Carcinoma de paratiroides en un paciente con hiperparatiroidismo terciario / Parathyroid carcinoma in a patient with tertiary hyperparathyroidism

Introducción: La mayoría de los pacientes con enfermedad renal crónica terminal en hemodiálisis desarrollan hiperparatiroidismo secundario. Algunas veces este tipo de hiperparatiroidismo secundario suele ser severo y refractario al tratamiento médico, inclusive algunos desarrollarán hiperparatiroidismo terciario, el tratamiento de ambas situaciones suele ser la paratiroidectomía subtotal o total. Reporte de Caso: Presentamos el caso de un paciente de 29 años, con enfermedad renal terminal en hemodiálisis durante 13 años, quien desarrolló hiperparatiroidismo terciario por lo que fue sometido a paratiroidectomía subtotal con autotrasplante de media glándula; el estudio patológico informó la presencia de un carcinoma de paratiroides en una de las cuatro glándulas paratiroides extirpadas. Conclusión: El carcinoma de paratiroides es una neoplasia rara que causa hiperparatiroidismo primario en menos de 1% de todos los casos, y se ha reportado en algunos pacientes operados por hiperparatiroidismo terciario.

Background: Most patients with terminal chronic kidney disease on hemodialysis develop secondary hyperparathyroidism. Sometimes this type of secondary hyperparathyroidism becomes severe and refractory to medical treatment, some will even develop tertiary hyperparathyroidism, the treatment of both situations is usually subtotal or total parathyroidectomy. Case Report: We present the case of a 29-year-old patient with end-stage kidney disease on hemodialysis for 13 years, who developed tertiary hyperparathyroidism, for which he underwent subtotal parathyroidectomy with half gland autotransplantation; the pathological study reported the presence of a parathyroid carcinoma in one of the four excised parathyroid glands. Conclusion: Parathyroid carcinoma is a rare neoplasm that causes primary hyperparathyroidism in less than 1% of all cases, and has been reported in some patients operated on for the development of tertiary hyperparathyroidism.

Hiperparatiroidismo terciario como presentación de enfermedad ósea metabólica en trasplante renal: un reto diagnóstico-terapéutico. Presentación de un caso clínico / Tertiaryhyperparathyroidism as a manifestation of bone mineral metabolismdisease in kidneytransplantrecipient: a diagnostic and therapeuticchallenge. Presentation of a clinical case

RESUMEN El hiperparatiroidismo terciario se caracteriza por hipercalcemia e hiperparatiroidismo autónomo en el contexto de hiperparatiroidismo secundario persistente. El HPT3 se relaciona con calcificaciones extraesqueléticas o calcifilaxis, fracturas, dolor óseo, pérdida progresiva de la densidad mineral ósea, nefrocalcinosis, litiasis, disfunción o rechazo del aloinjerto, alteraciones neuropsiquiátricas, enfermedad cardiovascular y aumento de la morbimortalidad. La paratiroidectomía subtotal es el tratamiento de elección, con altas tasas de curación. Presentamos el caso de una paciente con hiperparatiroidismo terciario como enfermedad ósea metabólica después de un trasplante renal exitoso, con evolución insidiosa y daño óseo severo, con adecuada respuesta al tratamiento oportuno con paratiroidectomía subtotal. El diagnóstico y tratamiento oportuno del hiperparatiroidismo terciario en el paciente con trasplante renal es fundamental para disminuir la incidencia de comorbilidades, mejorar el pronóstico del paciente y optimizar recursos de salud.

ABSTRACT Tertiary hyperparathyroidism (THPT) is characterized by hypercalcemia and autonomous hyperparathyroidism in the context of persistent secondary hyperparathyroidism (SHPT). THPT is related with extraskeletal calcifications, calciphylaxis, fractures, bone pain, progressive loss of bone mineral density, nephrocalcinosis, lithiasis, kidney allograft dysfunction and rejection, neuropsychiatric alterations, cardiovascular disease, and high morbimortality. Subtotal parathyroidectomy is the gold standard for treatment, with high cure rates. We described a case of THPT as a manifestation of Bone Mineral Metabolism Disease after a successful kidney transplant, with an insidious evolution and severe bone damage, with an adequate response to subtotal parathyroidectomy. We evidenced that early diagnosis and treatment of THPT in kidney transplant recipients is essential to the diminution of comorbidities, the improvement of prognosis and the optimization of health resources.

