Delayed Presentation of Functioning Metastasis in a Patient with Follicular Thyroid Carcinoma.

Functioning metastases from differentiated thyroid carcinoma are rare and present a great therapeutic challenge. Here, we present an unusual case of a patient with metastatic thyroid carcinoma who developed a hyperthyroid state a few years after the diagnosis due to functioning metastases. Radioiodine treatment was effective in controlling the hyperthyroidism; however, it had no effect on tumor burden. By sharing our experience with this case, we hope to raise awareness for this rare condition and the ways to manage it.

Isolated adrenal metastases of papillary thyroid carcinoma.

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Clinical outcomes of a cohort of 271 patients with lung metastases from differentiated thyroid carcinoma.

CONTEXT: Lung is the most common site of distant metastases from differentiated thyroid carcinoma (DTC). OBJECTIVE: To investigate the outcomes of a cohort of patients with DTC and lung metastases (LM). METHODS: A retrospective analysis of a cohort of 271 patients with LM was performed. RESULTS: The female-to-male ratio was 1:1 and the median follow-up time was 5.9 (1.1-38.4) years. Papillary thyroid carcinoma (PTC) was the most frequent type (83.4%), mainly the classic variant, followed by follicular thyroid carcinoma (FTC, 10.3%) and Hürthle cell carcinoma (HTC, 6.3%). The prevalence of PTC, FTC and HCC was different between the micronodular and macronodular LM groups [87.4%, 6.3% and 6.3% vs. 74.6%, 19.0% and 6.3%, respectively (p = .013)]. Only 5.0% of the patients had LM diagnosed after a period of remission. LM were submitted to radioactive iodine treatment (RAIT) in 84.5% (52.8% showed 131 iodine avid metastases). Complete remission was only achieved in 12.2%. Micronodular disease and age <55 years at LM diagnosis were associated with a better prognosis (p < .05). We found no difference in survival between patients with LM treated or not with RAIT. However, in patients submitted to RAIT, there was a tendency for longer survival in the group of patients with 131 I avid lesions. CONCLUSION: The classic variant of PTC was the most frequent histology found in LM of DTC. LM are rarely diagnosed in the follow-up when complete remission is achieved after surgery and 131 I. Younger age at LM diagnosis and a micronodular pattern are associated with a better prognosis.

Diffuse sclerosing variant of papillary thyroid carcinoma: outcomes of 33 cases.

Introduction: Diffuse sclerosing variant of PTC (DSV-PTC) is an uncommon subtype of thyroid cancer. Although an aggressive behavior is often recognized, prognostic significance is still under debate. Objectives: To describe the clinicopathological features and outcomes of a series of DSV-PTC patients. Methods: Retrospective data collection involving 33 patients diagnosed with DSV-PTC followed at the Endocrine Department of the Portuguese Institute of Oncology in Lisbon between 1981 and 2020. Results: Twenty-six patients (78.8%) were females with a mean age at presentation of 29.4 ± 11.7 years old. Mean time of follow-up was 19.5 ± 10.6 years (range 0.5-39). Histologically, bilateral tumors were present in 72.7% patients (n = 24), thyroid capsular invasion was documented in 57.6% (n = 19), 45.4% (n = 15) had extrathyroidal extension, and 42.4% (n = 14) had lymphovascular invasion. Most patients were staged pT3 (42.4%, n = 14) and pN1 (81.8%, n = 27). Median lymph nodes resected were 16. None of the patients showed distant metastases at presentation. All patients were treated at least once with 131I. During follow-up, four patients (14.8%), with persistent neck disease, were diagnosed with distant metastases, all of them in the lung. Two patients (1.8%) presented recurrent disease in the neck after being considered with no evidence of disease. At the last appointment, 18 patients (54.5%) were in remission, 4 (12.1%) had biochemical evidence of disease, 6 had structural disease, and for 5 patients disease status was considered as undetermined. There was no disease related mortality. Discussion/conclusion: Our study confirms that DSV-PTC is diagnosed more often in young patients and exhibits a local extensive disease at presentation. On the other hand, even in the presence of distant metastases, no patient died during follow-up.

