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Cranial and Spinal Leptomeningeal Dissemination In Surgical Neurology International OPEN ACCESS Editor: S. A. Enam, MD SNI: Unique Case Observations, a supplement to Surgical Neurology International For entire Editorial Board visit : Aga Kahn University; Karachi, http://www.surgicalneurologyint.com Sindh, Pakistan Cranial and spinal leptomeningeal dissemination in esthesioneuroblastoma: Two reports of distant central nervous system metastasis and rationale for treatment Walavan Sivakumar, Nathan Oh1, Aaron Cutler, Howard Colman, William T. Couldwell Department of Neurosurgery, Clinical Neurosciences Center, University of Utah, Salt Lake City, Utah 84132, 1Department of Neurosurgery, Loma Linda University, Loma Linda, California 92354, USA E‑mail: Walavan Sivakumar ‑ [email protected]; Nathan Oh ‑ [email protected]; Aaron Cutler ‑ [email protected]; Howard Colman ‑ [email protected]; *William T. Couldwell ‑ [email protected] *Corresponding author Received: 18 June 15 Accepted: 08 October 15 Published: 25 November 15 Abstract Background: Esthesioneuroblastoma is a locally aggressive cancer of the nasal cavity. While systemic metastasis can occur in 10-30% of patients, there are only six reported cases of distal metastasis from leptomeningeal dissemination. Access this article online Case Description: The authors report two cases of esthesioneuroblastoma treated Website: previously with multimodal therapy in which distal metastatic recurrence was found www.surgicalneurologyint.com and describe their treatment protocol, which has resulted in long-term success. DOI: 10.4103/2152-7806.170464 Conclusion: Understanding the drivers of leptomeningeal dissemination in Quick Response Code: more prevalent primary neuroectodermal tumors may hold the key to developing successful treatment algorithms for this disease. Key Words: Carcinomatosis, chemotherapy, esthesioneuroblastoma, leptomeningeal dissemination, olfactory neuroblastoma, primary neuroectodermal tumor, radiation, surgery INTRODUCTION leptomeningeal dissemination is an extremely rare sequela with a grave prognosis.[21] We describe our treatment Esthesioneuroblastoma, or olfactory neuroblastoma, protocol for two cases of esthesioneuroblastoma treated is a locally aggressive cancer of the nasal cavity.[4,10,23] previously with multimodal therapy in which the patients First described in 1924,[4] its estimated incidence is experienced distal leptomeningeal metastatic recurrence; 0.4/million.[15,22] The tumor originates from the olfactory in one patient, impressive progression‑free and long‑term epithelial cells and tends to invade the paranasal sinuses, survival were achieved. bony orbits, cribriform plate, and anterior cranial [1,7,13] fossa. Patients typically present with unilateral nasal This is an open access article distributed under the terms of the Creative Commons obstruction and/or epistaxis.[10] While there is variability Attribution-NonCommercial-ShareAlike 3.0 License, which allows others to remix, in the treatment modality chosen for patients, it typically tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms. involves surgery and radiotherapy, with or without For reprints contact: [email protected] chemotherapy.[10] Histopathologically, there are typically sheets of round blue cells with uniform round nuclei. How to cite this article: Sivakumar W, Oh N, Cutler A, Colman H, Couldwell [10] WT. Cranial and spinal leptomeningeal dissemination in esthesioneuroblastoma: Two There can be either true or pseudorosette formation. reports of distant central nervous system metastasis and rationale for treatment. Surg Neurol Int 2015;6:S628-32. Systemic metastases occur in 10–30% of patients, http://surgicalneurologyint.com/Cranial-and-spinal-leptomeningeal-dissemination-in- usually by hematogenous and lymphatic spread,[2,12,20] esthesioneuroblastoma:-Two-reports-of-distant-central-nervous-system-metastasis- but distal central nervous system (CNS) metastasis from and-rationale-for-treatment/ S628 © 2015 Surgical Neurology International | Published by Wolters Kluwer - Medknow SNI: Unique Case Observations 2015, Vol 6: Suppl 25 - A Supplement to Surgical Neurology International CASE REPORTS Patient 1 A 48‑year‑old man was referred to our clinic for evaluation of a new dural‑based lesion 6 years after excision, skin flap repair, and bone graft placement for a skull‑base esthesioneuroblastoma followed by adjuvant radiation. His posttreatment course was complicated by externalization of the bone flap requiring a second skull‑base bone flap resection. After 5 years of no radiographic evidence of residual disease, a routine follow‑up magnetic resonance imaging (MRI) scan at 6 years revealed an enhancing a b dural lesion over the right frontal