Solid pseudopapillary tumor of the pancreas with metastases to the lung and liver

Presented herein is the case of a 41-year-old woman who was admitted to Teikyo University Hospital with abdominal and back pain. Clinical examination revealed a large mass of the pancreas and multiple nodules in the liver. After surgical resection of the pancreatic and liver tumors, liver nodules recurred repeatedly, and a solitary mass lesion occurred in the right lung. Grossly, the pancreatic tumor was large and partially cystic. Histologically, small and uniform tumor cells proliferated, having solid and pseudopapillary patterns. These pathological findings enabled a pathological diagnosis of solid pseudopapillary tumor (SPT) of the pancreas to be made. The pathological appearance of the liver and lung tumors was similar to that of the pancreatic tumor. This is the first report of a case of pancreatic SPT that showed lung metastasis. It should be kept in mind that pancreatic SPT may take such an aggressive clinical course, although they are usually benign in nature.     

Cytology of solid and papillary epithelial neoplasms of the pancreas: a case report

A case of rare pancreatic tumor in a 39-yr-old woman is reported. Fine-needle aspiration biopsy was performed; frozen sections later allowed definite diagnosis. Cytologic features of the tumor are described, and histogenesis is discussed.     

Clinical, imaging, and cytopathological features of solid pseudopapillary tumor of the pancreas: a clinicopathologic study of three cases and review of the literature

Solid pseudopapillary tumors are rare pancreatic neoplasms of uncertain pathogenesis that rarely metastasize and usually occur in young women. We describe the clinical, imaging, and cytopathological features of solid pseudopapillary tumor of the pancreas. We reviewed the clinical presentation, imaging, morphologic/immunochemical features, and follow-up of three women (age range 26-44). Cases 1, 2, and 3 presented with abdominal wall abscess, multiple endocrine neoplasia, and solid/cystic mass in the pancreatic head, respectively, and computed tomography of abdomen revealed solid/cystic masses with heterogeneous enhancement in body, tail and head of the pancreas, respectively. Case 2 also exhibited a left adrenal mass. Case 3 underwent endoscopic ultrasound of the pancreas, which showed a complex solid/cystic mass with septations. Sampling consisted of fine-needle aspiration (percutaneous or endosonography-guided), and additionally, core biopsy of the pancreatic mass and adrenal lesion in case 2. Aspirates and core biopsy revealed vascular structures with attached monotonous neoplastic cells in papillary-like arrays. Tumor cells had bland nuclear features with grooves, cytoplasmic periodic acid Schiff-positive hyaline globules, and associated myxoid/stromal fragments. Immunochemistry expressed alpha-1-antitrypsin, alpha-1-antichymotrypsin, vimentin, and focal neuron-specific enolase. Cases 1 and 3 underwent pancreatectomy with follow-up consisting of yearly imaging and no recurrences. Case 2 proved metastatic disease to adrenal gland and no follow-up was available. In the setting of typical clinical and imaging findings, an accurate preoperative diagnosis of pancreatic solid pseudopapillary tumor can be established by aspiration cytology and immunochemistry with or without concomitant core biopsy, on the basis of which clinicians decide treatment. This tumor can behave in a malignant fashion.     

Solid pseudopapillary tumor: an invasive case report of primary ovarian origin and review of the literature

Solid pseudopapillary neoplasm occurring as a primary tumor outside the pancreas is a rare event. We report a case of an ovarian primary occurring with an ill-defined cystic mass in a 39-year-old woman. The morphologic and immunohistochemical features of the ovarian neoplasm described in this report are compatible with those of solid pseudopapillary neoplasm of the pancreas. Histologically, the tumor cells of the case we report infiltrate into the ovarian parenchyma. Because of the diagnosis is not clear before surgery, the patient had a reoccurrence two months after the operation in which laparoscopic simple ovarian cystectomy and part ovarian tissue removal, followed by the right salpingo-oophorectomy. The case herein confirms that solid pseudopapillary neoplasm of the ovary belongs to the class of low-grade malignant tumor with certain invasiveness. The diagnosis should be taken into serious consideration in order to avoid missed diagnosis and delay treatment. Through this case we have a better understanding of the biological behavior of solid pseudopapillary neoplasm of the ovary.     

