CASE REPORT |
https://doi.org/10.5005/jp-journals-11003-0145 |
A Rare Case of Corpus Callosum Lipoma with Corpus Callosum Dysgenesis and Subcutaneous Lipoma
1,2Department of Radiodiagnosis, Airforce Hospital, Jorhat, Assam, India
3Department of Pediatrics, Airforce Hospital, Jorhat, Assam, India
4Department of Anesthesiology, Airforce Hospital, Jorhat, Assam, India
Corresponding Author: Tooba Maryam, Department of Radiodiagnosis, Airforce Hospital, Jorhat, Assam, India, Phone: +91 9923359878, e-mail: tooba285@gmail.com
Received: 24 May 2024; Accepted: 14 June 2024; Published on: 31 December 2024
ABSTRACT
Background: Uncommon neoplasms seen within the brain include corpus callosum lipoma and pericallosal lipoma. Their percentage of all cerebral tumor lesions is <1%. The development of adipocytes from primitive meninges that persist into adulthood is the root cause of many conditions. Nevertheless, the exact cause of this lesion remains unknown. The Sylvian cistern, superior cerebellar cistern, suprasellar cistern, and quadrigeminal cistern are some other possible sites. Although headache is a rare symptom, many lesions are painless.
Case description: These parents took their 6-week-old baby to the doctor because their child had been experiencing nongrowing edema in the frontal region since birth. Upon external inspection, a soft swelling was detected in the midline of the frontal region. To characterize the lesion, ultrasonography (USG) was performed. On USG, a hyperechoic lesion was observed in the midline subcutaneous area of the anterior region. A hyperechoic lesion in the corpus callosum region with a hyperechoic stalk that extended into the subcutaneous region was seen on transcranial ultrasound. The results were validated by magnetic resonance imaging (MRI) scan. A stalk was found to be attached to the corpus callosum lipoma on the MRI, which was located in the frontal region and revealed it on the subcutaneous plane. Dysgenesis of the corpus callosum was also seen. As a result, a diagnosis of corpus callosum dysgenesis and subcutaneous lipoma was rendered.
Conclusion: The patient was kept on follow-up as it is an incidental finding. It is important to image any lesion in the head region to rule out the possibility of intracranial extension or intracranial complications.
Keywords: Case report, Magnetic resonance imaging, Ultrasound
How to cite this article: Maryam T, Raju AG, Bhargava H, et al. A Rare Case of Corpus Callosum Lipoma with Corpus Callosum Dysgenesis and Subcutaneous Lipoma. J Med Acad 2024;7(2):70–73.
Source of support: Nil
Conflict of interest: None
Patient consent statement: The author(s) have obtained written informed consent from the patient’s parents/legal guardians for publication of the case report details and related images.
BACKGROUND
Intracranial neoplasms of the corpus callosum or pericallosal type are extremely uncommon. Among tumors found within the brain, they constitute fewer than 1%.1 Their development into adipocytes is a result of the aberrant persistence of primitive meninges.2,3 Nevertheless, it remains uncertain where this lesion originated. Additionally, there are Sylvian, superior cerebellar, suprasellar, and quadrigeminal cisterns. Although headache is an uncommon symptom, the majority of lesions do not cause any discomfort.4,5 It is less common for there to be an intracranial lipoma that also has an exterior subcutaneous extension. We describe here the case of a 6-week-old baby who showed signs of subcutaneous edema with an intracranial component. To corroborate the diagnosis, a multimodal strategy was utilized.
CASE DESCRIPTION
The parents presented their 6-week-old child, who had been experiencing edema in the frontal region ever since birth. The pregnancy went smoothly, and the baby was born at the scheduled time. The baby weighed 3 kg at birth. The infant needed only breast milk. A soft, midline enlargement in the frontal region was seen during the clinical examination. To identify the condition and determine the extent of the swelling, the patient was subsequently sent for ultrasound. A clearly marked hyperechoic lesion in the subcutaneous plane of the frontal region, with a maximum diameter of 6 mm, was detected on ultrasonography (USG). The extent of the injury was determined to extend throughout the brain. Additionally, a 22 × 5 mm hyperechoic lesion was observed in the corpus callosum region (AP × ML). The subcutaneous plane revealed a hyperechoic stalk that began at the corpus callosum and continued to the lesion. Subcutaneous lipoma with corpus callosum lipoma was thus diagnosed on USG (Figs 1 and 2).
Figs 1A and B: Midline swelling in the frontal region of the infant
Figs 2A to C: A well-defined hyperechoic lesion noted in the region of the corpus callosum, with a hyperechoic stalk extending up to the subcutaneous plane. Hyperechoic lesions are also observed in the subcutaneous plane, suggestive of corpus callosum lipoma with subcutaneous lipoma
To validate the results of the USG and to search for any related structural abnormalities, a brain MRI was performed. A 22 × 6 mm (AP × ML) lesion with altered signal intensity was detected on magnetic resonance imaging (MRI) in the pericallosal area. While the lesion appears hyperintense on T1-weighted imaging (T1WI) and moderate on T2WI, it shows blooming on gradient recalled echo (GRE) and is completely suppressed on fat saturation imaging. The lesion in the pericallosal region is accompanied by a lipomatous tract that extends to the subcutaneous plane in the frontal region. The maximal width of the lesion in the subcutaneous plane is 6 mm, and its intensity is similar to that in the pericallosal region. When the splenium of the corpus callosum cannot be seen, it is also known as associated corpus callosum dysgenesis. Everything else within the skull appears to be normal. There was no evidence of cortical abnormality (Fig. 3).
