Design and Implementation of the Retinoblastoma Collaborative Laboratory.

PURPOSE: The purpose of this work was to describe the design and implementation of a digital pathology laboratory, the Retinoblastoma Collaborative Laboratory (RbCoLab) in Kenya. METHOD: The RbCoLab is a central lab in Nairobi that receives retinoblastoma specimens from all over Kenya. Specimens were processed using evidence-based standard operating procedures. Images were produced by a digital scanner, and pathology reports were disseminated online. RESULTS: The lab implemented standard operating procedures aimed at improving the accuracy, completeness, and timeliness of pathology reports, enhancing the care of Kenyan retinoblastoma patients. Integration of digital technology to support pathology services supported knowledge transfer and skills transfer. A bidirectional educational network of local pathologists and other clinicians in the circle of care of the patients emerged and served to emphasize the clinical importance of cancer pathology at multiple levels of care. A 'Robin Hood' business model of health care service delivery was developed to support sustainability and scale-up of cancer pathology services. DISCUSSION: The application of evidence-based protocols, comprehensive training, and collaboration were essential to bring improvements to the care of retinoblastoma patients in Kenya. When embraced as an integrated component of retinoblastoma care, digital pathology offers the opportunity for frequent connection and consultation for development of expertise over time.

Keratocystic odontogenic tumours of the jaws and associated pathologies: a 10-year clinicopathologic audit in a referral teaching hospital in Kenya.

AIM: To establish the pattern of occurrence and the clinicopathological features of keratocystic odontogenic tumour (KCOT) over a 10-year period. MATERIALS AND METHOD: Patients from the University of Nairobi Dental Hospital treated for KCOT were included in the study over a 10-year period. The study highlights the demographic, clinico-radiological and histological features of these tumours. RESULTS: A total of 22 confirmed cases of KCOTs were recorded with equal gender prevalence; (M:F = 1.44:1). The age range of the patients was from 10 to 69 years with a peak in the second decade of life (mean = 27.5 yrs). Of the 22 cases, 15 (68.2%) occurred in the mandible of which eight (53.3%) involved the body, five (33.4%) the angle and ramus. Six (27.3%) occurred in the maxilla, and one (4.5%) was in both jaws and was associated with Gorlin-Goltz Syndrome. The most common presenting complaint in most patients was swelling 54.6%, and in 18.2% was incidental finding. Eight (36.4%) cases showed satellite cysts upon pathologic evaluation. Thirteen (59.1%) cases were managed by surgical excision, while nine (40.9%) were managed by enucleation. CONCLUSION: Based on the outcome of this study, KCOT present mostly in body, angle and ramus of the mandible and its peak is in the second decade of life.

Retinoblastoma.

Retinoblastoma is an aggressive eye cancer of infancy and childhood. Survival and the chance of saving vision depend on severity of disease at presentation. Retinoblastoma was the first tumour to draw attention to the genetic aetiology of cancer. Despite good understanding of its aetiology, mortality from retinoblastoma is about 70% in countries of low and middle income, where most affected children live. Poor public and medical awareness, and an absence of rigorous clinical trials to assess innovative treatments impede progress. Worldwide, most of the estimated 9000 newly diagnosed patients every year will die. However, global digital communications present opportunities to optimise standards of care for children and families affected by this rare and often devastating cancer. Parents are now leading the effort for widespread awareness of the danger of leucocoria. Genome-level technologies could make genetic testing a reality for every family affected by retinoblastoma. Best-practice guidelines, online sharing of pathological images, point-of-care data entry, multidisciplinary research, and clinical trials can reduce mortality. Most importantly, active participation of survivors and families will ensure that the whole wellbeing of the child is prioritised in any treatment plan.

Clinical features and types of paediatric orofacial malignant neoplasms at two hospitals in Nairobi, Kenya.

AIM: To evaluate the clinical features and histopathological types of orofacial malignant neoplasms in children. PATIENTS AND METHODS: The study involved patients aged 15 years and below diagnosed with malignancy at two main referral hospitals in Kenya during the period from July, 2008 to December, 2008. A questionnaire and clinical examination chart were used to document data. Data analysis was done using SPSS 12.0 programme. RESULTS: 65 children (44 males, 21 females) with ages ranging from 0.25 to 14 years were evaluated. The main complaints were swelling 61 (94%) and visual disturbance 29 (45%). The mean duration of symptoms was 0.17-36 months. The commonest signs were leucocoria (white reflection from the retina) 23 (35%), proptosis 19 (29%) and loss of vision 15 (23%). The commonest sites were orbit 30 (46%) and maxilla 11 (17%). Most neoplasms were retinoblastoma 26 (40%), followed by 14 (21%) cases of Burkitt's lymphoma (BL) and occurred in patients under 5 years of age (40 cases) followed by 19 cases in children aged 5-10 years. CONCLUSIONS: Overall, malignancies were more common in males than females with most having been diagnosed in children aged less than 10 years. Retinoblastoma and BL were the most common neoplasms.

