e-ISSN: 2722-3558 Sriwijaya Journal of Surgery [SJS] https://sriwijayasurgery.
Synchronous Low-Grade Appendiceal Mucinous Neoplasm.
Perforated Ascending Colon Diverticulitis, and Mature Ovarian Cystic Teratoma in a 52-Year-Old Female: Diagnostic Challenges and Surgical Decision-Making I Made Aridana Sandika1*.
Maria Yustina2 General Practitioner.
Klungkung Regional General Hospital.
Semarapura.
Indonesia Department of Digestive Surgery.
Klungkung Regional General Hospital.
Semarapura.
Indonesia
A R T I C L E
I N F O
Keywords:
Appendiceal neoplasm Diverticular perforation Low-grade appendiceal mucinous neoplasm Ovarian teratoma Pseudomyxoma peritonei *Corresponding author:
I Made Aridana Sandika E-mail address:
arydana91@yahoo.
All authors have reviewed and approved the final version of the manuscript.
https://doi.
org/10.
37275/sjs.
Introduction The vermiform appendix is a small, blind-ending A B S T R A C T Introduction.
Low-grade appendiceal mucinous neoplasm (LAMN) is a rare mucin-producing epithelial tumor identified in 13Ae1% of appendectomies, with a recognized risk of pseudomyxoma peritonei (PMP) on rupture.
Synchronous coexistence with perforated ascending colon diverticulitis and an ipsilateral ovarian mature cystic teratoma is exceptional and creates a triple-layered diagnostic and operative challenge.
Case Presentation.
A 52-year-old Indonesian female presented with one month of right lower quadrant (RLQ) pain that worsened over forty-eight hours, accompanied by nausea and vomiting.
Examination revealed McBurney tenderness, local muscular defense, and a palpable RLQ mass.
Leukocytosis with neutrophilic predominance .
hite blood cells 19.
18y10A/AAL.
neutrophils 91%) was noted.
Ultrasonography demonstrated a lobulated, hyperechoic pelvic mass measuring 10.
46 cm.
Contrast-enhanced computed tomography showed a solidAecystic pelvic lesion with fat and calcification consistent with mature cystic teratoma alongside a periappendicular infiltrate.
Exploratory laparotomy disclosed 50 mL of purulent fluid, an abnormally enlarged appendix, multifocal ascending colon diverticulitis with perforation, dense adhesions, and a right ovarian mass.
Right hemicolectomy with transverse colon stump and end ileostomy together with total abdominal hysterectomy and bilateral salpingo-oophorectomy (TAH-BSO) were performed.
Histopathology confirmed LAMN with periappendicular infiltrate and a mature cystic teratoma of the right ovary.
Conclusion.
Coexistence of LAMN, perforated colonic diverticulitis, and mature ovarian teratoma can mimic adnexal or inflammatory disease and obscure recognition of the appendiceal Vigilant intra-operative inspection of the appendix and adjacent colon, oncologically appropriate resection, and definitive histopathology are essential to prevent PMP.
ranging from acute appendicitis and diverticula to neuroendocrine tumors and mucinous neoplasms.
Among low-grade lymphoid-rich tubular structure attached to the neoplasm (LAMN) is a distinctive entity defined by Although historically considered a vestigial neoplastic mucinous epithelium that replaces the organ, it is increasingly recognized for its immunologic role and as a reservoir for commensal flora that supports recovery of the colonic microbiome after extra-appendiceal 1,2 It is also home to a heterogeneous LAMN is uncommon, accounting for 0.
13Ae1% of all spectrum of neoplastic and non-neoplastic conditions appendectomy specimens depending on the series.
large German cohort of 108,247 appendectomies negative margins when disease is confined to the reported an incidence of only 0.
13%, whereas selected Right hemicolectomy is recommended for American and Asian series approached 1%.
3-5 Despite tumors at the appendiceal base, for positive margins, its rarity, recognition of LAMN matters because intra- for synchronous colonic pathology, and when intra- epitheliumAiwhether 1,6,17,18 When PMP is established, through inapparent transmural leakAican give rise to definitive treatment shifts to cytoreductive surgery pseudomyxoma peritonei (PMP), a slowly progressive but ultimately lethal condition characterized by chemotherapy (HIPEC) at high-volume centers.
gelatinous ascites and a redistribution phenomenon that coats peritoneal surfaces.
Despite this clarity at the extremes, an underdiscussed gray zone exists when LAMN coexists with Clinically.
LAMN is treacherous.
The classical perforated colonic diverticulitis and an ovarian mass teaching that it presents with right lower quadrant requiring its own oncologic clearance.
