Intravascular lymphomatosis (IL) in a child mimicking a posterior fossa tumor.

Intravascular lymphomatosis (IL) is a rare entity only recently included in lymphoma classification, whose main feature is the exclusive or predominant growth of neoplastic cells within blood vessels. The vast majority of the patients affected by IL belong to the 7th or 8th decade of life and present with skin rash or CNS diffuse necrotic or demyelinating lesions. Case report. SS, a 13-year-old girl, was admitted to a Neurosurgery Unit because of endocranic hypertension, where, after CT and MRI documenting a IV ventricle 3 cm diameter tumor, she was submitted to complete tumor excision: extemporary diagnosis was suggestive of medulloblastoma. When referred to us she had persistent fever with normal blood and spinal fluid cultures. Whole CNS MRI did not give evidence of residual or metastatic disease while CSF cytology showed only pleiocytosis. Treatment was started according to our ongoing protocol for medulloblastoma with pre-radiation chemotherapy. Before delivering radiotherapy (RT), upon review of histologic specimens, the definitive diagnosis of IL B-phenotype was made. The girl was re-admitted and, after a complete re-staging, chemotherapy was intensified according to our schedule for high-grade B-cell lymphoma and CNS was irradiated up to a total dose of 25 Gy. She remained alive in continuous complete remission at 21 months after diagnosis. The case here reported is unique for age, tumor presentation, and, so far, favourable outcome, in spite of the delayed histological diagnosis.

Management of medulloblastoma and ependymoma in infants: a single-institution long-term retrospective report.

To reduce the sequelae from CNS irradiation (RT), 16 children younger than 3 years with medulloblastoma-PNET (13 cases) and ependymoma (3 cases) were treated between 1987-1993 according to different postsurgical chemotherapy (CT) programs. None of these patients presented with metastases. Eleven patients were rendered disease-free by surgery, while 5 had residual tumor. Adjuvant therapy depended on patients' age, postsurgical status and parents' consent to radiotherapy (RT). Nine of the 16 infants remained alive in continuous complete remission from the first neoplasm (median follow-up 7 years). Three of them had been treated with CT alone and 6 with combined CT + RT (posterior fossa 4, whole CNS 2). Seven patients relapsed a median of 13 months after diagnosis, and all 7 of them died of their disease. Despite the omission of RT in 6 of the 16 patients and administration of only focal RT in 8 of the 16, the outcome of this series was satisfactory. Local failure (in 5/7 patients) was the major problem, despite the high dose of RT used in 2 of these 5. In 4 of 6 evaluable children school performance was satisfactory. One child in whom the entire CNS was irradiated developed glioblastoma multiforme 120 months after the first diagnosis of medulloblastoma.

Locally advanced breast cancer treated with primary chemotherapy: comparison between magnetic resonance imaging and pathologic evaluation of residual disease.

AIMS AND BACKGROUND: We evaluated the response of locally advanced breast cancer to induction chemotherapy using MRI techniques. The size and vitality of any residual pathologic tissue was quantified by means of morphologic and dynamic analysis. A curve derived from the dynamic parameters shows the uptake intensity with respect to the time elapsed since administration, which is related to vascularization and therefore indirectly reflects the angiogenesis of malignant tissue. METHODS AND STUDY DESIGN: A group of 30 patients were examined with MRI for staging purposes before undergoing treatment and subsequently to assess the response to treatment. Alterations in size and dynamic parameters were closely monitored. RESULTS: The overall accuracy was 90%, the sensitivity 96%, the specificity 75%, the positive predictive value 92.5% and the negative predictive value 66%. Interestingly, analysis of the dynamic curves made it possible to obtain additional information regarding the angiogenetic activity of the residual tumor. CONCLUSIONS: Evaluation of the response to treatment by means of conventional imaging and clinical examination can be particularly difficult because of the fibrosis induced by cytotoxic drugs or the small volume of residual disease. The additional information supplied by MRI could therefore allow a more conservative surgical approach in selected cases of optimal response to treatment, as well as a much more accurate follow-up. Furthermore, the variation in dynamic parameters according to the vitality of residual disease could in the future become a useful tool for monitoring the effectiveness of anti-angiogenetic drugs.

[Locally advanced breast tumors. Role of magnetic resonance in the assessment of response to preoperative therapy and of neoplastic residue before the operation]. / Tumori della mammella localmente avanzati. Ruolo della Risonanza Magnetica nella valutazione della risposta alla terapia prechirurgica e del residuo neoplastico prima dell'intervento.