One-stage treatment of acquired facial deformity caused by severe unilateral condylar hyperplasia.

Condylar hyperplasia (CH) is a rare and self-limiting process manifesting between the first and third decades of life. It causes facial asymmetry and derangement of occlusion. The management involves resection of the condylar head and orthognathic surgery followed by orthodontic treatment. We present a 33-year-old man with spontaneous onset of CH during a span of 10 years. This was managed with resection of the condyle alone, which dramatically improved facial asymmetry in addition to restoration of the occlusion within a few months of follow-up. Therefore, orthognathic surgery or orthodontic treatment was not needed.

Clinicopathologic features of ameloblastoma in Kenya: a 10-year audit.

This study describes the clinical and pathologic features of ameloblastomas seen in the 2 main craniofacial treatment centers in Kenya in the 10-year period between January 1995 and December 2005. A total of 184 patient records were analyzed for this study. Eighty-two (44.6%) of the patients were male, and 102 (55.4%) were female with an overall age range of 10 to 80 years (mean, 30.2 years; SD, 14.1 years). There was no significant difference in gender presentation of ameloblastomas, although females presented at a slightly older age. The mean age for males was 29.9 years, and for females, it was 30.5 years. Patients generally tended to seek medical advice late, with the mean duration at first presentation of 46.3 months for males and 44.4 months for females. Most of the ameloblastomas (n = 172; 93.5%) were located in the mandible, 11 (6.0%) were in the maxilla, and 1 (0.5%) was in the soft tissues. Presenting symptoms included swelling (n = 182; 98.9%), pain (n = 64; 36.0%), mobile teeth/history of extraction (n = 104; 57.5%), purulent discharge (n = 39; 21.7%) and paresthesia (n = 10; 5.6%). The posterior mandible was the most commonly affected site, whereas maxillary ameloblastomas tended to occur in anterior sites. One hundred fifty-three ameloblastomas (83.2%) were of the solid/multicystic subtype; 8 (5.3%) were unicystic; 1 (0.5%) was of extraosseous origin; 1 (0.5%) was desmoplastic; 9 (6.0%) were malignant, and 12 of the records had no histopathologic pattern specified.

Khat induces G1-phase arrest and increased expression of stress-sensitive p53 and p16 proteins in normal human oral keratinocytes and fibroblasts.

Khat is a psychostimulant plant used by over 10 million people daily, mainly in eastern Africa and the Middle East. Previous studies have suggested an association between khat use and oral lesions such as hyperkeratosis and oral cancer. This study investigated the effects of an extract of khat on primary normal human oral keratinocytes (NOK) and normal human oral fibroblasts (NOF). Low (sublethal) concentrations of khat inhibited the proliferation of both cell types in a dose-dependent and time-dependent manner. Both NOK and NOF treated with khat accumulated in the G1-phase of the cell cycle and showed increased expression of the stress-sensitive p53 protein after 24 h. Normal human oral keratinocytes showed a profound increase in p16(INK4A) (p16) after 24 h and showed morphological changes suggesting cell differentiation. Normal human oral fibroblasts showed growth inhibition and increased expression of p21(WAF1/CIP1) (p21) within 24 h. The concentrations of khat tested in this study were within the range of those found in the oral cavity of khat chewers. The results show that stress induced by khat modulates the cell cycle in oral keratinocytes and fibroblasts. It is further speculated whether khat could have similar effects in vivo, especially in keratinocytes.

Synchronous occurrence of ossifying fibroma of the mandible and maxilla: a case report.

Although ossifying fibroma (OF) is a slow growing neoplasm, some lesions may behave aggressively, reaching massive proportions that may demand special treatment. Synchronous presentation of this lesion in the maxilla and mandible is a rare occurrence. A case is presented of a 27-year-old woman who manifested synchronous OF lesions in the left maxilla and right mandible. The clinico-radiologic and histopathologic modalities of diagnosing OF are evaluated and discussed.