Melanomes malins primitifs anorectaux

Le melanome malin primitif anorectal represente 0;5 a 1 de toutes les tumeurs de cette region. C'est une pathologie agressive avec un pronostic souvent reserve. La rarete de cette localisation de melanome malin nous a pousses a en faire le point a partir des observations marocaines

Endometrial stromal sarcoma: prognostic factors and impact of adjuvant therapy in early stages.

BACKGROUND AND OBJECTIVES: Endometrial stromal sarcomas (ESS) are rare uterine neoplasms. surgery remains the cornerstone of treatment for early stages and consists of an abdominal hysterectomy with bilateral salpingo-oopherectomy. Despite appropriate surgical treatment, relapse rates are high (18% to 45%) and the value of adjuvant therapies is not clear. We evaluated prognostic factors and the impact of adjuvant treatment on localized ESS (stages I and II). DESIGN AND SETTING: Retrospective, case-control study conducted at the National Institute Of Oncology in Rabat, Morocco over 10 years from 2000 to 2009. PATIENTS AND METHODS: twenty-one cases of localized ESS were included in the analysis. RESULTS: standard surgery was performed in 71.4% of our patients. Myometrial invasion was noted in 57.1% of cases. Mitotic activity was considered high in five patients. Adjuvant treatment was given to 52.3% of patients: endocrine therapy in five patients and radiotherapy in six. Survival was significantly longer in the group of patients who underwent standard surgical treatment (P=.0007), in the absence of deep myometrial invasion (P=.0248) in cases with a low mitotic index (P<.0001) and in patients who received adjuvant therapy (hormone or radiotherapy) (P=.0048). In a multivariate analysis independent risk factors for monitoring were inadequate surgical treatment and absence of adjuvant treatment. CONCLUSIONS: Myometrial invasion and mitotic index appear to be important prognostic factors. the reference surgery is hysterectomy with bilateral salpingo-oopherectomy. Lymph node dissection does not appear to provide a benefit. finally adjuvant treatment may carry a significant survival benefit.

[Cardiac safety of trastuzumab in adjuvant: a review across 53 observations]. / Tolérance cardiaque du trastuzumab en adjuvant: revue à travers 53 observations.

Trastuzumab has been a revolution in the treatment of breast cancer overexpressing HER. Its use as an adjuvant for a period of 1 year is currently an international standard. Its major toxicity is cardiac, where the systematic monitoring of the LVEF before and during treatment. To evaluate the cardiac safety for our patients, we conducted this retrospective case-control study. The average in LVEF before the start of trastuzumab was 62.5% (51-80), and at the end of treatment 60.55 (40-77), a decrease in absolute value by 2%. This difference is statistically significant with P<0.001. Eighty-three percent of our patients have completed treatment, of whom 26.4% with a provisional arrest because of a regressive fall in LVEF. A final arrest has been made in 17% cases due to either a nonregressive reduction in LVEF or the appearance of symptomatic heart failure found in two patients. Analysis of risk factors toxicity found in this group of patients with a cardiotoxicity persisting an average age and average number of treatments received anthracyclines higher than the rest of our sample, and diminished baseline LVEF. But all these differences were not statistically significants. During the period of monitoring of these patients, six (67%) had spontaneous recovery of their LFEV 5 months ± 2.01 after discontinuation of trastuzumab. For two cases of symptomatic heart failure, they had a clinical improvement under medical treatment in February but is still less than 40%. The cardiac safety in our study seems comparable with the literature data but located in the upper range of levels of toxicity. The lack of statistical power of our study does not exclude a greater cardiac toxicity of trastuzumab among Moroccan women and should prompt a more cautious use of this drug and the achievement of larger studies that could answer this question.

[Treatment and prevention of acute radiation dermatitis]. / Traitement et prévention des radiodermites aiguës.

Acute radiation dermatitis is a common side-effect of radiotherapy which often necessitates interruption of the therapy. Currently, there is no general consensus about its prevention or about the treatment of choice. The goal of this work was to focus on optimal methods to prevent and manage acute skin reactions related to radiation therapy and to determine if there are specific topical or oral agents for the prevention of this acute skin reaction. The prevention and the early treatment are the two focus points of the management of the acute radiation dermatitis.

Identification of G2607A mutation in EGFR gene with a significative rate in Moroccan patients with nasopharyngeal carcinoma.

