Subtotal Tumor Removal Research Articles

Anterior transpetrosal-transtentorial approach for sphenopetroclival meningiomas

This report presents a new surgical method and the results in 10 patients with petroclival meningiomas extending into the parasellar region (sphenopetroclival meningiomas). Minimal but effective extradural resection of the anterior petrous bone via a middle fossa craniotomy offered a direct view of the clival area with preservation of the temporal bridging veins and cochlear organs. The dural incision was extended anteriorly to Meckel's cave, and in cases with invasion of the cavernous sinus, Parkinson's triangle was enlarged by mobilization of the trigeminal nerve. This approach offered an excellent view from the mid-clivus to the cavernous sinus. Extra-as well as intradural tumor masses and dural attachments could be cleared under direct view of the pontine surface. The risk of injury to the lower cranial nerve and of retraction damage to the temporal lobe and brain stem were kept minimal by this approach. Total tumor resection was achieved in 7 patients, with no resultant mortality. Eight patients had a satisfactory postsurgical course, extraocular paresis being their main complaint. The extent of tumor resection depended on the degree of tumor adhesion to the carotid artery, and operative morbidity on the degree of tumor invasion of the brain stem. Of the 3 patients in whom subtotal tumor removal was achieved, only one experienced regrowth of the tumor and underwent a second operation during the follow-up period (6 months-6 years).

Anterior Transpetrosal-Transtentorial Approach for Sphenopetroclival Meningiomas: Surgical Method and Results in 10 Patients

This report presents a new surgical method and the results in 10 patients with petroclival meningiomas extending into the parasellar region (sphenopetroclival meningiomas). Minimal but effective extradural resection of the anterior petrous bone via a middle fossa craniotomy offered a direct view of the clival area with preservation of the temporal bridging veins and cochlear organs. The dural incision was extended anteriorly to Meckel's cave, and in cases with invasion of the cavernous sinus, Parkinson's triangle was enlarged by mobilization of the trigeminal nerve. This approach offered an excellent view from the mid-clivus to the cavernous sinus. Extra-as well as intradural tumor masses and dural attachments could be cleared under direct view of the pontine surface. The risk of injury to the lower cranial nerve and of retraction damage to the temporal lobe and brain stem were kept minimal by this approach. Total tumor resection was achieved in 7 patients, with no resultant mortality. Eight patients had a satisfactory postsurgical course, extraocular paresis being their main complaint. The extent of tumor resection depended on the degree of tumor adhesion to the carotid artery, and operative morbidity on the degree of tumor invasion of the brain stem. Of the 3 patients in whom subtotal tumor removal was achieved, only one experienced regrowth of the tumor and underwent a second operation during the follow-up period (6 months-6 years).

An ACTH and FSH Producing Invasive Pituitary Adenoma with Crooke's Hyalinization

We report on a patient with ACTH and FSH producing invasive pituitary adenoma complaining of cutaneous pigmentation. Elevations in plasma ACTH, beta-endorphin and cortisol levels as well as urinary 17-OHCS and cortisol excretion were found. Serum FSH concentration was just within the upper limit of the normal range, whereas serum LH level was reduced and alpha-subunit level was normal. Roentogenographic examination showed an almost complete loss of sellar floor and destruction of the posterior clinoids and dorsum sella. CT scan and MRI demonstrated an enlarged tumor invasion of the clivus and its extension to the sphenoid sinus. After subtotal removal of the large pituitary tumor, serum cortisol and plasma beta-endorphin levels as well as plasma ACTH concentrations returned to normal and serum FSH levels also remarkably decreased. Histologically, the tumor corresponded to a chromophobe, slightly PAS positive adenoma. These tumor cells exhibited positive immunostaining with antibody to ACTH (1-24), beta-LPH, beta-endorphin and FSH, while immunostaining of the adenoma cells was negative for LH, TSH, GH and prolactin. The immunogold technique also demonstrated ACTH and FSH particles in the secretory granules in the cytoplasm of the adenoma cells. Some of the tumor cells disclosed Crooke's hyalinization and type I microfilament occupied most of the cytoplasm. In the present study, a very rare case of ACTH and FSH producing invasive pituitary adenoma is reported.

