A small intestine neuroendocrine tumor: case report: Case Report

Khalid JAMALEDDINE; Amal HAJRI; Anas EL HAYAL; Driss ERGUIBI; Rachid BOUFETTAL; Saad RIFKI JAI; Farid CHEHAB

doi:10.58372/2835-6276.1112

Authors

Khalid JAMALEDDINE Department of general surgery, Ibn ROCHD University hospital of Casablanca, Casablanca
Amal HAJRI Department of general surgery, IBN ROCHD University hospital of Casablanca, Casablanca, Morocco
Anas EL HAYAL Department of general surgery, IBN ROCHD University hospital of Casablanca, Casablanca, Morocco
Driss ERGUIBI Department of general surgery, IBN ROCHD University hospital of Casablanca, Casablanca, Morocco
Rachid BOUFETTAL Department of general surgery, IBN ROCHD University hospital of Casablanca, Casablanca, Morocco
Saad RIFKI JAI Department of general surgery, IBN ROCHD University hospital of Casablanca, Casablanca, Morocco
Farid CHEHAB Department of general surgery, IBN ROCHD University hospital of Casablanca, Casablanca, Morocco

DOI:

https://doi.org/10.58372/2835-6276.1112

Keywords:

Neuroendocrine tumors, Small bowel neoplasms, Small intestinal tract surgery, Metastatic neuroendocrine tumors, Jejuno-ileal tumors

Abstract

Introduction

Small intestinal neuroendocrine tumors (SI-NETs) are the most common small bowel tumors, they are recognized for their ability to produce hormones causing mesenteric fibrosis and carcinoid syndrome. They tend to have better surgical rate than adenocarcinomas.this work was made as an update on one of the most aggressive small intestine tumors.

Presentation of case

We discuss here the case of a sixth years old female woman admitted and operated for a small intestine neuroendocrine tumor with peritoneal carcinosis

Discussion

Neuroendocrine tumors are secreting tumors, locating mainly in the small intestine with a high malignancy risk. At the time of the diagnosis, lymph node, liver metastasis and peritoneal carcinosis are very common. Patients may be asymptomatic or consult for abdominal pain, rarely bowel obstruction, diagnosis is radiologically made and confirmed indeed histologically. Treatment involves surgery, chemotherapy, immunotherapy and radiotherapy and surveillance is assured by biomarkers, endoscopy and Octreoscan when metastatic.

Conclusion

The SI-NETs are rare but their incidence and prevalence have been increasing. Histologically, diagnosis is confirmed by positive immunohistochemical staining. Treatment and prognosis depend on the grade and stage of the tumor. Immunotherapy will serve as a future treatment modality.

References

Yao JC, Hassan M, Phan A et al (2008) One hundred years after ‘‘carcinoid’’: epidemiology of and prognostic factors for neuroendocrine tumors in 35,825 cases in the United States. J Clin Oncol 26:3063–3072

Mitry E. Tumeurs neuroendocrines digestives. EMC - Gastro-entérologie 2013;8(4):1-13 [Article 9-089-C-20].

Zar N, Garmo H, Holmberg L et al (2004) Long-term survival of patients with small intestinal carcinoid tumors. World J Surg 28:1163–1168

Makridis C, Oberg K, Juhlin C et al (1990) Surgical treatment of mid-gut carcinoid tumors. World J Surg 14:377–383 discussion 384–375

Modlin IM, Kidd M, Latich I, Zikusoka MN, Shapiro MD. Current status of gastrointestinal carcinoids. Gastroenterology 2005; 128: 1717-1751 [PMID: 15887161 DOI: 10.1053/j.gastro.2005.03.038]

Maggard MA, O'Connell JB, Ko CY. Updated population-based review of carcinoid tumors. Ann Surg 2004; 240: 117-122 [PMID: 15213627 DOI: 10.1097/01.sla.0000129342.67174.67]

Taal BG, Visser O. Epidemiology of neuroendocrine tumours. Neuroendocrinology 2004; 80 Suppl 1: 3-7 [PMID: 15477707 DOI: 10.1159/000080731] 8. Cancer. Net. View All Pages Neuroendocrine Tumors-Introduction. 2019 Apr. Available from: https://www.cancer.net/cancer-types/neuroendocrine-tumor/view-all

Wang R, Zheng-Pywell R, Chen HA, Bibb JA, Chen H, Rose JB. Management of Gastrointestinal Neuroendocrine Tumors. Clin Med Insights Endocrinol Diabetes 2019; 12: 1179551419884058 [PMID: 31695546 DOI: 10.1177/1179551419884058]

