Biomedical subjects

Jeremy L Freeman

Publications and source records attributed to Jeremy L Freeman.

At least 19 recordsLinked to original sources

PubMed2026

Late-onset epileptic spasms: presentation, aetiology and outcome.

Late-onset epileptic spasms (LOES) are epileptic spasms (ES) commencing after age 12 months, often misdiagnosed and having uncertain relationship to infantile spasms. Previous studies of mostly small LOES cohorts frequently reported 'cryptogenic' aetiologies. We studied the presentations, aetiologies, treatment responses and outcomes in a large LOES cohort, evaluated with modern neuroimaging and genomic testing. In this retrospective cohort study, 62 children with video-confirmed epileptic spasms, diagnosed between 2011 and 2021, were included. All had epileptiform activity on EEG, but none had hypsarrhythmia. Median age at epileptic spasms onset was 23 months (range 1-15 years) and median delay to diagnosis was 8 months (interquartile range: 3-15). Only 24% children were correctly diagnosed at presentation, common misdiagnoses being myoclonic epilepsies and non-epileptic phenomenon. Aetiology was identified in 95%. Structural-malformative aetiologies were present in 63% (most commonly focal cortical dysplasia and mild malformation of cortical development with oligodendroglial hyperplasia in epilepsy). Other aetiologies were structural-acquired in 13% (mostly postnatal stroke and CNS infections), genetic in 13% (mostly intragenic variants and chromosomopathies) and oncological in 6% (history of leukaemia, two with CNS involvement). Children with structural aetiologies often had normal development prior to onset of epileptic spasms, a later median age of epileptic spasms onset and were more likely to have asymmetric or subtle epileptic spasms and additional seizures prior to epileptic spasms. Epileptic spasms ceased in 29% children treated with prednisolone, most having genetic aetiologies and in 35% treated with vigabatrin, all having structural-malformative aetiologies. Apart from clobazam, which was effective in 17% of children, all other antiseizure medications, vagus nerve stimulation and ketogenic diet therapy were ineffective. Epileptic spasms ceased in 86% (18/21) of children who underwent epilepsy surgery. At median follow-up of 10.3 years, 53% children were free of all seizures and 76% were free of epileptic spasms. More children with unilateral structural-malformative aetiologies achieved seizure freedom than other aetiologies, most commonly following surgery but occasionally following treatment with vigabatrin or clobazam. Impairment of cognitive function or adaptive behaviour (formally assessed in 90%) was significantly more common in children with genetic than structural aetiologies, and in children with ongoing seizures than seizure freedom. Among children who underwent epilepsy surgery, 62% achieved average or low-average adaptive functioning or normal intellectual capacity. This study highlights the importance of prompt recognition of epileptic spasms in older children for improved seizure and developmental outcomes. Brain malformations and insults are the predominant causes of LOES, and when unilateral, respond best to epilepsy surgery.

cognitive outcome↗

PubMed2006

Risk of malignancy in Filipinos with thyroid nodules--a matched pair analysis.

BACKGROUND: Ethnicity is an infrequently cited risk factor for thyroid cancer. This study aims to determine whether patients of Filipino origin assessed at a tertiary referral center are at increased risk of thyroid cancer and to quantify that risk. METHODS: This is a retrospective analysis of 72 Filipino patients with thyroid nodules undergoing thyroidectomy individually matched to 72 controls according to demographics, risk factors, and pre-referral investigations. RESULTS: Thyroid cancer was found in 50 (69.4%) Filipino patients compared with 28 (38.9%) controls. Odds ratio for malignancy in Filipinos was 3.57 (95% confidence interval, 1.79-7.12). Pathologic characteristics of thyroid cancer were not significantly different in the two groups. The median follow-up for Filipino patients was 14.1 months (range, 1.4 months-19.7 years). Recurrence occurred in four Filipino patients, and one patient with anaplastic thyroid cancer died of the disease. CONCLUSIONS: Filipino patients with thyroid nodules are at significantly increased risk of thyroid cancer compared with matched controls, and hence the index of suspicion for malignancy should be high when evaluating these patients.

Adult↗

PubMed2006

Three-week thyroxine withdrawal: a thyroid-specific quality of life study.

