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Open Access, Volume 3
A Rare Adenocarcinoma of Ectopic Pancreatic fromDuodenum: A Case Report
Zhiwei Chen; Kangshou Liu; Mingrong Cao*
Department of General Surgery, The First Affiliated Hospital, Jinan University, Guangzhou, China.
Ectopic pancreas is a rare disease in which pancreatic tissue appears outside the pancreas. Malignanttransformation of ectopic pancreatic tissue is uncommon. We present a case of ectopic pancreaticmalignant transformation from the first part of the duodenum. A 68-year-old male was referred to ourhospital with recurrent upper abdominal pain for 3 months. A massive mass in the right upper abdomenwas revealed by computed tomography, which was closely related to the duodenum. The possibility of astromal tumor was discussed. The pancreatic adenocarcinoma was surgically resected, and postoperativepathology revealed that it was moderately differentiated. Imaging examination and tumor-specific antigentesting revealed no obvious signs of tumor recurrence up to 6 months after surgery. Ectopic pancreaticmalignant transformation is extremely rare, primarily occurring in the gastrointestinal tract’s submucosa,and diagnosis is difficult. CT, MR, endoscopic ultrasonography, and tumor-specific antigen should be addin diagnosis,and once detected, surgery should be performed as soon as possible.
Keywords: Duodenum; Ectopic pancreas; Ductal adenocarcinoma.
Abbreviations: CT: Computed Tomography; MRI: Magnetic Resonance Imaging; CA 19-9: Carbohydrate Antigen 19-9; CA 125: Carbohydrate Antigen 125; EUS-FNA: Endoscopic Ultrasonography-GuidedFine-Needle Aspiration.
Manuscript Information: Received: Mar 15, 2023; Accepted: Apr 05, 2023; Published: Apr 14, 2023
Correspondance: Mingrong Cao, Department of General Surgery, The First Affiliated Hospital, Jinan University, Guangzhou, China.Tel: +8613247377319 & +8613392692226; Email: firstname.lastname@example.org
Citation: Chen Z, Liu K, Cao M. A Rare Adenocarcinoma of Ectopic Pancreatic from Duodenum: A Case Report. J Surgery. 2023; 3(1):1088.
Copyright: © Cao M 2023. Content published in the journal follows creative common attribution license.
Ectopic pancreas, also known as abnormal pancreas, was firstobserved in 1727 in an ileal diverticulum . Ectopic pancreasis a condition in which pancreatic tissue appears outside of theoriginal pancreas and has no vascular or nerve connection topancreas . Ectopic pancreas is most commonly found in thegastrointestinal tract, but it can also be found in the biliary tract,liver, lung, brain, and other areas [3-7]. Malignant transformationof an ectopic pancreas is uncommon. According to a study, from2000 to 2020, only 12 cases of ectopic pancreatic malignancy ofduodenum were reported in Pubmed . We present a case ofectopic pancreatic cancer that originated in the first portion ofthe duodenum.
A 68-year-old male presented to our hospital with epigastricpain and weight loss for three months. PET-CT in the other hospital indicated a large abdominal mass involving the gastric antrum and duodenum, which was considered to be gastrointestinalstromal tumor with right superior phrenic, portal vein and retroperitoneal lymph node metastasis. Following admission, relevantexaminations were performed, and the patient’s serum CA19-9were 153.2 IU/L and CA125 were 101.3 IU/L. Combined with upper abdominal MRI (Figure 1 A,B) and gastroscopy (Figure 1 C,D),the possibility of gastrointestinal stromal tumor was still considered to be high. With the consent of the family, the operationwas performed.During the operation, it was discovered that thetumor originated from the first portion of the duodenum and wasclearly not associated with the pancreas. Then, a distal gastrectomy was performed along with regional lymph node dissection.Pathological findings revealed that it was a moderately differentiated ductal adenocarcinoma infiltrating the muscular layer of theduodenum, with vascular invasion and lymph node metastasis ofthe duodenal ligament, but no nerve invasion (Figure 2). Following surgery, the patient underwent two courses of gemcitabinechemotherapy and then refused further treatment. Six months after surgery, abdominal CT (Figure 3) revealed no signs of tumor recurrence, and serum CA19-9 and CA125 levels remained normal.
Figure 1A,B: Preoperative abdominal magnetic resonance imagingshowed a huge mass in the right upper abdomen, which was closelyrelated to the duodenum and gastric antrum. C,D Preoperative gastroscopy showed protruding lesions in the first part of duodenumand gastric antrum, which was considered as lateral wall compression.
