Loss of imprinting of the human-specific imprinted gene ZNF597 causes prenatal growth retardation and dysmorphic features: implications for phenotypic overlap with Silver-Russell syndrome.

BACKGROUND: ZNF597, encoding a zinc-finger protein, is the human-specific maternally expressed imprinted gene located on 16p13.3. The parent-of-origin expression of ZNF597 is regulated by the ZNF597:TSS-DMR, of which only the paternal allele acquires methylation during postimplantation period. Overexpression of ZNF597 may contribute to some of the phenotypes associated with maternal uniparental disomy of chromosome 16 (UPD(16)mat), and some patients with UPD(16)mat presenting with Silver-Russell syndrome (SRS) phenotype have recently been reported. METHODS: A 6-year-old boy presented with prenatal growth restriction, macrocephaly at birth, forehead protrusion in infancy and clinodactyly of the fifth finger. Methylation, expression, microsatellite marker, single nucleotide polymorphism array and trio whole-exome sequencing analyses were conducted. RESULTS: Isolated hypomethylation of the ZNF597:TSS-DMR and subsequent loss of imprinting and overexpression of ZNF597 were confirmed in the patient. Epigenetic alterations, such as UPD including UPD(16)mat and other methylation defects, were excluded. Pathogenic sequence or copy number variants affecting his phenotypes were not identified, indicating that primary epimutation occurred postzygotically. CONCLUSION: We report the first case of isolated ZNF597 imprinting defect, showing phenotypic overlap with SRS despite not satisfying the clinical SRS criteria. A novel imprinting disorder entity involving the ZNF597 imprinted domain can be speculated.

Impact of the practical guideline on appropriate usage of antimicrobial treatments for surgical site infections in reconstructive surgery of head and neck cancer.

OBJECTIVES: In 2016, Japanese Society of Chemotherapy and Japan Society for Surgical Infection presented the practical guideline for appropriate usage of antimicrobial agents to prevent postoperative infections. This study aims to exhibit the validity of the guideline as a series of effective strategies for prevention of surgical site infections (SSIs) during reconstructive surgery of the head and neck cancer. METHODS: We retrospectively evaluated patients who underwent head and neck reconstructive surgery with free or pedicle flaps in a single institute in Japan between July 2010 and July 2020. We evaluated the incidence of SSIs, patient backgrounds, and microbiological characteristics on the basis of antimicrobial prophylaxis recommended by the guideline. RESULTS: Enrolled in this study were 102 patients in our institution who underwent head and neck reconstructive surgery with free or pedicle flaps between July 2010 and July 2020. In the period between January 2018 to July 2020 after the SSI guideline was advocated (SSI guideline period), the ratio of administration of sulbactam/ampicillin (SBT/ABPC) was significantly higher (P < 0.001) and the duration of prophylactic antimicrobial treatment was significantly shorter than in the period between July 2010 to December 2017 before the SSI guideline was advocated (Pre-SSI guideline period) (P < 0.001). Incidence of SSIs were similar, even when antibiotic use was changed to be short-term single-agent administration in accordance with the practical guideline. CONCLUSIONS: Adherence to the current Japanese practical guideline on appropriate antimicrobial prophylaxis for SSIs can shorten the duration of usage of antimicrobial treatment without increasing the risk for occurrence of SSIs.

A case report of synchronous bilateral tonsillar carcinoma associated with human papilloma virus.

The case of a 69-year-old man with bilateral synchronous tonsillar carcinoma is reported. The patient complained of nasal closure, strange voice, and discomfort in his pharynx when he was admitted to the Department of Otolaryngology Head and Neck Surgery at Wakayama Medical University, Wakayama, Japan, in March 2017. The palatine tonsils were enlarged and the surface was irregular. Left cervical lymphadenopathy was also evident. Histological examination from both tonsils was performed and bilateral tonsillar squamous cell carcinoma was diagnosed. PCR analysis showed the same HPV-DNA pattern from bilateral tonsils. Concurrent chemoradiotherapy was performed. Total 70 Gy of irradiation (2Gy/day×35 day) was applied to bilateral tonsillar tumours and upper neck. Follow up was conducted every three months and the patient was free of recurrence for three years. Patient's informed consent was taken to publish the case report.

Update of the genotype and phenotype of KMT2D and KDM6A by genetic screening of 100 patients with clinically suspected Kabuki syndrome.

