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Review

Hard neck lumps: a review of uncommon and sometimes overlooked causes of these worrying presentations M.I. Ammar a,b , R.S. Oeppen c , C. Bowles a , P.A. Brennan a,∗ a b c

Maxillofacial Unit, Queen Alexandra Hospital, Portsmouth PO3 3LY, UK Department of Otolaryngology, Tanta University, Tanta, Egypt Department of Diagnostic Imaging, University Hospitals Southampton, SO16 6YD, UK

Accepted 16 June 2017

Abstract While a neck lump is a common presentation that can raise suspicion of a potentially serious underlying disease, a hard lump, though less common, may be even more concerning for the patient, and prompt urgent investigation. Metastatic squamous cell carcinoma is the commonest underlying diagnosis that must be excluded, but other diseases or even normal anatomy of the neck can be associated with lumps that are hard or bony. Many of these presentations are relatively rare and may not be familiar to oral and maxillofacial surgeons (OMFS) (particularly more junior clinicians) as a differential diagnosis of a hard neck mass. We have reviewed these lesions to raise awareness of possible unusual causes, particularly when patients are not initially examined in a specialist neck lump clinic where ultrasound is readily available. © 2017 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved. Keywords: Neck mass; hard; calcification; review; bone

Introduction A lump in the neck is a common presentation both in general practice, and to departments of oral and maxillofacial surgery (OMFS) and ear, nose, and throat (ENT). Since cancer of the head and neck is the main diagnosis that requires exclusion, guidelines from the National Institute for Health and Care Excellence (NICE) recommend an urgent two-week referral to a specialty service for patients with a persistent, unexplained lump.1 A hard lump is always worrying to both the patient and clinician,2 as it may be the only presentation of an asymptomatic cancer of the aerodigestive tract, particularly when on the left side.3 Other conditions or aberrant bony anatomy may present with a hard neck mass, but



Corresponding author. Tel.: +44 2392 286736; fax: +44 2392 286089. E-mail address: [email protected] (P.A. Brennan).

they are uncommon and therefore may be overlooked in the differential diagnosis. We review these conditions and highlight their clinical features to improve the awareness of colleagues, particularly junior trainees.

Bone-related masses in the neck The neck is demarcated by bony boundaries: superiorly from just above the lower margin of the mandible and base of the skull, and inferiorly by the clavicle and sternal notch. Together with the cervical spine and the hyoid bone, any part of this framework can be the origin of a hard bony mass on palpation. Other lesions, including inflammatory, neoplastic, or degenerative processes, may overlie prominent or aberrant bony structures.

http://dx.doi.org/10.1016/j.bjoms.2017.06.008 0266-4356/© 2017 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.

Please cite this article in press as: Ammar MI, et al. Hard neck lumps: a review of uncommon and sometimes overlooked causes of these worrying presentations. Br J Oral Maxillofac Surg (2017), http://dx.doi.org/10.1016/j.bjoms.2017.06.008

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Fig. 2. Plain radiograph of cervical spine showing prominent right cervical rib. Fig. 1. Coronal computed tomogram showing elongated styloid process in a patient who presented with neck and throat pain.

The styloid process is pinnacle-shaped, cylindrical, and bony. It projects from the lower surface of the temporal bone and tapers downwards, forwards, and medially towards the lateral pharyngeal wall against the tonsillar fossa. In about 4% of the population it is elongated (more than 30 mm long). However, only 4%–28% of them will have symptoms of Eagle syndrome, which are caused by compression of nervous structures by the elongated process, or by ossification of the stylohyoid ligament.4 Eagle syndrome is most common in middle-aged women in whom the tip of the styloid process or the ossified stylohyoid ligament can be palpated as a hard, tender lump in the area opposite to the tonsillar fossa and down to the hyoid bone.5 Elongation of the process can also be asymptomatic. In both situations imaging, including orthopantography (OPG) and computed tomography (CT) to delineate any extension (particularly when symptomatic) can confirm the diagnosis (Fig. 1).5 A cervical rib is a supernumerary rib that arises from the seventh cervical vertebra (C7) with variable extension from just beyond the transverse process of C7 to complete fusion with the first rib. Only about 10% of people with a cervical rib complain of manifestations of thoracic outlet syndrome.2 However, after weight loss it is common for a cervical rib to present with a hard lump in the lower part of the anterior triangle or the supraclavicular fossa, which will prompt a request for ultrasound-guided fine needle aspiration cytology (FNAC).2,6 Clinicians should be aware of this normal variant, as simple tests can unmask both neurological and vascular manifestations that are associated with undiagnosed thoracic outlet syndrome. Chest radiograph (CXR) or CT can readily confirm the presence of an ossified cervical rib (Fig. 2),6 but as unossified ribs are not shown clearly, particularly on CXR, magnetic resonance imaging (MRI) is recommended. Transverse mega-apophysis is a lateral elongation of the transverse process of a vertebra, most commonly at C7, which can be unilateral or bilateral, and present as an asymptomatic bony, hard neck lump or with thoracic outlet syndrome.

