Radiotherapy in the management of rare gastrointestinal cancers: A systematic review.

The aim of this analysis is to assess radiotherapy's role and technical aspects in an array of rare gastrointestinal (GI) cancers for adult patients. Collection data pertaining to radiotherapy and digestive rare cancers were sourced from Medline, EMBASE, and Cochrane Library. Preoperative chemoradiotherapy improved outcomes for patients with esophageal undifferentiated carcinoma compared with esophageal salivary gland types of carcinomas. For rare gastric epithelial carcinoma, perioperative chemotherapy is the common treatment. Adjuvant chemoradiotherapy showed no benefice compared with adjuvant chemotherapy for duodenal adenocarcinoma. Small bowel sarcomas respond well to radiotherapy. By analogy to anal squamous cell carcinoma, exclusive chemoradiotherapy provided better outcomes for patients with rectal squamous cell carcinoma. For anal adenocarcinoma, neoadjuvant chemoradiotherapy, followed by radical surgery, was the most effective regimen. For pancreatic neuroendocrine tumors, chemoradiotherapy can be a suitable option as postoperative or exclusive for unresectable/borderline disease. The stereotactic body radiotherapy (SBRT) is a promising approach for hepatobiliary malignancy. Radiotherapy is a valuable option in gastrointestinal stromal tumors (GIST) for palliative intent, tyrosine kinase inhibitors (TKIs) resistant disease, and unresectable or residual disease. Involved field (IF) radiotherapy for digestive lymphoma provides good results, especially for gastric extranodal marginal zone lymphoma (MALT). In conclusion, radiotherapy is not an uncommon indication in this context. A multidisciplinary approach is needed for better management of digestive rare cancers.

Primary neuroendocrine tumors of the breast: About a case and of the review of the literature.

primary neuroendocrine carcinomas of the breast represent a minority and are currently included in the latest WHO classification of breast tumors. Their morphological and immunohistochemical features (chromogranin and synaptophysin expression) allow the retain the diagnosis. we report a case of primary neuroendocrine carcinoma of the breast in 50 years old Moroccan women who presented nodule 4,2 cm palpable and mobile of the left breast. Lumpectomy axillary lymph node resection was performed. a histopathological examination disclosed the diagnosis of primary breast neuroendocrine tumors with negative surgical margins and positive lymph nodes (13 N+/19 N). The tumor cells were positive for neuroendocrine markers, a highKi67 proliferation index and the membrane expression of the invasive tumor cells to the anti-HER2 antibody was 2, a FISH done which was equivocal. Our patient received 6 courses of chemotherapythen radiotherapy; currently she received adjuvant hormonal treatment with Tamoxifene.

Influence of static compression on mechanical parameters of acoustic foams.

The modification of elastic properties of compressed acoustic foams is investigated. The porous sample is first submitted to a static compression and then to a dynamic excitation of smaller amplitude, corresponding to acoustical applications. The static compression induces the modification of the dynamic elastic parameters of the material. This work focuses on Young's modulus. The variation is measured with two different experimental methods: The classical rigidimeter and an absorption measurement. The effective Young's modulus is directly measured with the first method and is indirectly determined through the quarter-wave length resonance of the frame with the second one. The results of the two measurements are compared and give similar tendencies. The variation of the dynamic Young's modulus as a function of the degree of compression of the sample is shown to be separated in several zones. In the zones associated with weak compression (those usually zones encountered in practice), the variation of the effective Young's modulus can be approximated by a simple affine function. The results are compared for different foams. A simple model of the dependency of the Young's modulus with respect to the static degree of compression is finally proposed for weak compressions.

Lynch syndrome or hereditary non polyposis colorectal cancer (HNPCC) in a moroccan family: Case report.

