Long-Term Follow-Up Clinical Courses of Cerebellar Hemangioblastoma in von Hippel-Lindau Disease : Two Case Reports and a Literature Review

Although cerebellar hemangioblastomas are histopathologically benign, they yield a degree of malignant clinical behavior in long-term follow-up. We present two cases of long-term progression of renal cell carcinoma, which had been diagnosed as renal cysts during treatment for cerebellar hemangioblastoma. A 14-year-old male with von Hippel-Lindau disease was admitted for a cerebellar hemangioblastoma with multiple spinal hemangioblastomas and a renal cyst. After primary total resection of the cerebellar hemangioblastoma, the patient required two further surgeries after 111 and 209 months for a recurrent cerebellar hemangioblastoma. Furthermore, he underwent radical nephrectomy as his renal cyst had progressed to renal cell carcinoma 209 months after initial diagnosis. A 26-year-old male presented with multiple cerebellar hemangioblastomas associated with von Hippel-Lindau disease and accompanied by multiple spinal hemangioblastomas and multiple cystic lesions in the liver, kidney, and pancreas. He underwent primary resection of the cerebellar hemangioblastoma in association with craniospinal radiation for multiple intracranial/spinal masses. Unexpectedly, a malignant glioma developed 83 months after discovery of the cerebellar hemangioblastoma. At the same time, renal cell carcinoma, which had developed from an initial renal cyst, was diagnosed, and a radical nephrectomy was performed. In the view of long term clinical course, cerebellar hemangioblastoma associated with von Hipple-Lindau disease may redevelop even after primary total resection. In addition, associated lesions such as renal cysts may also progress to malignancy after the passing of a sufficient length of time.     

Surgical treatment options and management strategies of metastatic renal cell carcinoma to the lumbar spinal nerve roots

Spinal nerve root metastasis of renal cell carcinoma is a rare occurrence. In addition to treatment of the primary lesion, surgical resection of the nerve root metastasis, occasionally with sacrifice of the involved nerve, is the accepted standard of treatment. Resection often resolves presenting motor and pain symptoms due to relief of neural compression. We describe two patients with nerve root metastasis of renal cell carcinoma and their management. While locally advanced and metastatic renal cell carcinoma has been shown to be chemo- and radio-resistant, immunotherapy is a promising treatment. Given the high prevalence of systemic disease in patients with intradural metastases, systemic (and possibly intracranial) imaging can be used to identify other potential areas of disease.     

Ballistic-choreic movements as the presenting feature of renal cancer.

BACKGROUND: The paraneoplastic syndromes can involve multiple areas of the central nervous system and result in a variety of neurological symptoms. To our knowledge, severe, rapidly progressive, and drug-resistant ballistic-choreic movements have not been previously described as the presenting feature of renal cell carcinoma. PATIENT AND METHODS: A previously healthy 55-year-old man developed limb ballismus and involuntary choreic movements of his face over several weeks. Extensive laboratory, diagnostic, and radiographic studies failed to reveal a cause, until an abnormality on a chest x-ray film prompted a search for a primary neoplasm and a final diagnosis of renal cell carcinoma. High doses of medications traditionally used to treat choreic disorders had no effect on the abnormal movements. A biopsy specimen of the basal ganglia showed focal encephalitic changes but no malignant neoplasm. CONCLUSIONS: Whereas prior cases of paraneoplastic syndromes with chorea have been reported in other forms of cancer, our case was significant because, to our knowledge, renal cell carcinoma has not been previously reported in association with this syndrome. Furthermore, the chorea was categorically resistant to pharmacological treatment, and the movement disorder was the initial and only focal neurological feature of the primary illness.     

