scholarly journals Solid Pseudopapillary Neoplasm of the Pancreas: Clinical-Radiological-Pathological Characteristics of Four Pediatric Cases

2020 ◽  
Vol 04 (01) ◽  
pp. 01-07
Author(s):  
Zhongxin Yu

Solid Pseudopapillary Neoplasm (SPN) of the pancreas represents 1-3 % of all exocrine pancreatic tumors and is uncommon in children. We report four pediatric patients with SPN where each patient posed a unique diagnostic and therapeutic challenge. We describe these four cases with detailed clinical-radiological-pathological correlations. All patients are female, with a median age 13.5 years. Two patients presented with abdominal pain, one with jaundice and one with an incidental pancreatic mass on abdominal CT scan. Radiological studies included abdominal ultrasound, CT scan and MRI of abdomen. Pancreaticoduodenectomy was performed in three patients and laparoscopic distal pancreatectomy in one patient. Mean tumor size was 4.5 cm (ranged from 1.9 to 11.5 cm). All SPNs were benign on histological exam. One patient developed pancreatic insufficiency post-surgery. No tumor recurrence was observed over a mean follow up period of 1 year. We conclude that diagnosis of SPN in pediatric population can be challenging due to non-specific clinical findings, and surgical removal of the tumor is usually required for definitive histologic diagnosis and treatment. Most tumors are benign and recurrence is very rare.

2020 ◽  
Vol 50 (3) ◽  
Author(s):  
Ignacio Maldonado Schoijet ◽  
Javiera Vásquez Poblete ◽  
Sergio Álvarez Diaz ◽  
Javier González Moscoso

Background. Solid pseudopapillary neoplasm of the pancreas is an uncommon exocrine pancreatic tumor of epithelial origin, which represents only 1% to 2% of all pancreatic tumors and mainly affects young women. Objective. To describe clinical and imaging features of these rare entity in a symptomatic young woman. Case report. 41-year-old female patient, without morbid history, consulted for intermittent epigastralgia of one-month evolution. Abdominal ultrasound and magnetic resonance imaging demonstrated the presence of a solid-cystic mass of neoplastic appearance in the body-tail of the pancreas. The case was presented to the institutional hepato-biliary committee where surgical resection was decided. Discussion. Although solid pseudopapillary neoplasm of the pancreas are very uncommon pancreatic tumors, it is necessary to keep them within the differential diagnosis on the one hand due to their low but existing malignant potential and on the other hand due to their good prognosis with surgical treatment.


2020 ◽  
Vol 63 (6) ◽  
Author(s):  
Camilla de Laurentis ◽  
Julius Höhne ◽  
Claudio Cavallo ◽  
Francesco Restelli ◽  
Jacopo Falco ◽  
...  

2020 ◽  
Vol 154 (Supplement_1) ◽  
pp. S77-S77
Author(s):  
N C Jadhav ◽  
D L Gang

Abstract Casestudy: Pancreatic neuroendocrine neoplasms (PanNEN) are rare accounting for 2-5% of pancreatic tumors. Although mostly sporadic, 10-20% are associated with inherited syndromes, notably MEN-1, Von Hippel- Lindau disease, neurofibromatosis type 1, and tuberous sclerosis (TS). When compared to sporadic cases, PanNEN in hereditary syndromes occur at a younger age, are often multifocal, cystic, and may show characteristic microscopic patterns. TS is an autosomal dominant multi-system disorder with mutations involving TSC1 or TSC2 genes which function as tumor suppressors by inhibiting mTORC1 kinase. PanNEN is observed in 1.5-1.8% of patients with TS and no surveillance guidelines for the assessment of pancreatic lesions are established. Compared to other syndromes, PanNEN associated with TS are solitary. To our knowledge, only two cases of multifocal PanNEN in TS patients have been reported. We present a case of a 67-year-old gentleman with a history of TS also affecting two daughters. He presented to the emergency department with severe abdominal pain. Abdominal ultrasound suggested acute appendicitis and an incidental 2.0 cm solid lesion was noted in the head of the pancreas. Follow-up MRI revealed two additional non-cystic masses in the pancreatic tail. Endoscopic ultrasound-guided biopsy of a tail lesion revealed monomorphic tumor cells with stippled chromatin without cytologic atypia. Immunohistochemical staining was positive for synaptophysin and chromogranin. Ki-67 labelling index was under 1%. Diagnosis of a well-differentiated neuroendocrine tumor (G1) was made. The patient denied symptoms of the carcinoid syndrome and no biologically active hormones were detected. Gallium PET scan revealed multiple foci of radiotracer uptake throughout the pancreas in addition to those described on MRI. Although PanNEN are rare in TS, malignant behavior has been reported. This case reinforces the importance of early detection through active surveillance, especially as surgical options may be limited in multifocal disease.