Hiperplasia de las 4 glándulas paratiroides en hiperparatiroidismo terciario, una imagen infrecuente visualizada en la gammagrafía con Tc-99m MIBI

RESUMEN El hiperparatiroidismo terciario es una complicación presentada en pacientes con enfermedad renal crónica llevados a trasplante renal cuyo manejo quirúrgico requiere de la ubicación mediante imágenes diagnosticas para garantizar el éxito de esta intervención. Se presenta caso de mujer en hemodiálisis crónica que presentó hiperparatiroidismo terciario por hiperplasia paratiroidea confirmada con Tc-99m MIBI SPECT.

ABSTRACT Tertiary hyperparathyroidism is a complication presented in patients with chronic kidney disease subjected to renal transplantation whose surgical management requires location through diagnostic images to guarantee the success of this intervention. We present the case of a woman on chronic hemodialysis who presented tertiary hyperparathyroidism due to parathyroid hyperplasia confirmed by Tc-99m MIBI SPECT.

Clinical analysis of persistent hypercalcemia with osteoporosis caused by tertiary hyperparathyroidism after kidney transplantation / 中华器官移植杂志

Objective To explore the clinical characteristics of tertiary hyperparathyroidism (THPT) after renal transplantation .Methods The levels of bone mineral density (BMD) , serum calcium , phosphates , alkaline phosphatase (ALP) and intact parathyroid hormone (iPTH ) were retrospectively analyzed in 36 RTx recipients with persistent hypercalcemia and stable kidney function (eGFR 76 .71 ± 17 .44) ml/min/1 .73 m2 .Results Among them ,serum total calcium level increased (2 .97 ± 0 .20 ) mmol/L for 6 to 170 months ,blood phosphorus decreased (0 .59 ± 0 .19 ) mmol/L , serum alkaline phosphatase (ALP) increased to (295 .73 ± 194 .22)U/L and T-score of BMD decreased (T - 2 .78 ± 0 .84 in lumbar vertebrae and T - 2 .09 ± 0 .66 in hip joint) .And 11 /36 (30 .6％ ) cases had a complication of extraosseous calcification .Parathyroid hyperplasia was detected in 17 /36 cases (47 .2％ ) .iPTH was significantly higher at pre-operation and 1 week post-operation than that in control group (n= 45) (859 .50 ± 495 .44 vs 345 .56 ± 216 .55 pg/ml) , P = 0 .001 ,(759 .25 ± 907 .07 vs 197 .45 ± 249 .31 pg/ml) , P= 0 .001 .The value of iPTH at the last follow-up (198 .26 ± 155 .22) pg/ml was still higher than normal reference value (15 .0 - 65 .0 pg/ml) . Multivariate stepwise regression analysis showed the last iPTH was correlated with preoperative iPTH ,serum calcium and postoperative serum phosphor ,ALP and 25OHD3 (P= 0 .024 , P= 0 .002 , P = 0 .001 , P = 0 .037 , P = 0 .026 ) .Conclusions Renal recipients had a higher levels iPTH with persistent hypercalcemia , hypophosphatemia , osteoporosis and extraosseous calcification showing the features of tertiary hyperparathyroidism .

Treatment of renal hyperparathyroidism: Cinacalcet or surgery? / 中华内分泌外科杂志

Patients with end-stage renal disease have a 30％-50％ incidence of hyperparathyroidism.Renal hyperparathyroidism causes multiple systemic diseases,which affect the life quality of patients,and seriously endangers patients' life.Now there are two major treatments,Cinacalcet and surgery.This article is aimed to discuss the characteristics of the two methods and review the latest research on renal hyperparathyroidism.It is advised that Cinacalcet may apply to:1,patients with mild to moderate secondary hyperparathyroidism;2,patients who do not accept the surgery;3,patients with surgical contraindication.And surgical procedures are applicable to:1,patients with tertiary hyperparathyroidism after kidney transplantation;2,patients with invalid Cinacalcet treatment;3,patients who cannot tolerate the side-effect of Cinacalcet;4,patients unable to undertake economic burden of Cinacalcet.However,these conclusions still require higher levels of clinical trials to be validated.

