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ABSTRACT: Desmoplastic small round cell tumor (DSRCT) is a rare malignancy with poor prognosis that generally involves the peritoneum. Only rare cases occur outside the abdomen. Its diagnosis can be achieved only by immunohistochemistry and cytogenetic studies. We describe a case of a 61-year-old man referred to our department with a primary sinonasal tumor. The DSRCT diagnosis was confirmed by the presence of a polyphenotypic immunoprofile (positive for cytokeratin, desmin, and neuronspecific enolase) and the characteristic EWS-WT1 gene fusion resulting from the t(11;22)(p13;q12) reciprocal translocation. This reported case of DSRCT draws attention to the importance of including DSRCT in the differential diagnosis of sinonasal tumors.
Auris, nasus, larynx 02/2013; · 0.58 Impact Factor
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ABSTRACT: INTRODUCTION: Anterior craniofacial resection (CFR) is a standardised procedure for the treatment of tumours involving the anterior skull base. We present our experience in the endoscopic treatment of these tumours. MATERIAL AND METHOD: A retrospective analysis was performed of patients treated by endoscopic anterior CFR in our Department from 2004 until 2011. RESULTS: Thirty-two patients were analysed. Mean follow-up was 28 months (range: 6-84 months). The most frequent pathological entity was adenocarcinoma (60%), followed by undifferentiated carcinoma (13%). According to TNM classification, malignant epithelial tumour staging was T3 in 9%, T4a in 53% and T4b in 19% of the malignant epithelial tumours. The complication rate was 6% and the resection was complete in 91% of cases. During follow-up, 9% of patients developed recurrence. The 5-year overall survival rate was 70% and the 5-year disease-free survival rate was 85% CONCLUSION: These results seem to indicate that properly planned endoscopic CFR may be a valid alternative to traditional open approaches for the management of malignancies of the anterior skull base.
Acta Otorrinolaringológica Española 06/2012;
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ABSTRACT: Sinonasal carcinomas are rare tumours with an unfavourable prognosis whose management is difficult and complex, leading to high morbidity and mortality despite improvements in the field of surgery and radiotherapy. An elevated number of these tumours can be attributed to occupational exposure. In comparison with other head and neck malignancies, studies of molecular changes in these tumours are infrequent. This review was focused on findings about the epidemiology and molecular and phenotypic characterisation of sinonasal carcinomas, which can potentially be useful for diagnosis and treatment. The increasing knowledge about the molecular biology that underlies their carcinogenesis may help to identify precursor lesions, prognostic markers and markers that predict chemoradiotherapy response and, finally, to identify potential molecular targets that will expand treatment options.
Acta Otorrinolaringológica Española 05/2012;
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ABSTRACT: INTRODUCTION: The increasing expertise of transnasal endoscopic surgery has recently expanded its indications to include the management of tumours affecting the skull base. We report our experience with endoscopic management of these tumours, emphasising the indications and surgical technique used. MATERIAL AND METHOD: A retrospective analysis was performed of patients treated by an endoscopic endonasal approach (EEA) in our department from 2004 until 2011. RESULTS: Sixty-three patients were analysed. We performed an endoscopic craniofacial resection in 32 patients (51%), an expanded EEA in 22 (35%), a transclival approach in 6 (9%) and a transpterygoid approach in 3 (5%). The most frequent benign tumour was nasopharyngeal angiofibroma (24%), while adenocarcinoma (30%) was the most common among malignancies. Mean follow-up was 26 months (range: 6 to 84 months). The complication rate was 5% and resection was complete in 56 cases (89%). The 5-year overall-survival was 71% in patients with malignant tumours and the effectiveness was 100% in benign tumours. CONCLUSION: Our results support that endoscopic surgery, when properly planned, represents a valid alternative to standard surgical approaches for the management of skull base tumours.
Acta Otorrinolaringológica Española 04/2012; 63(5):339-347.
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ABSTRACT: The gold standard of treatment of cancer of the lateral wall of the oropharynx continues to be unclear, especially in advanced stages. In this study, we report our experience with surgical treatment of these cancers and describe the functional and oncological results of the procedures.
Retrospective review.
