Desmoid Tumor Presenting as a Typical Cervical Dumbbell Tumor: Case Report and Review of the Literature

Desmoid Tumor Presenting as a Typical Cervical Dumbbell Tumor: Case Report and Review of the Literature

Accepted Manuscript Desmoid Tumor Presenting as a Typical Cervical Dumbbell Tumor:A Case Report and Literature Review Ning Luo, Xia He, Guangzhou Li, ...

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Accepted Manuscript Desmoid Tumor Presenting as a Typical Cervical Dumbbell Tumor:A Case Report and Literature Review Ning Luo, Xia He, Guangzhou Li, Yehui Liao, Qiang Tang, Rupei Ye, Dejun Zhong PII:

S1878-8750(19)30033-6

DOI:

https://doi.org/10.1016/j.wneu.2019.01.001

Reference:

WNEU 11111

To appear in:

World Neurosurgery

Received Date: 15 August 2018 Revised Date:

31 December 2018

Accepted Date: 2 January 2019

Please cite this article as: Luo N, He X, Li G, Liao Y, Tang Q, Ye R, Zhong D, Desmoid Tumor Presenting as a Typical Cervical Dumbbell Tumor:A Case Report and Literature Review, World Neurosurgery (2019), doi: https://doi.org/10.1016/j.wneu.2019.01.001. This is a PDF file of an unedited manuscript that has been accepted for publication. As a service to our customers we are providing this early version of the manuscript. The manuscript will undergo copyediting, typesetting, and review of the resulting proof before it is published in its final form. Please note that during the production process errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.

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Desmoid Tumor Presenting as a Typical Cervical Dumbbell Tumor:A Case Report and Literature Review Ning Luo(the first author), Xia He,Guangzhou Li, Yehui Liao, Qiang Tang, Rupei Ye, Dejun Zhong

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Department of Spinal Surgery, Affiliated Hospital of Southwest Medical University, Southwest Medical University, Luzhou, China (N Luo MD, Yh Liao MD, Gz Li MD, Q Tang MD, Dj Zhong MD); Department of Pediatric Surgery, Affiliated Hospital of Southwest Medical University, Southwest Medical University, Luzhou, China (He Xia MD); Department of Pathology, Affiliated Hospital of Southwest Medical University, Southwest Medical University, Luzhou, China (R Ye PhD)

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Correspondence to:

Dr Dejun Zhong, Department of Spinal Surgery, The Affiliated Hospital of Southwest Medical University, Southwest Medical University, Luzhou, 64600, P. R. China Email: [email protected]

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Tel.: +86-0830-3165441

Keywords spinal tumor, head and neck neoplasm, dumbbell-shaped tumor, desmoid tumor, case

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Desmoid Tumor Presenting as a Typical Cervical Dumbbell Tumor:A Case

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Report and Literature Review

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Abstract

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Background: Dumbbell-shaped tumor is a type of the inner and outer cervical spinal canal

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tumor, and most of them are neurogenic tumors. Desmoid tumor is a rare tumor, and no case of

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them involving intervertebral foramen formed dumbbell-shaped in cervical spine have been

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reported before in English literature. Here we report a case of desmoid tumor arising in the

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cervical spine which is presented as typical dumbbell-shaped tumor.

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Case Description: A 47-year-old female was admitted to our department with a mass in her left

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neck. The tumor was initially thought to be a neurogenic cervical dumbbell tumor based on

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physical and radiological examination. Postoperative HE and immunohistochemical staining

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verified the diagnosis of a cervical dumbbell desmoid tumor, which had never been reported

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before. We report our experience and reviewed literature about desmoid tumor to share our

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experience and explore proper treatment option of such lesion.

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Conclusion:

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tumors. Before the treatment plan was undertaken, thorough examinations including surgical

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pathology were necessary.