儿童胰腺实性假乳头状瘤多排螺旋CT表现

目的:探讨儿童胰腺实性假乳头状瘤（SPTP）的多排螺旋CT（MDCT）表现。 方法:回顾性分析经手术病理证实为SPTP的10例患儿临床及MDCT资料,分析总结其特征表现。 结果:10例患儿均为女性,SPTP均为单发病灶,位于胰腺头颈部3例,胰腺体尾部7例。肿瘤最大径为2.5~13.8 cm,平均值为5.94 cm。8例呈类圆形,2例呈分叶状。10例均边界清晰,8例有完整包膜。平扫1例肿瘤呈均匀低密度,增强无明显强化;其余9例呈囊实性,8例以实性为主,1例囊实性比例相当,其中1例可见包膜下弧形钙化,实性成分动脉期呈轻度强化,强化程度低于正常胰腺组织,门脉期呈渐进性不均匀强化,囊性成分无强化。1例肿瘤出现肝内胆管和主胰管轻度扩张,1例出现主胰管轻度扩张。所有肿瘤均未见肝脏及淋巴结转移。 结论:儿童SPTP好发于女性,其CT表现具有一定的特征性,对诊断和鉴别诊断具有一定意义。     

Pancreatic solid pseudopapillary neoplasm

Pancreatic solid pseudopapillary neoplasm (SPN) is a rare low grade malignant tumour. Distinguishing this entity from other pancreatic neoplasms is critical for therapeutic decision making and prognostication. It predominantly affects young female patients <40 years of age, with excellent clinical outcome following surgical removal. The gold standard diagnostic test is cytopathological or histopathological assessment of fine needle aspirate. There are two main difficulties with this. First, SPN can present with morphological and immunohistochemical appearances that can closely mimic other pancreatic tumours, in particular, pancreatic neuroendocrine tumour (NET). Second, the amount of diagnostic material from fine needle aspiration can be limited. Here, we present a cytopathological case with both challenges during the pre-operative investigation of SPN. The case exemplifies the importance of combining morphological features with a targeted panel of immunohistochemistry to arrive at the diagnosis.     

Solid pseudopapillary neoplasms ofthe pancreas: clinicopathologic features and surgical treatment of 19 cases

Solid pseudopapillary neoplasms (SPNs) of the pancreas are very rare neoplasms. The present study is to summarize our experience of the diagnosis, surgical treatment and prognosis of SPNs. The clinical data of 19 cases that underwent surgery for pathologically confirmed SPNs, admitted in our hospital from Mar. 2007 to Mar. 2013, were analyzed retrospectively. The clinicopathologic feature, surgical treatment and prognosis were described in detail. The 19 patients were 17 females and 2 males, with a median age of 29 years. All patients had curative resections, including eight distal pancreatectomies with splenectomy, four spleen-preserving distal pancreatectomies, two pancreaticoduodenectomies, two pylorus-preserving pancreaticoduodenectomies, two duodenum-preserving pancreatic head resections and one central pancreatectomy. The tumors were 6.3 cm in diameter on average, and were mostly located in the body or tail of the pancreas (63.2%). Pathologically, the tumors contained a mixture of solid, cystic, and pseudopapillary patterns in various proportions. None of the patients had lymph nodes metastases and local invasion. All patients were alive and disease-free at a median follow-up of 38.4 months. SPNs are rare neoplasms, typically affecting young women without notable symptoms, with a low malignant potential but excellent prognosis. Radical surgical resection with clear margins is the treatment of choice.     

