Internationul Journal oj Pediatric ~tor~i~ola~n~o~o~, 16 (1988) 1?9-210 Ekevier

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POR a0553

alph ler l all oasic l ’ Diuision of Otobyngofo~ and ’ Division of General Surgery, CkildretA ff~~~i~af of ~~if~e~~i~

University of ~e~~~l~ia School of Medicine, Phifadebhia, PA (US.A.)

vised version recei

Key worck iatric neck mass;

masses are th few establi cervical masses includes many conditio clinical presentations is essential. To elucidate the c cal ch~act~~stics

Neck masses me ently encountered in t

adults, there are

cal characteristics which may aid in establis diagnosis, the charts of 493 patients with biopsies of neck masses pe

Presented at the American Society of Pediatric Otol gology (ASPO), Kiawah Island, SC, April 21.

31988. Correspondence: L.W.C. Tom, Street and Civic Center Blvd.,

SO 0 1988 Eisetier Science

200

Children’s Hospital of Philadelphia (CHOP) over a S-year period, January 1982 through December 1986, were reviewed. These cases were analyzed retrospectively for patients’ symptoms, physical findings, preoperative evaluation and impression, and postoperative diagnosis.

By reviewing the history and performing a thorough examination with attention to the head and neck, the physician can often establish a correct clinical impression. When they are indicated, appropriate laboratory and radiologic studies may confirm the diagnosis prior to any surgical intervention. In this study, 61% of the preoper- ative diagnoses were correct.

A review of the operative cases, performed at CHOP from January 1, 1982 through December 31, 1986, revealed that 529 biopsies of neck masses were performed on 493 children. Any procedure involving a biopsy or excision of a mass which originated primarily in the neck was included in this study. Both the outpatient and hospital records were examined to categorize the following: patient description, associated symptoms, characteristics of the mass, preoperative evalua- tion, prior treatment, and operative information. This information was analyzed.

esults

During the 5-year period, 529 procedures were performed on neck masses in 493 children. The charts of 48 patients (9%) were incomplete. The remaining underwent 481 operations and are the basis of this study. Four hundred children had their initial procedures at CHOP with 22 requiring more than one operation. The remaining 45 had prior surgery at other hospitals and were referred for further evaluation or treatment.

The patients ranged in age from 1 day to 21 years with a mean age of 6.2 years (Table I). There were 207 females and 238 males.

Congenital lesions were diagnosed in 244 (55%) patients. One hundred eighteen (27%) children had inflammatory lesions. Non-inflammatory benign masses were found in 23 (5%). Benign neoplasms were noted in 12 (3%) patients. Malignant lesions were diagnosed in 48 (11%) children (Table II). Twenty-six patients under- werit multiple procedures for further evaluation or treatment. The preoperative diagnosis was correct in 270 (61%) patients (Table III).

Congenital neck masses Congenital neck masses were diagnosed in 244 patients and generally, were noted

in younger children (Table I). The incidence of bran&al cleft cysts ( thyroglossal duct cysts (TDC) was almost equal, 17% and 16% respectively. Lymphangiomas, dermoid cysts, and hemangiomas were also common.

201

TABEE I

Age distribution

Branchid cleft cyst Thyroglossal duct cyst Dermoid cyst L~~~~~orna Wemangioma

Reactive lymphadenopathy

Granulomatous dii Suppurative lymphadenitis Sialadenitis

Inclusion cyst Fibromatosis

Lymphoma Thyroid carcinoma

Others

Range Auerage

6 months-16 years 3.6 years 9 months-17 years 6.1 years 9 months-15 years 3.7 years 3 months-10 years 3.6 years 1 day-15 years 5.6 years

3 months-18 years 8.0 years 1 year-14 years 6.0 years 4 months-l 5 years 7.3 years

