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LETTERS TO EDITOR  
Year : 2018  |  Volume : 61  |  Issue : 2  |  Page : 294-296
Intracranial chondrosarcoma: Series of three cases with varied and unusual presentations


1 Department of Pathology, ESIC Medical College, Faridabad, Haryana, India
2 Department of Pathology, PGIMER Dr. RML Hospital, New Delhi, India

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Date of Web Publication20-Apr-2018
 

How to cite this article:
Agarwal C, Bhardwaj M, Agarwal P, Ahuja A. Intracranial chondrosarcoma: Series of three cases with varied and unusual presentations. Indian J Pathol Microbiol 2018;61:294-6

How to cite this URL:
Agarwal C, Bhardwaj M, Agarwal P, Ahuja A. Intracranial chondrosarcoma: Series of three cases with varied and unusual presentations. Indian J Pathol Microbiol [serial online] 2018 [cited 2020 Jun 6];61:294-6. Available from: http://www.ijpmonline.org/text.asp?2018/61/2/294/230537




Editor,

Chondroid tumors occur predominantly in the axial skeleton and rarely may have an extraskeletal origin. Intracranial chondroid tumors constitute approximately <0.16% of all primary brain neoplasms.[1] They are subclassified into conventional (hyaline/myxoid), dedifferentiated, clear cell, and mesenchymal subtypes.[2] We discuss three cases of intracranial chondrosarcoma over a period of 6 years with varied presentations.

Case 1: A 52-year-old female presented with headache for the past 2 months. Noncontrast computed tomography (NCCT) of the head showed a heterogenous hypodense mass measuring 6 cm × 4 cm × 4 cm in the right frontoparietal white matter with areas of hyper-attenuation and central necrosis [Figure 1]a. Tumor was decompressed. We received multiple gray-white soft tissues, altogether measuring 5.5 cm × 3 cm × 2 cm with mucoid surface. Histopathology showed increased cellularity with chondrocytes arranged in the diffuse sheets and lobules with few bizarre cells in a background of chondromyxoid matrix [Figure 2]a and [Figure 2]b.
Figure 1: Noncontrast computed tomography of the head (a) large heterogenous hypodense space occupying lesion with few areas of hyper-attenuation, seen in right frontoparietal white matter with effacement of right ipsilateral lateral ventricle (b) partially calcified solid-cystic lesion involving left occipitotemporal lobe and cerebellum

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Figure 2: Tissue sections – chondrocytes (a) arranged in diffuse sheets and lobules (H and E, ×200) (b) moderate nuclear pleomorphism, round to vesicular nuclei, inconspicuous to prominent nucleoli and had abundant eosinophilic cytoplasm. Few bizarre cells along with bi-and multi-nucleated cells were also seen (H and E, ×400)

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On further interrogation, the patient gave a history of below-knee amputation 6 years back, for which she was operated outside, and the lesion was diagnosed as chondrosarcoma (Grade II) on histopathology. Hence, a final impression of metastatic intracranial chondrosarcoma was given [Table 1].
Table 1: Clinical, histomorphological, and immunohistochemical details of all the three cases

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Case 2: An 18-year-old female presented with complaints of frontal headache and progressive bilateral blurring of vision for the past 6 months. NCCT of the head showed an ill-defined hypo- to hyper-dense lesion in the bilateral frontal lobes with hyperostosis of the bone giving an impression of meningioma. Bilateral frontal craniotomy with tumor decompression was performed. We received multiple gray-white tumor tissue measuring 8 cm × 4 cm × 3 cm along with part of the skull bone with attached tumor tissue [Figure 3]a. On histology, both the specimens showed a biphasic tumor composed of sheets of undifferentiated oval-to-spindle-shaped small cells along with cartilaginous tissue. Undifferentiated cells were arranged in small aggregates in a hemangiopericytomatous pattern around dilated vascular channels. Bony invasion was seen [Figure 3]b. A diagnosis of extraskeletal mesenchymal chondrosarcoma was rendered [Table 1].
Figure 3: (a) Gross-part of the skull bone with attached tumor tissue (b) tissue section-biphasic tumor composed of sheets of undifferentiated small cells along with cartilaginous tissue (H and E, ×200)

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Case 3: A 13-year-old female child presented with visual disturbance and dull-aching headache for the past 2 years. On cerebellar examination, the patient swayed to left side on tandem walk. Fundus examination revealed bilateral papilledema. NCCT of the head showed a partially calcified solid-cystic lesion involving left occipitotemporal lobe and cerebellum [Figure 1]b. The patient underwent left paramedian suboccipital craniotomy with excision of tumor mass. Histopathology revealed hypercellular lobules of cartilage [Figure 4]a, separated by fibrovascular and myxoid stroma. Few binucleated and multinucleated chondrocytes with plump hyperchromatic nucleus were seen [Figure 4]b. A final diagnosis of chondrosarcoma Grade 1 was given. The patient was treated with intensity-modulated radiotherapy.
Figure 4: Tissue sections (a) irregularly shaped hypercellular lobules of cartilage (H and E, ×100) (b) Uninuclear with few binucleated and multinucleated chondrocytes with plump hyperchromatic nucleus (H and E, ×400)

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Postoperative period was uneventful except the third case in which there was recurrence 5 years later [Table 1].

