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.

[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 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.

[Clear cell carcinoma of the parotid gland]. / Carcinome à cellules claires de la glande parotide.

OBJECTIVES: Clear cell carcinomas of the parotid gland are hardly reported only fifty cases are known. They are characterized by a proliferation of acinic epithelial cells and of clear myo epithelial cells. What makes them remarkable is a slow process of evolution. MATERIAL AND METHODS: Three cases which were treated in the Ibn Rochd center of oncology are gathered from the 1999-2003 period into a review in order to discover the epidemic and anatomopathological characteristics of these tumours. The goal of study is to contribute to a best knowledge of the clinical features, pare clinical, anatomopathological and therapeutic characteristic of this tumour. RESULTS: Two men, aged of 62 and 71 and a 82-years-old-woman were concerned. Growing cervical mass was the most revealing symptom. The diagnosis was established by the parotid biopsy in 2 cases and after surgery in the third case. The treatment consisted in a total parotidectomy with conservation of the facial nerve and postoperative radiation at the extend of 65 Gy for the first patient. After 12 months the patient is still alive. As for the two other patients, given the extend of advancement of the tumour, palliative treatment was decided. It resulted in stabilization of the disease and a receding of 22 months for one patient and no trace of the second one because of a loss of the evolutionary pursuit. CONCLUSION: With an in-depth analysis we can notice that clear cell carcinomas of the parotid gland are rare and mostly occur to old patients. Immunohistochemistry is the essential first step to sort these tumour with other salivary ones. Basic treatment is surgery. Radiation therapy linked to surgery seems to improve the local control of the disease. The prognosis remains relatively good despite discusses. Because of the few published cases, it's hard to analyze these tumours.

[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.

[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.

[Vaginal leiomyosarcoma. Apropos of 2 cases and review of the literature]. / Léiomyosarcome du vagin. A propos de deux cas et revue de la littérature.

The leiomyosarcoma of the vagina is extremely rare. This paper reports two observations treated at the Ibn Rochd oncology center of Casablanca. The first patient was 32 years old, and presented a tumor of 15 cm to the detriment of the posterior wall of the vagina. The treatment consisted of a large exeresis of the tumor followed by postoperative radiotherapy. After 50 months, she is still alive. The second patient who was 42 years old, had a 10 cm tumor of the posterior wall of the vagina. A tumorectomy was realized and then the patient was not heard of. The evolution was marked by the appearance of pulmonary metastasis and a palliative chemotherapy was prescribed. This patient is deceased after 31 months. Seventy-five cases only are described in the literature. The surgery is the basic treatment whereas radiotherapy is still discussed. The prognosis of these tumors remains linked especially to the histological rank.

[Adenocarcinoma of the cervix uteri: a retrospective study of 83 cases]. / Adénocarcinome du col utérin: étude rétrospective de 83 cas.

UNLABELLED: The frequency of the adenocarcinoma of cervix uteri is increasing. AIM OF THE STUDY: To define the characteristics of the entity. PATIENTS AND METHODS: Between 1986 and 1992, 83 adenocarcinoma of cervix uteri, were treated at the Ibn Rochd Oncology Centre in Casablanca. It's a retrospective study with univariate analysis of the prognostic factors. This analysis was done for patients who were followed until the death or have 5 years or more follow up and the survival was calculated according the Kaplan & Meier method at 5 years. The local and regional recurrence factors were evaluated statically according the chi 2 and those for survival rate according the Logrank test. RESULTS: The frequency increased during the period with 2.1% in 1986 and 8.2% in 1992. The median of aged was 49 years. Fifty one patients have localised stage. Combined radiotherapy and surgery was performed in 66 cases and 17 received radiation alone. The loco-regional control was obtained in 83% among 73 available patients for results. The univariate analysis showed that the histological type was not a significant factor. However, age, tumor size, stage, histological grade and lymph node involvement were significant factors. These factors, the degree of differentiation and the lymph node involvement were significant for the 5-years survival. CONCLUSION: The prognosis factors of the cervix uteri adenocarcinoma are, size tumor more than 4 cm, lymph node involvement, microscopic involvement to the parameters and positives excision merges.

[Nephroblastoma in adults. Three case reports]. / Le néphroblastome de l'adulte. A propos de trois cas.

