Ectopic atypical parathyroid lipoadenoma: a rare cause of severe primary hyperparathyroidism

Parathyroid lipoadenomas are a rare and benign variant of parathyroid adenomas that are defined morphologically by an abundance of fat cells. They may be functional with secretion of parathyroid hormone or nonfunctional. We present a case of a parathyroid lipoadenoma in a patient presenting with clinical manifestations of severe primary hyperparathyroidism. Unusual features in this case included the ectopic location of the lipoadenoma in the mediastinum, the significant size of 6.6 × 3.4 cm, and weight of 30.1 g. In addition to the large size of this generally benign type of parathyroid neoplasm, histologic findings included focal band-forming fibrosis compatible with the diagnosis of an atypical parathyroid adenoma. In conclusion, this case illustrates a difficult case of an ectopic functioning parathyroid lipoadenoma with chronic sequelae due to severe hyperparathyroidism.     

Parathyroid lipoadenoma: a rare cause of primary hyperparathyroidism. Case report

Three patients with lipoadenoma of the parathyroid gland are reported. This rare tumour was encountered at surgical exploration for primary hyperparathyroidism in two patients and as an incidental finding at autopsy in the third patient. In the 2 operated patients the lipoadenoma was the cause of primary hyperparathyroidism and they were both cured by extirpation of the tumour. In the autopsy case there was no evidence of excessive parathyroid hormonal activity. The dominating stromal component of all three cases was abundant adult adipose tissue interspersed with epithelial trabeculae of mainly chief cells.     

Parathyroid adenoma associated with thymoma in a female adult with primary hyperparathyroidism

INTRODUCTIONParathyroid adenoma associated with a thymoma is a rare clinical entity and few cases have been reported in the literature. This association can be explained by the common embryologic origin of the parathyroid glands and the thymus. These patients may present only with clinical signs and symptoms of primary hyperparathyroidism making it difficult to suspect the coexistence of thymoma.PRESENTATION OF CASEA 68-year-old female with medical history of primary hyperparathyroidism underwent surgical excision of a single parathyroid adenoma. Intra-operatively a small mass was detected in the proximity of the parathyroid adenoma and was also removed. Pathologic examination revealed parathyroid adenoma as suspected pre-operatively whereas the small mass excised was identified as a type A thymoma. The postoperative course of the patient was favourable, with normal serum calcium levels and in six months follow-up the patient is symptom-free and with no recurrence.DISCUSSIONObjective of this study is to report the rare case of a female adult with a parathyroid adenoma and a coexisting thymoma in order to underline the importance of these two pathologies and the ideal treatment that should be followed according to the latest records.CONCLUSIONSurgical removal of parathyroid adenoma is the treatment of choice for primary hyperparathyroidism, with complete recovery and no postoperative complications. Thymoma may be associated with primary hyperparathyoridism, with or without clinical signs and symptoms of myasthenia gravis and this is a rare clinical entity as very few cases have been described worldwide. To our knowledge this is the first case reported in Greece.     

Outpatient video-assisted thoracoscopic surgery (VATS) for ectopic mediastinal parathyroid adenoma: a case report and review of the literature

Up to 20% of abnormal parathyroid glands causing primary or secondary hyperparathyroidism are located ectopically. Of these, approximately 1%-2% reside in the mediastinum and may not always be resectable through a traditional cervical approach. Recently, video-assisted thoracoscopic surgery (VATS) has arisen as a minimally invasive method for resecting mediastinal parathyroid glands and avoiding the complications and higher morbidity of a sternotomy. In this paper, we present a case of a patient with asymptomatic hyperparathyroidism and an ectopic parathyroid on sestamibi imaging in the mediastinum. Initially, the patient underwent a bilateral cervical exploration, left upper parathyroidectomy, and partial thymectomy; however, postoperatively, he continued to have persistently elevated serum calcium and parathyroid hormone levels. Ultimately, management consisted of parathyroidectomy through a VATS approach, along with intraoperative parathyroid assay monitoring and frozen-section pathologic analysis. The patient was successfully discharged to home several hours after surgery. To our knowledge, this is the first reported case of mediastinal ectopic parathyroid adenoma treated with outpatient VATS.     

