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.

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.

[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.