Non-resolving appendicular mass

By Dr.K.Priyatham 2nd year P.G

Dr.Y.Prabhakar Rao, M.S, M.Ch Professor, 2nd unit chief

Department of General Surgery

• A 50 year old female(I.P no- 15/958188) presented on 14/1/15 with complaint of pain in the lower abdomen since 10 days.

• Pain in right lower quadrant, insidious in onset, gradually progressive in nature, no radiation of pain, no aggravating factors

• H/o of fever for 2 days, continuous, high grade• Pain & fever subsided on medication after 2 days of onset of

symptoms.• The patient was initially treated by a R.M.P for 10 days.• As there is recurrence of pain after 10 days, she was referred

to NRIGH for further management.

• No H/o loss of weight & appetite.• No h/o of vomiting, diarrhea, constipation,

clay stools, mucus in stools, distension• No h/o jaundice, malena, bleeding per rectum,• No h/o tuberculosis

• History of past illness: No history of similar complaint in the past H/o caesarian done 20 years back• Personal history: Takes mixed diet, bowel and bladder habits

are normal, sleep and appetite are normal• Family history: Not significant

General condition:• Patient is conscious, oriented, moderately

built & moderately nourished. pallor+, no icterus, no cyanosis ,no clubbing ,

no significant lymphadenopathy.

• Vitals stable

Systemic examination

• Per Abdomen –• Inspection- fullness noted in the right lower quadrant.• Lower midline vertical scar is present.• Abdominal striae are present.• Umbilicus is central in position & inverted. • No visible pulsations, no dilated veins, no visible

peristalsis.• Renal angles are normal.• Hernial sites & external genitalia are normal.

• Palpation-• Palpable mass of size 8 × 10cms in the right iliac

fossa, minimal tenderness is present, surface is smooth , margins are well defined , firm in consistency, intra abdominal, no mobility with respiration, no intrinsic mobility.

• Hernial orifices- normal• Percussion – dull over the swelling, no free fluid• Auscultation -Bowel sounds -normally heard

• CVS- normal

• RS- bilateral normal vesicular breath sounds heard

• CNS-no neurological deficits

Differential diagnosis

1) Appendicular abscess

2) Ileocaecal tuberculosis

3) Carcinoma of ileocaecal junction

Investigations

• U/S abdomen – 4.4 cms × 5.4 cms irregular collection in right iliac fossa.

• Another well defined hypoechoic lesion measuring 4.4cms × 3.8 cms is seen in right iliac fossa with thickening of adjacent mesentery.

• Impression – Appendicular mass with abscess formation.

• U/S guided aspiration of 20 ml pus was done

• Hemoglobin: 5.5 gms%• White blood cell count: 18,000 cells/mm3 • Differential count: 82(N),10(L),02(E),03(M)• PCV – 18%• ESR – 140 mm/ 1st hour• RBC- 2.4 millions/Cu mm• Platelets – 6.8 lakhs/ mm3• MCV – 73 fl• MCH – 22 pg• MCHC – 30% • Peripheral smear – microcytic hypochromic anemia,

neutrophilic leucocytosis, thrombocytosis.

• Serum urea: 20 mg/dl• Serum creatinine: 1.0 mg/dl• Sodium: 132 mmol/l• Potassium : 3.1 mmol/l• Blood Grouping: O +ve• Viral Markers : negative

• Conservative management was done with• Inj Magnex forte 1.5gms IV BD• Inj Metrogyl 500 mg IV TID• Tab Pantop 40 mg OD• Tab Aceclo P BD• Blood transfusions were given to improve Hb

%

• Review U/S on 17/1/15 – (3 days after admission)

• Size of collection 3 × 1.3 cms (from 4.4cms × 5.4 cms)

• Significantly reduced in size• Mass in right iliac fossa is same as previous

scan.

• As there is no evidence of pus formation, conservative management was continued.

• Despite following conservative management for 8 days, appendicular mass did not decrease in size.

• Therefore CECT abdomen was done on 22/1/15 ( 8 days after admission)

Cect abdomen

• Ill defined heterogeneously enhancing lesion measuring approximately 6.1 × 5.1 cms noted in the right iliac fossa.

• The lesion shows cystic areas. Inflammatory changes with fat stranding is noted around the lesion.

• The inflammation is seen extending in to the anterolateral wall of urinary bladder, caecum & rectus abdominus muscle.

• Few enlarged lymph nodes are noted in right iliac fossa.• Appendix is not visualised.

Cystic Solid

Bladder

Caecum

Mass

Caecum

Ileum

• Impression - ? Appendicular abscess with mass formation.

• Conservative management was continued for 6 more days.

