INTRADUCTAL PAPILLARY MUCINOUS INTRADUCTAL PAPILLARY MUCINOUS NEOPLASM OF THE PANCREAS: NEOPLASM OF THE PANCREAS:

PRESENTATION IN A YOUNG ADULTPRESENTATION IN A YOUNG ADULT

INTRADUCTAL PAPILLARY MUCINOUS INTRADUCTAL PAPILLARY MUCINOUS NEOPLASM OF THE PANCREAS: NEOPLASM OF THE PANCREAS:

PRESENTATION IN A YOUNG ADULTPRESENTATION IN A YOUNG ADULT

RITA PASSANTINO - LORENZO MARASA’RITA PASSANTINO - LORENZO MARASA’

Department of Pathology, A.R.N.A.S. Civico, Department of Pathology, A.R.N.A.S. Civico, PalermoPalermo

RITA PASSANTINO - LORENZO MARASA’RITA PASSANTINO - LORENZO MARASA’

Department of Pathology, A.R.N.A.S. Civico, Department of Pathology, A.R.N.A.S. Civico, PalermoPalermo

INTRODUCTIONINTRODUCTIONINTRODUCTIONINTRODUCTION

Intraductal Papillary Mucinous Neoplasms (IPMNs) are a Intraductal Papillary Mucinous Neoplasms (IPMNs) are a distinct type of intraductal pancreatic tumours that have distinct type of intraductal pancreatic tumours that have been defined and segregated from other neoplams of this been defined and segregated from other neoplams of this organ (particularly Mucinous Cystic Neoplasms, MCNs) only organ (particularly Mucinous Cystic Neoplasms, MCNs) only recently.recently.

IPMNs are the most common intraductal tumours of the IPMNs are the most common intraductal tumours of the pancreas. pancreas.

Although they show an adenoma-carcinoma sequence, they Although they show an adenoma-carcinoma sequence, they have proved to have a more favorable prognosis than ductal have proved to have a more favorable prognosis than ductal adenocarcinoma, when resected in a preinvasive state. adenocarcinoma, when resected in a preinvasive state.

Intraductal Papillary Mucinous Neoplasms (IPMNs) are a Intraductal Papillary Mucinous Neoplasms (IPMNs) are a distinct type of intraductal pancreatic tumours that have distinct type of intraductal pancreatic tumours that have been defined and segregated from other neoplams of this been defined and segregated from other neoplams of this organ (particularly Mucinous Cystic Neoplasms, MCNs) only organ (particularly Mucinous Cystic Neoplasms, MCNs) only recently.recently.

IPMNs are the most common intraductal tumours of the IPMNs are the most common intraductal tumours of the pancreas. pancreas.

Although they show an adenoma-carcinoma sequence, they Although they show an adenoma-carcinoma sequence, they have proved to have a more favorable prognosis than ductal have proved to have a more favorable prognosis than ductal adenocarcinoma, when resected in a preinvasive state. adenocarcinoma, when resected in a preinvasive state.

INTRODUCTIONINTRODUCTIONINTRODUCTIONINTRODUCTION

IPMNs are mucin-producing epithelial tumours that usually IPMNs are mucin-producing epithelial tumours that usually show a papillary architecture and are associated with show a papillary architecture and are associated with involvement and dilatation of several major pancreatic involvement and dilatation of several major pancreatic ducts. ducts.

Clinically, these lesions often produce pancreatitis Clinically, these lesions often produce pancreatitis because of the hypersecretion of mucous. because of the hypersecretion of mucous.

Recently, it has become clear that IPMNs constitute a Recently, it has become clear that IPMNs constitute a heterogeneous group with at least four subtypes with heterogeneous group with at least four subtypes with different biological properties and different prognostic different biological properties and different prognostic implications. implications.

IPMNs are mucin-producing epithelial tumours that usually IPMNs are mucin-producing epithelial tumours that usually show a papillary architecture and are associated with show a papillary architecture and are associated with involvement and dilatation of several major pancreatic involvement and dilatation of several major pancreatic ducts. ducts.

Clinically, these lesions often produce pancreatitis Clinically, these lesions often produce pancreatitis because of the hypersecretion of mucous. because of the hypersecretion of mucous.

Recently, it has become clear that IPMNs constitute a Recently, it has become clear that IPMNs constitute a heterogeneous group with at least four subtypes with heterogeneous group with at least four subtypes with different biological properties and different prognostic different biological properties and different prognostic implications. implications.

CASE REPORTCASE REPORT CASE REPORTCASE REPORT

In this report, one of the youngest cases of IPMNs is de-In this report, one of the youngest cases of IPMNs is de-scribed.scribed.

The patient was a 34-year-old man with several episodes of The patient was a 34-year-old man with several episodes of acute recurrent pancreatitis in the past 6 months, on acute recurrent pancreatitis in the past 6 months, on February 2008 admitted for dyspepsia, nausea and loss of February 2008 admitted for dyspepsia, nausea and loss of appetite.appetite.

