Search

Sarcomas represent a heterogeneous group of tumors with a complex and poorly reproducible classification. However, in the last ten years, several specific genetic alterations have been described allowing a molecular classification with: 1) sarcomas with a specific translocation which can be used as a diagnostic marker. These translocations can be demonstrated by RT-PCR or by FISH with commercially available break apart probes ; 2) sarcomas with simple genomic profile showing amplification of a few genes. Well differentiated liposarcomas, dedifferentiated liposarcomas and intimal sarcomas show a simple genomic profile characterised by MDM2 and CDK4 amplifications associated with amplification of other genes in dedifferentiated liposarcomas ; 3) sarcomas with activating mutations: about 90% of GIST show activating mutation of a receptor tyrosine kinase gene, either KIT or PDGFRA. The most frequent mutation involves exon 11 of KIT followed by exon 9 of KIT and exon 18 of PDGFRA. Demonstration of these mutations is useful for the diagnosis of CD117 negative GIST, for predicting response to imatinib and to explain secondary resistance to imatinib ; 4) sarcomas with inactivating mutations: malignant rhabdoid tumors show biallelic inactivation of INI1 gene with a lost of INI1 expression which can be demonstrated by immunohistochemistry ; 5) other sarcomas usually show a complex genomic profile characterised by numerous gains and losses of genes with a frequent loss of RB1 and alterations of P53. Leiomyosarcomas, pleomorphic rhabdomyosarcomas, pleomorphic liposarcomas, myxofibrosarcomas, poorly differentiated sarcomas (so-called MFH and fibrosarcomas) belong to this category and show no specific molecular abnormality.

Regional tumour banks are nowadays an important tool for the management of patients with a sarcoma. Centralized databases with information on frozen or paraffin-embedded tumour samples are necessary tools for clinical and biological research projects for rare tumours and particularly sarcomas. Diagnostic procedures have evolved during the last years with a multidisciplinary management of patients by a national and structured network specialized in sarcomas, a common use of core needle biopsies, a daily use of immunohistochemistry and molecular analyses and the implementation of standardized and structured reports.

To state about adequacy of surgery margins in soft tissue sarcoma (STS).Review of the relevant literature.The end point for measuring the quality of margins in STS is local recurrence (LR). The rate of LR in specialised centres lies between 16 and 22%. The rates of LR should be given by the actuarial estimations. The methods determining margins are variable in the literature. By the quantitative methods, measuring the distance between the tumour edge and the limits of resection on the excision specimen, a twofold difference in LR is seen between adequate and inadequate margins. This difference is fourfold by the qualitative, consensual method from the French Sarcoma Group, appearing here more discriminatory. Obtaining adequate margins depends on the planned character of surgery, tumour size, tumour infiltration and grade.An actuarial 5-year LR rate ranging from 10 to 20% is a criterion for good quality of surgery. These results appear to be obtained best in specialised centres.

Epithelioid sarcoma is a rare type of soft tissue sarcomas with a high risk of recurrence both local and distant. The place of surgical conservative treatment and the role of radiation therapy remain controversial.A serie of 9 consecutive patients treated with initial conservative surgery and postoperative radiotherapy (median dose of 52.8 Gy) from 1987 to 2006 in the same institution was analyzed.With a median follow-up of 40 months (range 15-153 months), the rate of local, nodal and distant relapse is respectively 56%, 11% and 33%. The rate of death is 44.5%. No imputation has been performed.Even with a high rate of local relapse observed, a conservative treatment doesn't seem to influence badly the overall survival (55.5% alive at 40 months). Indeed the rate of distant relapse and death are comparable with those found in the literature. Moreover relapse occurred almost within the irradiated volumes. An improvement of dose could be also discussed.

