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Epineural glomus tumor of the posterior interosseous nerve: Case report
Journal of Clinical Neuroscience xxx (xxxx) xxx
Contents lists available at ScienceDirect
Journal of Clinical Neuroscience journal homepage: www.elsevier.com/locate/jocn
Case report
Epineural glomus tumor of the posterior interosseous nerve: Case report Philippe Drabent a, Franck Bielle a, Isabelle Bernat b, Matthieu Peyre c,d,⇑ a
Sorbonne Universités – Department of Neuropathology, Groupe Hospitalier Pitié-Salpêtrière, APHP, Paris, France Sorbonne Universités – Department of Electrophysiology, Groupe Hospitalier Pitié-Salpêtrière, APHP, Paris, France c Sorbonne Universités – Department of Neurosurgery, Groupe Hospitalier Pitié-Salpêtrière, APHP, Paris, France d Neurofibromatosis Type 2 and Schwannomatosis Referral Center, Groupe Hospitalier Pitié-Salpêtrière, APHP, Paris, France b
a r t i c l e
i n f o
Article history: Received 1 November 2019 Accepted 30 December 2019 Available online xxxx Keywords: Peripheral nerve sheath tumor Glomus tumor Radial nerve Uncertain malignancy Peripheral nerve schwannoma
a b s t r a c t Among peripheral nerve sheath tumors, epineural glomus tumors represent a rare differential diagnosis for peripheral nerve schwannoma, displaying the same appearance on MRI. Here we present the case of a 68-year-old man complaining of pain in the left forearm, with paresthesias in the first three fingers. MRI demonstrated a round mass along the course of the posterior interosseous nerve with homogeneous contrast enhancement. Complete surgical removal successfully relieved the symptoms. Pathological analysis led to the diagnosis of glomus tumor of uncertain malignant potential. This is to our knowledge the first reported case of epineural glomus tumor of the radial nerve. Ó 2020 Elsevier Ltd. All rights reserved.
1. Introduction Glomus tumors are exceedingly rare tumors arising from the normal glomus body, which consists of an arterio-venous shunt surrounded by a capsule of connective tissue. As normal glomus bodies are involved in body temperature regulation and are found mainly in the skin, glomus tumors have been frequently reported in the subungual regions of digits or the deep dermis of the hand and foot. Although the normal nervous system does not contain glomus bodies, glomus tumors involving peripheral nerves have been described, with 16 cases already reported in the literature. Most reported cases are intraneural glomus tumors [1–5] including two cases of glomangiomatosis [6,7]. Tumors of epineural cells developed outside the nerve are far less common [8]. Herein, we describe the first case of epineural glomus tumor involving the radial nerve.
2. Case report A 68-year-old man had a 3-year history of pain in the left forearm progressively extending to the whole limb. The patient also complained of paresthesias in the first three fingers. Physical ⇑ Corresponding author at: Department of Neurosurgery, Batiment Babinski, Groupe Hospitalier Pitié-Salpêtrière, 47-83 boulevard de l’Hôpital, 75013 Paris, France. E-mail address: [email protected] (M. Peyre).
examination was normal. MRI demonstrated a 1.2 cm lesion on the posterior interosseous nerve with homogeneous contrast enhancement (Fig. 1-A and -B). Both clinical and imaging features were consistent with a schwannoma of the posterior interosseous nerve. At surgery (Fig. 1-C), a round and reddish nodule was noted on the nerve. It was slightly adherent to the nerve fibers with a distinct cleavage plane, allowing for gross-total en bloc resection. No epineurial or surface hypervascularity of the nerve was apparent. On hematoxylin-eosin stain (Fig. 2-A and -B), the tumor was well limited by a thin fibrous capsule, and assumed a vaguely nodular and angiocentric architecture, interspersed with congestive capillaries of various size, admixed with a few smooth muscle bundles. A reticulin stain highlighted a delicate weaving surrounding every tumor cell individually (Fig. 2-C). Immunohistochemistry showed a strong and diffuse positivity to Smooth Muscle Actin (SMA), accompanied by a weaker staining to CD34 in 30% of the tumor cells. MIB-1 proliferation index was close to 1% (Fig. 2-D to 2-F). Consistent with the subaponeurotic location of the tumor, we rendered a diagnosis of glomus tumor of intermediate malignant potential. Postoperatively, the patient had complete resolution of pain and no new neurological deficit was observed. 3. Discussion Glomus tumors account for 1.6% of all soft tissue neoplasms. Histologically, they encompass three histological entities based on the presence or absence of three components: glomus cells,
https://doi.org/10.1016/j.jocn.2019.12.062 0967-5868/Ó 2020 Elsevier Ltd. All rights reserved.
