Pancreatitis aguda en el escenario de hipercalcemia por hiperparatiroidismo primario / Acute pancreatitis in the setting of hypercalcemia due to primary hyperparathyroidism

Introducción: describir el caso de un paciente con pancreatitis aguda secundaria a hipercalcemia por hiperparatiroidismo prImario. Esta es una causa poco frecuente de pancreatitis, asociada a morbimortalidad significativa en caso de no ser diagnosticada oportunamente Caso clínico: un hombre de 44 años, con antecedente de pancreatitis de presunto origen biliar que había requerido previamente colecistectomía, consultó por dolor abdominal y náuseas. Los estudios complementarios fueron compatibles con un nuevo episodio de pancreatitis aguda. Presentaba hipercalcemia y hormona paratiroidea (PTH) elevada, configurando hiperparatiroidismo primario. La gammagrafía informó hallazgos compatibles con adenoma paratiroideo. Se inició tratamiento con reanimación hídrica y analgesia con adecuada disminución de calcio sérico y resolución de dolor abdominal. Después de la paratiroidectomía se logró normalizar los niveles de calcio y PTH. Discusión: la pancreatitis aguda es una condición potencialmente fatal, por lo que la sospecha de causas poco frecuentes como la hipercalcemia debe tenerse en cuenta. El tratamiento de la hipercalcemia por adenoma paratiroideo se basa en reanimación hídrica adecuada y manejo quirúrgico del adenoma, con el fin de evitar recurrencia de pancreatitis y mortalidad. (AU)

Introduction: we describe the case of a patient with acute pancreatitis secondary to hypercalcemia due to primary hyperparathyroidism. This is a rare cause of pancreatitis associated with significant morbidity and mortality if not diagnosed in time. Clinical case: a 44-year-old man with a history of pancreatitis of presumed biliary origin, which had previously required cholecystectomy, consulted for abdominal pain and nausea. The laboratory findings were compatible with a new episode of acute pancreatitis. He presented hypercalcemia and an elevated parathyroid hormone (PTH), configuring primary hyperparathyroidism. Scintigraphy was performed, yielding findings compatible with parathyroid adenoma. Treatment with fluid resuscitation and analgesia was started, resulting in an adequate decrease in serum calcium and resolution of abdominal pain. After parathyroidectomy, calcium and PTH levels were normalized. Discussion: acute pancreatitis is a potentially fatal condition; therefore the suspicion of rare causes, such as hypercalcemia, should be considered. The treatment of hypercalcemia due to parathyroid adenoma is based on adequate fluid resuscitation and surgical management of the adenoma, to avoid recurrence of pancreatitis and death. (AU)

Crisis hipercalcémica, presentación inusual de adenoma paratiroideo: reporte de 2 casos / Hypercalcemic crisis, unusual presentation of parathyroid adenoma

La crisis hipercalcémica (CH) es una emergencia endocrina inusual, definida por la presencia de calcemia > 14 mg/dl asociada a disfunción renal, alteraciones cardiovasculares, gastrointestinales y del sensorio; también podría considerarse en pacientes con síntomas graves y calcemia menor. El hiperparatiroidismo primario (HPTP) y las neoplasias malignas son las etiologías más comunes de la hipercalcemia (90% de los casos); sin embargo, rara vez el primero se presenta como CH. Debido a la alta mortalidad asociada a esta entidad, es de gran importancia establecer diagnóstico y tratamiento precoces. Presentamos dos pacientes con crisis hipercalcémica como primera manifestación del HPTP, el 1.° con bloqueo auriculoventricular (AV) completo y el 2.° con pancreatitis aguda. La anatomía patológica (AP) reveló adenoma oxifílico en ambos casos, que es una variante histológica poco frecuente y puede manifestarse clínicamente de forma grave. Conclusiones: los adenomas paratiroideos son causa poco frecuente de CH. Consideramos el tipo histológico observado (adenoma oxifílico) como probable factor condicionante. La pancreatitis y especialmente el bloqueo AV son manifestaciones poco frecuentes de la CH. Resaltamos la importancia de la determinación de los niveles de calcio dentro de la evaluación inicial de todo paciente con bloqueo AV. (AU)

Hypercalcemic crisis (HC) is an unusual endocrine emergency, defined as the presence of serum calcium > 14 mg/dl related to kidney dysfunction, cardiovascular, gastrointestinal and sensory disturbances. It could also be considered in patients with severe symptoms and lower serum calcium levels. Primary hyperparathyroidism (PHPT) and malignant neoplasms are the most common hypercalcemia etiologies (90% of cases), nevertheless, the former hardly ever occurs as HC. Due to the high mortality associated with HC, it is crucial to establish early diagnosis and treatment.We report two patients with HC as the first manifestation of PHPT; the former with atrioventricular (AV) block and the latter with acute pancreatitis. Pathology revealed oxyphilic adenoma in both cases, which is an infrequent histological variant that can have a severe clinical manifestation. Conclusions: parathyroid adenomas are a rare cause of HC. We consider the histological type observed (oxyphilic adenoma) as a probable conditioning factor. Pancreatitis and especially AV block are rare manifestations of HC. We emphasize the importance of determining calcium levels in the initial evaluation of all patients with AV block. (AU)

