Leiomyosarcomas of the oral cavity: report of a radiation-associated and a metastatic case

Background

Leiomyosarcoma is rare in the oral cavity, where it may arise as primary, radiation-associated, or metastatic tumor. This article reports two cases of oral leiomyosarcoma, discussing the range of clinicopathological features and the significance of these presentations.

Case report

One case is a radiation-associated leiomyosarcoma arising in the tongue of a 71-year-old male occurring 22?years after radiation therapy for tonsil squamous cell carcinoma that was surgically treated. The other one is a mandible metastasis from a retroperitoneal widespread leiomyosarcoma in a 69-year-old man, who was treated by surgery and chemotherapy but died from the disease.

Discussion

Post-radiotherapy sarcomas of the oral cavity and oral metastasis from soft tissue sarcomas are very uncommon, but based on patient??s clinical history, they should be considered by oral health care providers in order to allow an early diagnosis and proper and timely management. Finally, to the best of our knowledge, this seems to be the first reported case of tongue leiomyosarcoma arising in a previously irradiated field.     

Positive effects of short course androgen therapy on the neurodevelopmental outcome in boys with 47,XXY syndrome at 36 and 72 months of age

The effects of early androgen treatment on neurodevelopmental performance in pre‐pubertal boys with 47,XXY have not been well investigated. The influence of hormones on brain development in humans suggests that a positive effect on neurodevelopmental outcome in young boys with XXY may be plausible with hormone replacement therapy. The aim of the study was to investigate retrospectively if an early course of androgen treatment (three injections of testosterone enanthate, 25 mg, each) had an impact on specific domains of neurodevelopmental function in boys with 47,XXY at 36 and 72 months of age. One hundred one boys with a karyotype of 47,XXY had neurodevelopmental assessments. The retrospective chart review resulted in one group (n = 34) who had received androgen treatment during infancy and the second group was untreated (N = 67). Statistical analysis was completed to determine if there was a positive effect from treatment observed at 36 and at 72 months on multiple domains of development. There were significant differences in multiple cognitive domains in the group who received androgen treatment, including multiple measures of language, intellectual, and neuromotor skills. Improved function was observed in neurodevelopmental outcome in boys with 47,XXY at 36 and 72 months who had been treated with a short course of androgen treatment in infancy. Continued research is underway to expand our understanding of the relationship of androgen, brain function, and neurobehavioral and neurodevelopmental outcome in boys with 47,XXY. © 2013 Wiley Periodicals, Inc.     

Oral vascular malformation in a patient with hereditary hemorrhagic telangiectasia: a case report

Hereditary hemorrhagic telangiectasia (HHT) is an inherited mucocutaneous disease characterized by recurrent epistaxis, lesions on skin and oral mucosa, and arteriovenous malformations of the soft tissues. This article describes the treatment of a 64-year-old woman with a bleeding nodule, which was diagnosed as an arteriovenous malformation of the gingival mucosa. She was treated using sclerotherapy. Patients with HHT can be treated in the dental office and vascular malformations of these patients can be successfully managed with sclerotherapy, which eliminates the need for invasive surgical procedures and the possibility of postsurgical complications.     

Human defensin alpha-1 causes Trypanosoma cruzi membrane pore formation and induces DNA fragmentation, which leads to trypanosome destruction