Please cite this article as: P. Drabent, F. Bielle, I. Bernat et al., Epineural glomus tumor of the posterior interosseous nerve: Case report, Journal of Clinical Neuroscience, https://doi.org/10.1016/j.jocn.2019.12.062
2
P. Drabent et al. / Journal of Clinical Neuroscience xxx (xxxx) xxx
Fig. 1. Radiological and per-operative features of the tumor. Axial (A.) and sagittal (B.) post-contrast T1-weighted images of the forearm showing a small and deeply-located mass (white arrow head) with homogeneous contrast enhancement that was initially diagnosed as radial nerve schwannoma. C. Per-operative view of the tumor showing a reddish and well-circumscribed tumor laying on the posterior interosseous nerve (asterisk).
Fig. 2. Histological features of the tumor. A. Hematoxylin-eosin (H&E), original magnification X20. B. H&E, original magnification X200. C. Reticulin staining, original magnification X200. D. SMA immunostaining, original magnification X100. E. CD34 immunostaining (note the internal positive control on endothelial cells), original magnification X100. F. MIB-1 (Ki67) immunostaining, original magnification X100.
blood vessels and smooth muscle cells. Tumors consisting mainly of glomus cells are called « solid glomus tumor ». The term « glomangioma » refers to a glomus tumor with a prominent vascular component. A spindled smooth muscle component is much rarer and defines a tumor called « glomangiomyoma », or « glomangiopericytoma » [10]. Although very rare, both malignant glomus tumors and glomus tumors of uncertain malignant potential (GTUMP) have been described, accounting for 2.9% and 3.6% of all glomus tumors respectively [11]. In particular, GT-UMPs encompass (a) superficial glomus tumors with high mitotic rate (>5 per 50 HPF) lacking cytologic atypias or exceeding 2 cm in size, and (b) microscopically conventional glomus tumors occurring in deep locations [12]. Few data exist about the prognostic value of this location: surgical resection with follow-up appears to be the best management. Glomus tumors rarely involve nerves, with only 17 cases reported to date to our knowledge, including our case. Tumors are equally distributed between superior and inferior limbs and have been found predominantly in the sciatic nerve (3 cases) and the digital nerves (4 cases). At surgery, nerve glomus tumors nerve may present as intraneural well-circumscribed masses [4], or diffuse intraneural tumors usually requiring sacrifice of the concerned fascicles or even the whole nerve [1,3,5–7,9]. Rarely,
purely epineural variants are encountered [2,8]. On pre-operative MRI, intraneural tumors generally present as an enlarged nerve with mild and/or intense contrast enhancement that may be focal [3] or fusiform and affect a large portion of the nerve [9], while epineural forms present as an oval and encapsulated mass with high and homogeneous contrast enhancement [8], similar to peripheral nerve schwannomas. Despite the diversity of anatomical variants, local excision improved pain in all reported cases. Appendix A. Supplementary data Supplementary data to this article can be found online at https://doi.org/10.1016/j.jocn.2019.12.062. References [1] Kline SC, Moore JR, deMente SH. Glomus tumor originating within a digital nerve. J Hand Surg 1990;15:98–101. [2] Mitchell A, Spinner RJ, Ribeiro A, Mafra M, Mouzinho MM, Scheithauer BW. Glomus tumor of digital nerve: case report. J Hand Surg 2012;37:1180–3. [3] Scheithauer BW, Rodriguez FJ, Spinner RJ, Dyck PJ, Salem A, Edelman FL, et al. Glomus tumor and glomangioma of the nerve. Report of two cases. J Neurosurg 2008;108:348–56. [4] Smith KA, Mackinnon SE, Macauley RJ, Mailis A. Glomus tumor originating in the radial nerve: a case report. J Hand Surg 1992;17:665–7.
