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.     

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.     

Accessory spleen presenting as a mass in the tail of the pancreas

In this case report, we describe an accessory spleen that presented as a mass in the tail of the pancreas and mimicked a neoplasm. Intrapancreatic accessory spleen have a relatively high prevalence and can be mistaken for tumors. We present a case of intrapancreatic accessory spleen in a 40-year-old man, which was discovered incidentally during a workup for an aortic dissection. Computerized axial tomography and magnetic resonance imaging scans demonstrated a hypervascular mass in the tail of the pancreas. The clinical and radiological differential diagnosis included pancreatic mucinous cystic neoplasm, pancreatic endocrine neoplasm, solid pseudopapillary tumor, ductal adenocarcinoma, and metastasis. After a distal pancreatectomy was completed, microscopic examination revealed heterotopic splenic tissue.     

Clinical Strategy for the Management of Solid Pseudopapillary Tumor of the Pancreas: Aggressive or Less?

Objective: To further delineate the clinicopathological and radiological features of solid pseudopapillary tumor (SPT) of the pancreas and summarize the surgical therapy strategy for this tumor. Methods: A retrospective review of 18 pathologically confirmed cases of SPT was performed and the clinical and pathological features, radiological findings and surgical interventions were analyzed. Results: The patients included 17 females and 1 male with a median age of 23 years. The median diameter of the lesions was 8.0 cm. Abdominal pain was the predominant complaint (8/18). The rest of the patients were asymptomatic and presented with a pancreatic mass detected incidentally. Radiological study revealed a well-demarcated mass which was composed of a solid-cystic portion. On post-contrast CT, the solid portions could be enhanced whereas the cystic parts remained unenhanced. With the preoperative diagnosis of SPT in 11 patients and pancreatic cyst, benign or malignant pancreatic tumor in the rest, pancreatic tumor resection was successfully completed. Surgical exploration findings, pathological characteristics and good prognosis of the patients with SPT, indicated its low-grade malignant potential. Conclusion: In combination with clinical findings, radiological features of SPT may help to make the correct diagnosis and differentiation from other pancreatic neoplasms. Once diagnosed, given the excellent prognosis and low-grade malignancy, less aggressive surgical resection of the primary lesion is proposed.     

A spindle cell predominant pancreatic solid-pseudopapillary tumor

A hitherto unrecognized variant of solid-pseudopapillary tumor (SPT) of the pancreas is reported. The tumor presented in the pancreatic tail of a 44-year-old female patient. It was a well-defined, solid nodule measuring 25 mm in diameter, with homogenous tan gray cut surface. Histologically, the neoplasm was mostly composed of sheets of spindle cells. No cellular atypia and mitosis was identified. The periphery of the tumor showed typical feature of SPT. Immunohistochemically, the tumor cells were positive for vimentin, CD10, CD56, beta-catenin, and alpha1-antichymotrypsin, but negative for cytokeratin, chromogranin, synaptophysin and S-100 protein. Ultrastructurally, the tumor showed a few acinar spaces with microvilli between tumor cells. This case is peculiar in that the tumor did not show gross cystic change and predominantly consists of spindle shaped tumor cells, so may cause difficult diagnostic problem.     

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 neoplasm, pancreas type, presenting as a primary ovarian neoplasm

Solid pseudopapillary neoplasm has historically been associated with the pancreas, categorized as a tumor of low malignancy. Recently, solid pseudopapillary neoplasm was reported to arise as a primary ovarian tumor in 3 women. We report a fourth case identified in a 48 year-old woman with an 8-cm left ovarian mass. A left salpingo-oophorectomy was performed. Microscopic examination demonstrated a predominately cystic neoplasm comprised of solid nests of cells with an epithelioid to plasmacytoid appearance, associated with blood vessels, hemorrhage, and degenerative changes, that is, pseudopapillary structures. The tumor cells stained focally for pancytokeratin, progesterone receptor, and CD57 with diffuse nuclear expression of β-catenin. Ki-67 was 5% to 10%. Synaptophysin, inhibin, and E-cadherin stains were negative. Clinical and radiologic follow-up of our patient demonstrated no pancreatic lesions. This is a rare report of a primary ovarian solid pseudopapillary neoplasm. Prolonged follow-up is needed to determine how this case will fare clinically.     

Solid pseudopapillary tumor of the pancreas: a review of salient clinical and pathologic features

Solid pseudopapillary tumor (SPT) of the pancreas is a rare tumor of uncertain histogenesis characterized, as the name suggests, by a cystic and solid pattern of growth with formation of pseudopapillae. Accounting for only a small percentage of pancreatic neoplasms, SPT occurs primarily in young women, although cases in older patients and men have been reported. The tumor is thought to have low-grade malignant potential, as the majority of the cases are cured by simple but complete surgical resection. Knowledge of the unique morphologic and demographic characteristics of this neoplasm is essential for accurate diagnosis. Herein, we review the clinical and pathologic features, which can help separate SPTs from other primary pancreatic tumors.     

