Tumores nasales congénitos de la linea media / Congenital nasal tumors of the midline in ENT

Las masas nasales congénitas de la línea media se presentan con una frecuencia muy baja ­1/20.000 a 1/40.000 nacidos vivos­. Se trata de hallazgos asintomáticos en el recién nacido y son resultado de anomalías congénitas del desarrollo embrionario, que suelen aparecer como masas en la línea media nasal en un punto cualquiera entre glabela y columela. Estas tumoraciones presentan un riesgo elevado de extensión al sistema nervioso central, lo que es especialmente importante tener en cuenta para prevenir consecuencias tales como la fístula de líquido cefalorraquídeo y/o la aparición de meningitis recidivante. Existen gran cantidad de tumores nasales de la línea media que aparecen en el recién nacido o en el lactante y que constituyen diagnósticos diferenciales de las lesiones congénitas antes descriptas. Describiremos brevemente los más frecuentes según nuestra experiencia. AU

Congenital nasal masses of the midline are very rare ­ 1/20,000 to 1/40,000 live births ­. Nasal tumors are asymptomatic findings in the neonate and are caused by congenital abnormalities during fetal development, usually appearing at the nasal midline between the glabella and columella. These tumors are associated with a high risk of extension to the central nervous system; therefore, it is especially important to prevent the development of a cerebrospinal fluid fistula and/or recurrent meningitis. There is a large number of nasal tumors of the midline in neonates or infants in the differential diagnosis of the above-described congenital lesion. Here we briefly describe the most common nasal tumors seen at our department. AU

Teratoma cervical congênito gigante: relato de caso e revisão quanto às opções terapêuticas / Giant congenital cervical teratoma: case report and review about therapeutic options

OBJETIVO:Relatar um caso de teratoma cervical congênito, destacando a gravidade e as dificuldades terapêuticas associadas. DESCRIÇÃO DO CASO: Mãe de 30 anos, com gestação por fertilização assistida. Com 23 semanas, diagnosticada malformação cervical fetal à direita. Parto cesáreo por indicação fetal com 31 semanas. Recém-nascido masculino, peso ao nascer de 1800g, Apgar 4 e 9, com volumoso processo expansivo à direita, ocupando toda a região cervical, comprometendo a mandíbula e estendendo-se para o terço superior do tórax. Com 40 horas de vida, apresentou insuficiência cardíaca congestiva de alto débito por roubo de fluxo pelo tumor. A partir de 54 horas de vida, houve progressiva deterioração hemodinâmica e respiratória, com hipotensão, anúria e labilidade de oxigenação, refratárias às aminas vasoativas, reposição de volume e aumento do suporte ventilatório. Indicada abordagem cirúrgica para ressecção tumoral, todavia o paciente não apresentou estabilidade clínica que permitisse seu transporte ao centro cirúrgico e faleceu com 70 horas de vida. COMENTÁRIOS: O caso demonstra as dificuldades relacionadas à abordagem pós-natal dos teratomas cervicais volumosos. Apesar do diagnóstico pré-natal, o paciente evoluiu com obstrução de vias aéreas, complicada por um choque cardiogênico refratário, que culminou no óbito. A abordagem intraparto é fundamental nesses pacientes, consistindo em exérese tumoral, enquanto a manutenção da circulação materno-fetal permite a oxigenação fetal contínua. A evolução neonatal no caso descrito é condizente com a literatura que mostra prognóstico reservado quando não é realizada a abordagem cirúrgica intraparto.

OBJECTIVE:To report a case of congenital cervical teratoma, highlighting the severity and the therapeutic difficulties associated. CASE DESCRIPTION: A 30-year old mother, with pregnancy by assisted fertilization. At 23 weeks, a cervical fetal malformation was diagnosed. A cesarean section was indicated with 31 weeks due to fetal distress. A male newborn infant with birth weight of 1800g and Apgar score of 4/9 presented a large right cervical tumor, with extensions to the mandible and to the upper chest. Patient presented congestive heart failure due to flow steal by the tumor 40 hours after birth, which caused progressive respiratory, hemodynamic and renal deterioration refractory to vasopressors, volume replacement and increased ventilatory support. Tumor resection was indicated, but the clinical instability of the patient did not allow his transport to the operating room and the infant died 70 hours after birth. COMMENTS: The case is representative of the difficulties related to postnatal treatment of bulky cervical teratomas. Despite prenatal diagnosis, the patient developed airway obstruction, complicated by refractory cardiogenic shock. The surgical approach during delivery is crucial for survival. Nowadays, management includes surgical removal of the tumor while maintaining the maternal-fetal circulation, allowing continuous fetal oxygenation. The clinical course described in the case is consistent with the literature that reports poor prognosis when the intra-partum surgical approach is not performed.

