Although usually harmless and asymptomatic, jejuno-ileal diverticulae are associated with various non-specific gastrointestinal symptoms, and rarely cause surgical emergencies. This case report describes the presentation and management of a patient with an acute abdomen, whose jejunal diverticulum was perforated. Unexpectedly, histopathological assessment demonstrated malignant melanoma lining the diverticulum. Whether this was primary or metastatic is discussed, together with a synopsis of the literature on small bowel diverticulae

Newton, RC

Nind, N

Penney, N

Sains, P

Sajid, MS

Gastroenterology Report

PubMed

Spiral - Imperial College Digital Repository

Gastroenterology Report (2014) 1–4, doi:10.1093/gastro/gou058Case reportSmall bowel malignant melanoma presentingas a perforated jejunal diverticulum: a casereport and literature reviewRichard C. Newton1,*, Nicholas Penney1, Nicholas Nind2, Muhammad S. Sajid1 andParvinder Sains11The Department of General Surgery, Worthing Hospital, Western Sussex Hospitals NHS Foundation Trust, Worthing, West Sussex, N11 2DH, UKand 2The Department of Histopathology, Worthing Hospital, Western Sussex Hospitals NHS Foundation Trust, Worthing, West Sussex, N11 2DH, UK*Corresponding author. The Department of General Surgery, Worthing Hospital, Western Sussex Hospitals NHS Foundation Trust, Worthing, WestSussex, N11 2DH, UK. Tel: +44-1903-205111; Fax: +44-1903-285010; Email: richardnewton22@yahoo.co.ukSubmitted 16 June 2014; Revised 21 July 2014; Accepted 26 July 2014Although usually harmless and asymptomatic, jejuno-ileal diverticulae are associated with various non-specific gastroin-testinal symptoms, and rarely cause surgical emergencies. This case report describes the presentation and management of apatient with an acute abdomen, whose jejunal diverticulum was perforated. Unexpectedly, histopathological assessmentdemonstrated malignant melanoma lining the diverticulum. Whether this was primary or metastatic is discussed, togetherwith a synopsis of the literature on small bowel diverticulae.Keywords: malignant melanoma; intestinal perforation; jejunal diverticulumINTRODUCTIONJejuno-ileal diverticulae (JID) are acquired outpouchings ofthe small bowel, emerging on the mesenteric border. Theydevelop probably as the result of intestinal dyskinesis andhigh segmental intraluminal pressures. They can be associ-ated with abdominal pain, diarrhoea, or unexplainedbleeding, but usually cause no harm. Occasionally theycause a surgical emergency through volvulus, obstruction,or, as in the case described below, through perforation. Theprecipitant for perforation in this case report was previ-ously undescribed: infiltration of malignant melanomainto the diverticulum.CASE PRESENTATIONA 64-year-old Caucasian female presented with a 48-hourhistory of rapid-onset severe epigastric and right upperquadrant pain after eating fish pie. The pain radiated tothe right shoulder, was exacerbated by movement, and wasassociated with rigors, a couple of episodes of vomiting andsome dyspnoea. After a chest infection a few months pre-viously, she had been left with a persistent, non-productivecough that had not responded to courses of antibiotics andsteroids. It had lowered her exercise tolerance; where sheworked as a manager of sheltered accommodation, she wasnow using the lift rather than the stairs. She had no otherpast medical or surgical history and had never smoked.On examination in the Accident and EmergencyDepartment her blood pressure was 170/85 mm Hg withSaO2 of 97% on room air, but her pulse was 140 beatsper minute with a respiratory rate of 38 breaths perminute. Her abdomen was distended, with marked tender-ness and guarding in the right upper quadrant, andMurphy’s sign was positive. Abdominal and erect chestradiographs showed a raised right hemidiaphragm(Figure 1a). Venous blood tests showed haemoglobin(Hb) 135 g/L, white blood count (WBC) 12.6 109/L, urea The Author(s) 2014. Published by Oxford University Press and the Digestive Science Publishing Co. Limited.This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted reuse, distribution, and reproduction in any medium, provided the original work is properlycited. Gastroenterology Report Advance Access published August 20, 2014 by guest on August 21, 2014http://gastro.oxfordjournals.org/Downloaded from 7.8 mmol/L, creatinine 122mmol/L, amylase 43 U/L, C-reac-tive protein (CRP) 332 mg/L, and liver function tests withinnormal range. Arterial blood gases on room air were