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Gangliocytic paraganglioma of filum terminale: Report of a rare case Sir, Gangliocytic paraganglioma (GP) is an extremely rare benign composite tumor, characteristically seen in the second portion of duodenum.[1,2] The lesion was first described by Dahl et al. in 1957. Kerpes and Zacharias termed this entity as “GP” in 1971.[3,4] Histologically GPs are of three cell types: Neuroendocrine, ganglion and spindle cells. The histomorphological features are common with both paraganglioma and ganglioneuroma.[4] The GPs of the filum terminale are extremely rare tumors with only few isolated cases described in the English literature. We herein report a case of GP as an incidentally detected lesion in the filum terminale. A 58‑year‑old man presented with complaint of backache radiating to the right lower limb for the last six months. There was no history of previous hospitalization for any medical or surgical illness. Neurological evaluation showed mild weakness in right external hallucis longus muscle (4/5) with no sensory deficits. Magnetic resonance imaging (MRI) of lumbo‑sacral region showed an intradural lesion (2.5 × 2.0 × 1.5 cm) at L2 spinal level. The lesion was hyperintense on T1 weighted image (WI), hypointense on T2 WI and showed homogenous enhancement following gadolinium administration. In addition, linear enhancing vascular structures were seen adjacent to the lesion [Figure 1a‑c]. Patient was taken up for surgery. Intraoperatively, a mulberry like, soft to firm, moderately vascular tumor was seen arising
a
b
from the filum and was adherent to the nerve roots of conus. Post‑operatively the patient was asymptomatic and is doing well after follow up of 16 months. Routine histological study showed a well circumscribed tumor comprising of three distinct types of cell populations, that is small neuroendocrine cells, ganglion cells and spindle cells lying separately as well as admixed with each other at places. Paraganglioma like neuroendocrine area showed uniform population of epithelial cells having round nuclei with speckled chromatin and anastomozing vascular channels in the background. These cells were diffusely immunopositive for cytokeratin, chromogranin and synaptophysin. Collections of ganglion cells (immunopositive for chromogranin and neurofilament protein) and schwannian cell population (immunopositive for S‑100 and neurofilament protein) formed the ganglioneuromatous component [Figure 2a‑c]. There was no evidence of pleomorphism, hyperchromasia, mitotic activity or necrosis. There was no evidence of hemorrhage, necrosis or mitotic activity. A final diagnosis of GP was rendered. The spectrum of intradural extramedullary tumors of the peripheral nervous system detected within the spinal canal in adults, predominantly includes schwannomas, neurofibromas and to a lesser extent paragangliomas while, retroperitoneal neuroblastomas are more common in children.[5] Paragangliomas are neuroendocrine tumors, 85-90% of which arise from the adrenal gland. Extra‑adrenal paragangliomas are most commonly seen in the carotid body, they have also been reported in the central nervous system (CNS) at the pineal region, petrous ridge, sella turcica and the spinal canal.[6] Paragangliomas of cauda equina have been well documented though, paraganglioma of the
c
Figure 1: MRI showing an (a) intradural lesion at L2 which is hyperintense on T1-WI (white arrow) and (b) Hypointense on T2-WI (white arrow) with (c) Linear enhancing vascular flow voids (arrow head)
Neurology India | Sep-Oct 2014 | Vol 62 | Issue 5
543
[Downloaded free from http://www.neurologyindia.com on Thursday, November 13, 2014, IP: 202.177.173.189] || Click here to download free Android application for journal Letters to Editor
Table 1: Literature review of gangliocytic paraganglioma involving exclusively filum terminale
a
b
c Figure 2: (a) Photomicrograph showing a tumor composed of small nests of neuroendocrine cells (*) admixed with ganglion cells (thin arrow) and schwanniann cells (thick arrow), H and E, ×100. (b) Immunohistochemical staining for chromogranin highlights neuroendocrine (*) and ganglion cell (thin arrow) components, IHC ×100. (c) The schwannian component, diffusely immunopositive for S-100, IHC ×100
