Intracranial Cisternal Lipoma Associated with Cerebellar Cortical Dysplasia Diagnosed Using Dixon Technique: A Case Report and a Review of Literature.

BACKGROUND: Intracranial lipomas are very rare congenital malformations. Previous studies have shown various brain anomalies related to intracranial lipomas, most of which are agenesis or dysgenesis of the adjacent structures. To the best of our knowledge, cortical dysplasia related to intracranial lipoma has yet to be reported. CASE REPORT: We present a rare case of intracranial lipoma in the quadrigeminal and superior cerebellar cisterns with combined cerebellar cortical dysplasia. A 43-year-old female underwent brain MRI to identify possible cause of headache. We made a confident diagnosis based on MR findings using Dixon technique, which is a fat-water separation method based on chemical shift. We also identified unique combined abnormalities of the right cerebellar hemisphere near the cisternal lipoma that showed an abnormal vertical orientation of the cerebellar folia and disorganized parenchymal pattern. CONCLUSION: This case exhibits the rareness of the intracranial lipoma related cerebellar cortical dysplasia by reviewing relevant literature and also highlights the usefulness of Dixon techniques in daily clinical practice.

Congenital Spinal Lipomatous Malformations. Part 1. Spinal Lipomas, Lipomyeloceles, and Lipomyelomeningoceles.

Background: Lumbosacral spinal lipomas and lipomyeloceles are usually identified in early childhood. Terminology, histopathology, and diagnosis for these malformations can be confusing. Materials and Methods: This is a PubMed review with comparison of embryology, gross, and histopathology, and reporting requisites for these and related closed spinal malformations. Results: The spinal lipoma group (congenital spinal lipomatous malformations) includes subcutaneous, transdural, intradural, and noncontiguous malformations stretching through the entire lower spinal region. This lipomyelocele trajectory overlaps the embryonic tail's caudal eminence. Histopathologically, the lipomyelocele spectrum is a heterogeneous, stereotypical set of findings encountered from dermis to spinal cord. Diagnosis requires detailed correlation of images, intraoperative inspection, and histopathology. Conclusions: Appropriate terminology and clinicopathologic correlation to arrive at a diagnosis is a critical activity shared by pathologist and clinician. Prognostic and management differences depend on specific diagnoses. Familial and genetic influences play little if any role in patient management in closed spinal malformations.

A possible link between intracranial lipomas and localization-related relapses in multiple sclerosis.

Intracranial lipomas are congenital malformations representing less than 0.5% of intracranial tumors. They are found incidentally and are asymptomatic in the majority of patients. Here we present three patients with Multiple sclerosis (MS) and intracranial lipomas (IL). The patients showed increased flares and burden of disabling and worsening MS symptoms with cognitive, neurovestibular dysfunction, and gait alterations associated with the localization of the Lipoma. The parenchyma near the Lipomas showed areas of demyelination and atrophy. We postulate that the location and content of the Lipomas may participate in the pathophysiology of MS symptoms in these patients. We conclude that in concurrent IL and MS, the lipomas localization may provoke incapacitating relapses.

Giant congenital melanocytic nevus associated with lipoma in an Indian man.

Congenital melanocytic nevus is a inborn melanocytic proliferation either present since birth or soon afterward and shows characteristic histopathological features comprising proliferation of benign melanocytes and extension of nevus cells into the deep reticular dermis and subcutis. We report the case of a 35-year-old male patient who presented with a progressively enlarging soft tissue mass in bilateral flank region. High-resolution ultrasound imaging showed multiple lipomatous masses within the soft tissues on the either flank region. Subsequent excision of the soft tissue mass showed a well-circumscribed lipomatous lesion with diffuse infiltration by benign appearing melanocytes within the fat lobules established by immunohistochemistry, which was positive for HMB45 and S100. This case serves to illustrate the initial diagnostic challenge in a male patient as well as the plasticity of the neural crest cells.

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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Fetal pericallosal lipomas - Clues to diagnosis in the second trimester.

