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+A 45-year-old woman presents to her general practitioner because of left-sided neck and shoulder pain.
+The pain was mild and non-specific and the patient's history was unremarkable.
+She denied a history of smoking, excessive alcohol use and radiation exposure.
+Physical examination demonstrated mild left sided cervical lymphadenopathy.
+Routine blood tests revealed borderline elevated calcium of 10.5 mg/dL and subsequent work up exhibited a markedly elevated parathyroid hormone level of 286 pg/mL, so the patient was referred to otolaryngology for further evaluation.
+Thyroid ultrasound showed a hypoechoic nodule in the left lower lobe of the thyroid.
+CT scan of the neck revealed a 2 cm hypodense nodule on the posterior inferior aspect of the left lower thyroid and sestamibi scan showed increased uptake along the lower pole of the left thyroid lobe.
+Elevated PTH along with an aberrant parathyroid gland on imaging suggests primary hyperparathyroidism.
+Historically, hyperparathyroidism is associated with bone disease, renal stones and neuromuscular dysfunction, however, with the current screening modalities, most patients are caught early and often asymptomatic.3 Primary hyperparathyroidism is most commonly caused by a parathyroid adenoma.
+Infrequent causes include parathyroid hyperplasia, which would affect all four glands and rarely caused by parathyroid carcinoma.
+Markedly elevated serum PTH and calcium levels leading to severe renal and bone manifestations are helpful in the diagnosis of cancer, however, it is usually discovered operatively based on local invasion and metastases.3
+The primary indication for parathyroidectomy, historically, is for symptomatic patients.
+Currently, since most patients are caught earlier, there are newer indications for surgery.
+These include an asymptomatic patient with any of the following: glomerular filtration rate <60 mL/min, bone density T-score <−2.5 at any site and/or previous fractures, age <50, and serum calcium 1.0 ng/dL above the upper limit of normal.3 This patient presented with pain, markedly elevated PTH, and was younger than 50 years old, so it was decided that surgery was the best choice in management.
+The patient was brought to the operating room and underwent a parathyroidectomy.
+The surgeon found the parathyroid gland to be firm and densely adherent to the thyroid capsule and overlying strap muscle, making it difficult to dissect.
+There was no visible invasion into the capsule, surrounding muscle or regional lymph nodes.
+The resected specimen was noted to be brownish grey in colour with scattered necrotic foci, and irregular texture.
+On postoperative pathology, the specimen was confirmed positive for parathyroid carcinoma with capsular invasion, focal tumour necrosis, reactive fibrosis and local skeletal muscle invasion (figures 1 and ​2).
+Margins were resected.
+Immunohistochemical stains showed increased Ki-67 reactivity as well as strong Bcl-1 (cyclin D1) reactivity, which support the diagnosis of parathyroid carcinoma (figure 3).
+Additionally, P57 staining was negative.
+Although most reports of parathyroid carcinomas are associated with marked hypercalcaemia, non-functioning cancers in patients tend to behave more aggressively.1 Owing to the severity and uncertainty of the lesion, the patient was followed up postoperatively for repeat imaging to determine if residual tumour remained.
+Repeat sestamibi and positron emission tomography (PET) scan revealed residual activity along the surgical area so the patient subsequently underwent a second operation for a radical left neck dissection and left hemithyroidectomy.
+The patient recovered very well postoperatively and it has now been 3 years since the second surgery.
+The patient continues to do very well and is followed up every 3 months to monitor serum PTH and calcium levels.
+She no longer reports of neck and shoulder pain.
+Sometimes the patient's blood tests show marginally elevated PTH levels, however, she remains eucalcaemic and follow-up nuclear scans and PET scans continue to be negative.
+The patient will continue to be monitored every 3 months indefinitely.