Hipercalcemia asociada a hiperparatiroidismo primario: enfoque y manejo a partir de un caso clínico

Introducción: el calcio es el electrolito más abundante del cuerpo humano y la hipercalcemia es el trastorno común causado normalmente por el hiperparatiroidismo primario o malignidad, su manejo depende de la presentación y causa subyacente. Además, una proporción de casos se presentan como una emergencia, lo que conlleva a una mortalidad significativa. Objetivo: mostrar un caso inusual de presentación clínica de hipercalcemia asociada a hiperparatiroidismo primario y, asimismo, dar una breve revisión acerca del enfoque y el manejo de esta patología. Presentación del caso: paciente femenina de 32 años, antecedente de adenoma paratiroideo no resecado y pancreatitis, asiste por tres días de dolor abdominal de tipo cólico y de moderada intensidad, acompañado de episodios eméticos de contenido alimentario, paraclínicos iniciales con hipercalcemia severa, electrocardiograma con bloqueo auriculoventricular grado I, gases arteriales con alcalosis respiratoria aguda e hiperlactatemia. También se le practicó un TAC de abdomen donde este apareció con tumores pardos. Se ingresó a la UCI para la administración de líquidos endovenosos, diuréticos de asa y cinacalcet, pero no presentó mejoría, por lo que se indicó terapia de hemodiálisis. Discusión y conclusión: la hipercalcemia es un hallazgo frecuente. El hiperparatiroidismo primario y la neoplasia maligna son las dos causas más frecuentes de aumento de los niveles de calcio sérico y, en conjunto, representan alrededor del 90 % de todos los casos, donde los valores en suero varían entre el calcio total (8,5 y 10,5 mg/dl) y el iónico (1,16-1,31). La concentración sérica de Ca 2+ está estrechamente relacionada por las acciones de la hormona paratiroidea y el calcitriol, donde el hiperparatiroidismo primario ocurre como resultado de adenomas, hiperplasias y carcinoma. Las manifestaciones clínicas y la severidad van a estar correlacionadas con el tiempo de duración de la enfermedad, los niveles de calcio y de PTH. Dentro del tratamiento, este será guiado por su causa, sin embargo, es posible clasificarlo en tratamiento urgente y no urgente. Además, el enfoque de la hipercalcemia aguda severa es un reto diagnóstico dadas las múltiples causas que pueden llevar a este trastorno hidroelectrolítico y la rápida instauración de tratamiento que se requiere cuando es detectada.

Background: Calcium is the most abundant electrolyte in the human body, hypercalcemia is a common disorder usually caused by primary hyperparathyroidism or malignancy. A proportion of cases presenting as an emergency, leading to significant mortality. The management of hypercalcemia depends on the presentation and underlying cause. Purpose: to present an unusual case of clinical presentation of hypercalcemia associated with primary hyperparathyroidism, as well as to give a brief review about the approach and management of this pathology. Case presentation: A 32-year-old female patient, with a history of unresected parathyroid adenoma and pancreatitis, attended for 3 days of abdominal pain, moderate intensity, accompanied by emetic episodes of food content, initial paraclinical findings showed severe hypercalcemia, electrocardiogram with block grade I atrioventricular, arterial gases with acute respiratory alkalosis and hyperlactatemia. CT of the abdomen with brown tumors. She was admitted to the ICU for administration of intravenous fluids, loop diuretics, and cinacalcet without showing any improvement, so hemodialysis therapy was indicated. Discussion and conclusion: hypercalcemia is a frequent finding. Primary hyperparathyroidism and malignancy are the two most common causes of elevated serum calcium levels, together accounting for about 90 % of all cases. Serum values vary between total calcium 8.5 and 10.5 mg/dl and ionic 1.16- 1.31. Serum Ca 2+ concentration is closely related by the actions of parathyroid hormone and calcitriol. Primary hyperparathyroidism occurs as a result of adenomas, hyperplasias, or carcinoma. The clinical manifestations and severity will be correlated with the duration of the disease, calcium and PTH levels. Within the treatment, this will be guided by its cause, however, it is possible to classify it into urgent and non-urgent treatment. The approach to severe acute hypercalcemia is a diagnostic challenge given the multiple causes that can lead to this hydroelectrolyte disorder and the rapid establishment of treatment that is required when it is detected.