OBJECTIVES/HYPOTHESIS: The purpose of this study is to determine the impact of a 3-week T4 withdrawal test on the quality of life (QOL) of patients undergoing investigation for residual/recurrent well-differentiated thyroid cancer. STUDY DESIGN: Prospective survey study. METHODS: 181 patients with well-differentiated thyroid cancer were surveyed using a thyroid-specific QOL-thyroid survey at three times throughout the study: prior to T4 withdrawal, after the 3-week withdrawal period, and 4 weeks after resuming T3/T4 combination therapy. RESULTS: A very small, though statistically significant, reduction was observed in the four domains of QOL defined by the QOL-thyroid survey from baseline values to 3 weeks after T4 withdrawal. The maximal difference was 2.04 (fatigue), out of a possible 10 point score, in the physical well-being category. CONCLUSIONS: The thyroid-specific QOL questionnaire demonstrates a statistically significant though very small and probably not clinically significant reduction in the QOL of thyroid cancer patients undergoing an induced 3-week hypothyroid state. Three-week T4 withdrawal is a simple, cost-effective, and readily available test.

Adolescent↗

PubMed2006

Quality of life in patients undergoing thyroid surgery.

OBJECTIVE: The extent of thyroidectomy in the management of low-risk, well-differentiated thyroid carcinoma (WDTC) has been debated extensively. Our objective was to determine if hemithyroidectomy has a less detrimental effect on quality of life (QOL) than total thyroidectomy. DESIGN: Prospective, nonblinded, nonrandomized, cohort study. SETTING: Tertiary care academic otolaryngology-head and neck surgery practice. METHODS: Using both disease-specific and global QOL instruments, patients treated with either hemi- or total thyroidectomy were prospectively followed. QOL was assessed preoperatively and for 12 months postoperatively. MAIN OUTCOME MEASURES: Scores on the two QOL instruments throughout a 12-month postoperative period. RESULTS: Patients with cancer experienced a greater drop in QOL during the first 6 months following surgery when compared with patients with benign disease (p < .03). Additionally, patients treated with total thyroidectomy did not have a significantly different QOL than patients treated with hemithyroidectomy (p > .2). CONCLUSION: These results suggest that QOL is not significantly impacted by the extent of surgery and that QOL should not be a factor in the decision-making process for the treatment of low-risk WDTC.

Adolescent↗

PubMed2005

Interspecialty and intraspecialty differences in the management of thyroid nodular disease and cancer.

INTRODUCTION: The management of thyroid cancer includes multiple medical specialties. Physicians from different specialties may vary in opinion regarding the optimal investigation and treatment of patients. Little data exist evaluating the differences within or between various specialties treating thyroid disease. This study aims to examine responses from a variety of specialty physicians closely involved in the medical or surgical management of thyroid disease to provide evidence as to whether any difference exists. METHODS: A cross-sectional survey of attendees at the 5(th) Biennial Course on the Management of Thyroid Nodular Disease and Cancer was conducted using an anonymous electronic touch pad system. Touch pads were given to 213 attendees who were asked to respond to 44 questions. This study analyzes the responses obtained from 19 selected questions (43%) and compares the results between endocrinologists (n = 48), general surgeons (n = 41), otolaryngologists (n = 61), and pathologists (n = 20). RESULTS: Responses were obtained from 69% of endocrinologists, 68% of general surgeons, 72% of otolaryngologists, and 65% of pathologists. Statistically significant interspecialty differences were observed in 12 (63%) of 19 questions. Each question and a summary of responses from all touch pads were recorded. CONCLUSIONS: Significant differences in the attitudes toward, and presumably the practice of, managing thyroid nodular disease and cancer exist between specialties. An understanding of these differences is helpful when working as a multidisciplinary team to optimize patient care.

Adult↗

PubMed2005

Zoster-associated intracranial hypertension.

A 14-year-old female presented with headache, vomiting, and a rash. She was found to have papilledema and herpes zoster. Examination of the cerebrospinal fluid revealed pleocytosis and an elevated protein concentration. Varicella-zoster virus deoxyribonucleic acid was detected in the cerebrospinal fluid by polymerase chain reaction. Intracranial hypertension was treated by repeated lumbar puncture and with acetazolamide. This case represents an unusual complication of the reactivation of varicella-zoster virus.