filum terminale is extremely rare and gangliocytic differentiation in a paraganglioma at this region is even rarer.[6] To the best of our knowledge the index case is the sixth reported case of a composite GP in the cauda equina/filum terminale region [Table 1]. [5,7‑10] Although histogenesis of GP is controversial they usually originate from neural crest cells which give rise to sympathoadrenal lineage which further undergo divergent differentiation to form neuroendocrine and ganglion cell components.[5] The GPs are benign, encapsulated and slow growing tumors. The most common histological differential is an ependymoma which has a poorer prognosis and higher frequency of occurrence in this region.[5] There are a few case reports of GPs with malignant features described in duodenum.[11] However, till date histological features predicting malignant transformation have not been defined. The patients usually present with lumbar pain, motor or sensory loss in lower extremities or bowel and bladder dysfunction.[12] The MRI of intradural extramedullary lesions is non‑diagnostic and differential diagnosis includes schwannoma, ependymoma, meningioma or solitary metastasis. The presence of vascular flow‑voids in the vicinity of a well‑defined intradural extramedullary mass narrows the differential diagnosis to hemangioblastoma or paraganglioma.[5] Few cases of spinal GP reported in the literature have been treated by surgical resection. Adjuvant radiotherapy does not guarantee prevention of tumor recurrence and should be reserved for unencapsulated or incompletely excised lesions.[12] To conclude, GPs of 544
Study
Age/ Clinical Follow‑up sex presentation
Lerman et al., 1972 Schmitt et al., 1982
29/M 33/M
Djindjian et al., 1990
36/M
Pytel et al., 2005 Shankar et al., 2010
74/F 48/M
Low‑back pain Low‑back pain, paralysis in left foot Low‑back pain, paraplegia Low‑back pain Low‑back pain
Present case
58/M
Low‑back pain
NED, 4 months follow up Slight sensorimotor deficiency in the left leg NED, 15 months follow up Not mentioned NED, 10 months follow up NED, 16 months follow up
NED - No evidence of disease
the filum terminale are extremely rare lesions with their origin in CNS being still unclear. In view of rarity, a long term follow up along with assessment of molecular pathogenesis is essential to identify their biological behavior and therapeutic interventions.
Mukund Namdev Sable, Aasma Nalwa, Vaishali Suri, Pankaj Kumar Singh1, Ajay Garg2, Mehar Chand Sharma, Chitra Sarkar Departments of Pathology, 1Neurosurgey, 2Neuroradiology, All India Institute of Medical Sciences, New Delhi, India E‑mail: [email protected]
References 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11.
Burke AP, Helwig EB. Gangliocytic paraganglioma. Am J Clin Pathol 1989;92:1‑9. Okubo Y, Wakayama M, Nemoto T, Kitahara K, Nakayama H, Shibuya K, et al. Literature survey on epidemiology and pathology of gangliocytic paraganglioma. BMC Cancer 2011;11:187. Dahl EV, Waugh JM, Dahlin DC.Gastrointestinal ganglioneuromas; Brief review with report of a duodenal ganglioneuroma. Am J Pathol 1957;33:953‑65. Kepes JJ, Zacharias DL. Gangliocytic paragangliomas of the duodenum. A report of two cases with light and electron microscopic examination. Cancer 1971;27:61‑7. Shankar GM, Chen L, Kim AH, Ross GL, Folkerth RD, Friedlander RM. Composite ganglioneuroma‑paraganglioma of the filum terminale. J Neurosurg Spine 2010;12:709‑13. Landi A, Mancarella C, Marotta N, Tarantino R, Lenzi J. Diagnosis and treatment of paragangliomas of the filum terminale, an extremely rare entity: Personal experience and literature review. J Spine 2013;S3:e001. Pytel P, Krausz T, Wollmann R, Utset MF. Ganglioneuromatous paraganglioma of the cauda equina ‑ A pathological case study. Hum Pathol 2005;36:444‑6. S c h m i t t H P, Wu r s t e r K , B a u e r M , Pa r s c h K . M i x e d chemodectoma‑ganglioneuroma of the conus medullaris region. Acta Neuropathol 1982;57:275‑81. Lerman RI, Kaplan ES, Daman L. Ganglioneuroma‑paraganglioma of the intradural filum terminale. Case report. J Neurosurg 1972;36:652‑8. Djindjian M, Ayache P, Brugières P, Malapert D, Baudrimont M, Poirier J. Giant gangliocytic paraganglioma of the filum terminale. Case report. J Neurosurg 1990;73:459‑61. Witkiewicz A, Galler A, Yeo CJ, Gross SD. Gangliocytic paraganglioma: Case report and review of the literature. J Gastrointest Surg 2007;11:1351‑4. Neurology India | Sep-Oct 2014 | Vol 62 | Issue 5
[Downloaded free from http://www.neurologyindia.com on Thursday, November 13, 2014, IP: 202.177.173.189] || Click here to download free Android application for journal Letters to Editor
12. Sonneland PR, Scheithauer BW, LeChago J, Crawford BG, Onofrio BM. Paraganglioma of the cauda equina region. Clinicopathologic study of 31 cases with special reference to immunocytology and ultrastructure. Cancer 1986;58:1720‑35. Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.144456
Received: 10-07-2014 Review completed: 07-08-2014 Accepted: 03-10-2014