INTRODUCTION AND IMPORTANCE: Colorectal cancer is a major global health problem. In 5% of cases, a genetic predisposition to cancer's syndrome is the etiology, such as Lynch syndrome. The population prevalence of Lynch syndrome has been estimated at 1/440. The objectives of this study are to show the interest of the oncogenetic consultation in the management of patients with suspicion of Lynch syndrome. CASE PRESENTATION: It is a 70-year-old patient with a family history of different neoplasms. The patient has also been followed for an adenocarcinoma of the colon. An oncogenetic consultation was indicated, which led to the diagnosis of Lynch syndrome, according to the Amsterdam II criteria. A study of the MisMatch Repair genes was requested, to allow a pre-symptomatic diagnosis of apparented subjects at risk, and thus to also allow monitoring and early diagnosis of neoplasms or prophylactic measures. DISCUSSION: Lynch syndrome is one of the most common cancer susceptibility syndromes. A constitutional deleterious mutation in one of the DNA MisMatch Repair genes, is responsible for nearly 70% of cases of this syndrome. The oncogenetic consultation and the identification of the genetics cause, makes it possible to set up specific monitoring and to offer a pre-symptomatic test to all major relatives of the index case. CONCLUSION: This medical observation shows the benefit of the oncogenetic consultation, if a genetic predisposition to cancer's syndrome is suspected. The diagnostic of this predisposition and monitoring of the propositus and his exposed, like in Lynch syndrome will help in the early management of cancers, specially colorectal cancer and endometrial adenocarcinoma.

[A rare tumor of the infratemporal fossa]. / Tumeur rare de la fosse infratemporale.

INTRODUCTION: Giant cell tumors of bone (GCT) are usually benign and relatively rare. They have an aggressive behavior and an unpredictable prognosis. They occur mainly in the young adult, with a preferential localization in long bones. We report a giant cell infratemporal fossa tumor. OBSERVATION: A 55-year-old female patient consulted for swelling in the right cheek. Surgical excision was incomplete because of the subtemporal tumor localization. Histological assessment proved a GCT. Forty-five grays postoperative external radiotherapy was applied to the surgical site. The patient had local control at the 12-month follow-up. DISCUSSION: GCTs are seldom observed in the facial skeleton (2%). The recommended treatment is surgery. Radiotherapy can be indicated in case of incomplete or impossible surgical excision, or when surgery would be responsible for a major functional deficit.

[Treatment de-intensification strategies for HPV-driven oropharyngeal cancer: A short review]. / Désescalade thérapeutique dans les cancers de l'oropharynx induit par les HPV : mise au point.

The incidence of oropharyngeal cancer induced by human papillomavirus (HPV) infection is steadily increasing in developed countries. These tumors are more chemoradiosensitive and have a better prognosis than HPV-negative one. In addition, they occur in younger and better-off patients with longer life expectancy. Current radiotherapy and chemotherapy protocols are currently being questioned as they may expose HPV-positive patients to excessive treatment and unnecessary toxic effects. Less intensive treatment regimens could possibly achieve similar efficacy with lower toxicity and improved quality of life. The aim of this work was to summarize the knowledge on these tumors and their implications for radiation oncologists. In this update, we will discuss ongoing de-escalation trials and highlight the issues raised by these studies. We will also comment on the results of recently published de-intensification studies. Three main strategies are analyzed in the present article: the de-escalation of the drug associated with radiotherapy, the de-escalation of the radiotherapy dose (in concomitant chemoradiotherapy, after induction chemotherapy, in a postoperative setting) and de-escalation of radiation target volumes. Our findings ultimately indicate that clinicians should not change the management of oropharyngeal cancer patients outside of clinical trials.

[Post-radiation choanal stenosis]. / Sténose choanale post-radique.

