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Case Reports
. 2013 May 28:4:70.
doi: 10.4103/2152-7806.112614. Print 2013.

Inflammatory myofibroblastic tumors of the central nervous system that express anaplastic lymphoma kinase have a high recurrence rate

Daniel J Denis  1 , Karim Elayoubi, Alexander G Weil, France Berthelet, Michel W Bojanowski
Affiliations
Case Reports

Inflammatory myofibroblastic tumors of the central nervous system that express anaplastic lymphoma kinase have a high recurrence rate

Daniel J Denis et al. Surg Neurol Int. .

Abstract

Background: Inflammatory myofibroblastic tumors (IMTs) of the central nervous system (CNS) are rare entities with diverse histopathological features and varying propensities to recur.

Case description: A 26 year-old male with an IMT of the CNS of the left tentorium had tumor progression 2 months after partial surgical resection. Histopathological studies confirmed expression of ALK. Macroscopic total resection was performed followed by radiotherapy. A recurrence occurred 20 months after the second surgery that necessitate reoperation. Including the present case, we identified 30 cases of IMT of the CNS corresponding to our search criteria in the literature. The extent of resection was reported in 26 of these cases. Gross total resection was done in 75% of ALK-positive and in 61% of ALK-negative cases. Recurrence rate after gross total resection for ALK-positive and ALK-negative cases was 33% and 9%, respectively. Every recurrence in ALK-positive patients occurred within 2 years after surgery.

Conclusion: IMT of the CNS are a heterogeneous group of tumors and the treatment of choice is complete surgical resection. Because of the high recurrence rate reported for IMT of the CNS expressing ALK, a closed follow-up is recommended. When faced with an early recurrence, a surgical resection followed by radiotherapy may be advised.

Keywords: Anaplastic lymphoma kinase; fibrohistiocytic; inflammatory myofibroblastic tumor; plasma cell granuloma; pseudotumor; recurrence.

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Figures

Figure 1
Figure 1
Preoperative axial (a), coronal (b) and sagittal (c) T1-weighted MRI studies showing a gadolinium enhancing extra-axial mass of the left tentorial incisure. Temporal lobe edema is seen on the axial T2-weighted MRI studies (d)
Figure 2
Figure 2
Postoperative axial CT-scan with contrast after a partial resection of the tumor through a supracerebellar-transtentorial approach
Figure 3
Figure 3
Histologic appearance of IMT of the CNS. (a) Fusiform cells organized in perpendicular oriented fascicules (arrow) (×100).(b) Diffuse lymphocytes and plasmocytes infiltrate (arrow) (×200). (c) Tumor cells have an oval shape nucleus, pale chromatin and a big purple nucleolus. Mitosis is seen (arrow) (×400). (d) ALK expression by tumor cells (×200)
Figure 4
Figure 4
Axial (a and b), coronal (c) and sagittal (d) postgadolinium T1-weighted MRI studies showing tumor progression 2-months following surgery
Figure 5
Figure 5
Axial (a and b) and coronal (c) post-gadolinium T1-weighted MRI studies performed 3 months after showing no recurrence after the second surgery and one cycle of radiotherapy
Figure 6
Figure 6
Axial head CT-scan with contrast showing tumor recurrence 20 months after the second surgery
See this image and copyright information in PMC

References

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