|Year : 2015 | Volume
| Issue : 2 | Page : 113-116
Gastrointestinal Carcinoids: Three contrasting presentations as surgical emergencies
S Yerrapragada, K Perumalla, S Daga, K Amirineni
Department of General Surgery, Kamineni Academy of Medical Sciences and Research Center, Hyderabad, Telangana, India
|Date of Web Publication||16-Jun-2015|
Dr. S Yerrapragada
Kamineni Academy of Medical Sciences and Research Center, LB Nagar, Hyderabad - 500 068, Telangana
Source of Support: None, Conflict of Interest: None
Carcinoids are neuroendocrine tumors and can be present anywhere in the gastrointestinal tract. These malignant tumors have relatively good prognosis after surgical resection when compared to other tumors of the gastrointestinal tract. Patients usually present with vague abdominal pain. Other rare presentations include intestinal obstruction, upper gastrointestinal bleed as hematemesis or malena, intestinal perforation, and carcinoid syndrome. Incidental finding on histopathology is another form of clinical presentation. We report three cases of carcinoid tumors of gastrointestinal tract which presented as different surgical emergencies namely, hematemesis, intra-abdominal abscess, and intestinal obstruction. Their clinical presentation, investigations, and treatment are hereby presented. Prompt evaluation and surgical intervention resulted in a good outcome.
Keywords: Carcinoid tumor, carcinoid syndrome, emergency presentation, intra-abdominal abscess, intestinal obstruction, surgical management
|How to cite this article:|
Yerrapragada S, Perumalla K, Daga S, Amirineni K. Gastrointestinal Carcinoids: Three contrasting presentations as surgical emergencies. Arch Int Surg 2015;5:113-6
|How to cite this URL:|
Yerrapragada S, Perumalla K, Daga S, Amirineni K. Gastrointestinal Carcinoids: Three contrasting presentations as surgical emergencies. Arch Int Surg [serial online] 2015 [cited 2020 Nov 30];5:113-6. Available from: https://www.archintsurg.org/text.asp?2015/5/2/113/158827
| Introduction|| |
Carcinoids are neuroendocrine tumors derived from enterochromaffin cells. They can arise from anywhere in the gastrointestinal tract, commonly in appendix, ileum, and rectum in descending order of frequency; but can also be seen in other organs. ,,
Patients with metastatic disease may live for years with indolent disease, but this same indolent nature makes these tumors difficult to diagnose at an early stage, when they are more likely to be cured by surgical resection.  Carcinoid tumors of the small bowel, and especially the ileum have been difficult to diagnose. They usually present as abdominal pain due to luminal narrowing or mesenteric fibrosis. Rarely these tumors present as an emergency with complications like obstruction, bleeding, or perforation. We present three patients with carcinoid tumors who presented with abdominal emergencies and were promptly evaluated and treated with a successful outcome.
| Case Report|| |
A 40-year-old female presented with severe hematemesis and pallor. She gave a history of recurrent episodes of bilious vomiting and melena of 6 months and hematemesis of 2 months' duration. She had palpitations and easy fatigability. Her abdominal examination did not reveal any abnormality. Her hemoglobin level at presentation was 5 g/dL. She was initially resuscitated with blood transfusions and was further evaluated with upper gastrointestinal endoscopy and abdominal computed tomography (CT) which showed a 3 × 4 cm mass in the distal body of stomach with stigmata of recent hemorrhage [Figure 1] and [Figure 2]. A diagnosis of gastric tumor was made. At laparotomy, a 3 × 4 cm tumor was found in the distal body of stomach for which a distal subtotal gastrectomy was performed [Figure 3] and [Figure 4]. There was no involvement of lymph nodes or other viscera. Postoperatively patient recovered well and is doing well on follow-up after 1 year. Histopathology of the tumor showed well-differentiated neuroendocrine tumor (carcinoid).
|Figure 1: CT of the stomach with oral and IV contrast showing tumor in the lesser curvature of the stomach|
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|Figure 2: Endoscopy of the stomach showing the tumor with stigmata of recent haemorrhage (case 1)|
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An 80-year-old male presented with complaints of pain in the right iliac fossa for 1 week. This was associated with fever and a mass in the right iliac fossa for 3 days. The mass was progressively increasing in size. Clinically, there was diffuse fullness and tenderness in the lower abdomen. He was evaluated with X-ray and contrast CT of the abdomen which showed a 15 × 15 cm collection, suggestive of an abscess in the right lumbar, right iliac regions, and pelvis [Figure 5]. After obtaining an informed consent, a laparotomy was done which revealed a large abscess cavity in the pelvis with 500 ml of pus. There was a perforation in mid ileum with a 3 × 3 cm inflammatory mass, adherent to the abscess cavity. The pus was drained and an ileal resection and anastomosis done. Histopathology report showed presence of carcinoid tumor in the ileal specimen. On follow-up after 6 months, the patient was doing well.