Successful long-term treatment of multiple metastases from renal cell carcinoma: combination of stereotactically guided percutaneous single-dose convergent beam irradiation and surgery

The case of a patient suffering from renal cell carcinoma and recurrent brain metastases shows how the survival period can be significantly prolonged by a combination of stereotactically guided percutaneous single-dose convergent beam irradiation and surgery. This 58-year-old man's left kidney was completely excised because of a renal cell carcinoma. After 18 months and 25 months, respectively, a right frontal brain metastasis was operated on. In the next 7 years, biannual MRI checks were carried out which successively showed five different brain metastases, each of which was immediately subjected to single-dose stereotactic irradiation (median dosage: 20 Gy prescribed to the 80% isodose). In the following 2 years, operations were carried out on two metastases which could not be treated by radiation because of their considerable size and partial compression of the ventricle. In the next 3 years, four more brain metastases were subjected to single-dose stereotactic irradiation. There were no metastases in the other organs. At present, the patient is in good clinical condition and mobile. A negative prognosis is usually delivered for patients suffering from renal cell carcinoma and brain metastasis. However, in individual cases, the survival period can be significantly prolonged by regular MRI examinations and a combination of neurosurgery and single-dose stereotactic irradiation.     

Intramedullary spinal cord metastasis as a first manifestation of a renal cell carcinoma: report of a case and review of the literature

The authors report the case of a 70-year-old woman who developed a Brown–Sequard-syndrome within 6 weeks caused by an intramedullary spinal cord metastasis of an occult renal cell carcinoma. Intramedullary metastases are rare and represent only 4–8.5% of central nervous system metastases. An important feature of intramedullary metastases is the rapid progression of neurological deficits which necessitates rapid treatment. There are only eight earlier reports of intramedullary metastasis due to renal cell carcinoma (Schiff D, O'Neill BP. Intramedullary spinal cord metastases: clinical features and treatment outcome. Neurology 1996;47:906–12; Belz P. Ein Fall von intramedullaerer Grawitz-Metastase im Lumbalmark. Frankfurt Z Pathol 1912;10:431–44; Gaylor JB, Howie JW. Brown–Sequard-syndrome. A case of unusual aetiology. J Neurol Neurosurg Psychiatry 1938;1:301–5; Kawakami Y, Mair WGP. Haematomyelia due to secondary renal carcinoma. Acta Neuro Pathol 1973;26:85–92; Strang RR. Metastatic tumor of the cervical spinal cord. Med J Aust 1962;1:205–6; Von Pfungen. Über einige Fälle von Haematomyelie nichttraumatischen Ursprungs. Wien Klin Rdsch 1906;20:44–50; Weitzner S. Coexistent intramedullary metastasis and syringomyelia of cervical spinal cord. Report of a case. Neurology 1960;674–8). To the best of our knowledge this is the first report on a patient in whom symptoms from the metastasis of a renal cell carcinoma preceded the detection of the primary tumor. This report presents the clinical, neuroradiological and histopathological findings of an intramedullary metastasis of a renal cell carcinoma and provides an overview of the literature on intramedullary spinal cord metastases.     

Cervical clear cell meningioma mimicking a vertebral metastasis.

Cervical vertebral involvement of clear cell meningioma is very rare. We report a case of clear cell meningioma in the cervical vertebral body in a 72-year old male. Seven years prior to this presentation, the patient underwent palliative surgery and posterior instrumentation for a cervical vertebral tumor at C5, which had been diagnosed as a metastatic renal cell carcinoma. On this admission, the patient presented with severe neck pain. Examination revealed hypesthesia on the left in a C6 nerve root distribution. Plain X-rays and MRI revealed an enlarging tumor in the C5 and C6 vertebral bodies. The tumor was resected via an anterior approach followed by fusion using a strut bone graft. Histological examination of the surgical specimen diagnosed a clear cell meningioma. Postoperatively, the patient achieved pain relief and resolution of the neurological deficit. At follow-up two years postoperatively, he remains asymptomatic. We emphasize that cervical clear cell meningioma with involvement of the vertebral bodies may mimic metastatic renal cell carcinoma.     

