Rare Non-Neuroendocrine Pancreatic Tumours

Mormul, Agata; Włoszek, Emilia; Nowoszewska, Julia; Fudalej, Marta; Budzik, Michał P.; Badowska-Kozakiewicz, Anna M.; Deptała, Andrzej

doi:10.3390/cancers15082216

Cited by 3 publications

(3 citation statements)

References 67 publications

(190 reference statements)

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“…Postoperative pathology showed a slightly hard mass of grayish-white nature, about 5 × 3 × 2cm in size, which was considered to have the possibility of recurrence. Therefore, it is recommended to use 5FU-based chemotherapy alone or in combination with cisplatin [ 34 ]. After full understanding, the patient refused; subsequently, it is recommended to improve the detection of tumor genes.…”

Section: Discussionmentioning

confidence: 99%

Kikuchi disease: A case report about Sintilimab-induced Kikuchi histiocytic necrotizing lymphadenitis and literature review

Ren,

Wang,

Yang

et al. 2024

Heliyon

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Section: Discussionmentioning

confidence: 99%

Kikuchi disease: A case report about Sintilimab-induced Kikuchi histiocytic necrotizing lymphadenitis and literature review

Ren,

Wang,

Yang

et al. 2024

Heliyon

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“…is possible in addition to histological markers and viscosity. This allows for better identification of patients with risk for aberration as opposed to those who do not require surgery with surveillance only (Mormul et al 2023 ) . However, a reliable diagnosis can only be made by a pathologist after processing the pancreatic resectate.…”

Section: Discussionmentioning

confidence: 99%

Rare tumours of the pancreas: monocentric study

Bauschke,

Altendorf-Hofmann,

Ali-Deeb

et al. 2024

J Cancer Res Clin Oncol

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Purpose The biology of rare pancreatic tumours, which differs from that of ductal pancreatic cancer, requires increased attention. Although the majority of rare pancreatic tumours are benign, it is difficult to decide whether an invasive component exists without complete removal of the lesion, despite considerable progress in diagnosis. We are investigating a large cohort of patients with histologically confirmed epithelial non-ductal non-neuroendocrine neoplasms of the pancreas. Methods Here we analyze long-term survival from patients, who underwent resection of histologically confirmed epithelial non-ductal non-neuroendocrine neoplasms of the pancreas. At our department between Jan 1st, 1999, and Dec 31st, 2019. The median follow-up was 61 (range 0–168) month. All statistical analyses were performed using SPSS 26.0 (IBM, Chicago, IL, USA) software. Results 46 patients (48%) were followed up for more than 5 years, 18 patients (19%) for more than 10 years. The 5-year and 10-year survival rates for rare non-invasive pancreatic tumours were 72% and 55% respectively. The proportion of rare tumour entities (non-ductal and non-neuroendocrine) increased continuously and statistically significantly (p = 0.004) from 4.2 to 12.3% in our clinic between 1999 and 2019. If there is no invasive growth yet, there is a varying risk of malignant degeneration in the course of the disease. Therefore, the indication for pancreatic resection is still the subject of discussion. Conclusion The long-term prognosis of rare epithelial pancreatic tumours after R0 resection—even if they are already malignant—is much better than that of ductal pancreatic cancer.

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“…For the definitive diagnosis of PACC, immunostaining with antibodies to identify acinar cell differentiation (trypsin and Bcl-10) ( Fig. 1 ), in addition to the use of general neuroendocrine markers (synaptophysin and Chromogranin A) to exclude pNEN, is important ( 16 , 44 , 52 , 53 ). Scattered neuroendocrine cells positive for synaptophysin and/or Chromogranin A can be observed in PACC, and a small subset of pNEN shows focal acinar differentiation ( 2 , 54 ).…”

Section: Epidemiology Clinical Features Diagnosis Molecular Features ...mentioning

confidence: 99%

Comprehensive review of pancreatic acinar cell carcinoma: epidemiology, diagnosis, molecular features and treatment

Ikezawa,

Urabe,

Kai

et al. 2023

Japanese Journal of Clinical Oncology

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Pancreatic acinar cell carcinoma is a rare form (0.2–4.3%) of pancreatic neoplasm with unique clinical and molecular characteristics, which largely differ from pancreatic ductal adenocarcinoma. Pancreatic acinar cell carcinoma occurs more frequently in males and can occur in children. Serum lipase is elevated in 24–58% of patients with pancreatic acinar cell carcinoma. Pancreatic acinar cell carcinomas tend to be large at diagnosis (median tumour size: ~5 cm) and are frequently located in the pancreas head. Radiologically, pancreatic acinar cell carcinoma generally exhibits a solid appearance; however, necrosis, cystic changes and intratumoral haemorrhage can occur in larger lesions. Immunostaining is essential for the definitive diagnosis of pancreatic acinar cell carcinoma. Compared with pancreatic ductal adenocarcinoma, pancreatic acinar cell carcinoma has a more favourable prognosis. Although radical surgery is recommended for patients with pancreatic acinar cell carcinoma who do not have distant metastases, the recurrence rate is high. The effectiveness of adjuvant therapy for pancreatic acinar cell carcinoma is unclear. The response to FOLFIRINOX is generally favourable, and some patients achieve a complete response. Pancreatic acinar cell carcinoma has a different genomic profile compared with pancreatic ductal adenocarcinoma. Although genomic analyses have shown that pancreatic acinar cell carcinoma rarely has KRAS, TP53 and CDKN2A mutations, it has a higher prevalence of homologous recombination-related genes, including BRCA1/2 and ATM, than pancreatic ductal adenocarcinoma, suggesting high sensitivity to platinum-containing regimens and PARP inhibitors. Targeted therapies for genomic alternations are beneficial. Therefore, genetic testing is important for patients with pancreatic acinar cell carcinoma to choose the optimal therapeutic strategy.

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Rare Non-Neuroendocrine Pancreatic Tumours

Cited by 3 publications

References 67 publications

Kikuchi disease: A case report about Sintilimab-induced Kikuchi histiocytic necrotizing lymphadenitis and literature review

Kikuchi disease: A case report about Sintilimab-induced Kikuchi histiocytic necrotizing lymphadenitis and literature review

Rare tumours of the pancreas: monocentric study

Comprehensive review of pancreatic acinar cell carcinoma: epidemiology, diagnosis, molecular features and treatment

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