2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Daishi Naoi ◽  
Koji Koinuma ◽  
Hideki Sasanuma ◽  
Yasunaru Sakuma ◽  
Hisanaga Horie ◽  
...  

Abstract Background Familial adenomatous polyposis (FAP) is characterized by the presence of hundreds to thousands of colonic polyps, and extracolonic manifestations are likely to occur. Pancreatic tumors are rare extracolonic manifestations in patients with FAP, among which solid-pseudopapillary neoplasm (SPN) are extremely rare. We report here a patient with an SPN of the pancreas found during the follow-up of FAP. Case presentation A 20-year-old woman was diagnosed with FAP 3 years previously by colonoscopy which revealed less than 100 colonic polyps within the entire colon. She complained of left upper abdominal pain and a 10-cm solid and cystic pancreatic tumor was found by computed tomography scan. Solid and cystic components within the tumor were seen on abdominal magnetic resonance imaging. Simultaneous laparoscopic resection of the distal pancreas and subtotal colectomy was performed. Histopathological findings confirmed the pancreatic tumor as an SPN without malignancy. Abnormal staining of beta-catenin was observed by immunohistochemical study. Multiple polyps in the colorectum were not malignant. Molecular biological analysis from peripheral blood samples revealed a decrease in the copy number of the promoter 1A and 1B region of the APC gene, which resulted in decreased expression of the APC gene. Conclusions A rare association of SPN with FAP is reported. The genetic background with relation to beta-catenin abnormalities is interesting to consider tumor development. So far, there are few reports of SPN in a patient with FAP. Both lesions were treated simultaneously by laparoscopic resection.


2017 ◽  
Vol 10 (3) ◽  
pp. 317-320 ◽  
Author(s):  
Seiichiro Tada ◽  
Taku Iida ◽  
Takayuki Anazawa ◽  
Shintaro Yagi ◽  
Satoru Seo ◽  
...  

2020 ◽  
Author(s):  
Dawei Yang ◽  
Xiaofang Yang ◽  
Yang Li ◽  
Peige Zhao ◽  
Rao Fu ◽  
...  

Abstract Background Lung cancer (LC) remains the deadliest form of cancer globally. While surgery remains the optimal treatment strategy for individuals with early-stage LC, what the metabolic consequences are of such surgical intervention remains uncertain. Methods Negative enrichment-fluorescence in situ hybridization (NE-FISH) was used in an effort to detect circulating tumor cells (CTCs) in pre- and post-surgery peripheral blood samples from 51 LC patients. In addition, targeted metabolomics analyses, multivariate statistical analyses, and pathway analyses were used to explore surgery-associated metabolic changes. Results LC patients had significantly higher CTC counts relative to healthy controls with 66.67% of LC patients having at least 1 detected CTC. CTC counts were associated with clinical outcomes following surgery. In a targeted metabolomics analysis, we detected 34 amino acids, 164 lipids, and 24 fatty acids. When comparing LC patients before and after surgery to control patients, metabolic shifts were detected via PLS-DA and pathway analysis. Further surgery-associated metabolic changes were identified when comparing LA and LB groups. We identified SM 42:4, Ser, Sar, Gln, and LPC 18:0 for inclusion in a biomarker panel for early-stage LC detection based upon an AUC of 0.965 (95% CI = 0.900–1.000). This analysis revealed that SM 42:2, SM 35:1, PC (16:0/14:0), PC (14:0/16:1), Cer (d18:1/24:1), and SM 38:3 may offer diagnostic and prognostic benefits in LC. Conclusions These findings suggest that CTC detection and plasma metabolite profiling may be an effective means of diagnosing early-stage LC and identifying patients at risk for disease recurrence.


