Desmoid tumors in neurosurgery: A review of the literature

Clinical Neurology and Neurosurgery 129 (2015) 78–84

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Clinical Neurology and Neurosurgery journal homepage: www.elsevier.com/locate/clineuro

Review

Desmoid tumors in neurosurgery: A review of the literature夽 Philippe De Vloo a,∗,1 , Jan De Vlieger a,1 , Vincent Vander Poorten b,c , Raf Sciot d , Johannes van Loon a , Frank Van Calenbergh a a

Department of Neurosurgery, University Hospitals Leuven, KU Leuven, Leuven, Belgium Department of Oncology, Section Head and Neck Oncology, University Hospitals Leuven, KU Leuven, Leuven, Belgium Department of Otorhinolaryngology, Head and Neck Surgery, University Hospitals Leuven, KU Leuven, Leuven, Belgium d Department of Pathology, University Hospitals Leuven, KU Leuven, Leuven, Belgium b c

a r t i c l e

i n f o

Article history: Received 18 November 2014 Accepted 15 December 2014 Available online 24 December 2014 Keywords: Desmoid tumor Aggressive fibromatosis Desmoid-type fibromatosis Cicatricial

a b s t r a c t Desmoid tumors (DTs) are rare myofibroblastic neoplasms, which are mostly sporadic, but sometimes associated with familial adenomatous polyposis syndrome. Neurosurgical cases of DT have been very scarce. We review the literature concerning neurosurgical DTs and describe the first case of a cicatricial DT after the resection of vestibular schwannoma, presenting as a painful swelling in the retrosigmoid scar. Contrary to other localizations in the body, standard-of-care wide margin resection cannot be performed in intracranial and spinal DTs. Therefore, maximally safe resection followed by radiotherapy when tumor margins are not free can be proposed as a treatment strategy in neurosurgical DTs. © 2014 Elsevier B.V. All rights reserved.

Contents 1. 2. 3.

4.

Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Methods . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 2.1. Case . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Discussion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3.1. Primary intracranial . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3.2. Primary head and neck DTs with an intracranial extension through the skull base . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3.3. Peripheral nerve involvement . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3.4. Plexus involvement . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3.5. Spinal and spinal cord involvement . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3.6. Cicatricial DTs after neurosurgery . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Conclusion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Funding . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Conflict of interest . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Acknowledgment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

夽 Portions of this work were presented in poster form at Annual Scientific Meeting of the Belgian Society of Neurosurgery, Ghent, Belgium, March 30, 2013. ∗ Corresponding author at: Department of Neurosurgery, University Hospitals Leuven, Herestraat 49, 3000 Leuven, Belgium. Tel.: +32 16 34 42 90; fax: +32 16 34 42 41. E-mail addresses: [email protected], [email protected] (P. De Vloo). 1 These authors contributed equally to this manuscript. http://dx.doi.org/10.1016/j.clineuro.2014.12.007 0303-8467/© 2014 Elsevier B.V. All rights reserved.