Figs 3A to E: (A) T1-weighted imaging axial image shows hyperintense lesion (blue arrow) in the midline measuring 22 × 6 mm; (B) Fluid-attenuated inversion recovery (FLAIR) axial image shows a hyperintense lesion (red arrow) in the midline measuring 22 × 6 mm; (C) Gradient-recalled echo (GRE) axial image shows the lesion (blue arrow) exhibiting blooming artifacts; (D) T1-weighted sagittal image shows a hyperintense lesion in the pericallosal region (red arrow) with extension through the lipomatous stalk (yellow arrow) into the subcutaneous plane (green arrow). The lesion in the subcutaneous plane exhibits similar intensity; (E) T2-weighted sagittal image shows nonvisualization of the splenium of the corpus callosum (blue arrow)
MANAGEMENT
The patient was kept on follow-up as he was asymptomatic.
DISCUSSION
Uncommon neoplasms seen within the brain include corpus callosum lipoma and pericallosal lipoma. Among tumors found within the brain, they constitute fewer than 1%.1 The aberrant persistence of primitive meninges, which eventually transforms into adipocytes, is the root cause of many conditions.2,3 Nevertheless, the exact cause of this lesion remains unknown. The Sylvian cistern, superior cerebellar cistern, suprasellar cistern, and quadrigeminal cistern are some other possible sites. Although headaches are uncommon, they can occur in rare cases among asymptomatic lesions.1,5,6 Fewer case studies have described intracranial lipomas with extracranial subcutaneous extensions; for example, Mitilian et al.7 and Chen et al.8 have recorded three such cases.9
There are two main categories of intracranial lipomas, as described by Tart and Quisling10—curvilinear and tubulonodular. Smaller in size and arranged in a linear fashion, curvilinear lipomas tend to be placed further back in the body. Tubulonodular lipomas, on the contrary, are more commonly found anteriorly and have a spherical or cylindrical shape. Most cases of the tubulonodular type have a midline tract defect and an expansion of the subcutaneous tissue into the frontal region.11
From a lack of direct connection6 to a fibrotic-lipomatous connection12,13 and finally a direct connection through the cranium,11 several communication patterns are noted between the intracranial and extracranial components of the lipoma. The dehiscence of the skull, together with the probable evagination of a small tuft of meninx primitiva, is the explanation put forth by Truwit and Barkovich3 for direct extension through the skull. Intracranial lipomas can spread to the subcutaneous tissue through a scalp defect.
Although most lipomas do not cause any noticeable symptoms, in rare cases they can be linked to neurological issues such as cerebral palsy, seizures, psychosomatic illnesses, and motor difficulties. The most prevalent symptoms are headaches and seizures. Obstructive hydrocephalus is a potential complication of seizures, which often begin before the age of 15.6 It is more common for women to experience these symptoms. These conditions are now more frequently detected starting at 26 weeks of gestation due to increased use of prenatal USG monitoring.1,5 The CT scan, which shows calcifications most commonly in the tubulonodular form, is used to make the clinical diagnosis.1,5 For accurate lesion characterization and size assessment, as well as for evaluating associated features and dysgenesis or agenesis of the corpus callosum, MRI is the examination of choice. All sequences demonstrate the lesion’s fatty intensity, with T1 and T2 sequences showing hyperintensity. It also shows suppression on fat saturation (fat SAT) sequences. If an electroencephalogram (EEG) is performed on a patient experiencing seizures, it may help identify the epileptic focus. Dermoid cysts and teratoma are among the possible alternative diagnoses. Unless hydrocephalus is present, surgical techniques are not advised for treating such anomalies.5,14 The strong vascularity, adhesion, and infiltration of the capsule into neighboring tissues, coupled with the fact that these lesions typically do not expand, are reasons why surgical intervention is not recommended for them.1,3,5,14 When dealing with individuals presenting with seizures, most literature reviews recommend anticonvulsant therapy for symptomatic relief. For patients experiencing developmental delays, recommendations include anti-migraine prophylaxis and interdisciplinary stimulation.
LEARNING POINTS
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There are two distinct types of lipomas in the corpus callosum, distinguished by their location and morphology—posterior lipomas and anterior (tubulonodular) lipomas.
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A majority of these lipomas do not cause any symptoms, but those that do can include epilepsy, cerebral palsy, seizures, persistent headaches, mental or motor impairments, and hypogenesis or agenesis of the corpus callosum.
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Additionally, the tubulonodular type of anterior lipoma is more commonly associated with these abnormalities.
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Clinical suspicion may be enhanced by the use of diagnostic tools such as EEGs.
CONCLUSION
Irrespective of the patient’s age, a comprehensive multimodality investigation of any scalp lesion should be performed to exclude cerebral pathology.
ORCID
Tooba Maryam https://orcid.org/0009-0004-0606-0976
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