The management of retroperitoneal sarcoma: The experience of a single institution and a review of the literature.

Objectives: Retroperitoneal sarcomas (RPSs) are rare mesenchymal tumors. The objective of this study was to discuss the different clinical, therapeutic and prognostic aspects of RPS in our institution. Methods: This was a retrospective study conducted at the Department of Medical Oncology in the Habib Bourguiba University Hospital in Sfax, including 19 patients who were treated for RPSs between 1999 and 2016. Results: The median age was 49 years (range: 18-83 years); 68.4% of the patients were female. The commonest symptom was abdominal pain (88%) and the median tumor size was 15 cm (range: 4-30 cm). Complete resection was achieved in only five cases (26.3%). The most common histological subtypes were liposarcoma (47.4%) and leiomyosarcoma (26.3%). Eight patients had a high-grade tumor (G2 = 2 or G3 = 6). Adjuvant radiotherapy was administered in 5 patients (26%). Seventeen patients were treated with chemotherapy; six received chemotherapy in an adjuvant treatment, three as a neoadjuvant treatment, and eight were treated during the palliative phase. Relapse was observed in 58% of cases. For all patients, the overall survival (OS) was 89.5% at 1 year and 40.3% at 5 years. Prognostic factors influencing OS were sex (p = 0.037), resection margins (p = 0.02), recurrence (p = 0.042), and radiotherapy (p = 0.023). In multivariate analysis, radiotherapy (p = 0.031) and histological subtype (p = 0.028) were independent factors influencing OS and disease-free survival (DFS) respectively. Conclusion: We showed that the treatment of RPSs relies on surgery with complete resection. Other factors, such as radiotherapy and the occurrence of relapse, also have an impact on OS. To facilitate surgery and to obtain negative resection margins, preoperative radiotherapy is indicated in selected patients with a high risk of relapse. Further prospective trials are warranted to select optimal therapies with less toxicity and better efficacy in reducing recurrences, mainly at a local level.

The Prognostic Significance of CD10 Expression in Invasive Breast Carcinoma in Tunisian Patients.

BACKGROUND: Breast cancer is the first female cancer worldwide. Its prognosis depends mainly on pathological stage and histological grade. These classical prognostic factors are essential but may be insufficient to predict the outcome of the disease. Research focuses on identifying new prognostic factors such as CD10, which is a cell surface metalloproteinase. OBJECTIVE: This study aims to evaluate CD10 expression on stromal and tumor cells in invasive breast carcinomas and its correlations with other clinicopathological factors and survival. METHODS: A series of 100 cases of breast carcinoma of no special type diagnosed from 2009 to 2011, was investigated in this study. CD10 expression was detected by immunohistochemistry. Stromal CD10 expression (=10% stromal positivity was considered positive) and tumor cells expression (=1% stained carcinomatous cells) were noted. Statistical correlations were analyzed with different known prognostic parameters; survival analysis were performed using SPSS 22.0. RESULTS: Stromal CD10 expression was seen in 60% of the cases. It showed positive correlation with high tumor grade (p=0,012) and distant metastasis (p=0,02). CD10 expression on tumor cells was observed in 10% of the cases. It was associated with high tumor grade (p=0,009), hormone receptor negativity (estrogen receptor: p<0,0001), progesterone receptor: p=0,005), triplenegative phenotype (p=0,001), and Ki67 overexpression (p=0,046). Stromal CD10 expression was significantly associated to a shorter overall survival (p=0,029) and disease-free survival (p=0,05) in univariate analysis. CONCLUSION: Given these results, it can be concluded that CD10 expression predict an aggressive behavior of breast cancer. This marker can be introduced as a determinant prognostic factor.

Germ Cell Tumors Revealing a Familial Persistent Müllerian Duct Syndrome.

Persistent Mullerian duct syndrome (PMDS) is a congenital disorder related to male sexual development. PMDS is usually diagnosed during an inguinal hernia cure. The diagnosis of PMDS following a testicular germ cell tumor is less common. We report the cases of three infertile male patients who were diagnosed with PMDS after surgery for germ cell tumors. They were 39, 27, and 37 years old men with a medical history of neglected cryptorchidism. All patients had a male karyotype and the ELISA test for the anti-Mullerian hormone was undetectable. Patients underwent chemotherapy followed by resection of residual mass in one patient. One patient is currently alive and disease-free. The two other patients died of systemic relapse. These cases highlight how early recognition and treatment of PMDS can prevent malignant germ cell tumors. The diagnosis of PMDS relies on a systemic assessment and analysis of mutations in the gene coding for AMH and AMHR-II. Key words: Persistent Müllerian duct syndrome (PMDS), anti mullerian hormone, germ cell neoplasm.