11 years-13 years 11.2 years

3 years-12 years 4.4 years 1 month-10 years 3.1 years

4 years-21 years 11.7 years 8 years-17 years 12.3 years

2 weeks-l8 years .4 years

majority (95%) were m

e masses were soft

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TABLE II

Dis!ribution of lesions

collplital lesions Bran&M cleft cyst ThyrogIossaI duct cyst Dermoid cyst Lymphangioma Hemangioma Taatoma Bronchogeniccyst Thymiccyst MyeIomeningocele

inflammatory lesions Reactive lymphadenopathy Undetermined etiology smus histiocytosis GranuIomatous disease Atypical mycobacteria cat bwatch disease Toxoplasmosis Sarcoid suppurative lymphadenitis SiaIade&is

Non-inflammatory beniga lesions Inclusion cyst Fibromatosis KeIoid

Benien-@-s Neurofibroma Lipoma Lipoblastoma ParagangIioma Goiter Benign mixed tumor Osteoblastoma

MaI&nant neoplasms Lymphoma

Hodgkin’s Non-Hodgkin’s

Thyroid carcinoma Rhabdomyosarcoma NeurobIastoma Fibrous histiocytoma Acinic all carcinoma Histioqtosis x chlolonla

Total

Total number

78 13 43 34 10 2 2 1 1

66 5

32 20 6 2 2

10 5

23 13 9 1

12 3 3 2 1 1 1 1

34 23 11 6 2 2 1 1 1

W of total

56 17 16 10 8 2

27 16

7

2 1 5 3 2

3

11 8

1

203

TABLE III

Preoperative diagnosis

Branchial cleft cyst ?l~yroglossal duct cyst

Hemangiioma

Reactive I~~hadenQ~a~y

tis

Lymphoma

Others

Total

Preoperative diagnmis

89

97 33 37

7

19 9

I.1 8

86

49

5

Correct w

68 76 68 70 18 55 24 65 4 57

13 68 9 10 9 82 4 5

27 31

27 55

271 62

204

were normal. Reactive lymphadenopathy was the preoperative diagnosis in 19 patients. This was correct in 68% (13).

Five children with reactive lymphadenopathy had sinus histiocytosis, and in 4 multiple masses were present. No cases of sinus histiocytosis were diagnosed preoperatively.

Suppurative cervical nodes were found in IO patients. Six masses were larger than 3 cm, and 5 were tender and fluctuant. Needle aspiration was performed in 2 patients. Stuphyiococcus aweus was the most common pathogen identified. Eleven patients were diagnosed as having suppurative adenitis, and this was correct in 9 Cases.

Granulomatous iesions including atypical mycobacteria, cat scratch disease, toxoplasmosis, and sarcoid were diagnosed in 32 patients. Atypical mycobacterial adenitis was the most frequent of these lesions occurring in 20 patients. Five children had associated fevers. These masses were larger than 3 cm in 15 (75%) of these patients and firm in I4 (70%). Diagnostic studies included tuberculin shin tests in 10 children, 5 of which were positive, and chest X-rays in 6 patients, all of which were normal. Nine children were diagnosed as having atypical mycobacterial adenitis preoperatively, and the diagnosis was correct in all cases.

Cat scratch disease, toxoplasmosis, and sarcoid were present in the remaining 12 patients with granulomatous lesions. In all cases, neither the history nor characteris- tics of the mass gave an indication to the final diagnosis. None of these cases were diagnosed preoperatively.

Five children with sialadenitis required surgery. All lesions were described as hard and fixed. Tenderness was present in 4 children. The submandibular gland was the site of involvement in 4 cases. The final diagnosis was correct in 4 of 8 children with this preoperative impression.

Non-inflmtmatoory benign lesions Non-inflammatory benign lesions were present in 23 patients. Five inclusion

cysts were in the midline, and 8 were in the lateral neck. They were most often misdiagnosed as dermoid cysts. The preoperative diagnosis was confirmed in 40%. None of the 9 children with fibromatosis were diagnosed correctly preoperatively. They were most often diagnosed as having rhabdomyosarcomas.

Benign neoplasms Benign neoplasms accounted for 3% (12) of all neck masses. This group included

neural, fatty, thyroid, salivary gland, and osseous neoplasms (Table II). Diagnostic studies were usually ordered to determine the extent of the lesions and included angiography, CT, and magnetic resonance imaging (MRI). The preoperative impres- sion was correct in only one case.