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1. Introduction

2.1. Case

Desmoid tumors (DTs), also called aggressive fibromatosis or desmoid-type fibromatosis, are histologically benign myofibroblastic neoplasms that exhibit slowly infiltrative growth. DTs do not metastasize but can be locally invasive and often recur after surgical excision [8,11,55]. It was recently shown that DTs arise from mesenchymal stem cells [68]. They are very rare, accounting for approximately 0.03% of all neoplasms and have an incidence of 2–4 per million per year [11,52]. The first DT description was made by MacFarlane in 1832 [31]. The majority of the cases are sporadic. Sporadic DTs appear to be three times more frequent in women than in men with a peak in the third and fourth decade [39,52]. The most frequent locations of sporadic DTs are the extremities, trunk musculature, head and neck and the abdominal cavity [1,52]. They mostly present as painless progressive swellings [39]. Besides these sporadic cases, there is group of DT with a clear association with familial adenomatous polyposis (FAP) or Gardner’s syndrome and other adenomatosis polyposis coli (APC) gene mutations [66]. Up to 29 percent of the FAP patients harbor DTs, a frequency 850 times greater than the general population [31]. Most FAP-associated DTs arise in the small bowel or the mesentery and cause more mortality, morbidity and recurrence when compared to sporadic DTs [38]. Based on the female predominance, an association with sex hormones is presumed. The direct relationship of the growth rate to the level of endogenous estrogen in the female patients and the demonstration of significant amounts of estradiol but not progesterone receptors in the tumor cytosol suggest that the growth rate of DT is regulated by steroid sex hormones [21]. Anti-estrogenic treatment was effective in about half of the cases in which it was applied [67]. Some cases, particularly with abdominal wall localization, are thought to be associated with local myofibroblastic trauma such as during pregnancy [2,21] or after traffic accidents [4,41]. Exceptionally, cases of DT have been found in surgical scars, especially after mammoplasty [35]. The current standard of care for initial management is surgical removal with wide margins [55]. If for functional or cosmetic reasons these tumor-free margins cannot be obtained, adjuvant radiotherapy decreases recurrence rates according to most studies [1,23,42]. It has been suggested that the outcome of radiotherapy alone is equal to that of combined surgery and radiosurgery, though this remains unclear [15,25]. Pharmaceutical therapy including chemotherapy is controversial [31]. In the neurosurgical literature, DTs are only anecdotally described. Firstly, there are a few, mainly pediatric, case reports of primary intracranial DTs [64]. Next, sometimes a neurosurgical approach is needed when primary head and neck DTs affect the skull base and secondarily grow intracranially [64]. Furthermore, DTs can compromise the brachial or lumbosacral plexus [13], peripheral nerves [59], or the spine and/or spinal cord [55]. Finally, a few authors reported on DTs arising in neurosurgical scars [26]. We describe a case of cicatricial DT encountered in the retrosigmoid scar after vestibular schwannoma resection and review the literature on neurosurgical DTs.

In January 2006, brain Magnetic Resonance Imaging [MRI] revealed a left-sided vestibular schwannoma in a 56-years old female with tinnitus and vertigo. Due to tumor growth on serial imaging, radiosurgical (Gamma Knife, Elekta, Stockholm, Sweden) treatment was performed in March 2007, complicated by a transient left facial nerve palsy. Following further tumor volume increase (Fig. 1a), a surgical resection via retrosigmoid approach was performed in October 2009. More than 2 years later, in January 2012, because of a painful progressive retro-auricular swelling, revision surgery was performed demonstrating a dense layer of yellowish tissue overlying the dura. No pathological specimen was retrieved. Since the retro-auricular swelling recurred soon, she was referred to our hospital in August 2012. On clinical examination there was a firm swelling behind the left ear, about 4 cm in diameter, with a normal appearance of the overlying skin and the scar (Fig. 1d), painful at palpation. No neurological deficits were found on clinical examination. Ultrasound imaging showed a hyporeflective and inhomogeneously vascularized lesion with no clear margins. Fine needle puncture biopsy was inconclusive. On computed tomography there was no bony infiltration nor erosion. MRI demonstrated an ovular and lobulated structure between the sternocleidomastoid and paraspinal muscles and the craniotomy cavity, T2-hypointense and slightly T1-hyperintense, with important but inhomogeneous contrast enhancement and measuring 40 mm× 50 mm × 50 mm (Fig. 1b and c). In November 2012, the retrosigmoid scar was reopened and a well-encapsulated tumor was found. Peritumoral dissection along the skin and the cervical muscles unto the craniotomy margins was relatively easy. Upon further dissection at the craniotomy site, we noted an important infiltration of the dura, which could be readily dissected from the underlying and indurated arachnoid. Macroscopically, we performed an apparently complete resection. Pathological examination demonstrated bundles of buckled fusiform cells, moderately densely embedded in a collagenous matrix and surrounded by multiple vessels. No clear cellular atypia, increase of mitosis or necrosis was noted (Fig. 1e). Tumor cells expressed alpha-smooth muscle actin and beta-catenin, mainly cytoplasmatic, but also nuclear (Fig. 1f). The tumor margins were not tumor free. The pathological diagnosis of a DT was made. Because of the positive tumor margins, adjuvant radiotherapy was performed (28 × 2 Gy) starting from December 2012. At last follow-up 2 years after tumor removal, there were no clinical or radiological signs of tumor recurrence.

