[Endometrial carcinoma: An histopathological and histoprognotic study about 62 patients in a center in the Tunisian north]. / Les carcinomes de l'endomètre : étude anatomopathologique et histopronostique à propos de 62 cas dans un centre du Nord tunisien.

Endometrial cancer is the most prevalent genital tract cancer in occident and the third most common cancer among women in Tunisia. It is dominated by carcinoma. The identification of prognostic factors allows a better understanding of its outcome and guides its therapeutic approach. We propose to describe the clinicopathological features and identify the histoprognostic factors of this cancer. It is a retrospective analysis of a series of 62 total hysterectomy specimens with bilateral salpingo-oophorectomy from women with primary carcinoma of the endometrium, colligated in Anatomy Laboratory and Pathology Salah Azaiz Institute of Tunis over a period of 5 years, from January 2003 to December 2007. The median age was 60 years. At the time of diagnosis, 25% of patients were nulliparous and 86% were menopaused. The endometrioid adenocarcinoma was the most common, accounting for 84% of cases (5% of them were grade 3). A myometrial invasion superior or equal to 50% was observed in 40% of cases. 42% of cases were classified as stage IA, 14% in stage IB, 16% in stage II, 18% stage III and 10% in stage IV. 22% of patients had nodal involvement. Overall survival at 5 years was 81%. In multivariate analysis, stage IV, nodal involvement and brachytherapy have influenced this rate. Event-free survival at 5 years was 71%. It was directly related to stage and nodal involvement. Stage, histological type, tumor grade, invasion of more than half of the myometrium and lymph node involvement were the most important adverse prognostic factors, dictating an appropriate management of these tumors.

[Care pathways before diagnosis in children and adolescents with malignancies]. / Parcours prédiagnostique des enfants et adolescents atteints de tumeurs solides.

OBJECTIVE: to describe medical care pathways between first symptoms and first oncologic consultation in children and adolescents with solid cancers in order to analyze a possible relationship between delayed diagnosis and its potential consequences. METHODS: Retrospective study on patients aged less than 25 years at first consultation in the oncology department of pediatric, adolescent and young adult in Institut Curie during one year. Were collected data on cancer characteristics, components of care pathways, and sociodemographic parents' characteristics. RESULTS: Hundred and six patients were selected, with median age of 6 years. Most represented tumor was low-grade cerebral tumor (17.0%). Pain was the most frequent type of disorder observed as first sign (34.3% of patients). First signs were unspecific in only 27.6% of cases. Most patients were first seen by a general practitioner (29.3%). Median total time to diagnosis was one month [ranges: 0-64]. Median number of consultations before referral to oncology expert was 2 [0-7]. Retrospective analysis found a possible delayed diagnosis in 44.3% of patients, with potential vital and functional risks estimated respectively at 14.1 and 20.7% of overall population. Time to diagnosis was shorter if father was of foreign nationality vs. French (34 days vs. 72 days, P<0.05), and longer if parents were separated (74.5 days vs. 42.5 days, P<0.03). CONCLUSIONS: Overall time to diagnosis is quite fast, even if first signs of pediatric cancers are very polymorphic. Some medical and sociodemographic factors could influence characteristics of care pathways.

Epidemiological and pathological characteristics of cardiac tumors: a clinical study of 242 cases.

The purpose of this study was to review the current clinical experience and pathological characteristics of cardiac tumors. We retrospectively reviewed 33,108 consecutive cases of cardiac operations performed at our institution from October 1996 to March 2005. There were 242 cases confirmed histologically as cardiac tumors. Among them, 234 patients were diagnosed with primary heart tumors, revealing a prevalence of 0.71% among the corresponding period cardiac operations. Of primary cardiac tumors, the incidence of benign neoplasm was much higher than malignant masses (90.6% vs. 9.4%, P<0.01). The most common benign cardiac tumor was myxoma (86.8%). Mesenchymoma and angiosarcoma were the most common primary malignant cardiac tumors. The prevalence rates of cardiac tumors were quite different among age groups. Rhabdomyoma and fibroma are prone to children. The number of female patients with myxoma was higher than that in male (P<0.01). Myxomas have a special predilection for the left atrium (93.5%). Benign non-myxoma tumors are more likely to occur in the ventricular (64.3%). The primary malignant tumors have a tendency to be of multi-center origination (23%). All the secondary cardiac tumors were located in the right side of the heart. This study, using a relatively large sample, reveals the clinical incidences and pathological characteristics of various cardiac tumors in the Chinese population.