Contemporary Management of Neurofibromatosis

The neurofibromatoses are two distinct entities with different genetic origins. The phenotypic expressions and required treatments are different. The devastating nature of neurofibromatosis-2 may be more effectively controlled through the application of advanced imaging techniques and contemporary neurotologic procedures. The most common manifestation of neurofibromatosis-2 is that of bilateral acoustic neuromas. The eventual total bilateral sensorineural deafness associated with this condition can be obviated in selected cases if the diagnosis is established early. Follow-up data are reported for three patients in whom hearing was preserved in at least one ear. When removal with hearing preservation is not possible, subtotal tumor removal with decompression of the internal auditory canals may delay progression of hearing loss. A new approach to tumors of the pterygomaxillary fossa that have extended to the middle cranial fossa has been successfully applied and is described.

Surgical Results of Brain Metastasis from Lung Cancer

Twenty-five patients receiving surgical treatment for brain metastasis from lung cancer were retrospectively studied to evaluate the prognostic factors for survival time. Twenty-two patients had died of respiratory distress by April, 1989. Favorable prognostic factors derived from the median survival time (MST) in these patients included; 1) resection of primary tumor (MST 10 months); 2) total or subtotal removal of metastatic tumor (MST 6.5 months); 3) adenocarcinoma (MST 13 months); 4) metachronous onset of brain metastasis (MST 12 months); 5) single metastasis (MST 8 months). These results suggest that therapy for the primary lung cancer is important before surgery for metastatic brain tumor.

The treatment and long-term prognosis of children with intracranial tumors: A study of 610 cases, 1950–1981

Six hundred and ten children aged under 16 years with intracranial tumors were referred for radiotherapy between 1950 and 1981: 579 were new cases and 31 had recurrent disease after primary treatment elsewhere. Radiotherapy was completed in 93% of all cases. The actuarial survival rate for all new cases was 53% at 5 years, 46% at 10 years, 40% at 20 years, and 39% at 30 years. The oldest children (10–15 years) had the best survival and the youngest (0–2 years) had the worst survival. Children treated with megavoltage x-ray equipment (1970 to 1981) had a significantly greater survival than those treated with orthovoltage X rays (1950–1969). Overall, a direct correlation was found between survival and maximum radiotherapy dose. Children having a total excision of the tumor prior to radiotherapy showed a greater survival than those treated by a subtotal or partial tumor removal. Children treated by radiotherapy alone had a survival comparable to those treated by sub-total excision and radiotherapy. There is a striking difference in survival expectation depending on initial functional category (I to III). The overall survival rates of 428 children completing treatment for glioma were 49% at 5 years, 43% at 10 years, and 40% at 15 years. The results according to certain specific tumor sites within the cerebral hemispheres are reported. Age is an important prognostic factor in low grade and also high grade astrocytomas, children having longer survivals than adults. Sub-total or partial excision of Canopharyngiomas combined with radical radiotherapy appears to give the best long-term results. Of 73 new cases, the 5-, 10-, and 15-year survival rates were 92%, 84%, and 79%. Recurrent craniopharyngiomas treated by surgery alone can be salvaged by further conservative surgery and radical radiotherapy. Optic gliomas are slow growing low grade astrocytomas. Survival rates at 5, 10, and 15 years for 20 children with mostly chiasmal lesions were 89%, 89%, and 78%, respectively. In 73 children with brain stem tumors, 17% remained alive for up to 15 years. The risk of CNS seeding from intracranial ependymomas depends on site of origin and grade of malignancy, with 50% incidence occurring in cases with high grade lesions situated in the posterior fossa. Survivals at 5, 10, and 15 years in 51 children were 51%, 40%, and 31%. Adjuvant chemotherapy improves survival. One hundred and forty-three children with medulloblastoma completing radiotherapy had survival rates of 42%, 33%, and 29% at 5, 10, and 15 years. Adjuvant chemotherapy has a profound effect on their survival. In a sample of 62 children treated and evaluated, 3–20 years after treatment, 15% had I.Q. scores of under 70 whereas in 60% the scores were average or above average. Seventy-three per cent were at normal school, at university or in full-time employment.