胰腺静脉的临床解剖学研究

在50具成人尸体上观察研究了胰腺静脉。胰头、胰颈的静脉血由胰十二指肠上下静脉和胰颈静脉引流。胰体下1/3由胰下静脉引流;上2/3由5～10条小静脉引流入脾静脉。胰尾多见1～4条小静脉,大都汇入脾静脉。胰腺静脉均汇入门静脉系。文中对胰腺静脉的临床应用进行了探讨。     

Serous cystic neoplasm in an intrapancreatic accessory spleen

Serous cystic neoplasm (SCN) of the pancreas is a benign epithelial neoplasm, except in extremely rare malignant cases. Development of SCN in tissues other than the pancreas has been never reported. Here we present the first reported case of SCN in an intrapancreatic accessory spleen (IPAS). A 54‐year‐old female patient with von Hippel‐Lindau (VHL) syndrome was found to have pancreatic tail mass. Pathologically the 25‐mm solid mass was an IPAS showing proliferation of clear cuboidal tumor cells without atypia, forming numerous small cysts. The tumor cells were rich in cytoplasmic glycogen and distributed in the splenic tissue almost diffusely. Immunohistochemically, tumor cells were positive for cytokeratins, MUC6, and neuron‐specific enolase, and negative for neuroendocrine markers. From these findings, we diagnosed the lesion as SCN in IPAS. This tumor is suggested to develop as a VHL‐associated SCN from coexisting pancreatic tissue in IPAS rather than as a metastatic tumor.     

The first experience of robot assisted spleen-preserving laparoscopic distal pancreatectomy in Korea

Spleen-preservation has recently been emphasized in benign and borderline malignant pancreatic diseases requiring distal pancreatectomy. Reports to suggest that laparoscopic distal pancreatectomy is feasible and safe have been increasingly published. Robotic surgical system has been introduced and is expected to provide unique advantages in laparoscopic surgery. However, robot-assisted pancreatic surgery has not yet been performed by many surgeons. A 45-year-old female patient with abdominal discomfort was found to have pancreatic cyst in the body of the pancreas. Mucinous cystic tumor of the pancreas was the most favourable preoperative diagnosis. She underwent spleen-preserving laparoscopic distal pancreatectomy by using da Vinci surgical robot system. Splenic artery and vein were so tightly adherent to the pancreatic cyst that segmental resection of splenic vessels was required. Postoperative course was uneventful. She was able to come home in 5 days after surgery. Postoperative follow up color doppler ultrasound scan, taken on 2 weeks after surgery, showed minimal fluid collection around surgical field and no evidence of splenic infarction with good preservation of splenic perfusion. Robot-assisted spleen preserving distal pancreatectomy is thought to be feasible and safe. Several unique advantages of robotic system are expected to enhance safer and more precise surgical performance in near future. More experiences are mandatory to confirm real benefit of robot surgery in pancreatic disease.     

Unusual pancreatic tumor resembling solid and cystic tumor, with somatostatin production and aberrant crystalloid structures.

An unusual pancreatic tumor in a middle-aged man is reported. The tumor was a large encapsulated mass with necrosis and hemorrhage, and microscopically, it showed a predominant microcystic pattern with solid pattern areas. The clinical and histological features of this tumor were similar to those of solid and cystic tumor. Immunohistochemical studies showed that the tumor was positive for alpha 1-AT, NSE, CA 19-9 and somatostatin. Electron microscopy studies showed neurosecretory granules, intercellular junctions, annulate lamellae and aberrant crystalloid structures. These findings indicated that the tumor had differentiated into acinar, ductal and endocrine cells, suggesting that solid and cystic tumor originates from multipotential immature cells.     

Non-cystic solid-pseudopapillary tumor of the pancreas showing nuclear accumulation and activating gene mutation of beta-catenin

Solid-pseudopapillary tumor (SPT) is an unusual pancreatic neoplasm that is characterized by a mixture of solid and cystic components and a fibrous capsule. Recently, the tumorigenesis of SPT has been reported to be associated with gene mutations of beta-catenin, which is a molecule participating in the Wnt signaling pathway. Reported herein is the case of a 53-year-old woman with SPT. The tumor, approximately 3 cm in diameter in the pancreas body, had a clear margin and central calcification but had neither a cystic component nor fibrous capsule. Several lines of pathological findings in the surgically resected specimen indicated SPT: (i) pseudopapillary proliferation of eosinophilic polygonal cells with oval nuclei; (ii) positive expression of several marker molecules indicating differentiation into acinar and endocrine cells; and (iii) zymogen granule-like structures in the cytoplasm on electron microscopy. Further, the tumor cells had intense nuclear accumulation of beta-catenin and an activating mutation, (34)Gly(GGA) to Arg(AGA), in exon 3 of the beta-catenin gene, as previously reported in most SPT. These findings suggest that association of the beta-catenin phenotype with development of the rare phenotype of SPT, a non-cystic and unencapsulated tumor, is unlikely.     

