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Parathyroid carcinoma: a 22-year experience.

PURPOSE: Because parathyroid carcinoma is rare, clear consensus is not available regarding the optimal management of patients with this condition. Treatment strategies generally derive from clinical and anecdotal experiences. We report our experience with this entity. METHODS: We included all patients with parathyroid carcinoma seen at The University of Texas M. D. Anderson Cancer Center since January 1, 1980. The medical records and pathology specimens were reviewed and verified in all cases. RESULTS: Since 1980, 27 patients (16 men and 11 women) registered at M. D. Anderson Cancer Center with parathyroid carcinoma and a minimum follow-up of 2 years. The age at initial diagnosis (mean +/- SD) was 46.7 +/- 15.3 years. All patients were seen with hypercalcemia (mean calcium, 13.4 +/- 1.5 mg/dL). Eighteen patients had locally invasive disease, eight had localized disease, and one had distant metastasis. Parathyroid cancer was treated with complete surgical excision with curative intent in 18 patients. In the other nine patients, who had clinical and/or radiographic evidence of soft tissue extension, the tumor was treated by comprehensive "en bloc" soft tissue resection. Of six patients who received adjuvant radiotherapy after initial surgery, only one had a local relapse. In contrast, of 20 patients who did not receive adjuvant radiotherapy, 10 had a local relapse, excluding the one patient who had distant metastases. The 5-year survival was 85%, and the 10-year survival was 77%. Five patients died of parathyroid carcinoma; all deaths were hypercalcemia related. CONCLUSIONS: Parathyroid carcinoma can be an indolent disease with morbidity and mortality related to hypercalcemia. Adjuvant radiotherapy may improve local control and limit the occurrence of local relapse. A comprehensive multidisciplinary approach with surgery, radiation therapy, and medical treatment for hypercalcemia is needed to optimize patient outcome.

Adolescent↗

Normocalcemic parathyroid carcinoma: an unusual clinical presentation.

BACKGROUND: Parathyroid carcinoma is a rare cause of primary hyperparathyroidism and may be associated with significant disease related morbidity and mortality. Preoperative diagnosis remains a challenge, which may jeopardize appropriate and successful patient treatment. CASE PRESENTATION: We report a case of parathyroid carcinoma diagnosed in a 60-year-old woman that presented with a tender nodule located at the left lower thyroid pole and had been present for several years. Ultrasound examination revealed a 2.7 x 1.6 x 2.7 cm mass within the lower left lobe of the thyroid with cystic and solid areas. Lab measurement of the intact PTH level revealed it to be three times the upper limit of normal and the serum calcium level was within normal limits. A left thyroid lobectomy and isthmusectomy was carried out. Histopathological evaluation was diagnostic for a parathyroid carcinoma. At greater than two years of follow-up, the patient has had no evidence of disease recurrence and her serum PTH and calcium levels have remained within normal. CONCLUSION: Parathyroid carcinoma is a rare endocrine tumor which must be considered in the differential diagnosis of a nodular thyroid mass. En bloc resection remains the treatment of choice for this malignancy. Disease prognosis is influenced by the extent of the initial resection, the presence of metastases, and adequate long-term follow-up.

Journal Article↗

Mediastinal parathyroid carcinoma.

OBJECTIVE: To describe a patient with a mediastinal parathyroid carcinoma and hypercalcemia as well as to compare the features of parathyroid carcinoma with those of hypercalcemia due to other causes of primary hyperparathyroidism. METHODS: We present a case report and discuss the diagnosis and management of this rare tumor. RESULTS: In a 44-year-old man, severe hypercalcemia and normal findings on neck exploration led to referral and ultimate detection of an anterior mediastinal mass. Surgical resection and pathologic examination of the mass revealed a cystic parathyroid carcinoma. Subsequently, normalization of serum calcium and parathyroid hormone levels ensued. CONCLUSION: Parathyroid carcinoma as an alternative to the diagnosis of primary hyperparathyroidism should be considered in patients with dramatically increased serum calcium and parathyroid hormone levels at the time of initial examination. The mainstay of therapy for parathyroid carcinoma is complete surgical resection. Accurate preoperative localization of parathyroid carcinoma is helpful, especially in instances of reoperation or hypercalcemic crisis.

