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Parathyroid carcinoma is one of the least frequent malignant neoplasms, diagnosis and treatment are often complex; however, the prognosis is not bad and, although recurrences are frequent, the mortality rate is low. We describe the clinical case of a 42-years-old patient, the evolution of the natural history and treatment.

The diseases of parathyroid glands have assumed a growing importance for innovations in diagnosis and surgery which have enabled a more precise identification and therapy. Use of Sesta-MIBI scintigraphy allows a correct localization of the adenoma; the introduction of intraoperative monitoring of intact parathyroid hormone made possible unilateral neck exploration with a mini-incision above the adenoma. The emergence of videotechnology has led the innovation of endoscopic parathyroidectomy, realized for the first time in 1996 and subsequently performed without the use of CO2. Currently, the SPECT scintigraphy allows a three-dimensional vision of the adenoma and is the more precise localization study, allowing to use mini-invasive access. Prerequisite for the use of minimally invasive techniques is the determination of intraoperative PTH, possible with various techniques. Our experience is based on 135 patients undergoing parathyroidectomy in the last 8 years with 5 cases of persistent hyperparathyroidism submitted to reoperation, an average hospital stay of 2 days and only 6 complications (1 bleeding and 5 temporary hypoparathyroidisms). The use of new technologies in parathyroid surgery can achieve optimal results, a better cosmetic result and quicker postoperative recovery, with a low incidence of recurrence and complications.

The authors, after reviewing parathyroid gland diseases, their location, and the modern strategies that can be used for their pre-operative detection, describe a case of primary hyperparathyroidism which recently came to their attention. The use of a combination of instrumental techniques (US, scintigraphy and SPEcT) enabled them to establish, prior to surgery, the mediastinal ectopic site of the parathyroid adenoma. Mini-invasive surgery proved to be the optimal technique to performing a targeted surgical excision that reduced the operative time and the hospitalisation.

Aim: The aim of the study was to draw up a management protocol in parathyroid surgery promoted by the Italian Association of Endocrine Surgery Units (UEC Club), based on the guidelines of the main international scientific societies and shared by the experts and applied by the operators in the sector., Methods and Consensus: The management protocols, already presented in 2003, on the occasion of the current review were examined by the 1st Consensus Conference called on the topic by the Italian Association of Endocrine Surgery Units (UEC). The Conference comprised two distinct sessions, the first in November 2006 within the framework of the 5th National Congress of the UEC Club in Verona, and the second in September 2007 within the framework of the 10th Multidisciplinary Scanno Prize Meeting. A selected board of endocrinologists and endocrine surgeons examined the individual chapters and submitted the consensus text for the approval of several experts., Conclusions: The diagnostic, therapeutic and healthcare management protocols in parathyroid surgery approved by the 1st Consensus Conference are officially those proposed by the Italian Association of Endocrine Surgery Units (UEC Club) and are subject to review by October, 2009.

We present the case of a male patient who needed surgery for a large undefined submandibular schwannoma and a small contralateral thyroid carcinoma associated with cervical lymph nodes of a dubious nature. During the operative procedure all the pathological conditions were resolved, with some remarkable surprises. A non-functioning parathyroid adenoma was found and removed. A fairly unusual anatomical complication was also detected with regard to the right inferior laryngeal nerve, i.e. an anastomotic branch connecting the main trunk to the vagus nerve.

Introduction: Parathyroid carcinoma is a rare endocrine neoplasm, difficult to define clinically and histopathologically., Case Report: The case concerns of a 20 years old male with situs viscerum inversus (with dextrocardia), and symptoms: asthenia, oliguresis, nausea, emesis, myalgia, lower limb paresthesia and very high levels of calcium and PTH. Laboratory findings (PTH 580 pg/ml; Ca 12.40 mg/dl; P 1.9 mg/dl), echography, TC, and parathyroid scintigraphy, associated with clinical data, have suggested hypothesis of parathyroid carcinoma confirmed by histological examination and immunochemistry. Surgery was efficacious with normalization of Calcium and PTH levels, and disappearance of symptoms. After two years no signs of local recurrence were present, but imaging show pulmonary micronodulations of uncertain pathological meaning., Discussion and Conclusion: No clinical or bio-humoral data allows a preoperative diagnosis of parathyroid carcinoma. Only with definitive pathology and immunohistochemistry it is possible to differentiate an adenoma from a carcinoma. Surgery is the only effective therapy and therefore should be always performed. This neoplasm usually relapses, locally first and later with distant metastases. For this reason after surgery the patients should always undergo a strict follow-up programme including evaluation of PTH and calcemia.

