The Clinical Practice Guidelines for Pancreatic Cancer were revised and re-published in July 2022, for the first time in 3 years. Revisions were incorporated according to the "Medical Information Network Distribution Service (Minds) Handbook for Clinical Practice Guideline Development 2020," which provides guidance for guideline development under direction of a new committee for revisions including 140 specialists. General remarks, introducing issues on which consensus is considered to have been reached, were placed in the first half of the revised guidelines. In the second half, important debatable clinical issues were carefully selected and 73 clinical questions and 112 statements with algorithms for diagnosis, treatment, chemotherapy and precision medicine for pancreatic cancer were provided. It is of particular significance in this revision that a group including 4 patient and public representatives and 4 health professionals was actively involved in both the development and implementation of the guidelines.

The Clinical Practice Guidelines for Pancreatic Cancer 2022 were revised in July 2022. In the new edition, 3 clinical questions (CQs) were described in "general statements" as background questions, while 10 CQs including 5 on "precision medicine" and a column were added. Four new CQs were added related to risk factors "diabetes", "intraductal papillary mucinous neoplasms", "chronic pancreatitis" and "genetic risks", which had been discussed in "general statements" in the previous edition. A column on "role of the medical checkup for the diagnosis of pancreatic cancer" was also added. Two previous CQs related to "contrast-enhanced computed tomography" were described in "general statements" as universally acknowledged concepts in the new edition. The diagnostic strategy in the new edition clarified that transabdominal ultrasonography is the first step for evaluation although it is limited for imaging the whole pancreas. A previous CQ for "general pathological diagnosis" was described in "general statements" as a background question, while two CQs on "transabdominal ultrasound guided fine needle biopsy" and "needle biopsy for the diagnosis of gene mutations" were added to the new edition.

In the Clinical Practice Guidelines for Pancreatic Cancer 2022 from the Japan Pancreas Society, a new "Precision Medicine Group" was established and Clinical Questions and statements were developed for diagnosis (D4~D8) and treatment (LC4/MC4). As for assessing the risk of cancer development in individuals without cancer, from the disease entity of familial pancreatic cancer, research on cancer predisposition genes or surveillance has been ongoing and germline genetic testing is weakly recommended (D6). As for finding effective treatments based on genetic information, a pivotal phase III study (POLO trial) indicated that olaparib as maintenance therapy after treatment with a platinum regimen prolongs progression-free survival in patients harboring germline BRCA1/2 pathogenic variants (D6, LC4/MC4). In addition, various cancer genetic panel tests have been recently implemented clinically (D5, D5L) and each situation will lead to increased chances of detecting secondary findings of germline pathogenic variants (D7/D8), and development of infrastructure. Although the evidence for these statements is currently insufficient and all are weak recommendations, this is an area with an extremely strong societal need.

The Clinical Practice Guidelines for Pancreatic Cancer 2022 were newly revised in July 2022. Compared with the previous 2019 version, three clinical questions (CQs) were created, five CQs/statements were modified, and two CQs were abolished, resulting in 20 CQs inquiring about surgical and adjuvant therapies being established in the 2022 version. This article describes the revision to CQs and statements in surgical and adjuvant therapies of the Clinical Practice Guidelines for Pancreatic Cancer 2022.

The clinical practice guidelines for Pancreatic Cancer 2022 from the Japan Pancreas Society established nine clinical questions (CQ) for the use of radiation therapy. As first-line treatment for patients with locally advanced unresectable pancreatic cancer (LAUPC), chemoradiotherapy or chemotherapy alone is recommended, as in the Clinical Practice Guidelines for Pancreatic Cancer 2019. Induction chemotherapy prior to chemoradiotherapy using gemcitabine hydrochloride alone is not recommended in patients with LAUPC. Two new CQs regarding high-precision radiotherapy (intensity modulated radiotherapy, stereotactic body radiotherapy, and particle therapy) and hyperthermia for LAUPC were adopted. An inncreased radiation dose using high-precision radiotherapy is recommended for patients with LAUPC. In a CQ about the utility of radiotherapy for patients with postoperative metastatic/recurrent pancreatic cancer, radiotherapy is recommended for patients with local recurrence and regional lymph node metastases or lung metastases as in the 2019 version.

For chemotherapy in the Clinical Practice Guidelines for Pancreatic Cancer 2022, as in the 2019 edition, first-line chemotherapy for patients with locally advanced, unresectable pancreatic cancer (LC1), second-line chemotherapy for patients with unresectable pancreatic cancer [LC2 (MC2)], and first-line chemotherapy for patients with distant metastases from pancreatic cancer (MC1) were established as Clinical Questions (CQ). The duration of treatment with chemotherapy for patients with unresectable pancreatic cancer, which had previously been designated as a CQ, was moved to the general consensus section, and a new CQ [LC3 (MC3)] was established for first-line chemotherapy for elderly patients. A new CQ (BA1) was established for preoperative adjuvant therapy for patients with borderline resectable pancreatic cancer. For each CQ, another systematic review was conducted and meta-analyses performed as necessary to determine recommendations.

