------------------------------------------------------------------------------- TITLE: DIAGNOSIS AND MANAGEMENT OF PEDIATRIC NECK MASSES SOURCE: Dept. of Otolaryngology, UTMB, Grand Rounds DATE: March 3, 1993 RESIDENT PHYSICIAN: Eric W. Bridges, M.D. FACULTY: Amy R. Coffey, M.D. DATABASE ADMINISTRATOR: Melinda McCracken, M.S. ------------------------------------------------------------------------------- "This material was prepared by resident physicians in partial fulfillment of educational requirements established for the Postgraduate Training Program of the UTMB Department of Otolaryngology/Head and Neck Surgery and was not intended for clinical use in its present form. It was prepared for the purpose of stimulating group discussion in a conference setting. No warranties, either express or implied, are made with respect to its accuracy, completeness, or timeliness. The material does not necessarily reflect the current or past opinions of members of the UTMB faculty and should not be used for purposes of diagnosis or treatment without consulting appropriate literature sources and informed professional opinion." I. Introduction: *Neck masses very common in children, almost all children have palpable adenopathy during some point in life *In the general population, children with neck masses may have a 2% incidence of malignancy, whereas those admitted for workup have about 15% incidence of malignancy *Multitude of causes for neck masses but most can be categorized as either congenital, inflammatory, or neoplastic *Suspicion of malignancy requires systematic, thorough evaluation to exclude or make diagnosis II. Embryology *Branchial arches give rise to heal and neck structures with disordered development sometimes leading to neck masses A. 1st branchial groove and pharyngeal pouch - external auditory canal and tympanic cavity and eustachian tube B. 2nd branchial groove and pharyngeal pouch - groove obliterated, palatine tonsils C. 3rd branchial groove and pharyngeal pouch - groove obliterated, inferior parathyroids, thymus D. 4th branchial groove and pharyngeal pouch - groove obliterates, superior parathyroids, ultimo branchial bodies E. Thyroid gland - midline endoderm of 2nd branchial arch area III. Diagnosis A. History *Establish onset and duration of symptoms and involvement of other organ systems, i.e., local disease vs. systemic disease *Obtain history of recent upper aerodigestive tract infection to explain cervical adenopathy *Constitutional symptoms of weight loss, fatigue, night sweats suggest malignancy *Exposure to less common infections agents - tuberculosis, cats, travel out of the country, birth outside U.S., zoonoses *Risks factors for HIV or syphilis in parents of newborn or in sexually active adolescents *Causative agents such as ionizing radiation predisposing to thyroid cancer *Pain associated with eating suggests salivary gland disease *Trauma in area can give rise to fibromatosis, hematoma with fibrosis, A-J fistula *Use of Dilantin can cause cervical adenopathy resembling neoplasm *Rate of growth of lesion B. Physical Examination A. Inspection *Establish rapport with child *Look for asymmetry in the neck, scars, skin discoloration, abnormal pulsations *Movement of mass with swallowing, tongue protrusion, Valsalva *Fistula openings B. Palpation *Hands clean, dry, warm *Establish mobility of mass, fixation to overlying skin or underlying deep structures *Establish whether mass is diffuse or localized,regular or irregular *Establish consistency - lipomas are soft, benign lymph nodes maybe rubbery and mobile,malignancies are hard or woody, cavernous hemangiomas may feel like a "bag of worms" due to phleboliths in the mass, laryngocele may be crepitant *Establish tenderness, usually associated with inflammatory process *Pulsation suggests aneurysm, A-V fistula, carotid body tumor *Palpate cervical organs (SCM muscles, thyroid vessels, salivary glands, larynx/trachea,lymph nodes) *Palpate salivary glands using bimanual technique, express pus from glands, identify stones in duct *Auscultation of the neck can help localize site of airway obstruction, may disclose bruit over thyroid in toxic goiter C. Laboratory *In a neck mass of undetermined etiology, routine blood work is indicated (CBC,liver, renal profile, sedimentation rate) *Serology for various viral, parasitic, venereal diseases -Toxoplasmosis, RPR, HIV, CMV, EBV, histoplasmosis, coccidioidomycosis *Skin testing can be done for mycobacterial disease, cat- scratch disease *Neck films are helpful if the lesion is bony or causing bony erosion or retropharyngeal abscess *Chest films should be obtained to look for associated mediastinal adenopathy *CT scan accurately defines extent of lesions, presence of abscess vs.cellulitis *U.S. shows cystic vs. solid nature of masses, less useful in inexperienced hands or when neck tender *M.R.I. probably imaging modality choice with rhabdomyosarcoma, other soft tissue sarcomas *Thyroid imaging necessary when evaluating for resection of presumed thyroglossal duct