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