Patients with ectopic pancreas usually have no specific symptoms, though a few may experience non-specific symptoms suchas abdominal pain, dyspepsia, or digestive tract obstruction dueto inflammation and tumor . Ectopic pancreas is typically foundin the submucosa, making imaging and general endoscopy difficult to diagnose. Although endoscopic ultrasound can help indiagnosis, a definitive diagnosis still requires a fine-needle aspiration biopsy or post-operative pathological biopsy .
The risk of malignant transformation of ectopic pancreas isvery low, estimated to be between 0.7% and 1.8% in the literature[10,11]. About one-third of all cases of ectopic pancreatic canceroccur in the stomach, followed by the duodenum and jejunum,with cases also occurring in the esophagus, spleen, rectum, mesentery, liver, Meckel diverticulum, and brain . Because of the disease's different location, ectopic pancreatic cancer can causea variety of nonspecific clinical symptoms, the most common ofwhich are gastrointestinal and systemic manifestations, such asabdominal pain, nausea, dyspepsia, and weight loss, and a fewpatients may experience abnormal defecation, gastrointestinalbleeding, hypoglycemia, and so on . About half of the patientshad elevated tumor markers, with the most common being an increase in serum CA 19-9, but the positive rate of tumor markerswere lower than pancreatic cancer . In our case, the patientexperienced abdominal pain, weight loss, and gastrointestinal bleeding, as well as an increase in the serum CA 19-9 and CA 125.However, these differences are insufficient to differentiate it fromgastrointestinal stromal tumor, gastrointestinal neuroendocrinetumor, gastric carcinoid, gastric lymphoma, and gastric cancer.Imaging, such as abdominal CT and MRI, can only pinpoint thelocation of the tumor and cannot provide a definitive diagnosis.Endoscopy has the function of differential diagnosis, but tissuebiopsy, even if performed, is not diagnostic because the sampling location is usually superficial. Endoscopic ultrasonographyis considered to be an effective method for the diagnosis of ectopic pancreas. Submucosal lesions can be cytologically evaluatedwhen combined with fine needle aspiration biopsy, making thediagnosis of ectopic pancreatic malignant transformation morereliable . The EUS-FNA was not performed in our case becausethe patient's abdominal pain was severe and required curative orpalliative surgery. Pathological diagnosis of surgical resection istypically used as the gold standard for ectopic pancreatic cancerdiagnosis. Some researchers proposed diagnostic criteria for ectopic pancreatic cancer as early as 1974 : (1) The tumor must bein or near the ectopic pancreas; (2) the transition zone betweenpancreatic structure and cancer shall be observed, while metastatic tumor or adjacent malignant tumor infiltration ought to beexcluded; and (3) non-neoplastic ectopic pancreatic tissue wassupposed to include well-developed acini and ductal structures.
Figure 2: Microscopically, the tumor was diagnosed as moderatelydifferentiated adenocarcinoma extending from the submucosa tothe musculi propria of the duodenum. Normal pancreatic tissue wasobserved near the tumor, suggesting the presence of an ectopic pancreas.
Figure 3: No tumor recurrence or metastasis was found in the wholeabdominal CT reexamination 6 months after surgery (the arrow indicates the original tumor location).
Because of its rarity, treatment for ectopic pancreatic canceris limited. Surgical resection is usually the first option, and different surgical methods, such as pancreaticoduodenectomy, distalgastrectomy, pylorus-preserving pancreaticoduodenectomy, andsubtotal stomach-preserving pancreaticoduodenectomy, can bechosen depending on the specific location of the tumor [13-15].If the intraoperative frozen pathological examination reveals thatthe tumor is malignant, regional lymph node dissection should beperformed routinely. There is currently no evidence that chemotherapy is effective in the treatment of ectopic pancreatic cancer[6,15]. According to studies, the overall survival time of ectopicpancreatic cancer after surgery is longer than that of pancreaticcancer, and approximately 39% of patients have a survival time ofmore than one year after operation, with a low probability of recurrence after surgical resection, which may be related to the factthat ectopic pancreatic cancer manifests clinical symptoms earlierthan pancreatic cancer .
Ectopic pancreas is becoming increasingly common as endoscopic detection and ultrasonic endoscopic technology becomemore widely available. Although the likelihood of malignant transformation of an ectopic pancreas is low, the possibility of malignant transformation should not be overlooked. Specialists shouldhave an understanding of the common sites, clinical characteristics, histological and pathological features, as well as diagnosisand treatment methods for ectopic pancreatic cancer.