Hiperparatiroidismo primario por carcinoma paratiroideo: caso problema y revisión / Primary hyperparathyroidism by parathyroid cancer: case report and review

El carcinoma paratiroideo (CP) es una neoplasia maligna con una incidencia de 0,015 cada 100.000 habitantes por año. Representa el 1% de los diagnósticos de hiperparatiroidismo primario y se manifiesta entre la 4.a y 5.a década de la vida, con una incidencia similar entre hombres y mujeres. La etiología del CP es incierta, ha sido asociada a formas esporádicas o familiares. Está caracterizado por altos niveles séricos de calcio y PTH y el desafío clínico-quirúrgico es el diagnóstico diferencial con otras entidades benignas como el adenoma o la hiperplasia de paratiroides. Aunque el diagnóstico de certeza es anatomopatológico, la sospecha clínica y el uso de métodos de baja complejidad (ecografía) con operadores avezados permite una correcta localización y abordaje pertinente del paciente para dirigir el tratamiento quirúrgico adecuado (resección en bloque) evitando persistencias y recurrencias de enfermedad. Se presenta el caso clínico de un paciente masculino que ingresa por síndrome de impregnación asociado a hipercalcemia, su abordaje diagnóstico, tratamiento y manejo interdisciplinario con discusión y revisión bibliográfica. (AU)

Parathyroid carcinoma (CP) is a malignant disease with an incidence of 0.015 per 100,000 inhabitants per year. It accounts for 1% of primary hyperparathyroidism diagnoses and occurs between the 4th and 5th decade of life, with a similar incidence between men and women. The etiology of CP is uncertain and has been associated with sporadic or family forms. CP is characterized by high serum calcium and PTH levels and the clinical-surgical challenge is the differential diagnosis with other benign entities such as parathyroid adenoma or hyperplasia. Although the diagnosis of certainty is achieved by pathological anatomy examination, the clinical suspicion and the use of low complexity methods (ultrasound) by experienced operators allows a correct localization and a patient-specific approach to direct the appropriate surgical treatment (block resection), avoiding persistence and recurrences of disease. The clinical case of a male patient admitted for severe hypercalcemia with multiple organ disfunction, the diagnostic approaches, treatment, and interdisciplinary management, together with review and discussion of the current literature are presented. (AU)

Preeclampsia as an inaugural manifestation of primary hyperparathyroidism: A case report / Pré-eclâmpsia como manifestação inaugural de hiperparatiroidismo primário: Um caso clínico

Abstract Primary hyperparathyroidism is an endocrine disorder characterized by hypercalcemia and elevated or inappropriately normal levels of parathyroid hormone. The diagnosis is based on a biochemical evaluation, and a neck ultrasound is the first choice during pregnancy to access the parathyroid glands. Manifestations during pregnancy are rare and can be present with life-threatening complications, so the diagnosis is challenging. The conservative treatment is limited, and there is not enough data about its safety and efficacy during pregnancy. Surgery is the only curative treatment, and a parathyroidectomy performed during the second or third trimesters is considered safe. Recently, some authors suggested an association between primary hyperparathyroidism and preeclampsia. We describe a case of preeclampsia with severe features at 27 weeks of gestational age. The severity of the preeclampsiamotivated an early termination of the pregnancy by cesarean section. During the postpartum period, the patient presented life-threatening complications, such as severe hypercalcemia and acute pancreatitis. An ultrasound exam found two parathyroid nodules, suggestive of parathyroid adenomas. The patient recovered after the pharmacological correction of the calcemia levels.

Resumo O hiperparatiroidismo primário é umdistúrbio endócrino caraterizado pela elevação do cálcio sérico associada a níveis de paratormona elevados ou inapropriadamente normais. O diagnóstico é baseado em análises bioquímicas, e, na gravidez, o exame de imagem de primeira linha é a ecografia cervical. É uma doença rara na gravidez, e pode se apresentar com complicações ameaçadoras de vida, pelo que o seu diagnóstico é desafiante. O tratamento médico disponível é limitado, havendo poucos dados relativos à sua eficácia e segurança na gravidez. A cirurgia é o único tratamento curativo, e pode ser realizada no segundo ou terceiro trimestres. Tem sido descrita uma relação entre hiperparatiroidismo primário e pré-eclâmpsia. Apresenta-se um caso de uma grávida de 27 semanas com pré-eclâmpsia com critérios de gravidade, o que obrigou ao término da gravidez por cesariana. Verificou-se agravamento clínico no período pós-parto, com aparecimento de complicações graves, tais como hipercalcemia grave e pancreatite aguda. Ecograficamente, constataram-se duas massas paratiróideias sugestivas de adenomas da paratiroide. A doente recebeu tratamento médico, e teve melhora apenas após a correção dos níveis de cálcio sérico.

