Paravertebral glomangioma mimicking a schwannoma

Neurochirurgie 59 (2013) 187–190

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Clinical case

Paravertebral glomangioma mimicking a schwannoma Glomangiome paravertébral simulant un schwannome rachidien F. Babeau a , S. Knafo c , V. Rigau b , N. Lonjon a,∗ a

Department of neurosurgery, hôpital Gui-de-Chauliac, 80, avenue Augustin-Fliche, 34091 Montpellier cedex 05, France Department of pathology, hôpital Gui-de-Chauliac, 34091 Montpellier cedex 05, France c Department of neurosurgery, hôpital Pitié-Salpétrière, université Pierre-et-Marie-Curie, 75013 Paris, France b

a r t i c l e

i n f o

Article history: Received 24 January 2013 Accepted 9 June 2013 Keywords: Paravertebral glomangioma Glomus tumor Spine

a b s t r a c t Glomus tumors are rare benign neoplasms usually arising from soft tissues. Surgical removal seems to be the best treatment. Here, we report the case of a 52-year-old female patient referred for chronic back pain, revealing a thoracic paravertebral tumor with no osseous extension. After surgical removal, this tumor turned out to be a glomangioma. Differential diagnosis with other more frequent tumors, such as schwannoma, is particularly difficult. A review of the relevant literature will be presented. A better knowledge of the natural history of those tumors, as well as the therapeutic options available, are necessary for spinal surgeons who may encounter such presentations. © 2013 Elsevier Masson SAS. All rights reserved.

r é s u m é Mots clés : Glomangiome paravertébral Tumeur glomique Rachis

Les tumeurs glomiques, lésions bénignes rares, se développent généralement au niveau des tissus mous. L’exérèse chirurgicale semble être le traitement de choix. Nous rapportons le cas d’une patiente de 52 ans, se plaignant de douleurs dorsales chroniques, qui présentait une tumeur paravertébrale thoracique sans atteinte osseuse se révélant être un glomangiome. Seulement quelques cas de la littérature rapportent ce type de localisation. La confusion diagnostic avec d’autres tumeurs paravertébrales plus fréquentes notamment avec un schwannome est classique mais ne change pas réellement la prise en charge thérapeutique. Nous avons effectué une revue de la littérature des tumeurs glomiques de topographie rachidienne. Une meilleure connaissance de l’histoire naturelle de ces tumeurs ainsi que des possibilités thérapeutiques est souhaitable pour améliorer la prise en charge de ces patients. © 2013 Elsevier Masson SAS. Tous droits réservés.

1. Introduction

2. Case report

Glomus tumors are rare neoplasms affecting mainly adults between 30 and 50 years old [1]. They account for 1 to 2% of soft tissues tumors [2], with a prevalence of 1.6% [3]. Those lesions arise from the neuromyo-arterial glomus of dermo-epidermic tissue [4] and correspond to a hyperplasia of glomus bodies [5]. Glomus tumors are benign lesions and paravertebral localization is very rare with only a few cases reported in the literature [2]. Such topographic forms are usually revealed by intense back pain, either at the medio-thoracic or lumbo-sacral level [1]. They are usually welldelimited tumors, although osteolysis and epidural extension can sometimes be found [1,4–6].

We report the case of a 52-year-old female patient, nurse, with no past medical history, presenting for the last 3 months a history of permanent dorsal back pain irradiating of the right side, increased at night, resistant to antalgic and anti-inflammatory medications (VAS: 5/10). Palpation of T6 spinous process triggered the pain on physical examination. Spine magnetic resonance imaging (MRI) showed a T6-T7 right foraminal lesion, iso-intense on T1-weighted sequences and with a clear hyper-signal in T2-weighted sequences (Fig. 1). There was a marked enhancement after gadolinium injection (Fig. 2), showing a typical dumbbell shape with intra and extracanalar extensions (Figs. 1 and 2). CT-Scan did not reveal osseous extension (Fig. 3A), while the spinal cord and lung were respectively deviated by the intracanalar and extracanalar parts of the tumor. The first hypothesis being a schwannoma, surgical removal was undertaken considering the potential neurological complications

∗ Corresponding author. E-mail address: [email protected] (N. Lonjon). 0028-3770/$ – see front matter © 2013 Elsevier Masson SAS. All rights reserved. http://dx.doi.org/10.1016/j.neuchi.2013.06.002

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Fig. 1. Coronal T2-weighted magnetic resonance imaging along with two different axial T1-weighted images showing tumoral extension after injection of gadolinium. IRM en coupe coronale et en séquence pondérée en T2, centrée sur la lésion avec deux différentes coupes axiales pondérées en T1 après injection de gadolinium montrant l’extension paravertébrale de la tumeur.

