|
1
|
de Jesus VHF, Donadio MDS, de Brito ÂBC, Gentilli AC. A narrative review on rare types of pancreatic cancer: should they be treated as pancreatic ductal adenocarcinomas? Ther Adv Med Oncol 2024; 16:17588359241265213. [PMID: 39072242 PMCID: PMC11282540 DOI: 10.1177/17588359241265213] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/17/2024] [Accepted: 06/13/2024] [Indexed: 07/30/2024] Open
Abstract
Pancreatic cancer is one of the deadliest malignancies in humans and it is expected to play a bigger part in cancer burden in the years to come. Pancreatic ductal adenocarcinoma (PDAC) represents 85% of all primary pancreatic malignancies. Recently, much attention has been given to PDAC, with significant advances in the understanding of the mechanisms underpinning disease initiation and progression, along with noticeable improvements in overall survival in both localized and metastatic settings. However, given their rarity, rare histological subtypes of pancreatic cancer have been underappreciated and are frequently treated as PDAC, even though they might present non-overlapping molecular alterations and clinical behavior. While some of these rare histological subtypes are true variants of PDAC that should be treated likewise, others represent separate clinicopathological entities, warranting a different therapeutic approach. In this review, we highlight clinical, pathological, and molecular aspects of rare histological types of pancreatic cancer, along with the currently available data to guide treatment decisions.
Collapse
Affiliation(s)
- Victor Hugo Fonseca de Jesus
- Oncoclínicas, Department of Gastrointestinal Medical Oncology, Santos Dumont St. 182, 4 floor, Florianópolis, Santa Catarina 88015-020, Brazil
- Department of Medical Oncology, Centro de Pesquisas Oncológicas, Florianópolis, Santa Catarina, Brazil
| | | | | | | |
Collapse
|
|
2
|
Yamada S, Motegi H, Kurihara Y, Shimbo T, Kikuchi I, Wakabayashi T, Sato T. A resected case of acinar cell carcinoma of the pancreas with liver metastasis following chemotherapy using modified FOLFIRINOX. Surg Case Rep 2023; 9:147. [PMID: 37610633 PMCID: PMC10447704 DOI: 10.1186/s40792-023-01729-1] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/19/2023] [Accepted: 08/08/2023] [Indexed: 08/24/2023] Open
Abstract
BACKGROUND Acinar cell carcinoma of the pancreas is a rare exocrine malignancy representing less than 1% of all pancreatic neoplasms. It has been reported that it responds to treatment differently from pancreatic ductal adenocarcinoma and the treatment algorithm for acinar cell carcinoma usually depends on the stage of the respective tumor and the patient's current status. CASE PRESENTATION A 60-year-old man presented with upper abdominal pain and anorexia. Abdominal ultrasonography showed a large-sized hepatic mass and he was referred to our hospital. Contrast-enhanced computed tomography demonstrated a 110-mm low-density area occupying the right hemi-liver and an enhanced mass of 70 × 56 mm in the tail of the pancreas, which seemed to directly infiltrate into the spleen. The case was diagnosed as acinar cell carcinoma with a simultaneous liver metastasis identified by liver biopsy. Upfront resection of pancreatic cancer with distant metastasis might not be considered as an optimal choice, and in this case chemotherapy was administered prior to curative resection. Chemotherapy using the modified FOLFIRINOX regimen was undertaken, resulting in a partial remission; the liver tumor reduced in size from 110 to 47 mm and the pancreatic tumor from 70 to 40 mm. The patient then safely underwent curative hepatic resection with distal pancreato-splenectomy. Histological examinations revealed small-sized atypical cells with large nuclei that had formed acinar patterns, and immunostaining with trypsin was positive in tumor cells, which was in accordance with acinar cell carcinoma. More than 3 years later, the patient is doing well without any recurrence. CONCLUSION Aggressive and curative surgery in combination with chemotherapy such as FOLFIRINOX could be a treatment option to achieve long-term survival in cases of acinar cell carcinoma with liver metastases.