Clinical features and surgical management of four peculiar cases of intracranial metastases from renal cell carcinoma

Intracranial metastasis from renal cell carcinoma is rare. In this study, four patients (age range 56–72 years) were treated for intracranial metastases from renal cell carcinoma. The metastasis was solitary in all four patients and was located in the temporoparietal region in two patients, cerebellum in one patient, and bilateral lateral ventricles in one patient. Preoperative magnetic resonance imaging showed hemorrhage and necrosis in the tumor in all four patients, and one patient had edema in the region of the tumor. All patients were treated with craniotomy for tumor resection, and histopathologic examination showed clear cell carcinoma. Marked bleeding occurred in all patients during the operation, but preoperative direct injection of ethanol into epidural metastases (2 patients) was effective in decreasing intraoperative blood loss. Prognosis was poor with limited survival time (range 2 weeks–45 months), but prognosis was best in the two patients who were treated with postoperative radiation therapy and chemotherapy (survival times 26 and 45 months). To our knowledge, this is the largest report of four uncommon intracranial metastases from RCC in Chinese patients. In summary, intracranial metastasis from renal cell carcinoma should be considered in the differential diagnosis of intracranial tumor because of the high risk of bleeding and poor prognosis.     

Reversible demyelinating neuropathy associated with renal cell carcinoma

Paraneoplastic neuropathy is a potential complication of renal cell carcinoma. The clinical and electrophysiologic features of such patients have not been well characterized. We describe a patient with a demyelinating neuropathy associated with papillary renal cell carcinoma that resolved following nephrectomy. The literature is reviewed with particular attention to the clinical, electrophysiologic, and kidney histopathologic characteristics of similar patients. Features arguing against other acquired demyelinating neuropathies are highlighted, and we conclude that abdominal imaging may provide important diagnostic data in some patients with evolving demyelinating neuropathies. The significance of papillary renal cell carcinoma (as opposed to clear renal cell carcinoma) is unknown, but may have important pathophysiologic implications.     

Solitary choroid plexus metastasis from carcinoma of the oesophagus.

Metastatic tumors to the brain presenting exclusively in the choroid plexus are rare and are most frequently associated with renal cell carcinoma. In this paper, the authors report an unusual case of intraventricular metastasis, and to the authors' knowledge, this is the first case of solitary metastasis from oesophageal carcinoma to the choroid plexus to be described in the literature. Metastatic disease is an important differential diagnosis which must be considered even for a patient without a documented primary malignancy who presents with a single lesion in the ventricle.     

A rare case of metastatic renal cell carcinoma resembling a nerve sheath tumor of the cauda equina.

We present a rare case of solitary metastasis to the cauda equina from the kidney. The patient was a 68-year-old man with a two-year history of low back pain. His past medical history revealed a renal cell carcinoma diagnosed seven years earlier. His lumbosacral MR imaging showed a well-demarcated, intradural extramedullary mass at the L3 level. He underwent an L2-4 laminectomy. The operative findings of the tumor quite resembled that of a nerve sheath tumor. It did not infiltrate into the subarachnoid space and involved only one spinal nerve. Pathology of the tumor was a metastasis of the renal cell carcinoma. Only 10 cases with such a metastasis to the cauda equina have been reported in the English literature. We added the 11th and reviewed the literature with reference to tumor pathologies, clinical findings and route of metastasis to the cauda equina.     

Annexin 2 and hemorrhagic disorder in vascular intimal carcinomatosis

Vascular intimal carcinomatosis refers to a characteristic tumor proliferation on vascular intima that replaces normal endothelium. This pathological event of unknown cause is quite different from tumor thrombotic microangiopathy due to the absence of thrombi on the tumor cell surfaces. We analyzed renal transitional cell carcinoma cases with metastasis to the main pulmonary arteries and marked hyperfibrino(geno)lysis. The fibrinogen-derived products from patients' plasma were identified as D1A/gamma, D1/gamma, and D1/beta by immunoblotting with the NH2-terminus of the fragment D specific antibody JIF-23. In all cases, the neoplastic cells with vascular intimal carcinomatosis were stained positive for anti-human annexin 2, which is a unique cell surface co-receptor for plasminogen and tissue-type plasminogen activator. In contrast, normal renal pelvic mucosa or renal transitional cell carcinoma without vascular intimal carcinomatosis did not express any annexin 2. The isolated transitional cell carcinoma cells contained annexin 2 mRNA and expressed its protein. Anti-annexin 2 antibody and transfection of annexin 2 small interfering RNA into these carcinoma cells significantly inhibited tissue-type plasminogen activator dependent plasmin generation. These findings suggest that annexin 2 mediated fibrinolysis on the transitional cell carcinoma cells may play a role in inducing hemorrhagic disorder in vascular intimal carcinomatosis.     

