thyroid hemiagenesis associated with papillary thyroid ... · ectopic thymus at thyroid bed, and...
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pISSN: 2384-3799 eISSN: 2466-1899
Int J Thyroidol 2016 May 9(1): 43-46
http://dx.doi.org/10.11106/ijt.2016.9.1.43
Received October 21, 2015 / Revised January 25, 2016 / Accepted February 22, 2016
Correspondence: Jee Soo Kim, MD, PhD, Division of Breast and Endocrine Surgery, Department of Surgery, Samsung
Medical Center, Sungkyunkwan University School of Medicine, 81 Irwon-ro, Gangnam-gu, Seoul 06351, Korea
Tel: 82-2-3410-0253, Fax: 82-2-3410-6982, E-mail: [email protected]
Copyright ⓒ 2016, the Korean Thyroid Association. All rights reserved.
This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creative-
commons.org/licenses/by-nc/4.0/), which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the
original work is properly cited.
Thyroid Hemiagenesis Associated with Papillary Thyroid Carcinoma
Inhye Park, Jun Ho Choi, Jung-Han Kim and Jee Soo KimDivision of Breast and Endocrine Surgery, Department of Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
Thyroid hemiagenesis is a rare congenital anomaly that is caused by a developmental defect of a thyroid.
Previous reports indicate that thyroid cancer associated with hemiagenesis is extremely rare. A 47-year-old
woman presented with single nodule in the right thyroid gland that was incidentally detected during a routine
medical checkup. Ultrasonography showed a 1.5×1.2 cm sized ill-defined irregular hypoehoic nodule in the right
thyroid and the isthmus was present. However, the left thyroid was not seen and thyroid was disconnected
at left paraisthmic area. Fine-needle aspiration cytology confirmed that the right thyroid nodule was papillary
thyroid carcinoma. Total thyroidectomy with bilateral central compartment node dissection was performed.
Permanent pathologic finding was 1.3×1 cm sized classical type papillary thyroid carcinoma with nodular
hyperplasia. There was extensive lymphatic invasion and 3 metastatic lymph nodes out of 4 in central
compartment. In conclusion, although thyroid hemiagenesis associated with thyroid carcinoma is extremely
rare, treatment strategy is not different with patients with normal anatomy. And the possibility of developing
a thyroid carcinoma should be considered in patients with hemiagenesis. Furthermore, it requires awareness
of anatomical difference around the thyroid gland during operation.
Key Words: Thyroid hemiagenesis, Thyroid cancer, Papillary thyroid carcinoma
Introduction
Thyroid hemiagenesis is an uncommon congenital
anomaly that is caused by a developmental defect of
a thyroid or failure of its precursor to migrate to the
normal location.1,2)
The prevalence of thyroid hemia-
genesis is reported as 0.05-0.2%.3-5)
Its occurrence
is mostly concomitant other pathologic conditions such
as Graves’ disease, multinodular goiter, and thyroiditis.
Previous reports indicate that thyroid cancer asso-
ciated with hemiagenesis is extremely rare.3) In this
study, we presented a case of hemiagenesis of thyroid
associated with papillary carcinoma.
Case Report
A 47-year-old woman presented with single nod-
ule in the right thyroid gland that was incidentally de-
tected during a routine medical checkup. There was
no history of previous radiation to the neck or any
other history of thyroid disease. Her older sister was
diagnosed papillary thyroid carcinoma without con-
genital anomaly of thyroid. Clinical examination re-
vealed no cervical lymphadenopathy. Ultrasonography
showed a 1.5×1.2 cm sized ill-defined irregular hy-
poechoic nodule in the right thyroid and the isthmus
was present. However, the left thyroid was not seen
and thyroid was disconnected at left paraisthmic area
Inhye Park, et al
Vol. 9, No. 1, 2016 44
Fig. 1. Ultrasonography of the thyroid gland showing one hypoechoic nodule (arrow) in the right thyroid (A) and no thyroid tissue
in the left thyroid (B).
Fig. 2. Operative finding of specimen showing absence of left lobe.
(Fig. 1). Fine-needle aspiration cytology confirmed that
the right thyroid nodule was papillary thyroid carcino-
ma; in addition, BRAFV600E mutation was not detected.
Neck CT, MRI or thyroid scan were not performed.
The patient was biochemically euthyroid (thyroid stim-
ulating hormone [TSH]: 1.57 μIU/mL [normal range,
0.3-6.0 μIU/mL], free T4: 1.25 ng/dL [normal range,
0.79-1.86 ng/dL], T3: 104.3 ng/dL [normal range,
76-190 ng/dL, Calcitonin: 7.8 pg/mL [normal range,
1.9-9.6 ng/dL]).