lobes [Figure 1]. Figure 1: Axial (a) and coronal (b) T1-weighted magnetic resonance imaging showing contrast-enhancing dural-based lesion with The patient underwent a right frontotemporal craniotomy parenchymal invasion along the right frontal lobe (arrow) for resection of the enhancing dura and tumor, which was found to be partially invading the brain parenchyma. Pathological analysis indicated the lesion was consistent with recurrent metastatic esthesioneuroblastoma. The patient received adjuvant stereotactic radiosurgery. His follow‑up MRIs approximately every 6 months remained negative until additional dural‑based lesions along the frontal sinus, right temporal lobe, and right sphenoid wing were observed at 3 years [Figure 2]. Follow‑up a b c imaging 5 months later revealed progressive interval Figure 2: Axial (a), coronal (b), and sagittal (c) T1-weighted magnetic growth of the dural‑based lesions. Treatment with cisplatin resonance imaging showing contrast-enhancing dural-based lesions and etoposide was stopped after one cycle because of through the frontal sinus and along the falx cerebri (a), right temporal lobe (b), and right sphenoid wing (c) (arrows) intractable nausea and vomiting. Approximately 1 year later, the patient restarted treatment with sunitinib, but the tumor showed clear progression 12 months later and the drug was discontinued. The patient succumbed to the disease 6 months later, 11 years after his initial diagnosis. Patient 2 A 48‑year‑old woman presented with a large sinonasal mass with extensive intracranial extension through the cribriform plate into the anterior cranial fossa [Figure 3]. Tumor was noted in the right orbit and maxillary and sphenoid sinuses and there was lymphatic spread to a retropharyngeal lymph node. She underwent radical resection of the Kadish stage D esthesioneuroblastoma a b with a right orbital exenteration and upper neck Figure 3: Axial (a) and sagittal (b) T1-weighted magnetic resonance dissection followed by adjuvant radiation therapy. Yearly imaging showing a contrast-enhancing sinonasal mass with surveillance imaging was stable until a 6‑year follow‑up intracranial extension through the cribriform plate into the anterior MRI revealed 1 cm of patchy enhancement in the cranial fossa, maxillary, and sphenoid sinuses right frontal lobe and the dura overlying the resection cavity. Follow‑up imaging 6 months later showed mildly carefully dissected and removed en bloc. Two days later, increased dural enhancement and interval development the patient underwent T8–T10 laminectomies, left T9 of a new dural‑based lesion in the posterior fossa. costotransversectomy, and ligation of the nerve root for resection of a large dural‑based mass displacing Spinal imaging revealed multiple dural‑based enhancing the thoracic cord and effacing the neural foramen. lesions in the cervical, thoracic, and lumbar spine Pathological analysis of both lesions affirmed a diagnosis with possible osseous involvement, consistent with of recurrent esthesioneuroblastoma with leptomeningeal drop metastasis [Figure 4]. The patient underwent a spread [Figure 5]. suboccipital craniectomy for resection of the posterior fossa lesion. Intraoperatively, the neoplasm was deep The patient initially began a course of systemic to the dura but above the arachnoid membrane; it was carboplatin, vincristine, and lomustine, as well as S629 SNI: Unique Case Observations 2015, Vol 6: Suppl 25 - A Supplement to Surgical Neurology International intrathecal methotrexate. She ultimately completed the locally into the brain parenchyma or distally through intrathecal methotrexate with carboplatin, etoposide, and the leptomeninges.[1] The cervical lymph nodes are cyclophosphamide. Follow‑up imaging 16 months after the most common site of metastasis. Other reported the initiation of chemotherapy revealed resolution of locations include the breast, lung, prostate, spine, the metastatic lesions and no radiographic appearance of bone, parotid, viscera, and abdomen.[1,16,21] While CNS residual disease, 9 years after her original diagnosis. She metastasis is seen in 20–30% of patients, leptomeningeal continues to receive surveillance for the recurrent disease dissemination remains extremely rare, with 2 and by evaluation of her cerebrospinal fluid (CSF) every 5 reported cases of cranial and spinal metastases, 3 months. respectively.[17,19,21,23] One case of distant intracranial, leptomeningeal spread of esthesioneuroepithelioma, a DISCUSSION rare variant, has been reported.[5] Leptomeningeal spread is an indicator of a poor prognosis with an expected Even with aggressive therapy, esthesioneuroblastomas survival of <2 years.[20] The most recent version of the exhibit a propensity for both
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