OBJECTIVE: Description of a rare complication of radiation therapy for nasopharyngeal carcinomas and the therapeutic value of surgical endoscopic desobstruction. CASE REPORT: A choanal stenosis observed in an 8-year-old boy, 3 years after radiation therapy and chemotherapy for a voluminous nasopharyngeal carcinoma. No initial involvement of the nasal fossa was observed. Concomitant relapse was excluded after clinical and histological examination. RESULTS: A good functional result was achieved after the choana was unblocked in videoendoscopic surgery. After 9 months of follow-up, the breathing and odor functions remained intact. CONCLUSION: Choanal stenosis is a rare complication of radiotherapy for nasopharyngeal carcinoma. Relapse should be excluded with biopsies beyond the stenosis. Treatment is based on endoscopic surgery.

[Breast metastasis from vulvar carcinoma: case report and review of literature]. / Métastase intramammaire d'un cancer vulvaire: à propos d'un cas avec revue de la littérature.

The breast metastases resulting of vulvar carcinoma are very rare, and represent exceptionally the first manifestation of the disease. We report the case of a 42 year-old patient who underwent a treatment because of vulvar epidermoid carcinoma, right away metastatic at the level of the inguinal ganglia. The treatment consisted in a total vulvectomy with bilateral ganglial curretage, followed by external radiotherapy about the perineum and the inguinal ganglia. Three months after the end of her treatment, the patient presented with a nodula on the left outer breast with features of malignancy noticed by clinic and mammographic examination. The histologic study of the mammary biopsy showed epidermoid carcinoma of likely metastatic origin. A left Patey has been realized and confirmed the metastatic localization of epidermoid carcinoma with axillary ganglial metastasis (2N+/-7). Besides, this patient presented a right cervical ganglial parcel that the biopsy showed a metastatic localization of a vulvar carcinoma. A palliative chemotherapy type cyclophosphamid, adriblastin, cisplatine (CAP) has been admistrated during three cycles spaced out three weeks. The patient died 11 months after the supervene of the cerebral metastasis. We present this case because its rarety and to show the possibility of metastasis at the level of breast due to vulvar cancer. The clinicians must remember this possible tropism of the vulvar cancer for the breast, not only during the supervision and the complete examination as regards the disease spreading but also when the affection revealed unknown primary tumor. The diagnostic orientation is based on the mammography and the mammary biopsy. In this stage, the treatment is unfortunately palliative, the survival until a year is not more than 20%.

[Small cell neuroendocrine carcinoma of the bladder: Report of five cases and a review of the literature]. / Carcinome neuroendocrine à petites cellules de la vessie : à propos de cinq cas et revue de la littérature.

Neuroendocrine small cell carcinoma of the bladder is a rare and aggressive tumour, accounting for less than 1% of all bladder tumours. Given its rarity and the absence of randomized trials, the therapeutic management of these tumours remains difficult. By analogy with small cell lung cancer, multimodal treatment is often proposed. Radical cystectomy plus chemotherapy and chemoradiation therapy are associated with better survival compared to monotherapy. We report our experience in the management of these tumours with literature review.

[Radiotherapy of primary breast sarcomas: Retrospective study]. / Radiothérapie des sarcomes mammaires primitifs : étude rétrospective.

PURPOSE: Primary breast sarcomas are heterogeneous tumours derived from non-epithelial mammary gland structures. Although they represent a rare entity, their incidence may increase in the coming years owing to conservative approach considered in the treatment of breast cancer. The aim of this work was to highlight the effect of postoperative irradiation in the treatment of these tumours. MATERIALS AND METHODS: This is a retrospective study conducted at the Mohammed-VI centre for cancer treatment between 2004 and 2011. Survival rates were calculated by the Kaplan-Meier method. RESULTS: Fifteen cases were collected. The median age was 41.9years. Phyllode sarcoma accounted for 66% of this series. Surgical treatment was performed in 93% of the patients with negative margins in 33.33% of the cases. Neoadjuvant chemotherapy was indicated in 46% of the patients with locally advanced tumours and 66% of the patients received postoperative radiotherapy for positive or close margins. Five years overall survival and relapse free survival was not significantly different with the use of adjuvant radiotherapy. CONCLUSION: Due to the rarity of this entity and the absence of randomized trials, evidence based management is still lacking. However, a multidisciplinary approach is to be required including surgical excision followed by radiotherapy, depending on the tumour characteristics.

