Klin Padiatr 2014; 226(06/07): 379-381
DOI: 10.1055/s-0034-1387731
Short Communication
© Georg Thieme Verlag KG Stuttgart · New York

Papillary Thyroid Carcinoma in an Adolescent Girl with Graves’ Disease

Diagnose eines papillären Schilddrüsenkarzinoms bei einem Mädchen mit Morbus Basedow
J. Gesing
,
R. Pfaeffle
,
H. Christiansen
,
A. Keller
,
T. Lincke
,
P. Lamesch
,
O. Sabri
,
W. Kiess
Further Information

Publication History

Publication Date:
28 November 2014 (online)

Case report

A 13-year-old girl presented to her pediatrician with headaches, tremor and restlessness of some weeks duration. The family had noticed swelling of the neck and protrusion of the eyes. The patient’s past medical history was uneventful. There was no endocrinopathy or malignancy known in the family. The parents migrated from Iraq.

Endocrine assessment detected a goiter (WHO 2°) and exophthalmos. Laboratory analysis revealed overt hyperthyroidism (thyroid-stimulating hormone (TSH) 0.04 mU/l (0.35–5); free thyroxine (fT4) 56.7 pmol/l (10.3–21); free triiodothyronine (fT3) 39.2 pmol/l (2.76–6.45)). Elevated serum TSH receptor antibodies (TRAb) of 2.36 U/l (0–1.5) confirmed the diagnosis of GD. An ultrasound of the thyroid showed a large gland with signs of inflammation and hyperperfusion, but no nodules. A treatment with 15 mg thiamazole daily was started. The management was complicated by constantly high fT3 levels, although increasing doses of thiamazole were administered and times of non-compliance. Thyroxine supplementation was needed after 6 months to normalize fT4 levels.

A year later the girl presented with clinical recurrence of GD. As the response to medical treatment was not satisfactory further treatment options were discussed. The progressive goiter and aggravating exophthalmos were the reason to opt for the surgical excision of the thyroid in a high volume center.

During thyroidectomy a small thyroid nodule was found in the left lower lobe of the enlarged gland (69 g; right lobe: 7.2×4.3×2.6 cm; left lobe 7×4.2×2.5 cm). A papillary thyroid carcinoma measuring 1.8×0.8×0.8 cm with penetration of the isthmus and the thyroid capsule was diagnosed by histology. Up to 0.1 cm infiltration into the perithyroid soft tissue and lymphatic vessels was detected. The surrounding thyroid tissue showed increased endocrine activity and signs of chronic inflammation in agreement with GD.

To assess tumor stage the patient was referred to our hospital. Sonography revealed enlarged cervical lymph nodes. On Iodine-123 scintigraphy lymph nodes around the trachea and in the left cervical region showed iodine tracer uptake ­indicating metastases ([Fig. 1]). No further evidence of distant metastases was detected.

Zoom Image
Fig. 1 a Planar iodine-123 scintigraphy after thyroidectomy revealed focal iodine uptake in left cervical and paratracheal regions (arrows), subsequently histologically proven as lymph node metastasis. b, c Diagnostic iodine-131 neck and thorax SPECT/CT two months after cervical lymphadenectomy and radioiodine ablation therapy raised suspicion of recurrent left cervical lymph node metastasis.

A multidisciplinary team discussed therapy options and recommended cervical lymphadenectomy prior to radioiodine ablative therapy. The patient was started on triiodothyronine. Clinical symptoms of hyperthyroidism occurred before a complete suppression of TSH could be achieved. A systematic cervical lymphadenectomy (K1a, K1b, K3) was performed 3 months after the initial surgery. 5 of 37 excised lymph nodes showed metastases. 3 were found in the central compartment opposite to the primary tumor and 2 in the left upper lateral ­compartment. The patient developed a superficial wound infection 10 days postoperatively treated with local antiseptic swathes and oral antibiotics. Hypoparathyroidism and laryngeal nerve lesion could be excluded ([Table 1]).

Table 1 Thyroid function, tumor markers and calcium after diagnosis of thyroid cancer.

Date

TSH (mU/l)

fT3 (pmol/l)

fT4 (pmol/l)

TG (ng/ml)

TG-Ab (U/ml)

TR-Ab (U/l)

Calcium (mmol/l)

physiological range

0.51–4.6

4.42–7.9

12–22

1.4–78

<33

(<1.8)

2.12–2.52

target value

<0.1

<1

06/13

pre-thyroidectomy

0.035 (0.4–4)

8.69 (2.8–7.7)

14.3 (10.3–24.5)

24.29

2.32

06/13

post-thyroidectomy

3.15

1.95

5.35

267.4

16.2

2.46

07/13

13.1

8.82

2.06

128.3

20.6

21.99

08/13

4.77

10.49

1.48

66.61

09/13

54.4

<0.4

0.87

137

18.7

19.63

2.22

10/13

lymphadenectomy

34.3

<0.4

0.94

32.6

16.1

2.37

10/13

radioablative therapy

>100

65.58

20

11/13

3.44

4.99

21.42

9.85

<10

01/14

0.33

4.84

19.13

10.92

12.9

2.35

01/14

pre-scintigraphy

>100

15.99

10.6

01/14

post-scintigraphy

28.1

17.54

10.1

02/14

1.34 (0.51–4-6)

5.11 (4.42–7.9)

26.07

(12–22)

12.45

12.4

4.72

2.41

Diagnostic procedures and interventions are indicated in the second column. After thyroidectomy the patient was treated with triiodithyronine, therefore fT4 values are ­beneath the physiological range between June and November 2013. Afterwards substitution with thyroxine with 137.5 µg daily, adjusted to 150 µg in January 2014 was started.

TSH=thyroidea stimulating hormone, fT3=free triiodine, fT4=free thyroxine, TR-Ab=TSH receptor antibody, TG-Ab=thyroglobulin-antibody, TG=thyroglobulin

10 days after surgery the patient underwent ablative radioiodine therapy (131I 3.579 GBq) without complications. A post therapy whole body radioiodine scan showed intense iodine uptake pretracheally, in the thyroid bed and along the thyroglossal duct, attributed to thyroid remnants. No signs of further metastases were found. The patient was started on hormone substitution with 137.5 µg thyroxine later adjusted to 150 µg daily.

At follow-up 2 months later a radioiodine scan revealed pathological tracer uptake in 2 cervical regions one in the previous left thyroid bed and one lateral of the left carotid artery, correlated to hypodense lesions on sonography. An elevated thyroglobulin level (17.54 ng/ml, TSH>100 mU/l) substantiated the suspicion of tumor metastases ([Table 1]). The surgical excision of the two lesions was recommended but the girl and her family refused to a third surgical approach. Therefore a second course of radioiodine ablation is planned.