Goblet cell adenocarcinoma

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Crypt cell carcinoma, also known as goblet cell carcinoid[1][2] and neuroendocrine tumour with goblet cell differentiation, is a rare malignant tumour that is typically seen in the vermiform appendix.

Goblet cell adenocarcinoma
Diagnosis in short

Crypt cell carcinoma. H&E stain.

LM small clusters of cells with stippled chromatin and a goblet cell-like appearance
LM DDx signet ring cell carcinoma, appendiceal neuroendocrine tumour
Stains alcian blue +ve, PASD +ve, mucicarmine +ve
IHC synaptophysin +ve, chromogranin +ve, S-100 +ve, CK20 +ve
Gross usu. no mass apparent
Site vermiform appendix, elsewhere in the GI tract

Prevalence rare
Prognosis moderate
Clin. DDx acute appendicitis, other appendiceal tumours, other abdominal pathology

General

  • Rare appendiceal tumour that typically has an aggressive course vis-a-vis other appendiceal carcinoids.[1]
  • Mixed (biphasic) tumour with endocrine and exocrine features.
  • Usually presents as acute appendicitis.[2]
    • Less common presentations: appendiceal mass, pain.
  • Five year survival in one series: 60-85%.[2]

Gross

  • Typically no mass is apparent at gross.[2]

Microscopic

Features:[2]

  • Mixed neuroendocrine-nonneuroendocrine tumour;[3] features of both carcinoid and adenocarcinoma.[2]
    • Archictecture: cells arranged in nests or clusters without a lumen.
    • Location: deep to the intestinal crypts (crypts of Lieberkühn); usually do not involve the mucosa.
    • Cytoplasm distended with mucin.
    • DNA: crescentic nucleus (similar to in signet-ring cells).
      • +/-Multinucleation.
      • +/-High mitotic rate.
      • Usually minimal nuclear atypia.

DDx:

Images

Stains

  • Mucin stains +ve:
    • Mucicarmine, perodic acid-Schiff diastase (PAS-D), alcian blue.

IHC

  • Classic neuroendocrine markers:
    • Synaptophysin +ve.
    • Chromogranin +ve.
  • S-100 +ve.
  • NSE +ve.
  • Serotonin +ve.

Keratins:

  • Usually CK20 +ve > CK7 +ve.
  • CEA +ve (membrane).

Notes:

  • Nice review of stains in Pahlavan and Kanthan.[2]

See also

References

  1. 1.0 1.1 van Eeden S, Offerhaus GJ, Hart AA, et al. (December 2007). "Goblet cell carcinoid of the appendix: a specific type of carcinoma". Histopathology 51 (6): 763–73. doi:10.1111/j.1365-2559.2007.02883.x. PMID 18042066.
  2. 2.0 2.1 2.2 2.3 2.4 2.5 2.6 Pahlavan, PS.; Kanthan, R. (Jun 2005). "Goblet cell carcinoid of the appendix.". World J Surg Oncol 3: 36. doi:10.1186/1477-7819-3-36. PMID 15967038. Cite error: Invalid <ref> tag; name "pmid15967038" defined multiple times with different content Cite error: Invalid <ref> tag; name "pmid15967038" defined multiple times with different content
  3. Volante M, Righi L, Asioli S, Bussolati G, Papotti M (August 2007). "Goblet cell carcinoids and other mixed neuroendocrine/nonneuroendocrine neoplasms". Virchows Arch. 451 Suppl 1: S61–9. doi:10.1007/s00428-007-0447-y. PMID 17684764.