Parathyroid cysts are rare lesions. A correct pre-operative diagnosis is rarely formulated. The Authors report a case of young woman with hyperparathyroidism do to a giant intrathyroidal parathyroid cyst. Aetiology, diagnosis and management are discussed.

Surgical management of primary hyperparathyroidism has undergone several chances in recent years and historically has required bilateral neck exploration with identification of the parathyroid adenoma together with three normal glands. The intraoperative hormone assay allows a more limited procedure by confirming complete removal of hypersecreting tissue. The Authors report surgical treatment of 24 consecutive hyperparathyroidism and conclude that evaluation of intraoperative hormone assay accurately predicts the determination of adequacy of resection and the correct outcome of surgery in patients with parathyroid adenomas.

Parathyroid carcinoma is a rare entity with an 0,5-1% of incidence on primary hyperparathyroidism (HPP) in literature. The very high values of calcium and parathormone (PTH) and the clinical aspects of hypercalcemia are the characteristics findings in these patients. We present our experience on 6 pts on 153 cases with HPP treated (3,9%). The clinical and diagnostic suspects are frequently intraoperative findings and the decision making for the surgeon is not always easy (parathyroidectomy with or without hemithyroidectomy, lymphadenectomy, surgical resection of other tissues). The mortality rate is high and we have registered three deaths at 8,14 and 64 months.

Parathyroid carcinoma is a rare malignancy. We report the case of a 66 years old man referred to our department for a large substernal goiter suspected as a thyroid cancer associated with severe hyperparathyroidism. After normalization of serum levels of calcemia, total thyroidectomy and subtotal parathyroidectomy was performed. Histopathology revealed a parathyroid cancer of 450 g.

This study aims to clarify the diagnostic value of ultrasonography, computerised tomography and magnetic resonance in adenomatous pathology of primary and persistent hyperparathyroidism. Although the diagnostic efficacy of the various imaging techniques is still the subject of current debate, the latest specific surgical techniques, require their use either individually or in association, in order to achieve faster and safer surgery. After an overview of the various methods, illustrating their indications and diagnostic limitations, the authors emphasise that the role played by ultrasonography, CAT and MR justifies their use in the preoperative localisation of parathyroid adenoma. It is not possible to assert in absolute terms that one methods is better than another, but each offers different potential. The use of US as the method of choice is indicated in primary hyperparathyroidism with hyperplastic glands owing to its innocuous nature and rapidity. In addition to US and scintigraphy, magnetic resonance may be appropriate in primary hyperparathyroidism with adenomatous glands. In persistent forms of primary hyperparathyroidism, magnetic resonance is the most reliable method, whereas CT and, to an even greater degree, ultrasonography play a marginal role.

Primary hyperparathyroidism is a rather frequent pathology characterised by hypersecretion of parathormone (PTH) which is caused by adenomas in 85% of all cases. At clinical onset, the most common symptoms are hypercalcemia-related (pain due to kidney stones, polyuria, gastrointestinal and neurological disorders) while rarer symptoms are due to brown tumors, expansive lesions often found in fibro-cystic osteitis. A case in which the patient showed recurrent mandibular brown tumors as initial clinical symptoms of primary hyperparathyroidism is described. This patient was examined for hypercalcemia, and a tumor mass at the left inferior mandibular branch was found. The patient had undergone surgical removal of a tumor in the left mandibular some years before, which was diagnosed as osteoclastoma. Primary hyperparathyroidism was diagnosed during recovery, and surgical removal of the parathyroid adenoma and mandibular tumor was performed. A histological diagnosis of large cell brown tumor was made. A microscopic observation of brown tumors which are made up of large multinuclear osteoclastic cells can often be confused with other large cell tumors during diagnosis. It is therefore necessary to exclude the presence of hyperparathyroidism with ionised calcium and, in cases of high values, intact PTH (iPTH), before performing a histological diagnosis of a large cell bone tumor. Throughout the course of primary hyperparathyroidism, brown tumors might appear in the absence of other specific symptoms and localize at the level of a single bone segment.

