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Multifocal intraductal tubulopapillary neoplasm of the pancreas with total pancreatectomy: report of a case and review of literature.

Kölby, David; Thilén, Johan; Andersson, Roland LU ; Sasor, Agata and Ansari, Daniel LU (2015) In International Journal of Clinical and Experimental Pathology 8(8). p.9672-9680
Abstract
Intraductal neoplasms of the pancreas are classified as intraductal tubulopapillary neoplasms (ITPNs) and intraductal papillary mucinous neoplasm (IPMNs) in the current WHO classification. ITPN is a rare tumor and there are only a few cases of ITPN reported in the literature. We present the case of an otherwise healthy 42-year-old male, who presented with upper abdominal pain. He was subsequently diagnosed with multifocal ITPN and underwent total pancreatectomy. The pathological report showed invasive growth. The postoperative course was uneventful and the patient received 6 months of adjuvant chemotherapy with gemcitabine-capecitabine. The patient is still alive 19 months after the procedure with no signs of recurrence. Literature review... (More)
Intraductal neoplasms of the pancreas are classified as intraductal tubulopapillary neoplasms (ITPNs) and intraductal papillary mucinous neoplasm (IPMNs) in the current WHO classification. ITPN is a rare tumor and there are only a few cases of ITPN reported in the literature. We present the case of an otherwise healthy 42-year-old male, who presented with upper abdominal pain. He was subsequently diagnosed with multifocal ITPN and underwent total pancreatectomy. The pathological report showed invasive growth. The postoperative course was uneventful and the patient received 6 months of adjuvant chemotherapy with gemcitabine-capecitabine. The patient is still alive 19 months after the procedure with no signs of recurrence. Literature review revealed only 30 individual cases of ITPN in the pancreas including our reported case. Mean age was 61 years (16 males/14 females; ratio 1.14:1). Mean tumor size was 3 cm. Immunohistochemical staining was positive for CK-7 in 100% of the patients, CK-19 in 95% and for MUC-1 in 88%. Trypsin was negative in all cases. β-catenin was negative in 94% and MUC-2 was negative in 96% of the cases. BRAF, KRAS, TP53 and PIK3CA mutations were infrequently seen. Invasive growth was present in 54% of the cases. Tumor size and Ki-67 index showed a statistically significant association with invasive growth. Survival rate could not be determined, due to short follow-up, and further research is needed to establish prognostic factors for disease recurrence and survival. (Less)
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Contribution to journal
publication status
published
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in
International Journal of Clinical and Experimental Pathology
volume
8
issue
8
pages
9672 - 9680
publisher
E-Century Publishing Corp
external identifiers
  • pmid:26464736
  • wos:000364775400122
  • scopus:85012963440
ISSN
1936-2625
language
English
LU publication?
yes
id
0fbdaae2-3751-4473-89e8-05641a5516e4 (old id 8152392)
alternative location
http://www.ncbi.nlm.nih.gov/pubmed/26464736?dopt=Abstract
date added to LUP
2015-11-03 12:51:06
date last changed
2017-10-22 04:03:11
@article{0fbdaae2-3751-4473-89e8-05641a5516e4,
  abstract     = {Intraductal neoplasms of the pancreas are classified as intraductal tubulopapillary neoplasms (ITPNs) and intraductal papillary mucinous neoplasm (IPMNs) in the current WHO classification. ITPN is a rare tumor and there are only a few cases of ITPN reported in the literature. We present the case of an otherwise healthy 42-year-old male, who presented with upper abdominal pain. He was subsequently diagnosed with multifocal ITPN and underwent total pancreatectomy. The pathological report showed invasive growth. The postoperative course was uneventful and the patient received 6 months of adjuvant chemotherapy with gemcitabine-capecitabine. The patient is still alive 19 months after the procedure with no signs of recurrence. Literature review revealed only 30 individual cases of ITPN in the pancreas including our reported case. Mean age was 61 years (16 males/14 females; ratio 1.14:1). Mean tumor size was 3 cm. Immunohistochemical staining was positive for CK-7 in 100% of the patients, CK-19 in 95% and for MUC-1 in 88%. Trypsin was negative in all cases. β-catenin was negative in 94% and MUC-2 was negative in 96% of the cases. BRAF, KRAS, TP53 and PIK3CA mutations were infrequently seen. Invasive growth was present in 54% of the cases. Tumor size and Ki-67 index showed a statistically significant association with invasive growth. Survival rate could not be determined, due to short follow-up, and further research is needed to establish prognostic factors for disease recurrence and survival.},
  author       = {Kölby, David and Thilén, Johan and Andersson, Roland and Sasor, Agata and Ansari, Daniel},
  issn         = {1936-2625},
  language     = {eng},
  number       = {8},
  pages        = {9672--9680},
  publisher    = {E-Century Publishing Corp},
  series       = {International Journal of Clinical and Experimental Pathology},
  title        = {Multifocal intraductal tubulopapillary neoplasm of the pancreas with total pancreatectomy: report of a case and review of literature.},
  volume       = {8},
  year         = {2015},
}