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Case presentation

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In March 2017, a 47-year-old female was admitted to hospital with a one-year history of

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recurrent, dull left-neck, shoulder, and upper-arm pain. This patient had not lost appetite or

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weight, and she had no significant past medical history. She and her families had farmed for

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generations, with no familial medical history of cancer and inherited disorder before. The pain

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occurred at night and was not accompanied by no numbness or pain of the limbs, dizziness or

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headache. She adopted self-medication with non-steroidal anti-inflammatory (Celecoxib, Pfizer,

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Desmoid tumors in the head and neck may present as cervical dumbbell-shaped

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USA), and analgesic drugs without relief of the symptoms. Over 3 months before admission, the

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neck and shoulder pain became worse, with radiation into the left deltoid and lateral upper arm.

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She noted a palpable subcutaneous mass in the anterolateral neck. At a local hospital, a magnetic

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resonance imaging (MRI) scan revealed a large mass on the left side of the cervical spine, which

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was considered a neurogenic tumor.

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Investigations

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On physical examination, a poorly mobile, tender, deep mass was palpable in the left neck. The

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mass was not warm, red or ulcerated. Paracanthosis and percussion pain in the C4–5 spinous

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processes were present. Acupuncture revealed hypoesthesia in the skin of the lateral left upper

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arm. Muscle strength of the left deltoid and the biceps brachii was level IV. Spurling's test was

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positive and the left biceps brachii was hyporeflexia. Visual Analogue Scale was 7 points.1

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Electromyography of the upper limbs yielded normal results. Radiology examination revealed a

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mass in the left lateral foramen of C4–5 and on the left side of the anterior C2–6, no bone

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destruction (Fig. 1).

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Clinical diagnosis and treatment

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Preoperative CT-guided biopsy was negative for neoplasm. The initial diagnosis was a dumbbell

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tumor, with high probability of being a neurogenic tumor. The tumor was resected with a

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combined anterior-posterior approach, and bone graft-fusion with screw-rod internal fixation was

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performed. During the operation, the tumor was found to be gray-white, hard, and completely

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enveloped. Most of the mass lay on the dorsal side of the longus colli, invaded the C4–5

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intervertebral foramen, and compressed the left C5 root. However, the tumor was not derived

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from the C5 nerve root and had no evident adhesion with the C5 root and surrounding tissues

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(Fig. 2). The tumor was completely removed while the left C5 nerve was protected.

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Postoperative hematoxylin-eosin staining, Van Gieson staining and immunohistochemical

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staining revealed the tumor had the phenotypic characteristics of a desmoid type fibromatosis

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(WHO 2013 ICD-O:8821) (Fig. 3). Local radiotherapy and chemotherapy was given after the

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specific diagnosis.2 The recurrence of tumor was not been found at 1 year after the operation and

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no nerve injury (Fig. 4). The cervical spine had normal range of motion, and there was no

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kyphosis.

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Genetic analysis

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We do genomic DNA sequence analysis in our patient for the presence of genome-wide. A

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mutation in the β-catenin gene in the Wnt signaling pathway, which binds to the β-catenin/TCF

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components of the IGFBP-6 promoter region, is found. This mutation leads to an abnormal

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increase in beta-catenin protein and abnormal Wnt pathway. These findings are consistent with

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reports of patients with desmoid tumors who had mutations in the β-catenin gene.3,4

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Literature review

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Dumbbell-shaped tumor

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Dumbbell-shaped tumor is a type of the inner and outer spinal canal tumor.5 Most are neurogenic

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tumors and are characterized by tumor encroachment on anatomic structures (intervertebral

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foramen/dura) and a dumbbell or hourglass configuration. Nerve-sheath cell tumors are

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reportedly the major type of dumbbell-shaped tumor in adults, accounting for about 80%, and

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neuroblastoma is common in children.6 A few non-neurogenic dumbbell-shaped tumors are

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angioma, angiolipoma, chondrosarcoma and other pathologic types.6 Dumbbell-shaped tumors

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account for about 15%–38% of inner cervical spinal canal tumors, of which 50% occur in the

