Spindle cell carcinomaof the larynx: A case report and literature review.

Introduction: Spindle cell carcinoma (SCC), also called sarcomatoid carcinoma or carcinosarcoma, represents a rare variant of squamous cell carcinoma that may involve multiple organs, including the larynx. It is a mixed tumor, composed of both malignant epithelial and mesenchymal elements. As the larynx appears to be an unusual site, we report a case of treated SCC laryngeal as well as a literature review of such tumor. Observation: A 72-year-old male, with 2 months' history of a progressive worsening dysphonia, was examined. The patient examination showed no cervical adenopathy, while direct laryngoscopy detected a voluminous budding formation of the anterior commissure. A biopsy was performed, in which an undifferentiated carcinoma was identified. The computed tomography (CT) confirmed the existence of a glottic tumor, which completely obstructs the lumen of the larynx. No metastasis were noticed elsewhere on the PET-CT. The tumor was then classified as T3 N0 M0. The patient underwent a total laryngectomy, thyroidectomy and neck dissection. The definitive histopathological analysis confirmed the diagnosis of SCC of the larynx with negative surgical margins. Afterward, the patient underwent adjuvant radiotherapy (RT) on the tumor bed. Conclusion: Primary laryngeal SCC is a very rare malignancy with a tendency to occur with controlateral lymph node metastasis at an early stage of the disease. Combining surgery and postoperative RT is recommended due to its aggressive profile.

Spontaneous regression of an orbital Langerhans cell histiocytosis after biopsy: A case report. Spontaneous regression of an orbital Langerhans cell histiocytosis.

Langerhans histiocytosis or Langerhans cell histiocytosis (LCH) is a rare benign pathology representing less than 1% of orbital tumors. It can cause either localized or generalized lesions, leading to the destruction of hard and soft tissues. Eosinophilic granuloma is the most benign form and the predominant clinical presentation of LCH. We report a case of eosinophilic granuloma with orbital involvement in an 18-year-old male patient. Orbital radiotherapy was initially planned, but finally it was not performed due to a spontaneous regression of the lesion after the incisional biopsy. The presented case supports an expectant attitude given the possibility of a spontaneous regression after the biopsy, especially in small lesions. However, long-term follow-up is essential given the risk of recurrence.

Li-Fraumeni syndrome in Tunisian carriers with different and rare tumor phenotype: genotype-phenotype correlation.

BACKGROUND: Li-Fraumeni syndrome (LFS) is a rare autosomal hereditary predisposition to multiples cancers, mainly affecting young individuals. It is characterized by a broad tumor spectrum. To our best knowledge, only one Tunisian study with a confirmed LFS was published. METHODS: Our study focused on the clinical, histopathological and genetic results of two patients with rare tumor phenotype and tried to establish genotype-phenotype correlation. The clinical diagnosis was based on Chompret-Bonaiti criteria relative to LFS. Molecular study was assessed using Sanger sequencing of the hotspot germline variants of TP53 gene. RESULTS: We report 2 Tunisian families fulfilling the clinical criteria of Chompret-Bonaiti. The tumor phenotype was bilateral breast cancer (BC) in 27-year-old woman and multiple tumors for the second proband, with an onset age of 14, 35 and 36 yo for osteosarcoma, BC and esophageal cancer respectively. Each of them had a rare histological type of breast cancer associated with LFS, phyllode tumor and intralobular carcinoma. Both patients had cancer family history. The molecular study showed deleterious heterozygous germline TP53 variants in each index case: The first had a well-known hotspot missense variation c.742C>T p.(R248W) with a rare histological association, explaining genotype phenotype correlation. The second case had a nonsense variation c.159G>A p.(W53*), rare worldwide, extending the phenotype spectrum in LFS. Immunohistochemistry study in tumor samples confirmed the lack of p53 protein expression. CONCLUSIONS: Conclusively, germline TP53 testing is primordial in patients with a family history suggestive of LFS for clinical practice avoiding genotoxic treatments and adapting the surveillance. National database in LFS listing clinical and mutational data is important to set, particularly for variants rarely reported worldwide. Experience from different countries must be integrated to harmonize global protocols for cancer surveillance in LFS.

