CD117 Is a Specific Marker of Intraductal Papillary Mucinous Neoplasms (IPMN) of the Pancreas, Oncocytic Subtype

Mattiolo, Paola; Hong, Seung‐Mo; Paolino, Gaetano; Rusev, Borislav; Marchegiani, Giovanni; Salvia, Roberto; Andrianello, Stefano; Capelli, Paola; Piccoli, P; Parolini, Claudia; Scarpa, Aldo; Lawlor, Rita T.; Luchini, Claudio

doi:10.3390/ijms21165794

Cited by 18 publications

(5 citation statements)

References 28 publications

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“…IPMNs with pancreaticobiliary differentiation mark with MUC1 and IPMNs with gastric-foveolar differentiation show variable expression with MUC6. For IOPNs, the immunohistochemical markers expressed include EMA (MUC1), MUC6, hepatocyte-1 (Hep Par-1), and CD117 (Mattiolo et al, 2020). MUC2 and MUC5AC expression are limited to goblet cells.…”

Section: Immunohistochemical Markersmentioning

confidence: 99%

Updates in the Diagnosis of Intraductal Neoplasms of the Pancreas

2022

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Pancreatic ductal adenocarcinoma (PDAC) is one of the deadliest types of cancer worldwide. There are many reasons for this dismal prognosis, including the advanced stage at the time of diagnosis and the lack of effective therapeutic approaches. Intraductal papillary mucinous neoplasms (IPMNs) represent detectable and treatable precursor lesions of PDAC. Our understanding of the pathology of IPMNs has evolved over the past few decades, and new advances in diagnostic tools have emerged. The new World Health Organization (WHO) classification scheme now recognizes the previously considered variants of IPMNs, such as intraductal oncocytic papillary neoplasms (IOPNs) and intraductal tubulopapillary neoplasms (ITPNs), as distinct neoplasms. New imaging and molecular diagnostic tests are being developed to recognize these PDAC precursor lesions better. Here, we review the advances in diagnostic tools for IPMNs, IOPNs, and ITPNs, emphasizing the new (5th edition, 2019) WHO classification for pathological diagnosis, molecular markers, new laboratory tests, and imaging tools.

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Section: Immunohistochemical Markersmentioning

confidence: 99%

Updates in the Diagnosis of Intraductal Neoplasms of the Pancreas

2022

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“…Mattiolo et al has investigated CD117 (c-KIT) expression in a cohort of 111 intraductal papillary neoplasms, including 17 IOPNs. CD117 positivity was able to distinguish IOPNs from all different IPMN subtypes, the former characterized by stronger and more diffuse pattern of expression (7).…”

Section: Discussionmentioning

confidence: 93%

Intraductal oncocytic papillary neoplasm (IOPN): two case reports and review of the literature

Innocenti¹,

Rotondo²,

Donati³

et al. 2023

Transl Cancer Res

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Background: Intraductal oncocytic papillary neoplasms (IOPNs) place at the oncocytic extreme of the intraductal pancreatic neoplasm spectrum and display typical morphological features. Their identification in 1996 by Adsay et al. has been followed by a growing number of cases, paving the way for a deeper understanding of this underestimated entity. Contrarily to intraductal papillary mucinous neoplasms (IPMNs), most IOPNs run an indolent course and surgery is usually curative. Pancreatic IOPNs tend to develop from the main pancreatic duct (MPD) and their diagnosis is either incidental or subsequent to mass-related symptoms. Up to 30% of cases show concomitant areas of minimal stromal invasion and loco-regional or systemic spread are confined to a minority of cases. Biological hallmarks of IOPNs are being identified, including recurrent kinase gene rearrangements. Morphological and biological traits of IOPNs seem to overlap with those of other malignancies. A deeper understanding of these entities is needed in order to shed light upon the nature of pancreato-biliary oncocytic neoplasms. This case report describes two patients with a diagnosis of IOPN-one of them accounting for the largest IOPN ever described-and provides a brief review of recent discoveries on the subject.Case Description: We describe two cases of IOPN occurring in adult male patients, respectively in their 60s and 70s. Both patients had unremarkable clinical history. In case 1 the diagnosis was coincidental to a right renal colic; case 2 complained a right lumbar pain radiating to the homolateral groin. In both cases imaging analyses revealed a voluminous pancreatic mass, posing the indication to laparoscopic pancreatectomy. Gross and histological features were consistent with the diagnosis of IOPN. Surgical margin were free from disease and the patient did not undergo further treatment. After a 10-and 7-month follow-up respectively, patients did not experience relapse.Conclusions: Recent immunohistochemical (IHC) and molecular data reveal unique characteristics of IOPNs, highlighting the substantial differences from IPMNs. Further research is needed in order to identify novel prognostic and predictive markers applicable to oncocytic neoplasms of the pancreato-biliary tract.

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“…Specific immunohistochemical staining were obtained following standardized procedures, as described elsewhere. [15][16][17] Further molecular analyses were performed following specific methods reported below.…”

Section: Clinicopathologic and Immunohistochemical Assessmentmentioning

confidence: 99%

Molecular Analysis of an Intestinal Neuroendocrine/Non-neuroendocrine Neoplasm (MiNEN) Reveals MLH1 Methylation-driven Microsatellite Instability and a Monoclonal Origin: Diagnostic and Clinical Implications

Sciammarella¹,

Bencivenga

Mafficini

et al. 2021

Applied Immunohistochemistry &Amp; Molecular Morphology

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Mixed neuroendocrine/non-neuroendocrine neoplasms (MiNEN) are rare mixed epithelial neoplasms in which a neuroendocrine component is combined with a non-neuroendocrine component. Here, we provide the clinical, pathologic, and molecular report of a 73-year-old-man presenting with an intestinal MiNEN. The lesion was composed of a well-differentiated G3 neuroendocrine tumor and a colloid adenocarcinoma. The molecular characterization was performed using a multigene nextgeneration sequencing panel. The neoplasm displayed microsatellite instability due to MLH1 promoter methylation. The extended molecular profile documented the same mutations affecting ARID1A, ASXL1, BLM, and RNF43 genes in both components, indicating a monoclonal origin of the tumor. Regarding component-specific gene mutations, BRCA2 was specifically altered in the neuroendocrine area. It may represent a new actionable target for precision oncology in MiNEN, but the lack of its alteration in the colloid component calls for further considerations on intratumor heterogeneity. The most important finding with potential immediate implications regards the presence of microsatellite instability: it indicates that this molecular alteration should become part of the diagnostic algorithm for these rare neoplasms.

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CD117 Is a Specific Marker of Intraductal Papillary Mucinous Neoplasms (IPMN) of the Pancreas, Oncocytic Subtype

Cited by 18 publications

References 28 publications

Updates in the Diagnosis of Intraductal Neoplasms of the Pancreas

Updates in the Diagnosis of Intraductal Neoplasms of the Pancreas

Intraductal oncocytic papillary neoplasm (IOPN): two case reports and review of the literature

Molecular Analysis of an Intestinal Neuroendocrine/Non-neuroendocrine Neoplasm (MiNEN) Reveals MLH1 Methylation-driven Microsatellite Instability and a Monoclonal Origin: Diagnostic and Clinical Implications

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