Clinics and Practice 2016; volume 6:813

Diffuse spinal leptomeningeal with dissemination in the spinal meninges have been published. Interestingly, only three Correspondence: Ameer Alyeldien, Department of spread of a pilocytic of these nine cases have been described in Neurosurgery, Sana Kliniken Duisburg, Zu den astrocytoma children;2-4 the other six cases concerned adult Rehwiesen 9, 47055 Duisburg, Germany. in a 3-year-old child patients.1,5-9 This may be due to the fact that Tel.: +49.152.0850-0875 - Fax: +49.203.733.2402. intradural extramedullary spinal dissemina- E-mail: [email protected] tion occurred even 10-20 years after the pri- Ameer Alyeldien,1 Key words: Pilocytic astrocytoma; metastasis; mary surgery of the cerebral tumor. Although Sarah Teuber-Hanselmann,2 spinal dissemination; optic chiasm. the first report of spinal seeding of pilocytic Azad Cheko,1 Tanja Höll,3 Martin Scholz,1 astrocytomas was published in 1976,10 no stan- Conflict of interest: the authors declare no poten- 4 Athanasios K. Petridis dard treatment modality is established until tial conflict of interest. 1Department of Neurosurgery, Sana today, due to its rarity. Here, we report the case Kliniken, Duisburg; 2Institute of of a 3-year-old boy with a pilocytic astrocytoma Received for publication: 14 October 2015. of the optic chiasm with contact to the third Revision received: 7 March 2016. Neuropathology, University of Duisburg- Accepted for publication: 13 March 2016. Essen, Essen; 3Department of Pediatrics, ventricle, hypothalamic invasion and lep- Sana Kliniken, Duisburg; 4Department of tomeningeal dissemination in the spinal This work is licensed under a Creative Commons Neurosurgery, Heinrich Heine University, meninges. Attribution NonCommercial 4.0 License (CC BY- Düsseldorf, Germany NC 4.0). Ethical approval All procedures performed in studies involv- ©Copyright A. Alyeldien et al., 2016 Licensee PAGEPress, Italy ing human participants were in accordance Clinics and Practice 2016; 6:813 Abstract with the ethical standards of the institutional doi:10.4081/cp.2016.813 and/or national research committee and with the 1964 Helsinki declaration and its later Pilocytic astrocytomas correspond to low- amendments or comparable ethical standards. grade gliomas and therefore metastasize anyonly of the spinal lesions because of the wide exceedingly rare. However, pilocytic astrocy- Patient consent spread of the tumor. tomas are able to and leptomeningeal dissem- The patient’s guardian (the father) has con- On neuropathological examination the ination may be seen. What are the treatment sented to the submission of the case report for scanty tumor fragments showed loosely tex- options of these cases? We present a case submission to the journal. use tured multipolar cells with round to oval, cyto- report of a 3-year-old child with a pilocytic logically bland nuclei and microcysts. astrocytoma of the optic chiasm with lep- Cellularity was low to moderate. Focally, a tomeningeal dissemination of the spinal basophilic mucoid matrix was seen. The tumor meninges. Partial resection of the cerebral Case Report lacked Rosenthal fibers, eosinophilic granular tumor has been performed. Since the lep- bodies and hyaline droplets. No satisfying tomeningeal dissemination was seen all over A 3-year-old child presented to our depart- angiocentric cell arrangement was observed. the spinal meninges, the child did not undergo ment after he had undergone a surgery 5 The scanty fragments showed no mitoses and further surgical treatment. A wait and watch months ago in another country to obtain a no necrosis. Only few slightly pathologic blood strategy were followed. Chemotherapy was ini- biopsy from the tumor around the optic nerve. vessels were seen. The tumor cells showed a tiated, if a 25% tumor growth was seen. Because of bleeding complications, the col- strong positivity for the glial fibrillary acidic Leptomeningeal dissemination of a pilocytic leagues performed only a biopsy and a further protein (GFAP). MIB-1 labeling was typically astrocytoma is seen so infrequently that no commercialtumor removal was aborted. Histological exam- low, showing proliferating activity in up to 2% standard therapy is established. Since these ination of this biopsy (realized in the country of tumor cells. metastases may occur even up to 2 decades where the operation was performed) revealed Because of the location of the tumor in the after primary tumor resection, long-term fol- a pilocytic astrocytoma. Now, six months after hypothalamic/chiasmic region, the focal low-up is indicated. In case of spinalNon metas- the first operation, the patient was admitted to basophilic myxoid matrix und lack of tases, surgical treatment should be performed our hospital. After the first surgery and before Rosenthal fibers, eosinophilic granular bodies if feasible. Otherwise observation should be the diagnosis was made, the child developed and hyaline droplets, the possibility of a possessed and/or chemotherapy should be ini- cry attacks, affective changes, loss of satiety, pilomyxoid astrocytoma (WHO grade II) was tiated. continuous thirsty, hyperactivity and sleep dis- considered. However, since we did not observe turbance. We renewed the cranial magnetic a markedly mucoid matrix and a predominant- resonance imaging (MRI) and performed addi- ly angiocenctric cell arrangement, which is tionally an MRI of the spine (Figure 1). The typical for this entity, the tumor was classified Introduction tumor did not show any further growth in the 5 as pilocytic astrocytoma (WHO grade I). months period, nevertheless the tumor was Liquor obtained from lumbar puncture Leptomeningeal spread of primary tumors of still large and a surgical reduction of the tumor revealed some tumor cell aggregates that var- the central nervous system (CNS) through was indicated. In the MRI of the spine we saw ied in size and showing tumor cells with round cerebrospinal fluid (CSF) is uncommon. It may multinodular tumors (intradural/ extrame - to oval cytologically bland nuclei and rarely appear in tumors like medulloblastomas dullary), which indicated a leptomeningeal definable cytoplasms, leading to the diagnosis [World Health Organization (WHO) grade IV], spread. Since the tumor was invading the of meningeosis gliomatosa. In accordance with ependymomas and high-grade gliomas, but hypothalamus, we only removed the large the radiological finding, the cytomorphology of exceedingly rare in low-grade gliomas.1 Up to intracranial tumor partially in order to prevent the tumor cells was consistent with that of the now, only nine cases of pilocytic astrocytoma hormonal insufficiencies. We did not remove glial tumor described above.

