1/18. Epignathus, double pituitary and agenesis of corpus callosum.Two infants from unrelated families died on the 1st day of life with epignathus, duplication of the entire pituitary, infundibulum and sella, and widening or separation of midline structures of the head including absent corpus callosum. We suggest that some infants surviving surgery for large epignathi may have relatively symptomless absent corpus callosum or double pituitary.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
2/18. A case of Burkitt's lymphoma that presented initially with resorption of alveolar bone.A 16-year-old male was evaluated for a 1-month history of alveolar bone resorption, which had been treated with endodontics by a neighborhood dentist. Intraoral examination showed slight gingival swelling and teeth mobility. However, no tumor mass was seen. The panoramic image showed resorption of alveolar bone and loss of teeth lamina dura. Because he complained of general fatigue, he was introduced to the internist. Biopsies of gingiva and bone marrow aspiration revealed a massive proliferation of lymphoblasts expressing CD10, 19, 20 and hla-dr antigens on the surface. Their karyotypes were abnormal; 46, XY, t (8;14) (q24;q32). Accordingly, he was diagnosed as Burkitt's lymphoma, and received intensive chemotherapy which relieved his symptoms and decreased his tumor. However, his disease soon became refractory to chemotherapy, and he died 11 weeks after the onset.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
3/18. Gardner's syndrome--the importance of early diagnosis: a case report and a review.Gardner's syndrome (familial polyposis coli) is a genetic condition characterised by colonic polyps that carry a 100% risk of malignancy if untreated. early diagnosis, in which an astute and knowledgeable dentist can play an essential role, is therefore of paramount importance. The presence of multiple unerupted teeth provides the dentist with a major pointer to the possible presence of this disease, which may be provisionally diagnosed on the detection of two other obvious features, namely osteomas and cutaneous lesions. This fact is clearly illustrated by the accompanying case report. Extracolonic manifestations increase the morbidity and make treatment more difficult. The presence of large desmoid tumours may be a serious complicating factor. Gardner's syndrome may be accompanied by adenomas of the stomach and duodenum and very rarely by malignant tumours of the central nervous system. Regular surveillance by means of colonoscopies must be carried out on all individuals suspected of having the disease in order to implement timeous life-saving prophylaxis.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
4/18. cherubism and its charlatans.cherubism is a rare hereditary condition characterised by progressive cystic proliferation of the mandible and maxilla in childhood, followed by post-pubertal involution of the process and jaw remodelling in adulthood. Its name is derived from the cherubic appearance that results from the jaw hypertrophy. Here, we present the case of a young boy with cherubism, in the context of his pedigree, to illustrate the clinical characteristics and their variable expression. We also seek to distinguish cherubism from central giant cell granuloma and giant cell tumour of the jaws, with which it holds a false synonymity.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
5/18. Malignant ameloblastoma, spindle cell variant.In this report, we document the histologic and clinical features of a previously undefined spindle cell variant of ameloblastoma that eventually behaved in a malignant fashion during a protracted course. The predominant histologic pattern was a well-differentiated, cellular, spindled epithelial proliferation arising in the maxilla of a 14-year-old African American girl. Over 19 years, the patient experienced numerous local recurrences, metastases to distant bones after 15 years, and finally bulky local recurrence with intracranial extension resulting in death. This ameloblastic malignancy histologically simulates a low-grade true sarcoma or an ameloblastic sarcoma, but differs in that the extensive spindle cell proliferation is epithelial, characterized by strong cytokeratin immunoreactivity and negative vimentin staining.- - - - - - - - - - ranking = 2keywords = life (Clic here for more details about this article) |
6/18. Desmoplastic fibroma-like tumor of maxillofacial region associated with tuberous sclerosis.Desmoplastic fibroma is a rare primary tumor of bone that histologically and biologically mimics the extra-abdominal desmoid tumor of soft tissue. It usually presents in patients during the first three decades of life and often involves the mandible or long bones of the skeleton. Its clinical behavior is characterized by a locally aggressive, infiltrating, and destructing course, often with invasion of surrounding tissues but without metastasis. We present herein the clinicopathological features of a desmoplastic fibroma-like tumor involving the left maxillofacial region in a 14-year-old Hispanic boy with tuberous sclerosis.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
