Goblet cell carcinoid tumors of the appendix: An overview

Table 1 Comparison of clinicopathological features of goblet cell carcinoid, carcinoid & adenocarcinoma

	Goblet cell carcinoid	Carcinoid	Adenocarcinoma
Clinical features
Age	5th-6th decade	4th decade	7th decade
Carcinoid syndrome	No	Yes	No
Primary symptoms	Acute appendicitis	Acute appendicitis	Mass
Gross appearance	> 2 cm, ill defined thickening	< 2 cm	> 2 cm, well defined mass
Microscopic appearance
Morphology	Clusters of goblet or signet-ring cells separated by fibrosis/pools of mucin	Nests of small cells	Well-formed glands to sheets of poorly differentiated signet-ring cells
Atypia	Minimal	Minimal	Marked
Mitosis	Rare	Rare	Increased
Vascular and perineural invasion	Present	Absent	Present
Infiltrative margins	Present	Absent	Present
Special stains
Argyrophil	Positive	Positive	Usually negative
Argentaffin	Negative	Positive	Negative
Mucicarmine/PAS/PASD	Positive in goblet cells	Negative	Positive
IHC
CEA	+	-	+
CDX2	+	-	+
CAM5.2	+	-	+
CK20	±	-	+
CK7	±	-	-
CK19	+	-	-
Neuroendocrine markers	±	±	-
Math1 and HD5	+	-	+
p53, p16	-	-	+
Molecular pathology
DPC4, Kras, β-catenin mutation and p53 over expression	-	-	+

Table 2 Treatment guidelines recommended by various authors (1974-2008)

Subbuswamy et al[2]	APX
	RH in case of cecal involvement
Klein[3]	APX
Haqqani et al[40]	RH if base of appendix or caecum is involved
Warkel et al[4]	RH in case of spread beyond appendix, atypia, and mitotic count ≥ 2/10 hpf
Chen et al[41]	APX alone unless there is cecal involvement
Olsson et al[42]	RH in case of spread beyond appendix, atypia, and mitotic count ≥ 2/10 hpf
Edmonds et al[33]	RH in all cases
Bak et al[39]	RH in case of spread beyond appendix, atypia, and mitotic count ≥ 2/10 hpf
Park et al[43]	RH in all cases
Rutledge et al[44]	RH in all cases
Butler et al[36]	RH for cecal involvement, BSO in females
Ramnani et al[13]	< 2 cm in size, without serosal & lymphatic involvement-APX
	More advanced tumor-RH
Kanthan et al[30]	RH
Li et al[31]	RH for N1, M1 or Mib1 > 3%
Varisco et al[23]	RH in case of spread beyond appendix, atypia, and mitotic count ≥ 2/10 hpf
Byrn et al[35]	No value of RH in non-metastatic cases
Pham et al[22]	RH with attendant mesenteric nodal resection for (1) T3/T4 disease or nodal involvement; (2) direct cecal involvement; and (3) clinically positive mesenteric nodes
	Bilateral oophorectomy for post menopausal women
O’Donnell et al[20]	RH irrespective of stage
Tang et al[17]	Group A T1, 2-no recommendations
	T3 or 4, group B/C, perforation, positive margin-RH with oophorectomy if possible. CT in stage III/IV
	Stage IV/group C- debulking/oophorectomy/systemic/intraperitoneal CT

APX: Appendicetomy; RH: Right hemicolectomy; hpf: High power fields; CT: Computed tomography.