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cervical segment.5

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The various morphological characteristics of dumbbell tumors necessitate various surgical

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procedures for treating them, but there is no consensus on surgical classification of dumbbell

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tumors. There are many different opinions on the surgical approach and resection maneuver of

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dumbbell-shaped tumors. Four types of dumbbell tumors, based on radiographic features, were

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described by Eden in 1941, whcih is still used.7 Xiao et al devised a surgical staging of the

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tumors based on division of the cervical spine anatomical regions, with corresponding treatment

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strategies for various stages of tumors.8 In 2009, the PUTH classification was proposed by Jiang

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et al, according to the cross-sectional image of the tumor, leading to the selection of

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corresponding surgical plans.9 Guo et al devised a classification based on the relationship

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between tumor and the sacrum and guided surgical approach selection, however the

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classification applied only to dummbell tumors which were arised in sacral canal.10 Asazuma et

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al put forward the Toyama classification.11 Based on this classification, tumors were divided into

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six types, different surgical approaches were chosen according to different types.

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Desmoid tumor

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Desmoid tumor, or aggressive fibromatosis, is a fibrous tumor formed by the proliferation of

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fibroblasts and myofibroblasts arising from the deep fascia or aponeurosis, which have been 3

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first reported by Muller, in 1838.12,13 Histologically, desmoid tumors have no abnormal mitotic

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figures. Nonetheless, they can invade into adjacent important structures and organs and have a

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high risk of recurrence.14 Desmoid-type fibromatosis often occurs as a sporadic lesion or in

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patients with familial adenomatous polyposis (FAP), which may be related to mutations of the

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adenomatous polyposis coli (APC) gene.15 At present, it is considered that the molecular genetic

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mechanism of desmoid tumor are mainly the APC gene inactivation or β-catenin activating can

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lead to nuclear β-catenin level rised, the Wnt pathways activated, activate the downstream target

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genes transcription, such as c-myc, cyclin D1 and cox-2, cause fiber cell proliferation and

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differentiation of out of control.16 Desmoid tumors are rare, and the incidence is low

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(0·24–0·43/100 thousand), accounting for 0·03% of all tumors.17,18 Tumors are located on the

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torso usually, and most originate in the abdominal wall.19,20 Desmoid tumors occurring outside

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the abdominal wall are called “extra-abdominal desmoid tumors”. Those of the head and neck

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account for about 11%–15% of extra-abdominal desmoid tumors.21,22 They rarely occur in the

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neck, only a few cases have been reported.23,24

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MRI features of desmoid tumor include 1) round or elongated tumor along muscle fibers, with

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uneven signals, and striped, punctate or flaky low-signal intensity fibrous tissues revealed in

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T1WI; 2) T1WI signals like those of muscle signals, T2WI signals slightly higher than muscle

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signals, and high signal if fat suppression is present; 3) infiltrative growth without envelope and

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clear boundaries, or growth with incomplete envelop; and 4) progressively enhancing tumor.25

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Because of the variations in the way desmoid tumours present, there is no specific method of

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treatment.26 For the treatment of desmoid tumors in the abdominal wall, one-stage surgical

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resection is the major recommended approach, combined with radiotherapy, chemotherapy and

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biotherapy.27 Extra-abdominal desmoid tumors are also treated with this strategy currently.21,23,24

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Althought there are conflicting reports on the extent of margin to be excised,28 the one-stage

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surgery should insure with negative surgical margins.29 Some research suggest that radiotherapy,

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as an adjuvant treatment for this disease, can significantly improve the cure rate of surgery and

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reduce recurrence.18 For patients who lose the opportunity of surgery, radiotherapy can

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effectively control tumor progression and make some tumors appear complete or partial

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regression. The total recommended dose of external radiotherapy is 50–60 Gy, with a dose of

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1·8–2·0 Gy per exposure.18 The radiotherapy should cover the whole region of the tumor when

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possible, with the margin of radiotherapy about 5–8 cm away from the tumor.30 Drug treatment

