Extraosseous Ewing's sarcoma of the spinal epidural space

Ewing's sarcoma is found exceptionally as a primary epidural tumor of the spine. Four cases of extraosseous Ewing's sarcoma of the spinal epidural space are presented. Another 17 cases were found in the pertinent literature and are discussed. There were 15 males and 6 females with mean age of 16 years (range, 4 to 30). Symptoms included back pain and/or radicular pain, paresis of one or more limbs, sensory disturbances, and bladder and bowel dysfunction. The mean diagnostic delay was 4,7 months. Each patient underwent surgery but complete resection of the tumor was obtained in only half of the cases. Most patients received radiation therapy and chemotherapy. Twelve patients died, 1 to 54 months (mean, 18) after diagnosis. Even though the number of patients was too small in this series for statistical analysis, partially resected tumors do appear to be associated with a higher mortality.     

Extraosseous sarcoma

Summary Extraskeletal osteosarcoma is very rare and is difficult to diagnose histologically. We report a case of a 63 year old women who had such a tumour in her thigh.

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Résumé L'ostéosarcome des parties molles est extrêmement rare et d'un diagnostic anatomo-pathologique difficile. Nous rapportons le cas d'une femme de 63 ans qui présentait une telle tumeur au niveau de la cuisse.

     

Treatment of Ewing's sarcoma in the cooperative Ewing's sarcoma study group

Multidisciplinary treatment approaches have greatly improved the prognosis of patients with Ewing's sarcoma. Four- or five-drug combination chemotherapy regimens are combined with radiation and/or surgery for local control. Currently, in the Cooperative Ewing's Sancoma Study (CESS) trials, approximately 60% of the protocol patients with Ewing's sarcoma show a relapse-free 10-year survival. For improvement of local control, preoperative radiotherapy and/or brachytherapy may be helpful. The early detection of blood and/or bone marrow contamination by molecular diagnosis can lead to a new clinical staging system. A myeloablative regimen consisting of simultaneous total-body irradiation and chemotherapy may improve the prognosis of patients with disseminated disease.     

肾脏尤文氏肉瘤1例报告并文献复习

目的探讨肾脏尤文氏肉瘤的临床诊治及免疫组化特点。方法分析1例原发性肾脏尤文氏肉瘤的临床资料,复习文献总结肾脏尤文氏肉瘤的特点。结果在全身麻醉下进行右肾癌根治术。肿瘤免疫组化标记结果：Vimentin＋＋＋、CD99＋＋、CD56＋＋、Ki-67＋、Calponin＋/-;SMA、Desmi、CK7、myoylobin、S-100、NSE、CgA、Syn、CD20、HMB45等为阴性。术后进行化疗和放疗。结论肾脏尤文氏肉瘤源于神经外胚层恶性软组织肿瘤,有较高的侵袭性,预后较差,手术切除应为首选治疗方案,术后配合放疗和化疗。     

Ewing's sarcoma of the mandible

Cure rates of Ewing's sarcoma have improved from 10% to 75% with the use of a combination of local therapy and aggressive multi-drug chemotherapy, to decrease both the incidence of local disease recurrence and the development of pulmonary and skeletal metastases. In Ewing's sarcoma of the mandible, complete surgical resection is preferred to irradiation for local control in those instances when surgery can be performed with minimal loss of function and disfigurement.     

Ewing's sarcoma of the pelvis

Summary Two hundred and thirty-nine patients with Ewing's sarcoma were treated at our institution between 1972 and 1987: 42 of these had lesions in the pelvis, 29 were in the wing of the ilium or involved the sacroiliac joint (type I), 5 were periacetabular (type II), and 8 were in the anterior pelvic arch (type III). Radiotherapy alone was used for the primary lesion in 11 cases, adjuvant chemotherapy in 20 and a neoadjuvant protocol in 22. The overall disease-free survival at a mean follow up of 34 months was 19%. There was no difference in survival related to age or the site in the pelvis, none in disease-free survival with adjuvant and neoadjuvant chemotherapy, or in the incidence of local recurrence and metastases in these two groups. Similarly, there was no difference in disease-free survival between operative treatment, with or without radiotherapy, and radiotherapy alone. There was a slight trend towards better local control of the disease in the former group compared to the latter, although this difference was not statistically significant. Our conclusion is that treatment needs to be planned for each individual patient.

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Résumé De 1972 à 1987, 239 patients atteints de sarcome d'Ewing ont été traités à l'Institut Orthopédique Rizzoli. Parmi eux 42 avaient des lésions pelviennes et ont été pris en compte dans cette étude. Vingt-neuf lésions étaient localisées à l'aile iliaque ou atteignaient l'articulation sacro-iliaque (type I), cinq lésions étaient periacétabulaires (type II) et huit étaient localisées à l'arc pelvien antérieur (type III). Dans 11 cas la lésion primitive à été traitée par résection seule ou par résection associée à la radiothérapie. Dans 31 cas on a traité la lésion primitive seulement par radiothérapie. La chimiothérapie adjuvante à été utilisée chez 20 patients alors qu'un protocole «néoadjuvant» a été suivi chez 22 d'entre eux. La survie sans rechute avec un recul moyen de 34 mois a été de 19%. Aucune différence de survie concernant l'âge ou la localisation pelvienne n'a été observée. En comparant les résultats de survie sans rechute aucune différence n'a été notée entre les patients traités par chimiothérapie adjuvante ou «néoadjuvante». Il n'a pas été noté non plus de différences de fréquence des récidives locales ou des métastases entre les 2 groupes. La survie sans rechute a été la même pour les patients dont la lésion primitive, irradiée ou non, a été traitée chirurgicalement (2/11=18,8%) ou simplement par irradiation (6/31=19,4%). La maladie a été un peu mieux contrôlée localement chez les patients traités par la chirurgie: 27,3% de récidives locales au lieu de 38,7% chez les malades irradiés seulement. Cette différence n'est cependant pas statistiquement significative. Les résultats de cette étude nous induisent à penser que le traitement du sarcome d'Ewing du bassin doit être adapté à chaque cas.

