Late Recurrence of a Rare Middle Ear Neuroendocrine Tumor With an Intracranial Extension to the Temporal Fossa: A Case Report.

We report a clinical case of an extremely rare neuroendocrine tumor of the right middle ear (MeNET) that recurred after 13 years with a local extension into the right temporal fossa. In the current medical literature, there are approximately 150 cases of MeNETs and even fewer cases with more than 10 years of follow-up, recurrence, and intracranial tumor progression. Therefore, we believe that this paper can make an important contribution to the existing and future knowledge about this disease. The purpose of this article is to present our experience in treating such a rare neoplasm in a 35-year-old woman. The patient initially complained of worsening hearing in her right ear over the past year. The final diagnosis was made based on the findings of computed tomography (CT), magnetic resonance imaging (MRI), and histological and immunohistochemical evaluation of excisional biopsies of the original and recurrent tumors. The primary tumor masses were removed with clear resection margins, and the ossicular chain was reconstructed. The patient has been monitored clinically and radiologically with temporal bone CTs every year and MRIs three times in general since then. A postoperative audiogram showed remaining mixed hearing loss in the right ear that eventually worsened as the tumor grew. Tumor recurrence and progression after 156 months (13 years) were seen on CT and MRI, requiring further treatment. After resection of the recurrent tumor, paresis of the right facial nerve developed, which was treated with dexamethasone. The surgical treatment caused the initial symptoms to disappear, but the facial nerve paresis persisted with mild functional improvement. The patient is not receiving adjuvant radiotherapy and is being monitored closely because the tumor may recur in the future.

A Case Report of Rare Sacral Solitary Fibrous Tumor.

Huge primary epidural solitary fibrous tumors in the sacrum are a rare clinical entity. The purpose of this article is to present our experience in treating such large and complex neoplasms in a 31-year-old woman. The patient complained of constant nocturnal bilateral hip and lower back pain and unilateral radicular symptoms (numbness, paresthesias) in the left S1/S2 dermatomal distribution. Diagnostic imaging, biopsy, preoperative endovascular embolization, two-staged tumor resection, and lumbosacroiliac fusion were performed. The treatment resolved the patient's neurological symptoms and resulted in overall good postoperative functionality. The patient has been in remission for more than five years despite her refusal of adjuvant radiotherapy.

Malignant mixed epithelial and stromal tumour of the kidney: a case report and a literature review.

INTRODUCTION: Mixed epithelial and stromal tumour of the kidney (MEST) is a rare and distinctive neoplasm accounting for 0.2% of all renal cancers. Most of these tumours behave in a benign fashion but 13 cases with malignant transformation have already been reported. We present the first case of an extremely aggressive MEST with rapid recurrence after radical treatment, demonstrating objective response to chemotherapy. CASE PRESENTATION: A 31-year-old female presented to the hospital complaining of gross hematuria. Computed tomography (CT) revealed an intraparenchymal mass in the left kidney forming a tumour thrombus in the inferior vena cava (IVC). Metastatic disease was ruled out and, under the clinical diagnosis of renal cell carcinoma, left radical nephrectomy with IVC thrombectomy was performed. The histopathological examination confirmed malignant MEST of the kidney. At the follow-up 12 months after surgery, a recurrent tumour in the left paravertebral area and a tumour thrombus in the IVC were detected. A second surgery was recommended and the mass from the paravertebral area was removed, so resection of the IVC with prosthetic replacement was performed. The histopathologic examination confirmed a recurrent malignant MEST. At the follow-up three months after the second surgery disease progression was diagnosed, so chemotherapy with ifosfamide and doxorubicin was initiated. The CT scan performed 14 months after the chemotherapy confirmed a stable process of the disease with no signs of progression. CONCLUSIONS: A literature review and our case report confirm the existence of extremely aggressive malignant MEST that shows response to chemotherapy. However, more reports are needed to improve our understanding about the biology of the MEST to develop any recommendations on personalized therapy.

Intrathecal Baclofen Therapy for the Treatment of Spasticity in Lithuania.

Spasticity of cerebral or spinal origin severely impairs an individual's functional ability and quality of life. Intrathecal baclofen (ITB) therapy via an implantable pump is indicated for use in patients unresponsive to oral antispasmodics. ITB therapy improves the daily caring for and relief of painful spasms. In Lithuania, ITB therapy was introduced in clinical practice just recently. We share our experience of spasticity management with the ITB pump system in five patients at Vilnius University Hospital Santariskiu Klinikos. Four patients had spastic tetraplegia associated with cerebral palsy, and one patient developed spastic paraplegia after a spinal epidural abscess.

Fatal Liver and Lung Alveolar Echinococcosis with Newly Developed Neurologic Symptoms due to the Brain Involvement.

The fox tapeworm Echinococcus multilocularis causes human alveolar echinococcosis, commonly affecting the liver. However, in ∼1% of cases, systematic spread of the disease involves the brain as well. A patient had a 6-year history of liver and lung alveolar echinococcosis that was considered not suitable for surgery, and treatment with albendazole was introduced. After the appearance of neurologic disturbances, an intracranial mass lesion was demonstrated by radiologic imaging. The lesion was surgically removed, and histologic analysis revealed metacestode tissue of E. multilocularis . Despite the surgical resection of the lesion, the patient died of progression of systemic alveolar echinococcosis. The authors highly recommend implementing neurologic monitoring to the follow-up algorithm for patients with systemically disseminated alveolar echinococcosis. When neurologic symptoms occur, radiologic imaging of the brain should be obtained immediately. Surgery should be considered for all intracranial echinococcal lesions, unless the lesion is located in the eloquent brain area.