|
| | Histology: |
| | (i) Scattered staining for chromogranin A and synaptophysin, along with mucus. |
| | (ii) Ki67 index. |
| | (iii) Staging according to Tang et al. [12]. |
| | Imaging: |
| Diagnostic procedures | (i) CT/MRI of the chest and the abdomen/pelvis |
| | (ii) MRI of the abdomen/pelvis |
| | (iii) Somatostatin receptor scintigraphy, Ga68-PET scans are usually negative |
| | (iv) FDG-PET and MIBG-PET scannings are usually negative |
| | Biochemistry: |
| | (i) CEA, CA-19-9, CA-125 |
| | (ii) Chromogranin A and U-5HIAA usually normal |
|
| | Appendectomy |
| Surgical therapy | Hemicolectomy (standard surgical treatment) |
| | Debulking surgery when possible |
|
| Medical therapy | 5-fluorouracil-based chemotherapeutic regimen |
| Cytoreductive surgery combined with HIPEC in selected cases |
|
| | (i) Clinical: abdominal pain, weight loss |
| Followup | (ii) Biochemistry: CEA, CA-19-9, and CA-125 |
| | (iii) Imaging: CT or MRI every 3–6 months, then yearly, mimicking the guidelines for colorectal adenocarcinoma. Lifelong followup. |
|