Henda Raies

Systemic therapy in the management of metastatic or advanced salivary gland cancers

Salivary gland cancers are very rare tumors. They are characterized by a histologic heterogeneity and a poor outcome. According to this rarity, few prospective data are available to date. No standard recommendations could be held for the use of systemic therapy in these tumors. Several case reports and small studies have investigated the contribution of different agents of chemotherapy. With the extension of molecular biology approach in oncology several signaling pathways have been discovered in different cancers including salivary gland cancers; thus a number of targeted therapies have been investigated. This paper reviewed exhaustively the studies investigating the role of systemic therapies (chemotherapy, targeted therapy, hormone therapy) in salivary gland cancers.

Larynx preservation: What is the best non-surgical strategy?

The concept of larynx preservation in locally advanced laryngeal or hypopharyngeal squamous cell carcinoma has evolved during the last three decades, especially with the advancement of nonsurgical strategies. These nonsurgical strategies include: (1) radiotherapy alone; (2) concomitant chemoradiotherapy (CCRT); and (3) induction chemotherapy followed by radiotherapy or CCRT and concurrent anti-epidermal growth factor receptor (EGFR). To date, the best approach for larynx preservation has yet to be defined. In this article, we review and discuss important recent randomized phase II/III trials investigating larynx preservation in order to facilitate the selection of an appropriate strategy in the clinical setting. However, the decision of larynx preservation should always be a multidisciplinary approach.

Primary anorectal melanoma: A case report with extended follow-up.

Summary Background: Anorectal melanoma is a rare and aggressive disease. The mainstay of treatment is usually surgical with curative or palliative intent, since radio- and chemotherapy do not really improve the outcome. The poor prognosis is attributable to delay in diagnosis and its inherent biologic aggressiveness. Case Report: We present a case of anorectal melanoma in a 68-year-old man who underwent solely abdominoperineal amputation and was doing well 30 months after surgery, without any evidence of recurrent disease. Conclusions: Treatment of anorectal melanoma should be by the least morbid means possible. Surgical procedure that can achieve a complete local excision and respect the functional aspects and quality of life of the patient remains the best therapeutic approach. No systemic regimen for metastatic anorectal melanoma is considered standard of care.

Squamous cell carcinoma of the gallbladder

Gallbladder cancer is the most common malignant tumor of the biliary tract. The majority of cases are adenocarcinoma (AC). Pure squamous cell carcinoma (SCC) of gallbladder accounts only 3% of the malignant neoplasm of this organ. Many patients are at advanced stage when diagnosed and have bad therapeutic efficacy. At present, radical surgery is the only chance to gain long-term survival for patients with early-stage gallbladder cancers. Recent reports have shown a benefit of adjuvant chemoradiation in this type of tumor. At present, no therapy is defined for unresectable cancer of the gallbladder, especially for SCC.

Testicular Synovial Sarcoma: A Case Report

This paper reports a case of testicular synovial sarcoma with molecular genetic analysis. A 24-year-old male presented with painless scrotal mass. Ultrasonography showed a heterogeneous mass of 66 mm × 34 mm in size involving the inguinal region. Histological examination of a surgical biopsy showed a grade III monophasic growth pattern of spindle cell proliferation. Immunohistochemical analyses indicated positive staining for pancytokeratine and epithelial membrane antigen. Cytogenetic analysis showed the presence of CYT-SSX1 mutation, and CT scan showed non-specific pleural micro-nodules with a size of 7.5 mm. The patient had an extended left orchidectomy but was lost to follow-up for 1 year. A local recurrent scrotal mass of 32 mm × 25 mm, multiple inguinal lymph nodes, and increased pleural nodules, which were confirmed by histological examination, were treated with three cycles of adriamycine and ifosfamide chemotherapy, surgical resection, and radiotherapy with complete response. After 3 months, the patient developed local recurrence and pulmonary metastases that did not respond to second-line chemotherapy based on gemcitabine and paclitaxel. The patient had dyspnea at the time of this writing and chest pain, and is under third-line chemotherapy based on Deticene after 30 months of following up. This patient died on November 16, 2012 after a resperatory failure and malignant pelural effusion. Synovial sarcoma should be considered in the differential diagnosis of soft tissue tumor and it should be aggressively treated to improve prognosis. Although our patient has shown numerous factors of bad prognosis, he has had a relatively long survival time.