[Osteosarcoma of the skull base: a case report]. / L'ostéosarcome de la base du crâne. A propos d'un cas.

Osteosarcoma, usually observed in long bones, is the second most frequent primitive malignant bone tumor after myeloma. The skull base is an exceptional localization. We report a case of skull base osteosarcoma managed in our department. A 23-year-old female was admitted for bilateral epistaxis, headache, decreasing visual acuity then blindness. Physical examination revealed bilateral blindness and exophthalmia. Cranial magnetic resonance imaging showed a voluminous mass in the skull base extending to the nasosinusal area. A rhinoseptal surgical approach was used but the tumor was so huge that excision was impossible. The biopsy identified at an osteoblastic osteosarcoma. Search for extension (chest computed tomography and abdominal ultrasonography) was negative. Chemotherapy was to be delivered before combination chemoradiotherapy but the patient died before any treatment could be started. Osteosarcoma of the skull base is very rare. The treatment is based on surgery which should be as complete as possible followed by chemoradiotherapy. Prognosis is poor. Median survival is around six months.

Lung adenocarcinoma with thyroid metastasis: a case report.

BACKGROUND: The metastases of a primary lung cancer over the thyroid gland are extremely rare. We report on an unusual presentation of thyroid metastasis of lung cancer in order to improve the management of similar cases. CASE PRESENTATION: Three years ago, a Moroccan male 59-year-old was admitted for dyspnea, dry cough, and chest pain. He had smoked about 30 cigarette packs a year. Clinical examination revealed a right thyroid nodule. Chest and neck computed tomography (CT) scan showed a proximal left tumor in contact with the pulmonary artery and revealed a suspected nodule in the right lobe of the thyroid with homolateral neck node. Transbronchial biopsy was performed and pathological examination revealed adenocarcinoma of the lung and positive for thyroid transcription factor. Other explorations carried out, such as brain CT, bone scan and abdominal ultrasound were normal. After a repeated negative fine needle aspiration biopsy of the suspected nodule of the right lobe of the thyroid, we performed total thyroidectomy with neck dissection. An anatomopathologic exam revealed a tubulopapillary adenocarcinoma poorly differentiated. An Immunohistochemistry showed positive tumor cells with TTF1 and cytokeratin (CK) 7 but negative cells with thyroglobulin and CK20. Thus, the pulmonary tumor was classified stage IV. Chemotherapy based on the combination of cisplatin and etoposide was conducted along with supportive care. The tumor grew up with brain metastases after three cycles of chemotherapy. Unfortunately, the patient died 2 months after despite brain radiotherapy. CONCLUSION: We presented a medical case of a patient with thyroid metastasis resulting from a pulmonary adenocarcinoma which has rapidly evolved to brain metastases. The prognosis was pejorative in our clinical case (5 months after admission).

[Small-cell carcinoma of the vulva]. / Le carcinome à petites cellules de la vulve: à propos d'un cas avec revue de la littérature.

Small-cell carcinoma is a rare and aggressive malignancy; this tumor is lethal due to the propensity to metastasize early in the course of the disease. It occurs most frequently in the lung. Small-cell cancer also rarely may occur in the female genital tract, usually in the cervix. This article concerns the fifth reported case of small-cell carcinoma of the vulva in a 34-year-old women who had developed a vulvar mass3 months earlier. The physical examination revealed bilateral inguinal lymph nodes. The mass was excised and the histological finding was a small-cell carcinoma. Postoperative search for metastasis included computed tomography scan of the abdomen, pelvis, chest and brain that showed right iliac lymph nodes. The osteo medullar biopsy was positive. The patient was treated with 6 cycles of chemotherapy including cisplatinum (80mg/m2 d1) and etoposide (100mg/m2 d1 d2 d3). Adjuvant radiotherapy (50Gy) was administered but the disease progressed and the patient died after 7 months. Small-cell carcinoma of the vulva is a very rare tumor. Similarly to small-cell cancers arising in other sites, it appears that regional therapy is not a sufficient treatment for this tumor. Chemotherapy should be used to improve outcome.

