Rita M. Linggood

Definitive radiation therapy for chordoma and chondrosarcoma of base of skull and cervical spine.

: Proton-beam radiation therapy has been developed for the treatment of chordomas or sarcomas of bone or soft tissue that abut the central nervous system. The authors report the results of treatment of 10 patients, six with chordoma, three with chondrosarcoma, and one with a neurofibrosarcoma. Local control has been achieved for all patients (with, however, one marginal failure) with a follow-up period ranging from 2 months to 6 years. High doses of radiation, up to 76 Cobalt Gray Equivalents (CGE), have been delivered without significant morbidity. In particular, no neurological sequelae have been observed.

Ocular adnexal lymphoma: Clinical behavior of distinct World Health Organization classification subtypes

PURPOSE To evaluate the clinical behavior and treatment outcome of ocular adnexal lymphomas classified by the World Health Organization system, with emphasis on marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT). MATERIALS AND METHODS The clinicopathologic materials from 98 consecutive patients treated for ocular adnexal lymphoma were reviewed. Fourteen patients had prior lymphoma and 84 patients had primary disease (75% Stage I, 6% Stage III, and 19% Stage IV). Radiation (photons/electrons) was administered to 102 eyes to a median dose of 30.6 Gy. The mean follow-up was 82 months. RESULTS The most common subtypes among the primary patients were MALT (57%) and follicular (18%) lymphoma. The 5-year actuarial local control rate in 102 irradiated eyes was 98%. Among the low-grade lymphomas, the 5-year local control rate correlated with the radiation dose in the MALT lymphoma subgroup (n = 53): 81% for <30 Gy and 100% for > or =30 Gy (p <0.01). For the non-MALT low-grade lymphomas such as follicular lymphoma (n = 30), the local control rate was 100% regardless of dose. For 39 Stage I MALT lymphoma patients treated with radiation alone, the distant relapse-free survival rate was 75% at 5 years and 45% at 10 years. Distant relapses were generally isolated and successfully salvaged by local therapy. The overall survival for this subgroup was 81% at 10 years, with no deaths from lymphoma. CONCLUSIONS Dose-response data suggest that the optimal radiation dose for MALT lymphoma of the ocular adnexa is 30.6-32.4 Gy in 1.8-Gy fractions and follicular lymphoma is adequately controlled with doses in the mid-20 Gy range. The substantial risk of distant relapse in Stage I ocular adnexal MALT lymphoma underscores the importance of long-term follow-up for this disease and the need for additional comparative studies of MALT lymphoma of different anatomic sites.

The role of radiotherapy in the treatment of subtotally resected benign meningiomas

SummaryThirty-six patients with benign meningioma were treated for primary or recurrent disease by subtotal resection and external beam irradiation from 1968–1986 at Massachusetts General Hospital. Comparison is made with 79 patients treated by subtotal surgery alone from 1962–1980.Progression-free survival for 17 patients irradiated after initial incomplete surgeery was 88% at 8 years compared with 48% for similar patients treated by surgery alone (p = 0.057). 16 patients incompletely resected at time of first recurrence were irradiated and 78% were progression-free at 8 years while 11% of a similar group treated by surgery alone were progression free (p = 0.001). Long term overall survival was high and similar in both control and study groups. Two patients were irradiated at second recurrence and 1 patient at third recurrence.Twenty-five patients were treated with photons alone and have a median follow-up of 57 months, 6 patients have recurred at doses 45-60 Gy. Eleven patients were treated with combined 10 MV photons and 160 MV protons utilizing 3-D treatment planning. These patients have been followed for a median of 53 months and none have failed to date. Eight of 11 received 54-60.4 Gy and 3/11 > 64.48 Gy. Sex, age, pathology grade and score, surgery and timing of radiation therapy were not associated with significant differences in failure patterns within the irradiated study group (p < 0.1). Complications have been seen in 6 irradiated patients.

Primary Non-Hodgkin's lymphoma of the mediastinum

Non‐Hodgkins lymphoma localized to the mediastinum and adjacent structures occurred in 12 of 215 (6%) non‐Hodgkins lymphoma patients seen at the Massachusetts General Hospital between 1975 and 1979. Lymphangiography, radionuclide scanning and whole body computerized tomography were used to exclude patients with extrathoracic disease at presentation. Eleven of the 12 patients presented with extensive contiguous extranodal disease (Stage IIE) with involvement of either the pericardium, sternum, chest wall, pulmonary parenchyma or, in four cases, with superior venacaval obstruction. Diffuse large cell lymphoma (eight cases) and diffuse poorly differentiated lymphocytic lymphoma (four cases) were the prevalent histlogic subtypes; no instances of lymphoblastic lymphoma without extrathoracic spread were encountered. None of four lymphomas studied could be characterized as either B‐ or T‐cell tumors utilizing conventional surface marker techniques. Ten of the 12 patients achieved complete remissions, either after treatment with combination chemotherapy alone (three patients) or after both chemotherapy and mediastinal irradiation (seven patients). Two of these ten have subsequently relapsed, but median survival has not been reached after a mean period of observation of 28 months. Primary nonlymphoblastic non‐Hodgkins lymphoma of the mediastinum is more common than previously realized, displays aggressive contiguous spread within the chest and responds well to combination chemotherapy with or without adjuvant mediastinal irradiation.

