Tumor pardo del paladar, presentación atípica de hiperparatiroidismo primario en adolescente: buena evolución y normalización de la masa ósea tras paratiroidectomía / Brown tumor of the palate, atypical presentation of primary hyperparathyroidism in an adolescent: good evolution and bone mass normalization after parathyroidectomy

El hiperparatiroidismo primario (HPTP) es poco frecuente en niños y adolescentes. Hay escasos datos para el manejo de estos pacientes en pediatría. Las glándulas paratiroideas son glándulas endocrinas que secretan hormona paratiroidea (PTH) y regulan el metabolismo del calcio y del fósforo. La sobreexpresión de PTH se llama hiperparatiroidismo, que se clasifica en primario, secundario y terciario. En los adolescentes, 80 a 92% de los hiperparatiroidismos primarios se deben a adenoma paratiroideo. Presentamos el caso clínico de una adolescente con una primera manifestación atípica de HPTP, la presencia de un tumor pardo del paladar, presentación rara de adenoma paratiroideo, acompañado de hipercalcemia, marcada elevación de PTH y varias lesiones óseas. (AU)

Primary hyperparathyroidism (PHPT) in children and adolescents is uncommon. Guidelines for management in pediatric patients are limited. Parathyroid glands are endocrine glands that secrete parathyroid hormone (PTH) and regulate calciumphosphate metabolism. The overexpression of PTH is called hyperparathyroidism, and is classified as primary, secondary, and tertiary. In adolescents, 80 to 92% of PHPT cases are due to a parathyroid adenoma. We present here a case report of an adolescent with a brown tumor of the palate as the first manifestation of the disease, atypical and rare presentation of parathyroid adenoma in an adolescent. She had hypercalcemia, marked elevation of PTH and bone lesions. (AU)

Pleomorphic adenoma of the hard palate with calcifications: an unusual presentation / Adenoma pleomórfico del paladar duro con calcificaciones: una presentación inusual

Pleomorphic Adenoma (PA) is the most common benign salivary gland tumor. The most common sites for minor salivary gland from which PA arises are the palate followed by the lips and buccal mucosa. Calcifications are a common finding in major salivary glands with chronic inflammatory disorders. Major salivary gland tumors rarely show calcifications and it is less common to find them in minor salivary gland tumors. We report a case of pleomorphic adenoma of the hard palate in a 67-year-old female patient with intra-tumoral, irregular and scattered calcifications visible on computed tomography (CT). The treatment was complete surgical excision of the lesion. The diagnosis was confirmed with the histopathological study.

El adenoma pleomórfico (AP) es el tumor benigno de las glándulas salivales más común. Los sitios de mayor frecuencia donde surge el AP en glándulas salivales menores es el paladar seguido de los labios y la mucosa bucal. Las calcificaciones son un hallazgo común en las glándulas salivales mayores con trastornos inflamatorios crónicos, pero en el caso de tumores rara vez muestran calcificaciones y es menos común encontrarlos en tumores de las glándulas salivales menores. Presentamos un caso de adenoma pleomórfico del paladar duro en una paciente de 67 años con calcificaciones intratumorales, irregulares y dispersas visibles en la tomografía computarizada. El tratamiento fue la extirpación quirúrgica completa de la lesión. El diagnóstico se confirmó con el estudio histopatológico

Endoscopic-assisted maxillectomy: Operative technique and control of surgical margins.

