Leiomiosarcoma radioinducido de la región posterior del cuello / Radiation-induced leiomyosarcoma of the posterior neck region

Los leiomiosarcomas son tumores mesenquimales malignos, que se originan en las células del músculo liso. Las localizaciones de aparición más frecuentes son el miometrio y el tracto gastrointestinal. En la región de cabeza y cuello los leiomiosarcomas aparecen de forma excepcional. Presentamos el caso de un leiomiosarcoma en la región posterior del cuello, en un paciente radiado 20 años antes por un tumor de nasofaringe. La incidencia de este tipo de tumores que cumple los criterios de tumor radioinducido se sitúa entre el 0,035 y el 0,2%. Los sarcomas radioinducidos son difíciles de diagnosticar en estadios tempranos, debido a la induración y la fibrosis de la zona radiada y a la sintomatología inespecífica que presentan. Su pronóstico es malo (AU)

Leiomyosarcomas are mesenchymal malignant tumours that appear in smooth muscle cells. Their most frequent locations are the uterus and gastrointestinal tract. Their occurrence in head and neck is considered exceptional. We present a patient with a posterior neck region leiomyosarcoma who had received radiation for a nasopharyngeal carcinoma 20 years earlier. The incidence ratio of these tumours in radiated patients (therefore considered radiation-induced) ranges from 0,035 to 0,2%. Radiation-induced sarcomas are difficult to diagnose due to the induration and fibrosis in the radiated area and the non-specific symptoms that they present. Their prognosis is very poor (AU)

Radiation-induced leiomyosarcoma of the posterior neck region.

INTRODUCTION: Leiomyosarcomas are mesenchymal malignant tumours that appear in smooth muscle cells. Their most frequent locations are the uterus and gastrointestinal tract. Their occurrence in head and neck is considered exceptional. We present a patient with a posterior neck region leiomyosarcoma who had received radiation for a nasopharyngeal carcinoma 20 years earlier. The incidence ratio of these tumours in radiated patients (therefore considered radiation-induced) ranges from 0,035 to 0,2%. Radiation-induced sarcomas are difficult to diagnose due to the induration and fibrosis in the radiated area and the non-specific symptoms that they present. Their prognosis is very poor.

Infecciones cervicales profundas. Revisión de 286 casos / Deep neck infection. Review of 286 cases

Introducción: Las infecciones cervicales profundas (ICP) son afecciones potencialmente letales y susceptibles de complicaciones graves. En este estudio se constata el aumento de la incidencia y se trata de buscar las causas. Más del 30% son idiopáticas, pero suelen relacionarse con infecciones dentarias y orofaríngeas. Material y métodos: Se presentan 286 ICP consecutivas en un estudio retrospectivo de los últimos 11 años en nuestro centro y se analizan antecedentes y enfermedades concomitantes y aspectos diagnósticos y terapéuticos de los pacientes que pueden influir en el desarrollo de las ICP. Resultados: Se objetiva un repunte de la incidencia de las ICP en nuestro medio. Las complicaciones más frecuentes fueron mediastinitis, shock séptico con coagulación intravascular diseminada, fascitis necrotizante y compromiso respiratorio agudo. Las secuelas más frecuentes observadas fueron la disfagia por parálisis de pares craneales bajos y neuroencefalopatía. Se produjeron un 10% de neumonías aspirativas. La mortalidad por ICP en los adultos es del 5,9% y en los niños del 6,2%. Conclusiones: Las ICP debe ser consideradas una urgencia médicoquirúrgica. Son susceptibles de complicaciones graves en cuestión de horas. Debemos estar atentos a la aparición de síntomas de alarma como: disnea, estridor, dolor a la palpación en el suelo de la boca, sialorrea, trismus, etc.El avance en los diversos procedimientos diagnósticos (imagen, microbiología) y terapéuticos (antibióticos, cirugía), así como la mejor atención del paciente crítico (UCI- REA), han sido decisivos en el diagnóstico y tratamiento precoz, identificar y tratar las complicaciones y mejorar el pronóstico y la mortalidad de los pacientes (AU)