Journal Article↗

Immunohistochemical Analysis of the Cell Cycle-Associated Antigens Ki-67 and Retinoblastoma Protein in Parathyroid Carcinomas and Adenomas.

The morphologic distinction between parathyroid carcinoma and adenoma can be a difficult diagnostic problem. We analyzed nuclear immunoreactivity for the cell cycle-associated antigen Ki-67 with monoclonal antibody (MAb) MIB-1 and for retinoblastoma (RB) protein with two polyclonal antisera in 24 parathyroid carcinomas and 35 adenomas, which were formalin fixed and paraffin embedded to determine if these antibodies could assist in distinguishing between carcinomas and adenomas. In addition, 10 cases of parathyroid hyperplasia and 5 cases of normal parathyroids were examined as control tissues. The Ki-67 labeling index was significantly higher in parathyroid carcinomas compared to adenomas (7.1 +/- 1.0% vs 2.4 +/- 0.2%, p <0.001). No patient with a parathyroid adenoma, parathyroid hyperplasia, or normal parathyroid gland had a Ki-67 labeling index >5.3%. Analysis of the primary tumors from patients with recurrent carcinomas and from those with nonrecurrent carcinomas showed a higher mean Ki-67 labeling index (7.8 +/- 1.5% vs 5.2 +/- 1.1%) in the former group. Although these differences were not statistically significant, the RB protein immunoreactivity was not useful in distinguishing between parathyroid carcinomas and adenomas in paraffin-tissue sections. These results indicate that nuclear immunoreactivity for the cell cycle-associated antigen Ki-67 may be another useful method to assist in distinguishing parathyroid carcinomas from adenomas.

Journal Article↗

Primary hyperparathyroidism secondary to parathyroid carcinoma: report of a case.

Distinguishing parathyroid carcinoma from benign hyperparathyroidism is often difficult. Clinical features most commonly associated with parathyroid carcinoma, such as palpable cervical mass, markedly higher serum calcium, high parathyroid hormone immunoassay, and evidence of bone disease may not be present. Therefore, intraoperative recognition is essential. We report a case in which the presenting symptoms, physical examination, and laboratory analysis were consistent with benign disease. During surgery, the finding of an enlarged firm gland with surrounding inflammatory reaction altered the approach to include the possibility of parathyroid carcinoma. The gland and surrounding tissue were removed, and pathologic examination led to the diagnosis of carcinoma. At 18-month follow-up, the patient was free from recurrence. Any parathyroid gland with a gray appearance, firm texture, and surrounding inflammatory reaction should be treated as carcinoma. Initial intraoperative recognition offers the best chance for cure, since local recurrences are rarely curable.

Carcinoma↗

[Parathyroid carcinoma. Therapeutic strategies derived from 20 years of experience].

