Journal of Cytology
Home About us Ahead of print Instructions Submission Subscribe Advertise Contact e-Alerts Login 
Users Online:423
  Print this page  Email this page Small font sizeDefault font sizeIncrease font size


 
 Table of Contents    
CASE REPORT  
Year : 2013  |  Volume : 30  |  Issue : 2  |  Page : 136-138
Metastatic appendiceal goblet cell carcinoid masquerading as mucinous adenocarcinoma in effusion cytology: A diagnostic pitfall


Department of Pathology, Gujarat Cancer and Research Institute, Ahmedabad, Gujarat, India

Click here for correspondence address and email

Date of Web Publication29-May-2013
 

   Abstract 

Goblet cell carcinoids are rare tumors of appendix having a mixed phenotype, with partial neuroendocrine differentiation and intestinal type goblet cell morphology. The reported incidence of this tumor is still limited. Till now, only two cases of metastatic goblet cell appendiceal carcinoid on effusion cytology have been reported in literature. We describe the clinico-pathological details and lay stress on fluid cytology of metastatic goblet cell carcinoid to ascitic fluid.

Keywords: Appendix; ascitic fluid; effusion cytology; goblet cell carcinoid; goblet cell; neuroendocrine.

How to cite this article:
Gupta A, Patel T, Dargar P, Shah M. Metastatic appendiceal goblet cell carcinoid masquerading as mucinous adenocarcinoma in effusion cytology: A diagnostic pitfall. J Cytol 2013;30:136-8

How to cite this URL:
Gupta A, Patel T, Dargar P, Shah M. Metastatic appendiceal goblet cell carcinoid masquerading as mucinous adenocarcinoma in effusion cytology: A diagnostic pitfall. J Cytol [serial online] 2013 [cited 2018 Nov 12];30:136-8. Available from: http://www.jcytol.org/text.asp?2013/30/2/136/112659



   Introduction Top


Goblet cell carcinoid (GCC), first reported by Gagne in 1969, is a rare tumor of appendix having a mixed phenotype, with partial neuroendocrine differentiation and intestinal type goblet cell morphology. [1],[2] It comprises approximately 6% of appendiceal carcinoids and usually occurs in the pure form. [3] Four types of carcinoid tumors that contain goblet cells are now recognized in the appendix: (1) Pure form composed entirely of goblet cells, (2) GCC with adenocarcinoma (adenocarcinoma ex GCC), (3) GCC with mucinous cystadenoma, and (4) the combined classical carcinoid with GCC tumor. [4] They are believed to behave in a more aggressive manner than usual appendiceal carcinoids. About 20% cases have been reported to present with metastases. [5] To the best of our knowledge, only two cases of metastatic goblet cell appendiceal carcinoid (GCAC) on effusion cytology have been reported in literature. [6],[7] Here, we report the cytological findings of another rare case of a metastatic GCAC to ascitic fluid.


