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 Table of Contents  
CASE REPORT
Year : 2015  |  Volume : 5  |  Issue : 1  |  Page : 43-45

Mixed goblet cell carcinoid-adenocarcinoma of the appendix: A rare presentation


Department of General Surgery, People's Education Society Institute of Medical Science and Research, Kuppam, India

Date of Web Publication13-Mar-2015

Correspondence Address:
MFR Shoeb
Room no 218, G-Block, P. E. S. Institute of Medical Science and Research, Kuppam - 517 425, Chittoor District, Andhra Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2278-9596.153158

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  Abstract 

Mixed goblet cell carcinoid-adenocarcinoma in the gastrointestinal tract is a rare disease. The incidence of such tumors have been reported to range from 0.3-4.3%. A 70-year-old female presented with acute intestinal obstruction. Explorative laparotomy revealed tumor in the tip of appendix, stricture of distal ileum around 30 cm proximal to ileo-cecal junction due to circumferential serosal deposits of metastatic tumor leading to acute intestinal obstruction. Widespread metastasis was present with ascitis. Histopathological examination revealed mixed goblet cell carcinoid-adenocarcinoma of appendix with metastasis of mixed features in ileum and omentum. Appendicectomy and resection of the stricture with end to end anastomosis was performed.

Keywords: Appendiceal tumor, Goblet cell carcinoid, signet ring adenocarcinoma


How to cite this article:
Shoeb M, Sadhu D. Mixed goblet cell carcinoid-adenocarcinoma of the appendix: A rare presentation . Arch Int Surg 2015;5:43-5

How to cite this URL:
Shoeb M, Sadhu D. Mixed goblet cell carcinoid-adenocarcinoma of the appendix: A rare presentation . Arch Int Surg [serial online] 2015 [cited 2021 May 15];5:43-5. Available from: https://www.archintsurg.org/text.asp?2015/5/1/43/153158


  Introduction Top


Cancer of the appendix is an uncommon disease. The goblet cell carcinoid (GCC), an uncommon neoplasm of the vermiform appendix, is a separate entity from adenocarcinoma and carcinoid tumors. [1] Appendiceal carcinoids however are found in 1 out of every 300 appendectomies. [2] Goblet cell carcinoid has both endocrine and glandular differentiation. [2],[3]

A 70-year-old female patient presented with acute intestinal obstruction, due to stricture caused by circumferential serosal deposits in the distal ileum. We are reporting this case to highlight the unique clinical presentation of our patient with Goblet cell carcinoid of appendix with acute intestinal obstruction. To the best of our knowledge such presentation was not previously described in the literature so far.


  Case Report Top


A 70-year-old female patient presented with complaints of colicky lower abdominal pain, abdominal distension, and constipation since 10 days. On examination she was febrile, pulse rate was 120/min, blood pressure was 90/60 mm of Hg, respiratory rate was 28/min. Abdomen was distended, lower abdomen was tender with rebound tenderness and visible peristalsis. Bowel sounds were sluggish. Per rectal examination was normal. Plain X-ray of abdomen and ultrasound abdomen showed dilated large and small bowel loops. Laboratory investigations revealed leukocytosis with total count 15,370 cells/mm 3 , predominantly neutrophils, serum albumin -3 gm/dl, serum potassium -3.4 mmol/L, serum creatinine -0.5 mg/dl.

A clinical diagnosis of acute intestinal obstruction with strangulation was made. At laparotomy, findings were:

  1. Tumor of size 2×3 cm at tip of appendix adherent to cecum [Figure 1].
    Figure 1: Tumour in appendix, shown by arrow

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  2. Stricture in distal ileum about 30 cm proximal to ileocecal junction, with dilated proximal bowel and collapsed distal ileum, due to serosal deposits covering the entire circumference of ileum causing the obstruction [Figure 2].
    Figure 2: Stricture in distal ileum showing proximaly collapsed and distally distented ileum, shown by arrow

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  3. Deposits on the serosal surface of entire jejunum, ileum, tranverse colon, sigmoid colon, uterus, liver and omentum.
  4. About 500 ml of hemorrhagic ascitic fluid was aspirated and sent for histological examination.


Appendicectomy and resection of seven cm of ileum on either side of stricture with end to end anastomosis was performed. Lymph nodes from mesentry and omentum were sent for histopathological examination. Biopsy reported as "Mixed goblet cell carcinoid-adenocarcinoma of appendix with metastasis of mixed features in ileum and omentum" [Figure 3] and [Figure 4]. Negative margins were attained in the resected segment of ileum. Lymph node was negative for malignancy. Cytology of ascitic fluid was positive for malignant cells.
Figure 3: Histopathology slide showing sheets of cells with fibrotic stroma suggestive of adenocarcinoma

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Figure 4: Histopathology slide showing tumour cells with vacuolated cytoplasm and peripherally placed nucleus (cluster of cells with goblet cell) and stromal fibrosis

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Carcinoembryonic antigen was 0.40 ng/ml, serum chromogranin was 2055 ng/ml. Urinary 5-hydroxy indole acetic acid (5-HIAA) level was 39 mg/24hrs. As GCC is crypt cell derived, immunohistochemistry showed synaptophysin and chromogranin A positivity [Figure 5].
Figure 5: Immunohistochemistry slide showing synaptophysin stain "cytoplasmic positivity"