Most frequent intracranial location of chondrosarcoma is the skull base (76.19%), in the middle cranial fossa, arising from the spheno-occipital synchondrosis. One of our cases of low-grade chondrosarcoma presented with a mass in the left cerebellum extending in the left parieto-temporo-occipital hemisphere. This is a rare site of involvement of the tumor. Very few case reports are being published of the same. Chondrosarcoma can occur in wide age groups. Most mesenchymal chondrosarcomas are reported in the second or third decade with a slight female preponderance.[3] Diplopia and headache are the two most common symptoms as increased tumor size causes compression or invasion of local structures and the optic pathway.[2] The present cases also came with similar complaints.

Their exact histogenesis is not known. In a study of review of 55 cases, majority of mesenchymal chondrosarcomas showed an attachment to meninges. Three theories were postulated to explain the presence of cartilage in meninges: first, metaplastic change of meningeal fibroblasts; second, origin from primitive multipotential mesenchymal cells; and third, tumor may arise from embryonal rests of cartilaginous matrix of the skull in the dura.[4]

Mesenchymal chondrosarcoma is a rare high-grade tumor with very high tendency for local recurrence and distant metastasis. They are mostly supratentorial, occurring in the frontoparietal region and attached to the meninges. Similarly, in our case, tumor was present in bilateral frontal lobes with bony attachment. Hemangiopericytoma or a cartilage-containing meningioma is their close differentials, especially when a limited amount of biopsy specimen is available. Intracranial mesenchymal chondrosarcoma is very difficult to differentiate from a meningioma by radiologic studies [3] as seen in our case also. Mesenchymal chondrosarcoma is easily distinguished from classical chondrosarcoma due to their characteristic biphasic pattern.

Conventional chondrosarcoma shows increased cellularity with abundant blue-gray cartilaginous matrix and lobules of cartilage. The chondrocytes are atypical varying in size and shape and contain enlarged hyperchromatic nuclei.

Although chondrosarcoma accounts for 40% of primary bone cancers in adults, it rarely metastasizes to the brain.[5] However, in patients with both advanced disease and systemic metastasis, over 10% develop metastases to the brain. In the present case of metastatic chondrosarcoma, the patient had a history of primary in the foot.

The prognosis depends on World Health Organization histological grades, which consist of three categories: Grade I (well differentiated), Grade II (moderately differentiated), and Grade III (poorly differentiated) based on cellular density, nuclear size, and nuclear characteristics. The metastatic potential of chondrosarcoma correlates largely to its histological grade. The most frequent sites of metastases are lungs, regional lymph nodes, and liver.

Management of mesenchymal chondrosarcoma is inadequately studied because of the rare and variable nature of the disease. Surgery is the mainstay with better survival in patients who underwent wide surgical resection followed by adjuvant chemotherapy and radiotherapy.[6] Long-term follow-up is essential because of local recurrence or distant metastasis. Recurrence rate depends on the treatment modality, surgery alone has a 5-year recurrence rate of 44%, and the addition of adjuvant radiation therapy reduced this rate to 9%.[2]

Skull base chondrosarcomas should always be considered in cases of unexplained headaches or other associated symptoms to obtain an early diagnosis before the tumor becomes extensive. A close follow-up of these patients with appropriate imaging studies on a regular basis is necessary to identify recurrences early.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Altinboga AA, Yalcin N, Akbulut M, Coskun E, Duzcan SE. Intracranial chondrosarcoma in a 22-years old woman: Report of a case. J Neurol Sci (Turkish) 2011;28:614-8.  Back to cited text no. 1
    
2.
Bloch OG, Jian BJ, Yang I, Han SJ, Aranda D, Ahn BJ, et al. A systematic review of intracranial chondrosarcoma and survival. J Clin Neurosci 2009;16:1547-51.  Back to cited text no. 2
    
3.
Chen JY, Hsu SS, Ho JT. Extraskeletal intracranial mesenchymal chondrosarcoma: Case report and literature review. J Med Sci 2004;20:187-90.  Back to cited text no. 3
    
4.
Salvati M, Caroli E, Frati A, Piccirilli M, Agrillo A, Brogna C, et al. Central nervous system mesenchymal chondrosarcoma. J Exp Clin Cancer Res 2005;24:317-24.  Back to cited text no. 4
    
5.
Lee YY, Van Tassel P, Raymond AK. Intracranial dural chondrosarcoma. AJNR Am J Neuroradiol 1988;9:1189-93.  Back to cited text no. 5
    
6.
Shakked RJ, Geller DS, Gorlick R, Dorfman HD. Mesenchymal chondrosarcoma: Clinicopathologic study of 20 cases. Arch Pathol Lab Med 2012;136:61-75.  Back to cited text no. 6
    

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Correspondence Address:
Charu Agarwal
Department of Pathology, ESIC Medical College and Hospital, Faridabad, Haryana
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJPM.IJPM_179_17

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