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Symptomatic subependymoma

Report of 21 cases with review of the literature

Bernd W. Scheithauer

✓ Of 48 cases of subependymoma reported to date, 22 were associated with symptoms. In a personal series of 47 additional cases of subependymoma reviewed by the author, 21 were symptomatic. The mean age of patients with symptom-producing subependymomas was 39 years, that is, 20 years younger than that of patients with asymptomatic tumors. Symptoms were most often produced by large tumors, particularly those arising from the septum pellucidum (100%), the floor of the fourth ventricle (65%), and the lateral ventricular walls (55%). The majority of symptomatic tumors displayed the classic gross and microscopic features of subependymomas; however, large tumors more frequently demonstrated cyst formation, microcalcification, and vessel degeneration accompanied by hemorrhage. Of all subependymomas, 15% were microscopically composed of an admixture of classic subependymoma and cellular ependymoma; of these, one-half occurred within the first decade, all were situated in the fourth ventricle, and 80% were symptomatic. In contrast to pure subependymomas, the mortality rate of patients with mixed tumors was 80%, reflecting their growth potential, large size, and dangerous location.

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Phil W. Tally, Edward R. Laws Jr. and Bernd W. Scheithauer

✓ Tumor-to-tumor metastasis is a rare occurrence. Fewer than 100 cases have been reported, many being metastases from carcinomas to benign intracranial neoplasms, most often meningiomas. A case is presented of carcinoma metastatic to a glioma. The patient, who presented for evaluation of bifrontal headache, was found on computerized tomography to have a partially calcified right frontal mass. Craniotomy revealed an oligodendroglioma containing foci of adenocarcinoma. Further work-up disclosed an infiltrative ductal adenocarcinoma of the breast. It has been suggested that tumors of the central nervous system may provide a fertile substrate or an immunological “haven” for metastases.

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Federico Roncaroli, Bernd W. Scheithauer and Savvas Papazoglou

✓ A case of polymorphous hemangioendothelioma of the spinal cord is described. This 55-year-old woman presented with an 18-month history of lower-extremity sensorimotor deficit. A magnetic resonance image revealed an enhancing, intradural, extramedullary nodule at the T1–2 level. On gross inspection, the lesion measured 3.5 cm and was firmly attached to spinal cord parenchyma and adjacent nerve roots. It was completely removed. Fourteen months after surgery the patient's neurological deficit had resolved. Polymorphous hemangioendothelioma is a rare vascular tumor of borderline malignancy. Most occur in lymph nodes. None has been reported to occur in the central and peripheral nervous system. Based on current experience, resection and close follow up seems the best therapeutic approach.

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Federico Roncaroli, Bernd W. Scheithauer and William E. Krauss

Object. Hemangiomas of the spinal nerve roots are rare. Only 12 cases have been reported in the literature, all since 1965. These lesions occurred in adults, primarily in males, and were located in the cauda equina. Of the 12 lesions, eight were cavernous hemangiomas. The authors report here the clinicopathological features of 10 cases of hemangioma of the spinal nerve root, nine of which were of the capillary type.

Methods. The group included six men and four women who ranged in age from 40 to 62 years. The majority of patients presented with pain and weakness. One patient also experienced worsening of pain during menses. In all cases, gadolinium-enhanced magnetic resonance imaging or computerized tomography myelography demonstrated a discrete intraspinal extramedullary mass. Administration of contrast agent resulted in uniform, intense enhancement. A gross-total resection was achieved in all but one case; in most cases, it was necessary to sacrifice the parent nerve root. Preoperative symptoms and signs improved in nine cases. On histological examination, all but one tumor, a spindle-cell hemangioendothelioma, were shown to be ordinary capillary hemangiomas. Two lesions exhibited a cavernous component, and five showed a partly solid growth pattern resembling juvenile capillary hemangioma. In all cases, the relationship with the nerve root was histologically confirmed. The tumor—nerve relation varied. The intact nerve was displaced by the epineurial mass in three cases. The fascicles appeared separated in six cases, and individual nerve fibers were seen throughout one lesion with endoneurial involvement.

Conclusions. Hemangiomas of spinal nerve roots pose a challenging diagnostic problem. Knowledge of their existence is relevant in that they may mimic tumors and are amenable to surgical treatment.