inter-preted as a lactic acidaemia (lactate 4.9 mmol/L, base excess6.5 mmol/L, and HCO3 19.9 mmol/L) fully compensated bytachypnoea (pH 7.47, pO2 12.7 kPa, pCO2 3.12 kPa). Despiteresuscitation with intravenous fluid and antibiotics for aprovisional diagnosis of severe acute cholecystitis, she re-mained oliguric, and her signs of sepsis failed to improve.An urgent contrast enhanced abdominoperineal computedtomography (CT) scan showed extraluminal air and fluid,mild thickening and inflammation of the right colon, sev-eral small pockets of portal venous air emboli within theliver, and a large, small-bowel diverticulum within thepelvis containing solid material and air (Figure 1b and c).A midline laparotomy was performed. Diffuse smallbowel contents were causing a generalised peritonitiswith adhesions and a widespread fibrinous exudate. A per-forated 10 cm diameter jejunal diverticulum, 80 cm fromthe ligament of Treitz, was found within an abscess cavity(Figure 1d). The diverticulum was gradually freed from sev-eral adhesions to more distal small bowel, before resectionalong with a short section of damaged ileum. The continu-ity of the small bowel was restored by hand-sewn inter-rupted seromuscular end-to-end anastamoses using 3/0Vicryl. After copious warm saline wash-out, two large-bore surgical drains were inserted and the abdomen wasclosed with loop nylon. The patient was returned to inten-sive care but became and remained acidotic, with fast atrialfibrillation, renal failure, chest sepsis and inotrope depen-dency. Owing to rising inflammatory markers, a repeat CTscan was performed on Day 4, which did not show anyformed intra-abdominal collections or evidence of anasta-motic leak, but in the lungs there was diffuse, predomi-nantly dependent consolidation and ‘ground glass’opacification. Furthermore, there was a 10 cm multicysticnecrotic lesion in the upper segment of the right lowerlobe, contiguous with grossly enlarged necrotic paratra-cheal adenopathy (Figure 1e).Histopathological examination of the prepared surgicalspecimen showed an ulcerated necrotic epithelium liningthe jejunal diverticulum, with normal small bowel epithe-lium proximally and distally. Pleomorphic tumour cellshad infiltrated the wall of the diverticulum (Figure 1f).Figure 1. The initial chest X-ray showed a raised right hemidiaphragm but no free air (a). The first CT scan showed a fluid-filleddiverticulum continuous with the small bowel (b), along with extraluminal air and fluid, and portal venous air within the leftlobe of the liver (c). The mucosal lining of the perforated diverticulum was necrotic and had a brown hue (d). The second CT scanextended into the chest and showed consolidation and a necrotic cystic lesion in the right lung (e). Histopathological assessmentshowed full-thickness infiltration of tumour across the bowel wall (f), staining for Melan A (inset; high magnification).Page 2 of 4Richard C. Newton et al. by guest on August 21, 2014http://gastro.oxfordjournals.org/Downloaded from The tumour cells had vesicular nuclei and many containedprominent nucleoli. Some cells contained brown pigmentthat was negative on Perl’s staining for iron/haemosiderin,but strongly expressed the immunohistochemical mela-noma markers S100 and Melan A (Figure 1f, inset). Thissupported a diagnosis of malignant melanoma of primaryor metastatic nature within the jejunal diverticulum.The lung lesion was therefore also likely to be malignantmelanoma. Notably, there were no suspicious cutaneousor ocular pigmented lesions. The patient continued todeteriorate and, when the prospects of recovery becamenegligible, after discussion with the family, the patientwas palliated and passed away on Day 13.DISCUSSIONThe prevalence of JID on autopsy ranges between 0.3 and4.6%, depending on whether the small bowel is distendedwith gas; with small bowel contrast studies the prevalenceis 0.5–2.3% [1–3]. Small antimesenteric hernias may bemissed during surgery, due to becoming hidden withinthe small bowel mesentery. Whilst JIDs are asymptomaticin the majority of patients, they are sometimes linked with(i) vague abdominal symptoms and signs such as intermit-tent pains, diarrhoea, malabsorption and steattorhoea,(ii) flatulence from small bowel bacterial overgrowth,(iii) bleeding and anaemia (pre-operative localisation ofthe bleeding is often delayed, especially as half of patientswith JID have co-existing colonic diverticulae), (iv) megalo-blastic anaemia secondary to the intraluminal metabolismof B12 from bile