The National French Federation of Comprehensive Cancer Centres (FNCLCC) initiated the update of clinical practice guideline for the management of patients with soft tissue sarcoma in collaboration with the French Sarcoma Group (GSF-GETO), specialists from French public universities, general hospitals and private clinics and with the French National Cancer Institute. This work is based on the methodology developed in the "Standards, Options and Recommendations" (SOR) project.To update SOR guidelines for the management of patients with soft tissue sarcoma previously validated in 1995.The methodology is based on a literature review and critical appraisal by a multidisciplinary group of experts who define the CPGsaccording to the definitions of the Standards, Options and Recommendations project. Once the guidelines have been developed, they are reviewed by independent reviewers.This article presents the updated recommendations for radiotherapeutic management. The main recommendations are: 1) irradiation before or after surgical treatment is the standard for soft tissue sarcoma of the extremity and uterine sarcoma; 2) no systematic irradiation should be done in case of retroperitoneal sarcoma.

Over the last fifteen years, pathology underwent significant changes in the field of soft tissue tumours. They were related to considerable advances in molecular biology and genetics. New data led to the revision of the WHO classification. Malignant fibrous histiocytoma is no longer considered as an entity. It has split up into several subgroups belonging to liposarcomas, leiomyosarcomas or undifferentiated sarcomas. Haemangiopericytoma underwent reappraisal and was put in the same category as solitary fibrous tumour. Many tools have improved. Immunohistochemistry performed with new antibodies had its specificity increased, and became appropriate for the prediction of therapeutic response in some cases, e.g. CD117 detecting mutations of the c-kit proto-oncogen in gastro-intestinal stromal tumours. Refinement of the techniques allows accurate diagnoses from core needle biopsies. Surgical specimens are collegially examined by surgeons and pathologists with special attention paid to resection margins. Although bound by some limitations, the grading system of the French Federation of Cancer Centers has currently remained the best predictor of metastasis-free survival and overall survival of patients. It is based on an assessment of three parameters: differentiation, amount of necrosis, and mitotic count of tumours. The pathologist sets up a diagnosis, and actively takes part in the prediction of the prognosis and therapeutic response. He is one of the major participants in decision making for multimodal treatment of sarcomas.

Soft tissue sarcomas are rare and may be a source of problems for diagnosis and treatment. Four types of genetic disorders can be distinguished: translocations, gene amplifications, mutations and complex genetic imbalances. Detection of these disorders may help in diagnosis and in determining prognosis. Detection of specific translocation is recommended in synovial sarcoma, alveolar rhabdomyosarcoma or PNET diagnosis because of therapeutic consequences; in case of rarer histologic type (low grade fibromyxoid sarcoma, clear cell sarcoma, infantile fibrosarcoma...), it may confirm the diagnosis. In some cases, some translocations have a prognostic value (alveolar rhabdomyosarcoma) whereas it is discussed in others (synovial sarcoma). The techniques used to detect these translocations are very sensitive so it may be used to detect microscopical metastasis (bone marrow metastasis of alveolar rhabdomyosarcoma for example). Detection of MDM2 and CDK4 genes amplifications (FISH or quantitative PCR) may be sometimes useful in well differentiated and dedifferentiated liposarcomas diagnosis. Mutation detection of KIT or PDGFRA may help in GIST diagnosis and type of mutation is predictive of response to treatment. Study of complex genomic imbalances in sarcomas is not used in routine practice but remains useful in research.

To evaluate the experience of a single cancer center with unusual tumors. To analyze Primary breast sarcomas (PBS). To investigate treatment and prognostic factors influencing overall survival (OS) and disease-free survival (DFS).Retrospective study of a series of 42 patients. We reviewed the clinical records and pathology slides of 42 women with PBS treated in our institution between 1970 and 2002. Log-rank tests were used to determine OS and DFS.The median age at diagnosis was 56.9 years (24-81 years). Surgery was part of the therapeutic strategy in all the patients. Patients with angiosarcoma and those with malignant cystosarcoma constituted distinct populations. The 10-year OS and DFS rates were 53% and 55% for angiosarcoma patients and 89% and 100% for cystosarcoma patients (p=0.009 and 0.01 respectively).Careful preoperative multidisciplinary assessment is required before making the decision to treat. Mastectomy is generally indicated. Axillary lymph node dissection is not indicated.