Four-dimensional computed tomography protocol for preoperative evaluation of the parathyroid glands and its correlations with other imaging methods: a pictorial essay / Protocolo de tomografia computadorizada quadridimensional para avaliação pré-operatória da paratireoide e correlação com outros métodos de imagem: ensaio iconográfico

Abstract Parathyroid adenoma is the most common cause of primary hyperparathyroidism. Advances in surgical techniques have made it possible to excise only the affected parathyroid gland in most cases. Imaging examinations play a fundamental role in the preoperative planning of parathyroidectomy. To localize the parathyroid glands, imaging tests such as scintigraphy, ultrasound, and, more recently, four-dimensional computed tomography (4D CT). The aim of this pictorial review was to illustrate the use of the 4D CT protocol in cases of parathyroid adenoma and to determine how well it correlates with other imaging methods, in order to improve understanding of the 4D CT method.

Resumo O adenoma de paratireoide é a causa mais comum de hiperparatireoidismo primário. Com o avanço das técnicas cirúrgicas, na maioria das vezes é realizada a retirada apenas da paratireoide afetada. Para que isso seja possível, os exames de imagem têm papel fundamental. São utilizados para a localização das paratireoides exames como a cintilografia e a ultrassonografia, e recentemente a tomografia computadorizada quadridimensional (TC 4D) com protocolo específico. O objetivo deste ensaio é descrever o uso do protocolo TC 4D em casos de adenoma da paratireoide e determinar sua correlação com os outros métodos de imagem, para facilitar o entendimento do método.

Hiperparatiroidismo primario por adenoma gigante de paratiroides. Abordaje quirúrgico selectivo / Primary hyperparathyroidism caused by giant parathyroid adenoma. Selective surgical approach / Hiperparatireoidismo primário devido a um adenoma gigante da paratireoide. Abordagem cirúrgica seletiva

Resumen: Introducción: el hiperparatiroidismo primario por un adenoma gigante de paratiroides es infrecuente. Los adenomas de mayor tamaño reportados ocurrieron sobre paratiroides ectópicas (mediastinales). Comparte con el carcinoma su gran tamaño y elevadas cifras de calcemia y de hormona paratiroidea, hecho que dificulta el diagnóstico. Su tratamiento quirúrgico es la paratiroidectomía mediante una cervicotomía transversa centrada en la región infrahioidea. Objetivo: presentar un caso clínico de hiperparatiroidismo primario por un adenoma gigante de paratiroides tratado quirúrgicamente mediante un abordaje selectivo. Caso clínico: paciente de 53 años, sexo femenino, con antecedentes de litiasis ureteral, dolores óseos y tumoración infrahiodea de 4 cm de diámetro que imagenológicamente presentó las características de un adenoma paratiroideo inferior izquierdo. La valoración funcional confirmó hiperparatiroidismo. Con diagnóstico de hiperparatiroidismo primario por adenoma gigante se trató quirúrgicamente a través de una incisión pequeña y centrada en la tumoración, realizándose la paratiroidectomía inferior izquierda con la cual remitió la sintomatología y normalizó la funcionalidad paratiroidea. Discusión: el hiperparatiroidismo primario por adenoma gigante de paratiroides tiene indicación quirúrgica y es curativo. El caso presentado demuestra la factibilidad y seguridad de un abordaje selectivo a lo que suma una menor afectación cosmética, dejando indemne la logia tiroidea contralateral ante futuras cirugías sobre ésta.

Summary: Introduction: primary hyperparathyroidism caused by giant parathyroid adenoma is a rather unusual condition. Reported large adenomas occurred in ectopic parathyroid glands (mediastinal). Just like carcinomas, they are large, present high calcemia and parathyroid hormone values, what complicates diagnosis. Surgical treatment consists in parathyroidectomy by means of transversal cervicotomy around the infrahyoid region. Objective: the study presents the clinical case of primary hyperparathyroidism caused by giant parathyroid adenoma that was treated by selective surgery approach. Clinical case: 53 year-old female patient with a history of uretheral lithiasis, bone pain and 4-cm-diameter infrahyoid tumor. Imagenology studies revealed the characteristics of lower left parathyroid adenoma. Functional assessment confirmed hyperparathyroidism. Upon the diagnosis of primary hyperparathyroidism caused by giant parathyroid adenoma it was surgically addressed by means of a small cut around the tumour and performing a lower left parathyroidectomy, what resulted in the remission of symptoms and normalized parathyroid functionality. Discussion: primary hyperparathyroidism caused by giant parathyroid adenoma has an indication for surgery and is therapeutic. The case presented shows the feasibility and safety of a selective approach, as well as its smaller cosmetic harm, managing to keep the contralateral thyroid loggia intact, in view of future surgeries involving it.

