INNV-24. METASTATIC HEMANGIOPERICYTOMA TO THE LIVER: DEFINING THE IMPORTANCE OF NAB-STAT2 FUSION

BACKGROUND Meningeal hemangiopericytoma (HPC) is a rare and aggressive vascular tumor which typically occurs in younger adults and has a tendency for local recurrence with delayed metastases outside the CNS. HPC is considered a variant of solitary fibrous tumor (SFT), and is associated with the presence of the fusion oncoprotein NAB2-STAT6. This fusion results in over-expression of the EGR1 gene and results in tumorigenesis. CASE REPORT We present a case of a 49 y/o female with a remote history of left occipital lobe HPC, initially treated with resection and radiotherapy in 2007, who presented with symptoms of right upper quadrant abdominal pain. She was found to have a hypervascular 7 cm mass in the right upper lobe of the liver on magnetic resonance imaging (MRI) studies and subsequently underwent hepatectomy for removal of the mass. Histopathological findings revealed metastatic HPC with features consistent with her primary meningeal tumor that was removed over 13 years agoearlier. Presence of the NAB2-STAT6 fusion was confirmed by targeted genomic analysis. MRI revealed a small focus of nodular enhancement abutting the confluence of the superior sagittal sinus and transverse sinus. This was considered her source of disease and possible dissemination. She was treated with stereotactic radiosurgery to target the lesion. DISCUSSION Given the rarity and often remote medical history of the primary intracranial HPC, this report suggests that 1) long term follow up of HPC is essential and 2) detection of the NAB2-STAT6 fusion can identify and confirm recurrent HPC at metastatic sites. In regards to the latter, distinguishing HPCs from other more benign spindle cell tumors that may mimic SFT (such as fibroblastic meningiomas) is also of therapeutic significance as HPCs typically require adjuvant treatment, (i.e. radiotherapy) as opposed to surgical resection alone.

EPIDEMIOLOGICAL PATTERNS OF PAEDIATRIC BRAIN TUMORS IN COIMBATORE

Background: Tumors of the nervous system are the second most common childhood tumors after leukemia,constituting approximately 35% of all childhood malignancies and remain the leading cause of cancer related deaths in children. In India,in the absence of a comprehensive population based national cancer registry,we depend on local hospital based registries for assessing the incidence of pediatric brain tumor.Hence,more and more institutional data are required to assess the actual disease load in India. Objective: The main objective of the present study is to assess the epidemiological patterns of brain tumors in children presenting in CMCH. Materials and Methods: Data regarding age,gender,topography and histopathology of 22 pediatric patients (0-18years)with brain tumors operated inCMCH over a period of 5 years(January 2015 to December 2019)was collected retrospectively and analysed. The results obtained were compared with available Indiandata and western literature. Results: Of 22 cases, males(63.6%) outnumbered females. In the present study, the most common anatomical site for brain tumors was cerebellum(45.5%) followed by cerebral hemispheres(36.2%), ventricles(13.6%) and sellar region(4.5%). The present study showed that Infratentorial tumors were more common (54.5%) as compared to Supratentorial tumors (45.5%).Thepresent study revealed that astrocytoma(36.4%) is the most common brain tumor in childhood. other common tumors include medulloblastoma(27.2%), followed by ependymoma(13.6%), oligodendroglioma(9%),pineal gland tumor(4.5%),craniopharyngioma(4.5%) and meningeal tumor(4.5%). Conclusion: From the present series, we conclude that, the frequencies of major histologic types of brain tumors found in the study do not differ substantially from that found in other developed and developing countries. Medulloblastomas and astrocytomas, which form the major histologic types in pediatric patients need special attention.

Sulcal FLAIR hyperintensity - Finding beyond meningitis

Introduction: The aim of this study was to enumerate the causes of Fluid-attenuated inversion recovery (FLAIR) hyperintensity in the sulcal space which could be due to cerebrospinal fluid (CSF) or non-CSF related pathologies. Methods and Materials: This is an observational retrospective study done in 100 patients in the department of Radio-diagnosis of Patna Medical College and Hospital, Patna from September 2019 to September 2020. Results: The mean age of patients was 40.13 +/- 13.88 years (Range 19 to 75 years). Male to female ratio was 1.32:1.00 (57:43). The most common cause of FLAIR sulcal hyperintensity was infection in 66% cases followed by meningeal tumor deposits in 16%, vascular cause in 6%, subarachnoid hemorrhage in 5%, mass effect in 5%, dermoid rupture in 1% and hyperoxygenation in 1%. Conclusion: Presence of sulcal hyperintensity on FLAIR images is a very strong sign to an underlying brain pathology. A keen observation of the same on FLAIR sequence in association with other findings can increase diagnostic confidence and thus lead to better patient care.

