Case Report

Management of Low-Grade Appendiceal Mucinous Neoplasm with Extensive Peritoneal Spread Diagnosed during Pregnancy: Two Case Reports and Literature Review

Table 1

Literature review of appendiceal tumors diagnosed during pregnancy.

#Author (year)AgePathologyPCGestational age at dxClinical presentationTreatment during pregnancyPregnancy outcomeStagingTreatment postpregnancyTime from pregnancy end to treatmentPCICC scoreComplicationsBreast-feedingStatusChild

1Present case #131LAMNYes2nd Tx (20 weeks)Routine prenatal screeningAppy, omentectomy, RSO, peritoneal bxSpontaneous vaginal delivery (38 weeks)MRI abd/pelv at 20 weeks; CT chest/abd/pelv after deliveryCRS/HIPEC (Mit-C)4 mos37CC-1POD 15: portal vein thrombosis POD 30: rectal bleeding4 mos+12 mos milk donor5 yrs, NEDHealthy
2Haase et al. (2010) [5]30Well-differentiated mucinous adenocarcinomaYes2nd Tx (17 weeks)Routine prenatal screeningAppy, RSO, omental bxEarly induce vaginal delivery (35 weeks)CT after deliveryCRS/HIPEC (Mit-C) + EPIC (5-FU); adjuvant XELOX x8 cycles2.5 weeks28CC-1Neutropenia, prolonged ileus (NGT, TPN), DVT-5 yrs, NEDHealthy
3Chiverto et al. (2012) [6]36Infiltrating mucinous adenocarcinoma (T3N1M1a)Yes2nd Tx (18 weeks)Abdominal pain x4 daysDx lap, open appyTerminated with misoprostolColonoscopy, CT chest/abd/pelv after terminationCRS/HIPEC (5-FU + Oxaliplatin; adjuvant FOLFOX x6 Mos-6Complete-NA10 mos, NEDNA
4Canuto et al. (2016) [7]38Well-differentiated mucinous adenocarcinoma (pT4apN1)Yes1st TxRoutine prenatal screening (R ovarian mass)Laparoscopic R adnexectomy, peritoneal washing/bx (16 weeks, 4 days)RDS prophylaxis; early C-section (33 weeks, 5 days)-CRS/HIPEC (Mit-C + Cisplatin); adjuvant FOLFOX x8 cycles2 mos-No residual tumor--2 yrs, NEDHealthy
5Sebire et al. (2000) [8]29Moderately differentiated adenocarcinoma; liver metastasisYes3rd Tx (29 weeks)Abdominal pain, vomiting, UTINoneC-section (30 weeks)Abd US (29 weeks); fine-needle liver bxCRS; adjuvant 5-FU/Epirubicin/carboplatin0 days (at C-section)-Residual tumor--6 mos, AWDHealthy

6Donnenfeld et al. (1986) [24]25Perforated invasive grade 1 mucinous adenocarcinomaNo3rd Tx (31 weeks)Acute appendicitis with peritonitisUrgent open appy, abscess drainInduced vaginal delivery (33 weeks)Chest X-ray, CT abd, colonoscopy 3 days after deliveryR hemicolectomy9 daysNANANone-30 daysHealthy
7Morgan et al. (2004) [25]30Well-differentiated mucinous adenocarcinoma, negative peritoneal washingsNo2nd Tx (26 weeks)Acute abdomenUrgent open R hemicolectomySpontaneous vaginal delivery (at term)-NoneNANANANone-36 mos, NED-
8Zeteroğlu et al. (2003) [26]35Non-perforated mucinous appendiceal cyctadenocarcinomaNo2nd Tx (21 weeks)Symptoms of acute appendicitisUrgent appyTerminated with misoprostol (21 weeks)-R hemicolectomy, omentectomy3 daysNANANoneNA1 yr, NEDNA
9Casey et al. (2003) [27]36Perforated appendiceal mucious cystadenomaNo2nd Tx (21 weeks)Symptoms of acute appendicitisOpen appy--NoneNANANA----
10Kalu and Croucher (2004) [28]42Mucus adenoma with mucoceleNo1st Tx (5 weeks)US due to vaginal bleedingNoneSpontaneous miscarriage (anembryonic pregnancy) (7 weeks)US at 5 weeks (adnexal mass); US in 3 Mos (enlarging ovarian mass)Laparotomy/appy3 mosNANANoneNA-NA
11Idris et al. (2015) [29]35MucoceleNo2nd Tx (22 weeks)Symptoms of acute appendicitisOpen appySpontaneous vaginal delivery (at term)-NoneNANANANone-1 yr, NED-
12Gilboa et al. (2008) [30]31Carcinoid tumorNo1st Tx (9 weeks)Symptoms of acute appendicitisAppySpontaneous miscarriage (5 days post-op)---NANA---NA
13Louzi et al.§ (2006) [31]36Well-differentiated carcinoid tumorNo3rd Tx (34 weeks)Symptoms of acute appendicitisUrgent appyVaginal delivery (35 weeks)-R hemicolectomy2 weeksNANA--23 mos, NEDHealthy
14Piatek et al. (2016) [32]28Well-differentiated carcinoid with gangrenous appendicitis (KI-67: <1%)No2nd Tx (25 weeks)Symptoms of acute appendicitisAppySpontaneous vaginal delivery (38 weeks)MRI abd at 29 weeks, normal 24-hour urine 5-HIAA; whole body SPECT 3 Mos after deliveryNoneNANANANone-1 yr, NEDHealthy
15Berrios (1965) [33]23Carcinoid tumorNo1st Tx (2 months)Symptoms of acute appendicitisAppySpontaneous vaginal delivery (at term)Negative liver scan during pregnancyNoneNANANA--23 mos, NEDHealthy
16Berrios (1965) [33]26Carcinoid tumorNo1st Tx (10 weeks)Symptoms of ruptured ectopic pregnancyBleeding luteum cyst, incidental appy--NoneNANANA--No FU-
17Pitiakoudis et al. (2008) [34]24Carcinoid tumor (0.5 cm)No3rd Tx (32 weeks)Symptoms of acute appendicitisAppySpontaneous vagina delivery (39 weeks)---NANANone--Healthy
18Korkontzelos (2005) [35]23Carcinoid tumor (2.2 cm)No2nd Tx (16 weeks)Symptoms of acute appendicitisUrgent appyC-section (36 weeks)-R hemicolectomy0 days (at C-section)NANA---Healthy

§Article in French. The cases reporting peritoneal spread at presentation are in italics. 5-FU: 5-fluorouracil; 5-HIAA: 5-hydroxyindoleacetic acid; Abd: abdomen; Appy: appendectomy; AWD: alive with disease; Bx: biopsy; CC: completeness of cytoreduction; CRS/HIPEC: cytoreductive surgery with hyperthermic intraperitoneal chemotherapy; C-section: cesarean section; CT: computed tomography; DVT: deep vein thrombosis; Dx: diagnosis/diagnostic; FOLFOX: folinic acid+5-fluorouracil+oxaliplatin; IVF: in vitro fertilization; LAMN: low-grade appendiceal mucinous neoplasm; Lap: laparoscopy; Mit-C: mitomycin C; mos: months; NA: not applicable; NED: no evidence of disease; NGT: nasogastric tube; PC: peritoneal carcinomatosis; PCI: peritoneal cancer index; Pelv: pelvis; R: right; RDS: respiratory distress syndrome; SPECT: single-photon emission computed tomography; TPN: total parenteral nutrition; Tx: trimester; US: ultrasound; UTI: urinary tract infection; XELOX: capecitabine+oxaliplatin; Yr (s): year (s).