Resumo: Introdução: o hiperparatireoidismo primário devido a adenoma de paratireoide gigante é raro. Os maiores adenomas relatados ocorreram em paratireoides ectópicas (mediastinais). Compartilha com o carcinoma seu grande tamanho e altos níveis de cálcio e hormônio da paratireoide, o que torna o diagnóstico difícil. Seu tratamento cirúrgico é a paratireoidectomia por meio de cervicotomia transversa com foco na região infra-hióidea. Objetivo: apresentar um caso clínico de hiperparatireoidismo primário por adenoma gigante da paratireoide tratado cirurgicamente por abordagem seletiva. Caso clínico: paciente do sexo feminino, 53 anos, com história de litíase ureteral, dor óssea e tumor infra-hióideo de 4 cm de diâmetro que apresentava características de imagem de adenoma de paratireoide inferior esquerdo. A avaliação funcional confirmou hiperparatireoidismo. Com diagnóstico de hiperparatireoidismo primário por adenoma gigante, foi tratada cirurgicamente por meio de pequena incisão focada no tumor, realizando paratireoidectomia inferior esquerda com remissão dos sintomas e normalização da funcionalidade da paratireoide. Discussão: o hiperparatireoidismo primário devido ao adenoma gigante da paratireoide tem indicação cirúrgica e é curativo. O caso apresentado demonstra a viabilidade e segurança de uma abordagem seletiva que apresenta um menor envolvimento estético, deixando o espaço contralateral da tireoide sem danos para futuras cirurgias.

Puesta al día: carcinoma paratiroideo / An update on parathyroid carcinoma

Parathyroid carcinoma is a rare malignant disease that presents as a sporadic or familial primary hyperparathyroidism (PHP). The latter is associated with some genetic syndromes. It occurs with equal frequency in both sexes, unlike PHP caused by parathyroid adenoma that is more common in women. It should be suspected in cases of severe hypercalcemia, with high parathyroid hormone levels and a palpable cervical mass. Given the difficulty in distinguishing between parathyroid carcinoma and adenoma prior to the surgery, the diagnosis is often made after parathyroidectomy. The only curative treatment is complete surgical resection with oncologic block resection of the primary tumor to ensure free margins. Adjuvant therapies with chemotherapy or radiation therapy do not modify overall or disease-free survival. Recurrences are common and re-operation of resectable recurrent disease is recommended. The palliative treatment of symptomatic hypercalcemia is crucial in persistent or recurrent disease after surgery since morbidity and mortality are more associated with hypercalcemia than with tumor burden.

Hiperparatiroidismo primario: conceptos para el cirujano general / Primary hyperparathyroidism: concepts for the general surgeon

Durante las últimas décadas, la incidencia del hiperparatiroidismo primario ha venido en aumento, muy probablemente relacionado con la mayor accesibilidad a los estudios diagnósticos; sin embargo, la forma más común de presentación clínica del hiperparatiroidismo primario es asintomática, en más del 80 % de los pacientes. En la actualidad, es menos frecuente el diagnóstico por las complicaciones renales (urolitiasis) u óseas (osteítis fibrosa quística) asociadas. Un tumor benigno de la glándula paratiroides (adenoma único), es la principal causa de esta enfermedad. Por tanto, su tratamiento usualmente es quirúrgico. A pesar de ello, no es frecuente el manejo de esta patología por el cirujano general. En este artículo se revisan conceptos claves para el diagnóstico y manejo de esta enfermedad para el médico residente y especialista en Cirugía general

During the last decades, the incidence of primary hyperparathyroidism has been increasing, most probably related to the greater accessibility to diagnostic studies; however, the most common form of clinical presentation of primary hyperparathyroidism is asymptomatic in more than 80% of patients. Diagnosis is less frequent due to associated renal (urolithiasis) or bone (osteitis fibrosa cystica) complications. A benign tumor of the parathyroid gland (single adenoma) is the main cause of this disease. Therefore, its treatment is usually surgical. Despite this, the management of this pathology by the general surgeon is not frequent. This article reviews key concepts for the diagnosis and management of this disease for the resident physician and specialist in General Surgery

Parathyroid hemangioma

Background Hemangiomas are benign neoplasms of capillary proliferation that arise from a developmental anomaly where angioblastic mesenchyme fails to form canals. Most hemangiomas arise in the head and neck region, either superficially in the skin or deeper within endocrine organs such as the parotid gland. Parathyroid hemangiomas, however, are extremely rare, with only five cases previously reported in the literature. Case presentation Herein, we present a case of a 68-year-old man with a hemangioma almost completely replacing the right upper parathyroid gland, grossly measuring 1.3 × 1.3 × 1.2 cm and weighing 700 mg, associated with primary hyperparathyroidism. Conclusions Parathyroid gland enlargement due to vascular neoplasms such as hemangiomas can mimic, both clinically and radiographically, hyperplasias and/or adenomas. Surgeons need to be aware of the presence of this entity and should consider it in the differential diagnosis of hyperparathyroidism or parathyroid gland enlargement.

