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Enomoto K.,Noguchi Thyroid Clinic and Hospital Foundation | Enomoto Y.,Noguchi Thyroid Clinic and Hospital Foundation | Uchino S.,Noguchi Thyroid Clinic and Hospital Foundation | Yamashita H.,Noguchi Thyroid Clinic and Hospital Foundation | Noguchi S.,Noguchi Thyroid Clinic and Hospital Foundation
Endocrine Journal | Year: 2013

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. © The Japan Endocrine Society.


Sakurai A.,Shinshu University | Imai T.,Nagoya University | Kikumori T.,Nagoya University | Horiuchi K.,Tokyo Women's Medical University | And 7 more authors.
Clinical Endocrinology | Year: 2013

Objective Thymic neuroendocrine tumour (Th-NET) occurs in 2-5% of patients with MEN1 and has high malignant potency accompanying recurrence and distant metastasis. While Th-NET is recognized to develop predominantly in men and heavy smokers, a number of female patients have been reported in the literature. The objective of this study is to clarify the clinical features of MEN1 patients with Th-NET using database analysis. Design/Patients Clinical data of patients with Th-NET were extracted and analysed from a recently constructed database of Japanese MEN1 patients. Results Among 560 registered cases, Th-NET was seen in 28 (5·0%) patients. Of note, 36% of patients (10/28) were women; only one patient among those was a smoker and another six patients were non-smokers. Age at diagnosis of Th-NET and MEN1, tumour size, prevalence of other MEN1-related tumours did not differ between male and female patients, and 10-year survival probability was 0·271 ± 0·106. Conclusions Although the prevalence of Th-NET in women (3·2%) is significantly lower than that in men (7·6%), a considerable proportion of female patients develop Th-NET. Given that Th-NET is a major determinant of life expectancy of patients, our results alert clinicians who treat patients with MEN1 that surveillance of Th-NET is essential even for female patients without a smoking habit. © 2012 Blackwell Publishing Ltd.


Uchino S.,Noguchi Thyroid Clinic and Hospital Foundation
Nihon Geka Gakkai zasshi | Year: 2012

Multiple endocrine neoplasia type 2 (MEN2) is an autosomal, dominantly inherited disease characterized by medullary thyroid carcinoma, pheochromocytoma, and primary hyperparathyroidism and is divided into types 2A and 2B. Familial medullary thyroid carcinoma (FMTC) is characterized by the presence of medullary thyroid carcinoma alone in family members and is considered to be one of the subtypes of MEN2. Clinical and genetic data on 505 Japanese patients from 275 MEN2 or FMTC families registered at 54 medical institutions were collected by the MEN Consortium of Japan. The diagnosis was MEN2A in 343 (67.9%) patients, MEN2B in 29 (5.7%), FMTC in 103 (20.4%), and unclassified in 30 (5.9%). Medullary thyroid carcinoma was found in 91.2% of patients (437/479), pheochromocytoma in 45.6% (212/465), and primary hyperparathyroidism in 8.1% (37/457). RET genetic testing was performed in 410 patients, and the germline RET mutation was found in 98.8% (397/402).


Tachibana S.,Noguchi Thyroid Clinic and Hospital Foundation | Murakami T.,Noguchi Thyroid Clinic and Hospital Foundation | Noguchi H.,Noguchi Thyroid Clinic and Hospital Foundation | Noguchi Y.,Noguchi Thyroid Clinic and Hospital Foundation | And 3 more authors.
Endocrine Journal | Year: 2010

The aim of this study was to demonstrate that the addition of orbital magnetic resonance (MR) imaging can provide improvement in sensitivity of detection of active disease and the prediction of the response to intravenous glucocorticoid therapy (ivGC), over clinical activity score (CAS) alone. A prospective case series was studied at our institution. Forty eight patients were examined by CAS and orbital MR imaging. The maximum of T2 relaxation times of extraocular muscles (maxT2RT) and other parameters were evaluated by MR imaging. Thirty five of 48 patients underwent ivGC. Twenty of 35 patients, whose CAS was 2 points or less, were evaluated for the response to ivGC. The correlation between CAS and maxT2RT was evaluated. Differentiation of active and inactive GO was performed by CAS and orbital MR imaging. The response to ivGC was evaluated by CAS, orbital MR imaging and ophthalmic parameters. As a result, CAS and maxT2RT showed significant positive correlation (r=0.58, p<0.0001), and 15 patients were positive by CAS and orbital MR imaging. However, 20 patients were positive by only MR imaging. In those 20 patients, there was significant improvement after ivGC. We concluded that orbital MR imaging combined with CAS could improve the sensitivity of detection of active disease and the prediction of the response to ivGC. In addition, even if only one parameter of CAS is positive, further examination with orbital MR imaging is advised. © The Japan Endocrine Society.