Unusual Pancreatic Tumor Resembling Solid and Cystic Tumor, with Somatostatin Production and Aberrant Crystalloid Structures

An unusual pancreatic tumor in a middle-aged man is reported. The tumor was a large encapsulated mass with necrosis and hemorrhage, and microscopically, it showed a predominant microcystic pattern with solid pattern areas. The clinical and histological features of this tumor were similar to those of solid and cystic tumor. Immunohisto-chemical studies showed that the tumor was positive for ax-AT, NSE, CA 19 9 and somatostatin. Electron microscopy studies showed neurosecretory granules, intercellular junctions, annulate lamellae and aberrant crystalloid structures. These findings indicated that the tumor had differentiated into acinar, ductal and endocrine cells, suggesting that solid and cystic tumor originates from multipotential immature cells. Acta Pathol Jpn 41: 629-635, 1991.     

Cystic lesion mimicking intraductal papillary mucinous tumor arising in heterotopic pancreas of the stomach and synchronous intraductal papillary mucinous adenocarcinoma of the pancreas

This article presents the study of a 66-year-old man with an asymptomatic pancreatic mass detected incidentally 4 months earlier. A magnetic resonance imaging scan revealed 2 distinct cystic masses in the pancreas and the gastric antrum. Microscopically, the pancreatic lesion showed dilated cysts containing papillary structures lined by mucinous epithelium, which showed a loss of polarity, an increased nucleus-to-cytoplasm ratio, a prominent nucleolus, and high proliferation on immunostaining for Ki-67. The gastric lesion was composed of heterotopic pancreatic tissue surrounding a large dilated cyst that was lined with mucinous epithelium and contained a few intraluminal papillae.     

门静脉癌栓与门静脉血栓的多层螺旋CT影像研究

目的 探讨肝硬化合并肝癌、门静脉癌栓患者和肝硬化合并门静脉血栓患者的癌栓与血栓的MSCT影像特点及病变解剖部位的差异。方法 回顾性分析2011年5月—2016年5月北京大学深圳医院和2013年8月—2014年8月广东省海丰县彭湃纪念医院经临床诊断的18例肝硬化合并肝癌、门静脉癌栓患者(癌栓组)和12例肝硬化合并门静脉血栓患者(血栓组)的临床资料。所有患者行MSCT平扫,以及动脉期、门静脉期及延迟期3期增强扫描,并采用多平面重建(MPR)和5 mm厚度最大密度投影(MIP)重建技术对门静脉行3D重建。观察门静脉癌栓和血栓的影像特征、分布情况及侧支循环血管形成情况,比较癌栓和血栓在平扫和增强扫描各时相的密度差异。结果 癌栓组中,CT平扫12例呈低密度并血管增粗,6例呈等密度;CT增强扫描显示动脉期癌栓均呈不均匀强化、15例可见滋养血管影,门静脉期4例呈稍高密度、5例呈等密度、9例呈低密度,延迟期均呈低密度;18例门静脉癌栓均累及门静脉左或/和右支, 仅6例累及门静脉主干。血栓组中,CT平扫3例呈等密度, 3例呈低密度,6例呈稍高密度;CT增强扫描血栓均无强化,门静脉期及延迟期血栓部位无对比剂充盈;12例中,11例门静脉血栓累及门静脉主干,6例血栓延伸至左叶或/和右叶门静脉分支。门静脉癌栓和血栓在CT平扫和增强扫描的延迟期密度的差异均无统计学意义(P值均＞0.05),而增强扫描的动脉期和门静脉期,癌栓密度明显高于血栓,差异均有统计学意义(P值均＜0.05)。癌栓累及门静脉左或/和右支的概率明显高于血栓,而血栓累及门静脉主干的概率明显高于癌栓,差异均有统计学意义(P值均＜0.05)。结论 结合MSCT平扫及3D重建技术,能客观显示肝硬化患者门静脉癌栓与门静脉血栓的影像特点及其累及范围,能为病变诊断提供客观依据,从而指导临床选择合适的治疗方案。     

Histogenesis of solid pseudopapillary tumor of the pancreas: the case for the centroacinar cell of origin

Solid pseudopapillary tumor (SPT) is an unusual pancreatic neoplasm of low malignant potential that most frequently occurs in young women. The tumor is indolent, with long patient survival, even in the presence of extension into adjacent organs and metastases. Histologically, it is a solid and cystic tumor with a prominent vascular network and degenerative pseudopapillae formation. Despite its distinctive morphology and cytological features, its histogenesis is unclear. Herein, we report a case of solid pseudopapillary tumor in a 41-year-old female in which the tumor cells immunohistochemically and ultrastructurally suggest a centroacinar cell origin. The tumor cells and the normal centroacinar cells stained positive for alpha-antitrypsin (alpha-AT), CD10, cyclin D1 and NSE. Ultrastructural examination shows similarities in nuclear shape, nucleoli location and cytoplasmic contents between neoplastic cells and normal centroacinar cells of the pancreas. Based on both immunohistochemical and ultrastructural features, we propose that the centroacinar cell is the origin of SPT.     

