Intracranial pilocytic astrocytoma with spinal drop metastasis: A case review, discussion of its current management and related literature

Pilocytic astrocytoma (PA) is a low grade benign tumor, commonly occurs in Cerebellum (42-60%), Optic & hypothalamic region (9-30%), Brain Stem (9%). They rarely spread. It rarely occurs within the ventricle. In this report, we described our experience with cranial pilocytic astrocytoma in a 14-year-old child, who initially treated with near total decompression of tumor. Four and half years later, recurrence of primary tumor with obstructive hydrocephalus occurred for which he underwent ventriculo-peritoneal shunt followed by re-excision. As further treatment was being evaluated for radiotherapy in view of recurrence at the primary site, he got detected to have significant spinal drop metastasis seen on MRI Spine. Finally, he underwent craniospinal irradiation (CSI).

Sporadic Intradural Extramedullary Hemangioblastoma of Cauda Equina with Large Peritumoral Cyst—A Rare Presentation

Cauda equina intradural tumors commonly reported include ependymoma, schwannoma, neurofibroma, meningioma, and drop metastasis. Hemangioblastoma of the neural axis is a rare benign vascular tumor comprising only 1.6 to 6.4% of spinal tumors, and are usually associated with Von-Hippel Lindau disease. Sporadic intradural extramedullary hemangioblastoma involving cauda equina is very rare with only countable reports, and the presence of peritumoral cyst has been reported only once. We report one such case of hemangioblastoma with a large peritumoral cyst, which was diagnosed radiologically and confirmed by histopathology following surgical excision. Pertinent radiological characteristics, diagnostic clues, treatment, and surgical outcomes are discussed.

Anaplastic pilocytic astrocytoma of the cerebellum presenting with conus medullaris drop metastasis

Due to its rarity, a complete understanding of the clinical behavior, pathogenesis, and diagnostic definition of anaplastic pilocytic astrocytoma (APA) is currently lacking. The optimal clinical management and use of adjuvant therapies has yet to be defined. We present a 64 year-old-female with progressive headaches, dysarthria, and ataxia, who was found to have right cerebellar mass. A gross total resection was achieved through two staged operations. Pathology demonstrated focal areas of necrosis, tumor infiltration, and increased mitotic activity most consistent with APA. Adjuvant chemotherapy and stereotactic radiosurgery were administered. Approximately two years later, the patient presented with symptoms of cauda equina syndrome, and lumbar spine imaging demonstrated a large intradural mass at the conus medullaris with diffuse leptomeningeal enhancement. A biopsy was performed and was consistent with metastatic APA. APA may rarely progress to metastatic disease, most frequently involving the leptomeninges of the posterior fossa and cervical spine. This report represents the first case of metastases distal to the cervicomedullary junction.

Cerebrospinal Fluid Drop Metastases of Canine Glioma: Magnetic Resonance Imaging Classification

Dissemination of glioma in humans can occur as leptomeningeal nodules, diffuse leptomeningeal lesions, or ependymal lesions. Cerebrospinal fluid (CSF) drop metastasis of glioma is not well-recognized in dogs. Ten dogs with at least two anatomically distinct and histologically confirmed foci of glioma were included in this study. The 10 dogs underwent 28 magnetic resonance imaging (MRI) examinations, with distant CSF drop metastasis revealed in 13 MRIs. The CSF drop metastases appeared as leptomeningeal nodules in four dogs, diffuse leptomeningeal lesions in six dogs, and ependymal lesions in seven dogs; six dogs had a combination of lesion types. Primary tumors were generally T2-heterogeneous and contrast-enhancing. Many metastases were T2-homogeneous and non-enhancing. Diffuse leptomeningeal lesions were seen as widespread extra-axial contrast-enhancement, again very dissimilar to the intra-axial primary mass. Primary masses were rostrotentorial, whereas metastases generally occurred in the direction of CSF flow, in ventricles, CSF cisterns, and the central canal or leptomeninges of the cervical or thoracolumbar spinal cord. Seven of the dogs had received therapy limited to the primary mass, such as surgery or stereotactic radiation, then developed metastasis in the following months. CSF drop metastasis of glioma may take a very different appearance on MRI to the primary mass, including periventricular lesions that are more homogeneous and less contrast-enhancing, rostral horn signal changes, or leptomeningeal enhancement ventral to the brainstem or encircling the spinal cord.