The epidermal growth factor receptor (EGFR) is involved in the regulation of several cellular processes and in the development of many human cancers. Somatic mutations of EGFR at tyrosine kinase domain have been associated with clinical response to tyrosine kinase inhibitors (TKIs) in lung cancer patients. In this study, we evaluated the frequency of point mutations in EGFR for future use of TKI in clinical treatment of nasopharyngeal carcinoma (NPC). Sixty Moroccan patient specimens of NPC were analysed for EGFR mutations in the region delimiting exons 18 and 21 by direct sequencing. Our results showed the absence of mutations in the EGFR kinase domain in these exons in all 60 analysed specimens. Sequence analysis of the EGFR—TK domain, revealed the presence of (G2607A) polymorphism at exon 20. The genotypes AA and GA were found respectively in 39 (65%) and 16 (26.6%) cases. Statistical analysis showed no difference between the polymorphism and either gender or age of patients. Mutations in EGFR kinase domain are rare events in NPC biopsies, suggesting, that treatment of NPC patients with TKI may not be effective. However, EGFR G2607A polymorphism at exon 20 is frequent in NPC cases and could be associated to clinical response to TKI therapy.

[Management of phyllodes tumors of the breast at the National institute of oncology of Rabat, Morocco]. / Prise en charge des tumeurs phyllodes du sein à l'Institut national d'oncologie de Rabat, Maroc.

OBJECTIVES: Phyllodes tumors of the breast (PTB) are rare fibro epithelial tumors. Their terminology, histological classification and their treatment are exposed to controversy. The aim of our work is to underline the epidemiological, clinical, histological, therapeutical, prognostic and evolutive features of these tumours through a retrospective study and a review of the literature. PATIENTS AND METHODS: We reviewed at the National Institute of Oncology of Rabat, Morocco, between 1985 and 1998, all the files of patients with histological certainty of PTB, doubtful PTB were excluded. We collected epidemiological, clinical, histological, therapeutical, prognostic and evolutive features of these tumours. STATISTICS: quantitative parameters were represented by mean +/- S.D. and qualitative parameters by percentage or effective. RESULTS: We studied nine cases of PTB, which represented 0.09% of all primitive tumors of the breast treated at our institution during the study period. All our patients were female. Mean age was 37.3 +/- 10.07 years. Two of our patients (22.2%) had a history of fibroadenoma and 44.4% were nulliparous. Mean delay before consulting was 60.7 +/- 17.56 months and the median tumour size was 13 +/- 7.47 cm. Pathological findings were six benign or borderline phyllodes tumours (66.7%) and three cystosarcomas phyllodes (33.3%). The treatment consisted in simple mastectomy in seven cases (77.8%) and tumorectomy in two cases (22.2%). All the surgical margins were clear. Two of the three cystosarcomas phyllodes received adjuvant external bean radiation therapy 50 Gy on the thoracic chest wall. After a median outcome of 3 +/- 2 years ranging from 3 to 74 months, we did not note any relapse or metastasis. DISCUSSION AND CONCLUSION: In our series, PTB happened exclusively in females. History of fibroadenoma within 22.2% of the patients suggests the hypothesis of a filiation between these two entities. Their distinctive features were young age in diagnosis, long delay before consulting, important tumor size, predominant benign and borderline histological types, treatment mainly surgical and good local and distant control.

[Angiocentric lymphoma of the face: report of the 2 cases]. / Lymphome angiocentrique de la face : à propos de 2 cas.

Angiocentric lymphoma of the face is a rare malignancy. During 11 years, only two patients were treated for a lymphoma of the face. The first patient, 31-year-old, had an angiocentric lymphoma of the nasal cavity, the second patient, 40-year-old, had an angiocentric lymphoma of the nasopharynx and the maxillary sinuses, with an extension the hard palate. According to the Ann Arbor classification, the disease was stage IEab and stage IIEbb, respectively. The first patient was treated by chemotherapy alone (COP regimen) and the second patient underwent combined chemotherapy (CHOP) and radiotherapy. The follow-up was marked by progression of the disease 7 and 9 months after the end of the treatment in the two cases. For the angiocentric lymphoma, treatment based on radiotherapy or chemotherapy alone or combined does not often provide local control and the overall survival rate does not exceed 40% at 5 years.

Cerebellous metastases in patients with uterine cervical cancer. Two cases reports and review of the literature.

Brain metastases from cervical cancer are extremely rare. We report on two patients who developed cerebellous metastases following uterine cervical cancer. The interval between diagnosis of the primary cancer and diagnosis of brain metastasis was 8 months. The main complaint was symptoms of increased intracranial pressure and cerebellous syndrome. Surgical excision of the brain lesion followed by radiation therapy was performed in the first case. The second patient received palliative radiation therapy. The first patient died 8 months after diagnosis. The second patient is alive 2 months after diagnosis.