[Mucinous carcinoma of the breast: Clinical, biological and evolutive profile]. / Carcinome mucineux du sein : profil clinique, biologique et évolutif.

PURPOSE: Mucinous carcinoma of the breast accounts for 1 to 4% of all breast cancer. There are two histological subtypes: mixed mucinous carcinoma, where the ductal carcinoma is associated with the colloid component, and pure mucinous carcinoma, with a favorable prognosis, where the mucus surrounds the tumour tissue and constitutes a mechanical barrier limiting cell invasion and making this form less aggressive. Our study aimed to determine retrospectively the main epidemiological, clinical, biological, and therapeutic features, as well as the prognosis of this rare form of breast carcinoma. MATERIALS AND METHODS: The authors report 32 cases of mucinous carcinoma of the breast diagnosed in Mohammed-VI centre for cancer treatment in Casablanca. RESULTS: The average tumour size was 4.5cm (0.5-7cm). We found ten positive lymph node dissections, seven of them were of mixed mucinous carcinoma with a tumour size ranging between 4 and 7cm. Mucinous carcinoma was pure in 16 cases, mixed in 14 and a neuroendocrine differentiation was found in two cases. Most tumours were of an intermediate histological grade (n=19) with positive hormonal receptors (68%). After a mean follow-up of 30 months, complete remission was maintained in 92% of evaluable patients. CONCLUSION: Mucinous carcinoma is a rare type of breast cancer, with a favourable prognosis for the pure form.

[Rhabdomyosarcoma of the salivary glands. Report of 3 cases]. / Le rhabdomyosarcome de la glande parotide. A propos de trois cas et revue de la littérature.

Rhabdomyosarcoma of the salivary glands is rare. Tumours develop mostly in children and young patients but can be diagnosed in older people. We report three new cases in women 7, 14 and 75 years old. Tumefaction of the parotid region and facial paralysis were the principle clinical symptoms. Two patients underwent a total parotidectomy and radiotherapy. The third patient had a locally advanced tumour and received chemotherapy followed by radiotherapy. Loco-regional recurrence was observed in all cases and median survival was 12 months. Rhabdomyosarcoma of the salivary glands is locally aggressive. Treatments include surgery and radiotherapy. The role of chemotherapy remains to be discussed.

[Therapeutic outcomes of medulloblastoma in Casablanca from 2000 to 2012]. / Résultats du traitement du médulloblastome à Casablanca de 2000 à 2012.

PURPOSE: To describe the therapeutic results, with the aim to contribute to improving the care of patients with medulloblastoma. PATIENTS AND METHODS: A retrospective study of 69 cases of medulloblastoma collected in the university hospital Ibn Rochd of Casablanca between 2000 and 2012. RESULTS: Fifty-three children with an average age of 9 years and 16 adults with an average age of 32.4 years were included in the study. Thirty-seven children and eight adults suffered from a high-risk tumour. The radiotherapy was received by all patients with a mean dose of 36 Gy to the whole brain and 54 Gy in the posterior fossa. All patients in the paediatric group and 10 patients in the adult group received concomitant chemotherapy, 44 children and four adults received adjuvant chemotherapy. Tumour recurrence was observed in 17 children after a mean follow-up period of 38 months. These recurrences were observed in five adults after a mean follow-up period of 42 months. The posterior fossa was the main site of relapses. Overall survival was 77.7% for the children and 61% for the adults. Overall survival was better (70% versus 25%) when the interval between radiotherapy and surgery was less than 40 days in the paediatric group. The recurrence rate was significantly higher for the high-risk group: 41% versus 13% for the standard risk. In the adult group, overall survival differences according to the risk group were significant (100% for the standard risk versus 37.5% for the high risk). CONCLUSION: The overall survival and recurrences rate obtained are encouraging. The risk group and time between surgery and radiotherapy were prognostic factors with significant impact on survival depending on the age group. We recommend reducing these times to improve therapeutic results.

