Large atypical parathyroid tumor - a diagnostic conundrum.

Atypical parathyroid tumor (APT) is a rare neoplasm of the parathyroid gland, which shows atypical cytological or architectural features and lacks definite diagnosis criteria for malignancy. These cases can cause diagnostic challenges owing to their rarity and similarity with thyroid neoplasm on imaging and fine needle aspiration cytology. Also, differentiating APT from giant parathyroid adenoma or parathyroid carcinoma can be challenging based on clinical, imaging or cytological features. A 49-year-old male presented with clinical features of hyperparathyroidism. On laboratory evaluation, his serum calcium and serum parathyroid hormone was elevated. Imaging studies suggested a possibility of left inferior parathyroid neoplasm, and fine needle aspiration cytology showed features suggestive of parathyroid neoplasm. However, exact categorization of parathyroid tumor was difficult in pre-operative work-up. Possibilities of giant parathyroid adenoma as well as parathyroid carcinoma were considered. A final diagnosis of an atypical parathyroid tumor was made after detailed histopathological evaluation given focal capsular invasion but lack of unequivocal evidence of malignancy in the resected specimen. APT is a rare neoplasm of uncertain malignant potential. Knowledge of the radiological and pathological features will be helpful in accurately identifying the lesion and avoiding misdiagnosis.

Time to Recurrence as a Prognostic Factor in Parathyroid Carcinoma.

Background: Parathyroid carcinoma (PC) is a rare and challenging disease without clearly understood prognostic factors. Adequate management can improve outcomes. Characteristics of patients treated for PC over time and factors affecting prognosis were analyzed. Methods: Retrospective cohort study including surgically treated patients for PC between 2000 and 2021. If malignancy was suspected, free-margin resection was performed. Demographic, clinical, laboratory, surgical, pathological, and follow-up characteristics were assessed. Results: Seventeen patients were included. Mean tumor size was 32.5 mm, with 64.7% staged as pT1/pT2. None had lymph node involvement at admission, and 2 had distant metastases. Parathyroidectomy with ipsilateral thyroidectomy was performed in 82.2%. Mean postoperative calcium levels were different between patients who developed recurrence vs those who did not (P = .03). Six patients (40%) had no recurrence during follow-up, 2 (13.3%) only regional, 3 (20%) only distant, and 4 (26.6%) both regional and distant. At 5 and 10 years, 79% and 56% of patients were alive, respectively. Median disease-free survival was 70 months. Neither Tumor, Nodule, Metastasis system nor largest tumor dimension (P = .29 and P = .74, respectively) were predictive of death. En bloc resection was not superior to other surgical modalities (P = .97). Time between initial treatment and development of recurrence negatively impacted overall survival rate at 36 months (P = .01). Conclusion: Patients with PC can survive for decades and have indolent disease course. Free margins seem to be the most important factor in initial surgery. Recurrence was common (60%), but patients with disease recurrence within 36 months of initial surgery had a lower survival rate.

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.

Manejo quirúrgico de carcinoma de paratiroides: a propósito de un caso / Surgical management of parathyroid carcinoma: a case report

Resumen Introducción: A pesar de que el carcinoma de paratiroides es uno de los cánceres menos frecuentes del mundo, es importante tenerlo en cuenta al plantear el diagnóstico diferencial del hiperparatiroidismo primario, ya que su diagnóstico temprano tiene repercusiones en el tratamiento y el pronóstico vital del paciente. Caso Clínico: A continuación, se expone un caso clínico de un paciente con sintomatología abigarrada de hiperfunción paratiroidea que, dada la sospecha clínica de carcinoma de paratiroides y la no infiltración de estructuras adyacentes, fue tratado con una paratiroidectomía. Conclusión: Esta cirugía supone una menor morbilidad, sin suponer un detrimento para la supervivencia global del paciente.

Introduction: Parathyroid carcinoma should be taken into consideration among the differential diagnosis of primary hyperparathyroidism, even though it is one of the less common malignant tumours in the world, because an early diagnosis is essential for the treatment and the prognosis of the patient. Case Report: We present the case of a patient whose symptoms were compatible with hyperfunction of parathyroid gland. Due to the malignant disease suspicion and the non-invasion of adjacent tissue, he was treated with a parathyroidectomy. Conclusión: This type of treatment supposes a lower morbidity without decrease in overall survival, according to bibliography.

Parathyroid carcinoma treated with parathyroidectomy and hemithyroidectomy.

