\s=b\ Deep, midline cervical cysts clinically diagnosed as thyroglossal duct cysts (TDCs), have been pathologically classified as dermoid cysts because of the presence of skin appendages and a squamous epithelial lining. In 75 midline cervical masses preoperatively diagnosed as TDC, we could classify only 54 as TDC, using the preexisting criteria of squamous or ciliated columnar epithelial lining associated with a tract or thyroid follicles. Eleven cysts were reclassified as dermoid, and six were called "mixed" because of features of both dermoid cysts (skin appendages) and TDC (epithelial tract or thyroid follicles). The morphological similarity of all these lesions suggests a common origin, perhaps from totipotential tissue entrapped during the descent of the embryonic thyroglossal duct from the base of the tongue. We conclude that these lesions should be grouped together under the eponym of "thyroglossal anomalies," and that treatment for all should consist of the Sistrunk procedure. (Arch Otolaryngol Head Neck Surg 1987;113:418-420) Certain midline cystic masses, clin¬ ically diagnosed as thyroglossal duct cysts (TDCs), do not fulfill the established histopathologic criteria for these lesions on microscopic exam¬ ination. The finding of skin append¬ ages in association with squamous epithelium, instead calls for the diagnosis of dermoid cyst. The treatment of choice for a TDC is the Sistrunk procedure, consisting of en bloc resec¬ tion of the midportion of the hyoid bone, while the surgical approach for a dermoid cyst is simple excision. Hitherto, the intraoperative differen¬ tiation of these midline neck cysts has been considered important because it dictates the surgical procedure to be selected. We reviewed the clinical and pathologic features of 75 midline cer¬ vical masses to determine whether the distinguishing characteristics were sufficiently clear as to warrant the different treatment protocols. PATIENTS AND METHODSA total of 75 cases with a preoperative diagnosis of TDC were retrieved from the surgical pathology files of Cardinal Glen¬ non Children's Hospital, St Louis. Patient medical records and operative notes were reviewed and information regarding depth of the cyst, attachment to bone, presence or absence of a tract, postoperative compli¬ cations, and incidence of recurrence were recorded.The surgical pathology reports and par¬ affin sections were examined, and infor¬ mation regarding the type of lining epithe¬ lium and presence or absence of skin appendages, tracts, attachment to bone, thyroid tissue, and mucous glands were recorded. Cysts lined by an epidermis with appendages, including hair, sebaceous glands, and eccrine glands, according to the criteria of Lever,1 were classified as dermoid cysts. Cysts lined by squamous or ciliated columnar epithelium, and associ¬ ated with a tract, thyroid follicles or attachment to bone, were classified as TDC. A "mixed" category was established to include those cysts fulfilling criteria for both dermoid cysts and TDC. These latter cysts were lined b...

The X-linked spinal and bulbar muscular atrophy (Kennedy’s disease) is a rare X-linked, recessive, lower motor neuron disease, characterized by weakness, atrophy, and fasciculations of the appendicular and bulbar muscle. The disease is caused by an expansion of the CAG repetition in the androgen receptor gene. Patients with Kennedy’s disease have more than 39 CAG repetitions. We report a case of 57-year-old man, resident of Monte Dourado (PA, Brazil) who complained of brachiocrural paresis evolving for 3 years along with fasciculations and tremors of extremities. In addition, he also developed dysarthria, dysphagia, and sexual dysfunction. The patient clinical picture included gait impairment, global hyporeflexia, proximal muscle atrophy of upper limbs, deviation of the uvula to right during phonation and tongue atrophy with fasciculations. The patient reported that about 30 years ago he had undergone gynecomastia surgery. His electroneuromyography suggested spinal muscular atrophy, and nuclear magnetic resonance imaging showed tapering of the cervical and thoracic spinal cord. Patient’s creatine kinase level was elevated. In view of the findings, an exam was requested to investigate Kennedy’s disease. The exam identified 46 CAG repetitions in the androgen receptor gene, which confirmed the diagnostic suspicion. This was the first case of Kennedy’s disease diagnosed and described in the Brazilian Amazon. To our knowledge only other four papers were published on this disease in Brazilian patients. A brief review is also provided on etiopathogenic, clinical and diagnostic aspects.

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Background: Brainstem abscess is a rare condition with a variety of treatment approaches. In this paper, we report an unusual case of a brainstem abscess with a positive outcome in an immunocompetent patient who was treated with antibiotic therapy. Case Description: A 22-year-old female presented with bilateral tetraparesis that was worse on the left hemibody, appendicular tremor, and left upper eyelid ptosis. Brain magnetic resonance imaging showed an abscess in the pons and midbrain due to possible nocardiosis. She was treated with dexamethasone, phenytoin, vancomycin, and meropenem for 8 weeks and trimethoprim-sulfamethoxazole for 6 weeks. The brain injury decreased, and the patient’s neurological status significantly improved. Conclusion: Brainstem abscess may be treated conservatively, leading to improvement of the clinical condition and decreased lesion size on imaging.

Background: Intracranial teratomas are rare and comprise about 0.5% of all intracranial tumors. Actually, a total of 15 cases of sellar-suprasellar teratoma have been described in the last 26 years. Teratomas occur more frequently in children and young adults than in the older population and in males as compared to females. Symptoms at diagnosis are usually neurological defects, diabetes insipidus, and hypopituitarism. Teratoma diagnosis can be suggested through neuroimaging findings. Magnetic Resonance Imaging (MRI) remains the preferred modality. Neuro-radiological findings of mixed-density mass, usually with mixed cystic and solid components inclusions of teeth, fat, and calcification can be suggestive. Tumor markers as beta-HCG and alpha-FP can be useful. Optimal treatment for mature teratoma is neurosurgical excision and in cases of immature teratomas or mixed germ cells tumors teratomas, a multi-step treatment is required with neurosurgical excision, adjuvant or neo-adjuvant radiotherapy and chemotherapy. Clinical Case : A 15-year-old woman, with a headache, secondary amenorrhea, syncope, progressive visual loss, polydipsia, and polyuria due to diabetes insipidus. A craniopharyngioma or another parasellar mass was suspected, an MRI showed an expansive suprasellar process with a cystic component with chiasm compression. A hormone panel showed corticotrophic and thyrotrophic deficiencies. Hormone replacement was started and the patient was referred for surgery. The immediate postoperative progressed well, although one month later she presented recurrence of the compressive symptoms (a headache and visual loss). She was submitted to second surgery. According to the pathology, the mass was identified as a suprasellar immature teratoma, with germ component, as well as cystic areas (ki67: 80%). In addition, tumor markers tests were requested: beta-HCG 5.47mIU/mL (<5.0), alpha-FP 3.1ng/mL (<7.0), DHL 635U/L (240-480). The patient was staged and referred to oncology unit that indicated radiotherapy, as a postoperative adjuvant therapy. At the most recent follow-up, approximately 9 months later, MRI shows a residual, stable tumor and patient presents with bitemporal hemianopsia. Conclusion: Intracranial germ cell tumors are uncommon, with only 15 cases of sellar-suprasellar teratoma described in the literature. It is important to recognize this entity in the differential diagnosis once they mimic many other lesions. Future reports on this rare location for a teratoma may help ascertain long-term outcomes. Reference: Chiloiro, S., Giampietro, A., Bianchi, A., & De Marinis, L. (2015). Clinical management of teratoma, a rare hypothalamic-pituitary neoplasia. Endocrine, 53(3), 636-642. doi:10.1007/s12020-015-0814-4