In low- and pain mimicking acute appendicitis applies to a middle-income regional hospitals, multidisciplinary input must be assembled rapidly without recourse to incidentally during imaging or surgery performed for intra-operative frozen-section pathology or on-site another indication.
2,10,11,12 The radiologic vocabulary peritoneal oncology programs.
The literature offers few developed around the mucoceleAi"onion-skin sign" on case-based templates that integrate the surgical ultrasonography, well-defined cystic tubular mass decision tree across all three pathologies.
11,14,15
The with mural calcification on CT, and the "volcano sign" To our knowledge, the simultaneous occurrence of at colonoscopyAiis helpful when present, but is absent histologically confirmed LAMN, multifocal perforated 10,12,13 ascending colon diverticulitis, and an ipsilateral Differentiating LAMN from an adnexal mass is mature cystic ovarian teratoma in a single patient has particularly difficult in women older than fifty, in not been previously documented in the surgical whom both pathologies coexist at appreciable rates literature from a district referral hospital setting.
This and the imaging features can overlap.
11,14
case report aims to .
describe the clinical, radiologic.
Coexistent intra-operative, and pathologic features of a 52-year- magnifies these difficulties.
Diverticular disease of the old female with this rare triad.
analyze the right colon, although less prevalent than left-sided disease in Western populations, is well described in preoperative diagnosis.
detail the intra-operative Asian and African cohorts.
Inflammation of ascending colon diverticula produces wall thickening, pericolic gynecologic procedure into a combined oncologic fat stranding, and on occasion perforation, all of which colectomy plus TAH-BSO.
synthesize current radiologically overlap with appendicular abscess and literature into a practical surveillance pathway aimed with the periappendicular infiltrate that frequently at preventing pseudomyxoma peritonei.
accompanies LAMN.
4,13,15 Adding a mature cystic teratoma of the right ovaryAiwith its tell-tale fat.
Case Presentation calcium, and dermoid plugAiproduces an imaging Ethics statement picture in which the gynecologic lesion captures the Written informed consent for publication of de- radiologistAos attention, while the more clinically identified clinical data and images was obtained from meaningful LAMN remains under-appreciated until the patient.
Institutional approval was waived for 14,16 single-patient case reports as per local policy.
Surgical management of LAMN is fundamentally about removing the lesion intact before mucinous spill Demographic and presenting complaint 52-year-old multiparous Indonesian female Current international guidance from the presented to the emergency department of Klungkung American Society of Colon and Rectal Surgeons Regional General Hospital with right lower quadrant (ASCRS) and PSOGI supports appendectomy with (RLQ) abdominal pain that had persisted for one month and acutely worsened over the forty-eight point, voluntary guarding, and a firm, ill-defined, hours preceding admission.
The pain was crampy, tender palpable mass in the RLQ approximately 10 cm intermittent at first and then constant, and was in greatest dimension.
RovsingAos sign was positive.
accompanied by three episodes of non-bilious, non- Bowel sounds were present but reduced.
A digital bloody vomiting and by nausea.
The patient denied rectal examination was unremarkable.
a per-vaginal examination revealed a non-tender cervix, no adnexal hematochezia, urinary symptoms, vaginal bleeding, or tenderness on bimanual palpation, and confirmed an vaginal discharge.
Flatus and stool passage were extra-uterine RLQ mass.
The patient was post-menopausal and had no Laboratory investigations history of malignancy, chronic illness, prior abdominal Initial laboratory studies are summarized in surgery, or recent travel.
She had been referred from Table 1.
Table 1 presents the complete blood count.
Gema Santi Nusa Penida Regional General Hospital leukocyte differential, hepatic and renal function, after an initial three-day in-patient stay during which electrolytes, and random blood glucose obtained on The principal abnormalities were marked intravenously every 24 hours, ibuprofen 400 mg three leukocytosis with neutrophilic predominance .
hite times daily, and intravenous omeprazole 40 mg twice suggesting an active inflammatory or infective process.
RingerAos 18y10A/AAL.
91%),
and mildly elevated serum creatinine .
4 mg/dL) Past medical and surgical history consistent with pre-renal azotemia from inadequate The patient initially experienced epigastric pain oral intake.
Hemoglobin and platelet counts were that migrated to the RLQ over one monthAia migration within reference ranges, lymphocytes and eosinophils pattern that may suggest acute appendicitis when it were depressed in a typical pattern of acute bacterial occurs over hours, but which over weeks favors a sub- stress, and electrolytes were within reference ranges.
Liver enzymes were normal.
Tumor markers (CEA.
CA- outpatient consultation with a general practitioner 125, and CA 19-.
were not available at the index during that month had yielded only oral analgesia without symptomatic improvement.
