The surgical management of metastatic pancreatic acinar cell carcinoma and associated pancreatic panniculitis—A case report and literature review

Clarke, Edward M.; Stevens, Sean; Bennett, T Z; Crowley, Peter; Starkey, Graham

doi:10.1016/j.ijscr.2020.10.062

Cited by 4 publications

(3 citation statements)

References 21 publications

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“…The cutaneous lesions preceded the diagnosis of underlying neoplastic disease in 48.9% of patients, with a mean lesion-to-diagnosis interval of 134 days. Resection of the tumour can often lead to the complete resolution of pancreatic panniculitis,9 as demonstrated twice in our patient.…”

Section: Discussionsupporting

confidence: 71%

“…The presenting symptoms are often non-specific and include abdominal and back pain, weight loss, lymphadenopathy and diarrhoea 8. Pancreatic panniculitis, the inflammation of subcutaneous fat associated with pancreatic diseases, is a characteristic feature of acinar cell carcinoma as seen in our case and in several previous reports 9 10. The cutaneous lesions preceded the diagnosis of underlying neoplastic disease in 48.9% of patients, with a mean lesion-to-diagnosis interval of 134 days.…”

Section: Discussionsupporting

confidence: 59%

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Acinar cell carcinoma with PRKAR1A and PTEN alterations and paraneoplastic panniculitis

et al. 2022

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Pancreatic acinar cell carcinoma is a rare type of pancreatic malignancy, which can be confused with pancreatic neuroendocrine neoplasm. Here, we describe a woman in her 80s who presented with abdominal pain and bilateral lower extremity panniculitis. She underwent surgery for a presumed diagnosis of neuroendocrine tumour with PTEN and PRKAR1A alterations; 19 months, later, a recurrence of her pancreatic malignancy was discovered. The patient underwent repeat resection and this time immunohistochemical staining confirmed the diagnosis of acinar cell carcinoma. Staining for acinar cell carcinoma should be prompted based on clinical suspicion in context of PTEN or PRKAR1A mutation when appropriate.

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Section: Discussionsupporting

confidence: 71%

Section: Discussionsupporting

confidence: 59%

Acinar cell carcinoma with PRKAR1A and PTEN alterations and paraneoplastic panniculitis

et al. 2022

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“…Similar to our study, Landa et al reported 12% of 566 resected PACC patients to be stage IV, with an estimated 5-year survival of 19% versus 4% ( p < 0.001) of not resected stage IV patients [ 5 ]. There have been several case reports of successful curative resections of metastases in PACC [ 24 , 25 , 26 , 27 , 28 , 29 ]. Ohara et al reported the successful resection of metachronous rectal and liver metastases, Kittaki et al and Villano et al performed resections of liver metastases after systemic therapy, and Suzuki et al published a case of a patient with long-term survival after repetitive resection of recurrent liver metastases [ 26 , 27 , 28 , 29 ].…”

Section: Discussionmentioning

confidence: 99%

Survival Outcome and Prognostic Factors for Pancreatic Acinar Cell Carcinoma: Retrospective Analysis from the German Cancer Registry Group

Petrova

Wellner

Nording

et al. 2021

Cancers

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Background: Pancreatic acinar cell carcinoma (PACC) is a distinct type of pancreatic cancer with low prevalence. We aimed to analyze prognostic factors and survival outcome for PACC in comparison to pancreatic ductal adenocarcinoma (PDAC), based on data from the German Cancer Registry Group. Methods: Patients with PACC and PDAC were extracted from pooled data of the German clinical cancer registries (years 2000 to 2019). The distribution of demographic parameters, tumor stage and therapy modes were compared between PACC and PDAC. The Kaplan–Meier method and Cox regression analysis were used to delineate prognostic factors for PACC. Propensity score matching was used to compare survival between PACC and PDAC. Results: There were 233 (0.44%) patients with PACC out of 52,518 patients with pancreatic malignancy. Compared to PDAC, patients with PACC were younger (median age 66 versus 70, respectively, p < 0.001) and the percentage of males was higher (66.1% versus 53.3%, respectively, p < 0.001). More patients were resected with PACC than with PDAC (56.2% versus 38.9%, respectively, p < 0.001). The estimated overall median survival in PACC was 22 months (95% confidence interval 15 to 27), compared to 12 months (95% confidence interval 10 to 13) in the matched PDAC cohort (p < 0.001). Surgical resection was the strongest positive prognostic factor for PACC after adjusting for sex, age, and distant metastases (hazard ratio 0.34, 95% confidence interval 0.22 to 0.51, p < 0.001). There was no survival benefit for adjuvant therapy in PACC. Conclusions: PACC has overall better prognosis than PDAC. Surgical resection is the best therapeutic strategy for PACC and should be advocated even in advanced tumor stages.

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Clinical characteristics and surgical outcomes of resectable acinar cell carcinoma of the pancreas-propensity score matching analysis with pancreatic ductal adenocarcinoma

Chen

Shen³

et al. 2022

European Journal of Surgical Oncology

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The surgical management of metastatic pancreatic acinar cell carcinoma and associated pancreatic panniculitis—A case report and literature review

Abstract: Highlights Pancreatic acinar cell carcinoma (ACC) may present with pancreatic panniculitis. Complete surgical resection of ACC can successfully treat pancreatic panniculitis. Aggressive surgery for ACC can lead to prolonged disease-free survival.

Cited by 4 publications

References 21 publications

Acinar cell carcinoma with PRKAR1A and PTEN alterations and paraneoplastic panniculitis

Acinar cell carcinoma with PRKAR1A and PTEN alterations and paraneoplastic panniculitis

Survival Outcome and Prognostic Factors for Pancreatic Acinar Cell Carcinoma: Retrospective Analysis from the German Cancer Registry Group

Clinical characteristics and surgical outcomes of resectable acinar cell carcinoma of the pancreas-propensity score matching analysis with pancreatic ductal adenocarcinoma

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