Survival and prognostic factors in adult medulloblastoma: the Salah Azaiz Institute experience.

BACKGROUND: Medulloblastoma is the most common malignant brain tumor in children. This entity in adulthood is rare. The aim of our study is to evaluate therapeutic results and prognostic factors of adult medulloblastoma treated at our institute with post-operative radiotherapy. METHODS: We retrospectively reviewed a cohort of 55 patients with medulloblastoma who underwent radiation in the department of radiation oncology of institute Salah Azaiz (Tunis) over a 18-year period (1994-2012). RESULTS: The surgery was total or subtotal resection in 73% of cases. Forty-eight patients received radiotherapy to the entire craniospinal axis as part of the curative treatment. The median interval from surgery to the initiation of radiotherapy was 83 days. Etoposide-cisplatin chemotherapy was only performed in metastatic patients (n = 4). The 5-years and 10-years overall survival rates were respectively 53 and 34%. The dose of radiotherapy to the craniospinal axis was a prognostic factor. The 5-years and 10-years event-free-survival rates were 64 and 41%. Reduction in the dose of radiotherapy to the craniospinal axis and fourth ventricular floor involvement were correlated with a worse event-free survival. CONCLUSION: Our results, compared to those of the literature, conclude that the reduction in the dose of radiotherapy to the craniospinal axis (<34 Gy) in the standard risk group of adult medulloblastoma could not be done without chemotherapy. In the high-risk group of adult medulloblastoma, radiotherapy to the cerebrospinal axis at the dose of 36 Gy with chemotherapy, is required for disease control.

Management of patients with cardiac implantable electronic devices (CIED) undergoing radiotherapy: A consensus document from the Tunisian Society of Oncology Radiotherapy and the Tunisian Society of Cardiology and Cardiovascular Surgery.

In patients with cardiac implantable electronic devices CIEDs, including cardiac pacemakers (PM) and implantable cardioverter-defibrillators (ICD), radiotherapy (RT) could compromise CIED function. Managing radiotherapy patients with CIED, has been a great practical and procedural challenge in radiotherapy and requires a structured multidisciplinary approach. A consensus document is presented as a result of a multidisciplinary working group involving cardiac electrophysiologists, Radiation Oncologists and Medical physicists. It aims to propose recommendations on risk stratification, management approach before, during and after radiation treatment/course of patients with CIED.

Primary Paratesticular Leiomyosarcoma: A Case Report and Literature Review.

Paratesticular soft tissue sarcomas are very rare malignant mesenchymal tumors. With only few cases reported in the literature, data regarding diagnostic and management of these tumors are limited. We reported a case of primary paratesticular leiomyosarcoma in a 72-year-old man complaining of a progressively growing painless right scrotal mass. The patient underwent radical inguinal right orchiectomy and adjuvant 3D conformal radiotherapy to the tumor bed including the surgical scar. The prescription dose was 54 Gy, and no pelvic irradiation was performed. He remained free of recurrence for the last 16 months.

Metastasis of colon adenocarcinoma to the mandible: A case report.

INTRODUCTION: Metastasis is the primary cause of death among patients with colon cancer. Metastatic tumors in the oral cavity originating from the colon are rare, and the number of relevant clinical studies is limited. CASE REPORT: We report a case of a 79 year old woman who developed a mandibular tumefaction. Biopsy was performed and made the diagnosis of metastasis from colon cancer. The patient received palliative radiotherapy for the mandibular mass lesion. CONCLUSION: Oral metastatic tumor from colorectal cancer is very uncommon and is often found with advanced recurrent cancer. Therapeutic management that includes palliative treatment is the usual therapeutic option.

Kaposi's sarcoma of the larynx: an unusual location in an HIV-negative patient (a case report).

Head and neck involvement of Kaposi's sarcoma is rarely encountered, especially for the Mediterranean classic subtype. Here we report a case of non-AIDS related laryngeal Kaposi's sarcoma in a 77-year-old Tunisian man complaining of 4-month history of hoarseness and dysphagia. The patient underwent exclusive local radiotherapy with a prescription dose of 45 Gy delivered in 1.8 Gy daily fractions. He remained complaint-free for 3 months.