[Malignant solid tumors in neonates: a study of 71 cases]. / Tumeurs solides malignes néonatales: à propos de 71 cas.

UNLABELLED: Malignant neonatal tumors are rare and comprise 2% of childhood malignancies. Clinical features, histologic types, prognosis were very different from those seen in older children, facing oncologists with diagnostic, therapeutic and ethical problems. PATIENTS AND METHODS: In a retrospective study from January 1987 to January 2004, we reviewed the management of neonates treated at the Institute Gustave Roussy for a malignant solid tumor for whom symptoms started in the first month of life. RESULTS: Seventy-one neonates were treated, comprising 1,2% of the overall patients treated during the same period of time. Of these 71 patients, 42 (59%) presented with neuroblastomas, 12 (17%) with mesenchymal tumors, 6(8%) with cerebral tumors and 11 with various other types of tumors. Fifty-nine patients underwent surgical resection. Thirty-eight neonates received chemotherapy, administered at a 30 to 50% reduced dose. Hematologic toxicities and infections were the main therapeutic complications. Very small doses of radiotherapy were used in only 5 children. There has been no therapy-related mortality. Twenty-two of the 57 survivors have sequelae, especially patients with intraspinal neuroblastoma. The 5 year overall survival was 79%. CONCLUSIONS: Neonatal malignant solid tumors, except for cerebral tumors, have a good prognosis. The young age of patients resulted in problems of treatment tolerance. The therapeutic regimen should take into account the risk of acute iatrogenic toxicity and long term sequelae. Surgery remains the treatment of choice but chemotherapy, with dose reduction, managed by expert teams, is essential and safer in a lot of case.

Trends in incidence and survival of mesenchymal neoplasm of the digestive tract within a defined population of northern Norway.

Population-based incidence and survival data for gastrointestinal stromal tumor (GIST) are sparse due to the fact that GIST is a rather novel entity both clinically and pathologically, and has not been registered as a separate entity in population-based cancer registries. The aim of the present study was to reclassify all mesenchymal tumors within a defined population of northern Norway over a time-span of 30 years with the purpose of estimating trends of incidence and survival. One hundred and forty-one patients with mesenchymal neoplasms of the digestive tract were identified: 102 as GISTs, 32 as leiomyomatous tumors, 4 as schwannomas, and 3 as fibromas. Incidence rates of GIST showed a significant increase over the whole period, which was not observed for the non-GIST cases. Analysis of GIST cases showed that cases with more than 5 mitoses per 50 high power fields had an increased expected mortality 4 times that of those with fewer mitoses, and the combination of mitotic count and size of tumor can be recommended for categorizing the tumors into different risk levels. The study confirms that GIST is by far the most frequent mesenchymal neoplasm of the digestive tract and that the incidence has increased over the last 30 years.

[Rare malignant tumors of the thyroid]. / I tumori maligni rari della tiroide.

The incidence of rare malignant tumors of the thyroid is about 4 cases/100,000 people and represent only 1.8% of all the thyroid cancers. When we talk about "rare" tumors, obviously, we do not refer to the most frequent cancers (papillary, follicular), or less frequent tumors (medullary, anaplastic), but to some types of thyroid tumors that have been almost always sporadically observed. Mucoepidermoid carcinoma and squamous carcinoma have been described in the literature. They present occasional papillary formation so that, according to some authors, could be considered as variants of the papillary carcinoma. Teratoma is another rare tumor which in the paediatric age is benign, but its prognosis could be unfavourable because it causes an important respiratory distress, while in the adult it presents a very aggressive clinical course like the anaplastic carcinoma. Lymphoma is the most frequent of the "rare" tumors of the thyroid (1-5% of all the thyroid cancers). It arises often in a setting of a long history of goitre and Hashimoto thyroiditis. Fine-needle aspirate is important to make diagnosis and to start a correct treatment which allows a 5-year survival up to 85% in the favourable cases.

