Rare case of remission of a patient with small cell carcinoma of the ovary, hypercalcaemic type (SCCOHT) stage IV: Case report.

INTRODUCTION: Small cell carcinoma of the ovary (SCC) is a very rare (less than 1 % of ovarien neoplasia), highly undifferentiated, aggressive malignancy affecting young women and linked to a poor prognosis. Overall survive rate is very low (about 16 %). SCCOHT has recently been shown to be associated with SMARCA4 gene mutations and shows some genetic similarities to malignant rhabdoid tumors (MRT). PRESENTATION OF CASE: After a reminder of the clinical, histological description of the SCCOHT and concensus about the medical management, we describe the rare case of a 22 years old patient with complete remission after diagnosis of un undifferentiated SCCOHT stage IV treated by conservative surgery and high-doses chemotherapy, 30 months after diagnosis. DISCUSSION: Thus far, no standard therapy exists for SCCOHT. Treatment modalities are surgery, chemotherapy, radiotherapy and autologous stem cell transplant after high-dose chemotherapy. Research for new treatments includes target therapy. CONCLUSION: Autologous stem cell transplant after high-dose adjuvant chemotherapy seems to lead to the best survival rates. Invasiveness of the treatment depends on the stage of the disease, age of the patient and her fertility-sparing desire. An international collaboration will be needed to standardise practices due of the small number of patients.

Accessory cavitated uterine mass: MRI features and surgical correlations of a rare but under-recognised entity.

OBJECTIVES: To describe MRI features of accessory cavitated uterine mass (ACUM) with surgical correlations. METHODS: Eleven young women with an ACUM at pathology underwent preoperative pelvic MRI. Two experienced radiologists retrospectively analysed MR images in consensus to determine the lesion location within the uterus, its size, morphology (shape and boundaries), and structure reporting the signal and enhancement of its different parts compared to myometrium. The presence of an associated urogenital malformation or other gynaecological anomaly was reported. MRI features were correlated with surgical findings. RESULTS: All 11 lesions were well correlated with surgical findings, lateralised (seven were left-sided), and located under the horn and the round ligament insertion. Nine were located within the external myometrium, bulging into the broad ligament. Two were extrauterine, entirely located within the broad ligament. On MRI, the mean size was 28 mm (range 17-60 mm). Nine lesions were round-shaped, two were oval; all had regular boundaries. At surgery, the ACUM were not encapsulated but were possible to enucleate. On MRI, all lesions were well defined and showed a central haemorrhagic cavity surrounded by a regular ring (mean thickness, 5 mm) which had the same signal compared to the junctional zone. ACUM was isolated in all women, without urogenital malformation, adenomyosis or deep endometriosis. CONCLUSIONS: On MRI, ACUM was an isolated round accessory cavitated functional non-communicating horn-like aspect in an otherwise normal uterus. MRI may facilitate timely diagnosis and appropriate curative fertility-sparing laparoscopic resection. KEY POINTS: • ACUM is rare, with delayed diagnosis in young women with severe dysmenorrhoea. Pelvic MRI facilitates timely diagnosis and appropriate curative fertility-sparing laparoscopic resection. • Quasi-systematically located under the uterine round ligament insertion, ACUM may be intramyometrial and/or in the broad ligament. • On MRI ACUM resemble a non-communicating functional accessory horn within a normal uterus; the mass, most often round-shaped, had a central haemorrhagic cavity surrounded by a regular ring which had the same low signal compared to the uterine junctional zone.

Thymoma associated with autoimmune diseases: 85 cases and literature review.

OBJECTIVES: To describe the clinical features, treatment, and outcome of autoimmune diseases (AD) in a cohort of patients with thymoma. DESIGN: Pathological records from three university hospitals, between 2005 and 2011, were reviewed to identify patients with thymoma. Patients with thymoma and AD were compared with patients with thymoma without AD. RESULTS: 47/85 (55%) cases of thymoma had AD, including myasthenia gravis (MG) (n=33), Hashimoto's thyroiditis (n=4), Isaac's syndrome (n=3), Morvan syndrome (n=2), pure red cell aplasia (n=2), systemic lupus (n=2), lichen planus (n=2), and one case of each following conditions: aplastic anemia, autoimmune hemolytic anemia, Good's syndrome, pemphigus, autoimmune hepatitis, Graves' disease, limbic encephalitis, and inflammatory myopathy. Six patients (7%) presented at least 2 ADs. The median duration of follow-up after surgery was 60 months (40-78 months). In 32 patients, the diagnosis of AD preceded the diagnosis of thymoma, in 9 patients, thymoma was diagnosed at the same time as the AD and 7 patients had been operated on when they developed an AD. We found a significative difference on the Masaoka stage between the MG patients and the patients who present another AD (p=0.028). No risk factor for developing an AD after thymectomy was identified. CONCLUSIONS: We describe here the long-term follow-up of a large series of AD related to thymoma. Our results confirm previous data concerning AD occurrence in patients with thymoma and suggest that preexisting autoimmunity is not a risk factor for developing autoimmune manifestations after thymectomy.