BACKGROUND: Carcinoma of the parathyroid is a rare endocrine tumour which can be difficult to diagnose even for expert anatomopathologists. METHODS: A retrospective study was carried out on all the cases of parathyroid pathology observed between January 1980 and October 2000: parathyroid carcinoma was diagnosed in 17 (3.59%) out of 478 patients treated for hyperparathyroidism. We describe their clinical presentation, treatment and results obtained. The patients included 9 women and 8 men, with a male/female ratio of 1.14 and a mean age at diagnosis of 56.9 years (range 30-83). All the patients, except one, the only non-secreting case, presented hypercalcemia, and 10 patients presented serum calcium levels above 3 mmol/L. The symptoms at onset included: nephrolithiasis in 10 cases, osteoporosis in 4 (3 of which presented uremic syndromes), gastrointestinal symptoms (gastritis) in 1 case, a palpable cervical mass in 1 patient and recurrent nerve palsy in one case suffering from familial IPT. A variety of imaging techniques were used for the preoperative localisation: high-resolution ultrasonography of the neck was carried out in all 17 patients and was positive in 15 cases; scintigraphy (99mTcO4/201Tl or 99mO4/MIBI) was carried out in 16 patients and was positive in 14; CAT was positive in 6 out of 17 patients. Three patients underwent the first operation in another hospital and were referred to our department for resistance or recidive. Initial surgery was restricted to simple parathyroidectomy in 4 cases; parathyroidectomy was extended to the entire gland in 3 patients with uremic syndrome and to the ipsilateral thyroid lobe in 7 cases. Three patients underwent parathyroidectomy extended en bloc to the adjacent structures, and recurrent lymph node dissection was also performed in 2 of these patients. Lymph node involvement was never demonstrated during the first operation. The dimensions of the tumour varied from 1 to 6.7 cm; we found signs of invasion of the neighbouring structures in 3 patients. RESULTS: Parathyroid carcinoma was correctly diagnosed during the first operation in 14 cases (this diagnosis was suspected in 10 cases following intraoperative frozen session), whereas the first diagnosis was of benign disease in 3 patients. Blood levels of calcium, phosphorus and PTH returned to normal after the first operation in 13 patients. These values diminished, but did not return to normal in 2 cases. Two patients relapsed, respectively 5 and 175 months after the first operation. A total of 10 reoperations were performed in 4 patients with persistent/recurrent symptoms (from a minimum of 1 to a maximum of 4). Recidive presented characteristics of local invasiveness in one case and the persistence was supported by micro-insemination of the pre-thyroid compartment and muscles in another two cases. At reoperation, lymph node metastasis was associated with local recidive only in one case. Two patients underwent radiotherapy after surgery and one received chemotherapy. At the last check-up (October 2000), 14 patients were alive and disease-free (82.25%). Two presented slight persistent hypercalcemia (with values ranging between 2.65 and 2.80 mmol/L), but without any macroscopic localisation of disease (11.76%). Only 1 patient died (5.88%) (one year after the first operation and 7 months after the last one). Death was caused by uncontrollable hypercalcemia supported by widespread metastasis to the bones and lungs. The 5 and 10-year survival rates were calculated as 94.12%. CONCLUSIONS: In conclusion, high blood levels of calcium and PTH, a palpable mass at the neck, with recurrent nerve paralysis, aspects of local invasiveness should alert the surgeon and guide him towards surgery that includes resection of the parathyroid en bloc with the adjacent structures, although there is no proof that a more extensive surgery is correlated with a more favourable prognosis. Being the majority of recidive functional, monitoring serum calcium and PTH levels offers a useful market which precedes their macroscopic demonstration.

Adult↗

Loss of parafibromin immunoreactivity is a distinguishing feature of parathyroid carcinoma.

PURPOSE: A reliable method for diagnosing parathyroid carcinoma has remained elusive over the years, resulting in its under-recognition and suboptimal therapy. Obtaining an accurate diagnosis has become an even more pressing matter with recent evidence that germline HRPT2 gene mutations are found in patients with apparently sporadic parathyroid carcinoma. There is a high prevalence of HRPT2 gene mutations and biallelic inactivation in parathyroid carcinoma. We hypothesize that loss of parafibromin, the protein product of the HRPT2 gene, would distinguish carcinoma from benign tissue. EXPERIMENTAL DESIGN: We generated a novel antiparafibromin monoclonal antibody and performed immunostaining on 52 definite carcinoma specimens, 6 equivocal carcinoma specimens, 88 benign specimens, and 9 hyperparathyroidism-jaw tumor (HPT-JT) syndrome-related adenomas from patients with primary hyperparathyroidism from nine worldwide centers and one national database. RESULTS: We report that the loss of parafibromin nuclear immunoreactivity has 96% sensitivity [95% confidence interval (CI), 85-99%] and 99% specificity (95% CI, 92-100%) in diagnosing definite carcinoma. Inter-observer agreement for evaluation of parafibromin loss was excellent, with unweighted kappa of 0.89 (95% CI, 0.79-0.98). Two equivocal carcinomas misclassified as adenomas were highlighted by parafibromin immunostaining. One of these tumors has since recurred, satisfying criteria for a definite carcinoma. Similarly, eight of nine HPT-JT syndrome-related adenomas showed absent nuclear immunoreactivity. CONCLUSIONS: Parafibromin is a promising molecular marker for diagnosing parathyroid carcinoma. The similar loss of parafibromin immunoreactivity in HPT-JT syndrome-related adenomas suggests that this is a pivotal step in parathyroid tumorigenesis.

