Journal of the Egyptian National Cancer Institute (2014) 26, 181–183
Cairo University
Journal of the Egyptian National Cancer Institute www.elsevier.com/locate/jnci www.sciencedirect.com
Letter to the Editor
Primitive neuroectodermal tumor masquerading meningioma Dear Sir, Extracerebellar occurrence of primitive neuroectodermal tumors (PNETs) is uncommon and mostly it is supratentorial intraparenchymal and less frequently in the spinal cord [1–5]. An 18 year old male patient presented with a gradually progressive swelling over his right temporal region of 4 months duration. He had one episode of generalized tonic–clonic seizures 9 months ago. He was started on anticonvulsants but not investigated further. There was no history of fever, trauma or altered sensorium. There was no papilledema, cranial nerves were normal. There were no focal neurological deficits. He showed a left temporal extra axial 88 mm · 77 mm · 47 mm solid signal intensity lesion with a broad based attachment to the dura, and lytic destruction of adjacent squamous temporal bone with an associated overlying extra calvarial sub galeal dome shaped solid component. Lesion shows heterogeneous signals and enhancement due to areas of necrosis. A focal breech was noted in the dura with a possible adjacent left temporal lobe invasion, with a T2 hyper intense edema in the compressed left temporal lobe (see Figs. 1 and 2). Though the MRI showed bone destruction, X-ray skull or a CT scan was
not done, it has been planned as a later investigation, for cranioplasty. The patient underwent left temporal craniotomy and total excision of the tumor. Intraoperatively, the lesion was vascular, with attachment to sphenoid ridge; some part of the lesion had involved the dura and the bone Fig. 3. It was having its blood supply from the bone and the dura. The surgical histopathology revealed cellular malignant small round (Blue) cell tumor composed of syncytial aggregates and lobular sheets of fairly monotonous appearing small round cells having round to oval hyperchromatic nuclei, finely stippled chromatin and smooth nuclear membranes with pale amphophilic to eosinophilic cytoplasm and indistinct margins. An occasional cell showed nucleoli. The minimal interstitial stroma revealed congested blood vessels, confluent foci of coagulative ischemic necrosis and few foci showing pseudorosettes. Overall the entire tumor showed scattered mitosis, however no endothelial – lined space invasion was seen. The immunohistochemistry profile showed the tumor cells to express CD 99 and was immunonegative for cytokeratin, EMA, desmin, GFAP, synaptophysin & LCA – concluding it to be Primitive neuroectodermal tumor (PNET). The patient made uneventful recovery in postoperative period without any neurological deficits.
Figure 1 MRI BRAIN Axial Post contrast T1w images show the lesion, lytic destruction of adjacent squamous temporal bone with an associated overlying extra calvarial sub galeal dome shaped solid component. Lesion shows heterogeneous enhancement due to areas of necrosis. Mass effect significant_ mid line shift of 13 mm to right with sub falcine as well as uncal herniation. Severe mid brain compression.
Peer review under responsibility of The National Cancer Institute, Cairo University. http://dx.doi.org/10.1016/j.jnci.2014.06.002 1110-0362 ª 2014 Production and hosting by Elsevier B.V. on behalf of National Cancer Institute, Cairo University.
182
Letter to the Editor
Figure 2 MRI BRAIN Coronal Post contrast T1w and Coronal T2w images show a left temporal extra axial 88 mm · 77 mm · 47 mm solid signal intensity lesion with a broad based attachment to the dura, and lytic destruction of adjacent squamous temporal bone with an associated overlying extra calvarial sub galeal dome shaped solid component. Lesion show heterogeneous signals and enhancement due to areas of necrosis. A focal breech was noted in the dura with a possible adjacent left temporal lobe invasion, with a T2 hyper intense edema in the compressed left temporal lobe.
[1,10,11,17]. Fortunately we performed the total excision of the tumor as for any other meningioma which is also a recommended option for the PNET [1,10,11,17]. In spite of the early and aggressive interdisciplinary adjuvant treatment even after complete neurosurgical resection PNETs show much more aggressive behavior and have poorer outcome [1,10,18–22]. Conflict of interest None declared. References
Figure 3 Intraoperative picture shows the lesion was involving the dura as well as the sphenoid bone. The lesion was extradural but also there was intradural spread. Blood supply of the lesion was from the sphenoid ridge and the dura.
