A lump in the neck may be the early manifestation of a host of pediatric conditions.1,2,3 The majority will prove to be inflammatory lymph nodes, while few of the remaining lesions can be said to be truly common. Rarely will the pediatrician or general practitioner have broad enough experience to feel at ease with diagnosing the full range of mass lesions that may present in the neck.
Nevertheless, most presentations are typical of the lesions they represent and with an appropriate history, physical examination, and an orderly approach, a reliable diagnosis can usually be made. Most neck masses are relatively innocent and common, while some are extraordinarily rare4 or atypical and no amount of clinical calisthenics short of excisional biopsy will provide an answer. Such is the nature of pediatric neck masses.
Many classifications of neck masses have been developed but a consideration of lesions presenting in a particular anatomic area is perhaps the most workable. The anatomy of the neck is compact and complex and an appreciation of the significant structures in an area is the key to a ready diagnosis. Anatomic minutiae are of little importance in this exercise.
It is also useful to divide these masses into two broad categories: cystic lesions, which consist mainly of pharyngeal cleft remnants and vascular malformations; and solid lesions, which are generally inflammatory or neoplastic. This distinction can usually be made clinically but ultrasound5 imaging is a quick, easy, noninvasive corroborating modality.
LATERAL NECK LUMPS
The lateral aspects of the neck contain a great diversity of anatomical structures and constitute the bulk of the pathologically significant neck volume. The bulk of disease, therefore, presents in this area.
For convenience, it is helpful to divide the lateral aspect of the neck into a superior and inferior region. The superior part is the home of the larger proportion of masses, most of which usually have a straightforward etiology. Lumps and humps in the inferior neck are not only less common, hut frequently obscure and often ominous.
Although lymphadenopathy6 may be present virtually anywhere in the neck in association with an inflammatory or neoplastic process, glandular involvement most frequently presents in the jugulodigastric area and somewhat less frequently in the submandibular triangle anteriorly or the posterior-triangle and sternomastoid area posteriorly.
An acute lymphadenitis presenting in these areas with marked tenderness, torticollis, trismus and dysphagia, and associated systemic signs of infection is seldom a diagnostic challenge to the clinician. These are almost always the result of a primary bacterial or viral infection, or secondary to pharyngotonsillitis or an inflammation in a related area of the upper aerodigestive tract. They respond readily to treatment. The less dramatic and particularly the asymptomatic adenopathies can be more difficult to assess and are often more disconcerting.
Infectious mononucleosis may present in the child with rapidly progressive massive cervical lymphadenopathy. Although the child may complain of local pain, tenderness is minimal and torticollis and trismus often absent. Dysphagia and obstructive airway complaints may be present but these are due to associated hypertrophy of adenoids and lingual and faucial tonsils. The tonsils and adenoids are covered with white necrotic debris but with little surrounding edema or redness as would be apparent in a bacterial tonsillitis. Petechiae may be present on the soft palate, and the presence of more generalized adenopathy and hepatosplenomegaly will support the diagnosis. Seroconversion may be late in this age group but a compatible peripheral blood smear is helpful. Should an apparently infective acute lymphadenopathy tail to settle on antibiotics or be associated with persistent fever, stomatitis, conjunctivitis, desquamation of the hands and feet, and/or a polymorphous exanthem, Kawasaki's disease should be considered and prompt management instituted.
Granulomatous infections usually develop over weeks and months, often with minimal systemic complaints or findings. These glands tend to be firm, with some degree of fixation and injection of the overlying skin. These glands may suppurate and drain (or be drained) only to reform. Tuberculosis is now very rarely seen in our population, but cat-scratch fever and atypical myobacterial infections may present in this way. Diagnosis is frequently uncertain until a biopsy or aspirate is obtained.
Progressive diffuse adenopathy, particularly with associated parotitis, should alert the clinician to the possibility ot toxoplasmosis or, in an at-risk infant, AIDS or AIDS related complex. Similarly, the disseminated form of histiocytosis with skeletal and visceral involvement may also be associated with generalized lymph node involvement in infancy.
Figure 1. This slowly progressive asymptomatic single mass of nodes, deep to the sternomastoid muscle, and in the midportion of the neck was found to be a Hodgkin's lymphoma.