Primary hyperparathyroidism with water clear cell content: the impact of histological diagnosis on clinical management and outcome

Water clear cell hyperplasia (WCCH) and water clear cell adenomas (WCCA) of the parathyroid glands are rare causes of primary hyperparathyroidism. We report in this series one case of WCCH and two cases of WCCA representing 0.3% of patients with primary hyperparathyroidism presenting to our institution. Increased parathyroid cellular water content was responsible for relatively larger parathyroid gland sizes. However, this was not associated with higher biochemical markers or more severe clinical presentations. Histological distinction between WCCH and WCCA is difficult but important since patients with WCCH who have had a parathyroidectomy via a unilateral neck exploration may carry an increased risk of future disease recurrence.     

Radiological considerations and surgical planning in the treatment of giant parathyroid adenomas

Giant parathyroid adenomas constitute a rare clinical entity, particularly in the developed world. We report the case of a 53-year-old woman where the initial ultrasonography significantly underestimated the size of the lesion. The subsequent size and weight of the adenoma (7cm diameter, 27g) combined with the severity of the hypercalcaemia raised the suspicion for the presence of a parathyroid carcinoma. This was later disproven by the surgical and histological findings. Giant parathyroid adenomas are encountered infrequently among patients with primary hyperparathyroidism, and appear to have distinct clinical and biochemical features related to specific genomic alterations. Cross-sectional imaging is mandated in the investigation of parathyroid adenomas presenting with severe hypercalcaemia as ultrasonography alone can underestimate their size and extent. This is important since it can impact on preoperative preparation and planning as well as the consent process as a thoracic approach may prove necessary for certain cases.     

A case of functioning parathyroid cyst]

We report a rare case of primary hyperparathyroidism with a functioning parathyroid cyst in a 45-year-old male. He was a recurrent stone former, and consulted our hospital for further examinations of hypercalcemia. Plasma levels of intact parathyroid hormone (PTH) were elevated to 130 pg/ml. Ultrasonography, computed tomography and magnetic resonance imaging revealed a parathyroid cyst on the right lobe of the thyroid gland. We performed right superior parathyroidectomy. Histological examination demonstrated a secondary pseudocyst resulting from cystic degeneration of a parathyroid adenoma. Plasma levels of intact PTH normalized after operation. To our knowledge, only 48 cases of functioning parathyroid cyst were reported in the Japanese literature. We discuss the clinical features and histological evidence of functioning parathyroid cyst.     

Non-Functional Parathyroid Carcinoma: A Review of the Literature and Report of a Case Requiring Extensive Surgery

Parathyroid carcinoma is a rare malignancy, and only accounts for 0.5–2% of cases of primary hyperparathyroidism. Less than 10% of parathyroid carcinomas are non-functional, and as such, they have been rarely reported in the literature. Importantly, margin status at resection is related to prognosis, and only a handful of case reports of non-functional carcinoma note this important parameter. Here we report the first case of non-functional parathyroid carcinoma with negative margins, and review the literature on this rare entity. Whether functional or non-functional, parathyroid carcinoma can often be difficult to differentiate from benign parathyroid adenoma. While diagnosis has been based on clinical and histological criteria, recent data concerning the molecular underpinnings of parathyroid carcinoma may allow for improved accuracy in distinguishing benign and malignant parathyroid tumors.     