cyst, must establish other functioning thyroid tissue separate from presumed TGDC *Arteriography helps diagnosis vascular lesions, delineates vascular supply of tumors, allows pre- operative embolization prior to planned resection D. Five Needle Aspiration Biopsy *Can be performed under local anesthesia as an outpatient *Should be performed in any solitary, non-tender mass greater than 3 cm with uncertain etiology *Can aid in infections/inflammatory conditions by obtaining material for gram stain and culture *Sensitivity and specificity are generally greater than 90%, approaching 100% at some institutions IV. Differential Diagnosis *Large series at Children's Hospital of Philadelphia over January 1982 to December 1986 reviewed 529 biopsies of neck masses in 493 children, 455 children had complete records *Congenital lesions were 55% of the total malignant lesions were 11% of all neck masses *Figure lists all lesions diagnosed by biopsy A. Differential Diagnosis by site *Eleven different areas of neck shown in figure, common lesions found in each region are listed *Various infectious agents shown in Table V. Cystic hygroma *Congenital mass *Sometimes unilocular, often multilocular endothelial- lined, fluid-filled mass of lymphatic origin *Spectrum of lymphangioma from superficial lymphangioma to diffuse systemic lymphangioma *90% diagnosed before 3rd birthday *Benign lesions,complete resection curative; however, neurovascular structures are not sacrificed Incomplete resection if necessary *Radiotherapy may be beneficial for multiply recurrent disease *Sclerotherapy diathermy not indicated *Surgery when obstructive symptoms or severe cosmetic deformities VI. Branchial Cleft Cyst A. 1st arch cyst *Two types - type I localized medial, inferior, or posterior to conchal cartilage and pinna. Sinus tract parallels to EAC - type II cyst or sinus tract terminates near bony cartilaginous junction of EAC, courses down over mandible toward hyoid. May be intimately involved with facial nerve *Surgery for cysts because most become infected at some point in time B. 2nd arch cyst *Comprise over 90% *Pharyngeal opening into tonsillar fossa *If fistula tract, opens along anterior border of SCM *Tract courses superficial to hypoglossal nerve, between internal and external carotid *Allow inflammation to subside before operating C. 3rd arch cyst *Tract courses over hypoglossal nerve, behind/deep to both carotid vessels, pierces thyrohyoid membrane to join pyriform sinus D. 4th arch cyst *Runs through larynx/cricoid cartilage *May cause recurrent suppurative thyroiditis *Ipsilateral thyroid resected with lesion E. Surgical techniques *Injection of fistula tracts with methylene blue or quick-hardening polymers. Embolectomy catheter allows fixation of catheter by inflating balloon *Complete excision of 2nd arch anomaly may require tonsillectomy for closure of internal tract *If recurrent thyroiditis, approach as standard thyroid VII. Thyroglossal Duct Cyst *Resect completely with removal of central part of hyoid bone *Recurrence rate`increased with cyst rupture, caused by previous inflammation, prior I & D, fistulous connections to skin *Resect skin around sinus opening *Operate before infection VIII. Dermoid Cyst *Often confused with TGDC *May demonstrate attachment thyroid *Resect hyoid (Sistrunk) if any doubt about lesion IX. Laryngocele *Asymptomatic require no treatment *May become infected *If no communication with lumen, then termed a saccular cyst *Internal laryngocele may cause airway comprise X. Rhabdomyosarcoma *Most common solid tumor in head and neck in children *Symptoms related to tumor site, may mimic persistent sinusitis, adenoiditis, otitis externa *Rapidly growing, most present for less than a month before diagnosis *Complete resection if possible, usually not possible with gross deforming and functional loss *Chemotherapy with radiation and/or surgical salvage has cure rates of approximately 50-65% XI. Lymphoma *Most common head and neck malignancy in children *Non-Hodgkin's lymphoma more common than Hodgkin's disease overall but Hodgkin's disease more common in head and neck, therefore, about equal incidence in head and neck region *1/3 of patients with supraclavicular masses had lymphoma and 1/3 of patients with lymphoma had supraclavicular masses *Oncologic consultation prior to biopsy to arrange intra- operative bone marrow biopsy XII.Mycobacterial Infection *Need to differentiate non-tuberculous lymphadenitis from M. tuberculosis *Excision of tuberculous lymph nodes may result in persistent draining sinuses *PPD strongly positive with tuberculosis, equivocal with atypical mycobacterial infection *Atypical infection in parotid/submandibular region, tuberculous infection in lower neck *CXR abnormal in M tuberculosis *May curettage atypical mycobacterial infection *Medication for M. tuberculosis ---------------------------------------------------------------------------- BIBLIOGRAPHY Bisceglia, M, Amini, M, Bosma, C. 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