Subject(s)
Bronchiectasis/diagnostic imaging , Neurofibroma/diagnostic imaging , Spinal Cord Neoplasms/diagnostic imaging , Bronchiectasis/complications , Cysts/complications , Cysts/diagnostic imaging , Humans , Lung Diseases/complications , Lung Diseases/diagnostic imaging , Magnetic Resonance Imaging , Male , Middle Aged , Neurofibroma/congenital , Neurofibroma/pathology , Radiography, Thoracic , Spinal Cord Neoplasms/complications , Spinal Cord Neoplasms/pathology , Thoracic Vertebrae , Tomography, X-Ray ComputedABSTRACT
Congenital skull defect is a rare malformation that is usually associated with congenital anomalies of the scalp and comparable lesions in the brain, spinal cord, limbs, and skeletal muscle. Most previously reported cases have described skull defects with aplasia cutis congenita and other congenital abnormalities. Very few patients with skull defects present with an intact scalp or neurofibroma. The authors report an adult patient with a rare congenital skull defect and local neurofibroma.
Subject(s)
Neurofibroma/congenital , Skull Neoplasms/congenital , Skull/abnormalities , Adult , Humans , Male , Neurofibroma/diagnostic imaging , Neurofibroma/surgery , Radiography , Skull/diagnostic imaging , Skull/surgery , Skull Neoplasms/diagnostic imaging , Skull Neoplasms/surgeryABSTRACT
Congenital giant benign tumours of the back are rarely seen or reported. They pose two challenges to plastic surgeons: copious intraoperative bleeding, and an extensive raw area left after removal of the tumour. We have treated five cases of congenital giant benign tumour of the back. The largest was a neurofibroma in a woman, which weighed 36.29 kg and she had 10,000 ml of blood transfused to replace the blood loss during operation. We achieved total excision of the tumours in all cases and covered the extensive wounds with skin harvested from the tumours themselves in one stage. Among the five cases, four were neurofibromas and one was a giant naevus. All grafted skin took, and there were no recurrences. The tumours were radically excised in one stage and the extensive wounds covered with the skin with good take and no risk of recurrence.
Subject(s)
Neurofibroma/congenital , Neurofibroma/pathology , Nevus, Pigmented/congenital , Nevus, Pigmented/pathology , Skin Neoplasms/congenital , Skin Neoplasms/pathology , Adolescent , Adult , Back/pathology , Back/surgery , China , Female , Follow-Up Studies , Humans , Male , Neurofibroma/surgery , Nevus, Pigmented/surgery , Rare Diseases , Plastic Surgery Procedures/methods , Retrospective Studies , Risk Assessment , Sampling Studies , Severity of Illness Index , Skin Neoplasms/surgery , Skin Transplantation , Treatment Outcome , Wound Healing/physiology , Young AdultABSTRACT
A case of an unusual congenital intramedullary tumor of the spinal cord is reported. A paraplegic 11-day-old boy with hypotonia and atrophy of the abdominal and lower-extremity muscles showed a complete myelographic block between T-5 and T-8. Surgical exploration disclosed an elongated tumor mass within the spinal cord, that blended with the surrounding nervous-system tissue. Light and electron microscopy showed that the tumor was composed of intermingled well differentiated astrocytes and fibroblasts. These two cell types often were surrounded by the same basal lamina. There were no intercellular junctions. Gliofibrils were abundant, and the interstitial spaces contained abundant collagen and reticulin fibers. There were no histological signs of malignancy. We conclude that this is a case of prenatally arising gliofibroma.
Subject(s)
Neurofibroma/congenital , Spinal Cord Neoplasms/congenital , Humans , Infant, Newborn , Male , Microscopy, Electron , Neurofibroma/ultrastructure , Spinal Cord Neoplasms/ultrastructureABSTRACT
Included in the discussion is the management of anomalies of abnormal formation of the lid folds, disorders of differentiation of the lid margins, and disorders of tissue differentiation. Also discussed are lid involvement with bacterial lesions and viral diseases.
Subject(s)
Eyelid Diseases/congenital , Eyelids/abnormalities , Bacterial Infections/complications , Blepharoptosis/congenital , Child, Preschool , Coloboma/congenital , Ectropion/congenital , Entropion/congenital , Eyelid Diseases/etiology , Eyelid Neoplasms/congenital , Female , Humans , Infant , Infant, Newborn , Male , Neurofibroma/congenital , Virus Diseases/complicationsABSTRACT
An eight-year-old child with congenital neurofibromatosis developed rapidly growing synchronous malignant schwannomas in the neck and mediastinum. These tumors, displaying a biphasic pattern of spindle cell sarcoma admixed with mucinous epithelium, represent the eighth and ninth known examples of glandular schwannoma. The clinical and pathological features of this case are detailed and the histogenesis of the tumors discussed.
Subject(s)
Head and Neck Neoplasms/pathology , Mediastinal Neoplasms/pathology , Neoplasms, Multiple Primary/pathology , Neurilemmoma/pathology , Neurofibroma/pathology , Child , Female , Head and Neck Neoplasms/congenital , Head and Neck Neoplasms/etiology , Humans , Mediastinal Neoplasms/etiology , Neoplasm Recurrence, Local/pathology , Neoplasms, Multiple Primary/etiology , Neurilemmoma/etiology , Neurofibroma/congenitalABSTRACT
A congenital malignant peripheral nerve sheath tumor contained small rhabdomyosarcomatous portions in both the retroperitoneal pelvic primary and in the subpleural lung metastasis. There was no family history of Von Recklinghausen's disease. Of the 10 patients with this tumor previously reported, the youngest is 14 yr old, and eight had familial neurofibromatosis.
Subject(s)
Neurofibroma/congenital , Pelvic Neoplasms/congenital , Peripheral Nervous System Neoplasms/congenital , Retroperitoneal Neoplasms/congenital , Rhabdomyosarcoma/congenital , Adult , Female , Humans , Infant, Newborn , Male , Neoplasm Metastasis , Neurofibroma/pathology , Pelvic Neoplasms/pathology , Peripheral Nervous System Neoplasms/pathology , Pleural Neoplasms/congenital , Retroperitoneal Neoplasms/pathology , Rhabdomyosarcoma/pathologyABSTRACT
An occipital scalp tumor first noted in a Nigerian girl during the first month of her life gradually increased in size and 13 years later measured 10 X 16 X 17 cm. A skull film revealed a 2 X 4-cm skull defect underneath the tumor over the lambdoid suture. Complete excision of the tumor was achieve although it was adherent to the dura of the transverse sinus. It was histologically confirmed to be plexiform neurofibroma. There were no other signs of van Recklinghausen's neurofibromatosis.