Surgical and medical treatment with cinacalcet in tertiary hyperparathyroidism / 国际外科学杂志

Tertiary hyperparathyroidism is commonly observed in patients with long-standing chronic kidney disease (CKD) after renal transplantation and is characterized by the autonomous hypersecretion of PTH leading to hypercalcemia.The primary treatment is surgery.After the introduction of cinacalcet,both surgical and medical methods have been commonly performed to treat tertiary hyperparathyroidism.However,which treatment is more appropriate has not been fully demonstrated.In this paper,the author will systematically review the related research of surgical treatment and cinacalcet in the treatment of 3HPT,and summarize their respective therapeutic effects.

Calcinosis tumoral: una rara entidad en hiperparatiroidismo terciario

Objetivo: La calcinosis tumoral es un desorden infrecuente caracterizado por el desarrollo de masas calcificadas en los tejidos periarticulares de las articulaciones. Se presenta caso representativo. Caso Clínico: Se presenta el caso de una paciente femenina de 34 años con enfermedad renal crónica e hiperparatiroidismo terciario quien presentó un tumor de 40 x 20 cm aproximadamente en la cadera derecha y 10 x 10 cm en la cadera izquierda, ambos sólidos, pétreos, no móviles y poco dolorosos, de 1 año de evolución. Bioquímica sanguínea reveló hiperfosfatemia, hipercalcemia y PTH 1125 pg/mL. En radiografías se apreció calcificación prominente en ambas caderas principalmente la derecha. El tratamiento ofrecido fue paratiroidectomía con autoimplante. Conclusión: La calcinosis tumoral es un síndrome de calcificación ectópica infrecuente y puede ser una rara complicación del hiperparatiroidismo terciario y de la enfermedad renal crónica.

Objective: The tumoral calcinosis is an infrequent disorder characterized by the development of calcified masses within the peri-articular soft tissues of large joints. A representative case is presented. Case presentation: We present a case of a woman patient of 34 years old with chronic renal failure and tertiary hyperparathyroidism who presented a solid tumor of 40 x 20 cm approximately in the right hip and a tumor of 10 x 10 cm in the left hip, stony, nonmovable and little painful since 1 year ago. Blood biochemistry revealed hyperphosphatemia, hypercalcemia and PTH 1125 pg/mL. In x-rays prominent calcification in both hips was appraised mainly the right. The offered treatment was parathyroidectomy with autoimplant. Conclusion: Tumoral calcinosis is an uncommon ectopic calcification syndrome and may be a rare complication of tertiary hyperparathyroidism and chronic renal failure.

Analysis on six cases of tertiary hyperparathyroidism and literature review / 中华内分泌代谢杂志

The data of 6 patients with tertiary hyperparathyroidism were retrospectively analyzed.The first symptom in 3 patients was bone ache,1 with lower extremity weakness,1 with swelling of lower limbs,and 1 with increase serum alkaline phosphatase.5 patients had past history of renal failure,1 case suffered from severe intestinal malabsorption,and 4 of them had renal transplantation.X-ray showed subperiosteal bone resorption,bone demineralization,or cellulose osteitis.5 subjects presented with osteoporosis,and 1 of them had fractures.Left atrioventricular membrane calcification and gluteal calcification were found in each of 2 cases.4 patients suffered from hypercalcaemia,2 patients with normal serum calcium,4 with hypophosphataemia,and 6 with high levels of PTH.Pathological results showed adenoma in parathyroid gland in 4 patients and enlarged parathyroid glands in 2 patients.Serum calcium and PTH levels returned to normal after operation and calcium supplement.

