A Giant Congenital Mastoid Cholesteatoma With Extension to the Occipital Condyle.

A congenital mastoid cholesteatoma (CMC) is a keratinizing epithelium originating from embryological epithelial tissue of the mastoid. It is often not diagnosed until it becomes large because of its rarity and indolent nature. Although there are a few reports on giant CMC, its exact extensions have not been well described, and detailed information regarding surgical methods is lacking, especially in giant CMC involving the occipital condyle and the middle and posterior cranial fossae. In this article, we report a case involving a 70-year-old woman with a giant CMC that extended inferiorly to the occipital condyle. The CMC eroded the middle and posterior cranial fossae, sigmoid sinus plate, and fallopian canal of the facial nerve. For complete removal, we used a subtotal petrosectomy in conjunction with an exposure of the cranial cervical junction and a wide decompression of the suboccipit. The boundaries of exposure were similar to those of a petro-occipital transsigmoid approach which is usually used for management of tumor involving the jugular foramen. The wide exposure allowed for complete removal of the lesion without any complications. Thus, we recommend this surgical approach for management of the giant CMC involving the occipital condyle and the middle and posterior cranial fossae.

Clinical Characteristics of Temporal Bone Metastases.

OBJECTIVES: The purpose of this study were to evaluate the clinical characteristics of temporal bone metastasis (TBM) and to determine whether the characteristics differed according to primary malignancy. METHODS: We retrospectively analyzed data on 20 patients diagnosed with TBM between January 2000 and January 2017. Demographics, the period from diagnosis of primary malignancy to TBM diagnosis, the period from TBM diagnosis to death, the type and staging of primary malignancy, otologic manifestations, and TBM sites were assessed. After the primary malignancies were divided into solid cancers and hematologic malignancies, each parameter was compared between the two groups. RESULTS: The most common primary malignancy with TBM was lung cancer (45%). The most common otologic symptoms and signs were facial palsy (30.5%) and hearing loss (30.5%). The temporal squama (23%) and the facial nerve (20%) were the most commonly involved. Most TBMs occurred late in the disease process after the primary malignancy first metastasized to other organs. Hematologic malignancies metastasized significantly more frequently to the external auditory canal and the middle ear/mastoid compared to solid cancers (P=0.001 and P=0.004, respectively). CONCLUSION: If otologic manifestations such as facial palsy and hearing loss are presented in patients at advanced stages of malignancy, TBM of primary malignancy should be suspected. In addition, hematologic malignancies tend to metastasize to the external auditory canal and the middle ear cleft more commonly than solid cancers do.

Clinical significance of extrathyroidal extension according to primary tumor size in papillary thyroid carcinoma.

BACKGROUND: Extrathyroidal extension (ETE) is a risk factor for poor papillary thyroid carcinoma (PTC) outcomes. However, the clinical significance of ETE according to primary tumor size has not been well-established. The purpose of this study was to compare differences in clinical outcomes, according to the presence and extent of ETE, between different primary tumor size groups. METHODS: In total, 381 patients with PTC underwent total thyroidectomy with or without lymph node (LN) dissection from 2004 to 2010. We divided the patients into two groups according to primary tumor size: ≤ 1 cm or >1 cm. Each group was further divided into subgroups according to the presence of ETE (ETE vs. no ETE) and degree of ETE (microscopic ETE vs. macroscopic ETE). The clinicopathological features and rate of recurrence during follow-up were compared among groups. RESULTS: Among the PTC patients with primary tumors >1 cm, patients with ETE had a higher recurrence rate than those without ETE, and only macroscopic ETE affected recurrence in patients with PTC > 1 cm (P < 0.05). However, there was no significant difference in recurrence rates between those without ETE and those with microscopic ETE (P = 0.100). When the primary tumor size was less than 1 cm, there were no difference in recurrence rates between the groups with or without ETE, or between the groups with microscopic and macroscopic ETE (P > 0.05). CONCLUSIONS: Our data suggests that the presence and degree of ETE may be associated with PTC outcome based on primary tumor size.

Relationship Between 18F-fluorodeoxyglucose Accumulation and the BRAF V600E Mutation in Papillary Thyroid Cancer.

BACKGROUND: To determine whether 18F-fluoro-2-deoxyglucose (18F-FDG)-PET/CT is useful for predicting the BRAF V600E mutation status of a primary papillary thyroid carcinoma (PTC). METHODS: A retrospective analysis was performed in 108 patients who underwent 18F-FDG positron emission tomography-computed tomography (PET/CT) for staging before thyroidectomy and BRAF analysis in biopsy-confirmed PTC. The maximum standardized uptake value (SUVmax) of the primary tumor was calculated according to FDG accumulation. Univariate and multivariate analyses were performed to assess the association between the SUVmax and clinicopathological variables. RESULTS: The BRAF V600E mutation was detected in 71 of 108 (65.7%) patients. In all subjects, the tumor size and BRAF V600E mutation were independently related to the SUVmax according to multivariate analyses (P = 0.002 and 0.007, respectively). The SUVmax was significantly higher in tumors with the BRAF V600E mutation than in tumors with wild-type BRAF (10.24 ± 11.89 versus 4.02 ± 3.86; P = 0.007). In the tumor size >1 cm subgroup, the BRAF V600E mutation was the only factor significantly associated with the SUVmax (P = 0.016). A SUVmax cutoff level of 4.9 was determined to be significant for predicting the BRAF V600E mutation status (sensitivity 77.4%, specificity 100.0%, area under the curve 0.929; P < 0.0001) according to ROC curve analysis. CONCLUSIONS: The BRAF V600E mutation is independently associated with high 18F-FDG uptake in PTC, especially in those with a tumor size >1 cm.

A retrospective analysis of 538 sinonasal fungus ball cases treated at a single tertiary medical center in Korea (1996-2015).

BACKGROUND: Sinonasal fungus ball (FB) is a type of noninvasive fungal rhinosinusitis affecting immunocompetent hosts. FB, previously considered rare, has been reported with increasing frequency. We reviewed our experience of 538 cases over the past 20 years. METHODS: We retrospectively examined clinical records including clinical presentations, radiological findings, management, and outcomes of FB patients who have undergone surgery for treatment. The number of FB patients who underwent endoscopic sinus surgery (ESS) was calculated annually. Causal relationships between structural variations and FB were also investigated. RESULTS: The number of FB patients who underwent sinus surgery has increased. The mean age was 58.3 years, and the gender ratio was approximately 2 (female): 1 (male). While the most common presenting symptoms of maxillary sinus FB patients were nasal symptoms, such as postnasal drip and nasal obstruction, sphenoid sinus FB patients presented with headache mostly. On computed tomography (CT) scans, the most common finding was intralesional hyperdensity (77.3%). There was no significant correlation between the presence of FB and structural variations (nasal septal deviation, concha bullosa, Haller cell). Median follow-up period of the patients was 11 months. Recurrence or residual disease occurred in only 6 (1.1%) cases. CONCLUSION: The number of FB patients who underwent surgery has increased steadily over the past 20 years. FB should be considered in patients with unilateral nasal symptoms and unexplained headaches. A preoperative CT scan is an essential tool in making diagnosis easier and faster. Endoscopic surgery is the treatment of choice, with a low morbidity and recurrence rate.