Echo-Planar MR Cerebral Blood Volume Mapping of Gliomas: Clinical utility

Neovascularization is a common phenomenon in gliomas. MR imaging cerebral blood volume (CBV) mapping utilizes ultrafast echo-planar imaging and simultaneous use of gadolinium-based contrast material. To determine the utility of MR CBV mapping in the clinical evaluation of gliomas, we followed 15 patients with serial studies. This technique provided functional information that was not evident with conventional CT or MR imaging. Low-grade tumors demonstrated homogeneously low CBV, while high-grade tumors often showed areas of both high and low CBV The maximum tumor CBV/white matter ratio was compared between low- (n = 3) and high-grade gliomas (n=5) in patients without previous treatment and with histologic verification (n=8) and was significantly higher in high-grade gliomas (p<0.01). High CBV foci in nonenhancing tumor areas were present in 2 cases. The distinction between radiation necrosis and active tumor could be made correctly in 3 of 4 cases. The information provided by MR CBV mapping has the potential to be an adjunct in the clinical care of glioma patients.

Mediastinal large cell lymphoma. An uncommon subset of adult lymphoma curable with combined modality therapy.

Thirty adults with large cell lymphoma predominantly localized to the mediastinum diagnosed at the Massachusetts General Hospital between 1976 and 1985 were identified. The median age of the 20 females and 10 males was 34 years. All but one presented with symptoms due to an enlarging mediastinal mass, which was localized in 22 patients (73%) and exceeded 10 cm in maximal diameter in 65%. Superior vena cava syndrome and large pleural and pericardial effusions were common. Employing CHOP chemotherapy (cyclophosphamide, doxorubicin, vincristine, prednisone) and consolidation radiation therapy in most cases, 80% achieved a complete remission and 59% survive failure‐free at 5 years by actuarial calculation, the size of the mediastinal mass adversely affected failure‐free survival (89% vs. 40%, P < 0.05). No other pretreatment risk factor predicted outcome, but more intense chemotherapy was associated with improved survival (P = 0.035). Large cell mediastinal lymphoma is a locally invasive, often bulky malignancy with a predilection for young women; disease of low or moderate bulk is curable with full dose CHOP chemotherapy and consolidation radiation, but bulky disease requires more aggressive treatment.

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.

CENTRAL NERVOUS SYSTEM NEUROCYTOMA AND NEUROBLASTOMA IN ADULTS-REPORT OF EIGHT CASES

SummaryThe clinical features, pathologic findings and treatment courses of eight adults with central nervous system small-cell neuronal tumors were reviewed. Five patients had central neurocytomas, two patients central nervous system neuroblastomas, and one patient a neurocytoma-like spinal cord tumor. The neurocytomas were intraventricular, moderately cellular tumors with bland nuclei and perinuclear halos. Patients with neurocytoma were treated with surgery, radiation therapy, and/or chemotherapy, and have followed favorable clinical courses. The neuroblastomas were intraparenchymal, hypercellular tumors with necrosis and frequent mitoses. Patients with neuroblastomas were treated with surgery, radiation therapy and chemotherapy, with some clinical response, but overall poor survival. One of the two patients developed extracranial metastasis. The spinal cord tumor had histologic features of neurocytoma, and responded well to biopsy and radiation therapy. The cases are compared with the varieties of small-celled neuronal tumors described in the literature, and pathologic, histogenetic and treatment implications are discussed.

Primary gastric lymphoma. An analysis with emphasis on prognostic factors and radiation therapy

Primary gastric lymphoma, lymphoma originating in the stomach, without involvement of peripheral or mediastinal lymph nodes, viscera, or the bloodstream, is sufficiently uncommon that the indications for radiation therapy, the dose of irradiation necessary for control of lymphoma, optimum field size, and patterns of failure have never been established. The authors identified 26 patients, and reviewed their charts and pathologic material. Their overall 5‐year survival was 57%. Factors significantly influencing 5‐year survival were serosal penetration (32% versus 91%), regional lymph node involvement (33% versus 81%), and location on the lesser curvature (20% versus 89%). Histologic characteristics, extent of surgery, and, provided the patient was irradiated, involvement of surgical margins did not influence survival. Overall, survival was not affected by irradiation, but in patients with poor prognostic factors there appeared to be a beneficial effect. Analysis of local control in this and other series suggests that patients should receive at least 40 Gy, and that whole abdominal irradiation is not necessary. The majority of failures were distant, indicating a need for effective systemic therapy. Cancer 52:2044‐2048, 1983.

Intramedullary spinal cord astrocytoma versus glioblastoma: the prognostic importance of histologic grade.

Fourteen patients with intramedullary spinal cord astrocytoma (Grades I, II) or glioblastoma (Grades III, IV) were seen at a major referral center over a 19‐year period. Although similar surgical and radiotherapeutic techniques were used for each group, the nine patients with astrocytoma had a five‐year actuarial survival rate of 89% with five patients alive and well at least five years after treatment; none of the five patients with glioblastoma survived past three years. Histologic grade is the most important factor affecting prognosis for patients with intramedullary spinal cord astrocytomas or glioblastomas, and long‐term survival can be achieved postirradiation for many patients with astrocytomas with improved neurologic functioning in most.