RESUMEN
(1) Background: The incidence of congenital cholesteatoma (CC) has rarely been discussed, particularly from a demographic viewpoint. Therefore, we conducted an epidemiological study of CC using local medical characteristics. (2) Methods: The participants were 100 patients (101 ears) who underwent initial surgical treatment at university hospitals in two rural prefectures between 2006 and 2022. A total of 68% of the patients were males and 32% were females, with a median age of 5 years. We reviewed the medical records for the date of birth, date of surgery, stage of disease, and first symptoms of the disease. (3) Results: The total incidence of CC was calculated to be 26.44 per 100,000 births and tended to increase. No significant difference was found between the incidences in the two prefectures. The number of surgeries performed was higher in the second half of the study period. No difference in the stage of progress was observed based on age. (4) Conclusions: The incidence of CC was estimated to be 26.44 per 100,000 newborn births. The number of patients with CC tended to increase; however, this can be attributed to an increase in the detection rate rather than the incidence.
RESUMEN
We report a case of amelanotic malignant melanoma (AMM) in a 66-year-old female. AMM of the lingual base was diagnosed based on a biopsy of late metastasis to the bone marrow of the L4 lumbar vertebra. The patient was initially treated with chemoradiotherapy after being misdiagnosed with poorly differentiated human papillomavirus- (HPV-) related squamous cell carcinoma of the oropharyngeal anterior wall. p16 immunostaining is used to diagnose HPV-related oropharyngeal cancer. However, while p16 expression is used as a surrogate marker of HPV infection, it is important to be aware that p16 protein overexpression can also be caused by other factors. Malignant melanoma is known to express the p16 protein. Morphologically differentiating between AMM and poorly differentiated squamous cell carcinoma based on hematoxylin-eosin staining is difficult. Therefore, in cases that are pathologically diagnosed as p16-positive poorly differentiated oropharyngeal squamous cell carcinoma, it is important to rule out AMM.
RESUMEN
OBJECTIVE: This study evaluated the clinical value of diffusion-weighted magnetic resonance imaging (DW-MRI) in the diagnosis and staging of congenital cholesteatoma (CC). PATIENTS AND METHODS: We retrospectively reviewed 24 patients with CC. All the patients underwent computed tomography (CT) and DW-MRI preoperatively; thereafter, surgery was performed. DW-MRI examination was performed with a 3â¯T MRI system using three-dimensional reversed fast imaging with steady-state precession and diffusion-weighted magnetic resonance sequence. The preoperative and operative CT and DW-MRI findings were compared. RESULTS: Using DW-MRI, cholesteatoma was successfully detected in 17 (71%) of the 24 patients with CC. Among the seven patients with false-negative results, the cholesteatoma mass diameter was <5â¯mm in six patients and ≥5â¯mm in one patient. One of these patients had open type congenital cholesteatoma (OTCC). The detection rates for closed type cholesteatoma and OTCC were 85% (17/20) and 0% (0/4), respectively, using DW-MRI. Using CT and DW-MRI, the correct stage was identified in 88% (15/17) and 59% (10/17) of the patients with aeration around the CC and in 0% (0/7) and 100% (7/7) of those without aeration around the CC, respectively. CONCLUSION: CT is the primary imaging tool for evaluating suspected CC in patients with aeration around the CC. However, CT is unreliable for the detection of the extension and staging of CC when the middle ear is filled with nonspecific imaging. DW-MRI is useful for the preoperative diagnosis and staging of CCâ¯>â¯5â¯mm in diameter with or without surrounding granulation tissue. Thus, we recommend using DW- MRI at least when CT fails to localize CC as a soft tissue mass because of non-specific tissue filling the middle ear and the mastoid.
