Biomedical subjects
Publications and source records attributed to Daniel Vanel.
Primary breast amyloidosis is rare, usually manifestating as suspicious masses in mammography, with or without associated microcalcifications. The final diagnosis is based on fine needle aspiration cytology or biopsy yielding clumps of amorphous material surrounded by lymphocytes and giant cells that characterize this entity. In our case the only mammographic finding was that of clustered, suspicious microcalcifications without any associated mass.
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The Breast Imaging Report and Data System (BIRADS) of the American College of Radiology (ACR) is today largely used in most of the countries where breast cancer screening is implemented. It is a tool defined to reduce variability between radiologists when creating the reports in mammography, ultrasonography or MRI. Some changes in the last version of the BIRADStrade mark have been included to reduce the inaccuracy of some categories, especially for category 4. The BIRADStrade mark includes a lexicon and descriptive diagrams of the anomalies, recommendations for the mammographic report as well as councils and examples of mammographic cases. This review describes the mammographic items of the BIRADS classification with its more recent developments, while detailing the advantages and limits of this classification.
OBJECTIVE: Germ-cell tumours (GCTs) with a non-GCT malignant component are a unique and rare phenomenon called teratoma with malignant transformation (TMT). The only published series of patients with TMT treated with chemotherapy comprised 10 patients. We report here our experience in treating 14 patients with TMT. PATIENTS AND METHODS: Sarcoma was identified in 10 of 14 patients, with rhabdomyosarcoma ranking first (n=4). Other histological types included adenocarcinoma (n=3) and bronchoalveolar carcinoma (n=1). Immunohistochemistry was performed to help in identifying the malignant non-GCT component. RESULTS: Primary treatment consisted of surgery alone in 4 patients. The remaining 10 patients received first-line cisplatin-based chemotherapy with resection of residual masses (n=5): 4 patients had a complete response and 5 had a partial response. Overall, 9 patients developed a relapse with a median time of 84 mo (range: 6-168). At relapse, 8 patients received a chemotherapy regimen directed to the non-GCT component. Four of these patients achieved a partial response. With a median follow-up of 59 mo (range: 3-180), 4 of 14 patients are alive, including 3 who are disease-free. CONCLUSION: To our knowledge, this is by far the largest reported European series of chemotherapy in TMT. Although TMT has a poor prognosis compared to GCT, its management may be improved by adapted chemotherapy associated with surgical resection of residual masses.
Retroperitoneal sarcomas are rare neoplasms. The aim of this article is to expose imaging protocols and useful radiological signs, to show the core biopsy technique for diagnosis, to precise imaging key points before treatment and for survey.
In soft tissue sarcoma, surgical resection remains the cornerstone of therapy for localized disease. Quality of margins is very important to evaluate. In case of marginal or incomplete resection, a new enlarged surgical resection should always be discussed before administration of any adjuvant treatments. Many retrospective studies and 2 randomized studies (one of adjuvant brachytherapy and one of external beam radiotherapy) have shown that adjuvant radiotherapy after complete surgery reduces significantly the risk of local recurrence in extremity soft tissue sarcomas. Combination of surgery and pre- or postoperative radiotherapy has therefore become the standard treatment with a local recurrence rate <or=25% and very few amputations. A recent randomized study has compared pre-op to postoperative radiotherapy. The results in terms of local control are similar in both arms (93 and 92% at 5 years) but the risk of early complications is higher in the preoperative arm and the risk of late sequela is higher in the postoperative arm. Surgical resection without radiotherapy may be considered after discussion at best within a multidisciplinary meeting, if surgical margins are considered satisfactory, in superficial, small tumours and low-grade. In retroperitoneal sarcomas, adjuvant radiotherapy is not a standard. It may decrease the risk of local recurrence but at the price of an increased gastro-intestinal toxicity. A randomized trial is warranted. Because of its rarity, and the risk of recurrence both local and metastatic, treatment should preferentially be discussed with a multimodality specialized approach.
Radiologists perform various examinations at every step of lymphomas. The role of imaging is atypical or a 'classical' oncologic radiologist, as multiple non-radiological criteria are combined to decide on treatments. A good knowledge of the practical use of the results helps the radiologist to seek the useful pieces of information. In treatment evaluation, uncertain complete response is only used in lymphomas. Imaging is changing, with the emergence of PET and whole body MRI but CT remains the key examination today. The WHO criteria are the only ones used to evaluate treatment results on CT, even though the use of PET is increasingly used, with better and better results.