Fig. 2. Sagittal T1-weighted MRI with gadolinium centered on the right foraminal T6–T7 region. Axial image showing the typical dumbbell shape lesion mimicking a schwannoma. IRM pondérée en séquence T1 après injection de gadolinium centrée sur la région foraminale droite T6–T7. Coupe axiale montrant l’aspect en sablier de la tumeur simulant un schwannome.

Fig. 3. A. Preoperative CT-scan revealed a right paravertebral mass without osseous extension. B. Postoperative CT-scan showing the unilateral approach to the foraminal right region. A. Scanner préopératoire révélant une masse paravertébrale droite sans extension intra-osseuse. B. Scanner postopératoire montrant l’approche chirurgicale de la région foraminale droite.

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Fig. 4. Histological study: A. Well-circumscribed nodule composed of numerous vessels with few nests of round and somewhat cohesive cells with abundant pale cytoplasm, smooth nuclear borders and occasional nucleoli (hematoxylin eosin × 200). B. Round cells positives with smooth muscle actin (SMA × 100); (C) Only CD34 positives vessels (CD34 × 200). Ki67 is low and PS100 do not show Schwann cell proliferation. A. Lésion nodulaire constituée par de nombreuses sections vasculaires délimitées par un endothelium aplati ne montrant pas d’atypie cytonucléaire. Certaines sections présentent une lumière dilatée et congestive contenant de nombreuses hématies (hématoxyline éosine × 200). B. L’étude immuno-histochimique met en évidence des cavités vasculaires avec le CD34 (C) et l’actine muscle lisse. Le Ki67 est faible et la PS100 ne montre pas de prolifération de cellules de Schwann.

of such a progressive tumor: a T6 hemilaminectomy with a superior T7 arthrectomy allowed access to the T6–T7 foramen and the intracanalar part of the tumor (Fig. 3B). The lesion appeared as violaceous, non-adherent to the dura, circumscribing the D6 nerve root. Though not very hemorrhagic; pleural adherences made the surgery more difficult. Pathological analysis found a nodular lesion made of numerous vascular subparts delimited by a thin endothelium, with no remarkable cytonuclear atypia. Some of these vascular subparts were dilated and contained many erythrocytes (Fig. 4A). Immunohistochemistry showed vascular cavities (CD34+) and smooth muscle fibers (Fig. 4B and C). Ki67 was low; PS100 showed no schwann cells proliferation. Analysis led to the diagnosis of glomangioma with no sign of malignity. Postoperative MRI showed no sign of residual enhancement. Pain resolved immediately in the postoperative course. At the most recent follow-up 9 months later, post-contrast MRI did not demonstrate any evidence of recurrent tumor (Fig. 5). 3. Discussion Glomus tumors are mostly benign tumors developing from structures specialized in thermoregulation, neuromyo-arterial glomus from dermo-epidermic tissues. They are slowly progressing tumors in most cases [5–8]. However, a few cases of malignant glomus tumors with regional or distant dissemination have been reported [9]. Glomangiomas are mainly located at fingers distal extremities, and look like violaceous and sensitive lesions [1]. Less frequent localizations have been reported such as digestive tract (stomach, colon), trachea, vagina and bone [9–11]. Over a series of 500 cases of soft tissue tumors from the Mayo Clinic, prevalence of glomus tumors was 1.6% [12]. Familial forms of glomus tumors exist and are secondary to a mutation within the gene encoding for glomuline, a protein involved in angiogenesis [1]. In the analysis of 52 cases of glomus tumor reported by Folpe, the immunohistochemical results are variable. CD34 and S-100 protein were expressed by

