J Korean Neurosurg Soc.  2015 Jun;57(6):469-472. 10.3340/jkns.2015.57.6.469.

Intracranial Undifferentiated Sarcoma Arising from a Low-Grade Glioma: A Case Report and Literature Review

• Kim BJ
• Kim JH
• Chung HS
• Kwon TH

• KMID: 2191257
• DOI: http://doi.org/10.3340/jkns.2015.57.6.469

Abstract

Undifferentiated sarcomas are rarely identified in the intracranial region. A 23-year-old man was admitted with a chief complaint of headache. Initial magnetic resonance images showed signs of low-grade glioma in the frontal lobe. Stereotactic biopsy was performed, and a diagnosis of diffuse astrocytoma was confirmed. Three months later, the patient presented with a high-grade tumor as seen on imaging studies. He underwent total resection of the tumor and histopathological tests identified an undifferentiated sarcoma. The patient died eight months later due to massive tumor bleeding. To the best of our knowledge, this is the first report of undifferentiated sarcoma arising from low-grade glioma without any chemotherapy or radiotherapy.

Keyword

Intracranial undifferentiated sarcoma; Malignant fibrous histiocytoma; Low-Grade glioma; Malignant transformation; Stereotactic biopsy

MeSH Terms

Astrocytoma
Biopsy
Diagnosis
Drug Therapy
Frontal Lobe
Glioma*
Headache
Hemorrhage
Histiocytoma, Malignant Fibrous
Humans
Radiotherapy
Sarcoma*
Young Adult

Figure

• 

  Fig. 1 Fluid-attenuated inversion recovery image reveals a hyperintense solid intraaxial lesion in the right frontal lobe (A). Contrast-enhanced T1 image shows a hypointense lesion without any enhancement (B). Magnetic resonance spectroscopy was used to define the biopsy target, which showed an increased choline/N-acetyl aspartate ratio (C and D). Cho : choline, Cr : creatine, NAA : N-acetyl-aspartate, Lac : lactate.

• 

  Fig. 2 Magnetic resonance (MR) imaging shows the heterogeneously enhancing tumor and subfalcine herniation (A). Tumor tissue obtained by gross total resection (B). Five months later, the recurred tumor was radically resected (C). Computed tomography showing large tumor regrowth with internal bleeding (D).

• 

  Fig. 3 Stereotactic biopsy of the initial tumor. A : Photomicrograph shows atypia without any necrosis or vascular proliferation (H-E, ×400). B : On immunohistochemistry, the tumor cells were immunoreactive for glial fibrillary acidic protein (×400). C : Staining for S-100 protein was positive (×400). D : The Ki-67 labeling index was 3% (×200).

• 

  Fig. 4 Resection of the heterogeneously enhancing tumor. A : Photomicrograph revealed hyperchromic pleomorphic nuclei, spindle cells, and giant cells (H-E, ×200). B : The tumor cells showed no immunoreactivity for glial fibrillary acidic protein (×400). C : Reticulin staining shows an encapsulating pericellular reticulin network (×400). D : The Ki-67 labeling index was increased to 20% (×400).

Reference

1. Alexandru D, Linskey ME, Kim RC, Bota D. Undifferentiated sarcoma induced by BCNU (1,3-bis(2-chloroethyl)-1-nitrosourea) wafers. J Interdiscipl Histopathol. 2013; 1:163–167.

2. Brennan MF, Antonescu CR, Maki RG. Undifferentiated Pleomorphic Sarcoma (UPS; Malignant Fibrous Histiocytoma : MFH) and Myxofibrosarcoma. In : Brennan MF, Antonescu CR, Maki RG, editors. Management of Soft Tissue Sarcoma. ed 1. New York: Springer Science & Business Media;2013. p. 129–136.

3. Fletcher CD. The evolving classification of soft tissue tumours - an update based on the new 2013 WHO classification. Histopathology. 2014; 64:2–11. PMID: 24164390.

4. Fletcher CD. Undifferentiated sarcomas : what to do? And does it matter? A surgical pathology perspective. Ultrastruct Pathol. 2008; 32:31–36. PMID: 18446665.

5. Hofer SO, Shrayer D, Reichner JS, Hoekstra HJ, Wanebo HJ. Wound-induced tumor progression : a probable role in recurrence after tumor resection. Arch Surg. 1998; 133:383–389. PMID: 9565118.

6. Kosucu P, Yavuz AA, Yavuz MN, Reis A, Ahmetoglu A. Primary intracranial malignant fibrous histiocytoma : case report. Eur J Radiol Extra. 2003; 47:78–81.

7. Lehnhardt M, Daigeler A, Homann HH, Schwaiberger V, Goertz O, Kuhnen C, et al. MFH revisited : outcome after surgical treatment of undifferentiated pleomorphic or not otherwise specified (NOS) sarcomas of the extremities-an analysis of 140 patients. Langenbecks Arch Surg. 2009; 394:313–320. PMID: 18584203.

8. Meng X, Zhong J, Liu S, Murray M, Gonzalez-Angulo AM. A new hypothesis for the cancer mechanism. Cancer Metastasis Rev. 2012; 31:247–268. PMID: 22179983.

9. Mitsuhashi T, Watanabe M, Ohara Y, Hatashita S, Ueno H. Multifocal primary intracerebral malignant fibrous histiocytoma-case report. Neurol Med Chir (Tokyo). 2004; 44:249–254. PMID: 15200060.

10. Nascimento AF, Raut CP. Diagnosis and management of pleomorphic sarcomas (so-called "MFH") in adults. J Surg Oncol. 2008; 97:330–339. PMID: 18286476.

11. Sasagawa Y, Tachibana O, Iizuka H. Undifferentiated sarcoma of the cavernous sinus after γ knife radiosurgery for pituitary adenoma. J Clin Neurosci. 2013; 20:1152–1154. PMID: 23587603.

12. Schmitt WR, Carlson ML, Giannini C, Driscoll CL, Link MJ. Radiation-induced sarcoma in a large vestibular schwannoma following stereotactic radiosurgery : case report. Neurosurgery. 2011; 68:E840–E846. discussion E846PMID: 21209595.

13. Weiss SW, Enzinger FM. Malignant fibrous histiocytoma : an analysis of 200 cases. Cancer. 1978; 41:2250–2266. PMID: 207408.

14. Yang T, Rockhill J, Born DE, Sekhar LN. A case of high-grade undifferentiated sarcoma after surgical resection and stereotactic radiosurgery of a vestibular schwannoma. Skull Base. 2010; 20:179–183. PMID: 21318035.

15. Yoo RE, Choi SH, Park SH, Jung HW, Kim JH, Sohn CH, et al. Primary intracerebral malignant fibrous histiocytoma : CT, MRI, and PET-CT findings. J Neuroimaging. 2013; 23:141–144. PMID: 21447025.

16. Yun LG, Shin WH, Kim BT, Choi SK, Byun BJ, Lee DW. Malignant fibrous histiocytoma presenting as osteolytic lesion on the left temporal bone : a case report. J Korean Neurosurg Soc. 1995; 24:471–476.