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H syndrome is an extremely rare autosomal recessive affection caused by biallelic mutations in the SLC29A3 gene encoding the human equilibrative nucleoside transporter hENT3. The hallmark signs are cutaneous consisting of hyperpigmentation and hypertrichosis patches. Besides, associated systemic manifestations are highly various reflecting phenotypic pleiotropism. Herein, we report a first case of pseudo-Meigs’ syndrome occurring in a young Tunisian H syndrome diagnosed patient with a novel homozygous frameshift mutation in exon 2 of the SLC29A3 gene: p.S15Pfs*86 inducing a premature stop codon. The patient developed ascites associated with left ovarian mass and she underwent surgery. After tumor resection, ascites disappeared rapidly. Histological examination showed serous cystadenoma of the ovary orienting the diagnosis towards pseudo-Meigs’ syndrome.

Background: The management of gastric adenocarcinoma is essentially based on surgery followed by adjuvant treatment. Adjuvant chemotherapy (CT) as well as chemoradiotherapy (CTRT) have proven their effectiveness in survival outcomes compared to surgery alone. However, there is little data comparing the two adjuvant approaches. This study aimed to compare the prognosis and survival outcomes of patients with gastric adenocarcinoma operated and treated by adjuvant radio-chemotherapy or chemotherapy Materials and methods: We retrospectively evaluated 80 patients with locally advanced gastric cancer (LGC) who received adjuvant treatment. We compared survival outcomes and patterns of recurrence of 53 patients treated by CTRT and those of 27 patients treated by CT. Results: After a median follow-up of 38.48 months, CTRT resulted in a significant improvement of the 5-year PFS (60.9% vs. 36%, p = 0.03) and the 5-year OS (55.9% vs. 33%, p = 0.015) compared to adjuvant CT. The 5-year OS was significantly increased by adjuvant CTRT (p = 0.046) in patients with lymph node metastasis, and particularly those with advanced pN stage (p = 0.0078) and high lymph node ratio (LNR) exceeding 25% (p = 0.012). Also, there was a significant improvement of the PFS of patients classified pN2–N3 (p = 0.022) with a high LNR (p = 0.018). CTRT was also associated with improved OS and PFS in patients with lymphovascular and perineural invasion (LVI and PNI) compared to chemotherapy. Conclusion: There is a particular survival benefit of adding radiotherapy to chemotherapy in patients with selected criteria such as lymph node involvement, high LNR LVI, and PNI.

Objectives To compare the survival outcomes between primary debulking surgery (PDS) and interval debulking surgery (IDS) in advanced epithelial ovarian cancer (EOC). Methods Data of 117 patients treated for a FIGO stage III-IV EOC between January 2000 to December 2010 were retrospectively reviewed. Results PDS was performed in 95 patients (81.2%) and IDS in 22 patients (18.8%). From all, 33 cases (28.2%) had maximal cytoreduction (R0), 39 had a residual disease (RD) ≤1 cm (33.3%) and 45 patients (38.5%) had a RD >1 cm.The 5-years OS was significantly associated the quality of resection (R0 resection:36.4%, RD ≤1 cm:25.5%, RD >1 cm:18.2%; p=0.041).The rate of complete and suboptimal resection (≤1 cm) was significantly higher in case of IDS compared to PDS (86.4% vs 55.8%, p=0.008). No significant difference on OS was found between PDS and IDS (28.2% vs 15.8%, p=0.364). Nonetheless, in the subgroup of patients with complete resection, PDS resulted in a significantly higher 5 years OS compared to IDS (44% vs 12.4%, p=0.045) with no significant difference in case of sub-optimal resection (28.6% vs 13.3%, p=0.830). However, IDS increased the 5 years OS of patient who had a RD greater than 1 cm compared to PDS (33% vs 17%, p=0.245). Neoadjuvant chemotherapy improved significantly the 5-year OS of patients staged FIGO IV compared to PDS (40% vs 12.5%, p=0.032). Conclusions Because of the lack of survival benefit of either treatment modality, the indication of PDS or IDS should depend on the extent of peritoneal carcinomatosis and the possibility of complete surgical resection.

