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.

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.

[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.

A novel missense mutation in ATRX uncovered in a Yemeni family leads to alpha-thalassemia/mental retardation syndrome without alpha-thalassemia.

BACKGROUND: Intellectual disability (ID) features in numerous heritable medical conditions that result from ATRX mutations. Alpha-thalassemia mental retardation syndrome (ATR-X syndrome) is the most notable manifestation of ATRX dysfunction. In addition to ID, genitourinary and craniofacial abnormalities are regularly observed with or without alpha-thalassemia. AIMS: The study sought to characterize two cases of ATR-X in a Yemeni family clinically and molecularly. METHODS: PCR amplification and Sanger sequencing were used to study the ATRX gene in a Yemeni family. Also, methylation-sensitive PCR was used to perform X-inactivation studies. CADD, SNAP2 and PolyPhen-2 helped to predict the functional consequences of the variant. RESULTS: Molecular testing revealed a novel hemizygous missense mutation (c.5666T>G) in the ATRX gene in the two Yemeni brothers. This mutation was found in a heterozygous state in the mother, with the chromosome harboring the mutated allele being under strongly skewed X-inactivation. CONCLUSIONS: The mutated gene is predicted to have a disrupted SNF-2 domain at a conserved residue; p.Leu1889Trp, which is deemed functionally damaging. This report offers, for the first time, full clinical and molecular characterization of a novel ATRX variant in an Arab family.

[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.

Induction of the anti-carcinogenic enzyme quinone reductase by food extracts using murine hepatoma cells.

Over 145 extracts of vegetables, fruits, herbs, spices and beverages which are consumed regularly in the European diet have been surveyed for potential anti-carcinogenic activity using an assay which measures the induction of NAD(P)H: (quinone acceptor) menadione oxidoreductase (quinone reductase, QR) activity in murine cells challenged with solutions of potential inducers. When appropriate the study has included extracts prepared from cooked and autolysed material. The results indicate that extracts of some brassicas, legumes (peas), lettuces, red pepper, grapefruit and some herbs including basil, tarragon and rosemary are inducers of QR activity. Inducing activity is strongly dependent on processing and on variety.

Radionuclide contamination of foods imported into Iraq following the Chernobyl nuclear reactor accident.

Since early 1986, a monitoring program for radionuclides in imported foods has been carried out by the Iraqi Atomic Energy Commission. After the Chernobyl nuclear reactor accident in the Soviet Union, the program was expanded; our laboratory was officially designated by the Iraqi Government to measure radionuclide activity concentrations in foodstuff imported from countries known to be severely contaminated by Chernobyl radioactive fallout. Gamma-spectrometric analysis was used. Food items such as powdered milk, lamb meat, poultry, cereals and grains imported into Iraq before the Chernobyl accident did not contain any detectable fission products. However, all lamb meat, 81% of the lentil, 44% of the powdered milk and chick-pea, and 17% of the roast beef samples were contaminated with 137Cs or 134Cs and 137Cs. The highest 137Cs contamination levels found were 82, 147, 420, 6 and 4 Bq kg-1, respectively. Contamination by 134Cs was approximately 50% of the values given above.

[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.

[Conservative treatment of extremity soft tissue sarcomas. Functional evaluation using LENT-SOMA scales and Enneking scoring]. / Traitement conservateur des sarcomes des tissus mous des membres. Evaluation du résultat fonctionnel selon l'échelle LENT-SOMA et le score de Enneking.

OBJECTIVE: The aim of this prospective study is the feasibility of late effects assessment by LENT-SOMA scales after conservative treatment of soft tissue sarcomas of the extremities and a comparison with the functional evaluation by the Enneking score. PATIENTS AND METHODS: During the systematic follow-up consultations, a series of 32 consecutive patients was evaluated in terms of late effects by LENT-SOMA scales and functional results by the Enneking score. The median time after treatment was 65 months. The treatment consisted of conservative surgery (all cases) followed by radiation therapy (29 cases), often combined with adjuvant therapy (12 concomitant radio-chemotherapy association cases out of 14). The assessment of the toxicity was retrospective for acute effects and prospective for the following late tissue damage: skin/subcutaneous tissues, muscles/soft tissues and peripheral nerves. RESULTS: According to the Enneking score, the global score for the overall series was high (24/30) despite four the scores zero for the psychological acceptance. According to LENT-SOMA scales, a low rate of severe sequellae (grade 3-4) was observed. The occurrence of high-grade sequellae and their functional consequences were not correlated with quality of exerese, dose of radiotherapy or use of concomitant chemotherapy. A complementarity was observed between certain factors of the Enneking score and some criteria of the LENT-SOMA scales, especially of muscles/soft tissues. CONCLUSION: The good quality of functional results was confirmed by the two mean scoring systems for late normal tissue damage. The routine use of LENT-SOMA seems to be more time consuming than the Enneking score (mean time of scoring: 13 versus five minutes). The LENT-SOMA scales are aimed at a detailed description of late toxicity and sequellae while the Enneking score provides a more global evaluation, including the psychological acceptance of treatment. The late effects assessment by the LENT-SOMA scales should be carried on in prospectives studies, especially in case of concomitant radio-chemotherapy.

[Colorectal cancer in patients younger than 40 years of age]. / Cancers rectocoliques chez le sujet de moins de 40 ans.

Between 1988 and 1994, 326 patients with colorectal cancers were treated at the Ibn Rochd Oncology Centre in Casablanca; 88 patients were less than 40 years old (27%). There were 79 males and nine females. A family history of colorectal cancer was noted in 5%. The most frequent symptoms were bleeding and rectal pain. The localisation of the tumour was the rectum in 70 cases. According to the anatomopathological profile, Dukes stages C and D were the most frequent and mucosal adenocarcinoma was noted in 24 cases. Seventy patients had surgery, associated with radiation therapy in 59 cases. Adjuvant chemotherapy was given in 44 cases and palliative treatment in 17 cases. Recurrence occurred in 14 cases and metastasis in other six cases. At five years, overall survival and disease-free survival rates were 24% and 17%, respectively. Worse prognostic factors were mucosal adenocarcinoma, poor differentiated adenocarcinoma and Dukes C and D stages.

[Salivary duct carcinoma of the parotid gland: report of two cases and literature review]. / Carcinome canalaire de la glande parotide. A propos de deux cas et analyse de la littérature.

Salivary duct carcinomas are scarce tumour developed mainly in the male in the 6th or 7th decades of their life. Authors report two new cases developed 43 and 60 years-old in females. Because this tumour was localised, a radical treatment associating surgery and radiotherapy was performed. Follow-up were 115 and 137 months, and the two patients remain free of disease. These two cases were opportunity to discuss literature data.

[Chemotherapy of soft tissue sarcoma in the adult]. / Chimioradiothérapie [correction of Chimiothérapie] des sarcomes des tissus mous de l'adulte.

The goal of postoperative treatment in adult soft tissue sarcoma is local control, and in high-risk patients prevention of distant failures. Radiation therapy is essential after non-radical surgery. The role of adjuvant chemotherapy on improvement of overall survival remains to be evidenced; however, recent meta-analysis data have confirmed its impact on both local and metastatic evolution of the disease. Because for both radiotherapy and chemotherapy, delay of treatment may be crucial for efficacy following tumor excision, concomitant radiochemotherapy should be considered. Review of the literature as well as personal results showed the feasibility of postoperative radiochemotherapy in adult soft tissue sarcoma, even when the chemotherapeutic associations used included an anthracycline. Prospective study of radiochemotherapy should be performed in order to assess its real impact in terms of efficacy and toxicity.