Solid and cystic tumor of the pancreas in an adult male.

A solid and cystic tumor (SCT) was located at the head of the pancreas in a 43-year-old Japanese male, and pancreatoduodenectomy was performed on the suspicion of papillary carcinoma or cystadenocarcinoma of the pancreas. The lesion, which measured 4.5 x 4.5 x 4.0 cm, was clearly demarcated by connective tissue. The cut surface showed solid grayish-white areas with central cystic degenerative changes. The solid areas consisted of small round cells proliferating in a small solid or a pseudopapillary pattern. The tumor cells partially invaded the surrounding normal pancreatic parenchyma. Immunohistochemical studies revealed positive staining for alpha-1-antitrypsin and neuron-specific enolase, but no staining for known pancreatic hormones. Moreover, ultrastructural studies showed the absence of zymogen granules and the presence of anullate lamellae and neurosecretory granules. On the basis of these findings, a diagnosis of SCT of the pancreas was established. In order to clarify the histogenesis and biological behavior of the tumor, it is necessary to accumulate and analyze similar cases, an endeavor which in turn will contribute to the successful management of this disease.     

Epithelial inclusion cyst (epidermoid cyst) formation with epithelioid cell granuloma in an intrapancreatic accessory spleen

The histologic characteristics of a case of epidermoid cyst formation with an epithelioid cell granuloma that developed in intrapancreatic accessory spleen were investigated, with the aim of clarifying its origin as well as etiologic factors. The patient, a 48-year-old male, was found to have a cystic lesion in the tail of the pancreas and renal cell carcinoma (clear cell carcinoma) during a medical check up. The pancreatic mass appeared to be a so-called "mucinous tumor" on imaging, and combined resection of the body and tail of the pancreas and the spleen was performed together with a left nephrectomy. The lesion in the tail of the pancreas was then demonstrated to have accessory splenic tissue with cyst formation in its central region. The cystic wall was covered with stratified squamous epithelium and ductal epithelium with squamous metaplasia, and pancreatic islet cells were evident at various sites within the accessory spleen. Formation of epithelioid cell granuloma tissue was observed around the cysts. The epithelium of the cyst was positive for CA19-9 and negative for antibodies against mesothelial cells, whereas the pancreatic island cells were positive for insulin. These results suggested that cystic epithelium in the accessory spleen could be derived from pancreatic duct epithelium. Frequent recurrence of chronic inflammation and epitheloid cell granuloma formation may have resulted from an aberration of the ectopic remaining pancreatic tissue in the spleen.     

Choriocarcinoma involving the pancreas as first manifestation of a metastatic regressing mixed testicular germ cell tumor.

We describe an unusual case of metastatic choriocarcinoma of the pancreas arising from a regressing testicular mixed germ cell tumor that clinically mimicked a primary pancreatic tumor. A 54-year-old male presented with a 2-month history of progressive upper abdominal pain, weight loss, and jaundice. He also had a history of recurrent epididymitis associated with the presence of a right testicular mass shown to be cystic by ultrasound and stable for at least 10 years. A computed tomography scan showed an isolated 6 cm mass in the head of the pancreas. A pancreaticoduodenectomy was performed. Upon histological examination, the pancreatic tumor showed extensive hemorrhage and necrosis. In the viable area, the tumor was composed of an intimate mixture of mononuclear cytotrophoblast cells and multinucleated syncytiotrophoblasts with vascular invasion. These characteristic features led to the correct diagnosis on frozen section. The cytology of the tumor was nonspecific and suggested undifferentiated carcinoma of the pancreas. The trophoblastic origin of the tumor cells was confirmed by immunohistochemistry staining. The testicular mass showed a regressed mixed germ cell tumor of predominantly seminoma with focal teratoma but without a choriocarcinoma component. In conclusion, we present a rare and unusual case of a regressing testicular mixed germ cell tumor that presented as a primary pancreatic tumor. Cytological features of the pancreatic mass were not specific and raised the possibility of a primary undifferentiated carcinoma of the pancreas. Characteristic histological features of choriocarcinoma led to the correct diagnosis on frozen section. Subsequent resection of the testicular mass confirmed the presence of a cystic and scarring (regressing) mixed germ cell tumor but without evidence of choriocarcinoma.