INTRODUCTION AND PURPOSE: Induction chemotherapy is the preoperative treatment for locally advanced breast carcinoma. The patients affected with this kind of tumor were previously considered inoperable. The sequential use of different cytotoxic drugs reduces the tumor mass effectively, thus allowing resection and improving patients prognosis. Tumor debulking is at times so significant that conservative treatment can even be considered. A reliable assessment of the response to drug therapy by conventional diagnostic procedures is usually hindered by chemotherapy-induced fibrosis. Magnetic resonance imaging (MRI) is a better tool for distinguishing fibrosis from still vascularized pathologic tissue and thus permits more accurate evaluation of tumor response to chemotherapy, namely tumor debulking and residual viability. MATERIAL AND METHODS: We selected 27 patients with breast cancer and submitted them to MRI both before and after chemotherapy. All examinations were performed with a high field system using 3D Flash sequences with optimized spatial and temporal resolution. RESULTS AND DISCUSSION: The morphologic and dynamic parameters of MRI were in agreement with pathologic findings. In case of persistent disease after chemotherapy, MRI demonstrated increased contrast agent uptake at restaging, with dynamic curves indicating early and intense uptake. In case of marked post-chemotherapy changes, the dynamic curves had a shorter and less steep trend. Finally, when no or very little (few microns) tumor tissue was left, MRI showed no uptake. CONCLUSIONS: Our initial experience indicates MRI as a valid too for monitoring chemotherapy response in breast cancer patients.

Treatment of childhood post-irradiation sarcoma of bone in cancer survivors.

PATIENTS AND METHODS: This is a retrospective review of five children with post-irradiation bone sarcoma (PIS). Age at PIS onset ranged between 10 and 17 years (median 11). They were treated with a chemotherapy regimen, similar to that in use for primary osteogenic sarcoma, consisting of vincristine and high-dose methotrexate alternated with cisplatinum and ifosfamide, given for 12 months. RESULTS: In all children chemotherapy induced a complete clinical remission. Four of them were alive in continuous complete remission at 1, 2, 4, and 12 years from the diagnosis of bone sarcoma. One girl recurred 3 years from PIS diagnosis and was salvaged by repeating the same chemotherapy program: she remained alive in second complete remission 8 years from relapse. CONCLUSIONS: In spite of an intensive treatment previously given for the primary tumor, this drug schedule proved to be feasible and short-term side effects were manageable. Chemotherapy alone, using an intensive regimen effective for primary osteogenic sarcoma, may be an adequate therapy for childhood post-irradiation sarcoma.

Langerhans cell histiocytosis mimicking malignancy: a radiological appraisal.

AIMS: To analyze the radiologic characteristics, clinical course and long-term follow-up of 7 radiologically uncommon pediatric cases of Langerhans cell histiocytosis and to identify prognostic factors related to imaging patterns. METHODS: The clinical records and complete imaging data of 75 patients with LCH diagnosed and treated at the National Cancer Institute of Milan between January 1975 and December 1993 were analyzed, and 43 cases presenting as unifocal bone lesions were identified. The plain film, computed tomography and magnetic resonance characteristics enabled the identification of 7 radiologically aggressive and rapidly progressive cases, which were analyzed at presentation and during follow-up. RESULTS: Although at disease presentation bone lesions appeared lytic destructive, rapidly progressive and often involved adjacent soft tissues, after adequate therapy the disease course was invariably benign and led to almost complete restoration of normal structure and function. Long-term follow-up confirmed the favorable outcome and lack of disease recurrence in all cases. CONCLUSIONS: There is no correlation between radiologically aggressive characteristics and final outcome in Langerhans cell histiocytosis. Radiologists and pediatric oncologists should be acquainted with less common radiologic forms which, at presentation, can mimic more ominous diseases. If recognized and adequately treated, monostotic forms almost invariably have a benign prognosis.

[Diagnostic imaging of liver metastasis]. / Diagnostica per immagini delle metastasi epatiche.

