| Abstract|| |
We report a 19-year-old female admitted to the Emergency Room with excruciating right lower abdominal pain of 1-day duration. The abdominal examination revealed a soft, lax abdomen with rigidity and guarding in her right iliac fossa without abdominal defense. Apart from a leukocytosis of 18.3/mm3, the rest of her baseline investigations and imaging, including abdominal X-rays, abdominal and pelvic ultrasound and abdominal CT scan were unremarkable. Patient's persistent pain prompted the treating surgeon to undertake exploratory laparotomy, which disclosed an inflamed Meckel's diverticulum and a normal-looking appendix. Meckel's diverticulectomy along with appendectomy was performed. The histopathological report demonstrated carcinoid tumor in the Meckel's diverticulum with free resection margins, whereas appendix was reported to be normal. The patient had an uneventful recovery and was discharged home on the sixth postoperative day.
Keywords: 5-hydroxyindole acetic acid, carcinoid syndrome, carcinoid tumors, Meckel′s diverticulum
|How to cite this article:|
Guraya SY, Khairi GA, Noureldin O, Ghallab A, Salman S. Carcinoid of the Meckel's diverticulum: Case report and review of literature. Saudi J Gastroenterol 2006;12:139-41
|How to cite this URL:|
Guraya SY, Khairi GA, Noureldin O, Ghallab A, Salman S. Carcinoid of the Meckel's diverticulum: Case report and review of literature. Saudi J Gastroenterol [serial online] 2006 [cited 2019 Jul 20];12:139-41. Available from: http://www.saudijgastro.com/text.asp?2006/12/3/139/29755
Carcinoid tumor is by far the most common gastrointestinal endocrine neoplasm, accounting for 55% of all endocrine tumors, 90% originating from the enterochromaffin or Kulchitsky's cells. Because of their neuroendocrine origin, carcinoid tumors are equipped with neurosecretory capability, which elaborates one of their defining clinical characteristics. The reported overall incidence of carcinoid tumors ranges from 1 to 2 / 100,000 per year. There is a slightly increased incidence in African Americans, with a female predominance in their reproductive years.,
By and large, gastrointestinal and, in particular, appendicular carcinoid tumors are incidentally discovered by the pathological examination of the specimen. We report a young female with a carcinoid tumor in the Meckel's diverticulum incidentally discovered by the histological review of the surgically removed specimen.
| Case report|| |
A 19-year-old female presented to the Emergency Room of King Khalid University Hospital, Riyadh, with sudden severe, colicky pain in the right lower abdomen. Pain was associated with repeated bouts of vomiting and fever. Patient's examination revealed temperature of 37.9°C, pulse 108/min, while her abdomen elicited tenderness and guarding in the right iliac fossa. No definite mass could be palpated in the abdomen, and bowel sounds were audible with normal frequency. White blood cell count amounted to 18.3/mm 3sub and abdominal X-rays (erect and supine) were unremarkable. An abdominal/pelvic ultrasound showed moderate amount of fluid in the pouch of Douglas, a finding further confirmed by CT scan of the same area. A few hours later as her symptomatology remained unchanged, the patient was subjected to exploratory laparotomy, and the operative findings demonstrated an apparently normal-looking appendix and a grossly inflamed Meckel's diverticulum sited about 26 cm from the ileocecal junction. Appendectomy and Meckel's diverticulectomy with primary anastomosis using GIA 50 stapler (US Surgical, Norwalk, Conn.) was undertaken. Postoperatively the patient required minimal analgesia, was advanced to regular diet on the fifth postoperative day and discharged home on the sixth day.
The histological examination reported carcinoid tumor in the Meckel's diverticulum, composed of neoplastic cells in the submucosa arranged in nesting pattern measuring about 1.2 cm [Figure - 1][Figure - 2]. There was no penetration of the muscular or serosal layer, and the resection margins were reported to be free of tumor. No pathological lesion was detected in the appendix. As her baseline (10 days post op) and follow-up (6 months post op) 5-hydroxyindoleacetic acid (5-HIAA) levels in the urine remained normal, no further treatment was considered.