There was no Ultrasonography history of tuberculosis, hepatitis, diabetes mellitus.
Figure hypertension, or coagulopathy.
The patient denied non-steroidal demonstrated a hyperechoic, lobulated lesion with smoking, alcohol intake, and herbal-medicine use.
well-defined borders, thickened walls, and regular Menarche occurred at age 13 and menopause at age edges in the midline of the pelvic cavity.
The lesion she had three vaginal deliveries and reported no measured AP 10.
20 y LL 10.
44 y CC 10.
46 cm.
The prior pelvic infections.
lesion abutted the bowel loops in the RLQ.
the typical Physical examination onion-skin layering described in classical mucocele anti-inflammatory On arrival, the patient was alert and oriented with was not appreciated, and the appendix could not be a Glasgow Coma Scale of E4V5M6.
Vital signs were reliably visualized due to overlying bowel gas.
The blood pressure 120/70 mmHg, heart rate 89 beats per provisional ultrasonographic interpretation was a minute, respiratory rate 20 breaths per minute, pelvic abscess versus an intra-abdominal mass of axillary temperature 36.
0 AC, and oxygen saturation indeterminate origin.
The probe was placed both 98% on room air.
The skin and conjunctivae were not pale or icteric.
Cardiopulmonary examination was vascularity within the lesion was mild on colour Abdominal examination revealed mild Doppler.
McBurneyAos distension with tenderness localized to McBurneyAos Table 1.
Initial laboratory test results on admission.
Test Group / Subgroup Parameter Result Unit Reference range Hematology Ae Complete blood count Hemoglobin g/dL 8 Ae 16.
White blood cells* y10A/AAL 5 Ae 10.
Red blood cells y10A/AAL 5 Ae 5.
Hematocrit 35 Ae 55 Platelets y10A/AAL 145 Ae 450 NeutrophilsA 3 Ae 73.
Lymphocytes 0 Ae 48.
Monocytes 4 Ae 12.
Eosinophils 60 Ae 7.
Basophils 00 Ae 1.
MCV
1 Ae 96.
MCH
0 Ae 31.
MCHC
5 Ae 35.
RDW-CV
5 Ae 14.
MPV
90 Ae 10.
AST
U/L
8 Ae 37
ALT
U/L
13 Ae 42 Urea mg/dL 10 Ae 50 CreatinineA mg/dL 6 Ae 1.
Sodium (N.
135 Ae 145 Potassium (K) 5 Ae 4.
Chloride (C.
95 Ae 105 Random blood glucose mg/dL 80 Ae 200 Leukocyte differential Red-cell indices Liver enzymes Renal function Electrolytes Glycemia Notes: *Marked leukocytosis with neutrophilic predominance .
%) suggested an acute inflammatory or infective focus.
ANeutrophil predominance >85% in a clinically septic-appearing abdomen mandated urgent imaging and surgical evaluation.
AMild creatinine elevation reflected pre-renal azotemia from poor intake.
it normalized after fluid resuscitation.
Figure 1.
Abdominal ultrasonography (A and B) showed a hyperechoic, lobulated lesion .
46 c.
with thick walls and well-defined borders in the midline pelvis, with adjacent bowel and the iliac vessels labelled.
appearance favored an abscess or intra-abdominal mass of indeterminate origin.
Contrast-enhanced computed tomography the RLQ a focal densification of the bowel wall with Contrast-enhanced CT of the abdomen and pelvis accompanied by an enhancing tubular structure (Figure .
was performed to characterize the pelvic within an inflammatory phlegmon, was suggestive of a mass and the right lower quadrant infiltrate.
Two periappendicular infiltrate with mucinous content.
principal abnormalities were demonstrated.
First, in classic features of LAMN such as mural calcification or an elongated low-density tubular cyst were not fat density, calcifications, and a discrete septation unequivocally present.
Second, in the pelvis strongly favored a heterogeneous solidAecystic mass with internal echoes, mature cystic Figure 2.
Contrast-enhanced abdominal CT.
(A) Densification with surrounding fat stranding and intramural air density in the RLQ, accompanied by an enhancing tubular structure within a colonic phlegmon, suggestive of periappendicular (B) A septated solidAecystic pelvic mass with fat, internal echoes, and calcificationAifeatures classical for a mature cystic teratoma.
On the basis of these imaging findings, the working Dense inflammatory adhesions were encountered preoperative diagnosis was: .
right ovarian mature between the ascending colon and the right lobe of the cystic teratoma, .
periappendicular infiltrate with liver, the gallbladder fundus and the transverse mesocolon, the cecum and the parietal peritoneum, inflammatory mass of indeterminate etiology.
