- Email: [email protected]
Intraneural perineurioma
1633
Case Reports / Journal of Clinical Neuroscience 16 (2009) 1633–1636
sure used for stent deployment, as well as the diameter of both the stent and balloon, which may result in underexpansion and incomplete apposition, as illustrated in our patient. For this reason, we always select undersized stents to use in those patients with intracranial stenosis, so as to reduce the risk of peri-interventional stroke. Acknowledgement This study is partly supported by Key Project from the Shanghai Committee of Science and Technology (074119506). References 1. Wong KS, Huang YN, Yang HB, et al. A door-to-door survey of intracranial atherosclerosis in Liangbei County, China. Neurology 2007;69:2031–4. 2. Jiang WJ, Xu XT, Jin M, et al. Apollo stent for symptomatic atherosclerotic intracranial stenosis: study results. Am J Neuroradiol 2007;28:830–4. 3. The SSYLVIA study investigators. Stenting of symptomatic atherosclerotic lesions in the vertebral or intracranial arteries (SSYLVIA): study results. Stroke 2004;35:1388–92.
4. Qureshi AI, Kirmani JF, Hussein HM, et al. Early- and intermediate-term outcomes with drug-eluting stents in high-risk patients with symptomatic intracranial stenosis. Neurosurgery 2006;59:1044–51. 5. Abou-Chebl A, Bashir Q, Yadav JS, et al. Drug-eluting stents for the treatment of intracranial atherosclerosis: initial experience and midterm angiographic follow-up. Stroke 2005;36:E165–8. 6. Jiang WJ, Xu XT, Du B, et al. Comparison of elective stenting of severe vs. moderate intracranial atherosclerotic stenosis. Neurology 2007;68: 420–6. 7. Holmes Jr DR, Leon MB, Moses JW, et al. Analysis of 1-year clinical outcomes in the SIRIUS trial: a randomized trial of a sirolimus-eluting stent versus a standard stent in patients at high risk for coronary restenosis. Circulation 2004;109:634–40. 8. Lee TH, Choi CH, Park KP, et al. Techniques for intracranial stent navigation in patients with tortuous vessels. Am J Neuroradiol 2005;26:1375–80. 9. Iakavou I, Schimdt T, Bonizzoni E, et al. Incidence, predictors and outcome of thrombosis after successful implantation of drug-eluting stents. JAMA 2005;293:2126–30. 10. Eisenstein EL, Anstrom KJ, Kong DF, et al. Clopidogrel use and long-term clinical outcomes after drug-eluting stent implantation. JAMA 2007;297:159–68. 11. Serruys PW, Daemen J. Late stent thrombosis: a nuisance in both bare metal and drug-eluting stents. Circulation 2007;115:1433–9.
doi:10.1016/j.jocn.2009.01.025
Intraneural perineurioma H.-Y. Lee a, R.G. Manasseh b, R.H. Edis b, R. Page c, J. Keith-Rokosh a,*, P. Walsh d, S. Song e, A. Laycock f, L. Griffiths a, V.A. Fabian a a
Department of Anatomical Pathology, Section of Neuropathology, Royal Perth Hospital, 2nd Floor North Block, Wellington Street Campus, Perth, Western Australia 6847, Australia Department of Neurology, Royal Perth Hospital, Perth, Western Australia, Australia c Department of Plastic Surgery, Princess Margaret Hospital for Children, Subiaco, Western Australia, Australia d Department of Neurology, Princess Margaret Hospital for Children, Subiaco, Western Australia, Australia e Department of Radiology, Royal Perth Hospital, Perth, Western Australia, Australia f Department of Anatomical Pathology, Princess Margaret Hospital for Children, Subiaco, Western Australia, Australia b
a r t i c l e
i n f o
Article history: Received 22 December 2008 Accepted 8 February 2009
Keywords: Intraneural perineurioma Nerve Pseudo-onion bulbs Neuropathology Management Nerve grafting
a b s t r a c t Intraneural perineurioma is a rare tumour that affects peripheral nerves and, based on its histological features, may be confused with hereditary motor and sensory neuropathies. Detailed neuropathology, including immunoperoxidase stains and electron microscopy, is vital to distinguish these conditions. We report two patients with intraneural perineurioma that demonstrate salient features of this tumour. The first patient is the longest reported follow-up of an intraneural perineurioma; extension of the lesion was observed over 14 years. The second patient is an 11-year-old female whose treatment highlights some of the controversy surrounding the management of these lesions, and the importance of thorough macroscopic and microscopic assessment by pathologists, including the status of surgical resection margins.
