A phase II trial of bevacizumab and everolimus as treatment for patients with refractory,progressive intracranial meningioma

Meningiomas that progress after standard therapies are challenging with limited effective chemotherapy options. This phase II trial evaluated the efficacy of everolimus plus bevacizumab in patients with recurrent, progressive meningioma after treatment with surgical resection and local radiotherapy when appropriate. Patients with recurrent meningioma (WHO grade I, II, or III) following standard treatments with surgical resection and radiotherapy received bevacizumab (10 mg/kg IV days 1 and 15) and everolimus (10 mg PO daily) each 28 day cycle. Evaluation of response occurred every 2 cycles. The primary endpoint was progression-free survival (PFS). Secondary endpoints included response rate, overall survival and safety. Seventeen patients with a median age of 59 years (29–84) received study treatment. WHO grades at study entry included: I, 5 (29?%); II, 7 (41?%); III, 4 (24?%); unknown, 1 (6?%). Patients received a median of 8 cycles (1–37); all patients are off study treatment. A best response of SD was observed in 15 patients (88?%), and 6 patients had SD for >12 months. Overall median PFS was 22 months (95?% CI 4.5–26.8) and was greater for patients with WHO grade II and III compared to grade I tumors (22.0 months vs 17.5 months). Four patients discontinued treatment due to toxicity (proteinuria, 2; colitis, 1, thrombocytopenia, 1). However, other grade 3 toxicity was uncommon, and no patient had grade 4 toxicity. The combination of everolimus and bevacizumab was well-tolerated, and produced stable disease in 88?% of patients; the median duration of disease stabilization of 10 months (2–29). The median PFS from this prospective trial was similar to previous retrospective reports of bevacizumab in the treatment of recurrent meningioma.     

Genetics: A New Frontier in Otology

Molecular genetics is a rapidly expanding field with possibilities for novel diagnostic and treatment strategies for otological diseases. Gene therapy, if theory is proven practical, could eliminate disease at the molecular level, thus obviating the need for pharmacologic or surgical treatment. Recent years have seen great advances in our understanding of the molecular genetic basis of many otological disorders. Building on the success of the Human Genome Project, new technologies are in development to identify disease-causing mutations through genetic testing. A basic understanding of the genetic basis of Otological diseases is crucial to the practising Otologist and the time has come for genetic services to be incorporated into regular Otological clinics.     

Endolymphatic sac papillary carcinoma treated with surgery and post-operative intensity-modulated radiotherapy: a rare case report

Endolymphatic sac tumor is a rare papillary neoplasm of the temporal bone which can occur sporadically or in association with von Hippel-Lindau disease. Many cases have been reported in neurosurgical literature wherein surgical excision has proved to be the gold standard of treatment. This article highlights the rarity of the pathology and the role of radiotherapy to the residual lesion after surgery. Benefits of intensity modulated radiotherapy in such irregular-shaped tumor, which is in close vicinity to the critical structures, are also discussed.     

Quality Matters–Hematopoietic Stem Cell Transplantation <Emphasis Type="Italic">versus</Emphasis> Transfusion and Chelation in Thalassemia Major

Objective

To compare quality of life of children with thalassemia major who have undergone stem cell transplantation with those on regular transfusion.

Methods

The study included 40 children who underwent transplantation and 40 children and 20 adults on regular transfusion and iron chelation therapy. The quality of life assessment was done using the Pediatric Quality of Life Inventory 4.0 Generic Core Scale.

Results

The mean total summary score, psychosocial summary score and physical score was 92, 91 and 92.8, respectively in transplant group and 83, 82.7 and 83.6, respectively in children in transfusion group. The adult group on transfusion showed overall poorer scores of 74.9, 76 and 73.9, respectively. The average scores in all domains were significantly (P<0.05) lower and drop steeply in second decade in transfusion group.

Conclusion

Allogeneic stem cell transplantation improves quality of life in thalassemia major.

     

Papillary carcinoma in a supra hyoid thyroglossal duct cyst — an unusual presentation

Carcinomatous transformation of ectopic thyroid elements within the thyroglossal tract is a rare entity. We report a case of primary papillary carcinoma of thyroid presenting within the thyroglossal duct cyst in a 23 year old gentleman. The patient presented to us, as a case of suprahyoid thyroglossal cyst with sub-lingual involvement and he underwent surgical excision of the entire thyroglossal tract by Sistrunk’s approach. The post-operative histopathological diagnosis was of a papillary carcinoma within the thyroglossal duct cyst. Hence, the patient was evaluated for a malignant focus in the thyroid which proved negative. He was counseled regarding the possibility of developing Carcinoma in the thyroid gland and offered two options of further management viz., total thyroidectomy followed by life long thyroid suppression or watchful observation and follow up. As the patient belonged to the low risk group, he opted for the second option. He is presently kept under meticulous follow up and remains asymptomatic till date. We present the pre- and post-operative imaging, intra-operative findings, histo-pathological features and review the present world literature on this rare entity.     

Papillary carcinoma in a supra hyoid thyroglossal duct cyst — an unusual presentation

Carcinomatous transformation of ectopic thyroid elements within the thyroglossal tract is a rare entity. We report a case of primary papillary carcinoma of thyroid presenting within the thyroglossal duct cyst in a 23 year old gentleman. The patient presented to us, as a case of suprahyoid thyroglossal cyst with sub-lingual involvement and he underwent surgical excision of the entire thyroglossal tract by Sistrunk’s approach. The post-operative histopathological diagnosis was of a papillary carcinoma within the thyroglossal duct cyst. Hence, the patient was evaluated for a malignant focus in the thyroid which proved negative. He was counseled regarding the possibility of developing Carcinoma in the thyroid gland and offered two options of further management viz., total thyroidectomy followed by life long thyroid suppression or watchful observation and follow up. As the patient belonged to the low risk group, he opted for the second option. He is presently kept under meticulous follow up and remains asymptomatic till date. We present the pre- and post-operative imaging, intra-operative findings, histo-pathological features and review the present world literature on this rare entity.     

Interface reaction at dental implants inserted in condensed bone

Purpose: The influence of the osteotome technique on the interface reaction of cylinder implants (SLA, ITI^®) was compared with the interface reaction of conventional implant insertion in an animal model. Material and methods: A total of 64 implants were placed in the cranial and caudal tibia of 8 Göttinger minipigs. The implant site was prepared either by a conventional technique with drills (control group A) or by the osteotome technique (experimental group B). Bone tissue responses were evaluated by histomorphometry, fluorescence microscopy and scanning electron microscopy after 7 and 28 days of osseointegration. Results: The average initial (7 days) bone‐to‐implant contact ratio was not statistically significantly different for the osteotome technique (35.88±2.94%) than for the control group (43.78±3.39%, P<0.095). After 28 days, the bone‐to‐implant contact ratio became statistically significantly higher when implants were inserted by conventional preparation (44.81±3.07% (group B), 63.47±4.87% (group A), P=0.003). Whereas fluorescence and immunhistologic examination revealed new bone formation with osteocalcin deposition directly at the implant surface in both groups, the extent of direct bone/implant contact was enhanced in conventionally prepared implant sites. SEM analysis confirmed an intimate bone to implant bond without fibrous tissue formation in places of direct contact at an ultrastructured level. Conclusion: Implant placement in conventionally prepared implantation sites is accompanied by an improved interface formation at an early stage of implantation.