Primary plasmacytoma of the anterior skull base: A rare case
Figure 1: T1 axial post contrast MRI showing the contrast enhancing tumor involving the anterior cranial fossa base extending from the frontal and ethmoidal sinuses to the roof of the left orbit
Sir, Skull base plasmacytoma is a rare condition and accounts for 10% of all plasmacytomas. The anterior skull base plasmacytomas are extramedullary plasmacytomas which have better prognosis with surgery and adjuvant therapy. Only about 42 cases of skull base plasmacytomas have been reported till date. We present a case of skull base plasmacytoma without evidence of multiple myeloma. A 30‑year‑old man presented with progressive protrusion of the left eye with headache since six months. Examination showed left extra‑axial proptosis with displacement of eyeball downwards. Visual acuity and fundus examination were normal. Magnetic resonance imaging (MRI) showed large lesion isointense in T1 weighted images and slightly hyperintense in T2 weighted images. It was homogenously enhancing with gadolinium [Figures 1 and 2]. It was involving the anterior cranial fossa base extending from the frontal and ethmoidal sinuses to the roof of the orbit in the extradural plane. Left fronto‑temporal craniotomy was performed. The tumor was soft to firm and was eroding the skull base in the extradural plane and was extending from the frontal and ethmoidal sinuses to the roof of the entire orbit upto the optic canal. The tumor could be excised completely. Histopathological examination showed sheets of neoplastic plasma cells including binucleate forms, suggestive of plasmacytoma [Figure 3]. Bone marrow aspiration did not show evidence of myeloma. Skeletal survey was normal. Patient has undergone post‑operative radiotherapy. He is doing fine at 15 months follow‑up. Neurology India | Sep-Oct 2014 | Vol 62 | Issue 5
Figure 2: T1 coronal post contrast MRI showing the contrast enhancing tumor involving the roof of left orbit in the extradural plane
Figure 3: Histopathology picture of the tumor (H and E, ×400) showing sheets of neoplastic plasma cells including bi-nucleate forms
Plasmacytoma is a focal form of plasma cell tumor. Only 36 cases have been documented till 2002 and 545
Copyright of Neurology India is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
Gangliocytic paraganglioma of filum terminale: Report of a rare case Sir, Gangliocytic paraganglioma (GP) is an extremely rare benign composite tumor, characteristically seen in the second portion of duodenum.[1,2] The lesion was first described by Dahl et al. in 1957. Kerpes and Zacharias termed this entity as “GP” in 1971.[3,4] Histologically GPs are of three cell types: Neuroendocrine, ganglion and spindle cells. The histomorphological features are common with both paraganglioma and ganglioneuroma.[4] The GPs of the filum terminale are extremely rare tumors with only few isolated cases described in the English literature. We herein report a case of GP as an incidentally detected lesion in the filum terminale. A 58‑year‑old man presented with complaint of backache radiating to the right lower limb for the last six months. There was no history of previous hospitalization for any medical or surgical illness. Neurological evaluation showed mild weakness in right external hallucis longus muscle (4/5) with no sensory deficits. Magnetic resonance imaging (MRI) of lumbo‑sacral region showed an intradural lesion (2.5 × 2.0 × 1.5 cm) at L2 spinal level. The lesion was hyperintense on T1 weighted image (WI), hypointense on T2 WI and showed homogenous enhancement following gadolinium administration. In addition, linear enhancing vascular structures were seen adjacent to the lesion [Figure 1a‑c]. Patient was taken up for surgery. Intraoperatively, a mulberry like, soft to firm, moderately vascular tumor was seen arising
a
b
from the filum and was adherent to the nerve roots of conus. Post‑operatively the patient was asymptomatic and is doing well after follow up of 16 months. Routine histological study showed a well circumscribed tumor comprising of three distinct types of cell populations, that is small neuroendocrine cells, ganglion cells and spindle cells lying separately as well as admixed with each other at places. Paraganglioma like neuroendocrine area showed uniform population of epithelial cells having round nuclei with speckled chromatin and anastomozing vascular channels in the background. These cells were diffusely immunopositive for cytokeratin, chromogranin and synaptophysin. Collections of ganglion cells (immunopositive for chromogranin and neurofilament protein) and schwannian cell population (immunopositive for S‑100 and neurofilament protein) formed the ganglioneuromatous component [Figure 2a‑c]. There was no evidence of pleomorphism, hyperchromasia, mitotic activity or necrosis. There was no evidence of hemorrhage, necrosis or mitotic activity. A final diagnosis of GP was rendered. The spectrum of intradural extramedullary tumors of the peripheral nervous system detected within the spinal canal in adults, predominantly includes schwannomas, neurofibromas and to a lesser extent paragangliomas while, retroperitoneal neuroblastomas are more common in children.