Intraductal tubulopapillary neoplasm of the pancreas with somatic BRAF mutation

Urata, Takahiro; Naito, Yoshiki; Nagamine, Michiko; Izumi, Yuichi; Tonaki, Ginga; Iwasaki, Hajime; Sasaki, Akira; Yamasaki, Akira; Minami, Nobuhiro; Yoshioka, Ritsuko; Kitada, Hideki; Takekuma, Yoshi; Yasuda, Hiroshi; Fukuda, Seiji; Yamaguchi, Hiroshi; Kuboki, Yuko; Furukawa, Toru; Hifumi, Michio

doi:10.1007/s12328-012-0342-1

Cited by 16 publications

(13 citation statements)

References 10 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Nevertheless, close clinical follow-up is considered essential to detect early recurrence. Fortunately, the prognosis of ITPN-associated invasive carcinoma is much better than that of traditional pancreatic 4 Kloppel et al, 6 Yamaguchi et al, 13 Reid et al, 28 Amato et al, 29 Urata et al, 36 Esposito et al, 38 Wu et al, 39 and Furukawa et al 40 ductal adenocarcinoma, even in patients with recurrent and metastatic disease. 6 The 5-year survival is more than 30%.…”

Section: Prognosis and Treatmentmentioning

confidence: 99%

“…Rare BRAF V600E mutations have also been reported. 36 Recent deep coverage, targeted next-generation sequencing was performed on 11 ITPN cases with a panel of 300 key cancer-associated genes and none of the known IPMN-associated genetic alterations were found in ITPN. 37 Two histone H3 methyltransferase genes, MLL2 and MLL3, each were mutated in 2 cases of ITPN.…”

mentioning

confidence: 99%

See 1 more Smart Citation

Intraductal Tubulopapillary Neoplasm of the Pancreas: An Update From a Pathologist's Perspective

Rooney

Shi

2016

Archives of Pathology &Amp; Laboratory Medicine

View full text Add to dashboard Cite

Context.-Intraductal tubulopapillary neoplasm (ITPN)is a rare intraductal epithelial neoplasm of the pancreas recently recognized as a distinct entity by the World Health Organization classification in 2010. It is defined as an intraductal, grossly visible, tubule-forming epithelial neoplasm with high-grade dysplasia and ductal differentiation without overt production of mucin. The diagnosis can be challenging owing to morphologic overlap with other intraductal lesions and its rarity. While recent advances in molecular genetic studies of ITPN have provided new tools to facilitate clinical diagnosis, the limited number of cases has yielded limited follow-up data to guide management.Objective.-To provide a clinical, pathologic, and molecular update on ITPN with respect to clinical presentation, imaging findings, histopathologic features, differential diagnosis, biological behavior, molecular characteristics, and treatment options.Data Sources.-Analysis of the pertinent literature (PubMed) and authors' research and clinical practice experience based on institutional and consultation materials.Conclusions.-Clinical presentation, imaging findings, histopathology, immunohistochemistry studies, molecular characteristics, prognosis, and treatment options of ITPN are reviewed. Important differential diagnoses with other intraductal neoplasms of the pancreas-especially intraductal papillary mucinous neoplasm-using histopathologic, molecular, and immunohistochemical studies, are discussed. Despite the recent progress, more studies are necessary to assess the biology and genetics of ITPN for a better understanding of the prognostic factors and treatment options.(Arch Pathol Lab Med. 2016;140:1068-1073; doi: 10.5858/arpa.2016-0207-RA) T he intraductal neoplasms of the pancreas are defined by the current 4th edition of the World Health Organization (WHO) classification as macroscopic epithelial neoplasms with ductal differentiation that grow in the pancreatic ductal system. The 2 entities included in this category are intraductal papillary mucinous neoplasm (IPMN) and intraductal tubulopapillary neoplasm (ITPN). Note that intraductal lesions with nonductal differentiation, such as acinar cell carcinoma, are not included in this category. Interestingly, before the current WHO classification, intraductal neoplasms of the pancreas were classified into 3 groups: IPMN, pancreatic intraepithelial neoplasia (PanIN), and intraductal tubular neoplasm.1,2 Intraductal tubular neoplasm was then further classified into intraductal tubular adenoma and intraductal tubular carcinoma, with intraductal tubular adenoma considered as the precursor of intraductal tubular carcinoma.3 Both IPMN and ITPN can be either cystic or solid-mass forming. The distinction between the intraductal neoplasms and PanIN is that PanIN is microscopic. On the contrary, by definition, both IPMN and ITPN are grossly and radiographically detectable lesions with an arbitrary minimal size criterion of 1 cm. Intraductal tubulopapillary neoplasms can have variable componen...

show abstract

Section: Prognosis and Treatmentmentioning

confidence: 99%

mentioning

confidence: 99%

Intraductal Tubulopapillary Neoplasm of the Pancreas: An Update From a Pathologist's Perspective