1. Case reports 1.1. Patient 1 A 16-year-old male presented with a 4-year history of a progressive limp. There was no history of trauma and no family history of pes cavus or neuromuscular disease. Clinical examination demonstrated shortening of the right leg and wasting and weakness of the right hamstring muscle and all of the muscles below * Corresponding author. Tel.: +618 9224 2463; fax: +61 9224 2556. E-mail address: [email protected] (J. Keith-Rokosh)
Ó 2009 Elsevier Ltd. All rights reserved.
the right knee. The right knee-jerk was normal but the right ankle-jerk was absent. There was marked hypoesthesia and hypoalgesia involving the entire right foot and the lateral aspect of the right calf. A fusiform area of thickening was palpated in the region of the right sciatic nerve from the mid-thigh to the popliteal fossa. Nerve conduction studies did not demonstrate right tibial and common peroneal motor responses, and the right sural response was absent. Needle electromyography examination demonstrated chronic active denervation in the right hamstring muscle and all of the muscles below the right knee. These abnormalities were consistent with a focal lesion of the right sciatic nerve between the mid-thigh and the knee.
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Case Reports / Journal of Clinical Neuroscience 16 (2009) 1633–1636
Fig. 1. (a) Axial T1-weighted post-gadolinium MRI of the lower thighs showing an enlarged right tibial nerve (arrow). (b) Axial T1-weighted MRI of the pelvis showing an enlarged right sciatic nerve (arrow). (c) Enlarged nerve fascicles with typical pseudo-onion bulbs formed by concentric proliferations of spindle cells (hematoxylin and eosin, 160). (d) Epithelial membrane antigen (EMA) immunoreactivity in concentrically arranged spindle cells (EMA, 160). (e) Perineurial cells in a concentric arrangement (electron microscopy (EM) ultrastructure, 3000). (f) Perineurial cells with pinocytotic vesicles (arrow). A single unmyelinated axon and an accompanying Schwann cell are shown centrally (EM ultrastructure 27 500). (g) Perineurial cell with incomplete basal lamina (arrow) (EM ultrastructure 57 500).
MRI of the thigh demonstrated thickening of the right distal sciatic and proximal tibial nerves (Fig. 1a). MRI of the lumbar spine was normal. A biopsy of the right posterior tibial nerve was initially reported as a chronic hypertrophic neuropathy. Over the next 14 years there was progressive wasting and weakness of the right hamstring muscle and all of the muscles below the right knee, as well as worsening sensory loss and pain involving the distal right leg. There was eventual wasting of the right gluteal muscles. To evaluate the proximal progression of motor symptoms, MRI of the pelvic area was performed (Fig. 1b). Fusiform thickening of the sciatic nerve extended through the sciatic notch into the upper buttock, affecting the superior and inferior gluteal nerves, and extended distally into the proximal tibial and peroneal nerves. Review of the nerve biopsy initially reported as a chronic hypertrophic neuropathy showed expansion of the nerve fascicles by concentric layers of spindle cells around nerve fibres (Fig. 1c), which were positive on immunoperoxidase staining with Epithelial Membrane Antigen (EMA), (1:200 dilution of clone E29, Dako, Glostrup, Denmark) (Fig. 1d). Ultrastructural examination confirmed concentric arrangements of perineurial cells, which were identified by their long, thin cytoplasmic processes, numerous
pinocytotic vesicles and fragmented basal lamina around a central axon and accompanying Schwann cell (Fig. 1e–g). These findings were consistent with a perineurioma.
1.2. Patient 2 An 11-year-old female presented with a left foot drop. There was no associated pain or sensory abnormality, and family history was non-contributory. On examination she had atrophy of the left peroneal muscle with a left foot drop. The left leg was 0.5 cm shorter, and the left foot 0.5 cm smaller, than the right. There was marked weakness of the left foot and toe extension and some weakness of ankle eversion. Sensation was intact. Nerve conduction studies demonstrated conduction was absent in the left peroneal nerve. Her MRI showed fusiform irregular expansion of the left common peroneal nerve in the mid-thigh, with segments of normal calibre nerve intervening between areas of tubular expansion (Fig. 2a). The T2-weighted MRI showed neither hyperintensity, nor contrast enhancement, of the nerve. The MRI findings were considered compatible with a hypertrophic neuropathy involving the left common peroneal nerve.
Case Reports / Journal of Clinical Neuroscience 16 (2009) 1633–1636
1635
Fig. 2. (a) Axial T1-weighted post-gadolinium MRI of the mid-thigh showing enlargement of the left common peroneal nerve (arrow). (b) Perineurial cells in a concentric arrangement around a central myelinated axon and a Schwann cell (electron microscopy (EM) ultrastructure 9750). (c) Perineurial cell processes showing pinocytotic vesicles (arrowhead) and surrounding incomplete basal lamina (long arrow) (EM ultrastructure, 45 150).
