Chondroblastoma of the Temporal Bone: A Case Report and Literature Review.

INTRODUCTION: Temporal bone chondroblastoma is a rare, locally aggressive tumour originating from immature cartilage, which recurs to a high degree. Treatment is surgical. Radiotherapy is reserved for recurrence. We describe a case of a 15-year-old-boy choosing a conservative surgical approach with reconstruction of the posterior canal wall. This study aims to report a rare pediatric case. METHODS: A literature review was performed to better understand temporal bone chondroblastomas, to describe their histopathological and radiological characteristics and to establish the optimal surgical and non-surgical treatments. The research of previous published data was done using PubMed with keywords mentioned below. RESULTS: Authors present a case of a 15-year-old boy with hearing impairment and facial nerve palsy. Conservative surgery with reconstruction of the tympanic membrane and posterior wall of the external auditory canal, restoring the hearing has been performed. We did not administer any adjuvant therapies. No sign of recurrence was observed 1 year after primary surgery. Facial nerve function is normal, and hearing is satisfactory. CONCLUSION: Chondroblastomas account for less than 1% of primary bone tumours. Temporal bone chondroblastoma is rare, locally aggressive, with a high prevalence of recurrence. This study describes specific histopathological and radiological findings, the chosen surgical approach and follow-up to improve the management and the prognosis of patients affected with this particular clinical entity.

Intraoperative calcitonin stimulation testing in the surgical treatment of C-cell disease.

OBJECTIVE: The prognosis of medullary thyroid carcinoma (MTC), derived from parafollicular C-cells, depends on the completeness of the initial surgical excision. The C-cells produce calcitonin, a peptide hormone used as a biochemical and immunohistochemical tumor marker. The aim of the study was to evaluate an individualized approach to patients with C-cell disease, i.e. MTC and C-cell hyperplasia (CCH), using the intraoperative calcitonin testing-assisted surgical strategy as a predictor of the final outcome. STUDY DESIGN: A unicentre cross-sectional study. METHODS: From June 2009 to May 2015, thirty one patients with MTC/CCH were surgically treated primarily (n=24) or reoperated for persistence of the disease (n=7). Depending on the result of intraoperative calcitonin stimulation testing (iCST), patients underwent total thyroidectomy with or without lymph node dissection. All patients were tested repeatedly in the postoperative period (range 1 to 48 months). RESULTS: The iCST was true negative in all CCH, and ten out of eleven N0 MTC primarily operated patients, and true positive in one N0 patient and six of the seven reoperated patients. The test was false negative in two patients preoperatively evaluated as N+, one primarily operated and one reoperated, respectively. CONCLUSION: The results encourage the use of an individualised approach on patients with MTC/CCH, e.g. to be less radical surgically in cases of negative iCST, and to be more radical in those patients with persistent increase of serum calcitonin. The absence of post-stimulation calcitonin elevation in iCST seems to be a good prognosis indicator in patients with an early-stage C-cell disease, but longer follow-up is needed.

Papillary Thyroid Carcinoma: Analysis of the Central Compartment's Lymph Nodes Metastases.

BACKGROUND: Papillary thyroid carcinoma is typical by regional lymph nodes metastases. Therefore we decided to analyse associated risk factors. OBJECTIVE: In this retrospective study we focused on the incidence of metastatic involvement of the central compartment's lymph nodes correlated with age, size of the primary tumour, infiltration of thyroid gland capsule, positive lymphangioinvasion in order to assess risk factors. METHOD: We analysed group of 156 patients with papillary carcinoma, who have undergone total thyroidectomy and bilateral elective central compartment neck dissection. We evaluated the occurrence of metastases, size, infiltration and lymphangioinvasion based on definitive histology of the whole group and separately for subgroups of patients under and over 45 years. RESULT: We found metastatic involvement in 88 (56.4%) patients. When comparing the subgroups of patients under (73 patients) and over 45 years (83 patients), we found metastases in 56 vs. 32 (76.7% vs. 38.6%) patients. In the subgroup of younger patients we found significant higher incidence of metastases compared with the group of over 45 years, P < 0.001 (P = 0.000027). We found significant higher incidence of metastases in patients with positive capsule infiltration in the whole group, P < 0.001 (P = 0.00049); in the subgroup of under 45 years, P < 0.001 (P = 0.00091) and in patients with positive lymphangioinvasion in the whole group, P < 0.01 (P = 0.00177); in the subgroup of over 45 years, P < 0.001 (P = 0.0002). In patients with metastases we found tumour size ≥1cm more frequently in all groups. CONCLUSION: We recorded higher incidence of regional metastases in patients under 45 years, positive capsule infiltration, lymphangioinvasion. Age under 45 years itself does not correlate with less aggressive disease, to the contrary some of other analysed risk factors correlate with more aggressive disease.

Recurrent salivary pleomorphic adenoma shows increased immunohistologic expression of bcl-2 oncoprotein.

BACKGROUND: Internal cell biology, including apoptotic regulation, is presumed to play a key role in the development of recurrent pleomorphic adenoma (PA). AIM: The aim of our study was to determine the relevance of B-cell lymphoma 2 (bcl-2) oncoprotein immunoexpression and distribution in primary PA, and its recurrence. METHODS: Ten primary-non-recurrent, 14 primary-to-recur, and 28 recurrences of parotid PA patients aged 19-73 (mean 40.7±16.7) years were enrolled. The bcl-2 expression was compared between groups using a semi-quantitative histoscore, defined as the multiple of the percentage of cells by the intensity of immunostaining. RESULTS: Widely varying bcl-2 immunoreaction was found in the epithelial areas of 91.7% of primary and 85.2% of recurrent PA. Similarly varying but much less, immunopositivity was found in the myxoid areas of 62.5% of primary and 71.4% of recurrent tumours. No obvious differences in the bcl-2 staining intensity or pattern of specific epithelial morphologic structures in either the primary-non-recurrent, primary-to-recur or recurrent tumours were found. In both the mesenchymal and epithelial areas of PA, the differences in bcl-2 immunohistoscore between the primary-non-recurrent and primary-to-recur groups were not statistically significant (P=0.62, respectively 0.51). In the mesenchymal areas, the study revealed a significantly increased histoscore in recurrent tumours compared to their corresponding primaries (P=0.01). Increased bcl-2 expression in recurrent PA suggests an exaggerated aggressiveness of that tumor. CONCLUSION: The fact that a significant difference in the histoscore was found exclusively in the myxoid component seems to accord with the reported prevalence of the latter in recurrent and metastatic PA.