Antibodies, Monoclonal↗

Parathyroid carcinoma: biochemical and pathologic response to DTIC.

Parathyroid carcinoma is a rare cause of hyperparathyroidism. Cure results from successful en bloc resection. However, because of its rarity, the malignant nature may not be appreciated at the initial operative procedure and as a result, definitive resection may not be accomplished. However, even with extensive en bloc resections, local recurrences do occur and patients die of metabolic derangements associated with hypercalcemia. Thus in addition to operative intervention, palliative chemotherapy may be required to control the hypercalcemia. Radiotherapy has been unsuccessful. A single case of nonfunctioning parathyroid carcinoma responding to treatment with methotrexate, Adriamycin, cyclophosphamide, and CCNU has been reported. We report a case of recurrent functioning parathyroid carcinoma treated with dacarbazine (DTIC) in which biochemical and pathologic evidence of at least a partial response was seen. The patient, a 33-year-old woman, had undergone five previous neck explorations during a 26-month period for aggressive locally recurrent disease. Before DTIC therapy the intact parathyroid hormone (PTH) level was 1032 pg Eq/ml (normal 163 to 347 pg Eq/ml) and the serum calcium level was 16.8 mg/dl (normal 8.8 to 10.0 mg/dl). After a course of DTIC there was a marked improvement in her clinical status and biochemical parameters (intact PTH 545 pg Eq/ml; serum calcium 11.8 mg/dl). For 2 months her condition stabilized, with PTH levels between 700 and 760 pg Eq/ml and serum calcium levels between 10.2 and 16.0 mg/dl. With a slowly progressive rise in biochemical parameters a second course of DTIC was initiated and a marked drop in serum calcium levels (5.7 mg/dl) occurred, but PTH levels remained unchanged. A progressive course of septicemia, malnutrition, and disseminated intravascular clotting ultimately lead to her death 4 weeks later. At autopsy examination the tumor was confined to the neck. Grossly and microscopically there was extensive central as well as peripheral necrosis of the tumor, which was thought to be the result of the cytotoxic effect of DTIC. From this experience and because of the grim prognosis in patients with recurring parathyroid carcinoma, it may be that aggressive use of chemotherapy with DTIC early in the course of treatment should be considered.

Adult↗

p53 abnormalities in human parathyroid carcinoma.

Two cell cycle regulators have been implicated in the pathogenesis of parathyroid neoplasms: rearrangement/overexpression of the PRAD1/cyclin D1 gene in parathyroid adenomas and inactivation of the retinoblastoma tumor suppressor gene in parathyroid carcinomas. We examined parathyroid tumors for evidence of molecular genetic abnormalities in another cell cycle regulator, the p53 tumor suppressor gene. Allelic loss of the p53 gene was observed in parathyroid carcinomas from 2 of 6 genetically informative patients. Moreover, 2 of 9 patients' parathyroid carcinomas had nuclear p53 protein detectable by immunohistochemical analysis, a finding that often reflects mutational stabilization of the p53 protein. Of these two p53-immunopositive carcinomas, 1 had p53 allelic loss and 1 was genetically uninformative. In contrast, none of 20 informative parathyroid adenomas exhibited p53 allelic loss; 1 of 19 adenomas had a focal region of nuclear p53 protein staining. Single strand conformation polymorphism analysis of exons 5-9 of the p53 gene did not reveal mutations in any parathyroid neoplasm, suggesting that such mutations in parathyroid tumors may lie outside of these conserved regions. The finding of both p53 allelic loss and abnormal p53 protein expression in parathyroid carcinomas implicates p53 in the pathogenesis of a subset of these tumors.