Yao JC, Hassan M, Phan A, Dagohoy C, Leary C, Mares JE, Abdalla EK, Fleming JB, Vauthey JN, Rashid A, Evans DB. One hundred years after "carcinoid": epidemiology of and prognostic factors for neuroendocrine tumors in 35,825 cases in the United States. J Clin Oncol 2008; 26: 3063-3072 [PMID: 18565894 DOI: 10.1200/JCO.2007.15.4377]

Moertel CG, Sauer WG, Dockerty MB, Baggenstoss AH. Life history of the carcinoid tumor of the small intestine. Cancer 1961; 14: 901-912 [PMID: 13771655 DOI: 10.1002/1097-0142(196109/10)14:5<901::aid-cncr2820140502>3.0.co;2-q]

Howe JR, Cardona K, Fraker DL, Kebebew E, Untch BR, Wang YZ, Law CH, Liu EH, Kim MK, Menda Y, Morse BG, Bergsland EK, Strosberg JR, Nakakura EK, Pommier RF. The Surgical Management of Small Bowel Neuroendocrine Tumors: Consensus Guidelines of the North American Neuroendocrine Tumor Society. Pancreas 2017; 46: 715-731 [PMID: 28609357 DOI: 10.1097/MPA.0000000000000846]

Klimstra DS, Modlin IR, Coppola D, Lloyd RV, Suster S. The pathologic classification of neuroendocrine tumors: a review of nomenclature, grading, and staging systems. Pancreas 2010; 39: 707-712 [PMID:20664470 DOI: 10.1097/MPA.0b013e3181ec124e]

Nagai T, Torishima R, Nakashima H, Tanahashi J, Iwata M, Ookawara H, Yokoyama S, Yada K, Sato R, Murakami K, Fujioka T. Duodenal gangliocytic paraganglioma treated with endoscopic hemostasis and resection. J Gastroenterol 2004; 39: 277-283 [PMID: 15065006 DOI: 10.1007/s00535-003-1289-2]

Burke AP, Thomas RM, Elsayed AM, Sobin LH. Carcinoids of the jejunum and ileum: an immunohistochemical and clinicopathologic study of 167 cases. Cancer 1997; 79: 1086-1093 [PMID: 9070484 DOI: 10.1002(sici)1097-0142(19970315)79:6<1086::aid-cncr5>.0.co;2-e]

Klöppel G, Perren A, Heitz PU. The gastroenteropancreatic neuroendocrine cell system and its tumors: the WHO classification. Ann N Y Acad Sci 2004; 1014: 13-27 [PMID: 15153416 DOI: 10.1196/annals.1294.002]

Pasieka JL. Carcinoid tumors. Surg Clin North Am 2009; 89: 1123-1137 [PMID: 19836488 DOI: 10.1016/j.suc.2009.06.008]

Druce MR, Bharwani N, Akker SA, Drake WM, Rockall A, Grossman AB. Intra-abdominal fibrosis in a recent cohort of patients with neuroendocrine ('carcinoid') tumours of the small bowel. QJM 2010; 103: 177-185 [PMID: 20123681 DOI: 10.1093/qjmed/hcp191]

Daskalakis K, Karakatsanis A, Stålberg P, Norlén O, Hellman P. Clinical signs of fibrosis in small intestinal neuroendocrine tumours. Br J Surg 2017; 104: 69-75 [PMID: 27861745 DOI: 10.1002/bjs.10333]

Bösch F, Bruewer K, D'Anastasi M, Ilhan H, Knoesel T, Pratschke S, Thomas M, Rentsch M, Guba M, Werner J, Angele MK. Neuroendocrine tumors of the small intestine causing a desmoplastic reaction of the mesentery are a more aggressive cohort. Surgery 2018; 164: 1093-1099 [PMID: 30076029 DOI: 10.1016/j.surg.2018.06.026]

Ramirez RA, Beyer DT, Chauhan A, Boudreaux JP, Wang YZ, Woltering EA. The Role of Capecitabine/Temozolomide in Metastatic Neuroendocrine Tumors. Oncologist 2016; 21: 671-675 [PMID: 27226359 DOI: 10.1634/theoncologist.2015-0470]

Ilett EE, Langer SW, Olsen IH, Federspiel B, Kjær A, Knigge U. Neuroendocrine Carcinomas of the Gastroenteropancreatic System: A Comprehensive Review. Diagnostics (Basel) 2015; 5: 119-176 [PMID: 26854147 DOI: 10.3390/diagnostics5020119]

von der Ohe MR, Camilleri M, Kvols LK, Thomforde GM. Motor dysfunction of the small bowel and colon in patients with the carcinoid syndrome and diarrhea. N Engl J Med 1993; 329: 1073-1078 [PMID: 8371728 DOI: 10.1056/NEJM199310073291503] 24. Pellikka PA, Tajik AJ, Khandheria BK, Seward JB, Callahan JA, Pitot HC, Kvols LK. Carcinoid heart disease. Clinical and echocardiographic spectrum in 74 patients. Circulation 1993; 87: 1188-1196 [PMID: 7681733 DOI: 10.1161/01.cir.87.4.1188]

