scholarly journals Pediatric patient with diagnosis of extra osseous Ewing’s sarcoma of the tongue: case report

2018 ◽  
Vol 66 (4) ◽  
pp. 399-403
Author(s):  
Neira CHAPARRO ◽  
Amaurys DIAZ ◽  
Luis HERRERA ◽  
Rafael PINEDA ◽  
Betty PEROZO ◽  
...  

ABSTRACT Ewing’s sarcoma is a malignant tumor that arises mainly from bone tissue, so that its extra-osseous presentation is not very common and even more unusual, in the soft tissues of the oral cavity. The objective of this case was to describe the clinical characteristics of an extra-osseous Ewing’s Sarcoma of the tongue in a pediatric patient. An 11-year-old male school patient, who attended an oral medicine consultation referring a volume increase in the tongue. Intraorally, a tumoral lesion was observed on the left lateral edge of the tongue, indurated, with the same color of the mucosa with ulcerated areas, well defined edges and symptomatic on palpation, with an approximate size of 2.5 cm. A cervical and maxillofacial MRI was indicated, observing a partially delimited hyperintense and non-infiltrating lesion in the described area. An incisional biopsy was performed with histopathological diagnosis of malignant neoplasm of blue round cells, theCD99 marker was found to be positive in the immunohistochemical study and was key to the definitive diagnosis of this tumor. A thoraco-abdomino-pelvic tomography was requested by the Pediatric Oncology service, as well as biopsy and aspiration of bone marrow, in which no neoplastic infiltrations were evidenced. After four cycles of chemotherapy, total removal of the lesion was performed with a consecutive histopathological study of the surgical piece, indicating free edges of the lesion. Ewing’s sarcoma is a tumor with aggressive behavior, so this case represents a finding of clinical and epidemiological relevance, both due to its extra-osseous appearance and its unusual behavior.

2002 ◽  
Vol 88 (4) ◽  
pp. 345-346 ◽  
Author(s):  
Marija Petković ◽  
Gordana Zamolo ◽  
Damir Muhvić ◽  
Miran Čoklo ◽  
Sanja Štifter ◽  
...  

Aims and Background To report an extremely rare case of Ewing's sarcoma located in the rectovaginal septum. Ewing's sarcoma is a highly malignant neoplasm of bone, which usually occurs during childhood. Common extraosseous localizations of Ewing's sarcoma include the trunk, extremities, uterus, cervix and vagina. Methods A 45-year-old woman presented to us with a six-month history of pain in the lower abdomen during intercourse. Pelvic examination was performed and a palpable mass was found. The mass had a size of 9 × 6 cm, a soft tissue consistency, was partially movable and the patient felt the pain during palpation. Examination of the inguinal lymph nodes revealed no signs of inguinal adenopathy. The results of laboratory tests, rectoscopy, chest X-rays, barium enema and bone scan were normal. Computed tomography (CT) showed an inhomogeneous expansive mass in the rectovaginal septum measuring 8.7 × 6.1 cm, without any signs of rectum or bladder invasion. The vascular structures of the pelvis were normal. At laparotomy the process was judged inoperable and only biopsy of the tumor mass was carried out. Histology showed a neoplasm with small, round to oval cells with scarce cytoplasm. Immunohistology with the monoclonal antibody CD99 (MIC-2 gene product, Ewing's sarcoma marker, clone 12E7, DAKO A/S, Glostrup, Denmark) revealed an extraosseous Ewing's sarcoma. The patient was treated with chemotherapy followed by whole-pelvis external beam radiation and intracavitary brachytherapy. Results A residual mass measuring 3.5 × 2.5 cm was visible on a control CT scan 18 months after treatment; however, the patient was feeling well and refused surgery to remove the residual mass. Conclusions To our knowledge this is the first reported case of extraosseous Ewing's sarcoma in the rectovaginal septum.


2019 ◽  
Vol 10 (1) ◽  
pp. 48-53
Author(s):  
Mohammad Sowkat Hossain ◽  
S M Mahbubul Alam ◽  
Sk Md Jaynul Islam ◽  
Wasim Selimul Haque ◽  
Shamoli Yasmin