A histological examination of a biopsy of the left peroneal nerve showed that the nerve fascicles were expanded by widespread ‘pseudo-onion bulbs’, which are concentric proliferations of spindle cells expressing EMA. The ultrastructure showed concentric arrangements of perineurial cells with long, thin cytoplasmic processes, pinocytotic vesicles and a fragmented basal lamina (Fig. 2b, c). These findings were consistent with a perineurioma. Five months later, the patient was readmitted for excision of the tumour and a sural nerve graft. A 1 cm ipsilateral sural nerve graft was performed using 4 cables. Histological examination of the longitudinally oriented paraffin-embedded sections through the inked resection margins showed that the tumour extended to the proximal surgical resection margin.
2. Discussion Intraneural perineurioma is a neoplasm of perineurial cells, classified as World Health Organization grade I.1,2 This rare lesion accounts for about 1% of nerve sheath tumours.2 Intraneural perineurioma occurs in patients of a wide age group and usually affects the upper extremities. Patients usually present with slowly progressive motor weakness and less prominent sensory symptoms in a single nerve distribution. Fusiform expansion of the affected nerve may be detected clinically or by MRI. Molecular cytogenetic studies have revealed an association with clonal chromosome 22 abnormalities.3,4 The histological and ultrastructural features of both patients’ tumours are typical of this lesion.5 The main histopathological differential diagnosis of intraneural perineurioma is hypertrophic neuropathy (mainly the demyelinating forms of hereditary motor and sensory neuropathies) where ‘‘onion bulbs” are the hallmark feature.1,6 Onion bulbs are morphologically similar to pseudoonion bulbs, but comprise concentric endoneurial periaxonal proliferations of Schwann cells. Unlike the perineurial cell proliferation in intraneural perineurioma, which is a neoplastic process, the Schwann cell proliferation that forms onion bulbs is reactive.
Perineurioma and hypertrophic neuropathy can be distinguished using immunohistochemistry (Schwann cells are S100 immunopositive whereas perineurial cells are EMA immunopositive) and/ or electron microscopy (Schwann cells have continuous basal lamina and lack pinocytotic vesicles). The two reported patients highlight salient points about this tumour. First, although intraneural perineuriomas do not metastasize or recur following excision, the prognosis of untreated intraneural perineurioma is poor, with most partial lesions becoming complete over time.7 Therefore, the pathologic distinction between a hereditary neuropathy and intraneural perineurioma is crucial. Patient 1 is the longest reported follow-up (14 years) of this condition, and demonstrates that, over time, intraneural perineuriomas are capable of extending into adjacent nerves. Second, as intraneural perineurioma is rare, there are no standard guidelines for treatment. Complete excision and nerve grafting is one treatment option. However, the timing of surgical intervention is controversial, and outcomes have been variable. Maximal functional recovery after nerve grafting is reportedly most favourable in young patients.8 Early identification of the lesion and the opportunity to proceed with nerve grafting prior to irreversible deterioration of distal intramuscular nerve sheaths and longstanding denervation atrophy is also an important factor.8 However, because excision with nerve grafting does not completely restore function, this must be carefully weighed against the degree of nerve compromise. A surgical dilemma exists: early excision and grafting of an incomplete lesion would result in the loss of any residual function with an uncertain prospect of useful recovery; however, by the time the slowly progressive lesion causes complete loss of nerve function, the chronic degeneration of distal nerve sheaths and muscle fibres would result in poor functional recovery.7 Perineuromas can extend into adjacent nerves over time, therefore it is important that pathologists assess the surgical margins of these tumours. Surgical resection margins should be deemed clear prior to undertaking a nerve graft. The impact of the positive surgical resection margin on the outcome of patient 2 remains to be seen.
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Case Reports / Journal of Clinical Neuroscience 16 (2009) 1636–1639
References 1. Shy ME, Lupski JR, Chance PF, et al. Hereditary motor and sensory neuropathies: an overview of clinical, genetic, electrophysiologic and pathologic features. In: Dyck PJ, Thomas PK, editors. Peripheral Neuropathy. 4th ed. Philadelphia: Elsevier Saunders Co.; 2005. p. 1623–53. 2. Scheithauer BW, Woodruff JM, Antonescu CR. Perineurioma. In: Louis H, Ohgaki H, Wiestler OD, Cavenee WK, editors. WHO Classification of Tumours of the Central Nervous System. Lyon: IARC Press; 2007. p. 158–9. 3. Emory TS, Scheithauer BW, Hirose T, et al. Intraneural perineurioma. A clonal neoplasm associated with abnormalities of chromosome 22. Am J Clin Pathol 1995;103:696–704.
