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Carcinoid Tumour of Caecum : A Rare Presentation as Palpable Mass in Right Iliac Fossa
BM Subnis*, Waqar Ahmed Ansari**, Dinesh Joshi***, Vinaya Ambore*****, PD Nichat****, Parag Kumthekar***, Sheetal Patil***
 

Abstract
A 67 year old female presented with lump in abdomen in right iliac fossa and pain which she noticed for the first time.
There was no history of previous such episodes, fever, melaena, tuberculosis.

General examination was unremarkable, per abdominal examination revealed nontender lump of size 5 cm x 5 cm in right iliac fossa with restricted mobility.

CT scan of abdomen revealed eccentric caecal mass with involvement of adjacent colon and ileocaecal junction. There was extra serosal extension with contiguous involvement of right psoas muscle, Liver was unremarkable.

Exploratory laparotomy with right hemicolectomy with ileo - transverse anastomosis was done. Patient stood procedure well. Histopathology revealed poorly differentiated neuroendocrine carcinoma.


 

Introduction
Carcinoid tumours are nonpancreatic digestive neuroendocrine tumours, generally well differentiated. Most of the time they develop from enterochromaffin cells of the digestive tract. Most of them are localized in ileum, rectum, stomach and appendix. Only bronchi are frequent extra-digestive localization. Most of these tumours are asymptomatic and discovered incidentally during a laparotomy or secondary to the exploration of a carcinoid syndrome. After tumour resection in carcinoid syndrome postoperative treatment is mainly with somatostatin analogues, and patients have a good recovery. Carcinoids, despite the fact that they are usually silent and diagnosed with advantages, have good prognosis and offer, after effective treatment, long survival.

Case Report
A 67 year old female presented with lump in abdomen in right iliac fossa, with dull aching pain continuous in nature since seven days. There was no history of previous such episodes, fever, melaena, tuberculosis. There was no history of vomiting, loose motions, fatigue, dizziness, palpitations, and asthma.
General examination was unremarkable, per abdominal examination revealed non-tender lump of size 5 cm x 5 cm in right iliac fossa, firm in consistency.
Ultrasonography showed thickening of caecal wall.

A plain and contrast enhanced CT scan of the abdomen and pelvis showed eccentric heterogeneously enhancing mass lesion involving caecum. There was no obstruction. There was pericaecal fat stranding and fat planes between the lesion and right psoas muscle which were obscured. The lesion measured 5 cm x 4.4 cm in maximum transverse diameter. Small pericaecal and right iliac nodes were seen. Liver and other abdominal viscera appeared normal.
Routine blood investigations were normal. X-raychest was normal.

Exploratory laparotomy revealed mass in caecal wall with involvement of serosa with presence of pericaecal and mesenteric lymphnode enlargement without any involvement of appendix. Right hemicolectomy was done with ileo-transverse colon end to end anastomosis.

Rest of the bowel was normal. Patient stood procedure well. Histopathology revealed poorly differentiated neuroendorine carcinoma extending through muscularis propria and involving pericolic fat. Serosal breach was noted. Lymphnodes were not involved.

In view of completely resected tumour without metastasis postoperative chemotherapy was not given.
Fig. 1 : CT scan showing mass involving caecum. Fig. 2 :Coronal CT scan image reconstruction showing mass involving caecum.
Fig. 3 : Histopathology slide showing carcinoid tumour 20x. Fig. 4 : Histopathology slide showing carcinoid tumour 50x.

Fig. 5 : Coronal CT scan image reconstruction showing normal appendix.

Discussion
Carcinoid syndrome is the syndrome complex due to hormones secreted from metastasis from carcinoid tumours.1

These tumours usually secrete excessive amounts of hormone serotonin. The carcinoid tumours arise from neuroendocrine cells, which are widespread in the human body, especially in the organs derived from the primitive intestine. In 1907, Obserndonfer called a group of small, benign - appearing tumours karzinoide tumoren. Some carcinoids could be malignant.

These intensely, vascularised tumours follow the so called “rule of one third” which states that one third of these tumours are multiple, one third of those in the GI tract are in small bowel, one third have second malignancy, and one third metastasize.

These tumours originate from any cell of aminoprecursor uptake and decarboxylation system and therefore produce several intestinal hormones.
These tumours are frequent in women (2-4:1).2 In order of frequency carcinoids may occur in appendix (35%), ileum (28%) rectum (13%) and bronchi. Incidence is less than 1% in the pancreas, gall bladder, liver, larynx, testes and ovaries.

Approximately 1 in 200 appendectomies result in discovery of carcinoid.3,4 Incidence of metastasis depends upon tumour size.

Tumour less than 1 cm has 15-25% incidence, 1-2 cm. has 60-80% incidence of metastasis, tumour > 2 cm. has more than 70% of metastasis.5

The colon and ileocaecal region are rare sites of origin for gastro intestinal carcinoid.6

The characteristic features of colonic and ileocaecal carcinoids include.

  1. Borer JG, Bauer SB. A single-system ectopic ureter draining an ectopic dysplastic kidney: delayed diagnosis in the young female with continuous urinary incontinence. Br J Urol 1998; 81 : 474-8.
  2. A higher incidence of carcinoids in the caecum as compared to those of the rest of the colon followed by ileocaecal region.
  3. A high incidence of palpable abdominal tumours resulting from a tendency of the tumours to be large (89% are above 2 cm).6
  4. A high incidence of metastasis and argentaffin cell type in ileocaecal carcinoids.
  5. A low detection rate of serotonin in immunohistochemical evaluation.
  6. A low post operative survival rate.
Diagnosis is made by the pathologist and staging by conventional radiologic procedures; dosage of neuroendocrine mediators such as 24 hours urinary 5 - HIAA. Now a days in III octreotide scintigraphy has an 86% sensitivity to detect the carcinoid2 and is useful for staging and for planning a surgical intervention. Simple appendectomy is adequate treatment for appendicial carcinoids less than 1 cm in diameter.

Adequate treatment for tumours greater than 2 cm is right hemicolectomy, for tumours in between 1-2 cm with mesoappendix7 involved or positive lymphnodes for malignancy right hemicolectomy is necessary.

When liver metastasis are encountered, octreotide can relieve symptoms and sometimes the progression of the disease. Other chemotherapeutic agents such as adriamycin, streptozotocin1, 5-Flurouracil, cisplatin can be used.

Symptomatic treatment is also given for diarrhoea, hypertension, bronchial constriction.

References
  1. Luigi S. Malignant carcinoid syndrome www.emedicine.com/med/topic 2649.htm.
  2. Spallitta SI, Termine G. Carcinoid of the appendix, A case report. Minerva Chirurgic 2002; 55 (1-2) : 77-87.
  3. Burke AP, Thomas RM. Carcinoids of jejunum and ileum. Cancer 1997; 79 : 1086.
  4. Mortel CG. History of carcinoid tumor of small intestine. Cancer 1961; 14 : 901.
  5. Mortel CG. Karnofsky memorial lecture. J Clinical Oncology 1987; 5 : 1502.
  6. Soga J. Carcinoids of the colon and ileocaecal region : a statistical evaluation of 363 cases collected from the literature. Jour Exp Clin Cancer Res 1998; 17 (2) : 139-48.
  7. Jensen R, Doherty GM. Carcinoid tumors and carcinoid syndrome; De-vita cancer principles and practice of oncology, 6th edition : 1815-8.
 
*Professor and Head; **Lecturer; ***Resident; ****Associate Professor, Department of Surgery, Grant Medical College and JJ Group of Hospitals, Mumbai 400 008.
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