[Ototoxicity in cancer survivors: experience and proposal of a surveillance protocol]. / Ototoxicidad en pacientes oncológicos: experiencia y propuesta de un protocolo de vigilancia.

INTRODUCTION: Ototoxicity occurs in different percentages in patients after treatment with platinum-based chemotherapy or cranial radiation therapy. The aim of this study was to present experience in ototoxicity monitoring. MATERIAL AND METHODS: A review was made of the registry of paediatric cancer patients referred to the Children's Hearing Loss Unit from 1999 to 2019. RESULTS: Of the 46 patients referred to this unit, 41 had received platinum as part of their treatment, 17 patients underwent neurosurgery, and 18 patients received cranial radiation therapy. An anamnesis and otoscopy were performed on all of them, and the monitoring was carried out with tone-verbal audiometry and/or distortion products. Hearing loss was observed in eight patients (21.05% of patients referred for audiological follow-up) as a consequence of the treatment. It was impossible to determine the audiological situation in eight patients at the end of treatment. Hearing aid adaption was necessary in two patients. In coordination with Paediatric Oncology, a change from cisplatin to carboplatin due to bilateral grade two ototoxicity was considered appropriate during treatment in one patient. CONCLUSION: Adequate coordination with Paediatric Oncology is essential to carry out active surveillance for ototoxicity and to modify, if possible, the dosage or type of chemotherapy in case hearing is affected. In our experience, and following current recommendations, a pre-treatment assessment is usually performed, as well as monitoring during treatment, at the end of treatment, and annually thereafter due to the risk of a later development of hearing loss.

Intestinal-type sinonasal adenocarcinomas. Immunohistochemical profile of 66 cases.

INTRODUCTION AND OBJECTIVES: Intestinal-type sinonasal adenocarcinomas are malignant epithelial tumours. Around 8-25% of all sinonasal malignant tumours are intestinal-type adenocarcinomas, which are related to wood dust exposure. Four histological subtypes have been described: papillary, colonic, solid and mucinous. We performed a pathological and immunohistochemical study in order to describe characteristics with prognostic, diagnostic and therapeutic value, and also to compare our results with previous studies. METHODS: Sixty six tumour samples were analysed and protein expression of p53, p16, E-cadherin, ß-catenin, epidermal growth factor receptor (EGFR), human epidermal growth factor receptor 2 (HER2/neu) and cyclooxygenase-2 (COX-2) was performed by tissue microarray blocks. RESULTS: The 63% of cases were p53 positive; 37% showed nuclear staining with ß-catenin and 100% with E-cadherin, while 98% showed membrane staining with ß-catenin, 7% with EGFR, 8% with HER2/neu and 52% with COX-2; and 59% of the cases lost p16 expression. CONCLUSIONS: Intracranial invasion was the worst prognostic associated event. Solid and mucinous tumours were the most aggressive histological subtypes. Intracranial invasion was more frequent in mucinous subtype tumours. Immunohistochemical results were similar in all tumour subtypes, except for mucinous tumours, which showed weak expression of E-cadherin and ß-catenin. Comparing with previous studies, we found a lower expression of EGFR, HER2/neu and COX-2. The p16 expression was associated with worse survival and metastatic disease.

Medullary thyroid carcinoma and 2q37 deletion in a patient with nevoid basal cell carcinoma syndrome: clinical description and genetic analysis.

BACKGROUND: Nevoid basal cell carcinoma syndrome (NBCCS) is a rare, inheritable, multisystem disorder characterized by numerous basal cell carcinomas (BCCs), maxillary keratocyst, and musculoskeletal malformations. Occasionally, it is associated with malignancies like rhabdomyoma, melanoma, and sinonasal undifferentiated carcinoma, to name a few. METHODS: A patient presented with NBCCS with a medullary thyroid carcinoma. Clinical, surgical details, and germline genetic analysis are herein described. RESULTS: A 32-year-old woman was referred to our department with suspicion of medullary thyroid carcinoma, which was confirmed by histopathological examination. The patient was diagnosed as also having NBCCS. Germline mutation analysis indicated wild-type genes PTCH1 and RET. DNA copy number analysis by high resolution microarray comparative genomic hybridization (CGH) revealed a small interstitial loss at chromosomal band 2q37.3. CONCLUSION: To our knowledge, this is the first described patient with NBCCS carrying a medullary thyroid carcinoma and a 2q37 deletion, which confirms that this syndrome can be associated with many different malignancies.

[Analysis of microsatellite instability in laryngeal squamous cell carcinoma]. / Análisis de la inestabilidad de microsatélites en el carcinoma escamoso de laringe.

