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23 February 2021: Articles

A Low-Grade Appendiceal Mucinous Neoplasia and Neuroendocrine Appendiceal Collision Tumor: A Case Report and Review of the Literature

Rare coexistence of disease or pathology

Massimo Villa A , Daniele Sforza A* , Leandro Siragusa A , Andrea Martina Guida B , Matteo Ciancio Manuelli A , Brunella Maria Pirozzi A , Marco Pocci C , Giampiero Palmieri C , Michele Grande A

DOI: 10.12659/AJCR.927876

Am J Case Rep 2021; 22:e927876

Table 1. Cases of collision ANET and LAMN.

Authors and yearSexAge (years)PresentationHistologySurgical treatmentFollow-up
Baena-del- Valle et al 2015 []14 F49Acellular mucin during epigastric hernia repairAppendiceal perforation, PCI 27 + LAMN (cytokeratin 20 and CDX-2 +, cytokeratin 7 –) and NET (CgA and synaptophysin +)First appendectomy + CRS + HIPECNot available
F45Epigastric hernia and free abdominal fluidMultiple abdominal mucinous implants + tumor-like lesion on the tip of the appendix + LAMN (cytokeratin 20 and CDX-2 +, cytokeratin 7 –) and NET (CgA and synaptophysin +)First diagnostic laparoscopic appendectomy + omentectomy + CRS + HIPECOne year later: progression of disease with perihepatic and pleural recurrences
Tan et al 2015 []13 M52Elevated CEA trendLAMN (3.5–5 cm diameter) without involvement of either the appendiceal base nor the surrounding structures + absence of peritoneal disease + carcinoid 3 mmAppendectomyRegular CT scan at 6 months after the surgery
Hajjar et al 2018 []11 M50Abdominal pain5.5 cm LAMN + perforation with PMP + 1.6 cm well-differentiated NET, infiltrating muscularis propria and mesoappendix, ki67 3%, grade of G2/3 + perineural invasion, no vascular invasion, free resection margins, no lymph nodesFirst appendectomy, second right hemicolectomy, omentectomy, cholecystectomy, peritoneal stripping, CRS, and HIPECDFS at 20 months after surgery
Ekinci et al 2018 []2 M60Abdominal discomfort, RIF pain, mild anemia, elevated WBC, elevated CEALAMN + NET WHO grade 1, infiltrating the entire thickness of the appendiceal wall, ki67First appendectomy, second right hemicolectomy indicated but refused by the patientDFS at 6 months after surgery
Sholi et al 2019 []12 F23Constipation and abdominal fullnessLAMN + well-differentiated NETFirst appendectomy, second right hemicolectomyDFS at 24 months after surgery
Sugarbaker et al 2020 []7 F39Right iliac fossa painRuptured LAMN with extrusion of mucin + foci of mucin and epithelial cells on the surface of the small bowel, but no involvement of the lymph nodes (pT3N0M1) +1.7 cm pT1BN0 NETFirst appendectomy + right hemicolectomy, second greater omentectomy, lesser omentectomy, cholecystectomy, hysterectomy, and bilateral salpingo-oophorectomy + HIPECDFS 5 years after surgery
M32Mucin fluid during left inguinal hernia repairLAMN pT3N0M1a + well-differentiated NET G2T2N1MX (ki67 5%)Right hemicolectomy, greater omentectomy, lesser omentectomy and cholecystectomy + HIPECFollow-up scheduled every 3 months, DFS 1 year after surgery
Cafaro et al 2020 []16 F35Epigastric pain with migration to right iliac fossa + >WBCLAMN + well-differentiated NETAppendectomyDFS at 15 months after surgery
LAMN – low-grade appendiceal mucinous neoplasia; ANET – appendiceal neuroendocrine tumor; NET – neuroendocrine tumor; CRS – colorectal surgery; HIPEC – hyperthermic intraperitoneal chemotherapy; CEA – carcinoembryonic antigen; CT – computed tomography; DFS – disease-free survival; WBC – white blood cell; CgA – chromogranin-A.

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American Journal of Case Reports eISSN: 1941-5923
American Journal of Case Reports eISSN: 1941-5923