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.

Hepatic metastasis of a parathyroid carcinoma treated with intra-arterial embolization / Metástasis hepática de un carcinoma de paratiroides tratada con embolización intraarterial

Abstract This paper reports the case of a patient with parathyroid carcinoma and metastatic parathyroid carcinoma in the liver, as well as persistent hypercalcemia resistant to the management with zoledronic acid and cinacalcet. The patient was treated with intra-arterial embolization using polyvinyl alcohol microspheres in the right hepatic artery, achieving an excellent biochemical control after the intervention.

Resumen Se reporta el caso de una paciente con carcinoma de paratiroides metastásico al hígado con hipercalcemia de muy difícil control pese a manejo con ácido Zoledrónico y Cinacalcet. La paciente fue llevada a embolización intraarterial con microesferas de alcohol polivinílico de la arteria hepática derecha, con lo que se obtuvo un excelente control bioquímico después de la intervención.

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.

Detección inmunohistoquímica de parafibromina en patología de paratiroides / Immunohistochemical detection of parafibromin in parathyroid pathology

Introduction: The definitive diagnosis of parathyroid cancer is extremely difficult, from the clinical approach to the molecular diagnosis. A gene mutation was detected recently in patients with parathyroid cancer. It is a suppressor tumor gene called HRPT2, which codifies for a protein that participates in PAF1 complex, the parafibromin. It has been observed that the expression of this protein it's altered in parathyroid cancer, what would serve like method of diagnosis by immunohystochemistry, with a sensitivity and specificity of 73-96 percent and 99-100 percent respectively. Material and Method: The anti-parafibromin immunohysto-chemistry staining was made in 23 parathyroids tissue samples (5 adenomas, 6 hyperplasia, 7 normal and 5 carcinomas). Results: A positive pattern is observed in almost 100 percent of benign pathology and 100 percent in normal tissue. In the cases of carcinoma only 2 of 5 had a strong positivity. Conclusions: The pathological clinical correlation does not allow the association of the loss of parafibromin immunoreactivity in some unequivocal cases of parathyroid cancer. The parafibromin immunostaining does not allow to discriminate between benign or malign pathologies.

Introducción: El diagnóstico definitivo de cáncer de paratiroides es extremadamente difícil, desde el acercamiento clínico hasta el diagnóstico molecular. Se detectó recientemente en pacientes con cáncer de paratirodes un gen supresor de tumor mutado (HRPT2), que codifica para una proteína que participa en el complejo PAF1, la parafibromina. Se ha observado que la expresión de esta proteína está alterada en los casos de cáncer de paratiroides, lo que serviría como método de diagnóstico por inmunohistoquímica, con una sensibilidad y especificidad de 73-96 por ciento y 99-100 por ciento, respectivamente. Material y Método: Se realizó tinción inmunohistoquímica anti parafibromina en 23 muestras de tejido paratiroideo (5 adenomas, 6 hiper-plasias, 7 normales y 5 carcinomas). Resultados: Se observa un patrón positivo fuerte en casi 100 por ciento de la patología benigna y 100 por ciento en tejido normal. En los casos de carcinoma sólo 2 de 5 tenían positividad fuerte. Conclusiones: La correlación clínico patológica no permite asociar la pérdida de tinción de parafibromina en algunos casos de cáncer inequívocos. La tinción de parafibromina no permite discriminar entre patología benigna y maligna.

A Case of Parathyroid Carcinoma Presenting Hyperparathyroidism that was Clinically Easy to Overlook

Parathyroid carcinoma is very rare in patients with primary hyperparathyroidism, only accounts for 1% to 2% of all cases. It is characterized by higher incidence of renal dysfunction, metabolic bone disease and gastrointestinal symptoms. Although the clinical manifestations of hyperparathyroidism are more severe in patients with parathyroid carcinoma when compared to those with those with parathyroid adenoma, it is difficult to differentiate carcinoma from adenoma without pathologic confirmation. To our knowledge, there have only been a few cases of hyperparathyroidism due to parathyroid carcinomas reported to date. Here, we report a case of 63-year-old woman developing osteoporosis, hypercalcemia and hypophosphatemia due to a parathyroid tumor and was diagnosed with parathyroid carcinoma postoperatively.

A Case of Parathyroid Carcinoma Presenting Hyperparathyroidism that was Clinically Easy to Overlook

Parathyroid carcinoma is very rare in patients with primary hyperparathyroidism, only accounts for 1% to 2% of all cases. It is characterized by higher incidence of renal dysfunction, metabolic bone disease and gastrointestinal symptoms. Although the clinical manifestations of hyperparathyroidism are more severe in patients with parathyroid carcinoma when compared to those with those with parathyroid adenoma, it is difficult to differentiate carcinoma from adenoma without pathologic confirmation. To our knowledge, there have only been a few cases of hyperparathyroidism due to parathyroid carcinomas reported to date. Here, we report a case of 63-year-old woman developing osteoporosis, hypercalcemia and hypophosphatemia due to a parathyroid tumor and was diagnosed with parathyroid carcinoma postoperatively.