Metastatic chromophobe renal cell carcinoma to the brain

Metastases represent the most common tumors of the central nervous system, with clear cell renal cell carcinomas showing a particular predilection to involve the brain. This report documents an unusual case of a patient with a chromophobe renal cell carcinoma presenting with a brain metastasis. A 58-year-old man presented with stroke-like symptomatology, including expressive aphasia, right side facial weakness, headaches and vomiting. CT imaging demonstrated a 4.7 cm left frontal lobe hemorrhagic mass. He underwent surgery with excision of the mass, which was marked by sheets of large cells with lightly eosinophilic to clear cytoplasm. PAX8 staining was positive and a diagnosis of clear cell carcinoma of probably renal origin was rendered. Subsequently, CT imaging of the abdomen revealed a 12.9 cm left renal mass. The patient underwent a left nephrectomy and a diagnosis of chromophobe renal cell carcinoma was made. The tumor was noted to extend into the perirenal fat and to have involved a paraaortic lymph node. Re-review of the frontal lobe lesion confirmed the tumor was the same. Chromophobe renal cell carcinomas are far less common than clear cell tumors, are less likely to metastasize, and generally have a more favorable overall prognosis. When they metastasize, they most commonly involve the liver, lung and lymph nodes, in contrast to the more common clear cell carcinomas that typically spread to the lungs, bone and brain.     

Renal cell carcinoma metastatic to the choroid mimicking intraventricular meningioma

BACKGROUND: Metastatic renal cell carcinoma to the choroid plexus is a rare condition and can be easily confused with meningioma. METHODS: A 61-year-old female presented with progressive neurologic deterioration and MRI findings of obstructive hydrocephalus and a homogeneously contrast enhancing 3 cm oval mass in the trigone of the left lateral ventricle. RESULTS: Despite radiologic, intraoperative gross features, and frozen pathology all consistent with meningioma, the final pathology revealed metastatic renal cell carcinoma. CONCLUSION: Renal cell carcinoma metastatic to the choroid plexus can mimic intraventricular meningioma. We present a review of the literature and comparison of the radiological features of meningiomas and metastatic renal cell carcinoma. We also discuss the use of an under-utilized technique, the contralateral transcallosal approach, in the surgical treatment of this intraventricular lesion.     

百忧解治疗抑郁性神经症的疗效观察

目的： 观察百忧解治疗抑郁性神经症的疗效。　方法： 对３３ 例抑郁性神经症患者给予每日２０ｍ ｇ 百忧解治疗, 不合用其它精神药物, 并用Ｚｕｎｇ 自评量表 （ＳＤＳ）、自评焦虑量表（ＳＡＳ） 进行治疗前后对照。　结果： 痊愈１３例, 显效１５ 例, 进步３ 例, 无效２ 例, 总有效率９４．０％ 。　结论：百忧解是一种快速、强效而安全的抗抑郁药。     

肝移植治疗肝原发性鳞状细胞癌1例

景：肝原发性鳞状细胞癌较罕见,各种治疗方法疗效不佳,预后差。 目的：随访观察1例肝移植治疗肝原发性鳞状细胞癌患者的近期疗效。 方法：观察中山大学附属第一医院1例肝原发性鳞状细胞癌患者经肝移植治疗后的情况,并回顾性分析迄今为止见于文献报道的国内外65例肝原发性鳞状细胞癌的临床表现、组织来源、治疗方法及预后。 结果与结论：该患者经肝移植后肝功能恢复正常,肿瘤标志物癌胚抗原、糖类抗原199、鳞状细胞癌抗原等均降至正常水平,恢复良好。国内外研究表明,肝原发性鳞状细胞癌的临床表现不典型,组织来源多样,手术切除、化疗等治疗方法疗效不佳,预后差。本例结果显示,肝移植治疗肝原发性鳞状细胞癌近期疗效良好。     

Peripheral neuropathy in oxalosis. A case report with electron microscopic observations.

