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

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

Tumor pardo del paladar, presentación atípica de hiperparatiroidismo primario en adolescente: buena evolución y normalización de la masa ósea tras paratiroidectomía / Brown tumor of the palate, atypical presentation of primary hyperparathyroidism in an adolescent: good evolution and bone mass normalization after parathyroidectomy

El hiperparatiroidismo primario (HPTP) es poco frecuente en niños y adolescentes. Hay escasos datos para el manejo de estos pacientes en pediatría. Las glándulas paratiroideas son glándulas endocrinas que secretan hormona paratiroidea (PTH) y regulan el metabolismo del calcio y del fósforo. La sobreexpresión de PTH se llama hiperparatiroidismo, que se clasifica en primario, secundario y terciario. En los adolescentes, 80 a 92% de los hiperparatiroidismos primarios se deben a adenoma paratiroideo. Presentamos el caso clínico de una adolescente con una primera manifestación atípica de HPTP, la presencia de un tumor pardo del paladar, presentación rara de adenoma paratiroideo, acompañado de hipercalcemia, marcada elevación de PTH y varias lesiones óseas. (AU)

Primary hyperparathyroidism (PHPT) in children and adolescents is uncommon. Guidelines for management in pediatric patients are limited. Parathyroid glands are endocrine glands that secrete parathyroid hormone (PTH) and regulate calciumphosphate metabolism. The overexpression of PTH is called hyperparathyroidism, and is classified as primary, secondary, and tertiary. In adolescents, 80 to 92% of PHPT cases are due to a parathyroid adenoma. We present here a case report of an adolescent with a brown tumor of the palate as the first manifestation of the disease, atypical and rare presentation of parathyroid adenoma in an adolescent. She had hypercalcemia, marked elevation of PTH and bone lesions. (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.

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.

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

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

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

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

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

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

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

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.

Tumores pardos múltiples en un paciente con hiperparatiroidismo primario / Multiple brown tumors in primary hyperparathyroidism: report of one case

Brown tumors are an uncommon manifestation of primary and secondary hyperparathyroidism. We report a 38 years old male consulting for generalized bone pain and prostration caused by multiple osteolytic lesions. Diagnostic work up disclosed a primary hyperparathyroidism secondary to a right parathyroid adenoma. The patient was subjected to a parathyroidectomy. After one year of follow up, symptoms have decreased considerably, laboratory parameters are normal and bone lesions are disappearing.

Severe hyperparathyroidism versus parathyroid carcinoma: a clinical dilemma

No clinical or laboratory data allow a preoperative diagnosis of parathyroid carcinoma and only occasionally does a definitive finding differentiate an adenoma from a carcinoma. We present a case of primary hyperparathyroidism presenting with severe elevation of parathyroid hormone and serum calcium as well as complications. We go on to discuss the case in the light of a literature review. The severity of the elevation of the parathyroid hormone, other biochemical alterations, the presence of skeletal and renal complications and of a neck mass should alert the clinician to a possible parathyroid carcinoma. Radical surgery is the only effective therapy for parathyroid carcinoma, and should always be performed if a preoperative suspicion is entertained

Carcinoma de paratireóide / Parathyroid carcinoma

O carcinoma de paratireóide uma neoplasia incomum. Corresponde a menos de 1 por cento dos casos de hiperparatireoidismo primário. Manifesta-se por hipercalcemia severa e mais de 50 por cento dos pacientes terão doença renal ou óssea concomitante...

Parathiroid carcinoma is a incommon malignancy. It accounts for less than one per cent of cases of primary hyperparathiroidism. It is manifested by severe hypercalcemia and up to 50 per cent of patients will have concomitant kidney or bone disease...