Parathyromatosis as a cause of recurrence primary hyperparathyroidism: A case report

Introduction and importanceParathyromatosis is a rare cause of recurrent hyperparathyroidism. The main cause of this pathology is secondary implantation into the surrounding tissues of the damaged parathyroid gland (rough manipulation of the gland tissue) during the primary operation. Nowadays, parathyromatosis remain a difficult diagnostic and therapeutic task.Case presentationA 57-year-old woman 12 years ago underwent right inferior parathyroid adenomectomy. For the last 2 years, the patient began to worry about pain in large tubular bones, thoracic spine. In the biochemical analysis of the patient's blood, the serum ionized calcium level was increased - 1.56 mmol/l, parathyroid hormone - 144 pg/ml. Ultrasound scan of the neck showed the presence of two hypoechoic formations with dimensions of 24 × 12 × 6 mm and 14 × 9 × 8 mm behind the right lobe of the thyroid gland (the site of a previously operation).The patient underwent cervicotomy, removal of 3 fragments of the parathyromatosis tissue. According to a histological study, there fragments are presented by diffuse-nodular hyperplasia from dark main cells. Remission of primary hyperparathyroidism was achieved.Clinical discussionThis clinical case shows the need for differential diagnosis in recurrence primary hyperparathyroidism with parathyroid cancer, secondary hyperparathyroidism, parathyromatosis.ConclusionThe main method of treatment is the surgical removal of all foci of parathyromatosis. In the postoperative period, observation of such patients is required with laboratory and visual screening to exclude recurrence hyperparathyroidism.     

Radio-guided surgery in recurrent renal hyperparathyroidism: report of a case

BACKGROUND: It has been demonstrated that radio-guided surgery offers several advantages in treating primary hyperparathyroidism. Even if it is considered less helpful in renal hyperparathyroidism, it could be of tremendous advantage in the treatment of persistent or recurrent secondary hyperparathyroidism. METHODS: We report a case of recurrent secondary hyperparathyroidism treated by the use of radio-guided surgery. The preoperative assessment consisting of ultrasonography, magnetic resonance imaging, and 99mTc-sestamibi scintigraphy identified a parathyroid in the upper mediastinum. The patient underwent a radio-guided neck re-exploration that allowed a rapid localization and excision of the ectopic gland, which was located in the anterosuperior mediastinum, in front of the trachea, between the innominant and the left common carotid artery. RESULTS: The operative time was 45 minutes. The patient was discharged on the first postoperative day. A decrease in serum calcium and parathyroid hormone was observed subsequently. A follow-up of 6 months did not show any recurrence. CONCLUSIONS: The case reported indicates that radio-guided surgery can help surgeons detect parathyroid tissue in selected cases of renal hyperparathyroidism.     

Predictors of single-gland vs multigland parathyroid disease in primary hyperparathyroidism: a simple and accurate scoring model

HYPOTHESIS: Preoperative clinical, biochemical, and imaging studies could be used to reliably select patients with single-gland primary hyperparathyroidism who could undergo minimally invasive parathyroidectomy and to determine whether additional perioperative testing is necessary. DESIGN: Retrospective analysis. SETTING: Tertiary referral center. PATIENTS: A total of 238 patients who underwent neck surgical exploration and parathyroidectomy for primary hyperparathyroidism from January 7, 2002, to December 23, 2004. MAIN OUTCOME MEASURES: Demographic, clinical, biochemical, and imaging factors that predict single-gland vs multigland parathyroid disease, and biochemical cure. RESULTS: Of the 238 patients, 75.2% had a single adenoma, 21.4% had asymmetric 4-gland hyperplasia, and 3.4% had double adenomas. A biochemical cure was achieved in 99.2% of the patients. Preoperative calcium and intact parathyroid hormone levels were significantly higher (P = .03 and .04, respectively) and ultrasound and sestamibi scan results were more likely to be positive (both P<.001) in single-gland primary hyperparathyroidism. A dichotomous scoring model based on preoperative total calcium level (>/=3 mmol/L [>/=12 mg/dL]), intact parathyroid hormone level (>/=2 times the upper limit of normal levels), positive ultrasound and sestamibi scan results for 1 enlarged gland, and concordant ultrasound and sestamibi scan findings reliably distinguished single-gland vs multigland cases (P<.001). The positive predictive value of this scoring model to correctly predict single-gland disease was 100% for a total score of 3 or higher. CONCLUSIONS: Preoperative biochemical and imaging study results reliably distinguished single-gland vs multigland parathyroid disease in primary hyperparathyroidism. Our findings suggest that patients with a score of 3 or higher can undergo a minimally invasive parathyroidectomy without the routine use of intraoperative parathyroid hormone or additional imaging studies, and those with a score of less than 3 should have additional testing to ensure that multigland disease is not overlooked.     