acid deconjugation from coliform over-growth due to stasis, (v) obstruction (due to volvulus, enter-olith formation or adhesions owing to previous bouts ofjejuno-ileal diverticulutis), and (vi) perforation (this can belocalised or cause widespread peritonitis). Perforation ofa JID usually mandates a laparotomy and carries a riskof mortality of 20–40% [4]. Our patient’s initial risk ofmortality was probably greater, owing to signs of severesepsis and the poor prognostic radiological finding ofhepatic portal venous air [5]. Interestingly, analogous tocolonic diverticulitis, if the patient is well and the signsminimal, conservative management has been successfullyemployed [6].Small bowel diverticulae are diagnosed with abdominalradiographs (multiple air fluid levels), enteroclysis (out-pouching with retained contrast after the main lumen hasemptied), CT, CT enteroclysis, or at laparoscopy/laparotomy.The mean age at diagnosis is 70 [7]. Small bowel diverticu-lae were first described by Baillie and Soemmering in 1794[8], Sir Astley Cooper described specifically jejunal diverti-culae in 1807 [9], Sir William Osler reported the conditioncausing fatal malaena in 1881 [10] and, in 1906, Gordinierand Sampson described emergency surgical management[11]. Unlike Meckel’s, which are antimesenteric, congenitaland true diverticulae, most JID are believed to be acquiredpulsion lesions, and therefore false diverticulae, comprisinga thin wall lacking a muscle coat. They are usually found onthe mesenteric aspect where the vasa recta (paired bloodvessels) penetrate and thus weaken the bowel wall [12];small bowel diverticulae are both more common andmore likely to be multiple within the jejunum, where feed-ing vessels have the greatest diameter. Abnormal neuro-muscular contractility has been proposed as anaetiological factor [13], and steroids and non-steroidalanti-inflammatory drugs have also been implicated [14].There is an association between small bowel diverticulaeand colonic diverticulae, suggesting connective tissue ordietary factors.Primary malignancies of the small bowel are rare, andmore commonly found in the jejunum than the ileum,but primary adenocarcinomas are the most common, fol-lowed by carcinoids, lymphomas and stromal tumours [15].Small bowel melanoma is usually metastatic, arising fromthe skin more often than the eye or anus; indeed, althoughneural crest cells embryologically migrate into the gut,mature melanocytes are a rare entity in the small bowel,so there is controversy as to whether small bowel mela-noma can ever be considered primary, with some authorssuggesting that an occult or regressed primary is always theoriginal source (spontaneous regression of cutaneous mel-anomas can occur) [16]. Nevertheless, there are nearly30 case reports on PubMed claiming a diagnosis of pri-mary gastrointestinal malignant melanoma, but thefollow-up after resection is often short. Evidence thatwould support a diagnosis of primary melanoma includesthe pathological identification, in the surrounding tissue, ofmelanosis or a precursor lesion, a long disease-free survivalperiod after resection [17], and absence of extraintestinalmetastatic spread. The case described here matches none ofthese and, without the potential help of a post-mortem,it therefore more likely represents metastatic cutaneousmelanoma to bowel and lung. In a post-mortem studyof 216 patients with advanced melanoma, multiple organdeposits were present in 95% of patients, with the organsmost commonly involved being lymph nodes (74%), lungs(71%), liver (58%), brain (49%), bone (49%), heart (47%),adrenal glands (47%), and gastrointestinal tract (44%) [18].The normal clinical presentation of gastrointestinal mela-noma is similar to other malignancies: a change inbowel habit or obstructive symptoms, a palpable mass,rectal bleeding or anaemia and fatigue, and pain [19].Presentation via a perforated JID has not been previouslydescribed.Many different primary cancers have been infrequentlyreported in cases of Meckel’s diverticulae: adenocarci-nomas, stromal tumours, sarcomas, neuroendocrinetumours [20], and malignant melanoma have been de-tected [21]. It has even been controversially proposed thatPage 3 of 4Perforated melanomatous jejunal diverticulum by guest on August 21, 2014http://gastro.oxfordjournals.org/Downloaded from incidentally discovered Meckel’s should be resected, owingto their carrying a relatively high risk of cancer [22]. Jejunaldiverticulae have contained sarcomas [23], stromal tumours[24], and