Infrequent clinicopathological findings in 108 ameloblastomas

One hundred and eight ameloblastomas diagnosed in a rural black Africa population were analysed for clinicopathologic findings other than those classically described. One patient had a polycystic ameloblastoma adjacent to an ameloblastic fibroma. Two other polycystic ameloblastomas showed aneurysmal bone cyst formation and one mandibular tumour was diagnosed as a keratoameloblastoma. Microscopic changes resembling an adenomatoid odontogenic tumour were present in association with two unicystic ameloblastomas and a HPV18-positive verrucous lesion occurred in the lining of a cystic space of a polycystic ameloblastoma. Two ameloblastomas contained eosinophilic granules in all tumor cells and melanocytes were diffusely present in another. One case exhibited a focus of mucous cell metaplasia. Two polycystic ameloblastomas showed diffuse interstitial ossification. One mandibular tumor was diagnosed as a desmoplastic ameloblastoma and another as an odontoameloblastoma. This study demonstrated that although ameloblastomas are regarded as a fairly homogeneous group of neoplasms, detailed investigations prove clinicopathologic diversity in a significant number of lesions.     

Impaction bone grafting and a cemented cup after acetabular fracture

Purpose

Patients suffering from post traumatic osteoarthritis of the acetabulum often require a total hip arthroplasty at a relatively young age. Long-term data outcome studies for this population are lacking. We report on the long-term outcome of 20 acetabular fractures in 20 patients treated with impaction bone grafting and a cemented cup after a mean follow-up of 18 years (range, 12–26 years).

Methods

The group consisted of 14 males (70 %) and six females (30 %) with an average age of 53.3 years (range, 35–75 years) at time of surgery. No patients were lost to follow-up. Four patients died and three patients underwent a revision; at review 13 patients were still living with their implant in situ. Survivorship analysis was performed at 20 years follow-up for three endpoints.

Results

Survival rate with endpoint revision for any reason at 20 years postoperative was 74.7 % (95 % confidence interval (CI), 40–91 %), 80.0 % (95 % CI, 41–95 %) for endpoint aseptic loosening, and 63.9 % (95 % CI 32–84 %) for endpoint radiographic failure. Three acetabular components were revised at 14.5, 15.3, and 16.7 years postoperative. Two cups failed for aseptic loosening and one cup failed due to septic loosening. The average postoperative Harris hip score was 82 (range, 56–100).

Conclusion

Acetabular reconstruction with impaction bone grafting and the use of a cemented cup after acetabular fracture is an attractive technique with acceptable long-term results and a low complication and re-operation rate.     

gp100/pmel 17 Is a Murine Tumor Rejection Antigen: Induction of “Self”-reactive,Tumoricidal T Cells Using High-affinity,Altered Peptide Ligand

Many tumor-associated antigens are nonmutated, poorly immunogenic tissue differentiation antigens. Their weak immunogenicity may be due to “self”-tolerance. To induce autoreactive T cells, we studied immune responses to gp100/pmel 17, an antigen naturally expressed by both normal melanocytes and melanoma cells. Although a recombinant vaccinia virus (rVV) encoding the mouse homologue of gp100 was nonimmunogenic, immunization of normal C57BL/6 mice with the rVV encoding the human gp100 elicited a specific CD8⁺ T cell response. These lymphocytes were cross-reactive with mgp100 in vitro and treated established B16 melanoma upon adoptive transfer. To understand the mechanism of the greater immunogenicity of the human version of gp100, we characterized a 9-amino acid (AA) epitope, restricted by H-2D^b, that was recognized by the T cells. The ability to induce specific T cells with human but not mouse gp100 resulted from differences within the major histocompatibility complex (MHC) class I–restricted epitope and not from differences elsewhere in the molecule, as was evidenced by experiments in which mice were immunized with rVV containing minigenes encoding these epitopes. Although the human (hgp100_25–33) and mouse (mgp100_25–33) epitopes were homologous, differences in the three NH₂-terminal AAs resulted in a 2-log increase in the ability of the human peptide to stabilize “empty” D^b on RMA-S cells and a 3-log increase in its ability to trigger interferon γ release by T cells. Thus, the fortuitous existence of a peptide homologue with significantly greater avidity for MHC class I resulted in the generation of self-reactive T cells. High-affinity, altered peptide ligands might be useful in the rational design of recombinant and synthetic vaccines that target tissue differentiation antigens expressed by tumors.     

An algorithm for the classification of mRNA patterns in eosinophilic esophagitis: Integration of machine learning

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Bloom syndrome does not always present with sun-sensitive facial erythema

Bloom syndrome is an autosomal recessive condition characterized by severe pre- and postnatal growth deficiency, immunodeficiency, an increased risk for malignancies, craniofacial dysmorphisms, and “typical” erythematous sun-sensitive skin lesions of the face. This facial rash has a butterfly-shaped distribution around the nose and is usually observed for the first time during the early years of life. Though reported as being a main feature of Bloom syndrome, there seems to be phenotypic variability regarding this facial skin rash among patients. It has been previously reported that in some individuals with Bloom syndrome these sun-sensitive lesions are less prominent or even absent. In this report we describe a 36 year old woman with short stature, microcephaly, several dysmorphisms, congenital hypothyroidism and premature ovarian failure. She was diagnosed with nasopharyngeal carcinoma at 36 years of age, only a few months after her consultation at the department of Clinical Genetics. Whole Exome Sequencing demonstrated that she had Bloom syndrome caused by a compound heterozygous mutation in BLM (c.2207_2212delinsTAGATTC; p.(Tyr736Leufs*5) and c.3681del; p.(Lys1227Asnfs*52)). She did not have facial sun-sensitive erythematous rash during childhood nor adulthood. We conclude that Bloom syndrome does not always present with erythematous sun-sensitive skin lesions of the face. We would like to underline that phenotypic variation regarding this “hallmark” feature of Bloom syndrome exists. Being aware of this might prevent a delay in diagnosing this rare short-stature syndrome and, subsequently, its potential clinical implications.