C A S E R EP O R T
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
Thyroid Hemiagenesis Associated with Papillary Thyroid Carcinoma Inhye Park, Jun Ho Choi, Jung-Han Kim and Jee Soo Kim Division 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
Case Report
Thyroid hemiagenesis is an uncommon congenital
A 47-year-old woman presented with single nod-
anomaly that is caused by a developmental defect of
ule in the right thyroid gland that was incidentally de-
a thyroid or failure of its precursor to migrate to the
tected during a routine medical checkup. There was
normal location.
1,2)
The prevalence of thyroid hemia-
no history of previous radiation to the neck or any
3-5)
Its occurrence
other history of thyroid disease. Her older sister was
is mostly concomitant other pathologic conditions such
diagnosed papillary thyroid carcinoma without con-
as Graves’ disease, multinodular goiter, and thyroiditis. Previous reports indicate that thyroid cancer asso-
genital anomaly of thyroid. Clinical examination re-
genesis is reported as 0.05-0.2%.
3)
vealed no cervical lymphadenopathy. Ultrasonography
In this
showed a 1.5×1.2 cm sized ill-defined irregular hy-
study, we presented a case of hemiagenesis of thyroid
poechoic nodule in the right thyroid and the isthmus
associated with papillary carcinoma.
was present. However, the left thyroid was not seen
ciated with hemiagenesis is extremely rare.
and thyroid was disconnected at left paraisthmic area 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://creativecommons.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.
43
Inhye Park, et al
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. 1). Fine-needle aspiration cytology confirmed that the right thyroid nodule was papillary thyroid carcinoma; in addition, BRAFV600E mutation was not detected. Neck CT, MRI or thyroid scan were not performed. The patient was biochemically euthyroid (thyroid stimulating 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 compartment node dissection was performed (Fig. 2). Thyroid nodule was hard in the right lobe and gross perithyroidal invasion was not detected. The left superior and inferior parathyroid glands were located on the tracheoesophageal groove and preserved
in
Fig. 2. Operative finding of specimen showing absence of left lobe.
situ.
Because thyroid hemiagenesis is rare, extent of lymph
ng/dL with levothyroxine 150 ug per day (normal
node dissection of thyroid carcinoma in patient with
range, 0.79-1.86 ng/dL). Parathyroid hormone level
hemiagenesis is still unclear. But in this patient, meta-
was 41.1 pg/dL (normal, 11.0-62.0).
static papillary carcinoma of right central lymph node
Discussion
was found in frozen biopsy. We considered risk of contralateral central lymph node metastasis and performed contralateral central lymph node dissection.
Thyroid hemiagenesis is a rare congenital anomaly
Permanent pathologic finding was 1.3×1 cm sized
that is caused by a developmental defect of a thyroid
classical type papillary thyroid carcinoma with nodular
or failure of its precursor to migrate to the normal
hyperplasia. There was extensive lymphatic invasion
location.
and 3 metastatic lymph nodes out of 4 in central com-
in 1866. The prevalence of thyroid hemiagenesis is re-
partment (right central lymph node 2/3, left central
ported as 0.05-0.2%, and is more frequently found in
lymph node 1/1). Postoperative TSH was 1.26 μIU/ml
women. Absence of the left lobe is more common and
(normal range, 0.3-6.0 μIU/ml) and free T4 was 1.85
absence of the isthmus occurs in 50% of cases.
1,2)
It was first described by Handfield-Jones
5,6)
Vol. 9, No. 1, 2016
But, 44
Thyroid Hemiagenesis with PTC Table 1. Thyroid hemiagenesis associated with thyroid cancer Study
Age
Sex
14 74 51 41 58 30 43 47 54 69 56 29 53 49 60 59 36 47
F F F F F F F F M F F F F F F F M F
14)
Hanburger et al. 15) Harada et al. 16) Greening et al. 11) Khatri et al. 10) McHenry et al. 12) Shaha et al. Park et al.17) 8) Huang et al. 4) Pizzini et al. Lee et al.3) 9) Kim et al. 13) Park et al. Nam et al.18) 5) Wang et al.
Karatag et al.19) 20) Campenni et al. Park (present report)
Site of thyroid hemiagenesis Left Right Left Right Left Right Right Right Left Left Left Right Right Right Left Left Left Left
Thyroid tumor
Isthmus
PTC＋FTC PTC PTC PTC FTC PTC PTC PTC PTC PTC FTC PTC PTC MTC PTC PTC PTC PTC
Absent Present Absent Present Present Present Unknown Present Present Unknown Present Present Absent Absent Present Unknown Absent Present
FTC: follicular thyroid carcinoma, MTC: medullary thyroid carcinoma, PTC: papillary thyroid carcinoma
the true prevalence of this congenital abnormality is dif-
with anatomically normal thyroid gland.3,5) However,
ficult to verify, because the absence of one thyroid lobe
the location of parathyroid gland and the course of re-
alone usually does not lead to clinical manifestations.
current laryngeal nerve could differ from the normal
The diagnosis is frequently incidental in the setting of
thyroid gland, hence, greater intra-operative care is
concomitant thyroid disorders.