Case Report: A Rare Association of Diffuse Thyroid Lipomatosis with Amyloid Deposition.

Diffuse thyroid lipomatosis is a rare histopathological condition of unknown etiology, characterized by diffuse fatty infiltration of the thyroid stroma, which can result in diffuse goiter with compressive symptoms. We report a case of a 46-year-old man with 1-year history of progressive goiter enlargement with compressive symptoms. Imaging studies revealed multiple coalescent nodules. The patient underwent surgery, and the microscopic appearance revealed a diffuse infiltration of thyroid stroma by mature adipose tissue with associated amyloid deposition. A final diagnosis of diffuse lipomatosis of the thyroid gland was established. This patient represents one of the few reported cases of diffuse lipomatosis with coexisting deposition of amyloid protein of the thyroid gland and contributes to the better understanding of this extremely rare condition.

Central diabetes insipidus caused by a pituitary stalk germinoma resembling infundibuloneurohypophysitis.

We report the case of a pituitary stalk germinoma initially misdiagnosed and treated as infundibuloneurohypophysitis (INH). A 27-year-old man presented with a 1-year history of polydipsia, polyuria, nycturia consistent with central diabetes insipidus and a hyperintense pituitary stalk lesion on MRI. A possible INH diagnosis was considered, after excluding other pathologies. Lesion biopsy was discarded at that time on the ground of a small target and the high risk of added morbidity. Oral desmopressin led to initial symptoms resolution but, in the following months, an anterior panhypopituitarism developed, in spite of appropriate treatment and, by that time, the brain MRI also revealed lesion growth, which prompted a biopsy recommendation. The pathology analysis revealed a germinoma. After chemotherapy and radiotherapy, there was complete disappearance of the pituitary lesion, but the panhypopituitarism persisted. In conclusion, this case highlights the importance and difficulty of precise diagnosis in the initial assessment of pituitary stalk lesions and the need for close monitoring of treatment response. Diagnostic reassessment and biopsy in atypical cases is the only path to achieve the correct diagnosis and treatment.

Bone Metastases from Thyroid Carcinoma of Follicular Origin: A Single Institutional Experience.

BACKGROUND/AIMS: Overall, 2-13% of patients with thyroid cancer develop bone metastases (BM). In addition to decreased survival, patients with BM may present skeletal-related events (SRE) that impair the quality of life. Our objectives were to characterize clinical features, treatment approaches, and outcomes of patients with thyroid cancer and BM. MATERIAL AND METHODS: We identified patients diagnosed with thyroid carcinoma of follicular origin and BM followed at the Instituto Português de Oncologia de Lisboa Francisco Gentil (IPOLFG), Lisbon, Portugal, from 1991 to 2017. SRE were defined as the need for bone irradiation, bone surgery, spinal cord compression, or pathologic fractures. RESULTS: The final cohort consisted of 86 patients, with a median follow-up time of 54 months (IQR 22.8-82.8), mainly women (67.4%), and a median age of 64 years (IQR 53.6-71.2). BM was the initial presentation of thyroid cancer in 36.0% of the patients. Bone involvement was multiple in 59.3% of the cases. Papillary carcinoma was the most frequent histological type, present in 47.7% of the patients, of which 56.1% presented the follicular variant. SRE were found in 76.7% of the patients. The most frequent SRE was radiotherapy (66.3%). Treatment with bisphosphonates was initiated in 19.8% of the patients. The 5-year specific survival was 60%, whereas the 10-year specific survival decreased to 50%. There were no differences in 5- or 10-year specific survival regarding gender, the occurrence of SRE, or histological type. However, patients with initial radioiodine non-avid lesions had a lower 5- and 10-year specific survival (p = 0.002). DISCUSSION: The high frequency of patients with SRE was notable. The follicular variant of papillary thyroid cancer was the variant most commonly associated with BM, reflecting a more similar behavior to follicular carcinoma than the classic variant.

