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.

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.

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.

[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.

[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.

[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.