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The surgeon’s view

The surgeon’s view Cancer Imaging (2001) 2, 17–22 Multidisciplinary Symposium — Carcinoma of the Pancreas Monday 15 October 2001, 15.30–Close R C G Russell 149 Harley Street, London W1N 2DE, UK and 51% weight loss. Significant correlation has been Introduction found between dyspnoea and length of survival. Pancreatic cancer remains a deadly disease in which the incidence and mortality are identical. Few patients sur- vive, and the long-term surgical survivors are prone to Pathology recurrence, even 10–15 years later. The incidence of pancreatic cancer has remained static for the last decade Currently there are two major classifications; the TNM with male and female incidence being similar; approxi- created by the UICC and a Japanese system (JPN) mately 10 new cases occur per 100 000 population per created by the Japan Pancreas Society. UICC is based annum. The recent trend has been for the disease to on tumour size and the presence or absence of nodal or occur in older age groups and now in the UK 50% remote metastases, whereas the JPN classification has of patients are over 75 years of age. Risk factors are more criteria; for example, a more detailed evaluation of few and the aetiology appears multifactorial; smoking the degree of invasion into vessels, retroperitoneum and appears the most important, with chronic pancreatitis, nerves. Evaluation of the remote nodal, as well as the hereditary predisposition, diabetes and carcinogens hepatic and peritoneal metastases is more neatly differ- being of importance. Approximately 90% of tumours are entiated. For individual tumours, the WHO classifica- [1] ductal carcinomas with the remaining 10% being a wide tion groups the exocrine tumours according to their range of different tumour types with a remarkably biological behaviour. The division between benign and variable prognosis. The importance of defining the his- malignant is not sharp, but rather a gradual transition tology is probably of greater importance in pancreatic analogous to some ovarian tumours. The borderline cancer than in most other tumours. Characteristically, group, known as ‘tumours of uncertain malignant tumours present late. potential’ are defined by the grade of dysplasia and their potential to become malignant. In the 1996 edition of the classification, a new name ‘intraductal papillary mucinous tumour’ replaced vague terms such as ‘mucin- Symptoms and signs producing tumour’, ‘mucinous duct ectasia’ and ‘solid The suspicion of pancreatic cancer arises because of pseudopapillary tumour’ (Table 1). Ductal adenocarci- symptoms such as pain, jaundice, weight loss, anorexia nomas compose 90% of pancreatic tumours. Sixty-five or early satiety. Less common symptoms include venous per cent of these tumours arise in the head of the thrombosis, diarrhoea, new onset diabetes or acute pancreas, 15% originate in the body and tail, and 20% pancreatitis. Back pain is ominous and associated with a diffusely involve the gland. The tumour infiltrates and shortened survival. Painless jaundice is the most com- creates a fibrous response such that the extent is difficult mon presentation in patients with a potentially resect- to appreciate and indeed, microscopically, extends able and curable lesion (52% of patients with a significantly beyond the grossly recognized tumour resectable lesion). Pain, however, is the most frequent mass. At the time of presentation most tumours have symptom and is present in 80% and 85% of patients with metastasized to the lymph nodes, and later extend to locally unresectable and advanced cancer, respectively. the liver (80%), peritoneum (60%), lungs and pleura The combination of pain and jaundice is present in 50% (50–70%), and the adrenal glands (25%). Most ductal of patients with a locally unresectable tumour. adenocarcinomas infiltrate the perineural, lymphatic In a prospective study of 39 patients with unresectable and vascular channels. pancreatic cancer, 82% had pain at the time of diagnosis, Endocrine pancreatic tumours arise from the diffuse 64% anorexia, 62% early satiety, 54% sleep problems, endocrine system (DES), the cells of which share a 1470-7330/01/01017 + 06  2001 International Cancer Imaging Society 18 Multidisciplinary Symposium — carcinoma of the pancreas Table 1 WHO classification of exocrine pancreatic transhepatic cholangiography (PTC) and computed tumours tomography (CT) scanning were associated with the highest provision of information, but the