The early diagnosis and monitoring of hepatic metastases are now achieved by different imaging modalities, some using ionizing radiations (computed tomography and angiography), some based on other energy sources (sonography and magnetic resonance), but all coming within the radiological area, which offers concrete possibilities of integration and the necessary organization. These modalities are sometimes used only for percutaneous histological samplings with minimal invasiveness. The progress in hepatic resective surgery and the possibility of orthotopic liver transplantation for some neoplastic histotypes, together with the alternatives provided by interventional radiology, have brought a continuous updating of the specialist' interest in the morphological and functional definition of hepatic metastatic disease, with the specific aim of choosing the best therapeutic strategy. Hepatic metastases have the greatest impact on the survival of patients with gastrointestinal neoplasms, especially colonic adenocarcinoma. Intraoperative sonography and CT arterial portography currently provide greatest diagnostic sensitivity in terms of spatial resolution but cannot be considered as methods of choice, the former for obvious reasons and the latter because of its invasiveness and complexity. The alternatives are to be sought in spiral CT and the new MR sequences which can undoubtedly provide a decisive improvement in the diagnostic standards currently available. Profoundly changed, but no less important, is the role of angiography, which still provides the anatomical support for hepatic surgery and the means for alternative treatments, such as chemoembolization and continuous infusional chemotherapy.

[Magnetic resonance in primary bone tumors: a review of 10 years of activities]. / La Risonanza Magnetica nei tumori primitivi dell'osso: revisione di dieci anni di attività.

All the MR exams of primary bone tumors performed during ten years were reviewed by three different radiologists. In all, 484 exams in 220 patients were considered--namely, 160 exams (33.1%) for staging purposes, 219 (45.2%) during therapy and 105 (21.7%) performed more than 8 months after the last treatment. Its well-known accuracy in the assessment of intra/extraosseous spread confirms the major role of MRI in the staging of primary bone tumors. During treatment, the overall accuracy of this method decreased to 88.8% because of the presence of therapy-induced tissue changes. MRI was 95.2% reliable in the detection of persistent disease or relapse in the exams performed long after therapy. Conventional radiology is still the method of choice in the study of primary bone tumors at presentation as it detects the lesion, differentiates malignant tumors and usually suggests the possible histotype. Nevertheless, MRI seems to be needed to depict actual tumor extent and to find the correct therapeutic approach. In the follow-up, MRI is the best single method to assess the response to therapy and to detect tumor persistence.

New imaging techniques in staging lymphomas.

A variety of imaging modalities are used to evaluate patients with lymphoma, mainly Hodgkin's disease. During the past few years, the improvement of diagnostic techniques has had a great impact on both staging and patient treatment. This review of the current status of imaging presents the possibilities of available diagnostic tools in the assessment of lymphomatous involvement of different organs and apparatus, emphasizing those aspects that might influence patient management and treatment. For central nervous system lymphomas, pre- and postcontrast computed tomography and magnetic resonance have completely replaced other radiologic methods. Similarly, because it seems clear that computed tomography will replace lymphography by the end of this decade, just as computed tomography and magnetic resonance imaging are expected to entirely replace staging laparotomy, the possibilities of these diagnostic tools and their impact on diagnosis and follow-up are also discussed.

[Value of magnetic resonance in the staging of non-small-cell lung carcinoma]. / Valore della risonanza magnetica nella stadiazione del tumore polmonare non-microcitoma.

In the last six years, 544 consecutive patients with nonsmall-cell bronchogenic carcinoma were studied with MRI. MR results were compared with surgical specimens and pathologic findings. Both TNM classification and ATS nodal mapping were considered. Local tumor extent (T parameter) was correctly identified in 67.2% of cases, understaged in 19.4% and overstaged in 13.4%. Lymph node metastases (N parameter) were adequately assessed in 55.5% of cases, understaged in 20.3% and overstaged in 24.2%. Considering two groups of patients, the first one with early stage disease (T1-T2, N0-N1) and the second one with advanced stages (T3-T4, N2-N3), MRI exhibited 84.6% overall accuracy, 57.3% sensitivity and 93.7% specificity for the T parameter and 72.3% accuracy, 65.2% sensitivity and 75.1% specificity for the N parameter. In conclusion, MRI did not provide the expected results in the staging of nonsmall-cell bronchogenic carcinoma, but improvement will probably be achieved with technological evolution.

Heart involvement in lymphomas. The value of magnetic resonance imaging and two-dimensional echocardiography at disease presentation.