4. Huguet P, de la Torre J, Pallares J, et al. Intraosseous intraneural perineurioma: a report of a case with morphological, immunohistochemical and FISH study. Med Oral 2004;9:64–8. 5. Lazarus SS, Trombetta LD. Ultrastructural identification of a benign perineurial cell tumor. Cancer 1978;41:1823–9. 6. Bilbao JM, Schmidt R, Hawkins C. Diseases of peripheral nerves. In: Love S, Louis DN, Ellison DW, editors. Greenfield’s Neuropathology. Vol. 2. 8th ed. London: Hodder Arnold; 2008. p. 1632–41. 7. Isaac S, Athanasou NA, Pike M, et al. Radial nerve palsy owing to localised hypertrophic neuropathy (intraneural perineurioma) in early childhood. J Child Neurol 2004;19:71–5. 8. Cortes W, Cheng J, Matoub HS. Intraneural perineurioma of the radial nerve in a child. J Hand Surg [Am] 2005; 30:820-5.
doi:10.1016/j.jocn.2009.02.013
Breast carcinoma metastasis to intracranial meningioma Jui-Wei Lin a, Feng-Wen Su b, Hung-Cheng Wang b, Tao-Chen Lee b, Jih-Tsun Ho b, Chiu-Hsien Lin c, Yu-Jun Lin b,* a
Department of Pathology, Chang Gung Memorial Hospital-Kaohsiung Medical Center, Chang Gung University College of Medicine, Kaohsiung, Taiwan Department of Neurosurgery, Chang Gung Memorial Hospital-Kaohsiung Medical Center, Chang Gung University College of Medicine, No. 123, Ta Pei Road, Niao Sung Hsiang, Kaohsiung County, 83301, Taiwan c Department of Family Medicine, Chang Gung Memorial Hospital-Kaohsiung Medical Center, Chang Gung University College of Medicine, Kaohsiung, Taiwan b
a r t i c l e
i n f o
Article history: Received 5 October 2008 Accepted 3 February 2009
Keywords: Intracranial metastasis Tumor-to-tumor phenomenon Meningioma
a b s t r a c t Meningiomas and breast cancers are common tumors among women in the fifth to seventh decade. However, metastasis from breast cancer to an intracranial meningioma is rare. A 63-year-old woman presented with headache, nausea and vomiting, and progressive right hemiparesis for one month. She had undergone a right modified radical mastectomy in another hospital 10 years prior. At that time, the pathological diagnosis was infiltrating ductal carcinoma. She required adjuvant radiotherapy and chemotherapy for a local recurrence 7 years later. On admission to our hospital, cranial CT scans showed a brightly enhancing, irregularly shaped lesion over the left high parietal lobe with surrounding parenchymal edema. Histopathological examination of the lesion revealed two distinct tumor types, meningioma and metastatic carcinoma of breast tissue origin. Although meningiomas have well-known radiological features, other tumors, including metastases from breast cancers may simulate them. In the clinical setting of previously diagnosed breast cancer, prompt craniotomy for removal of meningioma-like intracranial lesions is recommended to avoid missing the diagnosis of breast cancer metastasis which carries a poorer prognosis than meningioma and requires a different treatment strategy. Ó 2009 Elsevier Ltd. All rights reserved.
1. Introduction
2. Case report
The simultaneous occurrence of meningioma and breast cancer with brain metastasis is an unusual, but known event.1,2 Breast cancers have also rarely been reported to metastasize to meningiomas (tumor-to-tumor phenomenon).3–7 There are several hypotheses for this phenomenon;8 however, the exact mechanism remains obscure. The occurrence of metastasis into a primary brain tumor might be simply chance, but it is also possible that a combination of factors such as the vascularity of the ‘‘recipient” tumor, its cell surface, and its metabolic and immunologic properties are responsible for the metastatic affinity of some tumors.3,9–12 We report a rare case of breast cancer metastasizing to an intracranial meningioma.
