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Synchronous small intestinal and appendiceal neuroendocrine tumours: a rare case Ms BP is a 36-year-old woman who presented to the emergency department with a 4-day history of worsening lower abdominal pain on a background of anxiety and polycystic ovarian syndrome. On examination, she was haemodynamically stable, apyrexial and tender in both iliac fossae. Her C-reactive protein was 142 mg/L and white cell count was 7.93 × 109/L. A computed tomography (CT) scan of the abdomen and pelvis demonstrated a fluid collection in the right adnexa containing a small, central cystic structure. The appendix could not be traced. No other abnormalities were reported. At laparoscopy, there was mucopurulent free fluid in the pelvis, fibrinopurulent appendicitis and evidence of inflammation of the right adnexa. An appendicectomy was performed. Histopathology was consistent with an 11-mm World Health Organization (WHO) grade 1 well-differentiated neuroendocrine tumour (NET) at the appendiceal tip without lymphatic or perineural invasion. Review of this pathology at a multidisciplinary meeting demonstrated tumour cells at the serosal surface of the appendix.

Serum chromogranin A and urinary 5-hydroxyindoleacetic acid (5-HIAA) levels were normal. A Ga-68 Dotatate positron emission tomography (PET) scan showed two foci of moderate to intense uptake in the left mid abdomen at transaxial level L2/L3. One focus anterolateral to the left psoas muscle was reported as being located in the small bowel or an adjacent lymph node. The second focus appeared to be contained within small bowel loops. Capsule endoscopy demonstrated at least two tumours in the jejunum consistent with suspected NETs (Fig. 1). Given the size of these lesions and an inability to appreciate their presence on the CT component of the PET, it was decided to proceed with antegrage enteroscopy rather than CT or magnetic resonance imaging (MRI) enterography. These lesions were not demonstrated at enteroscopy, with the endoscopic limit to the proximal jejunum. This was marked with spot tattoo to assist with focusing intraoperative efforts to localize the lesions. Ms BP underwent a diagnostic laparoscopy. Intraoperatively, she was found to have multiple ileal and jejunal lesions with associated desmoplasia of the small bowel mesentery (Fig. 2). The residual mesoappendix was fibrotic and indurated. It was decided to proceed with a right hemicolectomy and resection of two small bowel segments. Histopathology was consistent with three WHO grade 2 (4–8 mm) and eight WHO grade 1 (1.2–5 mm) well-differentiated NETs (Fig. 3a). There was no evidence of residual or metastatic malignancy in the right hemicolectomy specimen or small bowel

Fig. 1. Capsule endoscopy: demonstrates one of the jejunal lesions. Differentiating these from prominent mucosal folds can be a challenge. The limited resolution that can be achieved demonstrates the difficulty in making further management decisions in these complex cases.

This case was presented at the St George Public Hospital surgical grand rounds for the month of September 2014.

© 2015 Royal Australasian College of Surgeons

Fig. 2. Macroscopic analysis of the resected surgical specimen. Demonstrates the classic finding of multicentric lesions in a small bowel segment, with associated desmoplasia of the small bowel mesentery.

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Images for surgeons

Fig. 3. Microscopic analysis of the resected surgical specimen (a) showing an intramucosal and submucosal portion of the largest well-differentiated neuroendocrine tumour of the ileum and (b) strong positive staining for Chromogranin A.