Adolescent↗

PubMed2005

Fine-needle aspiration biopsy findings suspicious for papillary thyroid carcinoma: a review of cytopathological criteria.

OBJECTIVES/HYPOTHESIS: The objective was to evaluate the usefulness of standard suspect cytological features on fine-needle aspiration biopsy (FNAB) in predicting papillary thyroid carcinoma. STUDY DESIGN: Retrospective chart review of consecutive fine-needle biopsies of the thyroid. METHODS: The study was a retrospective review of consecutive patients presenting with a diagnosis of suspected (group 1) or positive papillary thyroid carcinoma (group 2). The frequency of standard cytological features (i.e., papillary architecture, multinucleated giant cell, nuclear pseudo-inclusions, nuclear grooves, micronucleoli, powdery chromatin, and psammoma bodies) were recorded for each group. These were compared using chi test. Sensitivity and specificity for both individual and a combination of features were calculated for patients in group 1. RESULTS: One hundred eight patients were eligible for the study (group 1, n = 57; group 2, n = 51). Fifty-one patients (89%) in group 1 and all patients in group 2 had a histopathological diagnosis of papillary thyroid carcinoma. Respectively, the most frequent features present on fine-needle aspiration biopsy in group 1 versus group 2 were nuclear grooves (79% vs. 88%), micronucleoli (74% vs. 86%), pseudo-inclusions (58% vs. 88%), and powdery chromatin (47% vs. 59%); P values for these features were P > .05, P > .05, P < .05, and P > .05, respectively. In group 1, the sensitivities of nuclear grooves and micronucleoli were 80% and 71%, respectively. The presence of psammoma bodies was associated with a specificity of 100%. A combination of nuclear grooves, micronucleoli, pseudo-inclusions, powdery chromatin, and multinucleated giant cells was 100% specific in detecting papillary thyroid carcinoma. CONCLUSION: In choosing the most appropriate management of a finding suspect for papillary thyroid carcinoma on fine-needle aspiration biopsy, the surgeon must be aware of the diagnostic importance of certain cytopathological features. The presence of a combination of these factors may allow a more confident surgical approach.

Biopsy, Fine-Needle↗

PubMed2005

Variables predicting distant metastases in thyroid cancer.

OBJECTIVES: Distant metastases from thyroid cancer are uncommon and have a variable prognosis. We present a series of patients with distant metastases to determine which patients are at risk of developing distant disease and to examine the significant prognostic variables. STUDY DESIGN: Retrospective chart review of 30 patients with distant metastases compared with 633 controls from the Mount Sinai Thyroid Cancer Database and literature review. METHODS: The prevalence of distant metastases was 4.5%, and median follow-up of survivors was 12.7 years. Histologic type was Hurthle cell carcinoma in 3, follicular in 3, papillary in 19, and 5 patients had focal anaplasia either in the primary site or regional metastases. Predictors for distant metastases, locoregional control, and survival were analyzed. RESULTS: Cumulative survival for patients with distant metastases was 49.5% at 10 years and 12.9% at 20 years. Site of metastases was lung in 26, bone in 11 and brain in 1 patient, with 8 patients having multiple sites. The median time to diagnosis of distant metastases was 3 months. Variables that predicted for development of distant disease were male sex, age, size, extrathyroidal extension, regional metastases, and elevated thyroglobulin. Survival in patients without distant disease was significantly better than those with distant metastases (P < .001). Variables that predicted poor outcome in patients with distant metastases on analysis were age greater than 45 years (P = .003) and histologic type of thyroid cancer (P = .009). CONCLUSION: Although patients with thyroid cancer and distant metastases may live prolonged periods with disease, it does significantly impact on patient survival. Age remains an important variable in both predicting for development of distant metastases and also influences long-term survival in patients with existing distant metastases.

Adenocarcinoma, Follicular↗

PubMed2005

Prognostic variables and calcitonin in medullary thyroid cancer.

