Abstract
Neuroendocrine tumors (NETs) of the minor papilla/ampulla (MIPA) are rare and poorly studied. Only individual case reports and no comprehensive analysis are available from the literature. We collected 16 MIPA NETs and investigated their clinicopathologic and immunohistochemical features, including markers such as somatostatin, pancreatic polypeptide, gastrin, serotonin, MUC1, cytokeratin 7, and somatostatin receptors type 2A and 5. The median age at diagnosis was 57.5 years, and the female-to-male ratio was 2.2:1. The median NET size was 1.45 cm, and most (94%) were low-grade (G1) tumors. Similarly to what was observed in the major ampulla, 3 histotypes were found: (i) ampullary-type somatostatin-producing tumors (ASTs, 10 cases), characterized by somatostatin expression in most tumor cells, focal-to-extensive tubulo-acinar structures, often with psammoma bodies, MUC1 reactivity, and no or rare membranous reactivity for somatostatin receptor type 2A; (ii) gangliocytic paragangliomas (3 cases), characterized by the coexistence of 3 tumor cell types: epithelioid, often reactive for pancreatic polypeptide, ganglion-like cells, and S100 reactive sustentacular/stromal cells; and (iii) ordinary nonfunctioning NETs (3 cases), resembling those more commonly observed in the extra-ampullary duodenum. Comparable histotypes could also be recognized among the 30 MIPA NETs from the literature. No NET-related patient death among MIPA cases was observed during a median follow-up of 38 months; however, MIPA ASTs showed lymph node metastases and invasion of the duodenal muscularis propria or beyond in 44% and 40% of cases, respectively. In conclusion, MIPA NETs closely resemble tumors arising in the major ampulla, with predominance of ASTs.
| Original language | English |
|---|---|
| Pages (from-to) | 725-736 |
| Number of pages | 12 |
| Journal | American Journal of Surgical Pathology |
| Volume | 43 |
| Issue number | 6 |
| DOIs | |
| Publication status | Published - Jun 1 2019 |
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Keywords
- ampullary-type somatostatin producing tumor
- gangliocytic paraganglioma
- MUC1
- nonfunctioning neuroendocrine tumor
- somatostatin receptor
ASJC Scopus subject areas
- Anatomy
- Surgery
- Pathology and Forensic Medicine
Cite this
Neuroendocrine Tumors (NETs) of the Minor Papilla/Ampulla : Analysis of 16 Cases Underlines Homology With Major Ampulla NETs and Differences From Extra-Ampullary Duodenal NETs. / Vanoli, Alessandro; Albarello, Luca; Uncini, Stefania; Fassan, Matteo; Grillo, Federica; Di Sabatino, Antonio; Martino, Michele; Pasquali, Claudio; Milanetto, Anna C.; Falconi, Massimo; Partelli, Stefano; Doglioni, Claudio; Schiavo-Lena, Marco; Brambilla, Tatiana; Pietrabissa, Andrea; Sessa, Fausto; Capella, Carlo; Rindi, Guido; La Rosa, Stefano; Solcia, Enrico; Paulli, Marco.
In: American Journal of Surgical Pathology, Vol. 43, No. 6, 01.06.2019, p. 725-736.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Neuroendocrine Tumors (NETs) of the Minor Papilla/Ampulla
T2 - Analysis of 16 Cases Underlines Homology With Major Ampulla NETs and Differences From Extra-Ampullary Duodenal NETs
AU - Vanoli, Alessandro
AU - Albarello, Luca
AU - Uncini, Stefania
AU - Fassan, Matteo
AU - Grillo, Federica
AU - Di Sabatino, Antonio
AU - Martino, Michele
AU - Pasquali, Claudio
AU - Milanetto, Anna C.