Background: Undifferentiated tumours in the head and neck region are not uncommon. They can arise from different sites like in mucosa as well as in salivary glands, soft tissues or lymph nodes. Histopathological examination plays a central role in the diagnosis but difficulties arise with some tumours which are poorlydifferentiated due to their high inter- and intra-observer variability. In those cases, immunohistochemistry has greatly assisted to diagnose the tumours that cannot be accurately identified using routine histopathological procedures. The correct histopathological diagnosis is essential especially in case of malignant tumourwhere subsequent specific therapy is required. The aim of this study was to determine the role of immunohistochemistry for diagnosing undifferentiated malignancy in the head and neck region. Methods: This cross-sectional study was conducted during July 2014 to June 2015. A total of 35 Bangladeshi patient’s specimens of head and neck swelling were collected from two renowned laboratories in Dhaka city.These cases were diagnosed as undifferentiated tumour in histopathological examination.Standard protocol was followed for immunohistochemistry.Then primary immunohistochemical panel which included the markers for Epithelial CK (AE1/AE3),mesenchymal marker (Vimentin) and lymphoid marker (LCA) were used.The cases which were not resolved by primary immunohistochemistry panel, the second panel was applied for further sub classification (Desmin, Chromogranin, CK20, CEA, CD20, CD30, HMB45, NSE). Based on interpretation of immunohistochemical findings final diagnoses were made.Data analysis was performed using the Statistical Package for the Social Sciences for Windows version 22.0 (SPSS, Chicago, Illinois, USA). Results: A total of 35 undifferentiated tumorsof head-neck region were studied.The mean age was 46.3±17.6 years and male to female ratio was 4.8:1. The majority 13 (37.1%) patient had cervical lymphadenopathy, 11 (31.4%) had neck mass and 4(11.4%) had in nasal/sinonasal mass.Regarding histopathologicalcell types, round cell was 21 (60.0%), spindle cell 6 (17.1%), pleomorphic cells 6(17.1%) and epithelioid cells 2(5.7%). In initial histopathological examination, majority 25(71.4%) were undifferentiated malignant neoplasm, 8(22.8%) were metastatic undifferentiated carcinoma, 1(2.9%) was pleomorphic sarcoma and the remaining 1(2.9%) had malignant adnexal tumour. By application of immunohistochemistry, most (33, 94.3%) of the cases were resolved and the two cases remained unresolved. Among the resolved cases majority (15, 45.5%) were lymphoma, 4(12.1%) were metastatic carcinoma, 3(9.1%) were Ewing’s sarcoma and malignant melanoma were found in 3(9.1%) cases. Conclusion: This study supports that the immunohistochemical technique has a fundamental role in the investigation of undifferentiated tumour origin, to determine the correct guidance for treatment and improving the prognosis for head and neck tumour patients. Birdem Med J 2020; 10(1): 48-53


2021 ◽  
Vol 58 (4) ◽  
pp. 24-28
Author(s):  
S. BAITUROVA ◽  
K. OMAROVA ◽  
R. BORANBAEVA ◽  
G. ABDILOVA ◽  
O. PANKOVA ◽  
...  

Relevance: M6 variant of acute myeloid leukemia is extremely rare in pediatric practice. The diverse clinical manifestations of erythroid leukemia complicate the timely diagnosis of this group of diseases. Purpose: to describe a clinical case of congenital erythroid leukemia with multiple lesions of soft tissues and the skeletal system with complications in the form of the convulsive disorder, presented as a tumor of the Ewing’s sarcoma family, diagnosed at the Research Center of Pediatrics and Pediatric Surgery (Almaty, the Republic of Kazakhstan). Results: The presented clinical case demonstrated challenges in diagnosing and treating young patients with multiple life-threatening tumor lesions. The need to develop molecular genetic studies and expand diagnostic capabilities is an integral part of treating oncohematological diseases. Conclusion: The presented clinical case is of great interest due to its rareness. This case confirms the need to conduct all diagnostic manipulations to assess the process prevalence and choose and conduct molecular genetic studies on all examination and treatment stages.


Author(s):  
B. N. SUD

Review of 39 cases of sarcomas involving soft tissues, especially of the lower extremity and paravertebral region of children and young adults, disclosed a uniform histologic picture that was indistinguishable from that of Ewing's sarcoma of bone (1). Biopsy of a soft tissue tumor of the right thigh of a ten year old boy was studied by light microscopy and diagnosed as poorly differentiated sarcoma, probably extra skeletal Ewing's sarcoma (2). A portion of the biopsy was processed for electron microscopy to elucidate the ultrastructural pathology.The specimen revealed a neoplasm, consisting of sheets of viable as well as necrotic tumor cells. The viable cells had a single large rounded, oval or kidney- shaped nucleus with one or more dense nucleoli and chromatin with occasional clumping along the periphery and also in the interior.


KYAMC Journal ◽  
2019 ◽  
Vol 10 (3) ◽  
pp. 164-167
Author(s):  
Md Mofazzal Sharif ◽  
Khaleda Parvin ◽  
Umme Iffat Siddiqua ◽  
A.Q. Mehedi Hassan ◽  
Jabed Hossain ◽  
...  

