Capillary hemangioma involved in filar lipoma: A case report.

Filar lipomas are a subtype of spinal lipomas wherein adipose tissue accumulation is restricted to the filum terminale. Embryologically, filar lipomas are considered to occur because of the failure of secondary neurulation, although the precise mechanism is not yet completely understood. Involvement of ectopic mesodermal, ectodermal, and endodermal tissues in spinal lipomas has been occasionally reported, and the origin of these ectopic tissues has been supposed to be migration of pluripotent tissues, which exist during secondary neurulation. We report an infantile case of capillary hemangioma involved in filar lipoma. To our knowledge, this is the first report of a case of intradural extramedullary capillary hemangioma at the filum terminale. We suspected that the filar lesion arose during the late phase of secondary neurulation based on the clinical, anatomical, and histological characteristics.
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Schwannoma of the hand in an infant: case report.

Schwannomas (neurilemmomas) are the most common benign tumors of peripheral nerves and originate from the myelinating cells of the nerve sheath. We present a case of congenital schwannoma of the hand.

Pacinioma of the cauda equina.

Lesions composed of Pacinian corpuscles or showing Pacinian corpuscle differentiation have usually been described in relation to benign tumours of the peripheral nervous system or reactive hyperplastic processes. On the other hand, mature Pacinian corpuscles have occasionally been detected as part of intraspinal lumbosacral lipomas, a rare developmental anomaly usually associated with spina bifida. A lesion of the cauda equina composed of numerous mature Pacinian corpuscles and nerve fascicles embedded in adipose tissue in association with spina bifida occulta is described in a 5-month-old male with a sacral red papula. Magnetic resonance imaging (MRI) revealed a cord-like mass in the region of the cauda equina, presumably connected to the subcutis. With the exception of a low lying, tethered spinal cord, there was no neurological deficit and the range of motor development was normal. In March 2005, at 17 months, surgery was carried out. A cord of yellow tissue was found running from the subcutis through the bone defect into the lumbosacral spinal canal. Intradurally, it ran parallel to the cauda equina, terminating at the conus medullaris. Fifteen months after the surgery the development of the child was normal. Only two similar cases have been reported so far. Due to their occurrence in the sacrococcygeal region and association with developmental anomalies, they have been regarded as malformations and the term Pacinioma has been suggested. Our case with clusters of Pacinian corpuscles may represent a rare variant of complex intraspinal lumbosacral lipomas, closely related to Paciniomas reported by Bale.

Conservative management of asymptomatic spinal lipomas of the conus.

OBJECTIVE: The natural history of spinal lipomas of the conus (SLCs) has not been well studied. Because of disappointing long-term results with early surgical treatment of asymptomatic children with SLCs, we have followed a protocol of conservative management for these patients. The results are presented in this report. METHODS: Since 1994, all asymptomatic children with SLCs who were examined at Necker-Enfants Malades Hospital were subject to a protocol of conservative management. The records for those patients were reviewed, to determine the incidence and timing of neurological deterioration. The findings were compared with those for a previously reported historical cohort of asymptomatic patients who underwent early surgery at our institution. RESULTS: Fifty-three asymptomatic children (35 girls and 18 boys) with SLCs were monitored, with conservative management. During a mean follow-up period of 4.4 years (range, 12 mo to 9 yr), 13 patients (25%) exhibited neurological deterioration. At 9 years, the actuarial risks of deterioration, as determined with the Kaplan-Meier method, were 33% for the conservatively treated patients and 46% for the surgically treated patients. With a Cox proportional-hazards model, there was no significant difference in the risks of neurological deterioration for patients who were treated conservatively and those who underwent early surgery. CONCLUSION: The incidences and patterns of neurological deterioration seemed to be very similar, regardless of whether early surgery was performed. These results suggest that conservative treatment of asymptomatic patients is a reasonable option. A more definitive randomized study will be required to clarify the relative efficacy of early surgery for SLCs among asymptomatic patients.

Ineffectiveness of dietary folic acid supplementation on the incidence of lipomyelomeningocele: pathogenetic implications.

