Neurofibrosarcoma is a malignancy that occurs more frequently in patients with neurofibromatosis-1 (NF-1) and rarely may arise independently. This is a presentation of 3 consecutive patients with histologic diagnosis of neurofibrosarcoma who presented to the University of Calabar Teaching Hospital, Calabar from 2011-2013. Two patients presented were associated with NF-1 and were a decade younger than the one without NF-1. Late presentation and poor follow up were notable underlying factors. Long term follow up of patients with NF -1 for early diagnosis and adequate treatment will improve outcome.
Objective: To report a rare occurrence of giant, solitary, cystic, intramuscular plexiform neurofibroma in the thigh. Case Report: A 45-year-old architect presented with a 5-year history of painless, progressively enlarging soft tissue mass in the right thigh, which was diagnosed on histology as cystic plexiform neurofibroma after excision biopsy. Conclusion: A plexiform neurofibroma should be considered in the differentials of a solitary cystic mass in the thigh even in the absence of usual manifestations of Neurofibromatosis.
Background: Virchow in 1860 described liposarcoma which arise from fat cells. It is a common soft tissue sarcoma found commonly in the limbs and retroperitoneum. Objectives: This study is an attempt to highlight the biographic indices, pattern, and challenges of management and outcomes of liposarcoma in a tropical setting and proffer solutions for improved outcome.
Methods:Patients who presented to the University of Calabar Teaching Hospital from 2000-2014 with histologic diagnosis of liposarcoma were studied. This was compared with total soft tissue sarcoma and total malignancy seen during the same study period. Results: The 17 patients seen in the study period comprised 10 males and 7 females (M: F = 1.4:1) and their ages ranged from 37-70 years (mean 51 years). They accounted for 21.8% of soft tissue sarcoma and 0.9% of total malignancy. The commonest site involved was the lower limb 8(47%), while the abdomen ranked second 5(29%) with the retroperitoneum the commonest abdominal location. Pleomorphic liposarcoma was the commonest 8(47%) histologic type followed by myxoid 7(41%). The outcomes were generally poor with one hospital mortality. Sociocultural beliefs, ignorance and poverty were issues highlighted. Conclusions: Early presentation and diagnosis and treatment is advocated for improved outcome. Establishment of support groups in oncology will further provide health education services and compliment financing of treatment in view of the enormous cost of services and drugs.
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