     

Periosteal Ewing's sarcoma

Periosteal Ewing's sarcoma is a histologically typical Ewing's sarcoma arising in the periosteum with no involvement of the medullary canal or cancellous bone. We describe four cases in our experience and review the literature, recalling the usual computed tomography diagnostic criteria and the therapeutic consequences. Prognosis of periosteal Ewing's sarcoma is generally better than for ordinary Ewing's sarcoma. In order to avoid inoculating the canal, the operator must strictly avoid perforating the cortical during the biopsy. At surgical resection, cortical resection can be partial maintaining a continuous diaphysis; this should be examined as a possibility for young patients in order to avoid the problems encountered with massive reconstruction followed by chemotherapy.     

Laparoscopic removal of extraosseous Ewing's sarcoma of the kidney in a pediatric patient

In the pediatric population, to the best of our knowledge, only 2 cases of renal extraosseous Ewing's sarcoma/primitive neuroectodermal tumor (EES/PNET) have been published. We report the initial case of renal EES/PNET occurring in a 10-year-old girl treated by a laparoscopic radical nephrectomy. The regimen used is the first documented use of neoadjuvant chemotherapy prior to laparoscopic radical nephrectomy for PNET. This approach obviated the need for a large incision and a prolonged postsurgical recovery. The minimally invasive nature of the laparoscopic procedure allowed for a rapid convalescence and resumption of her chemotherapy regimen within 14 days of the surgery.     

肾脏未分化网状细胞肉瘤一例报告并文献复习

目的 探讨肾脏未分化网状细胞肉瘤(尤因肉瘤)的临床诊治及预后特点. 方法 肾尤因肉瘤患者1例,男,33岁.体检发现左肾肿物3d.B超检查见左肾实质下部占位病变,边界清楚,其内血流信号丰富.CT检查示左肾下极类圆形肿块,5.1 cmu×4.7 cm大小,平扫中等密度,内见多个小斑片状低密度区,增强扫描明显不均匀强化,静脉期强化明显,考虑为肾癌.行后腹腔镜下左肾根治性切除术,手术顺利.检索相关文献复习讨论. 结果 病理报告:左肾下极直径4.0 cm灰白色肿物,部分坏死,瘤细胞小圆形,较均匀一致,成片分布,胞界不清,胞质稀疏,细胞核大小较一致,可见核分裂象.免疫组化染色:Vimentin(+),CD99(+),PAS(+),WT-1(-).病理诊断为肾尤因肉瘤.术后化疗:短周期(环磷酰胺+长春新碱+吡柔比星,每天1次,连续2d),长周期(异环磷酰胺+依托泊苷,每天1次,连续6d),长短周期交替进行,共6个周期,周期间隔21 d.随访14个月未见复发. 结论 肾尤因肉瘤临床罕见,恶性程度高,生物学行为进展快,诊断主要依据病理组织学及免疫组化染色检查,治疗以手术切除加放、化疗为主,但预后不佳.     

Ewing's sarcoma in the retroperitoneum

The patient was a female aged 18. Hematuria was discovered at an examination two years previously at which time the patient was complaining of right lumbar pain. The patient visited our hospital with a chief complaint of right abdominal tumor in June, 1988. A retroperitoneal tumor, 77 X 45 mm in size, was observed by CT and ultrasonic examination, and the right kidney was in hydronephrosis. An operation was performed in September, 1988, based on a diagnosis of retroperitoneal tumor and hydronephrosis. The tumor was seen to have involved the right ureter. Subsequently the tumor was surgically removed; right total nephroureterectomy and partial cystectomy were performed at that time. The removed tumor was 90 X 70 mm in size and 120 g in weight; it was histopathologically diagnosed as extraskeletal Ewing sarcoma. A systemic examination was carried out postoperatively, but no abnormalities were observed. As of September, 1990, the patient had had no recurrence and was being kept observation on an outpatient basis. There have been a total of 210 case reports regarding extraskeletal Ewing sarcoma published worldwide, including our case and 26 cases in Japan, so far as we know. However those of a lesion of retroperitoneal origin are extremely rare, numbering only 11 cases worldwide and 2 Japan. Herein we report our case of extraskeletal Ewing sarcoma with reference to the other 209 cases reported in the literature to date.     

Metastatic Ewing's sarcoma/PNET of kidney in 40 year old patient

IntroductionPrimary renal Ewing sarcoma/PNET is an uncommon and very aggressive tumor.Presentation of case: We report the case of a young woman who underwent nephrectomy for a renal mass from unknown etiology. Histologic analysis found small tumoral cells in rosette formation, and immunohistochemical staining was positive for CD99, and focally positive for vimentin and Protein S-100. A post operative abdominal computed tomography (CT) scan revealed a residual renal processus with hepatic wounds and abdominal metastatic nodes.After 6 cycles of chemotherapy including Vincristine, Doxorubicin, Cyclophosphamide, the response was considered as good.DiscussionEwing's sarcoma/PNET of kidney is a member of the family of small round cell tumors and it should be differentiated from Wilms tumor, neuroblastoma, rhabdomyosarcoma and lymphoblastic lymphoma. The principle management of its treatment have been extrapolated from the treatment of osseous Ewing sarcoma of bone.ConclusionDespite aggressive treatment, primary renal Ewing sarcoma/PNET has a poor prognosis. It requires a multidisciplinary approach including oncologists, urologists and radiation oncologists.