[5] Paragangliomas are neuroendocrine tumors, 85-90% of which arise from the adrenal gland. Extra‑adrenal paragangliomas are most commonly seen in the carotid body, they have also been reported in the central nervous system (CNS) at the pineal region, petrous ridge, sella turcica and the spinal canal.[6] Paragangliomas of cauda equina have been well documented though, paraganglioma of the
c
Figure 1: MRI showing an (a) intradural lesion at L2 which is hyperintense on T1-WI (white arrow) and (b) Hypointense on T2-WI (white arrow) with (c) Linear enhancing vascular flow voids (arrow head)
Neurology India | Sep-Oct 2014 | Vol 62 | Issue 5
543
[Downloaded free from http://www.neurologyindia.com on Thursday, November 13, 2014, IP: 202.177.173.189] || Click here to download free Android application for journal Letters to Editor
Table 1: Literature review of gangliocytic paraganglioma involving exclusively filum terminale
a
b
c Figure 2: (a) Photomicrograph showing a tumor composed of small nests of neuroendocrine cells (*) admixed with ganglion cells (thin arrow) and schwanniann cells (thick arrow), H and E, ×100. (b) Immunohistochemical staining for chromogranin highlights neuroendocrine (*) and ganglion cell (thin arrow) components, IHC ×100. (c) The schwannian component, diffusely immunopositive for S-100, IHC ×100
filum terminale is extremely rare and gangliocytic differentiation in a paraganglioma at this region is even rarer.[6] To the best of our knowledge the index case is the sixth reported case of a composite GP in the cauda equina/filum terminale region [Table 1]. [5,7‑10] Although histogenesis of GP is controversial they usually originate from neural crest cells which give rise to sympathoadrenal lineage which further undergo divergent differentiation to form neuroendocrine and ganglion cell components.[5] The GPs are benign, encapsulated and slow growing tumors. The most common histological differential is an ependymoma which has a poorer prognosis and higher frequency of occurrence in this region.[5] There are a few case reports of GPs with malignant features described in duodenum.[11] However, till date histological features predicting malignant transformation have not been defined. The patients usually present with lumbar pain, motor or sensory loss in lower extremities or bowel and bladder dysfunction.[12] The MRI of intradural extramedullary lesions is non‑diagnostic and differential diagnosis includes schwannoma, ependymoma, meningioma or solitary metastasis. The presence of vascular flow‑voids in the vicinity of a well‑defined intradural extramedullary mass narrows the differential diagnosis to hemangioblastoma or paraganglioma.[5] Few cases of spinal GP reported in the literature have been treated by surgical resection. Adjuvant radiotherapy does not guarantee prevention of tumor recurrence and should be reserved for unencapsulated or incompletely excised lesions.[12] To conclude, GPs of 544
Study
Age/ Clinical Follow‑up sex presentation
Lerman et al., 1972 Schmitt et al., 1982
29/M 33/M
Djindjian et al., 1990
36/M
Pytel et al., 2005 Shankar et al., 2010
74/F 48/M
Low‑back pain Low‑back pain, paralysis in left foot Low‑back pain, paraplegia Low‑back pain Low‑back pain
Present case
58/M
Low‑back pain
NED, 4 months follow up Slight sensorimotor deficiency in the left leg NED, 15 months follow up Not mentioned NED, 10 months follow up NED, 16 months follow up
NED - No evidence of disease
the filum terminale are extremely rare lesions with their origin in CNS being still unclear. In view of rarity, a long term follow up along with assessment of molecular pathogenesis is essential to identify their biological behavior and therapeutic interventions.
Mukund Namdev Sable, Aasma Nalwa, Vaishali Suri, Pankaj Kumar Singh1, Ajay Garg2, Mehar Chand Sharma, Chitra Sarkar Departments of Pathology, 1Neurosurgey, 2Neuroradiology, All India Institute of Medical Sciences, New Delhi, India E‑mail: [email protected]
References 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11.
