Shu-Shong Hsu

Skull metastasis from uterine leiomyosarcoma: a case report.

Uterine leiomyosarcoma is a rare malignancy whose presenting symptoms usually are not specific for the disease. The clinical presentations include vaginal bleeding, pelvic pain or pressure, and awareness of an abdominal-pelvic mass. The diagnosis should be considered if rapid uterine enlargement occurs, especially in a post-menopausal woman. Local spreading of the tumor could involve the myometrium, pelvic blood vessels and lymphatics, contiguous pelvic structures, and then the abdominal organs, whereas distant metastasis most often involve the lungs. A 63-year-old female presented a rare uterine leiomyosarcoma metastasis to the skull with the manifestions of a bulging mass over her left occipito-parietal region. On admission, neurological examination revealed right hemiplegia. She underwent total resection of the tumor with the reconstruction of the dura, the skull plate and the scalp. Her post-operative course was smooth and the muscle power of her right limbs was improved from grade 1 to grade 3 after the procedure. The histological diagnosis is leiomyosarcoma. Aggressive management of the metastatic skull tumor is recommended in selected patients at least for a betterquality of life.

Spontaneous Regression of Lumbar Herniated Disc

Intervertebral disc herniation of the lumbar spine is a common disease presenting with low back pain and involving nerve root radiculopathy. Some neurological symptoms in the majority of patients frequently improve after a period of conservative treatment. This has been regarded as the result of a decrease of pressure exerted from the herniated disc on neighboring neurostructures and a gradual regression of inflammation. Recently, with advances in magnetic resonance imaging, many reports have demonstrated that the herniated disc has the potential for spontaneous regression. Regression coincided with the improvement of associated symptoms. However, the exact regression mechanism remains unclear. Here, we present 2 cases of lumbar intervertebral disc herniation with spontaneous regression. We review the literature and discuss the possible mechanisms, the precipitating factors of spontaneous disc regression and the proper timing of surgical intervention.

Primary vaginal extraosseous Ewing sarcoma/primitive neuroectodermal tumor with cranial metastasis.

Extraosseous Ewing sarcoma is now regarded as a member of the Ewing sarcoma/primitive neuroectodermal tumor (PNET) family. It typically involves the soft tissues of the chest wall, pelvis, paravertebral region, abdominal wall, retroperitoneal region and extremities of children, adolescents and young adults, but it seldom occurs in the female genital tract. We report an extremely rare case of retrospective diagnosis of vaginal extraosseous Ewing sarcoma/PNET which metastasized to the right frontoparietal scalp, skull, and dura. Surgical resection, followed by adjuvant radiotherapy and chemotherapy resulted in a favourable clinical outcome. Both the vaginal and head tumors had similar light microscopic features supporting the diagnosis.

Orbital apex syndrome due to aspergillosis with subsequent fatal subarachnoid hemorrhage.

Background: Orbital apex syndrome has been described previously as a syndrome involving damage to the oculomotor nerve (III), trochlear nerve (IV), abducens nerve (VI), and ophthalmic branch of the trigeminal nerve (V1), in association with optic nerve dysfunction. It may be caused by inflammatory, infectious, neoplastic, iatrogenic, or vascular processes. Case Description: A 73-year-old female having hypertension and rheumatoid arthritis stage 4 under long-term corticosteroid therapy presented to us with the right side orbital apex syndrome. Her magnetic resonance imaging (MRI) of orbit showed progression of a lesion at the right orbital apex and adjacent right superior orbital fissure with mild extension to the right posterior ethmoid sinus. She underwent endoscopic endonasal transethmoid approach with the removal of the lesion. The pathology showed a picture of fungal infection and the culture of the specimen proved Aspergillus fumigatus. Her postoperative course was smooth until 5 days after surgery, when she suffered a massive spontaneous subarachnoid hemorrhage resulting from a ruptured aneurysm, which was proven by computed tomography angiography (CTA) of brain. Unfortunately, she expired due to central failure. Conclusion: In cases of immunocompromised patients having orbital apex syndrome, fungal infection should be kept in mind. One of the most lethal but rare sequels of CNS fungal infection is intracranial aneurysms. Early diagnosis and radical resection, combined with antifungal medications is the key to save this particular group of patients.