Hiperparatiroidismo terciario: a propósito de tres pacientes con complicaciones diferentes / Tertiary hyperparathyroidism: representation of the complications in three different cases

El hiperparatiroidismo terciario ha sido reconocido como una hiperfunción autónoma de las glándulas paratiroides. Cuando esta enfermedad se instaura, la única opción terapéutica efectiva es la paratiroidectomía. Estos pacientes frecuentemente tienen hiperplasia paratiroidea por lo que la exploración cervical debe ser bilateral. Presentación de casos: Presentamos 3 pacientes, cada uno con una complicación diferente a propósito del hiperparatiroidismo terciario. La primera paciente con arteriolopatía cálcico-urémica, la segunda con calcinosis tumoral de la cadera, y la tercera paciente con tumor pardo bimaxilar. A todos los pacientes los tratamos con paratiroidectomía total con auto trasplante de la glándula más sana en el músculo recto anterior del abdomen. El estudio anatomopatológico reveló hiperplasia de las glándulas paratiroideas resecadas en todos los pacientes. La complicación principal de este procedimiento fue la hipocalcemia sintomática, requiriendo infusión de calcio endovenoso. Dos pacientes presentaron síndrome de hueso hambriento, que se resolvió progresivamente y en el seguimiento se observó normocalcemia. Hubo desenlace fatal en 2 pacientes como consecuencia de complicaciones sistémicas. El hiperparatiroidismo terciario es una enfermedad rara que el clínico debe reconocer para tratarla oportunamente. La paratiroidectomía total es terapéuticamente efectiva en esta condición, pero la hipocalcemia profunda posoperatoria es frecuente y necesita de un manejo cuidadoso.

Tertiary hyperparathyroidism has been recognized as an autonomous hyperfunction of the parathyroid glands. When this disease is established, the only effective therapeutic option is parathyroidectomy. These patients often have parathyroid hyperplasia so that the the neck exploration must be bilateral. Cases presentation: We report 3 patients, each with a different complication as a consequence of tertiary hyperparathyroidism. The first patient with calcic uremic arteriolopathy, the second with tumoral calcinosis of the hip, and the third patient with bimaxillary brown tumor. We treated all this patients with total parathyroidectomy, followed by healthy gland autotransplantation in the anterior rectus abdominal muscle. The anatomopathological study revealed hyperplasia of the resected parathyroid glands, in all the specimens. The main complication of this procedure were the symptomatic hypocalcemia, requiring intravenous calcium infusion. Two patients had the hungry bone syndrome, which was solved progressively. Two parients died due to systemic complications. Tertiary hyperparathyroidism is a rare disease that the clinician should recognize in order to treat it promptly. Total parathyroidectomy is therapeutically effective in this condition, but the postoperative profound hypocalcemia is frequent and needs a careful management.

Calcinosis tumoral: una rara entidad en hiperparatiroidismo terciario / Tumoral calcinosis: a rare entity in tertiary hyperparathyroidism

La calcinosis tumoral es un desorden infrecuente caracterizado por el desarrollo de masas calcificadas en los tejidos periarticulares de las articulaciones. Presentación del caso: se presenta un caso de una paciente femenina de 34 años con enfermedad renal crónica e hiperparatiroidismo terciario quien presentó un tumor de 40 x 20 cm aproximadamente en la cadera derecha y 10 x 10 cm cadera izquierda, ambos sólidos, pétreos, no móviles y poco doloroso de 1 año de evolución. La bioquímica sanguínea reveló hiperfosfatemia, hipercalcemia y PTH 1125 pg/ml. En las radiografías se apreció calcificación prominente en ambas caderas, principalmente la derecha. El tratamiento ofrecido fue paratiroidectomía con autoimplante. Conclusión: la calcinosis tumoral es un síndrome de calcificación ectópica infrecuente y puede ser una rara complicación del hiperparatiroidismo terciario y de la enfermedad renal crónica(AU)

The tumoral calcinosis is an infrequent disorder characterized by the development of calcified masses within the peri-articular soft tissues of large joints. Case presentation: we present a case of a woman patient of 34 years old with chronic renal failure and tertiary hyperparathyroidism, who presented a solid tumor from 40 x 20 cm approximately in the right hip and a tumor of 10 x 10 cm in left hip, stony, nonmovable and little painful from 1 year ago. Blood biochemistry revealed hyperphosphatemia, hypercalcemia and PTH 1125 pg/ml. In x-rays prominent calcification in both hips was appraised mainly the right. The offered treatment was parathyroidectomy with autoimplant. Conclusion: Tumor calcinosis is an uncommon ectopic calcification syndrome and it is can a rare complication of tertiary hyperparathyroidism and chronic renal failure(AU)