Operative Treatment of Intracranial Epidermoid Cysts and Cholesterol Granulomas: Report of 21 Cases

Abstract Thirteen patients had operations to remove intracranial epidermoid cysts, and long-term follow-up was obtained. Total or nearly total tumor and capsule removal was accomplished in 7 patients during the initial operation. This group required no additional operations. The other 6 underwent subtotal tumor removal and required multiple operations for symptomatic tumor recurrence. This latter group had a poorer neurological outcome. We conclude that initial total or near-total tumor resection is highly desirable in treating intracranial epidermoid cysts, particularly in physiologically young individuals. Five patients were followed after operations to remove pure cholesterol granulomas of the petroclival bone, and 3 additional patients were followed after operations to remove tumors with combined histopathological features of both an epidermoid cyst and cholesterol granuloma. Four patients with some component of a cholesterol granuloma had concurrent middle ear infections, and 4 did not. Intracranial subtotal excision and drainage of these lesions was the initial operative management in 7 patients. 5 of whom have required multiple operations for symptomatic tumor recurrence. Therefore, we conclude that subtotal excisional procedures for tumors with histopathological features of cholesterol granulomas are not usually successful in establishing long-term cures. Total excision, as recommended for epidermoid cysts, tumors frequently confused with cholesterol granulomas when occupying the petroclival region, may be warranted for these tumors as well. We postulate that when a congenital epidermoid cyst occurs in the petroclival bone, it may incite a local inflammatory reaction, producing lesions which have the histological features of both epidermoid cysts and cholesterol granulomas.

Operative treatment of intracranial epidermoid cysts and cholesterol granulomas

Thirteen patients had operations to remove intracranial epidermoid cysts, and long-term follow-up was obtained. Total or nearly total tumor and capsule removal was accomplished in 7 patients during the initial operation. This group required no additional operations. The other 6 underwent subtotal tumor removal and required multiple operations for symptomatic tumor recurrence. This latter group had a poorer neurological outcome. We conclude that initial total or near-total tumor resection is highly desirable in treating intracranial epidermoid cysts, particularly in physiologically young individuals. Five patients were followed after operations to remove pure cholesterol granulomas of the petroclival bone, and 3 additional patients were followed after operations to remove tumors with combined histopathological features of both an epidermoid cyst and cholesterol granuloma. Four patients with some component of a cholesterol granuloma had concurrent middle ear infections, and 4 did not. Intracranial subtotal excision and drainage of these lesions was the initial operative management in 7 patients, 5 of whom have required multiple operations for symptomatic tumor recurrence. Therefore, we conclude that subtotal excisional procedures for tumors with histopathological features of cholesterol granulomas are not usually successful in establishing long-term cures. Total excision, as recommended for epidermoid cysts, tumors frequently confused with cholesterol granulomas when occupying the petroclival region, may be warranted for these tumors as well. We postulate that when a congenital epidermoid cyst occurs in the petroclival bone, it may incite a local inflammatory reaction, producing lesions which have the histological features of both epidermoid cysts and cholesterol granulomas.

Infratentorial ependymomas in childhood: prognostic factors and treatment

The prognostic factors and survival data were analyzed for 35 children (aged under 16 years at diagnosis) with childhood infatentorial ependymomas treated surgically at The Hospital for Sick Children in Toronto during the years 1970 to 1987. Tumor histology was reviewed individually and grouped into three categories (Categories I to III) for survival analysis. An overall 5-year survival rate of 44.6% was obtained after the exclusion of perioperative mortality. Factors associated with an improved 5-year survival rate were: total tumor removal, noninvasive tumors, Category I histology, age greater than 6 years, and absent physical signs of parenchymal invasion or lower cranial nerve involvement. The 5-year survival rate was lower when associated with Category III histology, brain-stem or cranial nerve signs, age less than 2 years, tumor invasion and/or cranial nerve involvement, and subtotal tumor removal. Clinical evidence of spinal metastases was found to be uncommon (3.1%). Surgical excision followed by radiation therapy was the primary mode of treatment for these tumors. Different approaches regarding the volume of radiotherapy to be delivered and the use of adjuvant chemotherapy are discussed.

Radiation therapy in the management of low-grade supratentorial astrocytomas.