most cost- Benign tumours effective investigations were ultrasound and liver func- Serous cystadenoma tion tests. A software program produced a diagnostic Mucinous cystadenoma accuracy of 84%. Intraductal papillary-mucinous adenoma The role of investigation is to determine whether or Mature teratoma not a tumour is suitable for resection or palliation. The Borderline tumours (uncertain malignant potential) objective of palliation, which is the lot of the majority Mucinous cystic tumour with moderate dysplasia of patients, is to prolong life by relieving suffering, Intraductal papillary-mucinous tumour with moderate dysplasia Solid-pseudopapillary tumour namely the obstructive jaundice and the luminal obstruction. Non-operative approaches are the preferred Malignant method. Evidence is not available to suggest that pallia- Severe ductal dysplasia — carcinoma in situ Ductal adenocarcinoma tive resection has any advantage over non-operative Mucinous non-cystic carcinoma palliation. Surgical palliation is reserved for the patient Signet ring carcinoma who is considered operable but at surgery found not to Adenosquamous carcinoma be so. Thus the surgical view of pancreatic cancer is that Undifferentiated (anaplastic) carcinoma of operating on a suitable patient. Mixed ductal-endocrine carcinoma Osteoclast-like giant cell tumour Serous cystadenocarcinoma Mucinous cystadenocarcinoma Noninvasive Indications for resection Invasive Intraductal papillary-mucinous carcinoma The ideal tumour is one which is locally confined and Noninvasive has a good prognosis. The histology is important, but Invasive (papillary-mucinous carcinoma) this can usually be surmised from investigations. The Acinar cell carcinoma Acinar cell cystadenocarcinoma rare benign and good prognostic non-duct cell carci- Mixed acinar-endocrine carcinoma nomas have characteristic radiological features and are Pancreatoblastoma more prevalent in the body and tail of the pancreas than Solid-pseudopapillary carcinoma in the head of the gland. Ampullary tumours are diag- Miscellaneous carcinomas nosed endoscopically but their extent needs to be assessed radiologically. Biopsy, which is usually achieved by percutaneous techniques, is limited to the patient who is considered inoperable, however, rarely, as number of antigens with nerve elements, hence the name in neuroendocrine tumours, a biopsy is required if the ‘neuroendocrine’. The importance of these tumours is tumour is submitted for pre-operative chemoradio- that the relatively benign and differentiated tumours give therapy. Indeed, it is recommended that before starting rise to systemic endocrine syndromes (insulinoma, either chemotherapy or radiotherapy, a biopsy should be gastrinoma, glucagonoma, VIPoma, PPoma), while the performed. more malignant and non-hormone-producing tumours grow slowly and have a longer life-cycle than the ductal cancer. Similarly, ampullary tumours have different Operability behavioural patterns and present early due to obstruc- tion of the ampulla of Vater; their prognosis is similar to For the patient suitable for resection the surgeon needs colonic tumours. It is important to characterize precisely to know that the disease is confined to the pancreas and the pancreatic tumour in order to define a treatment that a standard resection will encompass the tumour. plan and consider prognosis. The standard resection is a pancreatoduodenectomy which excises the duodenum, the head of the pancreas and the lower bile duct. Extended procedures which involve radical lymphadenectomy have not improved Diagnosis survival. Incomplete excisions do not improve survival; The therapeutic advantages of an early, accurate and it behoves the surgeon to select the patient for precise diagnosis are obvious and indicate the need for resection in which the tumour is completely excised both standards for history-taking and physical examination macroscopically and microscopically. as well as non-invasive, highly sensitive and specific The markers of inoperability are distant metastases, methods of investigation. The diagnosis of carcinoma in involved lymph nodes away from the pancreas and the head of the pancreas can be made in 90% of patients involvement of the portal vein or superior mesenteric on the basis of clinical presentation. In a study of 356 artery. State-of-the-art helical CT scanning, with or patients with pancreatobiliary carcinoma, the clinical without magnetic resonance imaging (MRI) should and diagnostic tests reviewed showed that percutaneous accurately