Introduction: Deep neck infections (DNI) are potentially lethal processes and are susceptible to severe complications. This study shows an increment of the incidence and investigated the cause. More than 30% of cases are idiopathic, but they are commonly related to a dental or oropharyngeal infection. Material and methods: We present 286 consecutive cases in a retrospective 11-year study. We analysed the clinical picture, antecedents and concurrent diseases, and diagnostic and therapeutic approaches that could be related to developing a DNI. Results: A mayor increase in DNI incidence in our setting was seen in the last few years. The mayor complications were mediastinitis, septic shock with disseminated intravascular coagulation syndrome, necrotising fasciitis and acute respiratory failure. A lower cranial nerve palsy that develops into dysphagia and leukoencephalopathy are the most frequent sequela. We found 10% of aspiration pneumonia. Mortality in adults was 5.9% and in children, 6.2%. Conclusions: Deep neck infections constitute a medical and surgical emergency. Severe complications may arise in a short time. We must be vigilant to alarm symptoms such as dyspnea, stridor, pain in the floor of the mouth, sialohrrea, trismus, etc. Improvements in antibiotic therapy, diagnostic imaging and critical patient support modalities have decreased mortality and there is a better prognosis, with complications being identified and treated earlier (AU)

Deep neck infection. Review of 286 cases.

INTRODUCTION: Deep neck infections (DNI) are potentially lethal processes and are susceptible to severe complications. This study shows an increment of the incidence and investigated the cause. More than 30% of cases are idiopathic, but they are commonly related to a dental or oropharyngeal infection. MATERIAL AND METHODS: We present 286 consecutive cases in a retrospective 11-year study. We analysed the clinical picture, antecedents and concurrent diseases, and diagnostic and therapeutic approaches that could be related to developing a DNI. RESULTS: A mayor increase in DNI incidence in our setting was seen in the last few years. The mayor complications were mediastinitis, septic shock with disseminated intravascular coagulation syndrome, necrotising fasciitis and acute respiratory failure. A lower cranial nerve palsy that develops into dysphagia and leukoencephalopathy are the most frequent sequela. We found 10% of aspiration pneumonia. Mortality in adults was 5.9% and in children, 6.2%. CONCLUSIONS: Deep neck infections constitute a medical and surgical emergency. Severe complications may arise in a short time. We must be vigilant to alarm symptoms such as dyspnea, stridor, pain in the floor of the mouth, sialohrrea, trismus, etc. Improvements in antibiotic therapy, diagnostic imaging and critical patient support modalities have decreased mortality and there is a better prognosis, with complications being identified and treated earlier.

[Merkel cell carcinoma: a presentation of 5 cases and a review of the literature]. / Carcinoma de células de Merkel: presentación de 5 casos y revisión de la literatura.

Merkel cell carcinoma is an unusual, aggressive skin tumour, with a tendency to recurrence after its surgical extirpation. Five cases of tumours in the cervicofacial region seen at our Centre in the last five years are presented, along with a review of the literature, focusing on its etiopathogeneis, approach and treatment.

Mucormicosis rinoorbitocerebral, un estudio retrospectivo de 7 casos / Rhino-orbito-cerebral mucormycosis, a retrospective study of 7 cases

La mucormicosis es una infección oportunista, producida por los hongos del orden mucorales. Tiene una baja incidencia. Es potencialmente letal y afecta a pacientes inmunocomprometidos. Presentamos 7 casos de mucormicosis rinocerebral en un estudio retrospectivo de 8 años (2000–2008) en pacientes hematológicos. Es preciso realizar un diagnóstico precoz, para lo cual se ha de mantener un alto índice de sospecha clínica en pacientes con factores predisponentes. El diagnóstico de certeza requiere la realización de cultivos o biopsias de las zonas afectadas que demuestren la invasión de los tejidos por las hifas características. La clave del tratamiento es el desbridamiento quirúrgico precoz y agresivo, junto con altas dosis de amfotericina B intravenosa. A pesar de este tratamiento, el pronóstico es desfavorable y la mortalidad es de un 70–80% (AU)