The and the right adnexa and the right ovarian mass.
differential diagnoses entertained were appendiceal Adhesiolysis was performed sharp and blunt under mucocele, mucinous neoplasm of the appendix, direct vision.
The ascending colon revealed multiple complicated diverticulitis of the right colon, andAiless inflamed diverticula, two of which had frankly likelyAia cecal adenocarcinoma.
Tumor markers were perforated and were sealed by adjacent omentum and ordered but the result was not available before small bowel loops.
the perforation sites were proximal to the periappendiceal phlegmon, and pus tracked Operative findings and surgical strategy along the right paracolic gutter.
The right ovary .
broad-spectrum contained a 10-cm thick-walled cystic mass with antibiotics .
eftriaxone 2 g IV every 24 hours plus intra-cystic sebaceous content.
The contralateral metronidazole 500 mg IV every 8 hour.
, and informed adnexa appeared grossly normal.
The uterus showed consent for an exploratory laparotomy with possible mild fibroid changes.
After right hemicolectomy and TAH-BSO, the patient was After taken to the operating theatre.
A combined surgical team consisting of the digestive surgery service and .
the obstetrics and gynecology service performed the suspicious for a mucinous neoplasm, .
perforated The abdomen was entered via a lower ascending colon diverticulitis, and .
an ovarian Approximately 50 mL of purulent peritoneal fluid was menopausal patient, the operative plan was extended.
aspirated and sent for culture.
The appendix was An oncologically formal right hemicolectomy was identified as abnormally enlarged, with a thickened, performed with high ligation of the ileocolic and right dilated lumen and a serosa coated with fibrin and mucin-like material.
no frank free intra-peritoneal mucin pools or peritoneal nodules consistent with lymphovascular pedicles, and resection of the involved established pseudomyxoma peritonei were visualized.
Given pre-existing contamination from en-bloc diverticular perforation and the risk of an anastomotic complication in an already inflamed bed, a transverse inflammatory infiltrates with dilated congested vessels colon stump and an end ileostomy were fashioned and focal extra-appendiceal mucin extrusion without rather than a primary ileo-transverse anastomosis.
The appendiceal wall.
The resection margins of the ileum, hysterectomy with transverse colon stump, and mesentery were free of (TAH-BSO).
Copious neoplastic epithelium.
Multifocal diverticulosis with lavage, peritoneal drain placement, and a layered peri-diverticular abdominal closure completed the procedure.
fibropurulent inflammation was documented in the Histopathology ascending colon segment.
The constellation supported Gross examination of the right hemicolectomy a diagnosis of low-grade appendiceal mucinous specimen revealed an enlarged appendix measuring neoplasm (LAMN) with localized periappendicular 5 y 3 y 2.
5 cm with a dilated, mucin-filled lumen and mucinous and inflammatory spill but no high-grade a flattened mucosa.
Histopathology (Figure 3, panels dysplasia and no invasion.
Histopathology of the right AAeD) demonstrated a denuded mucosal lining with demonstrated a thick-walled fibrotic ovarian cyst lined epithelium of low-grade cytologic atypia, dispersed by keratinized stratified squamous epithelium with villous and undulating papillary configurations, loss of skin appendages, sebaceous glands, mature adipose tissue, and cartilaginous fociAifeatures diagnostic of a submucosal fibrosis, and abundant intraluminal mature cystic teratoma.
No immature elements, no germ-cell mucin-producing Lymphoid (Figure and no submucosa was markedly diminished.
The serosa of transformation were identified.
The contralateral the mesoappendix and adjacent appendicular bed ovary, both fallopian tubes, the uterine corpus, and showed dense polymorphonuclear and mononuclear the cervix were unremarkable on routine sections.
Figure 3.
Histopathology of the appendix and ovarian specimens .
ematoxylinAeeosi.
(A) Appendiceal lumen distended by intra-luminal mucin and flattened mucinous epithelium.
(B) Mucosal denudation with absent lymphoid tissue and loss of crypts.
(C) Higher-magnification view .
showing low-grade mucinous epithelium with villous undulation.
(D)
Periappendiceal infiltrate with polymorphonuclear and mononuclear cells and focal mucinous extravasation.
(E) Right ovarian cyst wall lined by keratinized stratified squamous epithelium with skin appendages, sebaceous glands, mature adipose tissue, and a focus of cartilageAifeatures diagnostic of a mature cystic teratoma.
Postoperative course The clinical phenotype was dominated by the most The patient was transferred to the intensive care acutely symptomatic processAicolonic diverticulitis with perforationAiand by the most radiologically hemodynamic monitoring, then to the general surgical striking lesionAithe teratoma.
The LAMN, the most ward for four additional days.