Extraparenchymal primitive neuroectodermal tumors can present either with diffuse involvement of the cranial and spinal lepto-meninges (no primary intraparenchymal or meningeal tumor) [2,6,7] or can present as a localized dural-based mass that can mimic meningioma [2,8–10]. Meningeal localization of primitive neuroectodermal tumors is extremely uncommon with only few reported cases in the literature [3,2,6–12]. Although involvement of the bone can be seen as erosive lesion of plain skull radiographs [13] CT and MR imaging (with contrast administration) will provide greater details of the lesions [1,14–16]. Based on clinical and imaging appearances it is difficult to suspect the diagnosis of PNET and only the histological findings can confirm the diagnosis of these rare lesions
[1] Kuroiwa T, Moriwaki K, Ohta T, Tsutsumi A, Tajima S. Extradural extension of primitive neuroectodermal tumor–case report. Neurol Med Chir (Tokyo) 1994;34:379–81. [2] Fujii M, Orita T, Nishizaki T, Aoki H, Tanaka K. Primitive neuroectodermal tumor of the leptomeninges. Neuroradiology 1991;33:260–3. [3] Hart MN, Earle KM. Primitive neuroectodermal tumors of the brain in children. Cancer 1973;32:890–7. [4] Gregorio A, Corrias MV, Castriconi R, Dondero A, Mosconi M, Gambini C, et al.. Small round blue cell tumours: diagnostic and prognostic usefulness of the expression of B7-H3 surface molecule. Histopathology 2008;53:73–80. [5] Meis-Kindblom JM, Stenman G, Kindblom LG. Differential diagnosis of small round cell tumors. Semin Diagn Pathol 1996;13:213–41. [6] Mendal RC, Pollay M, Bobele GB, Leech RW, Brumback RA. Primary primitive neuroectodermal tumor of the leptomeninges. J Child Neurol 1996;11:404–7. [7] Szpak GM, Papierz W, Liberski PP, Kulczycki J, KrystWidz´gowska T, Dymecki J. Primitive neuroectodermal tumor (PNET). A case report. Folia Neuropathol 1995;33: 35–40. [8] Antunes NL, Lellouch-Tubiana A, Kalifa C, Delattre O, PierreKahn A, Rosenblum MK. Intracranial Ewing sarcoma/ ‘peripheral’ primitive neuroectodermal tumor of dural origin with molecular genetic confirmation. J Neurooncol 2001;51:51–6.
Letter to the Editor [9] Katayama Y, Kimura S, Watanabe T, Yoshino A, Koshinaga M. Peripheral-type primitive neuroectodermal tumorarising in the tentorium. Case report. J Neurosurg 1999;90: 141–4. [10] Papotti M, Abbona G, Pagani A, Monga G, Bussolati G. Primitive neuroectodermal tumor of the meninges: an histological, immunohistochemical, ultrastructural, and cytogenetic study. Endocr Pathol 1998;9:275–80. [11] Dedeurwaerdere F, Giannini C, Sciot R, Rubin BP, Perilongo G, Borghi L, et al.. Primary peripheral PNET/Ewing’s sarcoma of the dura: a clinicopathologic entity distinct from central PNET. Mod Pathol 2002;15:673–8. [12] Ishii N, Sawamura Y, Aoki T, Nagashima K, Kashiwaba T, Abe H. Intracranial extracerebral neuroblastoma originating from the convexity dura mater. Neurol Med Chir (Tokyo) 1996;36:19–22. [13] Duffner PK, Cohen ME, Heffner RR, Freeman AI. Primitive neuroectodermal tumors of childhood. An approach to therapy. J Neurosurg 1981;55:376–81. [14] Chawla A, Emmanuel J, Seow W, Lou J, Teo H, Lim C. Paediatric PNET: pre-surgical MRI features. Clin Radiol 2007;62:43–52. [15] Dai AI, Backstrom JW, Burger PC, Duffner PK. Supratentorial primitive neuroectodermal tumors of infancy: clinical and radiologic findings. Pediatr Neurol 2003;29:430–4. [16] Altman N, Fitz CR, Chuang S, Harwood-Nash D, Cotter C, Armstrong D. Radiologic characteristics of primitive neuroectodermal tumors in children. AJNR Am J Neuroradiol 1985;6:15–8. [17] Rorke LB. The cerebellar medulloblastoma and its relationship to primitive neuroectodermal tumors. J Neuropathol Exp Neurol 1983;42:1–15. [18] Burkhardt JK, Kockro RA, Dohmen-Scheufler H, Woernle CM, Bellut D, Kollias S, et al.. Small supratentorial, extraaxial primitive neuroectodermal tumor causing large intracerebral hematoma. Neurol Med Chir (Tokyo) 2011;51:441–4. [19] Chang CH, Housepian EM, Herbert C. An operative staging system and a megavoltage radiotherapeutic technic for cerebellar medulloblastomas. Radiology 1969;93:1351–9. [20] Chintagumpala M, Hassall T, Palmer S, Ashley D, Wallace D, Kasow K, et al.. A pilot study of risk-adapted radiotherapy and chemotherapy in patients with supratentorial PNET. Neuro Oncol 2009;11:33–40.