[Tumor-bronchial actinomycosis simulating a recurrence of lung cancer 14 years after initial treatment: A case report]. / Actinomycose bronchique pseudo-tumorale simulant une ré-évolution d'un cancer bronchique 14ans après la prise en charge thérapeutique initiale : à propos d'un cas.

A patient with a history of squamous cell carcinoma of the right upper lung lobe treated 14 years before by concomitant chemo-radiotherapy was referred on account of dyspnea. Bronchial endoscopy revealed complete obstruction of the right main bronchus highly suggestive of a tumor recurrence. However, biopsy samples only showed inflammatory and necrotic tissue with no evidence of malignancy. Despite complete tissue resection by rigid bronchoscopy, a rapid and complete recurrence occurred requiring the placement of a Y-shaped bronchial prosthesis. Repeat histological, bacteriological and mycological analyses were negative. The patient was soon readmitted to hospital for a lung infection due to recurrence of obstruction inside and around the prosthesis. Bacterial examination of biopsy samples identified Actinomyces meyeri. Appropriate antibiotic therapy led to a complete regression of the bronchial obstruction. Unfortunately, the patient died a few months later due to massive hemoptysis after the removal of the prosthesis. Autopsy examination showed a fistula between the right main bronchus and pulmonary artery, with no evidence of neoplastic recurrence nor the persistence of lesions associated with actinomycosis.

Quality indicators in ovarian cancer surgery: report from the French Society of Gynecologic Oncology (Societe Francaise d'Oncologie Gynecologique, SFOG).

BACKGROUND: Based on registries, the European experience has been that <50% of patients are treated according to protocols and/or benefit from the minimum required surgery for ovarian cancer. The French Cancer Plan 2009-2013 considers the definition of qualitative indicators in ovarian cancer surgery in France. This endeavour was undertaken by the French Society of Gynaecologic Oncology (SFOG) in partnership with the French National College of Obstetricians and Gynecologists and all concerned learned societies in a multidisciplinary mindset. METHODS: The quality indicators for the initial management of patients with ovarian cancer were based on the standards of practice determined from scientific evidence or expert consensus. RESULTS: The indicators were divided into structural indicators, including material (equipment), human (number and qualification of staff), and organizational resources, process indicators, and outcome indicators. CONCLUSIONS: The enforcement of a quality assurance programme in any country would undoubtedly promote improvement in the quality of care for ovarian cancer patients and would result in a dramatic positive impact on their survival. Such a policy is not only beneficial to the patient, but is also profitable for the healthcare system.

[Comparison of three models to predict non-sentinel lymph node status in breast cancer patients with metastatic sentinel lymph nodes: a retrospective study of 77 patients]. / Comparaison de trois modèles prédictifs d'envahissement des ganglions non sentinelles en cas de métastases des ganglions sentinelles dans le cancer du sein: une étude rétrospective sur 77 patientes.