A retro-odontoid pseudotumor treated with fixation and tumor resection by the lateral approach: illustrative case

BACKGROUND A retro-odontoid pseudotumor is not a condition that requires resection. However, pathological diagnosis is required when a tumor such as a meningeal tumor or chordoma is suspected. The authors report a case of a large lesion treated with posterior fixation and tumor resection using a lateral approach. OBSERVATIONS A 77-year-old man visited the authors’ department complaining of neck pain and decreased dexterity of the upper extremities. Magnetic resonance imaging showed a large, beak-shaped lesion behind the dens and severe compression of the spinal cord. Surgery consisted of occipitocervical–C2 fixation, followed by tumor resection with a left lateral approach. The pathological diagnosis was consistent with a retro-odontoid pseudotumor. The tumor was resected to a relatively large extent and shrank over time, leading to complete disappearance. LESSONS Pathological examination is also possible with a posterior approach if the tumor can be reached through the lateral edge of the dura. In that situation, the amount of resection is limited, and there is a risk of spinal cord compression. Intradural dissemination of tumors is a concern with the transdural approach. If tumor resection by the posterior approach is difficult, the lateral approach can facilitate tumor resection.

Subcutaneous seeding following surgical excision of an intracranial meningioma in a cat

Traditionally patient owners express their concerns that surgical or diagnostic procedures on a tumor may induce metastasis. In pets, this has been documented in only very rare occasions, e. g. needle path metastases after diagnostic fine needle biopsies of urinary bladder or prostatic tumors. Here, we describe a case of subcutaneous seeding of a feline intracranial grade 1 meningioma 6 months after surgical resection. A 10-year-old male neutered domestic shorthaired cat with typical neurological signs was diagnosed with an extra-axial contrast enhancing mass in the dorsal frontotemporal lobes using magnetic resonance imaging (MRI). Transfronto-parietal bone craniotomy was performed and the 24 × 19 × 22 mm large tumor was largely removed. Tumor recurrence after 12 months resulted in a second surgical tumor removal. In addition, 2 subcutaneous masses of 10 × 4 × 4 mm in size were removed at the site of the original surgical site which were fully separated from the recurring meningeal tumor by the intact frontal bone. Histology and immunohistochemistry suggested the same tumor growth in all 4 masses. Most likely the tumor seeding had been caused during the first surgery. After all, the risk of surgical seeding of a benign tumor seems very low.

Case Report: A Malignant Liver and Thoracic Solitary Fibrous Tumor: A 10-Year Journey From the Brain to the Liver and the Spine

Solitary fibrous tumors are rare neoplasms that originate from mesenchymal tissues and have been found to occur in any site, including the spine and liver. Although most of solitary fibrous tumors have benign features, only 10–20% are malignant and prone to metastasis. No previous reports have described the malignant and metastatic Solitary fibrous tumor arising in both of the liver and thoracic vertebrae. In this article, we present the case of a 60-year-old woman who underwent gross total resection of a meningeal tumor in 2007. She presented 10 years later with a thoracic vertebral mass that caused relentless pain and a lesion in the right lobe of liver. She underwent marginal excision of the T3 tumor with T2-4 pedicular screw fixation in March 2017, then right hemi-hepatectomy was performed to remove the liver lesion in June 2017. Both of the lesions were confirmed to be a metastatic and malignant tumor after surgery. The literature lacks randomized controlled trials and large studies that define the natural history of malignant solitary fibrous tumors and recommendations of precise management plan for the disease. However, the best choice for treatment is gross total resection, which probably provide the optimal treatment to achieve long-term disease-free survival.

PRIMARY THORACIC INTRASPINAL HEMANGIOPERICYTOMA MIMICS MENINGEAL TUMOR: A CASE REPORT AND LITERATURE REVIEW

Hemangiopericytoma (HPC) accounts for less than 1% of all central nervous system tumors and is particularly rare in the spinal canal. HPC mimics other CNS intradural tumor radiologically. We present a rare case of intraspinal HPC initially diagnosed as thoracic intraspinal meningeal tumor radiologically. The patient was treated with T8–T9 partial laminectomy and en bloc tumor removal. Radiation therapy for tumor bed was prescribed. The patient had symptom recovery postoperatively, and remained free from local recurrence in the 3 years follow-up. Literature reviews focused on tumor behavior and treatment policy.