A renal angiomyolipoma with a challenging presentation: a case report.

INTRODUCTION: Renal angiomyolipoma is considered a benign mesenchymal tumor composed of fat, smooth muscle, and blood vessels. It represents 1-3% of solid renal tumors. Despite this tumor's benignity, it can be aggressive with a locoregional extension. CASE REPORT: A 41-year-old north African caucasian woman consulted with chief complaints of right lower back pain with no hematuria and no urinal sign. Thoracic-abdominopelvic contrast-enhanced computed tomography showed a right inferior polar heterogeneous renal mass complicated with venous thrombus ascending to the right atrium level. The patient underwent radical nephrectomy under extracorporeal circulation and direct supervision of the fatty thrombus at the right atrium level. The postoperative period was uneventful. The final histologic examination was concordant with renal angiomyolipoma. CONCLUSION: Renal angiomyolipoma is the most common benign kidney tumor. Despite its benignity, it can be associated with lethal complications such as hemorrhage, and it can also show signs of local extension mimicking malignant tumors. The cornerstone of the treatment remains surgery.

Is the systematic circumferential tumor cavity shaving a representative sample for the remaining mammary gland? About 75 cases.

INTRODUCTION: The conservative surgery is more and more indicated for breast cancer. However, we still fear local recurrence which is mostly due to residual tumors?. Several techniques have been used to minimize theses residual tumors; one of them is the systematic circumferential tumor cavity shaving (SCTCS). METHODS: We sampled 75 female patients who had conservative surgery with positive shaved margins in the anatomopathology examination and to whom a complementary treatment with mastectomy have been decided. RESULTS: The median age was 48 years old. The median tumor size was 23 mm. In the histological examination of the tumors, 93% were invasive ductal carcinoma associated in 50% of the cases to the presence of ductal carcinoma in situ (DCIS) where all the lumpectomies had clear margin. For the SCTCS, 62,2% were DCIS and in 17,6% of the cases were invasive ductal carcinoma. A complementary treatment with mastectomy was indicated to all the patients. A residual tumor was detected in the remaining mammary gland in 47,7% of the cases out of which 50% were DCIS. Local recurrence happened in three patients (4,6%) after a median of follow up of 36 months. The overall survival and the disease free survival at five years were respectively 83,6% and 75,5%. CONCLUSION: Standardized lumpectomy cavity shaving provides a backup to lumpectomy margins in conservative breast surgery but it can also be used as a sample for the remaining breast, helping to detect the residual tumor, and decreasing the rates of local recurrence after BCT.

Inflammatory breast cancer in Tunisia from 2005 to 2010: epidemiologic and anatomoclinical transitions from published data.

AIM: To report epidemiologic and anatomoclinical transitions of inflammatory breast cancer (IBC) in Tunisia. MATERIALS AND METHODS: Data including clinico-pathological data for208 cases of T4d or PEV 3 non-metastatic breast cancer diagnosed between 2005 and 2010 were collected from patient records. Chi2 and Z tests were used to compare variables with two Tunisian historical series and a series about Arab-American patients. RESULTS: Thirty three percent of our patients had their first child before 23 years of age and 56% had their menarche before 12 years, 75% never receiving oral contraception. Obesity was observed in 42% of women and IBC occurred during pregnancy in 13% of cases. Tumor grade was II-III in 90% of cases, HR was negative in 52%, HER2 was over expressed in 31% and invasion of more than 3 axillary nodes occurred in 18% of patients. We observed a pCR rate of 19% after neoadjuvant treatment (anthracyline-taxane used in 79%, trastuzumab in 27% ). Compared to historical Tunisian series (since 1996), IBC epidemiology remained stable in terms of median age, menopausal status and obesity. However we observed a significant decrease in median clinical tumor size and number of positive axillary lymph nodes. Comparison to IBC in Arab-Americans showed a significant difference in terms of median age, menopausal status, positivity of hormonal receptors and educational level. CONCLUSIONS: Our assessment of epidemiologic transition showed a reduction of clinco-pathological stage of IBC, keeping the same characteristics as compared to Tunisian historical series over a period of 14 years. Features seem to be different in Arab-American patients, probably related to migration, "occidentalization" of life style and improvement in socio-economic level.

[What is new in the local approach of limb sarcomas and desmoid tumours?]. / Actualisation de la stratégie thérapeutique locorégionale dans les sarcomes des tissus mous et les tumeurs desmoïdes des membres.