The postoperative prognostically meaningful of the three, was the regimen comprised intravenous ceftriaxone 2 g every 24 hours and metronidazole 500 mg every 8 hours for observation in published series that LAMN often seven days, escalating oral diet from clear fluids to soft masquerades as another disease and is recognized (ICU) intravenous paracetamol and rescue intramuscular This 11,14,15 ketorolac, and standard venous thromboprophylaxis.
Three principal pitfalls operated in this case.
First.
The peritoneal drain was removed on postoperative the temporal evolution of the painAian indolent day 4, the ileostomy was functioning by day 2, and the epigastric ache migrating over a month to the RLQAi abdominal wound healed by primary intention.
The patient was discharged on postoperative day 5 with trajectory and led both the patient and the initial oral cefixime 200 mg twice daily for one week, outpatient provider to rely on analgesia.
Second, the paracetamol 500 mg three times daily, ibuprofen 400 radiologic signal-to-noise ratio was dominated by the mg three times daily as needed, omeprazole 20 mg Fat, calcium, and septation on CT are twice daily, ileostomy care training, and an outpatient surveillance plan including serial CEA.
CA 19-9, and radiologistAos report.
meanwhile the LAMNAos tubular CA-125 measurements and a follow-up contrast- cystic enhancement was buried inside a phlegmon enhanced CT of the abdomen and pelvis at three attributed to periappendicular infiltrate, exactly the Reversal of the ileostomy was planned for pattern Kindie and colleagues describe in their report three months after the index operation pending of LAMN mimicking an adnexal tumor and Lu and surveillance and nutritional optimization.
colleagues describe as "diagnostic uncertainty" in "aha" acute-appendicitis chronic right-sided pain.
11,14 Third, in the absence of Discussion intra-operative Low-grade appendiceal mucinous neoplasm is in frozen-section pathology, the operative team had to itself uncommon.
PSOGI-defined LAMN was identified rely on macroscopic appearance and clinical judgment in only 0.
13% of 108,247 appendectomies in one large to identify the appendix as neoplastic.
only the German series and in 1% of selected American series.
unusual size and texture of the appendiceal wall.
Even rarer is its synchronous occurrence with histologically confirmed perforated ascending colon perforation, raised suspicion.
6,12,15
diverticulitis and ipsilateral mature cystic teratoma of LAMN arises from neoplastic mucinous epithelium the ovaryAia constellation that, to our knowledge, has replacing the normal columnar mucosa of the not been documented in detail in surgical literature.
Molecularly.
GNAS and KRAS mutations are The three pathologies share an anatomical address characteristic, supporting a clonal monoepithelial .
ight lower quadran.
but radically different biological origin distinct from cecal adenocarcinoma.
1,2 Mucin behaviors: LAMN is an indolent epithelial neoplasm retention within the obstructed appendix produces whose principal hazard is mucinous spill into the progressive distension, mural attenuation, loss of diverticulitis is an acute inflammatory disease whose hazard is perforation and sepsis.
lymphoid atrophyAieach documented in this case.
mature cystic teratoma is a benign germ-cell tumor intraluminal pressure rises, mucinous spill can occur whose hazard, in a post-menopausal woman, is occult through frank rupture or microscopic transmural peritoneal cavity, the redistribution phenomenonAi 6,16,17 Each Once gravitational pooling in the pelvis, sub-diaphragmatic recesses, and omentumAidrives the development of calcification, septatio.
are highly specific, and once PMP.
present the diagnosis is rarely missed.
what is more The presence of periappendicular mucinous and often missed is a second concurrent pathology, inflammatory extravasation in our specimen, in the especially LAMN, whose radiologic features are non-
absence of identifiable neoplastic epithelium beyond
12,13,16
the appendiceal wall, is best classified under PSOGIAos Ultrasonography is rapid and inexpensive, and in framework as "low-grade mucinous neoplasm confined to appendix with localized peritoneal extension" echogenic mucin within a tubular cyst is reasonably .
T4.
Aia category at intermediate risk of recurrence specific for an appendiceal mucinous neoplasm.
10,12 In as PMP that mandates structured imaging and tumor- our patient the lesion was hyperechoic and lobulated marker surveillance.
1,6,18,19
but without the classic layering.
we do not therefore Right-sided diverticulitis differs from its sigmoid "onion-skin criticize the original ultrasonographic interpretation.
counterpart in epidemiology and pathogenesis.
It is Contrast-enhanced more common in younger Asian patients and typically modality, with reported sensitivity around 83% and specificity around 92% for LAMN, but its diagnostic acquired pulsion diverticula.
When the diverticular accuracy collapses in the setting of an adjacent pouch obstructs, the same pathophysiologic sequence inflammatory mass, as occurred here.