To analyse the management and clinical outcome of patients treated for a first isolated local recurrence of soft tissue sarcoma (trunk or extremities) and to identify prognosis factors.This is a retrospective study of 83 adult patients treated between 1980 and 1999. Mean tumor size was 6 cm. Most sarcomas were located in extremities (N =74), were deep (N =60), and proximal (N =53). Thirty involved nerves or vessels. Histologic subtypes were mainly grade 2 (42%) or 3 (36%) histocytofibrosarcomas (49%) and liposarcomas (20%). Surgical treatment of recurrences consisted in wide excision (32 cases), marginal resection (46 cases), five patients requiring amputation. Final results were R0 (N =33), R1 (N =47) or R2 (N =3) resection. Beside surgery, six patients received neoadjuvant and seven others adjuvant chemotherapy. Twenty-three patients received postoperative external beam radiotherapy (EBRT) (mean dose 55 Gy) and 26 interstitial (192)Ir low dose rate brachytherapy (BCT) (mean dose 45 Gy for BCT alone, 22 Gy when associated with EBRT), 19 patients being re-irradiated.Mean follow up was 59 months. Thirty-seven (45%) tumors relapsed, 62% locally as first event. Nineteen patients developed secondary distant metastases. Multivariate analysis showed only tumour depth (P =0.05) and re-resection for primary R1 resection for the recurrence (P =0.018) being independent prognosis factors for tumour control, radiotherapy (EBRT and/or BCT) being significant in univariate analysis (P =0.05). Overall survival rate was 73, 54, and 47% at respectively 3, 5 and 10 years, and was 65, 35 and 32% after a further local recurrence. Multivariate analysis showed trunk (P =0.0001) or inferior extremity locations (P =0.023), symptomatic (P =0.001), high grade (P =0.01), deep (P = 0.01) tumours, and the occurrence of a further local failure (P =0.004) as unfavourable characteristics for overall survival.Because of the high relapse rate in this series, a first isolated local recurrence of STS increases mainly the risk of a subsequent local relapse. Quality of local treatment for the first local relapse is decisive. When a conservative treatment is feasible, it should combine surgical resection and radiotherapy, brachytherapy being the best suited in previously irradiated patients. Efforts have to be pursued to increase quality of the treatment of primary tumours, at best performed in centers that have expertise in this field.

The Standards, Options and Recommendations (SOR) project, which started in 1993, is a collaboration between the Federation of French Cancer Centers (FNCLCC), the 20 French Regional Cancer Centers, and specialists from French public universities, general hospitals and private clinics. The main objective is the development of clinical practice guidelines to improve the quality of health care and the outcome of cancer patients.To elaborate practice guidelines for difficult diagnoses in surgical pathology or cytopathology in cancer patients.The methodology is based on a literature review and critical appraisal by a multidisciplinary group of experts who define the CPGs according to the definitions of the Standards, Options and Recommendations project. Once the guidelines has been defined, the document is submitted for review by independent reviewers.The main recommendations to prevent and reduce the number of difficult diagnoses in surgical pathology or cytopathology are: The main recommendations to detect lesions associated with difficult diagnosis in surgical pathology or cytopathology are: The main recommendations to solve difficult diagnosis in surgical pathology or cytopathology are