Fibrous-cystic osteitis is a bone metabolic disorder related to hyperparathyroidism. This pathological condition shows a bone catabolism enhancement, due to increased level of PTH. Brown tumour is a uni- or multi-focal bone lesion, which represents the terminal stage of the hyperparathyroidism-dependent bone pathology. This focal lesion is not a real neoplasm showing itself as a cellular reparative process, mainly interesting the jaws, specially the mandible. Because of the similar radiological features (cyst-like radiolucency) showed by other lesions, the diagnosis can be difficult. Histology cannot guarantee a certain diagnosis, some lesions, such as giant cell tumour, giant cell granuloma, aneurysmal bone cyst and cherubism, show a similar macroscopical and microscopical features. Differential diagnosis is possible only by comparative evaluation of clinical, radiological and biochemical evidences. Personal experience with a patient affected by maxillary expansive lesion previously diagnosed as GCT is reported. Radiological examinations showed another cyst-like lesion involving the mandible. Clinical history and multifocality of lesions were suggestive for the presence of a systemic disease, laboratory data allowed a primary hyperparathyroidism diagnosis. Parathyroid scintigraphy was performed and detected a parathyroid adenoma. In first instance the patient underwent to surgical operation on the jaws in order to stop the rapid progression of bone lesions, and then another operation for the removal of parathyroid adenoma was performed.

Hyperparathyroidism and hypercalcaemia are considered to be a rare cause of acute pancreatitis. The relationship between hyperparathyroidism and pancreatic inflammatory disease remains controversial, but it may be related to the translation from inactive to active trypsinogen by hypercalcaemia. Surgical correction of parathyroid disease and normalization of serum calcium levels may ameliorate the acute pancreatitis. Also the mechanism of pathologic zymogen activation during acute pancreatitis remain unknown; probably the pancreatic "autodigestion" is the result of anomalous intracellular transport of secretory proteins activated by lysosomal hydrolases. A case of acute pancreatitis and hyperparathyroidism due to solitary parathyroid adenoma occurred in a 66-years-old woman is reported. After the excision of parathyroid adenoma the serum calcium levels and the function of the pancreas returned to normal. This suggests a cause and effect relationship between hyperparathyroidism and acute pancreatitis.

The authors present two cases of primary hyper-parathyroidism initially diagnosed as aneurysmal cyst. The aspects of differential diagnosis are discussed, and the importance of evaluating all of the diagnostic elements in order to formulate a correct diagnosis is emphasized.