Progressively enlarging firm to hard upper cervical lymph nodes with few or no overt systemic complaints are more ominous, particularly if any of the nodes are: 1) over 2.0 cm in diameter, 2) fixed to adjacent tissue, or 3) located in the posterior triangle or in some atypical place such as deep to the sternomastoid muscle or in the midportion of the neck (Figure 1). Multiple seemingly unrelated areas of lymphadenopathy (Figure 2) are similarly worrisome. The concern of course is that these glands are lymphomatous or occasionally, even in the pediatric age group, represent a secondary squamous cell carcinoma from the nasopharynx, the so-called lymphoepithelioma. If a diagnosis is not confirmed in the upper airway, then peripheral smear, bone marrow aspirate, chest x-ray, or prompt local biopsy is clearly indicated.
Frequently, one is faced with the healthy but perhaps thin child who presents with one or two jugulodigastric lymph nodes, which may be up to 1.5 cm or 2.0 cm in diameter. So often these nodes have been present for many months, fluctuate in size, are nontender, mobile, not fixed superficially, or deep. These children are systemically well, with no other significant clinical findings. Routine blood screening is normal. The concern of a possible neoplasm is natural, but this presentation is almost always innocent and biopsy is not indicated. Rarely, the above picture will be associated with a 3.0 cm or larger lymph node in the upper neck. This enlargement may simply be reactive, but it is the rare clinician who can refrain from obtaining a biopsy from such a lesion. Such a decision cannot be faulted.
Figure 2. Multiple regions of the neck were involved with nonHodgkin's lymphoma otherwise asymptomatic at the time of diagnosis.
Cystic lesions of this area are for practical purposes of two types, the cystic hygroma and second pharyngeal cleft remnants. The classical, large, multiloculated lesion is obviously cystic hygroma of the neck and may reach prodigious proportions. The diagnosis is almost impossible to miss, and complete removal of the lesion by surgery or other means is almost equally impossible. Smaller lesions may be somewhat more difficult to diagnose but ultrasonography is invaluable, although not pathognomonic. Occasionally, cystic hygroma may present as "a sudden lesion," literally over a few hours or days, around 2 to 3 years of age. The sudden appearance is presumably due to a vascular occlusion, hemorrhage, or infection. The appearance is otherwise the same as those that were apparent at birth.
A teratoma may present as firm, usually irregular lateral neck mass in the newborn. There may be some confusion with a cystic hygroma, but usually the teratoma is more solid and gritty to palpation due to calcified elements. Teratomata may be 5.0 cm or more in diameter and result in life-threatening airway obstruction and feeding disorders. Unlike teratomata elsewhere in the body, or those appearing in older individuals, these lesions are rarely malignant.
Second pharyngeal cleft cysts, although congenital anomalies, rarely present in early childhood. These too may appear suddenly when the child is only a few years of age and may be difficult to distinguish from a small cystic hygroma. However, second pharyngeal cleft cysts are characteristically unilocular and located just along the anterior border of the upper third of the sternomastoid muscle. They may be initially confused with a cervical lymphadenopathy and can become infected.
Progressive, often asymptomatic, growth of a neck mass over weeks to months is characteristic of neoplasia, most often a lymphoma in children. NonHodgkin's lymphoma and rhabdomyosarcoma, however, are characterized by alarmingly rapid growth. Increments may be apparent daily. More slowly growing tumors of fibrous origin or tumor-like lesions such as the various fibromatoses may be suspected but cannot usually be diagnosed reliably without biopsy.
Several neurogenic tumors present as lateral neck masses in children. These lesions are usually multiple and often represent a local manifestation of a systemic disease, ranging from the slowly progressive tumors of von Recklinghausen's disease, with its characteristic cutaneous, bony and CNS involvement, to the rapidly progressive neuroblastoma, which is usually found to be secondary to already established abdominal disease. Neurogenic tumors develop from multiple nerve roots or sympathetic elements and tend to have an irregular and often "ropy" feeling on palpation. The very slow growing neurofibromatosis may go unrecognized by the young patient or the family until it is remarkably large and obstructs air and food passages.