Hiperparatiroidismo primario: reporte de caso clínico / Primary Hyperparathyroidism: Case report

Antecedentes: El hiperparatiroidismo primario, tercer trastorno metabólico más común a nivel mundial, provoca un aumento del calcio sérico y de la hormona paratiroidea, causado por una hiperactividad de las glándulas paratiroideas. En el 85% de los casos, se debe a un adenoma paratiroideo. Es predominantemente asintomático (>80%), pero sus manifestaciones pueden ser musculo-esqueléticas, neurológicas, psiquiátricas, renales, cardiovasculares, y gastrointestinales. Descripción del Caso Clínico: Paciente femenina de 53 años de edad, con antecedente de hipercolesterolemia familiar heterocigoto e hipertensión arterial; presenta insomnio, náuseas, pérdida de peso, mialgias, astenia, relujo gastroesofágico e irritabilidad. Ante indicación de exámenes de labo-ratorio muestra hipercalcemia leve. Se comprueba un adenoma paratiroideo derecho y nódulos tiroideos coloides benignos en lóbulo izquierdo mediante gammagrafía Tc99m MIBI, siendo sometida a su resección con posterior evolución satisfactoria. Conclusiones: Considerando la predilección asintomática del hiperparatiroidismo primario y su preferencia de presentación como adenoma, resulta indispensable su sospecha ante toda hipercalcemia. Requiere entonces una concientización amplia no solo en el ámbito médico sino también en la comunidad, ampliando esfuerzos entre el clínico, cirujano, y patólogo. Se recomienda medir el calcio y fósforo sérico de forma rutinaria en la evaluación clínica cotidiana de cada paciente para su detección...(AU)

Cáncer de paratiroides e hipercalcemia de difícil manejo: un desafío diagnóstico / Parathyroid cancer and hypercalcemia difficult to manage: a diagnostic challenge

INTRODUCCIÓN: El carcinoma de paratiroides es una enfermedad de difícil diagnóstico, siendo perentorio una detección precoz y un tratamiento oportuno para prevenir las complicaciones. CASO CLÍNICO: Se presenta paciente de 42 años que debuta con hipercalcemia de 16.1 mg/dl, PTH 1573 pg/mL y lesión sugerente de adenoma de paratiroides. Biopsia quirúrgica identifica carcinoma paratiroideo sin invasión, realizándose posteriormente lobectomía derecha con foco de 0,1 mm de carcinoma paratiroideo, con bordes libres. En comité oncológico se decide seguimiento estricto; sin embargo, a los seis meses requiere hospitalización nuevamente por hipercalcemia, a la ecografía cervical presenta dos nódulos hipoecogénicos menores a 1 cm en lecho quirúrgico. Tomografía computada sin evidencia de lesiones. Con estos antecedentes, se decide exploración cervical, encontrándose tumor de 2 cm, multilobulado, paraesofágico. Biopsia evidencia carcinoma paratiroideo con invasión en tejido graso y músculo estriado. Se descarta radioterapia paliativa y quimioterapia debido a escasa evidencia, quedando en cuidados paliativos. DISCUSIÓN: El cáncer de paratiroides es una enfermedad de difícil diagnóstico. En muchos casos se ha descrito la crisis hipercalcémica como presentación inicial. La resección en bloque de la lesión de paratiroides con hemitiroidectomía ipsilateral es el tratamiento estándar. Es un tumor radio resistente y la quimioterapia adyuvante no ha demostrado aumento en la sobrevida. En pacientes con enfermedad inoperable, el pronóstico es pobre, siendo fundamental el control de calcemia y PTH, las cuales son la causa principal de morbimortalidad. CONCLUSIONES: El carcinoma paratiroideo es una enfermedad rara, cuyo diagnóstico y tratamiento representan un verdadero desafío clínico, siendo crucial el alto índice de sospecha. Su curso es crónico y de mal pronóstico, por lo que para pacientes de alto riesgo debe considerarse una cirugía radical desde el inicio.