Kabuki syndrome is characterized by a variable degree of intellectual disability, characteristic facial features, and complications in various organs. Many variants have been identified in two causative genes, that is, lysine methyltransferase 2D (KMT2D) and lysine demethylase 6A (KDM6A). In this study, we present the results of genetic screening of 100 patients with a suspected diagnosis of Kabuki syndrome in our center from July 2010 to June 2018. We identified 76 variants (43 novel) in KMT2D and 4 variants (3 novel) in KDM6A as pathogenic or likely pathogenic. Rare variants included a deep splicing variant (c.14000-8C>G) confirmed by RNA sequencing and an 18% mosaicism level for a KMT2D mutation. We also characterized a case with a blended phenotype consisting of Kabuki syndrome, osteogenesis imperfecta, and 16p13.11 microdeletion. We summarized the clinical phenotypes of 44 patients including a patient who developed cervical cancer of unknown origin at 16 years of age. This study presents important details of patients with Kabuki syndrome including rare clinical cases and expands our genetic understanding of this syndrome, which will help clinicians and researchers better manage and understand patients with Kabuki syndrome they may encounter.

False-positive staining of thyroglobulin distinguished from mixed medullary and follicular thyroid carcinoma by duplex in situ hybridization.

Medullary thyroid carcinoma (MTC) may mimic mixed medullary and follicular thyroid carcinoma (MMFTC). MTC originates from para-follicular cells, while MMFTC is an uncommon tumor characterized by coexistence of follicular and para-follicular cell-derived tumor populations. A 35-year-old woman was diagnosed with MTC but showed a hot nodule in thyroid scintigraphy. The tumor included diffusely-spread follicular lesions within it, which were immunostained with thyroglobulin and calcitonin. Immunofluorescence showed the presence of several tumor cells that were double-stained with thyroglobulin and calcitonin. To clarify whether or not the tumor was MMFTC, we used duplex in situ hybridization (ISH). Thyroglobulin and calcitonin-related polypeptide alpha mRNA were not expressed together in a single cell, so we suspected false-positive staining of tumor cells with thyroglobulin. To make comparisons with other follicular lesions in MTC, we searched our hospital database. Five cases within a ten-year period had been pathologically diagnosed as MTC. All had follicular lesions in the tumor, but unlike the other case, they were peripherally localized. Dual differentiation into follicular or para-follicular tumor cells was not indicated by either immunofluorescence or duplex ISH. Compared with the case suspected to be MMFTC, there was only mild invasion of tumor cells into the follicular epithelium. The extent of follicular lesions and invasiveness of tumor cells may be associated with pseudo-staining of thyroglobulin in MTC. Duplex ISH can distinguish MTC that are stained with thyroglobulin from MMFTC.

Health insurance status and survival among patients with head and neck cancer in Japan.

BACKGROUND: Healthcare systems vary among countries, and in many countries, insurance and economic statuses significantly impact the mortality associated with head and neck squamous cell carcinoma (HNSCC). Japan prides itself on its unique healthcare and health insurance system, which provides equal coverage and healthcare access with low individual payments to most citizens. Additionally, citizens in poverty are covered by insurance for the poor (public assistance) and receive medical and other types of assistance. Hence, they have no barriers to healthcare services. This study aimed to determine the impact of health insurance status on mortality in Japanese patients with HNSCC. METHODS: We reviewed 409 patients with HNSCC, using the Kaplan-Meier method to estimate overall survival. The association between insurance status and disease stage at diagnosis was analyzed via logistic regression. Cox and Fine-Gray proportional hazard models were employed to investigate the impact of insurance status on survival. RESULTS: The public assistance and other insurances groups did not significantly differ in clinical stage distribution. The 5-year overall survival, cumulative incidence of HNSCC death, and cumulative incidence of other death rates were 63.3 and 59.1 %, 27.0 and 31.8 %, and 10.3 and 9.7 % for the public assistance and other insurances groups, respectively. The adjusted subdistribution hazard ratio for the association between public assistance and HNSCC death was 0.73 (95 % confidence interval 0.44-1.21). CONCLUSIONS: The demonstrated non-inferiority of public assistance regarding HNSCC-specific mortality indicates the equality of healthcare in Japan, irrespective of the insurance status, and the superiority of the Japanese healthcare system.