predict operability in 60–70% of patients. Multidisciplinary Symposium — carcinoma of the pancreas 19 Laparoscopic examination of the abdominal cavity will, Key points at most, improve this yield by 10%. The effect of (1) Incidence of disease far more common in the elderly endoscopic ultrasound, peritoneal washings, bone mar- (2) Histological type of great importance prognostically row biopsy and PET scanning have yet to be fully (3) Early diagnosis essential evaluated. It is cost-effective to limit the investigations to (4) Operability determined by high quality imaging state-of-the-art CT scanning and accept a slightly higher (5) Resection associated with a <5% mortality and a rate of inoperable cases which can be managed by 20% 5-year survival surgical palliation. With selection the surgical outcome should be a mortality of under 5% and a 5-year survival of 20% for duct cell carcinomas. The quality of life for patients Further reading undergoing pancreatoduodenectomy is good and their [1] Kloppel G, Solcia E, Longnecker DS, Capella C, Sobin LH. initial recovery period is no longer than 3 months before Histological typing of tumours of the exocrine pancreas. achieving an acceptable normalization of life. Adjuvant International Histological Classification of Tumours, 2nd chemotherapy improves survival by 10%. Radio- Edition. Berlin: Springer. World Health Organisation, 1998: therapy does not improve survival and it may even be 1–61. harmful. [2] Lillemoe KD, Cameron JL, Yeo CJ, Sohn TA, Nakeeb A, Sauter PK, Hruban RH, Abrams RA, Pitt HA. Pancreaticoduodenectomy. Does it have a role in the palliation of pancreatic cancer? Ann Surg 1996; 223: 718–25. [3] Krech RL, Walsh D. Symptoms of pancreatic cancer. J Pain Conclusion Symptom Management 1991; 6: 360–7. [4] Yeo CJ, Cameron JL, Sohn TA, Coleman J, Sauter PK, The surgeons’ view of pancreatic carcinoma is that the Hruban RH, Pitt HA, Lillemoe KD. Pancreaticoduo- diagnosis should be made early, the investigations denectomy with or without extended retroperitoneal lym- phadenectomy for periampullary adenocarcinoma: comparison should be undertaken expeditiously and the investiga- of morbidity and mortality and short-term outcome. Ann Surg tions limited to helical CT scanning. For those patients 1999; 229: 613–22. with an inoperable tumour a biopsy is appropriate. Operative mortality should be low and the quality of life The digital object identifier for this article is: 10.1102/ following recovery from resection good. 1470-7330.2001.010 Carcinoma of the pancreas: detection and staging using CT and MRI Jay P Heiken Mallinckrodt Institute of Radiology, Washington University School of Medicine, St Louis, Missouri, USA Our ability to diagnose pancreatic carcinoma has improved reduce the percentage of patients who are unnecessarily substantially over the past 20 years, owing to major subjected to laparotomy. advances in pancreatic imaging, including the development CT has become established as the primary initial imaging of US, CT and MRI. Despite these advances, however, the method for both detection and staging of suspected pancre- prognosis of patients with pancreatic cancer remains dis- atic carcinoma. Most studies have found that CT is highly [1] mal. The overall 5-year survival rate is only 3% , although reliable when it demonstrates features indicating that a [5,6] the 5-year survival rate for patients who undergo pancreatic tumor is unresectable . The positive predictive value [2–4] resection is reported to be approximately 20% . Because (PPV) of a diagnosis of unresectability with helical CT has [7–11] of the very poor prognosis of patients with pancreatic ranged from 92% to 100% . Helical CT is less reliable, carcinoma, many physicians take a nihilistic approach to its however, for predicting that a tumor is resectable [7–11] diagnosis and staging. It is important to keep in mind, (PPV = 76–90%) . Nevertheless, this represents a sub- however, that a large percentage of patients with pancreatic stantial improvement over prediction of resectability with [12–14] cancer who undergo laparotomy for possible curative resec- conventional CT (PPV = 45–72%) . Limitations of CT tion are found to have unresectable disease. Thus, optimi- include: poor ability to demonstrate small hepatic or peri- zation of pre-operative imaging is important in order to toneal metastases; inability to demonstrate microscopic http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Cancer Imaging Springer Journals

The surgeon’s view

Russell, R.
