branchial cleft cyst

Girl With a Neck Mass: Reactive Lymphadenitis or Something Else?

Thomas E. Meek, DO, and Thy Dan Vo Tsai, DO

An otherwise healthy 15-year-old girl presented to our clinic for evaluation of an approximately 2 to 3 cm nodular lesion on the right anterior cervical area (Figures 1 and 2). The lesion had been diagnosed 3 months prior to presentation as reactive lymphadenitis and treated with amoxicillin/clavulanic acid. Treatment initially seemed to be associated with a reduction in the size of the lesion, but the lesion recurred periodically and seemed to fluctuate in size after the girl ate. However, the lesion remained nontender, and the patient denied any other associated symptoms since onset. The results of the physical examination were otherwise unremarkable.

Ultrasonography was ordered, and results revealed a cystic mass that measured 3.5 × 3.7 × 2.0 cm in the region of the submandibular gland. A computed tomography (CT) scan (Figure 3) was obtained for clarification, and the patient was referred to an otolaryngologist.

 

What is this cystic mass?

A. Parotid gland tumor

B. Pathologic lymph node

C. Branchial cleft cyst

D. Sialadenitis

(Answer and discussion are on the next page.)

Answer: C, Branchial cleft cyst

DISCUSSION 

Branchial cleft cysts account for approximately 20% of all pediatric neck masses and are also known as pharyngeal cleft cysts and lateral cervical cysts.1 The term “branchial” comes from the Greek root “branchia,” referring to gills. 

In normal embryologic development, the cervical sinus is completely obliterated.2,3 A branchial cleft cyst  is a congenital malformation that most commonly arises from the incomplete obliteration of the cervical sinus, which is a remnant of the second, third, and fourth branchial (pharyngeal) clefts. These cysts are consistently located on the lateral side of the neck, anterior to the sternocleidomastoid muscle, with some variation depending on their developmental origin. 

The cysts are classified as first, second, or third based on the specific embryologic cleavage of the mesenchymal tissue and superior-to-inferior location. The second branchial cleft cyst is the most common type, typically located just inferior to the angle of the mandible. The condition usually becomes apparent in late childhood or early adulthood when a previously undiagnosed cyst becomes infected.2 Rarely, the initial diagnosis is made well into adulthood. These anomalies are an important consideration in the differential diagnosis of any head and neck mass in children. 

DIFFERENTIAL DIAGNOSIS

Parotid gland tumor (A). Salivary gland tumors are rare, constituting approximately 6% to 8% of head and neck neoplasms. Of these, the parotid gland tumor is the most common, accounting for approximately 85% of all salivary gland neoplasms. Contributing risk factors include smoking, industrial environmental exposures, previous radiation exposure, and Epstein-Barr viral infections in endemic areas. Clinical presentation is usually as a painless mass without other symptomatology.4-6

Sialadenitis (D) is a generalized inflammation of salivary glandular tissue that may be related to a number of causes, including tumors, prior radiation exposure, salivary gland stones, infection, autoimmune syndromes, and malnutrition.7 Stones are typically characterized by pain and swelling in the involved gland and are aggravated by eating or anticipation of oral intake.8 Viral infection is most commonly associated with mumps, and onset usually includes a prodrome of fever, malaise, headache, myalgia, and anorexia. Other less common viral causes include coxsackievirus, Epstein-Barr, parainfluenza, influenza, and HIV.9 Bacterial infections are typically caused by Staphylococcus aureus and are limited to elderly, malnourished patients. These infections are usually sudden in onset with associated fever, chills, and often, systemic toxicity.10 Associated autoimmune syndromes include Sjögren syndrome and extrapulmonary sarcoidosis. These conditions are chronic and typically bilateral.11 Malnutrition can be related to noninfectious, noninflammatory sialadenosis often associated with anorexia nervosa, bulimia, and Type I and Type II diabetes11

A Pathologic Lymph Node (B) is typically characterized as greater than 1 cm in length at its longest diameter. Nodes larger than 2 cm significantly increase the suspicion of malignancy. The terms “lymphadenopathy” and “lymphadenitis” are often used interchangeably in the clinical setting, though “lymphadenopathy” denotes an enlarged node, whereas “lymphadenitis” is associated with inflammation and tenderness. Both conditions can be related to a number of causes, both infectious and otherwise, including systemic disease and various medications.12

DIAGNOSIS AND TREATMENT

Our patient underwent a CT scan, and results revealed a fluid-filled lesion anterior to the sternocleidomastoid muscle. She subsequently underwent fine needle aspiration, which revealed abundant lymphocytes without squamous or glandular cells.

Based on the girl’s clinical presentation and the absence of other suggestive histopathology, she was diagnosed as having branchial cleft cyst. Given the location of the cyst, the presentation was most consistent with a second branchial cleft cyst. The girl successfully underwent surgical excision. There were no complications related to the procedure, and she experienced an uneventful postsurgical course.

Thomas E. Meek, DO, and Thy Dan Vo Tsai, DO, are from the Carolinas HealthCare System Blue Ridge Family Medicine Residency in Rutherford College, North Carolina. 

REFERENCES 

1. Work WP. Cysts and congenital lesions of the parotid gland. Otolaryngol Clin North Am. 1977; 10(2):339-343. 

2. Sadler TW. Head and Neck. In: Coryell P, Taylor, Cady B, eds. Langman’s Medical Embryology. 7th ed. Baltimore, MD: Lippincott Williams & Wilkin; 1995:313-326. 

3. Waldhausen JH. Branchial cleft and arch anomalies in children. Semin Pediatr Surg. 2006;15(2):64-69. 

4. Eveson JW, Auclair P, Gnepp DR, El-Naggar AK, eds. Tumours of the salivary glands. In: Barnes L, Eveson JW, Reichart P, Sidransky D, eds. Pathology and Genetics of Head and Neck Tumours. Lyon, France:IARC Press; 2005.209. 

5. Guzzo M, Locati LD, Prott FJ, Gatta G, McGurk M, Licitra L. Major and minor salivary gland tumors. Crit Rev Oncol Hematol. 2010;74(2):134-148. 

6. Spiro RH. Salivary neoplasms: overview of a 35-year experience with 2,807 patients. Head Neck Surg. 1986;8(3):177-184. 

7. Ellies M, Laskawi R, Arglebe C, Schott A. Surgical management of non-neoplastic diseases of the submandibular gland. A follow-up study. Int J Oral Maxillofac Surg. 1996; 25(4):285-289. 

8. McKenna JP, Bostock DJ, McMenamin PG. Sialolithiasis. Am Fam Physician. 1987;36(5): 119-125. 

9. Chow A. Infections of the oral cavity, neck, and head. In: Principles and Practice of Infectious Diseases, Mandell G, Bennett JE, Dolin R, eds. Philadelphia, PA: Churchill Livingstone, Inc; 2000. 699. 

10. Koischwitz D, Gritzmann N. Ultrasound of the neck. Radiol Clin North Am. 2000;38:1029-1045. 

11. Rice DH. Noninflammatory, non-neoplastic disorders of the salivary glands. Otolaryngol Clin North Am. 1999;32(5):835-843. 

12. Margolin JF, Steuber CP, Poplack DG. Acute lymphoblastic leukemia. In:Pizzo PA, Poplack DG, eds. Principles and Practice of Pediatric Oncology. 4th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2001:489.