OBJECTIVES/HYPOTHESIS: Medullary thyroid cancer (MTC) is a nonepithelial, neuroendocrine tumor with a more aggressive clinical behavior than differentiated thyroid cancer. The purpose of the study was to review a single institution's experience with MCT since 1969. STUDY DESIGN: Retrospective cohort study. METHODS: A retrospective review of 30 patients treated for MTC at a tertiary care referral center between 1969 and 2000. There were 17 female and 13 male patients, median age at presentation was 38 years, and median follow-up for survivors was 12.4 years. RESULTS: Seventy percent of patients had sporadic MTC, 6.7% had familial MTC, 16.7% had multiple endocrine neoplasia syndrome type IIA, and 6.7% had multiple endocrine neoplasia syndrome type IIB. The cumulative overall survival rates at 5, 10, and 20 years were 97%, 88%, and 84%, respectively; disease-free survival rates were 97%, 74%, and 29%, respectively. Advanced tumor stage (P = .014) and multiple endocrine neoplasia syndrome type IIB predicted decreased disease-specific survival. Variables affecting disease-free survival were post-thyroidectomy calcitonin level (P = .001), vascular invasion (P = .005), perineural invasion (P = .010), extrathyroidal extension (P < .001), and the presence of nodal metastases (P = .001). Locoregional control rates were 83% at 5 years and 70% at 10 years. Vascular invasion (P = .004), extrathyroidal extension (P = .008), and post-thyroidectomy basal calcitonin level (P = .003) predicted locoregional failure. Many patients in the series experienced prolonged survival despite elevated calcitonin levels. CONCLUSION: Long-term disease-free survival is uncommon in MTC, but the study data indicate that the majority of patients with MTC live for prolonged periods, despite biochemical evidence of persistent disease. Adverse pathological features such as extrathyroidal extension and vascular and perineural invasion were predictors of disease recurrence. The use of serum markers in the follow-up of patients with MTC must be interpreted within the clinical context.

Adolescent↗

PubMed2005

Surgical strategy for thyroid bed recurrence in patients with well-differentiated thyroid carcinoma.

BACKGROUND: Well-differentiated thyroid carcinoma (WTC) has a low but definite rate of recurrence. The majority of these occur locoregionally and present a significant diagnostic and management challenge. It is the aim of this article to convey the complexities associated with revision surgery involving the thyroid bed and to present sound surgical strategies to deal with this problem in a manner that minimizes morbidity, adheres to oncologic principles, and achieves appropriate cure rates. METHOD: Between 1992 and 2002, 14 patients with revision surgery involving the thyroid bed were identified and managed according to an algorithm taking into account clinical, biochemical, and radiologic indices. All underwent revision surgery, and we applied our technique of wide field exposure by horizontally sectioning all of the ipsi- or bilateral strap muscles. We use blunt dissection to identify the recurrent laryngeal nerves and parathyroid glands. RESULTS: There were six males and eight females, with a median age of 38 years (range 23-62 years). The median time between procedures was 25 months (range 6-120 months). The diagnosis was established by clinical examination, thyroglobulin determination, and/or imaging. All were treated with surgery and postoperative iodine 131 (I131). The median follow-up was 6 months (range 2-48 months). Complications included two cases of temporary recurrent laryngeal nerve palsy, two patients with permanent and two patients with temporary hypocalcemia, two cases of temporary chyle leaks, and one recurrence. One patient underwent a negative exploration. CONCLUSION: The management of infield recurrence of WTC presents both a diagnostic and a therapeutic challenge owing to the disparity in presentation, the complexity of the anatomy, and indistinct tissue planes. The optimal treatment of these patients is surgical resection and postoperative I131. This can be accomplished safely and with little morbidity. The key to this type of surgery is a sound and systematic approach.

Adult↗

PubMed2004

Management and outcome of recurrent well-differentiated thyroid carcinoma.

