Dynamic Enhancement Pattern on CT for Predicting Pancreatic Neuroendocrine Neoplasms with Low PAX6 Expression: A Retrospective Observational Study

Paired box 6 (PAX6) is a transcription factor that plays a critical role in tumor suppression, implying that the downregulation of PAX6 promotes tumor growth and invasiveness. This study aimed to examine dynamic computed tomography (CT) features for predicting pancreatic neuroendocrine neoplasms (Pan-NENs) with low PAX6 expression. We retrospectively evaluated 51 patients with Pan-NENs without synchronous liver metastasis to assess the pathological expression of PAX6. Two radiologists analyzed preoperative dynamic CT images to determine morphological features and enhancement patterns. We compared the CT findings between low and high PAX6 expression groups. Pathological analysis identified 11 and 40 patients with low and high PAX6 expression, respectively. Iso- or hypoenhancement types in the arterial and portal phases were significantly associated with low PAX6 expression (p = 0.009; p = 0.001, respectively). Low PAX6 Pan-NENs showed a lower portal enhancement ratio than high PAX6 Pan-NENs (p = 0.044). The combination based on enhancement types (iso- or hypoenhancement during arterial and portal phases) and portal enhancement ratio (≤1.22) had 54.5% sensitivity, 92.5% specificity, and 84.3% accuracy in identifying low PAX6 Pan-NENs. Dynamic CT features, including iso- or hypoenhancement types in the arterial and portal phases and lower portal enhancement ratio may help predict Pan-NENs with low PAX6 expression.

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Surgical management for non-functional pancreatic neuroendocrine neoplasms with synchronous liver metastasis: A consensus from the Chinese Study Group for Neuroendocrine Tumors (CSNET)

International Journal of Oncology ◽

10.3892/ijo.2016.3711 ◽

2016 ◽

Vol 49 (5) ◽

pp. 1991-2000 ◽

Cited By ~ 7

Author(s):

Kaizhou Jin ◽

Jin Xu ◽

Jie Chen ◽

Minhu Chen ◽

Rufu Chen ◽

...

Keyword(s):

Liver Metastasis ◽

Surgical Management ◽

Neuroendocrine Tumors ◽

Neuroendocrine Neoplasms ◽

Study Group ◽

Synchronous Liver Metastasis ◽

Pancreatic Neuroendocrine Neoplasms ◽

Chinese Study

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Three-phase Dynamic CT Findings of Liver Abscess:Related Factors with Multiple Layering Enhancement Pattern

Journal of the Korean Radiological Society ◽

10.3348/jkrs.2001.44.1.69 ◽

2001 ◽

Vol 44 (1) ◽

pp. 69

Author(s):

Bae Ju Kwon ◽

Yong Soo Kim ◽

Hyun Chul Rhim ◽

Byung Hee Koh ◽

On Koo Cho ◽

...

Keyword(s):

Dynamic Ct ◽

Enhancement Pattern ◽

Phase Dynamic ◽

Ct Findings ◽

Three Phase

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Multifocal Pancreatic Neuroendocrine Neoplasms In Tuberous Sclerosis: A Rare Case

American Journal of Clinical Pathology ◽

10.1093/ajcp/aqaa161.168 ◽

2020 ◽

Vol 154 (Supplement_1) ◽

pp. S77-S77

Author(s):

N C Jadhav ◽

D L Gang

Keyword(s):

Tuberous Sclerosis ◽

Neurofibromatosis Type ◽

Abdominal Ultrasound ◽

Biologically Active ◽

Pancreatic Tumors ◽

Neuroendocrine Neoplasms ◽

Ki 67 ◽

Pancreatic Neuroendocrine Neoplasms ◽

Von Hippel Lindau ◽

Multifocal Disease

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.

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