Mucormycosis is an opportunistic fungal infection caused by fungi of the Mucorales order. It has a low incidence and is a potentially lethal infection which generally affects patients who are immunocompromised due to systemic disease. We report 7 cases of rhinocerebral mucormycosis in a retrospective study of 8 years (2000–2008) in hematologyc patients. An early diagnosis is essential, and therefore there must be a high level of clinical suspicion in patients with predisposing factors. Certainty diagnosis requires fungal cultures or biopsies of the affected areas which prove an invasion of the tissues by the characteristic hyphae. The key to treatment is early and aggressive surgical treatment, together with high intravenous doses of B amphotericyn. Despite this, prognosis is poor and mortality is about 70–80% (AU)

Rhino-orbito-cerebral mucormycosis, a retrospective study of 7 cases.

Mucormycosis is an opportunistic fungal infection caused by fungi of the Mucorales order. It has a low incidence and is a potentially lethal infection which generally affects patients who are immunocompromised due to systemic disease. We report 7 cases of rhinocerebral mucormycosis in a retrospective study of 8 years (2000-2008) in haematological patients. An early diagnosis is essential, and therefore there must be a high level of clinical suspicion in patients with predisposing factors. Certainty diagnosis requires fungal cultures or biopsies of the affected areas which prove an invasion of the tissues by the characteristic hyphae. The key to treatment is early and aggressive surgical treatment, together with high intravenous doses of amphotericin B. Despite this, prognosis is poor and mortality is about 70-80%.

[Small round cell desmoplastic tumour. Atypical morphology in the sub-maxillary gland]. / Tumor desmoplásico de células pequeñas y redondas. Morfología atípica en la glándula submaxilar.

Desmoplastic small round cell tumour (DSRCT) is a rare disease usually affecting young males. There are no other articles with a sub-maxillary location. The tumour consists of nests and masses of undifferentiated small round cells embedded in a desmoplastic stroma. The co-expression of epithelial, muscular and neuronal antigens distinguishes this entity from other small round cell tumours. The t(11;22)(p13;q12) translocation is a recurrent characteristic of this type of tumour. We report a case of desmoplastic small round cell tumour of the sub-maxillary gland, with an evolution of 8 months, affecting a 36 year old male. He suffered chronic lymphatic leukaemia five years ago and needed a bone marrow transplant. There was a 4x3 cm tumour. There were no signs of malignancy on the CT scan. A right sub-maxillectomy was performed. The pathology analysis gave a diagnosis of DSRCT. Post-surgical radiotherapy was given. The definitive diagnosis was reached using immunohistochemical techniques, such as polyphenotypical differentiation (epithelial, mesenchymal and neural), and by demonstration of translocation (11;22)(p13;q12). Sub-maxillary location is very rare.

Tumor desmoplásico de células pequeñas y redondas. Morfología atípica en la glándula submaxilar / Small round cell desmoplastic tumour. Atypical morphology in the sub-maxillary gland

El tumor desmoplásico de células pequeñas redondas (TDCPR) es una neoplasia infrecuente que afecta a varones jóvenes y se localiza en el abdomen principalmente. La localización submaxilar es extremadamente infrecuente y no hemos encontrado ningún caso en la literatura. La histología típica se caracteriza por nidos de células tumorales embebidas en una estroma fibromixoide densa. Su principal característica diagnóstica es la coexpresión de marcadores epiteliales, mesenquimales y neurales y la translocación recíproca t(11;22)(p13;q12). Presentamos el caso de un varón de 36 años con antecedente de leucemia linfática crónica y trasplantado de médula ósea con una tumoración submaxilar derecha de 8 meses de evolución. Exploración física: masa dura de 4x3 cm. La tomografía computarizada no muestra signos de malignidad. Se realiza submaxilectomía derecha. El análisis anatomopatológico reproduce la morfología y el inmunofenotipo característicos del TDCPR. Se completa el tratamiento con radioterapia. El diagnóstico definitivo se basa en la demostración inmunohistoquímica de diferenciación polifenotípica (epitelial, mesenquimal, neural), y en la demostración citogenética de la translocación (11;22)(p13;q12). La localización submaxilar es excepcional (AU)