INTRODUCTION: Parathyroid carcinoma is a difficult diagnosis, with early detection and timely treatment to prevent complications being imperative. CLINICAL CASE: A 42-year-old patient presenting with hypercalcemia of 16.1 mg / dl, PTH 1573 pg / mL and suggestive lesion of parathyroid adenoma is presented. Surgical biopsy identifies parathyroid carcinoma without invasion, subsequently performing right lobectomy with 0.1 mm focus of parathyroid carcinoma, with free borders. Oncological committee, strict follow-up is decided; However, at six months he requires hospitalization again for hypercalcemia, at cervical ultrasound he presents two hypoechogenic nodules smaller than 1 cm in the surgical bed. CT scan without evidence of injuries. With this background, cervical exploration is decided, finding a 2 cm, multilobed, paraesophageal tumor. Biopsy shows parathyroid carcinoma with invasion of fatty tissue and striated muscle. Palliative radiotherapy and chemotherapy are ruled out due to limited evidence, remaining in palliative care. DISCUSSION: Parathyroid cancer is a difficult diagnosis disease. In many cases the hypercalcemic crisis has been described as an initial presentation. Block resection of the parathyroid lesion with ipsilateral hemitiroidectomy is the standard treatment. It is a radioresistant tumor and adjuvant chemotherapy has not shown an increase in survival. In patients with inoperable disease, the prognosis is poor, with the control of calcemia and PTH being essential, which are the main cause of morbidity and mortality. CONCLUSIONS: Parathyroid carcinoma is a rare disease, the diagnosis and treatment of which represent a real clinical challenge, the high index of suspicion being crucial. Its course is chronic and has a poor prognosis, so for high-risk patients, radical surgery should be considered from the beginning.

Carcinoma de paratiroides: diagnóstico por fractura de fémur / Parathyroid carcinoma: diagnosis by a femur fracture

Resumen: Antecedentes: El carcinoma de las glándulas paratiroides (CP) descrito por De Quervain en 1909 representa la neoplasia menos frecuente, siendo su incidencia de 1.25/10,000,000 personas. Se han reportado aproximadamente 1,000 casos de carcinoma paratiroideo en la literatura mundial. En México existen dos series, una de ocho pacientes y otra de cuatro, además de tres reportes de casos aislados. Dado que el CP es funcionalmente activo el comportamiento clínico inicial es similar a las neoplasias paratiroideas benignas. Caso clínico: Femenino de 66 años de edad con dolor óseo en muslo y fractura espontánea de fémur, en la que se detectaron lesiones osteolíticas, hipercalcemia, niveles elevados de fosfatasa alcalina y de paratohormona; con gammagrama que mostró un tumor funcionante localizado en mediastino superior, fue sometida a extirpación en bloque con hemitiroidectomía derecha con tumor de la glándula paratiroides. El estudio histopatológico reportó CP. Discusión: El CP representa la neoplasia menos común; en pacientes con niveles de paratohormona mayores de 1,000 pg/ml e hipercalcemia mayor de 14 mg/dl debe sospecharse dicha patología.

Abstract: Background: Carcinoma of the parathyroid gland (PC) described by De Quervain since 1909, it represents the least common neoplasm, with an incidence of 1,25/10,000,000 peoples. It has been reported approximately 1,000 cases of CP in world literature. There are two series in Mexico, one of eight patients and other with four cases. Because CP is functionally active, its early clinical behavior is similar to that of parathyroid benign neoplasms. Clinical case: A 66-year-old female with history of thighbone pain and spontaneous femoral fracture, osteolytic lesions, hypercalcemia, elevated levels of alkaline phosphatase and parathyroid hormone detected; the scintigraphy showed a functioning tumor located in upper mediastinum. By hemithyroidectomy in block, the tumor was resected. Histopathological study reported parathyroid carcinoma. Discussion: PC is the least common neoplasia, in patients with parathyroid hormone levels greater than 1,000 pg/ml and hypercalcemia upper of 14 mg/dl this disease should be suspected.

Lesiones óseas multicéntricas por trastorno paratiroideo: Reporte de un caso clínico / Multicentric Bone Lesions Due to Parathyroid Disease: A Case Report

Resumen Introducción: El hiperparatiroidismo (Hpt) es un desorden endócrino que crea condiciones de hipersecreción de la hormona paratiroidea, propicias para el asentamiento de tumores en el tejido óseo. Si bien no es frecuente, los huesos maxilares, pueden estar involucrados en la patogenia de estas lesiones. Pacientes y métodos: En este trabajo, se presenta un caso clínico de una paciente femenina de 56 años de edad, con lesiones óseas multicéntricas maxilares y extramaxilares a predominio de células gigantes, vinculado a un hiperparatiroidismo. Discusión: El diagnóstico de un Tumor Pardo del Hiperparatiroidismo (TPH) es difícil en la clínica estomatológica, donde la metodología de diagnóstico debe reunir criterios serológicos, clínicos e histopatológicos. La multifocalidad de las lesiones, hacen a este caso un verdadero síndrome endócrino, siendo éste el aspecto más importante. Esta entidad, de incumbencia en la Endocrinología y en la Medicina Bucal, debe ser tratada fundamentalmente de forma interdisciplinar para lograr un tratamiento exitoso.

