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Year : 2009  |  Volume : 46  |  Issue : 2  |  Page : 174-175

Primary brain sarcoma or metastatic carcinoma?

1 Medical Center of Central Georgia, 840 Pine Street, Suite 880, Macon, GA 31201, USA
2 Mercer University, USA
3 Georgia Neurosurgical Institute, USA

Correspondence Address:
M S Walid
Medical Center of Central Georgia, 840 Pine Street, Suite 880, Macon, GA 31201
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0019-509X.49160

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How to cite this article:
Walid M S, Osborne T J, Robinson J S. Primary brain sarcoma or metastatic carcinoma?. Indian J Cancer 2009;46:174-5

How to cite this URL:
Walid M S, Osborne T J, Robinson J S. Primary brain sarcoma or metastatic carcinoma?. Indian J Cancer [serial online] 2009 [cited 2022 Aug 19];46:174-5. Available from:


Primary brain sarcomas are very rare tumors with less than 50 cases reported since 1929. [1],[2] We are describing the case of a patient with sarcoidosis and treated breast carcinoma who presented 18 years later with brain tumor, whose pathological features were more consistent with primary sarcoma of the brain.

A 56-year-old African-American woman presented with recent-onset confusion. The patient's history was significant for right mastectomy with transverse rectus abdominus myocutaneous flap reconstruction in 1989, total abdominal hysterectomy, and sarcoidosis. Physical examination and review of systems were remarkable for cough, delayed verbal response, and right-sided hemiparesis. Computed tomography (CT) images of the thorax, abdomen, and pelvis revealed mediastinal and hilar adenopathy. Brain magnetic resonance imaging (MRI) showed an intra-axial mass in the left frontal lobe with significant surrounding vasogenic edema and mild mass effect upon the surrounding structures [Figure 1]. The mass demonstrated intense contrast enhancement with findings suggestive of central necrosis. Bilateral digital diagnostic mammogram with computer-aided detection (CAD) was negative.

The patient was started on IV steroids and was taken to the operating room for craniotomy and excision of the mass on day one of hospitalization. Operative intervention revealed a hard, well-encapsulated lesion that was easily removed from the brain. The frozen section revealed sarcoma-type features. Immunohistochemistry showed that the tumor was negative for desmin, smooth muscle actin, S100, epithelial membrane antigen (EMA), glial fibrillary acidic protein (GFAP), and MOC-31. There was some positive staining with pankeratin [Figure 2]; however, this was interpreted as cross-reactivity with nonkeratin intermediate filaments. The vimentin and P16 staining were strongly positive. The GFAP and S100 stains highlighted the entrapped reactive astrocytes, with no evidence of a neoplastic glial component. P53 demonstrated 4+ nuclear staining in greater than 66% of the neoplastic cells. The Ki-67 labeling index was >50% and mitoses were 11 per 10 high power fields. Bcl-2 and repeat EMA stains were negative and the reticulin pattern was consistent with entrapped glial cells. Additional molecular diagnostic testing was performed, which showed no evidence of Phosphatase and tensin homolog (PTEN) deletions or epidermal growth factor receptor (EGFR) amplifications. The final diagnosis was spindle cell sarcoma, Fιdιration Nationale des Centres de Lutte Contre le Cancer (FNCLCC) grade 3. The old pathological report of the patient's breast cancer was retrieved; it indicated comedo type intraductal carcinoma, which meant the brain tumor was a primary sarcoma of the brain. The slides were sent to another hospital for consultation and the report came favoring metastatic carcinoma over primary or metastatic sarcoma in view of the strong and diffuse expression of cytokeratin.

The etiology of primary brain sarcomas is not well-established. Genetic, viral, and environmental factors have been implicated. Brain sarcoma has been reported years after radio- and chemotherapy. [3] Meningiomas have also been reported years after breast cancer treated with chemotherapy. [4] Our patient was successfully treated for breast cancer 18 years ago and old chemotherapy could have played a role in the genesis of her brain sarcoma. Besides, cases of Kaposi sarcoma associated with sarcoidosis have been described in literature. [5] In our case, primary sarcoma of the brain could be linked to previous chemotherapy and sarcoidosis. Understanding how these factors relate to each other requires further investigation. Finally, if this was a dedifferentiated metastasis from breast carcinoma, it would still be a rare case because of the long period that had passed before it revealed itself with a different tissue type.

  References Top

1.Kawafuchi J. Primary sarcoma of the brain: Report of two cases. Neurol Medico Chirurgica 1965;7:193.  Back to cited text no. 1    
2.Torres G, Petit F, Vilchez V, Romero Z, Dorfman S, Cardozo D, et al. Primary cerebral fibrosarcoma in a child. Clin Neuropathol 2007;26:284-7.  Back to cited text no. 2  [PUBMED]  
3.Kaminski JM, Yang CC, Yagmai F, Movsas B, Lee M, Barrett JT. Intracranial fibrosarcoma arising 5 years after chemotherapy alone for glioblastoma multiforme in a child. Pediatr Neurosurg 2000;33:257-60.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]
4.Kumpulainen EJ, Hirvikoski PP, Johansson RT. Long-term outcome of adjuvant chemotherapy cyclophosphamide, mitoxantrone and fluorouracil in women with breast cancer. Acta Oncol 2008;47:120-3.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]
5.Dessoukey MW, Dayem HA, Omer MF. Kaposi's sarcoma and sarcoidosis coexisting in lesions of HIV-seronegative patient. Int J Dermatol 1996;35:824-6.  Back to cited text no. 5  [PUBMED]  


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