Adenoma↗

Loss of nuclear expression of parafibromin distinguishes parathyroid carcinomas and hyperparathyroidism-jaw tumor (HPT-JT) syndrome-related adenomas from sporadic parathyroid adenomas and hyperplasias.

Parathyroid carcinoma is notoriously difficult to diagnose with confidence in borderline cases. Commonly there is a long lag time between diagnosis and clinical evidence of malignant behavior even in histopathologically straightforward lesions. There is therefore a need for a novel adjunctive marker to assist in the diagnosis of carcinoma. Parafibromin is the protein encoded by the putative tumor suppressor gene HRPT2. Mutations predicted to inactivate parafibromin were first detected in the germline of patients with hyperparathyroidism-jaw tumor (HPT-JT) syndrome. Subsequently, somatic mutations have been identified in the majority of sporadic carcinomas. We performed immunohistochemistry for parafibromin on 115 parathyroid tissues comprising 4 HPT-JT-related tumors (3 adenomas and 1 carcinoma), 11 sporadic parathyroid carcinomas, 79 sporadic adenomas, 3 multiple endocrine neoplasia 2A-related adenomas, 2 sporadic primary hyperplasias, 2 multiple endocrine neoplasia (MEN)-1-related hyperplasias, 6 secondary hyperplasias, 4 tertiary hyperplasias, and 4 normal parathyroid glands. There was complete absence of nuclear staining in 3 of 4 (75%) HPT-JT-related tumors and 8 of 11 (73%) sporadic parathyroid carcinomas and focal weak staining in 1 of 4 HPT-JT tumors and 2 of 11 sporadic parathyroid carcinomas. Only 1 parathyroid carcinoma exhibited diffuse strong nuclear expression of parafibromin. In contrast, 98 of 100 non-HPT-JT-related benign parathyroids showed diffuse strong nuclear positivity and 2 of 100 showed weak positive staining. We conclude that, in the correct clinical and pathologic context, complete absence of nuclear staining for parafibromin is diagnostic of parathyroid carcinoma or an HPT-JT-related tumor.

Biomarkers, Tumor↗

Familial parathyroid carcinoma in a child.

Familial hyperparathyroidism and parathyroid carcinoma are rare diseases. A case of parathyroid carcinoma in an 8-year-old girl whose mother had previously undergone parathyroidectomy for primary hyperparathyroidism is reported. Parathyroid carcinoma in a preadolescent child has not been described previously, and may have a familial basis.

Biopsy, Needle↗

Parathyroid carcinoma: diagnosis and clinical history.

BACKGROUND: Parathyroid carcinoma is often over-diagnosed based on histologic appearance alone. We limited the definition of the disease to patients with recurrence, metastases, or frank capsular invasion on histologic examination. METHODS: With these criteria, fourteen cases of parathyroid carcinoma seen at our institution from 1955 to the present were identified. RESULTS: All patients presented with hypercalcemia, and all deaths were due to hypercalcemia. Two patients have been free of disease after initial operation for 31 and 180 months. Six other patients had a prolonged course with a median survival more than 80 months. Two of these patients have undergone one reexcision each for local recurrence, and four have undergone multiple resections for local recurrence or metastases. These reoperations usually resulted in satisfactory, albeit temporary, control of hypercalcemia. Finally, six patients died of disease after an aggressive course, with a short median survival (47 months). Four of these patients were seen in the 1950s before an aggressive approach to metastatic disease was adopted. CONCLUSIONS: The types of clinical courses observed in this study may be more reflective of the varied biologic features of parathyroid carcinoma and the approach to recurrence than of the initial operations. For patients with recurrent or distant disease, an operation appears to prolong survival and palliate the symptoms of hypercalcemia.

Adult↗

[Sonographic diagnosis of parathyroid carcinoma].

