Elastofibroma dorsi: case report and review of the literature.

Elastofibroma dorsi (ED) is an uncommon benign soft tissue tumor with an uncertain pathogenesis. It mostly occurs in the infrascapular region of elderly people with a female predominance. Typically bilateral, ED can also be unilateral. While many patients remain asymptomatic, ED can be responsible of a periscapular arch source of ache. The diagnosis of ED is set on magnetic resonance imaging, and the pathological study ensures the diagnosis after surgical excision and establishes the differential diagnosis with malignant neoplasic process. The prognosis is excellent with extremely rare recurrence cases. Herein we report a case of a 54-years-old woman with a bilateral painful ED. The diagnosis was based on clinical and MRI findings that revealed bilateral tumors. Surgery was decided due to the symptomatic nature of the tumors. Pathological study confirmed the diagnosis. The post operative course was uncomplicated. We update through a review of the literature aspects of the diagnostic and therapeutic care of Elastofibroma dorsi.

Surgical management for squamous cell carcinoma of vulva.

To analyze our surgical management and the result of squamous cell carcinoma (SCC) of vulva. Retrospectively, we collected 38 cases of SCC; 17 cases of them were early SCC and 21 cases were locally advanced. The patients underwent primary surgery. The survival was estimated using Kaplan-Meier analysis and the log rank test. The mean age was 60.78 years. Total vulvectomy was performed in all patients. Superficial and deep incision of bilateral inguinal lymphadenectomy was performed by separates incisions for SCC infiltrating more than 1mm. The average tumor size was 53 mm (10 to 140mm). Morbidity was 42.1%. Lateral resection margin ≥8mm was obtained in 57.1%. Eighteen patients benefited from adjuvant radiotherapy. The follow-up median was 19.4 months (6 to 61.5 month) with 05 recurrences in 12 months. The survival using the Kaplan-Meyer analysis at 5 years, was 62.1% (71.2%N(-) vs 46.7%N(+); p = 0.13). We identified two groups for locally advanced vulva cancer. Primary surgery keeps its place. Neo adjuvant radio chemotherapy followed by surgery is the alternative treatment for locally extensive lesions.

Risk factors of poor functional results at 1-year after pseudocontinent perineal colostomy for ultralow rectal adenocarcinoma.

BACKGROUND: Pseudocontinent perineal colostomy is one of the techniques that helps recover the body image of patients undergoing abdominoperineal resection. This technique is rarely used internationally given its unknown functional results. OBJECTIVE: The study aimed to evaluate 1-year functional outcomes of perineal pseudocontinent colostomy and to determine the risk factors for "poor" functional results. DESIGN: This study is a retrospective interventional case series. SETTINGS: This study was conducted at a tertiary care university hospital and oncological center in Morocco. PATIENTS: From January 1993 to December 2007, 149 patients underwent pseudocontinent perineal colostomy after abdominoperineal resection for low rectal adenocarcinoma. INTERVENTION: Pseudocontinent perineal colostomy was performed with the use of the Schmidt technique after abdominoperineal resection. MAIN OUTCOME MEASURES: One-year functional results were assessed according to the Kirwan classification system. Functional results were considered "poor" when the Kirwan score was C, D, or E. Univariable and multivariable analyses were used to evaluate the impact of age, sex, type of surgery, irrigation frequency, palpable muscular ring, concomitant chemoradiotherapy, stage, and perineal complications on functional results. RESULTS: One hundred forty-six patients were analyzed. According to the Kirwan system, the scores showed that 100 (68.5%) patients had "good" continence results (stage A-B) and 46 (31.5%) patients had altered functional results (stage C-D-E). With the exception of pelvic recurrences, no conversions from a perineal colostomy to an abdominal colostomy were performed for dissatisfactory functional results. In multivariate analysis, the only independent predictive factors of poor functional results were the occurrence of perineal complications (OR, 3.923; 95% CI, 1.461-10.35; p = 0.007) and extended resection (OR, 3.03; 95% CI, 1.183-7.750; p = 0.021) LIMITATION OF THE STUDY:: This study is an observational retrospective study on selected patients (mainly a young population). CONCLUSIONS: This study showed that perineal complications and extended resection are associated with poor functional results after pseudocontinent perineal colostomy. These data can help clinicians to better inform patients about the outcomes of this technique and to assist them in choosing the right reconstruction technique after abdominoperineal resection.