Enomoto Y.,Noguchi Thyroid Clinic and Hospital Foundation | Enomoto K.,Noguchi Thyroid Clinic and Hospital Foundation | Uchino S.,Noguchi Thyroid Clinic and Hospital Foundation | Shibuya H.,Noguchi Thyroid Clinic and Hospital Foundation | And 2 more authors.
World Journal of Surgery | Year: 2012

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. Diseasefree 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. © Société Internationale de Chirurgie 2011.


Enomoto K.,Noguchi Thyroid Clinic and Hospital Foundation | Uchino S.,Noguchi Thyroid Clinic and Hospital Foundation | Ito A.,Noguchi Thyroid Clinic and Hospital Foundation | Watanabe S.,Noguchi Thyroid Clinic and Hospital Foundation | And 3 more authors.
World Journal of Surgery | Year: 2010

Background Parathyroid cancer is a rare endocrine tumor, and the prognostic factors for this cancer remain unclear. The standard therapy is en bloc resection of the primary tumor at the time of the initial operation. However, the clinical significance of prophylactic neck dissection (PND) in the management of parathyroid cancer has not yet been established. In this study, we investigated its clinical significance in patients with parathyroid cancer and the association of gene mutations with tumor progression. Methods A total of 12 patients with parathyroid cancer were treated and have been followed at Noguchi Thyroid Clinic and Hospital Foundation since 1977. In all, 11 patients were treated with the initial surgery for the cancer, and 1 patient underwent surgery for a metastatic lung lesion. Somatic and germ-line mutations of the HRPT2 and MEN1 were examined by polymerase chain reaction and automated DNA sequencing. Results En bloc resections of thyroid tissue were performed in 10 patients, and 1 patient underwent only parathyroidectomy with limited resection of the thyroid gland. PND was performed in eight patients, and no lymph node metastases were found in the histological specimens. Six of the eight patients had no evidence of the disease, and two had recurring disease (neck lymph node and lung in one patient and local, lung, and brain in another). PND was not performed in three patients, two of whom had no evidence of the disease and one with recurrence at the site of a regional lymph node. There were no significant differences in disease-free survival and cause-specific survival between the patients who underwent PND and the patients who did not (P = 0.98 and P = 0.32, respectively). Among the 12 patients with parathyroid cancer, 1 had a germ-line mutation of the HRPT2 at exon 7, codon 234, CGA (Arg) to TGA (Stop), and 1 patient had a tumor-specific mutation at exon 1, nucleotide 34-37 delAACA. Two of the four patients with recurrent disease had an HRPT2 gene mutation. MEN1 gene analysis revealed one somatic missense mutation at exon 2, codon 121, GTC (Val) to GAC (Asp) in one patient. Conclusions PND for patients with parathyroid cancer resulted in no evidence of lymph node metastasis and does not improve the prognosis. HRPT2 gene mutation may be associated with tumor recurrence. © The Author(s) 2010.


Imai T.,Aichi Medical University | Uchino S.,Noguchi Thyroid Clinic and Hospital Foundation | Okamoto T.,Tokyo Women's Medical University | Suzuki S.,Fukushima Medical University | And 3 more authors.
European Journal of Endocrinology | Year: 2013

Objective: The precise penetrance of pheochromocytoma (PHEO) in multiple endocrine neoplasia type 2 (MEN2) has not been reported in a large cohort. In this study, we aimed to clarify the codon-specific penetrance of PHEO in MEN2. Design: We established a study group designated the 'MEN Consortium of Japan' in 2008 and asked physicians and surgeons to provide clinical and genetic information on patients they had treated up to 2011. Methods: Data were collected on patients identified as carriers of the RET mutation or diagnosed with medullary thyroid carcinoma (MTC) and/or PHEO with family history from 52 institutions all over Japan. Results: Of 493 registered MEN2 patients, RET mutation data were available for 390. Of these, 144 developed PHEOs, while 246 did not. The penetrance of PHEO was 25% by age 30 years, 52% by age 50 years, and 88% by age 77 years in RET mutation carriers with a codon 634 mutation. All patients with a codon 918 mutation (MEN2B) developed PHEO by age 56 years. Less than 32% penetrance of PHEO was seen in patients with mutations at codons other than 634 and 918. Conclusions: Most patients with a codon 634 mutation develop PHEOs as well as MTC during their lifetime. © 2013 European Society of Endocrinology.