Exceptional Renal Metastasis from Adenoid Cystic Carcinoma of the Nasal Cavity and Literature Review.

Adenoid cystic carcinoma (ACC) is a rare malignant cancer that arises from secretory glands. Slow growth, perineural invasion, and late recurrences are the main characteristics of ACC. Only few cases of kidney metastases from ACC have been reported in the literature. We report here the case of a 66-year-old female patient who presented with bilateral renal metastases from ACC of the nasal cavity, detected 14 years after treatment of primary tumor and 6 years after metastasectomy of lung metastases. Histological examination confirmed diagnosis and the patient was treated with systemic chemotherapy. Radiological evaluation showed stability of the disease. However, a progression with occurrence of metastases in other sites (lung and bones) has been observed after 7 months. She is still receiving second-line chemotherapy. To the best of our knowledge, this is the second case of kidney metastases from ACC of the nasal cavity.

A case of severe paraneoplastic itch resistant to antihistamines and responding to serotonin reuptake inhibitors.

This article covers an interesting topic. Paraneoplastic pruritus is rare but can be severe. It can sometimes be resistant to usual treatments. In our case, it was resistant to antihistamines but was relieved by inhibitors of serotonin scrapping.

An uncommon breast metastasis of mediastinal neuroblastoma within a child: A case report.

A 13-year-old girl presented with dull lumbar pain and recent paraplegia. Clinical examination found a breast mass associated with an axillary adenopathy. Imaging revealed a large mass in the posterior mediastinum with spinal cord compression. Histological analysis confirmed the diagnosis of neuroblastoma with mammary metastases. To the best of our knowledge, breast metastasis is being reported for the first time in a mediastinal neuroblastoma.

Prognostic significance of E-cadherin and Cox 2 expression in Tunisian patients with colorectal mucinous adenocarcinoma.

INTRODUCTION: Mucinous colorectal carcinoma (MC) is a rare subtype of colorectal adenocarcinoma known to be associated with bad prognosis. Lately, research has turned to identify new prognostic markers allowing the use of targeted therapy. The aim of our study is to evaluate the prognostic impact of E-cadherin and Cox-2expression in MC. MATERIALS AND METHOD: A total of 40 formalin-fixed, paraffin-embedded MC specimens were collected within a period of 13 years and were studied for the expression of the two proteins. We used SPSS 22 software to study associations with clinicopathological parameters and overall survival (OS). RESULTS: A reduced or absent E-cadherin expression was noted in 52.5% of cases. It was associated with distant metastases (p = 0.049) and venous invasion (p = 0.049). Cox-2 was overexpressed in 17.5% of cases. It was associated with negative lymph node status (p = 0.020) and with early stage tumor (p = 0.020). A significant association between the two proteins was also noted (p = 0.04). No significant association with OS was found; However, there was an improvement in the survival of patients overexpressing Cox-2 (p = 0.16). CONCLUSION: Our findings link the loss of E-cadherin expression with spread and aggressiveness in MC and Cox-2 overexpression with better prognosis and survival. Because MC has a distinct genetic pathway we encourage the analysis of MSI and Cox-2 expression in all MC. Cox-2 inhibitors may not be effective chemopreventative agents in the setting of defective DNA mismatch repair. More molecular studies are needed to better understand the role of these markers and their prognostic significance in MC.

Tuberculosis and non-pulmonary malignancies : study of ten cases.