2. Methods The described patient has given written consent for submission of the case report to the journal. On November 1st 2014 we searched the MEDLINE database for “desmoid tumor” (total of 2112 articles), “desmoid-type fibromatosis” (total of 1555 articles) and for “aggressive fibromatosis” (total of 1502 articles). We further selected cases within the scope of neurosurgery.

3. Discussion Neurosurgeons can grossly encounter DTs in six occasions.

3.1. Primary intracranial Literature research showed very few intracranial cases of DT, all cases being pediatric (Table 1). Except for the peculiar case of Chung [3] with an APC mutation and a medulloblastoma followed by two intracranial and one spinal metachronous DTs, no patients were older than 3 years, suggesting a congenital origin. All were resected without recurrence, taking into account the limited length of follow-up. Due to its rarity, intracranial DT is poorly recognized. Histopathological differentiation with other spindle cell lesions such as fibrosarcoma, reactive fibrosis, nodular fasciitis, fibrous

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Fig. 1. (a) September 2009: coronal gadolinium-enhanced T1-weighted MRI of showing the left-sided vestibular schwannoma; (b) September 2012: coronal gadoliniumenhanced T1-weighted and (c) T2-weighted MRI showing a slightly T1-hyperintense and T2-hypointense inhomogeneous contrast enhancing lobulated mass measuring 40 mm × 50 mm × 50 mm; (d) November 2012: large subcutaneous mass near the lower border of the retrosigmoid approach scar; (e) Low power histological view, showing the typical bundles of wavy spindle cells with an open chromatin pattern and a small nucleolus. Hematoxylin and eosin stain, original magnification ×200×; (f) B-catenin immunostain, showing cytoplasmic and nuclear expression (arrows). Immunoperoxidase stain, original magnification 200×.

histiocytoma and infantile myofibromatosis remains an enormous challenge [63]. Some authors presume a dural origin of the intracranial DTs. In the two cases described by Wang et al. [63] there was an interface between the dura and DTs while the histological examination showed a positive vimentin test in the DT with a negative test in the dura, implying a mesenchymal origin as described by Wu [68]. As suggested by Wang et al. [63], these primary intracranial DTs may arise from cranial sutures. Reviewing the cases from Table 1, this might be the coronal suture for Chung (frontal) [3] and Wang [63] and the sagittal suture for Yazici [70] and Chung (parietal) [3]. Friede et al. [15] describe an adult case with a tumor centered on the coronal suture operated upon by professor Yas¸argil,

but due to fact that it was classified as a fibroma and had a recurrence 11 months postoperatively with a histological transformation considered to be malignant, we do not include this case. Interestingly, common carotid and vertebral angiography in the case of Dolman [7] showed an avascular tumor while in the case of Wang et al. [63] the superficial temporal artery supplied the tumor and underwent preoperative embolization. 3.2. Primary head and neck DTs with an intracranial extension through the skull base While the head and neck region is a predilection site for extraabdominal DTs, reported to constitute 6% to 15% of all DTs [1,34], we

Table 1 Primary intracranial DTs. Authors and year of publication

Patient age and sex

Site of DT

Signs and symptoms

Treatment

Follow-up (months)

Relapse

Dolman et al. [7]

6 m, M

Seizures

Subtotal resection

7

None

Yazici et al. [70]

36 m, M

Left petrous bone with temporal and infratentorial extension; extra-axial Right frontal; extra-axial

Painless swelling

Resection

12

None

Vertical diplopia (2 y after cerebellar medullobastoma resection and radiochemotherapy) Asymptomatic finding on routine imaging (5 y after cerebellar medulloblastoma resection and radiochemotherapy)

Complete resection

?

None

Complete resection

?

