A Nodule
on
the Side of the Neck in
a
Child
Mark\l=e'\taL\l=i'\mov\l=a'\,MD; Barbara M. Egbert, MD; Mary L. Williams, MD
University of California, REPORT OF A CASE
A 5-year-old white boy presented to the pediatric dermatology clinic with a somewhat tender nodule on the left side of his neck. His parents recalled that since birth he had had a small papule in this area. The papule remained unchanged until approximately 6 months before this visit, when a bluish
Figure 1.
San Francisco
nodule developed in the area. No discharge had been noted. On initial examination, the patient had a 0.5\m=x\0.5-cm, firm, freely mobile bluish nodule overlying the left sternocleidomastoid muscle (Fig 1). No ulceration or drainage was noted. The remainder of his skin examination was unremarkable. The nodule was excised in full and sent for
Figure
Downloaded From: http://archderm.jamanetwork.com/ by a University of California - San Diego User on 06/05/2015
2.
routine histopathologic examination. The sections showed a cystic structure lined by pseudostratified ciliated columnar epithelium. Goblet cells were present focally. The overlying epidermis was normal. Histopathologic sections are shown in Figs 2 and 3. What is your diagnosis?
Figure
3.
DIAGNOSIS: Branchial cleft
the
cyst.
DISCUSSION Branchial cleft cysts are commonly present in the second and third decade. Patients usually note a stable swelling on the lateral aspect of the neck. Unless secondary infection or hem¬ orrhage has occurred, the lesions are soft, flesh colored, round, and nontender. Branchial cleft cysts usually protrude somewhere below the angle of the mandible or along the anterior border of the sternocleidomastoid muscle. However, they may also be located on the anterior or posterior aspect of the shoulder and within or near the thyroid.1 The precise origin of these cysts is unclear, but they are considered to represent remnants of the cervical sinus or unenclosed branchial clefts. Those lesions presenting below the angle of the jaw or between the angle of the jaw and the external auditory meatus are remnants of the first bran¬ chial cleft. Occasionally, they may be connected by a sinus tract to the external auditory meatus. More common are cysts from the second branchial cleft, which are located along the anterior border of the sternocleidomastoid muscle and may have a communicating tract to the pharynx near the superior fold of the tonsil. Cysts related to the third and fourth clefts are usually supraclavicular or suprasternal and may also be associated with an internal sinus tract. These lesions usually occur sporadically; however, familial cases have been re¬
ported.2 The epidermis overlying
a branchial cleft cyst is usually unremarkable. The cyst may be lined by stratified squamous epithelium or by pseudostratified, ciliated columnar epithe¬ lium with goblet cells, as seen in our patient. Sequestration of
progenitor cells occurring early in fetal life and from the
parts of the branchial cleft result in differentiation toward respiratory epithelium.1 Hemorrhage into the cyst may be present as well as granulation tissue and inflammation in the surrounding stroma. If a communicating sinus is present, it
epithelium or by ciliated epithelium.3 Occasionally, seromucous glands, smooth muscle, or cartilage may be present.1 Squamous cell carcinomas have occasionally arisen in the lining of these cysts. The differential diagnosis of a bluish nodule on the lateral aspect of the neck includes unilateral lymph node enlarge¬ ment; epidermal inclusion cyst; blue nevus; pilomatricoma or other adnexal tumors; vascular lesions; carotid and thyroid tumors; thyroglossal, branchial, bronchogenic, and thymic may be lined by stratified squamous
cysts; and metastatic carcinoma.4 The diagnosis is made by
microscopic analysis. The developmental anomalies are de¬ fined by their histopathologic appearance and location. Thyroglossal duct cysts are the most common clinically sig¬ nificant congenital anomaly. These present in the midline of the neck anterior to the trachea. Lesions high in the neck are lined by stratified squamous epithelium; lesions more proxi¬ mal to the thyroid are lined by epithelium similar to that of the thyroid and surrounded by an intense lymphocytic infiltrate. Frequently, thyroid follicles are seen in the subjacent stroma. This thyroid tissue may undergo neoplastic changes, most commonly papillary carcinoma.4"6 Bronchogenic cysts present in the region of the suprasternal notch. These are lined by a pseudostratified, columnar cil¬ iated epithelium with goblet cells and may contain smooth muscle, seromucous glands, lymphoid follicles, or cartilage.7 Traditionally, these cysts have been thought to arise from ab¬ normal budding of the respiratory tree. However, more