183 [21] Dirks PB, Harris L, Hoffman HJ, Humphreys RP, Drake JM, Rutka JT. Supratentorial primitive neuroectodermal tumors in children. J Neurooncol 1996;29:75–84. [22] Niwa J, Shimoyama N, Takahashi Y. Primitive neuroectodermal tumor involving the frontal skull base in an infant. Childs Nerv Syst 2001;17:570–4.
Vijay P. Joshi * Department of Neurosurgery, S P Institute of Neurosciences, India * Corresponding author. Address: Ashwini Sahakari Rugnalaya, Yashodhara Superspeciality Hospital, India. E-mail address: [email protected]
Shirish Valsangkar Department of Neurology, S P Institute of Neuroscience, India
Anish Dekhne Department of Pathology, S P Institute of Neurosciences, India
Balaji Anvekar Department of Radiology, S P Institute of Neurosciences, India
Chakrapani Tumma Department of Anaesthesiology, S P Institute of Neurosciences, India
Amit Agrawal Department of Neurosurgery, Narayana Medical College Hospital, Chinthareddypalem, Nellore, Andhra Pradesh, India Available online 29 August 2014
Cairo University
Journal of the Egyptian National Cancer Institute www.elsevier.com/locate/jnci www.sciencedirect.com
Letter to the Editor
Primitive neuroectodermal tumor masquerading meningioma Dear Sir, Extracerebellar occurrence of primitive neuroectodermal tumors (PNETs) is uncommon and mostly it is supratentorial intraparenchymal and less frequently in the spinal cord [1–5]. An 18 year old male patient presented with a gradually progressive swelling over his right temporal region of 4 months duration. He had one episode of generalized tonic–clonic seizures 9 months ago. He was started on anticonvulsants but not investigated further. There was no history of fever, trauma or altered sensorium. There was no papilledema, cranial nerves were normal. There were no focal neurological deficits. He showed a left temporal extra axial 88 mm · 77 mm · 47 mm solid signal intensity lesion with a broad based attachment to the dura, and lytic destruction of adjacent squamous temporal bone with an associated overlying extra calvarial sub galeal dome shaped solid component. Lesion shows heterogeneous signals and enhancement due to areas of necrosis. A focal breech was noted in the dura with a possible adjacent left temporal lobe invasion, with a T2 hyper intense edema in the compressed left temporal lobe (see Figs. 1 and 2). Though the MRI showed bone destruction, X-ray skull or a CT scan was
not done, it has been planned as a later investigation, for cranioplasty. The patient underwent left temporal craniotomy and total excision of the tumor. Intraoperatively, the lesion was vascular, with attachment to sphenoid ridge; some part of the lesion had involved the dura and the bone Fig. 3. It was having its blood supply from the bone and the dura. The surgical histopathology revealed cellular malignant small round (Blue) cell tumor composed of syncytial aggregates and lobular sheets of fairly monotonous appearing small round cells having round to oval hyperchromatic nuclei, finely stippled chromatin and smooth nuclear membranes with pale amphophilic to eosinophilic cytoplasm and indistinct margins. An occasional cell showed nucleoli. The minimal interstitial stroma revealed congested blood vessels, confluent foci of coagulative ischemic necrosis and few foci showing pseudorosettes. Overall the entire tumor showed scattered mitosis, however no endothelial – lined space invasion was seen. The immunohistochemistry profile showed the tumor cells to express CD 99 and was immunonegative for cytokeratin, EMA, desmin, GFAP, synaptophysin & LCA – concluding it to be Primitive neuroectodermal tumor (PNET). The patient made uneventful recovery in postoperative period without any neurological deficits.