Collapse
Affiliation(s)
- Shuhei Yamada
- Department of Gastroenterological Surgery, Akita City Hospital, 4-30 Kawamoto Matsuoka-machi, Akita-city, Akita, Japan.
| | - Haruka Motegi
- Department of Gastroenterological Surgery, Akita City Hospital, 4-30 Kawamoto Matsuoka-machi, Akita-city, Akita, Japan
| | - Yoshiki Kurihara
- Department of Gastroenterological Surgery, Akita City Hospital, 4-30 Kawamoto Matsuoka-machi, Akita-city, Akita, Japan
| | - Tomonori Shimbo
- Department of Gastroenterological Surgery, Akita City Hospital, 4-30 Kawamoto Matsuoka-machi, Akita-city, Akita, Japan
| | - Isao Kikuchi
- Department of Gastroenterological Surgery, Akita City Hospital, 4-30 Kawamoto Matsuoka-machi, Akita-city, Akita, Japan
| | - Toshiki Wakabayashi
- Department of Gastroenterological Surgery, Akita City Hospital, 4-30 Kawamoto Matsuoka-machi, Akita-city, Akita, Japan
| | - Tsutomu Sato
- Department of Gastroenterological Surgery, Akita City Hospital, 4-30 Kawamoto Matsuoka-machi, Akita-city, Akita, Japan
| |
Collapse
|
|
3
|
Calimano-Ramirez LF, Daoud T, Gopireddy DR, Morani AC, Waters R, Gumus K, Klekers AR, Bhosale PR, Virarkar MK. Pancreatic acinar cell carcinoma: A comprehensive review. World J Gastroenterol 2022; 28:5827-5844. [PMID: 36353206 PMCID: PMC9639656 DOI: 10.3748/wjg.v28.i40.5827] [Citation(s) in RCA: 29] [Impact Index Per Article: 9.7] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/25/2022] [Revised: 09/14/2022] [Accepted: 10/14/2022] [Indexed: 02/06/2023] Open
Abstract
Acinar cell carcinoma (ACC) is a rare pancreatic malignancy with distinctive clinical, molecular, and morphological features. The long-term survival of ACC patients is substantially superior to that of pancreatic adenocarcinoma patients. As there are no significant patient series about ACCs, our understanding of this illness is mainly based on case reports and limited patient series. Surgical resection is the treatment of choice for patients with the disease restricted to one organ; however, with recent breakthroughs in precision medicine, medicines targeting the one-of-a-kind molecular profile of ACC are on the horizon. There are no standard treatment protocols available for people in which a total surgical resection to cure the condition is not possible. As a result of shared genetic alterations, ACCs are chemosensitive to agents with activity against pancreatic adenocarcinomas and colorectal carcinomas. The role of neoadjuvant or adjuvant chemoradiotherapy has not been established. This article aims to do a comprehensive literature study and present the most recent information on acinar cell cancer.