PURPOSE: We report computed tomography (CT) findings in 13 patients with a primary abdominal desmoplastic small round cell tumor. MATERIALS AND METHODS: 13 cases (12 men, 1 woman, mean age=24.8 years) were found in our hospital database between 1991 and 2003. Clinical, CT and histopathological features were studied retrospectively. RESULTS: Peritoneal involvement was the most common feature. In 10 cases, several lobulated peritoneal soft tissue masses (with a mean of four masses per patient) were seen. Two patients had diffused irregular peritoneal carcinomatosis without any distinct peritoneal masses. One patient had a solitary mass in the pelvic space. The main sites of peritoneal involvement were the pelvic space (n=7), omentum (n=5), retroperitoneal space (n=4), small bowel mesentery (n=3), paracolic gutter (n=2 on the right and n=1 on the left), transverse colon mesentery (n=1), peri-splenic space (n=1), peri-hepatic space (n=1). The soft tissue masses were often bulky (mean 6cm, range 1-28cm), lobulated and heterogeneous with hypodense areas (in 73% of cases). In six cases, moderate ascites was seen. In one case of pelvic involvement, unilateral hydronephrosis was seen. Adenopathies were present in seven cases at the time of the diagnosis (at intraperitoneal, retroperitoneal and pelvic sites in six patients and in the groin in one patient). Five patients had liver metastases (four lesions per case excepted one patient with 30 metastases). Associated thoracic metastases were seen in three patients. The diagnosis was confirmed with four CT-guided percutaneous biopsies. CONCLUSION: Although CT features are nonspecific, the diagnosis of desmoplastic small round cell tumor may be suspected in young men with multiple bulky heterogeneous peritoneal soft tissue masses. Imaging is useful for staging and also to guide biopsies.
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The purpose of our study was to explore and evaluate an innovative imaging approach, which consists on imaging the breast parenchyma by means of photoluminescence detectors (LED) and analysis of dynamic data. Breast magnetic resonance imaging (MRI) was chosen as the reference imaging method, as this is considered to be nowadays the gold standard for breast vascularisation evaluation. Preliminary results reveal a good correlation between breast MRI findings and light images.
PURPOSE: To prospectively analyze the evolution of hepatic and peritoneal unresectable metastases from gastrointestinal stromal tumors (GIST) under imatinib mesylate, a new targeted treatment, which induces changes in lesion structure. MATERIALS AND METHODS: 54 patients with metastases from GIST underwent an abdominal and pelvic computed tomography examination without and with contrast enhancement, before and during treatment with imatinib mesylate. The number and size of lesions and contrast enhancement were noted before treatment and every 2 weeks for the first 2 months, then every 2 months for the first year of treatment and every 3 months thereafter. RESULTS: 27 patients presented with both hepatic and peritoneal metastases, 14 had only peritoneal and 13, only hepatic disease. On baseline imaging, all metastases were hypodense heterogeneous lesions with progressive, concentric enhancement. After treatment (mean duration of follow-up: 23 months) metastases decreased in size number and enhancement in 35/54 patients, remained stable in 2 patients and increased in 14 patients. In 13/39 patients with hepatic metastases a cyst-like appearance was noted. Reactivation after a partial response appeared first as a focal, peripheral, solid nodule in the wall of a cystic lesion, or an increase in lesion density, before size regrew. CONCLUSION: Besides the classic size criterion, a decrease in density and in contrast enhancement with stable "near cystic" lesions signifies a good response. A more aggressive approach (surgery or radiofrequency ablation) may be indicated for initially focal recurrences with a stable size.
BACKGROUND: The management of gastrointestinal stromal tumors (GIST) has evolved very rapidly in the last years. A national consensus meeting was therefore organized in order to identify the optimal management procedures for patients with GIST in localized and advanced stages. METHODS: A panel of different specialties, including pathology, molecular biology, imaging, surgery, gastroenterology, medical oncology reviewed the current literature, in particular the recent Lugano conference, to identify consensus points and topics for future research in four different working groups: pathology and molecular biology, early management of small tumors and imaging, surgery, and medical treatment. Consensus points were categorized according to the Standard Options Recommendations (SOR) of the French Federation of Cancer Centers. RESULTS: The standard histological examination with immunohistochemical analysis using CD117, CD34, PS100, desmin and smooth muscle actin is considered standard. Molecular biology for the identification of KIT and PDGFRA mutation is advisable for GIST with negative CD117 staining, and otherwise is considered a research procedure. Complete tumor resection with negative tumor margins is the standard surgical treatment. Adjuvant imatinib after optimal tumor resection as well as neo-adjuvant imatinib remain experimental approaches to be performed within prospective clinical studies. Imatinib should be started at the date of diagnosis of metastatic relapse and given until development of intolerance or progressive disease. Resection of metastases is also considered as an experimental procedure which can not be recommended routinely. The criteria for tumor response to imatinib should include not only tumor size reduction or disease stabilization, but also reduction of tumor density (Hounsfield units) on computed tomography, metabolic activity (i.e. reduction of FDG uptake on positron emission tomography), and reduction of vascularisation of the tumors using contrast enhanced ultrasound evaluation. An increase in tumor size may be associated with pathologic response to imatinib therapy, and available survival data indicate that the survival of these patients is similar to that of patients with conventional tumor response. CONCLUSIONS: Consensus points in clinical management of GIST in this national conference adopted the majority of consensus points published in the Lugano conference. This multidisciplinary work will be published in the reference oncology, gastroenterology, and pathology journals in French languages.
