George M. Kleinman

Primary neuroectodermal tumors of the ovary. A report of 25 cases

Twenty-five primary ovarian neuroectodermal tumors occurred in females from 6 to 69 (average, 23) years of age; they had the usual presenting symptoms of abdominal swelling or pain. The tumors, which varied from cystic to solid, ranged from 4 to 20 cm (average, 14 cm) in diameter. Microscopic examination revealed three histologic categories—differentiated, primitive, and anaplastic— with the tumors in the first group having a better prognosis than those in the other two groups. Five of the six differentiated gliomas were pure ependymomas, and one was an ependymoma with an astrocytoma component; none contained teratomatous elements. Two patients with stage I tumors were alive 4 and 5 years postoperatively. The one patient with stage IIA tumor was free of disease at 3 years; one of the two patients with a stage III tumor died of tumor after 5 years, and one had two recurrences but was alive and well at 5 years. Twelve tumors were primitive, resembling medulloepithelioma, ependymoblastoma, neuroblastoma or medulloblastoma. Seven tumors had teratomatous foci of other types, including three dermoid cysts. Three patients with stage I tumors were alive at 7 months, 3 years, and 9 years postoperatively; six of seven patients with stage III tumors died of tumor 2 to 20 months postoperatively, and one was alive with disease at 1 year. Seven tumors were anaplastic, resembling glioblastoma. All contained foci of squamous epithelium. One patient with stage IA tumor died of tumor at 2 years, but two were free of tumor after 3 and 4 years. One patient with a stage IIA tumor died of disease after 5 years; another was alive with tumor at 1 year. One patient with a stage III tumor died after 4 months. The differential diagnosis of neuroectodermal tumors of the ovary includes many primary and metastatic ovarian neoplasms of diverse types, and distinction among them is important. Neuroectodermal tumors should be considered when examining unusual ovarian tumors, particularly if the patient is young.

Medulloblastoma: The identification of prognostic subgroups and implications for multimodality management

For 43 medulloblastoma patients who had five‐and ten‐year actuarial survival rates of 56%, prognostic factors of statistical significance included: T‐stage (82% T1,2; versus 46% T3,4; P < 0.02), M‐stage (63% M0,1 versus 0% M2,3; P < 0.03), and histopathologic tumor score (TS, based upon necrosis, desmoplasia, cytoplasmic processes, and mitoses) (81% TS ⩽ 5 versus 41% TS ⩾ 6; P < 0.05). Posterior fossa local control rates were also function of T‐stage (90% T1,2 versus 38% T3,4) and TS (83% TS ⩾ 5 versus 38% TS ⩾ 6). Combining TS with T‐stage, patients fell into three prognostic and local control groups, which may have different future management implications: Small (T1,2) tumors of favorable (TS ⩽ 5) histology had a 92% ten‐year actuarial survival rate with 100% (8/8) local control; no change from current management is suggested. For the intermediate prognosis group (T1,2‐TS ⩽ 6 or T3,4‐TS ⩾ 5 with 67% and 70% survival, respectively), increasing the irradiation dose alone may improve survival because these tumors exhibited an irradiation dose‐response relationship. However, it is the poor prognosis group (T3,4‐TS ⩾ 6 with 42% survival) which might be suitable for future adjuvant chemotherapy or radiosensitizer trials since there is no evidence that higher irradiation doses improve local control. This article identifies prognostic subgroups based on histologic type and TM staging in medulloblastoma patients which potentially may be utilized to improve therapeutic results, and confirms the value of staging patients with central nervous system malignancies.

Systemic metastases from medulloblastoma: report of two cases and review of the literature.

The clinical and pathologic data from two cases of medulloblastoma with systemic metastases, and 101 previously reported cases were evaluated to define better the clinical presentation and natural history. Patients ranged in age from six months to 48 years, with a mean of 13 years; two thirds of the patients were male. Ventricular shunts had been inserted in 20% of the patients. Systemic metastases occurred on an average of two years after the diagnosis of the primary tumor in patients without shunts, but only 1.3 years in patients with shunts. Fifty‐nine percent of the patients were known to have experienced recurrence or spread of medulloblastoma within the central nervous system by the time systemic metastases appeared. Ninety percent showed radiologic evidence of bone metastases, of which 60% were osteoblastic. Bones most frequently involved were pelvis, femur and vertebrae; pain was the most common initial symptom. At autopsy, lymph node metastases were found in 65% and liver metastases were found in 28% of all cases in addition to bone metastases in 82%. Lung metastases occurred in 9% of the patients without shunts, compared with 30% of patients with shunts. The average survival was seven months after the appearance of systemic metastases for patients both with and without shunts. Approximately 5% of patients with medulloblastoma may be expected to develop systemic metastases. This development is associated with increased morbidity and a shortened life expectancy.