Gastrointestinal stromal tumours of the oesophagus.

A rare case of gastrointestinal stromal tumour (GIST) of the oesophagus is presented. Pathological features with a review of the treatment options and the literature are presented.

Comparison of occurrence of oro-maxillo-facial tumor types in different regions of the People's Republic of China.

The frequencies of oro-maxillo-facial tumors, by type, seen at the Guangxi Medical College Hospital were surveyed in order to compare different regions of the People's Republic of China. Computer analysis was performed on data for all oro-maxillo-facial tumors, as confirmed by the hospital between 1957 and 1987, inclusive. The results were then used in a comparative study with those of five other medical colleges. Among a total of 4,052 cases, there were 1,593 benign tumors, 2,049 malignant tumors and 410 unknown tumors. Tumors derived from the epithelium were the most common type (42.0% of the total), whereas in the other five medical colleges the mean corresponding proportion was 27.8%, the difference being significant (p < 0.05). Epithelial tumors were also the most common type of malignant tumor (70.5% of the total), whereas the corresponding proportion reported by the other five institutions was 60.6%, which was also significantly different (p < 0.05). The proportion of malignant tumors among the total was more than 50.6%, which was higher than that reported by the other five colleges. This study shows that epithelium-derived tumors and malignant tumors were encountered more frequently at Guangxi Medical College than at the other five medical colleges in China.

Multiple primary neoplasms at a single institution: differences between synchronous and metachronous neoplasms.

During the 10-year period (1987-1996) of our study, 26,255 patients with cancer were admitted to our clinic and, of these, 271 (1%) patients had multiple primary malignant tumors. Ninety-two (34%) patients had synchronous tumors (synchronous group), and 179 (66%) patients had metachronous tumors (metachronous group). The mean age at first diagnosis was higher in the former group. The ratio of men to women was 1.36 in the synchronous group and 0.74 in the metachronous group (p = 0.018). Smokers and drinkers were more common in the synchronous group. Breast cancer and lung cancer were most prevalent, and associations between head/neck and lung cancer and between breast and breast cancer were the most frequent associations in both the synchronous and the metachronous group. The frequency of aerodigestive tumors was higher and that of mesenchymal tumors was lower in the synchronous group than in the metachronous group. Localization in the medial region and in the head/neck was more frequent in the synchronous group than in the case of metachronous secondary tumors.

Clinical and histopathological features of colonic stromal tumor in a child.

Neoplasms of the colonic submucosa are rare in children. Gastrointestinal stromal tumors (GISTs) are undifferentiated tumors, usually diagnosed by immunohistochemistry. We report a 4-year-old girl with a submucosal GIST of the ascending colon, which was detected by computed tomography. Diagnosis after ileocecal resection was established by histology. In addition, sections were examined immunohistochemically, using antibodies against vimentin, desmin, alpha-smooth muscle actin, S100, neuron-specific enolase, c-kit, and CD34. Hematoxylin and eosin-stained sections showed interlacing fascicles with occasional palisades of epithelioid and spindle cells. The tumor cells were positive for vimentin and CD34. To our knowledge, this is the first reported case of colonic stromal tumor in a child.

Primary malignant mesenchymoma of bone: case report, literature review, and distinction of this entity from mesenchymal and dedifferentiated chondrosarcoma.

The seventh case of primary malignant mesenchymoma of bone is reported. It is compared to the other six cases in the literature and clinicopathologic pattern of this entity is developed. Primary malignant mesenchymoma of bone is compared to mesenchymal chondrosarcoma and dedifferentiated chondrosarcoma. Differences as well as similarities are discussed and a more current classification scheme for malignant mesenchymoma of bone is proposed.

Fibrocartilagenous mesenchymoma of bone.