Abstract Introduction: Hyperparathyroidism is an endocrine disorder that could create parathyroid hormone hypersecretion conditions, propitious for bone tumors development. Although is not frequent, maxillary bones may be involved in the pathogenesis of these lesions. PATIENTS AND METHODS: In this case, a 56-year-old female is reported. An Hyperparathyroidism was diagnosed due to multiple maxillary and extra-maxillary multicentric bone lesions, with a predominance of giant cells, linked to her endocrine disorder. Discussion: The diagnosis of a Hyperparathyroidism Brown Tumor must meet serological, clinical and histopathological criteria. The multifocality of the lesions is the most remarkable clinical aspect in this case. For an adequate treatment of this entity is necessary an interdisciplinary and coordinate work between different areas of healthcare physicians and Oral Medicine specialist.

Hiperparatiroidismo primario en adolescente. Reporte de caso / Primary hyperparathyroidism in adolescent. Case report

INTRODUCTION: The hypercalcemia is infrequent in pediatrics, its clinical is diverse, and its etiology is determined by age. Among the dependent causes of parathormone (PHT) is the hyperthyroidism, state of hypersecretion of PHT by parathyroid glands (PG). The primary hyperparathyroidism (PHPT) is rare in children, there are 200 reported cases. In older children, the causes for PHPT correspond to parathyroid adenoma, multiglandular disease and parathyroid carcinoma. OBJECTIVE: Report a case of an 11 years old male adolescent. He presents three months symptoms of constipation, anorexia, vomiting and weight loss. Urgent consultation due to an increase of his symptoms, in exams stand out: calcemia 16.67 mg/dl (NV 8.8-10.8 mg/dl), phosphatemia 2.21 mg/dl (NV 4.5-5.5 mg/dl), parathormone (PHT) 308.7 pg/ml (NV 15-68.3 pg/ml), calciuria/creatininuria 0.56 (NV < 0.2). He was hospitalized to manage his severe hypercalcemia, it was indicated hyperhydration, monopotassium phosphate, intravenous hydrocortisone and furosemide. In his study was performed a cervical ultrasound which showed a solid node in the right parathyroid gland, hypoechogenic and scintigram parathyroid compatible with right superior parathyroid adenoma. In the waiting for surgery was necessary the administration of intravenous pamidronate. In the post-operatory, he evolved with hipocalcemia that was corrected with intravenous calcium carbonate, overlapping to oral calcium and calcitriol. CONCLUSION: The PHPT is a pathology of low prevalence in pediatrics, one hundred times less than adults. In the adolescent, the most frequent cause is the parathyroid adenoma. It should be considered as a differential diagnosis in cases of symptomatic hypercalcemia in this age group

Crisis hipercalcémica causada por adenoma paratiroideo: reporte de un caso clínico / Hypercalcemic crisis due to parathyroid adenoma: report of a clinical case

La hipercalcemia es un trastorno común que representa aproximadamente el 0,6% de todas las admisiones médicas agudas. El hiperparatiroidismo primario (HPTP) y las neoplasias malignas son las dos causas más comunes de elevación de los niveles séricos de calcio; constituyen, en conjunto, alrededor del 90% de todos los casos. La presentación sintomática clásica de la hipercalcemia se observa con relativa poca frecuencia en el mundo desarrollado; la presentación más común es la detección asintomática en las pruebas bioquímicas. Sin embargo, en casos raros, el HPTP puede desarrollar hipercalcemia aguda, grave y sintomática, llamada crisis hipercalcémica (CH). Esta condición se asocia a alteraciones profundas en el estado mental y las funciones cardíaca, renal y gastrointestinal en presencia de concentraciones marcadamente elevadas de calcio sérico y paratohormona (PTH). Mientras que algunas elevaciones en el calcio sérico pueden ser bien toleradas, los síntomas de la CH son severos. Si el tratamiento se retrasa, la CH puede provocar la muerte. Describimos el caso de un paciente masculino que ingresa en la unidad de cuidados críticos por una CH secundaria a un HPTP por adenoma paratiroideo. (AU)

Hypercalcaemia is a most common disorder, accounting for approximately 0,6% of all acute medical admissions. Primary hyperparathyroidism (PHPT) and malignancy are the two most common causes of increased serum calcium levels, together accounting for about 90% of all cases. The classical symptomatic presentation of hypercalcaemia is seen relatively rarely in the developed world, the most common presentation being asymptomatic and detected following on biochemical testing. However, in rare cases HPTP can result in acute, severe and symptomatic hypercalcemia, called hypercalcemic crisis (HC). This condition is associated with profound disturbances in mental status, and cardiac, renal, and gastrointestinal function in the presence of markedly increased serum calcium and parathyroid hormone (PTH) concentrations. While some elevations in serum calcium can be well tolerated, symptoms of HC are severe. If treatment is delayed, HC can result in death. We describe herein a case of a male patient who was admitted to the intensive care unit as a consequence of HC resulting from elevated PTH, secondary to a parathyroid adenoma. We describe the case of a male patient who was admitted to the critical care unit for a HC mediated by PTH secondary to a parathyroid adenoma. (AU)