Rooney

Shi

2016

Archives of Pathology &Amp; Laboratory Medicine

View full text Add to dashboard Cite

show abstract

“…The present case did not harbor any somatic mutations in TP53, PIK3CA, KRAS, BRAF, or GNAS. Some cases of ITPN harbor somatic mutations in PIK3CA and BRAF [ 4 , 7 ]; therefore, further molecular analyses of more cases are warranted to clarify the impact of these mutations on the development and progression of ITPNs.…”

Section: Discussionmentioning

confidence: 99%

A Rare Case of Intraductal Tubulopapillary Neoplasm of the Pancreas Rupturing and Causing Acute Peritonitis

Umemura

Ishida

Nitta

et al. 2017

Case Rep Gastroenterol

View full text Add to dashboard Cite

An intraductal tubulopapillary neoplasm (ITPN) is a very rare pancreatic tumor. Here we report an extremely rare case of an ITPN rupturing and causing acute peritonitis. A 50-year-old woman presented with left flank pain and vomiting. A computed tomography (CT) scan revealed gigantic multilocular cysts in the pancreatic tail and massive fluid collection in the abdominal cavity. The serum, urine, and abdominal fluid amylase levels were highly elevated, so she was conservatively treated with intraperitoneal drainage and antibiotics for a diagnosis of ruptured pancreatic cysts. After this patient recovered, a CT scan revealed a 2-cm low-density mass located in the body of the pancreas. This was diagnosed as a pancreatic ductal adenocarcinoma of the pancreatic body with an intraductal papillary mucinous neoplasm, and a distal pancreatectomy was performed. The tumor was composed of cuboidal high-grade dysplastic cells proliferating in a tubulopapillary growth pattern without mucin production. An immunohistochemical examination revealed that the tumor cells were positive for MUC1 and CK7, but negative for MUC5AC. These features led to the final diagnosis of ITPN. In this case, the solid ITPN growth obstructed the lumen of the main pancreatic duct, and the intraductal pressure of the distal side rose gradually. Then, pancreatic cysts formed and burst into the abdominal cavity when the intraductal pressure was at its maximum. However, an ITPN consists of high-grade atypical cells derived from the pancreatic ductal epithelium in principle, so the rupture may be an independent risk factor for peritonitis carcinomatosa in the future.

show abstract

“…In the data published (table 1), ITPN showed no sex prediliction, occurred in an age range of 35-80 years and was frequently located in the head and body of the pancreas [1,3,4,5,6,7,8,9,10,11,15]. On imaging studies, it presented as positron emission tomography (PET)-avid, as a cystic mass on computed tomography and as a hypoechoic mass on EUS.…”

Section: Discussionmentioning

confidence: 99%

“…The cytologic findings described in the literature are summarized in table 2 [3,4,5,6,7,8,9,11]. The slides were reported to show moderate-to-hypercellular smears with a background of cyst-type fluid, but no extracellular mucin was noted.…”

Section: Discussionmentioning

confidence: 99%

Intraductal Tubulopapillary Neoplasm of the Pancreas Masquerading as Pancreatic Neuroendocrine Carcinoma: Review of the Literature with a Case Report

Savant

Lee²,

Das³

2016

Acta Cytologica

View full text Add to dashboard Cite

Background: Intraductal tubulopapillary neoplasm of the pancreas (ITPN) is a rare tumor which was first described in 2009. We report a case with cytologic and histologic findings and discuss the pitfalls in diagnosing this entity on cytology. Case: An 82-year-old female presented with a pancreatic body mass measuring 3.3 cm. Endoscopic ultrasound-guided fine-needle aspiration showed cells in cohesive clusters with high-grade nuclear atypia. Immunohistochemistry (IHC) showed the neoplastic cells to be positive for CK19, CD56 and chromogranin (focal). Ki-67 was high at 50-60%, and chymotrypsin was negative. On the basis of this pattern of staining, the cytologic diagnosis rendered was ‘favors high-grade neuroendocrine carcinoma'. Distal pancreatic resection revealed a cystic 4-cm mass. Histologically, the tumor was seen arising from the duct with a solid growth pattern, tubule formation and papillary structures. IHC showed the tumor to be negative for chromogranin, synaptophysin, CD56, trypsin and chymotrypsin. The Ki-67 index was 70%. The final diagnosis was noninvasive ITPN. Conclusion: We review the literature and discuss the cytomorphologic features and IHC patterns characteristic of this new entity on cytology material in addition to the pitfalls of the cytologic diagnosis.

show abstract

Intraductal tubulopapillary neoplasm of the pancreas with somatic BRAF mutation

Cited by 16 publications

References 10 publications

Intraductal Tubulopapillary Neoplasm of the Pancreas: An Update From a Pathologist's Perspective

Intraductal Tubulopapillary Neoplasm of the Pancreas: An Update From a Pathologist's Perspective

A Rare Case of Intraductal Tubulopapillary Neoplasm of the Pancreas Rupturing and Causing Acute Peritonitis

Intraductal Tubulopapillary Neoplasm of the Pancreas Masquerading as Pancreatic Neuroendocrine Carcinoma: Review of the Literature with a Case Report

Contact Info

Product

Resources

About