Population-based comparison of prognostic factors in invasive micropapillary and invasive ductal carcinoma of the breast.

BACKGROUND: Invasive micropapillary carcinoma (IMPC) is a variant of breast carcinoma with a higher propensity for lymph node metastases compared with invasive ductal carcinoma (IDC). METHODS: Retrospective analysis of 636 IMPC and 297 735 IDC cases in the Surveillance, Epidemiology and End RESULTS database comparing disease-specific survival (DSS) and overall survival (OS) between IMPC and IDC. RESULTS: A higher percentage of IMPC cases (52.0%) had nodal metastases compared with IDC cases (34.6%). The 5-year DSS and OS for IMPC was 91.8% and 82.9%, respectively compared with 88.6% and 80.5% for IDC, respectively. For both IMPC and IDC, oestrogen-receptor positivity was associated with better survival, while having four or more positive lymph nodes or larger tumour size correlated with worse survival. Radiotherapy provided a survival benefit for both histological types. CONCLUSIONS: Despite IMPC's higher propensity for lymph node metastasis, IMPC has DSS and OS that compare favourably with IDC.

Computer visualization techniques (CVTs) foster evidence-based target delineation.

Computer visualization techniques (CVTs) are an emerging technology that can organize all cancer specialists. This article describes CVTs' ability to maximize the currently untapped advantages of intensity modulated radiotherapy (IMRT). The visual speed and dynamic strategies inherent in CVTs improves IMRT by distilling vast amounts of anatomic, multimodal imaging, textual/meaning, and surgical/outcome data into a large, rigorous, standardized evidence base of storable target delineation plans. This ability to standardize strategies will allow the collection of meaningful evidence based outcome data.

Amiodarone and radiation therapy sequelae.

With the aging of our population, amiodarone, a cardiac antiarrhythmic, is being used with increasing frequency. Anecdotal reports have appeared describing unexpected mucosal as well as cutaneous toxicity in patients treated concurrently with external beam radiotherapy and oral amiodarone. We retrospectively reviewed 12 years of experience at the University of Iowa Hospitals and Clinics and found 10 cases wherein amiodarone was taken concurrently with external beam radiation therapy. In our series, there were no missed treatment fractions because of unexpected acute sequelae. We conclude that amiodarone use does not preclude the delivery of therapeutic radiotherapy.

Relapsed Wilms tumor: is there a role for radiation therapy?

From 1968 to 1996, 21 of 97 (22%) patients with Wilms tumor treated at the University of Iowa Hospitals and Clinics relapsed after initial therapy. The most common sites of initial recurrence were the lungs in 12 (57%), abdomen in 3 (14%), bone in 3 (14%), and liver in 2 (10%). Treatment for recurrence included chemotherapy in 16, radiotherapy in 15 and surgery in 8. Radiotherapy was given to bilateral lungs of 8 previously untreated chests; total dose was 1,200 cGy to 1,500 cGy, with 2 patients receiving 1,000-cGy boost. One patient had abdominal radiotherapy after surgery for relapse; six patients had palliative radiotherapy to distant sites. The 1-, 3-, and 5-year postrelapse survivals were 62 +/- 11%, 38 +/- 11%, and 33 +/- 10%, respectively. Using Cox proportional-hazard regression models, abdominal recurrence (p = 0.0002; hazard ratio, 39.5) and initial stage IV disease (p = 0.019; hazard ratio, 6.27) were found to have a negative impact on postrelapse survival. Seven patients with previously nonirradiated chests are alive at a median follow-up of 123 months after relapse (range, 55--272 months); all had whole lung irradiation with or without chemotherapy at relapse for lung metastases. Radiotherapy was also found to be an effective treatment in the palliation of painful metastases.

The local field in infratentorial ependymoma: does the entire posterior fossa need to be treated?