Please cite this article as: P. Drabent, F. Bielle, I. Bernat et al., Epineural glomus tumor of the posterior interosseous nerve: Case report, Journal of Clinical Neuroscience, https://doi.org/10.1016/j.jocn.2019.12.062
P. Drabent et al. / Journal of Clinical Neuroscience xxx (xxxx) xxx [5] Tropet Y, Menez D, Billerey C. Vichard P [Glomus tumor of the sciatic nerve]. Ann Chir Plast Esthet 1991;36:204–8. [6] Kumar R, Vu L, Madewell JE, Herzog CE, Bird JE. Glomangiomatosis of the sciatic nerve: a case report and review of the literature. Skeletal Radiol 2017;46:807–15. [7] Laughlin RS, Suanprasert N, Dyck PJB, Spinner RJ, Folpe AL. Glomangiomatosis of the sural nerve. J Clin Pathol 2014;67:190–2. [8] Park DS, Choe WJ, Chun YI, Moon C-T. Glomus tumor in the femoral nerve. J Korean Neurosurg Soc 2013;54:540–3. [9] Dahlin LB, Scherman P, Besjakov J, Lindberg E, Solomon DA, Horvai AE, et al. Intraneural glomus tumor of ‘‘uncertain malignant potential” and with BRAF
3
mutation in the median nerve - an unusual case. Clin Neuropathol 2017;36:164–70. [10] Gombos Z, Zhang PJ. Glomus tumor. Arch Pathol Lab Med 2008;132:1448–52. https://doi.org/10.1043/1543-2165(2008) 132[1448:GT]2.0.CO;2. [11] Mravic M, LaChaud G, Nguyen A, Scott MA, Dry SM, James AW. Clinical and histopathological diagnosis of glomus tumor: an institutional experience of 138 cases. Int J Surg Pathol 2015;23:181–8. [12] Folpe AL, Fanburg-Smith JC, Miettinen M, Weiss SW. Atypical and malignant glomus tumors: analysis of 52 cases, with a proposal for the reclassification of glomus tumors. Am J Surg Pathol 2001;25:1–12.
Please cite this article as: P. Drabent, F. Bielle, I. Bernat et al., Epineural glomus tumor of the posterior interosseous nerve: Case report, Journal of Clinical Neuroscience, https://doi.org/10.1016/j.jocn.2019.12.062
Contents lists available at ScienceDirect
Journal of Clinical Neuroscience journal homepage: www.elsevier.com/locate/jocn
Case report
Epineural glomus tumor of the posterior interosseous nerve: Case report Philippe Drabent a, Franck Bielle a, Isabelle Bernat b, Matthieu Peyre c,d,⇑ a
Sorbonne Universités – Department of Neuropathology, Groupe Hospitalier Pitié-Salpêtrière, APHP, Paris, France Sorbonne Universités – Department of Electrophysiology, Groupe Hospitalier Pitié-Salpêtrière, APHP, Paris, France c Sorbonne Universités – Department of Neurosurgery, Groupe Hospitalier Pitié-Salpêtrière, APHP, Paris, France d Neurofibromatosis Type 2 and Schwannomatosis Referral Center, Groupe Hospitalier Pitié-Salpêtrière, APHP, Paris, France b
a r t i c l e
i n f o
Article history: Received 1 November 2019 Accepted 30 December 2019 Available online xxxx Keywords: Peripheral nerve sheath tumor Glomus tumor Radial nerve Uncertain malignancy Peripheral nerve schwannoma
a b s t r a c t Among peripheral nerve sheath tumors, epineural glomus tumors represent a rare differential diagnosis for peripheral nerve schwannoma, displaying the same appearance on MRI. Here we present the case of a 68-year-old man complaining of pain in the left forearm, with paresthesias in the first three fingers. MRI demonstrated a round mass along the course of the posterior interosseous nerve with homogeneous contrast enhancement. Complete surgical removal successfully relieved the symptoms. Pathological analysis led to the diagnosis of glomus tumor of uncertain malignant potential. This is to our knowledge the first reported case of epineural glomus tumor of the radial nerve. Ó 2020 Elsevier Ltd. All rights reserved.