The authors report three nephroblastoma's cases occurred in adult and treated at Ibn Rochd Oncology Center at Casablanca. The average of age was 24 years (19-29 years) and the delay of diagnosis was five months (3-8 months). The most frequent clinical sign was a lumbar fossa mass. At diagnosis, radiological exams revealed that patients presented an advanced stage and metastatic disease in one case. The treatment was a total nephrectomy with ganglioma dissection when the tumor was resectable followed by chemotherapy and radiotherapy. Two patients was in progressive disease despite treatment, and one patient was lost at follow-up. The prognosis of adult's nephroblastoma remains very severe, due to the advanced stage at diagnosis and the mediocre reply to the treatment.

[Pure choriocarcinoma of the testis: report of a case and review of the literature]. / Choriocarcinome pur du testicule: à propos d'un cas avec revue de la littérature.

We report a case of a young man 31 years old treated at the Ibn Rochd Oncology Center for a pure choriocarcinoma of the right testis. The first examination note a skin metastasis without another localization. The beta HCG level was 328 mu/mL. The diagnosis was confirmed by pathological examination of the testis after orchidectomy. The adjuvant treatment consisted in chemotherapy like using in germ cell neoplasm's of the testis. During the evolution, partial remission (50%) was obtained after the third course and complete remission one month after the end of treatment. The patient still alive after 20 months.

[Clear-cell carcinoma of the larynx: review of the literature apropos of a case]. / Carcinome à cellules claires du larynx: revue de la littérature, à propos d'un cas.

Clear cell carcinoma of the larynx is exceptional. Only six cases are described in the literature. We report a new case occurring in a 58-year-old man. The treatment consisted of a total laryngectomy with lymph node dissection followed by adjuvant irradiation. Local and regional recurrence occurred after 5 months. The patient died from the tumor's evolution 12 months after the diagnosis. The prognosis of clear cell carcinoma of the larynx is similar to the clear cell carcinoma of the lung and is unfavorable.

[Primitive seminoma of the mediastinum: a case report]. / Séminome primitif du médiastin: à propos d'un cas.

In this study, the case has been reported of a 36-year old male who was treated at the Ibn Rochd Oncology Center in Casablanca for a primary mediastinal seminoma revealed by a symptomatology including cough, dyspnea, laterocervical swelling, rachidial pain and gait disorder. The preliminary investigation showed significant mediastinal enlargement with a right pleuritis and vertebral metastases; tumor markers were normal. The diagnosis of seminoma was confirmed by pathological and immunohistochemical analysis of the cervical adenopathy. Disease management consisted of BEP/cisplatin type chemotherapy and lumbar, mediastinal, and supraclavicular radiotherapy. The response after four courses of combined chemo-/radiotherapy was estimated at 25%, but the patient died from respiratory failure five months after the initiation of treatment.

[Association of fallopian tube cancer and polymyositis. Apropos of 1 case]. / Association d'un carcinome tubaire primitif et polymyosite. A propos d'un cas.

A 51-year-old woman consulted for pelvic pain, metrorrhagia and leukorrhea. Physical examination revealed a renitent and mobile mass in the pelvis. A right lateral uterine mass with hydroxalpinx was found at ultrasonography. Pathology examination of the right annexectomy specimen provided the definitive diagnosis: fallopian tube cancer with polymyositis. No residual tumor was found at total hysterectomy with total bilateral annexectomy. The patient was lost to follow-up for three years without complementary treatment then consulted later for functional disability of the upper then lower limbs with myalgia, swallowing disorders and left supraclavian node enlargement resulting from pelvic relapse of the right fallopian tube adenocarcinoma and left supraclavian metastasis with paraneoplastic polymositis. The patient was given 6 courses of chemotherapy with radiotherapy (45 Gy) centered on the left clavian region. The patient exhibited a spectacular response, and remains in complete remission 50 months after diagnosis. The association of a fallopian tube tumor with polymyositis is exceptional, requiring rapid anticancer treatment effective against the cancer and the paraneoplastic polymyositis.

[Papillary carcinoma from the thyroglossal duct cyst: review of the literature and report of a case]. / Carcinome papillaire sur kyste du tractus thyréoglosse: revue de la littérature et à propos d'un cas.

Cancer of a thyroglossal duct cyst is very rare. Clinical presentation is identical that of a benign cyst, and the diagnosis is histopathological. We report the case of 36 year-old woman treated for papillary carcinoma of a thyroglossal duct cyst. The treatment was surgical excision according Sistrunk method. There was no distant spread. After 22 months follow up, the patient is alive, with no signs of recurrence.

[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.

[Liposarcoma of the larynx. Review of the literature apropos of a case]. / Liposarcome du larynx. Revue de la littérature à propos d'un cas.