INTRODUCTION AND OBJECTIVES: The literature on the involvement of microsatellite instability in head and neck squamous cell carcinoma shows great variability, probably due to differences in the testing methods. Using a consensus detection system, we aimed to reach a reliable estimate of microsatellite instability prevalence in a subset of head and neck squamous cell carcinoma cases. METHODS: The microsatellite instabilityI status of 43 patients with previously untreated primary laryngeal squamous cell carcinomas was analyzed by a multiplex polymerase chain reaction assay including 5 mononucleotide repeat markers. RESULTS: Thirty-six cases showed a stable phenotype or a microsatellite stable phenotype (83.7%) and 7 cases (16.3%) showed an microsatellite instability-positive phenotype. One case showed instability in 3 of 5 markers, 1 case in 2 markers and 5 cases in 1 marker. The microsatellite instability-positive and stable cases did not differ with respect to age, tumour stage, lymph node or distant metastases. CONCLUSIONS: Our data showed that a proportion of laryngeal squamous cell carcinomas are microsatellite instability positive. Knowledge of microsatellite instability patient status will allow adjusting anticancer therapy at an individual level.

Solitary fibrous tumor of the petrous bone: a successful treatment option.

The patient was a 48-year-old man complaining about right hemicranial pain and deafness of 1 year's duration. MRI showed a solid well-vascularized mass destroying the mastoid and petrous bone, and with an epidural component that pulled the right cerebellar hemisphere. The patient underwent a right modified type A infratemporal approach, which allowed complete resection of the tumor, with a low morbidity. The pathological diagnosis was solitary fibrous tumor. The patient received complementary treatment with radiotherapy. At 24 months after the initial treatment the patient is free of disease. We conclude that solitary fibrous tumor of the petrous bone can be satisfactorily treated with surgical excision followed by radiotherapy, with low morbidity and excellent facial function. To our knowledge this is the first description of a solitary fibrous tumor of the petrous bone. Due to the lack of consensus in treating rare tumors, we want to offer this management approach for treating this kind of tumor.

Malignant triton tumor of the infratemporal fossa.

Malignant triton tumor is a very aggressive type of sarcoma that comprises rhabdomyoblasts and malignant Schwann cells. It is a different entity from malignant schwannoma, characterized by their aggressiveness and poor prognosis. Head and neck location is frequent, and early diagnosis and complete resection followed by radiation therapy is important for long-term survival. However, the therapeutic plan should be individualized, taking into account the location and size of the primary tumor. The use of adjuvant chemotherapy and molecular therapies should be considered in the treatment of these tumors. We report an unusual presentation of a malignant triton tumor located in the infratemporal fossa, describing its clinical and pathologic features, and we try to update the knowledge in the management of these tumors, including the use of molecular therapies.

Conservative treatment of retropharyngeal and parapharyngeal abscess in children.

Parapharyngeal and retropharyngeal infections, which can potentially cause life-threatening complications, could be treated conservatively with no need for surgical drainage. A retrospective analysis of all patients diagnosed with retropharyngeal and parapharyngeal infections was performed. Information regarding age, sex, presenting symptoms, physical examination, laboratory and imaging evaluations, management, duration of hospital stay, and complications were reviewed. Seven children were identified, 4 with retropharyngeal abscess and 3 with parapharyngeal abscess. All but 1 patient were under 7 years old, and all were treated with intravenous amoxicilin/clavulanic acid and corticosteroids. Torticollis and fever were present in all the patients. The mean length of hospital stay was 7 days. There were no complications associated. We demonstrate that retropharyngeal and parapharyngeal abscesses can be treated medically, reserving the surgical drainage for complicated cases. Treatment with intravenous antibiotics and corticosteroids is a safe option, reducing the durations of symptoms and the length of hospital stay.

[Primary tumours of the parapharyngeal space. Our experience in 51 patients]. / Tumores primarios del espacio parafaríngeo. Nuestra experiencia en 51 pacientes.

INTRODUCTION AND OBJECTIVES: Tumours of the parapharyngeal space are rare, representing only 0.5 % of head and neck oncology. The aim of this study is to present our experience with the diagnosis and therapies for these tumours. PATIENTS AND METHOD: This study is a retrospective review of 51 patients diagnosed as having tumours of the parapharyngeal space and treated surgically between 1984 and 2006. Tumours originating in the deep lobe of parotid gland were excluded. RESULTS: Seventy percent of the parapharyngeal space neoplasms were benign and thirty percent malignant. Pleomorphic adenoma was the most common neoplasm (37 %), followed by miscellaneous benign tumours (34 %), paraganglioma (21 %), and neurogenic tumours (8 %). Surgery was chosen in all case, with the transcervical approach used in 32 cases, the cervical-transparotid approach in 13 cases, the transmandibular approach in 4 cases (with mandibulectomy in 1 patient), the transoral approach in 1 case, and the infratemporal fossa approach in 1 case. The most common complications were those deriving from damage to nerve structures. CONCLUSIONS: Most parapharyngeal space tumours can be removed surgically with a low rate of complications and recurrence. The transcervical approach is the most frequently used.