The records of 167 patients with grade 1 or 2 supratentorial pilocytic astrocytomas (41 patients), ordinary astrocytomas (91 patients), or mixed oligoastrocytomas (35 patients) diagnosed between 1960 and 1982 are retrospectively reviewed. The extent of surgical tumor removal was gross total or radical subtotal in 33 patients (20%) and subtotal or biopsy only in the remaining 134 patients (80%). Postoperative radiation therapy was given to 139 (83%) of the 167 patients, with a median dose of 5000 cGy (range 600 to 6500 cGy). Multivariate analysis revealed that a pilocytic histology was the most significant prognostic variable associated with a good survival. The 5- and 10-year survival rates were, respectively, 85% and 79% for the 41 patients with pilocytic astrocytomas compared to 51% and 23% for the 126 patients with ordinary astrocytomas or mixed oligoastrocytomas. Postoperative irradiation did not appear to be associated with improved survival times in the patients with pilocytic astrocytomas; however, in the 126 patients with ordinary astrocytomas or mixed oligoastrocytomas, those who received "high-dose" radiation (greater than or equal to 5300 cGy) had significantly better survival times than those who received "low-dose" radiation (less than 5300 cGy) or surgery alone. The 5- and 10-year survival rates were, respectively, 68% and 39% for the 35 patients receiving high-dose radiation, 47% and 21% for the 67 receiving low-dose radiation, and 32% and 11% for the 19 treated with surgery alone. The survival rate was poor for the 23 patients with ordinary astrocytomas and oligoastrocytomas who underwent gross total or radical subtotal tumor removal (14 of whom were also irradiated): 52% at 5 years and 21% at 10 years, with 19 of 23 patients developing tumor progression and dying 1 to 12 years postoperatively. In contrast, all 10 patients with pilocytic astrocytomas who had gross total or radical subtotal tumor removal alone were long-term survivors, with follow-up periods of about 4 to 25 years.

Preservation of Hearing in the Surgical Removal of Cerebellopontine Angle Tumors

It is claimed that postsurgical hearing preservation is possible in about 5 to 8 per cent of cases of acoustic neuroma. Even with small tumors, hearing preservation can be accomplished in about half of the patients who are fortunate to have anatomic integrity of facial and cochlear nerves, as well as intact inner ear blood supply at the end of the surgical procedure. Monitoring of seventh and eighth nerve function through evoked potentials may be important. On the other hand, even if evoked potentials are preserved during surgery with wave V latency and amplitude similar to preoperative recording, hearing may still be completely lost during the immediate postoperative period. There is no predictable pattern that assures postoperative preservation of hearing. In Case 2, the seventh and eighth nerves as well as the inner ear blood supply were carefully dissected and anatomically and electrophysiologically preserved during the intraoperative period. At the end of the surgical procedure, the evoked potentials were the same as preoperatively. Nevertheless, the patient ended up with no detectable hearing postoperatively. We feel that the suboccipital-retrosigmoid transcanal approach can be safely used for the removal of cerebellopontine angle tumors of all sizes. We believe the argument that "only the translabyrinthine approach can accomplish total tumor removal" is not valid. No complications have been attributed by having the patient in the semisitting position. Older patients, who cannot tolerate the semisitting position, are operated on while in the park bench position. Planned subtotal removal of a CPA tumor is done with the patient in the supine position (transmastoid-retrolabyrinthine or retrosigmoid). The relationship between the otologic surgeon and neurosurgeon is very important. The surgical approach used should be selected on the basis of the combined experience of the surgical team. Finally, an attempt should be made to preserve facial nerve function and hearing in all suitable patients. The postoperative course using the suboccipital-retrosigmoid approach is usually benign, and the patients are discharged from the hospital between 7 and 10 days following surgery. The translabyrinthine approach, in our opinion, should be reserved for smaller lesions in patients with anacusis or with residual hearing that is not worth saving.

