Optimizing Chemotherapy of Pancreatic Acinar Cell Carcinoma: Our Experiences and Pooled Analysis of Literature

Abstract

Background: Pancreatic acinar cell carcinoma (PACC) is rare, and its appropriate treatment remains unknown. We aim to explore the characteristics and optimal treatment of it. Methods: The data on clinicopathologic characteristics, molecular alteration, treatment, and survival of patients diagnosed with PACC at the Sun Yat-sen University Cancer Center from 2005 to 2020 were collected. The optimal treatment was explored by co-analyzing our results and published literatures. Results: Twenty-two PACC patients were enrolled. Eight of 17 non-metastatic patients received adjuvant chemotherapy. The patients receiving fluoropyrimidine-based regimen (n = 3) had a better median disease-free survival (mDFS) than those with gemcitabine-based regimen (n = 5) (unreached vs 27 months). Eight metastatic patients received first-line chemotherapy. Four patients received second-line chemotherapy. The objective response rate (ORR) of the fluoropyrimidine-based regimen was 85.7% (6/7), much better than that of the gemcitabine-based regimen (0/5). One patient who had responded to the first-line FOLFIRINOX (5-fluorouracil + oxaliplatin + leucovorin + irinotecan) regimen received olaparib as maintenance treatment for 5 months with good tolerance. Thirty-one published literatures, with a total of 86 cases, were included in the co-analysis. The ORR of the first-line fluoropyrimidine-based regimen (n = 47) was higher than that of gemcitabine-based regimen (n = 39) (59.6% vs 15.3%, P < .001). Eight of 11 patients treated with the FOLFIRINOX regimen achieved partial response (PR). Conclusions: For patients with metastasis, a fluorouracil-based regimen such as FOLFIRINOX may be preferred, and maintenance treatment of poly ADP-ribose polymerase (PARP) inhibitors after effective platinum-containing treatment for breast cancer susceptibility gene (BRCA) mutation patients must be assessed.

Síndrome de hipersecreción de lipasa y lesiones óseas en el cáncer de células acinares del páncreas

“…Los casos resecables, o con enfermedad localizada, pueden ser tratados con cirugía, asociado o no a quimioterapia adyudante. En casos metastásicos, los esquemas de quimioterapia como FOLFIRI-NOX o FOLFOX han demostrado mejorar las tasas de sobrevida 10,11 , pero también se usan ampliamente otras alternativas quimioterapeúticas, siendo una de ellas la capecitabina que, dentro de sus ventajas, tiene el poder ser administrado de forma oral, un perfil de toxicidad favorable y su efecto benéfico independiente de la etapa del cáncer [12][13][14] . Otros autores han planteado también el tratamiento de metástasis hepáticas a través de embolización selectiva, como una alternativa para disminuir los efectos del SHL 6,7 .…”

Roseate and merest-acinar cell carcinoma-pancreas

“…Extensive metastatic disease accompanying acinar cell carcinoma pancreas may demonstrate symptoms contingent to lipase hypersecretion as subcutaneous fat necrosis. 5,6 Upon gross examination, a solid, enlarged, well circumscribed, partially encapsulated tumefaction with fleshy consistency is encountered. Upon frozen section, acinar cell carcinoma pancreas appears as a hyper-cellular neoplasm composed of neoplastic cells reminiscent of pancreatic acinar cells.…”

“…Cytological examination demonstrates hyper-cellular or moderately cellular smears exemplifying dissemination of monomorphic nuclei with prominent nucleoli. 5,6 Acinar cell carcinoma pancreas is a significantly cellular neoplasm. Neoplastic cells appear incorporated with moderate quantities of granular, eosinophilic cytoplasm and are pervaded with zymogen granules stainable with periodic acid Schiff's (PAS) stain with diastase resistance.…”

“…Foci of perineural invasion and vascular invasion are frequently observed. 5,6 Tumefaction is endowed with variable architecture and exhibits configuration of cystic, acinar, glandular or intra-ductal growth patterns.…”

“…Acinar cell carcinoma pancreas may represent a non-neuroendocrine component composed of an admixture of neuroendocrine and nonneuroendocrine neoplasms (MiNEN), a tumefaction which may be appropriately discerned with morphological assessment and precise immunohistochemistry. 5,6 Pancreatic acinar cell carcinoma appears immune reactive to keratins as CK7, CK8,CK18, CK19, BCL10 or trypsin. Besides, immune reactivity to nuclear beta catenin or CD200 may ensue.…”