Neurofibromatosis type1 （NF1）, also known as Von Recklinghausen＇s disease with approximate occurrence of 1/3000 births, can be considered as a common autosomal dominant condition. Abdominal organs can be involved a...Neurofibromatosis type1 （NF1）, also known as Von Recklinghausen＇s disease with approximate occurrence of 1/3000 births, can be considered as a common autosomal dominant condition. Abdominal organs can be involved as neuro-fibroma and tumor growth in the liver, mesentery, retropedtoneum, stomach and bowel. Association of NF and gastrointes-tinal neoplasms occur in 2%-25% of patients. There is a 5%-10% lifetime risk of developing an NFl-associated malignant peripheral nerve sheath tumor （MPNST）, usually adsing within a pre-existing plexiform neurofibroma which metastasizes widely heraldinga poor outcome. We report a 55 year old man with gastrointestinal NF which ultimately developed MPNST and deceased.展开更多
Reactive lesions of bone and soft tissue can appear alarming on histologic examination because they are often cellular and have atypical cytologic features,such as distinct nucleoli,mild hyperchromasia,and mitotic act...Reactive lesions of bone and soft tissue can appear alarming on histologic examination because they are often cellular and have atypical cytologic features,such as distinct nucleoli,mild hyperchromasia,and mitotic activity.Reactive lesions of bone and periosteum also produce bone and cartilage matrix,resulting in confusion with osteosarcoma or chondrosarcoma.Careful attention to key cytomorphological features such as the pattern of bone formation,uniform appearance of cells,and absence of atypical mitoses should help identify the reactive nature of a lesion.Correlation with clinical and radiological findings is also imperative to avoid misclassification of the tumor because reactive lesions often arise at sites where osteosarcoma and chondrosarcoma are rare(eg,the hand)and lack aggressive radiological features.Here we present a case of exuberant callus formation after avulsion fracture of tibia in a three year-old Iranian girl which misdiagnosed as osteosarcoma.展开更多
文摘Neurofibromatosis type1 （NF1）, also known as Von Recklinghausen＇s disease with approximate occurrence of 1/3000 births, can be considered as a common autosomal dominant condition. Abdominal organs can be involved as neuro-fibroma and tumor growth in the liver, mesentery, retropedtoneum, stomach and bowel. Association of NF and gastrointes-tinal neoplasms occur in 2%-25% of patients. There is a 5%-10% lifetime risk of developing an NFl-associated malignant peripheral nerve sheath tumor （MPNST）, usually adsing within a pre-existing plexiform neurofibroma which metastasizes widely heraldinga poor outcome. We report a 55 year old man with gastrointestinal NF which ultimately developed MPNST and deceased.
文摘Reactive lesions of bone and soft tissue can appear alarming on histologic examination because they are often cellular and have atypical cytologic features,such as distinct nucleoli,mild hyperchromasia,and mitotic activity.Reactive lesions of bone and periosteum also produce bone and cartilage matrix,resulting in confusion with osteosarcoma or chondrosarcoma.Careful attention to key cytomorphological features such as the pattern of bone formation,uniform appearance of cells,and absence of atypical mitoses should help identify the reactive nature of a lesion.Correlation with clinical and radiological findings is also imperative to avoid misclassification of the tumor because reactive lesions often arise at sites where osteosarcoma and chondrosarcoma are rare(eg,the hand)and lack aggressive radiological features.Here we present a case of exuberant callus formation after avulsion fracture of tibia in a three year-old Iranian girl which misdiagnosed as osteosarcoma.
