BACKGROUND: Ewing\'s sarcoma usually presents in paediatric patients with its primary location being bone tissue. Nevertheless, we present such an adult case which arises from the small intestine. We registered thirty one cases of such origin published so far excluding ours.
METHODS: We report a case of 30 year old female who was admitted due to the persistent anaemia. Whole body computed tomography scan revealed abdominal mass in her left upper abdominal compartment. Surgery on the mass originating from jejunum was performed, although due to extremely complicated postoperative period and rapid dissemination no additional therapy had been performed. The tumour was positive for CD99, ERG, CD56, Synaptophysin, PanCK, Cam5.2.
CONCLUSIONS: Extraosseus Ewing\'s sarcoma is extremely rare entity, with poor prognosis.

Primary Ewing sarcoma (ES) of the lung is anecdotally rare, with few cases reported in literature. This report describes a 46 year-old man who presented with cough and chest pain. CT Thorax revealed a lesion in the right lung. Ultrasound guided fine-needle aspiration of the mass and subsequent cytologic examination exhibited a small round cell morphology. Immunohistochemistry done on the cell block revealed CD 99 and FLI-1 positivity, confirming the diagnosis of ES. FISH supported the diagnosis, showing the EWSR1 rearrangement. Radiologic investigations ruled out a primary tumour elsewhere, confirming the diagnosis of primary pulmonary ES. The patient was started on chemotherapy.

Lymphoblastic lymphomas (LBLs) are uncommon malignant neoplasms derived from immature T- or B-lymphoid progenitor cells. Although cutaneous involvement may reach 33% in B-LBL, only 12 cutaneous cases of T-LBL have been published. We report the case of a 49-year-old woman with 2-month history of erythematous-violaceous plaques in the sternal region and breasts. Histopathologic examination showed a dense monomorphus infiltrate in dermis and positive immunostainings for CD3, CD99 and terminal deoxynucleotidyl transferase, thus indicating T-LBL. Staging work-up only revealed a mediastinal mass at diagnosis. After a 51-month follow-up and different treatment regimens, the patient remains alive although she has presented four relapses, all of them extramedullary.

Solitary fibrous tumor is an uncommon neoplasm with unpredictable clinical behavior. Malignant solitary fibrous tumor is a rare morphological variant with more aggressive behavior and higher rates of local recurrences and distant metastasis, exceeding rare in oral cavity; our case occurred in the floor of the mouth in the sublingual gland.

Rare cases of Ewing\'s sarcoma/primitive neuroectodermal tumors (EWS/PNETs) arising from mesenteric tissue have been reported. This report describes an EWS/PNET in a 25-year-old woman who presented with abdominal pain lasting 3 days. Radiologic evaluation revealed a 9 cm × 6 cm homogeneous mass in the lower abdomen with homogeneous enhancement and invasion of the ileum. Surgical resection was completed during exploratory laparotomy. Immunohistochemically, the tumor cells revealed CD99, friend leukemia virus integration-1 and NKX2.2 (NK2 Homeobox 2, a protein coding gene) and subsequently showed EWSR1 rearrangement. The histological feature, immunohistochemical results and genetic fluorescence in situ hybridization analysis of this case were confirming the diagnosis of EWS/PNET. Adjuvant chemotherapy was suggested, but the patient was lost to follow-up.

暂无摘要。

Mesenchymal chondrosarcoma (MC) is a rare malignant neoplasm bearing characteristic dimorphic pattern histologically. We describe two rare cases of primary MC involving two different visceral organs (1) a 24-year-old man with solid renal mass and, (2) a 42-year-old man with cystic splenic mass. The histological and immunophenotypical features of both lesions were classical of MC. Although this lesion is uncommon in visceral organs, the possibility of this rare entity must be kept in differential diagnosis with compatible morphology.

BACKGROUND: Peripheral primitive neuroectodermal tumors are extremely rare tumors in the spine; only 18 cases of extra-dural peripheral primitive neuroectodermal tumor cervical region have been reported. The aim of this report is to highlight the challenges in diagnosis and management of this condition.
METHODS: We present a case of 5-year-old Moroccan boy, who presented with torticollis for 1 month. Computed tomography scan and Magnetic resonance imaging of the cervical spine revealed an extradural, dumbbell-shaped mass with extra-spinal extension at the left C1-C6 level. Multiple biopsy specimens were obtained. Histological examination revealed a highly cellular neoplasm composed of diffuse sheets of tumor cells having monomorphic, round to oval, finely vesicular nuclei. Immunohistochemical findings confirmed the diagnosis of intraspinal peripheral primitive neuroectodermal tumor.
CONCLUSIONS: After this illustrative case, we review the literature on clinicopathological and therapeutic aspects. In practice, it is important to consider the diagnosis of peripheral primitive neuroectodermal tumor in children and adolescents with an apparent soft-tissue mass located in the spine.

Although multiple primary malignancies are relatively rare, they have increased in frequency over the last decades, partly because of advances in diagnosis and therapy. This report describes for the first time the case of a patient with past occupational exposure to asbestos and no family history of cancer who developed 2 rare primary malignancies: a cardiac sarcoma and a gliosarcoma 11 months later. Molecular-cytogenetic studies did not identify common lesions to these 2 rare metachronous sarcomas. The gliosarcoma was associated with monosomy 10 and underlying PTEN monoallelic loss, which has been recurrently observed. In the cardiac sarcoma, MDM2 amplification and CDKN2AB/9p21 biallelic deletion suggested intimal sarcoma. No causal relationship was found between cardiac sarcoma and asbestos exposure, although MDM2 abnormalities were linked to malignant mesothelioma.

Adamantinoma-like Ewing sarcoma (AES) is a rare variant of the Ewing family of tumors that resembles classic adamantinoma of bone. AES shows epithelial differentiation and a more complex immunohistochemical expression profile with keratin and basal marker immunoreactivity and can resemble a variety of carcinomas. We report an unusual case of an AES of the parotid gland that mimicked a basal cell adenocarcinoma. Like basal cell adenocarcinoma, this AES showed a nested basaloid proliferation with peripheral palisading in tumor nests with \'basaloid\' epithelial differentiation as highlighted by cytokeratin AE1/3 and p40 positivity. However, unlike most basal cell adenocarcinomas, this tumor demonstrated high grade morphology, showed no true ductal or myoepithelial component, and also showed a tendency towards neuroectodermal phenotype with focal rosette formation, CD99 and weak synaptophysin immunoreactivity. EWSR1 and FLI1 fluorescence in situ hybridization confirmed the presence of a translocation supporting the diagnosis of AES. This is the first case of AES presenting as a primary parotid mass highlighting the potential to be mistaken for primary salivary gland carcinomas, which in addition to basal cell adenocarcinoma include other basaloid tumors such as adenoid cystic carcinoma.