7/18. Involvement of the jaw bones in systemic mastocytosis.Systemic mastocytosis is characterised by proliferation of mast cells and infiltration of organs. Severe bony pain may result from release of chemical mediators from mast cells and affected patients are at an increased risk of anaphylaxis. Traditional analgesics such as non-steroidal anti-inflammatory agents and opioids are contraindicated. Diagnosis is based on presentation, biopsy of bone marrow, and magnetic resonance imaging of the affected area. In the head and neck, the disease may present as facial pain, localised osteomyelitis, oral sinus formation, and oral ulceration. Treatment is with histamine antagonists and bisphosphonates to control symptoms. An adrenaline pen is provided for use in case of anaphylaxis.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
8/18. Hereditary familial polyposis and Gardner's syndrome: contribution of the odonto-stomatology examination in its diagnosis and a case description.Familial Adenomatous Polyposis (FAP) and its phenotype variant, Gardner's syndrome, constitute a rare autosomal dominant inherited disorder. They are characterised by the development, generally during the second and third decades of life, of multiple adenomatous polyps in the colon and rectum. These polyps have a high risk of subsequently becoming malignant, which normally occurs in the third and fourth decades of life. The phenotypical features of FAP can be very variable. As well as colorectal polyps, these individuals can present with extra-colonic symptoms, among which are particularly: gastro-duodenal polyps, dermoid and epidermoid cysts, desmoid tumours, congenital hypertrophy of the retinal pigment epithelium, disorders of the maxillary and skeletal bones and dental anomalies. In this paper the most important aspects of this syndrome are reviewed, showing an example based on a well documented clinical case. The importance of odonto-stomatological examinations should be pointed out, among others, as a means of reaching a presumptive diagnosis, whose confirmation is vital to the patient.- - - - - - - - - - ranking = 2keywords = life (Clic here for more details about this article) |
9/18. Cytologic findings in calcifying epithelial odontogenic tumor: a case report.BACKGROUND: Calcifying epithelial odontogenic tumor (CEOT), or Pindborg's tumor, is a rare, benign, odontogenic neoplasm first described by Pindborg in 1955. It is most commonly seen in the fourth and fifth decades of life, usually arises in the mandibular premolar-molar areas and accounts for approximately 1% of all intraosseous odontogenic tumors. This report describes the cytologic findings in a case of CEOT. CASE: A 62-year-old woman was referred to the ear, nose and throat clinic with a right maxillary mass. The fine needle aspiration (FNA) smears showed numerous calcifications; amorphous, eosinophilic material; and clusters of round epithelial cells embedded in a bloody background. The smears were diagnosed as suspicious for malignancy. Maxillectomy was done. The histologic sections were diagnosed as CEOT. CONCLUSION: FNA findings of calcifying epithelial odontogenic tumor have been described rarely. The clusters of epithelial cells with prominent nucleoli are mistaken for features of a malignant tumor.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
10/18. osteoblastoma of the maxilla and mandible: a report of 24 cases, review of the literature, and discussion of its relationship to osteoid osteoma of the jaws.osteoblastoma is a benign neoplasm of bone characterized by a proliferation of osteoblasts forming bone trabeculae set in a vascularized fibrous connective tissue stroma. We report 24 examples of this neoplasm arising in the maxilla and mandible and compare the clinical and radiographic characteristics of this neoplasm to 53 previously reported examples of osteoblastoma and osteoid osteoma in the jaws. Our results reveal more females reported in the new examples of osteoblastoma than in the previously reported examples of osteoblastoma and osteoid osteoma. This raises the overall female percentage from 47.2% to 58.4%. In addition, significantly fewer patients reported pain, tenderness, and discomfort associated with their neoplasms than in previously reported cases. When all cases are combined, a predominant trend is observed whereby osteoblastoma occurs predominantly on the left side of the posterior mandible and is associated with pain, tenderness, and discomfort. We also provide a rationale for use of the term "osteoblastoma" for any benign osteoblastic neoplasm arising in the jaws.- - - - - - - - - - ranking = 1keywords = life (Clic here for more details about this article) |
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