Solid‐pseudopapillary neoplasm of the pancreas: Awareness of unusual clinical presentations and morphology of the clear cell variant can prevent diagnostic errors

Solid‐pseudopapillary neoplasm (SPN) of the pancreas is a rare neoplasm predominantly reported in young women. It classically presents as a large tumor with cystic and solid components. The major differential diagnosis includes pancreatic neuroendocrine tumor (PanNET). This study presents our experience with this tumor with emphasis on the morphologic features of the clear cell variant of SPN. Fifteen histologically confirmed SPN were identified in our files. Endoscopic ultrasound‐guided fine needle aspirations (EUS‐guided FNA) were performed in 8/15 cases. Patients' demographics, cytohistologic correlation and tumor characteristics were evaluated. Eleven of the 15 subjects were female and four were male with an age range of 17–73 years. Twelve SPN were located in the pancreatic body/tail, and three in the head. Tumor size ranged from 1.5 to 8.5 cm and 11 were solid. Of the eight EUS‐guided FNA, four were diagnosed as SPN, two as SPN vs. PanNET, one as malignant with signet ring features, and one was nondiagnostic. Immunohistochemistry was performed on six/eight FNA cell blocks and 13/15 surgical specimens. Two of the 15 cases were classified as clear cell variants of SPN. Our study shows that SPN may occur in males and older adults, and present as a small or solid tumor. The clear cell variant of SPN, characterized by vacuolated cytoplasm and signet cell morphology, may pose a diagnostic challenge on FNA. Awareness of the wide spectrum of SPN clinical presentations, the morphology of its clear cell variant and the appropriate use of ancillary immunohistochemistry can prevent diagnostic errors. Diagn. Cytopathol. 2013;41:889–895. © 2013 Wiley Periodicals, Inc.     

Membrane loss and aberrant nuclear localization of E-cadherin are consistent features of solid pseudopapillary tumour of the pancreas. An immunohistochemical study using two antibodies recognizing different domains of the E-cadherin molecule

Aim:  To examine the expression of E-cadherin in solid pseudopapillary tumours (SPT) of the pancreas using two monoclonal antibodies recognizing two different domains of the E-cadherin molecule.
Methods and results:  Twenty cases of SPT were collected and a tissue microarray (TMA) constructed. The TMA was stained with commercially available antibodies to E-cadherin and β-catenin. All 20 cases displayed nuclear β-catenin as well as aberrant E-cadherin expression. With the antibody that stains the cytoplasmic domain of E-cadherin (clone 36, BD Transduction Laboratories), all 20 cases demonstrated nuclear E-cadherin reactivity, whereas with use of the antibody that recognizes the extracellular domain (clone 36B5, Vector Laboratories), no reactivity was observed in any of the cases.
Conclusion:  This study shows that aberrant β-catenin and E-cadherin protein expression occurs in 100% of SPT, is probably linked mechanistically to β-catenin nuclear localization, and two distinct patterns of E-cadherin immunoreactivity are seen in SPT: nuclear (with the antibody against the cytoplasmic domain), or immunonegativity (complete loss) when stained with the antibody for the E-cadherin extracellular fragment.     

Hepatoid carcinoma of the pancreas penetrating into the gastric cavity: a case report and literature review

A 79-year-old Japanese woman was admitted to our hospital for treatment of a pancreatic tumor measuring approximately 7 × 5 cm. The tumor had invaded the left adrenal gland and gastric wall and had penetrated into the gastric cavity. Surgical resection was performed. The tumor was composed of a brown to whitish solid area and a zone of hemorrhage, necrosis, and cystic degeneration resembling the gross features of solid pseudopapillary tumor (SPT). Histologically, the tumor showed a heterogeneous growth pattern with a combination of seat-like, trabecular, papillary and hemorrhagic-necrotic areas in various proportions. The differential diagnoses first considered were acinar cell carcinoma, neuroendocrine carcinoma and SPT with malignant transformation. Immunohistochemistry showed tumor cells were negative for pancreatic exocrine enzymes and endocrine markers. Tumor cells diffusely expressed cytokeratin 19, alpha-fetoprotein, carcinoembryonic antigen and glypican-3, but lacked vimentin or β-catenin expression. Small proportions of tumor cells expressed hepatocyte paraffin-1. Although typical morphological features of well-differentiated hepatocellular carcinoma (HCC) were not distinctly apparent, the tumor morphology partly resembled poorly differentiated HCC. Given these findings and considerations, the tumor was finally diagnosed as poorly differentiated hepatoid carcinoma of the pancreas.     

