Subject(s)
Vena Cava, Superior/abnormalities , Humans , Male , Middle Aged , Vena Cava, Superior/diagnostic imagingABSTRACT
Plexiform neurofibroma is a rare benign tumor of the peripheral nerves involving the conjunctiva cells of the perineurium. It is pathognomonic of neurofibromatosis type1 (NF1 or Von Recklinghausen disease). MRI is a great help in the diagnosis of this pathology. Anatomopathological confirmation is sometimes necessary, in particular in patients with no signs of NF1. We here report the case of a little girl with cervical plexiform neurofibroma revealing neurofibromatosis type 1.
Subject(s)
Head and Neck Neoplasms/diagnosis , Neurofibroma, Plexiform/diagnosis , Neurofibromatosis 1/diagnosis , Child , Female , Head and Neck Neoplasms/pathology , Humans , Magnetic Resonance Imaging/methods , Neurofibroma, Plexiform/pathology , Neurofibromatosis 1/pathologyABSTRACT
Renal angiomyolipomas are rare type of benign renal neoplasm. They are composed of vascular, smooth and fat elements and can be associated to phacomatosis as Tuberous Sclerosis disease. Symptomatic presentation is most frequently spontaneous retroperitoneal hemorrhage, which can be fatal. The risk of bleeding is proportional to the size of the lesion (>4 cm of diameter). Typical angiomyolipomas are benign but may have alarming properties: nuclear pleomorphism and mitotic activity, extension into the vena cava, and spread to regional lymph nodes without malignant progression. We report a Computed Tomography finding of a rare giant bilateral angiomyolipomas with spontaneous hemorrhage and inferior vena cava thrombus in a patient with tuberous sclerosis, emphasizing the importance of imagery in the positive and etiologic diagnosis.
Subject(s)
Angiomyolipoma/diagnosis , Kidney Neoplasms/diagnosis , Vena Cava, Inferior/pathology , Venous Thrombosis/etiology , Adult , Angiomyolipoma/complications , Angiomyolipoma/diagnostic imaging , Female , Hemorrhage/etiology , Humans , Kidney Neoplasms/complications , Kidney Neoplasms/diagnostic imaging , Retroperitoneal Space/pathology , Tomography, X-Ray Computed , Tuberous Sclerosis/pathology , Vena Cava, Inferior/diagnostic imaging , Venous Thrombosis/diagnostic imagingABSTRACT
INTRODUCTION: Neurogenic tumors account for 30% of mediastinal tumors in children. The thoracic region is the most common site for neurofibromas. We report a case of a voluminous neurofibroma in a small girl. CASE: After 8 months of paraplegia, a 4-year-old girl underwent thoracic radiography and computed tomography as well as magnetic resonance imaging of the spine. These revealed a huge mediastinal mass continuing into the vertebral canal and compressing the cord. Histological examination after surgery confirmed the diagnosis of neurofibroma. At the follow-up examination a month later, the paraplegia had resolved. DISCUSSION: Neurofibromas may manifest as solitary tumors or may be one manifestation of neurofibromatosis. In mediastinal neurofibroma, thoracic radiography, and computed tomography can show the tumor, posterior vertebral scalloping, and enlarged neural foramina. MR imaging provides the exact anatomic location, as well as any compression and displacement caused by the tumor. This information is essential for deciding the exact extent of resection. MRI may also be helpful in postoperative management.