Cancer Imaging , Volume 2 (1) – May 5, 2015
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Publisher
Springer Journals
Copyright
Copyright © 2001 by International Cancer Imaging Society
Subject
Medicine & Public Health; Imaging / Radiology; Cancer Research; Oncology
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1470-7330
DOI
10.1102/1470-7330.2001.010
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Abstract

Cancer Imaging (2001) 2, 17–22 Multidisciplinary Symposium — Carcinoma of the Pancreas Monday 15 October 2001, 15.30–Close R C G Russell 149 Harley Street, London W1N 2DE, UK and 51% weight loss. Significant correlation has been Introduction found between dyspnoea and length of survival. Pancreatic cancer remains a deadly disease in which the incidence and mortality are identical. Few patients sur- vive, and the long-term surgical survivors are prone to Pathology recurrence, even 10–15 years later. The incidence of pancreatic cancer has remained static for the last decade Currently there are two major classifications; the TNM with male and female incidence being similar; approxi- created by the UICC and a Japanese system (JPN) mately 10 new cases occur per 100 000 population per created by the Japan Pancreas Society. UICC is based annum. The recent trend has been for the disease to on tumour size and the presence or absence of nodal or occur in older age groups and now in the UK 50% remote metastases, whereas the JPN classification has of patients are over 75 years of age. Risk factors are more criteria; for example, a more detailed evaluation of few and the aetiology appears multifactorial; smoking the degree of invasion into vessels, retroperitoneum and appears the most important, with chronic pancreatitis, nerves. Evaluation of the remote nodal, as well as the hereditary predisposition, diabetes and carcinogens hepatic and peritoneal metastases is more neatly differ- being of importance. Approximately 90% of tumours are entiated. For individual tumours, the WHO classifica- [1] ductal carcinomas with the remaining 10% being a wide tion groups the exocrine tumours according to their range of different tumour types with a remarkably biological behaviour. The division between benign and variable prognosis. The importance of defining the his- malignant is not sharp, but rather a gradual transition tology is probably of greater importance in pancreatic analogous to some ovarian tumours. The borderline cancer than in most other tumours. Characteristically, group, known as ‘tumours of uncertain malignant tumours present late. potential’ are defined by the grade of dysplasia and their potential to become malignant. In the 1996 edition of the classification, a new name ‘intraductal papillary mucinous tumour’ replaced vague terms such as ‘mucin- Symptoms and signs producing tumour’, ‘mucinous duct ectasia’ and ‘solid The suspicion of pancreatic cancer arises because of pseudopapillary tumour’ (Table 1). Ductal adenocarci- symptoms such as pain, jaundice, weight loss, anorexia nomas compose 90% of pancreatic tumours. Sixty-five or early satiety. Less common symptoms include venous per cent of these tumours arise in the head of the thrombosis, diarrhoea, new onset diabetes or acute pancreas, 15% originate in the body and tail, and 20% pancreatitis. Back pain is ominous and associated with a diffusely involve the gland. The tumour infiltrates and shortened survival. Painless jaundice is the most com- creates a fibrous response such that the extent is difficult mon presentation in patients with a potentially resect- to appreciate and indeed, microscopically, extends able and curable lesion (52% of patients with a significantly beyond the grossly recognized tumour resectable lesion). Pain, however, is the most frequent mass. At the time of presentation most tumours have symptom and is present in 80% and 85% of patients with metastasized to the lymph nodes, and later extend to locally unresectable and advanced cancer, respectively. the liver (80%), peritoneum (60%), lungs and pleura The combination of pain and jaundice is present in 50% (50–70%), and the adrenal glands (25%). Most ductal of patients with a locally unresectable tumour. adenocarcinomas infiltrate the perineural, lymphatic In a prospective study of 39 patients with unresectable and vascular channels. pancreatic cancer, 82% had pain at the time of diagnosis, Endocrine pancreatic tumours arise from the diffuse 64% anorexia, 62% early satiety, 54% sleep problems, endocrine system (DES), the cells of which share a 1470-7330/01/01017 + 06  2001 International Cancer Imaging Society 18 Multidisciplinary Symposium — carcinoma of the