DefinePK

Abstract

Case Report


R.G. aged 14, female of Afghan ethnicity presently settled in the remote areas of Baluchistan, reported with a right sided maxillofacial deformity. She could barely walk because of another deformity that involved the right knee joint (Figures 1 and 2).The two lesions came into observation about nine months back as small swellings and both progressed in dimensions ever since. Maxillary growth extended into the hard palate disfiguring the anatomy of upper denture line from lateral incisor to the last molar. Plain facial radiograph corresponded to the clinical findings. CT scan confirmed that besides maxilla a little portion of the zygoma was implicated, but the orbit was spared by the growth. Biopsy done under local anaesthesia established the histopathology to be compatible with a giant cell tumour. The radiograph of the knee lesion showed involvement of proximal tibia with exuberant callus formation and this lesion was also diagnosed as giant cell tumour on histopathology. Other investigations showed low haemoglobin (9.0 Gms) with anisocytosis.Serum calcium, uric acid, phosphate, creatinine were within normal parameters. Alkaline phosphatase was 1260mg, (normal 800-1200mg).Serum Parathyroid hormones level was 678pg/ml (normal 7-53pg/ml).Repeat serum calcium showed it to be 7mg. An ultrasound of the parathyroid gland showed a hypo echoic mass measuring 1.8 by 1.1 cms posterior to the lower pole of the left lobe of the thyroid gland. It exhibited vascularity on colour Doppler study and represented a possible parathyroid adenoma. A dual phase Tc-99 Sestamibi parathyroid scan also picked up a focal area of abnormal tracer deposition in the left lower aspect indicative of a probable left lower parathyroid adenoma. Surgical exploration of the neck identified the enlarged parathyroid gland behind the left lower pole of thyroid. The parathyroid mass was removed in one piece (Figure 3). Histopathology report read "diffuse population of parathyroid tissue mainly composed of the chief cells with sinusoids in between. Focally a nodular pattern with fibro-collagenous trabeculae was identified. The features [(0)] Figure 1. Giant Cell involving maxilla. [(1)] Figure 2. Giant cell tumour involving tibia. [(2)] Figure 3. Parathyroid adenoma- excised specimen. [(3)] Figure 4. Facial asthetics- Postoperative. are compatible with a parathyroid adenoma". Parathyroid hormone levels dropped to 119pg/ml when checked after one week. Serum Calcium levels also registered a drop to 6mgs when parenteral calcium and vitamin D supplements restored it. Alkaline phosphatase was 1190 mg after two weeks.Two weeks later total maxillectomy was scheduled to relieve the patient off the social embarrassment that she had been facing due to facial deformity. En bloc removal of the lesion was done by a modified Weber-Ferguson incision.The tumour extension involved the greater portion of maxilla including the teeth, some part of zygoma, pterygoid plates, and soft tissue of the overlying cheek. A temporary obturator was fashioned to plug the defect.The histopathology report read" there is abundance of multi-nucleate giant cells with background of uniformly oval mononucleated stromal cells that have indistinct cell membranes. The diagnosis is maxillary giant cell tumour". Her facial aesthetics normalized once the final obturator was fitted (Figure 4). Two weeks later she suffered a pathological fracture in the lesion on tibia. An orthopaedic assessment was made. It was found that her fracture had healed with a 40% angulation at upper tibial metaphysis.. Anterior wedge osteotomy of the right proximal tibia was done followed by Ilizarov external fixator application. Daily physical therapy and ambulation using parallel bars was started. In the 4 weeks follow-up the patient had satisfactory progress. Her latest serum calcium was 10.9 Gms while the parathyroid hormone has returned to the normal range.