None

Ictal paralysis of the left leg, facial spasm

Total resection

36

None

Chung et al. [3]

Wang et al. [64] a

11 y, M

Right parietal with sagittal sinus compression; extra-axial

14 y, Ma

Right frontal; extra-axial

20 m, M

Right frontal; extra-axial

Same APC mutation patient with a second DT located elsewhere 3 years later.

P. De Vloo et al. / Clinical Neurology and Neurosurgery 129 (2015) 78–84

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Table 2 Primary head and neck DTs with intracranial extension through the skull base. Authors and year of publication

Patient age and sex

Site of DT

Signs and symptoms

Treatment

Follow-up (months)

Relapse

Pediatric Suarez Nieto et al. [71]

14 y, M

Prooptosis, facial assymmetry

Resection

84

Yes

O’Ryan et al. [45]

27 m, F

Sphenoid, maxilla, orbit, subtemporal region, anterior and middle cranial fossa Infratemporal fossa with skull base extension to middle cranial fossa

Mandular hypomobility, facial assymetry

Biopsy Chemotherapy

10

None

Infratemporal fossa, orbit wall, inferior temporal cranial base, gingivobuccal sulcus Infratemporal, temporal, middle cranial fossa Masticator space, infratemporal fossa, middle cranial fossa

Painless cheek mass

Gross total resection

39

None

Painless mass, masticator space Trismus, exophtalmos

Chemotherapy, gross total resection Gross total resection

20

None

27

Yes

Speech and swallowing disturbances for 2 m, facial asymmetry for 1y Exophthalmos, painless maxillary mass, eye movement limitation, absence of indirect light reflex Dysphagia

Biopsy Chemotherapy

?

?

Gross total resection

60

None

Gross total resection with positive margins

27

None

Facial hypoesthesia, cheek swelling Headache Temporal mass, trismus

Resection

9

None

Resection Radiotherapy

– 30

– None

Trismus, headache, abducens palsy, facial hypoesthesia, swelling Trismus, seizures, headache

Incomplete surgery

8

None

RT (primary); interferon (for quick relapse 10 m later)

16

None

Incomplete surgery, radiotherapy Incomplete surgery

–

–

?

Yes

Incomplete surgery

–

–

Perez-Cruet et al. [48]

17 m, M

5 y, F 5 y, F Flacke et al. [10]

3 y, M

Right skull base of the middle cranial fossa with extension to pontine cistern

Wang et al. [64]

1 m, F

Right temporal skull base

Wang et al. [63]

3 y, F

Parapharyngeal up to skull base

41 y, M

Maxillar sinus, pterygoid, middle cranial fossa Sphenoid, orbita, middle cranial fossa Infratemporal fossa, pterygomaxillary space parapharyngeal space, middle cranial fossa Infratemporal fossa, Meckel’s cave, cavernous sinus, Dorello’s canal

Adult Osguthorpe et al. [46] Crisi et al. [6] West et al. [65]

26 y, M 25 y, M

Corsten et al. [5]

27 y, M

Raguse et al. [51]

47 y, F

Gursoy et al. [18]

34 y, M

De Tella et al. [61]

20 y, F

Jenny et al. [24]

48 y, F

Infratemporal fossa, pterygopalatine fossa, ramus of the mandible, coranoid process, middle skull base, meningeal enhancement Sphenoid sinus, pterygoid recesses, retroorbital area, hypothalamic area Sphenoid sinus, ethmoid sinus, nasal cavity, orbit, anterior cranial fossa Sphenoid sinus, pterygoid recesses, cavernous sinus, sella floor, clivus, petrous temporal bone and mastoid

found only 16 cases with intracranial extension through the skull base described in the literature. In these cases there does not seem to be an age or gender predilection (Table 2). Most cases seem to have their bulk mass in the infratemporal fossa with extensions through the middle cranial skull base neuroforamina and present with a mass and/or trismus. In the very rare cases of temporal muscle [33,34,57] or scalp [58,62] DTs neurosurgical aid for excision might be asked.

