Radiological features of primitive neuroectodermal tumors in intra-abdominal and retroperitoneal regions: A series of 18 cases.
OBJECTIVE
To characterize the imaging and clinicopathological features of primitive neuroectodermal tumors (PNETs) arising in intra-abdominal and retroperitoneal regions.


METHODS
Eighteen patients with histopathologically proven intra-abdominal and retroperitoneal PNET were enrolled; computed tomography was performed for all cases, and magnetic resonance imaging was performed for a single case. Typical computed tomography and magnetic resonance imaging findings, including morphology, texture and enhancement features, as well as clinicopathological characteristics and prognosis data were retrospectively analyzed.


RESULTS
Of eighteen PNET patients, fifteen were male and three were female, with a median age of 36 years (range, 2-65 years). The onset of symptoms was most often nonspecific and insidious. The mean tumor diameter was 7.2 cm (range, 3.0-12.1 cm), with necrosis in fifteen cases, cystic changes in eight, partition structure in five, calcification in five, hemorrhage in two, and mural nodules in one. Contrast enhanced computed tomography showed multiple tiny feeding arteries within the masses in six cases, resulting in a crab-like appearance, and mild ring enhancement pattern in five cases. Eleven cases showed surrounding invasion and metastasis. Of the eighteen PNET cases, nine cases showed smooth, well-defined margins, and nine cases had irregular, ill-defined margins. A median survival was 10.0±1.6 months. However, chemotherapy had efficacy on patients even those with advanced disease.


CONCLUSIONS
Primary intra-abdominal and retroperitoneal PNETs are rare, and imaging features documented here may help the diagnosis of this severe disease. Notably, two signs present in retroperitoneal PNET tumors, including a mild ring enhancement pattern and a crab-like appearance of the tiny feeding arteries, may have the potential to help us improve the ability to make a relatively reliable diagnosis.
Department of Radiology, Xiangya Hospital, Central South University; Changsha, P.R. China.|Postdoctoral Research Workstation of Pathology and Pathophysiology, Basic Medical Sciences, Xiangya Hospital, Central South University; Changsha, P.R. China.
Xiaoping
X
Yi
Department of Radiology, Xiangya Hospital, Central South University; Changsha, P.R. China.
Wenguang
W
Liu
Department of Radiology, Xiangya Hospital, Central South University; Changsha, P.R. China.
Youming
Y
Zhang
Department of Pathology, Xiangya hospital, Central South University; Changsha, P.R. China.
Desheng
D
Xiao
Department of Pathology, Xiangya hospital, Central South University; Changsha, P.R. China.
Hongling
H
Yin
Department of Radiology, Xiangya Hospital, Central South University; Changsha, P.R. China.
Xueying
X
Long
Imaging Diagnosis and Interventional Center, Sun Yat-sen University Cancer Center; Changsha, P.R. China.
Li
L
Li
Departments of General Surgery, Xiangya Hospital, Central South University; Changsha, P.R. China.
Hongyan
H
Zai
Departments of Urology, Xiangya Hospital, Central South University; Changsha, P.R. China.
Minfeng
M
Chen
Department of Radiology, Xiangya Hospital, Central South University; Changsha, P.R. China.
Wenzheng
W
Li
Postdoctoral Research Workstation of Pathology and Pathophysiology, Basic Medical Sciences, Xiangya Hospital, Central South University; Changsha, P.R. China.|Center for Molecular Medicine, Xiangya Hospital, Central South University; Changsha, P.R. China.
Lunquan
L
Sun
D000008:Abdominal Neoplasms / Q000098:blood supply / Q000000981:diagnostic imaging* / Q000473:pathology / Q000628:therapy; D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D003287:Contrast Media; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D018242:Neuroectodermal Tumors, Primitive / Q000098:blood supply / Q000000981:diagnostic imaging* / Q000473:pathology / Q000628:therapy; D011379:Prognosis; D012186:Retroperitoneal Neoplasms / Q000098:blood supply / Q000000981:diagnostic imaging* / Q000473:pathology / Q000628:therapy; D016019:Survival Analysis; D014057:Tomography, X-Ray Computed; D047368:Tumor Burden; D055815:Young Adult
D003287:Contrast Media