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Macrocephaly and right arm disuse in a 7-month-old boy
Journal of Clinical Neuroscience xxx (2016) xxx–xxx
Contents lists available at ScienceDirect
Journal of Clinical Neuroscience journal homepage: www.elsevier.com/locate/jocn
Images in Neuroscience: Answer
Macrocephaly and right arm disuse in a 7-month-old boy Michael D. Staudt ⇑, Jason D. Morgenstern, Andrea Neufeld, Adrianna Ranger Department of Clinical Neurological Sciences, London Health Sciences Center, Western University, 339 Windermere Road, London, Ontario N6A 5A5, Canada
1. Answer B. Desmoplastic infantile ganglioglioma 2. Discussion The patient underwent gross total resection, and final pathology was consistent with desmoplastic infantile ganglioglioma (DIG). Intraoperatively, the tumor body was firmly attached to the dura and had an associated cystic nodule (Fig. 2A). Pathological examination of the specimen demonstrated tumor mainly in the leptomeninges, which was well-demarcated from the adjacent brain (Fig. 2B). The leptomeningeal component consisted primarily of desmoplastic areas and scattered foci with different cell types. Mitotic figures were very rare and only very minimal necrosis was visualized. The adjacent brain demonstrated mainly perivascular infiltrate, with few individual tumor cells infiltrating the cortex. Accordingly, the Ki-67 immunolabeling was low (1–5%). As the patient underwent gross total surgical resection (Fig. 2C, D) with no residual enhancement on MRI (Fig. 2E), no further adjuvant therapy was necessary. DIG is a very rare childhood tumor almost always presenting before 2 years of age [1]. Its etiology is unknown, although is likely prenatal in origin. Common presenting signs and symptoms include macrocephaly, hemiparesis, and/or signs of elevated intracranial pressure [2]. CT scan and MRI demonstrate an aggressive-appearing tumor, which can appear similar to primitive neuroectodermal tumors, atypical teratoid/rhabdoid tumors, pleomorphic xanthoastrocytoma, or ganglioglioma [3]; therefore the diagnosis cannot definitively be made with imaging alone. The mainstay of treatment is surgical resection, which is considered curative [1]. Chemotherapy is reserved for cases of brain invasion, or when partial resection is followed by radiographic evidence of non-operable disease progression. Radiotherapy may be used as a last resort in children over 5 years of age when other approaches have failed. The prognosis of both complete and partially resected DIG is excellent. Partial resection is often followed by disease
Fig. 2. (A) Operative resection of the enhancing lesion with attached cyst. (B) Hematoxylin and eosin staining of paraffin-embedded sections demonstrates tumor which is well-demarcated from adjacent brain (20 magnification). (C) Following gross total resection, the ipsilateral lateral ventricle is visible, (D) covered by a thin cortical rim. (E) Post-operative coronal T1-weighted MRI with gadolinium demonstrates improvement in overall mass effect, with small foci of enhancement favored to represent blood products and post-surgical changes.
stability or spontaneous regression, with a median disease-free survival of 8 to 30 years reported [3]. There are rare reports of local recurrence and/or metastasis, usually associated with deep-seated, particularly suprasellar tumor locations. Surveillance MRIs are generally recommended until stable disease has persisted for some time. Lifetime surveillance does not appear to be necessary as there are no reports of long-term recurrence [2].
References [1] Tamburrini G, Colosimo Jr C, Giangaspero F, et al. Desmoplastic infantile ganglioglioma. Child’s Nerv Syst: ChNS 2003;19:292–7. [2] Blount JP, Bauer DF. Desmoplastic infantile gangliogliomas. In: Tonn J-C, Westphal M, Rutka JT, editors. Oncology of CNS Tumors. Berlin Heidelberg: Springer; 2010. p. 477–81. [3] Alexiou GA, Stefanaki K, Sfakianos G, et al. Desmoplastic infantile ganglioglioma: a report of 2 cases and a review of the literature. Pediatr Neurosurg 2008;44:422–5.
DOI of question: http://dx.doi.org/10.1016/j.jocn.2016.06.016
⇑ Corresponding author.
E-mail address: [email protected] (M.D. Staudt). http://dx.doi.org/10.1016/j.jocn.2016.07.004 0967-5868/Ó 2016 Elsevier Ltd. All rights reserved.