Maiignant neoplasms M t neoplasms were diagnosed in 11% (48) of the patients (Table II). A

preoperative diagnosis of malignancy was suspected in 104 patients, but only 34

TABLE IV

Lyinphcnm data

Q5

erior cervical triangle supracIavicuIar Posterior cervicaI triangle ParOtid

Associated symptoms Fever weight loss Night sweats Wsphagia Respiratory s tom Fatigue ass: characteristics Larger &an 3 c Firm Soft Mobile Fixed

Chest X-ray Abnormal Nod

16 I2 S 1

9 S S 3 3 1

1s 24 10 25

9

11 6

ren were re atients initially treat

TABLE V

Supraclaviculaf ma.Ss

Lymph- 12 Reactivelymphadenopathy 9 LPPhaqoma 3 Inclusion cyst 3 Cat scratch 2 Lipoma 2 Hemangioma 1 !%lus bisti~tis 1 Sarcoid 1

Total 34

chl~roxna. All of these lesions presented as large hard masses. In the cases of the rhabdomyosarcomas and chloroma, multiple biopsies were required to establish the diagnosis.

Neck masses are frequent findings in the pediatric population. The etiology of cervical masses includes congenital masses, inflammatory lesions, non-inflammatory benign lesions, and benign and malignant neoplasms.

Congenital lesions In this series, congenital lesions were the most common sources of cervical

masses and were diagnosed in 55% of the patients. BCC and TDC were the most frequent entities and occurred almost equally ousattos [12] noted similar results in his series of 267 patients. The majority of lesions were correctly diagnosed preoperatively.

BCC were the most accurately diagnosed congenital masses. They usually pre- sented as soft, mobile masses along the anterior border of the stemocleidomastoid muscle and with a history of enlargement with upper respiratory tract infections. When they were associated with sinuses or skin appendages, the preoperative diagnostic accuracy increased from 75% to 94%. In these patients, preoperative studies were used to define the extent of the lesion rather than establishing a diagnosis.

Seventy percent of children with TDC were diagnosed correctly prior to any surgery, and this figure is similar to the 61% of correct preoperative diagnosis in Knight’s series (71. The presence of a firm, mobile mass in the midline of the neck n the hyoid bond makes a TDC likely.

ith TDC, thyroid scans and ultrasounds were the most common preoperative studies. tb of these studies were performed to determine the presence of a normal thyroid gland and to insure that the neck mass was not the patient’s only thyroid

287

aids were believ

en are c-au

secondary to viral or bacterial u

208

made in 25%, and Sinclair [17] recommends repeating biopsies when there are clinical indications.

reviewing the preoperative evaluations of these patients with cervical adenopa- thy revealed that a diverse number of laboratory and radiologic studies were ordered. This best exemplifies a lack of diagnostic direction when evaluating these Children.

Suppurative lymph nodes were biopsied in 2% of the patients, but this does not reflect the true incidence of suppurative cervical lymphadenopathy. Suppurative nodes may be treated with antibiotics, aspirated, and/or drained prior to any biopsy [8,18]. These are common procedures at C P and may avoid excision of an infected mass.

Atypical mycobacterial cervical adenitis is not an uncommon cause of a cervical mass and accounts for 4% of the present series. It is characterized by chronic unilateral cetvical adenitis frequently involving the submandibular nodes. Chest X-rays are usually normal. Tuberculin skin tests are ncommended for patients suspected of having atypical mycoba&erial adenitis. Excision of the nodes is the definitive treatment because the atypical strains are resistant to most antitubercu- lous drugs 1131.

Although the other inflammatory lesions are usually not diagnosed preoper- atively, they may be suspected on clinical grounds. This impression is confirmed by appropriate laboratory tests.

Non-inflammatory benign lesions and benign neoplasms Both these groups of lesions make up a small minority of neck masses and are

rarely diagnosed preoperatively. They are important because they are usually mistaken for malignant lesions. In most cases, biopsies are required to establish the diagnosis.

