Myxoid esophageal liposarcoma: A case report of a rare tumor.

INTRODUCTION: Esophageal liposarcoma represent a rare cause of esophageal tumor. According to the literature, since the first case reported in 1983, only 42 cases of esophageal liposarcoma were reported. PRESENTATION OF CASE: We present a case of liposarcoma in the lower oesophagus treated by surgical resection. DISCUSSION: Liposarcoma in the oesophagus gastrointestinal tract is an uncommon. An analysis of the literature reports 42 cases.In many cases the diagnosis was established in postoperative period.Surgery is the standard treatment including polypectomy, total or subtotal oesophagectomy. CONCLUSION: There is no conventional treatment of this pathology. The curative treatment requires surgical resection or endoscopic approach for selected tumor.

Retroperitoneal extra-adrenal non-Hodgkin lymphoma: An uncommon presentation.

Primary retroperitoneal non Hodgkin lymphoma is extremely rare, its diagnosis is often difficult and it may requires a time consuming and a costly diagnostic workup. We report the case of a 46-year-old patient complaining of abdominal fullness and dorsal pain, who was diagnosed with an extra-nodal non-Hodgkin lymphoma presenting as a unique and large retroperitoneal mass. The suggested diagnosis was a malignant retroperitoneal tumor and the patient underwent an excision of the tumor throw a lombotomy followed by an R-CHOP chemotherapy regimen with good outcome.

Facteurs prédictifs de malignité des tumeurs oncocytaires de la thyroïde

INTRODUCTION : Les tumeurs oncocytaires (TO) de la thyroïde regroupent les adénomes et les carcinomes. La distinction entre malignité et bénignité constitue une difficulté de taille sur le plan histologique. De cet aspect découlent des contraintes dans l'attitude thérapeutique à adopter. L'objectif du travail était de déterminer les particularités cliniques, pathologiques et pronostiques des TO de la thyroïde et dégager les facteurs prédictifs de malignité. MÉTHODES : Etude descriptive, rétrospective portant sur 35 cas de TO de la thyroïde opérés entre 2007 et 2015. Les prélèvements ont été étudiés au service d'anatomopathologie. Une étude statistique a été réalisée à la recherche des facteurs prédictifs de mali¬gnité.RÉSULTATS : L'âge moyen des patients était de 53 ans. Le sex-ratio a été de 0,09. Le traitement était chirurgical dans tous les cas. L'examen anatomopathologique définitif a confirmé le diagnostic de 26 adénomes oncocytaires, six carcinomes papillaires on-cocytaires et trois carcinomes vésiculaires oncocytaires. La taille moyenne de la tumeur était de 38,1 mm (10-120). Une irathérapie post-opératoire a été proposée pour les carcinomes. En analyse univariée, les facteurs prédictifs de malignité étaient : l'âge supé¬rieur à 55 ans, la consistance dure, les limites floues, la double vascularisation anarchique, le caractère hypoéchogène du nodule, les microcalcifications, la colloïde sombre et dense, la capsule épaisse et la thyroïdite associée. En analyse multivariée, aucun de ces facteurs n'était prédictif de malignité.CONCLUSION : Les tumeurs oncocytaires de la thyroïde représentent une entité anatomo-clinique distincte. La détermination des facteurs prédictifs de malignité est essentielle pour adapter la prise en charge thérapeutique

Round cell sarcoma of the colon with CIC rearrangement.

BACKGROUND: The CIC-rearranged sarcoma is a very rare highly aggressive malignant soft tissue group of tumors. It has recently been described as highly aggressive soft tissue tumors of children and young adults sharing similar morphological features with the Ewing sarcoma. The digestive localization is exceptional. CASE PRESENTATION: A 14-year-old male presented with a history of abdominal pain for 1 year, which increased in intensity over the last 2 months. Imaging findings showed a large heterogeneous mesenteric mass on the left flank of the abdomen. Exploratory laparotomy was performed and revealed a large cystic hypervascularized mass depending on the transverse colon and mesocolon. A wide excision of the lesion was performed with segmental colectomy. No postoperative complications were noted. The microscopic examination revealed a vaguely nodular growth of undifferentiated small round cells, arranged in solid sheets separated by thin fibrous septa with a scarce stroma. After an uncomplicated post-operative course, the patient was referred for chemotherapy. The patient died 2 months later with a peritoneal and pleural progression. CONCLUSIONS: The CIC-rearranged sarcoma is an aggressive tumor. There is no standard therapy for this rare disease. Their treatment includes surgery and chemotherapy. Resistance to chemotherapy is common. Further publications and studies will help to determine a standard therapy for this rare disease.

Facteurs prédictifs de l'atteinte ganglionnaire dans les carcinomes papillaires de la thyroïde

Objectifs:Le but de notre étude était de spécifier le profil de l'atteinte ganglionnaire en cas de carcinomes papillaire de la thyroïde et de déterminer les facteurs prédictifs de l'atteinte ganglionnaire.Méthodes: Etude rétrospective portant sur 39 patients pris en charge pour un cancer papillaire de la thyroïde durant la période entre 2012 et 2014.Résultats: L'âge moyen de nos patients était de 42,4 ans. Le sex-ratio était de 0,13. Tous nos patients ont été opérés d'une thyroïdectomie totale associée à un curage ganglionnaire central. Un curage fonctionnel unilatéral a été pratiqué chez cinq patients. Le secteur ganglionnaire central était envahi dans dix cas et le secteur latéral l'était dans quatre cas. Après analyse des critères épidémiologiques, cliniques et anatomopathologiques, nous avons établi une corrélation significative entre les stades avancés de latumeur (T3, T4) et l'envahissement ganglionnaire central. Aucun des facteurs étudiés n'a été corrélé à l'atteinte du secteur ganglion naire latéral.Conclusions: L'atteinte ganglionnaire au cours du carcinome papillaire de la thyroïde est non négligeable affectant surtout le secteur central. Le curage central bilatéral revêt une grande importance et doit être de réalisation systématique

[About a rare tumor of the kidney: Malignant mixed epithelial and stromal tumor]. / À propos d'une tumeur rarissime du rein: la tumeur mixte épithéliale et stromale maligne.