Disparity between Preoperative and Pre-Excisional Intraoperative Parathyroid Hormone in Parathyroid Surgery

Abstract Introduction Intraoperative parathyroid hormone (ioPTH) testing is a widely accepted standard for assessing the parathyroid gland function. A decline of preoperative parathyroid hormone (PTH) levels by more than 50% is one accepted measure of parathyroid surgery adequacy. However, there may be a variation between preoperative PTH levels obtained at a clinic visit and pre-excisional ioPTH. Objective Our study explores the differences between preoperative PTH and pre-excisional ioPTH levels, and the potential impact this difference has on determining the adequacy of parathyroid surgery. Methods A retrospective study that consisted of 33 patients that had undergone parathyroid resection between September 2009 and March 2016 at a tertiary academic center was performed. Each subject's preoperative PTH levels were obtained from clinic visits and pre-excisional ioPTH levels were recorded along with the time interval between the measurements. Results There was a significant difference between the mean preoperative PTH and the pre-excisional ioPTH levels of 147 pg/mL (95% confidence interval [CI] 11.43 to 284.47; p= 0.0396). The exclusion of four outliers revealed a further significant difference with a mean of 35.09 pg/mL (95% CI 20.27 to 49.92; p< 0.0001). The average time interval between blood draws was 48 days + 32 days. A weak correlation between the change in PTH values and the time interval between preoperative and pre-excision blood draws was noted (r2 = 0.15). Conclusion Our study reveals a significant difference between the preoperative PTH levels obtained at clinic visits and the pre-excisional intraoperative PTH levels. We recommend routine pre-excisional intraoperative PTH levels, despite evidence of elevated preoperative PTH levels, in order to more accurately assess the adequacy of surgical resection.

New features in diagnosis and treatment of parathyroid tumors co-existent with thyroid disease / 中国耳鼻咽喉头颈外科

OBJECTIVE To analyze the new features in diagnosis and treatment of parathyroid tumor co-existent with thyroid disease. METHODS The clinical data of 98 patients with parathyroid tumors from Jan.2007 to Dec.2016, in Zhejiang Cancer Hospital were retrospective analyzed. RESULTS In all 98 cases, there were 22 cases mistaken as thyroid disease before surgery. The misdiagnosis rate was higher(36.2％), when combined with thyroid disease. When co-existent with thyroid disease, the qualitative diagnosis rate of the ultrasound and CT will be decreased, it was easy to misdiagnosis. Logistic regression analysis showed that parathyroid tumor location, hyperparathyroidism, cystic parathyroid tumor affected the accuracy of the qualitative diagnosis of ultrasound. The size of the parathyroid tumor affected the accuracy of the qualitative diagnosis of CT. Three of 9 patients underwent bilateral thyroid cancer and parathyroid tumor resection presented permanent hypoparathyroidism(33％). CONCLUSION When combined with thyroid disease, parathyroid tumor is easy to be misdiagnosed, the qualitative diagnosis rate of the ultrasound and CT will also decreased. When combined with thyroid cancer, these will be a higher risk of hypoparathyroidism after bilateral thyroid cancer and parathyroid tumor resection.

Resultados del tratamiento quirúrgico del hiperparatiroidismo primario en un país en vías de desarrollo / Outcomes of Primary Hyperparathyroidism Surgical Treatment in a Low-Middle Income Country