INTRODUCTION: In the past decade, there have been multiple reports indicating that the predominant problem in the curative treatment of intracranial ependymoma is local failure. As a result, many have recommended local field radiotherapy. For infratentorial ependymoma, there is controversy regarding what constitutes the local field. Some radiation oncologists advocate coverage of the entire posterior fossa, whereas others recommend radiotherapy to the tumor bed and a safety margin. METHODS AND MATERIALS: From 1984 to 1998, 28 patients with posterior fossa ependymoma were diagnosed at our institution. There were 18 males and 10 females with a median age of 12 years (range, 2-81 years). Four patients (14%) had high-grade ependymoma and 3 (11%) had M+ disease at initial diagnosis. Gross total resection was achieved in 17 (61%) and postoperative radiotherapy (RT) was given to 22 (77%). Radiotherapy fields were craniospinal in 10, whole brain in 1, posterior fossa in 2, and tumor bed with a 2-cm. margin in 9. Median dose to the primary site was 54 Gy (range, 45-55 Gy). All 4 patients with high-grade ependymoma received craniospinal RT. Six patients did not receive RT after surgery. Magnetic resonance imaging (MRI) or computed tomography (CT) scans of the brain at initial diagnosis were compared to MRI or CT scans of patients at relapse to determine if the local relapse was in the tumor bed or nontumor bed posterior fossa. Median follow-up was 127 months (range, 14-188 months). RESULTS: Six patients have relapsed. For the 11 patients who had craniospinal or whole brain radiotherapy (RT), 3 recurred (tumor bed 1, spine 1, nontumor bed posterior fossa + spine 1). Both patients who failed in the spine had high-grade tumors. Neither of the 2 treated with posterior fossa fields relapsed. For the 9 patients who had tumor bed RT alone and the 6 who did not receive RT, there were 3 relapses; all were in the tumor bed. There were no relapses in the nontumor bed posterior fossa. CONCLUSION: For nondisseminated, low-grade infratentorial ependymoma, the radiotherapy volume does not need to include the entire posterior fossa. This information can be used to minimize late effects of RT in the era of three-dimensional (conformal) radiotherapy. No conclusion can be reached regarding the appropriate local field for high-grade infratentorial ependymoma because of the small number of patients.

Esthesioneuroblastoma: the University of Iowa experience 1978-1998.

OBJECTIVE: Esthesioneuroblastoma is rare and the best treatment has yet to be defined. The purpose of this study is to analyze the natural history, treatment, and patterns of failure of esthesioneuroblastoma treated at one institution. METHODS: Between 1978 and 1998, 13 patients with esthesioneuroblastoma were identified using the University of Iowa Tumor Registry. All patients were staged according to Kadish criteria. Mean follow-up was 6.3 years. Six patients had 5 or more years of follow-up and four had follow-up exceeding 9.5 years. One patient was lost to follow-up at 36 months. RESULTS: No patients had Kadish stage A disease, five were stage B, and eight stage C. Overall actuarial 5- and 10-year survival rate was 61% and 24%, respectively. Disease-free survival rate at 5 and 10 years was 56% and 42%, respectively. Seven patients have died, three of intercurrent disease and three of disease progression, one with an unknown disease status. Six patients remain alive, three without evidence of disease and three have experienced a local or regional recurrence. Five patients who were initially controlled developed recurrence, three local only, one locoregional, and one regional and distant. Median time to failure was 96 months. All patients with follow-up exceeding 12 years have experienced either a local or regional recurrence. Survival after salvage therapy in these patients ranged from 3 to 12 years. CONCLUSION: Esthesioneuroblastoma has a long natural history characterized by frequent local or regional recurrence after conventional treatment. Successful retreatment can lead to prolonged survival.

Pixel analysis of MR perfusion imaging in predicting radiation therapy outcome in cervical cancer.

The purpose of this study was to assess heterogeneity of tumor microcirculation determined by dynamic contrast-enhanced magnetic resonance (MR) imaging and its prognostic value for tumor radiosensitivity and long-term tumor control using pixel-by-pixel analysis of the dynamic contrast enhancement. Sixteen patients with advanced cervical cancer were examined with dynamic contrast-enhanced MR imaging at the time of radiation therapy. Pixel-by-pixel statistical analysis of the ratio of post- to precontrast relative signal intensity (RSI) values in the tumor region was performed to generate pixel RSI distributions of dynamic enhancement patterns. Histogram parameters were correlated with subsequent tumor control based on long-term cancer follow-up (median follow-up 4.6 years; range 3.8-5.2 years). The RSI distribution histograms showed a wide spectrum of heterogeneity in the dynamic enhancement pattern within the tumor. The quantity of low-enhancement regions (10th percentile RSI < 2.5) significantly predicted subsequent tumor recurrence (88% vs. 0%, P = 0.0004). Discriminant analysis based on both 10th percentile RSI and pixel number (reflective of tumor size) further improved the prediction rate (100% correct prediction of subsequent tumor control vs. recurrence). These preliminary results suggest that quantification of the extent of poor vascularity regions within the tumor may be useful in predicting long-term tumor control and treatment outcome in cervical cancer. J. Magn. Reson. Imaging 2000;12:1027-1033.