Water-Clear Cell Parathyroid Adenoma Causing Primary Hyperparathyroidism in a Patient with Neurofibromatosis Type 1: Report of a Case

Water-clear cell parathyroid adenoma is an exceedingly rare tumor, composed exclusively of tumor cells with abundant foamy cytoplasm. A combination of hyperparathyroidism and neurofibromatosis type 1 (NF1) is also a rare phenomenon. We report an 18-year-old woman with primary hyperparathyroidism caused by water-clear cell parathyroid adenoma in association with NF1. She had renal stones, hypercalcemia, and an elevated plasma level of intact parathyroid hormone. Physical examination revealed a palpable tumor in the right neck, and café-au-lait spots distributed over her entire body. An ultrasound examination showed an isoechoic mass in the right thyroid lobe. Thallium–technetium subtraction scintigraphy showed high thallium accumulation in the right thyroid lobe area. A surgical exploration revealed the palpable mass to be a parathyroid tumor. The pathological features were consistent with water-clear parathyroid adenoma. This is the first reported case of water-clear cell parathyroid adenoma associated with NF1.     

Hyperparathyroidism unmasked by the treatment of hypothyroidism secondary to Hashimoto's thyroiditis

Primary hyperparathyroidism masked by untreated hypothyroidism was first noted by Kissin and Bakst in 1947; since then there has been only a single case reported. As a result, it has been thought to be a rare complex of parathyroid disease. During the past 17 years we observed 17 patients who developed primary hyperparathyroidism after their treatment for hypothyroidism (secondary to Hashimoto's thyroiditis) with thyroxine had been well underway. The patients were all women whose ages ranged between 16 and 76 years. The clinical and biochemical manifestations of hyperparathyroidism were noted 2 months to 30 years after thyroxine therapy. All patients underwent standard subtotal parathyroidectomy. Fourteen patients had a single parathyroid adenoma and three had multiglandular adenomatous hyperplasia. It is of interest that all 17 patients were found to have Hashimoto's thyroiditis, based on antimicrosomal antibody titers or histopathologic criteria. Thus we present a series of patients who developed primary hyperparathyroidism in a background of Hashimoto's thyroiditis and hypothyroidism treated with thyroxine. We are intrigued by the association of hyperparathyroidism and Hashimoto's chronic thyroiditis. From this experience it seems appropriate to evaluate parathyroid function in patients with hypothyroidism secondary to Hashimoto's thyroiditis before the initiation of treatment with thyroxine and at intervals thereafter.     

Intracranial metastatic parathyroid carcinoma: case report

OBJECTIVE AND IMPORTANCE: Parathyroid carcinoma is a rare entity, and fewer than 200 cases have been described. It is a slowly progressive disease characterized by frequent recurrences and local metastases. Most patients with parathyroid carcinoma die from metabolic complications of hyperparathyroidism. Five-year survival rates range from 25 to 50%. Functional parathyroid carcinoma is a rare cause of hyperparathyroidism that affects only 0.32 to 5% of all patients who undergo surgery for hypercalcemia. A review of the literature revealed only one other reported case of metastatic intracranial parathyroid carcinoma, in a patient who experienced local recurrence and metastatic disease when she was diagnosed with an intracranial lesion. CLINICAL PRESENTATION: We report the case of a 44-year-old African-American man with recent-onset, right lower-extremity weakness and hypercalcemia 4 years after he underwent a parathyroidectomy for parathyroid carcinoma. At presentation, his parathyroid level was 467 pg/ml, and his serum calcium level was 15.2 mg/dl. Imaging studies revealed an isolated enhancing left mesial frontoparietal mass. A systemic Cardiolite study demonstrated a single focus of radiotracer uptake in this region. No abnormal uptake was demonstrated in the neck or elsewhere. INTERVENTION: The patient underwent a frameless stereotactic interventional magnetic resonance imaging-guided resection via a parasagittal interhemispheric approach. Pathological findings were consistent with parathyroid carcinoma. After resection, his right lower-extremity weakness and secondary hyperparathyroidism resolved. CONCLUSION: The typical natural history of parathyroid carcinoma concludes with death from complications of hyperparathyroidism. This case report supports aggressive surgical management to eliminate all parathyroid hormone-secreting malignant tissue and prevent metabolic complications. In this patient, intraoperative magnetic resonance imaging was helpful to ensure complete resection.     