Desmoplastic small round cell tumour (DSRCT) is a rare disease usually affecting young males. There are no other articles with a sub-maxillary location. The tumour consists of nests and masses of undifferentiated small round cells embedded in a desmoplastic stroma. The co-expression of epithelial, muscular and neuronal antigens distinguishes this entity from other small round cell tumours. The t(11;22)(p13;q12) translocation is a recurrent characteristic of this type of tumour. We report a case of desmoplastic small round cell tumour of the sub-maxillary gland, with an evolution of 8 months, affecting a 36 year old male. He suffered chronic lymphatic leukaemia five years ago and needed a bone marrow transplant. There was a 4x3 cm tumour. There were no signs of malignancy on the CT scan. A right sub-maxillectomy was performed. The pathology analysis gave a diagnosis of DSRCT. Post-surgical radiotherapy was given. The definitive diagnosis was reached using immunohistochemical techniques, such as polyphenotypical differentiation (epithelial, mesenchymal and neural), and by demonstration of translocation (11;22)(p13;q12). Sub-maxillary location is very rare (AU)

[Diagnosis and evaluation of head and neck paragangliomas. Clinical and biological manifestations]. / Diagnóstico y control evolutivo de los paragangliomas cervicocefálicos. Manifestaciones clínicas y biológicas.

Head and neck paragangliomas are slow-growing tumors and the initial symptoms are sometimes non-specific, often hampering and delaying diagnosis. These tumors may be asymptomatic, even when they have reached a considerable size. Symptomatology is highly varied in terms of anatomical location, stage (degree of invasion, local involvement and the presence of metastasis) and catecholamine secretion (pheochromocytoma), which not only produces a series of systemic manifestations but also serves as a guide to the search for specific genetic diseases, of which these tumors may be a component. Thus, in addition to identifying their anatomical location, excess catecholamine production must be assessed and genetic diagnosis must be completed before surgery is performed.

Diagnóstico y control evolutivo de los paragangliomas cervicocefálicos. Manifestaciones clínicas y biológicas / Diagnosis and evaluation of head and neck paragangliomas. Clinical and biological manifestations

Los paragangliomas cervicocefálicos son tumores de crecimiento lento y clínica inicial a veces muy inespecífica, lo que dificulta y retrasa su diagnóstico en muchas ocasiones. Pueden ser asintomáticos, incluso cuando ya alcanzan tamaños considerables. La semiología es muy variada en función de su localización anatómica y su estadio evolutivo (grado de invasión, compromiso local y existencia de metástasis) y de su capacidad para secretar catecolaminas (feocromocitoma), lo cual no sólo producirá una serie de manifestaciones sistémicas, sino que nos orientará en la búsqueda de determinadas enfermedades genéticas de lasque estos tumores pueden ser un componente más. Así pues, además del diagnóstico de localización, estos tumores entrañan la dificultad de descartar previamente a su intervención el exceso de producción de catecolaminas y completar el diagnóstico genético en caso de sospecha (AU)

Clinical and biological manifestations Head and neck paragangliomas are slow-growing tumors and the initial symptoms are sometimes non-specific, often hampering and delaying diagnosis. These tumors may be asymptomatic, even when they have reached a considerable size. Symptomatology is highly varied in terms of anatomical location, stage (degree of invasion, local involvement and the presence of metastasis) and catecholamine secretion (pheochromocytoma), which not only produces a series of systemic manifestations but also serves as a guide to the search for specific genetic diseases, of which these tumors may be a component. Thus, in addition to identifying their anatomical location, excess catecholamine production must be assessed and genetic diagnosis must be completed before surgery is performed (AU)

[PANDAS Syndrome: a new tonsillectomy indication?]. / Síndrome PANDAS: ouna nueva indicación de amigdalectomía?