Please cite this article in press as: Staudt MD et al. Macrocephaly and right arm disuse in a 7-month-old boy. J Clin Neurosci (2016), http://dx.doi.org/ 10.1016/j.jocn.2016.07.004
Contents lists available at ScienceDirect
Journal of Clinical Neuroscience journal homepage: www.elsevier.com/locate/jocn
Images in Neuroscience: Answer
Macrocephaly and right arm disuse in a 7-month-old boy Michael D. Staudt ⇑, Jason D. Morgenstern, Andrea Neufeld, Adrianna Ranger Department of Clinical Neurological Sciences, London Health Sciences Center, Western University, 339 Windermere Road, London, Ontario N6A 5A5, Canada
1. Answer B. Desmoplastic infantile ganglioglioma 2. Discussion The patient underwent gross total resection, and final pathology was consistent with desmoplastic infantile ganglioglioma (DIG). Intraoperatively, the tumor body was firmly attached to the dura and had an associated cystic nodule (Fig. 2A). Pathological examination of the specimen demonstrated tumor mainly in the leptomeninges, which was well-demarcated from the adjacent brain (Fig. 2B). The leptomeningeal component consisted primarily of desmoplastic areas and scattered foci with different cell types. Mitotic figures were very rare and only very minimal necrosis was visualized. The adjacent brain demonstrated mainly perivascular infiltrate, with few individual tumor cells infiltrating the cortex. Accordingly, the Ki-67 immunolabeling was low (1–5%). As the patient underwent gross total surgical resection (Fig. 2C, D) with no residual enhancement on MRI (Fig. 2E), no further adjuvant therapy was necessary. DIG is a very rare childhood tumor almost always presenting before 2 years of age [1]. Its etiology is unknown, although is likely prenatal in origin. Common presenting signs and symptoms include macrocephaly, hemiparesis, and/or signs of elevated intracranial pressure [2]. CT scan and MRI demonstrate an aggressive-appearing tumor, which can appear similar to primitive neuroectodermal tumors, atypical teratoid/rhabdoid tumors, pleomorphic xanthoastrocytoma, or ganglioglioma [3]; therefore the diagnosis cannot definitively be made with imaging alone. The mainstay of treatment is surgical resection, which is considered curative [1]. Chemotherapy is reserved for cases of brain invasion, or when partial resection is followed by radiographic evidence of non-operable disease progression. Radiotherapy may be used as a last resort in children over 5 years of age when other approaches have failed. The prognosis of both complete and partially resected DIG is excellent. Partial resection is often followed by disease
Fig. 2. (A) Operative resection of the enhancing lesion with attached cyst. (B) Hematoxylin and eosin staining of paraffin-embedded sections demonstrates tumor which is well-demarcated from adjacent brain (20 magnification). (C) Following gross total resection, the ipsilateral lateral ventricle is visible, (D) covered by a thin cortical rim. (E) Post-operative coronal T1-weighted MRI with gadolinium demonstrates improvement in overall mass effect, with small foci of enhancement favored to represent blood products and post-surgical changes.
stability or spontaneous regression, with a median disease-free survival of 8 to 30 years reported [3]. There are rare reports of local recurrence and/or metastasis, usually associated with deep-seated, particularly suprasellar tumor locations. Surveillance MRIs are generally recommended until stable disease has persisted for some time. Lifetime surveillance does not appear to be necessary as there are no reports of long-term recurrence [2].
References [1] Tamburrini G, Colosimo Jr C, Giangaspero F, et al. Desmoplastic infantile ganglioglioma. Child’s Nerv Syst: ChNS 2003;19:292–7. [2] Blount JP, Bauer DF. Desmoplastic infantile gangliogliomas. In: Tonn J-C, Westphal M, Rutka JT, editors. Oncology of CNS Tumors. Berlin Heidelberg: Springer; 2010. p. 477–81. [3] Alexiou GA, Stefanaki K, Sfakianos G, et al. Desmoplastic infantile ganglioglioma: a report of 2 cases and a review of the literature. Pediatr Neurosurg 2008;44:422–5.
DOI of question: http://dx.doi.org/10.1016/j.jocn.2016.06.016
⇑ Corresponding author.
E-mail address: [email protected] (M.D. Staudt). http://dx.doi.org/10.1016/j.jocn.2016.07.004 0967-5868/Ó 2016 Elsevier Ltd. All rights reserved.
Please cite this article in press as: Staudt MD et al. Macrocephaly and right arm disuse in a 7-month-old boy. J Clin Neurosci (2016), http://dx.doi.org/ 10.1016/j.jocn.2016.07.004