[Surgical treatment of vagal paragangliomas]. / Tratamiento quirúrgico de los paragangliomas vagales.

Vagal paragangliomas are much less frequent than carotid and jugulo-tympanic paragangliomas and, like these other forms, can be sporadic or familial. The familial forms usually occur as isolated tumors or within syndromic entities such as multiple endocrine neoplasia type IIA-B, Von Hippel- Lindau syndrome and neurofibromatosis type 1. The genes causing the disease are located in three loci: SDHD (11q23), SDHC (1q21) and SDHB (1p36.1p35). Approximately one-third of apparently sporadic paragangliomas also show germline SDH mutations. In familial forms, the rate of multicentric tumors is up to 30-40%. Vagal paragangliomas arise from arise from paraganglia associated with the vagus nerve. Lower cranial nerve deficits are frequently observed. An essential feature in the diagnosis of vagal paraganglioma is that they are situated behind the internal carotid artery. The treatment of vagal paraganglioma should be based on the biological behavior of the tumor, the age and health status of the patient, tumoral size and the possible treatment-associated morbidity. Surgery is important in the treatment of vagal paraganglioma but this option is controversial in patients without lower cranial nerve palsy. The approach of choice is usually cervical or transcervical. If the tumor involves the jugular fossa, in addition to the cervical approach, a transpetrous approach is also required. Expectant management and radiotherapy are often valid options.

Radiation-induced sarcomas of the head and neck.

The aim of this study was to report our experience on the management of radiation-induced sarcomas (RISs). A retrospective study from 1994 to 2003 was done at our institution reviewing the medical records of 5 patients who had RISs. Five patients diagnosed of head and neck cancer received irradiation to treat their diseases. Later on, these patients developed new neoplasms in the irradiation fields (3 malignant fibrous histiocytoma, 1 osteosarcoma, and 1 angiosarcoma). The mean period of latency between irradiation and diagnosis of new tumors was 13 years. Early symptoms included neck or face swelling, odynophagia, or trigeminal hypoesthesia. All of the patients underwent surgical treatment. In 4 cases, regional and free flaps for head and neck reconstruction were required. Three patients also needed neoadjuvant chemotherapy. In the follow-up, 2 patients are alive and free of disease. Wide excision is the treatment of choice for RISs. Previous radiation therapy limits the dose that can be administered to the involved area, and the response rate to the chemotherapy is always poor.

[Usefulness of video-assisted cervicotomy on the extraction of foreign bodies]. / Utilidad de la cervicotomía videoasistida en la extracción de cuerpos extraños.

Foreign bodies located in the upper aerodigestive tract, if not diagnosed and taken out speedily, may be the cause of great morbimortality. We present a female patient who arrived at the emergency department reporting she had swallowed a fish bone; it was finally located in the pre-vertebral muscle at the level of C3-C4, where it had entered from the oesophagus at 2 cm from the superior oesophageal sphincter. We used a cervicotomy assisted by endoscopy, showing a new application for videoendoscopy.

Voice quality after endoscopic laser surgery and radiotherapy for early glottic cancer: objective measurements emphasizing the Voice Handicap Index.

We analyzed the functional outcome and self-evaluation of the voice of patients with T1 glottic carcinoma treated with endoscopic laser surgery and radiotherapy. We performed an objective voice evaluation, as well as a physical, emotional and functional well being assessment of 19 patients treated with laser surgery and 18 patients treated with radiotherapy. Voice quality is affected both by surgery and radiotherapy. Voice parameters only show differences in the maximum phonation time between both treatments. Results in the Voice Handicap Index show that radiotherapy has less effect on patient voice quality perception. There is a reduced impact on the patient's perception of voice quality after radiotherapy, despite there being no significant differences in vocal quality between radiotherapy and laser cordectomy.

[Tumour recurrence in squamous head and neck cancer]. / Recidivas en el cáncer escamoso de cabeza y cuello.

INTRODUCTION: For most patients with squamous head and neck cancer (HN-SCC), locoregional tumour recurrence (TR) carries an extremely poor prognosis and is therapeutically challenging. OBJECTIVE: To define the clinical aspects of TR and their implication on the survival in patients with HN-SCC. PATIENTS AND METHOD: The clinical management and the outcome of 652 patients with HN-SCC were reviewed. RESULTS: The overall incidence of TR in this series of HN-SCC was 19.9% (n=130). The most frequent locations of the primary cancers were oropharynx (32%), hypopharynx (24%), and oral cavity (21%). The rates of recurrence were locoregional 50%, local 43 % and stomal recurrence 7%. The appearance of a TR reduces the overall survival of patients with HN-SCC to 15%. Survival is better in glottic (38%) and supraglottic (27%), carcinomas, and worse in oro-hypopharynx tumours (2-4%). CONCLUSIONS: RT are more frequent in pharyngeal tumours, especially locoregional recurrences. Patients with recurrence in pharynx were definitely associated with poor prognosis and in these cases salvage surgery seems not to improve survival rates.