Management of anorectal melanoma: report of 17 cases and literature review.

BACKGROUND: Primary anorectal melanoma is a rare and aggressive disease. It accounts for 0.5% of all rectal tumors. They are very agressive tumors with poor prognosis. The aim of this study is to report the clinical and evolutionary profile and therapeutical approach of these tumors. PATIENTS AND METHODS: A retrospective study of 17 patients with anorectal melanoma diagnosed between January 1998 and December 2007 was performed. The signs and symptoms, diagnostic study, and surgical and medical treatments were analyzed. RESULTS: The average age was 58 years. Sex ratio was 12 men per five women. Patients had symptoms present for an average of 6 months. The most common symptom was rectal bleeding. According to Slingluff classification, five patients had stage I (localized tumor), four cases had stage II (regional nodes metastasis), and eight cases had stage III (distant metastasis). Seven patients have radical surgery. Only two patients received adjuvant immunotherapy. Eight patients received palliative chemotherapy based on dacarbazine or cisplatinum. The median survival was 8 months. CONCLUSION: Prognosis of anorectal melanoma is still very poor. However, some patients when treated by radical resection may experience long-term survival. The use of adjuvant immunotherapy needs large collaborative studies in view of the rarity of the tumor.

Genetic testing and first presymptomatic diagnosis in Moroccan families at high risk for breast/ovarian cancer.

Germline mutations in the BRCA1 and BRCA2 genes highly predispose to breast and ovarian cancers and are responsible for a substantial proportion of familial breast and ovarian cancers. No female individuals from families from Morocco affected by breast cancer with mutations of these genes have previously been reported, and clinicians in Morocco are unaccustomed to dealing with healthy female individuals carrying mutations in the BRCA genes. This study aimed to report the initial experience of a group of Moroccan investigators carrying out predictive genetic testing to detect a known familial mutation in healthy Moroccan females with a high risk of developing breast cancer and to introduce supervision of these asymptomatic female carriers as a new approach in the prevention and early diagnosis of breast and ovarian cancers in Morocco. Presymptomatic diagnosis was carried out using DNA genetic testing in 5 healthy Moroccan female individuals from three families with an elevated risk of developing breast cancer. These are the first Moroccan families reported to be affected by breast cancers associated with BRCA mutations. Presymptomatic diagnosis was carried out for breast cancer in 5 female individuals from three Moroccan families with BRCA mutations. Two of the families are the first reported incidence of the founder mutation Ashkenazi BRCA1-185_186delAG in Moroccan patients. The third family carried the known BRCA2 mutation c.5073dupA/p.trp1692metfsX3. We tested the presence of these mutations in 5 asymptomatic healthy females from the three families. Two sisters from family 1 carried the BRCA1-185_186delAG mutation, whereas the third female individual from family 2 carried the c.5073dupA/p.trp1692metfsX3 mutation. However, one healthy female individual and her mother from family 3 did not carry the familial mutation of the BRCA1 gene. This study found BRCA mutations in three asymptomatic subjects, suggesting that this is the first step towards the development of persistent medical monitoring of females from families with a history of breast and ovarian cancers. Consequently, it is crucial for oncologists in Morocco to initiate the supervision of healthy female individuals with genetic defects which may lead to hereditary cancers.

Primary lymphoma of the ovary.

Involvement of the ovary by malignant lymphoma is a well-known late manifestation of disseminated nodal disease. Primary ovarian lymphoma is rare. We report a case of primary ovarian non-Hodgkin's lymphoma with bilateral involvement which was managed by surgery and chemotherapy. A 29-year-old woman was admitted with signs and symptoms suggestive of an ovarian cancer. Computed tomography revealed an abdominal tumor measuring 20 cm in diameter, without enlarged lymph nodes. The diagnosis of malignant lymphoma was established after bilateral adnexectomy and histological study of the excised tissue. The tumor was classified as a diffuse large B-cell lymphoma. The patient has been advised 8 cycles of standard CHOP regimen and is presently on treatment. She has now been without disease for 7 months after the surgery. According to previous reports the treatment principles and prognosis of primary ovarian lymphoma is the same as that of other nodal lymphomas.