BACKGROUND: The AMES (age, distant metastasis, tumor extent, and size), AGES (age, tumor size, histologic grade, tumor extent, distant metastasis), and MACIS (distant metastasis, age, completeness of primary tumor resection, local invasion, and tumor size) prognostic systems for well-differentiated thyroid carcinoma (WDTC) are well known. The development of disease recurrence is associated with a poor outcome; however, the prognostic importance of multiple treatment failures has not been clearly reported. OBJECTIVES: To identify patient, tumor, and treatment factors that may be associated with the development of multiple recurrences in WDTC. DESIGN AND SETTING: All patients treated for residual or recurrent WDTC were retrospectively identified from the thyroid cancer database at the Department of Otolaryngology-Head and Neck Surgery, Mount Sinai Hospital, Toronto, Ontario (1963-2000). Data on relevant patient, tumor, and treatment factors were collected. MAIN OUTCOME MEASURES: Patient, tumor, and treatment factors predicting the development of multiple treatment failures, disease-specific survival, and overall survival. RESULTS: A total of 574 patients (115 male, 459 female; median age, 42 years [range, 9-92 years]) were identified, whose final histopathologic diagnosis was papillary carcinoma in 468, follicular carcinoma in 76, and mixed in 30 cases. TNM staging was as follows: 409 (71%) stage I, 66 (12%) stage II, 68 (12%) stage III, and 31 (5%) stage IV. Initial management included total thyroidectomy for 217 patients (38%), subtotal thyroidectomy for 357 (62%), and adjuvant iodine 131 therapy for 492 (86%). Seventy-three patients (13%) developed recurrent WDTC (21 male, 52 female; median age, 44 years [range, 18-84 years]). Patients were divided into 3 groups: group 1 (no recurrence, n = 501), group 2 (1 recurrence only, n = 42), and group 3 (multiple recurrences, n = 31). Group 2 data were as follows: site of recurrence (locoregional, 25; distant, 7; unspecified, 10) and treatment (surgery, 12; iodine 131, 42) and for group 3: site of first recurrence (locoregional, 16; distant, 11; unspecified, 4) and treatment (surgery, 14, iodine 131, 22; palliation, 1). Actuarial disease-specific survival at 20 years was 100%, 94%, and 60%, respectively, for the 3 groups (median follow-up, 7 years; range, 1-34 years). Male sex, advanced stage, extrathyroidal spread, and primary treatment with total thyroidectomy were predictive factors for multiple recurrences on multivariate regression (all P<.05). CONCLUSIONS: Male sex, advanced initial stage, and presence of extrathyroidal spread within the primary tumor are the most significant independent predictors of developing multiple recurrences in patients with WDTC. These patients have a poor prognosis with a significant reduction in tumor-free survival.

Actuarial Analysis↗

PubMed2004

Operative technique: the anterior transcallosal transseptal interforniceal approach to the third ventricle and resection of hypothalamic hamartomas.

Background. We have previously described the resection of hypothalamic hamartomas (HH) using a transcallosal approach [Transcallosal resection of hypothalamic hamartomas, with control of seizures, in children with gelastic epilepsy, Neurosurgery, 2001]. Since then, we have refined the technique and now describe in detail an anterior transcallosal transseptal interforniceal approach to the third ventricle as a variation of the standard transcallosal interforniceal approach. The results of this series are presented to demonstrate the safety and efficacy of this approach. Method. HH were resected via an anterior transcallosal, transseptal, interforniceal approach to the third ventricle. This is a more anterior approach to the third ventricle with a more acute trajectory than has been described previously. Results. This approach provided excellent access to the floor of the third ventricle with minimal forniceal retraction and avoidance of dissection of the deep venous structures. Transcallosal resection of HH was performed in 45 patients aged 2.9-33 years (mean 11.3 years). Morbidity was minimal, including transient hemiparesis in 3, ongoing diabetes insipidus in 2, early short-term memory impairment in 16 (persistent in 6) and one patient developed pneumonia postoperatively but recovered. Conclusion. The anterior transcallosal transseptal interforniceal technique is an effective and relatively safe technique when used for the resection of HH. This operative approach is applicable to other pathology in the third ventricle or hypothalamic region and has advantages compared with the standard transcallosal approach to the third ventricle.

Adolescent↗

PubMed2004

Prognostic factors in well-differentiated thyroid carcinoma.