BACKGROUND AND METHODS: Thirty-six patients with mediastinal lymphoma were studied with chest magnetic resonance imaging (MRI) and two-dimensional echocardiography at presentation to define the extent of the disease in the paracardiac area. RESULTS: Involvement of cardiac structures was present in 23 of 36 patients (64%). Pericardial contiguity was detected in 23 of 23 patients (100%) by MRI and in 18 of 23 patients (78%) by echocardiography. Pericardial effusion, present in 17 patients (74%), and pericardial infiltration, present in 7 patients (30%), were detected by both techniques in 71% and 86%, respectively. Myocardial infiltration was identified in two of two patients (100%) by MRI and in one of two patients (50%) by two-dimensional echocardiography. Extrapericardial disease was identified in 100% of patients by MRI but only in 30% of patients by echocardiography. CONCLUSIONS: Extracardiac and intracardiac involvement is a frequent event in mediastinal lymphomas and should be carefully evaluated with different imaging modalities, mainly MRI, for correct diagnosis and proper management.

Current role of gallium scan and magnetic resonance imaging in the management of mediastinal Hodgkin lymphoma.

BACKGROUND: A residual mediastinal mass after treatment represents a common diagnostic problem in the management of patients with Hodgkin lymphoma (HD). Conventional diagnostic radiology, computed tomography (CT), or ultrasonography (US) do not adequately reflect changes as fibrosis or necrosis. Gallium-67 (67Ga) imaging has been proven to be useful for the evaluation of HD in the mediastinum. The authors compared the ability of gallium scan and magnetic resonance imaging (MRI) to evaluate the mediastinal disease in the follow-up of patients with HD. METHODS: Thirty-four patients previously treated for HD were investigated with gallium scan, MRI, and all the other investigations to evaluate the mediastinal region. Sixteen patients were in restaging after treatment, and 18 were investigated for suspected radiologic recurrence in the mediastinum (follow-up, 9-75 months). The results of gallium scan and MRI were matched with clinical findings during the follow-up. RESULTS: A sensitivity of 85.7% for 67Ga and 92.8% for MRI was found, while the specificity was 100% for the scan and 80.6% for MRI. The predictive positive value that resulted was 100% for 67Ga and 68.4% for MRI. CONCLUSIONS: Both examinations were accurate in assessing the activity of residual masses in the mediastinum after treatment. 67Ga showed a lower sensitivity in comparison with MRI, but 67Ga frequently overestimates the presence of pathologic tissue. The authors acknowledge the complementary role of these two tests, but if only one of these examinations can be performed for logistic or economic reasons, then gallium scan represents the single most adequate diagnostic procedure.

Hallervorden-Spatz disease: MR and pathologic findings.

PURPOSE: To compare the MR findings of eight cases with clinical diagnosis of Hallervorden-Spatz disease (HSD) with the pathologic findings of two other cases of HSD. MATERIALS AND METHODS: The eight imaged cases were studied with 0.5-T (seven cases) and/or 1.5-T (five cases) units. Six patients also had CT scans. The two other cases with proven HSD had detailed histologic evaluation. RESULTS: The 1.5-T findings showed abnormalities confined to the pallidum, which presented a diffuse low signal intensity in T2-weighted images, and an anteromedial area of high signal intensity (eye-of-the-tiger sign). In 0.5-T studies, low signal intensity was less evident and poorly detectable in spin echo, but gradient-echo images could enhance its demonstration; the area of high signal intensity was always well demonstrated. In three cases (three with 1.5 T, one with 0.5 T) a central spot of low signal intensity was seen in this area. The pathologic cases, in addition to neuroaxonal swellings and iron deposits, exhibited areas of "loose" tissue with vacuolization and lesser amounts of iron in the anteromedial part of the pallidum, in a location corresponding to the area of high signal intensity of the imaged cases. CONCLUSION: Comparison of MR findings with the pathologic studies demonstrates that the low signal intensity in T2-weighted images at 1.5 T corresponds to iron deposits in a dense tissue, and that the high signal intensity of the eye-of-the-tiger sign corresponds to an area of loose tissue with vacuolization. No correlation was found in the two pathologic cases for the central spot of low signal intensity.

Magnetic resonance imaging of the gastrointestinal tract: investigation of baby milk as a low cost contrast medium.