• Case was posted for exploratory laparotomy in view of non resolving mass of appendix on 29/1/15. (duration of hospital stay – 15 days, duration of symptoms – 25 days)

Surgery

• Procedure – Exploratory laprotomy• Intra-operative findings –1) Firm solid mass noted posterior to the

terminal ileum (8 × 5 cms)2) Uterus along with right ovary adherent to the

mass3) Omental adhesion are noted to the left of the

mass & previous scar site.

• Lower midline incision from umblicus to symphis pubis.

• On reaching the peritoneal cavity, omental adhesions are noted.

• Omental adhesions were seperated• Firm mass of size 8 × 5cms beneath the terminal

ileum.• Uterus & right ovary separated from the mass• Right colon is mobilized by incising white line of toldt.

Uterus

Terminal ileum

Omental adhesions

Ovaries

Mucin from the mass

• Right hemi-colectomy done after ligation of ileocolic artery, right colic artery, right branch of middle colic artery.

• Specimen was sent for HPE.• Ileum & transverse colon are anastomosed by using PDS

sutures• Hemostasis secured, drain placed in the morisson’s

pouch.• Abdomen closed in layers.• Cut section of tumor showed mucinous like substance.

Anastamosis

Ileum

Transverse colon

TerminalIleum

Ascending Colon

Biopsy

• Sections studied shows a well differentiated mucin producing adenocarcinoma exhibiting transmural infiltration, serosal nodular extension, lympho vascular embolization, surface ulceration & secondary inflammation.

• The adjacent colonic mucosa shows features suggestive of inflammatory bowel disease with mucosal ulceration

• Both the cut margins & small intestinal segment seperately received show mild chronic ileocolitis changes with sub mucosal edematous widening.

• There is no tumor extension• All the 4 lymphnodes identified in the

mesenteric fat show metastatic deposits from colonic carcinoma.

• Appendix is not seen.

• Opinion – histological appearances are in favor of well differentiated mucin producing adenocarcinoma with metastatic deposits in lymph nodes.

Final diagnosis

1) Mucinous adenocarcinoma of the appendix. (per-operative diagnosis)

2) Well differentiated mucin producing adenocarcinoma with metastatic deposits. (pathological diagnosis)

Normal colonic mucosa- 1Infiltrating mucin secreting adenocarcinoma-2

1 2

Large mucous distended tumorous gland with lining-1 & Lymphovascular emboli-2

1 2

Appendiceal Tumours

• Primary appendicular tumors occur in 0.9% to 1.4% of all appendectomies.

• <50% of cases are recognized pre-operatively.• Almost 85% are carcinoids.• Adenocarcinomas of the appendix are a category

of rare tumors of the gastrointestinal tract, with a frequency of 0.2% - 0.5% of all intestinal malignancies and 4% - 6% over neoplastic lesions of the appendix

• The first case of a primary adenocarcinoma of the appendix was reported by Berger on 1882

• Their main presentation is that of an acute appendicitis or as a palpable mass, mainly in the right lower quadrant.

• Approximately 30%–50% of patients present clinically with signs and symptoms of acute appendicitis, most often due to occlusion of the appendiceal lumen by tumor.

• Although at present they are a well studied pathologic entity, the crucial issue of their preoperative diagnosis remains unsolved.

• Diagnosis of underlying tumor is usually made only at the time of surgery or even later, during pathologic examination of the surgical specimen.

• This delay in diagnosis often necessitates modification of the surgical approach or a second surgical procedure such as right hemicolectomy.

Adenocarcinoma

• A malignant epithelial neoplasm of the appendix with invasion beyond the muscularis mucosae.

• 0.12 cases per 1,000,000 appendicectomies annually.• F=M• Occurs 6th decade of life• Rarer but more aggressive type.• It is of 2 types – 1) Mucin secreting adenocarcinoma 2) Non-mucin secreting adenocarcinoma

• Patients with chronic ulcerative colitis (UC) have an increased susceptibility to formation of epithelial dysplasia and malignancy in affected segments of bowel;

• Inflammatory involvement of the appendix is seen in approximately half of UC cases with pancolitis.

• Both adenoma and adenocarcinoma of the appendix have been described in patients affected by long-standing ulcerative colitis

• Spread to the peritoneal cavity may produce large volume of mucin causing psuedomyxoma peritonei – abdominal distension.

• Treatment–Appendectomy + right hemicolectomy.–Simple appendectomy for adenocarcinomas

that are confined to the mucosa or well-differentiated lesions that invade no deeper than the submucosa.–Role of adjuvant chemotherapy/RT is

unclear.–Adjuvant chemotherapy – 5 FU

• Prognosis – poorer than carcinoid. • Because of similarities with colon carcinoma,

appendiceal adenocarcinomas are classified as- Duke’s stage A – 100% 5 year survival rate B – 67% C – 50% D – 6%• Mucinous adenocarcinoma has better 5 year

survival rate of 70% over 40% of colonic type of adenocarcinoma

Carcinoid - Argentaffinoma

• Carcinoids account for 50-77% of all appendiceal neoplasms.