AU - Falconi, Massimo
AU - Partelli, Stefano
AU - Doglioni, Claudio
AU - Schiavo-Lena, Marco
AU - Brambilla, Tatiana
AU - Pietrabissa, Andrea
AU - Sessa, Fausto
AU - Capella, Carlo
AU - Rindi, Guido
AU - La Rosa, Stefano
AU - Solcia, Enrico
AU - Paulli, Marco
PY - 2019/6/1
Y1 - 2019/6/1
N2 - Neuroendocrine tumors (NETs) of the minor papilla/ampulla (MIPA) are rare and poorly studied. Only individual case reports and no comprehensive analysis are available from the literature. We collected 16 MIPA NETs and investigated their clinicopathologic and immunohistochemical features, including markers such as somatostatin, pancreatic polypeptide, gastrin, serotonin, MUC1, cytokeratin 7, and somatostatin receptors type 2A and 5. The median age at diagnosis was 57.5 years, and the female-to-male ratio was 2.2:1. The median NET size was 1.45 cm, and most (94%) were low-grade (G1) tumors. Similarly to what was observed in the major ampulla, 3 histotypes were found: (i) ampullary-type somatostatin-producing tumors (ASTs, 10 cases), characterized by somatostatin expression in most tumor cells, focal-to-extensive tubulo-acinar structures, often with psammoma bodies, MUC1 reactivity, and no or rare membranous reactivity for somatostatin receptor type 2A; (ii) gangliocytic paragangliomas (3 cases), characterized by the coexistence of 3 tumor cell types: epithelioid, often reactive for pancreatic polypeptide, ganglion-like cells, and S100 reactive sustentacular/stromal cells; and (iii) ordinary nonfunctioning NETs (3 cases), resembling those more commonly observed in the extra-ampullary duodenum. Comparable histotypes could also be recognized among the 30 MIPA NETs from the literature. No NET-related patient death among MIPA cases was observed during a median follow-up of 38 months; however, MIPA ASTs showed lymph node metastases and invasion of the duodenal muscularis propria or beyond in 44% and 40% of cases, respectively. In conclusion, MIPA NETs closely resemble tumors arising in the major ampulla, with predominance of ASTs.
AB - Neuroendocrine tumors (NETs) of the minor papilla/ampulla (MIPA) are rare and poorly studied. Only individual case reports and no comprehensive analysis are available from the literature. We collected 16 MIPA NETs and investigated their clinicopathologic and immunohistochemical features, including markers such as somatostatin, pancreatic polypeptide, gastrin, serotonin, MUC1, cytokeratin 7, and somatostatin receptors type 2A and 5. The median age at diagnosis was 57.5 years, and the female-to-male ratio was 2.2:1. The median NET size was 1.45 cm, and most (94%) were low-grade (G1) tumors. Similarly to what was observed in the major ampulla, 3 histotypes were found: (i) ampullary-type somatostatin-producing tumors (ASTs, 10 cases), characterized by somatostatin expression in most tumor cells, focal-to-extensive tubulo-acinar structures, often with psammoma bodies, MUC1 reactivity, and no or rare membranous reactivity for somatostatin receptor type 2A; (ii) gangliocytic paragangliomas (3 cases), characterized by the coexistence of 3 tumor cell types: epithelioid, often reactive for pancreatic polypeptide, ganglion-like cells, and S100 reactive sustentacular/stromal cells; and (iii) ordinary nonfunctioning NETs (3 cases), resembling those more commonly observed in the extra-ampullary duodenum. Comparable histotypes could also be recognized among the 30 MIPA NETs from the literature. No NET-related patient death among MIPA cases was observed during a median follow-up of 38 months; however, MIPA ASTs showed lymph node metastases and invasion of the duodenal muscularis propria or beyond in 44% and 40% of cases, respectively. In conclusion, MIPA NETs closely resemble tumors arising in the major ampulla, with predominance of ASTs.
KW - ampullary-type somatostatin producing tumor
KW - gangliocytic paraganglioma
KW - MUC1
KW - nonfunctioning neuroendocrine tumor
KW - somatostatin receptor
UR - http://www.scopus.com/inward/record.url?scp=85065911868&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85065911868&partnerID=8YFLogxK
U2 - 10.1097/PAS.0000000000001234
DO - 10.1097/PAS.0000000000001234
M3 - Article
AN - SCOPUS:85065911868
VL - 43
SP - 725
EP - 736
JO - American Journal of Surgical Pathology
JF - American Journal of Surgical Pathology
SN - 0147-5185
IS - 6
ER -