[Clinics and Practice 2016; 6:813] [page 13] Case Report

Discussion

Pilocytic astrocytomas are low-grade gliomas corresponding to WHO grade I with an excellent prognosis, particularly if complete resection can be achieved. The present case reveals a cerebral low-grade astrocytoma grow- ing from the optic chiasm towards the hypo- thalamus and the third ventricle with lep- tomeningeal spreading all over the spine through the CSF. Up to now, only two cases concerning pilocytic astrocytomas of the optic chiasm metastasizing into the spine have been published in the literature3,9 although the WHO classification of tumors of the CNS described the hypothalamic as the usual primary site of neuraxial seeding within this tumor entity. Other pilocytic astrocytomas with spinal dis- semination occurred in adult patients almost two decades after the primary surgical treat- ment emphasizing a long-term follow-up in Figure 1. Pilocytic astrocytom of the opticchiasm disseminated leptomeningeally into the patients with diagnosed pilocytic astrocytoma.6 spine. A) Cranial magnetic resonance image shows the pilocytic astrocytoma growing Nevertheless, the prognosis of this tumor from the optic chiasm and invading the hypothalamus and the third ventricle; B) remains excellent after resection of a solitary Leptomeningeal contrast enhanced areas in the spine with a node around the nerve roots S1 and S2. spinal metastasis since the proliferative activ- only ity is low.6 Interestingly, despite metastasizing, these tumors do not seem to show any signs of secondary malignisation.1,3 Indeed, very few examples of pilocytic astrocytoma undergoing use malignant changes have been reported.11 Since most of them had previously undergone radia- tion therapy, it should be taken into account that radiation may promote malignant trans- formation. One reported case even reveals a patient with a cervical low-grade astrocytoma with a cerebral metastasis, indicating the possibility of such history and also emphasizing that - to prevent further metastases - metastases should be rejected if possible.2 In the present case we performed a partial commercial resection of the cerebral tumor, since invasion into the hypothalamus did not allow complete resection. The CSF showed tumor cells in neu- ropathological examination. Radiologically, an intradural contrast enhancement throughNon a long distance was seen, building a thin film of tumor cells, which did not deliver a specific tar- get for surgical resection. Under these circum- stances we did not perform tumor resection around the nerve roots S1 and S2 where the tumor seemed to be solid. In addition to our case, two of three pediatric cases with spinal dissemination occurred after optic chiasm astrocytomas,3,7,9 revealing that contact to the ventricular system seems to increase the pos- Figure 2. Histological presentation of the pilocytic astrocytoma. A) Loosely textured mul- sibility of spinal cord dissemination. Because tipolar cells with round to oval, cytologically bland nuclei in a microcystic matrix with- of the rarity of dissemination within this out Rosenthal fibers and eosinophilic granular bodies. Mitotic figures and necrosis are benign tumor entity, a standardization of treat- absent (hematoxylin&eosin staining, 20-fold magnification); B) The tumor cells show a strongly positive reaction for glial fibrillary acidic protein (20-fold magnification); C) ment misses. Shapiro et al.10 advocate an Ki67 staining reveals few proliferating tumor cells (20-fold magnification); D) Liquor aggressive surgical treatment, while Fellgiebel obtained from lumbar puncture with few tumor fragments, varying in size and cell count. et al. admit that treatment of these cases is Cell morphology is consistent with a glial tumor in terms of meningeosis gliomatosa always difficult because of the lack of a stan- (May-Grünwald-Giemsa, 20-fold magnification).

