Background: Langerhans cell histiocytosis (LCH) of the temporal bone is an uncommon disease that primarily affects the pediatric population; fewer than 40 adult cases have been reported in the literature. We present a rare case of LCH of the temporal bone in an adult patient and describe its clinical presentation, histopathologic findings, and management. Case Report: A 21-year-old male presented to the emergency department with progressively worsening right-sided ear pain refractory to outpatient oral antibiotics. Physical examination revealed mastoid tenderness and decreased right-sided hearing. Computed tomography (CT) scan suggested coalescent mastoiditis; the patient responded to inpatient antibiotics and was discharged. He returned 9 days later with persistent symptoms. Repeat CT scan revealed an osteolytic lesion on the temporal bone, and the patient was indicated for surgery. Intraoperative histology was consistent with LCH. Subsequent surveillance magnetic resonance imaging (MRI) suggested persistence of disease, and the patient responded to a course of radiation. Three months following radiotherapy, surveillance MRI and positron emission tomography scans revealed no evidence of recurrent disease. Conclusion: Diagnosis of LCH of the temporal bone is frequently delayed because of misdiagnosis of more common otologic diseases, including otitis media, otitis externa, and mastoiditis. The clinician's index of suspicion for LCH should be high if imaging reveals an osteolytic defect of the temporal bone; confirmation is via immunohistostaining of biopsy samples. The majority of cases respond to surgery, radiation, chemotherapy, or combination therapy, but delays in diagnosis and treatment may increase morbidity. Increased physician awareness of LCH of the temporal bone, particularly among adults, may help to improve patient outcomes.
Nasal gliomas are benign congenital midline tumors with the potential for intracranial extension. They are most commonly seen in neonates and children but rarely in adults. The treatment of choice is surgical excision. Inadequate primary excision results in a 4 to 10 percent recurrence. Hence, a thorough preoperative evaluation is essential to delineate the exact site and extension of the tumor and to plan the appropriate surgical approach. Computerized tomographic (CT) scans are useful in visualizing bony defects, but are not well suited for soft tissue imaging. Magnetic resonance imaging (MRI) offers superior soft tissue contrast, without ionizing radiation. This is a report of a neonate with unexplained early respiratory distress. On day 5, a soft nasal mass became apparent. CT scans were inconclusive, so MRI scan was used to demonstrate intracranial extension. MRI is superior for imaging brain tissue, so it should be used preferentially to delineate intracranial extension and to help guide the surgical approach.
Nontoxic adenomatous multinodular goiter (AMNG) in infants is a rare condition. We discuss an extremely rare case of a nontoxic AMNG in a 3-month-old female presenting with airway obstruction. Surgical resection of the mass was performed to reduce the burden on the airway and for diagnosis. The literature is reviewed, and the clinical characteristics, pathology, and surgical treatment are discussed.
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