Brachytherapy of Uveal Melanomas with Ruthenium-106 Plaques

Background: Brachytherapy is the most commonly used conservative treatment for the uveal melanoma. The aim of this study was to evaluate therapeutic results of Ruthenium-106 plaque brachytherapy in the management of localized uveal melanoma cases. Methods: We reviewed retrospectively the clinical records of all patients treated in our department for an uveal melanoma, undergoing Ruthenium-106 plaque brachytherapy, from January 1996 to December 2015. We focused on clinical features, therapeutic characteristics, local and distant tumor control and side effects. Results: Nineteen patients were enrolled in our study. Mean age was 56.2 years (28-79) and the sex ratio was 1.37:1 males to females. Diagnosis was made on the basis of ophthalmological clinical examination, angiography, ultrasound and/or magnetic resonance. Median tumor diameter was 9.7 mm (6-13) and median thickness 4.4 mm (2.5- 8). The dose of Ruthenium-106 plaque brachytherapy prescribed to the apex of each tumor was 70 Gy in all cases. The median radiation dose to the sclera surface was 226.4 Gy (range: 179.6­342.3) and the median total application time 115.2 hours (range: 27 to 237). After a median follow-up of 61.5 months, local control was achieved in 17 patients (89%): 16 demonstrated a partial tumor response and 1 tumor stabilization. Two patients suffered local progression leading to enucleation, one dying of hepatic metastasis. Radiation-induced complications were cataracts in 3 cases and vitreal hemorrhage in 2. Conclusion: Ruthenium-106 plaque brachytherapy is an efficient treatment for localized uveal melanoma, offering good local control with low toxicity.

Sternal or parasternal involvement from breast cancer: a misleading clinical sign.

BACKGROUND: Sternal arch is a nonspecific clinical signs wich may arise during following of breast cancer, this sign reveals an involvement of internal mammary nodes secondary to breast cancer. AIM: To report a rare event such as a loco-regional sternal or parasternal invasion secondary to locally advanced cases of breast cancer (BC) about a Tunisian series. METHODS: We collected retrospectively from 1988 to 2012, 11 cases of BC treated at the Institut Salah Azaiez (ISA) of Tunis, with presence during the disease evolution (initial or at relapse) of a sternal or parasternal swelling. We analyzed their clinical history, clinical and imaging data (CT-scan and/or MRI), stage, time of occurrence, treatment and evolution RESULTS: All patients were females and their mean age was 46 years varying from 24 to 75 years. The sternal or parasternal swelling was found at diagnosis in 5 cases and on recurrence in 6 patients after a mean free interval of 24 months, > 12 months in 5 cases. The mean clinical tumor size of the primitive BC was 38 mm (24-75 mm) and lesions located in external quadrants in 3 cases and internal or central in 6 cases. Sternal involvement related to large Intrammmary Chain (IMC) adenopathies was diagnosed by CT-scan. 7 patients had synchronous metastases. 9 out of the 11 patients received a locoregional RT and two received chemotherapy (CT). CONCLUSION: Sternal or parasternal swelling is a clinical apparent symptom of advanced internal mammary lymph nodes in breast cancer. The first etiologic diagnosis in this context is an advanced internal mammary chain (IMC) node involvement from breast cancer.

[Internal mammary lymph node invasion in breast cancer: myth or reality?]. / L'atteinte de la chaîne mammaire interne dans le cancer du sein: mythe ou réalite?

The internal mammary nodes are often underestimated as breast cancer lymphatic pathway spread. It is yet the first site of lymphatic invasion in central or internal tumors and the second site in external tumors. The intra-thoracic situation of internal mammary nodes explains partly, the difficulty of its exploration. To evaluate the risk of internal mammary node invasion, some predictive factors are established (tumor size, internal or central tumor location, axillary node invasion and young age). Prognostic and therapeutic impact of invasion justifies its systematic research. Without exploring internal mammary nodes status, TNM classification remains, incomplete. CT scanner, magnetic resonnance imaging, positron emission tomography scanner and sentinel node exploration technique are helpful to explore this region and to adapt its irradiation.