Dyspnea and choking as presenting symptoms in primary medulla oblongata germinoma.

Background: The medulla oblongata is the lower half of the brainstem. It contains the cardiac, respiratory, vomiting, and vasomotor centers and deals with autonomic functions such as breathing, heartbeat, and blood pressure. Primary medulla oblongata germinoma is very rare and less than 20 cases have been reported in the English literature. Case Description: A 22-year-old female without any particular past medical history presented to us in October 2012 with the chief complaint of dyspnea and frequent choking for 1 month. Neurological examination revealed lower cranial nerve palsies and nystagmus. Her brain computed tomography (CT) and brain magnetic resonance imaging (MRI) demonstrated a mass lesion at the dorsal surface of medulla oblongata with extension into the inferior fourth ventricle and foramen magnum. She underwent bilateral suboccipital craniotomy and C1 laminoplasty with the grossly total resection of the tumor. The histological examination of the tumor proved germinoma. Postoperative adjuvant radiotherapy was arranged. The latest brain MRI and whole spine MRI done 1 year after surgery showed neither residual nor recurrent tumor in the whole axis. She is regularly followed-up at our outpatient department and is doing well except having left vocal cord palsy, which occurred before surgery. Conclusion: Medulloblastoma, ependymoma, glioma, hemangioblastoma, and cavernous angioma are common intraaxial tumors in the medulla oblongata and fourth ventricle. Intracranial germ cell tumors originate from extragonadal seminal cells and have been found in 0.4-3.4% of patients with primary central nervous system (CNS) tumors in Western countries, while the incidence is reported to be 5-8 times greater in Japan and the Far East. Although germinoma of medulla oblongata is rare and difficult to diagnose preoperatively, it should be included in the differential diagnosis of medulla masses with fourth ventricle extension, especially in Asian population.

Predictive Risk Factors for Fracture at Catheter of Totally Implantable Venous Access Devices via Subclavian Vein Insertion

Background: Fracture of totally implantable venous access devices (TIVAD) is a rare but potential serious complication. We aimed to explore the risk factors for fracture at catheter of TIVAD via subclavian vein insertion. Materials and Methods: From January 2008 to July 2010 the records of 34 patients with fractured TIVAD were retrospectively reviewed. The comparison group included 170 randomly selected cases (5 for every patient with a fractured catheter) from 3919 cases of TIVAD implantations without catheter fracture. Variables associated with catheter implantation and the relationship between the catheter and the clavicle were analyzed. Results: Statistical analysis revealed implantation method, duration of implantation, brand of device, and port-clavicle distance were predictive risk factors for catheter fracture on univariate analysis. Duration of implantation ＞200 days and port-clavicle distance ＜2.5 cm were independent risk factors for catheter fracture on multivariate analysis. Stratified analysis indicated that a port-clavicle distance ＜2.5 cm in patients with duration of TIVAD implantation ＞200 days was associated with a significantly increased risk of catheter fracture. Conclusions: We suggested that TIVAD with a port-clavicle distance ＜2.5 cm could be removed to prevent catheter fracture when the implantation duration is more than 200 days.

Left orbital roof giant cell tumor of bone: A case report

Background: Giant cell tumor of bone originating from the connective tissue within the bone marrow is benign but locally aggressive lesion. In all, 90% of the cases involve the epiphysis of long bones and less than 2% involve the skull. Giant cell tumors of the skull occur most frequently in the sphenoid and temporal bones, and very rarely in the ethmoid, frontal, parietal, and occipital bones. We would like to share a case of giant cell tumor of bone arising from the left orbital roof with involving ethmoid sinus, which was diagnosed to be a meningioma before surgery. Case Description: A 32-year-old lady presented to us with the chief complain of left proptosis, diplopia, and left eye soreness without decline of visual acuity for about 2 months. Her orbital magnetic resonance imaging (MRI) disclosed a mass lesion located in the left frontal base, orbital roof, and upper medial orbital region with adjacent dural-tail sign favoring meningioma. She underwent a left supraorbital pterional craniotomy with the gross total removal of tumor and dura reconstruction. Histology examination of the tumor showed a picture of giant cell tumor of bone. Considering giant cell tumor of bone is locally aggressive, postoperative adjuvant therapy with Denosumab was introduced after full explanation. Conclusion: Standard treatments of skull-base giant cell tumors have yet to be established due to small number of cases reported in the literature. The standard treatment of giant cell tumor of bone is complete resection of the tumor.