Collapse
Affiliation(s)
| | - Taher Daoud
- Department of Diagnostic Radiology, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, United States
| | - Dheeraj Reddy Gopireddy
- Department of Diagnostic Radiology, University of Florida College of Medicine Jacksonville, Jacksonville, FL 32209, United States
| | - Ajaykumar C Morani
- Department of Diagnostic Radiology, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, United States
| | - Rebecca Waters
- Department of Pathology and Lab Medicine, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, United States
| | - Kazim Gumus
- Department of Research and Diagnostic Radiology, University of Florida College of Medicine Jacksonville, Jacksonville, FL 32209, United States
| | - Albert Russell Klekers
- Department of Diagnostic Radiology, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, United States
| | - Priya R Bhosale
- Department of Diagnostic Radiology, The University of Texas MD Anderson Cancer Center, Houston, TX 77030, United States
| | - Mayur K Virarkar
- Department of Diagnostic Radiology, University of Florida College of Medicine Jacksonville, Jacksonville, FL 32209, United States
| |
Collapse
|
|
4
|
Survival Outcome and Prognostic Factors for Pancreatic Acinar Cell Carcinoma: Retrospective Analysis from the German Cancer Registry Group. Cancers (Basel) 2021; 13:cancers13236121. [PMID: 34885230 PMCID: PMC8656891 DOI: 10.3390/cancers13236121] [Citation(s) in RCA: 8] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/30/2021] [Revised: 11/29/2021] [Accepted: 12/01/2021] [Indexed: 12/18/2022] Open
Abstract
Simple Summary Less than 1% of all pancreatic malignancies are acinar cell carcinomas. Based on data from the German Cancer Registry Group, we performed a comparative analysis of characteristics and prognostic factors of pancreatic acinar cell carcinoma and the most common type of pancreatic cancer—pancreatic ductal adenocarcinoma. Compared to pancreatic ductal adenocarcinoma, patients with pancreatic acinar cell carcinoma were younger at the time of diagnosis and the percentage of males was higher. The prognosis of patients with pancreatic acinar carcinoma was better than that of patients with pancreatic ductal adenocarcinoma. Surgical resection was the strongest positive prognostic factor for pancreatic acinar cell carcinoma. The study shows that pancreatic acinar cell carcinoma has features distinct from pancreatic ductal adenocarcinoma. Radical resection should be advocated, whenever feasible. Abstract 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.
Collapse
|
|
5
|
Niger M, Prisciandaro M, Antista M, Monica MAT, Cattaneo L, Prinzi N, Manglaviti S, Nichetti F, Brambilla M, Torchio M, Corti F, Pusceddu S, Coppa J, Mazzaferro V, de Braud F, Di Bartolomeo M. One size does not fit all for pancreatic cancers: A review on rare histologies and therapeutic approaches. World J Gastrointest Oncol 2020; 12:833-849. [PMID: 32879662 PMCID: PMC7443847 DOI: 10.4251/wjgo.v12.i8.833] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/31/2020] [Revised: 05/01/2020] [Accepted: 05/20/2020] [Indexed: 02/05/2023] Open
Abstract
Exocrine pancreatic neoplasms represent up to 95% of pancreatic cancers (PCs) and are widely recognized among the most lethal solid cancers, with a very poor 5-year survival rate of 5%-10%. The remaining < 5% of PCs are neuroendocrine tumors that are usually characterized by a better prognosis, with a median overall survival of 3.6 years. The most common type of PC is pancreatic ductal adenocarcinoma (PDAC), which accounts for roughly 85% of all exocrine PCs. However up to 10% of exocrine PCs have rare histotypes, which are still poorly understood. These subtypes can be distinguished from PDAC in terms of pathology, imaging, clinical presentation and prognosis. Additionally, due to their rarity, any knowledge regarding these specific histotypes is mostly based on case reports and a small series of retrospective analyses. Therefore, treatment strategies are generally deduced from those used for PDAC, even if these patients are often excluded or not clearly represented in clinical trials for PDAC. For these reasons, it is essential to collect as much information as possible on the management of PC, as assimilating it with PDAC may lead to the potential mistreatment of these patients. Here, we report the most significant literature regarding the epidemiology, typical presentation, possible treatment strategies, and prognosis of the most relevant histotypes among rare PCs.