• They arise from argentaffin tissue.• 45% of carcinoids occur in the appendix.• Other sites of carcinoids – ileum(25%),

rectum(15%), others- pancreas, biliary tract, bronchus & testis.

• It is found 1 in 300-400 appendicectomies subjected to H.P.E.

• It is 10 times more common than any other neoplasm of appendix.

• The mean age at presentation is 32-43 years (range, 6 to 80 years)

• Appendiceal carcinoids occur more frequently in females than in males

• Majority of carcinoids are located at the tip of the appendix.

• The carcinoid mass is the cause of appendicitis in 25% cases only.

• About 75% of cases are less than 1cm in size.• 5-10% are over 2cms.• Lymph node invasion & distant metastases are

exceedingly rare except in tumors above 2cms.

• Carcinoid syndrome ( flushing, SOB, diarrhea, Right sided heart valve disease) caused by an appendiceal carcinoid is extremely rare and almost always related to widespread metastases, usually to the liver and retro-peritoneum.

Treatment• Treatment appendiceal carcinoids is dictated mainly by

tumor size. • Simple appendecectomy is sufficient for tumors less than

2cm & tumors at the tip of the appendix because of low likelihood of lymphnode involvement.

• For masses greater than 2cms right hemicolectomy is recommended.

• For carcinoids involving the base of the appendix – right hemicolectomy is advised.

• Carcinoids are less aggressive & carry a much favorable prognosis 0f 90% 5 year survival rate

Pseudomyxoma peritonei (PMP)• Pseudomyxoma peritonei refers to intraperitoneal accumulation of a

gelatinous ascites secondary to rupture of a mucinous tumour. The most common cause is a ruptured mucinous tumour of the appendix / appendiceal mucocoele. Other sources are colon, rectum, stomach, gallbladder, bile ducts, small intestine, urinary bladder, lung, breast, fallopian tubes and pancreas.

• Usually has metastased at time of presentation.• Spread

– direct– rarely through bloodstream or lymphatics.

• Sypmtoms– Bowel obstruction– Increase in abdominal size (Jelly Belly abdomen)– Pelvic discomfort– Ovarian masses

• If pseudomyxoma peritonei is noted during the operation, cytoreductive surgery plus intraperitoneal chemotherapy with or without hyperthermia therapy should be done.

• With traditional debulking surgery, the over-all five year survival rates is about 30-50% according to the literature, which is similar to our result.

• However, when cytoreductive surgery and hyperthermia intraoperative intraperitoneal chemotherapy is performed, five year survival rate can be improved to 52-96% by authors around the world.

Summary• Appendicular mass should be the top of the

differential diagnosis with RIF mass.• Appendicular cancer is a rare, usually an incidental

finding & should be suspected in any elderly person presenting with appendicitis like symptoms and signs.

• Non resolving appendicular mass should be explored.• All appendicectomy specimen should be sent for

HPE.

Leonid Rogozov

References• 1. Woodruff, R. and J. R. McDonald . Benign and malignant cystic tumors of the

appendix. Surg Gynecol Obstet 1940. 71:750–755. • 2. Gibbs, N. M. Mucinous cystadenoma and cystadenocarcinoma of the vermiform

appendix with particular reference to mucocele and pseudomyxoma peritonei. J Clin Pathol 1973. 26 (6):413–421. [CrossRef]

• 3. Higa, E. , J. Rosai , C. A. Pizzimbono , and L. Wise . Mucosal hyperplasia, mucinous cystadenoma, and mucinous cystadenocarcinoma of the appendix: a re-evaluation of appendiceal “mucocele.”. Cancer 1973. 32 (6):1525–1541. [CrossRef]

• Pickhardt PJ, Levy AD, Rohrmann CA et-al. Primary neoplasms of the appendix: radiologic spectrum of disease with pathologic correlation. Radiographics. 2003;23 (3): 645-62. Radiographics (full text) - doi:10.1148/rg.233025134 - Pubmed citation

• Bunch GH. Mucoid Disease of the Appendix. Ann Surg. 1945 May;121(5):704–709. [PMC free article] [PubMed]

• SCIMECA WB, DOCKERTY MB. Carcinoma of the vermiform appendix: a review of the literature and report of a case. Proc Staff Meet Mayo Clin. 1955 Nov 16;30(23):527–534. [PubMed]

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