INTRODUCTION: Tuberculosis (TBC) is a major public health problem with high mortality especially in developing countries. It is associated with a higher risk of developing pulmonary and non-pulmonary malignancies including solid and hematologic cancers. Association between TBC and nonpulmonary malignancies is rarely described in the literature. AIM: To describe the epidemiological, clinical, therapeutic modalities and the evolutive aspects of patients treated for cancer and TBC. METHODS: This is a retrospective study conducted over a period of 19 years (between 1993 and 2012), including 10 patients followed up for cancer and tuberculosis at the department of oncology and the department of infectious disease, CHU Habib Bourguiba Hospital and CHU HediChaker, Sfax, Tunisia. RESULTS: The average age of patients was 55 years old. The sex ratio was 1. The different locations of cancer were represented by the breast (4 cases), the nasopharynx (1 case), the colon (1 case), the kidney (1 case) the gum (1 case), the endometrium (1 case) and the blood (1 case).TBC and cancer were synchronous in 5 cases. Concerning the metachronous presentation that interested 5 patients, the average time betweenthe onset of TBC after cancer diagnosis was 3.5 years. Three of these patients were treated by chemotherapy with radiation therapy. TBClocalization was nodal in 6 cases, spinal one case, nasopharyngeal in one case, peritoneal in one case and urogenital in one case. The diagnosisof TBC was made incidentally in two cases during axillary lymph node dissection. The therapeutic management of cancer was based on surgery,chemotherapy and / or radiotherapy. All patients received anti TBC treatment for at least six months. Surgery was indicated in one case(laminectomy). A complete remission of cancer was observed in 9 patients. Concerning TBC, recovery was observed in 8 patients, 1 patient hada spinal recurrence and 1 patient is being treated. CONCLUSION: Chronic inflammation during TBC can lead to cancer development. The etiopathogenesis of this association is still imperfectly known. Association between TBC and non-pulmonary cancer, although rare, should be always kept in mind in order to handle in time these two diseases in order provide the best chances of recovery for patients.

Genetic Analysis of Mu and Kappa Opioid Receptor and COMT Enzyme in Cancer Pain Tunisian Patients Under Opioid Treatment.

BACKGROUND: Pain and its opioid treatments are complex measurable traits. Responses to morphine in terms of pain control is likely to be determined by many factors, including the underlying pain sensitivity of the patient, along with nature and extent of the painful process, concomitant medications, genetic and other clinical and environmental factors. This study investigated genetic polymorphisms implicated in the inter-individual pain response variability to opioid treatment in the Tunisian population. METHODS: This prospective association study investigated seven variations in the OPRM1, OPRK1 and COMT gene, which encode Mu and KAPPA opioid receptors, and Catechol-O-methyltransferase enzyme respectively, in a cohort of 129 Tunisian cancer pain patients under oral morphine treatment. Genotyping was performed by simple probe probes on Light Cyler for rs17174629, rs1799972, rs1799971, rs1051659, rs1051660 and rs4680 and by PCR assay for the indel in the promoter region of OPRK1 (rs35566036). A statistical associations study between dose (continuous), dose escalation (yes/no) and SNP or haplotypes were investigated using linear multiple regressions and logistic regressions respectively adjusted on metastases and pain covariates in the R software. RESULTS: We detected significant association of the rs1051660 adjusted on metastasis and pain (P=0.02), no other association has been detected between the 7 polymorphisms screened and the dose of morphine needed for pain relief. CONCLUSION: This can be explained by the strong genetic heterogeneity in the cosmopolitan areas where our patients were recruited for this study, compared to more homegenous population recruited in other studies.

Ewing's sarcoma of the cranial vault: a case report.

Ewing's sarcoma is a malignant tumor that mainly affects young patients. It represents 10% of primary malignant tumors of the bone and 3% of malignant tumors of the child. Cranial localization is extremely rare representing less than 1% of all the localizations. We report a case of a 10-year-old girl who presented with an intracranial hypertension syndrome with left parietal mass of progressive installation. The X-ray skull showed a lytic lesion with irregular margins involving the left parietal bone. Brain magnetic resonance imaging revealed extensive parietal bone destruction involving both the inner and outer tables. The girl was operated in emergency. Histological examination concluded to Ewing's Sarcoma. The resection was incomplete (R1). The girl received induction's chemotherapy. The cerebral scanner evaluation showed no abnormalities. Then, she received consolidation's chemotherapy with concomitant local radiation therapy. Currently, the girl is in complete remission with a seven-month decline.

Primary Gingival Melanoma: An Important Entity.

Primary melanoma of the mandibular gingiva is extremely rare. It is often misinterpreted as a benign pigmented process. The prognosis of this entity is very poor. We report here the first case of primary gingival melanoma described in the Tunisian literature about a 55-year-old smoker having cerebral and pulmonary metastases from gingival melanoma at diagnosis. Our patient underwent brain radiotherapy at a dose of 18 Gy in three sessions but he died with a decline of 3 months before starting systemic therapy. Therefore, each new case should be illustrated to make clinicians aware about the importance of the early diagnosis to improve the poor diagnosis of this entity.