Collapse
Affiliation(s)
- Monica Niger
- Medical Oncology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
| | - Michele Prisciandaro
- Medical Oncology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
| | - Maria Antista
- Medical Oncology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
| | - Melissa Anna Teresa Monica
- First Pathology Division, Department of Pathology and Laboratory Medicine, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
| | - Laura Cattaneo
- First Pathology Division, Department of Pathology and Laboratory Medicine, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
| | - Natalie Prinzi
- Medical Oncology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
| | - Sara Manglaviti
- Medical Oncology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
| | - Federico Nichetti
- Medical Oncology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
| | - Marta Brambilla
- Medical Oncology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
| | - Martina Torchio
- Medical Oncology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
| | - Francesca Corti
- Medical Oncology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
| | - Sara Pusceddu
- Medical Oncology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
| | - Jorgelina Coppa
- Hepato-biliary-pancreatic Surgery and Liver Transplantation Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
| | - Vincenzo Mazzaferro
- Hepato-biliary-pancreatic Surgery and Liver Transplantation Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
- Università degli studi di Milano, Milan 20133, Italy
| | - Filippo de Braud
- Medical Oncology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
- Università degli studi di Milano, Milan 20133, Italy
| | - Maria Di Bartolomeo
- Medical Oncology Department, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan 20133, Italy
| |
Collapse
|
|
6
|
Robot-assisted combined pancreatectomy/hepatectomy for metastatic pancreatic acinar cell carcinoma: case report and review of the literature. Clin J Gastroenterol 2020; 13:973-980. [PMID: 32583372 DOI: 10.1007/s12328-020-01146-z] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 03/30/2020] [Accepted: 06/01/2020] [Indexed: 12/14/2022]
Abstract
Acinar cell carcinoma (ACC) of the pancreas is a rare neoplasm with less aggressive behavior than ductal carcinoma. As a result, surgical resection for metastatic ACC is a therapeutic option which can result in long-term survival. There is a paucity of data describing institutional approaches to these challenging patients, and therefore, we herein describe our institution's approach to a patient with a distal pancreatic ACC and isolated liver metastasis. The patient underwent neoadjuvant chemotherapy (FOLFIRINOX), followed by a robot-assisted distal pancreatectomy/splenectomy and non-anatomic segment 6 resection. He was discharged to home post-operative day 2. Final pathology revealed complete tumor response of the liver metastasis and a margin negative resection of the primary tumor. He remains disease free and without complications at 3 months. We highlight that combined modality therapy for metastatic ACC can yield long-term survival in selected patients. Similarly, the robotic platform enables performance of complex multivisceral resections with rapid recovery. Future research investigating precision medicine for metastatic ACC is warranted given widely variable tumor biology in this disease.
Collapse
|
|
7
|
Ang C, Herran LA, Lagunes DR, Klimstra DS, Kemeny NE. A Case Report of a Patient with Advanced Acinar Cell Carcinoma of the Pancreas: Long-Term Survival with Regional, Systemic and Targeted Therapy. TUMORI JOURNAL 2018; 99:e61-4. [DOI: 10.1177/030089161309900230] [Citation(s) in RCA: 7] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/16/2022]
Abstract
Acinar cell carcinoma of the pancreas is an uncommon malignancy for which there are no prospective, randomized data to guide therapy. We describe the clinical course and management of a patient with advanced pancreatic acinar cell carcinoma who is alive seven years after diagnosis using systemic and regional chemotherapies as well as molecularly targeted agents.
Collapse
Affiliation(s)
- Celina Ang
- Department of Medicine, Gastrointestinal Oncology Service, Memorial Sloan-Kettering Cancer Center, New York, NY
| | | | - Diane Reidy Lagunes
- Department of Medicine, Gastrointestinal Oncology Service, New York, NY, USA
| | - David S Klimstra
- Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, NY, USA
| | - Nancy E Kemeny
- Department of Medicine, Gastrointestinal Oncology Service, Memorial Sloan-Kettering Cancer Center, New York, NY
| |
Collapse
|
|
8
|
Multimodal approach and long-term survival in a patient with recurrent metastatic acinar cell carcinoma of the pancreas: A case report. Pancreatology 2015; 16:153-6. [PMID: 26456669 DOI: 10.1016/j.pan.2015.09.006] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 07/12/2015] [Revised: 09/12/2015] [Accepted: 09/15/2015] [Indexed: 12/11/2022]
Abstract
Pancreatic acinar cell carcinoma is an uncommon neoplasm of the exocrine pancreas associated with a poor prognosis, especially when found to be metastatic. Since there are a lack of large studies and prospective, randomized data, no consensus treatment guidelines are available. Here, we report a case of a patient with recurrent metastatic acinar cell carcinoma involving the liver who had presented initially with pancreatic panniculitis. She received chemotherapy with capecitabine and oxaliplatin prior to resection of her primary tumor and liver metastases, after which she experienced a 30 months recurrence-free survival. Upon relapse, she was treated with a combination of capecitabine and oxaliplatin followed by maintenance capecitabine. Now, more than seven years after initial diagnosis, the patient remains stable without evidence of active disease. This case highlights the possibility of therapeutic success even for a patient initially deemed unresectable due to a poor performance status who responded to fluoropyrimidine-based therapy.
