背景:胰腺实性假乳头状瘤(SPNP)是一种罕见的原发性肿瘤,具有独特的临床病理特征。肿瘤最常见于年轻(绝经前)妇女,以恶性潜能低和总体预后良好为代表。
方法:在两个转诊的三级护理机构(费萨尔国王医院和研究中心和阿卜杜勒阿齐兹国王大学医院,吉达,沙特阿拉伯王国)发现12名女性患者被诊断患有SPNPs。还对诊断时进行的辅助研究的结果进行了审查,并将其置于当前建议的背景下。
结果:回顾了临床和病理发现。所有患者均为女性,18至30岁。八名患者出现腹痛,其中两人经历了显著的体重减轻,4人出现腹部肿块/不适。肿瘤大小为1.5和15cm。2例最初诊断为神经内分泌肿瘤(NETs)。其中一例表现为多灶性疾病。所有患者均接受手术治疗,随访期为1至11年。只有1例患者经过7年的随访出现腹膜转移,但总的来说,都做得很好。
结论:在这项研究中,我们分析了20年(2001-2021年)人口中的12例SPNP病例。简而言之,SPNP是一种低度恶性潜能肿瘤。尽管SPNP是公认的实体,诊断挑战可能会出现,特别是在有限的采样设置。病理学家必须了解SPNP的经典形态特征和免疫组织化学的特征性特征,并能够将SPNP与其他模拟者区分开来。尤其是分化良好的胰腺NET,并最终避免误诊和不必要的肿瘤治疗。切缘阴性的充分手术切除与出色的结果相关。
BACKGROUND: Solid pseudopapillary neoplasm of the pancreas (SPNP) is a rare primary neoplasm with distinct clinicopathological features. The tumor most commonly occurs in younger (premenopausal) women and is typified by low malignant potential and an excellent overall prognosis.
METHODS: A retrospective search over 20 years at two referral tertiary care institutions (King Faisal Hospital and Research Center and King Abdulaziz University Hospital, Jeddah, Kingdom of Saudi Arabia) revealed 12 female patients diagnosed with SPNPs. The reslts of ancillary studies performed at the time of diagnosis were also reviewed and placed in the context of current recommendations.
RESULTS: The clinical and pathological findings were reviewed. All patients were females, aged 18 to 30 years. Eight patients presented with abdominal pain, of which two experienced significant weight loss, and four presented with abdominal mass/discomfort. The tumor size ranged from 1.5 and 15 cm. Two cases were initially diagnosed as neuroendocrine tumors (NETs). One of the cases presented as a multifocal disease. All patients were treated surgically with a follow-up period between one and 11 years. Only one patient presented with peritoneal metastasis after seven years of follow-up, but generally, all are doing well.
CONCLUSIONS: We have analyzed 12 SPNP cases in our population over 20 years (2001-2021) in this study. In brief, SPNP is a low-grade malignant potential tumor. Even though SPNP is a recognized entity, diagnostic challenges can arise particularly in the setting of limited sampling. Pathologists must be aware of the classic morphological features of SPNP and the characteristic profile of immunohistochemistry and be able to differentiate SPNP from other mimickers, especially well-differentiated NETs of the pancreas, and ultimately to avoid misdiagnosis and unnecessary oncologic treatment. Adequate surgical resection with negative margins is associated with an excellent outcome.