Paratirotoxicosis y tumor cervical palpable: caso clínico de cáncer de paratiroides / Parathyrotoxicosis and palpable neck mass: a clinical presentation of parathyroid cancer

Introducción: El cáncer de paratiroides es poco frecuente. Suele presentarse como hiperparatiroidismo primario, en ocasiones como crisis hipercalcémica, con malestar general, náuseas, vómitos, alteraciones del ánimo y pérdida de peso. En algunos casos se presenta como osteítis fibrosa quística, osteopenia difusa, osteoporosis y fracturas patológicas. El diagnóstico suele estar dado por biopsia quirúrgica que muestra invasión a estructuras adyacentes, o metástasis locales y distantes. El tratamiento es la resección quirúrgica del tumor, sin uso de terapias adyuvantes. Su tasa de recurrencia es alta, de 25 a 100%. Algunos factores de mal pronóstico son metástasis a linfonodos en la presentación inicial, metástasis distantes y carcinomas no funcionantes. Caso clínico: Paciente masculino de 64 años que consultó por compromiso del estado general, bradipsiquia, poliuria, cefalea y masa cervical. Además presentaba hipercalcemia y gran elevación de PTH. Se realizó resección quirúrgica de la masa cervical, con biopsia rápida con atipias y mitosis 1/10, y de un nódulo hiperplásico tiroideo. Tras esto, sus niveles de PTH disminuyeron, así como también descendieron sus niveles de calcio. Discusión: Los pacientes que presentan cáncer de paratiroides suelen tener valores más elevados de calcemia y PTH. Para su diagnóstico, no se recomienda usar biopsia por punción, por riesgo de diseminación y por el bajo rendimiento de esta técnica. Conclusión: Pese a ser poco frecuente, se debe pensar en cáncer de paratiroides en el contexto de un paciente con hipercalcemia, especialmente si presenta PTH muy elevada. La sospecha clínica de este diagnóstico tiene directa relación con la posibilidad de tratamiento y pronóstico de la enfermedad.

Introduction: Parathyroid cancer is rare. Usually presented as primary hyperparathyroidism, sometimes as hypercalcemic crisis, with general malaise, nausea, vomiting, mood disturbances and low weight. In some cases it occurs as osteitis fibrosa cystica, diffuse osteopenia, osteoporosis and pathological fractures. The diagnosis is usually made by surgical biopsy shows invasion of adjacent structures, or local and distant metastases. The treatment is surgical resection of the tumor, without the use of adjuvant therapies. Their recurrence rate is high, 25 to 100%. Poor prognostic factors are lymph node metastases at initial presentation, distant metastases and nonfunctional carcinomas. Case report: Male patient consulted for 64 years in general conditions, bradypsychia, polyuria, headache and neck mass. He also had hypercalcemia and high elevation of PTH. Surgical resection of the cervical mass was performed, with rapid biopsy atypia and mitosis 1/10, and hyperplastic thyroid nodule. After this, PTH decreased levels as well as levels of calcium. Discussion: Patients with parathyroid cancer tend to have higher serum calcium and PTH of values. For diagnosis, it is not recommended to use needle biopsy, risk of spread and the poor performance of this technique. Conclusion: Despite being rare, you should think parathyroid cancer in the context of a patient with hypercalcemia, especially if you have very high PTH. The clinical suspicion of this diagnosis is directly related to the possibility of treatment and prognosis of the disease.

Association between atypical parathyroid adenoma and neurofibromatosis

Primary hyperparathyroidism is a disease characterized by excessive production of parathyroid hormone (PTH), which is due to a parathyroid adenoma in 85% of cases. An atypical parathyroid adenoma, with some histopathological features of parathyroid carcinoma, may be found in some of the cases, although it may not fulfill all the criteria for this diagnosis. Neurofibromatosis type 1 (NF1) is an autosomal dominant systemic disease that may be associated with hyperparathyroidism. We report here the rare combination of a patient with NF1 and clinical manifestations of hyperparathyroidism due to an atypical parathyroid adenoma.

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.

Importance of careful Tc-MIBI interpretation in patients with thyroid cancer and primary hyperparathyroidism

No abstract available.