Ruszniewski P, Ducreux M, Chayvialle JA, Blumberg J, Cloarec D, Michel H, Raymond JM, Dupas JL, Gouerou H, Jian R, Genestin E, Bernades P, Rougier P. Treatment of the carcinoid syndrome with the longacting somatostatin analogue lanreotide: a prospective study in 39 patients. Gut 1996; 39: 279-283 [PMID: 8977344 DOI: 10.1136/gut.39.2.279]

Kvols LK, Moertel CG, O'Connell MJ, Schutt AJ, Rubin J, Hahn RG. Treatment of the malignant carcinoid syndrome. Evaluation of a long-acting somatostatin analogue. N Engl J Med 1986; 315: 663-666 [PMID: 2427948 DOI: 10.1056/NEJM198609113151102]

Givi B, Pommier SJ, Thompson AK, Diggs BS, Pommier RF. Operative resection of primary carcinoid neoplasms in patients with liver metastases yields significantly better survival. Surgery 2006; 140: 891-7; discussion 897-8 [PMID: 17188135 DOI: 10.1016/j.surg.2006.07.033]

Gupta S, Yao JC, Ahrar K, Wallace MJ, Morello FA, Madoff DC, Murthy R, Hicks ME, Ajani JA. Hepatic artery embolization and chemoembolization for treatment of patients with metastatic carcinoid tumors: the M.D. Anderson experience. Cancer J 2003; 9: 261-267 [PMID: 12967136 DOI: 10.1097/00130404-200307000-00008] 29. Kulke MH, Hörsch D, Caplin ME, Anthony LB, Bergsland E, Öberg K, Welin S, Warner RR, LombardBohas C, Kunz PL, Grande E, Valle JW, Fleming D, Lapuerta P, Banks P, Jackson S, Zambrowicz B, Sands AT, Pavel M. Telotristat Ethyl, a Tryptophan Hydroxylase Inhibitor for the Treatment of Carcinoid Syndrome. J Clin Oncol 2017; 35: 14-23 [PMID: 27918724 DOI: 10.1200/JCO.2016.69.2780]

Oberg K, Funa K, Alm G. Effects of leukocyte interferon on clinical symptoms and hormone levels in patients with mid-gut carcinoid tumors and carcinoid syndrome. N Engl J Med 1983; 309: 129-133 [PMID: 6191217 DOI: 10.1056/NEJM198307213090301]

Frank M, Klose KJ, Wied M, Ishaque N, Schade-Brittinger C, Arnold R. Combination therapy with octreotide and alpha-interferon: effect on tumor growth in metastatic endocrine gastroenteropancreatic tumors. Am J Gastroenterol 1999; 94: 1381-1387 [PMID: 10235222 DOI: 10.1111/j.1572-0241.1999.01090.x]

Bainbridge HE, Larbi E, Middleton G. Symptomatic Control of Neuroendocrine Tumours with Everolimus. Horm Cancer 2015; 6: 254-259 [PMID: 26245686 DOI: 10.1007/s12672-015-0233-2]

Bushnell DL Jr, O'Dorisio TM, O'Dorisio MS, Menda Y, Hicks RJ, Van Cutsem E, Baulieu JL, BorsonChazot F, Anthony L, Benson AB, Oberg K, Grossman AB, Connolly M, Bouterfa H, Li Y, Kacena KA, LaFrance N, Pauwels SA. 90Y-edotreotide for metastatic carcinoid refractory to octreotide. J Clin Oncol 2010; 28: 1652-1659 [PMID: 20194865 DOI: 10.1200/JCO.2009.22.8585]

Vasseur B, Cadiot G, Zins M, Flejou JF, Belghiti J, Marmuse JP, et al. Peritoneal carcinomatosis in patients with digestive endocrine tumors. Cancer 1996;78:1686-92.

Makridis C, Rastad J, Oberg K, Akerstrom G. Progression of metastases and symptom improvement from laparotomy in midgut carcinoid tumors. World J Surg 1996;20:900-6; discussion 7.

Kianmanesh R, Ruszniewski P, Rindi G, Kwekkeboom D, Pape UF, Kulke M, et al. ENETS consensus guidelines for the management of peritoneal carcinomatosis from neuroendocrine tumors. Neuroendocrinology 2010;91: 333-40.

Das S, Shi C, Koyama T, Huang Y, Gonzalez R, Idrees K, et al. (2019) Peritoneal Carcinomatosis in Well-Differentiated Small-Intestinal Neuroendocrine Tumors with Mesenteric Tumor Deposits. J Med Surg Pathol. 4:166. doi: 10.35248/2472-4971.19.4.166