Computed tomography showed a cystic lesion in the head Computed tomography showed a cystic lesion in the head of pancreas which communicated and extensively involved of pancreas which communicated and extensively involved the large pancreatic ducts. the large pancreatic ducts.

Endoscopic retrograde cholangiopancreatography demon-Endoscopic retrograde cholangiopancreatography demon-strated mucin oozing from ampulla of Vater.strated mucin oozing from ampulla of Vater.

Cephalopancreaticoduodenectomy was performed.Cephalopancreaticoduodenectomy was performed.

In this report, one of the youngest cases of IPMNs is de-In this report, one of the youngest cases of IPMNs is de-scribed.scribed.

The patient was a 34-year-old man with several episodes of The patient was a 34-year-old man with several episodes of acute recurrent pancreatitis in the past 6 months, on acute recurrent pancreatitis in the past 6 months, on February 2008 admitted for dyspepsia, nausea and loss of February 2008 admitted for dyspepsia, nausea and loss of appetite.appetite.

Computed tomography showed a cystic lesion in the head Computed tomography showed a cystic lesion in the head of pancreas which communicated and extensively involved of pancreas which communicated and extensively involved the large pancreatic ducts. the large pancreatic ducts.

Endoscopic retrograde cholangiopancreatography demon-Endoscopic retrograde cholangiopancreatography demon-strated mucin oozing from ampulla of Vater.strated mucin oozing from ampulla of Vater.

Cephalopancreaticoduodenectomy was performed.Cephalopancreaticoduodenectomy was performed.

GROSS EXAMINATIONGROSS EXAMINATIONGROSS EXAMINATIONGROSS EXAMINATION

The tumour massively involved either the main pancreatic duct or its The tumour massively involved either the main pancreatic duct or its branches. There was marked dilatation of a major pancreatic duct with branches. There was marked dilatation of a major pancreatic duct with fibrosis and atrophy of the surrounding parenchima. This duct contained fibrosis and atrophy of the surrounding parenchima. This duct contained large amounts of mucin in its lumen. large amounts of mucin in its lumen.

The tumour massively involved either the main pancreatic duct or its The tumour massively involved either the main pancreatic duct or its branches. There was marked dilatation of a major pancreatic duct with branches. There was marked dilatation of a major pancreatic duct with fibrosis and atrophy of the surrounding parenchima. This duct contained fibrosis and atrophy of the surrounding parenchima. This duct contained large amounts of mucin in its lumen. large amounts of mucin in its lumen.

HISTOLOGICAL EXAMINATIONHISTOLOGICAL EXAMINATIONThere was a multicentric involvement of major ducts by a predominantly There was a multicentric involvement of major ducts by a predominantly papillary lesion with an “intestinal” differentation: a large mucin-papillary lesion with an “intestinal” differentation: a large mucin-producing papillary neoplasm, with a main focus of 1.8 cm in greatest producing papillary neoplasm, with a main focus of 1.8 cm in greatest diameter, made up columnar cells with cigar-shaped nuclei and mild diameter, made up columnar cells with cigar-shaped nuclei and mild dysplasia, in a context of an obstructive chronic pancreatitis.dysplasia, in a context of an obstructive chronic pancreatitis.

IMMUNOHISTOCHEMICAL EXAMINATIONIMMUNOHISTOCHEMICAL EXAMINATION

The tumour showed the characteristic immunohistoche-mical profile of The tumour showed the characteristic immunohistoche-mical profile of “intestinal type” of IPMN with expression of MUC-2, MUC5AC, CDX2 and “intestinal type” of IPMN with expression of MUC-2, MUC5AC, CDX2 and absent expression of MUC-1.absent expression of MUC-1.

HISTOLOGICAL EXAMINATIONHISTOLOGICAL EXAMINATION

Involvement of major duct by a predominantly papillary Involvement of major duct by a predominantly papillary

lesion with an “intestinal” differentationlesion with an “intestinal” differentation

HISTOLOGICAL EXAMINATIONHISTOLOGICAL EXAMINATION

An intraductal mucin-producing papillary neoplasm, An intraductal mucin-producing papillary neoplasm,

in association with a chronic pancreatitisin association with a chronic pancreatitis

IMMUNOHISTOCHEMICAL EXAMINATIONIMMUNOHISTOCHEMICAL EXAMINATION IMMUNOHISTOCHEMICAL EXAMINATIONIMMUNOHISTOCHEMICAL EXAMINATION

MUC-2MUC-2MUC-2MUC-2 MUC5ACMUC5ACMUC5ACMUC5AC

CDX-2CDX-2CDX-2CDX-2 MUC-1MUC-1MUC-1MUC-1

CONCLUSIONSCONCLUSIONS CONCLUSIONSCONCLUSIONS

IPMNs are now being reported more frequently with larger case IPMNs are now being reported more frequently with larger case series. The reason for the increasing recognition of the disease is series. The reason for the increasing recognition of the disease is unclear, but is at least partially the result of a rising awareness of unclear, but is at least partially the result of a rising awareness of the disease and an increasing use of cross-sectional imaging.the disease and an increasing use of cross-sectional imaging.