Parathyroid Carcinoma Presenting as a Hyperparathyroid Crisis

No abstract available.

Hiperparatiroidismo primario y cáncer de paratiroides: caso clínico / Primary hyperparathyroidism and parathyroid cancer: report of one case

We report a 51 years old female operated for a primary hyperparathyroidism that relapsed in two occasions. After the first and second operation, the pathological study of the excised glands disclosed an adenoma. After the second relapse, a parathyroid gland in the right lateral and upper cervical region, located with technetium (Tc)-99m Sestamibi scintigraphy, was excised. The pathological study of the surgical piece disclosed a parathyroid carcinoma without local lymph node involvement. A postoperative 18F-fluorodexyglucose positron emission tomography did not show tumor dissemination.

Presentamos un caso de sexo femenino, operada por hiperparatiroidismo primario, con 2 recidivas posteriores. En las 2 primeras oportunidades, el diagnóstico histológico fue compatible con adenoma. La última intervención se debió realizar por una glándula ubicada, de acuerdo al Sestamibi Tc-99m, en la región cervical lateral alta derecha. Se realizó exploración supraomohiodea, efectuando la extirpación de la glándula y adenopatías peritumorales. La biopsia definitiva informó carcinoma de paratiroides y ganglios linfáticos negativos para neoplasia. Se realizó estudio de diseminación y PET- FDG de control en el postoperatorio alejado, sin nuevos hallazgos. Entre la primera y la segunda intervención, se realizó una tiroidectomía total por un bocio uninodular, cuya biopsia informó 3 paratiroides normotípicas, intratiroideas. En suma, se lograron identificar histológicamente 6 glándulas paratiroides.

A Case of Multiple Primary Parathyroid Cancer / 대한이비인후과학회지

Parathyroid cancer is a rare disease, of which about 700 cases have been reported since first being described by de Quevain in 1904. In particular, multiple primary parathyroid cancer is extremely rare and to our knowledge only 4 cases have been reported in the literature since the first report in 1990. A 41-year-old male patient was presented with bilateral neck mass, polydypsia and polyuria with hypercalcemia and high parathyroid hormone level. The patient underwent surgery at Kosin University Gospel Hospital and was diagnosed as double parathyroid cancer on pathologic examination. So, we report this rare case with literature.

A Case of Nonfunctioning Parathyroid Carcinoma / 대한이비인후과학회지

Parathyroid carcinoma is an uncommon endocrine tumor. Its usual clinical presentation is that of primary hyperparathyroidism with elevated serum calcium and parathyroid hormone levels. Nonfunctioning carcinoma of the parathyroid gland with normal serum calcium levels is very rare and more aggressive. Histologically, the neoplastic cells may be of variable size and shape but are usually uniform and not too dissimilar from normal parathyroid cells. There is a general agreement that a diagnosis of carcinoma based on cytologic detail is unreliable, and local invasion and metastasis constitute the only reliable criteria of malignancy. In both functional and nonfunctional carcinoma, early surgery has proven to be the only curative treatment approach whereas both chemotherapy and radiation therapy fail to produce systemic or regional benefit when used alone. En bloc excision of primary lesion with removal of any involved structures should be performed. This paper is a case report of a nonfunctioning parathyroid carcinoma and a review of the literature.

Hyperfunctioning Parathyroid Carcinoma / 대한내분비외과학회지

PURPOSE: Most cases of primary hyperparathyroidism are due to either a parathyroid adenoma or parathyroid hyperplasia. Parathyroid carcinoma is a very rare cause of hyperparathyroidism. Although the diagnosis of parathyroid carcinoma is usually established by pathologic criteria especially of vascular or capsular invasion, some clinical and biochemical features differentiate it from benign forms of hyperparathyroidism. We undertook a retrospective study in 4 patients with functioning parathyroid carcinoma, with the aim of conveying experience from management of this rare cause of kyperparathyroidism. METHODS: Clinical simptoms, biochemical laboratory, radiologic, and intraoperative findings, extent of surgical resection. histologic findings, local recurrence and distant metastasis were analysed in 4 patients diagnosed pathologically as a parathyroid carcinoma after operation from 1992 to 1998. RESULTS: Mean age was 46.7 years (33~51 years) and male to female ratio was 1:3. Neck mass was found in 3 patients, multiple bone pain in 3 patients and renal stone in 1 patient. One case has suffered from chronic renal failure for 19 years. Although preoperative laboratory evaluations showed the aspects of hyperparathyroidsm in all the cases, mean serum calcium level was 11.1 mg/dl (10.5~12.0 mg/ dl), slightly elevated. Laboratory values after surgery were within the normal range in 3 cases. However, in one case with chronic renal failure, serum PTH levels, serially checked, were above the normal range. Any of imaging methods failed to suggest a parathyroid carcinoma preoperatively. Parathyroid adenoma was suspected in 2 cases, thyroid cancer in the other cases before surgery. The extent of resection was radical resection of parathyroid lesion with more than node dissection and in 2 cases, the resection of recurrent laryngeal nerve or strap muscles was added. During followup period, any local or systemic recurrence were not evident in all the cases. CONCLUSION: Although functioning parathyroid carcinoma is a rare disease and its preoperative diagnosis, in general, cannot easily be made, the understanding of characteristic clinical and biochemical feature could help diagnosis at first surgery. Radical resection without remaining residual tumor is most important fo the management of the parathyroid cancer.