Malignant Lymphoma of the Spinal Epidural Space

Three patients with non-Hodgkin's lymphoma involving the spinal epidural space are reported. All three patients, a 3-year-old boy (Case 1), a 63-year-old female (Case 2), and a 64-year-old male (Case 3), manifested signs and symptoms of neurological involvement before the diagnosis of malignant lymphoma was established by tissue examination. The tumors were located in the thoracic (Case 3) and thoracolumbar regions (Cases 1 and 2). The initial signs and symptoms of all cases were low back pain and progressing myelopathies. Bony abnormalities of the vertebrae, seen on both plain X-rays and computed tomographic scans, were unremarkable except for bilateral L1 pedicle erosion observed in Case 2. Total or subtotal tumor removal was followed by radiation therapy with or without chemotherapy in all cases. The primary lesion in Case 1 was a paravertebral lymphoma that had invaded the adjacent thoracolumbar epidural space through the intervertebral foramina. In Case 2, the primary lesion, documented by post-mortem examination, was a submucosal lymphoma of the stomach. In Case 3, the site of primary lesion could not be determined, although splenic and retroperitoneal nodal involvement was found at autopsy. The authors take a skeptical view of the existence of "primary" spinal epidural lymphoma, which has been reported a few times. Each of the three patients reported here presented with an epidural mass as the initial clinical manifestation of malignant lymphoma.

Adoptive immunotherapy for recurrent glioblastoma multiforme using lymphokine activated killer cells and recombinant interleukin-2.

Thirteen patients with recurrent glioblastoma were treated with adoptively transferred autologous lymphokine activated killer (LAK) cells and recombinant interleukin-2 (rIL-2). Patients' blood mononuclear cells (MNC) obtained by leukapheresis were cultured at 2.5 million MNC per ml for 3 to 5 days in media containing 1000 U rIL-2/ml. After incubation, the nonadherent MNC from all cultures (0.5-5 X 10(9] were combined and concentrated for infusion in 5 to 10 ml saline containing 10(6) U rIL-2. Nine patients received one injection of LAK cells and rIL-2 into the brain tissue immediately surrounding the tumor cavity during craniotomy for subtotal tumor removal (Group 1). On each of the 3 days after surgery, patients received boosters of 10(6) U rIL-2 delivered into the tumor cavity through a skin flap or via an Ommaya reservoir. Approximately 1 to 2 weeks after this series of injections, these patients were treated with a second cycle of LAK cells and rIL-2 injected into the tumor cavity using the reservoir. Four patients received both adoptive immunotherapy cycles by intracavitary injection (Group 2). In this relatively small patient pool, neither age, sex, Karnofsky score, treatment history, nor anticonvulsant and steroid dosage appeared to influence a patient's ability to make LAK cells. The therapy, itself, was well-tolerated by all patients although they all displayed symptoms of aseptic meningitis and increased intracranial pressure, i.e., headache, fever, malaise on the days of LAK cell and/or rIL-2 infusion. The therapy did not appear to have a significant impact on patient survival (mean, 30 weeks) especially for those patients with a high postsurgical tumor burden. As the therapy is safe, the authors believe its efficacy can best be tested in patients with a newly diagnosed or recurrent glioblastoma which lies in an area where a near-total resection is possible.

Clinical and Electromyographic Features of “Intermittent Tonic Facial Spasm” due to Acoustic Neurinoma

The authors report two cases of acoustic neurinoma involving intermittent tonic contractions of the facial muscles on the affected side. The clinical features, and in one case the electromyographic (EMG) findings, were compared to those in cases of hemifacial spasm (HFS) due to neurovascular compression (NVC). Both patients exhibited intermittent facial tonic muscle contractions and deep nasolabial folds on the affected side despite facial weakness. In one case, EMG showed continuous muscle activity not only during contractions but also at rest. On the other hand, in HFS due to NVC, EMG demonstrated intermittent bursts of activity during spasm but little or no activity at rest. In both of these cases the tumor was large enough to compress the brainstem. In neither case could the facial nerve be identified during subtotal tumor removal. These findings suggest that the nature of the facial muscle contractions in these two cases was quite different from that of HFS due to NVC. The former appeared to be an incomplete form of the so-called “continuous tonic facial spasm” attributable to glioma of the brainstem. Such contractions might better be termed “intermittent tonic facial spasm.”

