Branch duct intraductal papillary mucinous neoplasms of the pancreas (BD-IPMN) without malignant features rarely developed into invasive cancer. However, invasive cancer is aggressive once an invasive change occurs. We report three cases of invasive cancers which developed in patients with BD-IPMN and they showed grave clinical courses. All patients were diagnosed with BD-IPMN < 3 cm without malignant features on imaging. Invasive cancer was detected at 2.5 years, 3.0 years, and 4.0 years after BD-IPMN detection in each patient. The intervals of invasive cancer and the last follow-up were 9 months, 3 years, and 1.5 years in the three patients, respectively. All patients were diagnosed with locally advanced pancreas invasive cancers and were treated with palliative chemotherapy or conservative management. The patients died at 3 months, 9 months, and 10 months after the diagnosis of invasive cancers, respectively. We report three cases of invasive cancer developed in BD-IPMN patients and followed fatal courses.
Intraductal papillary mucinous neoplasms of the pancreas (IPMNs) are a spectrum of diseases ranging from adenoma, high grade dysplasia, to invasive cancer [
In the present study, we report three cases diagnosed as locally advanced PDAC developed in the patients having BD-IPMN without malignant features on imaging; the patients followed grave clinical courses (
A 74-year-old man was admitted at our hospital following a five-day history of yellow skin and febrile sense. His past medical history included a pancreas cystic lesion detected two and half years ago by health surveillance. Abdominal computed tomography (CT) scan and magnetic resonance cholangiopancreatography (MRCP) revealed a 2.5 cm-sized multiseptated cystic lesion located at the body of pancreas that was suggested as BD-IPMN (
A 62-year-old man was admitted for abdominal distension and weight loss. Pancreas cystic lesion had been diagnosed three years before. The abdominal CT scan and MRCP revealed a 2.5 cm-sized well-defined multiloculated cystic mass at the head and uncinate process of pancreas with mild MPD dilatation and pancreatic parenchymal calcification, suggested as BD-IPMN with chronic pancreatitis (
A 78-year-old man was admitted for abdominal pain and weight loss. He had received transurethral resection of a bladder tumor 4 years ago and had been followed with the abdominal CT scan. The abdominal CT scan at that time had revealed a 1.5 cm-sized cystic lesion at the pancreatic body, suggested as a BD-IPMN (
In this study, we reported three cases of BD-IPMN without malignant features on imaging that progressed to invasive cancers and showed grave clinical courses. BD-IPMN without malignant features usually revealed a favorable clinical course that rarely developed into invasive cancers. Also, PDAC concomitant or derived from IPMN may have more favorable biological behaviors than typical PDAC [
However, a recent study reported that 38% of resected invasive cancer of BD-IPMN show metastases and worse outcomes than those derived from MD-IPMN [
It was difficult to clarify between derived from or concomitant of PDAC in BD-IPMN. Invasive IPMN was defined when the invasive solid mass extended continuously from IPMN, while concomitant cancer was considered to be the case when the invasive solid mass was located separately from the IPMN [
International consensus guidelines to manage BD-IPMN were suggested in 2012 [
Our case series were BD-IPMN < 3 cm without worrisome features (mural nodules, thickened cyst walls, dilated MPD 5-9 mm, abrupt change in MPD caliber with distal pancreatic atrophy, lymphadenopathy). Cases 2 and 3 did not follow the guidelines. Case 2 had been lost for three years and case 3 for one and half years before the diagnosis of invasive cancer. Therefore, it is important to comply with surveillance guidelines in BD-IPMN, as pancreatic cancer is a lethal disease and some portion of BD-IPMN can progress to invasive cancer. However, case 1 was followed by appropriate image studies and by proper interval satisfying the two different guidelines outlined above [
We report three cases of invasive PDAC which developed in the patients having BD-IPMN without malignant features on imaging and they followed grave clinical courses.
The author has no conflicts to disclose.
Image studies of case 1. (A, B) Abdominal CT scan (A) and MRCP (B) at 2.5 years before admission. A 2.5 cm-sized multiseptated cystic lesion(arrows) was visible at the pancreatic body. (C) EUS at 9 months before admission. Bilocular cystic lesion (arrow), measuring 1.8 cm and 1.1 cm, was seen. (D) Abdominal CT scan at admission. A 3.5 cm ill-defined low-density mass and vascular invasion to celiac axis and hepatic artery were observed (arrow). CT, computed tomography; MRCP, magnetic resonance cholangiopancreatography; EUS, endoscopic ultrasonography.
Image studies of case 2.(A-C) Abdominal CT scan and MRCP three years before admission. A 2.5 cm-sized multiloculated cystic mass was seen at head and uncinate process of pancreas (A and C, arrows). Main pancreatic duct dilation (arrow head) and parenchymal calcification (arrow) were revealed at pancreas body and tail (B).(D) Abdominal CT scan at admission. An ill-defined 5 cm-sized low density lesion in the pancreatic head (arrow) was observed. CT, computed tomography; MRCP, magnetic resonance cholangiopancreatography.
Image studies of case 3. Abdominal CT scans at 4 years (A), 3 years (B), 1.5 years (C), and 0 year (D) before admission. A 1.5 cm cystic lesion at body of pancreas was seen (A, arrow). A pancreatic cystic lesion remained unchanged (B, arrow). A pancreatic cystic lesion was increased and pancreatic duct was dilated (C, arrow). A 5.0 cm-sized of ill-defined low density lesion at body of pancreas with invasion to para-aortic and peripancreatic area was observed (D, arrow). CT, computed tomography
Baseline and clinical characteristics of the three eligible patients
Patient number | Age/Sex | Comorbidity | BD-IPMN |
Pancreatic ductal adenocarcinoma |
|||||
---|---|---|---|---|---|---|---|---|---|
Location | Size | Clinical symptoms | CA19-9 | Method of pathologic diagnosis | Interval from BD-IPMN to PDAC | Overall survival | |||
Case 1 | M/74 | None | Body | 2.5 cm | Jaundice, Febrile sense | Normal | Brush cytology | 2.5 years | 3 months |
Case 2 | M/62 | Diabetes mellitus | Head | 2.5 cm | Abdominal distension, Weight loss | Normal | EUS-FNA | 3.0 years | 9 months |
Case 3 | M/78 | Urinary bladder tumor | Body | 1.5 cm | Abdominal pain, Weight loss | Normal | EUS-FNA | 4.0 years | 10 months |
BD-IPMN, branch duct intraductal papillary mucinous neoplasm; PDAC, pancreatic duct adenocarcinoma; M, male; EUS-FNA, endoscopic ultrasonography-fine needle aspiration.