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Congenital cystic masses of the neck: radiologic-pathologic correlation.

Cervical congenital cystic masses constitute an uncommon group of lesions usually diagnosed in infancy and childhood. The most common congenital neck mass is the thyroglossal duct cyst. The diagnosis is easily established from the presence of a cystic lesion in the anterior midline portion of the neck. The vast majority of branchial cleft cysts arise from the second branchial cleft. They can occur anywhere from the oropharyngeal tonsillar fossa to the supraclavicular region of the neck. Cystic hygroma is the most common form of lymphangioma. In the neck, cystic hygromas are most commonly found in the posterior cervical space. They typically extend into adjacent structures without respecting the fascial planes. Dermoid and epidermoid cysts result from sequestration of ectodermal tissue. The floor of the mouth is the most common location in the neck. Cervical thymic cysts are very uncommon lesions and are found anywhere from the angle of the mandible down to the sternum. Laryngoceles are classified into internal, external, and mixed types and have a frequent association with laryngeal carcinoma.

Cysts↗

Papillary carcinoma in an ectopic thyroid.

Ectopic thyroid tissue can be seen anywhere from the foramen caecum to the lower neck. Most frequently it presents as a thyroglossal duct cyst associated with a normal thyroid gland. Less common is total ectopia, which can occur as a lingual thyroid or occasionally in non-lingual locations. Malignant transformation of such ectopic tissue is extremely uncommon. Only 21 cases of lingual thyroid carcinomas have been reported until 1971. We present, to our knowledge, the first case of a papillary carcinoma in a non-lingual ectopic thyroid in the absence of an orthotopic thyroid.

Adolescent↗

Endoscopic botulinum toxin injection for cricopharyngeal dysphagia.

Twelve patients underwent 17 endoscopic injections of botulinum toxin type A in the cricopharyngeus muscle for the treatment of dysphagia and cricopharyngeal spasm over a 3-year period. The patient's charts were reviewed. Preoperative and postoperative symptoms, examination, and swallowing studies were reviewed. Eleven of the 12 patients had improvement in their symptoms, which lasted for a mean of 3.8 months. Two patients elected cricopharyngeal myotomy for permanent correction of their dysphagia. There was 1 case of postoperative neck cellulitis in an immunocompromised patient undergoing simultaneous excision of a thyroglossal duct cyst. We conclude that endoscopic injection of botulinum toxin is a relatively safe and viable technique for the treatment of dysphagia associated with cricopharyngeal spasm. It requires simple tools readily available to otolaryngologists. Larger, prospective controlled studies are necessary to establish its effectiveness and role in the management of this condition.

Aged↗

Adenocarcinoma originating in the thyroglossal duct.

Eighty-six cases of carcinoma of the thyroglossal duct have been reported in the literature, most of these being well-differentiated adenocarcinomas. Three new cases of the latter type are presented. Cases where carcinoma of the thyroglossal duct is suspected should be investigated with fine-needle biopsy, thyroid scintigraphy, skeletal and pulmonary X-ray, determination of TSH, T 3, PBI and blood serum calcium. The treatment of well-differentiated adenocarcinomas is a combination of surgical and medical procedures.

Adenocarcinoma, Papillary↗

Thyroglossal duct remnants in infants and children: reevaluation of histopathology and methods for resection.

Established principles for the management of thyroglossal duct remnants have been accepted widely since Sistrunk's description of the operative procedure in 1928. Nevertheless, inadequate excisions and troublesome recurrences still are encountered. We have reviewed 90 infants and children treated at The Children's Hospital in Boston over a 25-year period. Ten patients referred to our institution after an average of 2.4 prior operations per patient elsewhere suffered a recurrence rate of 30% after very wide reexcision. The remaining 80 patients who underwent primary excision at our institution had a 6.3% recurrence rate. All recurrences were managed successfully by further, wider excision. Histologic review demonstrated variability in patterns of drainage of the tract into the oropharynx, with accessory tracts and alveolar outpouchings off the main duct being present in 7.8% of specimens. This study demonstrates that the greatest opportunity for curative resection is at initial presentation, and that previous inadequate or unsuccessful excision is a major risk factor for further recurrence. The variability in microscopic anatomy of thyroglossal duct remnants can account for recurrent disease after lesser procedures, and underscores the importance of wide dissection above the hyoid bone. Our operative methods are illustrated.

Child↗

Thyroid hemiagenesis is a rare variant of thyroid dysgenesis with a familial component but without Pax8 mutations in a cohort of 22 cases.