pancreas Table 1 WHO classification of exocrine pancreatic transhepatic cholangiography (PTC) and computed tumours tomography (CT) scanning were associated with the highest provision of information, but the most cost- Benign tumours effective investigations were ultrasound and liver func- Serous cystadenoma tion tests. A software program produced a diagnostic Mucinous cystadenoma accuracy of 84%. Intraductal papillary-mucinous adenoma The role of investigation is to determine whether or Mature teratoma not a tumour is suitable for resection or palliation. The Borderline tumours (uncertain malignant potential) objective of palliation, which is the lot of the majority Mucinous cystic tumour with moderate dysplasia of patients, is to prolong life by relieving suffering, Intraductal papillary-mucinous tumour with moderate dysplasia Solid-pseudopapillary tumour namely the obstructive jaundice and the luminal obstruction. Non-operative approaches are the preferred Malignant method. Evidence is not available to suggest that pallia- Severe ductal dysplasia — carcinoma in situ Ductal adenocarcinoma tive resection has any advantage over non-operative Mucinous non-cystic carcinoma palliation. Surgical palliation is reserved for the patient Signet ring carcinoma who is considered operable but at surgery found not to Adenosquamous carcinoma be so. Thus the surgical view of pancreatic cancer is that Undifferentiated (anaplastic) carcinoma of operating on a suitable patient. Mixed ductal-endocrine carcinoma Osteoclast-like giant cell tumour Serous cystadenocarcinoma Mucinous cystadenocarcinoma Noninvasive Indications for resection Invasive Intraductal papillary-mucinous carcinoma The ideal tumour is one which is locally confined and Noninvasive has a good prognosis. The histology is important, but Invasive (papillary-mucinous carcinoma) this can usually be surmised from investigations. The Acinar cell carcinoma Acinar cell cystadenocarcinoma rare benign and good prognostic non-duct cell carci- Mixed acinar-endocrine carcinoma nomas have characteristic radiological features and are Pancreatoblastoma more prevalent in the body and tail of the pancreas than Solid-pseudopapillary carcinoma in the head of the gland. Ampullary tumours are diag- Miscellaneous carcinomas nosed endoscopically but their extent needs to be assessed radiologically. Biopsy, which is usually achieved by percutaneous techniques, is limited to the patient who is considered inoperable, however, rarely, as number of antigens with nerve elements, hence the name in neuroendocrine tumours, a biopsy is required if the ‘neuroendocrine’. The importance of these tumours is tumour is submitted for pre-operative chemoradio- that the relatively benign and differentiated tumours give therapy. Indeed, it is recommended that before starting rise to systemic endocrine syndromes (insulinoma, either chemotherapy or radiotherapy, a biopsy should be gastrinoma, glucagonoma, VIPoma, PPoma), while the performed. more malignant and non-hormone-producing tumours grow slowly and have a longer life-cycle than the ductal cancer. Similarly, ampullary tumours have different Operability behavioural patterns and present early due to obstruc- tion of the ampulla of Vater; their prognosis is similar to For the patient suitable for resection the surgeon needs colonic tumours. It is important to characterize precisely to know that the disease is confined to the pancreas and the pancreatic tumour in order to define a treatment that a standard resection will encompass the tumour. plan and consider prognosis. The standard resection is a pancreatoduodenectomy which excises the duodenum, the head of the pancreas and the lower bile duct. Extended procedures which involve radical lymphadenectomy have not improved Diagnosis survival. Incomplete excisions do not improve survival; The therapeutic advantages of an early, accurate and it behoves the surgeon to select the patient for precise diagnosis are obvious and indicate the need for resection in which the tumour is completely excised both standards for history-taking and physical examination macroscopically and microscopically. as well as non-invasive, highly sensitive and specific The markers of inoperability are distant metastases, methods of investigation. The diagnosis of carcinoma in involved lymph nodes away from the pancreas and the head of the pancreas can be made in 90% of patients involvement of the portal vein or superior mesenteric on the basis of clinical presentation. In a study of 356 artery. State-of-the-art helical CT scanning, with or patients with pancreatobiliary carcinoma, the clinical without magnetic resonance