Total thyroidectomy with bilateral central compart-
ment node dissection was performed (Fig. 2). Thyroid
nodule was hard in the right lobe and gross perithyr-
oidal invasion was not detected. The left superior and
inferior parathyroid glands were located on the tra-
cheoesophageal groove and preserved in situ.
Because thyroid hemiagenesis is rare, extent of lymph
node dissection of thyroid carcinoma in patient with
hemiagenesis is still unclear. But in this patient, meta-
static papillary carcinoma of right central lymph node
was found in frozen biopsy. We considered risk of
contralateral central lymph node metastasis and per-
formed contralateral central lymph node dissection.
Permanent pathologic finding was 1.3×1 cm sized
classical type papillary thyroid carcinoma with nodular
hyperplasia. There was extensive lymphatic invasion
and 3 metastatic lymph nodes out of 4 in central com-
partment (right central lymph node 2/3, left central
lymph node 1/1). Postoperative TSH was 1.26 μIU/ml
(normal range, 0.3-6.0 μIU/ml) and free T4 was 1.85
ng/dL with levothyroxine 150 ug per day (normal
range, 0.79-1.86 ng/dL). Parathyroid hormone level
was 41.1 pg/dL (normal, 11.0-62.0).
Discussion
Thyroid hemiagenesis is a rare congenital anomaly
that is caused by a developmental defect of a thyroid
or failure of its precursor to migrate to the normal
location.1,2)
It was first described by Handfield-Jones
in 1866. The prevalence of thyroid hemiagenesis is re-
ported as 0.05-0.2%, and is more frequently found in
women. Absence of the left lobe is more common and
absence of the isthmus occurs in 50% of cases.5,6)
But,
Thyroid Hemiagenesis with PTC
45 Int J Thyroidol
Table 1. Thyroid hemiagenesis associated with thyroid cancer
Study Age SexSite of thyroid
hemiagenesisThyroid tumor Isthmus
Hanburger et al.14)
14 F Left PTC＋FTC Absent
Harada et al.15)
74 F Right PTC Present
Greening et al.16)
51 F Left PTC Absent
Khatri et al.11)
41 F Right PTC Present
McHenry et al.10)
58 F Left FTC Present
Shaha et al.12)
30 F Right PTC Present
Park et al.17)
43 F Right PTC Unknown
Huang et al.8) 47 F Right PTC Present
Pizzini et al.4) 54 M Left PTC Present
Lee et al.3) 69 F Left PTC Unknown
Kim et al.9) 56 F Left FTC Present
Park et al.13)
29 F Right PTC Present
Nam et al.18)
53 F Right PTC Absent
Wang et al.5) 49 F Right MTC Absent
60 F Left PTC Present
Karatag et al.19)
59 F Left PTC Unknown
Campenni et al.20)
36 M Left PTC Absent
Park (present report) 47 F Left PTC Present
FTC: follicular thyroid carcinoma, MTC: medullary thyroid carcinoma, PTC: papillary thyroid carcinoma
the true prevalence of this congenital abnormality is dif-
ficult to verify, because the absence of one thyroid lobe
alone usually does not lead to clinical manifestations.
The diagnosis is frequently incidental in the setting of
concomitant thyroid disorders.7)
In the remnant thyroid lobe, pathologic conditions
including follicular adenoma, multinodular goiter, chronic
thyroiditis and Graves’ disease are common.2-4,8)
However, thyroid hemiagenesis associated with papil-
lary thyroid carcinoma is extremely rare with only 18
patients reported including 14 papillary carcinoma, 2
follicular carcinoma, 1 medullary carcinoma, 1 mixed
papillary and follicular carcinoma. Sixteen patients
were women and hemiagenesis occurred on the left
in 11 cases and the right in 7 cases.5) Mean age was
48.3 years old (range, 14-74), without significant dif-
ference from patients with thyroid cancer, except de-
velopment defect. The isthmus could be found in 10
patients (Table 1).4,5,8-13)
Hemiagenesis is usually asymptomatic, if not con-
comitant with another condition causing hyper- or
hypothyroidism. It is diagnosed by imaging methods
including ultrasonography, CT, MRI and/or thyroid
scanning.3,5)
Treatment strategy for thyroid carcinoma
patients with hemiagenesis was not different in patients
with anatomically normal thyroid gland.3,5)
However,
the location of parathyroid gland and the course of re-
current laryngeal nerve could differ from the normal
thyroid gland, hence, greater intra-operative care is
required from the surgeon.
In conclusion, although thyroid hemiagenesis asso-
ciated with thyroid carcinoma is extremely rare, the
possibility of developing a thyroid carcinoma should be
considered in patients with hemiagenesis. Further-
more, it requires awareness of anatomical difference
around the thyroid gland during operation.
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