PANDAS Syndrome (Paediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcus) is a rare disease described in 1998. In this disease, there is a relationship between group A beta haemolytic streptococcal tonsil infections and the exacerbation of neuropsychiatric disorders. A case report of a 9-year-old child with PANDAS syndrome is presented. This child has had no further symptoms after tonsillectomy. The understanding about PANDAS syndrome and tonsillectomy is reviewed.

Síndrome PANDAS: ¿una nueva indicación de amigdalectomía? / PANDAS syndrome: a new tonsillectomy indication?

El síndrome PANDAS (pediatric autoinmune neuropsychiatric disorders associated with Streptococcus) es una entidad rara que se describió en 1998. Los pacientes con esta afección presentan una exacerbación de los síntomas neuropsiquiátricos coincidiendo con las infecciones amigdalares causadas por Streptococcus betahemolítico del grupo A. Presentamos el caso clínico de un paciente de 9 años con síndrome PANDAS sometido a amigdalectomía que mejoró de su sintomatología tras la intervención. Del mismo modo revisamos la literatura sobre esta entidad

PANDAS Syndrome (Paediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcus) is a rare disease described in 1998. In this disease, there is a relationship between group A beta haemolytic streptococcal tonsil infections and the exacerbation of neuropsychiatric disorders. A case report of a 9-year-old child with PANDAS syndrome is presented. This child has had no further symptoms after tonsillectomy. The understanding about PANDAS syndrome and tonsillectomy is reviewed

[Facial paralysis of infectious origin in patients receiving cochlear implants]. / Parálisis facial de origen infeccioso en paciente intervenido de implante coclear.

Peripheral facial paralysis during cochlear implant surgery appears in 0.43 % of adults and 0.39 % of children. Peripheral facial paralysis secondary to acute otitis media is very rare in adults, while the incidence in children remains between 0.19 % and 0.45 %. We present 3 cases of patients who underwent cochlear implant surgery at our department, and who presented peripheral facial paralysis secondary to acute otitis media. The procedure is similar to facial paralysis secondary to acute otitis media in children. Treatment consists of parenteral antibiotic and corticosteroid treatment. Prognosis is favourable, with a total recovery of facial function in 1 or more months.

Parálisis facial de origen infeccioso en paciente intervenido de implante coclear / Facial paralysis of infectious origin in patients receiving cochlear implants

La parálisis facial periférica durante la cirugía del implante coclear se presenta en el 0,43 % de los adultos y en el 0,39 % de los niños. La parálisis facial periférica secundaria a otitis media aguda es excepcional en el adulto. En el niño tiene una incidencia del 0,19-0,45 %. Presentamos 3 casos clínicos de pacientes sometidos a cirugía de implante coclear en nuestro servicio, que sufrieron parálisis facial periférica en el contexto de una otitis media aguda. El manejo es semejante a la parálisis facial secundaria a otitis media aguda en el niño. El tratamiento consiste en antibioterapia y corticoterapia parenterales. El pronóstico es bueno, con una recuperación completa de la función facial en uno o varios meses

Peripheral facial paralysis during cochlear implant surgery appears in 0.43 % of adults and 0.39 % of children. Peripheral facial paralysis secondary to acute otitis media is very rare in adults, while the incidence in children remains between 0.19 % and 0.45 %. We present 3 cases of patients who underwent cochlear implant surgery at our department, and who presented peripheral facial paralysis secondary to acute otitis media. The procedure is similar to facial paralysis secondary to acute otitis media in children. Treatment consists of parenteral antibiotic and corticosteroid treatment. Prognosis is favourable, with a total recovery of facial function in 1 or more months