Fig. 3. Transverse mega-apophysis (arrowed) at C7.

Fig. 4. Orthopantogram showing an osteoma at the right angle of the mandible.

Absence of a costocervical articulation on plain film radiographs differentiates between a cervical rib and transverse apophysomegaly (Fig. 3).7 Benign bony tumours Osteomas that arise from the mandible or clavicle may present in the neck as slowly growing, stony, hard, painless, smooth masses. They characteristically move with movement of the bone of origin and, depending on their size and anatomical location, may cause compressive symptoms. Peripheral osteoma of the mandible can present as a hard mass on the lower border of the mandible (Fig. 4),8 in the submandibular

Please cite this article in press as: Ammar MI, et al. Hard neck lumps: a review of uncommon and sometimes overlooked causes of these worrying presentations. Br J Oral Maxillofac Surg (2017), http://dx.doi.org/10.1016/j.bjoms.2017.06.008

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postmenopausal women and labourers being at highest risk. Abduction or flexion of the arm above 90◦ typically causes pain, but the only symptom may be a non-tender swelling with medial clavicular osteophytes. Crepitus over the joint can often be palpated when the shoulder is moved.14 Similarly, cervical osteophytes caused by cervical spondylosis, ankylosing spondylitis, or diffuse idiopathic skeletal hyperostosis, may be large enough to cause a bulge on the surface of the neck or the upper aerodigestive tract and present as a slowly-growing, non-tender, hard mass. They are found most often in older patients, and may be associated with long-term complaints of neck pain, stiffness, limited movement, or other symptoms related to compression of other structures by large osteophytes. Calcification that presents as hard masses in the neck Dystrophic calcification results in progressive deposition of calcium phosphate in non-viable or degenerate tissues secondary to changes in their microenvironment, often with normal serum calcium concentrations. Many pathological processes in the neck can result in calcification and present later with a hard mass. Fig. 5. Osteoarthritis of the right sternoclavicular joint, presenting as a neck lump.

and submental triangles, or even in the parapharyngeal space, but it rarely causes dysphagia, dyspnoea, or obstructive sleep apnoea. These patients should be assessed to exclude Gardner syndrome, particularly when there are multiple neoplasms, as this may help with the early detection of intestinal lesions that require intervention.9 Osteomas of the clavicle are rare and may be associated with pain and tenderness.10 Single case reports of those arising from the hyoid bone and stylohyoid complex have been published.11 Chondromas are uncommon, potentially hard swellings in the head and neck that may be multiple. They arise mainly in the larynx and cause endolaryngeal swelling. They can also present with a hard swelling that arises from the laryngeal framework or the hyoid bone.12 Osteosarcomas and chondrosarcomas are rare in the neck and may manifest as firm-to-hard swellings. This applies particularly to the osteoblastic variants of osteosarcoma and calcified chondrosarcoma. While the osteoblastic type of osteosarcoma is commonest overall, chondroblastic osteosarcoma is most common in elderly patients.13 Degenerative disease Degenerative disorders of the sternoclavicular joint (most commonly osteoarthritis) can give rise to a hard mass in the root of the neck (Fig. 5). This is estimated to occur in more than half of patients over 60 years of age, with

Calcification of lymph nodes Calcification of the cervical lymph nodes is rare. It affects only about 1% of patients with cervical lymphadenopathy, and may cause a node to feel hard.15 A study of 2300 neck CT found that 62% of calcified lymph nodes were secondary to cancer that had either been treated (for example, after radiotherapy) or had not. It concluded that a calcified lymph node should raise the suspicion of malignancy and be investigated accordingly.16 Papillary thyroid carcinoma is the most common neoplastic condition that underlies de novo nodal calcification, but other thyroid malignancies – squamous cell carcinoma or other rare metastases from non-cervical primaries – are other potential causes.16 Calcification can also present in nonviable nodal tissue after non-surgical treatment of metastatic squamous cell carcinoma or Hodgkin lymphoma.15 Calcification of cervical lymph nodes after caseous necrosis in tuberculous lymphadenitis is well known but less likely to present in the neck than in the chest and abdomen (Fig. 6). Calcified cervical lymph nodes may also occur with sarcoidosis and amyloidosis.16 Calcification in the thyroid gland The thyroid gland is a common site of dystrophic calcification, which can occur with both benign and malignant disease, and may present as a hard mass in a long-standing multinodular goitre. Calcification within cysts or other benign lesions in the gland, which are most commonly coarse and dense, can be seen on ultrasound. After ablation with radioactive