Collapse
|
|
9
|
Pitman MB, Centeno BA, Ali SZ, Genevay M, Stelow E, Mino-Kenudson M, Castillo CFD, Schmidt CM, Brugge WR, Layfield LJ. Standardized terminology and nomenclature for pancreatobiliary cytology: The Papanicolaou Society of Cytopathology Guidelines. Cytojournal 2014; 11:3. [PMID: 25191517 PMCID: PMC4153338 DOI: 10.4103/1742-6413.133343] [Citation(s) in RCA: 44] [Impact Index Per Article: 4.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/15/2014] [Accepted: 02/06/2014] [Indexed: 02/07/2023] Open
Abstract
The Papanicolaou Society of Cytopathology has developed a set of guidelines for pancreatobiliary cytology including indications for endoscopic ultrasound (EUS) guided fine-needle aspiration (FNA) biopsy, techniques of EUS-FNA, terminology and nomenclature of pancreatobiliary disease, ancillary testing and post-biopsy treatment and management. All documents are based on the expertise of the authors, a review of the literature, discussion of the draft document at several national and international meetings over an 18 month period and synthesis of online comments of the draft document on the Papanicolaou Society of Cytopathology web site [www.papsociety.org]. This document selectively presents the results of these discussions and focuses on a proposed standardized terminology scheme for pancreatobiliary specimens that correlate cytological diagnosis with biological behavior and increasingly conservative patient management of surveillance only. The proposed terminology scheme recommends a six-tiered system: Non-diagnostic, negative, atypical, neoplastic [benign or other], suspicious and positive. Unique to this scheme is the “neoplastic” category separated into “benign” (serous cystadenoma) or “other” (premalignant mucinous cysts, neuroendocrine tumors and solid-pseudopapillary neoplasms (SPNs)). The positive or malignant category is reserved for high-grade, aggressive malignancies including ductal adenocarcinoma, acinar cell carcinoma, poorly differentiated neuroendocrine carcinomas, pancreatoblastoma, lymphoma and metastases. Interpretation categories do not have to be used. Some pathology laboratory information systems require an interpretation category, which places the cytological diagnosis into a general category. This proposed scheme provides terminology that standardizes the category of the various diseases of the pancreas, some of which are difficult to diagnose specifically by cytology. In addition, this terminology scheme attempts to provide maximum flexibility for patient management, which has become increasingly conservative for some neoplasms.