Myoview [technetium-99m tetrofosmine] scintigraphy of parathyroid gland in primary hyperparathyroidism

Minimal surgical techniques are increasingly used to remove parathyroid adenomas. Such surgery depends on accurate preoperative localization of the parathyroid swelling. The preoperative accuracy of Tc-99m tetrofosmin was evaluated in 45 patients presented with primary hyperparathyroidism and compared with ultrasonography and Tc-99m pertechnetate studies / Thallium-201 [Tc-O[4]/TL-20I] subtraction. The overall sensitivity of Tc-99m tetrofosmin was 88.8% for double phase study and 91.1% for dual tracer subtraction and that for Tc-99mO[4]/TL-201 subtraction was 80% and US was 73.3% with high significant difference. High reliability and accuracy of Tc-99m tetrofosmin in pre-operative localization of parathyroid adenoma

Hiperparatireoidismo primário familiar isolado: análise e descrição de uma família com seis casos índices: [carta ao editor] / Familial isolated primary hyperparathyroidism: description and analyses of six cases: [letter to the editor]

Objetivamos descrever e analisar uma família com seis casos de hiperparatireoidismo familiar isolado (HFI), uma rara doença hereditária de padrão autossômico dominante, caracterizada por hiperparatireoidismo primário sem associação com outras doenças ou tumores endocrinológicos. O diagnóstico foi realizado através da demonstração de hipercalcemia, aumento dos níveis de paratormônio e tumores de paratireóide à histopatologia, excluindo-se neoplasias endócrinas múltiplas do tipo 1 (NEM 1) e do tipo 2a (NEM 2a), além da síndrome hiperparatireoidismo/tumor de mandíbula (HPT/TM). Analisamos a descrição dos exames diagnósticos iniciais, a abordagem cirúrgica, os laudos histopatológicos pós-operatórios e suas evoluções. A primeira paciente operada neste instituto há 20 anos, recidivou onze anos após, e possuía uma irmã com diagnóstico prévio, o que motivou a investigação de outros familiares. A observação do caráter familial nesses pacientes contribuiu para a facilitação diagnóstica e encaminhamento terapêutico dos mesmos, assim como a orientação clínica e genética à família.

Our objective is to evaluate and describe one family with six cases of familial isolated primary hyperparathyroidism (HFI), a rare hereditable disorder with an autossomal dominant mode of inheritance. It is characterized by a primary hyperparathyroidism without association with other endocrine tumors or diseases. The HFI diagnosis relied on the demonstration of hypercalcemia, inappropriately high levels of parathyroid hormone, and parathyroid adenomas, plus exclusion of NEM 1/2a and HPT/TM syndrome in this family. We analyzed the description of the first diagnosis, surgical approach, postoperative histopathological results and their development process. The first patient, treated in our institute twenty years ago, has recidivated eleven years after the treatment. Her sister had had the same diagnosis, which motivated us to investigate theirs relatives. The analysis of the characteristics that run in these patients' family has contributed to facilitate their diagnosis and therapeutic treatment, including clinical and genetic orientation of this family.

Ectopic parathyroid adenoma--the hidden culprit.

Primary Hyperparathyroidism is known to present with protean manifestations leading to misdiagnosis in the initial stages of the disease. Inability to locate the adenoma in an ectopic parathyroid gland may further delay the diagnosis of these cases. Aberrant migration during development may lead to intrathyroidal or other ectopic locations of parathyroid glands. This may lead to their misdiagnosis as a thyroid nodule or failure to locate parathyroids during surgery. Similarity in cytological picture between thyroids and parathyroids may further complicate diagnosis by fine needle aspiration cytology. Nuclear imaging scintigraphy accurately localizes the tumor in 90% of cases and simplifies the surgical management. We encountered three such cases with the parathyroid gland adenomas in ectopic locations in which pre-operative nuclear imaging played a major role.

Case report of parathyroid carcinoma with raised urinary catecholamines: a diagnostic dilemma.

Parathyroid carcinoma is often misdiagnosed as thyroid carcinoma clinically and also with fine needle aspiration cytology. Moreover in cases misdiagnosed as thyroid carcinoma pre operatively, raised urinary catecholamines may mislead to a diagnosis of MEN2 A. We report a case of a patient admitted to the surgery department ofour hospital with a swelling in the thyroid region, raised urinary catecholamines and urinary VMA levels, with hypercalciuria and elevated parathyroid hormone levels. It was clinically suspected as thyroid carcinoma with parathyroid adenoma and associated MEN 2A syndrome.