A total body CT scan was performed, which excluded any associated tumorous lesion. The patient was planned to be regularly reviewed in the surgical clinic every 6 months for 3 years with urinary 5-HIAA assays.
| Discussion|| |
Historically, the term carcinoid was first coined by Oberndorfer in 1907. The prevalence of carcinoid tumors is expected to be around 0.5 per 100,000, although autopsy studies suggest that it may be as high as 2 per 100,000. In the great majority of cases, carcinoids remain silent but can present with a wide spectrum of symptoms due to their ability to secrete a variety of peptides and bioactive amines, mainly 5- hydroxytryptamine (5-HT), corticotrophin (ACTH), histamine and dopamine. Carcinoids occur in about 9% of patients with multiple endocrine neoplasia (MEN), predominantly MEN type I. These tumors are most frequently reported in the GIT (74%), followed by bronchopulmonary system (25%); the remaining 1% have been found in ovary, thymus, testis, spleen and breast. About 36% of all carcinoids are found in the appendix, 25% in the small bowel, 16% in the rectum, 6 percent in the colon and 3% in the duodenum. Lafrancesco et al. have documented the existence of carcinoid tumors in the Meckel's diverticulum.
A Meckel's diverticulum possesses all three coats of the intestinal wall and has its own blood supply. In 20% of the cases, the mucosa contains heterotopic epithelium - namely, gastric, colonic or sometimes, pancreatic tissue. A substantial number of carcinoid tumors remain clinically silent. Symptomatic carcinoids declare either through their mass effect (pain, luminal obstruction) or secretory products. Carcinoid syndrome consists of a constellation of symptoms which arise as a result of massive release of serotonin and neuropeptides directly into the systemic system. The classic symptoms include flushing, diarrhea, bronchospasm, valvular heart disease and pellagra. ACTH-producing carcinoids lead to Cushing's syndrome, and gastrin-secreting tumors may cause Zollinger-Ellison syndrome. Our patient complained of excruciating abdominal pain, which necessitated urgent surgical exploration. Urinary 5-HIAA measured in a 24-hour urine sample is the most frequently applied test in the endocrine work-up of the carcinoid tumors. However, it lacks sensitivity and specificity as it may also be elevated in tropical sprue, celiac disease, Whipple's disease, small bowel obstruction, as well as by the ingestion of 5-HIAA-rich foods. Chromogranin A, a glycoprotein secreted by the neuroendocrine cells, has 80% sensitivity to carcinoids and serves as a valuable marker in the early detection of recurrences and during follow-up after the primary treatment. Since 87% of carcinoids express somatostatin receptors, octreotide labeled with indium 111 is the most widely used radionuclide analog for somatostatin receptor imaging. Histologically, a striking feature of the carcinoid tumors is intense desmoplasia, which may lead to vascular occlusion secondary to anatomic distortion caused by the surrounding tissue reaction. Two types of silver stainings, argyrophilic and argentaffin, are commonly used to identify neuroendocrine cells. Classically, foregut and hindgut carcinoids are argyrophilic, and midgut serotonin-secreting tumors are argentaffin.
The surgical treatment of carcinoid tumors depends on the localization and the size of the lesion. Lymph node metastasis is most frequently reported in small bowel carcinoids (20-45%), providing the rationale for an extended resection including the regional lymph nodes. Carcinoids of the appendix that are ≤1 cm in diameter rarely metastasize, simply requiring appendectomy for treatment. A right hemicolectomy is recommended for tumors ≥2 cm, while for those lesions ranging 1-2 cm, the extent of surgery depends on other factors (positive lymph nodes, extension of the tumor to mesoappendix or subserosal lymphatics). Carcinoids of the colon and rectum measuring ≤2 cm require local excision, whereas those ≥2 cm are best treated with wide resection. The present case was surgically managed in accordance with the recommended treatment pathways.
To conclude, carcinoid tumors are extremely rare, and the diagnosis is often made after surgery. Carcinoids frustrate the physicians by the complex symptoms and varied biochemical affections and demand a high index of suspicion. Surgery is the treatment of choice for such lesions, and the patients should be followed up with serial urinary 5-HIAA assays and somatostatin receptor imaging, where available.