Large choroid plexus teratoma: A rare cause of congenital hydrocephalus.

Teratomas form the most common type of congenital brain tumors, frequently presenting as stillbirth. The largest neonatal series of intracranial teratomas reported a 12% survival rate. Although the first teratoma of the lateral ventricle was reported in 1961 by Maier, neonatal intracranial teratoma of the lateral ventricle is an extremely rare entity. We report here a large intracranial poorly differentiated teratoma arising from choroid plexus of lateral ventricle. This typically presented at birth with a large congenital hydrocephalus.

Congenital orbital teratoma presenting as microphthalmos with cyst.

We report a rare case of non-communicating large orbital cyst with microphthalmos which was surgically separated from the globe and excised. Histopathology reported it to be a teratoma. Congenital cystic teratoma should be a part of the differential diagnosis in an infant presenting with a clinical picture of microphthalmos with orbital cyst, in view of the different management required.

Oropharyngeal True Teratoma.

Oropharyngeal teratoma in newborn is very rare. Here we report a case of oropharyngeal true teratoma where a 17-day-old female baby presented with a protruding mass from oropharynx with episodic respiratory distress and feeding difficulty complicated by aspiration pneumonia, and treated successfully with coordinated team approach.

Extragonadal teratomas presenting at birth and originating from 3 different sites--a report of 3 cases.

Three cases of extragonadal teratomas presented at birth and all the cases arisen from three separate sites are reported in the present study. A huge sacrococcygeal teratoma is being reported and the other two retroperitoneal and nasopharyngeal teratomas, are reported for the rarity of their location in neonatal period. Teratomas of all three babies were mature benign on histopathology and surgical removal sufficed as mode of treatment. No recurrence was noted on follow-up.

Teratoma cístico da órbita: estudo clínico patológico: relato de caso / Cystic orbital teratoma: clinicopathologic study: case report

Apresentamos um caso de tumor orbitário congênito de grande tamanho, ocorrendo em criança recém-nascida. A paciente foi submetida a exenteração da órbita e o diagnóstico anatomopatológico foi de teratoma cístico. Os aspectos clínico-patológicos desta rara doença são comentados.

We report on a case of an congenital orbital tumor of impressive size, occurring in a newborn. The patient underwent orbital exenteration with a histopathologic diagnosis of cystic teratoma. The clinicopathological aspects of such a rare disease are commented.

Congenital Cavernous Sinus Cystic Teratoma

Teratomas represent 0.5% of all intracranial tumors. These benign tumors contain tissue representative of the three germinal layers. Most teratomas are midline tumors located predominantly in the sellar and pineal regions. The presence of a teratoma in the cavernous sinus is very rare. Congenital teratomas are also rare, especially those of a cystic nature. To our knowledge, this would be the first case report of a congenital, rapidly growing cystic teratoma within the cavernous sinus. A three-month-old boy presented with a past medical history of easy irritability and poor oral intake. A magnetic resonance image (MRI) scan of the head disclosed a large expanding cystic tumor filling the right cavernous sinus and extending into the pterygopalatine fossa through the foramen rotundum. These scans also demonstrated a small area of mixed signal intensity, the result of the different tissue types conforming to the tumor. Heterogeneous enhancement was seen after the infusion of contrast medium. However, this was a cystic tumor with a large cystic portion. Thus, a presumptive diagnosis of cystic glioma was made. With the use of a right frontotemporal approach, extradural dissection of the tumor was performed. The lesion entirely occupied the cavernous sinus, medially displacing the Gasserian ganglion and trigeminal branches (predominantly V1 and V2). The lesion was composed of different tissues, including fat, muscle and mature, brain-like tissue. The tumor was completely removed, and the pathological report confirmed the diagnosis of a mature teratoma. There was no evidence of recurrence. Despite the location of the lesion in the cavernous sinus, total removal can be achieved with the use of standard microsurgical techniques.

Teratoma facial / Facial teratoma

Ilustra un recién nacido del sexo masculino con presencia de una tumoración localizada en hemicara derecha de diez por nueve centímetros de tamaño y que ocupaba la región temporal, parotidea, geniana, maseterina, pre y retro auricular con desplazamiento del pabellón auricular hacia abajo y atrás.