   Case Report Top


A 45-year-old female presented with one-month history of gradual distension of abdomen and vomiting. Clinically, gross ascites was present. Past history of patient revealed that three years back, the patient was operated for bilateral ovarian tumor in a private hospital. She was asymptomatic for the next three years. The slides of ovarian tumor were reviewed, and a diagnosis of bilateral mucinous cystadenoma was made. There was no component of invasive tumor or carcinoid component in the ovarian tumor. Computed tomography (CT) scan of abdomen was done, which revealed ascites and diffuse omental thickening. Ascitic fluid was tapped. Slides were prepared on Shandon cytocentrifuge. Smears were made on glass slides, which were fixed immediately in 95% alcohol and stained by Papanicolaou method. Air-dried smears were stained by May-Grünwald-Geimsa (MGG) method. Smears of ascitic fluid were moderately cellular and composed of numerous cells disposed in acini, clusters as well as lying singly in a background of mucin. There were gland-like formations, some with central lumina. Predominant cell type was the signet ring cell with abundant vacuolated cytoplasm and nucleus pushed to periphery. A second population of cells was identified, which were smaller in size with folded nuclei, fine chromatin and scant to moderate amount of eosinophilic cytoplasm [Figure 1]. Our cytological diagnosis was compatible with that of metastatic mucinous adenocarcinoma. In view of a primary ovarian tumor and fluid cytology findings of metastatic mucinous adenocarcinoma, patient was given six cycles of chemotherapy. Repeat computed tomography (CT) scan after six months did not show any significant change in size of omental thickening. Infracolic omentectomy along with appendicectomy was performed. Gross examination of the specimen of appendix revealed a small diffuse shiny thickened area (less than 1 cm) at the tip of appendix. Hematoxylin and eosin-stained sections were obtained from formalin-fixed, paraffin-embedded tissue blocks, which revealed a submucosal infiltrate of dual population of cells, majority of which comprised of classical carcinoid tumor cells and a minor component of GCC lying in a pool of mucin. Mitosis was occasional. Necrosis was not seen. Tumor infiltrated upto peri-appendiceal fat. Sections from the omental thickening revealed infiltration by goblet cells (signet cells) only. Histopathological diagnosis of combined classical carcinoid and GCC with metastases to omentum was made. Immunohistochemical studies were performed on a Ventana Benchmark automated stainer on formalin-fixed, paraffin-embedded tissue block from both the sites. Appropriate positive and negative controls were obtained. The antibodies tested were cytokeratin (CK) 20, and the neuroendocrine markers chromogranin, synaptophysin, and neuron-specific enolase. Immunohistochemistry (IHC) in both the classical carcinoid and goblet cell component revealed positivity for neuroendocrine markers. The ascitic fluid smears were re-examined in light of histopathology findings. On carefully reviewing the slides, the second population of cells, which were fewer in number, was identified as classical carcinoid cells [Figure 1]. These were admixed with goblet cells. We could not make a diagnosis of metastatic GCAC to ascitic fluid because of our lack of experience with such a finding on cytological smears coupled with the fact that there was a frank predominance of signet ring cells in the smear. Identification of the classical carcinoid cells in ascitic fluid earlier would have prevented us from misdiagnosing this case.
Figure 1: Metastatic goblet cell carcinoid in ascitic fluid. Goblet cells (black arrow) and carcinoid cells (white arrow) are present in clusters. (a and c, Pap stain, × 400; b and d, Pap stain, × 1000)

Click here to view



   Discussion Top


GCC are unusual neoplasms with dual mucinous and neuroendocrine differentiation and a distinct predilection for the appendix. Other rare sites are colon, ampulla of Vater and duodenum. [8] They commonly present as acute appendicitis often with intra-abdominal mass as opposed to appendiceal carcinoid tumors, which are commonly incidental finding. The tumor is thought to arise from pleuripotent, intestinal crypt-base stem cells that are able to differentiate along neuroendocrine and mucinous cell lines. [1],[3] GCC behave more aggressively than classical carcinoid tumors. [4],[7],[8] However, they have relatively better prognosis when compared with traditional adenocarcinoma. Cytological findings of primary appendiceal GCC have not been described yet. Pathologists are seldom approached for FNAC as the patients usually present with acute appendicitis. Wojcik et al.[6] and Zafar et al.[7] described the cytological features of the two cases of metastatic GCAC in effusion cytology. They emphasized the presence of carcinoid component and signet ring cells in effusion cytology as was evident in our case. Other entities that contain signet ring cells including metastatic signet ring cell carcinomas from gastrointestinal tract, lung and also metastatic lobular carcinoma from breast may be considered in the differential diagnosis. Metastatic lobular carcinoma in effusion fluids are typically manifested as small uniform cells in an Indian file pattern. Presence of signet ring cells in these cases may show drop-like mucin in an intra-cytoplasmic vacuole. These features in combination reflect its origin from breast. Cytological picture in metastatic signet ring carcinoma from stomach is more likely to be one of single population of virtually all isolated cells rather than one of clusters of cells. [9]

Cytological misdiagnosis in present case was due to the fact that that there was a frank predominance of goblet cells in the smears, which masked the presence of minor population of classical carcinoid cells. Though the histopathological features of this tumor are classical, it is difficult to diagnose the tumor on cytology smears, especially when one cell type far exceeds the other. However, a careful search will lead to a correct diagnosis.