Cancers ◽  
2021 ◽  
Vol 13 (16) ◽  
pp. 4170
Author(s):  
Satomi Tomida ◽  
Dayan B. Goodenowe ◽  
Teruhide Koyama ◽  
Etsuko Ozaki ◽  
Nagato Kuriyama ◽  
...  

The polyunsaturated fatty acid (PUFA) elongase, ELOVL5, is upregulated in breast cancer (BC) vs. adjacent normal tissue. We performed a comprehensive lipid metabolomic analysis of serum using high-resolution accurate mass spectrometry from two case-control studies that included non-BC, BC subjects pre-surgery, and BC subjects one-month post-surgery to determine if the metabolic signatures of over-active fatty acid elongation and other lipid changes could be detected in BC vs. non-BC subjects: study 1 (n = 48: non-BC, n = 69: pre-surgery BC); study 2 (blinded validation: n = 121: non-BC, n = 62: pre-surgery BC, n = 31: one month post-surgery). The ratio of the ELOVL5 precursor, linoleic acid (18:2) to a non-ELOVL5 precursor, oleic acid (18:1) was evaluated in multiple lipid pools (phosphatidylethanolamine (PtdEtn), phosphatidylcholine (PtdCho), lyso-PtdCho, and free fatty acids). This ratio was lower in pre-surgery BC subjects in all pools in both studies (p < 0.001). At one-month post-surgery, the 18:2/18:1 ratios increased vs. pre-surgery and were no longer different from non-BC subjects (p > 0.05 expect for lyso-PtdCho). In contrast to the elongation biomarkers, docosahexaenoic acid (22:6n-3) containing ethanolamine plasmalogen (EtnPls) species were observed to be further decreased in BC subjects one-month post-surgery vs. pre-surgery levels (p < 0.001). These results are consistent with the hypothesis that ELOVL5 is upregulated in BC tissue, which would result in the selective depletion of 18:2 vs. 18:1 containing lipid species. Surgical removal of the tumor removes the overactive ELOVL5 effect on serum lipids. In contrast, the low EtnPls levels do not appear to be caused by BC tumor activity and may be pre-existent and a possible risk factor for BC. These results indicate that it may be possible to screen for both breast cancer risk and breast cancer activity using a simple blood test.


Author(s):  
Joana Braga ◽  
Francesca Pereira ◽  
Cristiana Fernandes ◽  
Marinha Silva ◽  
Teresa Boncoraglio ◽  
...  

Splenosis is a benign condition which results from the self-implantation of splenic tissue on intra or extraperitoneal surfaces, after splenic trauma or splenectomy. Patients are usually asymptomatic but may present with varied symptoms related to the implantation site. The diagnosis is a challenge because abdominal splenosis can mimic several diseases, including neoplasm. The gold standard examination for its diagnosis is scintigraphy with 99mTc-labelled heat-denatured erythrocyte. When splenosis is found in an asymptomatic patient, surgical removal is not indicated. A 57-year-old male patient presented with sporadic epigastric pain and a suspected mass in the recto-sigmoid transition. Abdominal ultrasound, CT and MRI identified this mass, its characteristics and location, but failed to distinguish its nature. However, given the patient’s past history of splenectomy and because the mass showed a similar sign to that of the splenic parenchyma, a hypothesis of abdominal splenosis was raised, which was confirmed by scintigraphy with 99mTc-labelled heat-denatured erythrocyte. In this case, the diagnosis was obtained before the patient was subjected to more invasive procedures, which are associated with high morbidity, and, as in most cases, no targeted intervention was necessary.