Introducción: El hiperparatiroidismo primario es una enfermedad común y con una distribución similar en todo el mundo. El propósito del estudio fue establecer si la presentación clínica y bioquímica, así como los resultados de su tratamiento quirúrgico, difieren en un país en vías de desarrollo, que no cuenta con todos los recursos diagnósticos y terapéuticos recomendados actualmente. Metodología: Análisis retrospectivo de pacientes operados por un mismo equipo, entre 1992 y 2015. Se obtuvo información sobre presentación clínica, resultados de estudios preoperatorios, procedimientos quirúrgicos, diagnóstico histopatológico y evolución postoperatoria. Resultados: Se operaron 55 pacientes con edad promedio de 45 años, 78% mujeres. El 65% eran sintomátcos. El valor promedio preoperatorio de calcio sérico fue 11.2 mg/dl, PTH 167.1 pg/ml, fósforo 2.6 mg/ dl, 25-hidroxi vitamina D 17.3 ng/ml y calcio urinario de 24 horas 294.7 mg. Al 59% se realizó estudios de localización preoperatoria. La sensibilidad del ultrasonido fue 57.14% y 75% para centellografa con tecnecio sestamibi. La positividad de los estudios de localización determinó el tipo de exploración quirúrgica (p=0.02). Se practcaron 27 (49%) exploraciones unilaterales y 28 (51%) bilaterales, resecando 47 (85.5%) adenomas solitarios y 3 ½ glándulas en 7 (12.7%) casos de hiperplasias. En 27 (36%) coexista patología tiroidea. Las tasas de curación, persistencia y recurrencia fueron 94.5%, 5.5% y 3.6% respectivamente. Conclusiones: La mayoría de nuestros pacientes operados son jóvenes y sintomátcos. La estrategia quirúrgica fue condicionada por los estudios de localización. Nuestras tasas de curación, persistencia y recurrencia son comparables a las reportadas.

Background: Primary hyperparathyroidism (HPTP) is a common disease with widespread distribution around the world. The aim of this study was to establish if clinical and biochemical disease characteristics and long term results differ in patents with HPTP in a low-middle income country without all recommended diagnostc and therapeutc resources. Methods: Retrospective collection of clinical diagnosis, biochemical, operative details, histology and long term results of all surgically treated patents with HPTP, from 1992 to 2015, by the same surgical team. Results: 55 patents with HPTP were analyzed. Average age is 45 years old with 78% of female patents. Sixty five percent were symptomatic. The mean preoperative serum calcium level was 11.2 mg/dl, PTH 167.1 pg/ml, phosphorus 2.6 mg/ dl, vitamin D 17.3 ng/ml and 24 hour urinary calcium 294.7 mg. Fifty nine percent of the patents had preoperative imaging. Ultrasound and sestamibi scan sensitivity was 57.1% and 75% respectively. Unilateral localization in preoperative imaging determined surgical exploration (p=0.02). Unilateral approach was used in 27 (49%) patents and bilateral in 28 (51%); 47 (85.5%) solitary adenomas and 7 (12.7%) 3 ½ gland resections of hyperplastic glands were performed. Thyroid pathology co-existed in 27 (36%) patients. Cure, persistence and recurrence rates were 94.5%, 5.5% y 3.6% respectively. Conclusions: In this study most of the patents were young and symptomatc. Surgical strategy was determined by preoperatve imaging. Cure, persistence and recurrence rates were comparable to published literature.

Análisis de falsos negativos en la cintigrafía SPECT de paratiroides con sestamibi en pacientes con hiperparatiroidismo primario sometidos a cirugía entre 2008-2015 en hospital universitario / False negative spect parathyroid scintigraphy with sestamibi in patients with primary hyperparathyroidism

Background: 99mTc-sestamibi parathyroid SPECT scintigraphy is a useful tool in the pre-operative study of hyperparathyroidism. False negatives (FN) have been reported in 5.7-14% of the examinations. Aim: To characterize 99mTc-sestamibi FN in cases referred for primary hyperparathyroidism (PHP) to a university hospital. Material and Methods: Descriptive retrospective analysis. We included patients with PHP, studied with SPECT scintigraphy, operated at our center between 2008 and 2015. Clinical and surgical data were recorded; biopsies of the FN were blindly reviewed by one pathologist. Results: One hundred twenty one scintigraphies fulfilled the inclusion criteria. Seven (5.8%) were negative and 114 positive. There was no difference in age, sex and PTH levels between FN and true positive scintigraphies. At surgery, one FN case had two hyperplasic glands and two cases had ectopic glands. Pathology reported adenoma in three cases, hyperplasia in three and carcinoma in one. The largest diameter of the lesion was lower in FN (1.3 and 2.1 cm respectively, p = 0.02) and the proportion of adenomas was higher in true positive cases (29% and 75% respectively; p < 0.01). The interval between scintigraphy and parathyroidectomy was greater in FN with a median of 92 days (range 20 days-3.2 years, p < 0.01). The percentage of oxyphilic cells observed was similar in both groups. Conclusions: FN parathyroid SPECT scintigraphies in PHP are uncommon. They corresponded to lesions under the equipment's resolution limit and resulted in longer time lags between scintigraphy and surgery.