Spontaneous remission of primary hyperparathyroidism: a case report and meta-analysis of the literature

BACKGROUND: In a minority of patients, primary hyperparathyroidism spontaneously remits either by autoinfarction or by hemorrhage into or around the adenoma. We describe a case of autoparathyroidectomy occurring in a 63-year-old man 9 years after three parathyroid glands were removed during a total thyroidectomy. This case is compared with 50 previously reported cases of autoparathyriodectomy, and a meta-analysis is performed. METHODS: Case report, literature review, and meta-analysis were done using statistical software (SigmaStat 2.0, SPSS, Chicago). RESULTS: Fifty cases of autoparathyroidectomy were summarized according to the three etiologies. The greatest biochemical aberration was found in the acute intracapsular hemorrhage group, with [Ca(++)] falling from a mean 15.1 mg/dL to 8.9 mg/dL. The average drop in parathyroid hormone was 69% across all groups, comparing favorably to surgical resection. CONCLUSIONS: Autoparathyroidectomy is a rare but described outcome of unoperated primary hyperparathyroidism that may delay or supplant operative management.     

Functioning oxyphil cell adenomas of the parathyroid gland. A study of 15 cases

Parathyroid adenomas composed predominantly of chief cells are the most frequent cause of primary hyperparathyroidism. Until as recently as 1978, the rare oxyphil cell parathyroid adenoma was generally considered nonfunctioning. A retrospective review of 500 consecutive patients at the Massachusetts General Hospital with a diagnosis of hyperparathyroidism associated with parathyroid adenoma during the years 1979-1987 yielded 15 (3.0%) oxyphil cell adenomas. A total of 65 case reports of hyperparathyroidism associated with a diagnosis of oxyphil cell adenomas were reviewed, applying the same diagnostic criteria used in case selection for the present series. These criteria include: (a) at least 90% composition of the adenoma by oxyphil cells; (b) biopsy or excision of a second histologically normal parathyroid gland to help rule out hyperplasia; and (c) postoperative alleviation of hypercalcemia. More than 50% of the previously reported cases did not conform to these criteria. The findings in the present study further document the entity of hyperparathyroidism caused by oxyphil cell parathyroid adenomas and suggest criteria guidelines for this rare diagnosis.     

Primary hyperparathyroidism related to a parathyroid adenoma: the dramatic clinical evolution of a misdiagnosed patient and its surgical solution

Primary hyperparathyroidism is a clinical condition related to an excessive and abnormally regulated secretion of parathyroid hormone (PTH) from the parathyroid glands which is responsible for an alteration of the calcium and phosphorus metabolism. Parathyroid adenomas are the most important cause of primary hyperparathyroidism (80-85%). A case of parathyroid adenoma observed in a patient aged 47, admitted to the Emergency Medicine Department of our Hospital with a diagnosis of hypertensive crisis, cephalea, vomiting, and a clinical history of recurrent episodes of severe abdominal and renal pain, is presented. Lab data showed severe hypercalcemia and a progressive worsening of the renal function. A severe neurological involvement with stupor, derangement of mind, the arising of acute respiratory depression, lethargy compelled the colleagues to transfer him to the Intensive Care Unit; a neck ultrasonography showed a poor-echogenous area under the right thyroid inferior pole, with signs of vascularization. The suspect of a primary hyperparathyroidism related to a single adenoma of the parathyroid gland suggested a surgical treatment. A 'concise parathyroidectomy' was performed. Our surgical approach was confirmed by the comparison of the preintervention and the post-intervention iPTH values: 2080 pg/mL (normal range: 12-65 pg/mL) before excision vs 101 pg/mL after the removal. The histologycal exam reported a parathyroid adenoma with large areas with haemorrage. Three days after surgery the patient was in good general conditions. Patients affected by primary hyperparathyroidism are often misdiagnosed because their clinical conditions can create differential diagnosis problems with other diseases. However the surgical option remains the gold standard treatment.     