The authors report their 3-year experience with the diagnosis of parathyroid lesions in primary hyperparathyroidism patients in a geographic area where the occurrence of endemic goiter is medium. Our study was aimed at prospectively assessing preoperative imaging results in these patients. The following imaging methods were used: high-definition and color-Doppler ultrasonography (US), double-tracer 201Thallium-99mTechnetium (T1/Tc) subtraction scintigraphy, Computed Tomography (CT), Magnetic Resonance Imaging (MRI) and US-guided fine-needle aspiration of the suspected parathyroid lesions. Preoperative US and scintigraphy were performed in 50 patients with primary hyperparathyroidism; in addition, color-Doppler US studies were performed in 33 patients for vascular characterization of the lesions. In 19 patients, the suspected lesions were punctured under US guidance to measure parathormone (PTHa) and thyroglobulin (TGa) levels in the aspirated material. CT and MRI were performed in 9 patients, to identify a possible ectopic parathyroid gland. Surgery demonstrated 48 solitary parathyroid lesions and one double parathyroid adenoma. In one patient no abnormal parathyroid gland was found. Overall sensitivity rates of US and scintigraphy were 85.7% and 61.2%, respectively. In multinodular goiter patients, the sensitivity rates of US and scintigraphy were 71.4% and 47.6%, respectively. At color-Doppler US the presence of parenchymal vascularization was specific of parathyroid nodules and the method helped differentiate parathyroid lesions from thyroid nodules in 14 multinodular goiter patients. Overall PTHa sensitivity was 72.2% and its specificity 100%. Overall TGa sensitivity was 100% and specificity 94.7%. CT and MRI allowed the detection of 8 ectopic parathyroid lesions. In conclusion, in our personal experience, US should be preferred to double-tracer T1/Tc subtraction scintigraphy in the early examination of primary hyperparathyroidism patients. When US detects a suspected parathyroid lesion, color-Doppler US and PTH and TG sampling can make useful diagnostic tools for reducing false-positive results, especially when thyroid disease is associated.

Parathyroid carcinoma is a rare cause of hyperparathyroidism (rate of occurrence of 0.5% of all parathyroid neoplasms). In this report we describe three cases of parathyroid carcinoma seen in our Institution and we analyse the clinical, diagnostic, therapeutic and pathological findings of this disease. The three patients were 14, 22 and 45 year old respectively. Familial history was negative for endocrine diseases. The laboratory and instrumental findings of thyroid and adrenal glands were negative. Predominant symptoms were in all cases weakness, lethargy, bone and muscular pain, nausea, vomiting. The two young patients presented fractures of the inferior limb and of the forearm respectively, five years and one year before the diagnosis. X-ray examination and MNR easily demonstrated the "brown tumors". In two cases a symptomatic nephrolithiasis was present. The 14 year old child presented polyuria and polydipsia. In all cases a mass was palpable in the neck (two in the right side and one in the left one). The elevated serum calcium concentration (15, 18, and 20.2 mg/dl respectively) and the elevated serum PTH (480, 651, and 680 pg/ml respectively) allowed the diagnosis of hyperparathyroidism. Ultrasound scan and thallium-technetium scanning identified in all cases a mass adjacent the thyroid. A radical resection of the malignant parathyroid gland and the ipsilateral thyroid lobe was performed in two cases, while only a resection of the involved parathyroid gland in one case. The diagnosis of parathyroid cancer was established using pathologic criteria.(ABSTRACT TRUNCATED AT 250 WORDS)

Parathyroid gland carcinoma is a rare cause of primary hyperparathyroidism. The authors discuss the case of a 56-year-old man who presented with hypercalcemia and multiple bone lesions suggestive of tumor metastases. Laboratory and radiological investigations revealed primary hyperparathyroidism in a patient with evidence of osteitis fibrosa cystica, sustained by carcinoma of a mediastinal parathyroid gland. Early titration of parathyroid hormone (PTH) levels in all patients with hypercalcemia of unclear cause is important for early diagnosis of cases that are tumor-sustained and prevention of the most severe complications. The role of immunostaining with anti-PTH antibodies in demonstrating parathyroid gland tissue in ectopic and/or non-functioning primary tumors, as well as metastases, is also discussed.

The authors describes a case of tertiary hyperparathyroidism (HPTH) in a uremic patient on intermittent dialysis treatment: the term refers to an adenoma with ensuing uncontrolled parathormone (PTH) secretion rate arising on the ground of hyperstimulated hypertrophied parathyroid glands. The syndrome was heralded clinically by bone pain, psychiatric disorder and biochemically by increased levels of calcium and alkaline phosphatase (AP), while parathormone (PTH), did not change from basal very high levels as commonly found in uraemic patients. As hypercalcemia in the hemodialyzed is an infrequent finding the only alternative explanation could have been hypercalcemic secondary HPTH related to hyperplastic autonomous parathyroids. For no clinical and laboratory findings as well as US findings and double scintigraphy (99mTc and 201mTl) may suggest differential diagnosis the patient underwent total parathyroidectomy which actually revealed an adenoma of the left superior parathyroid gland. Bone pain and psychiatric disturbances disappeared and now get well on chronic dialysis treatment and 1.25-OH Vit D3 supplement.