[A Case of Hereditary Medullary Thyroid Cancer (MEN2A/FMTC) Diagnosed at the Time of Recurrence].

We report a 42-year-old man with hereditary medullary thyroid cancer (multiple endocrine neoplasia, MEN2A/familial medullary thyroid carcinoma, FMTC), which was diagnosed at the time of tumor recurrence. He had a past history of a left thyroidectomy with neck dissection 7 years previously. A RET gene analysis revealed a point mutation (codon 618), and we diagnosed him as having hereditary medullary thyroid cancer. We resected the recurrent tumor in the right thyroid lobe together with performing a right lateral and central neck dissection. A RET gene analysis should be performed for patients with medullary thyroid cancer. When a RET gene mutation is present, a total thyroidectomy must be performed for the medullary thyroid cancer.

Pure duplication of 19p13.3.

Chromosomal abnormalities involving 19p13.3 have rarely been described in the published literature. Here, we report on a girl with a pure terminal duplication of 6.1 Mb on 19p13.3, caused by an unbalanced translocation der(19)t(10;19)(qter;p13.3)dn. Her phenotype included severe psychomotor developmental delay, skeletal malformations, and a distinctive facial appearance, similar to that of a patient previously reported by Lybaek et al. [Lybaek et al. (2009); Eur J Hum Genet 17:904-910]. These results suggest that a duplication of >3 Mb at the terminus of 19p13.3 might represent a distinct chromosomal syndrome.

Follicular thyroid cancer in children and adolescents: clinicopathologic features, long-term survival, and risk factors for recurrence.

Children and adolescents represent 1-1.5% of all patients with thyroid cancer (TC). The vast majority of TC in children and adolescents is papillary TC; follicular TC (FTC) is exceedingly rare. In this study, we evaluate the clinical and pathological features of FTC in children and adolescents. We also report the risk factors for post-operative tumor recurrence and the associated outcomes. Twenty children and adolescents (under 21 years old) with FTC have been treated and followed at Noguchi Thyroid Clinic and Hospital Foundation since 1946. All patients underwent surgery (lobectomy, 11; subtotal thyroidectomy, 8; and total thyroidectomy, 1), and 8 patients received postoperative external beam radiation therapy. The incidence of FTC in children and adolescents was 1.9% among all FTC patients treated in our hospital. Histopathology revealed vascular and capsular invasion in 9 and 20 patients, respectively. The tumor recurrence rate in FTC with vascular invasion is significantly higher than in those without it (p = 0.038). No other factors were significant. Patients with recurrences were treated with completion thyroidectomy and ¹³¹I radioactive iodine therapy. There were no significant differences in the rates of disease-free survival or cause-specific survival when pediatric/adolescent FTC patients were compared to adults with FTC. FTC is very rare among children and adolescents, but the outcomes are similar to those observed among adults. Vascular invasion is poor prognostic indicator in pediatric/adolescent FTC patients.

Surgical strategy of locally advanced differentiated thyroid cancer.

Approximately 20% of patients with differentiated thyroid cancer (DTC) have direct tumor extension with invasion of the surrounding tissues such as the larynx, trachea, esophagus, or recurrent laryngeal nerve. Recent progress of molecular-targeted therapy, such as the use of tyrosine kinase inhibitors, improves survival outcome in patients with advanced DTC. However, induction of tyrosine kinase inhibitors for locally-advanced DTC has presented novel fatal adverse events including fistula in patients with infiltration toward to the trachea, pharynx and esophagus, and fatal bleeding in patients with great vessel invasion. Surgery therefore still has an important role in DTC management, particularly in local control. The surgical strategy for laryngeal/tracheal invasion, which commonly occurs by DTC, is decided according to the extension (depths and area) of the tumor. The "shave procedure" is performed when the tumor has superficially invaded the larynx/trachea. However, intra-luminal extension requires resection and reconstruction of the larynx/trachea wall. Large veins, such as the internal jugular vein and the subclavian vein, are also frequently directly invaded by DTC. Three types of jugular vein reconstruction have been advocated to avoid fatal complications according to bilateral jugular vein ligation. The majority of carotid artery invasion by DTC can be managed with tumor resection of the sub-adventitial layer without reconstruction surgery using an artificial vessel. In this review article, we examine surgery for advanced DTC, showing the surgical strategy toward DTC that has invaded the laryngotracheal, recurrent laryngeal nerve, esophagus/hypopharynx, or great vessels.