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consists mainly of non-cytotoxic non-steroidal anti-inflammatory drugs (sulindac and

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indomethacin), anti-estrogen drugs (tamoxifen and others), cytotoxic chemotherapy (doxorubicin

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scheme), and drugs such as interferon-α and imatinib.31-34

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Reconstruction of cervical spine stability

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Cervical instability has been reported in 20% of patients following posterior surgery for cervical

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spinal cord tumors.35 Therefore, cervical kyphosis is prone to appear in the later stages because of

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the postoperative cervical instability,36,37 and mostly in patients without internal fixation 1–3

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years after operation.8 The kyphosis is thought related to factors such as the surgical method used,

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method of internal fixation, and whether a sufficient segment is fixed.36 To avoid postoperative

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cervical instability, Some studies suggest that achieving spinal stability at the time of tumor

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resection is essential. But there is controversy about whether the operation requires internal

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fixation. Some studies suggest that bone grafting is performed on the “hinge” side to prevent

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postoperative abnormalities of cervical curvature.11,38 In addition, Asazuma et al indicated that

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spinal reconstruction including bone grafting and instrumentation should be considered when the

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spinal column is weakened structurally in removal of tumors invading at multiple levels.11 Other

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study indicated that for severe vertebral damage caused by the tumor, the vertebral body should

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be removed through the anterior approach and titanium mesh scaffold used to achieve

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anterior-plate internal fixation together with posterior internal fixation with the spinal column.9

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Besides, some studies attempt to use unilateral instrumented fixation for cervical tumors.39

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Discussion

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This is a report to a desmoid tumor presenting as a cervical dumbbell tumor. We all know

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desmoid tumor in the head and neck are unusual, and most have been described only in case

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reports.23,24,40-42 Very few reports of them which involving centrum, interspinal canal and

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paravertebral region simultaneously and almost all of them are associated with bone destruction

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and formed a giant paravertebral mass with or without invasion of spinal canal.43 In the present

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case, the tumor on the MRI T2WI and CT of the cervical spine was like that of neurofibroma,

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and no bone destruction. Further, the preoperative pathological biopsy did not obtain tumor cells

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and the morphology of the tumor was highly consistent with common neurogenic

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dumbbell-shaped tumors. Accordingly, the patient was considered to have a neurogenic cervical

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dumbbell tumor before histopathologic examination established the diagnosis.

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In this patient, the tumor was on the left side of the head and neck, invading the left paravertebral

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region of C2-6 and the left intervertebral foramen of C4-5. According to the Toyama

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classification, it was divided into type IIc IF2 TF1. Based on this classification, a combined

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posterior and anterior approach had been selected for.11 The posterior resection of the tumor

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required removal of the left lamina and lateral mass of C5 to expose the dural sac and nerve root

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and the anterior approach achieved separation of the mass. In fact, we recognized that it is

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controversial whether the posterior fixation is necessary. In our opinion, because of her spinal

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column is weakened structurally in removal of tumor, the spinal reconstruction including bone

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grafting and instrumentation should be considered. To avoid instability and kyphosis, the

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posterior cervical internal fixation system and lateral mass screw fixation were performed. This

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is also consistent with some of studies.8,9,11

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Postoperative histological test and genetic analysis certified the ultimately diagnosed as desmoid

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tumor. For the treatment of desmoid tumors , one-stage surgical resection were recommended by

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many researches. When the diagnosis was clear, a chemoradiotherapy was added, which was

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based on the combined reported experience of surgical resection and non-surgical treatment of

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cervical dumbbell tumors and of desmoid tumors.11,18

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recurrence of tumor is not be found and her cervical spine have normal range of motion with no

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kyphosis.