We studied 12 cases of a rare primary bone neoplasm--fibrocartilaginous mesenchymoma--including five from the original report on this condition. The seven male and five female patients were 9 to 25 years old. The metaphyses of the long bones were the most common site, with the fibula accounting for a third of all cases. Histologically, the lesion contained spindle cells, bone trabeculae, and islands of cartilage. At least some of the cartilage was in the form of plates that resembled epiphyseal plates. Intralesional excision led to a high rate of recurrence, but no metastasis or death was related to the tumor. Our results indicate that the entity described by Dahlin and co-authors is histologically distinct and that its behavior does not merit the term malignant.

Hepatic mesenchymal sarcoma: MRI findings.

Hepatic undifferentiated mesenchymal sarcoma is a rare pediatric malignant neoplasm. We present three children, aged 7, 8, and 12 years, with this tumor. Clinical presentation was abdominal pain, palpable mass, asthenia, anorexia, and weight loss. One had jaundice. All three lesions were detected on ultrasound (US), computed tomography (CT), and magnetic resonance imaging (MRI). MRI localized the lesions more accurately than the other methods, with good resectability correlation. On MRI, these tumors were markedly hyperintense on long TR/TE spin-echo (SE) and short-time inversion recovery (STIR) sequences. This was due to the cystic areas with myxoid material and necrosis. The internal separations were hypointense on these sequences. On short TR/TE SE sequences the lesions presented a fibrous pseudocapsule (two cases), and internal hyperintense areas representing hemorrhage (two cases). MRI also detected vascular invasion (one case), biliary obstruction (one case), and hilar adenopathies (one case). The combination of hemorrhage (hyperintense on short TR/TE SE) and cystic or myxoid components (markedly hyperintense on long TR/TE SE and STIR sequences) is common in this tumor.

Undifferentiated (embryonal) sarcoma of the liver. Clinical and pathologic study of 16 cases with emphasis on immunohistochemical features.

Undifferentiated (embryonal) sarcoma of the liver is a primitive mesenchymal neoplasm with predilection for individuals in the first 2 decades of life. In this study (10 boys, 6 girls), children in the age range of 6-10 years were most commonly affected (63%). Clinical features most frequently noted on presentation were abdominal pain or a palpable mass. In two cases there was cardiac involvement caused by invasion of the inferior vena cava with extension into the right atrium and ventricle; both children died of progressive dyspnea from tumor embolization to the lungs. One patient was a member of a kindred with the cancer family syndrome (Li-Fraumeni syndrome). There were 13 tumor-related deaths (86% mortality); on child was alive with recurrent tumor in the upper abdomen. Complete surgical resection was attempted in 10 of 15 children who underwent exploratory laparotomy; 2 were alive and well 1 and 5 years later, whereas 1 patient had a recurrence in the upper abdomen 3 years after diagnosis. Ultrastructural study (five cases) and immunohistochemistry (11 cases) supported a mesenchymal origin for the tumor, but failed to identify any diagnostic immunophenotype or specific line of differentiation. Coexpression of vimentin and cytokeratin was seen in three cases. Prompt detection of this aggressive tumor with complete surgical resection is the key to a successful outcome, but this is very difficult to achieve. Recent experience suggests that aggressive adjuvant chemotherapy may improve survival in some cases.

Second primaries in children with central nervous system tumors.

Among 670 children diagnosed with central nervous system tumors before age 20 and recorded in the Connecticut Tumor Registry, nine had a second neoplasm as well. From known age-specific and year-specific cancer incidence figures for Connecticut, the expected number of second neoplasms for the series of 670 is 0.99; therefore the relative risk is 9.1 (95% confidence limits: 4.0, 17.3). Three patients developed two central nervous system tumors, while 0.16 were expected, giving a relative risk of 19 (95% confidence limits: 3.8, 55). Six patients developed two neoplasms in childhood, versus 0.66 expected; the relative risk of this event is 9.1 (95% confidence limits: 3.3, 20). In four cases of lapse in time between the diagnosis of the first and second tumor, the first tumor had been treated with radiation. Five cancers occurred in parents or siblings of these nine patients, versus 0.91 expected; the relative risk is 5.5 (95% confidence limits: 1.2, 10.0). Three of these relatives had leukemia, while only 0.04 cases were expected (relative risk = 75). We conclude that not only is a child with CNS cancer at increased risk for other cancers, but such a child with two cancers is often part of a familial cluster with increased risk of cancer.