Hyperfunctioning Parathyroid Carcinoma / 대한내분비외과학회지

PURPOSE: Most cases of primary hyperparathyroidism are due to either a parathyroid adenoma or parathyroid hyperplasia. Parathyroid carcinoma is a very rare cause of hyperparathyroidism. Although the diagnosis of parathyroid carcinoma is usually established by pathologic criteria especially of vascular or capsular invasion, some clinical and biochemical features differentiate it from benign forms of hyperparathyroidism. We undertook a retrospective study in 4 patients with functioning parathyroid carcinoma, with the aim of conveying experience from management of this rare cause of kyperparathyroidism. METHODS: Clinical simptoms, biochemical laboratory, radiologic, and intraoperative findings, extent of surgical resection. histologic findings, local recurrence and distant metastasis were analysed in 4 patients diagnosed pathologically as a parathyroid carcinoma after operation from 1992 to 1998. RESULTS: Mean age was 46.7 years (33~51 years) and male to female ratio was 1:3. Neck mass was found in 3 patients, multiple bone pain in 3 patients and renal stone in 1 patient. One case has suffered from chronic renal failure for 19 years. Although preoperative laboratory evaluations showed the aspects of hyperparathyroidsm in all the cases, mean serum calcium level was 11.1 mg/dl (10.5~12.0 mg/ dl), slightly elevated. Laboratory values after surgery were within the normal range in 3 cases. However, in one case with chronic renal failure, serum PTH levels, serially checked, were above the normal range. Any of imaging methods failed to suggest a parathyroid carcinoma preoperatively. Parathyroid adenoma was suspected in 2 cases, thyroid cancer in the other cases before surgery. The extent of resection was radical resection of parathyroid lesion with more than node dissection and in 2 cases, the resection of recurrent laryngeal nerve or strap muscles was added. During followup period, any local or systemic recurrence were not evident in all the cases. CONCLUSION: Although functioning parathyroid carcinoma is a rare disease and its preoperative diagnosis, in general, cannot easily be made, the understanding of characteristic clinical and biochemical feature could help diagnosis at first surgery. Radical resection without remaining residual tumor is most important fo the management of the parathyroid cancer.

A Case of Functioning Parathyroid Cancer with Hypokalemia / 대한내분비학회지

The parathyroid carcinoma is rare disorder and its reported incidence is 1-2% of primary hyperparathyroidism. Parathyroid cancer shows more profound clinical symptoms and signs than adenoma or hyperplasia: more severe renal and bony complications, higher calcium levels (>15 mg/dL) and palpable neck mass. It usually diagnosed after operation when it shows malignant pathologic findings, local invasion, distant metastasis, or even recurrence. The treatment is surgical resection. Hypokalemia is relatively common feature of primary hyperparathyroidism but it should be marked because of its arrhythmogenic effect when accompanies with hypercalcemia. We experienced a hyperfunctioning parathyroid carcinoma in 53 year old rnan confirmed postoperatively which showed hypokalemia normalized after operation.

A Case of Parathyroid Carcinoma with Systemic Calcification / 영남의대학술지

Hyperparathyroidism due to parathyroid cancer is rare. It is difficult to diagnose preoperatively but there should be an increased index of suspicion in those parathyroid Patient with palpable neck masses, profound hypercalcemia(greater than 14mg/dl), made increase of the parathyroid hormone level to greater than twice normal, and significant metabolic complications. In parathyroid cancer, systemic calcinosis is an extremely rare manifestation. The most common metastatic calcification site is lung and the other involved site is stomach, liver, skin and heart. After resection of parathyroid tumor, this systemic calcinosis is self-limiting. We experienced a patient with primary hyperparathyroidsm, presented with metastatic calcification in the lung and stomach disappeared by successful parathyroidectomy.