Unravelling a hidden pathology of a vertebral fracture in a teenage girl.

BACKGROUND: Although the skeleton remains a common target of primary hyperparathyroidism, the classic bone disease "osteitis fibrosa cystica" is currently rare due to early diagnosis. This case represents severe classic bone manifestations of primary hyperparathyroidism due to delayed diagnosis and delayed medical attention. CASE PRESENTATION: A 19-year-old young female was symptomatically managed for chronic back pain and nonspecific bone pain in the small joints of both hands over 2 months by a general practitioner. The patient had delayed seeking for treatment for 3 months. Later, she was evaluated for tuberculosis, hematological malignancies and rheumatic disorders following a fractured T12 vertebra and underwent pedicle screw fixation. However, clinical examination and investigations, including biochemistry, imaging and histology, ruled out the above conditions. Unfortunately, serum calcium level was not performed at the initial presentation. Later, primary hyperparathyroidism was diagnosed on the basis of moderate hypercalcaemia and elevated intact PTH levels (2064 pg/ml). She had sufficient vitamin D levels and normal kidney function. Her DXA scan revealed severe secondary osteoporosis with the lowest Z score of -8 at the total lumbar spine. Ultrasonography of the thyroid revealed a hypo echoic mass in the left lower neck, and localization studies with technetium-99 m sestamibi and 4D-CT revealed a left inferior parathyroid adenoma (1.6 × 1.5 × 1.6 cm). CT scan also revealed brown tumors in the mandible and vertebrae and diffuse bony changes in the skull, sternum, humerus and vertebrae. Her radiographs revealed subperiosteal bone resorption on the radial aspects of the middle and distal phalanges and brown tumors in both the ulna and fibula. We excluded MEN and other hereditary syndromes in our patient with a personal and family history and with a normal pituitary hormone profile because of poor resources for genetic testing. She underwent parathyroid adenoma excision, and the postoperative period was complicated with hungry bone syndrome, requiring high doses of calcium and active vitamin D supplements. These supplements were gradually weaned off over 6 months, and she recovered with normal biochemical investigations. Histology revealed parathyroid adenoma without malignant features. CONCLUSION: In developing countries where routine calcium screening is not available, clinicians should be aware of various manifestations of primary hyperparathyroidism to allow diagnosis as soon as possible without delay to prevent further progression, as it is a treatable condition.

Manifestations of hyperparathyroidism in the jaws: Concepts, mechanisms, and clinical aspects.

Hyperparathyroidism is one of the most common endocrine disorders worldwide. In countries where routine biochemical screening is not common, symptomatic hyperparathyroidism predominates. Its manifestations include skeletal alterations, calcification of soft tissues, kidney stones, and functional alterations in other systems. Notably, jaw alterations can be the first clinical sign of hyperparathyroidism, including brown tumor, renal osteodystrophy, osteitis fibrosa, and leontiasis ossea, and knowing such conditions is of core importance for the multidisciplinary diagnosis and management of hyperparathyroidism. We aimed to perform a concise review, systematizing the concepts and mechanisms underlying hyperparathyroidism and associated gnathic alterations. In addition, a detailed description of the clinical aspects of the jaw manifestations is presented.

Quantitative analysis of argyrophilic nuclear organizer regions in giant cell lesions of jaws.

BACKGROUND: Giant cell lesions of the jaws are considerably similar according to histopathologic characteristics yet show different clinical behaviors. These lesions include central giant cell granuloma (CGCG), aneurysmal bone cyst, Cherubism, and Brown tumor associated with hyperparathyroidism. The present study aimed to investigate AgNORs count in these lesions as a proliferative marker and to determine whether it can be used to discriminate between them or not. METHODS: Forty-one cases of giant cell lesions of jaws were retrived from Oral Pathology Department (1987-2007). They included 21 cases of CGCG, eight cases of aneurysmal bone cyst (ABC), six cases of Cherubism, six cases of Brown tumor. The mean AgNORs count was calculated for all cases. To compare mean AgNORs in groups of lesions, ANOVA test was performed. RESULTS: Mean AgNOR counts were: (0/85 +/- 0/29) in CGCG, (0/76 +/- 0/32) in ABC (0/87 +/- 0/10) in Cherubism and (0/82 +/- 0/16) in Brown tumor. A significant difference was not observed in AgNOR counts among these groups of lesions. CONCLUSIONS: Jaws giant cell containing lesions have no acceptable differences in mean AgNORs.

[Course of Osteodystrophia fibrosa generalisata in a satin guinea pig]. / Krankheitsverlauf einer Osteodystrophia fibrosa generalisata bei einem Satinmeerschweinchen.