A case of delayed tension pneumocephalus nine years after craniectomy

Introduction: Pneumocephalus is a collection of air in the cranial cavity. It appears commonly after supratentorial craniotomies, whereas tension pneumocephalus is a rare but potential life-threatening complication of intracranial surgical procedures. Case Report: A 27-yearold female patient, who was diagnosed with right cerebellopontine angle tumor, received twice suboccipital craniectomies for tumor removal. Postoperatively, she had subclinical pneumocephalus, which progressed into tension pneumocephalus after nine years. Conclusion: Tension pneumocephalus may develop in a delayed manner. Therefore, close follow-up, early diagnosis and management of recurrent pneumocephalus are crucial to prevent morbidity and mortality.

Glioblastoma with Both Oligodendroglioma and Primitive Neuroectodermal Tumor-Like Components in a Case with 9-Year Survival

Introduction Glioblastoma multiforme (GBM), the most common primary malignant brain tumor in adults, is characterized by extensive heterogeneity in its clinicopathological presentation. A primary brain tumor with both astrocytic differentiation and neuronal immunophenotype features is rare. Here, we report a long-term survival patient who presented this rare form of GBM in the disease course. Presentation of Case A 23-year-old woman, presenting with rapidly progressive headache and right-side weakness, was diagnosed with brain tumor over the left basal ganglion. She underwent the first craniectomy for tumor removal, and histopathology revealed classic GBM. Tumor recurrence occurred 8 years later. Another gross total resection was performed and pathology revealed GBM with the oligodendroglioma component (GBM-O). Due to disease progression, she received debulking surgery the following year. The third pathology revealed glioblastoma with primitive neuroectodermal tumor-like component (GBM-PNET). Discussion GBM-PNETs are collision tumors with both neuronal and glial components. They are rare, and a few case reports have suggested that these tumors are associated with favorable outcomes but a higher risk of cerebrospinal fluid dissemination. Conclusion We report a patient who developed the distinct pathologic variants of classic GBM, GBM-O, and GBM-PNET, throughout the disease course. Young age, aggressive surgical resection, and pathologic and genetic features may have contributed to the long-term survival of the patient.

Intracranial Atypical Meningiomas: A Case Series

Background: Atypical meningiomas fall into a category World Health Organization Grade II, which have higher local recurrence rates and lower survival rates than their benign counterparts. The aim of this study is to review the outcome of newly diagnosed patients with atypical meningioma after therapy. Methods: We conducted a retrospective review of the medical records of patients having atypical meningiomas who were treated in our hospital between January 2005 and December 2014. Their age, sex, initial presentation, tumor location, tumor size, extent of resection, tumor recurrence or tumor progression, duration of follow-up, adjuvant therapy, and outcome were reviewed. Results: There were 27 consecutive patients (15 male and 12 female) having fresh intracranial atypical meningiomas treated in our hospital between January 2005 and December 2014. Their mean age at diagnosis was 60.81 years. Twenty-three patients (85.19%) underwent total resection of the tumor, whereas 4 patients (14.81%) had partial resection of their tumors during their first time of surgery. Fifteen patients (55.56%) had finished adjuvant radiotherapy. Nine patients (33.33%) had tumor progression or recurrence during follow-up, and 4 of them were proved to have malignant transformation to anaplastic meningiomas in the following operations. The mean time to tumor progression or recurrence of these nine patients was 17.67 months. Nineteen patients (70.37%) had a favorable outcome, 7 patients (25.93%) had an unfavorable outcome, and we lost 1 patient (3.7%) due to disease progression. Conclusions: Surgery remains the standard treatment to atypical meningioma, and postoperative adjuvant radiotherapy is still controversial especially to those who undergo total surgical resection of the tumors. Our study reveals that early postoperative adjuvant radiotherapy seems to play a role in local control. Atypical meningioma can have malignant transformation to anaplastic meningioma, so aggressive treatment and follow-up are essential to manage this particular tumor.