Spinal Metastasis detected 11 Years after Initial Treatment for Pineal Germ Cell Tumor

A metastatic spinal tumor was found in a 28-year-old male 11 years after initial treatment for a pineal tumor. The original tumor had been treated by ventriculoatrial shunting, irradiation, and chemotherapy. The patient underwent laminectomy and subtotal removal of the spinal tumor, which was histologically diagnosed as germinoma. The final diagnosis of metastatic mixed germ cell tumor (mainly teratoma with a germinomatous component) of pineal origin was based on the findings of computed tomography and magnetic resonance imaging, the clinical course, tests for tumor markers, the response of the tumor to radiation therapy, and the histopathological findings. It was inferred that only the germinomatous component of the pineal mixed germ cell tumor had metastasized to the cauda equina.

Postoperative radiotherapy of intracranial ependymoma in pediatric and adult patients

In 33 patients undergoing operation and postoperative irradiation for intracranial ependymomas between January 1963 and December 1983, the tumor was grade 1 or 2 in 26 (79%) patients and grade 3 or 4 in 7 (21%). Operation consisted of only biopsy in 1 (3%), subtotal removal of tumor in 28 (85%), and gross total resection in 4 (12%). All patients received brain irradiation with a median dose of 4800 cGy. Seventeen (52%) patients also received spinal axis irradiation (median dose, 3000 cGy) which included 5 with high-grade tumors and 12 with low-grade infratentorial tumors. The relapse-free and overall survival rates at 5 years were 61% and 62%, respectively. Prognostic factors analyzed for statistically significant survival differences included age, sex, hydrocephalus, site, grade, extent of operation, extent of brain field, spinal axis irradiation, and brain dose. Grade was the only significant factor found: the 5-year survival of patients with low-grade ependymomas, 71%, was significantly better ( p < 0.04) than that of patients with high-grade ependymomas, 29%. Among the 31 patients evaluable for patterns of failure, treatment failed in 12 (39%) (10 only in the brain, 1 in the brain and spinal cord, and 1 only in the spine). All but one of the brain failures were at the site of the original primary lesion. Treatment failed in 4 of the 6 (67%) patients with high-grade tumor but in only 8 of the 25 (32%) with low grade tumor. Among the 7 low-grade infratentorial ependymomas treated with brain irradiation only, there was 1 treatment failure (in the spine; salvaged with further irradiation). Among the 12 patients with low-grade infratentorial tumors who received spinal axis irradiation, treatment failed in 1 (8%) (in the spine and also in the brain; patient subsequently died of disease). Nineteen (58%) patients remain alive; all but 2 of the patients who had recurrence died of their disease. This retrospective study suggests that: (a) patients with high-grade tumors have significantly poorer survival compared with those with low-grade tumors; (b) the main cause of death in ependymoma patients is intracranial failure at the primary site; and (c) craniospinal axis irradiation may not be necessary for patients with low-grade infratentorial ependymoma (localized irradiation alone may be adequate).

Cerebellar astrocytomas: therapeutic management.

The therapeutic management of cerebellar astrocytomas is almost exclusively surgical. Although a few patients survive for long periods without treatment, the majority die without surgery. Total excision is advised to prevent recurrence which almost always follows non-total removal of tumour. Moreover, radical excision is feasible since the cerebellum has a remarkable capacity to compensate after large amounts of tissue have been removed. Morbidity is related to damage to the deep cerebellar nuclei, infiltration of the brain stem, secondary adhesions, and infection. Tumours may not be macroscopically visible at the time of first operation which in turn emphasizes the need for a detailed radiological work-up using, in particular, the CT scan. Biopsy alone, decompression alone, and/or aspiration are usually followed by rapid recurrence and no more than 30% of patients thus treated are recurrence-free five years after surgery. Approximately 40% of patients have subtotal resections and, of these, only 35% are recurrence-free five years post-operatively. Despite the high risk of recurrence following subtotal removal, subtotal excision may still be followed by prolonged survival since two thirds of the patients in the present study were still alive ten years or more after surgery. This is due in part to the unpredictable behaviour of cerebellar astrocytomas, a fact clearly demonstrated by serial CT studies of patients with partially excised tumours which demonstrate that residual tumour may occasionally regress or even remain static for many years. Total removal, when possible, is the treatment of choice and was carried out in 41% of patients in the present study. Ninety-five per cent of patients were free of recurrence for 25 years or more following total removal. In fact, recurrence following total removal has only rarely been recorded and is more often found when the initially excised tumour contains atypical and/or malignant features. Still, a benign histology does not preclude recurrence even when a total macroscopic excision has been achieved. This again emphasises the unpredictable nature of these tumours and the need for long-term radiological follow-up. Overall, operative mortality should be around 5% and even less for unilateral, hemispheric, circumscribed, nodular cerebellar astrocytomas. Conversely, the operative mortality for tumours of the vermis may approach 30% and generally increase with each subsequent operation, being maximal in the first post-operative month. Radiotherapy does not reduce the rate of recurrence nor prolong the overall survival period to death in patients with subtotal removal of tumour.(ABSTRACT TRUNCATED AT 400 WORDS)