In a 14 months old satin guinea pig Osteodystrophia fibrosa generalisata was diagnosed by clinical and x-ray examination. The guinea pig was treated palliatively with Meloxicam and Natriumrisedronate as well as periodic dental treatment. At the age of 3 years and 10 months the guinea pig died, but necropsy was denied by the owner.

Brown tumor of the jaws as a manifestation of tertiary hyperparathyroidism: A literature review and case report.

Brown tumor of the jaws is a manifestation of hyperparathyroidism consisting of osteolytic lesions that show proliferation of multinucleated giant cells in the maxilla and/or mandible. Differential diagnosis of these lesions from local central giant-cell granuloma is mandatory for the correct treatment of the patient. Radiographic and histopathological exams of the jaw lesion are not sufficient to determine the diagnosis, which requires laboratory tests including serum levels of calcium, alkaline phosphatase, parathyroid hormone (PTH) and phosphate, and radiographic examination of other bones as well, such as hand-wrist, pelvis, and femur. We present here a brief literature review focusing on the clinical and radiographic features, diagnostic criteria and treatment of brown tumor and also report a case of the disease affecting the jaw.

Giant mediastinal parathyroid adenoma presenting as bilateral brown tumour of mandible: a rare presentation of primary hyperparathyroidism.

Hyperparathyroidism (HPT) is becoming increasingly common endocrinopathy in clinical practice. Nowadays, it is mostly diagnosed in subclinical or early clinical stage. Bony involvement in HPT has seen significant fall in incidence. Brown tumour of bone is exceptionally rare as a first manifestation of primary HPT (PHPT). Its radiological and histopathological features may be mistaken for other bony pathologies. If possibility of underlying HPT is overlooked the disease is bound to recur after surgery adding to morbidity of the patient. Here we present a case of bilateral brown tumour of mandible which was mistakenly treated as giant cell granuloma by surgical curettage. That the patient was harbouring an ectopic parathyroid adenoma with hypercalcemia causing non-specific symptoms was missed by the referring physician. This led to recurrence of the lesion. On subsequent evaluation, a giant mediastinal parathyroid adenoma causing PHPT was detected at our centre and was removed via mini sternotomy approach.

From mild to severe primary hyperparathyroidism: The Brazilian experience.

Primary hyperparathyroidism often presents as an asymptomatic disorder. In our institution, routine serum calcium measurements have now been used as part of medical examination for 23 years. Out of 124 patients consecutively seen at our institution, 47% presented with no symptoms related to the disease, while 25% presented with severe skeletal involvement and osteitis fibrosa cystica, 25% with renal stone disease without overt bone involvement, and 2% with the typical neuropsychiatric syndrome. This same pattern is seen in the city of São Paulo. In severe disease pathological fractures are frequently seen, especially in long bones of the lower extremities, and also loss of lamina dura of the teeth and salt-and-pepper appearance of the skull. Bone mineral density is extremely low in these patients but usually show remarkable recovery following surgical cure. Serum PTH and bone markers are considerable higher in severely affected patients, who also have a high rate of vitamin D deficiency, and the parathyroid lesion is easier located compared with asymptomatic patients. From pathological specimens 87% had histological confirmation of a single adenoma, 6.4% multiple gland hyperplasia and 3.8% carcinoma.

Progress of brown tumors in patients with chronic renal insufficiency undergoing dialysis.

In a group of 73 patients, affected by chronic renal insufficiency, whose skeleton was periodically checked, three cases of brown tumors were found. These cases demonstrated a variety of locations that were involved, especially the pelvis, ribs and mandible. During therapeutic treatment the brown tumors presented a different behavior; while some lesions tended toward complete sclerosis, others increased in size and in both instances new lesions appeared. Parathyroidectomy, carried out in two patients, determined a definitive sclerosis of these lesions. Brown tumors also correlate with high PTH levels and with lesions from osteitis fibrosa.

Fibrous osteodystrophy in an opossum.

A free living opossum (Didelphis marsupialis) was found to have severe fibrous osteodystrophy of the maxilla and mandibles. No significant lesions were found in the kidneys, ruling out an etiology of renal secondary hyperparathyroidism. An etiology of primary phperthyroidism or nutritional secondary hyperparathyroidism is suggested.

Brown tumor of the maxilla associated with primary hyperparathyroidism.