The "Standards, Options and Recommendations" (SOR) project, started in 1993 is a collaboration between the Federation of the French Cancer Centres (FNCLCC), the 20 French Cancer Centres and specialists from French Public Universities, General Hospitals and Private Clinics. The main objective is the development of clinical practice guidelines to improve the quality of health care and outcome for cancer patients. The methodology is based on literature review and critical appraisal by a multidisciplinary group of experts, with feedback from specialists in cancer care delivery.To develop clinical practice guidelines according to the definitions of the Standards, Options and Recommendations project for difficult diagnoses in surgical pathology or cytopathology in cancer patients.Data were identified by searching Medline and using the personal reference lists of members of the expert groups. Once the guidelines were defined, the document was submitted for review to 71 independent reviewers.The main recommendations to prevent and reduce the number of difficult diagnoses in surgical pathology or cytopathology are: 1) The development of quality insurance programs with use of written procedures in each pathology laboratory (standard). 2) The knowledge of clinical data in order to explain surgical pathology or cytopathology results (standard). 3) The availability of complementary patient informations (radiologic data . . .) can be useful to explain surgical pathology or cytopathology results (option). The main recommendations to detect lesions associated with difficult diagnosis in surgical pathology or cytopathology are: 1) Tumor types known as potential difficult diagnosis in surgical pathology or cytopathology should be reviewed by a second pathologist. 2) The systematic second reviewing for every case is expensive but has to be done when the difficulty is know (sarcoma, lymphoma . . .) by experienced pathologists. The main recommendations to solve difficult diagnosis in surgical pathology or cytopathology are: 1) Block recuts, use of special techniques (immunocytohistochemistry and molecular biology), additional data from clinicians, second opinion by a local pathologist, or new specimen can be required for establishing the diagnosis (options). 2) Outside second opinion by expert pathologist has to be considered once the other steps did not allow to establish surgical or cytopathology diagnosis (recommendations, expert agreement).

Cutaneous location of multiple myeloma is rare, and generally develops as a consequence of direct spread from an underlying bony focus of the disease. Metastatic skin lesions without adjacent bone involvement are uncommon. The prognosis is very poor.A 74-year-old man consulted for a hemorrhagic cutaneous nodule localized on the left inguinal area. This patient had been treated for five months for a stage I IgG lambda multiple myeloma. The histopathologic examination of the lesion showed a predominantly nodular configuration made up of masses of atypical cells with numerous hemorrhagic areas. The diagnosis of cutaneous metastasis of multiple myeloma was confirmed by the positivity of the cells for anti-IgG lambda antibodies.Cutaneous involvement in multiple myeloma without extension from underlying bony focus is exceptional. The lesions generally consist of firm, erythematous nodules involving the neck and lower extremities. To our knowledge, we present here the first case mimicking clinically and histologically a malignant vascular proliferation.

Thirty six cases of clear cell sarcoma of soft tissue are reported. The median age was 44 years (5 to 80 years). The principal sites were the foot (11 cases), the hand and wrist (7 cases) and the knee (6 cases). The architecture was fascicular with lobular arrangement of cells delimited by delicate fibrous septa intimately bound to tendons or aponeuroses. Tumoral cells were round or fusiform with abundant clear cytoplasm sometimes epithelioid with round nuclei and prominent nucleoli. The mitotic rate was evaluated to 9/10 HPF. S100 protein was expressed in 33/36 cases and HMB45 marked 29/31 cases, without expression of cytokeratin. Three-year and 5-year survival rate were respectively 72% and 62%. Prognosis factors for global survival were efficiency of initial treatment with distal location and necrosis and FNCLCC grade. The distinction of clear cell sarcoma from metastatic melanoma is important because of the difference of prognosis.

Adult soft tissue sarcomas comprise a heterogeneous group of rare tumors having a wide range of clinical, gross and histological presentations. Outcome is variable. The pathologist's task is difficult but must be based on a rigorous method. The aim of these recommendations is to describe the main steps of management: collection of clinical information, performing biopsy, handling the fresh specimen, gross examination, histological examination, use of special techniques, definition of the main prognostic factors and key features of the pathology report.

Hyalinizing spindle cell tumor with giant rosettes is a slowly growing tumor with a risk of local recurrence in case of incomplete surgical excision that could be regarded as a distinctive type of low-grade fibroblastic tumor. We report a case involving the presacral area. This tumor was composed of areas of bland spindle cell proliferation with a fascicular pattern in a fibrous or myxoid stroma, intermixed with giant rosettes consisting of rounded cells surrounding a central collagen core. The tumor expressed vimentin, and, for cells comprising the rosettes, S-100 protein, NSE and CD-57. These latter cells exhibited ultrastructural features of Schwann cells, the tumoral cells of fascicular area exhibiting features of fibroblastic cells. Flow cytometry showed DNA-aneuploidy and a very low S-phase fraction. This tumor appeared to be composed of two cellular components, fibroblastic and Schwann cells, located, at the opposite of neurofibroma, in two distinct areas.