The treatment of soft tissue sarcomas of limbs should be discussed within an experienced multimodality team. Surgical resection remains the cornerstone of therapy for localized disease and achieves a five years overall survival around 75% and a local recurrence rate as low as 10% in the best series. In complex cases, neo-adjuvant treatments may be used such as systemic chemotherapy, isolated limb perfusion, or radiotherapy to achieve an optimal conservative approach. Molecular genetics of sarcomas and quality of margins are essential to guide diagnosis and therapeutic selection. In case of marginal or incomplete resection, a new enlarged surgical resection should always be discussed before administration of any adjuvant treatments. Many retrospective studies and two randomized studies (one of adjuvant brachytherapy and one of external beam radiotherapy) have shown that adjuvant radiotherapy after complete surgery significantly reduces the risk of local recurrence in extremity soft tissue sarcomas. A randomized study has compared pre- to postoperative radiotherapy. The results in terms of local control are similar in both arms. The risk of surgical complications is higher in the preoperative arm and the risk of late sequelae is higher in the postoperative arm. A randomized study within the French sarcoma group is ongoing evaluating the omission of postoperative radiotherapy in favourable cases. Presently, the role of systematic first-line invasive treatment (including surgery and/or radiotherapy) of desmoids is debated. It is becoming evident that up to 50% of patients with desmoids benefit from a front-line non-aggressive policy, because growth arrest is a common feature of this disease. Additional study of the molecular determinants of desmoid behaviour is needed to guide treatment.

[Surgical news of soft tissue sarcomas, fibromatosis and GIST]. / Actualités chirurgicales des sarcomes, des fibromatoses et des tumeurs stromales gastro-intestinales.

Excellent local control rates can be achieved using multidisciplinary approach and combined surgical technics. Better vascular and nervous dissections, use of different flaps and isolated limb perfusion have been determinant. Resection's extent of retroperitoneal sarcoma is still debated, but compartmental surgery seems to achieve better local control. The impact of pre operative radiotherapy will be explored soon in a randomized EORTC trial. Concerning desmoids, authors address the question whether surgery and other aggressive treatments should systematically be part of first-line treatment, as growth arrest occurred in 2/3 of non-operated patients. The objective of on going biological studies is to use molecular findings to individualize the selection of management protocols. In the same way, surgical indications for gastrointestinal stromal tumors evolved: with the development of investigations, more micro-GIST are discovered, rising the question of wait and see policy for some of them. In locally advanced inoperable patients and metastatic patients, imatinib is the standard treatment. Secondary excision of residual disease has been shown to be related to a good prognosis in responding patients to imatinib, but it is still not demonstrated whether this is due to surgery itself or to a selection bias. An ongoing phase III EORTC randomises this secondary surgery after 6 to 12 months of imatinib in responding patients.

[Radio-histological correlation of microcalcifications of the breast: a Tunisian center experience]. / Corrélation radio-histologique des microcalcifications du sein. Experience d'un centre Tunisien.

PURPOSE: Evaluation of surgical biopsy for mammary microcalcifications, in Tunisian patients according to the American College of Radiology's recommandations (ACR). MATERIAL AND METHODS: [corrected] 100 patients treated in Salah Azaiez Institute from January 2005 to December 2006, who underwent surgical biopsy for breast microcalcifications The diagnosis was based on mammography associated in 60% of the cases, to ultra-sonography. We correlated biopsies results to ACR classification. RESULTS: 123 surgical biopsies are related to ACR 3 lesions in 72 cases (58.5%) ACR 4 in 39 cases (32%) and ACR 5 in 14 case (11.5%). The histological examination showed malignant lesions in 4 cas/72 ACR3 (5.5%), 19/39 ACR4 (48.7%) and 10 among the ACR5 (71.4%). CONCLUSION: Our preliminary study introduces a diagnostic approach of mammary microcalcifications, that are usually infra-clinic lesions, in a general context of breast tumors detected with an average size of more than 3 cm.

[Typical medullary carcinoma of the breast: a retrospective study about 33 cases]. / Carcinome médullaire typique du sein : étude rétrospective à propos de 33 cas.

OBJECTIVE: Typical medullary carcinoma of the breast is a rare histological form of breast carcinoma. It represents less than 5% of all breast cancer. It is known for its favourable prognosis. Considering the rarity of the series we could retrieve, we aimed at underlining the particularities of this pathology. PATIENTS AND METHODS: Retrospective study about 33 cases of typical medullary carcinoma managed at the Salah-Azaïz Institute during a period of six years between 1994 and 1999. RESULTS: The mean age was 47.5 years. The left breast was concerned in about 55% of cases. Mean tumoral size was 46mm. Sixty-one percent of our patients were treated by radical surgery. Seventy-three percent undergo radiotherapy, 57% chemotherapy and 42% hormonotherapy. The five-year free-disease survival was about 85%. DISCUSSION AND CONCLUSION: Typical medullary carcinoma of the breast is a rare histological form of breast carcinoma. Its treatment is similar to the other breast cancers. Our results agree with the different published studies and confirm its favourable prognosis.