INTRODUCTION: Pericallosal lipomas (PCL) are congenital soft masses of adipose cells encapsulated by a thin layer of fibrous tissue, appearing adjacent to the corpus callosum (CC). The lipomas are usually diagnosed prenatally during the third trimester. The purpose of this study was to identify 2nd trimester ultrasound findings that may hint to a later diagnosis of PCL and to evaluate their MRI evolution. METHODS: A multicenter study of fetuses diagnosed during the 3rd trimester with a PCL in 7 fetal ultrasound units between 2001 and 2017. We reevaluated the ultrasound and MRI images starting from the referral examination and until the time of diagnosis, searching for clues that could have prompted an earlier diagnosis. Parents were contacted at the end of the study period to obtain information regarding development and neurological examination. RESULTS: Fifteen patients with PCL were diagnosed during the study period; fourteen had second trimester scans. A 2nd trimester diagnosis was established in only 2 (13.3%). Anomalies of the CC were evident in 8/14 patients during the 2nd trimester scan and included: short length (n = 4), increased thickness (n = 1), complete agenesis (n = 2) and partial agenesis (n = 1). Third trimester ultrasound scans were considered diagnostic of PCL in all 12 remaining cases, in eight it was considered an isolated finding. Postnatal neurological evaluation in the isolated cases revealed normal development in all children. One child was diagnosed with attention deficit disorder. CONCLUSIONS: Non visualization of a PCL during the 2nd trimester is common, and should not be considered a diagnostic error. An underlying PCL should be included in the differential diagnosis of CC anomalies during this time period, necessitating further follow up into the 3rd trimester.

Split cord malformation type 1 with two hemicord lesions.

Split cord malformations are rare entities which may present in an occult manner or in association with other lesions or congenital anomalies. Rarely, these cases may have associated hemicord lesions. We report an unusual case, the first of its kind, a type 1 split cord malformation with two pathologically different lesions (lipoma and dermoid) on one hemicord.

Aggressive Resection of Congenital Lumbosacral Lipomas in Adults: Indications, Techniques, and Outcomes in 122 Patients.

OBJECTIVE: The authors reviewed the treatment of adult patients with congenital intraspinal lipomas with total/near-total resection and discussed their preoperative characteristics, prognostic factors, and surgical outcomes. METHODS: Medical records of 122 adult patients with congenital lumbosacral lipomas undergoing total/near-total resection were systematically analyzed. The cohort was subdivided into 3 groups depending on symptom onset age: group 1 (≤5 years, n = 40), group 2 (>5 years but <18 years, n = 33), and group 3 (>18 years, n = 49). Preoperative and postoperative neurologic status were compared between groups and analyzed as a whole. RESULTS: The most common symptom was bladder dysfunction (82.0%), followed by constipation (76.2%). At the 3-month follow-up, improvement was noted in most patients presenting with pain (87.2%) and neuropathic ulcers (70.0%). Overall, neurologic status was improved in 73.0% of patients and stabilized in 19.7% of patients. A binary logistic regression model identified shorter preoperative duration (P = 0.013) and preoperative pain (P = 0.005) as independent predictors of postoperative improvement. Neurosurgical complications developed in 16 patients, and wound complications occurred in 2 patients. Two of 3 patients who had recurred symptoms underwent repeated detethering surgery during long-term follow-up. CONCLUSIONS: Despite longer preoperative duration than the pediatric population, adult patients with lumbosacral lipomas can still benefit from total/near-total resection especially regarding pain and foot ulcers, with low surgery-related morbidity. The long-term advantage of resecting additional lipoma in adults remains a point of discussion.

Prenatal diagnosis of pericallosal curvilinear lipoma: specific imaging pattern and diagnostic pitfalls.

We report the first series of cases of pericallosal curvilinear lipoma (CL) diagnosed prenatally and highlight the limitations in identifying a specific prenatal imaging pattern using ultrasound and magnetic resonance imaging (MRI). In all five of our cases, on ultrasound, the main feature leading to referral was a short corpus callosum. This subtle callosal dysgenesis was associated with a band of hyperechogenicity surrounding the corpus callosum, mimicking the pericallosal sulcus, which increased in size during the third trimester in three of the four cases in which sonographic follow-up was performed. On T2-weighted MRI, this band showed typical hypointensity in all cases; in contrast, on T1-weighted imaging, in only one case was there hyperintensity, suggestive of fat, as seen typically in the postnatal period. For appropriate prenatal counseling regarding outcome, it is important to identify or rule out CL when mild corpus callosal dysgenesis is observed. One should be aware of subtle diagnostic findings, such as a thin band of echogenicity surrounding the corpus callosum that is seen as a band of hypointensity on T2-weighted fetal MRI, and which may increase in size during gestation. Copyright © 2017 ISUOG. Published by John Wiley & Sons Ltd.

Congenital Infantile Fibrosarcoma Associated With a Lipofibromatosis-Like Component: One Train May Be Hiding Another.

Congenital infantile fibrosarcoma (CIFS) is a soft tissue sarcoma of infants mainly involving lower extremities and usually developing during the first year of life. At another end of the spectrum of pediatric fibroblastic lesions, lipofibromatosis is a rare benign infiltrative soft tissue tumor that affects children. The authors report in this study a particular presentation with a CIFS surrounded by lipofibromatosis-like areas. The presence of a surrounding benign tumor confused and delayed CIFS diagnosis.

A Rare Diagnosis in the Neck During Childhood: Congenital Chondrolipoma.