Outpatient ablation treatment with two doses of 30 mCi of radioactive iodine for non-low-risk papillary thyroid cancer.

Outpatient ablation therapy with low-dose radioactive iodine (RAI) is applied to non-low-risk papillary thyroid cancer patients due to a chronic shortage of inpatient RAI treatment wards in Japan. We used the maximum dosage available for outpatient therapy of 30 mCi of RAI for ablation and diagnostic (Dx) whole-body scintigraphy (WBS). This study aimed to examine the significance of the second dose of 30 mCi. DxWBS was performed 6 months after ablation, and assessment of success or failure was performed 12 months after ablation. A second WBS was performed in the remaining RAI accumulation cases in the neck on DxWBS. The criteria for successful ablation was negative cervical accumulation on WBS, thyroid stimulating hormone-suppressed thyroglobulin (sup-Tg) below 1.0 ng?/?mL, and no increase in thyroglobulin antibody (TgAb) level. At the time of DxWBS, 35?/?68 cases met the successful criteria, and 45 cases achieved success at assessment. Sup-Tg values decreased significantly after ablation and decreased further after DxWBS in successful ablation cases, whereas those were not changed in ablation failure cases. Findings indicated that RAI used in DxWBS had therapeutic effects. It makes sense to use 30 mCi for DxWBS, given the current difficulty of inpatient ablation therapy with high-dose RAI. J. Med. Invest. 70 : 17-21, February, 2023.

Case Report: Solid variant of papillary thyroid carcinoma in a young adult with Turner syndrome with chronic thyroiditis.

Turner syndrome is associated with an increased risk of developing several neoplasms. In particular, a clinical feature of Turner syndrome with chronic thyroiditis implies a relationship with thyroid malignancies. We report a very rare case of a solid variant of papillary thyroid carcinoma that was identified during a follow-up of chronic thyroiditis in a 22-year-old woman with Turner syndrome. The patient had no notable history of radiation exposure. No genetic mutations relating to the occurrence of the solid variant of papillary thyroid carcinoma, including RET/PTC rearrangements and mutations in the BRAF or RAS, were detected by a gene panel test, namely, the Oncomine™ Dx Target test. To the best of our knowledge, this is the first report of a solid variant of papillary thyroid carcinoma in a young adult with Turner syndrome with chronic thyroiditis. Our case suggests that in patients with Turner syndrome, there may be different pathogeneses from those previously reported, including exposure to radiation or known genetic mutations for the development of a solid variant of papillary thyroid carcinoma.

12q14 microdeletion syndrome and short stature with or without relative macrocephaly.

Patients with 12q14 microdeletion can present with short stature with or without relative macrocephaly. When associated with relative macrocephaly, the phenotype resembles Silver-Russell syndrome (SRS). Short stature is attributable to haploinsufficiency of HMGA2, but a patient with a deletion at the HMGA2 locus only did not have macrocephaly. Hence, the presence of a separate locus for a relative macrocephaly phenotype was suggested. Herein, we present a girl with a 12q14 microdeletion involving the HMGA2 locus who had short stature but did not have macrocephaly. Inclusion and exclusion mappings based on a quantitative review of the degree of relative macrocephaly and the extent of the deletions in previously reported patients with a 12q14 microdeletion demonstrated a presumptive interval for relative macrocephaly spanning a few megabases. These results confirm that a deletion spanning both HMGA2 and this presumptive interval locus would cause an SRS-like phenotype.

Expression analysis of a 17p terminal deletion, including YWHAE, but not PAFAH1B1, associated with normal brain structure on MRI in a young girl.

Tyrosine 3-monooxygenase/tryptophan 5-monooxygenase activation protein, epsilon polypeptide (YWHAE), on chromosome 17p13.3, has been shown to play a crucial role in neuronal development. The deletion of YWHAE, but not platelet-activating factor acetylhydrolase, isoform 1b, subunit 1 (PAFAH1B1), underlies a newly recognized neurodevelopmental disorder, characterized by significant growth retardation, developmental delay/intellectual disability (DD/ID), distinctive facial appearance, and brain abnormalities. Here, we report on a girl with a terminal deletion of 17p13.3, including YWHAE but not PAFAH1B1, showing normal brain structure on MRI. She had mild developmental delay, a distinctive facial appearance, and severe growth retardation despite normal growth hormone levels, which was improved by growth hormone therapy. Expression analysis of YWHAE and PAFAH1B1 yielded results consistent with array CGH and FISH results. These results indicate that the dosage effect of YWHAE varies from severe to very mild structural brain abnormalities, and suggest that the expression of YWHAE is associated with a complex mechanism of neuronal development.