Important advances have been obtained in the care of soft tissue sarcoma in adults, mainly in the field of locoregional treatment. Surgery or combination of surgery and radiotherapy allow adequate tumor control with preservation of function for the majority of patients. However, the management of locally advanced primaries remains problematic. Moreover, although patients survival mainly depends on the metastatic risk of the disease, controversies remain in definition of pronostic factors as well as in the evaluation of the role of chemotherapy in curative therapeutical strategies. The case reported here allows a discussion of the different modalities of treatment for adults with soft tissue sarcomas and stresses the necessity of a multimodal approach in these patients.

We report one case of Solitary infantile Myofibromatosis of Bone in a 14-year old girl. Radiologic features of the tibial lesion were consistent with the diagnosis of a benign bone tumor. The final diagnosis was made on a total resection of the tumor. Histologically, the tumor consisted of nodules, hyalinized or cellular, with spindle-shaped cells resembling fibroblasts or smooth-muscle cells. There were numerous vascular spaces in close contact with these nodules. The tumor cells were immunoreactive with anti-alpha-smooth muscle actin antibodies. These data were consistent with the diagnosis of Myofibromatosis. The histologic features were similar to those of Infantile Myofibromatosis of other sites such as skin and soft tissue. The case reported is rare and not yet published, due to the age and sex of the patient, and the location of the tumor.

These recommendations regard the immunohistochemical evaluation of estrogen and progesterone receptors in paraffin sections of breast cancers. All the components of the procedure are dealt with: fixation, antigen retrieval, antibodies, controls, analysis and interpretation of immunostaining, report and quality assurance parameters. The purpose of these guidelines is to serve as a basis for standardization of techniques and results and to improve quality control.

Eight cases of breast myofibroblastomas are reported: two in men and six in post-menopausal women. This study and a review of the 19 published cases permit to define more precisely the particular features of this rare and benign mesenchymal tumour. Good demarcation, lack of necrosis and nuclear atypia argue for the benign nature of the lesion. It is made up of spindle cells (mainles myofibroblasts) which are positive for vimentin and not for S 100 protein or epithelial markers. Alpha-smooth muscle cell actin and desmin labelling are heterogeneous. This immunohistochemical study underlines the variable results of myofibroblasts for smooth muscle markers reflecting different degrees in their differentiation, without resolving the question of their origin. A simple surgical excision is required for this benign lesion. No recurrence has never been observed.

During a period of 15 years, 6 cases of dedifferentiated liposarcomas were found among 542 cases of adult soft tissue sarcomas, 77 of which were liposarcomas. They were huge tumors of the retroperitoneum, containing distinct areas of well-differentiated liposarcoma most often of sclerosing type and malignant fibrous histiocytoma or undifferentiated sarcoma most often of high grade malignancy. Immunohistochemistry on the dedifferentiated component showed a positivity with anti-vimentin and alpha-1-antichymotrypsin in 5 cases and with anti-alpha smooth muscular actin in 4 cases. Three patients developed local recurrence and a fourth one quickly died with bone metastasis. Other types of dedifferentiated sarcomas, the process of dedifferentiation and links between malignant fibrous histiocytoma and dedifferentiated sarcomas are discussed.

The prognosis for adenocarcinoma of the endometrium is dependent on the findings of the histopathological assessment of the tumor. In a retrospective study of patients treated in initially by surgery, the estrogen and progesterone receptors were assayed 89 times and the androgen receptors 64 times. No statistically significant correlation was found between any of these receptors and the degree of structural differentiation or degree of infiltration of the myometrium. The absence of any one of these receptors had no negative impact on the overall survival nor on recurrence-free survival. The same was true for the 9 tumors which were devoid of both estrogen and progesterone receptors. In the authors' experience, the results of these hormone assays did not provide any further information on which to base the prognosis of endometrial cancers.