recently, branchial cleft origin of these lesions has been pro¬ posed because intrathoracic bronchial cysts are frequently associated with other congenital anomalies; these superficial cysts are not.1 Thymic cysts are rare. They may be located anywhere be¬ tween the angle of the mandible to the manubrium sterni. The
histopathologic appearance is diagnostic; remnants of thymic tissue with characteristic Hassall's corpuscles are present in the fibrous tissue wall. Cholesterol granulomas are common.8 Treatment for branchial cleft cyst is surgical excision. For deeply located lesions or those with draining sinus tracts, ex¬ cision by a head and neck surgeon is recommended.9 References 1. Shareef DS, Salam R. Ectopic vestigial lesions of the neck and shoulders. J Clin Pathol. 1981;34:1155-1162. 2. Wheeler CE, Shaw RF, Cawley EP. Branchial anomalies in three generations of one family. Arch Dermatol. 1958;77:715-719. 3. Foote JE, Anderson PC. Branchial cleft remnants suggesting tuberculous lymphadenitis. Arch Dermatol. 1968;97:536-539. 4. Rook A, Wilkinson DS, Ebling FJG, Champion RH, Burton JL. Textbook of Dermatology. 4th ed. Boston, Mass: Blackwell Scientific Publications; 1986:218-221. 5. Rosai J. Ackerman's Surgical Pathology. 7th ed. St Louis, Mo: CV Mosby Co; 1989:393-394. 6. Cotran RS, Kumar V, Robbins SL. Robbins Pathologic Basis of Disease. Philadelphia, Pa: WB Saunders Co; 1989:1241-1242. 7. Fraga S, Helwig EB, Rosen SH. Bronchogenic cysts in the skin and subcutaneous tissue. J Clin Pathol. 1971;56:230-238. 8. Sanusi LD, Carrington PR, Adams DN. Cervical thymic cyst. Arch Dermatol. 1982;118:122-124. 9. Arnold HL, Odom RB, James WD. Andrews' Diseases of the Skin: Clinical Dermatology. 8th ed. Philadelphia, Pa: WB Saunders Co; 1990:679-680.
Clinicians, local and regional societies, and residents and fellows in dermatology are invited to submit quiz cases to this section. Cases should follow the established pattern and be submitted double-spaced and in triplicate. Photomicrographs and illustrations must be clear and submitted as positive color transparencies. If photomicrographs are not available, the actual slide from the specimen will be acceptable. Material should be accompanied by the required copyright transfer statement, as noted in "Instruc¬ tions for Authors. Material for this section should be submitted to Antoinette F. Hood, MD, Department of Dermatology, The Johns Hopkins Medical Institutions, 600 N Wolfe St, Baltimore, MD 21205. Reprints are not available. "
Downloaded From: http://archderm.jamanetwork.com/ by a University of California - San Diego User on 06/05/2015
on
the Side of the Neck in
a
Child
Mark\l=e'\taL\l=i'\mov\l=a'\,MD; Barbara M. Egbert, MD; Mary L. Williams, MD
University of California, REPORT OF A CASE
A 5-year-old white boy presented to the pediatric dermatology clinic with a somewhat tender nodule on the left side of his neck. His parents recalled that since birth he had had a small papule in this area. The papule remained unchanged until approximately 6 months before this visit, when a bluish
Figure 1.
San Francisco
nodule developed in the area. No discharge had been noted. On initial examination, the patient had a 0.5\m=x\0.5-cm, firm, freely mobile bluish nodule overlying the left sternocleidomastoid muscle (Fig 1). No ulceration or drainage was noted. The remainder of his skin examination was unremarkable. The nodule was excised in full and sent for
Figure
Downloaded From: http://archderm.jamanetwork.com/ by a University of California - San Diego User on 06/05/2015
2.
routine histopathologic examination. The sections showed a cystic structure lined by pseudostratified ciliated columnar epithelium. Goblet cells were present focally. The overlying epidermis was normal. Histopathologic sections are shown in Figs 2 and 3. What is your diagnosis?
Figure
3.
DIAGNOSIS: Branchial cleft
the
cyst.