Long-term effects in children treated with radiotherapy for head and neck rhabdomyosarcoma.

PURPOSE: To examine the long-term effects of treatment in children receiving radiotherapy for head and neck rhabdomyosarcoma. METHODS: From 1967 to 1994, a total of 30 children with head and neck rhabdomyosarcoma received megavoltage radiotherapy at one institution. Seventeen patients (57%) have survived and have at least a 5-year follow-up. There were 11 males and 6 females, with a median age of 5.7 years (range 2.2-11.6) at the time of radiotherapy. Tumor location was orbit in 6 patients, infratemporal fossa in 4, paranasal sinuses in 2, and supraglottic larynx in 2; the nasopharynx, pterygopalatine fossa, and parotid gland were sites for the remaining children. All but 2 patients had tumors of embryonal histology. The Intergroup Rhabdomyosarcoma Study (IRS) Group was I in 2, II in 3, and III in 11 children; 1 patient had a recurrent tumor after surgery alone. Radiotherapy volume was the primary tumor or tumor bed in 13, tumor and whole brain in 3, and tumor and craniospinal axis in 1. Median radiotherapy dose to the primary site was 5,040 cGy (range 4,140-6,500) and to the whole brain was 3,000 cGy. All but 1 were treated with 150-200-cGy fractions; 1 patient received 250-cGy fractions for a tumor in the larynx. Chemotherapy was vincristine (V), actinomycin-D (A), and cyclophosphamide (C) in 10 patients, VAC + adriamycin in 2, VA in 1, VA + ifosfamide in 1, VC + adriamycin in 1, and none in 2. One patient had salvage chemotherapy consisting of cisplatin and etoposide. Median follow-up time was 20 years (range 7.5-33). RESULTS: Late effects of treatment were seen in all patients and included facial growth retardation in 11, neuroendocrine dysfunction in 9, visual/orbital problems in 9, dental abnormalities in 7, hearing loss in 6, and hypothyroidism in 3. Intellectual and academic delays were documented in 3 patients who had received whole brain radiotherapy. While neuroendocrine, thyroid, dental, and cognitive sequelae were primarily attributed to radiotherapy, hearing loss was thought to be a direct result of tumor destruction and, in 1 case, cisplatin chemotherapy. Late effects at or beyond 10 years from radiotherapy were few, but severe, and included chondronecrosis, esophageal stenosis, second malignancy, and brain hemorrhage. CONCLUSION: Late effects of treatment in children receiving radiotherapy for head and neck rhabdomyosarcoma are frequent. Although radiotherapy is a significant contributor of neuroendocrine, dental, thyroid, and cognitive toxicity, it is not usually implicated with hearing loss. Late toxicity of treatment beyond 10 years is not as frequent as those occurring within 10 years of therapy.

Comparison of posterior fossa and tumor bed boost in medulloblastoma.

To quantify the difference between the area of brain irradiated using the posterior fossa boost (PFB) and tumor bed boost (TBB) in medulloblastoma, we studied 15 simulation radiographs of patients treated in our institution from 1990 and 1999. The PFB was compared with the TBB, which was defined as the tumor bed plus 2-cm margin as demonstrated by postoperative magnetic resonance imaging. The PFB field treated a mean area of 9.43 cm2 more brain than the TBB. In 3 patients (20%), the area of the brain in the TBB was larger than the PFB. In 11 patients (73.3%), the PFB field had more than 10% more brain than the TBB. The cochlea was in the PFB and TBB field in all patients. In more than two thirds of patients, the area of brain irradiated with the PFB was at least 10% greater than the TBB. Future studies are needed to determine whether the TBB can replace the PFB in patients with medulloblastoma.

The significance of radiotherapy treatment duration in intracranial ependymoma.

