Ectopic Hamartomatous Thymoma: A Review Of The Literature With Report Of New Cases And Proposal Of A New Name: Biphenotypic Branchioma.

Ectopic hamartomatous thymoma (EHT) is a rare benign neoplasm of the lower neck suggesting branchial origin. Despite use of the term thymoma in the nomenclature, there is no evidence of thymic origin or differentiation. It affects middle-aged adults with a remarkable male predominance. To date less than 80 cases have been reported in the English literature. We present here two additional cases of EHT. The first is a benign case in a 31-year-old man, showing typical histological features. The second is a malignant case in a 70-year-old woman, showing intraductal carcinoma arising in intimate association with an EHT. These cases are presented in the context of a review of cases reported in the English literature. The exact origin has not been identified, but is considered to be of branchial apparatus, creating a quandary about the best terminology. Recently, the designation "branchial anlage mixed tumor" or "thymic anlage tumor" were proposed, but do not quite reflect the true nature of the neoplasm. To avoid taxonomic confusion, international consensus on terminology is desired. As this entity is a neoplasm that shows dual mesoderm and endoderm derivation/differentiation, we propose a new name "biphenotypic branchioma."

Cytologic separation of branchial cleft cyst from metastatic cystic squamous cell carcinoma: A multivariate analysis of nineteen cytomorphologic features.

BACKGROUND: The separation of branchial cleft cysts from metastatic cystic squamous cell carcinomas in adults can be clinically and cytologically challenging. Diagnostic accuracy for separation is reported to be as low as 75% prompting some authors to recommend frozen section evaluation of suspected branchial cleft cysts before resection. We evaluated 19 cytologic features to determine which were useful in this distinction. METHODS: Thirty-three cases (21 squamous carcinoma and 12 branchial cysts) of histologically confirmed cystic lesions of the lateral neck were graded for the presence or absence of 19 cytologic features by two cytopathologists. The cytologic features were analyzed for agreement between observers and underwent multivariate analysis for correlation with the diagnosis of carcinoma. RESULTS: Interobserver agreement was greatest for increased nuclear/cytoplasmic (N/C) ratio, pyknotic nuclei, and irregular nuclear membranes. Recursive partitioning analysis showed increased N/C ratio, small clusters of cells, and irregular nuclear membranes were the best discriminators. CONCLUSION: The distinction of branchial cleft cysts from cystic squamous cell carcinoma is cytologically difficult. Both digital image analysis and p16 testing have been suggested as aids in this separation, but analysis of cytologic features remains the main method for diagnosis. In an analysis of 19 cytologic features, we found that high nuclear cytoplasmic ratio, irregular nuclear membranes, and small cell clusters were most helpful in their distinction. Diagn. Cytopathol. 2016;44:561-567. © 2016 Wiley Periodicals, Inc.

[Brachial cyst: a case report]. / Cisti branchiale: case report.

The Authors present the clinical case of a 41 years old patient, presenting since two years a right cervical swelling, progressively grown up in the last two months with tight-elastic thickness. Pre-operative diagnostic tests suggested the suspect of II branchial arch cyst. By surgery we removed a cystic formation that appeared, at histopathologic exam with a multi-stratified coating, surrounded by lymphoid tissue, organized in germinative centres, compatible with branchial cyst structure.

[Nasopharyngeal cysts. Report of four cases and literature review]. / Quistes nasofaríngeos: descripción de cuatro casos y revisión de la literatura.

Nasopharyngeal cysts although common are not completely understood. They are classified according to their localization and pathogenesis in congenital or acquired and medial or lateral. We report 4 cases: a branchiogenic causing serious otitis, a Tornwaldt's cyst also producing otitis serosa, another asymptomatic Tornwaldt's cyst and a retention cyst which produce an obstructive apnea syndrome. All but the asymptomatic one were operated on transoral, by grasping the cyst and its implantation site. They all remain till now asymptomatic. We review the chapter of nasopharyngeal cysts, discussing their clinicopathological classification, their diagnosis and treatment.

Branchiogenic carcinomas: do they exist?

The existence of branchiogenic carcinomas is questioned in this review, which also discusses the different possibilities in an adult patient who presents with a cystic swelling in the upper lateral aspect of the neck. Most cases of branchiogenic carcinomas reported in the literature may actually have been secondary metastatic lesions from an occult tonsillar carcinoma. We will also discuss the different features of cystic metastasis that distinguish them from solid metastasis, and explain why oropharyngeal carcinomas with cystic neck metastasis should be in a separate category from other head and neck cancers.

Pharyngeal dermoids ("hairy polyps") as accessory auricles.

The purpose of this study is to clarify the origin and nature of so-called hairy polyps or dermoids of the pharynx, which are often thought to be a variant of pharyngeal teratoma. For this purpose, a case is reported of a dermoid polyp involving the middle ear of an infant, the features of multiple examples of pharyngeal dermoid polyps and teratomas received for consultation by the Armed Forces Institute of Pathology are examined, and selected pertinent reports from the literature are reviewed. All three means are used to support the conclusion that these lesions are choristomatous developmental anomalies arising from the first branchial cleft area and that they essentially represent heterotopic accessory "ears" (auricles) without the growth potential of a teratoma.

Evaluation of first branchial anomalies by CT and MR.

