Surgical resection of extremely rare primary giant splenic angiomyolipoma: a case report

Background Angiomyolipoma is a benign mesenchymal tumor that develops commonly in the kidney and rarely in other organs. The involvement of the spleen in angiomyolipoma is extremely rare, and only one such case has been reported in the English literature. Case presentation A 27-year-old man presented with adenoid hyperplasia and bilateral palatal tonsillar hyperplasia. During the treatment for adenoid hyperplasia, a 15-cm tumor was detected in the spleen using abdominal ultrasonography and enhanced computed tomography. Partial resection of the spleen was successfully performed. A giant tumor of approximately 13 cm with a smooth surface was observed in the upper left quadrant of the abdomen. The tumor was confirmed to be continuous with the upper spleen, and there was no invasion of the other organs. The postoperative course was good, and the patient was discharged on the 7th postoperative day. The excised specimen was a smooth, extremely soft tumor measuring 123 × 120 × 82 mm. The cleaved surface of the tumor was reddish brown, and a distressing yellow color was observed. Pathological examination revealed a proliferation of mature adipocytes and an increase in the number of blood vessels of various sizes. Furthermore, spindle-shaped cell proliferation foci were visible between the adipocytes and the surrounding blood vessels. Profuse leakage of erythrocytes from the blood vessels, hemosiderin deposition, and small round cell infiltration were also noted. Immunostaining disclosed that the spindle-shaped cells were weakly positive for smooth muscle antibody and were identified as smooth muscle cells. The adipocytes and spindle cells were negative for HMB 45, Melan A, MDM, and CDK4. However, some parts of the cells were positive for estrogen and progesterone receptors. Besides, vascular endothelial cells were positive for CD31 and CD34 and negative for CD8. Based on these findings, the patient was diagnosed to have primary angiomyolipoma of the spleen. Conclusions We have reported the surgical treatment for an extremely rare case of giant splenic angiomyolipoma in a young man. Globally, this is the second report on this condition. We believe that partial splenic resection is a feasible option for the management of giant tumors.

Association between the microbiomes of tonsil and saliva samples isolated from pediatric patients subjected to tonsillectomy for the treatment of tonsillar hyperplasia

Oral microbes have the capacity to spread throughout the gastrointestinal system and are strongly associated with multiple diseases. Given that tonsils are located between the oral cavity and the laryngopharynx at the gateway of the alimentary and respiratory tracts, tonsillar tissue may also be affected by microbiota from both the oral cavity (saliva) and the alimentary tract. Here, we analyzed the distribution and association of the microbial communities in the saliva and tonsils of Korean children subjected to tonsillectomy because of tonsil hyperplasia (n = 29). The microbiome profiles of saliva and tonsils were established via 16S rRNA gene sequencing. Based on the alpha diversity indices, the microbial communities of the two groups showed high similarities. According to Spearman’s ranking correlation analysis, the distribution of Treponema, the causative bacterium of periodontitis, in saliva and tonsils was found to have a significant positive correlation. Two representative microbes, Prevotella in saliva and Alloprevotella in tonsils, were negatively correlated, while Treponema 2 showed a strong positive correlation between saliva and tonsils. Taken together, strong similarities in the microbial communities of the tonsils and saliva are evident in terms of diversity and composition. The saliva microbiome is expected to significantly affect the tonsil microbiome. Furthermore, we suggest that our study creates an opportunity for tonsillar microbiome research to facilitate the development of novel microbiome-based therapeutic strategies.

IS THERE ANY ASSOCIATION BETWEEN ORAL HYGIENE AND THE DEVELOPMENT OF TONSILLITIS OR TONSILLAR HYPERPLASIA

Background: The number of recurrent tonsillitis and tonsillar hyperplasia requiring tonsillectomies, annually is huge. Poor oral hygiene has been linked with the occurrence of these tonsillar diseases. Objective: To determine the association between poor oral hygiene and tonsillitis and/or tonsillar hyperplasia. Methodology: This was a study of association between oral hygiene and tonsillitis and/or tonsillar enlargement determined by correlating the Oral hygiene index scores (OHIS) and Decay Missing and Filled Teeth (DMFT) of subjects with complaint of sore throat with development of tonsillitis or tonsillar hyperplasia. A corresponding number of subjects with no symptoms of sore throat were used as control. Results: A total of 80 subjects with sore throat were recruited into the study, comprising 49(61.3%) females and 31(38.7%) males with age ranging from 16 to 32 years. The control group comprised 45(56.3%) females and 35(43.7%) males with age ranging from 16 to 48 years.. OHIS of the test group was poorer than that of the control. Erythema (redness) of the tonsil was significantly correlated with OHIS in the test group but not in the control. Conclusion: Poor oral hygiene seems to have an association with tonsillar infection and so the, otolaryngologists managing tonsillar infection should consider inter-disciplinary approach in the management of tonsillar diseases.