The lower half of the lateral neck may also be involved by most of the lesions mentioned above, but adenopathy in this area is far more likely to be neoplastic (eg, Hodgkin's lymphoma or thyroid carcinoma) than inflammatory. Cystic hygromas are also found in the lateral neck area but exotic cystic lesions, such as a venous aneurysm (Figure 3), thymic cyst, or pneumocele, should also be considered. All three of these rare lesions may only appear when the child is crying or straining, and the latter two may even disappear into the chest at rest. A bruit may be heard over an aneurysm and breath sounds over a pneumocele.
Congenital muscular torticollis or torticollis tumor of the sternomastoid muscle is an uncommon fibromatous process involving the lower third of the sternomastoid muscle. It presents within a few days of birth and may appear to progress over a few weeks. There is a transient shortening of the affected muscle and rotation of the neck to the affected side; thus the name, congenital torticollis. The presentation is classic, and it should not be confused with any other lesion.
Figure 3. This soft, fluctuant lesion in the lower part of the neck was found to be a jugular aneurysm of unknown etiology. The diagnosis was made at attempted (but deferred) biopsy.
THE SUBMANDIBULAR REGION
The primary anatomical structures in this area are lymph nodes and the submandibular salivary gland. These lymph nodes may be involved by any of the preceding inflammatory or neoplastic processes. Occasionally these lymph nodes are the only nodes involved by a granulomatous process such as an atypical mycobacterial infection.
The submandibular gland is the salivary gland most likely to form calculi in children and adults. The history is one of repeated identical swellings, often repeatedly diagnosed as cervical adenitis but without inflammatory or suppurative process. The affected gland swells suddenly, literally over seconds to minutes, as a result of ductal occlusion. The gland is rubbery hard and tender, but inflammation is not evident unless the gland is occluded for several days, at which point it may become secondarily infected. In the early stage, diagnosis is most likely to be confirmed by the presence of a small white calculus impacted into the orifice of Wharton's duct in the floor of the mouth.
A firm solid mass in the submandibular gland, which progressively increases in size is likely to be neoplastic and may well be malignant (eg, mucoepidermoid carcinoma), although most are benign (eg, pleomorphic adenoma). Such a mass, if solitary, is best diagnosed and treated by excisional biopsy. Lesions primarily of mandibular origin, suchas a periostitis, histiocystosis, or osteosarcoma, may present here as well but can readily be distinguished by appropriate radiographic examination.
Significant lymph node enlargement in the midline of the neck is uncommon in children. Occasionally, a submental lymph node may be a presenting sign of oral infection but such inflammation is more likely to be diffuse than limited to a node or two.
The midline is the domain of the thyroid gland and all other pathologies should be secondary considerations. A midline or paramedian solid thyroid mass in the newborn is most likely to be a congenital goiter or a thyroid teratoma.
Although thyroglossal duct remnants may be found anywhere from the base of tongue to the mediastinum, a midline cyst just over the thyroid-hyoid membrane is by far the most common presentation. These lesions may be present at birth but are easily missed in the chubby-chinned infant. They are more likely to be noted in the 3- or 4-year-old and are frequently mistaken for a midline inflamed lymph node. These cysts are characteristically associated with surrounding lymphoid tissue, which reacts to upper respiratory tract infections as do lymph nodes. With regression of the inflammation, the nodule may persist. Because these cysts are tethered to the foramen caecum by the thyroglossal duct remnant, protrusion of the tongue will result in visible and palpable elevation of the cyst. Clinical diagnosis of this lesion can be made with confidence and definitive surgery recommended.
Ultrasonography demonstrates that this lesion is cystic, and thyroid functions demonstrate a normally situated and functional thyroid gland. Occasionally the ectopic thyroid lesion may represent the only functional thyroid tissue present, and although surgery may still be indicated this information is clearly vital. Thyroglossal duct cysts may be found above or below the classic thyrohyoid site and may be multiple (Figure 4), but the management is essentially the same as that for thyroglossal duct remnants.
Thyroid malignancies, although rare in the pediatric age group, do occur and range from relatively low grade papillary carcinomas to highly aggressive medullary types. Any solid nodule on or near the thyroid gland should be considered as potentially malignant. Fine needle aspiration biopsy, ultrasonography, and radionuclide scan should be carried out for evaluation. Further management is dependent on specific diagnosis. Bilateral paratracheal nodes may be involved and may have been noted prior to diagnosis.