A 63-year-old woman presented with headache, nausea and vomiting and progressive right hemiparesis for one month. Ten years prior, she had undergone a right modified radical mastectomy for a breast carcinoma at another institution. The pathological diagnosis was infiltrative ductal carcinoma. The tumor was progesterone receptor (PR)-positive using immunohistochemical staining, and estrogen receptor (ER)-negative and HER-2/neu-negative. She required irradiation and chemotherapy 7 years later due to local recurrence and axillary lymph node metastasis. On admission to our hospital, a right mastectomy incision scar was observed. Neurological examination revealed disorientation to time and place and right hemiparesis. Neuro-ophthalmological examination revealed bilateral papilledema. However, other neurological and physical examinations were normal. A cranial CT scan without contrast enhancement revealed a left high parietal
* Corresponding author. Tel.: +886 7 7317123x8011; fax: +886 7 7317562. E-mail address: [email protected] (Y.-J. Lin)
Case Reports / Journal of Clinical Neuroscience 16 (2009) 1633–1636
sure used for stent deployment, as well as the diameter of both the stent and balloon, which may result in underexpansion and incomplete apposition, as illustrated in our patient. For this reason, we always select undersized stents to use in those patients with intracranial stenosis, so as to reduce the risk of peri-interventional stroke. Acknowledgement This study is partly supported by Key Project from the Shanghai Committee of Science and Technology (074119506). References 1. Wong KS, Huang YN, Yang HB, et al. A door-to-door survey of intracranial atherosclerosis in Liangbei County, China. Neurology 2007;69:2031–4. 2. Jiang WJ, Xu XT, Jin M, et al. Apollo stent for symptomatic atherosclerotic intracranial stenosis: study results. Am J Neuroradiol 2007;28:830–4. 3. The SSYLVIA study investigators. Stenting of symptomatic atherosclerotic lesions in the vertebral or intracranial arteries (SSYLVIA): study results. Stroke 2004;35:1388–92.
4. Qureshi AI, Kirmani JF, Hussein HM, et al. Early- and intermediate-term outcomes with drug-eluting stents in high-risk patients with symptomatic intracranial stenosis. Neurosurgery 2006;59:1044–51. 5. Abou-Chebl A, Bashir Q, Yadav JS, et al. Drug-eluting stents for the treatment of intracranial atherosclerosis: initial experience and midterm angiographic follow-up. Stroke 2005;36:E165–8. 6. Jiang WJ, Xu XT, Du B, et al. Comparison of elective stenting of severe vs. moderate intracranial atherosclerotic stenosis. Neurology 2007;68: 420–6. 7. Holmes Jr DR, Leon MB, Moses JW, et al. Analysis of 1-year clinical outcomes in the SIRIUS trial: a randomized trial of a sirolimus-eluting stent versus a standard stent in patients at high risk for coronary restenosis. Circulation 2004;109:634–40. 8. Lee TH, Choi CH, Park KP, et al. Techniques for intracranial stent navigation in patients with tortuous vessels. Am J Neuroradiol 2005;26:1375–80. 9. Iakavou I, Schimdt T, Bonizzoni E, et al. Incidence, predictors and outcome of thrombosis after successful implantation of drug-eluting stents. JAMA 2005;293:2126–30. 10. Eisenstein EL, Anstrom KJ, Kong DF, et al. Clopidogrel use and long-term clinical outcomes after drug-eluting stent implantation. JAMA 2007;297:159–68. 11. Serruys PW, Daemen J. Late stent thrombosis: a nuisance in both bare metal and drug-eluting stents. Circulation 2007;115:1433–9.
doi:10.1016/j.jocn.2009.01.025
Intraneural perineurioma H.-Y. Lee a, R.G. Manasseh b, R.H. Edis b, R. Page c, J. Keith-Rokosh a,*, P. Walsh d, S. Song e, A. Laycock f, L. Griffiths a, V.A. Fabian a a
Department of Anatomical Pathology, Section of Neuropathology, Royal Perth Hospital, 2nd Floor North Block, Wellington Street Campus, Perth, Western Australia 6847, Australia Department of Neurology, Royal Perth Hospital, Perth, Western Australia, Australia c Department of Plastic Surgery, Princess Margaret Hospital for Children, Subiaco, Western Australia, Australia d Department of Neurology, Princess Margaret Hospital for Children, Subiaco, Western Australia, Australia e Department of Radiology, Royal Perth Hospital, Perth, Western Australia, Australia f Department of Anatomical Pathology, Princess Margaret Hospital for Children, Subiaco, Western Australia, Australia b
a r t i c l e
i n f o
Article history: Received 22 December 2008 Accepted 8 February 2009
Keywords: Intraneural perineurioma Nerve Pseudo-onion bulbs Neuropathology Management Nerve grafting
a b s t r a c t Intraneural perineurioma is a rare tumour that affects peripheral nerves and, based on its histological features, may be confused with hereditary motor and sensory neuropathies. Detailed neuropathology, including immunoperoxidase stains and electron microscopy, is vital to distinguish these conditions. We report two patients with intraneural perineurioma that demonstrate salient features of this tumour. The first patient is the longest reported follow-up of an intraneural perineurioma; extension of the lesion was observed over 14 years. The second patient is an 11-year-old female whose treatment highlights some of the controversy surrounding the management of these lesions, and the importance of thorough macroscopic and microscopic assessment by pathologists, including the status of surgical resection margins.
