Malignant diseases of the pancreas are very rare in children. Only 0.2 % of all malignant diseases are classified as malignant pancreatic tumors.
Treatment in so-called competence centers is also absolutely necessary here in order to use the very special knowledge of these generally rare diseases for the patients. At Hannover Medical School, a team (Department of Pediatric Hematology and Oncology, Radiology and Pediatric Surgery) therefore draws up a treatment plan for each patient that corresponds to the national and international state of knowledge. In this context, there is constant cooperation with the GPOH study groups and international networks. Of course, this intensive cooperation also covers the psycho-social aspects in order to meet the holistic treatment requirements that we have for the sick children, the parents affected and the siblings.

The cells of this type of tumor originate from an epithelial tissue (cover cells). They spread continuously in the pancreas in a field-like manner (blastomatous), and the majority of them release a specific hormone (alphafoetoprotein (AFP)), which can be determined as a marker in the peripheral blood. About 10% of the diseases occur in adulthood - the remaining, i.e. the majority, in early childhood. Genetic changes (which are known, for example, in the so-called Beckwith-Wiedemann syndrome or mutations in the beta-catenin gene) may be present.

In principle, the tumor can develop anywhere in the gland. The disease becomes symptomatic through the more general symptoms such as palpable hardening and resistance in the abdominal area, loss of weight and appetite, jaundice (icterus). The tumor can give rise to secondary tumors (metastasis).

 

Treatment:

After and while performing the necessary so-called imaging diagnostics (e.g. computer tomography (CT), magnetic resonance imaging (MRI)), a computer-assisted puncture of the focus of the disease is performed under anesthesia (if possible during the examination procedure) in order to obtain tissue material for fine tissue diagnostics (examination at the MHH and in parallel to obtain maximum accuracy in the Pediatric Pathology Section / Pediatric Tumor Registry of the University in Kiel) in order to be able to plan the exact, individually suitable therapy. As with other pancreatic tumors, this therapy consists of the complete removal of the tumor. The required radicality is determined together with the pediatric oncology colleagues in the team and, if necessary, also intraoperatively with the examining pathologists. Particular attention is paid to the quality of life after the operation, so that the aim is generally to preserve the stomach and restore the defect using small intestinal structures (pylorus-preserving duodenal pancreatectomy, also partial according to Traverso / Longmire). Traverso / Longmire). In principle, however, all resecting surgical procedures can be used depending on the findings. Pediatric surgery and special visceral surgery expertise are available.

5-year survival rates of up to 80% have been reported for pancreatoblastoma. For less favorable findings with corresponding metastasis, neoadjuvant therapy (chemotherapy) is usually carried out after the operation, which is planned and also carried out by the pediatric oncology colleagues.

 

 

These tumors were also first described by Virginia K. Frantz in 1959. They are considered very rare (2-3% of all pancreatic tumors) and mainly affect young female patients. Although the average age of onset is 21.9 years, these tumors also occur in childhood. The number of cases observed is increasing. However, this observation correlates with the increasing level of awareness.

In this case, too, the tumor can occur at any site of the pancreas. The tumor is malignant to a low degree (low-grade malignant), but it is capable of metastasizing.

The symptoms of the disease are again - as described above - non-specific. An increase in tumor markers in the blood is not recorded.

The measures described above (computer- and image-guided puncture with escalation of invasive diagnostic measures) should also be referred to for diagnostics prior to surgery.

The treatment of choice is also complete surgical removal as described above.

The prognosis is good in the case of complete resection. Factors associated with a less favorable prognosis include tumor rupture, male gender and advanced age.

These tumors are also extremely rare in children and are therefore only briefly mentioned here. As in adults, insulinomas and gastrinomas are the most common. These tumors can also be present as part of a so-called multiple endocrine neoplasia (MEN).
As a rule, these extremely rare diseases are diagnosed by detecting the irregularly secreted hormones in the blood and by imaging procedures (such as sonography, scintigraphy, CT, MRI) (insulinomas lead to considerable changes in the blood sugar level, gastrinomas to increases in gastric acid secretion and to the development of stomach and duodenal ulcers).

Treatment consists of surgery with localization of the tumorous changes, which are often present in several parts of the pancreas, and localized removal (enucleation) of the processes.

If a pancreatic tumor is suspected or diagnosed, your child will be examined by a team of pediatric surgeons, pediatric oncologists and radiologists on the day of admission. It is important that we introduce ourselves as a whole team from the first contact with you so that you know what to expect and we can plan the next steps together.

The final diagnosis can often not be made immediately. For this reason, we usually require a so-called "cross-sectional imaging examination" (e.g. magnetic resonance imaging or "MRI"). A tissue sample of the tumor is also often taken. This can be done either by surgery or by a CT-guided puncture. Possible spread of the tumor to other organs (e.g. lungs or skeleton) can be examined with a normal chest X-ray or other special radiological procedures.

Once these initial examinations have been carried out, we will discuss a rough treatment plan with you. The exact treatment regimen depends on the spread of the tumor and the tumor structure.

An inpatient stay of around 1-3 weeks must be planned for the surgical removal of the tumor. You will be admitted to hospital one day before the actual operation so that we can discuss the upcoming procedure with you in peace and quiet and carry out final blood tests. During the operation, an incision is made across the abdominal wall. The organs above the pancreas are then carefully pushed aside and the tumor is isolated from all surrounding tissue. The aim of the operation is to completely remove the tumor together with the surrounding affected tissue. Particular attention is paid to the quality of life after the operation, so that the aim is generally to preserve the stomach and restore the defect using small intestinal structures (pylorus-preserving duodenal pancreatectomy, also partial n. Traverso / Longmire). Traverso / Longmire). In principle, however, all resecting surgical procedures can be used depending on the findings. The removed tumor is then examined under the microscope by a tissue specialist (pathologist).

The pediatric oncologists will take over the further care of your child after the operation. Here at the center, there is very close cooperation between surgery and oncology. However, the prognosis depends on the type and location of the tumor and therefore cannot be generalized here.