Pediatric and Adult Low-Grade Gliomas: Where Do the Differences Lie?

Two thirds of pediatric gliomas are classified as low-grade (LGG), while in adults only around 20% of gliomas are low-grade. However, these tumors do not only differ in their incidence but also in their location, behavior and, subsequently, treatment. Pediatric LGG constitute 65% of pilocytic astrocytomas, while in adults the most commonly found histology is diffuse low-grade glioma (WHO II), which mostly occurs in eloquent regions of the brain, while its pediatric counterpart is frequently found in the infratentorial compartment. The different tumor locations require different skillsets from neurosurgeons. In adult LGG, a common practice is awake surgery, which is rarely performed on children. On the other hand, pediatric neurosurgeons are more commonly confronted with infratentorial tumors causing hydrocephalus, which more often require endoscopic or shunt procedures to restore the cerebrospinal fluid flow. In adult and pediatric LGG surgery, gross total excision is the primary treatment strategy. Only tumor recurrences or progression warrant adjuvant therapy with either chemo- or radiotherapy. In pediatric LGG, MEK inhibitors have shown promising initial results in treating recurrent LGG and several ongoing trials are investigating their role and safety. Moreover, predisposition syndromes, such as neurofibromatosis or tuberous sclerosis complex, can increase the risk of developing LGG in children, while in adults, usually no tumor growth in these syndromes is observed. In this review, we discuss and compare the differences between pediatric and adult LGG, emphasizing that pediatric LGG should not be approached and managed in the same way as adult LCG.

Extradural malignant peripheral nerve sheath tumor of the thoracic spine: A rare case report

Background: Malignant peripheral nerve sheath tumors (MPNSTs) typically found in the trunk, limbs, head, and neck represent 3–10% of all soft-tissue sarcomas. Although they typically originating from peripheral nerve Schwann cells, 2–3% arise from the spinal nerves and may be found within the spinal canal. Here, we present a 43-year-old male with an extradural thoracic MPNST contributing to marked cord compression and a progressive paraparesis. Case Description: A 43-year-old male presented with a progressive paraparesis of 16 months’ duration. The MRI showed a posterior T2-T4 extradural tumor in the thoracic spine resulting in significant cord compression. Following a T2-T4 laminectomy and gross total excision of the epidural mass, the patient regained modest neurological function. Immunohistochemistry staining supported the diagnosis of thoracic spinal MPNST. Conclusion: Rarely, spinal MPNST can be considered amongst the differential diagnoses of an extradural spinal tumor. In this case, gross total excision of a posterior T2-T4 epidural MPNST resulted in improvement in the patient’s original paraparesis. Notably, immunohistochemistry staining helped confirm the diagnosis of a MPNST.

Surgical Outcome of Giant Vestibular Schwannomas: A Retrospective Analysis

Background Giant vestibular schwannomas, more than 4.5 cm in diameter, are still representing the majority of vestibular schwannomas in developing countries. The philosophy of incomplete and intracapsular tumor excision has been introduced in the management of these giant tumors, balancing the long-term tumor control and postoperative clinical outcome. Objective The aim was to review the cases with giant vestibular schwannomas and studying their prevalence, morbidity, and mortality rates. Patients and Methods This study was conducted retrospectively on data of patients who had undergone microsurgical excision of vestibular schwannomas in our institute between January and December 2017. The functional outcome of the patients was assessed by comparing the preoperative and the postoperative neurological examination, as well as the Karnofsky performance score. Results Twenty-two cases with vestibular schwannomas were included in this study. Among these 22 cases, 15 cases had giant vestibular schwannomas (68.2%). The tumors’ largest extracanalicular diameters ranged from 4.5 cm to 6.2 cm. Postoperative images showed gross total excision of the tumor in 11 cases (73.3%), and subtotal excisions in four cases (26.7%). Twelve cases (80%) had postoperative facial palsy. We encountered no mortality in our cases and three cases (20%) were symptom-free postoperatively (apart from hearing affection). Conclusion Large and giant vestibular schwannomas are still commonly met in neurosurgical practice in developing countries; they have different behaviors and presentations from those of smaller tumors. Both patient and surgeon expectations from surgery should be toward no mortality and mild or no morbidities.