1. Case reports 1.1. Patient 1 A 16-year-old male presented with a 4-year history of a progressive limp. There was no history of trauma and no family history of pes cavus or neuromuscular disease. Clinical examination demonstrated shortening of the right leg and wasting and weakness of the right hamstring muscle and all of the muscles below * Corresponding author. Tel.: +618 9224 2463; fax: +61 9224 2556. E-mail address: [email protected] (J. Keith-Rokosh)
Ó 2009 Elsevier Ltd. All rights reserved.
the right knee. The right knee-jerk was normal but the right ankle-jerk was absent. There was marked hypoesthesia and hypoalgesia involving the entire right foot and the lateral aspect of the right calf. A fusiform area of thickening was palpated in the region of the right sciatic nerve from the mid-thigh to the popliteal fossa. Nerve conduction studies did not demonstrate right tibial and common peroneal motor responses, and the right sural response was absent. Needle electromyography examination demonstrated chronic active denervation in the right hamstring muscle and all of the muscles below the right knee. These abnormalities were consistent with a focal lesion of the right sciatic nerve between the mid-thigh and the knee.
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Case Reports / Journal of Clinical Neuroscience 16 (2009) 1633–1636
Fig. 1. (a) Axial T1-weighted post-gadolinium MRI of the lower thighs showing an enlarged right tibial nerve (arrow). (b) Axial T1-weighted MRI of the pelvis showing an enlarged right sciatic nerve (arrow). (c) Enlarged nerve fascicles with typical pseudo-onion bulbs formed by concentric proliferations of spindle cells (hematoxylin and eosin, 160). (d) Epithelial membrane antigen (EMA) immunoreactivity in concentrically arranged spindle cells (EMA, 160). (e) Perineurial cells in a concentric arrangement (electron microscopy (EM) ultrastructure, 3000). (f) Perineurial cells with pinocytotic vesicles (arrow). A single unmyelinated axon and an accompanying Schwann cell are shown centrally (EM ultrastructure 27 500). (g) Perineurial cell with incomplete basal lamina (arrow) (EM ultrastructure 57 500).
MRI of the thigh demonstrated thickening of the right distal sciatic and proximal tibial nerves (Fig. 1a). MRI of the lumbar spine was normal. A biopsy of the right posterior tibial nerve was initially reported as a chronic hypertrophic neuropathy. Over the next 14 years there was progressive wasting and weakness of the right hamstring muscle and all of the muscles below the right knee, as well as worsening sensory loss and pain involving the distal right leg. There was eventual wasting of the right gluteal muscles. To evaluate the proximal progression of motor symptoms, MRI of the pelvic area was performed (Fig. 1b). Fusiform thickening of the sciatic nerve extended through the sciatic notch into the upper buttock, affecting the superior and inferior gluteal nerves, and extended distally into the proximal tibial and peroneal nerves. Review of the nerve biopsy initially reported as a chronic hypertrophic neuropathy showed expansion of the nerve fascicles by concentric layers of spindle cells around nerve fibres (Fig. 1c), which were positive on immunoperoxidase staining with Epithelial Membrane Antigen (EMA), (1:200 dilution of clone E29, Dako, Glostrup, Denmark) (Fig. 1d). Ultrastructural examination confirmed concentric arrangements of perineurial cells, which were identified by their long, thin cytoplasmic processes, numerous
pinocytotic vesicles and fragmented basal lamina around a central axon and accompanying Schwann cell (Fig. 1e–g). These findings were consistent with a perineurioma.
1.2. Patient 2 An 11-year-old female presented with a left foot drop. There was no associated pain or sensory abnormality, and family history was non-contributory. On examination she had atrophy of the left peroneal muscle with a left foot drop. The left leg was 0.5 cm shorter, and the left foot 0.5 cm smaller, than the right. There was marked weakness of the left foot and toe extension and some weakness of ankle eversion. Sensation was intact. Nerve conduction studies demonstrated conduction was absent in the left peroneal nerve. Her MRI showed fusiform irregular expansion of the left common peroneal nerve in the mid-thigh, with segments of normal calibre nerve intervening between areas of tubular expansion (Fig. 2a). The T2-weighted MRI showed neither hyperintensity, nor contrast enhancement, of the nerve. The MRI findings were considered compatible with a hypertrophic neuropathy involving the left common peroneal nerve.
Case Reports / Journal of Clinical Neuroscience 16 (2009) 1633–1636
1635
Fig. 2. (a) Axial T1-weighted post-gadolinium MRI of the mid-thigh showing enlargement of the left common peroneal nerve (arrow). (b) Perineurial cells in a concentric arrangement around a central myelinated axon and a Schwann cell (electron microscopy (EM) ultrastructure 9750). (c) Perineurial cell processes showing pinocytotic vesicles (arrowhead) and surrounding incomplete basal lamina (long arrow) (EM ultrastructure, 45 150).