Brown tumors represent the terminal stage of the remodeling processes during primary or secondary hyperparathyroidism. During the last three decades primary hyperparathyroidism has been recognized much more commonly and the increase has generally been attributed to the routine determination of calcium by new automated methods and the advent of new and more objective parathyroid hormone radioimmunoassay techniques. Early diagnosis and successful treatment of the disease have made clinical evidence of bone disease uncommon. While, the mandible is the most frequently involved bone in the head and neck region, maxillary involvement is extremely rare. A case of brown tumor on the maxilla associated with primary hyperparathyroidism is reported. This patient presented multiple skeletal lesions, which are uncommonly seen nowadays. The diagnosis was suggested by the clinical history and confirmed by biochemical, radiological and histopathological determinations. Excision of a parathyroid adenoma normalized the metabolic status. Excision of the maxillary mass led both histopathological confirmation of the disease and early masticator rehabilitation.

[Von Recklinghausen osteitis fibrosa cystica. A clinical case]. / Osteite fibroso cistica di Von Recklinghausen. Caso clinico.

In Recklinghausen's disease the skeleton lesions are often the first signal of the pathology. The main clinical manifestations are represented by bony lesions which appear as multicystic lesions with loss of the hard lamina and skull malformation and asymmetry. In this disease there is a relevant osteoclastic activity which prevails over the osteoblastic one associated to the fibrous substitution of the marrow, sometimes producing micro or macro cysts. The typical alteration consists of an increase along the endosteal and trabecular surfaces in the number of osteoclasts which can be found in small reabsorption gaps. This is the cause of a cortical and trabecular reduction which can appear as interrupted. Histologic lesions consist in the replacement of bone tissue with fibrous and osteoid tissue. These bony lesions are not characteristic of the disease but to be distinguished from other pathologies such as for example Paget's disease and other forms of fibrous dysplasia (Gardner's syndrome, Leontiasis ossea). A case personally observed is described: a women, 29 years old, suffering from Recklinghausen's disease with face and skull asymmetry, condyles and glenoid cavity deformation, abnormal face reduction. Observing the planigraphy on the right side of the temporomandibular articulation, flattened glenoid cavity and condyles with irregular outlines can be noticed, aplastic coronoid cuts, altered jaw. The patient was submitted to surgery for dental extraction followed by a biopsy which showed some regressive alterations on cellular level of the bony structure.

[Brown tumor of the jaws]. / Tumore bruno dei mascellari.

Brown tumour is one of the forms in which fibrous-cystic osteitis, which represents the terminal stage of the bone remodelling processes during primary or secondary hyperparathyroidism, is manifested. For years brown tumour was regarded as a typical lesion of primary hyperparathyroidism, but cases of brown tumours in patients with hyperparathyroidism secondary to renal failure were increasingly often reported in the literature. From an epidemiological point of view, the frequency of brown tumours in patients with renal insufficiency is extremely variable, as is the bone site affected. Several bone segments can be affected at once, but the ethmoid and frontal sinus are rarely reported. Symptoms are caused by the considerable dimensions of the brown tumour and its localisation: in the jawbones it may present sometimes painful, hard and clearly palpable swellings; if large, the tumour may deform the appearance of the bone segments affected or alter the function of the masticatory apparatus. In other cases, there is a complete absence of clinical symptoms and diagnosis may be totally coincidental during the radiological examinations. In histological terms, brown tumours are made up by a cell population consisting of rounded or spindle-like mononucleate elements, mixed with a certain number of plurinucleate giant cells, resembling osteoclastic cells, among which recent haemorrhagic infiltrates and hemosiderin deposits (hence the brown colour) are often found. The aim of this study was to report three cases from a population of 107 patients undergoing haemodialysis at the Turin University Centre. In conclusion, the localisation of maxillary brown tumours appears to prefer a young, female population; brown tumours are rarely an early sign of hyperparathyroidism in haemodialysis patients, but they often appear in conditions of advanced hyperparathyroidism which have escaped medical control either owing to unsuitable therapy or scant patient compliance; they are rapidly evolving lesions whose regression may be very slow or not occur even after total parathyroidectomy; the severity of the lesion caused by a brown tumour may lead to evident osteolysis in the maxillofacial district, thus suggesting the need for early and regular radiological screening; in the event of lesions which are already present, from the authors' point of view, the choice of treatment must be oriented towards parathyroidectomy.

"An exophytic mandibular brown tumor": an unusual presentation of primary hyperparathyroidism.

CASE REPORT: A case of 35-year-old male patient with previously undiagnosed primary hyperparathyroidism who presented with an atypical exophytic mandibular swelling is reported. PURPOSE: The aim is to alert the clinicians to include this entity although extremely rare, in the differential diagnosis of swellings in the maxillofacial region and to highlight another remarkable aspect in the multitude of presentations associated with primary hyperparathyroidism especially in the setting of normocalcemia.