Human defensins play a fundamental role in the initiation of innate immune responses to some microbial pathogens. Here we show that human defensin alpha-1 displays a trypanocidal role against Trypanosoma cruzi, the causative agent of Chagas' disease. The toxicity of human defensin alpha-1 against T. cruzi is mediated by membrane pore formation and the induction of nuclear and mitochondrial DNA fragmentation, leading to trypanosome destruction. Exposure of trypomastigote and amastigote forms of T. cruzi to defensin alpha-1 significantly reduced parasite viability in a peptide concentration-dependent and saturable manner. The toxicity of defensin alpha-1 against T. cruzi is blocked by anti-defensin alpha-1 immunoglobulin G. Electron microscopic analysis of trypomastigotes exposed to defensin alpha-1 revealed pore formation in the cellular and flagellar membranes, membrane disorganization, and blebbing as well as cytoplasmic vacuolization. Furthermore, human defensin alpha-1 enters the trypanosome when membrane pores are present and is associated with later intracellular damage. Trypanosome membrane depolarization abolished the toxicity of defensin alpha-1 against the parasite. Preincubation of trypomastigotes with defensin alpha-1 followed by exposure to human epithelial cells significantly reduced T. cruzi infection in these cells. Thus, human defensin alpha-1 is an innate immune molecule that causes severe toxicity to T. cruzi and plays an important role in reducing cellular infection. This is the first report showing that human defensin alpha-1 causes membrane pore formation in a human parasite, leading to trypanosome destruction.     

Maxillary lesion presenting as a first sign of multiple myeloma: case report

Plasma cell neoplasia is a lymphoid neoplastic proliferation of B cells. This denomination encloses multiple myeloma (MM), solitary bone plasmacytoma and extramedullary plasmacytoma. MM consists of a clonal proliferation of plasma cells based in the bone marrow, with various degrees of differentiation. Neoplastic cells usually produce great amounts of monoclonal light or heavy chains of immunoglobulin that can be detected in serum or urine. The disease is more frequently in men and the average age at diagnosis is about 60 years. The diagnosis is established by blood and urine exams and medullary biopsy. Patients may present renal failure, bone pain, fatigue, recurrent infections and nervous system dysfunction. Oral manifestations may be the first sign of MM, highlighting the importance of the dentist in the early diagnosis of the disease. Treatment involves mainly irradiation and chemotherapy and the prognosis is generally poor. This paper reports a case of a 65 years old black female who had a complaint of a painful mass in the maxilla that prompted a MM diagnosis.     

Die postoperative Prognose des chromophoben Nierenzellkarzinoms

Background

The chromophobe subtype represents the third most common histological subtype of renal cell carcinoma (chRCC). Due to the rarity of this subtype only one publication regarding the specific analysis of clinical and histopathological criteria as well as survival analysis of more than 200 patients with chRCC is known to date.

Materials and methods

A total of 6,234 RCC patients from 11 centres who were treated by (partial) nephrectomy are contained in the database of this multinational study. Of the patients 259 were diagnosed with chRCC (4.2?%) and thus formed the study group for this retrospective investigation. These subjects were compared to 4,994 patients with a clear cell subtype (80.1?%) with respect to clinical and histopathological criteria. The independent influence of the chromophobe subtype regarding tumor-specific survival and overall survival was determined using analysis by Cox proportional hazards regression models. The median follow-up was 59 months (interquartile range 29-106 months).

Results

The chRCC patients were significantly younger (60 vs. 63.2 years, p?<?0.001), more often female (50 vs. 41?%, p?=?0.005) and showed simultaneous distant metastases to a lesser extent (3.5 vs. 7.1?%, p?=?0.023) compared to patients with a clear cell subtype. Despite a comparable median tumor size a ≥?pT3 tumor stage was diagnosed in only 24.7?% of the patients compared to of 30.5?% in patients with a clear cell subtype (p?=?0.047). In addition to the clinical criteria of age, sex and distant metastases, the histological variables pTN stage, grade and tumor size showed a significant influence on tumor-specific and overall survival. However, in the multivariable Cox regression analysis no independent effect on tumor-specific mortality (HR 0.88, p?=?0.515) and overall mortality (HR 1.00, p?=?0.998) due to the histological subtype was found (c-index 0.86 and 0.77, respectively).

Conclusions

Patients with chRCC and clear cell RCC differ significantly concerning the distribution of clinical and histopathological criteria. Patients with chRCC present with less advanced tumors which leads to better tumor-specific survival rates in general; however, this advantage could not be verified after adjustment for the established risk factors.