PURPOSE: To determine if radiation therapy treatment duration has an impact on the outcome of intracranial ependymoma. METHODS AND MATERIALS: From 1965 to 1997, 34 patients with intracranial ependymoma were seen and treated with postoperative radiotherapy (RT). There were 26 male and 8 female patients with a median age of 14 years (range, 18 months to 60 years). Tumor location was infratentorial in 23 (67.6%) and supratentorial in 11 (32.4%). Pathology was low-grade in 25 (73.5%) and high-grade in 9 (26.5%). Two (5.9%) patients had M+ disease at initial diagnosis. Gross total resection was achieved in 12 (35.3%), subtotal resection in 19 (55. 9%), and biopsy alone in 3 (8.8%). Median RT dose to the primary site was 5400 cGy (range, 4500 cGy to 6600 cGy). The entire neuraxis was treated in 14 (41.2%), whole brain in 9 (26.5%), and the primary site alone in 11 (32.4%). Median radiotherapy treatment duration was 43 days (range, 26 to 122 days). Patients treated with craniospinal RT were more likely to have treatment duration of > or =50 days. Median follow-up was 108 months (range, 24 to 252 months). RESULTS: The 5-, 10-, and 15-year overall survival rates were 71.5%, 63.3%, and 55.4%. Local control rates at 5, 10, and 15 years were 61.8%, 57.7%, and 57.7%. For patients with RT treatment duration <50 days, the 5-, 10-, and 15-year overall survivals were 85.5%, 78.9%, and 65.7% whereas for those with treatment duration > or = 50 days, the corresponding rates were 45.5%, 36.4%, and 36.4% (p = 0.01, log-rank test). Local control rate at 5, 10, and 15 years was 70.6% for patients with RT treatment duration <50 days and 45.5%, 36.4%, and 36.4% for patients with RT treatment duration > or =50 days (p = 0.05, log-rank test). Age, gender, tumor location, tumor grade, degree of surgical resection, RT volume, and RT dose (4500-5399 cGy vs. 5400-6600 cGy) were not found to have a statistically significant impact on overall survival, disease-free survival, or local control. The most common site of failure was local, found in 13 (38.2%) patients. Three patients also had spinal failure in addition to a primary site failure. There were no isolated nonprimary site brain or spine failures. CONCLUSIONS: Radiation therapy treatment duration is an important prognostic factor for survival and local control with patients having treatment duration <50 days having a better outcome than those with treatment duration > or =50 days. Because the overwhelming site of failure was local, we recommend local field irradiation in patients with M0 disease.

Unresectable adenocarcinoma of the pancreas: patterns of failure and treatment results.

We retrospectively reviewed the outcome of 36 consecutive patients seen in our department with nonmetastatic unresectable adenocarcinoma of the pancreas. There were 28 men and 8 women with a mean age of 63.7 years (range, 43-78 years). Radiation therapy (RT) was given to all patients. 5-Fluorouracil (5-FU) was given as a bolus (500-1000 mg/m2/day) during the first and last weeks of RT in 22 patients, whereas continuous 5-FU (225 mg/m2/day) was given to 3 patients. The median survival for all patients was 10 months. Overall survival for all patients at 1 and 3 years was 47.2% and 19.1%, respectively. The pancreas, peritoneum, liver, and lungs were components of failure in 66.7%, 33.3%, 14.8%, and 14.8%, respectively. Local control was a major problem in patients treated for unresectable pancreatic cancer. The results of this study confirm the dismal prognosis of these patients.

Late effects in children treated with radiation therapy for Wilms' tumor.