Panhypopituitarism and diabetes insipudus Prooptosis, nasal obstruction Facial pain

Ferraresi reports on an exceptional case in which the DT selectively invaded the nerve fascicles and did not invade the nerve from the neighboring structures [9]. Other authors recently reported on two cases in which they used synthetic vascular grafts to cover neurovascular structures in order to prevent tumor invasion [47]. For patients who are not suitable for radiotherapy and in whom the neurovascular structures need to be secured because of the risk of local recurrence, the authors state that this method can prevent possible future invasion of vessels and nerves.

3.3. Peripheral nerve involvement 3.4. Plexus involvement Considering the wide distribution of DTs over the body and their infiltrative character, they can compress, incorporate or adhere to virtually all peripheral nerves. We found cases involving upper extremity nerves [27,59], thoracic roots [55] and lower extremity nerves [27]. The standard treatment seems to be a wide surgical exposure of the lesion with resection of the epineurium. Recurrence rate is considered to be more than 50% [27].

The largest series of brachial plexus DTs published so far is that by Gaposchkin et al., describing 11 cases [13]. The authors advocate maximally safe resection, preserving neurological status, followed by RT. Seinfeld added four cases and also supports this strategy [53]. In a large surgically treated peripheral non-neural sheath nerve tumor series from Louisiana 11 out of 146 tumors were DTs, of which 6 involved the brachial plexus [28].

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Table 3 Spinal DTs. Authors and year of publication Pediatric Friede et al. [12] Kriss et al. [30]

Patient age and sex

Site of DT

Signs and symptoms

Treatment

Follow-up (months)

Relapse

11 y, F

T2–T5 intramedullar

Resection

–

–

19 m, F

C2–C4 spinous process periosteum extension of paraspinal DT T9–T10 intraspinal extradural transforaminal extension of posterior mediastinal and chest wall DT T7–T10 intraspinal extradural extension of posterior thoracic cavity DT with vertebral body erosion C7 vertebral body

Hypesthesia, spasticy and later paraparesis Cervical mass

Resection

17

None

Posterior thoracic painless mass

Subtotal resection

0

–

Parapareseis, scoliosis

Subtotal resection, radiotherapy

108

None

Asymptomatic finding on routine imaging (4 y after cerebellar medulloblastoma resection and radiochemotherapy)

Resection

–

None

Scoliosis, mass

Subtotal resection, radiotherapy Needle biopsy, chemotherapy, radiotherapy

36

None

12

None

Ko et al. [29]

3 y, F

Shindle et al. [56]

12 y, F

Chung et al. [3]

13 y, Ma

Adult Oberthaler et al. [43] Hara et al. [20]

Shakur et al. [55]

21 y, M 50 y, M

45 y, F 38 y, F

Kim et al. [27] a b

32 y, Mb

T10–L4 pedicles, transverse processe, facet joints T3–T4 vertebral body invasion by thoracic inlet DT

Shoulder pain

C5–T1 posterior element erosion by paraspinal DT T9–T10 transforaminal extension from paraspinal DT

Neck pain, radiating arm pain, paraesthesia Back pain, paraesthesia

Subtotal resection

40

None

Resection, chemotherapy

10

None

L3–4 facet joint erosion by paraspinal DT

Painful swelling with sciatica

Resection with safe margins

10

None

Same patient as in Table 1 with APC mutation. Same patient as reported by Nam et al. [41].

Table 4 Cicatricial DTs post neurosurgery. Authors and year of publication

Patient age and sex

Site of DT

Type of previous neurosurgery

Interval neurosurgery Treatment and DT surgery (months)

Follow-up (months)

Relapse

Spinal Gonatas et al. [16]

45 y, F

Cervical

Cervical laminectomy

‘years’

14

None

Wyler et al. [69] Maurer et al. [36]

47 y, F 18 y, F

Cervical Lumbar

12 13?

13 6

Yes Probable

Lynch et al. [32]

49 y, F

Thoracic

Cervical laminectomy Lumbar instrumented fusion after traumatic vertebral fracture Thoracic meningioma

?

?

