Table 2.
Thyroid disease in cases with MEN2B syndrome
| Case | Age at surgery (yr) | Age at last FU (yr) | First available Ctn (ng/l) | Initial thyroid surgery | Histology | TNM (stage) at Dxa | Operation curable | Disease status at last FU |
|---|---|---|---|---|---|---|---|---|
| 1 | 0.6 | 12.4 | 60b,c | TT | CCH | T0N0M0 (n/a) | Yes | Cured |
| 2 | 0.6 | 7.9 | Ud | TT ± LND | CCH | T0N0M0 (n/a) | Yes | Cured |
| 3 | 0.5 | 6.4 | 5e | TT | MTC | T1aNxMx (l) | Yes | Cured |
| 4 | 12.0 | 25.5 | 360c | TT | MTC | T1aNxMx (l) | No | Progressive |
| 5 | 6.1 | 35.0 | 0.32c,f | TT | CCH with possible MTC | T0N0M0 or T1aNxMx (n/a or l) | Yes | Recurrence |
| 6 | 16.0 | 21.8 | 8000c | TT + cLND + bLND | MTC, IR | T4aN1bM1 (lVc) | No | Progressive |
| 7 | 6.5 | 9.8 | 3500c | TT + cLND + uLND | MTC, IR | T3N1bMx (lVa) | No | Persistent |
| 8 | 16.0 | 54.0 | 30c,g | TT ± LND | MTC | TxNxMx (?) | No | Persistent |
bLND bilateral lymph node dissection (LND), C cured (no biochemical signs of thyroid disease), CCH C-cell hyperplasia, cLND central LND, Ctn calcitonin, Dx diagnosis, FU follow-up, IR irradical resection (tumor identified at the resection margin), ±LND unknown if LND is performed, MTC medullary thyroid carcinoma, n/a not applicable, ng/l nanogram/liter, P persistent disease (biochemical signs), Pr progressive disease, R recurrent disease (biochemical signs), TNM tumor node metastasis—classification, TT total thyroidectomy, U undetectable, uLND unilateral LND, yr years, ? unknown
aStaging based upon the American Joint Committee on Cancer (AJCC) Cancer Staging Manual, eight edition (Rosen et al. [16])
bCalcitonin values can be elevated in (very) young children. For reference values in children, see: Basuyau et al. [17]
cPreoperative calcitonin level
d3 years postoperative calcitonin level. Patient was under treatment in another country at time of surgery; calcitonin levels were not measured earlier
e3 months postoperative calcitonin level
fµg/l, basal ctn (normal range <0.3 µg/l)—not stimulable
gng/ml, basal ctn (normal range <0.4 ng/ml). pentagastrin-stimulated ctn: 455 ng/ml (at 2 min), 310 ng/ml (at 5 min)