The natural history of these lesions is to spread slowly over the The natural history of these lesions is to spread slowly over the ductal system with eventual progression in some cases to ductal system with eventual progression in some cases to invasive carcinoma. In the “intestinal type” of IPMNs the invasive invasive carcinoma. In the “intestinal type” of IPMNs the invasive component is an invasive mucinous (colloid) adenocarcinoma component is an invasive mucinous (colloid) adenocarcinoma with a better prognosis than ordinary ductal adenocarcinoma. with a better prognosis than ordinary ductal adenocarcinoma.

IPMNs are now being reported more frequently with larger case IPMNs are now being reported more frequently with larger case series. The reason for the increasing recognition of the disease is series. The reason for the increasing recognition of the disease is unclear, but is at least partially the result of a rising awareness of unclear, but is at least partially the result of a rising awareness of the disease and an increasing use of cross-sectional imaging.the disease and an increasing use of cross-sectional imaging.

The natural history of these lesions is to spread slowly over the The natural history of these lesions is to spread slowly over the ductal system with eventual progression in some cases to ductal system with eventual progression in some cases to invasive carcinoma. In the “intestinal type” of IPMNs the invasive invasive carcinoma. In the “intestinal type” of IPMNs the invasive component is an invasive mucinous (colloid) adenocarcinoma component is an invasive mucinous (colloid) adenocarcinoma with a better prognosis than ordinary ductal adenocarcinoma. with a better prognosis than ordinary ductal adenocarcinoma.

CONCLUSIONSCONCLUSIONS CONCLUSIONSCONCLUSIONS

The potential for this neoplasms to diffusely involve the pancreatic The potential for this neoplasms to diffusely involve the pancreatic ducts has been recognized, making treatment decisions difficult. ducts has been recognized, making treatment decisions difficult.

The crux of the issue is whether a partial pancreasectomy is The crux of the issue is whether a partial pancreasectomy is adequate treatment and what effect the histologic status of the adequate treatment and what effect the histologic status of the margin has on outcome.margin has on outcome.

More extensive surgery may be needed in IPMNs, such as total More extensive surgery may be needed in IPMNs, such as total pancreasectomy, especially in cases of diffuse ductal dilation of pancreasectomy, especially in cases of diffuse ductal dilation of the pancreas.the pancreas.

Anyway, because of a limited number of cases, prognostic factors Anyway, because of a limited number of cases, prognostic factors and the natural history of IPMNs have not been well defined. and the natural history of IPMNs have not been well defined.

The potential for this neoplasms to diffusely involve the pancreatic The potential for this neoplasms to diffusely involve the pancreatic ducts has been recognized, making treatment decisions difficult. ducts has been recognized, making treatment decisions difficult.

The crux of the issue is whether a partial pancreasectomy is The crux of the issue is whether a partial pancreasectomy is adequate treatment and what effect the histologic status of the adequate treatment and what effect the histologic status of the margin has on outcome.margin has on outcome.

More extensive surgery may be needed in IPMNs, such as total More extensive surgery may be needed in IPMNs, such as total pancreasectomy, especially in cases of diffuse ductal dilation of pancreasectomy, especially in cases of diffuse ductal dilation of the pancreas.the pancreas.

Anyway, because of a limited number of cases, prognostic factors Anyway, because of a limited number of cases, prognostic factors and the natural history of IPMNs have not been well defined. and the natural history of IPMNs have not been well defined.

MAIN REFERENCESMAIN REFERENCES MAIN REFERENCESMAIN REFERENCES

Andrejevic-Blant S et al. Pancreatic intraductal papillary-Andrejevic-Blant S et al. Pancreatic intraductal papillary-mucinous neoplasms: a new and evolving entity. mucinous neoplasms: a new and evolving entity. Virchows Arch. Virchows Arch. 2007 Nov; 451(5): 863-869.2007 Nov; 451(5): 863-869.

D’Angelica M et al. Intraductal papillary mucinous D’Angelica M et al. Intraductal papillary mucinous neoplasms of the pancreas. An analysis of neoplasms of the pancreas. An analysis of clinicopathologic features and outcome. clinicopathologic features and outcome. Ann Surg. Ann Surg. 2004 2004 March; 239(3): 400-408. March; 239(3): 400-408.

Andrejevic-Blant S et al. Pancreatic intraductal papillary-Andrejevic-Blant S et al. Pancreatic intraductal papillary-mucinous neoplasms: a new and evolving entity. mucinous neoplasms: a new and evolving entity. Virchows Arch. Virchows Arch. 2007 Nov; 451(5): 863-869.2007 Nov; 451(5): 863-869.

D’Angelica M et al. Intraductal papillary mucinous D’Angelica M et al. Intraductal papillary mucinous neoplasms of the pancreas. An analysis of neoplasms of the pancreas. An analysis of clinicopathologic features and outcome. clinicopathologic features and outcome. Ann Surg. Ann Surg. 2004 2004 March; 239(3): 400-408. March; 239(3): 400-408.