Elisabetta Zanoletti

Temporal bone squamous cell carcinoma: Analyzing prognosis with univariate and multivariate models

Temporal bone squamous cell carcinoma (SCC) is an uncommon malignancy accounting for less than 0.2% of head and neck cancers. Despite advances in its early diagnosis, skull base microsurgery, radiotherapy, and integrated treatments, prognosis in advanced SCCs remains dismal. The present study aimed to analyze the clinicopathological variables potentially influencing outcome in a series of temporal bone SCCs.

Characterization of endolymphatic sac tumors and von Hippel–Lindau disease in the International Endolymphatic Sac Tumor Registry

Endolymphatic sac tumors (ELSTs) are, with a prevalence of up to 16%, a component of von Hippel–Lindau (VHL) disease. Data from international registries regarding heritable fraction and characteristics, germline VHL mutation frequency, and prevalence are lacking.

The problem of nodal disease in squamous cell carcinoma of the temporal bone

Abstract Conclusion: The prognosis of the clinically positive neck in temporal bone squamous cell carcinoma is bad and failures never occur in the neck but at the site of the primary. A clinically positive neck at presentation is a sign of aggressiveness of the primary and, in such cases, therapeutic neck dissection is to be performed. The role of elective neck dissection is partially supported by our results, but as prognosis worsens and survival drops when a positive node appears in the neck, elective dissection is still advocated. In our material, no recurrence was recorded in the neck, but it was the resection of the primary tumor that proved to be oncologically inadequate. This led to the conclusion that in cases of clinically positive necks a more extended and adequate approach to the primary is thus advocated. Objective: This work aimed to determine the prognostic role of positive neck nodes in squamous cell carcinoma of the temporal bone. Methods: We studied the homogeneous case material of 47 surgical cases of temporal bone squamous cell carcinoma, operated on between the years 1983 and 2004. All the cases were reviewed retrospectively, staged according to the Pittsburgh classification, and the follow-up was updated at June 2008 with MRI. The minimum follow-up was set at 3 years. Results: All the cases but two were treated with therapeutic or elective neck dissection. The neck was assessed both clinically and pathologically. The cases with clinically positive neck nodes at presentation had unfavorable prognosis. All the failures recurred at the site of the primary and never in the neck. The comparison of outcomes between the cases with clinically positive and negative necks showed a difference in survival. The rate of micrometastasis in the clinically negative neck was investigated as well. Our results indicate that though the necessity of elective neck dissection is still controversial, the bad prognostic value of the clinically positive neck is such to support the indication to treat the clinically negative neck.

Evaluation of the prognostic role of pSTAT3 expression in temporal bone squamous cell carcinoma.

Objective Temporal bone squamous cell carcinoma (SCC) accounts for less than 0.2% of all head and neck tumors. Although some progress has been made in treating this aggressive tumor, the prognosis in advanced cases remains poor. More effective therapeutic strategies need to be considered, including receptor-mediated carcinoma-targeted therapy. Phosphorylated STAT3 (pSTAT3) regulates many genes that are necessarily expressed in cancer initiation, development, and progression, being involved in proliferation, anti-apoptosis, invasion, angiogenesis, and immune surveillance evasion. The aim of the present study was to preliminarily investigate the potential prognostic role of pSTAT3 expression in temporal bone SCC. Study Design Retrospective clinicopathologic investigation. Setting Tertiary referral centers. Patients Twenty-five consecutively operated patients with primary temporal bone SCC. Intervention pSTAT3 immunohistochemical expression in primary temporal bone SCCs was assessed with the aid of computer-based image analysis. Main Outcome Measures Conventional clinicopathologic parameters and pSTAT3 expression were correlated with SCC prognosis. Results pT, stage, and surgical margin status were significantly related with recurrence rate (p = 0.002, p = 0.01, and p = 0.047, respectively) and disease-free survival (DFS) (p = 0.0049, p = 0.031, and p = 0.035, respectively). pT classification was also related with disease-specific survival (DSS) (p = 0.035). The SCC recurrence rate did not correlate with pSTAT3 expression. Statistical analyses ruled out any significant difference in DFS or DSS when patients were stratified by pSTAT3 expression (>80.0% or ⩽80.0%). Conclusion Despite our preliminary results, the role of pSTAT3 in temporal bone SCC warrants further investigation in larger series because there is increasing evidence in preclinical models to indicate that inhibiting STAT3 phosphorylation can be a useful addition to different anticancer strategies.

Expression of the tumour-suppressor maspin in temporal bone carcinoma.

Although it accounts for fewer than 0.2% of all head and neck tumours, temporal bone squamous cell carcinoma (SCC) is an aggressive malignancy with a poor prognosis in advanced cases. Novel therapeutic strategies should be developed focusing on specific targeted therapies. Maspin is a serpin showing tumour‐suppressing activity which has therapeutic potential. The present study is the first to investigate maspin expression in temporal bone SCCs, using a series of 29 cases.

A critical look at persistent problems in the diagnosis, staging and treatment of temporal bone carcinoma

Temporal bone squamous cell carcinoma (TBSCC) is an uncommon malignancy with a distinctly poor prognosis in advanced cases. There is still much controversy surrounding the rational diagnostic/therapeutic approach to TBSCC. Diagnostic differences are due mainly to: the small number of cases reported (even in the largest available series); the inappropriate histological heterogeneity of several case series; the lack of an internationally-accepted staging system for TBSCC; the frequent absence of adequate radiological imaging to enable a malignancys local, regional and distant extension to be studied in detail; and a non-standardized approach to final histological assessment of the surgical margins. As for the therapeutic approaches, several issues are still debated, including the choice between en bloc and piecemeal primary surgery for the tumors removal, and the role of elective neck dissection. Although radiotherapy seems to be an effective adjuvant therapy in advanced cases, its role in low-stage tumors or as a primary treatment has yet to be established. The value of chemotherapy is also still unclear. The treatment strategy for TBSCC is often based on the combined experience of a given surgeon and institution, bearing the results reportedly achieved by other oncology centers in mind. To date, the optimal management of TBSCC is still elusive. We aimed to critically review the ongoing crucial issues concerning the management of TBSCC, analyzing how it is diagnosed, staged and treated, the management of recurrences, rational follow-up schedules, and prognostic factors for this disease.