UNLABELLED: Various statistic predictive models have been developed to predict the status of the non-sentinel lymph nodes (NSLN) when the sentinel one was invaded by tumor cells in the case of breast invasive carcinoma. The objective of this study was to compare the accuracy of three of these statistic models: the MSKCC nomogram, the Tenon score and the MDA score. The study was conducted at Croix Rousse Hospital, Lyon, France. OBJECTIVE: When sentinel lymph node is invaded by metastasis, complete lymph node dissection of the axilla can be avoided using statistic predictive models especially when talking about micrometastases and moreover about isolated tumor cells in the sentinel lymph node. Over the different existing models, we tested the three most used ones to determine the model that is most adapted to our everyday practice. METHOD: The study population consisted of 77 women with an invasive breast cancer treated by complete axillary lymph node dissection for metastatic sentinel lymph node. Over the nine models already published, we focused on three of them due to their ease to use as well as their good preliminary results: the Memorial Sloan-Kettering Cancer Center nomogram (MSKCC), the Tenon score and the M.D Anderson score (MDA). Different criteria are used by these models, but all of them take in consideration: tumor size, multifocality, tumor type, lymphovascular invasion, hormonal receptors, number of sentinel lymph node resected, degree of sentinel lymph node invasion (i.e. macrometastasis, micrometastasis or isolated tumor cells) and histologic method used to diagnose sentinel lymph node invasion. These scores are validated depending on their false negative ratio as well as the proportion of patient selected as having a low risk of non-sentinel lymph node invasion. The major criterion for the MSKCC score is the calibration that compares the predicted likelihood of invasion and the histologically proved one. RESULTS: The rate of non-sentinel lymph node invasion was 37.6%. The discrimination of the three models was good with an AUC of 0.74 for both MSKCC nomogram and Tenon score and of 0.72% for MDA score. MSKCC nomogram was well calibrated (P=0.23). The false negative ratio was 0 (CI 95%: 0-18.1%) for MSKCC nomogram, 16.7% (CI 95%: 8.6%-27.8%) for Tenon score and 16% (CI 95%: 6.8-31%) for MDA score. The percentage of patients considered as having a low risk of invasion of the non-sentinel lymph node was 20.5% for MSKCC nomogram, 34.7% for MDA score and 47.4% for Tenon score. CONCLUSION: MSKCC nomogram seems to be the statistic model that is most adapted to the population we studied. To validate the Tenon and MDA score, we should have a most important population.

[Sarcoma of the pulmonary artery]. / Sarcomes de l'artére pulmonaire.

Two cases of pulmonary artery angiosarcomas are reported. Pulmonary artery tumor was diagnosed during exploratory thoracotomy in the first case. It was suspected on magnetic resonance imaging and confirmed by surgery in the second case. The therapeutic was a right pneumonectomy for the first patient and a tumor resection followed by chemotherapy and radiotherapy on cerebral metastases for the second patient. Both our patients died, 13 and 44 months after surgery. On the basis of histological and immunohistochemical findings both tumors were angiosarcomas. Ultrastructural examination in the second case revealed an endothelial differentiation. The review of 142 previously reported cases of pulmonary artery sarcomas and these ultrastructural findings suggest that pulmonary artery sarcomas may have multiple origins; leiomyosarcomas deriving from arterial smooth muscle; angiosarcomas deriving from the endothelium and undifferentiated sarcomas or "myointimal sarcomas" deriving from myointimal cells.

[Extracranial cephalic schwannomas. Apropos of 13 cases]. / Les schwannomes exocraniens de l'extrémité céphalique. A propos de 13 observations.

The authors report a series of 13 extracranial cephalic schwannomas, treated between 1981 and 1998. Schwannomas are rare and benign tumors derived from Schwann cells. Cephalic sites represent 2.5% of all schwannomas. Their diagnosis is often delayed, because symptoms mainly consist of compression disorders. There is a female predominance. The age distribution is homogeneous. There are no risk factors. There is no predominant side. The orbit and jugular foramen are the most frequent sites. The trigeminal nerve is usually involved. CT scan and magnetic resonance imaging (MRI) specify the topography and characteristics of the tumor and can contribute to the diagnosis. Macroscopically, schwannoma is a well-demarcated, ovoid or spherical, brownish tumor, off-centered from the nerve. It is composed of soft, easily fragmented tissue. The diagnosis is often obvious on microscopic examination and rarely requires immunohistochemical confirmation. The only treatment is surgical. It consists enucleation after opening the epineurium, using on operating microscope, without disruption of the nerve. Total excision ensures recovery. The authors have observed only one recurrence (first patient operated without microscope). Compression disorders regress completely. Nerve injuries have a variable prognosis. Malignant transformation is exceptional. Type II neurofibromatosis must be excluded in young patients.

Another two cases of ovarian tumours in women who had undergone multiple ovulation induction cycles.