Management of recurrent schwannoma of the cauda equina: A case report

Background: Schwannomas of the cauda equina are rare intradural primary spinal tumors. Many of these patients initially present with cauda equina syndromes, and only 2.2% demonstrate clinical recurrence. Gross total excision is the procedure of choice. Case Description: A 62-year-old female had undergone resection of a cauda equina schwannoma 5 years previously. She newly presented with cauda equina symptoms attributed to a recurrent schwannoma. Following gross total secondary tumor resection, the patient’s preoperative deficits fully resolved, and the tumor never recurred. Conclusion: Secondary gross total excision of schwannomas of the cauda equina is critical to avoid further tumor recurrence.

Surgical considerations for maximal safe resection of exophytic brainstem glioma in the pediatric age group

Background: Brainstem glioma is the leading cause of morbidity and mortality among all central nervous system tumors, especially in childhood as it represents about 20% of all pediatric brain tumors. Therefore, this study aimed to present our experience in a tertiary center in a developing country with limited resources for the surgical management of exophytic brainstem gliomas. Methods: This retrospective study included pediatric patients with brainstem (midbrain, pontine, or medullary) focal or diffuse gliomas whether low or high grade that had dorsal, ventral, or lateral exophytic component who were presented to our hospitals from January 2019 to January 2021. The patients’ data were collected, such as age, sex, preoperative and postoperative clinical condition, radiological data, surgical approach, extent of tumor removal, histopathology, follow-up period, and adjuvant therapy. Results: A total of 23 patients were included in this study. The telovelar approach was used in 17 patients, the supracerebellar infratentorial approach in three patients, and the retrosigmoid, transcerebellar, and occipital transtentorial approach once for each patient. Twenty patients underwent near-total excision, and three underwent subtotal excision. Two-thirds of our cases (17 patients) were low-grade gliomas, with the remaining one-third comprising entirely of either anaplastic astrocytoma (five patients) or glioblastoma multiforme (one patient). The follow-up period of the patients extended from 3 months to 24 months. Conclusion: Exophytic brainstem glioma surgery can result in good outcomes with minimal complications when near-total excision is attempted through a properly chosen approach and adherence to some surgical techniques and considerations.

Xanthoma of Tendoachilles: Management and Follow-Up

Xanthoma is a rare pseudotumor of connective tissue, consisting of histiocytes with lipid deposits. Usually, it manifests as encapsulated lobular yellowish mass, often associated with familial hypercholesterolemia (FH). We present a case of xanthoma of tendoachilles just proximal to its insertion site, secondary to FH. The patient was a 28-year-old female presenting with a 2-year history of difficulty ambulating and complaints of swelling over the posterior aspect of her left lower leg just proximal to her heel. She had a deranged lipid profile level and was started on a lipid lowering agent for 3 weeks followed by total excision of the lesion with flexor hallucis longus tendon graft augmentation. The patient was able to bear full weight at 6 weeks and could stand on her toes by 4 months. No recurrences were noted at 1 year follow-up.

Extra-axial tentorial medulloblastoma: a rare presentation of a common posterior fossa tumour

Medulloblastoma is a common paediatric posterior fossa tumour typically presenting as midline intra-axial mass involving the cerebellar vermis and/or roof of fourth ventricle with typical radiological features. These can be extra-axial in extremely rare instances with less than 50 cases reported so far in literature. We present a case of 18-year-old boy presenting with ataxia and headache. MRI showed dural mass (involving the left tentorium cerebellum) with typical imaging features of extra-axial lesion. The patient underwent near total excision of the tumour. Histopathology along with immunohistochemistry revealed the mass to be medulloblastoma. We present this case to highlight rarity of this location for medulloblastoma and the importance of considering this in the differential diagnosis of atypical posterior fossa extra-axial lesions. This can help in performing other relevant preoperative workup similar on the lines of medulloblastoma and planning of relevant management.

An unusual presentation of acute abdomen: infarcted peritoneal cyst-a probable asbestos-related benign cystic mesothelioma

ABSTRACT This is a report of a rare case of an infarcted pelvic intra-abdominal cyst, having no mesenteric connection presenting as an acute abdomen. The patient had significant asbestos exposure. The cyst was treated successfully by surgical excision. Histopathology showed an infarcted cyst; the lining was destroyed, precluding marker studies. A diagnosis of benign cystic peritoneal mesothelioma (BCPM) was made by excluding other causes of solitary pelvic intra-abdominal cysts. BCPM has been classified as an asbestos-related neoplasm and is usually seen in the pelvis adjunct to the urinary bladder. One-year post-surgery, there was no recurrence. The case report shows that infarcted pelvic mesothelial cysts can present as an acute abdomen and can be treated successfully by total excision with no recurrence.