Thyroid hemiagenesis is a rare form of thyroid dysgenesis of which some familial cases have been reported, including one associated with a heterozygous mutation in the Pax8 gene. However, the physiopathology remains not well known. The objectives of this study were 1) to describe the clinical features, 2) to look for familial clustering, and 3) to search for Pax8 mutations in a relatively large cohort of affected patients. A family history of thyroid dysgenesis was found in nine patients (40%), whose affected relatives had ectopic thyroid (n = 4), athyreosis (n = 1), thyroid hemiagenesis (n = 2), or thyroglossal duct cysts (n = 2). Screening for Pax8 mutations identified abnormal migration profiles by SSCP analysis in 3 patients, but direct sequencing did not show coding region mutations in any of the 22 patients. In conclusion, this study provides the first evidence that thyroid hemiagenesis can occur as a familial disorder associated with any form of thyroid dysgenesis. This finding supports both a common underlying mechanism to the various abnormalities in thyroid development and a role for genetic factors; however, our results from Pax8 analysis suggest that this gene may not be a key factor.

Abnormalities, Multiple↗

Management of well-differentiated thyroglossal remnant thyroid carcinoma: time to close the debate? Report of five new cases and proposal of a definitive algorithm for treatment.

BACKGROUND: Thyroid carcinoma is diagnosed in approximately 1% to 2% of thyroglossal duct remnants (TGDR). No definite agreement exists concerning the management of TGDR carcinoma, especially regarding the role of total thyroidectomy and postoperative adjuvant therapy. We report five new cases of TGDR carcinoma and review relevant articles in an attempt to clarify this issue. METHODS: We studied the demographic, clinical, tumor-related, treatment, pathologic, and outcome data on five patients treated at our institution for a TGDR carcinoma and compared the results with the four most important series published. RESULTS: All five patients were women, and diagnosis occurred after surgery in four. The sizes of the papillary tumors were 40, 38, 25, 23, and 15 mm (mean, 28.2 mm; range, 15-40 mm). Fine-needle aspiration biopsy of the TGDR had a low sensitivity (positive for one in four). A Sistrunk procedure was performed for resection of the TGDR in four patients, and plain TGDR resection was performed for the other patient. Three patients underwent repeat surgery; total thyroidectomy was performed in all cases, and cervical bilateral node dissection was performed in one case. With a median follow-up of 123.8 months (range, 8-284 months), all the patients are alive and free of disease, with no recurrences. CONCLUSIONS: TGDR carcinoma is a rare malignant tumor that is usually diagnosed after surgery; papillary carcinoma is the most common type. The currently recommended treatment is a Sistrunk procedure, with a tendency to deferred total thyroidectomy in selected cases (similar criteria exist for papillary carcinoma of the thyroid gland). The prognosis is excellent, with a good long-term survival.

Adult↗

Management of thyroglossal tract disease after failed Sistrunk's procedure.

Sistrunk's procedure for thyroglossal duct remnants has a very high success rate, there remains the occasional patient, however, that will have recurrent disease despite a competently performed operation. Applied anatomy and embryology proffer a solution to this problem. Extending the Sistrunk operation, with an anterior wide local excision remaining within normal tissue, enables removal of the entire thyroglossal tract remnant. A retrospective case note review was conducted to study our experience using this extended procedure to treat patients with thyroglossal tract disease that had recurred after a previous Sistrunk's operation. Six patients aged from five to 33 years were included in the study. There was one recurrence and the complication rate was comparable to the standard operation. It was concluded that a wide local excision is a valuable extension of the Sistrunk operation for the management of recurrent disease.

Adult↗

Mucin-secreting papillary adenocarcinoma of the hyoid bone: a unique case.

We present a unique case of a mucin-secreting papillary adenocarcinoma of intestinal type which has invaded and completely destroyed the hyoid bone and metastasized to the cervical lymph nodes bilaterally. The tumour is believed to have originated from a malignant thyroglossal duct remnant, and was managed with surgery and radiotherapy. We describe the case and discuss the literature regarding hyoid bone tumours.

Adenocarcinoma, Mucinous↗

Carcinoma in thyroglossal duct remnants.

