Enhancement; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed; D014682:Vena Cava, Inferior / Q000000981:diagnostic imaging