only five of 27 (19%) and none of 34 specimens (0%) respectively [9]. In 30% of cases, three clinical signs are found: persisting and insidious pain, exacerbation due to coldness, hypersensitivity to contact [13]. Such symptoms might be explained by P-substance secreting nervous fibers associated with glomus tumors [1,10]. Paravertebral localization with both intra and extracanalar development, as described in the present case, is exceptional with only two cases reported in the literature [2,4]. Axmann et al. [4] describe the case of a 50-year-old man presenting a paravertebral glomus tumor in the lumbar region, with significant extension to soft tissues and non-homogenous enhancement on MRI. Delay in diagnosis is frequent, particularly for spinal localizations where clinical signs are sparse [8]. Typical findings of glomus tumors on MRI are: hypo-intense signal on T1-weighted sequences, hyper-intense on T2-weighted sequences and a strong and homogenous enhancement following gadolinium injection [1,4,6,8]. If there is an osseous extension, it usually consists in well-circumscribed osteolytic lesions with secondary sclerosis [1]. Differential diagnoses on MRI are schwannoma, meningioma, Ewing sarcoma, primitive neuro-ectodermal tumors, paraganglioma (tumors that arise from the extra-adrenal paraganglion of the autonomic nervous system, typically benign with slow-growing) and carcinoid or epithelioid leiomyosarcoma [2,14]. A particular attention can be made with melanocytic schwannoma. This is a rare soft tissue tumor, which arises most commonly in the paraspinal sympathetic chain with an unpredictable prognosis. Twenty-five percent of the patients develop metastasis [15,16]. Based only on imaging data, distinguishing these tumors from atypical glomus tumors is not possible and diagnostic are discussed according to their incidence. Moreover, in our case, the dumbbell shape was very evocative of a schwannoma. Regarding the risk of malignant progression, Folpe et al. have classified glomus tumors in 4 distinct groups [9]: from malignant glomus tumors (38% risk of metastatic progression) to glomangiomatosis (no report of metastasis). Histologically, glomus tumors are made of glomus cells along with dilated vessels and smooth

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damaged. This includes ligation of segmental vessels medial to the costotransverse joint or behind the formation of anastomoses at the lateral aspects of the vertebral bodies. For this reason, a selective angiography of the spinal cord could have been performed. 4. Conclusion Intracanalar spinal glomus tumors with paravertebral extension are rare. Such presentations are almost always misdiagnosed with intracanalar extramedullary tumors such as schwannoma or meningioma. Radiological criteria for glomus tumors being unspecific, definitive diagnosis rely exclusively on histological findings. Surgical treatment should be considered every time it is possible, with alternatives such as radiosurgery or radiotherapy available for more difficult localizations, remnants or recurrences. Fig. 5. Postoperative MRI at 9-month after surgical resection. Axial T1-weigthed post-gadolinium of the thoracic spine without evidence of recurrence. IRM postopératoire à 9 mois de l’intervention chirurgicale. Coupe axiale de la région opérée en séquence pondérée en T1 et après injection de gadolinium ne montrant pas de reliquat tumoral et de signe de récidive.

muscle cells. Glomus cells have a round shaped nucleus, with no mitosis or nuclear polymorphisms [4,6]; atypia and necrosis are rare but possible [5]. Three kinds of glomus tumors are described according to the main cellular subtype: vascular (in this case, it is often called a glomangioma [17], myxoid and solid) [7]. Complete surgical removal is usually considered the treatment of choice for spinal glomus tumors [8]. In most cases, clinical results are satisfactory with complete pain relief [18]. The usefulness of preoperative embolization is still discussed for large vascular tumors where hemorrhagic risk is significant [1,6,8]. One case of an intra-pedicular right T11 glomus tumor treated with CT-guided radiofrequency has been reported with interesting results although follow-up was only 6 months [8]. Radiosurgery or radiotherapy has been proposed for treatment of tumor residues or recurrences [2]. Given the limited follow-up available for cases reported in the literature, with a mean duration of 8 months, the risk of recurrence is not well assessed for glomus tumors [8]. Although surgical removal seems to be the main prognostic factor [18], complete resection should not always be the aim if it involves a significant neurological risk given the slow progression and the availability of adjuvant therapies. A rare, but devastating, complication of spine surgery to this region is spinal cord ischemia or infarction. If this occurs, the resulting neurologic damage is usually irreversible and permanent. Commonly the third through seventh thoracic segments are supplied by only one radicular artery that enters along the fourth or fifth thoracic nerve root and the eighth thoracic segment to the conus medullaris receives its main supply via the artery of Adamkiewicz. This artery usually (85% of the time) originates from a left posterior intercostal or lumbar segmental artery between T9 and L2 [19]. In general, ischemic injuries to the spinal cord are obviated during posterior approach surgery (the radiculomedullary arteries enter the epidural space ventral to the approach and ligation or cauterization of these vessels is not required) [20]. But in our case regarding the tumor localization in the right side with a lateral body extension, the risk is very low. Effectively a segmental spinal cord ischemia could occur if even one radiculomedullary artery was

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