Malignant neophns Failure to diagnose a malignancy remains a concern when evahtating children

with neck masses. Biopsies are often performed to rule out a mahgnancy. In the present study, surgery was performed on 104 patients with a suspected malignancy. This impression was correct in 34 (33%) of these patients. In addition, 14 (29%) of the malignant tumors were not suspected prior to a biopsy.

With the exception of primary i.ntracraniaI neophtsms, mahgnant lesions of the head and neck are not common in the pediatric population [16]. In a lo-year study, Jaffe [S] reported 178 head and neck malignancies, and 241 malignant neoplasms were identified in a 20-year study from Pittsburgh 121.

The most common presentation of a head and neck malignancy in children is an asymptomatic neck mass [2]. Despite the relatively high frequency of other lesions, Cunningham [2] believes that a firm, non-tender neck mass in a child should be considered a malignancy until proven otherwise. All of these children require thorough otolaryngologic and systemic evaluations. Jaffe [5] noted that one out of every 6 chiklren with a mahgnant neck mass had a primary or associated lesion in the oral cavity or pharynx. :

ese tumors u

roid carcinoma was the second most ties have accounted for 50% of

common neck ma the neck either as

nitial biopsies were not

if the clinical situation warrants, a repeat biopsy should be performed.

erences

1 Bamji, M., Stone, R., Kad, A., Usmani, G., Schachter, F. and Wasserman, E., Paipabk !pph n

2 tumors of the head and n ren: a

210

3 DeMello, D., Lima, J. and Liapas, H., midline cervicai cysts in children, Arch. Dtol. Head Neck surg., 113 (1987) 418-420.

4 Herzo& L., prevalence of lymphadenopathy of the head and neck in chikhen, CIin. Pediatr. (Bologna), 22 (1983) 485-487.

5 Jaffe, B., Pediatric head and neck tumors: a study of 178 cases, Laryngoscope. 83 (1973) 1644-1651. 6 Kissane, J. and Gephardt, G., Lymphadenopathy in childhood, Human Pathol., !I (1974) 431-439. 7 Knight, P., Hamoudi, A. and Vassy, L., The diagnosis and treatment of midline neck masses in

chiIdren. Surgery, 93 (1983) 603-611. 8 Knight, P., MuIne., A. and Yassy, L., When is lymph node biopsy indicated in children with enlarged

peripheral nodes? Pediatrics, 69 (1982) 391-3%. 9 Koop, C., Visible and Palpable Lesions in Children, Grune & Stratton, New York, 1976.

10 Lake, A. and O&i, F., Peripheral lymphadenopathy in childhood, Am. J. Dis. Child.. 132 (1978) 357-359.

11 May, M., Neck masses in chihlrea: Diagnosis and treatment, RNT J., 57 (1978) 12-54. 12 Moussatos, 6. and Baffes, T., CetvicaI masses in infants and children, Pediatrics, 37 (1963) 251-256. 13 Pelton, S., Cervical Adenopathy. In C. Bluestone and S. Stool (Eds) Pediatric Otolaryngology, W.B.

Saunders, Philadelphia, 1983, pp. 1402-1411. 14 Pounds, L., Neck masses of congenital origin, Ped. CIin. North Am., 28 (1981) 841-844. 15 Putney, F., The diagnosis of head and neck masses in children, Otol. CIin. North Am., 3 (1970)

277-294. 16 Raney, R. and Handler, S., Management of neoplasms of the head and neck in children. II.

MaIignant tumors, Head Bt Neck 3, (1981) 580-507. 17 Sinclair, S., Beckman, IL and Rlhnan, L., Biopsy of enlarged, superficial lymph nodes, J. Am. Med.

Assoc., 228 (1974) 602-603. 18 Wald, II. and Sivasubramanian. K., Cervicai adenitis in infancy, CIin. Pediatr. (Bologna), 15 (1976)

1168-1169. 19 Zitelli, R., Neck masses in chiklren: adenopathy and maIignant disease, Ped. CIin. North Am., 28

(1981) 323-334.