Ewing's sarcoma is a common malignancy of the bone and soft tissues in pediatric patients. It mostly affects the long bones and pelvis, and less commonly the flat bones and vertebrae. Primary Ewing's sarcoma affecting the spine is very rare. The patient has non-specific symptoms for a prolonged period of time before the correct diagnosis is given. Patients can present with acute paraplegia due to spinal cord compression, which needs prompt surgical intervention. Early diagnosis and treatment are important for neurological recovery. The definitive management includes three main modalities: surgery, radiotherapy and combination chemotherapy. Adequate surgical excision may not be feasible because of anatomical limitations and local control is mainly achieved by radiotherapy. Because of the low incidence of these tumors, a multitude of therapeutic strategies have been employed with varying success. Currently there are no clinical guidelines outlining optimal management KYAMC Journal Vol. 10, No.-3, October 2019, Page 164-167


2000 ◽  
Vol 118 (6) ◽  
pp. 198-200 ◽  
Author(s):  
Adriano Santana Fonseca ◽  
Raquel Mezzalira ◽  
Agrício Nubiato Crespo ◽  
Antônio Emílio Bortoleto Junior ◽  
Jorge Rizzato Paschoal

CONTEXT: Ewing's sarcoma is a rare neoplasm, which usually arises in long bones of the limbs and in flat bones of the pelvis, with the involvement of head and neck bones being very unusual. CASE REPORT: a case of Ewing's sarcoma occurring in the mandible of a 35-year-old female. Pain and swelling of the tumor were the main complaints. The early hypothesis was an undifferentiated malignant neoplasm, possibly a sarcoma. The CT scan depicted an expansive lesion, encapsulated, with septa and characteristics of soft tissue, involving the left side of the mandible and extending to the surrounding tissues. The patient underwent surgical excision of the lesion, the definitive diagnosis of Ewing's sarcoma was established, and the patient commenced on radiotherapy.


1982 ◽  
Vol 90 (4) ◽  
pp. 491-493 ◽  
Author(s):  
Ray O. Gustafson ◽  
Nicolas E. Maragos ◽  
Herbert M. Reiman

Extraskeletal Ewing's sarcoma is a rare malignant neoplasm. Arising from a primitive mesenchymal stem cell and primarily affecting young adults, this lesion demands aggressive therapy, including surgical treatment, radiation therapy, and chemotherapy.


Author(s):  
Nikolai G. Rainov ◽  
Dimitar Haritonov ◽  
Volkmar Heidecke

Abstract Background and Objective Primary Ewing's sarcoma of the skull is a very rare malignant neoplasm, predominantly occurring in children and adolescents. We describe here the clinical, neuroradiologic, and histopathologic features of a patient with primary Ewing's sarcoma of the skull and discuss the standards of therapy for this type of tumor. Clinical Presentation This 18-year-old male patient presented with a primary Ewing's sarcoma of the skull, involving the dura of the frontal and parietal lobes of the left cerebral hemisphere. He was treated with gross total surgical excision of tumor, skull reconstruction, chemotherapy, and irradiation. Twelve years after the surgery, the patient has no evidence of local recurrence or distant metastases. Radical surgical excision of the primary tumor with safety margins is thought to play a role in the favorable clinical course. Conclusion The presented case is the longest surviving patient after treatment of primary Ewing's sarcoma of the skull bone. This rare type of tumor may allow better survival rates under adequate management than sarcoma elsewhere in the body.


1997 ◽  
Vol 15 (2) ◽  
pp. 574-582 ◽  
Author(s):  
R B Raney ◽  
L Asmar ◽  
W A Newton ◽  
C Bagwell ◽  
J C Breneman ◽  
...  

PURPOSE One hundred thirty of 2,792 patients (5%) registered on three Intergroup Rhabdomyosarcoma Study clinical trials (IRS-I, -II, and -III) from 1972 to 1991 had an extraosseous Ewing's sarcoma (EOE). We report here the results of multimodality therapy for this tumor. PATIENTS AND METHODS The 130 patients were less than 21 years of age; 70 (54%) were males. Primary tumor sites were on the trunk in 41 patients, an extremity in 34, the head/neck in 23, the retroperitoneum/pelvis in 21, and other sites in 11. One hundred fourteen patients had no metastases at diagnosis. In 21 patients, the tumor was completely resected; in 30, the localized or regional tumor was grossly resected, and in 63 patients, grossly visible sarcoma was left behind. Sixteen patients (12%) had distant metastases at diagnosis. All patients were given multiagent chemotherapy and most received irradiation (XRT); none were treated with bone marrow transplantation. RESULTS One hundred seven patients (82%) achieved a complete response. At 10 years, 62%, 61%, and 77% of the patients were alive after treatment on IRS-I, IRS-II, or IRS-III therapeutic protocols, respectively, similar to figures obtained in all IRS patients. At last follow-up evaluation, 42 patients had died of progressive tumor and one of infection. Survival at 10 years was most likely for patients with tumor that arose in the head and neck, extremities, and trunk, and for those who underwent grossly complete tumor removal before initiation of chemotherapy. For patients with localized, gross residual tumor, adding doxorubicin (DOX) to the combination of vincristine, dactinomycin, cyclophosphamide (VAC), and XRT did not significantly improve survival in 39 patients (62% alive at 10 years) compared with that of 24 patients treated with VAC and XRT without DOX (65% alive at 10 years, P = .93). CONCLUSION This series indicated that EOE in children is similar to rhabdomyosarcoma (RMS) in its response to multimodal treatment. No benefit was apparent from the addition of DOX to VAC chemotherapy in patients with gross residual EOE.


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