Two cases of carcinoma arising in thyroglossal duct remnants are reported to add to the previously documented 78 cases in the literature. This rare lesion, which occurs is less than 1% of thyroglossal duct abnormalities, pathologically and clinically is similar to primary tumors arising in the thyroid gland. Papillary adenocarcinomas constitute 75-85% of all cases reported and most are confined to the duct remnant itself with metastasis being infrequently recorded. Therapy consists of wide excision of the tumor-bearing tissue (Sistrunk procedure) in those cases localized to the duct remnant itself. In instances where metastasis or additional lesions are detected in the thyroid gland, thyroid ablation and neck dissections are indicated. Additional modalities such as suppressive thyroid therapy, radioactive iodine, and external radiation have been employed. Distant metastasis is rare and the long-term prognosis is very favorable.

Adenocarcinoma, Papillary↗

Same-day-stay head and neck surgery.

The purpose of this study was to determine if selective head and neck surgical procedures on a same-day basis are justifiable. Two hundred consecutive head and neck same-day procedures were reviewed, including 84 parotidectomies and 116 other procedures previously managed as inpatients. Of the 200 patients, 36 (18%) were admitted, 33 for overnight observation, and 164 (82%) were discharged the same day. There were no complications reported in the discharged patients and a later questionnaire showed that 97% of the patients were satisfied. The advantages of selective same-day procedures outweigh the disadvantages for otolaryngologist and patient. The study shows that same-day-stay head and neck surgery for selective procedures is safe, reasonable, and cost-effective, but the combined efforts of the otolaryngologists, nurses, and administrators are required. Because of rising healthcare costs, experts are predicting a significant increase in the next few years of outpatient surgery, including surgery of the head and neck.

Adult↗

Pathogenesis, diagnosis and management of thyroid nodules in children.

According to the literature thyroid nodules are quite rare in the first two decades of life. However, there are some exceptions, relating to areas with an iodine deficiency or affected by radioactive fallout, where the risk of nodules and carcinomas is increased. Therefore, it is a great challenge for the physician to distinguish between benign and malignant lesions preoperatively, and not only in these areas of greater risk. A careful work-up, comprising the patient's history, clinical examination, laboratory tests, thyroid ultrasound, scintigraphy, fine-needle aspiration biopsy (FNAB) and molecular studies, is mandatory to improve the preoperative diagnosis. The differential diagnosis should also include benign thyroid conditions such as: (i) congenital hypothyroidism due to dyshormonogenesis or ectopy, (ii) thyroid hemiagenesis, (iii) thyroglossal duct cyst, (iv) simple goiter, (v) cystic lesion, (vi) nodular hyperplasia, (vii) follicular adenoma, (viii) Graves' disease and (ix) Hashimoto thyroiditis, all of which can predispose to the development of thyroid nodules. The majority of thyroid carcinomas derive from the follicular cell (papillary, follicular, insular and undifferentiated (or anaplastic) thyroid carcinoma), whereas medullary thyroid carcinoma derives from calcitonin-producing cells. Inherited forms of thyroid cancer may occur, especially in relation to medullary thyroid carcinoma. FNAB is a critical factor in establishing the preoperative diagnosis. However, we should keep in mind the fact that a conventional cytological evaluation can miss the neoplastic nature of a lesion and the employment of immunocytochemical and molecular studies of aspirates from FNAB can give us a more precise diagnosis of neoplasia in thyroid nodules once they are detected.

Biopsy, Fine-Needle↗

Papillary carcinoma in thyroglossal duct remnants: presentation of four cases and decision procedure for prophylactic thyroid gland dissection.

Papillary carcinoma in thyroglossal duct remnants is a rare and usually unexpected finding. It is controversial whether or not prophylactic thyroid gland dissection is necessary in such circumstances. We present our experience of four cases. Based on this, a consideration of published risk factors, and evaluation of the likelihood of a primary versus metastatic origin of the malignancy, we present a therapeutic decision procedure. When the thyroid is normal, the patient presents low-risk factors for thyroid cancer, and there is evidence that the malignancy is primary, removal of all thyroglossal duct remnants by the Sistrunk procedure is sufficient.

Adult↗

Common lumps and bumps of the head and neck in infants and children.

The majority of lesions in the neck of children will prove to be benign and of congenital origin arising from such structures as the thyroglossal duct and the branchial clefts in addition to hemangiomas and lymphangiomas. However, it is essential that lymphadenopathy be proven infectious and any asymptomatic lymph node enlargement must be considered Hodgkin's disease until proven otherwise. The occasional neuroblastoma or rhabdomyosarcoma presenting in the neck can usually be identified as solid by sonography, leading to early investigation and biopsy. Because of the abundance of important structures that course through the neck, surgery should be conducted in an operating suite with sophisticated, modern anesthetic techniques and with a surgeon experienced in dealing with the full array of lesions that occur.

Branchioma↗