BACKGROUND: When amenable to radical excision, cancer involving the maxilla is typically treated with maxillectomy followed by adjuvant therapy. Posterior tumor extension beyond the maxillary box leads to the invasion of complex areas, where achieving clear margins may be challenging. METHODS: Patients undergoing endoscopic-assisted maxillectomy for nasoethmoidal, maxillary, or hard palate cancer between 2007 and 2017 were included in the study. Surgical technique, margin status, and recurrences were analyzed. Extension of posterior resection was classified in 3 types (type 1: resection of the pterygopalatine fossa; type 2: resection of the pterygoid plates and related muscles; type 3: resection of the upper parapharyngeal space). The analysis of putative risk factors for involvement of margins and local recurrence was performed with special focus on the posterior and medial margin. RESULTS: The study included 79 patients (75 with available follow-up; mean: 20.6 months, range: 6-101 months), 37 (46.8%) of whom underwent type 1 resection, 34 (43.0%) type 2, and 8 (10.1%) type 3. According to pT category, 57 (72.2%) tumors were classified as T4a/T4b. Posterior and medial clear margins were achieved in 76/79 (96.2%) and 75/79 (94.9%) patients, respectively. T4b category, extension to the ethmoid, sphenoid sinus, pterygoid process, orbital cavity, and premaxillary tissues were significantly associated with a higher rate of margin involvement. None of the factors was significantly associated with medial margin involvement. CONCLUSION: Endoscopic-assisted maxillectomy combines several refinements including the facilitated detachment of the maxilla from the skull base and precise delineation of the posterior and medial margins of resection.

Um raro diagnóstico de osteoma periférico em palato duro: relato de caso / A rare diagnosis of peripheral osteoma in the hard palate: case report

Objetivo: relatar uma biópsia excisional de um osteoma periférico no palato duro direito de uma paciente jovem, do sexo feminino e não sindrômica. Relato de caso: paciente do sexo feminino, 32 anos de idade, melanoderma e normossitêmico, buscou atendimento no ambulatório de diagnóstico estomatológico da Faculdade de Odontologia da Universidade Federal de Uberlândia com queixa quanto ao surgimento de lesão na região de palato. Após avaliação clínica e imaginológica, foi decidido realizar biópsia excisional sob anestesia local da lesão. Foram levantadas três hipóteses de diagnóstico: osteoma periférico, exostose ou osteossarcoma. O material coletado foi enviado para análise histopatológica, que confirmou a hipótese de osteoma periférico. Considerações finais: os osteomas periféricos na região de palato duro são raríssimos. Essa lesão geralmente é assintomática ­ exceto quando há impacto traumático durante a mastigação ­, de crescimento lento, podendo se desenvolver em osso cortical ou medular. O tratamento de escolha é a remoção cirúrgica e o índice de recorrência é raro. O caso enfatiza a condição rara, pois foram encontrados apenas seis casos semelhantes relacionados na literatura. (AU)

Objective: To report an excisional biopsy of a peripheral osteoma in the right hard palate in a non-syndromic young female patient. Case Report: A 32-year-old female patient, black, and normosystemic sought assistance in the stomatological diagnosis outpatient clinic of the School of Dentistry of the Federal University of Uberlândia, Brazil, complaining about the appearance of a lesion in the palate region. After clinical and imaging assessment, it was decided to perform an excisional biopsy under local anesthesia of the lesion. Three diagnostic hypotheses were raised - peripheral osteoma, exostosis, or osteosarcoma. The material collected was sent for histopathological analysis, which confirmed the hypothesis of peripheral osteoma. Final considerations: Peripheral osteomas in the hard palate region are very rare. This lesion is usually asymptomatic, except when there is a slow-growing chewing impact during mastication, which may develop in cortical or spinal bone. Surgical removal is the treatment of choice and the rate of recurrence is rare. The case emphasizes the rare condition, considering only six similar cases were found in the literature. (AU)

Computed tomographic features of adenoid cystic carcinoma in the palate.

BACKGROUND: To evaluate the computed tomographic features and create a prediction model for clinical diagnosis of adenoid cystic carcinoma (ACC) in the palate with intact mucosa. METHODS: From March 2016 to May 2018, 102 patients with palatal tumors and intact mucosa, including 28 patients with a pathological diagnosis of ACC after surgery, were enrolled in this study. The patients' clinical symptoms, computed tomographic features and pathological diagnoses were recorded and analyzed. Independent predictors of ACC were determined by using univariate analysis and multivariate logistic regression, and the discrimination and calibration of the prediction model was evaluated, and internal validation was performed. RESULTS: Univariate analysis of patients showed that ACC patients were more likely than non-ACC patients to be older (P = 0.019); to have palatine bone destruction (P<0.001) and greater palatine foramen (GPF) enlargement (P<0.001); to have involvement of the pterygopalatine fossa (P<0.001), foramen rotundum (P<0.001), nasal cavity (P<0.001) and maxillary bone (P<0.001); and to have numbness (P = 0.007) and pain (P<0.001). Multivariate logistic analysis showed that age and GPF enlargement were independent predictors of ACC in palatal tumors. The diagnostic prediction model showed good discrimination and calibration, as evaluated by the area under the receiver operating characteristic curve (0.98) and the Hosmer-Lemeshow goodness-of-fit test (P = 0.927). CONCLUSIONS: The palate ACC prediction model based on age and GPF enlargement shows excellent discrimination with no evidence of poor calibration. Older patients with palatal tumors and intact mucosa should be considered for ACC when they have GPF enlargement.