Liposarcoma of the larynx is an uncommon tumor. Only 27 cases have been described in literature. We report a new case occurring in a 50-year-old patient. The treatment consisted of a total laryngectomy with lymph node dissection followed by adjuvant irradiation. Prognosis for laryngeal liposarcoma is better than that of non laryngeal liposarcoma. This tumor is at high risk of local recurrence and seldom has metastatic potential.

Nasopharyngeal carcinoma in children under 15 years of age: a retrospective review of 65 patients.

BACKGROUND: Nasopharyngeal carcinoma constitutes comprises up to 5% of primary childhood cancers but literature lacks modern uniformly treated large series. PATIENTS AND METHODS: A retrospective review was performed of sixty-five previously untreated patients younger than 16 years of age diagnosed and treated at the Ibn Rochd Centre in Casablanca between 1988 and 1992. Forty-four percent of them were stage T3 to T4 and 66% stage N2 or N3. All patients were irradiated. Prior adjuvant chemotherapy was administered in 33 patients. Thirteen patients were lost to follow-up. RESULTS: Local control was obtained in 85% of the 52 evaluable patients. The five-year overall survival was 42% and disease-free survival 38%. Ten relapses occurred at local and/or regional sites. Six patients have distant metastases. All 24 patients with relapse or persistent disease died despite salvage therapy. Stage, histology and dose of radiation, were statistically significant prognostic variables. Patients treated with chemotherapy followed by irradiation had a better outcome than those treated with radiation alone. CONCLUSIONS: Nasopharyngeal carcinoma in children is a rare chemosensitive tumor. However, conclusive treatment guidelines cannot be drawn from this series and prospective co-operative studies are needed for the development of more effective and less toxic therapeutic strategies.

[Preoperative radiotherapy of rectal cancer. The Lyons experience, 1985-1996. Prognostic study apropos of 312 patients]. / La radiothérapie pré-opératoire du cancer du rectum. Expérience lyonnaise de 1985 à 1996. Etude pronostique à propos de 312 patients.

AIM OF STUDY: Preoperative radiotherapy is used increasingly in rectal cancer in Europe. This study is a retrospective analysis of a series of 312 patients with rectal adenocarcinoma treated by preoperative radiotherapy. MATERIAL AND METHOD: From 1985 to 1996, 312 patients were included in this study. Preoperative staging was: T2: 83, T3: 192 et T4: 21. On digital rectal examination, 25 patients were classified as N1. Endorectal sonographic staging was: uT1: 3, uT2: 77, uT3: 163, uN0: 122, uN1-2: 127. After surgery, pathological staging was: pT0: 43 (14%), pT1 24, pT2: 81, pT3: 151, pN0: 229, pN1-2: 81. Radiotherapy was delivered to the posterior pelvis with an accelerated schedule 39 Gy/13 fractions/17 days with x 18 MV. RESULTS: Median follow-up is 54 months. For pM0 patients (297 patients), the overall 5-year survival rate is 67%. Local failure rate is 9%. Since 1986, the rate of sphincter saving surgery is close to 65%. Various parameters related to the tumor were found to be significant prognostic factors on multivariate analysis in relation to 5-year overall survival rate: the T stage as judged by digital rectal examination and endorectal sonography, the N stage as evaluated on digital rectal examination but not with endorectal sonography. Pathological examination of the operative specimen retains a very strong prognostic value for pT and pN. CONCLUSION: Pathological examination of the specimen of rectal carcinoma retains a very strong prognostic value after preoperative radiation therapy. Endorectal sonography is of interest to evaluate T staging of the tumor but is not reliable for N stage.

[Chondrosarcoma in Ollier's disease. Apropos of 2 cases and review of the literature]. / Les chondrosarcomes sur maladie d'Ollier. A propos de deux cas et revue de la littérature.

Ollier's disease is a rate affliction (not more than approximately 100 cases have been reported over a 30-year period) characterized by bone dysplasia in which evolution to degenerative neoplasia has been described. Diagnosis of chondrosarcoma is delicate in these cases. It is a kind of slowly evolving low grade malignant tumor. Surgery is the primary treatment course, and can lead to remission. Prognosis, depending on the number and dimension of the lesions, their localization and metastasis prevention, is usually good, but relapses are relatively frequent: 25 to 50%. In case of relapse, radiotherapy and chemotherapy were tested but their efficacy is limited. We report here two clinical cases and discuss literature data.