MRI offers as left-sided disease applies: bacterial overgrowth, superior soft-tissue contrast and may distinguish solid from cystic components and characterize mucin.
The proximity of perforated ascending but data on its accuracy for LAMN are limited.
10,12,13
colon diverticulitis to a LAMN-bearing appendix is Colonoscopy can demonstrate the "volcano sign" at the appendiceal orifice and contribute to differential tether the appendix to a diseased segment and can diagnosis, but it is rarely available before emergency either occlude the appendiceal lumen and predispose surgery and is uncomfortable in a patient with it to mucinous distension, or distort the appendiceal Tumor markers (CEA.
CA 19-9.
CA-.
wall during inflammation and contribute to the lack sensitivity and specificity individually but, taken periappendicular infiltrate that confounds radiology.
serially, are useful for surveillance.
6,8,17,18
4,15
Inflammatory We did not find a published anatomically detailed Definitive diagnosis of LAMN is histopathologic.
The coexistence series, but the case literature suggests PSOGI that any inflammatory process that thickens the classifications define LAMN as a low-grade mucinous caeco-colonic epithelial neoplasm with one or more of: loss of Delphi muscularis mucosae, fibrosis of the submucosa.
In a post-menopausal woman presenting with RLQ "pushing" invasion, undulating or flat epithelial pain and a 10-cm pelvic mass, the differential growth, mucin dissection of the wall, and rupture with diagnosis is broad.
Adnexal neoplasiaAibenign extra-appendiceal mucin.
1,2,20 Our patientAos specimen serous or mucinous cystadenomas, mature cystic displayed all of these features.
The differential under teratoma, borderline ovarian tumors, and frank the microscope includes appendiceal diverticulumAi where the mucosa herniates through a wall defect Appendiceal pathologyAi periappendicular abscess, mucocele of any etiology.
LAMN, epitheliumAiand high-grade mucinous neoplasm (HAMN), which appendiceal adenocarcinoma.
Colonic pathologyAi features high-grade cytologic dysplasia.
2,4 Lowes and diverticular abscess, cecal colleagues recently codified the histomorphologic CrohnAos stricture.
Uterine pathologyAipyometra, large fibroid, leiomyosarcoma.
Mesenteric or appendiceal diverticular disease in the largest series pathologyAituberculous to date, demonstrating that misclassification of LAMN The diverticular disease as LAMN occurred in 71% of radiologic "fingerprints" of mature cystic teratoma .
at, pathologist consultations.
their criteriaAiretention of normal frank pus tracked along the right paracolic gutter, and arrangement, and absence of nuclear abnormalities pre-existing diverticular perforation rendered the operative field heavily contaminated.
The risk of an cryptsAianchor adopted in our case.
anastomotic leak under those conditions has been The decisive question in our case was whether to reported to exceed 15% in some series.
An end limit resection to an appendectomy plus drainage of pericolic abscess and a planned interval gynecologic proximal diversion, allowed unhurried recovery of the procedure, or to perform a synchronous oncologic inflamed colonic wall, right hemicolectomy plus TAH-BSO.
Five arguments reversal in three months.
This staged strategy follows damage-control .
The transverse-colon and permitted controlled macroscopically suspicious for a neoplasm.
The hospitals where intensive postoperative imaging and ascending colon was the site of multiple perforated re-laparotomy resources are limited.
6,15,17
a damage-control approach with right mass, the residual ovarian function is negligible, and substantial leak risk of a primary anastomosis in an the risk of occult malignancy or torsion exceeds the actively infected bed.
6,17 .
The ovarian mass required benefit of preserving the contralateral ovary.
The oncologic clearance because, in a post-menopausal decision for bilateral salpingo-oophorectomy plus patient, the probability of an occult borderline or hysterectomy followed standard gynecologic-oncologic malignant component was In a post-menopausal woman with a 10-cm pelvic non-trivial.
The practice and was performed by the obstetrics and gynecology team in the same operative sitting, taking unsuccessful conservative management at an outside further temporization risked frank rupture of histopathology fortunately confirmed a benign mature the appendix and frank establishment of PMP.
7,8,9 .
Multi-staged surgery imposes a logistic and financial malignant transformation, an outcome which was not burden on the patient and her family in a regional assured pre-operatively and which justifies oncologic district setting.
one definitive procedure was likely to clearance in similar scenarios.
be both more oncologically adequate and more Final Even when LAMN is judged to be confined and extra-appendiceal Counter-arguments included the operative time of mucinous extravasationAias identified histologically a combined three-team procedure, the loss of bowel in our patientAiplaces her at intermediate risk of continuity from an end ileostomy, and the absence of subsequent PMP.