[Secondary breast cancer after treatment for Hodgkin's disease. About seven cases]. / Cancer du sein secondaire après traitement pour maladie de Hodgkin. A propos de sept cas.

OBJECTIVE: Therapeutic results of Hodgkin disease (HD) have improved by the use of combined radio-chemotherapy. However, this association can increase the risk of on-term effects including secondary cancers. In a retrospective study, we collected secondary breast cancer (BC) in patients previously treated with chemoradiotherapy for Hodgkin disease at Salah-Azaïz institute of Tunis. PATIENTS AND METHODS: Between 1975 and 2003, seven patients (six women and one man) treated for HD subsequently developed BC. Mean age at diagnosis of HD was 21 years (12-29). The first treatment was combined chemotherapy (MOPP-ABVD) and radiotherapy for all patients. Radiotherapy was delivered with cobalt 60 with large fields. The median dose was 41.3 Gy (2 Gy/fraction in 6 patients and 3.3 Gy in one). RESULTS: The breast tumours occurred after a median delay of 204 months (132-276). According to the TNM classification, we showed two stage T2, one stage T3, two stage T4b and two stage T4d. The mean clinical size was 47 mm (25-80 mm). All patients had infiltrating carcinoma. Axillary node histological involvement was found in 6 cases. All patients were treated by mastectomy and chemotherapy. Only one patient had a locoregional irradiation. Median survival was 26.5 months (12-48). Four patients died and three are still alive at respectively 24, 31 and 144 months. DISCUSSION AND CONCLUSION: According to the previous data, breast cancer represents 6.3 to 9% of all second cancers occurring after HD treatment. We conclude that especially young women and girls treated for HD should be carefully monitored. We suggest that secondary BC be sometimes treated by conservative radiosurgical approach.

[Benign schwannoma of the breast: about two cases]. / Schwannome mammaire bénin: à propos de deux cas.

Schwannoma is a rare nervous tumor developed on schwan cells. Only 22 cases of breast schwannoma have been published since 2005. It usually appears as a breast lump having clinical and radiological characteristics suggestive of kindness. Its diagnosis is histological. Its treatment is surgical. Through two observations and a review of the literature we would try to remind the characteristics of this tumor.

[Mammary tuberculosis: a retrospective study of 65 cases]. / Tuberculose mammaire: étude rétrospective de 65 cas.

OBJECTIVES: Remember the clinical, morphologic and histological particularities of mammary tuberculosis. Discuss clinical and radiological diagnostic problems caused by this localization, as far as breast cancer is concerned. PATIENTS AND METHODS: This is a retrospective study concerning 65 women suffering from mammary tuberculosis, diagnosed at Salah Azaiez Institute between January 1980 and December 2001. RESULTS: In this series, mammary tuberculosis represents 0.2% of the declared tubercular localizations and 0.3% of the mammary pathologies treated at the institute. The mean age of our patients was 36 years (19 to 79). Clinical findings were misleading. In 60% of cases the aspect was of a malignant tumor, in 24.6% of cases of a benign nodule and in 16.4% of cases of an abscess. The mammography concluded to a malignant lesion in 49% of cases. The fine needle aspiration achieved at 8 patients was negative in 6 cases and brought back the caseum in the 2 others. The diagnosis has been made, for all our patients, after histological study when we found typical tuberculosis lesions on pieces of tumorectomy, or biopsy. The diagnosis of tuberculosis was established; all patients had an anti-tuberculosis medical treatment in a department of infectious diseases out of the institute. DISCUSSION AND CONCLUSION: Clinical and radiological features of mammary tuberculosis are very confusing and cause a diagnostic problem with breast cancers. In front of symptoms evoking tuberculosis, biopsies must be done to eliminate an eventual cancer.

Intrahepatic Osler's disease: report of two cases and review of the literature.

Osler-Weber-Rendu disease is a hereditary vascular disease with multiple manifestations. The liver is involved rarely. Vascular abnormalities include telangiectasis and arteriovenous fistulas, sometimes associated with fibrosis and cirrhosis. Hepatic arteriovenous shunting may include secondary portal hypertension, reduced liver function and high cardiac output. Two cases of Osler-Weber-Rendu disease with extensive hepatic arteriovenous fistulation were described in detail and we report on their clinical features. In the first patient, treatment was symptomatic since liver transplantation is not indicated because the patient was asymptomatic. Embolization treatment of hepatic arteries was indicated in the second patient because he had biliary disease and recurrent cholangitis secondary to vasculo-biliary shunts. Therapy with arterial embolization, banding, or ligature of hepatic arteries is still limited and provides unsatisfactory long-term results. Liver transplantation offers now another therapeutic option for patients with intrahepatic high shunting and secondary pulmonary hypertension.