Tumor pardo multifocal como manifestación del hiperparatiroidismo primario por adenoma paratiroideo asociado a carcinoma papilar de tiroides / Brown tumor of the pelvis associated with hyperparathyroidism

Background: Brown tumors of bones are an uncommon manifestation of hyperparathyroidism. Case report: We report a 35 years old male presenting with pain and paresis of the left superior limb. Part of his humerus was excised due to a diagnosis of a giant cell tumor. He was admitted again to the hospital due to pelvic pain, malaise and constipation. A right cervical nodule was found. Laboratory evaluation confirmed the presence of a hyperparathyroidism. The biopsy of the pelvic lesion disclosed a brown tumor. The patient was subjected to a parathyroidectomy and the pathological study of the surgical piece showed a right parathyroid adenoma and a right thyroid papillary micro carcinoma. In the postoperative period the patient had a hungry bone syndrome, which was adequately treated.

Introducción: La paratohormona es una hormona encargada de la homeostasis del calcio, el hiperparatiroidismo es una patología con manifestaciones renales y óseas, el Tumor Pardo es una rara presentación de esta enfermedad. Caso clínico: Hombre de 35 años con dolor y paresia en extremidad superior izquierda, fue resecado parte del húmero por un diagnóstico de Tumor de Células Gigantes; reingresa con dolor pélvico derecho, malestar general, astenia y estreñimiento. Se descubre un nódulo cervical derecho e hipersensibilidad en la pelvis derecha. Los exámenes de laboratorio muestran hiperparatiroidismo; la biopsia de la lesión pélvica es diagnóstica de Tumor Pardo, encontrándose además una hipercaptación paratiroidea derecha. Operado, el diagnóstico histopatológico fue: Adenoma paratiroideo derecho y un micro carcinoma papilar tiroideo; en el post-operatorio desarrolló un Síndrome de Bone Hunger, el cual fue superado y dado de alta. Discusión y conclusiones: El Tumor Pardo no es una verdadera neoplasia; producido por intensa actividad osteoclástica, tiene características histológicas y radiológicas inespecíficas y su diagnóstico se realiza por datos clínicos y bioquímicos. El hiperparatiroidismo puede llevar a la formación de Tumores Pardos; se sugiere realizar estudios de la glándula tiroides en pacientes con hiperparatiroidismo.

A rare case of a parathyroid adenoma inside a parathyroid cyst / Um caso raro de adenoma de paratireoide em um cisto de paratireoide

Parathyroid cysts (PCs) are rare lesions, located in the neck and anterior mediastinal region. The vast majority are non-functioning, presented as nodular cervical lesions. Large, non-functioning PCs can manifest with compressive symptoms of the surrounding tissues. Rarely, PCs produce excessive amounts of parathyroid hormone (PTH), resulting in primary hyperparathyroidism. We report a case of functional PC, describing its diagnostic and therapeutic approach.

Os cistos de paratireoide (PCs) são lesões raras, localizadas no pescoço e na região do mediastino anterior. A grande maioria é não funcionante, apresentando-se como lesões cervicais nodulares. PCs não funcionantes de grandes dimensões podem se manifestar com sintomas compressivos. Raramente, PCs produzem quantidades excessivas de hormônio da paratireoide (PTH), o que resulta em hiperparatiroidismo primário. Relatamos um caso de PC funcional, descrevendo sua abordagem diagnóstica e terapêutica.

Normocalcemic primary hyperparathyroidism: long-term follow-up associated with multiple adenomas / Hiperparatireoidismo primário normocalcêmico associado a múltiplos adenomas

Normocalcemic primary hyperparathyroidism (NPHPT) is a condition characterized by elevation of the parathyroid hormone (PTH) in the presence of normal serum calcium and the absence of secondary causes. The case described illustrates the long-term follow-up of a postmenopausal woman with NPHPT patient who progressed with multiple adenomas. This case reports a 77-year-old female who has chronic generalized pain and osteoporosis. Her initial serum PTH was 105 pg/mL, with total serum calcium of 9.6 mg/dL, albumin 4.79 g/dL, phosphorus 2.8 mg/dL, and 25OHD after supplementation was 34.6 ng/mL. The bone densitometry (BMD) results were as follows: lumbar spine: T-score -3.0, femoral neck: T-score -2.6 and distal radius: -4.2. Other causes of secondary hyperparathyroidism were ruled out and cervical ultrasound and Tc-99-Sestamibi scan were negative. She used oral alendronate and three infusions of zoledronic acid for treatment of osteoporosis. In the 10th year of follow-up, after successive negative cervical imaging, ultrasound showed a nodule suggestive of an enlarged right inferior parathyroid gland. PTH levels in fluid which was obtained during fine-needle aspiration (FNA) were over 5,000 pg/mL and a Sestamibi scan was negative. The patient underwent parathyroidectomy, and a histological examination confirmed parathyroid adenoma. Post-operatively serum PTH remained elevated in the presence of normal serum calcium levels. A follow-up cervical ultrasound showed a new solid nodule suggestive of an enlarged right superior parathyroid gland. PTH levels in the aspiration fluid were remarkably high. A second parathyroidectomy was performed, with the excision of a histologically confirmed parathyroid adenoma. In conclusion, this is an unusual presentation of NPHPT and highlights the long-term complications.