imaging (MRI) should and diagnostic tests reviewed showed that percutaneous accurately predict operability in 60–70% of patients. Multidisciplinary Symposium — carcinoma of the pancreas 19 Laparoscopic examination of the abdominal cavity will, Key points at most, improve this yield by 10%. The effect of (1) Incidence of disease far more common in the elderly endoscopic ultrasound, peritoneal washings, bone mar- (2) Histological type of great importance prognostically row biopsy and PET scanning have yet to be fully (3) Early diagnosis essential evaluated. It is cost-effective to limit the investigations to (4) Operability determined by high quality imaging state-of-the-art CT scanning and accept a slightly higher (5) Resection associated with a <5% mortality and a rate of inoperable cases which can be managed by 20% 5-year survival surgical palliation. With selection the surgical outcome should be a mortality of under 5% and a 5-year survival of 20% for duct cell carcinomas. The quality of life for patients Further reading undergoing pancreatoduodenectomy is good and their [1] Kloppel G, Solcia E, Longnecker DS, Capella C, Sobin LH. initial recovery period is no longer than 3 months before Histological typing of tumours of the exocrine pancreas. achieving an acceptable normalization of life. Adjuvant International Histological Classification of Tumours, 2nd chemotherapy improves survival by 10%. Radio- Edition. Berlin: Springer. World Health Organisation, 1998: therapy does not improve survival and it may even be 1–61. harmful. [2] Lillemoe KD, Cameron JL, Yeo CJ, Sohn TA, Nakeeb A, Sauter PK, Hruban RH, Abrams RA, Pitt HA. Pancreaticoduodenectomy. Does it have a role in the palliation of pancreatic cancer? Ann Surg 1996; 223: 718–25. [3] Krech RL, Walsh D. Symptoms of pancreatic cancer. J Pain Conclusion Symptom Management 1991; 6: 360–7. [4] Yeo CJ, Cameron JL, Sohn TA, Coleman J, Sauter PK, The surgeons’ view of pancreatic carcinoma is that the Hruban RH, Pitt HA, Lillemoe KD. Pancreaticoduo- diagnosis should be made early, the investigations denectomy with or without extended retroperitoneal lym- phadenectomy for periampullary adenocarcinoma: comparison should be undertaken expeditiously and the investiga- of morbidity and mortality and short-term outcome. Ann Surg tions limited to helical CT scanning. For those patients 1999; 229: 613–22. with an inoperable tumour a biopsy is appropriate. Operative mortality should be low and the quality of life The digital object identifier for this article is: 10.1102/ following recovery from resection good. 1470-7330.2001.010 Carcinoma of the pancreas: detection and staging using CT and MRI Jay P Heiken Mallinckrodt Institute of Radiology, Washington University School of Medicine, St Louis, Missouri, USA Our ability to diagnose pancreatic carcinoma has improved reduce the percentage of patients who are unnecessarily substantially over the past 20 years, owing to major subjected to laparotomy. advances in pancreatic imaging, including the development CT has become established as the primary initial imaging of US, CT and MRI. Despite these advances, however, the method for both detection and staging of suspected pancre- prognosis of patients with pancreatic cancer remains dis- atic carcinoma. Most studies have found that CT is highly [1] mal. The overall 5-year survival rate is only 3% , although reliable when it demonstrates features indicating that a [5,6] the 5-year survival rate for patients who undergo pancreatic tumor is unresectable . The positive predictive value [2–4] resection is reported to be approximately 20% . Because (PPV) of a diagnosis of unresectability with helical CT has [7–11] of the very poor prognosis of patients with pancreatic ranged from 92% to 100% . Helical CT is less reliable, carcinoma, many physicians take a nihilistic approach to its however, for predicting that a tumor is resectable [7–11] diagnosis and staging. It is important to keep in mind, (PPV = 76–90%) . Nevertheless, this represents a sub- however, that a large percentage of patients with pancreatic stantial improvement over prediction of resectability with [12–14] cancer who undergo laparotomy for possible curative resec- conventional CT (PPV = 45–72%) . Limitations of CT tion are found to have unresectable disease. Thus, optimi- include: poor ability to demonstrate small hepatic or peri- zation of pre-operative imaging is important in order to toneal metastases; inability to demonstrate microscopic

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Cancer Imaging – Springer Journals

Published: May 5, 2015

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