[page 14] [Clinics and Practice 2016; 6:813] Case Report dard therapy scheme.8 malignisation. Long-term follow-up is impor- ing multiple subtotal resections. Pediatr Chemotherapy does not seem to be the opti- tant for early detection of multifocal spreading Neurosurg 2005;41:248-52. mum therapeutic approach since at least vin- of pilocytic astrocytomas and MRI of the spine 5. Bohner G, Masuhr F, Distl R, et al. Pilocytic cristine and carboplatin did not exhibit good should be performed always when the primary astrocytoma presenting as primary diffuse treatment results in one patient.5 Again, pilocytic astrocytoma is in proximity to the leptomeningeal gliomatosis: report of a because of the very few reports it is impossible ventricular system. Treatment options in cases unique case and review of the literature. to conclude if a therapeutic approach with of spinal dissemination are the surgical tumor Acta Neuropathol 2005;110:306-11. chemotherapy could be efficient or not. Since resection in cases of solid lesions as a first line 6. Crabtree KL, Arnold PM. Spinal seeding of in our case the meninges were infested by therapy, we recommended that further follow- a pilocytic astrocytoma in an adult, initial- tumor over a long distance, complete surgical up and observation of tumor growth and/or resection was not possible. Therefore, we chemotherapy, if the tumor is disseminated ly diagnosed 18 years previously. Pediatr decided, after many consultations in the pedi- over a great area of leptomeninges. Neurosurg 2010;46:66-70. atric oncology department in our hospital as 7. Faria AV, Azevedo GC, Zanardi VA, et al. well as in other expert pediatric oncology cen- Dissemination patterns of pilocytic astro- ters to choose a chemotherapeutic approach cytoma. Clin Neurol Neurosurg 2006;108: and the tumor growth should be observed in References 568-72. the 3-month-follow-up. As the child moved with 8. Fellgiebel A, Erbguth F, Neundörfer B. his Family to another city, the child was admit- 1. Jusué-Torres I, Alcázar-Vaquerizo L, Polyradiculopathy and ataxia: clinical ted there to another hospital for the further Gómez-Angulo JC, et al. Leptomeningeal manifestation of late recurrence of pilocyt- treatment, where the colleagues decided to fol- spread of an intramedullary cervical pilo- ic astrocytoma with cerebral and spinal low the wait and watch strategy and no cytic astrocytoma: case report and litera- dissemination. Nervenarzt 2001;72:143-6. chemotherapy was initiated. A 3-month follow- ture review. Neurocirugia Astur 2011; 9. Versari P, Talamonti G, D’Aliberti G, et al. up MRI did not show any further growth of the 22:445-51. Leptomeningeal dissemination of juvenile tumor, the patient had no complaints and 2. Jang SY, Kong MH, Song KY, Frazee JG. pilocytic astrocytoma: case report. Surg therefore chemotherapy is not needed. Intracranial metastases of cervical Neurol 1994;41:318-21. intramedullary low-grade astrocytoma only 10. Shapiro K, Shulman K. Spinal cord seed- without malignant transformation in ing from cerebellar astrocytomas. Childs adult. J Korean Neurosurg Soc 2009;45: Conclusions 381-5. Brain 1976;2:177-86. 3. Kocks W, Kalff R, Reinhardt V, et useal. Spinal 11. Tomlinson FH, Scheithauer BW, Hayostek Leptomeningeal dissemination of pilocytic metastasis of pilocytic astrocytoma of the CJ, et al. The significance of atypia and astrocytomas into the spine is rare. Even two chiasma opticum. Childs Nerv Syst 1989;5: histologic malignancy in pilocytic astrocy- decades after resection of the primary tumor 118-20. toma of the cerebellum: a clinicopatholog- metastases of the spine could be observed. 4. Zorlu F, Selek U, Akyuz C, et al. Spinal ic and flow cytometric study. J Child Fortunately, there is usually no secondary seeding of a pilocytic astrocytoma follow- Neurol 1994;9:301-10.

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