[Medulloblastoma with extracentral nervous system metastases: clinical presentation and risk factors]. / Medulloblastoma with extracentral nervous system metastases: clinical presentation and risk factors.

PURPOSE: Extra-central nervous system (extra-CNS) metastases are relatively unknown failure patterns in medulloblastoma. The aim of this study was to analyse epidemiological, clinical and aetiopathological aspects of these extra-CNS localisations. PATIENTS AND METHODS: Extra-CNS metastases were retrospectively identified in patients treated in the department of radiation therapy at Salah-Azaïz institute (ISA) for medulloblastoma. These metastases were diagnosed as extra-CNS for all secondary localisations not related to other tumour aetiology. Aetiopathological aspects are discussed with a literature review. RESULTS: Among 103 patients treated and followed-up in the department of radiation therapy of ISA from 1970 to 1992, 8 developed extra-CNS metastases (7.7%). Age at diagnosis of primitive tumour varied from 3 to 23 years. Sex ratio was 1. Primitive tumour treatment was: complete surgical resection in 4 patients with preoperative cerebrospinal fluid shunting in two, cerebrospinal axis irradiation in 7 patients and a cerebral-limited irradiation in 1. Two patients received chemotherapy for their initial treatment (systemic in one case and intrathecal in the other). The mean free-interval from diagnosis of primitive tumour to extra-CNS metastases was 23 months, varying from 8 to 53 months. These metastases were located in the liver (1 case), cervical lymph nodes (2 cases), bone marrow (1 case) and bone (2 cases). Two patients had multiple metastases: bone and bone marrow (in one), lung, pleura, cervical lymph node and bone localisations (in one). Treatment of these metastases was: chemotherapy in 5 cases, chemotherapy and radiation in one, radiation therapy in one and 2 patients were given only supportive care treatment. All patients died or are in progressive disease in less than one year from the diagnosis of extra-CNS metastases. CONCLUSION: Extra-CNS metastases are not rare and have a poor prognosis. The most commonly involved sites are bone, cervical lymph nodes and bone marrow. A complete work-up at initial diagnosis is recommended to screen early metastases. Literature review showed that histopathologic grading might help to identify groups at risk.

Radio-induced malignancies of the scalp about 98 patients with 150 lesions and literature review.

INTRODUCTION: - The induction of malignant diseases is one of the most concerning late effects of ionizing radiation. The topic of this study deals with skin tumors developed in the irradiated areas in children given X-ray therapy for tinea capitis. MATERIAL AND METHODS: - All patients with malignant tumors of the scalp referred to Salah Azaiz Institute between 1970 and 2001 have been questioned in order to determine if there had been a prior X-ray irradiation for tinea capitis, its modality, and its consequences. The first scalp irradiation goes back to 1922 and the last was performed in 1963. RESULTS: - Ninety-eight patients with 150 radio-induced cancers of the scalp following irradiation for tinea capitis are reported (1.5 lesion per patient). The patients were irradiated in various hospitals and dispensaries throughout the country. Eighty-one patients (82%) had only one session of radiation. The average age at irradiation was 12 (+/-6) years, the latent period for radiation-induced skin cancers was 36 (+/-14) years. In 61 patients (62%), the scalp appeared normal and in 38% radiodermatitis was noted. Patient age at diagnosis of malignancy varied from 20 to 83 years with an average of 47 years. Basal cell carcinomas (125 cases) and spinocellular carcinomas (16 cases) were the most common, three other cases of annexial tumors, two malignant non-Hodgkin's lymphomas and four melanoma lesions are also present. Radiotherapy was used for the treatment of 74 patients (alone in 42 and associated with surgery in 32 patients); 14 patients had exclusive surgical excision. CONCLUSIONS: - Basal cell carcinomas are the most frequent tumors arising on chronic radiodermatitis. In spite of the long latency period, patients' young age at irradiation explained the occurrence of these cancers at a relatively young age. Literature review is suggesting recessive mutation of tumor-suppressor genes as the characteristic abnormality in radio-induced cancer.