Figure 4. Midline lumps are usually thyroid related. Thyroglossal duct cysts are usually single but may be multiple as in this patient.
Figure 5. Repeated sudden swellings of the parotid gland is characteristic of chronic recurrent parotitis of childhood.
A dermoid cyst may also present in the midline as a discrete, smooth, soft lesion much the same as a thyroglossal duct cyst. As they are not tethered to the base of the tongue, they will not elevate with tongue protrusion. Occasionally, clinical distinction cannot be made. If surgical excision is elected, such uncertain lesions should be treated as though they are of thyroid origin rather than as dermoids. The incidence of recurrence and the need for a second surgical procedure is thus minimized.
A number of diverse lesions can present about the external ear, and an appreciation of the key anatomical structures in the area will facilitate clinical diagnosis.
Lymph nodes abound in front of, below, and behind the pinna. A readily palpable, single postauricular lymph node in an otherwise asymptomatic child is not uncommon, but multiple tender nodes are an indication of a viral infection, such as German measles, or a scalp infection, or infestation. An isolated preauricular node in a child may be secondary to facial or periorbital infection.
Infra-auricular nodes are usually multiple and associated with other, often extensive, inflammatory or neoplastic adenopathy. The infra-auricular area is also the site of several cystic lesions. Sebaceous cysts, which are relatively common in young adults and adolescents, are decidedly uncommon in infants and young children. In the younger age group, a cystic lesion in this area should be considered as being of first pharyngeal cleft origin. They may present as smooth, slowly progressive, painless swellings below the ear lobe or as an acute inflammation or even abscess. The presence of an associated pit or dimple in the ear canal or just below the body of the mandible will confirm the diagnosis. These cysts may be intimately related to the facial nerve and may, in part, course deep to the nerve. Failure to recognize the true nature of such a lesion may result in catastrophic facial nerve injury as a result of incision and drainage, simple excision, or even biopsy.
Acute parotitis or mumps is usually readily appreciated by most practitioners. "Recurrent mumps," of course, does not exist, yet children suffering from chronic recurrent parotitis7 are frequently so labeled and repeatedly sent home from school.
Chronic recurrent parotitis (Figure 5) or sialadenitis is a condition of unknown etiology characterized by repeated sudden swelling of one or both parotid glands associated with sialectasia and stasis of secretions. The glands are hard and tender to some degree but will usually decompress with gentle massage. Systemic symptoms are not present although the glands may become secondarily infected. They respond to antibiotics both acutely and prophylactically, and ablative surgery is rarely indicated. Recurrent parotitis tends to regress after months or occasionally years of flare-up. This condition is often much more distressing to the parents, who are certain some ominous condition is being missed, than it is to the patient. Ongoing support and reassurance are the key to management.
Figure 6. The most common tumor of the parotid gland is the capillary hemangioma, which may increase progressively over several years and ultimately involute spontaneously at 5 or 6 years of age.
Primary neoplasms of the parotid gland in children are uncommon and usually benign, but the relative incidence of malignancy is greater than in adults. Any solid, slowly progressive (over weeks or months) lesion of the parotid should therefore be considered as neoplastic and potentially malignant. In the child, such a lesion is best dealt with by excisional biopsy via a superficial parotid lobectomy. This should be therapeutic as well as diagnostic and, in experienced hands, of minimal risk to the facial nerve.
The most common tumors about the parotid region are vascular or lymphatic in origin. The capillary hemangioma (Figure 6) may not be apparent at birth but over the next few months may grow to alarming size. When some visible cutaneous or mucosal component is present, the diagnosis may be readily apparent. A deep capillary hemangioma may be more difficult to diagnose. These lesions are diffuse, soft, compressible, warm and may enlarge if the infant is crying. In spite of their size and appearance these lesions will regress in a few yeaTS as opposed to the arteriovenous malformations composed of larger vessels, which may be relatively progressive and destructive. Ultrasonography, angiography, and even open biopsy may be necessary to distinguish between the two lesions.
The clinical presentation of a neck mass in a child is obviously not always pathognomonic but a careful clinical appraisal can certainly limit the diagnostic options and reduce the need for complex and sometimes invasive investigations.
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