Please cite this article in press as: Ammar MI, et al. Hard neck lumps: a review of uncommon and sometimes overlooked causes of these worrying presentations. Br J Oral Maxillofac Surg (2017), http://dx.doi.org/10.1016/j.bjoms.2017.06.008

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most commonly as a reaction to trauma, but it can also start after infection or muscular atrophy, or because of an underlying genetic disorder. The posterior neck is an uncommon site of traumatic myositis, which commonly presents in adolescence with a well-circumscribed, usually solitary, stony, hard mass, and often in patients with a bleeding or coagulation disorder. A useful clue for diagnosis is to enquire about any trauma to the neck that may have been trivial enough to pass unnoticed, repeated trauma, violent exercise, or operation.22 Fig. 6. Orthopantogram showing calcified lymph node, which presented as a hard neck lump.

Fig. 7. Coronal computed tomogram showing bridge of calcification on the inferior aspect of a fibular flap that had been used for mandibular reconstruction.

iodine, thyroid tissue may also present with asymptomatic calcification.17 While many authors describe the pattern of calcification as a predictor of malignancy, others argue that a pattern of nodularity, together with calcification, can be more suggestive of the nature of the underlying lesion. A retrospective analysis of 462 thyroidectomies concluded that calcification in a long-standing multinodular goitre is common and associated with a low risk of malignancy. On the other hand, in a solitary thyroid nodule it is more likely to be malignant.18

Hard thyroid masses Rare inflammatory conditions that involve the thyroid gland may present with a hard thyroid swelling. These include Riedel thyroiditis, tuberculosis, sarcoidosis, and plasma cell granuloma. Riedel thyroiditis or invasive fibrous thyroiditis is a rare inflammatory condition that affects middle-aged women and usually presents with a hard, diffuse thyroid swelling that is associated with pain, compressive symptoms, or paralysis of the vocal cords, with or without hypothyroidism. Thyroid tuberculosis, which is rare with fewer than 200 cases reported in English publications, is generally described as a soft, tender thyroid swelling, but can also present as a hard goitre.23 Anaplastic thyroid carcinoma accounts for about 2% of thyroid malignancies and predominantly affects women in their sixth or seventh decade. Unlike differentiated thyroid cancers, anaplastic thyroid carcinoma is highly aggressive and usually presents as a rapidly-growing, hard, fixed, anterior neck mass that is associated with compressive symptoms.24 The thyroid gland may also be a site of direct extension of advanced laryngeal tumours and metastases from distant primaries, which may be hard in consistency. Metastasis to the thyroid gland is rare (between 1.4% and 3% of all thyroid malignancies) from primary sites including kidney, lung, head and neck, breast, and metastatic chondrosarcoma.25

Calcification in other soft tissues Investigation of hard lumps Around 40% of venous malformations are found in the head and neck with a high incidence of associated phleboliths, which may develop after thrombosis. They may present as a calcified, hard swelling in the neck that may be painful or inflamed, and may be the first presentation of a previously undiagnosed vascular malformation.19 Calcification in the neck after reconstructive surgery has also been reported to present with a hard neck mass. Subcutaneous calcification of a pectoralis major flap was thought to be a long-term complication of radiotherapy.20 Late calcification of the pedicles of free flaps or other parts of bony free flaps may be symptomatic enough to justify excision (Fig. 7).21 Myositis ossificans is a pathological process in which new mature bone forms heretopically in skeletal muscle fibres,

Most patients with a hard mass in the neck will present to a neck lump clinic and have ultrasound as a first-line investigation. In many cases this will confirm the diagnosis, but plain films may be required to show bony structures and normal variants, and to reassure the patient. Sometimes – for example, in patients with symptoms from a calcified styloid process, CT may be required, and other investigations can be arranged as clinically or radiologically indicated. MRI is particularly useful for the assessment of unossified cervical ribs. For a patient who does not present to a lump clinic, both ultrasound and plain radiographs, including an OPG when appropriate, might help with the diagnosis. FNAC should not be done in cases of hard bony lumps, as this can be painful

Please cite this article in press as: Ammar MI, et al. Hard neck lumps: a review of uncommon and sometimes overlooked causes of these worrying presentations. Br J Oral Maxillofac Surg (2017), http://dx.doi.org/10.1016/j.bjoms.2017.06.008