Mixed epithelial and stromal tumor of the kidney is a recently described neoplasm that predominantly affects perimenopausal women. Few cases with malignant features have been reported. Here, we report the case of malignant mixed epithelial and stromal tumor of the kidney with sarcomatous transformation arising in a 27-year-old female. She presented with abdominal discomfort. Computed tomography of the abdomen revealed a large compressive mass arisen from the left kidney with solid and focal cystic components. The patient underwent left radical nephrectomy. Histologic sections showed benign and malignant components. The benign component consisted of multiple tubules and variably sized cysts lined by benign epithelium. The malignant component was composed of undifferentiated cellular spindle cell sarcoma. By immunohistochemical studies, the epithelial component was positive for cytokeratins and epithelial membrane antigen (EMA). The stromal component displayed strong immunohistochemical expression of vimentin, CD99, bcl2; and was negative for cytokeratins, desmin, SMA, S-100, estrogen receptor (ER) and progesterone receptor (PR). Analysis by reverse transcriptase polymerase chain reaction (RT-PCR) failed to identify the SYT-SSX1 or SYT-SSX2 fusion transcripts characteristic of synovial sarcoma.

[Paraganglioma of spermatic cord]. / Paragangliome du cordon spermatique.

Paragangliomas can exist anywhere in the distribution of neural crest derived cells. In the spermatic cord, they are exceedingly rare. We report a case of histologically discovery, occurring in a man of 50 years who had a para-testicular mass, painless, firm without other signs associated. In view of this observation and a review of the literature, we propose to establish the epidemiological profile and study the prognostic and the clinicopathologic features of this tumor.

[Sweet's syndrome: retrospective case series of 47 patients]. / Syndrome de Sweet : étude rétrospective de 47 cas.

PURPOSE: Also called acute febrile neutrophilic dermatosis, Sweet's syndrome is an inflammatory disorder with a prominent cutaneous expression. It is characterized by a variety of manifestations, clinical and histological findings. The objective of this study was to describe their clinical, pathological and therapeutic characteristics. METHODS: We report on a series of 47 patients who presented a Sweet's syndrome, collected in our institution in Tunis between 1997 and 2011. RESULTS: The patient population consisted of 11 men and 36 women. The mean age was 47 years with extreme ranging from 28 to 74 years. An associated disorder was observed in ten patients: inflammatory disease (three cases), inflammatory bowel disease (two cases), tuberculosis (three cases) and diabetes (three cases). One case of pregnancy was observed. Cutaneous lesions consisted of erythematous plaques or nodules. Lesions were located mainly on the upper or lower extremities. All biopsy specimens demonstrated a dermal infiltrate composed predominantly of neutrophils. Fibrinoid necrosis and intramural inflammation were observed in eight cases. CONCLUSION: The skin disorder can precede, follow, or appear concurrent with the diagnosis of an associated disease which requires careful surveillance.

[Pediatric mandibular ameloblastic fibro-odontoma]. / Fibrome odonto-améloblastique de l'enfant.

INTRODUCTION: Ameloblastic fibro-odontoma (AFO) is a very rare mixed odontogenic tumor (2% of all odontogenic tumors). It is considered as a non-extensive non-aggressive tumor but recurrence and malignant transformation remain possible. We report a voluminous AFO in a child. CASE REPORT: A 7-year-old boy presented with a right mandibular and facial swelling. The panoramic radiograph and CT scan revealed a voluminous unilocular radiolucent lesion (10 cm) with an impacted tooth that involved the ramus area and the angle of the right mandible. Enucleation and curettage was performed with a good outcome. There was no recurrence at ten months. DISCUSSION: This case is exceptional because of the young patient's age, the large tumor diameter, and the good outcome after ten months. This tumor occurs more often in the posterior region of the mandible and is frequently seen in the first two decades of life. The diagnosis is made on radio-clinical data and may be confirmed by histology. A surgical treatment with complete tumor removal is recommended to prevent recurrence.

[Primary mediastinal sarcomas]. / Les sarcomes primitifs du médiastin.

INTRODUCTION: Primary sarcomas of the mediastinum are rare and account for 2 to 8% of malignant mediastinal tumours. The aim of this study is to describe their clinical and pathological characteristics. PATIENTS AND METHODS: Fifteen cases of primary mediastinal sarcoma, diagnosed between 1993 and 2009, were reviewed retrospectively, noting the clinical, radiological and pathological findings, and the treatment given. RESULTS: The patient population consisted of eight females and seven males with mean age of 40 years (14 to 73 years). The symptomatology was predominately respiratory. Imaging showed a mediastinal mass invading adjacent organs in nine cases. The diagnosis was made in all cases by histological examination. These 15 mediastinal sarcoma comprised 12 malignant peripheral nerve tumours, two liposarcomas and one angiosarcoma. Three were grade III, six grade II and six grade I. Ten were treated surgically, of which seven had radical resections. Associated treatments were neoadjuvant (one case) or adjuvant (one case) chemotherapy and postoperative radiotherapy (five cases). Radiotherapy was undertaken alone in three inoperable tumours. Eight patients (53%) had died. CONCLUSION: Management of primary mediastinal sarcoma needs a multidisciplinary approach, and is based mainly on radical resection. The prognosis is poor and depends mainly on surgical excision and histological grade.