Incidental thyroid papillary carcinoma in a thyroglossal duct cyst – management dilemmas

INTRODUCTIONBenign thyroglossal duct tract remnants typically thyroglossal duct cysts, (TDCs) are one of the commonest congenital childhood midline neck masses. Clinical presentation of persistent TDCs in adults is uncommon and the occurrence of incidental primary thyroid papillary carcinoma (TPC) in these cysts is rare.PRESENTATION OF CASEWe report the case of a 32-year-old female with an asymptomatic midline neck mass compatible with a TDC that was excised by Sistrunk's procedure. Histopathological examination revealed an incidental primary intraluminal TPC arising from the wall of the TDC.DISCUSSIONManagement dilemmas regarding the roles for total thyroidectomy, regional lymph node dissection, radioactive iodine, and suppressive thyroxine therapy are reviewed in the context of relevant evidence based literature.CONCLUSIONThe occurrence of incidental TPC in a TDC is rare. Though Sistrunk's procedure is adequate treatment for TDC, based on low, moderate, and high risk stratification, recommendations for further management of incidental TPC in TDC is discussed.     

Respiratory complications in cervical thymic cysts

Thymic cysts are considered uncommon lesions in the differential diagnosis of pediatric neck masses. They have been described as asymptomatic and of little clinical consequence. Recent reports have stressed the possibility of respiratory compromise associated with these lesions. We reviewed our experience with cervical thymic cysts with emphasis on respiratory problems. Ten pediatric patients underwent surgery and were found to have cervical thymic cysts. Ages ranged from newborn to 14 years. There were four boys and six girls. Two were found to have the thymic cysts at time of neck exploration for Grave's disease and hyperparathyroidism. Of the remaining eight patients, all had mobile cystic masses, located anterior to but extending beneath the lower third of the sternocleidomastoid muscle. The size of the mass ranged from 3.0 to 8.5 cm. Preoperative diagnosis included cystic hygroma/branchial cleft cyst (five), lymphoma (one), teratoma (one), and thymic cyst (one). All had a history of rapidly developing neck mass. Seven of the eight gave a history of upper respiratory tract infection (URI) prior to the development of the mass. Five had imaging studies that showed tracheal compression. Three of these required airway management in the early postoperative period. All were excised through a neck incision, with two requiring sternal extension. Histology showed cholesterol crystals, Hassall's corpuscles, and giant cell reaction diagnosis of thymic cysts. There has been no recurrence and no permanent respiratory sequela in the ten patients. Cervical thymic cysts are benign lesions that may be more common than literature suggests.(ABSTRACT TRUNCATED AT 250 WORDS)     

Case series: variations in the embryology of congenital midline cervical clefts

Congenital midline cervical clefts (CMCC) is a clinical diagnosis and represents a spectrum of rare developmental anomalies. Fewer than 100 cases have been reported overall, the first being described by Bailey in 1924 (1). It is not a true cleft because it does not include a gap between adjacent skin flaps. It is thought to represent a failure of midline fusion of the branchial arches although this is controversial. It is a clinical diagnosis and presents at birth with a ventral midline defect of the skin of the neck. This consists of a skin tag (nipple-like projection), an atrophic mucosal surface and a caudal sinus. It may be associated with a subcutaneous fibrous cord, which can cause a vertical midline tethering restricting cervical extension. Associated defects may be a median cleft of the mandible, tongue and lower lip. There may be an associated delay in mandibular development and hypoplasia or absence of neck structures such as the hyoid bone. Associated thyroglossal and bronchogenic cysts may occur as well as defects in other parts of the body such as a sternal cleft (2). CMCC has been previously reported in the literature as being of a branchial origin, however a review of the histology of previous cases suggests a combined branchial and bronchogenic component (2-6). We report on two cases that presented with the clinical picture of a classical CMCC. Our first case appears to have a bronchogenic origin with possible branchial components and the second case appears to be solely branchial in origin. There is much debate as to the embryology of this clinical entity; we shall endeavour to address the main theories.     

