Aim- To describe the pattern of clinical presentation of patients with neurofibromatosis in a homogeneous black African population with emphasis on ophthalmological presentation in a multidisciplinary management setting. Methods- Ophthalmology clinic records from the Department of Ophthalmology and database of the Department of Pathology, both of University College Hopsital, Ibadan, Nigeria were reviewed for cases of neurofibromatosis over a 10-year period (Jan 2010 and Dec 2019). Relevant demographic, clinical information, management, complications and patient follow up were extracted from the records and entered into a spreadsheet and analysed. Results - The 34 cases included in this study comprised 19 males (55.9%) and age ranged from 18 months – 60 years, with a median age of 15 years. The male to female ratio was 1.3:1 with approximately 35.3% (12 patients) in the paediatric age group. The most frequent reason for consultation was unilateral progressive painless lid swelling (plexiform neurofibroma) often associated with ptosis. There was a positive family history of neurofibromatosis in 9 out of 32 cases (28.1%). Café au laît macules were observed in 22 out of 25 (88%) of cases. Typical neurofibromas were present in 84.8% of the patients. There was no significant difference in prevalence of plexiform neurofibromas with age (p= 0.05) or sex (p= 0.79). Bone and joint abnormalities was present in 17.6% of the patients. Ophthalmic examination showed conjunctiva changes in 3 cases, prominent cornea nerves in 2 cases. Iris lisch nodules was present in 75.9% of cases that had documentation, cataract in 2 cases and optic atrophy in 6cases.Three patients had ophthalmic pathway gliomas. Patients were managed by multidisciplinary teams depending on their needs. Conclusion- Multidisciplinary team management is advocated because of the multi-system disorders these patient have and the need for holistic, patient centred care that is of good quality, and sustainable.
Aim: To describe the pattern of paediatric soft tissue sarcomas with emphasis on the grade and stage at the first surgical or medical oncologic intervention. Methods: We retrospectively reviewed all cases of histologically confirmed paediatric Soft Tissue Sarcomas in the Department of Pathology, University College Hospital (UCH), Ibadan, Nigeria in children age 0-14 years. The study period was January 1991 to December 2016. Information obtained included age, gender, morphology and site of the tumours. The tumour grade and pathologic/clinical staging of all cases that had surgical biopsy or surgical resection were also obtained and verified from the clinical records. Tumour grading was carried out using the Fédération Nationale des Centres de Lutte Contre le Cancer (FNCLCC) Sarcoma group grading system and staging was done using TNM. Results: One hundred and twelve cases were seen with an almost equal male-to-female ratio and age range from 5 months to 14 years (median 8.5 years). The peak age group was 5-9 years. Rhabdomyosarcoma was by far the most common histological type (73.2%). Other types were undifferentiated sarcomas (6.3%), fibrosarcoma (5.4%), Kaposi sarcoma (4.5%), and 2.7% each for synovial sarcoma, dermatofibrosarcoma protuberans. The common primary sites were the head and neck (including the orbit) 51(45.5%), and the abdominopelvic 27(24.1%) regions. Other sites included the lower limb 13(11.6%), trunk 12(10.7%), upper limb 6(5.4%). Majority (67.9%) had histologic grade 3 at presentation based on the FNCLCC grading system. About 48.2% had Stage T2b while 9.8% were stage T2a based on American Joint Committee on Cancer Staging (AJCC) system. Conclusion: Rhabdomyosarcoma, undifferentiated sarcomas and Kaposi sarcoma are the commonest paediatric malignant soft tissue tumours in our practice. Majority of our patients present with late stage tumour and with high histologic grade thereby conferring poor prognosis at presentation/first surgical or medical oncologic intervention.