Appendiceal Carcinoid / Well Differentiated Neuroendocrine Neoplasm / Tumor
Differential Diagnosis
- Conventional GI adenocarcinoma
- Goblet cell carcinoid vs. appendiceal carcinoid
- Site of origin of metastatic well differentiated endocrine carcinoma
| Appendiceal Carcinoid / Well Differentiated Neuroendocrine Cell Neoplasm |
Appendiceal or Other GI Adenocarcinoma |
| Bland cytologic features |
Atypical cytology |
| Chromogranin or synaptophysin positive |
Chromogranin and synaptophysin negative |
| No desmoplastic response |
Desmoplastic response frequent |
| Rare mitotic figures |
Mitotic figures frequently numerous |
| Cohesive nests and cords |
Frequent single cell infiltration |
| No surface adenoma or in situ lesion |
May have surface adenoma or in situ component |
| No intracytoplasmic mucin |
May have intracytoplasmic mucin and/or signet ring cells |
| Appendiceal Carcinoid / Well Differentiated Neuroendocrine Cell Neoplasm |
Goblet Cell Carcinoid and/or Carcinoma ex-GCC |
| Chromogranin or synaptophysin positive |
Chromogranin and synaptophysin stain rare to scattered cells only |
| No intracytoplasmic mucin (lumenal mucin may be present in tubular carcinoid) |
Intracytoplasmic mucin in goblets |
| CK7 negative, CK20 variable |
CK7 variable, CK20 100% |
| Rare mitotic figures at most |
Mitotic figures may be numerous in carcinoma ex-GCC |
| Cohesive nests and cords |
Single cell infiltration or sheet like growth in carcinoma ex-GCC |
| No desmoplasia |
Desmoplasia may be present in carcinoma ex-GCC |
Determination of common sites of origin for metastatic well differentiated endocrine neoplasms
| |
TTF1 |
CDX2 |
PDX1 |
ISL1 |
PAX8 |
| Lung |
40-50% |
Negative |
Negative |
Negative |
Negative |
| Pancreas |
Negative |
0-18% |
28% |
68% |
50-67% |
| Stomach |
Negative |
0-17% |
60% |
Negative |
20% |
| Duodenum |
Negative |
0-17% |
60% |
Negative |
100% |
| Ileum |
Negative |
>90% |
Negative |
Negative |
Negative |
| Appendix |
Negative |
>90% |
55% |
Negative |
21% |
| Rectum |
Negative |
0-55% |
17% |
Negative |
85% |
Detection of specific islet hormones may be useful; PAX8 data has been questioned (see
Pancreas).
Determination of common sites of origin for metastatic well differentiated endocrine neoplasms-keratins
| |
CK7 |
CK20 |
| Lung |
Variable |
Negative |
| Pancreas |
Variable |
Variable |
| Stomach |
Variable |
Negative |
| Duodenum |
Negative |
Variable |
| Ileum |
Negative |
Variable |
| Appendix |
Negative |
Variable |
| Rectum |
Variable |
Variable |
CK7/20 staining is only helpful in instances of positivity where a negative result is expected