[Adenoid cystic carcinoma of the nasopharynx: a case report]. / Carcinome adénoïde kystique du nasopharynx: à propos d'un cas.

Nasopharyngeal adenoid cystic carcinoma is a rare tumor, few cases have been reported in the literature. The aim of this report is to present a new case of an adenoid cystic carcinoma of the nasopharynx. We report the case of a 50 years-old woman presenting with an adenoid cystic carcinoma of the nasopharynx with diffuse pulmonary metastasis. The treatment combined chemotherapy, based on cisplatin and adriamycin drugs, and palliative radiation therapy at the dose of 30 Gy. The patient died 6 months after diagnosis establishment. The best treatment of adenoid cystic carcinoma is complete surgical resection but extended resection can be difficult in some locations as nasopharynx. The adjuvant radiation therapy seems to improve local control.

[Muscle metastasis of squamous cell carcinoma of the uterine cervix]. / Métastase musculaire d'un carcinome épidermoïde du col utérin.

Muscular metatasis are rare, their number is probably underestimate. The authors report a case of a patient treated, in 1993, for a squamous cell carcinoma of the uterine cervix. Five years later, the patient developed a metatasis to muscular psoas. She underwent combined surgical excision and adjuvant radiation therapy. The patient is alive and well 30 months after diagnosis of the metastasis.

[Primitive intracranial adenoid cystic carcinoma. A case report]. / Le carcinome adénoïde kystique primitif intracrânien. A propos d'une case.

We report the case of a 50-year-old woman presenting a primitive adenoid cystic carcinoma of the middle part of skull base diagnosed by transphenoidal biopsy. She was treated by exclusive radiation therapy with cobalt 60 1.25 MeV and the dose received was 66 Gy in 33 fractions. Our patient remains alive 50 months after the completion of treatment. Adenoid cystic carcinoma is a slow growing malignant tumor with perineural invasion which arises more frequently from salivary glands. The occurrence of primary intracranial adenoid cystic carcinoma is exceptional. The best treatment is radical surgery followed by radiotherapy. Chemotherapy is rarely used. Prognosis is worse than for salivary glands.

Male breast cancer. A report of 71 cases.

Male breast cancer is rare; it constitutes 0.2-1.5% of all malignant tumours in men and 1% of all breast cancers. The goal of this retrospective study is to analyse the epidemiologic, clinic, therapeutic and evolutive profiles of this disease in 71 cases collected at the National Institute of Oncology in Rabat, Morocco, between the years 1985 and 1998. The median age was 60 years. No significant risk factor was found. The average consultation's delay was 35 months. The main clinical complaint was a mass beneath the areola in 86% of the cases, associated with ulceration in 18% of the cases. For that, the disease was diagnosed at an advanced stage. Infiltrating ductal carcinoma was the most frequent pathologic type (91.5% of cases). Management consisted especially of radical mastectomy, followed by adjuvant radiotherapy and hormonal therapy with or without chemotherapy. It was possible to follow 58 of the patients. The median of follow-up was 30 months. The evolution has been characterized by local recurrence, after a median delay of 36 months, in five cases (8.5% of all patients). Metastasis occurred, after a median delay of 12 months, in 14 cases (24% of all patients). The site of metastasis was the bone in six cases, lung in five cases, liver in one case, liver and skin in one case and pleura and skin in one case. There were three cases of death. In one case, death was related to lung metastases. In the two remaining cases, death was due to comorbid disease. Progression was observed in one case.

[Primary adenocarcinoma of the male urethra. A case report]. / L'adénocarcinome primitif de l'urètre masculin. A propos d'un cas.

Primary adenocarcinoma of the male urethra is very uncommon, accounts for 5% of primary urethral cancers. All types of urethral carcinomas account for less than 1% of urinary malignancies in man. The prognosis remains poor despite the wide surgical treatment. The place of chemotherapy combined with radiotherapy must be defined by other studies. We report a case of primary locally advanced adenocarcinoma arising in the bulbo-membranous urethra. The patient was 29 years old, without any previous history of venerian disease. He was treated by combined external beam radiotherapy (total dose 67Gy) and chemotherapy (Cisplatinum). A marked reduction of tumor volume has been noted but the patient died because of the appearance of bone metastasis which failed to the systemic therapy.

[Primary squamous cell carcinoma of the breast. Report of three cases]. / Carcinome épidermoïde primitif du sein. A propos de 3 cas.