The clinical features of tertiary hyperparathyroidism in 7 cases / 中华内分泌代谢杂志

ObjectiveTo analyze the clinical features of tertiary hyperparathyroidism. MethodsThe data including symptoms, past history, bone and joint X-ray, bone mass density, bone SPECT, blood routine, serum calcium, phosphorus, alkaline phosphatase, parathyroid hormone ( PTH), as well as parathyroid ultrasonic or CT,parathyroid ECT, and parathyroid pathology were collected from seven patients with tertiary hyperparathyroidism.ResultsThe first symptom in 6 patients was bone ache, one of them was thirsty, and 1 patient had fracture. Four patients had past history of gallbladder stone or renal stone. Four subjects presented with osteoporosis and 2 of them had fractures. All 7 patients suffered from hypercalcaemia, along with high PTH level, 6 with hypophosphataemia and 6 with high alkaline phosphatase level. The enlarged parathyroid glands were detected in five patients by ultrasonic or CT. Tc99 scans of parathyroid gland revealed hyperfunctioning nodes in 3 subjects. Six patients were operated, and pathological results showed adenoma in parathyroid gland in 5 patients and adenocarcinoma in 1 patient. Serum calcium and PTH levels returned to normal in all the operated patients.ConclusionsPatients who present bone ache, osteoporosis, spontaneous fracture, past history of gallbladder stone or renal stone, high serum calcium, and PTH levels, should be aware of the probability of tertiary hyperparathyroidism. The confirmed diagnosis depends on pathological result.

Surgical Treatment of Tertiary Hyperparathyroidism after Renal Transplantation / 대한이식학회지

PURPOSE: Tertiary hyperparathyroidism (tHPT) means a persistent secondary hyperparathyroidism even after successful renal transplantation. Parathyroidectomy (PTX) is an efficient way to treat tHPT. In this study, we reviewed our single center Experience of PTX in regard to postoperative outcomes and analyzed any differences by the types of surgery. METHODS: Among 2,589 recipients who underwent renal allograft between April. 1979 and Dec. 2006, 11 patients (0.4%) were identified to have tHPT and underwent PTX. Levels of intact parathyroid hormone (iPTH) and serum calcium were measured before and after PTX to evaluate therapeutic effect, and serum-creatinine and GFR using the Modification of Diet in Renal Disease (MDRD) equation to investigate any effect to graft function. RESULTS: One patient showed persistent hyperparathyroidism and hypercalcemia after subtotal PTX. We experienced 10 successful PTXs in which 2 total PTX with autotransplantations, 4 subtotal PTXs and 4 limited PTXs. Level of iPTH and serum calcium were at normal range after PTX. Serum creatinine increased and estimated GFR decreased after PTX. Total PTX with autotransplantation showed a tendency of more decrease in the values of iPTH, and GFR after PTX than Subtotal PTX. CONCLUSION: PTX can cure tHPT-specific symptoms and sign by the recovery of hypercalcemia but may carry the risk of deterioration of kidney graft function. Subtotal PTX rather than total PTX might be recommended in the surgical treatment of tHPT to prevent any risk of kidney graft deterioration.

