Department of Pathology and Laboratory Medicine, Aga Khan University Hospital, Karachi, Pakistan.
Department of Pathology and Clinical Laboratory, French Medical Institute for Mothers and Children (FMIC), Kabul, Afghanistan.
Diagn Pathol. 2020 Dec 9;15(1):139. doi: 10.1186/s13000-020-01058-z.
Pancreatic Solid Pseudopapillary Neoplasms (SPNs) are rare low-grade malignant tumors with a marked preponderance for young females. Objective was to describe the morphology, differential diagnosis, and prognosis of SPNs in patients under 20 years of age and present a detailed review of literature.
A total of 29 cases in patients under 20 years of age reported as SPN during the period January 2014 to December 2019, were included in the study. These included 19 resection specimens, 4 incision biopsies and 6 cases received as blocks for second opinion. Hematoxylin and eosin (H&E) slides as well as immunohistochemistry (IHC) slides of all cases were retrieved and reviewed by the authors. TFE3 and Progesterone Receptor were performed retrospectively.
Twenty-eight of the 29 patients were females. Ages of patients ranged from 12 to 19 years. Nineteen cases were resections. Tail was the commonest location. Mean tumor size was 9.5 cm. In 89.5% cases, tumor was confined to the pancreas. In 2 cases, distant metastasis was present. In 2 cases, extension beyond pancreas was seen. Solid and pseudopapillary areas were seen in all cases while other features were variable. Beta catenin and Cyclin D1 were positive in most cases while TFE3 was positive in 57% cases. Progesterone Receptor (PR) was positive in all 13 cases in which it was performed. Follow up was available in 14 patients. Follow up period ranged from 3 to 70 months. Twelve were alive and well without recurrence or metastasis while 2 were alive with recurrence and metastasis to liver and omentum respectively.
Although many studies on SPNs have been published, surgeons, oncologists and even pathologists in this part of the world are often not aware of these rare tumors leading to inaccuracies and delays in diagnosis. In addition, this paper focusses on the interesting observation that the majority of SPNs diagnosed in our department during study period occurred in patients under 20 years of age (29 versus 21 in patients over 20). However, clinico-epidemiological, morphologic and prognostic features were similar in both age groups. Possibility of SPNs should always be considered in case of pancreatic neoplasms occurring in patients under 20 years of age as well. We believe that this is a very interesting and helpful study for the clinicians as well as the pathologists.
胰腺实性假乳头状瘤（SPN）是一种罕见的低度恶性肿瘤，主要发生在年轻女性。本研究旨在描述 20 岁以下患者 SPN 的形态学、鉴别诊断和预后，并对文献进行详细回顾。
纳入 2014 年 1 月至 2019 年 12 月期间诊断为 SPN 的 29 例年龄小于 20 岁的患者，包括 19 例切除术标本、4 例切开活检和 6 例作为第二意见的组织块。作者对所有病例的苏木精和伊红（H&E）切片和免疫组织化学（IHC）切片进行了检索和复习。回顾性进行 TFE3 和孕激素受体检测。
29 例患者中 28 例为女性，年龄 12-19 岁。19 例为切除术标本，尾部是最常见的部位，肿瘤平均大小为 9.5cm。89.5%的病例肿瘤局限于胰腺，2 例存在远处转移，2 例肿瘤超出胰腺。所有病例均可见实性和假乳头状区域，其他特征不一。大多数病例β连环蛋白和 Cyclin D1 阳性，57%的病例 TFE3 阳性。孕激素受体（PR）在 13 例中均为阳性，其中 14 例获得随访。随访时间为 3-70 个月，12 例患者存活且无复发或转移，2 例患者存活且肝和网膜转移。
尽管已有许多关于 SPN 的研究，但该地区的外科医生、肿瘤学家甚至病理学家通常对这些罕见肿瘤认识不足，导致诊断不准确和延误。此外，本文还重点观察到，在研究期间，我们科室诊断的大多数 SPN 发生在 20 岁以下的患者（29 例比 20 岁以上的患者多 21 例）。然而，两组的临床流行病学、形态学和预后特征相似。对于 20 岁以下发生的胰腺肿瘤，也应始终考虑 SPN 的可能性。我们认为，这对临床医生和病理学家来说都是一项非常有趣和有益的研究。