Collapse
Affiliation(s)
- Martha B Pitman
- Address: Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA
| | - Barbara A Centeno
- H. Lee Moffitt Cancer Center and Research Institute, Tampa, Florida, USA
| | - Syed Z Ali
- The Johns Hopkins University School of Medicine, Baltimore, Maryland, USA
| | | | - Ed Stelow
- University of Virginia Medical Center, Charlottesville, Virginia, USA
| | - Mari Mino-Kenudson
- Address: Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA
| | | | - C Max Schmidt
- Deparment of Surgery, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA
| | - William R Brugge
- Deparment of Surgery, Indiana University Medical Center, Indianapolis, USA
| | - Lester J Layfield
- Department of Medicine, Division of Gastroenterology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts, USA
| |
Collapse
|
|
10
|
Pitman MB, Centeno BA, Ali SZ, Genevay M, Stelow E, Mino-Kenudson M, Fernandez-del Castillo C, Max Schmidt C, Brugge W, Layfield L. Standardized terminology and nomenclature for pancreatobiliary cytology: the Papanicolaou Society of Cytopathology guidelines. Diagn Cytopathol 2014; 42:338-50. [PMID: 24554455 DOI: 10.1002/dc.23092] [Citation(s) in RCA: 158] [Impact Index Per Article: 14.4] [Reference Citation Analysis] [Abstract] [Key Words] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/03/2013] [Accepted: 01/08/2014] [Indexed: 12/20/2022]
Abstract
The Papanicolaou Society of Cytopathology has developed a set of guidelines for pancreatobiliary cytology including indications for endoscopic ultrasound (EUS)-guided fine needle aspiration (FNA) biopsy, techniques of EUS-FNA, terminology and nomenclature of pancreatobiliary disease, ancillary testing, and postbiopsy treatment and management. All documents are based on the expertise of the authors, a review of the literature, discussions of the draft document at several national and international meetings over an 18-month period and synthesis of online comments of the draft document on the Papanicolaou Society of Cytopathology web site (www.papsociety.org). This document selectively presents the results of these discussions and focuses on a proposed standardized terminology scheme for pancreatobiliary specimens that correlate cytological diagnosis with biological behavior and increasingly conservative patient management of surveillance only. The proposed terminology scheme recommends a six-tiered system: Nondiagnostic, Negative, Atypical, Neoplastic (benign or other), Suspicious and Positive. Unique to this scheme is the "Neoplastic" category separated into "benign" (serous cystadenoma), or "Other" (premalignant mucinous cysts, neuroendocrine tumors, and solid-pseudopapillary neoplasms). The positive or malignant category is reserved for high-grade, aggressive malignancies including ductal adenocarcinoma, acinar cell carcinoma, poorly differentiated neuroendocrine carcinomas, pancreatoblastoma, lymphoma, and metastases. Interpretation categories do not have to be used. Some pathology laboratory information systems require an interpretation category, which places the cytological diagnosis into a general category. This proposed scheme provides terminology that standardizes the category of the various diseases of the pancreas, some of which are difficult to diagnose specifically by cytology. In addition, this terminology scheme attempts to provide maximum flexibility for patient management, which has become increasingly conservative for some neoplasms.
Collapse
Affiliation(s)
- Martha B Pitman
- Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts
| | | | | | | | | | | | | | | | | | | | | |
Collapse
|
|
11
|
Sumiyoshi T, Shima Y, Okabayashi T, Kozuki A, Nakamura T. Comparison of pancreatic acinar cell carcinoma and adenocarcinoma using multidetector-row computed tomography. World J Gastroenterol 2013; 19:5713-5719. [PMID: 24039366 PMCID: PMC3769910 DOI: 10.3748/wjg.v19.i34.5713] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/12/2013] [Revised: 03/30/2013] [Accepted: 07/13/2013] [Indexed: 02/06/2023] Open
Abstract
AIM: To distinguish acinar cell carcinoma (ACC) from pancreatic adenocarcinoma (AC) by comparing their computed tomography findings.
METHODS: Patients with ACC and AC were identified on the basis of results obtained using surgically resected pancreatectomy specimens. The preoperative computer tomographic images of 6 acinar cell carcinoma patients and 67 pancreatic adenocarcinoma patients in 4 phases (non-contrast, arterial, portal venous, and delayed phase) were compared. The scan delay times were 40, 70, and 120 s for each contrast-enhanced phase. The visual pattern, tomographic attenuation value, and time attenuation curve were assessed and compared between AC and ACC cases using the χ2 test, Wilcoxon signed-rank test, and Mann Whitney U test.