Hyperparathyroidism (HPT), once considered a rare disease, is nowadays observed and diagnosed more frequently. Consequently, surgical treatment of HPT is often performed with good therapeutic results. The success of parathyroid surgery depends mainly on the accuracy of histopathologic diagnosis through intraoperative examination of frozen tissue specimens. Yet, parathyroid gland pathology is very complex and some of its topics even controversial. Terminology and clinico-pathological concepts in this field are constantly changing and even basic characteristics such as weight, stromal fat content and "normal " histologic patterns of these glands are still actively discussed. The pathologist must often determine, merely by studying a small bioptic specimen, not only the parathyroidal (or non-parathyroidal) origin of the examined tissue, but also the histological "normal" or "abnormality" of the tissue. In carrying out his work the experienced pathologist takes on an important task and responsibility in distinguishing between normal, early hyperplastic (asymmetric), hyperplastic or adenomatous parathyroid glands. New diagnostic technical approaches (such as Electron Microscopy, intracellular fat morphometric evaluation, parenchymal cell mass study by density gradient, etc.) enhance the possibility of reaching a reliable pathologic diagnosis, but they do not solve all the present problems and sometimes can even lead the pathologist astray. Presently the diagnosis and treatment of primary HPT should be considered an open and current problem inasmuch as definite criteria to be followed in distinguishing between normal or single enlarged glands microscopically are still lacking. Neither the degree of accuracy of intraoperative frozen section examination during parathyroid surgery nor the possibility of misleading observations in such a study has been fully evaluated. A close cooperation between the pathologist and head and neck surgeon is of utmost importance to assure the best therapeutical results obtainable from parathyroid surgery in HPT.

The Authors introduce their experience of primary and secondary surgical treatment of hyperparathyroidism, pointing out at first the good confidence of echography, scintigraphy and TAC, in the pre-operating diagnostic of the parathyroid glands localization and then the satisfactory clinical results with a follow-up which, up to date, has not shown any relapse.

Hyperparathyroidism due to parathyroid carcinoma is rare. The natural history of this neoplastic disease is poorly understood for the extremely small number of cases reported in literature. The Authors observed three cases and discuss the clinical features illustrating how to recognize and treat this neoplasia.

Many hormones initiate their biologic actions by augmenting the intracellular concentrations of 3',5'-adenosine monophosphate (cyclic AMP). The nucleotide has been found in body fluids; its determination in plasma and urine can be performed by a rapid, simple and specific method: the cyclic AMP assay kit of the Radiochemical Centre (Amersham, England). The assay is based on the competition between unlabelled cAMP and a fixed quantity of the tritium labelled compound for binding to a bovine muscle protein which has a high specificity and affinity for cAMP. Different factors must be considered in evaluating the 24 h urinary content of the nucleotide: the renal or extrarenal origin of cAMP and the functional status of the kidneys. In basal conditions the urinary cAMP excretion is significantly correlated with creatinine excretion (n = 67; r = 0.47; p less than 0.001) thus confirming that the most part of cAMP excreted is derived from the plasma by glomerular filtration. Parathyroid hormone (PTH) stimulates adenylate cyclase predominantly in the renal cortex, whereas vasopressin (ADH) stimulated the enzyme in the medulla; thus PTH and ADH could increase the amount of cAMP in the urine from the renal source. In a case of diabetes insipidus and infusion of ADH caused a prompt rise in cAMP urinary excretion. In 5 normals an infusion of bovine synthetic parathyroid hormone caused an increased excretion of cAMP that preceded the phosphaturic response. An infusion of salmon synthetic calcitonin caused a rise in phosphate excretion and no increase in cAMP urinary content. As it concerns the two calciotopic hormones, PTH and CT, it is reasonable to assume that renal receptors are distinct. The 24 h urinary excretion of cAMP in 55 control subjects (3613 +/- 1460 D.S. n moles) was contrasted with the lower excretion in 25 elderly subjects (70-93 years: 1804 +/- 699 n moles), with the high cAMP excretion in a patient with hyperparathyroidism (that fell to normal values following removal of the parathyroid adenoma) and with the low cAMP excretion in patients with primary or surgical hypoparathyroidism. The mean 24 h cAMP excretion in patients with renal insufficiency was significantly decreased when compared to control subjects. These findings and recent reports confirm that the 24 h urinary output of cAMP may be considered an useful index of pharathyroid function in man.