[Carcinomas of the nasopharynx in children. Analysis of 50 cases]. / Carcinomes du nasopharynx chez l'enfant. Analyse de 50 observations.

From 1980 to 1990, 50 children were treated for nasopharyngeal carcinoma in the Department of Oncology at the hospital Center Ibn Rochd in Casablanca. There were 34 boys and 16 girls with a median age of 12.3. UCNT were observed in 70% of cases. Fourty six percent were stage T3-T4 and 70% were N2-N3 disease. All children received irradiation therapy and 16 of them have had a neo-adjuvant chemotherapy. Thirty seven children were evaluable, the immediate response was complete in 26/37 cases. Recurrence of disease was noted for four children and metastatic for four others children. Two children have both recurrence and metastatic disease. The overall survival was 40.5% at 3 years. Disease free survival was 37.8% at 3 years.

[Changes of the cutaneous micro-relief in superficial radiation-induced fibrosis: a qualitative study]. / Modifications du microrelief cutané dans la fibrose radio-induite superficielle: étude qualitative.

Cutaneous radiation-induced fibrosis (RIF) is characterized by a skin retraction or atrophy, toughness to the palpation and often entails functional limitation. Its clinical evaluation remains poorly quantified. The aim of this study was to propose an analytical method to quantify RIF skin surface with the replica technique. In this preliminary study, we report the qualitative and quantitative evaluation of the cutaneous microrelief in 44 healthy controls and in four patients presenting a superficial RIF, 3 to 20 years after radiotherapy for cancer. The microrelief of these RIF presented an abnormal anisotropy with a parallel reorganization of cutaneous valleys in three cases out of four, suggesting a premature radiation-induced ageing of the skin. Each subject being his own control, the relative vertical amplitude of the skin microrelief was +/-15% in control skin. Vertical amplitude was respectively increased by 84% in one inflammatory fibrosis (3 years after RT), decreased by 18% in one evolutive fibrosis (6 years after RT), decreased by 26% in one voluminous stabilized fibrosis (8 years after RT) and decreased by 53% in one atrophic fibrosis (20 years after RT). The present study suggests that the variations of the microrelief parameters could reflect the RIF evolution. This technique requires a validation in a larger series of patients, including patients with telangiectasia.

[Cancer of the uterine cervix in young women. A retrospective study of 337 cases]. / Cancer du col utérin chez la femme jeune. Etude rétrospective de 337 cas.

A retrospective study of 337 cases of cervix cancers in women aged 35 years and under, treated between 1980-1990 at the Ibn Rochd Oncology Center in Casablanca, was analyzed in order to determine the main characteristics of this disease and the influence of age on prognosis. The incidence of these cases was stable throughout the 11 years study period with a mean of 7.4% of all cervix cancers. The rate of early sexual activity in age under 18 years was particularly high, with 80%, of which 45.4% before the puberty. The advanced cases were predominating (58.2%). Earlier cases were treated with combined radiotherapy and surgery and advanced cases with radiotherapy alone. Overall survival rate was 41% at 5 years. It was 45.2% for the 31-35 years of age group and 32% for women aged 30 years and less. The prognosis of these younger women was especially poor in tumoral volume more than 3 cm, in advanced cases, in undifferentiated carcinoma and in cases of lymph node involvement. The comparative analysis of all prognostic factors in function of the age had permitted to show that women aged 31-35 years old have been a good prognosis, concerning survival at 5 years, in regard to women less 30 years.