Duct-acinar-islet cell tumor of the pancreas

A case of a rare pancreatic tumor, duct-acinar-islet cell tumor is presented. The tumor was incidentally found in the pancreatic body on computed tomography of a 21 year old male suffering from mumps. It was well demarcated from surrounding pancreas, and spherical in shape, measured 2.5 cm in diameter. Histologic and immunohistochemical examinations showed the tumor to consist of three distinct cell populations: duct, acinar and islet cells. Small cell nests consisting of these cellular components, either solely of one cell type or mixed of the three cell types, were separated by broad desmoplastic stroma. Islet (endocrine) cells, which were most predominant, were arranged in a tra-becular pattern or small cell nests. Most of them were positive for glucagon, and a few cells expressed insulin, somatostatin, serotonin or pancreatic polypeptide. These cells were distributed randomly within the cell nests. Ducts, some of which contained goblet cells, were found among the endocrine cell nests. Duct-islet complexes were also observed. The acinar cells were the least conspicuous component. They expressed pancreatic α-amylase. An electron microscopic examination revealed duct cells with intercellular attachments and interdigitations, endocrine cells containing secretory granules, and acinar cells with zymogen granules. No definite evidence suggesting malignancy could be obtained.     

胰腺实性假乳头状肿瘤临床病理分析

目的 探讨胰腺实性假乳头状瘤（solid-pseudo-papillary tumor of pancreas,SPT）的临床病理学特点及其生物学行为。方法 对6例SPT临床及病理资料进行回顾性复习,光镜观察其形态学特征,并采用免疫组化S-P法检测肿瘤细胞α-AT等9种抗体的表达,并对其中1例做AB／PAS染色。结果 6例SPT中5例为女性、1例为男性,平均年龄34岁,瘤体平均直径9．3cm;光镜下瘤细胞圆形,大小较一致,无明显异型性,排列成实性片状区和假乳头状结构,假乳头轴心黏液变性,囊性区常见出血、坏死;其中1例除上述特征外还出现了大量的印戒细胞样瘤细胞,这些瘤细胞AB／PAS染色阴性,CEA阴性,S-100蛋白阴性,随访至今已4年余,无复发及转移。2例浸润包膜及周围胰腺组织;6例中3例有随访的均健在且无复发及转移。免疫组化检测：6例Vim阳性（6／6）,4例α-AT阳性（4／6）,3例Syn阳性（3／6）,1例CK弱阳性（1／6）、PR阳性（1／6）,ER阴性（o／6）,EMA阴性（0／6）,CEA阴性（0／6）,S-100蛋白阴性（0／6）。结论 SPT好发于年轻女性;组织形态学特征为瘤细胞大小一致,排列成实性片状区与独特的假乳头状结构,偶见瘤细胞呈印戒细胞样,间质可黏液变性;该肿瘤生物学行为交界性或恶性潜能未定的肿瘤,即使出现包膜及周围胰腺浸润,预后亦较好。     

Papillary-cystic tumor of the pancreas

A case of papillary cystic tumor (PCT) of the pancreas in a 40-yr-old woman is reported. This rare neoplasm was discovered fortuitously by a CT-scan examination for a palpable abdominal uterine leiomyoma. Percutaneous fine-needle aspiration (FNA) of the pancreatic mass was performed under CT-scan guidance. Cytologic examination of the material gave the diagnosis of PCT, which was confirmed by histologic examination of the resected tumor. Immunohistochemical staining showed a high degree of positivity to wide-spectrum anticytokeratin and anticytokeratin 20, and a weak positivity to anti-NSE antibody. In addition, the tumor cells were highly reactive for progesterone antibody, while they were negative for estrogen. These findings suggest a ductal origin for PCT, and also suggest that sex hormones may play a role in its growth, but not in its genesis. We emphasize the value of FNA cytologic features in the diagnosis of these rare tumors. Diagn Cytopathol 1996;15:398–402. © 1996 Wiley-Liss, Inc.