OBJECTIVES: To test the prognostic significance of standard clinicopathologic factors in patients with well-differentiated thyroid carcinoma (WDTC). STUDY DESIGN: A retrospective chart review of the thyroid cancer database at Mount Sinai Hospital (Toronto, Canada 1963-2000) was carried out. METHODS: All patients consecutively treated for WDTC with a follow-up period of at least 5 years were eligible for inclusion. Relevant patient, tumor, treatment, and outcome data were collected. The main outcome measures were recurrence rate, actuarial overall, and disease-specific survival at 20 years. RESULTS: Three hundred and thirty-three patients (F 275, M 58) with a median age of 39.7 (range 9-82.9) years were eligible for inclusion in this study (median follow-up 10.4 years, range 5-34.4 years, minimum 5 years). The recurrence rate was 15.6% (52 /333). The overall and disease-specific survival at 10 years was 97.5% and 98.5%, respectively. Likewise, the overall and disease-specific survival at 20 years was 88.4% and 93.3%, respectively. Clinicopathologic factors significant on multivariate regression for the development of disease recurrence included family history of WDTC, advanced stage, and total thyroidectomy (all P < .05). Similarly, advanced stage on presentation was associated with a worse disease-specific survival on multivariate regression (all P < .05). There was a trend for age 60 or greater to predict disease-specific survival (P = .09). CONCLUSIONS: WDTC is associated with a significant recurrence rate but good disease-specific survival. The most important prognostic factors are family history of WDTC, extent of surgical treatment (i.e., total thyroidectomy), and advanced initial stage of disease, with a trend for age 60 years and older.

Adenocarcinoma, Follicular↗

PubMed2004

Familial non-medullary thyroid cancer: a matched-case control study.

OBJECTIVES: Familial non-medullary thyroid cancer (FNMTC) is a newly recognized disease entity and can be distinguished from the more common sporadic non-medullary thyroid cancer. The purpose of this study was to determine some of the potential distinguishing features of FNMTC. STUDY DESIGN: Retrospective association study and matched-case control study. METHODS: Five hundred forty-three cases of well-differentiated follicular origin thyroid cancers were identified and collected in a database. Among this population, 24 cases of FNMTC were identified. A case of FNMTC was defined as a patient with the following two criteria: a well-differentiated follicular origin thyroid cancer and at least one first-degree relative with a well-differentiated epithelial origin thyroid cancer. The unmatched sporadic and FNMTC groups were compared using t test, Phi test, Cramer V test, and Pearson and Spearman correlation tests. Twenty-four FNMTC cases were matched to 24 sporadic cases based on age, gender, stage of disease at presentation, and tumor size. Clinicopathologic features, management, and outcome were analyzed statistically using a matched-proportional z test. Disease-free survival and disease-specific survival were analyzed using log-rank test and the Kaplan-Meier function. A P-value less than .05 was considered statistically significant. RESULTS: : There was no significant difference in ionizing radiation exposure, disease multifocality, surgical management, or recurrence between the sporadic and FNMTC patients. Although FNMTC patients tend to have improved disease-free survival and disease-specific survival, the difference was not significant at the 5% level. CONCLUSION: Although FNMTC is characterized by strong family history, these patients do not tend to have worse prognosis.

Adenocarcinoma, Follicular↗

PubMed2004

Thyroid colloid nodules diagnosed by fine-needle aspiration: efficacy of suppression.

BACKGROUND: This study reviewed the accuracy of fine-needle aspiration biopsy (FNAB) and the efficacy of thyroid suppression for colloid nodules in our population to determine the utility of these two modalities on the decision to operate. METHODS AND MATERIALS: A retrospective chart review of patients with colloid nodules diagnosed by FNAB from January 1993 to July 1995 was conducted. A 52-patient cohort underwent surgical management, and their needle aspirate cytologies and final pathologies were reviewed. RESULTS: A 7.7% false-negative rate in the detection of thyroid malignancy by FNAB was obtained. This is in keeping with data reported in the literature. Virtually no efficacy of hormonal suppression in our population was found. CONCLUSION: When the literature is reviewed and compared with the results of this study, the use of FNAB as a decision tool to operate is valid. The decision to operate based on the outcome of hormonal suppression, however, is not valid based on our results.

Biopsy, Needle↗

PubMed2004

Recurrent pleomorphic adenoma of the parotid gland.

This is a retrospective analysis of 35 patients treated for a first recurrence of pleomorphic adenoma of the parotid gland from 1982 to 1997. All patients were treated surgically; no patient received radiotherapy for benign disease. We experienced a locoregional control rate of 77%. Our malignant transformation rate was 5.7%. Our results suggest that in carefully selected patients, local excision of recurrent disease is sufficient in controlling further recurrence.

