Final Diagnosis -- A 68 year old female with goblet cell carcinoid

FINAL DIAGNOSIS   GOBLET CELL CARCINOID

GOBLET CELL CARCINOID
Also called mucinous carcinoid, adenocarcinoid, microglandular goblet cell carcinoma.
Shows divergent differentiation of normal crypt stem cells (columnar, endocrine, goblet cell, Paneth cell lineages).
Occurs in an older age group then conventional carcinoid.
Often associated with perforative appendicitis.
The behaviour of the Goblet Cell Carcinoid is more aggressive than that of the classic carcinoid particularly if the tumor has transmural involvement, extends into the cecum, is larger than 2 cm and shows the presence of signet ring cells.

Bad PROGNOSTIC FACTORS

• transmural involvement
• extension into the cecum
• size larger than 2 cm
• solid pattern in >50%+ of tumor
• perineural invasion
• lymphatic invasion
• spread beyond appendix
• serosal involvement at presentation
• incomplete excision at appendix base

Metastasis has been documented particularly to the ovary.

CYTOGENETICS

loss of heterozygosity of 18q (56%), 16q (38%), 11q (25%), resemble ileal carcinoids more than appendiceal adenocarcinoma

TREATMENT
Right hemicolectomy is recommended for this tumor type especially with
-spread beyond the appendix
-size of the tumor more than 2cm

DIFFERENTIAL DIAGNOSIS

• Metastatic signet-ring carcinoma
  infiltrative pattern of signet-ring cells

• Classic carcinoid tumor
  no goblet cell differentiation
  no intracellular mucus vacuoles
  no Paneth cell differentiation

• Mucinous adenocarcinomas
  more aggressive
  irregular fused structures without distinct lumina
  often merges with mucosal adenoma

Contributed by Sonal Kamat, MD