Many questions have been raised recently about the possible association between ovulation induction and ovarian cancer. In order to contribute to the limited literature on this important issue, two cases of ovarian cancer in women who had multiple ovulation induction are presented here. In the first case the patient had ovarian induction by clomiphene citrate while she already had an ovarian cyst. The cyst enlarged under induction. During laparoscopy, the cyst was removed. Histological examination showed a borderline and invasive sero-papillary cystadenoma. In the second case, the patient underwent ovulation induction and intrauterine insemination. During the first ultrasound in the beginning of the cycle, an ovarian cyst was discovered. Laparoscopy was performed and the cyst removed. Histological examination showed a borderline and invasive mucinous cystadenoma.

Expression of urokinase-type plasminogen activator, stromelysin 1, stromelysin 3, and matrilysin genes in lung carcinomas.

We have previously shown that the extracellular-matrix-degrading enzymes, urokinase-type plasminogen activator (u-PA), stromelysin 1, stromelysin 3, and matrilysin, may play an important role in the transition from lung preneoplasia to invasive carcinoma. Using in situ hybridization and immunohistochemistry, we analyzed serial frozen sections for the expression of these enzymes in 89 lung carcinomas including 25 neuroendocrine (NE) carcinomas (10 small-cell lung carcinomas, 7 large-cell NE carcinomas, 1 atypical, and 7 typical carcinoids) and 64 non-small-cell, non-NE carcinomas (29 squamous and 7 basaloid carcinomas, 23 adenocarcinomas, and 5 large-cell carcinomas). Proteases, except matrilysin, were more often expressed in stromal than cancer cells. In non-small-cell, non-NE carcinomas, stromal co-expression of u-PA and stromelysin 3 was seen in 80 to 90% of the tumors and was highly correlated (P < 0.0001). Stromal u-PA and stromelysin 3 expression was linked to tumor size (P = 0.01 and 0.03, respectively) and lymph node involvement (P = 0.001 and 0.02, respectively). Epithelial expression of u-PA was correlated to tumor size (P = 0.04). Epithelial expression of stromelysin 3 predominated in squamous and basaloid carcinomas (P = 0.0005) and was inversely correlated to squamous differentiation (P = 0.018). Epithelial expression of matrilysin predominated in adenocarcinomas and large-cell carcinomas (P = 0.07). In NE carcinomas including small-cell lung carcinomas, stromal expression of u-PA was correlated to lymph node metastasis (P = 0.017). Epithelial expression of all enzymes were significantly less frequent in NE than in non-NE tumors. We conclude that 1) epithelial expression of matrix proteases in lung cancer is linked to cell phenotype (NE versus non-NE, squamous versus glandular) and 2) their stromal, rather than epithelial, expression influences local metastasis.

Expression of c-ets-1, collagenase 1, and urokinase-type plasminogen activator genes in lung carcinomas.

The c-ets-1 transcription factor has been involved in the in vitro transactivation of matrix-degrading protease genes that might play an important role in tumor invasion. Using in situ hybridization, we analyzed serial frozen sections for c-ets-1, collagenase 1, and urokinase-type plasminogen activator gene expression in 54 lung carcinomas including 34 non-neuroendocrine carcinomas (18 squamous carcinomas, 10 adenocarcinomas, 3 large cell carcinomas, and 3 basaloids) and 20 neuroendocrine carcinomas (7 small cell lung carcinomas, 4 large cell neuroendocrine carcinomas, 4 well differentiated neuroendocrine carcinomas, and 5 carcinoids). c-ets-1 gene was expressed in stromal cells in 44/54 lung carcinomas including one metastasizing carcinoid. c-ets-1 transcripts were also detected in cancer cells more frequently in neuroendocrine than in non-neuroendocrine carcinomas (P = 0.0059) and in stages III and IV and metastasis more frequently than in stages I and II ( P = 0.0065). Collagenase 1 gene was expressed in 16/34 non-neuroendocrine tumors and in 1/20 neuroendocrine tumors, either in stromal (12/17) or in cancer cells (6/17). Urokinase-type plasminogen activator mRNAs were expressed in 45/54 lung carcinomas in stromal and/or cancer cells. In non-neuroendocrine tumors, c-ets-1 and collagenase 1 gene expressions in stromal cells were correlated. These results demonstrate that the transcription factor c-ets-1, collagenase 1, and urokinase-type plasminogen activator are involved in lung cancer invasion and suggest that c-ets-1 protein might transactivate collagenase 1 gene during tumor invasion.