| References|| |
|1.||Deeg KH, Reisig A, Seitz G. Sonographic diagnosis of a carcinoid tumor of the appendix in a 14-year-old boy. Ultraschall Med 2003;24:120-2. |
|2.||D'Aleo C, Lazzareschi I, Ruggiero A, Riccardi R, Carcinoid tumors of the appendix in children: Two case reports and review of the literature. Pediatr Hematol Oncol 2001:18:347-51. |
|3.||Modlin IM, Sandor A. An analysis of 8305 cases of carcinoid tumors. Cancer 1997;79:813-29. [PUBMED] [FULLTEXT]|
|4.||Newton JN, Swerdlow AJ, Dos SS. The epidemiology of carcinoid tumors in England and Scotland. Br J Cancer 1994;70:939-42. |
|5.||Bartos M, Narebski JM, Kazka K, Pomorski L. Colorectal carcinoid own-own experience. Neoplasia 2000;47:409-12. |
|6.||Oberndorfer S. Karzinoide Tumoren des Dunndarms. Frankf Z Pathol 1907;1:425-9. |
|7.||Bax ND, Woods HF, Batchelor A, Jennings M. Clinical manifestations of carcinoid disease. World J Surg 1996;20:142-6. [PUBMED] [FULLTEXT]|
|8.||Janmohammed S, Bloom SR. Carcinoid tumors (review). Post grad Med J 1997;37:207-14. |
|9.||Timmins PF, Kuo DY, Anderson PS. Ovarian carcinoid: Management of primary and recurrent tumors. Gynecol Oncol 2000;76:112-4. |
|10.||Fukai I, Masaoka A, Fujii Y. Thymic neuroendocrine tumor (thymic carcinoid): A clinicopathological study in 15 patients. Am Thorac Surg 1999;67:208-11. |
|11.||Glazier DB, Murphy DP, Barnard M. Primary carcinoid tumor of the testis. BJU Int 1999;83:153-4. |
|12.||Ng JW, Liu KW, Mak KO. Carcinoid tumor of the spleen. Aust NZ J Surg 1999;69:70-2. [PUBMED] |
|13.||Jablon LK, Somers RG, Rim PY. Carcinoid tumor of the breast: Treatment with breast conservation in three patients. Ann Surg Oncol 1998;5:261-4. |
|14.||Vinik AI, McLeod MK, Fig LM, Shapiro B, LIoyd RV, Cho K. Clinical features, diagnosis and localization of carcinoid tumors and their management. Gastroenterol Clin North Am 1989;18:865-96. |
|15.||Iafrancesco D, Mazzoni G, Vagni V, Mazzuca F, Rocco R, Mazzarella Farao R. Carcinoid of Meckel's diverticulum: Presentation of a clinical case. Giornale di Chirurgia 2000;21:399-401. [PUBMED] |
|16.||Caplin ME, Buscombe JR, Hilson AJ. Carcinoid tumor (review). Lancet 1998;352:799-805. |
|17.||Pasieka JL, Mckinnon JG, Kinnear S, Yelle CA, Numerow L, Paterson A, et al . Carcinoid syndrome symposium on treatment modalities for gastrointestinal carcinoid tumors. Symposium summary. Can J Surg 2001;44:25-32. |
|18.||Roberts LJ, Anthony LB, Oates JA. Disorders of vasodilator hormones: Carcinoid syndrome and mastocytosis. In : Wilson JD, editor. Text book of endocrinology. WB Saunders: Toronto; 1998. p. 1711-20. |
|19.||Nobels FR, Kwekkeboom DJ, Coopmans W, Schoenmakers CH, Lindemans J, De Herder. Chromogranin A as serum marker for neuroendocrine neoplasia: Comparison with neuro-specific enolase and the alpha-subunit of glycoprotein hormones. J Clin Endocrinol Metab 1997;82:2622-8. |
|20.||Kisker O, Weinel RJ, Geks J, Zacara F, Joseph K, Rothmund M. Value of somatostatin receptor scintigraphy for pre operative localization of carcinoids. World J Surg 1996;20:162-7. |
|21.||Cotran RS, Kumar V, Robbins SL. Robbin's Pathologis Basis of Disease, 5th ed. WB Saunders: Philadelphia; 1994. p. 818-20. |
|22.||Mani S, Modlin IM, Ballantyne G. Carcinoids of the rectum (review). J Am Coll Surg 1994;179:231-48. |
|23.||Stinner B, Kisker O, Zielke A, Rothmund M. Surgical management for carcinoid tumors of small bowel, appendix, colon and rectum. World J Surg 1996;20:183-8. |
|24.||Rothmund M, Kisker O. Surgical treatment of carcinoid tumors of the bowel, appendix, colon and rectum. Digestive 1994;55;86-91. |
Salman Y Guraya
College of Medicine and King Khalid University Hospital, King Saud University, PO Box 286896, Riyadh 11323
Source of Support: None, Conflict of Interest: None
[Figure - 1], [Figure - 2]