Clinical features, treatment, and long-term outcome of papillary thyroid cancer in children and adolescents without radiation exposure.

BACKGROUND: Cancer of the thyroid gland is rare in children and adolescents. A history of neck irradiation is a well-established risk factor for tumor development, and most previous reports focused on cases that were induced by radiation exposure. We present here a retrospective review of the clinical features, treatment, and long-term outcome of children and adolescents with papillary thyroid cancer (PTC) without a history of radiation exposure who were treated at our institution over a period of ~50 years. METHODS: We retrospectively investigated 142 PTC patients without an irradiation history who were younger than 20 years of age when treated from 1961 to 2005 (17 males and 125 females; mean age = 16.3 ± 2.7 years; follow-up = 21.8 ± 12.0 years). The clinicopathological results were evaluated using the medical records. Disease-free survival (DFS) and cause-specific survival (CSS) were assessed with the Kaplan-Meier method and compared with the log-rank test. Parametric analyses were performed using Student's t test and nonparametric analyses were performed using the Mann-Whitney U test. RESULTS: At diagnosis, three patients had distant lung metastasis and 33 had gross neck lymph node (LN) metastasis. All patients were treated with surgery (hemi/partial thyroidectomy in 45 patients, subtotal thyroidectomy in 85, total thyroidectomy in 12, no LN dissection in 50, central compartment dissection in 20, and modified radical neck dissection in 72), and postoperative external beam radiation therapy was administered to 59. Postoperative ablative therapy using I(131) was not performed in this series. Recurrence was found for regional LN (n = 25), lung (n = 9), remnant thyroid (n = 5), and others (n = 4). DFS and CSS at 40 years were 74.1 and 97.5%, respectively. DFS was significantly worse in patients aged <16 years with a family history of thyroid cancer, preoperative neck gross LN metastasis, maximum tumor diameter, and extrathyroidal invasion. Preoperative gross neck LN metastasis and distant metastasis at diagnosis were significant factors for CSS. No other factors contributed to DFS and CSS. When the clinical features of children and adolescents were compared, the incidence of preoperative gross neck LN metastasis and distant metastasis at diagnosis and tumors with a maximum diameter >10 mm were significantly higher in the children group than in the adolescent group. DFS was significantly shorter in the children group than in the adolescent group, but no significant difference was found in CSS between these two groups. CONCLUSIONS: The prognosis of PTC in children and adolescents is excellent, regardless of the extent of thyroidectomy and LN dissection. We recommend that only children or adolescents with preoperative gross neck LN metastasis and distant metastasis at diagnosis should be subjected to postoperative ablative therapy.

Proportion of malformations and genetic disorders among cases encountered at a high-care unit in a children's hospital.

Genetic disorders and birth defects account for a high percentage of the admissions in children's hospitals. Congenital malformations and chromosomal abnormalities are the most common causes of infant mortality. So their effects pose serious problems for perinatal health care in Japan, where the infant mortality is very low. This paper describes the reasons for admissions and hospitalization at the high-care unit (HCU) of a major tertiary children's referral center in Japan. We retrospectively reviewed 900 admission charts for the period 2007-2008 and found that genetic disorders and malformations accounted for a significant proportion of the cases requiring admission to the HCU. Further, the rate of recurrent admission was higher for patients with genetic disorders and malformations than for those with acquired, non-genetic conditions. Over the past 30 years, admissions attributed to genetic disorders and malformations has consistently impacted on children's hospital and patients with genetic disorders and malformations form a large part of this facility. These results reflect improvements in medical care for patients with genetic disorders and malformations and further highlight the large proportion of cases with genetic disorders, for which highly specialized management is required. Moreover, this study emphasizes the need for involvement of clinical geneticists in HCUs at children's hospitals.

A case of Sjögren-Larsson syndrome with minimal MR imaging findings facilitated by proton spectroscopy.

