Surgical management of primary Ewing’s sarcoma of the petroclival bone extend into the sphenoid sinus: A case report and review of literatures

Background: Ewing’s sarcoma (ES) is a malignancy that arises from bones or soft tissue, characterized by primitive small and round blue cells. Primary ES typically occurs in the long bones, vertebrae, or pelvis, and is extremely rare in the skull base. Case Description: A 14-year-old girl presented with posterior cervical pain and dysfunction of multiple cranial nerves (CNs). Radiological investigation revealed a solid mass of the petroclival bone extending into the sphenoid sinus. The patient underwent endoscopic transsphenoidal surgery for diagnosis of the pathology, and partial resection was safely achieved. Histopathological, genetic, and radiological examinations confirmed the diagnosis of primary ES. Subsequently, the patient underwent adjuvant chemotherapy and radiotherapy following which the clinical symptoms resolved. Complete response was achieved after multimodal treatment. Twenty months after treatment, the patient remains in remission without recurrence or metastatic disease. Primary ES of the petroclival bone has been reported in only three cases in the literature. As seen in the present case, dysfunction of multiple CNs is the most common manifestation of petroclival ES. Diagnosis should be confirmed by histopathological and genetic examinations considering the nonspecific clinical symptoms and radiological features. Conclusion: Multimodal treatment, including surgery, chemotherapy, and radiotherapy, can result in favorable outcomes. Clinicians should consider safe resection during surgical management to prevent complications that can delay postoperative multimodal treatment.

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311The role of radiotherapy in the multimodal treatment of Ewing's sarcoma of the chest walk (Askin tumors)

Radiotherapy and Oncology ◽

10.1016/s0167-8140(96)80320-3 ◽

1996 ◽

Vol 40 ◽

pp. S81

Author(s):

A. Schuck ◽

Ch. Rübe ◽

J. Hofmann ◽

C. Hoffmann ◽

J. Dunst ◽

...

Keyword(s):

Multimodal Treatment ◽

Ewing’S Sarcoma ◽

Ewing's Sarcoma

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Ewing's sarcoma of soft tissues in childhood: a report from the Intergroup Rhabdomyosarcoma Study, 1972 to 1991.

Journal of Clinical Oncology ◽

10.1200/jco.1997.15.2.574 ◽

1997 ◽

Vol 15 (2) ◽

pp. 574-582 ◽

Cited By ~ 150

Author(s):

R B Raney ◽

L Asmar ◽

W A Newton ◽

C Bagwell ◽

J C Breneman ◽

...

Keyword(s):

Soft Tissues ◽

Ewing’S Sarcoma ◽

Ewing's Sarcoma ◽

Distant Metastases ◽

Residual Tumor ◽

Complete Response ◽

Left Behind ◽

Multiagent Chemotherapy ◽

Extraosseous Ewing’S Sarcoma

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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A rare case of primary Ewing's sarcoma presenting in the posterior nasal cavity with extension into the sphenoid sinus and a review of the literature

Otolaryngology Case Reports ◽

10.1016/j.xocr.2018.01.003 ◽

2018 ◽

Vol 6 ◽

pp. 34-37

Author(s):

Koorosh Rahmani ◽

Shokouh Taghipour zahir ◽

Mohammad Baghi Yazdi ◽

Mohammad-hossein Vahedian-Ardakani ◽

Maryam Vajihinejad

Keyword(s):

Nasal Cavity ◽

Sphenoid Sinus ◽

Rare Case ◽

Ewing’S Sarcoma ◽

Ewing's Sarcoma ◽

Review Of The Literature

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Multimodal Treatment of Primary Extraskeletal Ewing's Sarcoma of the Chest Wall: Report of 2 Cases

Cancer Research and Treatment ◽

10.4143/crt.2009.41.2.108 ◽

2009 ◽

Vol 41 (2) ◽

pp. 108 ◽

Cited By ~ 6

Author(s):

Woo Surng Lee ◽

Yo Han Kim ◽

Hyun Keun Chee ◽

Jae Joon Hwang ◽

Jun Seok Kim ◽

...

Keyword(s):

Chest Wall ◽

Multimodal Treatment ◽

Ewing’S Sarcoma ◽

Ewing's Sarcoma ◽

Extraskeletal Ewing’S Sarcoma

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Cyclophosphamide Plus Topotecan in Children With Recurrent or Refractory Solid Tumors: A Pediatric Oncology Group Phase II Study

Journal of Clinical Oncology ◽

10.1200/jco.2001.19.15.3463 ◽

2001 ◽

Vol 19 (15) ◽

pp. 3463-3469 ◽

Cited By ~ 205

Author(s):

Robert L. Saylors ◽

Kimo C. Stine ◽

Jim Sullivan ◽

James L. Kepner ◽

Donna A. Wall ◽

...