We describe the clinical and histologic features of non-Hodgkin's lymphoma in 26 patients with human immunodeficiency virus 1 (HIV) infection. These represent 10 per cent of AIDS cases recorded in the Bordeaux area. Mean age was 42. Contamination was mostly related to homosexuality (50 per cent) and blood transfusion (27 per cent) with 5 female cases. The initial presentation of lymphoma was extranodal (69 per cent). Lymphoma spread was diffuse (65 per cent), involving the bone marrow (38 per cent), lymph nodes (35 per cent), central nervous system (27 per cent), oral and digestive mucosae (23 per cent), liver (19 per cent) and genital tract (12 per cent). Histologic types were of intermediate or high grade malignancy (88 per cent) with 38 per cent large, non cleaved-cell (centroblastic) subtype. Median survival was 4 months. Lymphoma caused death in 65 per cent of patients and opportunistic infection in 18 per cent. Lymphoma was the first manifestation of HIV infection in 10 patients (38 per cent) and was responsible for AIDS in 14 (54 per cent). Diagnosis of lymphoma could be established at an early stage on extranodal biopsy. In these patients a prolonged disease-free survival was obtained after chemotherapy alone or associated with radiotherapy.

From October 1985 to October 1988, 31 patients with a soft tissue sarcoma of extremities were treated in our institute by conservative resection and post-operative radiation therapy. The initial target volume encompasses the entire musculoskeletal area occupied by the tumor. The compartmental definition of this initial target volume is based on clinical, radiographic, histopathologic and operative findings. We analyzed the irradiated volume in terms of the anatomical definition and feasibility. We then evaluated the distribution of the dose in the irradiated volume. The variation of the dose is always less than 10%. Accordingly, the range of dose in the irradiated volume varies from 45 to 50 Gy in the compartmental volume after a complete resection, and from 55 to 60 Gy in the tumor bed after a marginal or incomplete resection. For this series, the median follow-up is 27 months. We observed 3 distant metastases and 1 progressive disease in the irradiated volume. No locoregional recurrence appeared in the margins or outside the irradiated volume. These preliminary results validate the compartmental definition of the initial target volume. Furthermore, they point out the lack of opportunity to encompass tendinous insertions in the irradiated volume and to boost the scar. Finally, the definition of optimal dose for the control of microscopic residual tumor is discussed.

Five cases of primary non-Hodgkin's lymphomas of the breast (stage IE in the Ann Arbor staging system) are described. No clinical or radiological feature has permitted to make the diagnosis before surgery. Histologically, all lymphomas are diffuse and B type. Three cases are high grade lymphomas, one of lymphoblastic type. Two cases are of lymphoplasmacytoid type, one of them associated with an occult invasive ductal carcinoma within the same breast, the other changing in high grade lymphoma at the recurrence time. Three of them reveal histological aspects of lymphoepithelial lesions and appear as mucosa-associated lymphoid tissue lymphomas (MALT). The differential diagnosis with a carcinoma is now easily done by immunohistochemical techniques. The importance of a diagnosis before surgery by cytopunction and drill-biopsy has to be emphasized, to prevent an useless mastectomy.

The number and distribution of Leu-7 + cells, a subset of Natural Killer cells clustered as CD 57 + cells, were studied with an immunoperoxidase technique on reactive lymph nodes (n = 13), malignant lymphomas (n = 60) and Hodgkin's disease (n = 22). Results of paraffin-section immunocytochemistry were compared with those obtained of frozen sections of the same tissues. CD 57 + cells are small lymphocytes mainly located in the germinal centers of reactive lymph nodes and in their malignant counterpart, i.e. the follicular lymphomas. The paragranuloma of Hodgkin's disease, type I nodular, contained high numbers of CD 57+ cells. Nine cases of CD 57+ lymphomas are reported, which were of high grade of malignancy. Their histological subtypes were anaplastic (3 cases) immunoblastic (2 cases), pleomorphic medium and large cell (3 cases), unclassifiable (1 case). Their diverse T-cell (4 cases) or B-cell (2 cases) origin and the various expression of epithelial membrane antigen (EMA) or Kil/Ber-H2 (CD 30) suggest an aberrant CD 57+ phenotype of a subset of large cell lymphomas.

