11. Appendix – Pancreatic Cancer

• Members of families with a known, likely pathogenic/pathogenic gene variant predisposing to pancreatic cancer

Surveillance examinations shall be offered to the following individuals due to the high risk of developing pancreatic cancer:

• all patients with Peutz-Jeghers syndrome or carriers of a probable pathogenic/pathogenic germline variant in the STK11 gene,
• all carriers of a probable pathogenic/pathogenic germline variant in the CDKN2A gene.

Screening/surveillance examinations should be carried out at the following ages:

• in high-risk individuals for familial pancreatic cancer without a known germline mutation: from 50-55 years of age. or at an age of 10 years before the youngest age of onset in the family.
• in individuals with probable pathogenic/pathogenic CDKN2A germline variants or Peutz-Jeghers syndrome (probable pathogenic/pathogenic germline variants STK11 gene): From 40 years of age or at 10 years before the youngest age of onset in the family.
• for carriers of a probable pathogenic/pathogenic variant in the germline in one of the predisposing genes APC, ATM, BARD1, BRCA1, BRCA2, CHEK2, CDKN2A, MLH1, MSH2, MSH6, PMS2, EPCAM, PALB2, TP53, WT1 and at least one first-degree relative with pancreatic cancer: From the age of 50 or 10 years before the youngest age of onset of the disease in the family.
• in patients with hereditary pancreatitis: from the age of 40 if the onset of the disease is unknown, or 20 years after the onset of the disease if this is before the age of 40.

Diagnostic laparoscopy should be used for resectable pancreatic carcinomas in the following constellations:

• if imaging shows a large tumor (see background text)
• if ascites are present
• if an elevated tumor marker value is present (CA 19-9 > 500 U/ml, without cholestasis).
• In these situations, the presence of occult organ metastasis (liver metastasis and/or peritoneal carcinomatosis) is suspected.

When resecting a pancreatic carcinoma, a standardized lymphadenectomy shall be performed.

An extended lymphadenectomy shall not be performed.

8.3

Various chemotherapy regimens can be used in the first-line treatment of advanced or metastatic pancreatic cancer. These include the combination regimens FOLFIRINOX, gemcitabine+nab-paclitaxel and gemcitabine+erlotinib as well as monotherapy with gemcitabine.

8.11

FOLFIRINOX or NALIRIFOX* shall be offered to patients with metastatic pancreatic cancer if the following criteria are met: ECOG performance status of 0-1, favorable comorbidity profile, patient preference, adequate options for supportive therapy.

* off label use

8.12

Gemcitabine-based combination therapies can be used in patients who do not tolerate or do not prefer FOLFIRINOX or NALIRIFOX.

8.22

If a KRAS-G12C mutation is present, treatment with a selective KRAS-G12C inhibitor can be carried out after all therapeutic options for pancreatic cancer have been exhausted.

9.1

All patients with pancreatic cancer shall have access to information about palliative care (e.g. by displaying flyers) regardless of the stage of the disease.

EK: Adopted from S3 Guideline Palliative Medicine (5.1.)

9.2

All patients shall be offered palliative care following the diagnosis of incurable pancreatic cancer, irrespective of whether tumor-specific therapy is carried out.

9.3

Patients shall be offered a needs assessment by an SPC team (specialized palliative care team) following the diagnosis of incurable pancreatic cancer.

9.4

Patients with incurable pancreatic cancer and a highly complex situation shall receive specialized palliative care

9.5

Supportive therapy shall be used in all phases of the diagnosis and treatment of patients with pancreatic cancer.

9.6

All patients with pancreatic cancer shall be screened for typical distressing symptoms. Symptom screening shall be carried out as early as possible at appropriate intervals if clinically indicated or repeatedly during the course of the disease if a patient's disease status changes (e.g. recurrence or progression of the disease).

9.7

Validated and standardized screening instruments shall be used to record the symptoms.

9.8

All patients shall be screened for psychosocial stress. A psycho-oncological screening shall be carried out as early as possible at appropriate intervals if clinically indicated or repeatedly during the course of the disease if a patient's disease status changes (e.g. recurrence or progression of the disease).

EK: Adopted from S3 Guideline Psycho-oncology (7.2)

9.9

Validated and standardized screening instruments shall be used to record psychosocial stress.

The Distress Thermometer or the HADS-D, for example, are recommended as screening instruments.

In addition, individual psychosocial support needs shall be assessed.

EK: Adopted from S3 Guideline Psycho-oncology (7.3)

9.10

Pain history and pain-related clinical examination shall be part of every clinical presentation.

EK: Adopted from S3 Guideline Palliative Medicine (9.1)

9.11

Patients with pancreatic cancer should undergo regular screening for malnutrition (e.g. Nutritional Risk Screening (NRS) or Malnutrition Universal Screening Tool (MUST)).

9.12

In the event of nutritional problems, an individual nutritional assessment and appropriate advice shall be carried out. This should be carried out by a qualified nutritionist.

9.13

If spontaneous food intake remains inadequate despite nutritional advice, supplementary or total enteral nutrition or parenteral nutrition may be considered. The aim is to maintain or improve the patient's nutritional status and quality of life while carefully weighing up the benefits and risks.

9.14

Patients with exocrine pancreatic insufficiency due to pancreatic carcinoma shall receive pancreatic enzyme therapy.

9.15

Patients with pancreatic cancer shall receive an individualized nutritional intervention before and after oncological pancreatic resection.

9.22

In patients with incurable cancer and with a tumor-related obstruction in the gastric outlet and duodenum, endoscopic stenting can be performed to relieve symptoms.

EK: Adopted from S3 Guideline Palliative Medicine (14.31)