Recurrent squamous cell carcinoma of the temporal bone: critical analysis of cases with a poor prognosis ☆,☆☆

PURPOSE Temporal bone squamous cell carcinoma (TBSCC) is an uncommon, aggressive malignancy with a significant recurrence rate. We reviewed our experience with recurrent TBSCCs. MATERIALS AND METHODS Clinicopathological and therapeutic variables potentially associated with disease-free survival (DFS) and disease-specific survival (DSS) were assessed in 17 TBSCC patients who died of their disease after treatment. RESULTS TBSCC recurrences were treated with surgery in 12 cases (palliative in 11, with curative intent in 1) and palliative chemotherapy in 5; the median DFS and DSS were 6 and 16 months, respectively. The mean DFS and DSS were longer in patients who had primary lateral temporal bone resection (LTBR) rather than subtotal temporal bone resection (STBR) (p=0.0173 and p=0.03, respectively). Patients given non-surgical palliative treatment for recurrences had a longer mean DSS than those who underwent surgery (trend toward significance, p=0.09). CONCLUSIONS Our results reflect the aggressive nature of TBSCC recurrences. Our findings seem to support the use of non-surgical treatments (chemotherapy, radiotherapy, or specialist palliative care) in patients with loco-regionally advanced recurrent TBSCC. Salvage surgery might be considered for early recurrences when radicality is still achievable. Precise guidelines for the rational follow-up of surgically-treated TBSCCs need to be shared between tertiary centers.

Intracranial venous sinus thrombosis as a complication of otitis media in children: Critical review of diagnosis and management

OBJECTIVES Otogenic lateral sinus thrombosis (LST) is a rare intracranial complication of acute otitis media (AOM), which can lead to severe neurological sequelae and death. The aim of this study was to analyze the clinical presentation, management and outcome of LST in children, investigating a possible correlation between clinical aspects, radiological findings and anatomical variations. METHODS At a tertiary Italian hospital, a retrospective review was conducted on the medical records of eight patients diagnosed with otogenic LST over a 3-year period. Four children were males and mean age was 4.7 years. RESULTS All patients had a history of otitis media at diagnosis and 4/8 presented also with more than one neurological sign or symptom. Mastoiditis signs were detected in 5/8 patients. Thrombosis was diagnosed by computed tomography, enhanced magnetic resonance and magnetic resonance venography. Treatment was medical, alone or combined with surgery. Medical treatment consisted in anticoagulants eventually combined with anti-edema medication on clinical basis. Mastoidectomy and/or myringotomy±trans-tympanic drainage placement were performed in 7/8 patients. Complete vessel recanalization was obtained in 6/8 children after a median follow-up time of 4.8 months. No complications, neither clinical sequelae occurred. In our series, neurological signs and symptoms were significantly associated with the presence of hypoplasia of the contralateral venous sinus (p=0.029). CONCLUSION LST is a severe condition occurring even in absence of otological signs, and despite adequate antibiotic therapy for AOM, which should be ruled out and promptly treated. A dominant neurological presentation is associated in our series with anatomical variations of cerebral sinus venous drainage patterns. This should be carefully evaluated and considered in diagnosis, treatment planning and prognosis.

Microsurgical endoscopy-assisted presigmoid retrolabyrinthine approach as a minimally invasive surgical option for the treatment of medium to large vestibular schwannoma

Background Treatment of vestibular schwannomas presents many controversial aspects, from the indication to the selection of the best treatment option. In the era of stereotactic radiotherapy, microsurgery has to be competitive in terms of providing the best chances of functional preservation and complete tumor removal. The two most commonly used surgical approaches are the retrosigmoid suboccipital and the presigmoid translabyrinthine. We describe the endoscopy-assisted presigmoid retrolabyrinthine approach (EAPRA) aiming at combining the advantages of the retrosigmoid and translabyrinthine techniques.

Relaxin-2 expression in temporal bone carcinoma

Temporal bone squamous cell carcinoma (TBSCC) is an uncommon, aggressive malignancy with a significant local recurrence rate even in patients with postoperative pathology reports of free surgical margins. This raises the question of how “free” negative margins should be to be oncologically safe, especially in bone tissue. A potential role for relaxin-2 hormone in tumor-driven osteolysis has recently been reported. The aim of this study was to assess the prognostic role of relaxin-2 expression in TBSCC tissue specimens and pathologically negative bone margins. Relaxin-2 immunohistochemical expression was assessed in 25 consecutively operated TBSCC patients. Several pathological variables correlated with recurrence rate (pT stage, dura mater involvement), disease-free survival (DFS) (pT stage, pN status, grade, and dura mater involvement), and disease-specific survival (DSS) (pT stage, pN status, grade, and dura mater involvement). The recurrence rate, DFS, and DSS did not correlate with relaxin-2 expression in TBSCC specimens or pathologically negative bone margins. Although local recurrence in TBSCC could relate to neoplastic bone invasion not apparent on conventional pathological investigations, the present preliminary findings seem to rule out any role of relaxin-2 in mediating this local aggressiveness. Molecular mechanisms of TBSCC recurrence after curative treatment should be further investigated.