Recurrence-free survival following aggressive multimodal treatment of an isolated recurrence of adrenocortical carcinoma

A 43-year old woman demonstrated an intra-abdominal drop metastasis 6 years after initial presentation of stage-III adrenocortical carcinoma (ACC), previously treated with resection, adjuvant radiation and mitotane therapy. This recurrence was managed aggressively with complete excision, adjuvant radiation and mitotane. Imaging at 3.25-year follow-up showed no evidence of recurrence, a remarkable response given ACC’s poor prognosis and high recurrence rates. Management of oligometastatic ACC remains unclear and intra-abdominal drop metastases are particularly rare in ACC; both require further documentation. Aggressive management with adjuvant radiation and mitotane therapy may represent an effective and well-tolerated approach for improving local control for recurrent ACC, including drop metastases. Further research is required to codify potential benefits.

Successful Multimodality Management of Atypical Teratoid/Rhabdoid Tumour of the Lower Dorsal Spine with Spinal Drop Metastasis: Illustrated Review

<b><i>Introduction:</i></b> Spinal atypical teratoid/rhabdoid tumour (AT/RT) is exquisitely rare and constitutes 2% of all AT/RTs. <b><i>Case Presentation:</i></b> A 6-year-old boy presented with low backache for the last 5 months. MRI of the spine showed a 1.5 × 1.5 × 4.7 cm intradural extramedullary mass extending from D10 to D12, causing compression of the conus medullaris. With a preoperative diagnosis of ependymoma, a gross total resection (GTR) of tumour was performed. Post-operative histopathology showed AT/RT. The tumour cells were immunopositive for cytokeratin, epithelial membrane antigen, smooth muscle actin, and p53 and immunonegative for MIC2, desmin, glial fibrillary acidic protein, and INI1. Post-operative neuraxis MRI revealed post-operative changes (D10–D12) with a 9 mm enhancing lesion at L5-S1 junction suggesting drop metastasis. There was no lesion in brain. Cerebrospinal fluid cytology did not show any malignant cell. The metastatic work-up was normal. He received 3 cycles of chemotherapy with ICE regimen (ifosfamide, carboplatin, and etoposide). Subsequently, he received craniospinal irradiation (CSI)-36 Gy/20 fractions/4 weeks followed by focal boost to primary tumour bed and spinal drop metastasis-14.4 Gy/8 fractions/1.5 weeks. Thereafter, he received 3 more cycles of ICE regimen. End-of-treatment MRI spine showed post-op changes (D10–D12) and 38.9% reduction of the L5-S1 lesion suggesting partial response. Six monthly spinal MRI showed serial reduction of the metastatic lesion leading to complete response (CR) 1 year after completion of treatment. On last follow-up (30 months from the initial diagnosis), he was neurologically intact and in CR. <b><i>Conclusion:</i></b> Multimodality management comprising GTR of tumour, CSI followed by focal boost, and multiagent chemotherapy (ICE) can lead to successful outcome in patients with this rare and aggressive spinal tumour.

ATRT-31. SUCCESSFUL MULTIMODALITY MANAGEMENT OF ATRT OF THE LOWER DORSAL SPINE WITH SPINAL DROP METASTASIS

A 6 year old boy presented with low backache for the last 5 months. MRI of the spine showed a 1.5x1.5x4.7cm intradural mass extending from D10-D12, causing compression of the conus medullaris. With a preoperative diagnosis of intradural ependymoma, a gross total resection (GTR) of tumour was performed. Post-operative histopathology showed a markedly cellular, malignant tumour with frequent mitotic figures. Cells were round to polygonal with vesicular nuclei, prominent nucleoli and were immunopositive for CK,EMA,p53 and immunonegative for MIC2,desmin,SMA,GFAP,INI-1(MIB1 labeling index-35–40%). The overall impression was spinal atypical teratoid rhabdoid tumour(ATRT). Post-operative neuraxis MRI revealed post-operative changes(D10-D12) with a 9 mm enhancing lesion at L5-S1 junction suggesting drop metastasis. There was no brain lesion. CSF cytology did not show any malignant cell. The metastatic work-up was normal. He was started on chemotherapy with ICE regimen (Ifosfamide-2g/m2IVD1–D3,Carboplatin-500mg/m2IVD3,Etoposide-100mg/m2IVD1–D3q3weeks). Subsequently he received craniospinal irradiation (CSI)-36Gray/20fractions/4weeks→ focal boost to primary tumour bed and spinal drop metastasis-14.4Gray/8fractions/1.5 weeks. Thereafter he received 3 more cycles of ICE regimen. End-of-treatment MRI spine showed post-op changes(D10-D12) and 38.9%reduction of the L5-S1 lesion suggesting partial response. Six monthly spinal MRI showed serial reduction of the metastatic lesion leading to complete response (CR) 1 year after completion of treatment. On last follow-up (30 months from initial diagnosis), he was neurologically intact and in CR. Multimodality management comprising GTR,CSI followed by focal boost and multiagent chemotherapy(ICE) can lead to successful outcome in patients with this rare and aggressive spinal tumour.