Hyperfunctioning intrathyroid parathyroid gland: a potential cause of failure in parathyroidectomy

BACKGROUND: The surgical success rate of parathyroidectomy is 95%. However, in rare patients, ectopic hyperfunctioning parathyroid glands may be located in the thyroid but missed on imaging studies or during surgery. The aim of the present study was to evaluate the perioperative findings in 6 patients with intrathyroid parathyroid glands treated for hyperparathyroidism in our center over a 5-year period and to review the relevant literature. METHODS: Of the 426 patients who underwent parathyroidectomy for hyperparathyroidism between 1998 and 2003 in our center, 6 (1.4%) were found to have intrathyroid glands. Their files were reviewed for clinical features, surgical approach, intraoperative findings, parathyroid gland location, and morphologic and histopathologic characteristics. RESULTS: The group included 2 men and 4 women of mean age 66 years. The diagnosis in all patients was based on an increase in serum calcium concentration (10.1-13 mg/100 mL) and parathyroid hormone level (102-320 pg/mL). The clinical symptoms were kidney stones, gastrointestinal complaints, hypertension, and osteopenia. Localization studies included ultrasonography in all patients and sestamibi in 4. When the parathyroid glands were not identified, bilateral neck and mediastinum were explored. Adenoma was diagnosed histologically in 4 patients; 2 had a double adenoma. CONCLUSION: Intrathyroid parathyroid gland is a rare condition in patients with hyperparathyroidism. In these patients, imaging may miss the pathologic gland. Despite its rarity, the possibility of an intrathyroid parathyroid should be kept in mind, and, when meticulous bilateral exploration of the neck fails to identity the hyperfunctioning gland, the surgeon should consider hemithyroidectomy.     

甲状旁腺癌诊断治疗的研究进展

甲状旁腺癌是一种罕见的恶性肿瘤,主要临床表现为甲状旁腺功能亢进和继发于高钙血症引起的各种代谢紊乱以及相应组织器官的损害.完善的实验室检查、准确的影像学定位和可靠的组织病理学特征是甲状旁腺癌诊断的重要依据.甲状旁腺癌对放疗、化疗不敏感,肿瘤及其周围受侵组织的"整块切除"是首选的治疗方式.抗PTH免疫治疗作为一种新的治疗方...     

Parathyroid gland radionuclide scanning--methods and indications

The usefulness of preoperative radionuclide scanning of the parathyroid glands in patients with primary or secondary hyperparathyroidism was long controversial because available techniques were of limited diagnostic efficacy. Technetium-99m-labeled sestamibi (99Tc-sestamibi) is a new radiopharmaceutical agent easily detected by gamma cameras. The first parathyroid imaging studies done with 99Tc-sestamibi about 10 years ago used a double-phase technique to separate thyroid and parathyroid tissue. Although promising, this method was less than ideal, particularly in multiple gland primary hyperparathyroidism and in secondary hyperparathyroidism. For several years, we have been using subtraction between two images acquired simultaneously, one with 99Tc-sestamibi, which binds to thyroid and parathyroid tissue, and the other with 123-iodine, which binds only to thyroid tissue. The remarkable efficacy of this technique in both primary and secondary hyperparathyroidism invites a reappraisal of the place of radionuclide imaging as a preoperative localization procedure done to reduce the need for repeat surgery. The usefulness of this technique in selecting candidates for unilateral surgery among patients with primary hyperparathyroidism is discussed.