PURPOSE: To determine the frequency and types of late effects in children receiving radiation therapy (RT) for Wilms' tumor. MATERIALS AND METHODS: From 1968 to 1994, 55 children received megavoltage RT at our institution as part of treatment for Wilms' tumor. A total of 42 (76.4%) have survived and have a minimum follow-up of 5 years. There were 25 female and 17 male patients with a median age at diagnosis of 48 months (range, 7-126 months). There were 12 Stage I, eight Stage II, 15 Stage III, six Stage IV, and one Stage V patient. RT was delivered to the hemiabdomen in 36 and whole abdomen in six patients. RT dose was 1000-1200 cGy (Group A) in 12, 1201-2399 cGy (Group B) in 11, and 2400-4000 cGy (Group C) in 19. Whole-lung RT was delivered to 13 patients either at diagnosis or pulmonary relapse. All patients received chemotherapy; the most common agents were actinomycin-D/vincristine/adriamycin in 13 and actinomycin-D/vincristine in 18. Median follow-up was 181 months (range, 60-306 months). RESULTS: Of 42 patients, 13 (31.0%) did not have late effects of treatment. The number of patients who developed muscular hypoplasia, limb length inequality, kyphosis, and iliac wing hypoplasia were seven (16.7%), five (11.9%), three (7.1%), and three (7.1%), respectively. Scoliosis was seen in 18 (42.9%) with only one patient requiring orthopedic intervention. Median time to development of scoliosis was 102 months, with a range of 16-146 months. The actuarial incidence of scoliosis at 5, 10, and 15 years after RT was 4.8 +/- 3.3%, 51.8 +/- 9.0%, and 56.7 +/- 9.3%, respectively. Only one of 12 Group A patients developed scoliosis. The 10- and 15-year actuarial incidences of scoliosis for Group A and B patients were 37.7 +/- 12.4% and 37.7 +/- 12.4%, whereas for Group C patients the incidences were 65.8 +/- 12.0% and 74.4 +/- 11. 7% (p = 0.03, log rank test). The actuarial incidence of bowel obstruction at 5, 10, and 15 years was 9.5 +/- 4.5%, 13.0 +/- 5.6%, and 17.0 +/- 6.5%. Of 23 patients, five irradiated within 10 days of surgery and one of 19 irradiated after 10 days developed bowel obstruction (p = 0.09, log rank test). Three patients developed hypertension with normal blood urea nitrogen (BUN) and creatinine levels; another patient had chronic renal insufficiency in a nonirradiated kidney. One patient developed diffuse interstitial pneumonitis. Of the 19 female patients who have reached puberty, three have given birth, and 15 have regular and one has irregular menstrual periods. Four patients developed benign neoplasms; three were in the RT field (two osteochondroma, one lipoma) and one outside (cervical intraepithelial neoplasia II). There were three second malignancies (chronic myelogenous leukemia at 9 years, osteosarcoma at 11 years, and breast cancer at 25 years after initial diagnosis of nephroblastoma); both solid malignancies occurred in the RT field. CONCLUSIONS: Late effects of therapy were seen in more than two thirds of children treated for Wilms' tumor. Children treated with lower doses (<2400 cGy) had a lower incidence of scoliosis compared with those who received more than 2400 cGy. There is also a suggestion that the incidence is lower in patients who received 1000-1200 cGy. Severe physical and functional deformity from RT was uncommon.

Posterior fossa boost in medulloblastoma: an analysis of dose to surrounding structures using 3-dimensional (conformal) radiotherapy.

PURPOSE: The purpose of this study was to compare three different techniques of delivering the posterior fossa boost in patients with medulloblastoma. METHODS AND MATERIALS: Five patients underwent CT simulation for treatment planning of the posterior fossa boost. For each slice, the posterior fossa was contoured in addition to the cochlea, non-posterior fossa brain, pituitary gland, mandible, parotid glands, thyroid gland, pharynx, and cervical spinal cord. For each patient, plans for three techniques of delivering the posterior fossa boost were compared. Technique A utilized parallel-opposed lateral fields using bony landmarks (2-dimensional radiotherapy); the other two techniques were planned using 3-dimensional radiotherapy. Technique B utilized a pair of coplanar wedged posterior oblique beams, whereas Technique C utilized a pair of posterior oblique fields and a vertex field. Dose-volume histograms (DVH) were obtained for each of the organs contoured and for each technique and patient. The maximum, minimum, and mean dose to each organ were determined using the DVH program in our treatment planning system. RESULTS: In 3 of 5 patients, the planning target volume (PTV) was not included in the treatment field with Technique A. The cochlea received 100%, 50%, and 42% of the prescribed posterior fossa dose using Techniques A, B, and C respectively. The mean dose to the non-posterior fossa brain was highest with Technique C, intermediate with Technique A, and lowest for Technique B. The mean dose to the non-posterior fossa brain with Technique B was comparable to the mean non-posterior fossa brain dose delivered using parallel-opposed lateral fields based on the CT definition of the PTV. Likewise, mean dose to the pituitary gland was lowest for Technique B. Both Techniques B and C were associated with a higher mean dose to the thyroid gland, mandible, parotid glands, and pharynx. CONCLUSIONS: The use of Technique B minimized the radiotherapy dose to the cochlea, pituitary gland, and non-posterior fossa brain. Contrary to what one may expect, conformal radiotherapy using Technique B did not deliver a higher dose to the non-posterior fossa brain over standard parallel-opposed lateral fields. Other advantages of conformal techniques B and C over 2-dimensional radiotherapy are the inclusion of the PTV in all patients and a lower mean dose to the pituitary gland. The main disadvantage of conformal Techniques B and C employed in our patients is a higher mean dose to the thyroid gland and other tissues in the neck.