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H. Gordon Deen Jr., Bernd W. Scheithauer and Michael J. Ebersold

✓ The clinical features and pathological materials of 51 cases of histologically verified meningiomas in patients under 21 years of age were reviewed. The age range was 7 to 20 years, with the majority of cases being clustered in the second decade of life. There was virtually a 1:1 sex ratio, with 26 females and 25 males. Thirty-eight patients had intracranial meningiomas, three had intraorbital tumors, and 10 had intraspinal tumors. Twelve patients (24%) had neurofibromatosis. Twenty patients (39%) had tumor recurrence. The cases were subdivided into five histological categories: meningotheliomatous, fibrous, transitional, psammomatous, and papillary. In each case, the clinical course was correlated with histological subtype. The 15-year survival rate in patients with intracranial meningiomas of all types was 68%. Factors adversely affecting survival included infratentorial location, papillary histology, and evidence of brain invasion.

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James T. Wolfe III, Bernd W. Scheithauer and David C. Dahlin

✓ Giant-cell tumor rarely affects the sphenoid bone. Ten cases of this tumor seen at the Mayo Clinic are reviewed, bringing the number of cases in the world literature to 31. These tumors, which present in the second and third decades of life and are slightly more frequent in women than in men, may present with various symptoms, including headache, visual field defects, blindness, and diplopia. The symptom complex and roentgenographic findings are seldom, if ever, sufficiently characteristic to allow preoperative diagnosis. The microscopic differential diagnosis of giant-cell tumor of the sphenoid region includes giant-cell reparative granuloma, aneurysmal bone cyst, fibrous dysplasia, and “brown tumor” of hyperparathyroidism. Interesting features of this series include the presence of multifocal giant-cell tumor in one case, and the absence of Paget's disease of bone in every case. Complete follow-up review in these cases allowed a discussion of therapy and prognosis; it appears that subtotal excision with postoperative radiation therapy is the treatment of choice. While the histological appearance of giant-cell tumor is characteristic, caution must be exercised in interpreting small samples, and various reparative and metabolic disorders must be considered in the differential diagnosis.

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Michael D. Partington, Bernd W. Scheithauer and David G. Piepgras

✓ An elevated serum carcinoembryonic antigen (CEA) without evidence of neoplasia was noted in an 84-year-old woman. She subsequently developed a lytic skull lesion, which at surgery proved to be an atypical meningioma. Immunohistochemical analysis demonstrated that this tumor was producing CEA. This aggressive meningioma required two further resections and adjuvant radiotherapy in the following 18 months. Serum elevation of CEA has been reported only twice in association with meningiomas; both of those tumors were of the secretory subtype.

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Federico Roncaroli, Bernd W. Scheithauer and H. Gordon Deen Jr.

✓ A case of multiple hemangiomas of the cauda equina nerve roots, conus medullaris, and lower spinal cord is described. The 74-year-old male patient presented with a 9-month history of progressive bilateral leg weakness. He had a history of lymphoma at the age of 39 years and renal cell carcinoma in his early 40s. Neither disease was evident at the time of this presentation. A magnetic resonance image revealed multiple enhancing nodules in the cauda equina region as well as on the pial surface of the lower thoracic spinal cord and conus medullaris. The patient underwent an L2–3 laminectomy. Cauda equina nerve roots were found to be studded with numerous purple nodules, the largest measuring 6 to 8 mm. The nodules were adherent to nerve roots from which they could not be resected. Two lesions were histologically examined and found to be capillary hemangiomas. Twelve months into an uneventful postoperative course, the patient is neurologically unchanged. This unique case might represent a distinct form of hemangiomatosis confined to the cauda equina nerve roots and spinal cord.

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Federico Roncaroli, Bernd W. Scheithauer and William E. Krauss

✓ The authors describe the clinicopathological features of four cases of capillary hemangioma of the spinal cord. All occurred in adult patients. The presenting symptoms were similar to those of more common intramedullary tumors. Radiologically, they resemble other vascular spinal cord tumors. All patients underwent surgery, and the outcomes varied. Histologically, the lesions resembled capillary hemangioma of skin or of soft tissue that is composed of lobules of small capillaries with associated feeding vessels, all enveloped by a delicate fibrous capsule. Capillary hemangiomas of the central and peripheral nervous system are extremely rare. Although examples of these lesions have been described as occurring in the dura mater and in peripheral nerve, including spinal nerve roots, none has occurred within the spinal cord. Knowledge of their existence may help practitioners to avoid misdiagnosis of tumor and resultant overtreatment of these benign lesions.