[Characterization of patients with type 2 diabetes treated with vildagliptin]. / Caracterização de doentes com diabetes mellitus tipo 2 medicados com vildagliptina.

INTRODUCTION: Diabetes mellitus is a progressive disease and the rapid growth of this global prevalence has been a worldwide concern. About a third of Portuguese population has type 2 diabetes or pre-diabetes. 2 DM is associated with significant morbidity and mortality, although the treatment so far available it is a high percentage of patients who do not achieve the proposed objectives. Vildagliptin is an inhibitor of oral DPP-4, the most studied of this new class. Inhibiting the rapid degradation of incretins, the vildagliptin increases levels of GLP-1, getting this hormone available to modulate the function of a and ß cells. AIMS: This study aims to characterize the first patients with DM2 treated with vildagliptin in the Department of Endocrinology, Diabetes and Metabolism at the Military Hospital. METHODS: Retrospective study with the first 70 patients treated with vildagliptin, between October and December 2008. The information collected was demographic data, disease duration, associated diseases and their medication, metabolic control in the beginning of the disease (values HbA1c) and criteria for use of vildagliptin. RESULTS: Among the patients included in the study, 55, 7% were male, with the average age of 63, 3 years. These patients had a average duration of diabetes of 11, 7 years. Hypertension was the most frequent associated pathology (85.7% of patients), although dyslipidemia and obesity have a high percentage, 80% and 51% respectively. All patients were overweight (BMI =25 Kg/m(2)). More than half of the patients (55,7%) were on monotherapy until the introduction of vildagliptin, having been associated with other oral antidiabetic agents in all patients. CONCLUSIONS: Most of patients showed risk factors, for witch they were medicated. Vildagliptin has been added mostly in patients medicated with metformin. It is suggested that the therapeutic approach in type 2 diabetes is more and more early, effective and secure.

[Quality of care in diabetic outpatient clinics]. / Avaliação da qualidade de cuidados numa consulta de diabetes.

UNLABELLED: Type 2 Diabetes Mellitus affects an increasing number of people throughout the world. Several studies have shown that it is possible to prevent and minimize type 2 diabetes complications, be it treated appropriately over time. This study aimed to determine the quality of care provided to type 2 diabetic patients in our institution, through metabolic control and risk factors evaluation. SUBJECTS AND METHODS: We reviewed the medical records of 776 type 2 diabetic patients, followed at our outpatient clinic between 1998-2004. RESULTS: A total of 588 patients were included in the study, with a mean age of 66,8 ± 27,2 years. 58% were men. HbA1c levels averaged 7,2 ± 1,6. 57% had HbA1c = 7%. 25,3% met the target blood pressure of 130/80 mmHg; 48% met the goal LDL cholesterol level < 100 and 80% < 130 mg/dl. 6,8% of patients met the combined ADA goal for BP, LDL and HbA1c. Concerning therapeutic regimens: 71,5% used oral hypoglycaemic agents (OAD) alone (52,1% of these were using 2 or more agents); 28,5% were treated with insulin (16,2% in combination with OAD). 52,1% of the patients were anti-aggregated with aspirin. CONCLUSIONS: The metabolic control (HbA1c) and LDL values were favourable in our patients sample, comparing to other studies. The percentage of patients treated to the recommended BP of 130/80 mmHg is consistent with the literature. Only 6,8% of patients met the combined ADA goal for BP, LDL and HbA1c. Despite our comparable results to published data, we would like to highlight the difficulty to accomplish international recommendations to metabolic and risk factors control in clinical practice and the necessity of an aggressive approach to diabetes treatment.

[Hyperthyroidism in a hospital environment: 5-year evaluation]. / Hipertiroidismo em meio hospitalar: avaliação em cinco anos.

