Solid pseudopapillary tumor of the pancreas and concomitant urogenital malformations in a young woman

Guan, Zhiwei; Sun, Long; Wang, Yanqiu; Xu, Baixuan

doi:10.1186/1746-1596-8-35

Cited by 9 publications

(7 citation statements)

References 31 publications

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“…In SPN a combination of solid and pseudopapillary growth pattern is frequently present but these tumors are usually immunoreactive for CD10, vimentin, chromogranin A and nuclear β-catenin [16,17]. Although a clear cell variant and a chromogranin A negative SPN has already been reported, our case did not show nuclear β-catenin nor vimentin expression, therefore ruling out SPN [16,18]. MCN is not connected to the pancreatic duct system and contains epithelium (in rare cases squamous epithelium), surrounded by an ovarian-like stroma that stains positive for progesterone receptor, inhibin, CEA, and chromogranin A [19].…”

Section: Discussionmentioning

confidence: 67%

Case report: intraductal tubulopapillary neoplasm of the pancreas with unique clear cell phenotype

et al. 2014

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Intraductal tubulopapillary neoplasms of the pancreas are very rare tumors characterized by intraductal tubulopapillary growth, ductal differentiation, scant intracellular mucin production and cellular dysplasia. Here, we report the first case of an intraductal tubulopapillary neoplasm of the pancreas with clear cell morphology. The tumor was detected during the diagnostic work-up of acute pancreatitis in a 43- year old female. Histological examination revealed a tumor with the typical architecture of an intraductal tubulopapillary neoplasm of the pancreas with tumor cells showing abundant clear cytoplasm and Di-PAS negativity. Immunohistochemistry revealed positivity for Pan-CK, CK7, CK8/18, MUC1, MUC6, carbonic anhydrase IX, CD10, EMA, β-catenin and e-cadherin. Sanger sequencing did not detect mutations for β-catenin, BRAF, KRAS, PIK3CA and GNAS. Altogether, histology, immunohistochemical expression profile (MUC1+, MUC6+, MUC2-, MUC5AC-, thrypsin-, chymotrypsin-, CDX2-) and sequencing results led to the diagnosis of intraductal tubulopapillary neoplasm. However, the neoplasm consisted of cells showing abundant clear cytoplasm, a morphological pattern not being described so far in the current classification of pancreatic intraductal neoplasms. Potential differential diagnosis and the molecular basis of clear cell morphology are discussed. In conclusion, we consider this tumor as intraductal tubulopapillary neoplasm of the pancreas with unique clear cell phenotype. After surgery and without adjuvant therapy, the patient’s clinical course has been uneventful for over two years now.Virtual slidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1051828790117127

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Section: Discussionmentioning

confidence: 67%

Case report: intraductal tubulopapillary neoplasm of the pancreas with unique clear cell phenotype

et al. 2014

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“…Furthermore, the most common location in adults is body or tail, whereas, in children, it is the head of the pancreas; however, in our child, it was in the tail. [ 4 ]…”

Section: Discussionmentioning

confidence: 99%

“…The association between these anomalies and SPN needs to be further confirmed, because only one case of such kind has been reported in a 25-year-old female who presented with SPN and solitary kidney and uterus didelphys. [ 4 ] Till date, more than fifty cases of SPN have been reported in the pediatric population, but none of them had associated urogenital anomalies.…”

Section: Discussionmentioning

confidence: 99%

Solid pseudopapillary neoplasm of the pancreas in a pediatric patient with associated urogenital anomalies

Sharma

Sarin

Luthra

2019

J Indian Assoc Pediatr Surg

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“…Generally, there is no specific clinical syndrome associated with SPT of the pancreas. However, SPT has been reported to occur occasionally in patients with congenital pancreatic anomalies (pancreatic dorsal agenesis, pancreatic divicum), congenital urogenital anormalies (solitary kidney and uterus didelphys) and congenital mental anomaly (Mulvihill-Smith syndrome) [ 6 ]. Here we present a case of pancreatic SPT with concomitant cervical cancer.…”

Section: Discussionmentioning

confidence: 99%

Solid pseudopapillary tumor of the pancreas in a patient with cervical cancer: relation of E-cadherin/ -catenin adhesion complex in their carcinogenesis

Vijay

Ram

Mathew

et al. 2015

Journal of Surgical Case Reports

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Solid pseudopapillary tumor (SPT) of the pancreas is one of the most uncommon histotypes of all exocrine pancreatic neoplasms. Disorganization of E-cadherin and β-catenin mutations, two key components of the Wnt signal transduction pathway, has been implicated in the development of SPT, but not other pancreatic tumors. Loss of E-cadherin/β-catenin proteins and tyrosine phosphorylation of E-cadherin/β-catenin have been postulated in cervical carcinogenesis and cancer invasion. A 38-year-old married woman, who had undergone brachytherapy, radiotherapy and chemotherapy for cervical cancer in Philippines in 2011, was admitted to our hospital after follow-up CT scan of abdomen in 2012 revealed a lesion in the tail of pancreas. The patient underwent distal pancreatectomy and splenectomy. The pathological diagnosis was SPT of pancreas. We suspect that the concurrent SPT pancreas and cervical cancer in this woman were triggered by a primary insult, a process in which E-cadherin/β-catenin/Wnt-signaling pathway played important roles.

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Solid pseudopapillary tumor of the pancreas and concomitant urogenital malformations in a young woman

Cited by 9 publications

References 31 publications

Case report: intraductal tubulopapillary neoplasm of the pancreas with unique clear cell phenotype

Case report: intraductal tubulopapillary neoplasm of the pancreas with unique clear cell phenotype

Solid pseudopapillary neoplasm of the pancreas in a pediatric patient with associated urogenital anomalies

Solid pseudopapillary tumor of the pancreas in a patient with cervical cancer: relation of E-cadherin/ -catenin adhesion complex in their carcinogenesis

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