Malignant neoplasms after treatment for metachronous bilateral Wilms' tumor: a literature review.

Information regarding malignant neoplasms after treatment for metachronous bilateral Wilms' tumor is limited. A MEDLINE search was performed of all English-language articles from 1950 to 1997 pertaining to metachronous bilateral Wilms' tumor. A total of 108 different cases were identified and analyzed. Mean follow-up was 5.8 years after initial diagnosis of Wilms' tumor (range, 1 month to 25.6 years). Eleven of 63 evaluable children (17.5%) had a congenital anomaly. Four patients (3.7%) developed a malignant neoplasm after treatment of a metachronous bilateral Wilms' tumor. Three of 18 patients followed for at least 10 years developed a solid tumor, including two sarcomas in the irradiated areas. Two of the 4 children who developed a malignant neoplasm had a congenital anomaly. Malignant neoplasms after treatment for metachronous bilateral Wilms' tumor can occur. Health-care professionals caring for these patients should be aware of this late sequelae of treatment.

Resected pancreatic cancer treated with adjuvant radiotherapy with or without 5-fluorouracil: treatment results and patterns of failure.

There are relatively little data regarding patterns of recurrence after curative resection and postoperative radiotherapy with or without 5-fluorouracil (5-FU) for patients with adenocarcinonima of the pancreas. Between 1978 and 1997, 41 patients underwent postoperative radiotherapy (RT) at Loyola-Hines Department of Radiotherapy. Of the 38 evaluable patients, 30 had RT + 5-FU and 8 had RT alone. Twenty-nine patients (76.3%) had a Whipple's resection, seven (18.4%) had distal pancreatectomy, and two (5.2%) had total pancreatectomy. Thirty-three (86.8%) of the 38 patients received > or =4,500 cGy to the tumor bed. Median survival for all patients was 21 months. The median survivals for patients who received RT + 5-FU and RT alone were 26 months and 5.5 months (p = 0.004). The most common site of failure was the liver, as seen in 79.2% of all recurrences. The peritoneum, other distant sites (lungs, bone, distant lymph nodes), and locoregional tumor bed were components of failure in 33.3%, 29.2%, and 25.0%, respectively. Locoregional failure alone was found in only one patient. Our median survival with postoperative RT + 5-FU is consistent with results reported by the Gastrointestinal Tumor Study Group and Mayo Clinic. Although patients who had RT + 5-FU had a better median survival than those who received RT alone, our RT-alone group had an inferior survival outcome compared to other published reports and may represent patient selection bias. Efforts in controlling this disease should be directed to prevention of intraabdominal relapse.

Medulloblastoma and supratentorial primitive neuroectodermal tumors: an institutional experience.