INTRODUCTION: Thyroid diseases are an area of particular interest to this Department, constituting 30% of the 3561 patients observed over the last 5 years (when group consultations were set up and outpatient data computerised). OBJECTIVES: To determine the prevalence and aetiologies of Hyperthyroidism (HT) in outpatient clinic and characterise diagnostic and therapy practice. METHODOLOGY: All files of patients diagnosed with HT treated in the department over the last 5 years were reviewed. Of a total of 105 files, 96 were analysed. The data analysed included: demographic data, aetiology of disease, complementary diagnostic test reported, treatment options and follow-up. The data was analysed independently depending on whether 1st consultation occurred before or after 1999. The variables were analysed using descriptive statistical methods, namely frequency and contingency tables for categoric variables and mean, standard deviation, maximum and minimum values for continuous variables. RESULTS: Of the patients evaluated, 72% were female and 28% male, with an average age of 50. 49.0% had Graves' Disease (GD); 14.6% toxic multinodular goitre (TMNG); 11.5% autoimmune HT; 10.4% iatrogenic HT; 7.3% subacute thyroiditis and 7.3% toxic adenoma (TA). Medical treatment was 1st option for 92% of patients with GD (40.4% of cases combined with levothyroxine); 5% received 131Iodine treatment and 3% underwent surgery. In this group, 6 months after suspension of medical treatment, 30.4% had suffered a relapse. Of the patients who suffered a relapse, 42% underwent surgery and 28% received 131Iodine treatment. The remaining 30% made a second attempt with synthetic antithyroid drugs. For patients with TMNG, surgery was the 1st treatment option for the majority of patients (78.5%), followed by 131Iodine (14.2%). 70% of patients with toxic adenoma underwent surgery and 30% 131Iodine treatment. For other pathologies, namely autoimmune HT, iatrogenic HT and subacute thyroiditis, the option was medical treatment. CONCLUSIONS: Hyperthyroidism was diagnosed in 3.5% of patients observed as outpatients in this Department. GD was the most prevalent form of HT (49%). Medical treatment is the first treatment option (92%) for GD. 6 months after suspension of medical treatment, 30% of patients had suffered a relapse of GD. For these patients, surgery was the 1st option. For both TMNG and TA, surgery was the first treatment option. COMPLICATIONS: The adverse effects that occurred with ATDs (antithyroid drugs) were detected in 4,1% patients; one case of agranulocytosis with propylthiouracil, 2 cases of allergic reactions--rash (one due to the use of methimazole and the other to propylthiouracil), and one case of minor hepatocellular toxicity. In all patients there was improvement after stopping medical treatment.

[Thyrotoxic periodic paralysis: a case report]. / Paralisia periódica tireotóxica.

Thyrotoxic periodic paralysis (TPP) is characterized by recurrent episodes of muscle weakness and hypokalemia associated with thyroid hyperactivity. Reported predominantly among individuals of Asian descent, TPP is a rare manifestation of hyperthyroidism in western populations. We describe a typical case of TPP in a 33-year-old portuguese male, who presented with acute tetraparesis associated with hypokalemia (K+ 2.6 mEq/L). The patient had a 8-week history of several episodes of paraparesis of variable intensity; these attacks were usually nocturnal, preceded by strenuous exercise and resolved spontaneously after a few hours. The presence of tachycardia, weight loss, and goiter led to the diagnosis of Graves's disease. The correction of the hyperthyroid function with metimazol and propranolol prevented further episodes of muscle weakness. Thyrotoxic periodic paralysis should be considered in the differential diagnosis of acute episodes of motor paralysis in young patients. The maintenance of a euthyroid status is an essential part of management as this prevents the paralytic episodes.

[Pituitary macroadenoma associated with pulmonary and cutaneous sarcoidosis--a clinical case]. / Macroadenoma da hipófise Associado a Sarcoidose Pulmonar e Cutânea.