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lymph nodes. Post-operatively, Ms BP developed an ileus, which was managed conservatively. She was discharged home on day 11. NETs represent a rare and complex group of neoplastic lesions that arise from neuroendocrine cells of the gastrointestinal tract and bronchopulmonary system. Australian cancer registry data suggest an annual incidence of 3.3 cases per 100 000.1 In addition to this, rates of malignant non-skin NETs in theAustralian population have been steadily increasing at an average of 0.52% per year over the past three decades. This rising incidence is thought to be secondary to increasingly sensitive diagnostic tools and increased awareness amongst physicians.2 The majority of ileal and jejunal NETs are well-differentiated and have an indolent course. Patients will often present with vague abdominal pain, cramping, bloating and episodic diarrhoea. Nonspecific symptoms lead to frequent delays in diagnosis and advanced disease.3 Locally advanced small intestine-NETs (SI-NETs) tend to invade the mesenteric root, with potential complications of intestinal obstruction and ischaemia.4 Although it is well recognized that 10–20% of carcinoid tumours are associated with a second histologically disparate primary malignant neoplasm, synchronous carcinoids in two different organs are extremely rare.5 There are very few reported cases of synchronous small intestinal and appendiceal NETs. The standard imaging procedures for diagnosing NETs include ultrasound, contrast-enhanced helical CT or MRI in association with somatostatin receptor scintigraphy (SRS) and endoscopy; SRS with single photon emission computed tomography (sensitivity 80–90%) remains the standard for staging. Recently, some centres are using PET with Ga-68-labelled somatostatin analogues as an alternative for diagnosis.6 Japanese researchers have confirmed the efficacy of Ga-68 DOTATE for diagnosing small bowel NETs in a meta-analysis of 416 patients. The pooled sensitivity and specificity of Ga-68 DOTATATE PET was 96 and 100%, respectively.7 PET with Ga-68 has been found to be superior for the detection of small primary tumours and lymph node metastases compared with SRS or CT/MRI.6 Surgery remains the only potentially curative therapy for patients with well-differentiated NETS of the midgut. Curative resection of the primary lesion with wide lymphadenectomy has been shown to improve survival outcomes in patients with localized disease.6 Currently there is insufficient evidence to support a role for neoadjuvant or adjuvant treatment after complete resection of loco-regional disease.3 Due to the uncommon nature of SI-NETs there is limited data regarding recurrent disease following curative resection of local or locoregional disease.8 The ENETS guidelines recommend 6–12 monthly examination, serum chromogranin A, urinary 5-HIAA and triple-phase CT following curative resection. Because of limited high-level evidence

regarding median time to recurrence, guidelines recommend lifelong follow-up.6 Specific issues highlighted by the Australian Neuroendocrine Tumours consensus workshop report include the need for extended surveillance in patients with multiple endocrine neoplasia-1 and 2, and heightened vigilance for the second non-neuroendocrine malignancy that one in four patients will develop.1

Acknowledgements Thanks to Dr Robyn Levingtson of Douglas Hanley Mior, for providing descriptions to accompany images of the histopathology specimens. Thanks also to Professor Philip Craig for proving images of the capsule endoscopy.

References 1. Australian Neuroendocrine Tumours (NETs) Consensus Workshop Meeting: Management Challenges in Australia. [Cited 1 Sep 2014.] Available from URL: https://www.cosa.org.au/media/1060/cosa_report_ nets-consensus-workshop_2008.pdf 2. Pinchot SN, Holen K, Sippel RS, Chen H. Carcinoid tumors. Oncologist 2008; 13: 1255–69. 3. Boudreaux JP, Klimstra DS, Hassan MM et al. The NANETS consensus guideline for the diagnosis and management of neuroendocrine tumours: well-differentiated neuroendocrine tumours of the jejunum, ileum, appendix and caecum. Pancreas 2010; 39: 753–66. 4. Norlen O, Stalberg P, Oberg K et al. Long-term results of surgery for small intestinal neuroendocrine tumours at a tertiary referral centre. World J. Surg. 2012; 36: 1419–31. 5. Sembawa H, Lamoureux E, Gologan A, Gotlieb W, Gordon PH. Multiple carcinoids of the intestine. Cancer Ther. 2007; 5: 471–6. 6. Pape UF, Perren A, Niederle B et al. ENETS consensus guidelines for the management of patients with neuroendocrine neoplasms from the jejuno-ileum and the appendix including goblet cell carcinomas. Neuroendocrinology 2012; 95: 135–56. 7. Yang J, Kan Y, Ge BH, Yuan L, Li C, Zhao W. Diagnostic role of Gallium-68 DOTATOC and Gallium-68 DOTATATE PET in patients with neuroendocrine tumors: a meta-analysis. Acta Radiol. 2014; 55: 389–98. 8. Srirajaskanthan R, Ahmed A, Prachialias A, Srinivasan P. ENETS TNM staging predicts prognosis in small bowel neuroendocrine tumours. Int. Sch. Res. Not. Oncol. 2013; 13: 1–7.

Rebecca Lendzion,* BBiomedSc, MBBS Kim-Chi Phan-Thien,† BSc (Med), MBBS (Hons), MS, FRACS *Hurstville Private Hospital, Sydney, New South Wales, Australia and †St George Public Hospital, Sydney, New South Wales, Australia doi: 10.1111/ans.13053

© 2015 Royal Australasian College of Surgeons

Synchronous small intestinal and appendiceal neuroendocrine tumours: a rare case.

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