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  Discussion Top


In 1927, Hamperl was first to notice the presence of neuroendocrine cells in adenocarcinomas of GIT. [1] All GCCs occurred in adults. Patients age ranged from 29 to 80 years with a mean age of 50 ± 1 year. There was an overall female predominance, with a male to female ratio of 1:2.2(20:43). [2] A higher incidence was found among white races. [3] The incidence of a carcinoid tumor of the Gastrointestinal tract co-existing with an adenocarcinoma synchronously has been reported to range from 0.3-4.3%. [4] When both the epithelial tumor and neuroendocrine tumor are present in the same area without any transitional changes in the tumor cells, they are termed as "collision" tumors. Conversely "composite" tumor is used when endocrine and epithelial cells are intermixed within the same tissue and transitions can be observed. [5] Collision tumors are considered to be a biclonal derivation, resulting from two separate but adjacent neoplasms, while composite tumors are believed to result from multidirectional differentiation of a single neoplasm. [5],[6] In addition, a single tumor component should be observed in the metastases in case of collision tumor. Both glandular and carcinoid elements should be present in metastases from composite tumors. [5],[7] Our patient showed metastasis of both glandular and carcinoid elements.

The most common clinical presentation is abdominal pain and a palpable mass (50%), followed by symptoms related to acute appendicitis (44%), incidental diagnosis (3%), and other non-specific findings (3%). Patients with advanced disease most commonly present with abdominal masses. [2] Other rare manifestations include intussusception, gastrointestinal bleeding, secondary genitourinary complications, and appendicovesical fistula. [7] Goblet cell carcinoid metastasize in 15-30% of cases, to the ovaries, pelvis, abdominal cavity, rib, vertebrae, and lymph nodes through lymphatic vessels by trans-coelomic and intraperitoneal invasion. However, hematogenous spread is rare. [7] There is insufficient data for recommending management of goblet cell tumor of appendix where in tumor is localized and confined to appendix. [9] For tumors spreading beyond appendix or in positive appendicectomy resection margin or in case of localized perforation, right hemicolectomy is recommended. For tumors spreading intraperitoneally, a debulking procedure is advised. Chemotherapy is advised for patients with advanced disease. [2]

In mixed tumors, 5-year survival rate is 38% for stage IV tumors. Five-year survival rate for adenocarcinoma are typically in the 15-20% range. Five-year survival rates for carcinoid tumor are 25-35% in those with distant metastasis. [10] Prognosis of mixed tumors is better than de novo adenocarcinoma or de novo carcinoid tumor for the same stage. The presence of carcinoid component makes the tumor less aggressive. [2],[8] In our patient, Goblet cell carcinoid was arising from tip of appendix with widespread metastasis and malignant cells in the ascites. Only palliative appendicectomy and resection of strictured segment of ileum, to relieve the cause of obstruction was performed. Patient had an uneventful recovery. Adjuvant chemotherapy with 5-flurouracil and leucovorin has been planned depending upon the fitness of the patient.

Though rare, the possibility of this clinical entity should be kept in mind, while treating patients who present with intestinal obstruction.


  Acknowledgement Top


Authors are thankful to Dr Sunil Kumar M.D, Associate professor of Pathology, P.E.S.I.M.S.R. and the Department of pathology P.E.S.I.M.S.R., Kuppam for their support and valuable guidance.

 
  References Top

1.
McCusker ME, Cote TR, Clegg LX, Sobin LH. Primary malignant neoplasms of the appendix: A population-based study from the surveillance, epidemiology and end-results program, 1973-1998. Cancer 2002;94:3307-12.  Back to cited text no. 1
    
2.
Pahlavan PS, Kanthan R. Goblet cell carcinoid of the appendix. World J Surg Oncol 2005;3:36.  Back to cited text no. 2
    
3.
Capella C, La Rosa S, Uccella S, Billo P, Cornaggia M. Mixed endocrine - exocrine tumors of the gastrointestinal tract. Semin Diagn Pathol 2000;17:91-103.  Back to cited text no. 3
    
4.
Tang LH, Shia J, Soslow RA, Dhall D, Wong WD, O'Reilly E, et al. Pathologic classification and clinical behavior of the spectrum of goblet cell carcinoid tumors of the appendix. Am J Surg Pathol 2008;32:1429-43.  Back to cited text no. 4
    
5.
McCusker ME, Cote TR, Clegg LX, Sobin LH. Primary malignant neoplasms of the appendix: A population-based study from the surveillance, epidemiology and end-results program, 1973-1998. Cancer 2002;94:3307-12.  Back to cited text no. 5
    
6.
Chaturvedi KU, Kaur H. Composite carcinoid carcinoma of the colon. Indian J Cancer 1994;31:203-6.  Back to cited text no. 6
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7.
Mitra B, Pal M, Paul B, Saha TN, Maiti A. Goblet cell carcinoid of appendix: A rare case with literature review. Int J Surg Case Rep 2013;4:334-7.  Back to cited text no. 7
    
8.
Pecorella I, Memeo L, Ciardi A, Rotterdam H. An unsual case of colonic mixed adenoendocrine carcinoma: Collision versus composite tumor. A case report and review of the literature. Ann Diagn Pathol 2007;11:285-90.  Back to cited text no. 8
    
9.
Chen V, Qizilbash AH. Goblet cell carcinoid tumor of the appendix. Report of five cases and review of the literature. Arch Pathol Lab Med 1979;103:180-2.  Back to cited text no. 9
    
10.
Shaun McKenzie, Evers MB. "Small intestine": Townsend, Beauchamp, Evers Matlox (Editors) Sabiston textbook of surgery. 19 th ed. Elsevier; 2012. p. 1259-63.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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