Primary squamous cell carcinoma of the breast is a rare neoplasma included in metaplastic breast cancer. The histogenesis remains unknown. Clinical and radiological appearances are not specific. Nodal involvement is rare and hormones receptors are negative. The treatment is based on surgery associated to radiation therapy and chemotherapy. Prognosis seems to be similar to others breast carcinoma. We report three cases of primary squamous cell carcinoma of the breast recruited at National Institute of Oncology with review of the literature.

[Sarcoma botryoïde of the uterine cervix: report of a case]. / Rhabdomyosarcome botryoïde du col utérin : à propos d'un cas.

Botryoïde sarcoma of the uterine cervix is a very rare tumor of the young woman or during genial activity. Less than 100 cases have been reported in the literature. The treatment ranged from radical surgery to conservative surgery followed by chemotherapy. We report a case of cervical botryoide rhabdomyosarcoma occurred in a 33 year-old woman treated primary by chemotherapy followed by surgery and adjuvant chemotherapy. The patient died by extensive metastatic retroperitoneal nodes and lung.

[Intracranial Hodgkin disease: case report]. / Localisation intracrânienne de la maladie de Hodgkin: à propos d'un cas.

Hodgkin disease intracranial lesions are uncommon. We report the case of a 24-year-old man who presented with an intracranial relapse without other evidence of disease 24 months after multiple drug therapy and extended field radiation therapy for stage III Bb type 3 Hodgkin disease. The patient was treated by whole brain irradiation (40 Gy/20 fractions/4 weeks). Six months after completion of therapy, the patient had a second relapse, with bilateral inguinal metastatic nodes, which were treated by a second line polychemotherapy. The patient was free of disease 9 months later.

[Non-Hodgkin's lymphoma of the uterus: apropos of 4 cases and review of the literature]. / Lymphome non hodgkinien de l'utérus. A propos de quatre cas et revue de la littérature.

The primary non hodgkin's lymphoma of the uterus is rare. This rarity explains of one part certain difficulties of the histological diagnosis and on the other hand the absence of a therapeutic strategy clearly established. We report 4 cases of primary non-hodgkin lymphoma of the uterus. Two patients had a cervical location, the two other had corpus location. The average age of our patients is of 59 years (extremes: 54-68). Histological diagnosis was confirmed by biopsy for the cervical location. For the corpus location, it is study of the uterus after hysterectomy which retained the diagnosis of lymphoma. The type of the lymphoma was low grade in two cases and high grade in the two other cases. The disease was limited to the pelvis for all our patients (stage IE according to Ann-Arbor's classification). The treatment consisted of an association of chemotherapy and radiotherapy in both cases of lymphoma of the cervix and in a radical hysterectomy followed by chemotherapy for the two cases of lymphoma of the corpus. Our patients are regularly followed, with an average follow-up of 56 months. Two patients are in disease free, the third patient presented a dissemination of the disease and the fourth patient presented a squamous cell carcinoma of the lung.

Antibody responses to recombinant Epstein-Barr virus antigens in nasopharyngeal carcinoma patients: complementary test of ZEBRA protein and early antigens p54 and p138.

Serological tests based on the antibodies directed against the Epstein-Barr virus early antigen (EA) and viral capsid antigen (VCA), which have been recognized as tumor markers for nasopharyngeal carcinoma (NPC), are routinely used to help in the diagnosis of this malignancy. The detection of these antibodies reveals very low titers, found only in a small proportion of young compared with older NPC patients. This is a problem for the diagnosis of NPC, especially among Maghrebians, among whom young people are also affected, and emphasizes the necessity to search for more reliable markers. The present study reports results of immunoglobulin G (IgG) and IgA responses of NPC patients to recombinant EA antigens p54 (BMRF1) and p138 (BALF2), VCA complex antigens p18 (BFRF3) and p23 (BLRF2), and EBNA antigen p72 (BKRF1). Our results show that IgA-EA-p54 and -p138 (IgA-EA-p54+138) antibodies have a diagnostic value for detection of NPC (70%), compared with IgA-VCA-p18+23 and IgA-EBNA-p72, which have limited diagnostic value, especially in young patients. It is also noteworthy that IgA-EA-p54+138 can detect a high percentage (64%) of NPC cases negative by immunofluorescence. These results, however, clearly show that a single test cannot achieve the objective of detecting all NPC patients, and it seems advisable to combine different tests for the diagnosis of NPC. The combination of IgG-ZEBRA with IgA-EA-p54+138 improved the sensitivity of detection of NPC to 95% in the overall NPC population. The use of IgA-EA-p54+138 in combination with IgG-ZEBRA will facilitate detailed studies on the pattern of antibody response, which may result in the development of useful serological markers to guide the treatment of NPC.