Güzey et al. [19]

50 y, F

Lumbosacral

L5–S1 instrumentation

22

14

None

Sonmez et al. [60]

55 y, F

Thoracic

Thoracic schwannoma

12

?

?

Sevak et al. [54]

48 y, F

Cervical

Cervical schwannoma

24

Puvanesarajah et al. [49]

57 y, F

Thoracic

Thoracic haemangioma

Cranial Quest et al. [50]

7 y, M

Frontal

Mitchell et al. [40] Okamoto et al. [44] McCall et al. [37]

17 y, F 15 y, M 30 y, F

Suboccipital Suboccipital Suboccipital

Frontal subependymal giant cell astrocytoma Vascular malformation Medulloblastoma Low grade astrocytoma

Kenning et al. [26]

65 y, F

Suboccipital

Foramen magnum meningioma

48

This article (2014)

63 y, F

Retromastoid

Vestibular schwannoma

36

Abdominal wall

Ventriculo-peritoneal shunting

19

Other González-Dander et al. 19 m, ? [17]

24

Resection with clear margins Resection Resection

6

None

10

Resection with wide margins Resection with wide margins Resection with wide margins Resection with wide margins Resection

24

None

17

Resection

?

?

25 8 9

Resection Resection Resection with clear margins Resection with wide margins Resection without clear margins, radiotherapy

? ? ?

None ? ?

84

None

18

None

Resection

30

None

P. De Vloo et al. / Clinical Neurology and Neurosurgery 129 (2015) 78–84

Though most often presenting with neurological symptoms, sometimes patients only notice an axial mass [13]. Gehman describes a case mimicking a thoracic outlet syndrome [14].

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Conflict of interest The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

3.5. Spinal and spinal cord involvement Acknowledgment Although the trunk musculature is a typical localization for DTs, DTs with spinal involvement are very rare, with only 10 reported cases in the literature (Table 3). They mostly present as a painless subcutaneous mass or with pain and neurological symptoms [55]. The distribution along the spine seems to be in accordance with the number of vertebra, being most common in the thoracic spine. They tend to affect children and adults in about equal numbers. Most were treated by resection alone. Recurrence rate, at least within the short mentioned follow-up periods, seems to be low. Even paraspinal DTs without spinal extensions may necessitate neurosurgical interventions for spinal stability preservation. In the case described by Hood et al. occipitocervical instrumented fusion was performed after the extensive resection of a cervical paraspinal recurrent DT [22]. 3.6. Cicatricial DTs after neurosurgery To the best of our knowledge, up to now only 14 cases of DTs in neurosurgical scars have been reported in the literature (Table 4). Eight described cases previously underwent spinal procedures, while 5 patients had cranial neurosurgical scars. We add one case, being the first one to be described after a retrosigmoid approach for a vestibular schwannoma. One described case developed an abdominal wall cicatricial DT after ventriculoperitoneal shunting. The proportion of pediatric cases seem to be lower than in the aforementioned groups, in accordance with the frequency of neurosurgical procedures in the different age groups. In these cicatricial cases, female patients tend to be affected more often than males. In cranial cicatricial cases there seems to be a preponderance for fossa posterior surgery patients. A possible explanation might be that these approaches usually create bigger muscle trauma. Also spinal cases seem to develop after surgery necessitating rather extensive approaches with relatively big muscular trauma. This could explain why no cicatricial DT was reported after simple either cervical or lumbar discectomy, being amongst the most commonly performed spinal procedures. 4. Conclusion While being well-known by general and head and neck surgeons, DTs are never encountered by most neurosurgeons. We describe the first case of a cicatricial DT after a retrosigmoid vestibular schwannoma resection and provide a literature overview of neurosurgical DTs. Due to their rarity, no clear conclusions concerning treatment strategy can be made. Contrary to other localizations in the body, standard-of-care wide margin resection cannot be performed in intracranial and spinal DTs without neurological impairment. Therefore, maximally safe resection followed by radiotherapy when tumor margins are not free can be proposed as a treatment strategy in neurosurgical DTs. Funding No funding for this study was obtained.

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