Transnasal surgery in the treatment of craniopharyngiomas.

The results of transnasal-transsphenoidal surgery in 31 patients with craniopharyngioma are evaluated. To assess the value of transnasal surgery in cases with visual impairment, the results are compared to cases operated transcranially. There was only one death occurred on the 5th postoperative day. Two patients developed rhinoliquorrhoea, and one a transient oculomotor palsy. Recurrences leading to re-operation occurred frequently after mere cyst aspiration and only once after subtotal tumour removal. Normal glandotrope functions of the hypophysis were preserved in 75%, disturbed functions were normalized in 19% of primary transnasal surgery. Visual disturbances were normalized or improved in 94% cases after transnasal surgery, whereas an improvement was observed in only 65% after transcranial surgery. A deterioration never appeared after transnasal surgery, but in 26% after transcranial operations.

High complete response rate in children with advanced germ cell tumors using cisplatin-containing combination chemotherapy.

A consecutive series of 13 children (five girls) with advanced malignant germ cell tumors (MGCTs) were treated with between four and seven (median, six) courses of cisplatin, bleomycin, and either vinblastine (BVP) or VP-16 (BEP). There were seven gonadal primaries (four testis, three ovary) and six at extragonadal sites (three sacrococcyx, two thoracic, one extradural). Total or subtotal removal of primary tumor was carried out in nine patients at diagnosis and two others after some chemotherapy. Clinical complete remission (CR) was achieved in nine of ten patients with measurable disease and serum markers returned to normal in all 13 patients. Eleven remain disease-free 17 to 48 months (median, 28 months) after diagnosis. One patient (stage IV sacrococcygeal tumor) relapsed at the primary site 3 months after completing treatment, but is disease-free after further surgery, radiotherapy, and chemotherapy. Serial glomerular filtration rates were performed during treatment. Audiometry and pulmonary function tests were carried out where possible. Toxicity led to alteration of drug scheduling in two cases, but there were no permanent clinical renal, auditory, or pulmonary sequelae. These encouraging results confirm that MGCTs in children are as responsive as those in adults to cisplatin-containing chemotherapy and indicate that they may be as curable. The regimens are relatively well-tolerated and, with close monitoring, clinically significant toxicity should be avoidable.

Long-term follow-up of children with craniopharyngioma.

Management of craniopharyngiomas is still controversial. 28 children with this tumor were studied. GH deficiency was present in 22 patients following surgery, 10 of these GH-lacking patients had normal or accelerated growth (usually associated with rapid weight gain) postoperatively. Somatomedin levels were normal in three of six normally growing patients. After craniotomy their basal and TRH-stimulated prolactin levels were in the normal range, but their insulin secretion was markedly increased. Postoperatively there was a significant correlation between peak insulin levels following arginine infusion and growth velocity in all patients. Complete tumor removal could be performed in 28% of our patients. Altogether 36% of all patients had at least one tumor recurrence. Recent literature with the addition of our series showed tumor recurrence in 22% of patients with "total" tumor excision and in 72% of patients with partial tumor removal. Radiotherapy seems to be capable of destroying craniopharyngioma tissue. The recurrence rate was only 26% in patients with subtotal excision plus radiotherapy. Unless radical tumor removal can be attempted with safety, subtotal tumor removal plus radiotherapy appears to be the treatment of choice for craniopharyngioma.