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and is not likely to be adequate or to contribute to the diagnosis. Further information about imaging guidelines is readily available from the Royal College of Radiologists.26 Conflict of interest We have no conflicts of interest. Financial support None. Ethics statement/confirmation of patient permission Permission of all patients received. References 1. National Institute for Health and Care Excellence. Suspected cancer: recognition and referral. Full guideline (NG12), June 2015. Available from URL: https://www.nice.org.uk/guidance/NG12. 2. Leong SC, Karkos PD. A “hard” neck lump. Singapore Med J 2009;50:e141–2. 3. Barakat M, Flood LM, Oswal VH, et al. The management of a neck mass: presenting feature of an asymptomatic head and neck primary malignancy? Ann R Coll Surg Engl 1987;69:181–4. 4. Baba A, Okuyama Y, Ojiri H, et al. Eagle syndrome. Clin Case Rep 2017;5:201–2. 5. Sowmya GV, Singh MP, Manjunatha BS, et al. A case of unilateral atypical orofacial pain with Eagle’s syndrome. J Cancer Res Ther 2016;12:1323. 6. Buyukkaya A, Buyukkaya R, Ozel MA, et al. Cervical rib mimicking supraclavicular mass. Joint Bone Spine 2015;82:464. 7. Pepper T, Singh M, Brennan PA. The transverse mega-apophysis—an unusual neck lump. Br J Oral Maxillofac Surg 2010;48:201–2. 8. Manjunatha BS, Das N, Sutariya R, et al. Peripheral osteoma of the body of mandible. BMJ Case Rep 2013;2013.

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9. Brucoli M, Giarda M, Benech A. Gardner syndrome: presurgical planning and surgical management of craniomaxillofacial osteomas. J Craniofac Surg 2011;22:946–8. 10. Shaffrey CI, Moskal JT, Shaffrey ME. Osteoid osteoma of the clavicle. J Shoulder Elbow Surg 1997;6:396–9. 11. Hagiwara A, Nagai N, Ogawa Y, et al. Osteoma of the pharynx that developed from the hyoid bone. Case Rep Otolaryngol 2014;2014:732096. 12. Temsamani H, Mouhsine A, Benchafai I, et al. Bilateral extraskeletal chondroma of the neck. Eur Ann Otorhinolaryngol Head Neck Dis 2016;133:295–6. 13. Tudor-Green B, Fonseca FP, Gomez RS, et al. Current update on the diagnosis and management of head and neck hard tissue sarcomas. J Oral Pathol Med 2017 (Epub ahead of print). 14. Robinson CM, Jenkins PJ, Markham PE, et al. Disorders of the sternoclavicular joint. J Bone Joint Surg Br 2008;90:685–96. 15. Keberle M, Robinson S. Physiologic and pathologic calcifications and ossifications in the face and neck. Eur Radiol 2007;17:2103–11. 16. Gormly K, Glastonbury CM. Calcified nodal metastasis from squamous cell carcinoma of the head and neck. Australas Radiol 2004;48:240–2. 17. Kim JH, Park JH, Kim SY, et al. Symptomatic calcification of a thyroid lobe and surrounding tissue after radioactive iodine treatment to ablate the lobe. Thyroid 2011;21:203–5. 18. Khoo ML, Asa SL, Witterick IJ, et al. Thyroid calcification and its association with thyroid carcinoma. Head Neck 2002;24:651–5. 19. Ahmed T, Moonis G, Loevner L, et al. Venous malformation of the neck with giant phleboliths. Ear Nose Throat J 2008;87:E1. 20. Plzak J, Kalitova P, Urbanova M, et al. Subcutaneous calcification in the pectoralis major flap: a late complication of radiotherapy. Br J Radiol 2011;84:e221–3. 21. Myon L, Ferri J, Genty M, et al. Consequences of bony free flap’s pedicle calcification after jaw reconstruction. J Craniofac Surg 2012;23:872–7. 22. Onoda N, Yamazoe S, Tanaka T, et al. Myositis ossificans associated with subclinical idiopathic thrombocytopenic purpura: report of a case. Int J Surg Pathol 2011;19:207–11. 23. Das SK, Bairagya TD, Bhattacharya S, et al. Tuberculosis of the thyroid gland. Indian J Lepr 2012;84:151–4. 24. Taccaliti A, Silvetti F, Palmonella G, et al. Anaplastic thyroid carcinoma. Front Endocrinol (Lausanne) 2012;3:84. 25. Hegerova L, Griebeler ML, Reynolds JP, et al. Metastasis to the thyroid gland: report of a large series from the Mayo Clinic. Am J Clin Oncol 2015;38:338–42. 26. iRefer guidelines: making the best use of clinical radiology (version 7.0.2). London: Royal College of Radiologists, January 2012.

Please cite this article in press as: Ammar MI, et al. Hard neck lumps: a review of uncommon and sometimes overlooked causes of these worrying presentations. Br J Oral Maxillofac Surg (2017), http://dx.doi.org/10.1016/j.bjoms.2017.06.008

Hard neck lumps: a review of uncommon and sometimes overlooked causes of these worrying presentations.

While a neck lump is a common presentation that can raise suspicion of a potentially serious underlying disease, a hard lump, though less common, may ...
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