Prognosis and survival of patients suffering from parathyroid carcinoma are completely dependent on correct surgical therapy in form of an en-bloc resection of the tumour. A correct preoperative diagnosis of this rare carcinoma is therefore absolutely mandatory in order to perform a curative operation. We demonstrate a case of parathyroid carcinoma in which scintigraphy and CT diagnostics did not produce the right diagnosis. Preoperative high-resolution ultrasound (8 MHz) in combination with power-Doppler-sonography, however, led to the right diagnosis by demonstrating the correct topography, signs of malignancy and the feeding vessels of the tumour. As it could be demonstrated that the inferior thyroid artery was displaced by the tumour and was not a tumour feeding artery, the inferior thyroid artery was an important additional landmark for making the decision between thyroid or parathyroid carcinoma. The use of power-Doppler-sonography for identification of feeding arteries associated with parathyroid carcinoma in addition to high-resolution small parts sonography is of great value for the distinction between thyroid or parathyroid tumour.

Carcinoma↗

Parathyroid carcinoma: report of three cases and review of the literature.

Parathyroid carcinoma is a rare endocrine malignancy characterized by the exaggerated metabolic effects of the parathyroid glands. The preoperative differential diagnosis between parathyroid carcinoma and primary hyperparathyroidism is often difficult because many of the signs and symptoms are very similar. Intraoperative differentiation is obscured by the strict anatomic and histologic criteria required for diagnosis of parathyroid carcinoma. We have encountered three patients with parathyroid carcinoma during the last 10 years and managed them successfully. Two of them presented with recurrence of hypercalcemia, one 11 years after and the other 3 years after the primary operation for hyperparathyroidism; both patients were eventually diagnosed with parathyroid carcinoma. The third case was suspected as primary hyperparathyroidism preoperatively but confirmed as carcinoma subsequent to histologic examination.

Adult↗

Localization and reoperation results for persistent and recurrent parathyroid carcinoma.

HYPOTHESIS: Reoperation is safe and benefits patients with persistent and recurrent parathyroid carcinoma. DESIGN: Retrospective study. The mean follow-up time was 8.1 years (median, 7 years; range, 1-23 years). SETTING: A university tertiary referral center. PATIENTS: Eighteen patients treated for parathyroid carcinoma from 1966 to 1999. RESULTS: The mean serum calcium level was 13.7 mg/dL (3.43 mmol/L), and the parathyroid hormone (PTH) level was 1.6 to 20 times the upper limit of normal. Fourteen of 18 patients had persistent or recurrent parathyroid carcinoma and underwent 54 reoperations (28 at our institution). Mean time to recurrence was 4.8 years (range, 1-20 years). Symptoms of hyperparathyroidism were relieved in 86% of patients who had reoperation (P<.05). Reoperation for parathyroid carcinoma (25 locoregional and 3 distant) significantly reduced and normalized the serum calcium and PTH levels in 75% and 62% of the cases, respectively (P<.001). The preoperative serum calcium level was a significant predictor of postreoperative normalization of the serum calcium level but not extent of initial resection, PTH level, time to recurrence, concordance of localization studies, or patient age and sex (P<.01). Surgical complications consisted of 5 unilateral and 1 bilateral permanent recurrent laryngeal nerve palsies (2 intentionally resected en bloc), 1 transient hypoparathyroidism, 1 wound seroma, and 1 tracheoesophageal fistula. The sensitivity rates of sestamibi scan (n = 14), magnetic resonance imaging (n = 15), computed tomographic scan (n = 6), ultrasound (n = 13), and selective venous catheterization with PTH measurement (n = 6) were 79%, 93%, 67%, 69%, and 83%, respectively. CONCLUSIONS: Recurrence is common in patients with parathyroid carcinoma. Patients with this disease should have frequent, lifelong follow-up to ensure early detection of recurrence. Although reoperation for persistent or recurrent parathyroid carcinoma provides significant symptomatic relief and normalizes serum calcium and PTH levels in most patients, it is associated with some morbidity. Localizing studies of parathyroid carcinoma are helpful but do not detect all tumor foci.