7)
required from the surgeon.
In the remnant thyroid lobe, pathologic conditions
In conclusion, although thyroid hemiagenesis asso-
including follicular adenoma, multinodular goiter, chronic
ciated with thyroid carcinoma is extremely rare, the
thyroiditis and Graves’ disease are common. However, thyroid hemiagenesis associated with papil-
2-4,8)
possibility of developing a thyroid carcinoma should be
lary thyroid carcinoma is extremely rare with only 18
more, it requires awareness of anatomical difference
patients reported including 14 papillary carcinoma, 2
around the thyroid gland during operation.
considered in patients with hemiagenesis. Further-
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 difference from patients with thyroid cancer, except development defect. The isthmus could be found in 10 patients (Table 1).
4,5,8-13)
Hemiagenesis is usually asymptomatic, if not concomitant 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 45 Int J Thyroidol
References 1) Lipin R, Kandil E. Thyroid hemiagenesis. J La State Med Soc 2012;164(4):205-6. 2) Sari O, Ciftci I, Toru M, Erbas B. Thyroid hemiagenesis. Clin Nucl Med 2000;25(10):766-8. 3) Lee YS, Yun JS, Jeong JJ, Nam KH, Chung WY, Park CS. Thyroid hemiagenesis associated with thyroid adenomatous hyperplasia and papillary thyroid carcinoma. Thyroid 2008; 18(3):381-2. 4) Pizzini AM, Papi G, Corrado S, Carani C, Roti E. Thyroid hemiagenesis and incidentally discovered papillary thyroid cancer: case report and review of the literature. J Endocrinol Invest 2005;28(1):66-71. 5) Wang J, Gao L, Song C. Thyroid hemiagenesis associated with medullary or papillary carcinoma: report of cases. Head
Inhye Park, et al
Neck 2014;36(11):E106-11. 6) Maiorana R, Carta A, Floriddia G, Leonardi D, Buscema M, Sava L, et al. Thyroid hemiagenesis: prevalence in normal children and effect on thyroid function. J Clin Endocrinol Metab 2003;88(4):1534-6. 7) Shabana W, Delange F, Freson M, Osteaux M, De Schepper J. Prevalence of thyroid hemiagenesis: ultrasound screening in normal children. Eur J Pediatr 2000;159(6):456-8. 8) Huang SM, Chen HD, Wen TY, Kun MS. Right thyroid hemiagenesis associated with papillary thyroid cancer and an ectopic prelaryngeal thyroid: a case report. J Formos Med Assoc 2002;101(5):368-71. 9) Kim B, Kim IA. Means of monitoring the course of postoperative inflammatory processes in the abdominal cavity. Vestn Akad Med Nauk SSSR 1981(6):46-8. 10) McHenry CR, Walfish PG, Rosen IB, Lawrence AM, Paloyan E. Congenital thyroid hemiagenesis. Am Surg 1995; 61(7):634-8; discussion 8-9. 11) Khatri VP, Espinosa MH, Harada WA. Papillary adenocarcinoma in thyroid hemiagenesis. Head Neck 1992;14(4):312-5. 12) Shaha AR, Gujarati R. Thyroid hemiagenesis. J Surg Oncol 1997;65(2):137-40. 13) Park J-Y, Kim SJ, Cho YU. Thyroid hemiagenesis associated
14)
15) 16) 17)
18)
19)
20)
with micropapillary thyroid carcinoma. J Korean Surg Soc 2010;78(2):116-8. Hamburger JI, Hamburger SW. Thyroidal hemiagenesis. Report of a case and comments on clinical ramifications. Arch Surg 1970;100(3):319-20. Harada T, Nishikawa Y, Ito K. Aplasia of one thyroid lobe. Am J Surg 1972;124(5):617-9. Greening WP, Sarker SK, Osborne MP. Hemiagenesis of the thyroid gland. Br J Surg 1980;67(6):446-8. Park SG, Ryu JW, Myung NH. Thyroid hemiagenesis and ectopic thymus at thyroid bed, and papillary cancer in opposite thyroid lobe with hyperthyroidism. J Korean Surg Soc 2000; 58(3):433-7. Nam YM, Park JS, Na KH, Ahn D. A case of thyroid hemiagenesis with concurrent papillary thyroid carcinoma. Korean J Otorhinolaryngol-Head Neck Surg 2011;54(8):557-9. Karatag GY, Albayrak ZK, Onay HK, Karatag O, Peker O. Coexistence of thyroid hemiagenesis, nodular goitre and papillary carcinoma. Kulak Burun Bogaz Ihtis Derg 2013;23(2):115-8. Campenni A, Giovinazzo S, Curto L, Giordano E, Trovato M, Ruggeri RM, et al. Thyroid hemiagenesis, Graves' disease and differentiated thyroid cancer: a very rare association: case report and review of literature. Hormones (Athens) 2015;14(3):451-8.
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