Liposarcoma of the spermatic cord: A case report and review of literature

a Department of Oncology, Salah Azaiez Institute, Faculty of Medicine of Tunis, Boulevard 9 Avril, Tunis El Manar University, Tunis, France Department of Pathology, Charles Nicolle Hospital, Faculty of Medicine of Tunis, Boulevard 9 Avril, Tunis El Manar University, Tunis, France c Department of Urology, Charles Nicolle Hospital, Faculty of Medicine of Tunis, Boulevard 9 Avril, Tunis El Manar University, Tunis, France

Para testicular adult rhabdomyosarcoma: A case report and review of litterature

Paratesticular adult rhabdomyosarcoma (RMS) is a rare pathology that has the specificity of being more aggressive than that of the child. It has high malignant potential, quick spread and requires prompt and adequate care. In this context we report the case of an adult paratesticular RMS. We aimed to describe its clinical features and natural history.

Atteinte synchrone pulmonaire et des glandes lacrymales par un lymphome de type MALT

Primary lung lymphomas are rare tumors representing less than 1% of malignant tumors of the lung. The most frequent form is the mucosa-associated lymphoid tissue (MALT). Ocular involvement is also rare and it is mostly located in the lachrymal glands. We report the case of a patient with pulmonary MALT lymphoma associated with synchronous involvement of the lachrymal glands. This study illustrates the nonspecific clinical, radiological and evolutionary features of this disease.

Granulosa Cell Tumor of the Ovary: A Retrospective Study of 31 Cases and a Review of the Literature

Background Adult granulosa cell tumors (AGCTs) are the most common sex cord-stromal tumors. Unlike epithelial ovarian tumors, they occur in young women and are usually detected at an early stage. The aim of this study was to report the clinical and pathological characteristics of AGCT patients and to identify the prognostic factors. Methods All cases of AGCTs, treated at Salah Azaïz Institute between 1995 and 2010, were retrospectively included. Kaplan-Meiers statistical method was used to assess the relapse-free survival and the overall survival. Results The final cohort included 31 patients with AGCT. The mean age was 53 years (35–73 years). Patients mainly presented with abdominal mass and/or pain (61%, n = 19). Mean tumor size was 20 cm. The majority of patients had a stage I disease (61%,  n = 19). Two among 3 patients with stage IV disease had liver metastasis. Mitotic index was low in 45% of cases (n = 14). Surgical treatment was optimal in almost all cases (90%, n = 28). The median follow-up time was 14 years (1–184 months). Ten patients relapsed (32%) with a median RFS of 8.4 years (6.8–9.9 years). Mean overall survival was 13 years (11–15 years). Stage I disease and low-to-intermediate mitotic index were associated with a better prognosis in univariate analysis (resp., p = 0.05 and p = 0.02) but were not independent prognostic factors. Conclusion GCTs have a long natural history with common late relapses. Hence, long active follow-up is recommended. In Tunisian patients, hepatic metastases were more frequent than occidental series. The prognosis remains good and initial staging at diagnosis is an important prognostic factor.

Giant Cell Tumor of Soft Tissues: A Case Report and Review of Literature

Background: Primary giant cell tumor (GCT) of soft tissue (GCTST) is an extremely rare slow-growing entity bearing a high similarity to conventional bone TCG (GCTB). The term, malignant tumor of giant cells of soft tissues have been reserved for histologically high-grade lesions. Although the gold standard remains surgical carcinological resection, bisphosphonates are beginning to prove their benefit in the treatment of GCTST. Results and Discussion: A 37-year-old man came to the outpatient department of medical oncology with a painful swelling arising from his right elbow. Magnetic resonance imaging (MRI) of the right elbow was done and revealed a 19 cm × 7 cm, T1 and T2 hypointense lesion with significant postcontrast enhancement of calcified tissue nodules, distance extension report was negative. An echo-guided biopsy of the right elbow was performed. The anatomopathological examination showed a poorly delimited encapsulated tumor proliferation composed of sheets of histiocytic cells admixed with multinucleated giant cells dispersed uniformly among this tumor. Cells were embedded in a richly vascularized tissue. No significant nuclear pleomorphism or mitotic activity was appreciated. There were focal areas of osseous metaplasia. On the basis of these date, the diagnosis of giant cell tumor of low malignant potential was retained. Due to its intra-articular extension, the mass was judged unresecable. The case was discussed in a multidisciplinary consultation meeting indicating medical treatment with zoledronic acid given the unavailability of denosumab. After 8 monthly injections of zoledronic acid, a control imaging of the right elbow and forearm concluded to tumor stability. Conclusion: GCTST is a slow-growing tumor known as soft tissue tumor. Numerous studies show the role of bisphosphonates when complete surgical excision cannot be performed. Further studies are needed to establish a standardized treatment protocol particularly in the context of inoperable large primary GCTST.