Parathyroid carcinoma is a very rare oncological entity, presenting insidiously in the second and fourth decades of life, characterized by hypercalcemia and hyperparathyroidism of difficult control, accompanied by general malaise, complications secondary to hypercalcemia and cervical tumor, being the main differential diagnosis of parathyroid adenoma. Complementing imaging studies of neck, scintigraphy, and contrast neck computed tomography. The diagnostic suspicion of parathyroid carcinoma is usually confirmed by the pathology of the surgical specimen. The surgery of choice is parathyroidectomy plus ipsilateral thyroidectomy. Monitoring of hypercalcemia and hyperparathyroidism is performed.

El carcinoma paratiroideo es una afección oncológica muy infrecuente, que se presenta de manera insidiosa en la segunda y cuarta décadas de la vida. Su clínica se caracteriza por hipercalcemia e hiperparatiroidismo de difícil control, y se acompaña de malestar general, complicaciones secundarias a la hipercalcemia y tumoración cervical, siendo el principal diagnóstico diferencial el adenoma paratiroideo. Son necesarios estudios de imagen, como ecografía de cuello, gammagrafía y tomografía computarizada de cuello con contraste. La sospecha diagnóstica de carcinoma paratiroideo usualmente se confirma con el estudio de patología de la pieza quirúrgica. La cirugía de elección es la paratiroidectomía más tiroidectomía homolateral. Se realiza seguimiento para el control de la hipercalcemia y del hiperparatiroidismo.

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

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.

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.

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.

Carcinoma de paratiroides: como causa de muerte / Parathyroid carcinoma: as a cause of death

ResumenSe presenta el caso de un masculino de 25 años de edad, conocido sano, trabajador en un piñera, con un cuadro de 3 semanas de evolución de malestar general, adinámia, astenia, síntomas gastrointestinales y pérdida de peso. Fue valorado en el servicio emergencias de un hospital rural donde se describió que presentaba fascies de crónicamente enfermo, los laboratorios evidenciaron calcio en 21,10 g/dl, diagnosticándosele hipercalcemia maligna de probable etiología neoplásica e insuficiencia renal aguda, presentó cuadro de convulsiones generalizadas y falleció el mismo día que ingresó. El carcinoma de paratiroides es un tumor infrecuente con una incidencia menor a 1 en 1 millón, su clínica se basa en los efectos por la excesiva secreción de hormona paratiroidea y por niveles elevados de calcio en sangre como en el presente caso.

AbstractWe report the case of a 25-year-old male, known as a healthy, hard-working worker, with a 3-week history of malaise, adynamia, asthenia, gastrointestinal symptoms and weight loss. It was evaluated in the emergency service of a rural hospital where it was described that it presented fascies of chronically ill, the laboratories evidenced calcium in 21,10 g / dl, being diagnosed malignant hypercalcemia of probable neoplastic etiology and acute renal failure, he presented generalized convulsions and died the same day he entered. Parathyroid carcinoma is an uncommon tumor with an incidence of less than 1 in 1 million. Its clinical features are based on the effects of excessive secretion of parathyroid hormone and elevated levels

Tumores pardos mimetizando enfermedad metastásica ósea como presentación inicial de un carcinoma paratiroideo: A propósito de un caso / Brown tumors mimetizing bone metastasic disease as an initial presentation of a parathyroid carcinoma: About a case

Objetivo: Describir caso inusual de paciente con tumor pardo, manifestación poco frecuente del hiperparatiroidismo, como presentación inicial de carcinoma de paratiroides. Caso Clínico: Paciente masculino de 25 años, inicia enfermedad actual en el 2011, caracterizada por dolor en encía inferior de hemiarcada izquierda, acompañada de tumoración en región maxilar inferior ipsilateral. Se evidencia progresión de dicha lesión y aparición de nueva tumoración en maxilar superior derecho, además de dolores óseos, limitación a la movilización y fracturas patológicas en húmeros. En abril de 2015 presenta disartria, dificultad en la alimentación por progresión de la tumoración en la cavidad oral, además de sangrado en el sitio de la lesión, por lo que es hospitalizado. Examen físico: lesión tumoral en maxilar superior e inferior que deforma la arcada dentaria, con sangrado activo que imposibilita la oclusión completa de boca, con asimetría facial. Extremidades: asimetría en ambos húmeros. Paraclínicos: PTH: 990 pg/mL, calcio: 13 mg/dL, fosfatasa alcalina: 300 UI/L. Se realiza paratiroidectomía y lobectomía tiroidea izquierda. Biopsia: carcinoma paratiroideo. A los meses se evidencia mejoría del dolor óseo y del tamaño de las lesiones. Conclusión: El carcinoma de paratiroides es una neoplasia rara, la prevalencia es menos de 1% de los casos de hiperparatiroidismo, y la incidencia es de 0.015 por 10000 casos. El tumor pardo es una forma infrecuente de manifestación de un hiperparatiroidismo, reflejando un desafío en el diagnóstico diferencial de una enfermedad metastásica ósea, y más aún cuando el carcinoma paratiroideo generalmente al momento del diagnóstico ya presenta metástasis a distancia.