Successful Radiofrequency Ablation of Parathyroid Adenoma in an Older Patient with Primary Hyperparathyroidism / 대한내과학회지

Primary hyperparathyroidism is one of the most common endocrine diseases and is defined as the inappropriate overproduction of parathyroid hormone, resulting in hypercalcemia. It occurs mostly as a result of parathyroid adenoma or hyperplasia. The incidence of primary hyperparathyroidism increases with advancing age. The standard treatment of symptomatic primary hyperparathyroidism is parathyroidectomy; however, in older patients with multiple comorbidities, the risks associated with surgical treatment involving general anesthesia are high. Compared with surgery, radiofrequency abalation (RFA) is a minimally invasive procedure, in which the mass is removed. We here present a case of an elderly patient with primary hyperparathyroidism associated with parathyroid adenoma who was successfully treated with ultrasonography-guided RFA. RFA is an alternative therapeutic option for treatment of primary hyperparathyroidism for high-risk elderly patients, and further evaluation of its clinical value is warranted.

Synchronous parathyroid carcinoma and papillary thyroid carcinoma in a patient with long-standing schizophrenia

No abstract available.

Multiple endocrine neoplasia type 1 with anterior mediastinal parathyroid adenoma: successful localization using Tc-99m sestamibi SPECT/CT

The most common manifestation of multiple endocrine neoplasia type 1 (MEN1) is hyperparathyroidism. Treatment of hyperparathyroidism in MEN patients is surgical removal of the parathyroid glands, however ectopic parathyroid gland is challenging for treatment. A 51-year-old female, the eldest of 3 MEN1 sisters, had hyperparathyroidism with ectopic parathyroid adenoma in the mediastinal para-aortic region, which was detected by technetium-99m (Tc-99m) sestamibi scintigraphy and single-photon emission computed tomography/computed tomography (SPECT/CT). She underwent total parathyroidectomy with video-assisted thoracoscopic surgery on an anterior mediastinal mass. Anterior mediastinal parathyroid adenoma in MEN1 patients is rare. Precise localization of an ectopic parathyroid gland with Tc-99m sestamibi SPECT/CT can lead to successful treatment of hyperparathyroidism. This is the first reported case in the literature of mediastinal parathyroid adenoma in MEN1 patient visualized by Tc-99m sestamibi SPECT/CT.

Macroadenoma cervical palpable: Una presentación muy inusual de hiperparatiroidismo primario / Cervical macroadenoma causing hyperparathyroidism: Report of one case

We report a 59-year-old man with a history of hypertension, recurrent renal stones and a severe hypercalcemia of 14.9 mg/dl with a serum phosphorus of 2.4 mg/dl and a serum albumin of 3.6 g/dl. Physical examination showed a 4 cm left cervical nodule, consistent with the diagnosis of thyroid nodule. Parathyroid hormone (PTH) levels were 844 pg/mL (normal 15-65 pg/ml) and a cervical ultrasound examination disclosed a solid nodule in the lower left lobe of 40 x 30 x 25 mm, adjacent to the thyroid parenchyma. Abdominal ultrasound revealed bilateral renal stones. Parathyroid scintigraphy showed a high uptake of the left lower parathyroid mass and a bone densitometry showed bone density t scores of -1.2 in the spine, -2.0 in the right femoral neck and -3.5 in the distal radius. A review of his medical record revealed the presence of hypercalcemia for at least 4 years. He was admitted for hydration and administration of 4 mg zoledronic acid iv. At 24 hours, serum calcium dropped to 11.0 mg/dl, and a left thyroid lobectomy was performed including the lower left parathyroid gland. The pathology report showed a 22.6 g parathyroid adenoma. Intraoperatory PTH descended > 50%, consistent with successful parathyroidectomy. At 7 days after surgery serum calcium was 8.8 mg/dl, phosphorus 2.1 mg/dl, alkaline phosphatase 166 U/L, albumin 3.9 g/dL, PTH 230 pg/ml and 25-OH vitamin D 12.4 ng/ml. This finding was interpreted as secondary hyperparathyroidism due to vitamin D deficiency and “hungry bone”, being less likely the presence of residual or metastatic parathyroid tissue. A cholecalciferol load was administered, with significant descent of PTH.