Figure 1 MRI BRAIN Axial Post contrast T1w images show the lesion, lytic destruction of adjacent squamous temporal bone with an associated overlying extra calvarial sub galeal dome shaped solid component. Lesion shows heterogeneous enhancement due to areas of necrosis. Mass effect significant_ mid line shift of 13 mm to right with sub falcine as well as uncal herniation. Severe mid brain compression.
Peer review under responsibility of The National Cancer Institute, Cairo University. http://dx.doi.org/10.1016/j.jnci.2014.06.002 1110-0362 ª 2014 Production and hosting by Elsevier B.V. on behalf of National Cancer Institute, Cairo University.
182
Letter to the Editor
Figure 2 MRI BRAIN Coronal Post contrast T1w and Coronal T2w images show a left temporal extra axial 88 mm · 77 mm · 47 mm solid signal intensity lesion with a broad based attachment to the dura, and lytic destruction of adjacent squamous temporal bone with an associated overlying extra calvarial sub galeal dome shaped solid component. Lesion show heterogeneous signals and enhancement due to areas of necrosis. A focal breech was noted in the dura with a possible adjacent left temporal lobe invasion, with a T2 hyper intense edema in the compressed left temporal lobe.
[1,10,11,17]. Fortunately we performed the total excision of the tumor as for any other meningioma which is also a recommended option for the PNET [1,10,11,17]. In spite of the early and aggressive interdisciplinary adjuvant treatment even after complete neurosurgical resection PNETs show much more aggressive behavior and have poorer outcome [1,10,18–22]. Conflict of interest None declared. References
Figure 3 Intraoperative picture shows the lesion was involving the dura as well as the sphenoid bone. The lesion was extradural but also there was intradural spread. Blood supply of the lesion was from the sphenoid ridge and the dura.
Extraparenchymal primitive neuroectodermal tumors can present either with diffuse involvement of the cranial and spinal lepto-meninges (no primary intraparenchymal or meningeal tumor) [2,6,7] or can present as a localized dural-based mass that can mimic meningioma [2,8–10]. Meningeal localization of primitive neuroectodermal tumors is extremely uncommon with only few reported cases in the literature [3,2,6–12]. Although involvement of the bone can be seen as erosive lesion of plain skull radiographs [13] CT and MR imaging (with contrast administration) will provide greater details of the lesions [1,14–16]. Based on clinical and imaging appearances it is difficult to suspect the diagnosis of PNET and only the histological findings can confirm the diagnosis of these rare lesions
[1] Kuroiwa T, Moriwaki K, Ohta T, Tsutsumi A, Tajima S. Extradural extension of primitive neuroectodermal tumor–case report. Neurol Med Chir (Tokyo) 1994;34:379–81. [2] Fujii M, Orita T, Nishizaki T, Aoki H, Tanaka K. Primitive neuroectodermal tumor of the leptomeninges. Neuroradiology 1991;33:260–3. [3] Hart MN, Earle KM. Primitive neuroectodermal tumors of the brain in children. Cancer 1973;32:890–7. [4] Gregorio A, Corrias MV, Castriconi R, Dondero A, Mosconi M, Gambini C, et al.. Small round blue cell tumours: diagnostic and prognostic usefulness of the expression of B7-H3 surface molecule. Histopathology 2008;53:73–80. [5] Meis-Kindblom JM, Stenman G, Kindblom LG. Differential diagnosis of small round cell tumors. Semin Diagn Pathol 1996;13:213–41. [6] Mendal RC, Pollay M, Bobele GB, Leech RW, Brumback RA. Primary primitive neuroectodermal tumor of the leptomeninges. J Child Neurol 1996;11:404–7. [7] Szpak GM, Papierz W, Liberski PP, Kulczycki J, KrystWidz´gowska T, Dymecki J. Primitive neuroectodermal tumor (PNET). A case report. Folia Neuropathol 1995;33: 35–40. [8] Antunes NL, Lellouch-Tubiana A, Kalifa C, Delattre O, PierreKahn A, Rosenblum MK. Intracranial Ewing sarcoma/ ‘peripheral’ primitive neuroectodermal tumor of dural origin with molecular genetic confirmation. J Neurooncol 2001;51:51–6.