The Difficulties in the Management of Breast Cancer and Pregnancy.

INTRODUCTION: Breast cancer and pregnancy is rare. It is defined by the discovery of breast cancer during pregnancy or within one year after delivery. Throughout this study, we analyzed the characteristics of this entity with review of the literature. MATERIALS AND METHODS: We report through a retrospective study of breast cancer in pregnant women, a series of 11 cases collected at the oncology department in Sfax between 1994 and 2012. RESULT: The average age of our patients at diagnosis was 35 years. The average term of pregnancy was 22 weeks. Nine cases were diagnosed during pregnancy. The pregnancy outcome was well with babies in a good health, in 10 cases. The prognosis was excellent with complete remission after a mean follow of 39 months in 7 cases (2years-9years). CONCLUSION: The diagnosis of breast cancer in pregnant women is often delayed because of physiological changes of pregnancy and the young age which explain the poor prognosis of this entity. The prognosis was excellent in our series compared to the literature.

[Mixed breast carcinoma with melanocytic differentiation in a man]. / Carcinome mammaire mixte avec différenciation mélanocytaire chez un homme.

INTRODUCTION: Male breast cancer is rare; the lobular type is exceptional. Only one case of mixed ductal and lobular type is reported in the literature. This is the first report on a mixed ductal and lobular carcinoma with melanocytic differentiation in a man. The aim of our study is to describe the clinicopathological characteristics of this rare type of breast tumor and to discuss its histogenesis. CASE REPORT: A 63-year-old man presented with cutaneous ulceration of the left breast. Ultrasound of the breast revealed a solid hypoechoic lesion, 13 mm in diameter. Microscopic evaluation of the biopsy showed an invasive ductal carcinoma. The patient received three cycles of chemotherapy and lost of view. Then consulted for increasing of the tumor size reaching 3 cm. Histological examination of the mastectomy specimen showed a mixed ductal and lobular carcinoma with melanocytic differentiation, confirmed by the immunohistochemical study. The patient received adjuvant chemotherapy and the evolution was favorable with an average follow-up of 9 months. CONCLUSION: Breast carcinoma with melanocytic differentiation is extremely rare; only seven cases are reported in the literature and all occurs in females. Its histogenesis is unclear; tumors exhibiting this combination of cell types may occur as collision or composite tumors.

Primary Signet Ring Cell Adenocarcinoma of the Urinary Bladder: A Report of 2 Cases.

Primary signet ring cell carcinoma of the urinary bladder is a rare and aggressive histologic subtype of adenocarcinoma. In general, this tumor occurs in the middle age, and clinical presentation does not differ from transitional cell carcinomas. The prognosis is often poor, given the advanced stage at diagnosis. To our knowledge, <100 cases of signet ring cell adenocarcinoma of the urinary bladder have been reported. We report 2 cases with bladder linitis plastica primitive, and we draw attention to its pathologic, anatomoclinical, and evolution specificity to optimize its therapeutic management.

Mammary neuroendocrine carcinoma with mucinous differentiation: a clinicopathological study of 15 cases.

BACKGROUND: Mammary neuroendocrine carcinoma (NEC) displays morphological features including mucinous type. OBJECTIVE: To describe clinicopathological of NEC with mucinous differentiation. METHODS: A total of 15 cases of mammary NEC with mucinous differentiation were reviewed. RESULTS: All patients in this study were women aged from 37 to 78 year olds (median 68.1 years). The tumors ranged in size from 1.2 cm to 16 cm (mean 3.74 cm). The amount of extracellular mucin varied from 10% to 90%. Histological grade was I in 7 cases and II in 8 cases. Immunohistochemically, estrogen receptor (ER) and progesteron receptor (PR) were expressed in 12 and 14 cases, respectively. All tumors were negative for Her-2. Ki-67 proliferative index was lesser than 1% in all cases and no cases had demonstrated p53 overexpression. Three patients died of disease with a follow-up of 3 to 6 months. One patient was alive with metastasis at 96 months. Ten patients were disease free (follow-up range from 15 to 125 months). CONCLUSIONS: Mammary NEC with mucinous differentiation affects mostly older women. All tumors were low grade and immunoreactive for ER/PR and negative for Her-2. Mammary NEC with mucinous differentiation seems associated with well survival parameters.