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To the best of our knowledge, this is the first case to extra-abdominal desmoid tumor presenting

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as a cervical dumbbell-shaped tumor. We can regarded it as a special type of desmoid tumor

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occurring in the head and neck, while it also conformed to the neurosurgical definition of

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dumbbell-shaped tumor. Therefore, it can be temporarily named as dumbbell-shaped desmoid

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tumor. For desmoid tumor involving the inner and outer cervical spinal canal, it may be

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temporarily classified according to the types of cervical dumbbell tumors and the related

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operations. Furthermore, the different diagnosis of dumbbell-shaped desmoid tumor included

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Schwannoma, Neurofibromatosis, Inflammatory myofibroblastic tumor, Nodular fasciitis.44-51

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In the operation, the patient’s giant paravertebral dumbbell-shaped desmoid tumor was hard and

According to present situation, the

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required careful separation from the vertebral artery to avoid tearing the artery. Although this

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tumor have a complete capsule, most desmoid tumors may invaded surrounding muscle and have

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an unclear boundary. In the end, because the rate of recurrence of desmoid tumors is high,

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long-term follow-up is needed to guarantee a cure.

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Summary

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We have reported here a dumbbell-shaped desmoid tumor. The diagnosis of desmoid tumor was

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verified with HE staining and immunohistochemical staining with antibodies to smooth muscle

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actin(SMA) and β-catenin. Genomic DNA sequence analysis revealed a mutation in the

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β-catenin gene, which can lead to an increase in β-catenin and has been identified in patient with

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desmoid tumor. This special disease is easily misdiagnosed. We draw attention to neurosurgeons,

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spinal surgeons, otolaryngological surgeons and others that cervical desmoid tumors in the head

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and neck may as well present as cervical dumbbell-shaped tumors.

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Figure Legends

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Figure 1: X-ray films of cervical spine with lateral view showing cervical degeneration and

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calcification of the anterior longitudinal ligament and the nuchal ligament (A). CT scan and

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three-dimensional reconstruction of cervical vertebra revealing mass shadow in the left lateral

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intervertebral foramen of C4–5 and on the left side of the anterior C2–6 in the horizontal

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direction (arrows), with its internal density close to that of muscle tissue (B). Compression of the

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left vertebral artery, left common carotid artery and carotid bifurcation at the corresponding

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plane (C). MRI revealing mass shadow in the left lateral foramen of C4–5 and on the left side of

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the anterior C2–6 (arrows), enlarged left intervertebral foramen of C4–5 (D), about 2·9 × 3·7 ×

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7·9 cm in size (E, F). Inhomogeneous tumor signal of low signal like that of muscle tissue by

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T1-weighted (D), high signal by T2-weighted (E). Enhanced scan displayed uneven and

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moderate enhancement of the lesion (F).

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Figure 2: Posterior approach to resected tumor, which has invaded the C4/5 intervertebral

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foramen, and C3–6 screw-rod system internal fixation (A). During the operation, the tumor was

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gray white in color, hard in texture, complete enveloped, and most of the mass lay on the dorsal

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side of the longus colli, about 2 × 3 × 7 cm large (dashed. B).

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Figure 3: Histopathology (hematoxylin and eosin stain; ×100 magnification) revealing collagen

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fiber bundles and spindle fibroblast cells, arranged in a wavy pattern. A proliferation of uniform

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fibroblasts in a collagenous stroma (black star. A), immunohistochemical staining revealed the

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tumor to be smooth muscle actin (SMA) (+), β-catenin (+), Ki-67 (+) (B, C, D).

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Figure 4: Six months after operation, MRI showed the tumor had been resected completely (A,

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B, C). One year after operation, MRI showed no recurrence of tumor (D, E, F). X-ray films of

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the cervical spine with lateral and frontal view showed C3–6 screw-rod system internal fixation,

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the cervical curvature had recovered to normal, and there was no cervical kyphosis (G, H).

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Abbreviation Magnetic resonance imaging: MRI Computed tomography: CT

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Nonsteroidal anti-inflammatory drugs: NSAIDs

ACCEPTED MANUSCRIPT Declaration of interests

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We have no conflicts of interest.