1. Introduction Glomus tumors are exceedingly rare tumors arising from the normal glomus body, which consists of an arterio-venous shunt surrounded by a capsule of connective tissue. As normal glomus bodies are involved in body temperature regulation and are found mainly in the skin, glomus tumors have been frequently reported in the subungual regions of digits or the deep dermis of the hand and foot. Although the normal nervous system does not contain glomus bodies, glomus tumors involving peripheral nerves have been described, with 16 cases already reported in the literature. Most reported cases are intraneural glomus tumors [1–5] including two cases of glomangiomatosis [6,7]. Tumors of epineural cells developed outside the nerve are far less common [8]. Herein, we describe the first case of epineural glomus tumor involving the radial nerve.
2. Case report A 68-year-old man had a 3-year history of pain in the left forearm progressively extending to the whole limb. The patient also complained of paresthesias in the first three fingers. Physical ⇑ Corresponding author at: Department of Neurosurgery, Batiment Babinski, Groupe Hospitalier Pitié-Salpêtrière, 47-83 boulevard de l’Hôpital, 75013 Paris, France. E-mail address: [email protected] (M. Peyre).
examination was normal. MRI demonstrated a 1.2 cm lesion on the posterior interosseous nerve with homogeneous contrast enhancement (Fig. 1-A and -B). Both clinical and imaging features were consistent with a schwannoma of the posterior interosseous nerve. At surgery (Fig. 1-C), a round and reddish nodule was noted on the nerve. It was slightly adherent to the nerve fibers with a distinct cleavage plane, allowing for gross-total en bloc resection. No epineurial or surface hypervascularity of the nerve was apparent. On hematoxylin-eosin stain (Fig. 2-A and -B), the tumor was well limited by a thin fibrous capsule, and assumed a vaguely nodular and angiocentric architecture, interspersed with congestive capillaries of various size, admixed with a few smooth muscle bundles. A reticulin stain highlighted a delicate weaving surrounding every tumor cell individually (Fig. 2-C). Immunohistochemistry showed a strong and diffuse positivity to Smooth Muscle Actin (SMA), accompanied by a weaker staining to CD34 in 30% of the tumor cells. MIB-1 proliferation index was close to 1% (Fig. 2-D to 2-F). Consistent with the subaponeurotic location of the tumor, we rendered a diagnosis of glomus tumor of intermediate malignant potential. Postoperatively, the patient had complete resolution of pain and no new neurological deficit was observed. 3. Discussion Glomus tumors account for 1.6% of all soft tissue neoplasms. Histologically, they encompass three histological entities based on the presence or absence of three components: glomus cells,
https://doi.org/10.1016/j.jocn.2019.12.062 0967-5868/Ó 2020 Elsevier Ltd. All rights reserved.
Please cite this article as: P. Drabent, F. Bielle, I. Bernat et al., Epineural glomus tumor of the posterior interosseous nerve: Case report, Journal of Clinical Neuroscience, https://doi.org/10.1016/j.jocn.2019.12.062
2
P. Drabent et al. / Journal of Clinical Neuroscience xxx (xxxx) xxx
Fig. 1. Radiological and per-operative features of the tumor. Axial (A.) and sagittal (B.) post-contrast T1-weighted images of the forearm showing a small and deeply-located mass (white arrow head) with homogeneous contrast enhancement that was initially diagnosed as radial nerve schwannoma. C. Per-operative view of the tumor showing a reddish and well-circumscribed tumor laying on the posterior interosseous nerve (asterisk).
Fig. 2. Histological features of the tumor. A. Hematoxylin-eosin (H&E), original magnification X20. B. H&E, original magnification X200. C. Reticulin staining, original magnification X200. D. SMA immunostaining, original magnification X100. E. CD34 immunostaining (note the internal positive control on endothelial cells), original magnification X100. F. MIB-1 (Ki67) immunostaining, original magnification X100.