Objectives The aim of this study was to identify predictive factors of axillary lymph node metastasis (ALNM) in early breast cancer (EBC). Methods It was a retrospective study of 209 patients with T0-T1-T2 breast cancer who underwent resection of the primary tumor and axillary staging by sentinel lymph node biopsy (SLNB) and/or axillary lymph node dissection (ALND) over 2012 at our institute.The χ2 test and Fisher’s exact probability tests were used for categorical variables,and t-test for continuous variables.Predictors of ALNM were identified by univariate and multivariable logistic regression analyses using SPSS statistical software package (version 20.0). Results Among the 209 patients, 48.8% (102 cases) had ALNM.Factors associated with ALNM in univariate analyses were tumor clinical size (23.5% in stage T0,41.9% in stage T1,55.4% in stage T2; p=0.02),multifocality (73.7% vs 43.3%,p=0.001),lymphovascular invasion (LVI) (77.1% vs 43.1%,p Conclusions Our results suggest that LVI, tumor size and the rate of positive ER are predictive factors for ALNM in EBC.

Background This study aimed to evaluate the severity of intraoperative and post operative complications of gastric cancer surgery and to investigate the predictive factors correlated to surgical morbidity. Methods We included 145 patients operated for gastric cancer. We investigated the risk factors associated with complications, length of hospital stay, operative time, and intraoperative blood transfusion (BT). Significant risk factors were analyzed by multiple logistic regression analysis. Results Postoperative complications occurred in 32 patients (22.1 %) and the rate of major complications was 7.6%. The rate of anastomotic fistula was 6.9% and was correlated to diabetes, tumor size, operative time, surgical margin, and extended lymphadenectomy. The mean risk factors for postoperative morbidity were the presence of comorbidities and ASA score (p = 0.021), intraoperative BT (p = 0.045) and prolonged operative time (p = 0.055). Conclusion surgical morbidity of gastric cancer is correlated to the extent of resection as well as the clinical and histological characteristics.

Cutaneous leiomyosarcoma is a rare soft tissue sarcoma. We present a case of a 50-year-old man, with a rare location in the flank. Clinical presentation showed an ulcerative lesion. Imaging features were evocative of malignancy. Surgical excision was performed, involving oblique muscles. The diagnosis of low-grade leiomyosarcoma with free margins was made. No adjuvant therapy was indicated. Clinical and radiological follow-up of the patient during two years did not reveal recurrences. Cutaneous leiomyosarcoma of the flank is extremely rare and takes part in a group of infrequent and unknown tumors. Clinical presentation is unspecific and histological evidence is mandatory for diagnosis. Tumor extension in depth constitutes the major determinant factor for therapeutic management and prognosis. Wide surgical resection is crucial to minimize recurrence risk especially when poor prognostic factors take place.

The fibrosarcomatous variant of dermatofibrosarcoma protuberans (DFSP) represents an uncommon form of DFSP which has a higher risk of local recurrence and distant metastases than ordinary DFSP. The aim of our study is to investigate clinicopathologic characteristics, treatment modalities and prognostic factors of nine cases of transformed DFSP admitted in Salah Azaiez Institute between 2002 and 2009. They were five men and four women. Median age at diagnosis was 52 years (35–87 years). The lesions were located on the abdominal wall (three cases), the upper limb (two cases), the back (two cases), the lower limb (one case) and the chest wall (one case). Tumor size ranged from 25 mm to 150 mm. After diagnosis, six patients were treated by wide local excision with margins ⩾2 cm, two patients had local excision without defined margins and one patient had incomplete local excision. Three patients underwent radiotherapy because of either cramped or unknown limits. Local recurrence was diagnosed in 5 cases and distant metastasis occurred in one patient. Fibrosarcomatous DFSP exhibits more aggressive behavior than DFSP. Their similar clinical presentation requires histopathological differentiation for prognosis. Treatment is based on wide local excision, radiation and targeted therapy.