This 16-year-old boy with Proteus syndrome suffered acute spinal cord compression secondary to hemorrhage into an extradural paraspinal angiolipomatous hamartoma. A magnetic resonance (MR) imaging study performed 12 years earlier had revealed the presence of this tumor at the apex of a mild thoracic scoliosis, which had been stable during the intervening decade. Spinal cord neurological deficit in Proteus syndrome is rare. It can arise either from vertebral abnormalities and kyphoscoliotic deformities or from tumor encroachment. The authors describe the temporal evolution of scoliosis and acute spinal cord compression caused by a paraspinal angiolipomatous hamartoma over a period of 12 years. A review of the literature suggests that regular follow up, including serial MR imaging, is indicated in children with Proteus syndrome and scoliosis. Earlier resection of extradural tumor mass encroaching on the cord is warranted in the treatment of these children.
[Show abstract][Hide abstract] ABSTRACT: Angiolipoma is a rare vascular variant of the benign lipomatous tumors and is generally seen in subcutaneous tissues. We report a 70-year-old female with abdominal distension not related to rectal small polypoid mass with peduncule described as angiolipoma by histologically, and review the literature.
World Journal of Gastroenterology 04/2007; 13(9):1460-5. · 2.37 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: A 2-year-old boy presented with weakness of both lower limbs with bladder and bowel involvement and history of frequent falls. Magnetic resonance imaging of the spine revealed a T2 altered signal intensity enhancing mass lesion seen in the spinal epidural space extending from sixth cervical to fourth thoracic vertebrae, which was compressing the adjacent spinal cord. Histopathology of the lesion was suggestive of hamartoma. A brief review of the literature including its embryogenesis is discussed here. The child subsequently developed pseudopancreatic cysts possibly because of repeated falls leading to blunt trauma abdomen.
The Indian Journal of Pediatrics 04/2008; 75(3):281-3. DOI:10.1007/s12098-008-0060-3 · 0.87 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Proteus syndrome is a complex and highly variable disorder comprising malformations and overgrowth of multiple tissues. We present a 65-year-old Japanese man who had multiple spinal meningiomas and accompanying neural symptoms. His right leg showed hypertrophy with cerebriform connective-tissue naevus on the sole, and macrodactyly. Chest computed tomography imaging revealed mild cystic and emphysematous lung changes, which were possibly related to Proteus syndrome. Otherwise, he had no particular cutaneous, musculoskeletal or visceral involvements. Because of the rather insignificant clinical features, he had not been accurately diagnosed in the past and yet had survived to this age. In particular, the presence of spinal meningiomas as an exceptional complication was sufficiently confusing to consider that he had neurofibromatosis. Doctors should be familiar with the diverse clinical pictures of this rare syndrome for its correct diagnosis and proper management.
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