Treatment of nasolabial fold rhabdomyosarcoma in children: A single-institution experience.

OBJECTIVES: To summarize the clinical characteristics and prognosis of children with nasolabial fold rhabdomyosarcoma (RMS). METHODS: Retrospective review of children treated for nasolabial fold RMS from January 2014 to September 2019. RESULTS: Of 21 patients with nasolabial fold RMS, 90.48% were alveolar subtype, in which PAX3/7-FOXO1 fusion positive accounted for 87.5%. Ten patients (47.62%) had nodals invasion. Almost all patients received comprehensive treatment (chemotherapy [100%], radiation therapy [100%], and surgery [95.24%]). The median follow-up time was 34.3 months. The 3-year overall survival (OS) and event-free survival (EFS) was 67.7% ± 14.1% and 42.1% ± 13.5%, respectively. Four patients had regional lymph node relapse (NR), all in the ipsilateral submandibular lymph node region. CONCLUSION: Majority of the patients with RMS in the nasolabial fold area were alveolar subtype and had positive PAX3/7-FOXO1 gene fusion. In addition, the nasolabial fold RMS had a high probability of regional lymph node metastasis in the submandibular area. To maintain the facial aesthetics and functions, the surgical area for nasolabial fold RMS is often very conservative and restricted. This could be one of the contributors for the poor prognosis of nasolabial fold RMS beside its worse pathological subtype and gene fusion.

Placa abollonada en surco nasogeniano / Nodular Plaque in the Nasolabial Fold

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Quiste de klestadt. Revisión de la literatura y presentación de un caso / Klestadt cyst. Review of the literature and presentation of a case

El quiste nasolabial es un quiste muy infrecuente, no odontogénico y que tradicionalmente se ha clasificado dentro de los llamados fisurados. Su etiología es desconocida, se supone que este origen puede estar en la línea de fusión de los procesos frontonasal y maxilares, por retención de restos ectodérmicos. Clínicamente se observa una tumefacción de crecimiento lento, consistencia blanda que produce elevación del ala de la nariz y es asintomático, a no ser que exista infección. En algunos casos de gran expansión se puede llegar a producir una reabsorción ósea del proceso alveolar. Para el diagnóstico son necesarias tomografías computerizadas o resonancias magnéticas, con la finalidad de estudiar los tejidos blandos. El tratamiento de la lesión habitualmente consiste en su resección quirúrgica a través de un acceso intraoral sublabial. Se preenta un caso clínico de una paciente de 66 años de edad que acude a consulta con un aumento de volumen de la narina izquierda y rinorrea derecha. A la exploración extraoral se observa un nódulo en el vestíbulo a nivel de la narina izquierda. Tras el estudio radiológico se conculye que la lesión es compatible con un quiste nasolabial, por lo que se procede asu exéresis mediante abordaje intraoral sublabial. Posteriormente, el análisis anatomopatlogico confirmó el diagnóstico de presunción

The nasolabial cyst is a very infrequent cyst, not odontogenic, that traditionally has been classified within those called fissured. Itsetiology is unknown and signifies that this origin can be in the fusion line of the frontonasal and maxillary processes, due to retention of ectodermal residue. Clinically, a swelling of slow growth and softconsistency is observed that produces the elevation of the lateral part of the nostril and it is asymptomatic, unless there is infection. In some cases of large expansion it can produce an osseous resorption of the alveolar process. For the diagnosis computerized tomographs or magnetic resonances are necessary, for the purpose of studying the soft tissues. The treatment of the lesion usually consists of surgical resection through an intraoralsublabial access. A clinical case is presented of a patient of66 years of age, who comes to the consultation with an increase of the volume of the left nostril and right rhinorrhoea. In the extraoral examination a nodule is observed in the vestibule at the level of the left nostril. After the radiological study it is concluded that the lesion is compatible with a nasolabial cyst, which was removed through a sublabial intraoral approach. Afterwards, the anatomopathological analysis confirmed the presumed diagnosis

Espiradenoma ecrino maligno en el surco nasolabial. Caso clínico / Malignant eccrine spiradenoma of the nasolabial fold. A case report

El espiradenoma ecrino maligno (EEM) es un tumor maligno poco frecuente de las glándulas sudoríparas ecrinas. Suele presentarse como un pequeño nódulo eritematoso, firme, solitario y doloroso. La cabeza y el cuello son una localización excepcional. Se desconoce la etiología aunque se considera que un traumatismo previo es un factor implicado. El EEM se origina sobre un espiradenoma benigno previo. La conducta clínica es agresiva con una elevada tasa de recidivas y metástasis a distancia. El pronóstico es infausto. El diagnóstico se basa en los hallazgos histológicos y el tratamiento ha de ser agresivo desde el principio para obtener los mejores resultados. Desde que, en 1956, Kersting y Helwig describieran el primer caso, y, en 1971, Beekley y cols. documentaran su transformación maligna, sólo se han publicado unos pocos casos. En función de estas características específicas, describimos a un hombre de 75 años de edad, en el que se estableció el diagnóstico de este tumor, originado en una localización poco habitual, con una histopatología y conducta peculiares(AU)

Malignant eccrine spiradenoma (MES) is a rare malignancy of the eccrine sweat glands. It usually presents as a small, firm, reddish painful and small solitary nodule. Head and neck are rare locations. Etiology is unknown although previous trauma is believed to be an implicated factor. MES arises over a prior benign spiradenoma. Clinical behavior is aggressive with a high rate of recurrences and distant metastases. Prognosis is poor. Diagnosis is based on histological findings and treatment must be aggressive from the beginning to achieve the best results. Since Kersting and Helwig first described the case in 1956, and Beekley et al., reported its malignant transformation in 1971, only a few cases can be found in the literature. Based on these particular features we report a case of a 75-year-old man diagnosed on a MES that arises in a very unusual location, with a peculiar histopathology and behavior(AU)