Congenital nasopharyngeal immature teratoma: a first case report in Thailand.

The authors report the very rare case of a congenital immature teratoma arising from the nasopharyx in a full term female neonate. The tumor also extended to the oral cavity, particularly the right tonsillar fossa without intracranial involvement leading to upper airway obstruction and secondary Escherichia coli pneumonia. The immature part of the tumor in the head and neck region is not a poor prognostic indicator and chemotherapy is useless. In the presented case, the mass was widely excised without postoperative complications. To the best of our knowledge, this is the first reported case in Thailand.

Diagnóstico prenatal de teratoma sacrococcígeo, utilidad del ultrasonido y resonancia magnética / Sacrococcygeal teratoma prenatal diagnosis, utility of ultrasound and magnetic resonance image

El teratoma sacrococcígeo (TS) es una malformación de muy baja frecuencia y de alta letalidad. El objetivo de este trabajo es presentar un caso clínico de diagnóstico prenatal de teratoma sacrococcígeo, utilizando ultrasonido y resonancia magnética. Paciente de 21 semanas de gestación es enviada para segunda opinión a nuestro centro. Realizamos examen ultrasonográfico que reveló una lesión perineal quística predominante externa de gran tamaño, con componente presacro de menor volumen y escasos vasos sanguíneos en su pared, lo que sugiere el diagnóstico de teratoma quístico. Se realiza resonancia magnética que comprueba el diagnóstico de TS quístico predominantemente externo. El resto de la evaluación anatómica y hemodinámica fue normal. Evoluciona con aumento del tamaño de la lesión, sin complicaciones maternas o fetales. A las 38 semanas de gestación, se realiza operación cesárea que comprueba diagnóstico de TS, y a las 24 horas de vida se efectúa extirpación del tumor en su totalidad con resección del cóccix y parte del sacro, con plastía del defecto remanente. El diagnóstico prenatal de TS quístico, en nuestro caso, mediante el uso de ultrasonido y resonancia magnética permite por una parte predecir una evolución favorable durante la gestación, y por otra, posibilita el manejo multidisciplinario con interrupción programada y cirugía definitiva del TS.

Congenital cervical teratoma--an unusual presentation.

We report a case of congenital benign cervical teratoma in a female child. The unusual asymptomatic nature of the tumour and its relationship with the thyroid is highlighted.

Sacrococcygeal teratoma with anorectal malformation.

A 7-month-old child presented with imperforate anus, penoscrotal hypospadias and transposition, and a midline mucosa-lined perineal mass. At surgery the mass was found to be supplied by the median sacral artery. It was excised and the anorectal malformation was repaired by posterior sagittal anorectoplasty. Histologically the mass revealed well-differentiated colonic tissue. The final diagnosis was well-differentiated sacrococcygeal teratoma in association with anorectal malformation.

Congenital intracranial teratoma of the lateral ventricle.

A case of lateral ventricular teratoma in neonate, where near total excision of tumour was done, is being reported with the review of literature.

Congenital nasopharyngeal teratoma (epignathus).

The case report of a congenital nasopharyngeal teratoma presented as an intra-amniotic sac mass was described. Histologically, the tumor was composed of highly differentiated tissue derived from three germ layers. Those tissues were partially developed as infant organs with a central core made up by bone. Haphazardly arrangement of such tissues with disorganized sketal axis helped in differentiating the mass from an asymmetric twins. The evidence of tumor attachment was seen at the retro-uvular region.

Teratoma orbitario congénito. Presentación de un caso / Orbital congenital teratoma. Case report

Se presenta el caso clínico de paciente del sexo femenino de 24 horas de nacida, con teratoma de órbita izquierda, diagnosticada por su cuadro clínico radiológico característico y confirmado histopatológicamente, tratado quirúrgicamente con exenteración orbitaria, con seguimiento por nueve meses

Teratoma gástrico inmaduro con gliomatosis torácica en un niño / Gastric teratoma with pleural gliomatosis

Se informa un caso de teratoma gástrico congénito con gliomatosis pleural en un niño del sexo masculino a quien se le detectó un tumor abdominal que fue resecado de la curvatura mayor del estomago. Días después de la cirugía el niño murió y en la autopsia se encontraron implantes gliales en la superficie torácica del diafragma. La mayoría de los teratomas gástricos descritos en la literatura son tumores benignos sin capacidad de matástasis, la gliomatosis torácica asociada con teratoma gástrico no ha sido descrita anteriormente.