Angiosarcomas of the breast are exceedingly rare tumors that are only infrequently seen by pathologists examining fine-needle biopsy specimens. We report two cases in two women aged 52 and 57 years. Cytologic features are analyzed and differential diagnosis is discussed.

Lymph nodes in breast cancer have been examined by two histological techniques in 324 patients who underwent axillary node dissection. A routine pathologic examination consisted in the examination of only one pathological section while in the other, the lymph nodes were macroscopically serially sectioned. With the latter, an increase of 18.8 per cent in the number of detected metastases was noted. However this increase can be considered of prognostic and therapeutic importance only in 8.6 per cent of cases: half corresponding to those cases in which more than the initial threshold number of three lymph nodes were found, the other half corresponding to those cases in which one lymph node was found while the initial examination was negative. In the latter case, the increase of lymph node involvement was mainly due to "clandestine" metastases, i.e. lymphatic embolisation of micrometastases.

The variations of histologic patterns inside the primary tumor have been studied in 15 patients with locally advanced soft tissue sarcomas who were given induction (neo adjuvant) chemotherapy. Increase of tumor necrosis and decrease of tumor cellularity were the predominant aspects identified. The mitosis count tend to be lower, while the nuclear atypies and the number of giant malignant cells tend to increase. In 2 cases, a better tumor differentiation, attributable to the induction chemotherapy was noted. In 10/15 cases, it was concluded to a tumoricidal effect of the chemotherapy. According to tumor grade, 6 among 10 patients with grade III tumor presented a histological response (greater than 50 p. cent of tumor destruction) while none among 5 lesions of grade I or II exhibited a response of such a level. So, the histological study allows an evaluation of the response to chemotherapy which usefully completes the clinical appreciation, and that may be useful for the selection of postoperative adjuvant treatment, as it is in osteosarcomas.

Five cases of mammary carcinoma with osteoclast-like giant cells are reported. This study as well as review of the literature underline characteristic features of this rare histological type of carcinoma: a round well-circumscribed dense nodule of benign appearance on mammograms in half of the cases; brown colour of tumour specimens; stromal hypervascularity; negativity of epithelial, vascular and most histiocytic immunohistochemical markers in giant cells; positivity of leucocyte common antigen and vimentin in giant cells. Giant cells most probably originate from the fusion of histiocytic type mononucleated stromal cells, but results of immunohistochemical stainings cannot actually bring formal arguments to support this hypothesis.

One thousand two hundred and twenty-five partial breast resections, examined in serial gross sections have been retrospectively reviewed. The percentage of benign lesions in comparison with malignant lesions is 70 per cent-30 per cent. The percentage of malignant lesions is maximum (43%) in clustered microcalcifications which are so the best criteria of malignancy. A diagnosis of non infiltrating carcinoma has been misdiagnosed at the first examination in 6 per cent of cases. This misdiagnosis is partially explained by very small foci of carcinoma and recent recognition of certain histological types of early carcinoma. It must be noticed the unsuspected frequence of carcinoma (17%) detected by random in ipsilateral breast tissue from plastic surgery, after controlateral reconstruction for carcinoma. The analysis of benign lesions associated with carcinoma shows a significative frequent association with carcinoma in two types of lesion: radial scar and late phase of blunt duct adenosis.

In pathology, reproducibility of lesional diagnostic and classification is not perfect, particularly when dealing with rare tumors like soft-tissue sarcomas, the classification of which is complex. Reproducibility among many independent observers can be well appreciated by kappa statistics, which take into account the agreement rate expected by chance only. A study of the reproducibility of histological classification and grading of 25 soft-tissue sarcomas by 15 pathologists showed that the agreement was better for the grading. A review of some other applications of kappa statistics in pathology reported in the literature is presented.

