Intralesional injection of triamcinolone hexacetonide as an alternative treatment for central giant-cell granuloma in 21 cases

Central giant-cell granulomas are benign, but occasionally aggressive, lesions that traditionally have been treated surgically. 21 cases of central giant-cell granuloma of the jaw were treated with intralesional injection of corticosteroids. The treatment protocol adopted was intralesional injection of 20 mg/ml triamcinolone hexacetonide diluted in an anaesthetic solution of 2% lidocaine/epinephrine 1:200,000 in the proportion 1:1; 1.0 ml of the solution was infiltrated for every 1 cm³ of radiolucid area of the lesion, totalling 6 biweekly applications. Ten patients had aggressive lesions and 11 nonaggressive. Two patients showed a negative response to the treatment and underwent surgical resection, 4 showed a moderate response and 15 a good response. 8 of the 19 who had a moderate-to-good response to the drug treatment underwent osteoplasty to reestablish facial aesthetics. In these cases, only mature or dysplastic bone was observed, with the presence or absence of rare giant multinucleated cells. The advantages of this therapy are its less-invasive nature, the probable lower cost to the patient, lower risk and the ability to treat the lesion surgically in the future, if necessary.     

Medical management of polymyalgia rheumatica

Importance of the field: Polymyalgia rheumatica (PMR) is a relatively frequent condition in individuals older than 50 who originate from Western countries. Corticosteroids constitute the cornerstone therapy in the management of patients with PMR.

Areas covered in this review: This review summarizes the current literature on clinical clues for the diagnosis of PMR, conditions mimicking PMR, relapses in the setting of PMR and the main therapeutic strategies.

What the reader will gain: With this information, the reader receives an overview on the current available data on clinical diagnosis and treatment options in PMR.

Take-home messages: An initial dose of prednisone of 10 – 20 mg/day yields clinical improvement in the majority of patients with PMR. This is generally achieved within 7 days of the onset of this therapy. Conditions different from isolated PMR should be considered in atypical cases or when a good response to 20 mg/day of prednisone is not achieved. Relapses of PMR are not uncommon when the dose of prednisone is equal to or below than 5 mg/day. Methotrexate is the most commonly used corticosteroid sparing agent. Osteoporosis prophylaxis is also recommended.     

Tongue necrosis in temporal arteritis provoked by ergotamine.

A case of tongue necrosis in a patient with temporal arteritis who was taking ergotamine is described, and the role of ergotamine tartrate in provoking the tongue necrosis is considered. The literature on this unusual complication is critically reviewed, and the value of a carotid angiography in assessing the tongue ischaemia is exemplified.     

Giant-cell chondrodysplasia in a male infant with clinical and radiological findings resembling the Piepkorn type of lethal osteochondrodysplasia

We report on a patient whose clinical, radiologic, and histopathologic findings are compatible with the Piepkorn type of lethal short-limb osteochondrodysplasia, but who also showed multinucleated giant chondrocytes in cartilage. Multinucleated giant cells are an unusual finding in osteochondrodysplasias, having been reported in atelosteogenesis type I and boomerang dysplasia. This uncommon histopathologic finding and the clinical and radiographic findings strongly support the diagnosis of boomerang dysplasia in the present patient. Our patient supports the previously suggested existence of an entity including atelosteogenesis and boomerang dysplasia. If this is so, the current patient and that described by Piepkorn et al. [1977: Teratology 16:345–350] could represent the most severe clinical expression of that condition. Am. J. Med. Genet. 68:342–346, 1997. © 1997 Wiley-Liss, Inc.     

Early predictors of clinical and mental outcome in tuberous sclerosis complex: A prospective study

Aim

We aimed to identify early predictors of intractable epilepsy, intellectual disability (ID) and autism spectrum disorders (ASD) in the cohort of TSC patients initially diagnosed with cardiac rhabdomyomas (CR).

Everolimus: an mTOR inhibitor for the treatment of tuberous sclerosis

Tuberous sclerosis complex (TSC) is a devastating disease affecting virtually all organ systems of the body and is characterized by multiple hamartomas and neurodevelopmental disorders. The majority of patients with TSC have mutations in TSC1 or TSC2, resulting in constitutive activation of mTOR. Because the pathogenesis of the disease is mTOR hyperactivity, mTOR inhibitors have the potential to treat the underlying cause in TSC patients. Everolimus is the first mTOR inhibitor approved in the USA for the treatment of patients with subependymal giant-cell astrocytomas (SEGAs) associated with TSC. Evidence supports and ongoing studies are evaluating the role of mTOR inhibitors in the treatment of a wide spectrum of disease manifestations, including reduction in tumor volume (SEGAs, renal angiomyolipoma) and improvement in epilepsy, lung function and skin manifestations, including facial angiofibromas. In time, the use of mTOR inhibitors in patients with TSC will likely be very well established.     

Giant-cell arteritis of the female genital tract

Abstract. A 56-year-old woman with long-lasting fever of unknown origin was diagnosed as having a giant-cell arteritis of the genital tract with no evidence of temporal arteritis. Diagnosis relied on pathological examination, which showed a segmental panarteritis of ovaries, myometrium, endometrium and uterus cervix. Corticosteroid therapy led to clinical cure within a few weeks. Twenty-five cases of giant-cell arteritis of the female genital tract have been published of which only four were associated with temporal arteritis. We recommend that such a diagnosis should be considered in women presenting with long-lasting fever of unknown origin, even in the absence of temporal arteritis and the clinical evidence of genital abnormalities.     

Fine-needle aspiration cytology of osteoclastic giant-cell tumor of the pancreas.

Osteoclastic giant-cell tumor (OGCT) of the pancreas is a rare tumor. We present the fine-needle aspiration (FNA) and bile cytology findings of an OGCT arising in the head of the pancreas in a 72-yr-old male, along with immunocytochemical studies that were done on the cytologic material. The smears showed numerous giant cells with clustered, overlapping, uniform, bland-appearing nuclei with prominent nucleoli consistent with osteoclastic-type multinucleated giant cells. A second population of mononucleated cells appearing singly or in groups having similar nuclear features was also present. Immunocytochemical studies performed on the FNA and bile duct fluid material demonstrated positive staining of the malignant cells for vimentin, alpha-1 antichymotrypsin, and alpha-1 antitrypsin and negative staining for high- and low-molecular-weight cytokeratin, pooled monoclonal cytokeratin, epithelial membrane antigen, and carcinoembryonic antigen. Although not definitive, these studies are supportive of a mesenchymal-stromal histogenesis of this unusual pancreatic malignancy.     

Pulmonary metastasis from a benign giant-cell tumor of the hand: Report of a case diagnosed by fine-needle aspiration cytology

We report on the fine-needle aspiration cytologic findings of a metastatic giant-cell tumor of the right long digit to the lungs. The patient had undergone curettage of his giant-cell tumor of the finger twice 2 yr before his last admission. The lesion recurred 9 mo later, and a Ray resection of the right long digit was performed. Recently, a routine follow-up chest X-ray disclosed pulmonary metastasis. The aspirate from the lung yielded a highly cellular double-cell population, composed of mononuclear stromal cells and an osteoclast-like giant-cell population. The rarity of this tumor and its metastatic potential can cause considerable difficulty in diagnosing this entity. Clinical data, radiologic findings, and cytologic features are important to reach the correct diagnosis. Fine-needle aspiration cytology is a reliable, relatively noninvasive, and cost-effective diagnostic tool in the diagnosis of visceral metastasis in giant-cell tumors. Diagn Cytopathol 1996;15:157–160. © 1996 Wiley-Liss, Inc.