RESULTS: The adenocarcinomas tended to be hypodense in all 4 phases. The acinar cell carcinomas also tended to be hypodense in the 3 contrast-enhanced phases, although their computed tomographic attenuation values were higher. Further, 5 of the 6 acinar cell carcinomas (83%) were isodense in the non-contrast phase. The time attenuation curve of the adenocarcinomas showed a gradual increase through the 4 phases, and all adenocarcinomas showed peak enhancement during the delayed phase. The time attenuation curve of the acinar cell carcinomas showed peak enhancement during the portal venous phase in 4 cases and during the arterial phase in 2 cases. None of the 6 acinar cell carcinomas showed peak enhancement during the delayed phase.
CONCLUSION: The tumor density in the non-contrast phase and time attenuation curve pattern clearly differ between acinar cell carcinomas and adenocarcinomas, and multidetector-row computed tomography can thus distinguish these tumors.
Collapse
|
|
12
|
Matsumoto S, Sata N, Koizumi M, Lefor A, Yasuda Y. Imaging and pathological characteristics of small acinar cell carcinomas of the pancreas: A report of 3 cases. Pancreatology 2013; 13:320-3. [PMID: 23719608 DOI: 10.1016/j.pan.2013.03.007] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.4] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/27/2012] [Revised: 03/04/2013] [Accepted: 03/04/2013] [Indexed: 12/11/2022]
Abstract
We present three patients with T2 acinar cell carcinomas (ACC) (two of which were small) and discuss their clinical and pathological features. Case 1: A 34 year-old woman had a 2.6 cm iso- or hyperdense tumor. Enucleation was performed but final diagnosis was ACC and a pylorus preserving pancreaticoduodenectomy was performed. Case 2: A 35 year-old woman was found to have a 4.5 cm hypervascular tumor in the tail of the pancreas. Distal pancreatectomy was performed. Case 3: A 61 year-old man suffered from acute pancreatitis and was found to have an encapsulated 2.5 cm hypervascular tumor in the head of pancreas. Pylorus-preserving pancreaticoduodenectomy was performed. These three patients with T2 ACCs had common characteristics including well-demarcated exophytic tumors with slow and limited progress, distinctively different from large ACCs and pancreatic ductal adenocarcinomas. ACCs are important in the differential diagnosis of homogeneously or heterogeneously enhancing small pancreatic tumors.
Collapse
Affiliation(s)
- Shiro Matsumoto
- Department of Surgery, Jichi Medical University, Yakushiji 3311-1, Shimotsuke City, Tochigi Prefecture, Japan.
| | | | | | | | | |
Collapse
|
|
13
|
Clinicopathologic study of 62 acinar cell carcinomas of the pancreas: insights into the morphology and immunophenotype and search for prognostic markers. Am J Surg Pathol 2013; 36:1782-95. [PMID: 23026929 DOI: 10.1097/pas.0b013e318263209d] [Citation(s) in RCA: 113] [Impact Index Per Article: 9.4] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 12/14/2022]
Abstract
Acinar cell carcinoma (ACC) of the pancreas is a very rare tumor that has various morphologic features, which may give rise to diagnostic difficulties. Because of its rarity, many clinicopathologic characteristics remain to be further elucidated, and prognostic factors are yet to be well established. With the aim of better characterizing this carcinoma and searching for prognostic indicators, we collected 62 ACCs and investigated the following parameters: site, size, local infiltration, node and distant metastases, architectural pattern, nuclear atypia, presence of necrosis, lymphovascular and perineural invasion, proliferation, BCL10, trypsin, carboxyl ester lipase, amylase, lipase, PDX1, cytokeratin 19 (CK19), CK7, p53, and β-catenin expression. Twelve cases showing >30% of endocrine cells were reclassified as mixed acinar-neuroendocrine carcinomas, whereas 1 tumor was reclassified as a mixed ductal-acinar carcinoma and was excluded from the statistical prognostic evaluations. BCL10 and trypsin were the most reliable immunohistochemical markers, whereas amylase and lipase were not. Surgery was statistically correlated with a better prognosis (P=0.0008). Among resected tumors there was no difference in survival between ACCs and mixed acinar-neuroendocrine carcinomas, and factors that significantly correlated with poor prognosis were size >6.5 cm (P=0.004), lymph node (P=0.0039) and distant (P=0.008) metastases, and UICC stage (P=0.009). Stage was the only independent prognostic factor at multivariable analysis, and the best prognostic discrimination was observed on grouping together stages I and II and grouping together stages III and IV, suggesting a simplification of the UICC staging for such cancers. In addition, vascular and perineural invasion and CK19 and p53 expression showed a trend for poor prognosis, not reaching statistical significance.