A histological examination of a biopsy of the left peroneal nerve showed that the nerve fascicles were expanded by widespread ‘pseudo-onion bulbs’, which are concentric proliferations of spindle cells expressing EMA. The ultrastructure showed concentric arrangements of perineurial cells with long, thin cytoplasmic processes, pinocytotic vesicles and a fragmented basal lamina (Fig. 2b, c). These findings were consistent with a perineurioma. Five months later, the patient was readmitted for excision of the tumour and a sural nerve graft. A 1 cm ipsilateral sural nerve graft was performed using 4 cables. Histological examination of the longitudinally oriented paraffin-embedded sections through the inked resection margins showed that the tumour extended to the proximal surgical resection margin.
2. Discussion Intraneural perineurioma is a neoplasm of perineurial cells, classified as World Health Organization grade I.1,2 This rare lesion accounts for about 1% of nerve sheath tumours.2 Intraneural perineurioma occurs in patients of a wide age group and usually affects the upper extremities. Patients usually present with slowly progressive motor weakness and less prominent sensory symptoms in a single nerve distribution. Fusiform expansion of the affected nerve may be detected clinically or by MRI. Molecular cytogenetic studies have revealed an association with clonal chromosome 22 abnormalities.3,4 The histological and ultrastructural features of both patients’ tumours are typical of this lesion.5 The main histopathological differential diagnosis of intraneural perineurioma is hypertrophic neuropathy (mainly the demyelinating forms of hereditary motor and sensory neuropathies) where ‘‘onion bulbs” are the hallmark feature.1,6 Onion bulbs are morphologically similar to pseudoonion bulbs, but comprise concentric endoneurial periaxonal proliferations of Schwann cells. Unlike the perineurial cell proliferation in intraneural perineurioma, which is a neoplastic process, the Schwann cell proliferation that forms onion bulbs is reactive.
Perineurioma and hypertrophic neuropathy can be distinguished using immunohistochemistry (Schwann cells are S100 immunopositive whereas perineurial cells are EMA immunopositive) and/ or electron microscopy (Schwann cells have continuous basal lamina and lack pinocytotic vesicles). The two reported patients highlight salient points about this tumour. First, although intraneural perineuriomas do not metastasize or recur following excision, the prognosis of untreated intraneural perineurioma is poor, with most partial lesions becoming complete over time.7 Therefore, the pathologic distinction between a hereditary neuropathy and intraneural perineurioma is crucial. Patient 1 is the longest reported follow-up (14 years) of this condition, and demonstrates that, over time, intraneural perineuriomas are capable of extending into adjacent nerves. Second, as intraneural perineurioma is rare, there are no standard guidelines for treatment. Complete excision and nerve grafting is one treatment option. However, the timing of surgical intervention is controversial, and outcomes have been variable. Maximal functional recovery after nerve grafting is reportedly most favourable in young patients.8 Early identification of the lesion and the opportunity to proceed with nerve grafting prior to irreversible deterioration of distal intramuscular nerve sheaths and longstanding denervation atrophy is also an important factor.8 However, because excision with nerve grafting does not completely restore function, this must be carefully weighed against the degree of nerve compromise. A surgical dilemma exists: early excision and grafting of an incomplete lesion would result in the loss of any residual function with an uncertain prospect of useful recovery; however, by the time the slowly progressive lesion causes complete loss of nerve function, the chronic degeneration of distal nerve sheaths and muscle fibres would result in poor functional recovery.7 Perineuromas can extend into adjacent nerves over time, therefore it is important that pathologists assess the surgical margins of these tumours. Surgical resection margins should be deemed clear prior to undertaking a nerve graft. The impact of the positive surgical resection margin on the outcome of patient 2 remains to be seen.
1636
Case Reports / Journal of Clinical Neuroscience 16 (2009) 1636–1639
References 1. Shy ME, Lupski JR, Chance PF, et al. Hereditary motor and sensory neuropathies: an overview of clinical, genetic, electrophysiologic and pathologic features. In: Dyck PJ, Thomas PK, editors. Peripheral Neuropathy. 4th ed. Philadelphia: Elsevier Saunders Co.; 2005. p. 1623–53. 2. Scheithauer BW, Woodruff JM, Antonescu CR. Perineurioma. In: Louis H, Ohgaki H, Wiestler OD, Cavenee WK, editors. WHO Classification of Tumours of the Central Nervous System. Lyon: IARC Press; 2007. p. 158–9. 3. Emory TS, Scheithauer BW, Hirose T, et al. Intraneural perineurioma. A clonal neoplasm associated with abnormalities of chromosome 22. Am J Clin Pathol 1995;103:696–704.