We present a 5-year-old girl who was ultimately diagnosed with Sjögren-Larsson syndrome (SLS). Although her MRI findings were minimal compared to previously published cases, prominent and characteristic abnormal lipid peaks on single-voxel proton MR spectroscopy ((1)H-MRS) facilitated the diagnosis. This case emphasizes the importance and usefulness of (1)H-MRS in diagnosing SLS.

The Role of Anatomical Imaging and Intraoperative Neuromonitoring (IONM) for Successful Prediction of a Nonrecurrent Laryngeal Nerve.

A nonrecurrent laryngeal nerve (NRLN) is a rare anatomical variant of laryngeal nerves that branches directly from the vagus nerve. The anatomical abnormality makes it difficult to identify the NRLN and results in high incidence of accidental nerve injury during surgery. A 76-year-old woman complained of swelling in the right side of her neck and visited our university hospital for further examination. Ultrasonography showed a right thyroid lobe mass with calcification and fine needle aspiration biopsy was classified as class III. Computed tomography revealed that the right subclavian artery branched directly from the descending aorta without branching from the brachiocephalic artery and ran behind the esophagus. Since it was afraid that the accidental injury of NRLN was likely to occur, a right thyroid lobe dissection using intraoperative neuromonitoring (IONM) was performed. After separating the connective tissue on the thyroid capsule from the right side of the trachea to the inferior pole laterally, the NRLN running across the level of the inferior margin of the cricoid cartilage was identified by using IONM 0.5 mA stimulation. After complete dissection of right thyroid lobe, we again stimulated the NRLN by 0.5 mA and the electromyographic response was confirmed. The pathological analysis confirmed nodular hyperplasia without malignancy; the condition was diagnosed as an adenomatous goiter. There was no vocal cord dysfunction and hoarseness after the surgery. IONM contributed to the prevention of NRLN injury during the surgery. We believe that it is important to confirm the presence or absence of an aberrant subclavian artery on preoperative imaging, and that IONM should be considered to identify the NRLN to prevent vocal cord paralysis if its presence is suspected.

Lateral approach contributes to shortened surgical time in video-assisted neck surgery (VANS) for thyroid nodule.

Background: Despite endoscopic thyroid surgery contributing to excellent cosmetic outcomes, it requires longer surgical time than open surgery. This study evaluates the factors associated with operative time in video-assisted neck surgery. Methods: We retrospectively reviewed patients who underwent hemithyroidectomy by video-assisted neck surgery at a single hospital between 2017 and 2021. The clinical parameters were evaluated: age, sex, body weight, body mass index, coexistence of Hashimoto thyroiditis, side of operation, surgical approach to thyroid (midline approach or lateral approach), operation time, amount of blood loss during surgery, maximum tumor diameter and tumor volume, and postoperative complications. These parameters were compared to investigate the association of operative time and surgical approach. Results: Seventy consecutive patients were enrolled in this study, the median age at operation was 50 years, and there were 6 (8.6%) men and 64 (91.4%) women. The median body weight and body mass index at operation were 56.6 kg and 21.5, respectively. Coexistence of Hashimoto thyroiditis was found in 17 (24.3%) patients. Significant differences were found between the groups with longer and shorter operation time than median 201 minutes in surgical approaches to the thyroid (P < .001) and the amount of bleeding during surgery (P = .039). There were no differences in other candidates between the groups. Median operation times were 242 minutes in midline approach and 131 minutes in lateral approach (P < .001). Conclusion: The lateral approach of video-assisted neck surgery to the thyroid can contribute to shortened surgical time in the case of benign thyroid nodules.

A case of concurrent occurrence of carcinoma showing thymus-like differentiation and follicular variant of papillary thyroid cancer in the same thyroid.

Carcinoma showing thymus-like differentiation (CASTLE) is a rare thyroid cancer. This is the first report of a case of concurrent occurrence of CASTLE with papillary thyroid carcinoma (PTC). A 66-year-old male patient had hoarseness with right vocal cord paralysis. Ultrasonography revealed a hypoechoic nodule in the inferior pole of the right thyroid lobe. Ultrasound-guided fine-needle aspiration cytology suggested differentiated thyroid cancer. The patient underwent total thyroidectomy with neck dissection. Pathological examination revealed two different thyroid cancers: a CASTLE and a follicular variant of PTC. Postoperative radiation therapy was performed. The patient was still alive after 5 year following the initial treatment without evidence of recurrence. The oncological management of patients with concurrent occurrence of different thyroid cancers should consider the biological behavior of both tumors.