leiomyosarcomas [25], but this is the first reportedcase of one containing malignant melanoma.Conflict of interest: none declared.REFERENCES1. Noer T. Non-Meckelian diverticula of the small bowel. The inci-dence in an autopsy material. Acta Chir Scand 1960;120:175–9.2. Wilcox RD and Shatney CH. Surgical implications of jejunal diver-ticula. South Med J 1988;81:1386–91.3. Miller RE, McCabe RE, Salomon PF et al. Surgical complications ofsmall bowel diverticula exclusive of Meckel’s. Ann Surg 1970;171:202–10.4. Roses DF, Gouge TH, Scher KS et al. Perforated diverticula of thejejunum and ileum. Am J Surg 1976;132:649–52.5. Kinoshita H, Shinozaki M, Tanimura H et al. Clinical features andmanagement of hepatic portal venous gas: Four case reports andcumulative review of the literature. Arch Surg 2001;136:1410–4.6. Longo WE and Vernava AM 3rd. Clinical implications of Jejuno-ilealdiverticular disease. Dis Colon Rectum 1992;35:381–8.7. de Bree E, Grammatikakis J, Christodoulakis M et al. The clinicalsignificance of acquired Jejuno-ileal diverticula. Am J Gastroenterol1998;93:2523–8.8. Baillie M and Soemmerring ST. Anatomie des krankhaften Bauesvon einigen der wichtigsten Theile im menschlichen Körper.Berlin: In der Vossischen Buchhandlung, 1794.9. Cooper A. The anatomy and surgical treatment of crural andumbilical hernia. Longman 1807.10. Osler W. Notes on intestinal diverticula. Ann Anat Surg 1881;4:202–7.11. Gordinier HC and Sampson JA. Diverticulitis (not Meckel’s) causingintestinal obstruction: multiple mesenteric (acquired) diverticula ofthe small intestine. JAMA 1906;46:1585–90.12. Mendonca HL, Vieta JO and Ling WS. Jejunal diverticulosis withmassive hemorrhage. Am J Gastroenterol 1978;70:657–9.13. Krishnamurthy S, Kelly MM, Rohrmann CA et al. Jejunal diverticu-losis. A heterogenous disorder caused by a variety of abnormalitiesof smooth muscle or myenteric plexus. Gastroenterology 1983;85:538–47.14. Palanivelu C, Rangarajan M, Rajapandian S et al. Perforationof jejunal diverticula in steroids and nonsteroidal anti-inflammatory drug abusers: a case series. World J Surg 2008;32:1420–5.15. Chang HK, Yu E, Kim J et al. Adenocarcinoma of the small intes-tine: a multi-institutional study of 197 surgically resected cases.Hum Pathol 2010;41:1087–96.16. Bramhall RJ, Mahady K and Peach AH. Spontaneous regressionof metastatic melanoma - clinical evidence of the abscopal effect.Eur J Surg Oncol 2014;40:34–41.17. Poggi SH, Madison JF, Hwu WJ et al. Colonic melanoma, primaryor regressed primary. J Clin Gastroenterol 2000;30:441–4.18. Patel JK, Didolkar MS, Pickren JW et al. Metastatic pattern ofmalignant melanoma: A study of 216 autopsy cases. Am J Surg1978;135:807–10.19. Liang KV, Sanderson SO, Nowakowski GS et al. Metastatic malig-nant melanoma of the gastrointestinal tract. Mayo Clin Proc 2006;81:511–6.20. Poncet G, Hervieu V, Walter T et al. Neuroendocrine tumors ofMeckel’s diverticulum: lessons from a single institution study ofeight cases. J Gastrointest Surg 2011;15:101–9.21. Bloch T, Tejada E and Brodhecker C. Malignant melanoma inMeckel’s diverticulum. Am J Clin Pathol 1986;86:231–4.22. Thirunavukarasu P, Sathaiah M, Sukumar S et al. Meckel’s divertic-ulum–a high-risk region for malignancy in the ileum. Insightsfrom a population-based epidemiological study and implicationsin surgical management. Ann Surg 2011;253:223–30.23. Antes G, Bassermann R and Lessel W. Sarcomas originating inthe small bowel diverticula. Abdom Imaging 1994;19:339–41.24. Schepers S and Vanwyck R. Small bowel gastrointestinalstromaltumor (GIST) arising in a jejunal diverticulum. JBR-BTR2009;92:23–4.25. Fiori E, Ansali A, Galati G et al. Leiomyosarcoma in jejunaldiverticulum. A case report and review of the literature. G Chir1996;17:269–75.Page 4 of 4Richard C. Newton et al. by guest on August 21, 2014http://gastro.oxfordjournals.org/Downloaded from 

Small bowel malignant melanoma presenting as a perforated jejunal diverticulum: a case report and literature review.

Richard C. Newton

Nicholas Penney

Nicholas Nind

Muhammad S. Sajid

Parvinder Sains

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Small bowel malignant melanoma presenting as a perforated jejunal diverticulum: a case report and literature review

http://spiral.imperial.ac.uk/bitstream/10044/1/27201/2/Jejunal%20Melanoma%20Case%20Report.pdf

Small bowel malignant melanoma presenting as a perforated jejunal diverticulum: a case report and literature review.

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