4. Huguet P, de la Torre J, Pallares J, et al. Intraosseous intraneural perineurioma: a report of a case with morphological, immunohistochemical and FISH study. Med Oral 2004;9:64–8. 5. Lazarus SS, Trombetta LD. Ultrastructural identification of a benign perineurial cell tumor. Cancer 1978;41:1823–9. 6. Bilbao JM, Schmidt R, Hawkins C. Diseases of peripheral nerves. In: Love S, Louis DN, Ellison DW, editors. Greenfield’s Neuropathology. Vol. 2. 8th ed. London: Hodder Arnold; 2008. p. 1632–41. 7. Isaac S, Athanasou NA, Pike M, et al. Radial nerve palsy owing to localised hypertrophic neuropathy (intraneural perineurioma) in early childhood. J Child Neurol 2004;19:71–5. 8. Cortes W, Cheng J, Matoub HS. Intraneural perineurioma of the radial nerve in a child. J Hand Surg [Am] 2005; 30:820-5.
doi:10.1016/j.jocn.2009.02.013
Breast carcinoma metastasis to intracranial meningioma Jui-Wei Lin a, Feng-Wen Su b, Hung-Cheng Wang b, Tao-Chen Lee b, Jih-Tsun Ho b, Chiu-Hsien Lin c, Yu-Jun Lin b,* a
Department of Pathology, Chang Gung Memorial Hospital-Kaohsiung Medical Center, Chang Gung University College of Medicine, Kaohsiung, Taiwan Department of Neurosurgery, Chang Gung Memorial Hospital-Kaohsiung Medical Center, Chang Gung University College of Medicine, No. 123, Ta Pei Road, Niao Sung Hsiang, Kaohsiung County, 83301, Taiwan c Department of Family Medicine, Chang Gung Memorial Hospital-Kaohsiung Medical Center, Chang Gung University College of Medicine, Kaohsiung, Taiwan b
a r t i c l e
i n f o
Article history: Received 5 October 2008 Accepted 3 February 2009
Keywords: Intracranial metastasis Tumor-to-tumor phenomenon Meningioma
a b s t r a c t Meningiomas and breast cancers are common tumors among women in the fifth to seventh decade. However, metastasis from breast cancer to an intracranial meningioma is rare. A 63-year-old woman presented with headache, nausea and vomiting, and progressive right hemiparesis for one month. She had undergone a right modified radical mastectomy in another hospital 10 years prior. At that time, the pathological diagnosis was infiltrating ductal carcinoma. She required adjuvant radiotherapy and chemotherapy for a local recurrence 7 years later. On admission to our hospital, cranial CT scans showed a brightly enhancing, irregularly shaped lesion over the left high parietal lobe with surrounding parenchymal edema. Histopathological examination of the lesion revealed two distinct tumor types, meningioma and metastatic carcinoma of breast tissue origin. Although meningiomas have well-known radiological features, other tumors, including metastases from breast cancers may simulate them. In the clinical setting of previously diagnosed breast cancer, prompt craniotomy for removal of meningioma-like intracranial lesions is recommended to avoid missing the diagnosis of breast cancer metastasis which carries a poorer prognosis than meningioma and requires a different treatment strategy. Ó 2009 Elsevier Ltd. All rights reserved.
1. Introduction
2. Case report
The simultaneous occurrence of meningioma and breast cancer with brain metastasis is an unusual, but known event.1,2 Breast cancers have also rarely been reported to metastasize to meningiomas (tumor-to-tumor phenomenon).3–7 There are several hypotheses for this phenomenon;8 however, the exact mechanism remains obscure. The occurrence of metastasis into a primary brain tumor might be simply chance, but it is also possible that a combination of factors such as the vascularity of the ‘‘recipient” tumor, its cell surface, and its metabolic and immunologic properties are responsible for the metastatic affinity of some tumors.3,9–12 We report a rare case of breast cancer metastasizing to an intracranial meningioma.
A 63-year-old woman presented with headache, nausea and vomiting and progressive right hemiparesis for one month. Ten years prior, she had undergone a right modified radical mastectomy for a breast carcinoma at another institution. The pathological diagnosis was infiltrative ductal carcinoma. The tumor was progesterone receptor (PR)-positive using immunohistochemical staining, and estrogen receptor (ER)-negative and HER-2/neu-negative. She required irradiation and chemotherapy 7 years later due to local recurrence and axillary lymph node metastasis. On admission to our hospital, a right mastectomy incision scar was observed. Neurological examination revealed disorientation to time and place and right hemiparesis. Neuro-ophthalmological examination revealed bilateral papilledema. However, other neurological and physical examinations were normal. A cranial CT scan without contrast enhancement revealed a left high parietal
* Corresponding author. Tel.: +886 7 7317123x8011; fax: +886 7 7317562. E-mail address: [email protected] (Y.-J. Lin)