Burke AP, Helwig EB. Gangliocytic paraganglioma. Am J Clin Pathol 1989;92:1‑9. Okubo Y, Wakayama M, Nemoto T, Kitahara K, Nakayama H, Shibuya K, et al. Literature survey on epidemiology and pathology of gangliocytic paraganglioma. BMC Cancer 2011;11:187. Dahl EV, Waugh JM, Dahlin DC.Gastrointestinal ganglioneuromas; Brief review with report of a duodenal ganglioneuroma. Am J Pathol 1957;33:953‑65. Kepes JJ, Zacharias DL. Gangliocytic paragangliomas of the duodenum. A report of two cases with light and electron microscopic examination. Cancer 1971;27:61‑7. Shankar GM, Chen L, Kim AH, Ross GL, Folkerth RD, Friedlander RM. Composite ganglioneuroma‑paraganglioma of the filum terminale. J Neurosurg Spine 2010;12:709‑13. Landi A, Mancarella C, Marotta N, Tarantino R, Lenzi J. Diagnosis and treatment of paragangliomas of the filum terminale, an extremely rare entity: Personal experience and literature review. J Spine 2013;S3:e001. Pytel P, Krausz T, Wollmann R, Utset MF. Ganglioneuromatous paraganglioma of the cauda equina ‑ A pathological case study. Hum Pathol 2005;36:444‑6. S c h m i t t H P, Wu r s t e r K , B a u e r M , Pa r s c h K . M i x e d chemodectoma‑ganglioneuroma of the conus medullaris region. Acta Neuropathol 1982;57:275‑81. Lerman RI, Kaplan ES, Daman L. Ganglioneuroma‑paraganglioma of the intradural filum terminale. Case report. J Neurosurg 1972;36:652‑8. Djindjian M, Ayache P, Brugières P, Malapert D, Baudrimont M, Poirier J. Giant gangliocytic paraganglioma of the filum terminale. Case report. J Neurosurg 1990;73:459‑61. Witkiewicz A, Galler A, Yeo CJ, Gross SD. Gangliocytic paraganglioma: Case report and review of the literature. J Gastrointest Surg 2007;11:1351‑4. Neurology India | Sep-Oct 2014 | Vol 62 | Issue 5
[Downloaded free from http://www.neurologyindia.com on Thursday, November 13, 2014, IP: 202.177.173.189] || Click here to download free Android application for journal Letters to Editor
12. Sonneland PR, Scheithauer BW, LeChago J, Crawford BG, Onofrio BM. Paraganglioma of the cauda equina region. Clinicopathologic study of 31 cases with special reference to immunocytology and ultrastructure. Cancer 1986;58:1720‑35. Access this article online Quick Response Code:
Website: www.neurologyindia.com PMID: *** DOI: 10.4103/0028-3886.144456
Received: 10-07-2014 Review completed: 07-08-2014 Accepted: 03-10-2014
Primary plasmacytoma of the anterior skull base: A rare case
Figure 1: T1 axial post contrast MRI showing the contrast enhancing tumor involving the anterior cranial fossa base extending from the frontal and ethmoidal sinuses to the roof of the left orbit
Sir, Skull base plasmacytoma is a rare condition and accounts for 10% of all plasmacytomas. The anterior skull base plasmacytomas are extramedullary plasmacytomas which have better prognosis with surgery and adjuvant therapy. Only about 42 cases of skull base plasmacytomas have been reported till date. We present a case of skull base plasmacytoma without evidence of multiple myeloma. A 30‑year‑old man presented with progressive protrusion of the left eye with headache since six months. Examination showed left extra‑axial proptosis with displacement of eyeball downwards. Visual acuity and fundus examination were normal. Magnetic resonance imaging (MRI) showed large lesion isointense in T1 weighted images and slightly hyperintense in T2 weighted images. It was homogenously enhancing with gadolinium [Figures 1 and 2]. It was involving the anterior cranial fossa base extending from the frontal and ethmoidal sinuses to the roof of the orbit in the extradural plane. Left fronto‑temporal craniotomy was performed. The tumor was soft to firm and was eroding the skull base in the extradural plane and was extending from the frontal and ethmoidal sinuses to the roof of the entire orbit upto the optic canal. The tumor could be excised completely. Histopathological examination showed sheets of neoplastic plasma cells including binucleate forms, suggestive of plasmacytoma [Figure 3]. Bone marrow aspiration did not show evidence of myeloma. Skeletal survey was normal. Patient has undergone post‑operative radiotherapy. He is doing fine at 15 months follow‑up. Neurology India | Sep-Oct 2014 | Vol 62 | Issue 5
Figure 2: T1 coronal post contrast MRI showing the contrast enhancing tumor involving the roof of left orbit in the extradural plane
Figure 3: Histopathology picture of the tumor (H and E, ×400) showing sheets of neoplastic plasma cells including bi-nucleate forms
Plasmacytoma is a focal form of plasma cell tumor. Only 36 cases have been documented till 2002 and 545
Copyright of Neurology India is the property of Medknow Publications & Media Pvt. Ltd. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