Keyword(s):

Solid Tumors ◽

Stable Disease ◽

Pediatric Patients ◽

Ewing’S Sarcoma ◽

Ewing's Sarcoma ◽

Complete Response ◽

Median Number ◽

Pediatric Oncology Group ◽

Hematopoietic Toxicity ◽

Grade 3

PURPOSE: To determine the response rate of the combination of cyclophosphamide and topotecan in pediatric patients with recurrent or refractory malignant solid tumors. PATIENTS AND METHODS: A total of 91 pediatric patients, 83 of whom were fully assessable for response and toxicity, received cyclophosphamide (250 mg/m2/dose) followed by topotecan (0.75 mg/m2/dose), each given as a 30-minute infusion daily for 5 days. All patients received filgrastim (5 mcg/kg) daily until the absolute neutrophil count (ANC) was ≥ 1,500 μL after the time of the expected ANC nadir. RESULTS: A total of 307 treatment courses were given to the 83 fully assessable patients. Responses (complete response plus partial response) were seen in rhabdomyosarcoma (10 of 15 patients), Ewing’s sarcoma (six of 17 patients), and neuroblastoma (six of 13 patients). Partial responses were seen in two of 18 patients with osteosarcoma and in one patient with a Sertoli-Leydig cell tumor. Twenty-three patients had either minor responses (n = 6) or stable disease (n = 17); the median number of courses administered to patients with partial or complete response was six (range, two to 13 courses), and the median administered to those with stable disease was three (range, one to 11 courses). The toxicity of the combination was limited principally to the hematopoietic system. Of 307 courses, 163 (53%) were associated with grade 3 or 4 neutropenia, 84 (27%) with grade 3 or 4 anemia, and 136 (44%) with grade 3 or 4 thrombocytopenia. Despite the severe myelosuppression, only 34 (11%) of 307 courses were associated with grade 3 or 4 infection. Nonhematopoietic toxicity of grades ≥ 3 was rare and consisted of nausea and vomiting (two courses), perirectal mucositis (one course), transaminase elevation (one course), and hematuria (two courses). CONCLUSION: The combination of cyclophosphamide and topotecan is active in rhabdomyosarcoma, neuroblastoma, and Ewing’s sarcoma. Stabilization of disease was seen in osteosarcoma, although objective responses were rare in this disease. The therapy can be given with acceptable hematopoietic toxicity with the use of filgrastim support.

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Primary Ewing's sarcoma of the sinonasal tract, eroding the ethmoid and sphenoid sinus with intracranial extension: A rare case report

Molecular and Clinical Oncology ◽

10.3892/mco.2015.548 ◽

2015 ◽

Vol 3 (4) ◽

pp. 807-810 ◽

Cited By ~ 8

Author(s):

MARIA EMANUELA NEGRU ◽

ANDREA PIETRO SPONGHINI ◽

DAVID RONDONOTTI ◽

FRANCESCA PLATINI ◽

MARCO GIAVARRA ◽

...

Keyword(s):

Case Report ◽

Sphenoid Sinus ◽

Rare Case ◽

Ewing’S Sarcoma ◽

Ewing's Sarcoma ◽

Sinonasal Tract ◽

Intracranial Extension ◽

Rare Case Report

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Primary Ewing’s Sarcoma of Body of Mandible, Multimodal Treatment with Excellent Spontaneous Bone Regeneration: a Case Report

Journal of Maxillofacial and Oral Surgery ◽

10.1007/s12663-021-01623-z ◽

2021 ◽

Author(s):

Rangila Ram ◽

Priyanka Bhardwaj ◽

Yogesh Bhardwaj ◽

Narotam Ghezta ◽

Ravi Bhatt ◽

...

Keyword(s):

Case Report ◽

Bone Regeneration ◽

Multimodal Treatment ◽

Ewing’S Sarcoma ◽

Ewing's Sarcoma

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Survival After Recurrence of Ewing’s Sarcoma Family of Tumors

Journal of Clinical Oncology ◽

10.1200/jco.2005.05.105 ◽

2005 ◽

Vol 23 (19) ◽

pp. 4354-4362 ◽

Cited By ~ 119

Author(s):

Lisa M. Barker ◽

Thomas W. Pendergrass ◽

Jean E. Sanders ◽

Douglas S. Hawkins

Keyword(s):

Relative Risk ◽

Ewing’S Sarcoma ◽

Ewing's Sarcoma ◽

Medical Center ◽

Univariate Analysis ◽

Complete Response ◽

High Dose ◽

Line Treatment ◽

Second Line ◽

Risk Of Death

Purpose The overall survival (OS) of patients with relapsed Ewing’s sarcoma family of tumors (ESFT) is poor, and the relative benefit of high-dose therapy (HDT) is controversial. Patients and Methods We retrospectively identified 55 consecutive ESFT patients with adequate medical records for review, who were treated at Children’s Hospital and Regional Medical Center and who developed disease recurrence between January 1, 1985 and December 31, 2002. Results The median relapse-free interval (RFI) from diagnosis to first recurrence was 17 months (range, 5 to 90 months). Most recurrences were metastatic only (39 patients) or local and metastatic (10 patients). Twenty-seven patients (49%) achieved a partial or complete response to second-line treatment, with a median duration of response of 27 months (range, 5 to 119+ months). The 5-year OS rate for all relapsed patients was 23% (95% CI, 11% to 35%). By univariate analysis, improved OS was associated with response to second-line treatment versus no response (46% v 0%, respectively; P < .0001), RFI ≥ 24 months versus less than 24 months (48% v 12%, respectively; P = .0001), and no metastases at initial diagnosis versus presence of metastases (31% v 12%, respectively; P = .05). Because all 13 patients who received HDT also had responsive relapse, we performed a multivariate analysis. Reduced risk of death was associated with response to second-line therapy (relative risk, 0.14; 95% CI, 0.05 to 0.40), RFI ≥ 24 months (relative risk, 0.29; 95% CI, 0.13 to 0.66), and receiving HDT (relative risk, 0.26; 95% CI, 0.08 to 0.85). Conclusion HDT as consolidation therapy for relapsed ESFT seems to be associated with improved OS, even after adjusting for RFI and response to second-line treatment.

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