Between June, 1979 and December, 1983, 23 adult patients with non-metastatic soft tissue sarcoma were treated with induction chemotherapy since local spread precluded primary conservative surgery. Eighteen patients received a combined cyclophosphamide, vincristine, adriamycin and dacarbazine treatment and 5, a combined adriamycin, cis-platinum and vindesine treatment. Both drug combinations exhibited toxic effects, notably on blood and digestive tract. However, tumoral volume was reduced by 50 p. cent or more in 14/23 patients, including 2 complete remissions; the course of the disease remained unchanged under treatment in only 3 cases. Chemotherapy facilitated surgical excision in 14 patients, and subsequent radiotherapy resulted in complete remission in 13 of these. None of the 13 patients relapsed during a mean follow-up period of 12 months (range: 3 to 48 months). Among the 9 patients who could not be operated upon, 5 benefited from radiotherapy with satisfactory local control, but only one still remains in complete remission. Thus, induction chemotherapy made it possible to achieve local control in two-thirds of patients with locally advanced sarcoma. However, a more prolonged follow-up is necessary to confirm that these results are long-lasting and to determine the effects of chemotherapy, if any, on the metastatic potential of these tumours.

We report two cases of giant cell tumor of bone (osteoclastoma) associated with Paget's disease. One appeared on the left cubitus in a 68-year-old male patient while the second involved the right maxillary sinus in a 79-year-old woman. Histopathological study was completed by ultrastructural and immunocytological studies. They were demonstrated to contain intranuclear virus-like filamentous inclusions in the osteoclasts. However, we cannot obtain positive response with antisera against measles virus and respiratory syncitial virus. These data are confronted to the hypothesis for a viral aetiology of Paget's disease and giant cell tumor of bone, associated or not to Paget's disease.

A rare tumor, primary choriocarcinoma of the stomach occurred in a 68 year-old man. Histologic examination of the tumor revealed two components, choriocarcinoma and adenocarcinoma. The liver metastasis displayed only choriocarcinoma, the lymph nodes metastasis only adenocarcinoma. The diagnosis was confirmed by demonstration of beta HcG in serum and urines by radioimmunoassay and within syncytiotrophoblast cells by immunoperoxidase staining. A review of the literature is done. The pathogenesis is discussed with hypothesis that this tumor represents a form of retro-differentiation of a preexisting carcinoma. However, this diagnosis will only be certain when a metastasis of a non gestational, non gonadal choriocarcinoma will be eliminated.

Bone pains observed in patients undergoing estrogen therapy, and presenting with osteoblastic metastases from prostatic cancer are usually related to unsuccessful treatment. In some patients, these pains may result from osteomalacia--ie incomplete mineralization of the new bone--because of the drainage of calcium by the osteoblastic metastases. A clinical, biological and histomorphometric study of bone specimens without decalcification was conducted in ten patients with osteoblastic disease secondary to prostatic carcinoma, who were under estrogen therapy, and for whom a change of therapy was contemplated. The study reports three cases of osteomalacia. Their bone pains were more intense, more diffuse and more permanent than those registered by patients without osteomalacia. All three had had previous fractures of the neck of the femur and a low urinary and serum calcium and phosphorus content. The discovery of osteomalacia by histomorphometric study is important because it allows effective, etiological treatment of the bone pains in these patients.

An histomorphometric study with double tetracycline labelling was carried out on iliac biopsies taken from patients suffering from Paget's bone disease; 64 of the cases revealed non-pagetic bone in radiology, and 72 revealed a pagetic incidence. The purpose was to better compare non-pagetic bone with pagetic bone. 5 biopsies performed in a radiologically non-pagetic area were shown to be histologically pagetic. An histomorphometric profile of non-pagetic bone in a patient suffering from Paget's disease was established: increase in the reabsorption surfaces, of the periosteocytic uneven surfaces, of the volume and of the osteoid surfaces in the women; decrease in the osteoid thickness indicator and in the speed of normal calcification. These signs of hyperremodelling were, for the authors, connected with a hyperparathyroid condition which was secondary to calcium needs in the pagetic areas. The effects of diphosphonates on non-pagetic bone in the patients suffering from Paget's disease are clarified. This therapy has a beneficial action on the bone volume of non-pagetic bone, when the initial bony trabecular column is small.