|Figure 5: CT of the abdomen showing abscess in the pelvis with bowel adhered to the abscess cavity (case 2)|
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A 50-year-old male presented with abdominal pain in right lower abdomen of 5 months' duration. He was not passing stools for 5 days and was having fever for 3 days. Clinical examination and abdominal radiograph were suggestive of acute small bowel obstruction. A contrast CT of the abdomen showed a 3 × 3 cm mass lesion in the distal ileum with obstruction [Figure 6]. A laparotomy was done which showed a 3 × 3 cm obstructing mass lesion in the terminal ileum with enlarged lymph nodes mass in the ileal mesentry [Figure 7] and [Figure 8]. Right hemicolectomy with ileocolic anastomosis was done. Histopathology showed carcinoid tumor. The patient was discharged without any complications and was doing well on follow-up at 3 months.
|Figure 6: CT of the abdomen showing growth and narrowing in the distal ileum with bowel obstruction (case 3)|
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|Figure 7: Intra operative image showing narrowing and stricture in the distal ileum (case 3)|
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|Figure 8: Mesenteric fibrosis and enlarged mesenteric lymph nodes (case 3)|
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| Discussion|| |
Carcinoid tumor is a malignant neuroendocrine tumor which may arise anywhere in gastrointestinal tract. Carcinoid tumors are neuroendocrine tumors with a relatively good prognosis, and long-term survival is possible despite advanced stages of disease.  The spectrum of presentation can vary from the usual incidental finding in an appendix; vague abdominal pain to a life-threatening surgical emergency like obstruction, perforation, or bleeding; or as a classical carcinoid syndrome. Carcinoid syndrome occurs when they metastasize to the liver because the vasoactive peptides escape the filtering action of the liver. The syndrome comprises of flushing attacks, diarrhea, asthmatic attacks, pulmonary and tricuspid stenosis. Neuroendocrine markers like 24-h urinary 5-hydroxyindoleacetic acid (5-HIAA) levels, and chromogranin and synaptophysin levels in blood are helpful in diagnosis.
The primary is usually small, but lymph node metastasis can be large. The tumors can be multiple and can produce a dense fibrotic reaction in the surrounding bowel and mesentery. Appropriate management can lead to cure, especially if the tumor can be fully resected, or to long-term palliation with medical treatment or cytoreductive surgery, or both, with good survival periods. Carcinoid tumors are usually not sensitive to chemo- or radiotherapy and surgical resection of primary remains the main stay of treatment. Unless a patient displays the carcinoid syndrome, which is generally indicative of metastatic disease, there is no clinical feature that leads the clinician to suspect a carcinoid tumor specifically. These tumors secrete various substances like serotonin, histamine, and prostaglandins; which produce carcinoid syndrome. In metastatic carcinoid tumors, long-term use of octreotide is reported. 
Treatment is aimed at removing the primary tumor with adequate margins even in the presence of liver metastasis. The procedure depends on the location of the tumor. These tumors grow slowly in contrast to most metastatic cancers and the survival period may be long even with metastasis. 
| Conclusion|| |
Gastrointestinal tract carcinoid tumors have a nonspecific clinical presentation, except in the case of the carcinoid syndrome. The final diagnosis is usually made by histopathology and immunohistochemistry. Surgical resection is the treatment of choice for improving survival and the role of aggressive surgery is paramount irrespective of the stage of disease and presentation. These three cases of gastrointestinal carcinoids help in renewing awareness and highlight some of the modes of presentation of contrasting surgical emergencies and a good outcome following appropriate surgery.
| References|| |
Soga J, Yakuwa Y, Osaka M. Evaluation of 342 cases of mediastinal/thymic carcinoids collected from literature: A comparative study between typical carcinoids and atypical varieties. Ann Thorac Cardiovasc Surg 1999;5:285-92.
Piura B, Dgani R, Zalel Y, Nemet D, Yanai-Inbar I, Cohen Y, et al
. Malignant germ cell tumors of the ovary: Astudy of 20 cases. J Surg Oncol 1995;59:155-61.
Murali R, Kneale K, Lalak N, Delprado W. Carcinoid tumors of the urinary tract and prostate. Arch Pathol Lab Med 2006;130:1693-706.
Shebani KO, Souba WW, Finkelstein DM, Stark PC, Elgadi KM, Tanabe KK, et al
. Prognosis and survival in patients with gastrointestinal tract carcinoid tumors. Ann Surg 1999;229:815-21.
Söreide JA, van Heerden JA, Thompson GB, Schleck C, Ilstrup DM, Churchward M. Gastrointestinal carcinoid tumors: Longterm prognosis for surgically treated patients. World J Surg 2000;24:1431-6.
Corleto VD, Angeletti S, Schillaci O, Marignani M, Caratozzolo M, Panzuto F, et al
. Long-term octreotide treatment of metastatic carcinoid tumor. Ann Oncol 2000;11:491-3.
Søreide O, Berstad T, Bakka A, Schrumpf E, Hanssen LE, Engh V, et al
. Surgical treatment as a principle in patients with advanced abdominal carcinoid tumors. Surgery 1992;111:48-54.
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]