Arteriovenous malformations are congenital vascular malformations with a high flow rate. They are made up of a complex vessel system that forms a nidus by connecting feeding arteries to draining veins. Arteriovenous malformations can be fatal due to progressive symptoms and infiltrative disease. The head and neck are the most affected areas by extracranial arteriovenous malformations, followed by the limbs. Hormonal changes during pregnancy lead to the expansion of arteriovenous malformations, which may lead to aggressive progression. We present a case of a patient who had a very rare presentation of ruptured forehead arteriovenous malformations during her fifth pregnancy. A combination of radiological embolization followed by surgical excision was used to treat the condition.
Extensive soft tissue injuries with exposed joints, bones, and tendons due to trauma of the handparticularly fingers-necessitate soft tissue reconstruction and coverage. However, these injuries are challenging; therefore, various management options for traumatic skin loss over fingers are widely performed. These options comprise wound care permitting wound contracture without surgical intervention, full or split-thickness skin grafting, skeletal shortening and primary closure, and various types of flaps. We present a case of successfully placed Integra over the exposed phalangeal bones followed by split-thickness skin grafting a few weeks later, with a good outcome. We conclude that Integra is an alternative, safe and effective method for reconstructing severely traumatized fingers with exposed bone, tendon, or joint without sacrificing outcome.
Despite being the most common primary malignant bone tumor in children and adolescents, the presence of osteosarcoma at the wrist is infrequent; only less than 1% of osteosarcomas arise in the distal radius. The clinical presentation may mimic common musculoskeletal problems or benign lesions such as osteomyelitis, and a high index of suspicion is necessary so that the treating surgeon does not miss such lesions. We reported a case treated initially as osteomyelitis before being diagnosed as distal radius osteosarcoma. We conclude that an unusual location of osteosarcoma may be easily misdiagnosed, and therefore, osteosarcoma should be considered one of the main differential diagnosis in such cases until proven otherwise.
Neurofibromatosis type 1 (NF1) is a complex autosomal dominant, multisystem genetic disease affecting about 1 in 3500 individuals. Plexiform neurofibromas represent a rare variant (30%) of NF1 in which the spread of tumor cells along nerve fascicles leads to a diffuse mass of thickened nerve fibers. Affected patients with NF1 have a greater chance of developing soft tissue sarcomas than the general population. Leiomyosarcoma is one of the most frequent soft tissue sarcomas, seldom observed in patients with NF1. Herein we report a rare concurrency of bone leiomyosarcoma in a patient with a plexiform neurofibroma, adding to the few reported cases of leiomyosarcomas in patients with NF1. Our case is a 14-year-old male who is a known case of NF1 and presented with a four-month history of pain and swelling on the medial side of the right knee. Imaging and biopsy confirmed the diagnosis of leiomyosarcoma. Based on the authors' knowledge and search, this is the first reported case of plexiform neurofibroma with a primary bone leiomyosarcoma, representing an extremely rare concurrency. Patients with such uncommon tumors should be assessed regularly, and continuous follow-up is essential.
Malignant peripheral nerve sheath tumours (also called neurofibrosarcomas) are a rare, highly aggressive soft tissue sarcomas that arise from the peripheral nerves or cells associated with the nerve sheath, such as Schwann cells, peri-neural cells and fibroblasts. It is representing 10% of all soft tissue sarcomas in which it is considered as an extremely rare malignancy, especially in patients with neurofibromatosis type I. In the general population, it affects approximately 1 in 100,000 people. This article is reporting a 56-year-old Malay female patient who is a known case of neurofibromatosis type I for 20 years, presented with a lower back, pruritic, gradually increasing swelling during the last five months. Last month before the presentation, the lesion rapidly grows, reaching a size of (15×15 cm), accompanied by foul-smelling discharge and pain exacerbated with movement. Although no history of preceding trauma or accident, the mass bleeds within contact. In conclusion, only a few cases of giant malignant peripheral nerve sheath tumours reported in the literature describing its location and growth progression. We present a massive, extremely rapid growth of cutaneous exophytic malignant peripheral nerve sheath tumours over the lower back.
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