Recurrent melanotic neuroectodermal tumor of infancy: a proposal for treatment protocol with surgery and adjuvant chemotherapy

The case of a 4-month-old male infant treated with combined surgery and chemotherapy for an aggressive recurrent melanotic neuroectodermal tumor of infancy (MNTI) on the top of the alveolar process of the mandible with a long-term follow-up is presented. Initial treatment comprised conservative local excision and curettage of the mandible. After several local recurrences and because radical surgical excision would give gross functional and aesthetic mutilation, finally complete, long-lasting remission was achieved with adjuvant chemotherapy, according to a neuroblastoma protocol (10-year follow-up). The reason for this protocol was because molecular genetic studies of this tumor showed loss of heterozygosity of chromosome 1p and gain of chromosome 7q analogue to neuroblastomas. A combination of surgery and chemotherapy should be the preferred treatment in case of a recurrence MNTI because optimal functional and aesthetic outcome.     

Mandibular reconstruction with bone transport in a patient with osteogenesis imperfecta

Osteogenesis imperfecta (OI) was originally considered a connective tissue disorder, primarily involving type 1 collagen. It is characterized by numerous skeletal and extraskeletal defects, including bone fragility, multiple fractures, blue sclerae, hearing deficits, skin and ligament laxity, and dentinogenesis imperfecta (DI). The authors describe a 24-year-old Japanese man with OI and DI who had an ossifying fibroma of the mandible. Segmental resection was performed, and the mandible was reconstructed by distraction osteogenesis with the transport segment technique. This is the first report to describe a patient with OI undergoing mandibular reconstruction with bone transport, to the authors' knowledge.     

Common variable immunodeficiency with unusual vegetative lesions of the tongue and labial mucosa: a case report

A case of common variable immunodeficiency with unusual vegetative lesions of the tongue and lower lip in a 28-year-old man is presented. The vegetative lesions developed over the preceding 10 months and clinically were suggestive of malignancy. The biopsy specimens showed no malignancy, and a bacterial culture of the tongue detected abundant Staphylococcus aureus. Combined treatment with a corticosteroid antibiotic ointment and povidone iodine rinse produced remarkable resolution of the lesions. Laboratory examination showed markedly decreased levels of serum immunoglobulins. Intravenous gamma globulin replacement therapy resulted in good control of infection and disappearance of the lesions.     

Possible participation of isolated epicardial cell clusters in the formation of chick embryonic epicardium

The embryonic epicardium is formed by the spreading of cells derived from the extracardiac proepicardial organ over the myocardial surface after transfer to the dorsal side of the myocardium via a bridge of villous projections. Using whole-heart immunostaining for keratin, we found that the chronology and pattern of epicardial formation in the chick was basically identical to that reported previously in the quail. However, discrete epicardial islands were observed on the ventrolateral surface of the atrioventricular canal as well as in two previously reported areas. Closer examination by scanning electron microscopy demonstrated the presence of isolated, sparsely distributed epicardial cell clusters on both the dorsal and ventral surfaces of the myocardium. These cells showed a surface morphology similar to that of the epicardial cells at the advancing edge of the spreading epicardial sheet and possessed numerous well-developed filopodia, suggesting active motility. These clusters are probably seeded onto the myocardium by vesicular transport from proepicardial villi, and our findings suggest that the resulting small, localised patches of epicardial cells might accelerate, supplement and tune the epicardial formation mediated by radial spreading of the epicardial sheet in the chick embryonic heart.     

Mixed infection of Porphyromonas gingivalis and Bacteroides forsythus in a murine abscess model: involvement of gingipains in a synergistic effect

Several microorganisms including Porphyromonas gingivalis and Bacteroides forsythus have been implicated to be etiologically important agents of periodontal disease. In this study, we determined the ability of combinations of periodontopathogenic microorganisms to cause tissue destruction in a murine abscess model. Although all bacterial combinations used in this study produced larger abscesses than did monoinfection of each bacterium, the combination of P. gingivalis and B.forsythus showed a synergistic effect on abscess formation. Since these two bacteria have been frequently found together in lesions of periodontitis, these results suggest the significance of their co-infection in the progression of periodontitis. P. gingivalis produces extracellular and cell-associated cysteine proteinases (gingipains) which appear to be involved in its virulence. The rgpA rgpB double and kgp mutants induced significantly smaller abscesses than the wild type. Moreover, the rgpA rgpB kgp triple (gingipain-null) mutant hardly showed lesion formation at all with the experimental conditions used in this study, indicating that these genes encoding gingipains are important for virulence of P. gingivalis. Mixed infection of these P. gingivalis mutants with B. forsythus showed an additive effect on abscess formation, indicating that the gingipains of P. gingivalis may play an important role in the pathological synergism between P. gingivalis and B. forsythus.     

Rare incidence of three consecutive primary tumors in the maxillofacial region: retinoblastoma, leiomyosarcoma, and choriocarcinoma: case report

Multiple primary tumors occur more commonly in the region of the head and neck than elsewhere in the body. The chance of this is particularly high in patients treated for retinoblastoma, in part because of a genetic predisposition, and in part because of the possibility of irradiation treatment. However, triple tumors occur in only 0.5% of multiple tumors. A rare case of a triple (metachronous) tumor is reported: 12 years after the treatment of bilateral retinoblastoma (enucleation and irradiation), secondary leiomyosarcoma developed in the maxillofacial region, followed 5 years later by choriocarcinoma. Surgery was performed on all three types of tumor. As a result, the female patient (currently 21 years old) is now free of complaints and has married. It is extremely rare for either leiomyosarcoma or choriocarcinoma (CHC) to appear in the maxillofacial region. The long-term, systematic control of such patients is absolutely necessary, for the multiple tumors tend to develop only after a long latency period of 10 to 20 years.