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.

[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.

[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.

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).

[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.

[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.

Metachronous bilateral isolated adrenal metastasis from rectal adenocarcinoma: a case report.

We report a case of adrenal metastasis from colorectal cancer in a 54-year-old woman. Nine months after resection for advanced rectal carcinoma, a computed tomography scan revealed bilateral adrenal metastasis. The level of serum carcinoembryonic antigen was normal. A bilateral adrenalectomy was performed after chemotherapy. Histopathological examination showed adenocarcinoma, compatible with metastasis from the rectal cancer. Adrenal metastasis should be considered in the patients' follow-up for colorectal cancer.

Pseudomyxome peritoneal

Le pseudomyxome du peritoine est une affection dont la genese est peu connue. De plus sa rarete est confirmee par les 2 cas decouverts de maniere fortuite sur 10.000 laparotomies. Le tableau clinique est pauvre; fait de distension et douleurs abdominales. Il semble que le pseudomyxome soit consecutive a la rupture d'un muccocele appendiculaire. L'association soit a des tumeurs malignes bien differenciees soit a des tumeurs frontieres a souvent ete retrouvee. La production extracellulaire de mucine dans la cavite peritoneale constitue un critere majeur de diagnostic. Le traitement adequat associe la chirurgie complete des lesions macroscopiques et la chimiotherapie intraperitoneale en utilisant certaines molecules qui ont montre leur efficacite

[Granular cell tumor: report of a complicated vulvar localization of pulmonary metastases]. / Tumeur à cellules granuleuses : à propos d'une localisation vulvaire compliquée de métastases pulmonaires.

Granular cell tumour, or Abrikossoff's tumour, is a rare, ubiquitous tumour, with mostly benign evolution. The malignant forms represent 1-3% of the cases. The vulvar localization is exceptional. We report the case of a 28-year-old patient who presented a vulvar Abrikossoff's tumour. Initially considered benign, the tumour was treated by exclusive local surgery. Five months later, the tumour showed a malignant transformation. The tumour size was 14cm in its major axis with the presence of not systematized scattered mitoses, PS 100 was positive at 100% and Ki 67 to 20%. Because of malignant and economic excision of the neoplasm, the patient received adjuvant radiotherapy. After 20 months, the patient had a local recurrence and lung metastasis, supporting the diagnosis of malignancy. She received two chemotherapy regimens of 5-fluoro-uracil/cisplatin and pacliatxel/carboplatin without any response. Following the failure of chemotherapy, the patient received symptomatic medical treatment. This observation leads to advocate an aggressive surgical treatment and strict supervision of all granular cell tumours.

[Orbital metastasis from mucinous adenocarcinoma of the rectum]. / Métastase orbitaire d'un adénocarcinome mucineux du rectum.

Orbital metastasis from colorectal cancer are extremely rare. Only six cases are described in the literature. The discrepancy between the frequency of colorectal cancer and the rarity of metastasis to the eye and orbit remains paradoxical and enigmatic. We report the case of an 18-year-old young woman with orbital metastasis as the presenting sign of rectal cancer. Proptosis is the most frequent presenting clinical sign. Once the diagnosis is made, the prognosis is poor and treatment is palliative.

[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.

[Ocular metastasis heralding gastric adenocarcinoma]. / Métastase oculaire inaugurale d'un adénocarcinome gastrique.

Ocular metastasis is a rare presenting feature of gastric adenocarcinoma. We report a 48-year-old woman who presented with a decrease in visual acuity of the right eye leading to the discovery of an ocular metastasis. Diagnostic work-up identified a gastric adenocarcinoma with pulmonary metastases. She received four cycles of chemotherapy combining epirubicin, cisplatin and fluorouracil. The patient died 6 months after the diagnosis of respiratory failure.

[Adjuvant chemotherapy followed by conformal chemoradiotherapy in gastric carcinoma]. / Chimiothérapie adjuvante suivie d'une chimioradiothérapie conformationnelle dans les cancers de l'estomac.

PURPOSE: Analysis of the feasibility and results of adjuvant chemotherapy followed by conformal chemoradiotherapy after surgery for gastric carcinoma. PATIENTS AND METHODS: Twenty-six patients (R0 or R1) were treated postoperatively by three cycles of 5-fluorouracil (5-FU) and cisplatin, followed by a concomitant association of LV5FU2 chemotherapy with a conformal radiotherapy of 45 Gy. RESULTS: The tumor was classified pT3-T4 in 77% of the patients and 92.5% had a nodal involvement (pN1: 54%; pN2: 31%). FEASIBILITY: (1) Adjuvant chemotherapy: nausea/vomiting grade II/III: 12 patients (48%); neutropenia grade III/IV: two patients; completed in all patients, except one. (2) Chemoradiotherapy: nausea/vomiting grade II/III: 10 patients; diarrhea grade II/3: two patients; oesophagitis grade II/III: two patients; myocardial infarction/pulmonary embolism: two patients. All patients except one received the planned dose of 45Gy. Radiotherapy was interrupted in six cases, with a median duration of 14 days. Survival: with a median follow-up of 30 months, 65% of the patients were alive without disease; median survival was 32 months. CONCLUSION: This postoperative schedule was judged feasible. It allowed the deliverance of a more intensified chemotherapy than the classical schedule. Its clinical benefit must be evaluated in a phase III trial.

[Multiple metastases of a mandibular ameloblastoma]. / Métastases multiples d'un améloblastome mandibulaire.

INTRODUCTION: Ameloblastoma is a benign odontogenic tumor which can be locally aggressive and invasive. Metastases are rare but possible and must be considered as a malignant form of the tumor. OBSERVATION: A 50-year-old woman presented a jugal metastasis of a mandibular ameloblastom which had been treated several times 28 years earlier. Six months after resection of the metastasis, the patient developed multiple cervical node metastases which were removed by radical curettage. Five months later, new metastases developed on the scalp and three months later a voluminous metastasis involving the right hemiface extended to the base of the skull. Surgical resection was only partial followed by radiotherapy. Ten months later the outcome was favorable. DISCUSSION: The absence of any histological sign of malignity in the primary tumor and in the metastases, as observed in our patient, is remarkable. Metastases generally develop in the lung (61-80% of cases). Metastases to the scalp have never been described. The time to development of a metastasis is generally very long and metastases usually remain asymptomatic. Progression is very slow, like for the primary tumor. Several factors predictive of metastasis have been described: female gender, age at onset of primary tumor (2nd to 3rd decade) and multiple local recurrences. There is no standard treatment for metastases. Chemotherapy is not effective. Radiotherapy may be effective, particularly when lesions are not accessible to surgery. Surgical resection remains the treatment of choice.