PubMed | KEM Hospital, Seth G S Medical College, Noguchi Thyroid Clinic and Hospital Foundation and TATA Memorial Hospital
Type: Journal Article | Journal: Familial cancer | Year: 2016

Multiple endocrine neoplasia type 1 syndrome (MEN1) is a rare autosomal dominant familial cancer syndrome affecting multiple endocrine glands. Published literature on MEN1 from Indian subcontinent is scarce. We report here a case series of MEN1 patients (n=18) from 14 unrelated families. Retrospective study describing the clinical profile of MEN1 patients from endocrine unit of a tertiary care hospital from western India. Additionally clinical profile of primary hyperparathyroidism (PHPT) in MEN1 patients was compared with that of apparently sporadic PHPT cohort from our centre. Eighteen patients (10 males, 8 females) diagnosed as MEN1 were included. Mean age at diagnosis was 31.510.6years (range 17-54). Incidence of primary hyperparathyroidism (PHPT), pituitary adenoma (PA), and gastro-entero-pancreatic neuroendocrine tumor (GEP-NET) was 94.4, 72.2, and 72.2%, respectively. GEP-NET was the commonest presenting lesion (33.3%), followed by PA (27.7%), PHPT (16.6%), thymic carcinoid (5.5%), while 16.6% cases were identified on family screening. PHPT manifestations (clinical and biochemical) in MEN1 were less severe as compared to those of sporadic PHPT. Contrast enhanced computed tomography (CECT) and (68)Ga-DOTANOC PET/CT were equally sensitive (64.7 vs. 63.5%) in identifying multiglandular parathyroid disease. Non functioning tumors (NFT) were the most common GEP-NET, followed by insulinoma (5/13, two were metastatic). (68)Ga-DOTANOC PET/CT had higher sensitivity in detecting GEP-NET lesions than CECT (100 vs. 62.5%). The most common pituitary lesion was prolactinoma, and all were cabergoline responsive. Genetic analysis was available in 13 patients and 11 patients showed mutation in MEN1 gene. The clinical profile of MEN1 in Asian Indian patients is largely comparable to that reported in other cohorts. Peculiar findings of our cohort are predominance of GEP-NET as a presenting manifestation and relatively higher prevalence of insulinoma with higher occurrence of metastatic insulinoma. Clinical and biochemical profile of MEN1 associated PHPT is less severe than that of our sporadic PHPT.


PubMed | Noguchi Thyroid Clinic and Hospital Foundation
Type: Journal Article | Journal: The Indian journal of surgery | Year: 2016

The most effective treatment for thyroid cancer (TC) invading into the larynx and trachea is a complete surgical resection of the tumor, but currently employed techniques are less than ideal. We report a novel surgical technique, which we named Windmill resection and Tetris reconstruction, for patients with TC invading into the laryngeal lumen. We treated eight cases of TC with invasion into the laryngeal lumen by Windmill resection and Tetris reconstruction. We analyzed complications, clinical data, and pathological findings for all patients. Patients included one man and seven women (mean age 6910years). Histopathology of TC indicated papillary cancer in five patients, poorly differentiated cancer in one patient, anaplastic cancer in one patient, and squamous cell carcinoma in one patient. Unilateral recurrent laryngeal nerve (RLN) palsy was confirmed preoperatively by laryngoscope in four patients, and none had bilateral RLN palsy. All patients underwent Windmill resection and Tetris reconstruction along with total thyroidectomy (three patients), subtotal thyroidectomy (three patients), and lobectomy (two patients). Neck dissection was performed in all patients. The average resected length of the larynx and trachea was 296mm. Air leakage at the suture line occurred in three patients; two required further surgery, while the third was closed by insertion of a Penrose drain. Postoperative RLN palsy occurred in five patients. Aspiration was observed in two patients and resolved within 4weeks. Pneumonia, atelectasis, and pleural effusion occurred in some patients. No other complications, including hemorrhage, wound infection, or airway stenosis, occurred. There was no postoperative mortality and no recurrence at the anastomotic site. Two patients underwent permanent tracheostomy due to permanent bilateral RLN palsy. Two patients, one with anaplastic cancer and the other with poorly differentiated cancer, recurred 13 and 21months after surgery, while patients with papillary thyroid cancer had no local recurrence. Importantly, laryngeal functions such as phonation and swallowing were preserved in all patients. This novel surgical technique may be as effective as window resection of the larynx for local control of TC and contributes to the quality of life of patients by resulting in a less unsightly surgical wound.


PubMed | Oita University and Noguchi Thyroid Clinic and Hospital Foundation
Type: Case Reports | Journal: Asian journal of endoscopic surgery | Year: 2016

Multiple endocrine neoplasia type 2 (MEN2) is an autosomal-dominant cancer syndrome with major components of medullary thyroid carcinoma, pheochromocytoma, and hyperparathyroidism. MEN2B is the most aggressive and rarest of the MEN2 variants. Pheochromocytoma in MEN2 is virtually always located in the adrenal medulla, but MEN2-associated extra-adrenal pheochromocytomas (paraganglioma) are rare. A 59-year-old man who has been diagnosed with MEN2B consulted our hospital for surgical treatment of a 10-mm left adrenal mass and a 30-mm retroperitoneal mass. He had paroxysmal elevations in blood pressure and in urinary metanephrine and vanillylmandelic acid values. Laparoscopic excision of the left adrenal gland and retroperitoneal mass was performed. We experienced an extremely rare case of composite paraganglioma-ganglioneuroma concomitant with adrenal metastasis of medullary thyroid carcinoma in a patient with MEN2B.

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