OBJECTIVE: We present the spectrum of CT and MR findings of first branchial anomalies (FBAs) in our 10 year experience and attempt to integrate these anomalies into current classification systems. MATERIALS AND METHODS: The hospital charts and all relevant imaging studies were retrospectively reviewed in 11 patients with pathologically proven FBAs. Radiographic analysis consisted of lesion size, morphology, location, relation to the expected course of the facial nerve, enhancement pattern, attenuation compared to CSF, and T1- and T2-weighted signal intensity. These findings were correlated with clinical symptoms and surgical findings. RESULTS: Seven lesions were unilocular cystic lesions within the parotid gland. Two lesions were multilobular and two consisted only of sinus tracts. Rim enhancement occurred with signs and symptoms of infection. Sinus tracts were present in 5 of 11 patients. One patient had an associated epidermoid tumor. The Arnot classification system was the most useful for categorizing our lesions. CONCLUSION: Imaging was helpful in preoperative surgical planning, and none of the patients in our series had residual neurological deficits. Computed tomography was preferred to MR due to the visualization of bony detail and better capability to determine the cystic nature of these lesions.

[Congenital cysts and fistulae of the face and the neck]. / Kystes et fistules congénitaux de la face et du cou.

The authors review the embryological, clinical and therapeutic aspects of congenital facial and cervical cysts (C) and fistulae (F), based on a personal series of 85 cases (facial: 18, latero-cervical: 29, mediocervical: 38) observed during a 5-year period. The facial forms are the result of an incomplete coalescence of the facial buds and most often present as helical F (17/18). Laterocervical C and F are due to abnormal evolution of branchial clefts; the main clinical forms are related to anomalies of the 2nd branchial cleft (24/29), usually presenting as sinus localized at the anterior border of the lower third of the sternocleidomastoid muscle (8) and amygdaloid cysts. Thyroglossal duct cysts are the most frequent of the medio-cervical C and F (35/38); they usually present as a mediocervical cyst in the thyro-hyoid space which may be revealed by an infection or a fistulization. The only appropriate treatment of congenital facial and cervical C and F is surgery providing that the resection is meticulous with complete resection of the fistula in order to avoid relapse. Complete resection also suppresses the risk of secondary malignant degeneration of amygdaloid and thyroglossal duct cysts.

Second branchial cleft-pouch set fistulae, sinuses and cysts in children.

A perfect understanding of the embryology of the branchial apparatus and the cervical anatomy is absolutely essential for the classification and treatment of branchial fistulae, sinuses and cysts, particularly with respect to those of the second cleft-pouch set. Some of our recent cases provide evidence of the need for precise dissection, a condition sine qua non to avoid surgical failures.

[Anomalies of the first branchial cleft]. / Anomalies de la première fente branchiale.

These are cysts and fistulae which closely adherent to the external auditory canal and drain through the high lateral cervical suprahyoid site. Misdiagnosis is common with these lesions and inadequate removal leads to surgical failure. A review of the embryology of this region is necessary since we intend to explain the clinical aspects and the variable relationship to the facial nerve. Eight cases are described and the literature pertaining to first branchial cleft syndrome is reviewed. Diagnostic and management problems will be discussed. A full exposure of the parotid gland and facial nerve is essential to complete removal and facial nerve preservation.

Thymopharyngeal duct cyst: a form of cervical thymus.

The various types of cervical thymus may present as a neck mass, usually laterally, from the angle of the mandible to the manubrium. Since it is rare to diagnose the entity preoperatively, the differential diagnosis includes the more common branchial cleft cyst, thyroglossal duct cyst, cystic hygroma, cystic dermoid, and lesions of the salivary gland, thyroid, parathyroid, and cervical lymph nodes. Because cervical thymic tissue in various forms has been reported so frequently, we feel the entity should be considered in the clinical differential diagnosis of lateral neck mass, especially in the younger age group.

An unusual first branchial cleft cyst.

First branchial cleft anomalies have been documented with increasing frequency within the past decade, with more than 200 cases reported to date. A case of a first branchial cleft cyst with some unusual features is presented. The literature of such anomalies is reviewed, with some distinct clinical differences being noted between cysts and other branchial cleft anomalies.

First branchial cleft anomalies.

Our experience with anomalies of the first branchial cleft is reviewed. This includes 38 cases, the largest series collected to date. A new classification is proposed based on the anatomic findings--whether cyst, sinus, or fistula is present. The embryology and pathology of first cleft anomalies are discussed and an approach to the diagnosis and management of these lesions is given. In addition, a patient with a branchial cleft defect involving the middle ear space is reported.

Branchiogenic carcinoma -- fact or fallacy?

The ongoing debate concerning the existence of malignant transformation of branchial cleft cysts has been analyzed, with the aid of previous reports and reviews in the literature. Six new cases are presented and illustrated. These occurred in 4 male and 2 female patients, having an average age of 60 years. Two of the lesions were within the parotid salivary gland. The longest period of survival was 3 1/2 years. New criteria for the acceptability of tumors as primary branchiogenic cancers are proposed. We believe that, using these strict criteria, rare examples of primary branchiogenic carcinomas do exist; their treatment should include whide local excision, followed by ipsilateral radical neck dissection.

Bilateral first branchial cleft cysts: case report and literature review.

Instances of first branchial cleft anomalies are quite uncommon. A patient with a first branchial cleft cyst was seen and surgically treated at UCLA Hospital in 1972. Three years later he developed a similar lesion on the opposite side of his neck and this also was surgically excised. A review of the literature has revealed only one prior report of bilateral first branchial cleft anomalies. The embryogenesis, diagnosis, and surgical management of these lesions are discussed in this paper.