Metastatic Malignant Glomus Jugulare Tumor: A Rare Case Report with Clinical Manifestations and Treatment Approach.

BACKGROUND Paragangliomas are neuroendocrine neoplasms derived from paraganglia of the sympathetic and parasympathetic nervous systems. Parasympathetic ganglia-derived tumors, also called non-chromaffin, are located almost exclusively in the neck and skull base and are usually non-secretory and inactive. A case of malignant glomus jugulare with a metastatic cervical lymph node is described here. CASE REPORT A 24-year-old woman was referred to an otolaryngology clinic for concern of voice change for 1 month, which was associated with right progressive hearing loss, pulsating tinnitus, and right facial weakness. A clinical examination revealed a reddish mass in the right ear behind an intact tympanic membrane with right facial weakness of House-Brackmann grade VI. A bedside flexible nasopharyngoscopy revealed an immobile right vocal fold. A computed tomography scan of the brain revealed a destructive lesion within the right jugular foramen. The patient underwent embolization followed by glomus tumor resection via infra-temporal fossa with Fisch type A approach. Pathology revealed that the tumor was an infiltrative epithelioid tumor with a spindle and nesting pattern separated by fibrovascular stroma. The submitted lateral neck lymph node revealed a metastatic tumor. CONCLUSIONS Glomus jugulare tumors are uncommon paragangliomas, and malignant behavior with metastasis is extremely rare. Metastatic tumors are often associated with facial and vagal nerves palsy. There are no histological features that distinguish malignant glomus jugulare tumors. Malignant neoplasms are characterized by the presence of metastases. Tumors of the glomus jugulare that are malignant are treated with surgery, radiotherapy, or both. However, our search of the literature revealed no clear guidelines, given the scarcity of cases. Moreover, the presence of metastasis increases the risk of death.

A misdiagnosed aneurysm of the petrous internal carotid artery: A case report.

INTRODUCTION: An aneurysm is characterized by the weakening of the arterial wall, which leads to a bulge that can be filled with blood. Aneurysms of the petrous portion of the internal carotid artery are rare and predominantly detected incidentally. This is a report of multiple misdiagnoses of an aneurysm of the petrous segment of the internal carotid artery (ICA) that highlights its imaging-based diagnosis and risk of mortality. PRESENTATION OF CASE: A 60-year-old woman with chronic kidney disease and a history of stroke presented with left ear discharge, decreased hearing, and non-pulsatile tinnitus that had persisted for four months. Clinical examination showed wet tympanic membrane perforation, and imaging revealed an ill-defined infiltrative mass involving the left petrous apex initially misdiagnosed as glomus jugulare. Diagnostic computed tomography (CT) angiography revealed a left aneurysm in the petrous part of the ICA, which was successfully treated with interventional radiology. Follow-up was planned for infectious diseases and internal medicine, but she was lost to follow-up by the otolaryngology department. DISCUSSION: Aneurysms in the petrous portion of the ICA are rare and usually asymptomatic. However, their clinical manifestations vary, and they have various differential diagnoses. CT and magnetic resonance imaging are essential for diagnosis, and CT angiography is the gold standard. CONCLUSION: Diagnosing petrous ICA aneurysms requires a high level of suspicion and CT angiography. Their clinical presentations vary from asymptomatic to severe. Case-specific management and endovascular treatment yield positive neurological outcomes.

Malignant Otitis Externa in a 20-Year-Old Male Patient With Erdheim-Chester Disease: A Case Report.

Malignant otitis externa (MOE) is an uncommon, potentially life-threatening, invasive infection of the external auditory canal and lateral skull base. It is mainly seen in older adults with diabetes or immunocompromised patients and rarely seen in children. Erdheim-Chester disease (ECD) is a rare, non-Langerhans histiocytosis disorder. It is a multisystem disease with a poorly understood etiology. It commonly affects the skeletal system, central nervous system (CNS), cardiovascular system, lungs, retroperitoneum, kidneys, and skin. CNS involvement confers poor prognosis and reduced response to treatment. Cardiovascular involvement is another indicator of poor prognosis. This report describes the case of a 20-year-old male with ECD who had bilateral malignant otitis externa/skull base osteomyelitis and hearing loss. The patient was not responding well to treatment. He was on intravenous antibiotics, underwent left mastoidectomy, received targeted immunotherapy, and had four rounds of chemotherapy. The patient died after six months from the diagnosis and targeted treatment, which indicates the aggressiveness of the disease. MOE should be suspected in any patient presenting with intractable otalgia with otorrhea that is not responsive to local treatment for uncomplicated otitis externa or otalgia with temporomandibular joint (TMJ) pain aggravated by chewing. This is the first report of malignant otitis externa in a young patient with ECD to the best of the author's knowledge. This case emphasizes the importance of suspecting MOE in young patients, especially if the risk factors of the disease are present because early diagnosis can prevent or minimize life-threatening complications.