Objective: To describe the unusual case of a patient with brown tumor, rare manifestation of hyperparathyroidism, as initial presentation of a parathyroid carcinoma. Case Report: Two years later evidence progression of the lesion, also appearance of new tumor in the right upper jaw, in addition to bone pain, limitation mobilization and pathological fractures in the humerus. In April 2015 the patient presents dysarthria and difficulty in feeding due to progression of the tumor in the oral cavity, in addition to bleeding at the site of injury, so it is hospitalized. Physical examination: tumor lesion in upper and lower jaw, which deforms the dental arch, with active bleeding that precludes complete occlusion of the mouth, with facial asymmetry. Limbs: asymmetry in both humerus. Paraclinical: PTH: 990 pg/mL, calcium: 13 mg/dL, alkaline phosphatase: 300 IU/L. Parathyroidectomy and left thyroid lobectomy was performed. Biopsy: parathyroid carcinoma. Months later, improvement in bone pain and lesion size was evident. Conclusions: Parathyroid carcinoma is a rare neoplasm, the prevalence is less than 1% of cases of hyperparathyroidism, and the incidence is 0.015 per 10,000 cases. The brown tumor is a rare form of manifestation of hyperparathyroidism, reflecting a challenge in the differential diagnosis of a metastatic bone disease, and even more when the parathyroid carcinoma usually at the time of diagnosis already presents distant metastases.

[Parathyroid disease: The full spectrum, from adenoma to carcinoma. Report of 3 cases]. / Enfermedad paratiroidea: el espectro completo, de adenoma a carcinoma. Reporte de 3 casos.

BACKGROUND: Primary hyperparathyroidism is a disease characterised by the autonomous production of parathyroid hormone. The most common cause is an adenoma, followed by hyperplasia, and rarely carcinoma. CLINICAL CASES: Three cases are presented. The first case is associated with a brown tumour that was diagnosed as hyperplasia after study and surgery. The second case was related to pathological fractures, and a lower right adenoma 236 times bigger than a normal parathyroid was excised. The last case presented with abdominal pain and heartburn. Histopathology reported a carcinoma, which was removed using surgery en bloc. All patients have improved. CONCLUSION: Hyperparathyroidism symptoms are very difficult to identify and diagnose, thus a detailed and broad approach is needed when hyperparathyroidism is suspected.

Evaluation of preoperative ultrasonographic and biochemical features of patients with aggressive parathyroid disease: is there a reliable predictive marker?

ABSTRACT Objective Parathyroid cancer (PC) represents < 1% of cases of PHPT. Tumors demonstrating atypical histopathologic features and don’t fulfill criteria for carcinoma are classified as atypical adenomas (APA). The purpose of this study was to determine a biochemical or ultrasonographic feature that can predict aggressive disease requiring more extensive surgery and closer follow-up. Subjects and methods Twenty eight patients operated for PHPT and diagnosed with atypical adenoma (23 patients) or carcinoma (5 patients) were enrolled in this study. The control group consisted of 102 patients operated between the same dates and diagnosed with classical PA. Classical adenomas, atypical adenomas, and carcinomas were compared according to their biochemical and ultrasonographic parameters. Results Serum Ca levels were significantly higher in the PC group compared with the APA and classical PA groups. Serum median PTH, Serum ALP and UCa was significantly higher in the APA and carcinoma groups compared to the classical PA group. ROC analysis was made to determine the best cut off values for predicting aggressive disease were 12.45 mg/dL, 265.05 pg/mL, 154.5 IU/l, 348.5 mg/day and 21.5 mm for Ca, PTH, ALP, UCa and the adenoma diameter, respectively. Multivariate analysis showed that serum Ca, ALP and isoechoic/cystic appearance were independent predictors for aggressive disease. Conclusion Preoperatively high PTH, ALP, and UCa levels and large lesions with isoechoic or cystic appearances may be predictive of atypical adenoma or carcinoma in patients being evaluated for PHPT. In such cases, surgeons may prefer en bloc parathyroidectomy to minimally invasive surgery.