DISCUSSION Branchial cleft cysts are commonly present in the second and third decade. Patients usually note a stable swelling on the lateral aspect of the neck. Unless secondary infection or hem¬ orrhage has occurred, the lesions are soft, flesh colored, round, and nontender. Branchial cleft cysts usually protrude somewhere below the angle of the mandible or along the anterior border of the sternocleidomastoid muscle. However, they may also be located on the anterior or posterior aspect of the shoulder and within or near the thyroid.1 The precise origin of these cysts is unclear, but they are considered to represent remnants of the cervical sinus or unenclosed branchial clefts. Those lesions presenting below the angle of the jaw or between the angle of the jaw and the external auditory meatus are remnants of the first bran¬ chial cleft. Occasionally, they may be connected by a sinus tract to the external auditory meatus. More common are cysts from the second branchial cleft, which are located along the anterior border of the sternocleidomastoid muscle and may have a communicating tract to the pharynx near the superior fold of the tonsil. Cysts related to the third and fourth clefts are usually supraclavicular or suprasternal and may also be associated with an internal sinus tract. These lesions usually occur sporadically; however, familial cases have been re¬
ported.2 The epidermis overlying
a branchial cleft cyst is usually unremarkable. The cyst may be lined by stratified squamous epithelium or by pseudostratified, ciliated columnar epithe¬ lium with goblet cells, as seen in our patient. Sequestration of
progenitor cells occurring early in fetal life and from the
parts of the branchial cleft result in differentiation toward respiratory epithelium.1 Hemorrhage into the cyst may be present as well as granulation tissue and inflammation in the surrounding stroma. If a communicating sinus is present, it
epithelium or by ciliated epithelium.3 Occasionally, seromucous glands, smooth muscle, or cartilage may be present.1 Squamous cell carcinomas have occasionally arisen in the lining of these cysts. The differential diagnosis of a bluish nodule on the lateral aspect of the neck includes unilateral lymph node enlarge¬ ment; epidermal inclusion cyst; blue nevus; pilomatricoma or other adnexal tumors; vascular lesions; carotid and thyroid tumors; thyroglossal, branchial, bronchogenic, and thymic may be lined by stratified squamous
cysts; and metastatic carcinoma.4 The diagnosis is made by
microscopic analysis. The developmental anomalies are de¬ fined by their histopathologic appearance and location. Thyroglossal duct cysts are the most common clinically sig¬ nificant congenital anomaly. These present in the midline of the neck anterior to the trachea. Lesions high in the neck are lined by stratified squamous epithelium; lesions more proxi¬ mal to the thyroid are lined by epithelium similar to that of the thyroid and surrounded by an intense lymphocytic infiltrate. Frequently, thyroid follicles are seen in the subjacent stroma. This thyroid tissue may undergo neoplastic changes, most commonly papillary carcinoma.4"6 Bronchogenic cysts present in the region of the suprasternal notch. These are lined by a pseudostratified, columnar cil¬ iated epithelium with goblet cells and may contain smooth muscle, seromucous glands, lymphoid follicles, or cartilage.7 Traditionally, these cysts have been thought to arise from ab¬ normal budding of the respiratory tree. However, more
recently, branchial cleft origin of these lesions has been pro¬ posed because intrathoracic bronchial cysts are frequently associated with other congenital anomalies; these superficial cysts are not.1 Thymic cysts are rare. They may be located anywhere be¬ tween the angle of the mandible to the manubrium sterni. The
histopathologic appearance is diagnostic; remnants of thymic tissue with characteristic Hassall's corpuscles are present in the fibrous tissue wall. Cholesterol granulomas are common.8 Treatment for branchial cleft cyst is surgical excision. For deeply located lesions or those with draining sinus tracts, ex¬ cision by a head and neck surgeon is recommended.9 References 1. Shareef DS, Salam R. Ectopic vestigial lesions of the neck and shoulders. J Clin Pathol. 1981;34:1155-1162. 2. Wheeler CE, Shaw RF, Cawley EP. Branchial anomalies in three generations of one family. Arch Dermatol. 1958;77:715-719. 3. Foote JE, Anderson PC. Branchial cleft remnants suggesting tuberculous lymphadenitis. Arch Dermatol. 1968;97:536-539. 4. Rook A, Wilkinson DS, Ebling FJG, Champion RH, Burton JL. Textbook of Dermatology. 4th ed. Boston, Mass: Blackwell Scientific Publications; 1986:218-221. 5. Rosai J. Ackerman's Surgical Pathology. 7th ed. St Louis, Mo: CV Mosby Co; 1989:393-394. 6. Cotran RS, Kumar V, Robbins SL. Robbins Pathologic Basis of Disease. Philadelphia, Pa: WB Saunders Co; 1989:1241-1242. 7. Fraga S, Helwig EB, Rosen SH. Bronchogenic cysts in the skin and subcutaneous tissue. J Clin Pathol. 1971;56:230-238. 8. Sanusi LD, Carrington PR, Adams DN. Cervical thymic cyst. Arch Dermatol. 1982;118:122-124. 9. Arnold HL, Odom RB, James WD. Andrews' Diseases of the Skin: Clinical Dermatology. 8th ed. Philadelphia, Pa: WB Saunders Co; 1990:679-680.
Clinicians, local and regional societies, and residents and fellows in dermatology are invited to submit quiz cases to this section. Cases should follow the established pattern and be submitted double-spaced and in triplicate. Photomicrographs and illustrations must be clear and submitted as positive color transparencies. If photomicrographs are not available, the actual slide from the specimen will be acceptable. Material should be accompanied by the required copyright transfer statement, as noted in "Instruc¬ tions for Authors. Material for this section should be submitted to Antoinette F. Hood, MD, Department of Dermatology, The Johns Hopkins Medical Institutions, 600 N Wolfe St, Baltimore, MD 21205. Reprints are not available. "
Downloaded From: http://archderm.jamanetwork.com/ by a University of California - San Diego User on 06/05/2015
