What Is a Pancreatic Endocrine Tumor?

The incidence of pancreatic endocrine tumors is about (1 to 4) per 100,000, accounting for only 1% to 2% of pancreatic tumors, and can occur at any age. The ratio of male to female is about 13: 9, which has gradually increased in recent years.

Liu Zhe

(Deputy Chief Physician)

Department of Hepatobiliary Surgery, Beijing 301 Hospital

Pancreatic endocrine tumors are a class of tumors derived from pancreatic pluripotent neuroendocrine stem cells. They are rare in the clinic, and their symptoms are complex and diverse. They can progress from benign to malignant, have a slow course, and are easily confused with the primary endocrine disease. Insufficient, misdiagnosed and missed diagnosis are common, which can easily lead to long-term misdiagnosis and mistreatment, and even serious irreversible damage.

Western Medicine Name: Pancreatic endocrine tumor
Affiliated Department: Internal Medicine-Oncology

Disease site: pancreas
Multiple groups: Can occur at any age
Contagious: Non-contagious

Clinical manifestations of pancreatic endocrine tumors

Pancreatic endocrine tumors

Pancreatic endocrine tumor disease symptoms

Pancreatic neuroendocrine tumors can be divided into "functional" and "non-functional" tumors according to whether they cause clinical symptoms. The former has corresponding clinical syndromes due to the production of a certain hormone. According to the type of hormone secretion, it can be divided into insulinoma and gastric Tumors, glucagon tumors, vasoactive intestinal polypeptide secretion tumors, somatostatin tumors, and so on, and "non-functional" tumors may not not produce neuroendocrine materials, but do not cause special clinical symptoms. Common clinical manifestations of pancreatic endocrine tumors are shown in Table 1.

Table 1 Pancreatic neuroendocrine tumors and their secreted hormones
Tumor name	Secreted hormone	Clinical manifestation
Insulinoma	insulin	Weakness, sweating, tremor, tachycardia, anxiety, fatigue, headache, dizziness, disorientation, seizures, confusion
Gastrinoma	Gastrin	Refractory or recurrent peptic ulcer (bleeding, perforation), complications of peptic ulcer, diarrhea
VIP tumor	Vasoactive intestinal peptide	Excessive watery diarrhea, flushing, hypotension, abdominal pain
Glucagonoma	Glucagon	Necrotizing migratory rash, glossitis, stomatitis, angular cheilitis, diabetes, severe weight loss, diarrhea
Somatostatin tumor	Somatostatin	Weight loss, gallstones, diarrhea, multiple neurofibromas
Nonfunctional islet cell tumor	None or ambiguous	Symptoms of tumor compression, obstructive jaundice, pancreatitis, duodenal obstruction

Diagnosis and identification of pancreatic endocrine tumors

Diagnosis of pancreatic endocrine tumors

According to the different clinical manifestations caused by different hormones, combined with the corresponding serum hormone levels and other tests, is the first step in the diagnosis of endocrine tumors, and then pancreatic imaging examination for localization, and then make a diagnosis of pancreatic endocrine tumors. For non-functional pancreatic endocrine tumors, they are usually diagnosed by imaging examinations due to compression symptoms or physical examination. Pancreatic endocrine tumors often require surgery, and finally the pathological type and nature are determined by immunohistochemical staining.

There are many methods for the localization and diagnosis of pancreatic endocrine tumors, including ultrasound, CT, magnetic resonance imaging, PET / CT, endoscopic ultrasound, laparoscopic ultrasound or laparoscopic ultrasound, angiography, and so on.

Differential diagnosis of pancreatic endocrine tumors

Mainly differentiating from primary endocrine diseases: rely on imaging examination. If the imaging examination reveals the presence of pancreatic tumors and changes in hormone levels, it can be distinguished from the primary endocrine disease. If the imaging examination cannot confirm the presence of pancreatic tumors, it is difficult to identify.

Early detection requires the clinician's awareness and experience, combined with the patient's clinical manifestations and positive imaging studies to be clear. At present, regular and regular medical examinations are required.

Once a pancreatic endocrine tumor is diagnosed, attention must be paid to the possibility of multiple endocrine tumor type I (MEN1). While testing the corresponding hormone levels, serum calcium, calcitonin, parathyroid hormone, growth hormone, prolactin, etc. Hormone levels to exclude MEN1. ^[1] ^[2]

Pancreatic endocrine tumor disease treatment

Pancreas Endocrine Tumor Treatment Principles

Pancreatic endocrine tumors are treated according to different hormone secretion characteristics and different clinical manifestations.

Pancreatic endocrine tumor surgery

Local excision, pancreatoduodenectomy or pancreatic head resection with duodenum preservation, pancreatic segment resection, pancreatic body and tail resection, etc. were performed according to tumor location, size, and pathological type. According to different hormone secretion characteristics before surgery, we need to control blood sugar, suppress acid, and correct disorders of water, electrolyte, and acid-base balance. Postoperatively supplemented with somatostatin and similar drugs. Commonly used chemotherapy drugs include streptozotocin, fluorouracil, and doxorubicin. For malignant cases with recurrence after surgery, if they can be removed, they should be removed again. For patients with malignant pancreatic endocrine tumors with liver metastasis that cannot be radically resected, tumor reduction surgery, gastrointestinal short circuit surgery, chemotherapy, transcatheter arterial embolization, B-frequency or CT guided radiofrequency ablation thermocoagulation therapy or Cryotherapy for liver metastases and other measures. For cases with limited primary lesions with extensive liver metastases and good general condition, strict selection can be considered for primary lesion resection and combined liver transplantation.

Table 2 Clinicopathological classification criteria for pancreatic endocrine tumors (WHO, 2004)
I. Well-differentiated endocrine tumors
Benign
Limited to the pancreas, no vascular invasion, no perineural invasion, diameter <2 cm; mitosis <2 / 10HPF; Ki-67 positive cells <2%
2. Undefined biological behavior:
Limited to the pancreas, but with one or more of the following characteristics: diameter 2 cm; mitosis 2 to 10/10 HPF; Ki-67 positive cells> 2%; vascular invasion, peripheral nerve infiltration
2. Well-differentiated endocrine cancer
*Low-grade malignant:*
Local infiltration generally visible, surrounding tissues or organs and / or metastases, Ki-67 (+)> 5%
Third, poorly differentiated endocrine cancer
*Highly malignant:*
Nuclear fission> 10 / 10HPF, Ki-67 (+)> 15%, obvious invasion of blood vessels and / or nerves
Attachment: HPF: High Power Field (10 high magnification fields)

^[1] ^[3] ^[2]

Prognosis of pancreatic endocrine tumor disease

The diagnosis and treatment of pancreatic endocrine tumors have made rapid progress in recent years. Especially, non-invasive localized diagnosis represented by pancreatic perfusion CT and three-dimensional reconstruction of blood vessels has basically replaced the complicated and invasive methods in the past. The rate has increased year by year. The survival time of pancreatic endocrine tumors is better than other types of pancreatic tumors. The median survival time of pancreatic endocrine tumors with liver metastases can reach 48 months, and the 5-year survival rate is nearly 40%. In short, the improvement of early diagnosis rate, active radical resection of tumors, extensive clinical trials of drug treatment and other comprehensive treatment measures have significantly improved the prognosis of pancreatic endocrine tumors.