Comparison of Operative Methods for Tertiary Hyperparathyroidism

PURPOSE: Tertiary hyperparathyroidism (THPT) occurs in less than 8% of the patients with secondary hyperparathyroidism after successful kidney transplantation. Tertiary hyperparathyroidism is commonly due to parathyroid hyperplasia, but about 2.6% of the THPT cases may be due to single or double adenomas. We investigate the usefulness of limited resection of single or two-gland parathyroid by comparing the blood calcium and PTH levels with respect to operative strategy. METHODS: We analyzed a total of 13 cases of tertiary hyperparathyroidism that were diagnosed and operated on at Asan Medical Center from May 1996 to April 2005. The patients were grouped according to the operative strategy: 3 and 1/2-parathyroidectomy (n=5), 3-parathyroidectomy (n=6), and 2-parathyroidectomy (n=2), and then we compared the mean blood PTH, calcium and creatinine levels. One patient in the 3-parathyroidectomy group underwent hemodialysis due to kidney transplant rejection, and this data was excluded from the analysis. RESULTS: The mean blood calcium and PTH levels of the 2-parathyroidectomy group were higher than that of the other two groups. There were no cases of transient hypocalcemia in the 3 groups (<7.5 mg/dl), and there were no cases of hypercalcemia at one year postoperative in the 3 & 1/2-parathyroidectomy and 2-parathyroidectomy groups. The blood calcium and blood PTH levels were less than 9.1 mg/dl and more than 100 pg/ml, respectively, in the one 3-parathyroidectomy patient who underwent hemodialysis due to kidney transplant rejection. CONCLUSION: Our preliminary conclusions, based on our small groups and the short follow-up period, are that the blood calcium and PTH levels will be higher in the limited resection group if kidney transplant rejection occurs, and tumor recurrence will be also more often found in the limited resection group. Therefore, our recommendation is that 3 and 1/2-parathyroidectomy and 2-parathyroidectomy are preferable operative strategies for tertiary hyperparathyroidism.

A Case of Hungry Bone Syndrome after Parathyroidectomy / 대한신장학회잡지

Patients with secondary hyperparathyroidism usually present with a history of underlying disease such as chronic renal failure. Tertiary hyperparathyroidism usually exists in situations of secondary hyperparathyroidism. It occurs when parathyroid hyperplasia becomes so severe that removal of the underlying cause does not eliminate the stimulus for PTH secretion and hypertrophic chief cells become autonomous. Surgical parathyroidectomy sould be considered in patients with uncontrolled hyperparathyroidism. Hungry bone syndrome is known to be developed due to extensive remineralization of skeleton after parathyroidectomy. It is characterized by prolonged symptomatic hypocalcemia, as a complication of the parathyroidectomy for hyperthyroidism. We have experienced a female patient with hypercalcemia, who had been on maintenance hemodialysis for 15 years. She had elevated intact PTH and alkaline phosphatase. We decided parathyroidectomy because of uncontrolled hyperthyroidism despite of medical treatment. A few weeks after the operation she developed a muscle pain and arthralgia, which were found to be due to severe hypocalcaemia. Calcium suppletion led to normalization of the serum calcium level. she was discharged from the hospital in good condition after calcium supplement. We report a case of hungry bone syndrome developed after parathyroidectomy in this patient.

A Case of Hungry Bone Syndrome after Parathyroidectomy in Patient with Tertiary Hyperparathyroidism and Hemodialysis Patient / 대한내분비외과학회지

Hyperparathyroidism is a common finding in patients with chronic renal failure. Among the hyperparathyroidism, tertiary hyperparathyroidism, which is secreting parathyroid hormone autonomously in spite of hypercalcemia. Sometimes it requires surgical intervention due to not only symptomatic hypercalcemia, but also longstanding asymptomatic hypercalcemia. Hungry bone syndrome was known to be developed due to extensive re-mineralization of skeleton after parathyroidectomy. It is characteristic of persistent hypocalcemia, hypophosphatemia and often with tetany. The patient's condition improved without complication after the calcium chloride and calcium carbonate administration. We report a case of hungry bone syndrome developed after parathyroidectomy in patient with tertiary hyperparathyroidism and chronic renal failure.

A Case of Tertiary Hyperparathyroidism Combined with Thyroid Papillary Carcinoma / 대한이식학회지

Secondary hyperparathyroidism is the condition which leads to excessive production of the parathyroid hormone secreted to compensate for longstanding hypocalcemia in chronic renal failure. After restoration of normal renal function, some patients continue to have autonomous parathyroid hyperfunction. In 1963 St. Goar termed it tertiary hyperparathyroidism. Hyperparathyroidism in the chronic renal failure is mostly well managed medically, but sometimes may require surgical intervention in refractory hyperparathyroidism. Recently, we have experienced a female patient diagnosed as tertiary hyperparathyroidism with persistent hypercalcemia after renal transplantation and report the results of subtotal parathyroidectomy and thyroid right lobectomy due to hyperparathyroidism and thyroid papillary carcinoma found coincidentally.