Collapse
|
|
14
|
Kittaka H, Takahashi H, Ohigashi H, Gotoh K, Yamada T, Shingai T, Motoori M, Kishi K, Noura S, Fujiwara Y, Ohue M, Tomita Y, Yano M, Ishikawa O. Multimodal treatment of hepatic metastasis in the form of a bile duct tumor thrombus from pancreatic acinar cell carcinoma: case report of successful resection after chemoradiation therapy. Case Rep Gastroenterol 2012; 6:518-22. [PMID: 22949891 PMCID: PMC3433019 DOI: 10.1159/000341915] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/30/2022] Open
Abstract
Pancreatic acinar cell carcinoma (ACC) is a rare tumor, and its pathophysiology has not been well understood. Treatment strategies for hepatic metastasis originating from ACC remain controversial. We report the case of a 66-year-old woman who had undergone total pancreatectomy from ACC 7 years prior to clinical presentation. Contrast-enhanced computed tomography imaging revealed a tumorous lesion measuring 7 cm in length and 1 cm in diameter and extending along the intrahepatic bile duct (B6), which showed mild enhancement in the early phase and modest washout in the late phase. This lesion was diagnosed as hepatic metastasis primarily in the form of a bile duct tumor thrombus originating from the prior ACC by the pathological evaluation of the fine needle biopsy specimen. The patient underwent preoperative gemcitabine-based chemoradiation therapy followed by subsequent surgical resection, which included subsegmentectomy (S6) of the liver and complete removal of the bile duct tumor thrombus. The patient has had no recurrence during the past 8 months since her last surgery. Multimodal treatment including preoperative chemoradiation therapy might be beneficial especially for marginally resectable cases of ACC.
Collapse
Affiliation(s)
- Hirotada Kittaka
- Department of Surgery, Osaka Medical Center for Cancer and Cardiovascular Diseases, Osaka, Japan
| | | | | | | | | | | | | | | | | | | | | | | | | | | |
Collapse
|
|
15
|
Armstrong MD, Von Hoff D, Barber B, Marlow LA, von Roemeling C, Cooper SJ, Travis P, Campbell E, Paz-Fumagalli R, Copland JA, Colon-Otero G. An effective personalized approach to a rare tumor: prolonged survival in metastatic pancreatic acinar cell carcinoma based on genetic analysis and cell line development. J Cancer 2011; 2:142-52. [PMID: 21475719 PMCID: PMC3053537 DOI: 10.7150/jca.2.142] [Citation(s) in RCA: 21] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/12/2011] [Accepted: 02/23/2011] [Indexed: 12/14/2022] Open
Abstract
Acinar cell carcinoma of the pancreas is an uncommon malignancy, accounting for less than 1% of all pancreatic neoplasms. Because of its rarity, only a few retrospective studies are available to help guide management. We report the case of a patient with metastatic ACC who achieved prolonged survival as a result of personalized treatment designed in part on the basis of molecular and in-vitro data collected on analysis of the tumor and a cell line developed from the liver metastasis. To our knowledge, this represents the first human cell line of ACC. The molecular findings on this case and this patient's cell line may be of use in the management of future cases of this rare tumor and allow the identification of potential novel targets for the effective treatment of this disease.
Collapse
|