Chondrolipomas are mesenchymal tumors that found as mature cartilage tissue in a fat tissue. A 2-year-old boy was seen with a complaint of a mass of the neck. On physical examination of the child, there was a one-centimeter mass above the sternocleidomastoid muscle on the lateral neck. Pathological examination of this mass after excision was reported as chondrolipoma. Chondrolipomas are seen mostly in the breast and in the adult. They are rare tumors of the head and neck area and seen mostly the in oral cavity here. This is the first case of chondrolipoma with this age and localization combination in the literature. Chondrolipoma should therefore also be considered in the differential diagnosis of congenital masses located at lateral cervical area.

Spinal lipoma as a dysembryogenetic anomaly: Four unusual cases of ectopic iliac rib within the spinal lipoma.

BACKGROUND: Congenital spinal lipomas are closed spinal dysraphisms belonging to the neural tube defects (NTDs) group. They include a broad spectrum of lesions ranging from simple lipomas of the filum terminale to complex malformations. On histological evaluation, various tissue components of ectodermal, mesodermal or endodermal origin are found within the lipomas, with prevalence for nerves and striated muscle and, more rarely, cartilage and bone. Overall, rib malformations have been occasionally observed in patients with NTDs and in NTD mouse models. However, an ectopic rib arising within the spinal lipoma and articulating with the iliac crest has not been reported in either animal models or in humans. CASES: We describe four patients affected by lipomyeloschisis or lipomyelomeningocele, with an unusual fibrocartilaginous protuberance arising within the lipoma and connecting to one iliac crest, strongly resembling an ectopic rib. Histological evaluation confirmed the presence of cartilaginous tissue. CONCLUSION: We expand the clinical spectrum of fibrocartilaginous anomalies associated with spinal lipoma, suggesting the presence of an ectopic rib as a new possible phenotype in NTDs. A careful analysis by neuroradiologists and pathologists should be performed in spinal lipomas to assess the presence of an ectopic rib or other uncommon developmental anomalies. Furthermore, molecular studies are required to detect the genetic cause of this unusual phenotype. Birth Defects Research (Part A) 106:530-535, 2016. © 2016 Wiley Periodicals, Inc.

Congenital infiltrative lipomas and retroperitoneal perirenal lipomas in a calf.

BACKGROUND: Congenital lipocytic tumours have rarely been reported in cattle. Lipomas are benign tumours, but infiltrative lipomas have significant health implications due to their aggressive infiltrative growth pattern. CASE PRESENTATION: A calf was born with skeletal malformations and soft tissue proliferations, primarily on the external thoracic wall. The calf was euthanized for welfare reasons and submitted for post mortem examination. Necropsy, histopathology and post mortem computed tomography scanning revealed two types of lipocytic tumours. Widespread infiltrative lipomas were present in the muscles and connective tissues along the vertebral column and diffusely invaded the external soft tissues of the right thoracic wall. The neoplastic lipocytes had invaded intervertebral spaces thus causing congenital vertebral malformations, and further invaded the vertebral canal and the bone marrow of coccygeal vertebrae. Periosteal localization of the tumour was associated with costal hyperostosis. Two large retroperitoneal lipomas enclosed the kidneys and occupied much of the abdominal space. CONCLUSION: The development of congenital bone malformation in this calf illustrates the severe consequences of the infiltrative and aggressive growth of infiltrative lipomas during foetal development. The congenital retroperitoneal lipomas occupied a large part of abdominal cavity, but did not invade the adjacent tissues. Due to their large size, perirenal lipomas should be considered in calves with distended abdomen, even in cases without other signs of tumours.

Congenital Sialolipoma in an Infant.

Sialolipoma is a newly recognized tumor of the major and minor salivary glands and represents only 0.3% of all salivary gland tumors. Only 3 cases of congenital sialolipoma are available in the literature. In the current case, we performed a total parotidectomy with facial nerve preservation on a 12-week-old infant exhibiting huge mass in the parotid region. Histopathology results showed sialolipoma. There was no recurrence at the 18-month follow-up. Although it is a very rare disease in infants, congenital sialolipoma should be kept in mind in patients with parotid mass. The primary treatment is parotidectomy with facial nerve preservation.

Massive intrathoracic lipoma: a report of two cases, one being congenital.

Massive intrathoracic lipomas are uncommon. Few cases have been reported worldwide. We report two cases, one of which was congenital. They were managed by thoracotomy and complete excision, with excellent outcomes.

Lower lip cleft, bifid tongue and fibrolipoma: a case report of rare congenital anomaly.

A non-syndromic bifid tongue with fibrolipoma, ankyloglossia, and clefts of the lower lip and alveolus is rare. We describe a case in which a bifid anterior tongue was separated by a fibrolipomatous mass. We excised the mass and repaired the lip and the tongue with satisfactory results.