In the previous Clinical Practice Guideline 2019, Clinical Questions (CQ) included ①approach routes for biliary drainage for patients with unresectable pancreatic cancer (PC), ②preoperative biliary drainage for PC with obstructive jaundice, ③biliary drainage for unresectable PC with obstructive jaundice, and ④selection of treatment for unresectable PC with gastrointestinal obstruction. In this revision, we reviewed current clinical issues, including ⑤respond to acute pancreatitis and cholecystitis associated with biliary stents, ⑥respond to obstruction of self-expanding metallic stents (SEMS), ⑦methods of biliary drainage for PC with gastrointestinal obstruction and obstructive jaundice, and ⑧indications for SEMS for chemotherapy and radiotherapy for unresectable PC with obstructive jaundice. As a result of careful consideration and discussion, ⑦ and ⑧ were set as CQs, and ⑤ and ⑥ were designated as columns. The 2022 edition consists of six CQs and two columns.

Since many patients with pancreatic cancer are diagnosed at a time when the lesion is unresectable and present with a variety of symptoms at diagnosis, it is necessary to initiate interventions aimed at palliation of symptoms and improvement of quality of life in the early stages of the disease. In the Pancreatic Cancer Treatment Guideline 2022, clinical questions (CQs) were defined for approaches required for early palliative treatment in the treatment of patients with pancreatic cancer (mental and psychological care and advance care planning for patients with pancreatic cancer and their families, and the utility of communication skills training) and for symptom palliation (pain management, peripheral nerve damage control, cachexia, thrombosis and ascites management) in the treatment of patients with pancreatic cancer, respectively. Although there is insufficient evidence for any of the CQs, they are considered important in the management of patients with pancreatic cancer, and a systematic review resulted in recommendations.

Focal pancreatic stenosis and focal pancreatic atrophy are features of early-stage pancreatic cancer including carcinoma in situ. We have performed endoscopic retrograde pancreatography (ERPs) on patients with focal pancreatic stenosis with no apparent tumor seen despite multiple imaging studies. Clinical data were collected from 36 patients over 37 sessions who underwent pathological examination by ERPs from January 2010 to December 2020. This included 18 males and 19 females, median age 74 years, and median observation period of 952 days. Of the 37 sessions, 12 were malignant, and 25 benign lesions. The sensitivity of ERP cytology was: pancreatic juice cytology 50%, brush cytology 57%, SPACE 50%, and whole ERP cytology 67%. The malignant group had less acute pancreatitis history, and more males, more stenosis length less than 5mm, and more focal pancreatic atrophy compared to the benign group. Within the 4 malignant sessions that could not be diagnosed by ERP cytology, 2 were diagnosed with metastases within one year of the final ERP session. About 30% of focal pancreatic stenosis without tumor being detected was malignant, especially in patients with shorter length stenosis and with focal pancreatic atrophy. ERP cytology is a valuable method for detecting pancreatic cancer.

A 45-year-old man diagnosed with a serous cystic neoplasm of the pancreatic body was followed. He had an unremarkable medical history. He was referred with an enlarging cyst and increasing CA19-9 level. Computed tomography scan showed a multifocal cystic lesion with a maximum diameter of 45mm in the pancreatic body and portal annular pancreas (PAP) with suprasplenic vein fusion. The CA19-9 level (reference: <37) was 12 at the beginning, but increased to 122 six months later.

Laparoscopic distal pancreatectomy was performed. The ventral and dorsal portions were separated using a stapler at the level of the portal vein. The postoperative course was uneventful, with no postoperative pancreatic fistula (POPF). PAP is often unrecognized due to its lack of clinical significance. However, it should be accurately diagnosed preoperatively because of the increased risk for of POPF. In distal pancreatectomy, it is recommended to separate the ventral and dorsal pancreas, individually, at the level of the portal vein because the post-fusion pancreas is thicker. The surgeon should be aware of the anatomical characteristics of PAP and the risks of POPF before performing a resection.

A 71-year-old man was treated with tumor-forming pancreatitis in the pancreatic head. The tumor presented with rapid growth and duodenal invasion. Pancreaticoduodenectomy was performed with the diagnosis of carcinoma based on duodenal invasion with hemorrhage. Histopathological findings showed acinar structures with rosette-arranged circular cells. Immunohistochemical examination was positive for BCL-10 and INSM-1 and acinar cell carcinoma with neuroendocrine differentiation. CEA elevation and multiple liver metastases were found six months after resection. Needle biopsy of the liver metastases showed a recurrent acinar cell carcinoma component which was immunohistochemically positive for BCL-10 and INSM-1. A BRCA2 genetic mutation was shown by multi-gene cancer panel testing from the primary pancreatic tumor. Modified FOLFILINOX therapy followed by maintenance olaparib therapy was administered. The patient is alive with continuous shrinkage of metastatic lesions 20 months after resection.

A 60-year-old man presented with worsening diabetes. Computed tomography scan showed a mass in the tail of the pancreas. Distal pancreatectomy with lymph node dissection was performed with a preoperative diagnosis of pancreatic cancer. Pathological findings showed pancreatic intraductal tubulopapillary carcinoma, invasive pN1b (9/31+), pT3N1bM0 pStage IIB. The postoperative course was uncomplicated. Yamaguchi first described intraductal tubulopapillary neoplasm (ITPN) in 2009 as a rare pancreatic neoplasm. ITPN can be difficult to distinguish from other intraductal tumors and typical pancreatic cancer, and it was challenging to make a preoperative diagnosis for this patient. Since tumor progression is relatively slow, lymph node and distant metastases are rare. Lymph node metastases are reported to be present in about 3.4%. In this patient, lymph node metastases were present, and close follow-up is necessary.