Computed Tomography of Herpes Simplex Encephalitis, with Clinicopathological Correlation

The computed tomographic (CT), clinical, and pathologic findings in 9 patients with biopsy-proven herpes simplex encephalitis (HSE) were reviewed. Seventeen scans (5 plain, 12 contrast enhanced) were analyzed. The major CT finding was a low-absorption abnormality, present in 11 of 17 scans (64%), affecting primarily the temporal lobes. Mass effect was evident in 9 of 17 (52%). Of the 12 contrast-enhanced scans, 6 (50%) showed an abnormal enhancement pattern affecting primarily the sylvian fissure and opercular areas. CT abnormalities are characteristic enough that, in the proper clinical setting, they lend support to the correct diagnosis.

Malignant tumors of nerve sheath origin

A clinicopathologic study of malignant nerve sheath tumors diagnosed between 1962 and 1979 at the Massachusetts General Hospital was undertaken. During this 17‐year period, 24 patients with such a neoplasm were seen while 607 patients were treated for schwannoma or neurofibroma. Criteria were developed for establishing the nerve sheath origin and malignancy of a given neoplasm which allowed a secure diagnosis of malignant nerve sheath tumor to be made in 11 of the 24 cases. In 7 of the remaining cases, the malignant tumors were of uncertain histogenesis and in 6 cases, a new diagnosis was made. The malignant tumors constituted 2% of all neoplasms of nerve sheath origin diagnosed during this period. An attempt was made to grade these neoplasms, but clinical and histologic parameters were only approximately predictive of their behavior. The treatment and survival of the 11 patients with malignant nerve sheath tumors and the 7 with malignant neoplasms of uncertain histogenesis are detailed and compared. In the former group, 4 patients died within four and a half years and there were eight recurrences in 4 patients. In the latter group, 4 died within three and a half years and there were five recurrences in 3 patients.

Whipple disease of the nervous system

A 58-year-old man with dizziness and unsteady gait had a 10-year history of behavioral change, impotence, and a progressive peripheral neuropathy. CT revealed low-density, contrast-enhancing lesions in the right pontine tegmentum and the right medial temporal lobe. Temporal lobe biopsy contained a collection of mature histiocytes, with PAS-positive rod-shaped inclusions. These inclusions, when studied by electronmicroscopy, were seen to be membrane-bound bacilliform bodies. Peroral jejunal biopsy contained no such inclusions. Despite treatment with antibiotics, the patients neurologic illness progressed, and he succumbed to intercurrent sepsis. We believe this to be the first instance in which a lesion of Whipple disease has been identified within the CNS by CT scan, and the diagnosis made antemortem, in the absence of demonstrable systemic disease.

Medulloblastoma in adults. Improved survival with supervoltage radiation therapy

During the period from January 1962‐June 1979, 17 adults (⩾16 years of age) received postoperative supervoltage neuraxis radiation therapy for medulloblastoma. An actuarial five‐ and ten‐year survival rate of 46% was achieved, and the major site of recurrence postirradiation was in the posterior fossa. Compared to previous pediatric series, adults may demonstrate more visceral metastases and fare less well after tumor recurrence. Compared to prior series of adult patients, the demonstrated improved survival is attributed to increased doses delivered to the posterior fossa.

Ependymoma of the ovary: report of three cases

Three apparently pure ependymomas of the ovary occurred in young women 25 to 35 years of age who presented with abdominal swelling or pain. At operation one tumor was stage IC, and two were stage III. On microscopic examination the tumors contained cells with fibrillary cytoplasmic processes, often arranged around blood vessels to form perivascular pseudorosettes. Immunohistochemical staining for glial fibrillary acidic protein confirmed the nature of the tumor in each case. The patient with the stage IC tumor was alive and well five years postoperatively. One patient with stage III disease died of tumor six years postoperatively, and insufficient time elapsed after treatment of the second to allow meaningful follow-up data. These cases illustrate the rare occurrence of ovarian tumors resembling differentiated tumors of the central nervous system. Recognition and distinction of these tumors from common epithelial tumors, which they may resemble, have important prognostic and therapeutic implications.

Glioma of the uterus. Report of a case with comments on histogenesis.

A 15-year-old girl had a hysterectomy for intractable vaginal bleeding. Examination of the specimen revealed a polypoid tumor that filled the endometrial cavity; microscopical examination disclosed a low-grade fibrillary astrocytoma. Glial tissue in the uterus has been generally considered to arise from implantation of fetal tissue. A reliably negative coital history makes such an explanation unlikely in the present case in which the tissue appeared to be truly neoplastic and of either germ cell or mesodermal origin. The patient is free of disease 6 years later.

Computed tomography in primary malignant lymphoma of the brain.

Primary malignant lymphoma of the brain is a rare lesion characterized clinically by varied nonspecific neurologic deficits and usually a rapid fulminant deterioration and death. This course may be altered by radiotherapy, and early diagnosis assumes major importance. Laboratory tests. EEG. radionuclide scans, angiography, and pneumoencephalography may not show definite abnormality in the early stages. This report concerns four cases in which good correlation between the location of the brain lesion and the abnormality on computed tomography scan was demonstrated.