Aberrant cervical thymus in children: three case reports and review of the literature

Aberrant migration of thymic tissue occurs with ectopic thymus in the mediastinum, base of the skull, tracheal bifurcation, and cervical region. A recent review of the literature showed a total of 76 reported cases of aberrant thymus or thymic cysts in patients who presented with primary neck masses. We report three additional cases of ectopic cervical thymus. All three patients presented with asymptomatic cervical masses, and preoperative diagnosis included branchial cleft cyst, cervical lymphangioma, and cervical teratoma. All patients underwent complete surgical resection of the masses. Aberrant cervical thymus rarely produces symptoms because it does not invade contiguous strictures. Despite its rarity, it should be considered in the differential diagnosis of asymptomatic neck masses in children.     

Computed tomography and the thyroglossal duct cyst

Thyroglossal duct cysts (TDC) classically present as midline neck masses in close relation to the hyoid bone; yet--not uncommonly--their locations may be varied from the midline and from the hyoid. By means of the diagnostic modality of computed tomography (CT), high-resolution images of several cases of alternate presentations and locations of the TDC are examined. Included are examples of TDC in the suprahyoid, transhyoid, infrahyoid, and lateral positions. The potential value of CT in the diagnosis of the unusual cyst will be demonstrated and discussed.     

Branchial Cleft Cyst with Xanthogranulomatous Inflammation

Xanthogranulomatous inflammation (XGI) is a special type of inflammation presenting as mass lesions and mimicking malignant tumors. It is rarely described in the head and neck region, and to the best of our knowledge, there are no cases associated with branchial cleft cyst. A 39-year-old woman with an infiltrative cystic neck mass that radiologically mimicks a malignant tumor is herein presented. The histopathologic diagnosis was a branchial cleft cyst with XGI. XGI may be associated wih branchial cleft cysts. Patients may present with radiologic findings mimicking an invasive malignant tumor, and should be taken into account during the differential diagnosis with cystic squamous cell carcinoma.     

Unusual presentations of branchial cysts: a trap for the unwary.

Twenty-three cases of branchial cysts seen over 4 years are described. Only 11 patients presented with simple non-tender fluid swellings of the neck. Five patients presented with tender masses during infections and were diagnosed as acute abscesses. Seven patients presented with hard fixed masses mimicking lymphadenopathy. None of these were diagnosed correctly before surgery. Misdiagnosis led to significant morbidity from persistent cervical discharges. One patient had a partial nerve palsy. Infection of branchial cysts was common in this series and caused difficulties in diagnosis due to thickening of cysts walls following chronic inflammation in lymphoid tissue.     

Treatment of asymptomatic preauricular sinuses: challenging conventional wisdom

Congenital preauricular sinuses must not be considered trivial lesions. They can and should be differentiated from branchial cleft cysts. Cure is achieved only through complete excision of the sinus tract and its associated cysts. Total extirpation of the sinus tract is difficult in the presence of infection. The embryoanatomy and treatment of congenital preauricular sinus tracts as well as our experience with 6 patients are reported. We recommend excision of asymptomatic congenital preauricular sinus tracts.     

The problem of recurrent thyroglossal duct remnants

The removal of a thyroglossal duct or sinus is a common pediatric surgical operation. It has been taught that if the duct remnant is removed down to and including the middle third of the hyoid bone, the "correct" operation has been done. The three authors have an experience totaling over 60 years in pediatric surgery during which time they operated on 270 thyroglossal duct cysts and sinuses. In this group were 27 recurrences of which three belonged to the authors. Most recurrent thyroglossal duct remnants were found to have the middle third of the hyoid bone still in place, and with its removal the patients were cured. However, during this period of time, there were thyroglossal duct cysts and sinuses which recurred in spite of the "correct" surgical procedure having been done. These patients had all been operated on between three and five times thus creating a difficult and unusual problem. The solution to these recurrent thyroglossal duct cysts and sinuses were deeper excisions to remove residual tracts deep to the previously removed hyoid bone, and/or wider excisions to excise previously missed respiratory epithelial remnants which deviated laterally from the midline. The latter tissue was found to be a more centrally directed branchial cleft sinus.     