Adenoma, Pleomorphic↗

PubMed2004

Prognostic implications of site of recurrence in patients with recurrent well-differentiated thyroid cancer.

OBJECTIVE: The site of treatment failure in patients with recurrent well-differentiated thyroid carcinoma (WDTC) has implications for both the mode of salvage therapy and disease-specific prognosis. The objective of this study was to evaluate the prognostic significance of the site of failure in patients with recurrent WDTC. DESIGN: Patients with recurrent thyroid cancer were identified retrospectively from 1963 to 2000. SETTING: Data were retrieved from the thyroid cancer database at the Department of Otolaryngology-Head and Neck Surgery, Mount Sinai Hospital, Toronto (1963-2000). METHODS: Patients were placed into four different groups according to their site of recurrence: group 1, local recurrence; group 2, regional recurrence; group 3, distant recurrence; and group 4, unspecified recurrence. Patient, tumour, and treatment data were collected and compared. The Kaplan-Meier method was used to calculate survival data, and curves were compared using the log rank test. MAIN OUTCOME MEASURES: Outcome included alive, no disease; alive with disease; dead, no disease; and dead of disease. RESULTS: Seventy-three patients (21 male, 52 female; median age 44 years, range 18-84 years) were eligible for inclusion in this study. Relevant data were divided according to group 1, 22 patients; group 2, 24 patients; group 3, 13 patients; and group 4, 14 patients. The groups were comparable in terms of primary tumour and treatment factors. The actuarial disease-specific survival rate after salvage therapy was less significant in patients who developed a distant recurrence versus a local recurrence, a regional recurrence, or an unspecified recurrence (ie, 66% vs 95%, 88%, and 92%, respectively; p = .06). CONCLUSIONS: Patients with distant recurrences have a poor prognosis, with a significant reduction in the actuarial disease-specific survival rate.

Adolescent↗

PubMed2004

MR imaging and spectroscopic study of epileptogenic hypothalamic hamartomas: analysis of 72 cases.

BACKGROUND AND PURPOSE: Reports of MR imaging in hypothalamic hamartomas associated with epilepsy are few, and the number of patients studied is small. We aimed to detail the relationship of hypothalamic hamartomas to surrounding structures, to determine the frequency and nature of associated abnormalities, and to gain insight into mechanisms of epileptogenesis. METHODS: We systematically examined MR imaging studies of 72 patients with hypothalamic hamartoma and refractory epilepsy (patient age, 22 months to 31 years). A dedicated imaging protocol was used in 38 cases. Proton MR spectroscopy of the hypothalamic hamartoma was performed for 19 patients and compared with the metabolite profile of the thalamus in 10 normal children and the frontal lobe in 10 normal adults. RESULTS: Compared with normal gray matter, hypothalamic hamartomas were hyperintense on T2-weighted images (93%), hypointense on T1-weighted images (74%), and had reduced N-acetylaspartate and increased myoinositol content shown by MR spectroscopy. Hypothalamic hamartomas always involved the mammillary region of the hypothalamus, with attachment to one or both mammillary bodies. Intrahypothalamic extension (noted in 97%) tended to displace the postcommissural fornix and hypothalamic gray matter anterolaterally, such that the hypothalamic hamartomas nestled between the fornix, the mammillary body, and the mammillothalamic tract. Larger hamartoma size was associated with central precocious puberty. Associated findings of questionable epileptic significance included anterior temporal white matter signal intensity abnormalities (16%) and arachnoid cysts (6%). Malformations of cortical development were observed in only two patients, and hippocampal sclerosis was not observed. CONCLUSIONS: Hypothalamic hamartomas can be readily distinguished from normal hypothalamic gray and adjacent myelinated fiber tracts, best appreciated on thin T2-weighted images. MR imaging and spectroscopy suggest reduced neuronal density and relative gliosis compared with normal gray matter. Associated epileptogenic lesions are rare, supporting the view that the hypothalamic hamartoma alone is responsible for the typical clinical features of the syndrome. The intimate relationship to the mammillary body, fornix, and mammillothalamic tract suggests a role for these structures in epileptogenesis associated with hypothalamic hamartomas.

Adolescent↗