Letter to the Editor [9] Katayama Y, Kimura S, Watanabe T, Yoshino A, Koshinaga M. Peripheral-type primitive neuroectodermal tumorarising in the tentorium. Case report. J Neurosurg 1999;90: 141–4. [10] Papotti M, Abbona G, Pagani A, Monga G, Bussolati G. Primitive neuroectodermal tumor of the meninges: an histological, immunohistochemical, ultrastructural, and cytogenetic study. Endocr Pathol 1998;9:275–80. [11] Dedeurwaerdere F, Giannini C, Sciot R, Rubin BP, Perilongo G, Borghi L, et al.. Primary peripheral PNET/Ewing’s sarcoma of the dura: a clinicopathologic entity distinct from central PNET. Mod Pathol 2002;15:673–8. [12] Ishii N, Sawamura Y, Aoki T, Nagashima K, Kashiwaba T, Abe H. Intracranial extracerebral neuroblastoma originating from the convexity dura mater. Neurol Med Chir (Tokyo) 1996;36:19–22. [13] Duffner PK, Cohen ME, Heffner RR, Freeman AI. Primitive neuroectodermal tumors of childhood. An approach to therapy. J Neurosurg 1981;55:376–81. [14] Chawla A, Emmanuel J, Seow W, Lou J, Teo H, Lim C. Paediatric PNET: pre-surgical MRI features. Clin Radiol 2007;62:43–52. [15] Dai AI, Backstrom JW, Burger PC, Duffner PK. Supratentorial primitive neuroectodermal tumors of infancy: clinical and radiologic findings. Pediatr Neurol 2003;29:430–4. [16] Altman N, Fitz CR, Chuang S, Harwood-Nash D, Cotter C, Armstrong D. Radiologic characteristics of primitive neuroectodermal tumors in children. AJNR Am J Neuroradiol 1985;6:15–8. [17] Rorke LB. The cerebellar medulloblastoma and its relationship to primitive neuroectodermal tumors. J Neuropathol Exp Neurol 1983;42:1–15. [18] Burkhardt JK, Kockro RA, Dohmen-Scheufler H, Woernle CM, Bellut D, Kollias S, et al.. Small supratentorial, extraaxial primitive neuroectodermal tumor causing large intracerebral hematoma. Neurol Med Chir (Tokyo) 2011;51:441–4. [19] Chang CH, Housepian EM, Herbert C. An operative staging system and a megavoltage radiotherapeutic technic for cerebellar medulloblastomas. Radiology 1969;93:1351–9. [20] Chintagumpala M, Hassall T, Palmer S, Ashley D, Wallace D, Kasow K, et al.. A pilot study of risk-adapted radiotherapy and chemotherapy in patients with supratentorial PNET. Neuro Oncol 2009;11:33–40.
183 [21] Dirks PB, Harris L, Hoffman HJ, Humphreys RP, Drake JM, Rutka JT. Supratentorial primitive neuroectodermal tumors in children. J Neurooncol 1996;29:75–84. [22] Niwa J, Shimoyama N, Takahashi Y. Primitive neuroectodermal tumor involving the frontal skull base in an infant. Childs Nerv Syst 2001;17:570–4.
Vijay P. Joshi * Department of Neurosurgery, S P Institute of Neurosciences, India * Corresponding author. Address: Ashwini Sahakari Rugnalaya, Yashodhara Superspeciality Hospital, India. E-mail address: [email protected]
Shirish Valsangkar Department of Neurology, S P Institute of Neuroscience, India
Anish Dekhne Department of Pathology, S P Institute of Neurosciences, India
Balaji Anvekar Department of Radiology, S P Institute of Neurosciences, India
Chakrapani Tumma Department of Anaesthesiology, S P Institute of Neurosciences, India
Amit Agrawal Department of Neurosurgery, Narayana Medical College Hospital, Chinthareddypalem, Nellore, Andhra Pradesh, India Available online 29 August 2014