Adult↗

Ultrasonographic features of parathyroid carcinoma.

Although several authors have reported single cases illustrative of some ultrasonographic characteristic of parathyroid carcinoma, the value of ultrasonography for diagnosing this entity remains to be determined. The purpose of our study was to investigate the ultrasonographic features of parathyroid carcinoma in a large number of cases. We assessed the shape, contour, echogenicity, and depth-width (DW) ratio of 16 parathyroid carcinomas and 61 parathyroid adenomas. Ultrasonography showed that parathyroid carcinomas tend to be large, inhomogeneous, hypoechoic masses with lobulated contours. In contrast, parathyroid adenomas were small, homogeneous, hypoechoic masses with smooth borders. The mean (range) DW ratios for parathyroid carcinomas were 1.21 (0.91-2.5) and 0.64 (0.33-1.47) for adenomas; the difference was statistically significant (p<0.0001). The DW ratio was > or =1 in 15 (94%) of the 16 cases of carcinoma, whereas only 3 (5%) of the 61 adenomas had a similar ratio. Ultrasonographic examination is useful not only for preoperative localization but also for differentiating parathyroid carcinoma from adenoma. Parathyroid tumors with irregular margins, inhomogeneous echogenicity, and a DW ratio > or =1 are likely to be malignant.

Adenoma↗

Parathyroid carcinoma: clinical and pathologic features in 43 patients.

Parathyroid carcinoma accounts for 0.5 to 5% of all cases of hyperparathyroidism. We reviewed the clinical, surgical, and pathologic features observed in all patients with parathyroid carcinoma evaluated at the Mayo Clinic from 1920 through 1991. Forty-three patients (22 women, 21 men; mean age, 54 yrs, range 29-72) were identified, including 2 with familial hyperparathyroidism. Information on initial presentation was available in 40 patients: 15 (38%) presented with polydipsia or polyuria, 11 (27%) with myalgias or arthralgias, 7 (17%) with weight loss, and 4 (10%) with nephrolithiasis; 3 patients (7%) were asymptomatic at presentation. Of 31 patients in whom the initial neck examination was recorded, 14 (45%) had a palpable neck mass. The mean serum calcium and serum phosphorus levels were 14.6 mg/dl and 2.3 mg/dl, respectively. Parathyroid hormone levels were elevated in 21 of 21 patients (mean elevation, 10.2 times upper limit of normal). Complications included nephrolithiasis in 14 of 25 patients (56%), bone disease in 20 of 22 patients (91%) and both in 8 of 15 patients (53%). All patients underwent primary surgical resection of parathyroid carcinoma. Twenty-six of 43 patients (60%) required a second operation with 18 patients requiring multiple re-explorations. At the second operation, residual tumor was found in the neck (68%), mediastinum (16%), or both (12%). Six patients received radiation therapy to the neck (5 patients) or bones (1 patient) for recurrent or metastatic disease. Of these, 1 patient appeared cured of parathyroid carcinoma by radiation therapy 11 years after documented tumor invasion of his trachea. Repeated excision of tumor recurrences was an effective means of controlling hypercalcemia in these patients.(ABSTRACT TRUNCATED AT 250 WORDS)

Academic Medical Centers↗

Radiographic findings in recurrent parathyroid carcinoma.

Seven cases of recurrent parathyroid carcinoma were reviewed. Subperiosteal resorption was seen in 6, pulmonary nodules in 4, renal calculi in 5, brown tumors in 5, and pancreatic calcification in 1. Angiography was performed in 5 patients, showing 1 definite and 2 probable cervical recurrences, 1 mediastinal lesion, and 1 case of possible bone metastases. Venous sampling for parathyroid hormone was carried out in 4 patients and suggested residual disease in 2; in addition, there were 1 false and 1 true negative. CT was performed in 4 patients and was useful in delineating metastatic pulmonary nodules, cervical recurrence, and hepatic metastases. The radiographic approach to recurrent parathyroid carcinoma and the use of CT and angiography are discussed.

Adult↗