A 61 year old man had chronic renal failure because of oxaluria and renal calculi. Two years before death, while on hemodialysis, he developed severe progressive peripheral neuropathy. At autopsy calcium oxalate crystals were found in the peripheral nerves and other tissues. Nerve lesions included segmental demyelination, axonal degeneration and crystalline deposits within the myelin sheath. Ultrastructurally there were foci of osmiophilic granular material within myelin lamellae and endoneurium, and pleomorphic lamellar bodies in the perinuclear Schwann cell cytoplasm. It is probable that chronic hemodialysis favors the deposition of oxalate in the Schwann cells and the development of neuropathy in patients with primary hyperoxaluria and renal failure.     

Calvarial metastases as clinical presentation of renal cell carcinoma: report of two cases and review of the literature

Calvarium is a frequent target site of involvement for common neoplasms. Some cases of calvarial metastases have been reported in literature as secondary lesions from renal cell carcinoma (RCC), but only five cases have been described concerning calvarial mass as the first clinical presentation of this kind of tumor. In this report, we discuss the clinical aspects of two further cases we observed, in which the renal cell carcinoma was found thanks to the histological examination of a calvarial mass after surgery. We also briefly review the literature.     

Dural metastases mimicking meningioma. Case report and review of the literature.

The typical appearance of meningioma on CT and MRI is well known. Particularly in the elderly, the imaging appearance is sometimes considered diagnostic of these benign tumours without histopathological confirmation. However, other more aggressive neoplasms can present with a classical CT and MRI appearance of meningioma, indicating the need for histopathological confirmation wherever possible. We report a case of dural metastases which, on both pre-operative CT and MRI and at surgery, had the typical appearance of a falcine meningioma. Histopathology and immunohistochemistry revealed adenocarcinoma of renal cell origin, and the renal primary was identified on subsequent abdominal investigation. The literature regarding dural metastases is reviewed. To our knowledge, this is the first reported case of a renal carcinoma metastasizing directly to the dura. Although rare, dural metastases can mimic meningioma, and this needs to be considered if conservative therapy or radiosurgery are to be offered to a patient with radiological diagnosis of meningioma.     

Intracranial collision tumor mimicking an octreotide-SPECT positive and FDG-PET negative meningioma.

OBJECTIVE/IMPORTANCE: Cancer metastasis to a pre-existing intracranial tumor is rare, but several cases have been reported. We report an unusual case of a "collision tumor" consisting of a renal cell carcinoma metastasis to an intracranial meningioma. CLINICAL PRESENTATION: A 67-year old male with renal cell carcinoma had an asymptomatic right posterior frontal dural-based lesion identified on a screening CT scan. MRI characteristics of the tumor were consistent with meningioma. On octreotide-SPECT and F-18 fluorodeoxyglucose (FDG)-PET scans, the lesion showed octreotide uptake but did not accumulate FDG, both of which are consistent with a diagnosis of benign meningioma. One week later, he presented with a 1-day history of progressive left-sided weakness. The intracranial tumor was resected, and subacute subdural blood was found overlying a soft, reddish tumor. Microscopic examination was consistent with renal cell carcinoma with a minor portion consisting of meningioma. The meningothelial component was strongly immunoreactive to vimentin and weakly reactive to epithelial membrane antigens. Neither area reacted with glucose transporter-1 (GLUT-1), correlating with low FDG-PET uptake. CONCLUSION: Collision tumor involving metastatic renal cell carcinoma to an intracranial meningioma is a rare occurrence. Diagnosis by non-invasive means, with use of a combination of octreotide-SPECT and FDG-PET may not accurately reflect the malignant component of such a collision tumor. In this case, the collision tumor also demonstrated a propensity to undergo spontaneous hemorrhage. A high degree of suspicion of intracranial metastasis should be maintained for patients who have known systemic cancer and are found incidentally to have a dural-based mass lesion.     

Myasthenia gravis and primary squamous cell carcinoma of the thymus: a case report

We detail a case of primary squamous cell carcinoma of the thymus in a patient with myasthenia gravis. The clinical course of the patient and the gross and histologic appearance of the thymus are discussed. Appropriate therapy for patients with these tumors includes resection of the tumor followed by radiation therapy and continued surveillance for recurrence. Review of the literature indicates less than 50% survival at 10 years and 22% recurrence rate at 5 years for patients with this tumor.