Sarcoidosis is a granulomatous disease of unknown etiology that can affect all organs and systems, including the central nervous system in approximately 5% of patients. Although the hypothalamo-hypophyseal system may be one of the areas affected by neurosarcoidosis, pseudotumoral lesions are very rare. This paper reports the case of a 42-year-old male Caucasian patient followed by the Pneumology and Dermatology services for pulmonary and cutaneous sarcoidosis. Corticotherapy was initiated five months from diagnosis and the patient showed clear clinical and imagiological signs of improvement. In the following two months, the patient experienced visual disturbances and a slight decrease in libido, but denied having other symptoms associated with hypothalamo-hypophyseal hypofunction. Laboratorial evaluation (basal determinations and stimulation tests) revealed panhypopituitarism. The EC-NMR showed a bulky intrasellar lesion extending into the suprasellar and right parasellar regions, with evidence of optic chiasm compression, erosion of the floor of the sella and cavernous sinus invasion. Campimetry showed a bitemporal hemianopsia. For the purposes of chiasmatic decompression and histological study of the lesion the patient underwent a right frontal craniotomy, with a subtotal resection of the hypophyseal lesion. The histological diagnosis revealed a pituitary adenoma with no immunohistochemical expression. The patient began radiotherapy six months after surgery. Although today's diagnostic tools allow for a high degree of accuracy, hypophyseal lesions still present problems to the endocrinologist as their identification is essential for determining the appropriate treatment. At times the final diagnosis is only achieved after a histological identification of the lesion (biopsy/surgery). It should be noted that the described cases of association between sarcoidosis and hypophyseal adenoma are very rare, making the differential diagnosis with neurosarcoidosis difficult--a condition which has a preferential medical approach and a different prognosis.

[Diagnosis and treatment of solitary thyroid nodule--study and evaluation in Portugal]. / Diagnóstico e tratamento do nódulo solitário da tiróide. Estudo de Avaliação em Portugal.

INTRODUCTION: The best diagnostic and treatment strategy for an approach to the nodular thyroid disease continues to be a controversial issue. OBJECTIVES: The aim of this study was to characterise medical practice in the diagnosis and treatment of nodular thyroid disease by endocrinologists and surgeons in Portugal in 2002. METHODS: A questionnaire based on that used by the European Thyroid Association and the American Thyroid Association was drawn up. The questionnaire, based on a well-defined index case, was circulated by the Portuguese Endocrinology Society to endocrinologists and surgeons: 42 year-old woman with solitary thyroid nodule measuring 2 x 3 cm, with no history of malfunction or painful symptoms. Each doctor was asked to reply as to the adopted diagnosis and therapy procedures for the index case. Eleven variations to the original case were proposed in order to evaluate the alterations for each variation. RESULTS: 1492 questionnaires were sent out, 163 to endocrinologists and 1329 to surgeons. A total of 104 were returned. The global response rate was 7%. The response rate for endocrinologists was 27% and 4.5% for surgeons. Of the 104 questionnaires returned, 42% were from endocrinologists and 58% from surgeons. Concerning tests prescribed, surgeons would use more tests than endocrinologists for the index case. The main differences in laboratory terms were the higher number of prescriptions for total T4 and T3 and thyroglobulin by surgeons and more prescriptions for AATPO by endocrinologists. The average number of tests was 4.6, 4.1 for endocrinologists and 5.1 for surgeons. Relative to imaging and cytology, 32% of doctors advocated a scintigraphy to diagnose the index case, with no significant differences between endocrinologists and surgeons. Ultrasonography was used by over 85% of respondents. 90% prescribed a cytology, 83% guided by palpation and 18% ultrasonography-guided. Concerning treatment, 33% of doctors advocated levothyroxin treatment; surgery was advocated by 16.3% of endocrinologists and 36.6% of surgeons. Meanwhile, the majority of doctors (68%) would opt for no treatment and simply maintain the patient under surveillance. CONCLUSIONS: There are important differences in the approach to nodular thyroid disease among the various doctors and specialists, which highlight the difficulty in achieving a diagnostic and therapeutic consensus.