Congenital cervical cysts, sinuses, and fistulae in pediatric surgery

Congenital cervical anomalies are essential to consider in the clinical assessment of head and neck masses in children and adults. These lesions can present as palpable cystic masses, infected masses, draining sinuses, or fistulae. Thyroglossal duct cysts are most common, followed by branchial cleft anomalies and dermoid cysts. Other lesions reviewed include median ectopic thyroid, cervical teratomas, and midline cervical clefts. Appropriate diagnosis and management of these lesions requires a thorough understanding of their embryology and anatomy. Correct diagnosis, resolution of infectious issues before definitive therapy, and complete surgical excision are imperative in the prevention of recurrence.     

The fourth branchial cleft anomaly

The fourth branchial cleft anomaly has long been postulated. A case in which a lower neck sinus tract extended under the clavicle near the subclavian vessels is presented. The distinguishing features of bronchogenic and branchogenic anomalies are discussed. It appears that unless a complication (e.g., abscess, inflammatory process) of the fourth branchial cleft sinus tract occurs within the mediastinum, mandating full exploration, the complete form of the anomaly may continue to remain undocumented.     

An intra-thyroidal branchial cyst: a case report.

Branchial cleft cysts were previously thought not to occur in the thyroid but have recently been described in two patients with Hashimoto's disease. This case report describes a patient with a branchial cleft cyst in an otherwise normal thyroid gland and could provide further evidence that thyroidal follicular cells are derived from the branchial clefts as well as from the primitive gut.     

AN INTRA-THYROIDAL BRANCHIAL CYST: A CASE REPORT

Branchial cleft cysts were previously thought not to occur in the thyroid but have recently been described in two patients with Hashimoto's disease. This case report describes a patient with a branchial cleft cyst in an otherwise normal thyroid gland and could provide further evidence that thyroidal follicular cells are derived from the branchial clefts as well as from the primitive gut.     

Lateral cervical bronchogenic cyst: an unusual cause of a lump in the neck

BACKGROUND: Congenital cysts of the neck in children are not uncommon. Most of these are thyroglossal, branchial cleft, or less commonly, thymic cysts. Bronchial cysts rarely are initially seen as a neck mass. METHODS: Use of an illustrative case of a bronchogenic cyst initially seen as an upper lateral neck mass. CONCLUSIONS: We emphasize that although ectopic bronchogenic cysts are rare lesions of the head and neck, especially in the upper lateral neck, they should be included in the differential diagnosis in the evaluation of congenital neck cysts.     

An unusual presentation of a fourth pharyngeal arch (branchial cleft) sinus

Fourth pharyngeal arch (branchial cleft) sinuses and fistulas are a rare cause of recurrent neck abscesses presenting in the first 2 decades of life. Because they are uncommon, the diagnosis and subsequent treatment can be delayed, even with proper radiographic imaging and clinical evaluation. This case illustrates a life-threatening presentation of a ruptured fourth branchial cleft sinus with extravasation into the mediastinum, chest, and abdomen.     

Congenital cervical salivary fistula

A study of 35 lesions diagnosed as branchial cleft sinuses or fistulas identified a homogeneous subgroup characterized by ectopic salivary tissue, draining serous or mucoid fluid through a short sinus tract to the base of the neck anteriorly. These lesions should more properly be called congenital cervical salivary fistulas, since they may not arise from the branchial apparatus but rather from the embryonic placodal duct.     