Comparison of Ultrasonography and 99mTc-sestamibi Scan for Preoperative Localization of Parathyroid Adenoma / 대한내과학회지

BACKGROUND/AIMS: Primary hyperparathyroidism can be cured by minimally invasive surgery (MIS) with optimized preoperative localization. Ultrasonography (US) and 99mTc-sestamibi (MIBI) scan are the imaging modalities most widely used for the localization of the affected glands. In this study, we defined the roles of US and MIBI scan. METHODS: We retrospectively reviewed 40 patients who underwent parathyroidectomy for a single parathyroid adenoma between 2004 and 2013. US and scintigraphic findings were compared with operative findings. RESULTS: Adenomas were accurately localized using US and MIBI scan in 38 patients (95%) and 37 patients (92.5%), respectively. Twenty-nine patients (76.3%) showed typical extrathyroidal hypoechoic nodule with central or peripheral vascularity, and, after MIS, we confirmed that they were suffering from a single parathyroid adenoma. Eight patients with atypical US findings and two patients with an undetectable lesion on US underwent MIS after localization using MIBI scan or computed tomography (CT). Only one patient showed an extrathyroidal cystic nodule evidenced by high parathyroid hormone cystic fluid on ultrasound-guided fine-needle aspiration and negative MIBI scan. All lesions not localized on US were located in the superior portion. CONCLUSIONS: US is a sensitive and accurate method for the preoperative localization of parathyroid adenoma, especially if the lesion has typical US features and is located inferiorly. We suggest that US be the first localization modality and that MIBI scan or CT be used in the limited number of cases with negative US findings.

Primary Hyperparathyroidism with Extensive Brown Tumors and Multiple Fractures in a 20-Year-Old Woman

A brown tumor is a benign fibrotic, erosive bony lesion caused by localized, rapid osteoclastic turnover, resulting from hyperparathyroidism. Although brown tumors are one of the most pathognomonic signs of primary hyperparathyroidism, they are rarely seen in clinical practice. In this report, we present a case of 20-year-old woman with recurrent fractures and bone pain. Plain digital radiographs of the affected bones revealed multiple erosive bone tumors, which were finally diagnosed as brown tumors associated with primary hyperparathyroidism due to a parathyroid adenoma. This case shows that multiple, and clinically severe form of brown tumors can even occur in young patients.

Hiperparatireoidismo primário: apresentação atípica. Relato de caso / Primary hyperparathyroidism: an atypical presentation. Case report

O objetivo deste estudo foi relatar um caso de hiperparatireoidismo primário com características clínicas, laboratoriais e de imagem atípicas, levando a suspeita diagnóstica de carcinoma de para tireoide que é uma causa rara de hiperparatireoidismo primário. Paciente do gênero masculino, 55 anos, apresentava quadro de perda ponderal significativa, astenia e mal estar generalizado. Ao exame físico, foi encontrada uma massa cervical palpável à esquerda. Procedeu-se assim à investigação laboratorial que evidenciou aumento das concentrações de cálcio e de paratormônio. Foi realizada ultrassonografia cervical que confirmou a presença de nódulo cervical e cintilografia por SESTAMIBI-99mTc que mostrou nódulo hipercaptante, de 2,5cm de diâmetro, próximo à tireoide. Outros exames evidenciaram litíase renal e lesões ósseas líticas disseminadas. Tais achados levaram à hipótese de hiperparatireoidismo primário causado por carcinoma de paratireoide. O paciente foi então submetido a procedimento cirúrgico e à biópsia de congelamento da lesão. Esta revelou características benignas, afastando a hipótese de carcinoma e a necessidade de uma ressecção inbloc associada à linfadenectomia, procedimento indicado em caso positivo para malignidade. A análise anatomopatológica do nódulo evidenciou um adenoma. Um ano após o tratamento,o paciente permanece livre de doença. O carcinoma de paratireoide deve ser considerado quando da presença de níveis extremamente elevados de cálcio sérico e paratormônio. Apesar do diagnóstico de carcinoma não ter se confirmado na análise histopatológica, a manutenção desta patologia entre os diagnósticos diferenciais foi essencial para a abordagem adequada do caso e para sua exclusão diagnóstica com maior segurança...

The aim of this article is to report a case of primary hyperparathyroidism with atypical clinical, laboratory and imaging features. These findings raised a diagnostic suspicion for parathyroid carcinoma, a rare cause of primary hyperparathyroidism. A 55 year-old male patient presented complaints of significant weight loss, weakness and general malaise. He also had a palpable cervical mass. Imagingm studies evidenced nephrolithiasis and diffuse lytic bone lesions. Laboratory tests showed very high levels of serum calcium and parathormone. These findings prompted a neck ultrasonography and SESTAMIBI-99mTc scintigraphy, which revealed a 2.5cm nodule with increased captation near the thyroid gland. The patient was submitted to surgical exploration and a frozen section biopsy of the lesion. The lesion showed benign patterns, and there was, therefore, no need for an in bloc resection and lymphadenectomy, which is the appropriate procedure in malignancy cases. The histopathologic analysis of the nodule revealed an adenoma. At the one year follow-up, the patient remained disease-free. Parathyroid carcinoma must be considered in cases with very high levels of serum calcium and parathormone. Although the diagnosis of carcinoma was not confirmed histologically, keeping this pathology in mind among the differential diagnosis was essential for the appropriate management of the case and the safe diagnostic exclusion of that malignancy...