After the incidental observation of the high signal intensity of the upper GI tract in a nourished baby, we tested eight baby milks; five different fresh commercial milks, one sweetened and condensed and two lyophilized milks in order to compare their ability to contrast MR images. The images were obtained with a 1.5 T magnet whereas the "in vitro" water proton relaxation time (T1 and T2) measurements were carried out at 0.5 T. After having selected the most effective lyophilized product, that was prepared according to the manufacturer's instructions, a group of 23 adult patients, 17 males and 6 females, with a mean age of 55.8 years (range 37 to 71 years) were examined. Thirteen patients had gastric cancer and ten patients had rectal or rectosigmoid junction tumors. The most effective imaging sequence was a spin-echo T1.w. After oral intake of milk a good contrast of the stomach, with sufficient distribution in the duodenum and the very proximal bowel, was achieved in all 13 patients with gastric cancer, as was a good depiction of the rectum and the recto-sigmoid junction after enema achieved in the 10 patients with rectal cancers. Disadvantages of lyophilized milk as a contrast agent are due to partial intestinal absorption, inhomogeneous distribution and irregular intestinal passage, whereas a clear advantage of lyophilized milk as a contrast agent is its good acceptance and palatable, inexpensive and non invasive properties. Because of these limitations lyophilized milk cannot be considered a real oral contrast medium but it can enhance MR imaging of the upper abdomen, and mainly of the lower GI tract in infants and adults.

[Radiological staging of metastases of urologic tumors]. / La stadiazione radiologica delle metastasi dei tumori urologici.

The present contribution considers the diagnostic possibilities of radiological imaging procedures in the evaluation of the lymphatic and bloodborne metastases in urological tumors. As regards the evaluation of the lymph node chains the presently available imaging modalities are discussed. For each of them, diagnostic accuracy and the percentage of false positive and false negative readings are presented. For the evaluation of the bloodborne metastases, the available imaging procedures for each organ and site are reported and a diagnostic flow chart is suggested.

Early stage Hodgkin's disease: ten-year results of a non-randomised study with radiotherapy alone or combined with MOPP.

From September 1976 to June 1982, 201 consecutive patients with stage I (A and B)-IIA Hodgkin's disease were stratified in two groups according to prognostic factors. The F group included 116 patients with favourable presentation: they were staged with laparotomy and treated with subtotal or total nodal radiotherapy alone. The U group included 85 cases with unfavourable presentation who were staged by laparoscopy and treated with 3MOPP (mechlorethamine, vincristine, procarbazine, prednisone)-radiotherapy-3MOPP. At 10 years the F group showed a freedom from progression (FFP) of 71% with significant difference between stage I and II (85% vs. 59%; P = 0.003) and an overall survival of 84%. The results of the U group were: FFP 83%, overall survival 74%, and the findings were not influenced by stage. FFP in patients with bulky vs. not bulky lymphoma was 70% vs. 87% (P = 0.04). No secondary acute non-lymphocytic leukaemia developed among patients treated with radiotherapy and in continuous complete remission, while acute leukaemia occurred in the F group patients who received salvage chemotherapy (4 of 31 cases) and in the U group (3 of 85 cases). Present results confirm the usefulness of radiotherapy alone in favourable pathological stage IA. All other disease stages will require a different strategy that should consist of radiotherapy combined with short-term effective regimens, such as ABVD (doxorubicin, bleomycin, vinblastine and decarbazine) or VBM (vinblastine, bleomycin and methotrexate) to reduce the incidence of MOPP-associated gonadal dysfunction and leukaemogenesis.

[Feasibility of magnetic resonance-guided fine-needle percutaneous biopsy]. / Possibilità di utilizzazione della risonanza magnetica nella guida alla biopsia percutanea con ago sottile.

Fluoroscopic, US, and CT guidance to aspiration cytology are valuable tools for oncologic diagnosis. MR imaging is now replacing CT as the imaging method of choice to guide biopsy mainly in the abdomen and pelvis. The standard needles used for CT-guided biopsy are unsuitable for MR guidance because ferromagnetic artifacts shade the underlying anatomy. A new needle specifically designed for MR guidance allowed MR-guided aspiration biopsies to be carried out in a group of 17 patients with different neoplastic diseases. To locate the lesion and to assess its depth, a glass pipette containing a diluted solution of paramagnetic contrast medium (Gd-DTPA) was placed on the skin surface. In 13/17 patients (76.5%), biopsy was successful and histology and/or cytology allowed a diagnosis to be made. In spite of their longer measurement times, T1-weighted SE images clearly demonstrated both needle and lesion. On the contrary, fast images (FLASH, 15 degrees-90 degrees), with/without breath holding, although much shorter (7 s), were often useless, due to artifacts, mainly in small lesions.