Hiperparatiroidismo primário normocalcêmico (NPHPT) caracteriza-se pela elevação do hormônio da paratiroide (PTH), na ausência da elevação dos níveis séricos de cálcio e exclusão de causas secundárias. O caso descrito ilustra o seguimento de uma mulher na pós-menopausa com NPHPT que evoluiu com múltiplos adenomas. Este caso relata uma paciente de 77 anos de idade que tem dor generalizada crônica e osteoporose. O PTH inicial foi elevado com níveis séricos de cálcio, albumina, fósforo e 25OH vitamina D normais. A densitometria óssea (DMO) evidenciou um T-SCORE da coluna lombar: -3.0, colo do fêmur: -2.6 e rádio distal: -4.2. Outras causas de hiperparatireoidismo secundário foram descartadas e a ultrassonografia cervical e varredura com Sestamibi foram negativos. Fez uso de alendronato e três infusões de ácido zoledrônico para o tratamento da osteoporose. No décimo ano de seguimento, depois de sucessivas imagens negativas, a ultrassonografia evidenciou um nódulo sugestivo de adenoma de paratireoide inferior direita. A paciente foi submetida à paratireoidectomia, e um exame histológico confirmou a hipótese. A elevação dos níveis séricos de PTH no pós-operatório se manteve com níveis normais de cálcio. A nova ultrassonografia cervical evidenciou outro nódulo sugestivo de adenoma de paratireoide superior direita. Uma segunda paratireoidectomia foi realizada, cujo histológico confirmou outro adenoma de paratireoide. Conclui-se que essa é uma apresentação incomum de NPHPT e destaca as complicações a longo prazo.

Nonadenomatous nonencapsulated thymic parathyroid tissue concomitant with primary hyperparathyroidism due to ectopic parathyroid adenoma / Tecido tímico paratireoidiano não edematoso e não encapsulado concomitante com hiperparatireoidismo devido a adenoma paratireoidiano ectópico

Primary hyperparathyroidism due to ectopic parathyroid adenoma is not infrequent. Primary hyperparathyroidism caused by unusual thymic nonadenomatous nonencapsulated parathyroid tissue has been reported before. Both can cause unsuccessful neck explorations. Here we presented for the first time a patient with hyperparathyroidism due to ectopic parathyroid adenoma concomitant to the presence of thymic nonadenomatous nonencapsulated parathyroid tissue.

O hiperparatireodismo primário devido a adenoma ectópico paratireoidiano não é raro. O hiperparatireodismo primário causado por tecido tímico paratireoidiano não edematoso e não encapsulado incomum já foi relatado anteriormente. Ambos podem levar à exploração cervical malsucedida. Apresentamos aqui, pela primeira vez, uma paciente com hiperparatireoidismo decorrente de um adenoma paratireoidiano concomitante com a presença de tecido tímico paratireoidiano não edematoso e não encapsulado.

Total parathyroidectomy in a large cohort of cases with hyperparathyroidism associated with multiple endocrine neoplasia type 1: experience from a single academic center

Most cases of sporadic primary hyperparathyroidism present disturbances in a single parathyroid gland and the surgery of choice is adenomectomy. Conversely, hyperparathyroidism associated with multiple endocrine neoplasia type 1 (hyperparathyroidism/multiple endocrine neoplasia type 1) is an asynchronic, asymmetrical multiglandular disease and it is surgically approached by either subtotal parathyroidectomy or total parathyroidectomy followed by parathyroid auto-implant to the forearm. In skilful hands, the efficacy of both approaches is similar and both should be complemented by prophylactic thymectomy. In a single academic center, 83 cases of hyperparathyroidism/ multiple endocrine neoplasia type 1 were operated on from 1987 to 2010 and our first surgical choice was total parathyroidectomy followed by parathyroid auto-implant to the non-dominant forearm and, since 1997, associated transcervical thymectomy to prevent thymic carcinoid. Overall, 40% of patients were given calcium replacement (mean intake 1.6 g/day) during the first months after surgery, and this fell to 28% in patients with longer follow-up. These findings indicate that several months may be needed in order to achieve a proper secretion by the parathyroid auto-implant. Hyperparathyroidism recurrence was observed in up to 15% of cases several years after the initial surgery. Thus, long-term follow-up is recommended for such cases. We conclude that, despite a tendency to subtotal parathyroidectomy worldwide, total parathyroidectomy followed by parathyroid auto-implant is a valid surgical option to treat hyperparathyroidism/multiple endocrine neoplasia type 1. Larger comparative systematic studies are needed to define the best surgical approach to hyperparathyroidism/multiple endocrine neoplasia type 1.