PURPOSE: To prospectively evaluate the use of MRI with dynamic sequences during isolated limb perfusion (ILP) for soft tissue sarcomas, an aggressive local treatment using very high-dose chemotherapy and tumor necrosis factor aimed at avoiding limb amputation. DESIGN AND PATIENTS: Twenty-six patients were referred for ILP over one and a half years; eight were excluded as the lesions were either too proximal or suspicious inflammatory changes without tumor were found on the initial MRI, or the vascular status was poor. The indications for ILP were: vessel nerve involvement (13), multiple lesions (8), tumor size (4) or the presence of pulmonary metastases (2). MRI was performed 1 and 2 months after ILP, immediately prior to surgery and histological analysis. The MR examinations included T1-weighted SE and fast SE T2-weighted fat-saturated sequences, as well as dynamic sequences (T1-weighted SE repeated six times every 40 s), displaying the maximum intensity slope in each pixel. RESULTS: The tumor had disappeared in three patients. One patient still had histologically proven isolated widespread tumor cells without a mass. The tumor size had increased in two patients. In six patients, the size of the tumor had not changed but it had become completely necrotic, with a thin wall. In three patients, after an initially good result MRI demonstrated that the tumor wall had become thickened from 1 to 2 months after ILP. Dynamic MRI was mainly useful during the initial examination, demonstrating two patients with inflammatory changes without tumor. Three amputations and a second ILP were proposed based on poor results. Conservative limb-sparing surgery was successful in the other cases. CONCLUSION: MRI proved valuable in demonstrating the variable responses to ILP.
OBJECTIVE: Bone allografts and vascularized fibula autografts were combined (the fibula inside the massive allograft) for skeletal reconstruction in a homogeneous group of patients. To verify the biologic behavior of the grafts, we followed the series using conventional radiography and CT analysis. MATERIALS AND METHODS: Twenty-four patients with bone tumors had intercalary segments of tibia or femur reconstructed and were followed up for 36-120 months. Sequential radiographs and CT scans were analyzed. RESULTS: Three types of behavior were observed. In 13 patients, the allograft maintained its architecture without fracture, although a regular enlargement of the inlaid fibula led to progressive integration with the allograft. A dense line on allograft endosteum was the first sign of bone bridges heralding fusion of the two grafts. In eight patients, fracture or nonunion of the allograft occurred, and the autograft reacted with rapid appearance of dense hypertrophy that again induced bridges to the allograft. In three patients, no changes in autograft size and density were followed by fracture with no callus formation. This behavior was interpreted as unsuccessful vascularization of the autograft. CONCLUSION: Sequential radiography and CT analysis enabled us to understand the changes in a combined graft offering an original way to revascularize bone allografts.
Local recurrence is the determinant of tumor-related mortality in retroperitoneal sarcomas because death often occurs as a result of local progression mostly without synchronous metastasis. Complete resection of the lesion and surgical margins status are the only therapeutic factors significantly associated with local control. Further outcome improvements need multimodal therapy since prognosis of these recurrences is poor with lower rates of complete resection and higher grade of malignancy than primary. Complete resection of the recurrence often requires removal of adjacent organs to achieve negative margins. External beam radiotherapy (EBRT), eventually associated with a boost of intra-operative electron beam radiotherapy (IORT) could improve the outcome in these patients. Preoperative timing could limit its toxicity. Chemotherapy protocols may enhance local and systemic outcome and can reduce the volume of high grade tumors and therefore allow a higher rate of complete resection. Isolated pelvic perfusion with local high doses chemotherapy is under investigation. Surgical excision of lung metastases should remain the treatment of choice, if preoperative evaluation indicates that complete clearance of the metastases is possible. Intra-operative chemotherapy after cyto-reductive surgery for the treatment of sarcomatosis is disappointing and complete surgery remains the cornerstone of the treatment with best results for low grade sarcomatosis. Adequate management at the time of primary presentation is likely to afford the best chance for long-term survival.
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We report a rare case of Hodgkin's disease primarily involving the oropharyngeal region. The patient presented with stage IIEA disease with a favorable response to treatment. Our literature search revealed that this disease affected rarely the lymphoid tissues of Waldeyer's ring. In this atypical location, tonsil and nasopharynx represent the most frequently involved sites. The involvement of the posterior wall of the oropharynx by Hodgkin's disease has been reported only once so far. When Waldeyer's ring is involved, the disease is usually localized, with occasional involvement of cervical lymph nodes. In this location, mixed cellularity is the most frequent histologic subtype and the Epstein-Barr virus is found in the majority of cases. This report is followed by a discussion on the clinical and pathological features, the implication of Epstein-Barrs virus and a literature review for Hodgkin's disease involving Waldeyer's ring.