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| LETTER TO EDITOR |
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| Year : 2013 | Volume
: 61
| Issue : 6 | Page : 677-679 |
Primary pilomyxoid astrocytoma of the thoracolumbar spinal cord in an adult
Liang Wu, Tao Yang, Chenlong Yang, Yulun Xu
Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Dongcheng District, Beijing, China
| Date of Submission | 09-Jul-2013 |
| Date of Decision | 05-Aug-2013 |
| Date of Acceptance | 18-Dec-2013 |
| Date of Web Publication | 20-Jan-2014 |
Correspondence Address:
Yulun Xu
Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, Dongcheng District, Beijing
China
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0028-3886.125376
How to cite this article:
Wu L, Yang T, Yang C, Xu Y.
Primary pilomyxoid astrocytoma of the thoracolumbar spinal cord in an adult. Neurol India 2013;61:677-9 |
Sir,
Pilomyxoid astrocytoma (PMA) is a rare tumor that was previously classified as pilocytic astrocytoma (PA) because of similar histological features. [1] Compared with PA, PMA demonstrates more aggressive biological behavior, and was assigned to World Health Organization (WHO) α grade according to the 2007 WHO Classification. [2] PMA in the spinal cord is extremely rare. We documented probably the first case of primary thoracolumbar spinal PMA.
A 40-year-old female presented with intermittent burning pain and progressive numbness in both legs of 8 months duration. On admission, mild incomplete voiding was noted. Neurological examination revealed reduced superficial sensation below T12 level. Preoperative magnetic resonance imaging (MRI) of the spine demonstrated an abnormal intramedullary lesion with cord swelling at the T11-L1 level. The lesion showed heterogeneous isointensity on the T1-weighted image (WI) with ill-defined margins from the normal spinal cord, mild hyperintensity on the T2WI, and heterogeneous speckled enhancement after gadolinium administration [Figure 1]. A T11-L1 laminectomy was performed through the posterior approach. Due to its adhesion to the cord and ill-defined margins with the conus medullaris, the intramedullary tumor was subtotally removed. Histopathologically, the tumor was composed of monomorphous bipolar piloid cells with elongated cytoplasmic processes in a prominent myxoid background without Rosenthal fibers [Figure 2]a and b. The tumor cells were arranged around the small blood vessels, showing an angiocentric pattern [Figure 2]c. Immunohistochemical staining revealed immunoreactivity for glial fibrillary acidic protein, and the MIB-1 labeling index was 3%. These findings were consistent with the diagnosis of PMA. After surgery she had relief of pain. Postoperatively she received external beam radiotherapy (40 Gy in 25 fractions, 1 fraction per day, 5 fractions per week). She had gradual improvement in numbness and bladder dysfunction in the follow-up. At 3 years follow-up MRI revealed no regrowth, or metastasis of the residual tumor was found [Figure 3].
PMAs may originate from anywhere along the neuroaxis but the most frequent locations are the hypothalamic and chiasmatic regions. [2] Spinal PMAs of spinal cord are extremely rare, especially in adult patients. Since the first description in 2005 by Komotar et al., [3] only nine cases have been reported in the literature. [1],[2],[3],[4],[5],[6] Spinal PMAs affect pediatric age group more than adult patients with a female preponderance, mostly the cervical and thoracic spinal regions. Most of the lesions were intramedullary, and only one case was intradural extramedullary lesion. [5] The MRI features of spinal PMA are variable and could be misleading. Pathological examination is the gold standard in the differential diagnosis of PMA. | Figure 1: Preoperative magnetic resonance imaging showing a T11 - L1 intramedullary mass with heterogeneous isointensity on the sagittal T1 - weighted image (WI) (a), mild hyperintensity on the sagittal T2WI (b), and heterogeneous speckled enhancement with ill-defined margins from the normal spinal cord on the sagittal (c), coronal (d), and axial (e) T1WI with gadolinium
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 | Figure 2: Histology sections showing monomorphous tumor cells in a prominent myxoid background (a, H and E, ×200), bipolar piloid cells with elongated cytoplasmic processes (b, H and E, ×400), and tumor cells arranged in angiocentric pattern (c, H and E, ×400)
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 | Figure 3: Postoperative magnetic resonance imaging on the sagittal T1-weighted images with gadolinium showing the resection and the residual tumor at 25 days after operation (a), and no regrowth of the residual tumor at 3 years after adjuvant radiotherapy (b)
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Because of the ill-defined margins or dense adhesions to the neural tissue, partial removal or even biopsy had to be performed to avoid unacceptable postoperative complications for the treatment of spinal PMA. The main goal of surgery is preservation of neurological function. Adjuvant therapy is recommended considering the high risk of local recurrence and cerebrospinal fluid dissemination, [5] particularly when total removal cannot be achieved. However, the efficacy of adjuvant radiation therapy for controlling the tumor is still uncertain, and cisplatin/carboplatin-based multidrug chemotherapy is recommended for the initial treatment of PMAs. [6] Cisplantin + etoposide was shown to be effective in a 15-month-old girl and the tumor has not relapsed for 64 months. [4]
Paraskevopoulos et al. reported a female child who had early recurrence and transformation within 3 months into a glioblastoma with a fatal outcome in spite of adjunctive chemotherapy. [7] This case suggests that the biological behavior of PMA may be variable. Because of possible variable biologic behavior of PMA, adjuvant therapy may be recommended at an early stage of diagnosis, particularly for the tumor with more aggressive findings, such as high expression of Ki-67, rapid progression, and malignant transformation, although overall prognosis is uncertain.
In conclusion, PMAs should be included into the differential diagnosis of intradural tumors of the spine. Multidisciplinary treatment consisting of surgical removal and adjuvant radiochemotherapy should be considered.
| » References | |  |
| 1. | Eigenbrod S, Thon N, Jansen N, Janssen H, Mielke J, Ruiter M, et al. Intramedullary pilomyxoid astrocytoma with intracerebral metastasis exhibiting oligoden-droglioma-like features. Rare Tumors 2012;4:e30. 
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| 2. | Komotar RJ, Carson BS, Rao C, Chaffee S, Goldthwaite PT, Tihan T. Pilomyxoid astrocytoma of the spinal cord: Report of three cases. Neurosurgery 2005;56:191.
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| 3. | Arulrajah S, Huisman TA. Pilomyxoid astrocytoma of the spinal cord with cerebrospinal fluid and peritoneal metastasis. Neuropediatrics 2008;39:243-5.
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| 4. | Matsuzaki K, Kageji T, Watanabe H, Hirose T, Nagahiro S. Pilomyxoid astrocytoma of the cervical spinal cord successfully treated with chemotherapy: Case report. Neurol Med Chir (Tokyo) 2010;50:939-42.
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| 5. | Mendiratta-Lala M, Kader Ellika S, Gutierrez JA, Patel SC, Jain R. Spinal cord pilomyxoid astrocytoma: An unusual tumor. J Neuroimaging 2007;17:371-4.
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| 6. | Tsugu H, Oshiro S, Yanai F, Komatsu F, Abe H, Fukushima T, et al. Management of pilomyxoid astrocytomas: Our experience. Anticancer Res 2009;29:919-26.
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| 7. | Paraskevopoulos D, Patsalas I, Karkavelas G, Foroglou N, Magras I, Selviaridis P. Pilomyxoid astrocytoma of the cervical spinal cord in a child with rapid progression into glioblastoma: Case report and literature review. Childs Nerv Syst 2011;27:313-21.
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[Figure 1], [Figure 2], [Figure 3]
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