A total of 155 previously untreated patients with squamous cell carcinoma of the lateral wall of the oropharynx who underwent a surgical resection of the lesion at our department from January 1990 to January 2008 were included. Sixty-seven percent of these patients received postoperative radiotherapy. The records of these patients were reviewed to obtain measures such as local and regional control, disease-specific survival, and speech and swallowing function.
Six patients had a stage I disease, 15 had a stage II disease, 31 had a stage III disease, 86 had a stage IVA, and 17 had stage IVB disease. The overall recurrence rate was 60%, and the local recurrence rate was 40%. The 5-year overall survival and disease-specific survival rates were 33% and 43%, respectively. Five-year disease-specific survival rates by stage were as follows: 100%, 59%, 57%, 31%, and 33% for stages I to IVB, respectively. Multivariate analysis showed two parameters that were independent predictors of a reduced disease-specific survival: cervical lymph node metastases pN2-3 (P = .027) and primary tumor classified as pT3-4 (P = .029). In 122 patients, a tracheotomy was performed, and it couldn't be sealed in 23% of them. Oral alimentation was successfully recovered in 93% of the patients.
Surgical treatment of cancer of the lateral wall of the oropharynx provides acceptable oncological and functional results, especially in early and moderately advanced stages (stages I-III). In advanced stages (stage IV), we obtained good functional preservation rates but poor oncological outcomes. Consequently, these groups of patients could be considered for another treatment modality, such as radiochemotherapy.
The Laryngoscope 07/2011; 121(7):1449-54. · 1.75 Impact Factor
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ABSTRACT: Intestinal-type sinonasal adenocarcinoma (ITAC) is a rare tumor etiologically related to professional exposure to wood dust. The overall prognosis is poor, mainly due to the difficulty to resect the tumor completely in this anatomically complex region. Therefore, there is great need for alternative treatments. However, the lack of a good tumor model system for ITAC has hampered the development and testing of new therapeutic agents. Here, we report the establishment and characterization of the first human ITAC cell line named ITAC-3.
The cell line was initiated from small explants of a T4bN0M0 colonic type ITAC from the ethmoid sinus. Growth and invasion parameters as well as genetic characteristics were analyzed.
The population doubling time was 18 h and the cell line was capable of invasion in matrigel. Chromosomal analysis showed a tetraploid karyotype with both numerical and structural aberrations. High resolution microarray CGH analysis identified many copy number alterations, including homozygous deletions. TP53 carried a mutation c.818G>T in exon eight concurring with a strong nuclear protein overexpression. Immunohistochemical analysis showed protein overexpression of EGFR and normal expression of β-catenin and p16.
This is the first report of the establishment of a cell line derived from a primary ITAC. The genomic profile of the cell line was the same as the primary tumor from which it was derived. This new cell line will be a useful tool for the development and testing of new therapeutic agents for this tumor type.
Cellular oncology (Dordrecht). 02/2011; 34(1):23-31.
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ABSTRACT: Juvenile nasopharyngeal angiofibroma (JNA) is a rare benign tumour in adolescent males. It may be associated with a significant morbidity because of its anatomical location and its locally destructive growth pattern. Severe haemorrhage constitutes a high risk in JNA and its surgical management could be complex.
We retrospectively analysed the clinical data from 43 patients with JNA surgically treated in our Department from 1993 until 2010. Mean postoperative follow-up time was 85 months.
Analysis was performed on 42 males and one female. Mean patient age was 16 years old. The most common presenting symptoms were unilateral epistaxis (56%) and nasal obstruction (56%). Using the Fisch staging scale, tumours were classified as stage I in 2 patients, stage II in 9, stage III-a in 13, stage III-b in 13 and stage IV-a in 6. Preoperative selective embolisation was performed on 32 patients (74%). Thirty-three patients (77%) underwent an open surgical approach and 10 (23%) were treated by endoscopic approach. Complete resection of the lesion was achieved in 35 patients (81%) and tumour recurrence was observed in 2 (5%). All lesions treated via transnasal endoscopic approach were stage I and stage II lesions.
Surgery is the treatment of choice for JNA. An endoscopic approach is feasible for early-stage lesions (Fisch I and II) and conservative external approaches are still useful in advanced stages (Fisch III and IV). The open approaches proved helpful with respect to exposure, safety, cosmetic outcome and low morbidity. Preoperative embolisation, if possible, is mandatory.
Acta Otorrinolaringológica Española 62(4):279-86.
