Raman Siva P, Kawamoto Satomi, Law Joanna K, Blackford Amanda, Lennon Anne Marie, Wolfgang Christopher L, Hruban Ralph H, Cameron John L, Fishman Elliot K
From the *Department of Radiology, Johns Hopkins University; †Division of Gastroenterology, Johns Hopkins Hospital; ‡Division of Oncology Biostatistics, Johns Hopkins University School of Medicine; §Department of Surgery, Johns Hopkins University School of Medicine; and ∥The Sol Goldman Pancreatic Cancer Research Center, Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, MD.
J Comput Assist Tomogr. 2013 Sep-Oct;37(5):824-33. doi: 10.1097/RCT.0b013e31829d44fa.
Solid pseudopapillary neoplasms (SPNs) are low-grade malignancies with an excellent prognosis, albeit with the potential for metastatic disease. This study details our institution's experience with the diagnosis and treatment of SPN, including clinical presentation, multimodality imaging findings, and potential predictors of aggressive tumor behavior.
The institutional pathology database was searched through for all cases of SPN since 1988, yielding 51 patients. The electronic medical record was searched for clinical and demographic information regarding these patients, including age, sex, presenting symptoms, type of surgery, postoperative length of stay, tumor markers, and postsurgical follow-up. All available imaging data were reviewed, including those of 30 patients who underwent multidetector computed tomography, those of 9 patients who underwent magnetic resonance imaging (MRI), those of 3 patients who underwent conventional ultrasound, and those of 11 patients who underwent endoscopic ultrasound.
A total of 84% of patients were females, with a mean age of only 33 years. Prognosis was excellent, with a mean follow-up of 3 years without recurrence. Only 1 of the 51 patients developed metastatic disease to the liver 8 years after the surgery. On computed tomography, lesions tended to be large (5.3 cm), well circumscribed (29/30), round/oval (20/30), and encapsulated (23/30). The lesions often demonstrated calcification (14/30) and typically resulted in no biliary or pancreatic ductal dilatation. The lesions ranged from completely cystic to completely solid. On MRI, the lesions often demonstrated a T2 hypointense or enhancing capsule (6/9) and demonstrated internal blood products (5/9). The lesions tended to be devoid of vascularity on conventional ultrasound. Ten patients were found to have "aggressive" histology at presentation (T3 tumor, nodal involvement, perineural invasion, or vascular invasion). No demographic, clinical, or multidetector computed tomographic imaging features were found to correlate with aggressive histology.
Certain imaging features (eg, well-circumscribed mass with calcification, peripheral capsule, internal blood products, and lack of biliary/pancreatic ductal obstruction) on computed tomography and MRI are highly suggestive of the diagnosis of SPN, particularly when visualized in young female patients. However, it is not possible to predict aggressive histology on the basis of imaging findings, clinical presentation, or patient demographic features.
实性假乳头状肿瘤(SPN)是低级别恶性肿瘤,预后良好,尽管有发生转移性疾病的可能。本研究详细介绍了我们机构在SPN诊断和治疗方面的经验,包括临床表现、多模态影像学表现以及侵袭性肿瘤行为的潜在预测因素。
检索机构病理数据库中自1988年以来的所有SPN病例,共得到51例患者。检索电子病历以获取这些患者的临床和人口统计学信息,包括年龄、性别、症状表现、手术类型、术后住院时间、肿瘤标志物以及术后随访情况。回顾所有可用的影像学数据,包括30例行多排螺旋CT检查的患者、9例行磁共振成像(MRI)检查的患者、3例行传统超声检查的患者以及11例行内镜超声检查的患者的数据。
84%的患者为女性,平均年龄仅33岁。预后良好,平均随访3年无复发。51例患者中只有1例在术后8年发生肝转移。在CT上,病变往往较大(5.3 cm),边界清晰(29/30),呈圆形/椭圆形(20/30),有包膜(23/30)。病变常显示钙化(14/30),通常不会导致胆管或胰管扩张。病变范围从完全囊性到完全实性。在MRI上,病变常显示T2低信号或强化包膜(6/9),并显示内部血液成分(5/9)。在传统超声检查中,病变往往无血管。10例患者在初诊时发现有“侵袭性”组织学表现(T3肿瘤、淋巴结受累、神经周围侵犯或血管侵犯)。未发现人口统计学、临床或多排螺旋CT影像学特征与侵袭性组织学相关。
CT和MRI上的某些影像学特征(如边界清晰的肿块伴钙化、外周包膜、内部血液成分以及无胆管/胰管梗阻)高度提示SPN的诊断,尤其是在年轻女性患者中出现时。然而,根据影像学表现、临床表现或患者人口统计学特征无法预测侵袭性组织学表现。