Combined Goblet Cellcarcinoid and Mucinous Cystadenoma of The

I Clin Pathol 1995;48:869-870 869 Combined goblet cell carcinoid and mucinous cystadenoma of the appendix J Clin Pathol: first published as 10.1136/jcp.48.9.869 on 1 September 1995. Downloaded from R K Al-Talib, C H Mason, J M Theaker Abstract Case reports Two cases of combined goblet cell car- CASE ONE cinoid and mucinous cystadenoma oc- An adherent pelvic appendix was resected with curring in the appendix are reported. The difficulty from a 54 year old woman admitted histogenesis of the goblet cell carcinoid for an interval appendicectomy, two months remains one of its most controversial as- after an attack of appendicitis. The appendix pects and the occurrence of both of these measured 60 x 15 mm and was irregular, dis- relatively uncommon tumours in the same torted and showed serosal fibrosis. On sec- organ may lend support to the unitary tioning, the tip of the appendix was distended stem cell hypothesis on the origin of this and a mucus containing diverticulum pen- tumour. Alternatively, this occurrence etrating the muscular wall of the appendix was may represent an example ofthe adenoma/ identified. carcinoma sequence. ( Clin Pathol 1995;48:869-870) Department of CASE TWO Histopathology, Keywords: Goblet cell carcinoid, mucinous cyst- A 64 year old woman was a Southampton adenoma, appendix, histogenesis. admitted with four University Hospitals month history of a dull ache in the right iliac NHS Trust, fossa which had become increasingly severe Southampton S09 4XY R K Al-Talib Goblet cell carcinoid is an uncommon tumour over the last week. Eight months earlier, the J M Theaker of patient had been admitted with a similar epi- the appendix showing features of both a sode of pain which had been treated con- Department of carcinoid and an adenocarcinoma.12 The mu- underwent Histopathology, cinous cystadenoma is an epithelial neoplasm servatively. The patient appen- Eastbourne District which represents the appendiceal counterpart diectomy. The resected appendix measured Hospital 65 x 12 mm and had a distended, fibrotic tip C H Mason of the more common colonic adenoma.34 In which contained two small diverticulae on sec- this report we wish to describe the existence of http://jcp.bmj.com/ Correspondence to: tioning. Dr J M Theaker. both tumours within the appendices of two Accepted for publication patients. This is an unusual occurrence which 18 January 1995 raises the possibility of a related histogenesis. Pathology Both appendices were of very similar ap- pearance on histology. The diverticulae noted were produced on the basis of a mucinous cystadenoma. The lumen was focally dilatated on September 30, 2021 by guest. Protected copyright. and lined by mucus secreting columnar epithelium. The epithelium was mainly flattened with focal papillary areas. Cellular crowding with pseudo-stratification and mild to moderate atypia were noted on cytology (fig 1). The appendiceal walls were also widely infiltrated by nests, clumps and rosettes of tumour cells with features resembling those of a goblet cell carcinoid (fig 2). The tumour cells were uniform, distended with mucus and had crescentic nuclei arranged around the periphery of the tumour nests. There was little pleomorphism and mi- totic activity was absent. Moderate numbers of Paneth cells, both singly and as part of the tumour nests, were present in the second case but were scarce in the first. A Gremilius stain for agyrophilia performed in case 1 revealed numerous positive cells, occurring both singly and in the periphery ofsmall tumour trabeculae but not in the larger tumour nests. Only scattered single positive cells were identified in the second case. The tumour nests in both Figure 1 Mucinous cystadenoma of the appendix showing cystic dilatation of the lumen lined by mucus secreting epithelium (haematoxylin and eosin, x 100) (inset: detail, cases appeared to arise from the basiglandular x 360). region ofthe intestinal crypts, in close proximity 870 Al-Talib, Mason, Theaker endocrine/exocrine cell termed an amphicrine cell, hence the amphicrine cell carcinoma.8 The striking resemblance of the tumour nests to J Clin Pathol: first published as 10.1136/jcp.48.9.869 on 1 September 1995. Downloaded from intestinal crypts prompted Isaacson to pos- tulate an origin from lysozyme producing cells 7,- X ',!-';. o t ,, QW >- s ... v , normally present in intestinal crypts.9 common .. is the most b cystadenoma -49'w 5 Mucinous - A~~~~~~~~~~~~~~~~~~~WE .s.I ~0 cause of the so-called mucocele of the appendix. In this condition there is focal cystic dilatation of the appendix which is lined by 4>A. columnar/cuboidal, mucus secreting epithelium arranged in variable patterns, ranging r t.. ........ *. from well developed papillary formations pro- . jecting into the lumen to a single layer of flat cuboidal cells.3 Mucus filled diverticulae of the ~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~~. appendiceal wall are commonly associated with this condition because of increased intra- }~~~~~~~~~~~~~~~~~~~~~~~~~~~ luminal pressure. It is now widely accepted that the mucinous cystadenoma is the appendiceal counterpart ofvillous adenomas occurring else- where in the intestine, differing from the latter only because they are restricted to the appendix.34 Figure 2 Goblet cell carcinoid nests infiltrating the wall of the appendix (haematoxylin Despite wide interest, the histogenesis of and eosin, x 100). the goblet cell carcinoid remains controversial, hence the importance of this report. If this tumour is a true member of the carcinoid fam- to the mucinous cystadenoma. The walls and contained ily, as suggested by Subbuswamy et al, then its serosal surfaces of both appendices co-existence with a mucinous cystadenoma, pools of extravasated mucin, in which tumour itselfan uncommon epithelial appendiceal neo- cells were identified. plasm, would seem to support the unitary stem cell hypothesis. If this tumour, however, is a carcinoma of crypt cell origin rather than a Discussion carcinoid, as claimed by Isaacson,9 then this et an appendiceal In 1969 Gagne al5 described combination may represent an example of the tumour with features intermediate between adenoma/carcinoma sequence, a widely re- those of a carcinoid and those of an ad- cognised and accepted concept. '0 Furthermore, et a16 later sug- enocarcinoma. Subbuswamy the coincidental occurrence of these two neo- name carcinoid" for http://jcp.bmj.com/ gested the "goblet cell cannot be entirely ruled out. Further tumour in view of its growth pattern and plasms this reports of similar occurrences may explain this relatively good prognosis, which resembled that unusual association. of carcinoids more than adenocarcinomas, and because the principle cell type in the tumour 1 Berardi RS, Lee SS, Chen HP. Goblet cell carcinoids of the closely resembled the goblet cells of the in- appendix. Surg Gynecol Obstet 1988;167:81-6. testinal tract. Since then, this tumour has been 2 Warkel RL, Cooper PH, Helwig EB. Adenocarcinoid, a mucin-producing tumour of the appendix. Cancer 1978; described by a wide variety of names including 42:2781-93. on September 30, 2021 by guest. Protected copyright. mucinous carcinoid, adenocarcinoid, am- 3 Higa E, Rosai J, Pizzimbono CA, Wise L. Mucosal hy- perplasia, mucinous cystadenoma, and mucinous cyst- phicrine cell carcinoma, and crypt cell car- adenocarcinoma of the appendix. Cancer 1973;32:1525- cinoma.' This broad nomenclature reflects the 41. 4 Qizilbash AH. Mucoceles ofthe appendix; their relationship identification of goblet cells, Paneth cells and to hyperplastic polyps, mucinous cystadenomas and cyst- enterochromaffin cells within this tumour, the adenocarcinomas. Arch Pathol 1975;99:548-55. 5 Gagne F, Fortin P, Dufour V, Delage C.Tumeurs de l'ap- presence ofwhich has given rise to considerable pendice associant de carreteres histologique de carcinoide discussion regarding its histogenesis. As a et d'adenocarcinome. Ann Anat Pathol 1969;14:393-406. 6 Subbuswamy SG, Gibbs NM, Ross CF, Morson BC. Goblet result, several hypotheses have been proposed. cell carcinoid of the appendix. Cancer 1974;34:338-44. Supporters of the unitary hypothesis suggest 7 Warner TFCS, Seo IS. Goblet cell carcinoid of appendix; ultrastructural features and histogenetic aspects. Cancer that the tumour derives from a single neoplastic 1979;44:1700-6. intestinal cell, albeit with divergent differ- 8 Olsson B, Ljunberg 0. Adenocarcinoid of the vermiform appendix. Vtrchows Arch A Pathol Anat Histopathol 1980; entiation.7 A second hypothesis suggests that 386:201-10. the tumour is the result of the simultaneous, 9 Isaacson P. Crypt cell carcinoma of the appendix (so-called adenocarcinoid tumour). AmjSurgPathol 1981;5:213-24. integrated, neoplastic proliferation of several 10 Day DW, Morson BC. The adenoma-carcinoma sequence. histogenetically different elements.2 Other au- In: Morson BC, ed. 7he pathogenesis ofcolorectal cancer. Vol 10 in the series Major problems in pathology. Philadelphia: thors suggest that it originates from a combined W B Saunders, 1978:58-71..

Load more