6,18,19 Recommended surveillance frozen-section LAMN comprises .
clinical review at three months and every diagnosis intra-operatively.
After balancing these six months thereafter for the first three years.
serial considerations and obtaining informed consent, the CEA.
CA 19-9, and CA-125 measurements every three decision to proceed with a combined definitive to six months.
annual contrast-enhanced CT or procedure was made by consensus.
Glasgow and MRI of the abdomen and pelvis for five years.
colleaguesAo ASCRS guidelines explicitly endorse right prompt referral to a peritoneal-surface oncology center hemicolectomy when synchronous colonic pathology at the first evidence of mucinous peritoneal deposits.
coexists with a suspected appendiceal neoplasm.
6 The Although 2019 abdominal-radiology and HIPEC review by cytoreductive surgery with HIPEC, the surveillance Bartlett and colleagues, and the 2025 integrated pathway above is achievable in a district setting and review by Mitamura and colleagues, both reinforce ensures timely referral if PMP develops.
Chua and that oncologically appropriate resection at the index colleaguesAo operation reduces downstream PMP risk.
12,17
meaningful long-term survival after CRS plus HIPEC Primary ileo-transverse colonic anastomosis is the standard reconstruction after right hemicolectomy in multi-center on-site for appendiceal-origin PMP, reinforcing the value of early detection.
the absence of contamination.
In our case, 50 mL of Several recent reports illuminate the diagnostic colonic perforation, and should construct an operative difficulty of LAMN.
Kindie and colleagues described a roadmap that can flex from gynecologic-only surgery LAMN mimicking a malignant ovarian tumor in an to combined oncologic surgery on the basis of intra- Ethiopian patient.
Lu and colleagues reviewed eleven operative findings.
The 2025 integrative review by reports of preoperative diagnostic uncertainty.
Liu and Mitamura colleagues reported a peri-orificial LAMN managed by treatment-strategy double purse-string appendectomy.
Edwards and histological and molecular features.
colleagues distinguished a perforated appendiceal and colleagues Table 2 presents a structured comparison of our case with the four most directly relevant published 11,14,15,21 The unique feature of the present case is the pathology phenotype of those reports, our patient synchronous occurrence of three independent right carried three concurrent pathologies.
Our case is lower quadrant pathologies and the corresponding therefore best understood as an extension of those need for a combined three-team operative plan, reports: when imaging suggests adnexal pathology in whereas the comparator series uniformly describe a the RLQ in a woman over 50, the surgical team should single appendiceal pathology with at most one plan operatively for the possibility of a coexistent additional finding.
appendiceal mucinous neoplasm and a coexistent Table 2.
Comparison of the present case with the most relevant published reports of LAMN with diagnostic or operative complexity.
Author.
Year (Countr.
Patient .
ge / se.
Clinical Imaging findings Operative procedure Final pathology & USG: lobulated pelvic 46 cm.
CT:
solid-cystic adnexal mass with fat and calcification, plus Right hemicolectomy transverse colon stump end ileostomy TAH-BSO .
ombined surgery and gynecology team.
LAMN perforated ascending colon diverticulitis mature ovarian cystic ICU 1 d, ward 4 d, structured 5yr PMP surveillance Present case.
Sandika & Yustina, 2026 (Indonesi.
A 52 / F One-month RLQ pain with two-day McBurney tenderness, palpable RLQ mass Kindie et al.
, 2025
(Ethiopi.
45 / F Chronic RLQ pain with palpable mass CT suggested malignant adnexal lesion Right hemicolectomy right salpingophorectomy LAMN initially mistaken for ovarian malignancy.
uneventful recovery Lu et al.
, 2024 (USA)
61 / F
Intermittent abdominal pain with uncertainty over 14 CT: cystic appendiceal mass mimicking abscess Appendectomy with mucinous-spill LAMN confined to instituted, no PMP at 6 Liu et al.
, 2025
(Chin.
58 / F Incidental finding during colonoscopy for screening Cystic lesion at appendiceal orifice on Appendectomy with double purse-string suture at appendiceal LAMN at appendiceal resection, no recurrence at follow-up Edwards et al.
, 2024
(USA)
70 / M
Chronic right lower abdominal pain CT showing chronic appendicitis with cystic Laparoscopic Perforated appendiceal diverticulum Ai distinguished from LAMN on histology Notes: AThe present case is highlighted in the first row.
Abbreviations: F = female.
M = male.
LAMN = low-grade appendiceal mucinous RLQ = right lower quadrant.
USG = ultrasonography.
CT = computed tomography.
TAH-BSO = total abdominal hysterectomy with bilateral salpingo-oophorectomy.