BACKGROUND: To the authors' knowledge there are relatively few data concerning supratentorial primitive neuroectodermal tumors (PNET). The authors retrospectively reviewed all cases of PNET of the brain treated at the study institution to determine whether there was a difference in presentation, overall survival, and recurrence-free survival with regard to tumor location (supratentorium vs. posterior fossa). METHODS: Between 1977-1996 33 patients with PNET were diagnosed and treated at 1 radiotherapy center. The median age of the patients was 9 years. The location of the tumor was in the posterior fossa in 25 patients and the supratentorium in 8 patients. The tumor had spread to the neuraxis in six patients; four patients with disseminated neuraxis disease had a supratentorial PNET and two had a posterior fossa PNET. All but three patients received craniospinal irradiation. The primary tumor received > or = 5000 centigray in 27 patients and chemotherapy was employed in 26 patients. The median follow-up was 60 months. RESULTS: The 5-year overall and recurrence-free survival rates for all patients were 77.2% and 79.6%, respectively. The 5-year overall survival rates were 86.3% for patients with medulloblastoma (posterior fossa PNET) and 46.9% for patients with supratentorial PNET (P = 0.01, log rank test). For overall survival, prognostic factors included radiotherapy dose to the primary site, metastases (M) status, and location of the primary tumor. The 5-year recurrence free survival rates were 89.8% for patients with medulloblastoma and 46.9% for patients with supratentorial PNET (P = 0.003, log rank test). For recurrence free survival, prognostic factors included M status and primary tumor site location; radiation dose to the primary tumor site and patient gender were of borderline significance. In the ten patients with inadequate posterior fossa boost fields judged by Children's Cancer Group criteria, there were two failures, both of which were in the original tumor bed. CONCLUSIONS: Supratentorial PNET has a worse overall survival and recurrence free survival than medulloblastoma. There is a suggestion that radiotherapy boosts in medulloblastoma may not need to encompass the entire posterior fossa because posterior fossa failures primarily are in the tumor bed. Larger studies with longer follow-up are needed to determine whether craniospinal irradiation followed by a boost to the tumor bed is adequate for medulloblastoma patients.

Trilateral retinoblastoma: is the location of the intracranial tumor important?

BACKGROUND: Trilateral retinoblastoma refers to bilateral retinoblastoma associated with an intracranial primitive neuroectodermal tumor in the pineal or suprasellar region. The purpose of this study was to review patient and tumor characteristics and treatment outcome in patients with trilateral retinoblastoma and to determine whether there is a difference in presentation or outcome according to the location of the intracranial tumor. METHODS: A MEDLINE search of all English language articles pertaining to trilateral retinoblastoma published between 1977-1997 was performed. A total of 94 different cases were identified and analyzed. RESULTS: The gender was male in 39 patients (41.5%), female in 50 patients (53.2%), and unknown in 5 patients (5.3%). Family history for retinoblastoma was positive in 44 patients (46.8%), negative in 39 patients (41.5%), and unknown in 11 patients (11.7%). The median age at the time of diagnosis of retinoblastoma was 6 months. The median time interval to the development of an intracranial tumor from the time of diagnosis of retinoblastoma was 21 months. In 78 patients (83.0%) the intracranial tumor was in the pineal region and in 16 patients (17.0%) it was in the suprasellar region. The median time interval from the time of diagnosis of retinoblastoma to the development of a pineal region tumor was 24 months whereas the median time interval for the development of a suprasellar region tumor was 1 month. At 6 months after the diagnosis of intraocular tumors, 6 of 61 children with pineal region tumors and 10 of 14 patients with suprasellar region tumors had developed intracranial disease (P = 0.005). Unilateral intraocular retinoblastoma associated with intracranial tumor was more likely to occur in patients with suprasellar region tumors than pineal region tumors (P < 0.015). The median survival after the diagnosis of an intracranial tumor was 6 months regardless of the location of the intracranial tumor. For patients who received no treatment for the intracranial tumor the median survival was 1 month whereas it was 8 months for those who received treatment. Children who were asymptomatic at the time of diagnosis of the intracranial tumor had a better overall survival than those who were symptomatic (P = 0.002). CONCLUSIONS: The prognosis of children who develop trilateral retinoblastoma is dismal with current treatment strategies. Tumors of the suprasellar region present earlier than tumors of the pineal region after the diagnosis of intraocular tumors. Because patients who were asymptomatic at the time of diagnosis of intracranial disease had a better overall survival than those who were symptomatic, screening for intracranial tumors may be a valuable strategy in the management of patients with bilateral and/or hereditary retinoblastoma. [See editorial on pages 3-5, this issue.]

Diffuse malignant leptomeningeal gliomatosis in a child: a case report and review of the literature.

Diffuse leptomeningeal gliomatosis is a rare condition characterized by glioma in the leptomeninges without a dominant mass lesion. The difficulty in diagnosis of this condition, its rarity. and its extensive nature have hampered its successful treatment. Most cases of primary diffuse leptomeningeal gliomatosis have occurred in adults. Reported here is a case of this condition in a 9-year-old girl; to the authors' knowledge, she is the youngest patient with diffuse leptomeningeal gliomatosis and the longest survivor of the malignant variety.