Present case highlights the importance of effusion cytology in diagnosing this rare tumor. The practicing pathologists need to keep this entity in mind to arrive at a correct diagnosis so that appropriate therapy can be instituted. One must not overlook even if few cells with different nuclear features are visualized on fluid cytological smears.


   Acknowledgments Top


The authors wish to express their thanks to Dr. P. M. Shah, Honorary Director, Dr. K. M. Patel, Dr. S. N. Shukla Deputy Directors of The Gujarat Cancer and Research Institute to allow us to publish this case report.

 
   References Top

1.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. 1
    
2.Gagne F, Fortin P, Dufour V, Delage C. Tumors of the appendix associating histologic features of carcinoid and adenocarcinoma. Ann Anat Pathol 1969;14:393-406.  Back to cited text no. 2
    
3.Pahlavan PS, Kanthan R. Goblet cell carcinoid of the appendix. World J Surg Oncol 2005;3:36.  Back to cited text no. 3
    
4.Chetty R, Klimstra DS, Henson DE, Albores-Saavedra J. Combined classical carcinoid and goblet cell carcinoid tumor: A new morphological variant of carcinoid tumor of the appendix. Am J Surg Pathol 2010;34:1163-7.  Back to cited text no. 4
    
5.Toumpanakis C, Standish RA, Baishnab E, Winslet MC, Caplin ME. Goblet cell carcinoid tumors (adenocarcinoid) of the appendix. Dis Colon Rectum 2007;50:315-22.  Back to cited text no. 5
    
6.Wojcik EM, Selvaggi SM. Goblet cell carcinoid in peritoneal fluid: A case report. Diagn Cytopathol 1991;7:155-7.  Back to cited text no. 6
    
7.Zafar S, Chen H, Sun W, Das K. Cytology of metastatic appendiceal goblet cell carcinoid in pleural effusion fluid: A case report. Diagn Cytopathol 2008;36:894-8.  Back to cited text no. 7
    
8.Albores-Saavedra J, Henson DE, Batich K. Pathological classification and clinical behavior of the spectrum of goblet cell carcinoid tumors of appendix. Am J Surg Pathol 2009;33:1259-60.  Back to cited text no. 8
    
9.Naylor B. Pleural peritoneal and pericardial fluids. In: Bibbo M, editor. Comprehensive cytopathology, 2 nd ed. Philadelphia: WB Saunders; 1997. p. 551-621.  Back to cited text no. 9
    

Top
Correspondence Address:
Anuja Gupta
Room #404, Department of Pathology, The Gujarat Cancer and Research Institute, NCH Campus, Asarwa, Ahmedabad - 380 016, Gujarat
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0970-9371.112659

Rights and Permissions


    Figures

  [Figure 1]

This article has been cited by
1 Intestinal goblet cell carcinoid presenting with recurrent sterile peritonitis in a patient on peritoneal dialysis: a case report
Chih-Wei Chen,Jan-Show Chu,Li-Chun Hsieh,Chih-Chin Kao,Yen-Chung Lin,Hsi-Hsien Chen
BMC Nephrology. 2017; 18(1)
[Pubmed] | [DOI]
2 Cytological findings of appendiceal mixed adenoneuroendocrine carcinoma in pleural effusion: Morphological changes evident after metastasis
Akiko Tonooka,Ken-Ichi Oda,Mamoru Hayashi,Ko-Ichi Sakazume,Hiroki Tanaka,Kyo-Hei Kaburaki,Toshimasa Uekusa
Diagnostic Cytopathology. 2015; 43(7): 577
[Pubmed] | [DOI]



 

Top
 
 
  Search
 
  
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Email Alert *
    Add to My List *
* Registration required (free)  


    Abstract
   Introduction
   Case Report
   Discussion
   Acknowledgments
    References
    Article Figures

 Article Access Statistics
    Viewed2341    
    Printed63    
    Emailed0    
    PDF Downloaded91    
    Comments [Add]    
    Cited by others 2    

Recommend this journal