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Pancreatic cancer is notorious for being highly aggressive and difficult to treat.How rare is ectopic pancreas? ›
Ectopic pancreatic tissue has also been seen in the ileum, Meckel diverticulum, gallbladder, common bile duct, splenic hilum, umbilicus, lung, and in perigastric and periduodenal tissue. In autopsy series, the average frequency of ectopic pancreas is between 1% and 2% (range 0.55% to 13%).How rare is pancreatic adenocarcinoma? ›
So, affecting 13 out of 100,000 people, pancreatic cancer is considered rare. While about 90% of pancreatic cancer cases are pancreatic adenocarcinoma, there are also types of pancreatic cancer – such as pancreatic neuroendocrine tumors (PNETs) – that are even less common.What is ectopic pancreatic tissue in duodenum radiology? ›
Ectopic pancreatic tissue, also known as heterotopic pancreatic tissue, refers to the presence of pancreatic tissue in the submucosal, muscularis or subserosal layers of the luminal gastrointestinal tract outside the normal confines of the pancreas and lacking any anatomic or vascular connection with the pancreas ...What is the best treatment for adenocarcinoma of the pancreas? ›
Chemotherapy is the main type of systemic therapy used for pancreatic cancer. However, targeted therapy and immunotherapy are occasionally used and are being studied as potential treatments in select individuals with specific molecular or genetic features (see Latest Research).What is the survival rate of pancreatic adenocarcinoma? ›
Up to 10 percent of patients who receive an early diagnosis become disease-free after treatment. For patients who are diagnosed before the tumor grows much or spreads, the average pancreatic cancer survival time is 3 to 3.5 years.What is the prognosis for ectopic pancreas? ›
An analysis of the eight cases of ectopic pancreatic tissue that had reported on patient prognoses after surgery, including the present case, revealed a 5-year survival rate of 64.3%. The corresponding survival rate for ordinary pancreatic cancer is about 10% .How do you treat an ectopic pancreas? ›
Most patients with ectopic pancreas are asymptomatic although a minority may present with a variety of symptoms, the most common being epigastric pain. Options for treatment for gastric ectopic pancreas include observation, surgery[7,8], or endoscopic resection[6,9-11].What is the rarest type of ectopic? ›
Intramyometrial pregnancy is the rarest subtype of ectopic pregnancy. The first case of intramural pregnancy was reported in 1924. Preoperative diagnosis is difficult and hysterectomy is always required because of delayed diagnosis.Is adenocarcinoma of the pancreas Fatal? ›
About 95% of people with pancreatic cancer die from it, experts say. It's so lethal because during the early stages, when the tumor would be most treatable, there are usually no symptoms.
Surgery is the only chance a patient has to remove all of the pancreatic cancer, according to the ACS. Even if it's not resectable, a pancreatectomy as a palliative option may help in reducing side effects, even when the tumor has spread.Is pancreatic adenocarcinoma fast growing? ›
Pancreatic cancer progresses rapidly, and the prognosis of patients is usually poor. Because most pancreatic cancers show rapid progression, an early detection of pancreatic tumors seems to be difficult.What are the symptoms of ectopic pancreatic tissue? ›
Although ectopic pancreata are usually an incidental finding, they can present with nonspecific symptoms such as abdominal pain, abdominal fullness, nausea, vomiting, anorexia, weight loss, anemia, and melena.What are the 2 types of ectopic tissue? ›
Meckel diverticula may contain ectopic tissue. The two most common types are gastric mucosa and pancreatic tissue.What is an ectopic pancreas? ›
Ectopic pancreas (EP), also recognized as heterotopic, aberrant, accessory, or pancreatic rest [1,2], is a rare congenital condition in which pancreatic tissue is detected in areas where is normally absent and it has no vascular, anatomical, or ductal association with the orthotopic pancreas.Is pancreatic adenocarcinoma painful? ›
Pancreatic cancer can cause severe pain in the abdomen or back. Helping patients manage cancer-related pain is one of the most important aspects of cancer care. The best management for pain combines aggressive therapy with continual assessments to ensure that patients can maintain their quality of life.What is the most effective treatment for adenocarcinoma? ›
Usually the first line of treatment for adenocarcinoma, surgery is done to remove cancer and some of the surrounding tissue. Chemotherapy. This treatment involves using drugs to kill cancer cells. Chemotherapy may be used in a specific area or throughout your entire body.What is the most common pancreatic adenocarcinoma? ›
Also called ductal carcinoma, adenocarcinoma, is the most common type of pancreatic cancer, accounting for more than 90 percent of pancreatic cancer diagnoses. This cancer occurs in the lining of the ducts in the pancreas. It's also possible for adenocarcinoma to develop from the cells that create pancreatic enzymes.Can you live 20 years after Whipple surgery? ›
For some pancreatic patients, however, a complex surgery known as the Whipple procedure may extend life and could be a potential cure. Those who undergo a successful Whipple procedure may have a five-year survival rate of up to 25%.What age does pancreatic adenocarcinoma occur? ›
Most people who develop pancreatic cancer are older than 45. In fact, 90% are older than 55 and 70% are older than 65. However, adults of any age can be diagnosed with pancreatic cancer, as can children rarely.