[Primary tumours of the external auditory canal. Our experience in 34 patients]. / Tumores primarios del conducto auditivo externo. Nuestra experiencia en 34 pacientes.

OBJECTIVES: Malignant neoplasms of the external auditory canal (EAC) and middle ear are rare but have a poor prognosis. The aim of this study is to identify the variables associated with worse prognosis. PATIENTS AND METHOD: Thirty-four patients were treated in our department between 1990 and 2006 for EAC and middle ear tumours. The patients were staged according to the 1990 Pittsburgh classification. In most cases, surgery was followed by post-operative radiotherapy. RESULTS: The overall disease-free survival was 49 % after 5 years. It reached 87 % in stages I and II, whereas the survival for stages III and IV was 21 % (P=.001). Pre-operative facial nerve paralysis (P=.03), lymph node metastasis (P=.01) and dural extension (P=.02) were associated with decreased survival rates. CONCLUSIONS: In carcinomas of the EAC and middle ear, lymph node involvement, facial nerve palsy, and dural extension were associated with a poorer outcome. For tumours in advanced stages, new therapeutic protocols should be evaluated.

[Head and neck paragangliomas: revision of 89 cases in 73 patients]. / Paragangliomas de cabeza y cuello: revisión de 89 casos en 73 pacientes.

INTRODUCTION: Paragangliomas (PGL) are uncommon neuroectodermal tumours. PGL are usually clinically benign tumours, although metastasis has been reported and invasive growth can occur in adjacent tissues (<10 %). Mutations in SDHB, SDHC, and SDHD, which encode sub-units of mitochondrial complex II (succinate dehydrogenase), play an important role in the pathogenesis of these tumours. MATERIAL AND METHOD: Retrospective review of 73 patients with 89 paragangliomas who had undergone resection of the PGL in our hospital. There were 8 patients who displayed multiple PGL. PGL were distributed as follows: 33 were jugular, 17 tympanic, 26 carotid body tumours, and 13 vagal paragangliomas. All these patients had a follow-up time of at least a year. The surgical approach was evaluated in terms of tumour origin, sequelae, and subsequent evolution, as well as the relapses and their relation with location of the primary tumour. RESULTS: The treatment was surgical, using complementary radiosurgery in just 1 patient. The type A infratemporal fossa approach was used in jugular paragangliomas, the approach was cervical in the carotid and vagal ones and, in the tympanics, a transmeatal or transmastoid approach was performed. In the 73 patients making up our study group, there were 11 recurrences which appeared in jugular paragangliomas (two of them in multiple PGL cases). The post-operative sequelae were mainly cranial nerve paralysis (VII, IX, X, XI, and XII), along with cerebrospinal fluid fistulas in 14 of the jugular PGLs. CONCLUSIONS: With this article we try to reflect our experience in the treatment of this type of tumour. Surgical treatment achieves excellent control of the disease with an acceptable morbidity in young or middle-aged patients. In order to diminish the probabilities of facial nerve paralysis in jugular PGL we must avoid the facial nerve transposition in the infratemporal approach.

Cirugía endoscópica nasal en complicaciones orbitarias por sinusitis / Nasal endoscopic surgery in orbital complications caused by sinusitis

Las complicaciones a partir de infecciones en los senos paranasales, aunque raras veces, pueden provocar lesiones oculares. Clásicamente estas afecciones eran abordadas por técnicas abiertas con las siguientes secuelas y defectos estéticos. La evolución endoscópica nasal permite hoy día un manejo de una forma eficaz y segura sin los inconvenientes de las técnicas abiertas. Presentamos 11 pacientes con complicaciones orbitarias provocadas en 6 casos por sinusitis complicadas y 5 por mucoceles de senos paranasales que provocan una clínica ocular (exoftalmos, diplopia, disminución de la agudeza visual) y que fueron tratados por cirugía endoscópica nasal bajo anestesia general. En todos los casos la evolución fue satisfactoria

Cirugía Endoscópica Nasal en Complicaciones Orbitarias de Procesos Inflamatorios Nasosinusales / Endoscopic nasal surgery in orbital complications of nasal sinus inflammatory disease

Propósito: Comunicar nuestra experiencia con cirugía endoscópica en complicaciones orbitarias de infecciones paranasales. Material y método: Estudio retrospectivo de 11 pacientes, 6 con infección orbitaria y 5 con mucoceles, todos intervenidos por cirugía intranasal exclusivamente. Resultados: En todos los casos se resolvió el problema naso-sinusal y orbitario sin complicaciones ni secuelas originadas por el procedimiento quirúrgico