blood vessels and smooth muscle cells. Tumors consisting mainly of glomus cells are called « solid glomus tumor ». The term « glomangioma » refers to a glomus tumor with a prominent vascular component. A spindled smooth muscle component is much rarer and defines a tumor called « glomangiomyoma », or « glomangiopericytoma » [10]. Although very rare, both malignant glomus tumors and glomus tumors of uncertain malignant potential (GTUMP) have been described, accounting for 2.9% and 3.6% of all glomus tumors respectively [11]. In particular, GT-UMPs encompass (a) superficial glomus tumors with high mitotic rate (>5 per 50 HPF) lacking cytologic atypias or exceeding 2 cm in size, and (b) microscopically conventional glomus tumors occurring in deep locations [12]. Few data exist about the prognostic value of this location: surgical resection with follow-up appears to be the best management. Glomus tumors rarely involve nerves, with only 17 cases reported to date to our knowledge, including our case. Tumors are equally distributed between superior and inferior limbs and have been found predominantly in the sciatic nerve (3 cases) and the digital nerves (4 cases). At surgery, nerve glomus tumors nerve may present as intraneural well-circumscribed masses [4], or diffuse intraneural tumors usually requiring sacrifice of the concerned fascicles or even the whole nerve [1,3,5–7,9]. Rarely,
purely epineural variants are encountered [2,8]. On pre-operative MRI, intraneural tumors generally present as an enlarged nerve with mild and/or intense contrast enhancement that may be focal [3] or fusiform and affect a large portion of the nerve [9], while epineural forms present as an oval and encapsulated mass with high and homogeneous contrast enhancement [8], similar to peripheral nerve schwannomas. Despite the diversity of anatomical variants, local excision improved pain in all reported cases. Appendix A. Supplementary data Supplementary data to this article can be found online at https://doi.org/10.1016/j.jocn.2019.12.062. References [1] Kline SC, Moore JR, deMente SH. Glomus tumor originating within a digital nerve. J Hand Surg 1990;15:98–101. [2] Mitchell A, Spinner RJ, Ribeiro A, Mafra M, Mouzinho MM, Scheithauer BW. Glomus tumor of digital nerve: case report. J Hand Surg 2012;37:1180–3. [3] Scheithauer BW, Rodriguez FJ, Spinner RJ, Dyck PJ, Salem A, Edelman FL, et al. Glomus tumor and glomangioma of the nerve. Report of two cases. J Neurosurg 2008;108:348–56. [4] Smith KA, Mackinnon SE, Macauley RJ, Mailis A. Glomus tumor originating in the radial nerve: a case report. J Hand Surg 1992;17:665–7.
Please cite this article as: P. Drabent, F. Bielle, I. Bernat et al., Epineural glomus tumor of the posterior interosseous nerve: Case report, Journal of Clinical Neuroscience, https://doi.org/10.1016/j.jocn.2019.12.062
P. Drabent et al. / Journal of Clinical Neuroscience xxx (xxxx) xxx [5] Tropet Y, Menez D, Billerey C. Vichard P [Glomus tumor of the sciatic nerve]. Ann Chir Plast Esthet 1991;36:204–8. [6] Kumar R, Vu L, Madewell JE, Herzog CE, Bird JE. Glomangiomatosis of the sciatic nerve: a case report and review of the literature. Skeletal Radiol 2017;46:807–15. [7] Laughlin RS, Suanprasert N, Dyck PJB, Spinner RJ, Folpe AL. Glomangiomatosis of the sural nerve. J Clin Pathol 2014;67:190–2. [8] Park DS, Choe WJ, Chun YI, Moon C-T. Glomus tumor in the femoral nerve. J Korean Neurosurg Soc 2013;54:540–3. [9] Dahlin LB, Scherman P, Besjakov J, Lindberg E, Solomon DA, Horvai AE, et al. Intraneural glomus tumor of ‘‘uncertain malignant potential” and with BRAF
3
mutation in the median nerve - an unusual case. Clin Neuropathol 2017;36:164–70. [10] Gombos Z, Zhang PJ. Glomus tumor. Arch Pathol Lab Med 2008;132:1448–52. https://doi.org/10.1043/1543-2165(2008) 132[1448:GT]2.0.CO;2. [11] Mravic M, LaChaud G, Nguyen A, Scott MA, Dry SM, James AW. Clinical and histopathological diagnosis of glomus tumor: an institutional experience of 138 cases. Int J Surg Pathol 2015;23:181–8. [12] Folpe AL, Fanburg-Smith JC, Miettinen M, Weiss SW. Atypical and malignant glomus tumors: analysis of 52 cases, with a proposal for the reclassification of glomus tumors. Am J Surg Pathol 2001;25:1–12.
Please cite this article as: P. Drabent, F. Bielle, I. Bernat et al., Epineural glomus tumor of the posterior interosseous nerve: Case report, Journal of Clinical Neuroscience, https://doi.org/10.1016/j.jocn.2019.12.062