0881 Clinical Evaluation of Adeno-Tonsillar Hyperplasia in Non-Syndromic Children and Adolescents During Growth Hormone Treatment

Introduction Recombinant human Growth Hormone (rhGH) is an important pharmacological agent for linear growth stimulation and body composition in children with growth hormone deficiency (GHD). However, reports indicate that treatment with rhGH can be associated with adeno-tonsillar hyperplasia. This condition can lead to occurrence of obstructive sleep apnea (OSA) and worsening of sleep and life quality. Nonetheless, studies assessing this outcome in non-syndromic children are scarce. Methods We evaluated the difference in size of pharyngeal and palatine tonsils in patients aged between 5 and 17 being treated with recombinant human Growth Hormone (rhGH). We conducted a prospective longitudinal observational study. Twelve patients in treatment with rhGH were evaluated by otorhinolaryngological physical examination, nasofibrolaryngoscopy and obstructive sleep apnea (OSA)-18 questionnaire in two different time-points: when selected (T0) and after 6 months (T1). Results No significant associations were found regarding palatine and pharyngeal tonsil size with rhGH treatment. In relation to OSA 18 questionnaires, there was no statistically significant result in the absence of covariables for the general score as well as for the five domains that it comprises. When covariables were included in the analyses, controlled by the patients age, we observed statistically significant increases in the general score and in the domains relating to sleep disorders, emotional suffering, diurnal problems and the caretaker′s concerns. Conclusion In the present study, pharyngeal or palatine tonsils hyperplasia were infrequent during treatment with rhGH in the non-syndromic children and adolescents. However, the impact on sleep and quality of life that may arise warrant careful monitoring during therapy. Support Associação Fundo de Incentivo à Pesquisa (AFIP).

Impact of Superantigen-Producing Bacteria on T Cells from Tonsillar Hyperplasia

Staphylococcus aureus and Group A Streptococcus (GAS) are common occupants of the tonsils and many strains produce potent exotoxins (mitogens) that directly target T cells, which could be a driver for tonsillar hyperplasia. Tonsil tissues from 41 patients were tested for these bacteria in conjunction with profiling of B and T cells by flow cytometry. S. aureus and GAS were detected in tonsil tissue from 44% and 7%, respectively, of patients by bacteriological culture; immuno-histology showed bacteria in close proximity to both B and T lymphocytes. The presence of tonsillar S. aureus did not alter B or T cell populations, whereas peripheral blood mucosal-associated invariant T (MAIT) cells were significantly increased in S. aureus culture positive individuals (p < 0.006). Alterations of tonsil CD4+ TCR Vβ family members relative to peripheral blood were evident in 29 patients. Three patients had strong TCR Vβ skewing indicative of recent exposure to superantigens, their tonsils contained mitogenic bacteria, and supernatants from these bacteria were used to partially recapitulate the skewing profile in vitro, supporting the notion that superantigens can target tonsillar T cells in situ. Tonsils are a reservoir for superantigen-producing bacteria with the capacity to alter the composition and function of key immune cells.

SURGICAL AND NON-SURGICAL THERAPY OF OBSTRUCTIVE SLEEP APNEA SYNDROME IN CHILDREN

Interventions of paediatric obstructive sleep apnea syndrome are complex, varied and multidisciplinary. The goal of the treatment is to restore optimal breathing during the night and to relieve associated symptoms. Evidence suggests that the surgical intervention with removal of the tonsils and adenoids will lead to significant improvements in the most incomplicated cases, as recently reported from a meta-analysis. However, post-operative persistence of this syndrome in paediatric population is more frequent than expected, which supports the idea of the complexity of this syndrome. Adenotomy alone may not be sufficient in children with OSAS, because it does not address oropharyngeal obstruction secondary to tonsillar hyperplasia. Continuous positive airway pressure can effectively treat this syndrome in selected groups of children, improving both nocturnal and daytime symptoms, but poor adherence is a limiting factor. For this reason, CPAP is not recommended as first-line therapy for OSAS when adenotonsillectomy is an option. It is now being investigated the incorporation of nonsurgical approaches for milder forms and for residual OSAS after surgical intervention. Althought adeno­tonsillar hypertrophy is the most common for OSAS in children; obesity is emerging as an equally important etiological factor. Therefore an intensive weight reduction program and adequate sleep hygiene are also important lifestyle changes that may be very effective in mitigating the symptoms of this syndrome. Pharmacological therapy (leukotriene antagonists, topical nasal steroids) is usually use for mild forms of OSAS and in children with associated allergic diseases. Special orthodontic treatment and oropharyngeal exercises are a relatively new and promising alternative therapeutic modality used in selected groups of children with OSAS.