title: Pancreatic acinar cell carcinoma with bilateral ovarian metastases, panniculitis and polyarthritis treated with FOLFIRINOX chemotherapy regimen. A case report and review of the literature.
abstract: Pancreatic acinar cell carcinoma (PACC) is a rare pancreatic tumor, with an estimated frequency of less than 1% of pancreatic malignancies. There are no prospective studies to guide diagnostic or therapeutic algorithms. We report the case of a 36 year-old woman, diagnosed of a pancreatic tumor with liver and peritoneal metastases that was initially managed as a neuroendocrine tumor with temozolomide and capecitabine. After two cycles a severely painful arthritis developed in her left ankle with panniculitis and extensive fat necrosis, and CT scan demonstrated progressive disease. Pathology of the primary was reassessed establishing the diagnosis of PACC. The patient started treatment with FOLFIRINOX regimen, achieving clinical benefit and disease stabilization. We also briefly reviewed the literature on this rare subtype of pancreatic tumor.
authors.affiliation: Department of Medical Oncology, Hospital Clínico San Carlos, Madrid, Spain. Electronic address: hector.callata@hotmail.com.
authors.forename: Hector R
authors.initials: HR
authors.lastname: Callata-Carhuapoma
authors.affiliation: Department of Rheumatology, Hospital Clínico San Carlos, Madrid, Spain.
authors.forename: Esperanza
authors.initials: E
authors.lastname: Pato Cour
authors.affiliation: Department of Medical Oncology, Hospital Clínico San Carlos, Madrid, Spain.
authors.forename: Beatriz
authors.initials: B
authors.lastname: Garcia-Paredes
authors.affiliation: Department of Radiology, Hospital Clínico San Carlos, Madrid, Spain.
authors.forename: Ramiro Mendez
authors.initials: RM
authors.lastname: Fernandez
authors.affiliation: Department of Pathology, Hospital Clínico San Carlos, Madrid, Spain.
authors.forename: Maria Luisa
authors.initials: ML
authors.lastname: Mendoza Fernandez
authors.affiliation: Department of Medical Oncology, Hospital Clínico San Carlos, Madrid, Spain.
authors.forename: Aranzazu Manzano
authors.initials: AM
authors.lastname: Fernandez
authors.affiliation: Department of Medical Oncology, Hospital Clínico San Carlos, Madrid, Spain.
authors.forename: Carlos Aguado
authors.initials: CA
authors.lastname: De La Rosa
authors.affiliation: Department of Medical Oncology, Hospital Clínico San Carlos, Madrid, Spain.
authors.forename: Miguel J
authors.initials: MJ
authors.lastname: Sotelo Lezama
authors.affiliation: Department of Medical Oncology, Hospital Clínico San Carlos, Madrid, Spain.
authors.forename: Santiago
authors.initials: S
authors.lastname: Cabezas-Camarero
authors.affiliation: Department of Medical Oncology, Hospital Clínico San Carlos, Madrid, Spain.
authors.forename: Javier
authors.initials: J
authors.lastname: Sastre Varela
mesh_terms: D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001168:Arthritis / Q000209:etiology*; D002166:Camptothecin / Q000008:administration & dosage / Q000031:analogs & derivatives; D018267:Carcinoma, Acinar Cell / Q000188:drug therapy* / Q000473:pathology; D005218:Fat Necrosis / Q000209:etiology; D017809:Fatal Outcome; D005260:Female; D005472:Fluorouracil / Q000008:administration & dosage; D006801:Humans; D000077146:Irinotecan; D002955:Leucovorin / Q000008:administration & dosage; D008113:Liver Neoplasms / Q000556:secondary; D009944:Organoplatinum Compounds / Q000008:administration & dosage; D010051:Ovarian Neoplasms / Q000556:secondary*; D000077150:Oxaliplatin; D010190:Pancreatic Neoplasms / Q000188:drug therapy* / Q000473:pathology; D015434:Panniculitis / Q000209:etiology*; D010534:Peritoneal Neoplasms / Q000556:secondary; D014057:Tomography, X-Ray Computed
chemical_list: D009944:Organoplatinum Compounds; D000077150:Oxaliplatin; D000077146:Irinotecan; D002955:Leucovorin; D005472:Fluorouracil; D002166:Camptothecin
keywords: Arthritis; Bilateral ovarian metastases; FOLFIRINOX; Fat necrosis; Lipase levels; Pancreatic acinar cell carcinoma