We report here the unusually difficult cases of two patients with end-stage renal failure who suffered from severe hyperparathyroidism requiring surgical correction. The first patient had previously undiagnosed primary hyperparathyroidism. A first surgical neck exploration led to the discovery of four glands, paradoxically normal in size and aspect, which were removed. Subsequently, a supernumerary gland was localized in the mediastinum by computerized tomography and removed via sternotomy. After confirming the hypoparathyroid state, parathyroid autotransplantation was performed using cryopreserved tissue. The second patient had five hyperplastic parathyroid glands removed during the first neck exploration, followed by immediate parathyroid autografting. Because of the persistence of severe hyperparathyroidism, forearm autografts were subsequently removed and a sternotomy performed. Both failed to improve parathyroid hyperfunction. Numerous localization procedures remained negative. A repeat surgical neck exploration was performed because of positive double isotope scanning but was of no success in preventing fatal outcome, as were all medical treatments. These observations of two patients illustrate the difficulties in localizing and removing ectopic parathyroid lesions. Even when relying on the presently available powerful diagnostic means, correction of severe hyperparathyroidism may be extremely difficult.

The authors evaluated the diagnostic reliability of echotomography in detecting parathyroid masses: 37 patients were studied with primary hyperparathyroidism, in four cases recurrent. They all underwent surgical ablation of the mass, identification of all glands, and biopsy of an apparently normal parathyroid. Echotomography was accurate in 91.4% of cases: it identified 27 out of 39 pathological glands (sensibility 69.2%) and gave no false-positive results (specificity 100%). Since parathyroid is often ectopic, oblique scans are suggested, i.e. of jugulum while the patient is swallowing. Such scans were always used in our study, in order to identify lesions even in case of "difficult" locations, such as the superior mediastinum.

The Authors after a review of literature and on the basis of their direct experience, emphasize the importance of right protocol for diagnosis of parathyroid tumors. The techniques investigated, continuously evolving and with different sensitivity, permit improving diagnosis in order to right surgery.

Ultrasonography (US) of parathyroid glands was performed in 56 patients with clinic and/or laboratory findings diagnostic or strongly suggesting a primary hyperparathyroidism. In 42 cases CT and in 8 US-guided fine needle biopsy (FNB) were performed. Surgical or autoptic confirmation was obtained in 34 patients. In controlled cases US yielded an overall sensibility of 88.46%, specificity of 95.14% and accuracy of 94.58% in identifying enlarged parathyroid glands. Doubtful US findings can be due to atypical pattern or site and associated thyroid pathology: in these cases, also in our experience, US-guided FNB can be usefully employed. US was more accurate than CT in detecting small size glands. In our opinion CT is mandatory only in negative US and/or scintigraphic cases. According to some clinical and surgical problems US may have a localizing or diagnostic role.

Parathyroid surgery needs an appropriate diagnosis and a preoperative localization. We conducted a prospective study to compare the efficacy of 4 different imaging modalities in 17 patients: thallium-technetium subtraction scintigraphy, ultrasonography, computed tomography and arteriography. The sensitivity was: scintigraphy 58%, echotomography 86%, Tc 92% and arteriography only 33%. Neck exploration confirmed the imaging results. We found 15 cases of adenomas (2 cases of double adenoma) and 1 case of hyperplasia; in 1 patient, no lesions were found. We conclude that the association of such techniques appears to be the optimal strategy in about 100% of the patients.