OBJECT: Periconceptual folic acid supplementation is effective in myelomeningocele prevention. The relationship between folic acid and lipomyelomeningocele (LMM) and the overall incidence of this occult form of spina bifida has never been studied. The objectives of this study were to determine the impact of dietary folic acid supplementation on the incidence of LMM and to measure its overall incidence. METHODS: In a retrospective population-based study the authors calculated the incidence of LMM in Nova Scotia between 1985 and 2001. Because of changes in public policy during this period, there are three intervals defined in relation to the treatment of the food supply with folic acid: 1) prior to folic acid fortification (1985-1994); 2) postsupplementation but prefortification (1995-1998); and 3) postfortification. The overall incidence of LMM in Nova Scotia between 1985 and 2001 was 16 per 100,000 live births or one case per 6121 live births. Its incidence between 1985 and 1994 was 15 per 100,000 live births, and between 1995 and 1998 it was 12 per 100.000 live births (relative risk [RR] 0.82, 95% confidence interval [CI] 0.31-2.22; p = 0.7). Between 1999 and 2001, the incidence of LMM was 29 per 100,000 live births, which was not significantly different from that between 1995 and 1998 (RR 2.41. 95% CI 0.79-7.36; p = 0.11) or between 1985 and 1994 (RR 1.98, 95% CI 0.86-4.56; p = 0.1). CONCLUSIONS: The overall incidence of LMM between 1985 and 2001 in Nova Scotia was 16 per 100,000 live births and has not been reduced by dietary folic acid supplementation. This finding provides epidemiological evidence that the embryogenesis of LMM is fundamentally different from that of myelomeningocele.

Lipofibromatous hamartoma of the median nerve associated with macrodactyly and port-wine stains.

Lipofibromatous hamartoma of the nerve is a very uncommon congenital tumor. An association between this condition and vascular malformations is not well known. We present an 11-year-old girl with a lipofibromatous hamartoma of the right median nerve with macrodactyly. She had small red macules on her right neck, chest, and arm, which were diagnosed clinically as port-wine stains. The specimens of the enlarged nerves showed fibrous and fatty growth surrounding the nerve bundles and proliferation of the small veins. We suggest that this disorder can be accompanied by a vascular malformation.

Neurofibromatosis and associated neuroectodermal tumors: a congenital neurocristopathy.

The synchronous occurrence of neurofibromatosis and neuroblastoma has been labeled in the recent literature as a chance event. We report 2 cases of newborn infants with congenital neurofibromatosis and a similar midline pattern of multiple Schwann cell and neuroblastic tumors; other types of ectomesenchymal tumor differentiation are documented, along with supportive ultrastructural and immunohistochemical studies. The tumors may take an aggressive, fatal course despite maximal multimodality antitumor therapy. These 2 cases are reported, with additional literature review, to document a clinically recognizable neurocristopathy that links neuroblastic tumors and neurofibromatosis.

Surgically confirmed myelographic classification of congenital intraspinal lipoma in the lumbosacral region.

Twenty-four cases of histologically confirmed congenital intraspinal lipoma of the lumbosacral region were studied by means of myelography with metrizamide. The findings were compared with intraoperative observations. Myelography with metrizamide clearly revealed the detailed intrathecal structures and allowed a classification of intraspinal lipomas into four types, in terms of their insertion into the conus medullaris: dorsal type, either with direct or indirect (via an intrathecal stalk) insertion of the extrathecal lipoma into the dorsal aspect of the conus medullaris; caudal type; combined type; and filar lipoma. Based on our surgical experience in untethering and decompression of the lesions, the classification was found to be useful in designing a safe and effective surgical procedure which minimized all possible trauma to the intrathecal neural structures.

Congenital primitive neuroectodermal tumor (neuroepithelioma) of the chest wall.

Primitive neuroectodermal tumor (neuroepithelioma) is a relatively common central nervous system tumor in children. Those arising from a peripheral nerve are extremely rare in childhood. There is only one reported case in 6-year-old where the tumor arose from the sciatic nerve. A case of neuroectodermal tumor of the chest wall, arising from the intercostal nerve, in a newborn is presented. The tumor metastasized to the brain. Prominent Homer-Wright rosettes, with central eosinophilic fibrillar substance similar to that seen in neuroepithelioma of the central nervous system, were present in the primary tumor and brain metastases. Ultrastructure, as revealed by transmission electron microscopy, is also described.

Congenital neurogenous sarcoma with rhabdomyosarcomatous differentiation.

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.