ICU = intensive care unit.
PMP = pseudomyxoma peritonei Several limitations merit explicit acknowledgement.
First, preoperative tumor markers (CEA.
CA 19-9.
CA- Third, molecular profiling (GNAS.
KRAS, .
were not available in time to inform the operative TP.
was not performed, limiting comparison with this is common in regional Indonesian hospitals but constrains the diagnostic algorithm we can cannot extrapolate from a single case to a general frozen-section further multi-center experience will be pathology was not available.
the operative escalation required to define when prophylactic peritonectomy or to right hemicolectomy was therefore informed by empirical HIPEC may be justified at the index Second, intra-operative Fourth, operation District referral hospitals in low- and middle- Fifth, our follow-up at the time of income countries provide care for the majority of writing is short.
long-term PMP risk will be quantified surgical patients globally yet contribute only a small only after several years of surveillance.
fraction of published surgical literature.
Several The reported incidence of appendiceal mucinous practical realities shape decisions here that differ from neoplasms has risen progressively over the past two those in tertiary centers.
Frozen-section pathology is Marmor and colleagues, in a population- rarely available.
intra-operative consultation must rely based analysis of United States data between 2000 on macroscopic appraisal and on the operating and 2009, documented an increase in age-adjusted surgeonAos appendiceal cancer incidence from 0.
97 to 1.
32 per turnaround time is days rather than hours, so surgical 100,000 person-years, attributable in part to improved plans cannot await results in an unstable patient.
pathologic recognition of mucinous tumors and to Magnetic resonance imaging is often not accessible increased use of cross-sectional imaging.
5 Smeenk and colleagues, in a Dutch population-based study, tomography may be limited to a single dual-phase Cytoreductive Tumor-marker HIPEC account for approximately half of all cases of centralized at a few tertiary referral centers, often in a pseudomyxoma peritonei.
22 Female predominance is different province, with delays in referral that are consistent across these series, with the mean age at measured in months rather than weeks.
These diagnosis between fifty-five and sixty years.
Our constraints place a premium on definitive index patientAos demographic profile is therefore typical of the operations whenever it can be done safely, and on at-risk population, and the prevalence of the disease robust postoperative surveillance pathways that flag is high enough that surgeons in district hospitals PMP early so that timely referral can salvage long-term should expect to encounter several cases over a career.
Reliable incidence data for Indonesia are not available.
oncologically formal right hemicolectomy with end the present case is a small contribution to addressing ileostomy, concurrent TAH-BSO, and structured five- that gap and emphasizes the need for a national year surveillance with tumor markers and cross- appendiceal pathology registry.
sectional imagingAireflects an attempt to apply the Molecular characterization of LAMN has matured.
Whole-exome and targeted sequencing studies have The caseAi principles of evidence-based surgical oncology to a resource-realistic delivery model.
6,8,17
repeatedly identified activating mutations of GNAS Drawing the threads of this case together, several .
odons 201 and .
and KRAS .
odons 12, 13, and practical lessons emerge for surgeons working in district referral hospitals.
A high index of suspicion for 1,2,17 TP53 mutations and chromosomal an unrecognized appendiceal mucinous neoplasm complexity are more common in high-grade mucinous should accompany every evaluation of a woman over neoplasms and in invasive adenocarcinoma, whereas fifty who presents with a right lower quadrant pelvic LAMN typically displays a relatively quiet genome.
The mass, even when imaging is dominated by an adnexal clinical implication is that biologic targetingAieither of the cyclic adenosine monophosphateAeprotein kinase A pathway downstream of GNAS or of the MAPK cascade carbohydrate antigen 19-9 and CA-125, together with defining drivers of low-grade KRASAiremains a radiology report that actively searches for a tubular current trials of MEK inhibitors and of GNAS-targeted cystic mass, mural calcification and the onion-skin strategies are preliminary.
Molecular profiling is not sign in addition to commenting on the dominant currently mandated for management decisions in lesion, materially improves diagnostic accuracy.
When LAMN confined to the appendix, but emerging data the appendix appears macroscopically abnormal at suggest that in patients with established PMP, laparotomy, an en-bloc oncologic resection is generally mutational status may stratify the response to preferable to a simple appendectomy, especially when cytoreductive surgery and HIPEC.
6,8,9,17
the adjacent colon is also diseased or when frozen- section pathology is unavailable.
in the setting of cross-sectional imaging to ensure that pseudomyxoma peritonei, the principal long-term threat, is detected right-sided contamination, an end ileostomy and transverse colon stump remain safer than a primary ileo-transverse The operative note should document the precise location and amount of any extra-appendiceal promptly if it develops.
References