Brown tumors of the femur and pelvis secondary to a parathyroid carcinoma: Report of one case / Tumores pardos de fémur y pelvis secundarios a carcinoma de paratiroides: Informe de un caso

Brown tumors result from excess osteoclast activity and consist of collections of osteoclasts intermixed with fibrous tissue and poorly mineralized woven bone. They are secondary to hyperparathyroidism (HPT). Their incidence is higher in primary than in secondary hyperparathyroidism. We report a 69 years-old male, admitted in a state of confusion, lethargy and bedridden, with a pathological fracture of the femur caused by a brown tumor. The laboratory examination revealed a hypercalcemia (8.85 mEq/L), with high levels of ionized Ca (5.48mEq/L), serum alkaline phosphatases (416 U/L) and serum parathormone (120 pg/mL). Ultrasound examination of the neck showed a large parathyroid tumor, probably corresponding to a carcinoma. A primary HPT was diagnosed. The patient was hydrated and high doses of diuretics and bisphosphonates were administered. After correction of serum calcium and neurologic symptoms, the patient was operated, performing an extensive resection of the tumor. The pathology report confirmed the diagnosis of parathyroid carcinoma.

Los tumores pardos son una consecuencia de una actividad osteoclástica excesiva y consisten en osteoclastos mezclados con tejido fibroso y tejido óseo mal mineralizado. Son secundarios a hiperparatiroidismo y más comunes en hiperparatiroidismo primario. Informamos de un hombre de 69 años que ingresa confuso y letárgico con una fractura patológica del fémur causada por un tumor pardo. El laboratorio mostró hipercalcemia de 8,85 mEq/L, fosfatasas alcalinas de 416 U/L y parathormona de 120 pg/mL. La ecografía del cuello mostró un tumor paratiroideo sospechoso de carcinoma. Se diagnosticó un hiperparatiroidismo primario. El paciente se hidrató y estabilizó con diuréticos y bifosfonatos. Una vez estabilizado, se operó efectuando una extensa resección del tumor. El estudio anatomopatológico confirmó el diagnóstico de cáncer de paratiroides.

Intrathyroidal parathyroid tumor: A report of three cases and literature review / 肿瘤

Objective: To enhance the understanding of the diagnosis and treatment of intrathyroidal parathyroid tumor by retrospective review of the medical records of three patinets with intrathyroidal parathyroid tumor. Methods: A retrospective analysis of medical records was conducted for three patients with intrathyroidal parathyroid tumor in Tianjin Medical University Cancer Institute and Hospital between January 2011 and June 2013, and the review of the related literatures was performed. Results: All of the three patients with intrathyroidal parathyroid tumor were females, aged 37 to 56 years; of the three patients, two had parathyroid adenoma, one had parathyroid carcinoma. All patinets did not have hyperparathyroidism. Conclusion: Intrathyroidal parathyroid tumor is very rare. The error in diagnosis is common in clinical practice. Accuracy in positioning and qualitative diagnosis is essential to achieve success in treatment. Copyright © 2014 by TUMOR.

Incidental Findings with Technetium-99m Methoxyisobutrlisonitrile Imaging of Ectopic Parathyroid Hyperplasia in Patient with Intrathyroidal Parathyroid Hyperplasia

We report a case of intrathyroidal and ectopic parathyroid hyperplasia in a 47-year-old female patient with secondary hyperparathyroidism. During evaluation for end stage renal disease, hypercalcemia and hyperparathyroidism were detected in laboratory exam and thyroid nodules were found in computed tomography, and thyroid ultrasonography. In subsequent dual-phase technetium-99m methoxyisobutrlisonitrile (Tc-99m MIBI) scan, delayed focal uptakes were found in thyroid gland and the upper mediastinum. I-123 scan showed photon defects in thyroid gland and no uptake in the upper mediastinum. The imaging findings indicate intrathyroidal and mediastinal ectopic parathyroid hyperplasia. The intrathyroidal and ectopic parathyroid hyperplasia were confirmed by surgical approach. Tc-99m MIBI scan was useful for detecting ectopic parathyroid hyperplasia concomitant intrathyroidal parathyroid lesion.