Branchial remnants: a review of 58 cases

Most congenital lateral cervical cysts, fistulae, and skin tags are considered to be from the branchial apparatus. This is a 13-year review of 58 patients (with 66 branchial lesions) who were operated on. There were eight simple cysts and six cysts with a fistula; 43 external fistulae with or without an internal opening, and nine skin tags. Eighty-seven percent (39/45) of patients with skin tags and external fistulae were less than 5 years of age at the time of operation. On the other hand, all eight patients with cysts but no fistula were greater than 9 years of age. Eight lesions were considered to be the first branchial remnants, and 44 lesions were suspected to be from the second branchial cleft. One external fistula with an internal communication to the pharynx at the level of thyrohyoid membrane was considered to be a third (or fourth) branchial remnant. The other branchial cyst with thyroid tissue in its wall was suggested to be a fourth branchial remnant. Pathology of the excised lesions showed columnar, squamous, or a mixed epithelium. Lymphoid aggregates were documented in 31 patients. Duration of hospital stay was short, except for four patients with first cleft defects who stayed more than five days. Three of the four recurrent cases were first branchial remnants, including one case with the first operation performed at another hospital. In view of these findings, first branchial remnants must be excised with extra care.     

Investigation prior to thyroglossal duct cyst excision

INTRODUCTION

Investigation of the anterior midiine neck lump has been debated over the years with little agreement on best practice. Thyrogiossai duct cysts (TDCs) are the most common aetiology. A TDC may contain ectopic thyroid tissue, which may affect the decision to excise.

METHODS

A computerised survey was sent to a representative sample of UK-based ENT surgeons to determine current practice in investigation of presumed TDCs and the incidence of ectopic thyroid tissue.

RESULTS

Overall, 95% of those surveyed use ultrasonography, with 32% also arranging thyroid function tests. Fifteen per cent had encountered absent normal thyroid tissue in the presence of a midiine neck swelling. In 64% of cases this represented the only functioning thyroid tissue. Thyroid function tests were normal in all but two cases.

CONCLUSIONS

The results show a significant change in practice over the last decade. All surgeons would arrange some form of investigation of a presumed TDC, with the vast majority using ultrasonography. Radioisotope scanning should only be used if the ultrasonography or thyroid function tests are abnormal. The incidence of ectopic thyroid tissue in this survey was higher than previously calculated, with a 0.17% prevalence of midiine neck lumps representing the only functioning thyroid tissue.     

Recurrent branchial sinus tract with aberrant extension

Second branchial cysts are the commonest lesions among congenital lateral neck anomalies. Good knowledge of anatomy and embryology are necessary for proper treatment. Surgical treatment involves resection of all branchial remnants, which extend laterally in the neck, medial to the sternocleidomastoid muscle with cranial extension to the pharynx and ipsilateral tonsillar fosa. However, infections and previous surgery can distort anatomy, making the approach to branchial anomalies more difficult. We present a case of a 17-year-old patient who presented with a second branchial tract anomaly with an aberrant extension to the midline and part of the contralateral neck. Previous surgical interventions and chronic infections may have been the primary cause for this aberrant tract. All head and neck surgeons should bear in mind that aberrant presentations may exist when reoperating on chronic branchial cysts fistulas.     

Cystic metastases of the neck from occult thyroid adenocarcinoma.

In 9 of 118 patients with differentiated thyroid carcinomas, a solitary cystic lateral cervical mass simulating a branchial cleft anomaly was the sole presenting sign of the disease. These masses were nodal metastases of occult papillary adenocarcinoma of the thyroid that underwent liquefaction necrosis. Sonographically, the masses presented a complex pattern. Accordingly, it is suggested that, in patients presenting with an asymptomatic solitary lateral cystic cervical mass, the possibility of metastasis from an occult thyroid malignancy should be considered, and consent for definitive surgery should be obtained. The ultrasonic pattern of the cystic mass is of importance in the differentiation of a cavitated lymph node from a branchial cleft cyst.