In conclusion, our study identified predictive factors for actual long-term survival in pancreatic ductal adenocarcinoma using population-based real-world data. Actual survival has only marginally improved over the past decades and patients of all stages still retain a 5-year survival below 5%.What are the complications of ectopic pancreas? ›
Complications of heterotopic pancreas include pancreatitis, pseudocyst formation, malignant degeneration, gastrointestinal bleeding, bowel obstruction, and intussusception. Certain complications may be erroneously diagnosed as malignancy.What are the different types of ectopic pancreas? ›
- proximal duodenum.
- gastric antrum including within gastric duplication cysts.
- proximal jejunum.
- Meckel diverticulum.
Ectopic pancreatic tissue is most often reported in the stomach, duodenum and jejunum, yet many other locations have been reported (Wlaz et al.What helps the pancreas repair itself? ›
Rest and recovery, and fasting gives time to your pancreas to recover and work again for producing insulin and enzymes. Sleeping or fasting enables pancreas to regenerate enzymes.How do you resolve an ectopic? ›
The main treatment options are: expectant management – your condition is carefully monitored to see whether treatment is necessary. medicine – a medicine called methotrexate is used to stop the pregnancy growing. surgery – surgery is used to remove the pregnancy, usually along with the affected fallopian tube.What does pancreatic pain feel like? ›
The main symptom of acute pancreatitis is a severe, dull pain around the top of your stomach that develops suddenly. This aching pain often gets steadily worse and can travel along your back or below your left shoulder blade. Eating or drinking may also make you feel worse very quickly, especially fatty foods.What is the mortality rate of ectopic? ›
Risks of Ectopic Pregnancy
Bleeding from ectopic pregnancy causes 10% of all pregnancy-related deaths, and it's the leading cause of first-trimester maternal death.
Tubal ectopic pregnancy - ampullary, isthmic and fimbrial.Should I be worried about ectopic? ›
An ectopic pregnancy can be serious, so it's important to get advice right away. Your GP will ask about your symptoms and you'll usually need to do a pregnancy test to determine if you could have an ectopic pregnancy.
When a person has stage 4 pancreatic cancer, it means that the cells that grew into a tumor in the pancreas have spread in the bloodstream. The cells start to make tumors in other parts of the body, such as the bones or lungs. This stage is also called metastatic pancreatic cancer.What is stage 3 pancreatic adenocarcinoma? ›
Stage III pancreatic cancer. The tumor is any size and cancer has spread to (a) 4 or more nearby lymph nodes; or (b) the major blood vessels near the pancreas. These include the portal vein, common hepatic artery, celiac axis (trunk), and superior mesenteric artery.What is stage 4 pancreatic adenocarcinoma? ›
What is stage 4 pancreatic cancer? Stage 4 cancer means that the cancer has spread to other parts of your body. It may have spread to the lungs, liver or lining of your tummy (peritoneum). This is advanced or metastatic cancer.How does pancreatic adenocarcinoma spread? ›
Lymph-angiogenesis and lymphangio-invasion and its role in metastasis of pancreatic cancer. Tumor cells spread via the lymphatic system to regional lymph nodes and finally into larger lymphatic vessels which re-enter into the blood vascular system.Why can't you remove a cancerous pancreas? ›
the cancer has grown into or around the blood vessels near the pancreas which means that it can't be removed.What is the surgery in pancreatic adenocarcinoma? ›
As the least common pancreatic cancer surgery, this procedure is used when tumors extend throughout the pancreas. In a total pancreatectomy, surgeons remove the entire pancreas, spleen, gallbladder, common bile duct and portions of the small intestine and stomach.
Adenocarcinoma may be aggressive depending on the organ involved. For example, adenocarcinoma of the lung is the leading cause of cancer deaths in the United States. Lung adenocarcinoma is fairly aggressive and results in a 5-year survival rate of 59.8% if there is no metastasis at the time of diagnosis.What is the actual 5-year survivors of pancreatic adenocarcinoma? ›
In conclusion, our study identified predictive factors for actual long-term survival in pancreatic ductal adenocarcinoma using population-based real-world data. Actual survival has only marginally improved over the past decades and patients of all stages still retain a 5-year survival below 5%.