2019 ◽  
Vol 43 (2) ◽  
pp. 4-14
Author(s):  
N. B. Gubergrits ◽  
N. V. Byelyayeva ◽  
A. Ye. Klochkov ◽  
G. M. Lukashevich ◽  
P. G. Fomenko ◽  
...  

The article presents a detailed review of the research results in the field of pancreatology published in 2018. Certain parts of the review are devoted to the pathogenesis, diagnostics, course of pancreatitis, its treatment, as well as autoimmune, hereditary pancreatitis, pancreatic pathology in children, as well as treatment. In studying the pathogenesis of pancreatitis, attention is paid to genetic markers of pancreatitis along with a role of bacterial overgrowth syndrome in the small intestine, both in terms of worsening of the course of pancreatitis and the lack of effectiveness of enzyme replacement therapy. The study of the role of alcohol abuse and smoking in the pathogenesis of pancreatic pathology is still in progress. Diagnostics is going on. Endosonography remains the most informative method. A number of studies have been devoted to the studying of exocrine and endocrine pancreatic insufficiency both upon pancreatitis, pancreatic tumors, and in functional dyspepsia and HIV infection. Autoimmune pancreatitis is increasingly being diagnosed, a number of studies are devoted to its diagnostics and treatment. Pancreatic diseases in children develop mainly on the background of genetic predisposition, while functional pancreatic insufficiency occurs in adult patients. The study of the peculiarities of the effect of enzyme replacement therapy continues. The immediate and remote results of the surgical treatment of pancreatic pathology are assessed.


2020 ◽  
Vol 24 (3) ◽  
pp. 132
Author(s):  
U. G. Kolbik ◽  
A. V. Gorustovich ◽  
Yu. I. Linnik ◽  
M. M. Shved ◽  
V. V. Drozdovskaya ◽  
...  

<p>This publication presents the clinical observation in a paediatric patient with haemangioma of the left stomach. The effectiveness of surgical treatment of this disease is also noted. We assess the challenges in diagnosis and the choice of treatment of this pathology in children and the effectiveness of surgical treatment disease.<br />Primary cardiac tumours are rare; vascular tumours and haemangiomas are rarer. The clinical picture of heart haemangioma is non-specific and varies as per its location and size. Echocardiography is the main diagnostic method for this disease. However, in most cases of preoperative diagnostic examination, the pre-surgery diagnosis is not confirmed. The final diagnosis of capillary haemangioma is established following immunohistochemical staining of the surgical material. Thus far, owing to the rare occurrence, a generally accepted tactic for the treatment of such patients with vascular heart tumours has not been developed.<br />This clinical case describes our experience of treating a rare pathology in children with unusual localisation as well as the rapid successful surgical removal of the tumour without complications and with a favourable post-surgery recovery period.</p><p>Received 14 May 2020. Revised 15 June 2020. Accepted 26 June 2020.</p><p><strong>Funding:</strong> The study did not have sponsorship.</p><p><strong>Conflict of interest:</strong> Authors declare no conflict of interest.</p><p><strong>Author contributions</strong> <br />Literature review: U.G. Kolbik, A.V. Gorustovich, Y.I. Linnik<br />Illustrations: U.G. Kolbik, I.V. Sakharov, V.V. Drozdovskaya <br />Drafting the article: U.G. Kolbik, A.V. Gorustovich, I.V. Sakharov<br />Critical revision of the article: I.V. Sakharov, M.M. Shved, Yu.I. Linnik<br />Surgical treatment: A.V. Gorustovich, M.M. Shved, U.G. Kolbik<br />Final approval of the version to be published: U.G. Kolbik, A.V. Gorustovich, Yu.I. Linnik, M.M. Shved, V.V. Drozdovskaya, <br />I.V. Sakharov, K.V. Drozdovski</p>


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