Cleft Palate Repair Using Single Flap Palatoplasty in Patient With Associated Palatal Hemangioma.

Hemangiomas are the most common tumors of childhood with an average incidence of 10%. It is unusual for hemangiomas, which are already rarely seen isolated on the palate, to coexist with cleft palate as in the authors' patient. Four months old baby was admitted with isolated Veau 2 cleft palate and a red-purple colored raised lesion involving almost the whole right side of the hard palate. Magnetic resonance imaging revealed intensive contrasting solid nodule with a lobulated contour that was covering the right half of the hard palate, measuring 2.2×1.3 cm. To reduce the size of the vascular lesion 7 mg/d oral propranolol treatment was initiated. Following reduction in the size of hemangioma, the cleft palate was repaired at 11 months of age. Single mucoperiosteal flap from the left side of the palate preserving the major palatine artery was elevated whereas right mucoperisteal flap was minimally dissected not to interfere with the hemangioma. No intraoperative and postoperative complications in both the early and late term were experienced. As a result, since early palatal repair is important to obtain ideal speech outcomes in cleft patients, repair should be performed in similar patients with hemangiomas without delaying the timing.

Cribriform adenocarcinoma of the soft palate with multiple lymph node metastasis and long-term follow-up.

INTRODUCTION: Cribriform adenocarcinoma of the tongue and minor salivary glands (CATMSG) is a recently described entity, with most cases previously published as polymorphous low-grade adenocarcinoma (PLGA). Typical cases share some main characteristics, such as oral sites (mainly tongue), regional lymph node metastasis, and morphology resembling solid and follicular variants of papillary thyroid carcinoma. OBJECTIVE: To present a CATMSG and emphasize the importance of reclassifying PLGAs with unusual behavior. CASE REPORT: A 78-year-old male presented with an ulcerated mass in the soft palate treated as PLGA. The patient developed 5 regional metastases over 11 years of follow-up, all diagnosed as PLGA. He died due to the disease, and because of the very aggressive behavior of PLGA, all histopathologic slides were revised and the tumor was reclassified as CATMSG. CONCLUSION: This report emphasizes the importance of reevaluating aggressive PLGA and contributes to a better understanding of CATMSG.

Imaging of palatal lumps.

Palatal tumours are relatively rare and of variable aetiology, rendering radiological evaluation a daunting process for many. A systematic approach to the imaging of a palatal lump is therefore essential. The hard and soft palates are oral cavity and oropharyngeal structures, respectively. They have different tissue compositions, and therefore, lesions occur with different frequencies at each site. The hard palate has the highest concentration of minor salivary glands in the upper aerodigestive tract and most tumours here are salivary in origin, whereas most tumours at the soft palate are epithelial in origin, i.e., squamous cell carcinomas, in line with other oropharyngeal subsites. The most common malignant tumours of the palate, after squamous cell carcinoma, are minor salivary gland tumours, predominantly adenoid cystic and mucoepidermoid carcinomas. These tumours have a propensity to spread perineurally; understanding the anatomy and imaging features of perineural spread is vital, as it can have significant implications for patient management and tumour resectability. When confronted with a palatal lump, it is important to consider the following: its location on the hard or soft palate; whether it is mucosal or submucosal; the frequently occurring lesions at that site; the most suitable imaging techniques (ultrasound, computed tomography, magnetic resonance imaging); whether there are typical imaging features for any of the common lesions; and whether there are aggressive features, such as bone erosion or perineural spread. This approach allows the radiologist to narrow the differential diagnosis and assist the clinicians with planning treatment.