Surgical Treatment and Prognosis of Primitive Neuroectodermal Tumors of the Thorax

Demir, Adalet; Günlüoğlu, Mehmet Zeki; Dağoğlu, Nergiz; Turna, Akif; Dizdar, Yavuz; Kaynak, Kamil; Dilege, Şükrü; Mandel, Nil Molınas; Yılmazbayhan, Dilek; Dinçer, Seyyit İbrahim; Gürses, Atilla

doi:10.1097/jto.0b013e318194fafe

Cited by 34 publications

(23 citation statements)

References 16 publications

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“…These tumors are treated in the same manner due to the general conception that they all share a common neural histogenesis and tumor genetics 1 . However, a few cases have been reported in the thoracopulmonary region, such as the lung 2–10 . To the best of our knowledge, an ESFT originating in the central airway has not ever been reported.…”

Section: Introductionmentioning

confidence: 99%

“…1 However, a few cases have been reported in the thoracopulmonary region, such as the lung. [2][3][4][5][6][7][8][9][10] To the best of our knowledge, an ESFT originating in the central airway has not ever been reported. In this study, we report an extremely rare case of ESFT that originated in the main bronchus, which was subsequently resected by endobronchial snare, followed by chemotherapy and radiotherapy.…”

Section: Introductionmentioning

confidence: 99%

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Ewing's sarcoma family of tumors originating in the main bronchus

et al. 2012

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Ewing's sarcoma family tumors (ESFT), which include Ewing's sarcoma and primitive neuroectodermal tumors (PNET), have been reported to originate in a variety of sites, mostly in the extremities. Previous reports have shown ESFT originating in the thoracic region, such as chest wall and peripheral lung. We herein report the first case of the ESFT that originated in the main bronchus. Endobronchial snare resection was followed by five courses of chemotherapy (VDC-IE; including vincristine, doxorubicin, cyclophosphamide, ifosfamide and etoposide) and sequential radiation. After the treatment, the patient's condition has improved, and he has remained disease-free for the past year.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Ewing's sarcoma family of tumors originating in the main bronchus

et al. 2012

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“…PNET is usually diagnosed by histologic and immunohistochemical analysis. It was histologically composed of small round cells with scanty cytoplasm [13–16]. The chromosomal translocation t (11;22) (q24;q12) is an additional firm diagnostic criterion.…”

Section: Discussionmentioning

confidence: 99%

Benefit of Sunitinib in the treatment of pulmonary primitive neuroectodermal tumors: a case report and literature review

Zhang

Wang

et al. 2016

Oncotarget

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Primitive neuroectodermal tumor (PNET) is a highly aggressive small round celltumor but is extremely rare in the lung. Next-generation sequencing (NGS) has led to breakthroughs for genetic analyses and personalizedmedicine approaches for cancer treatment.We report the case of a 30-year-old woman with an advanced pulmonary PNET treated with multiple chemotherapeutic regimens, and achieved a partial response (PR) as a best response. However, there was a disease progression after these treatment regimens.The NGS revealed the presence of a copy number loss (CNL) of Von Hippel-Lindau (VHL), CDKN2A/B and TP53 genes. The specific VHL CNL has not previously been associated with PNET, but has been reported in other tumors and has been associated with response to Sunitinib. Sunitinibwas then instituted for this patient and resulted in PR after the failure of multiple chemotherapeutic regimens. To our knowledge, this is the first report of pulmonary PNET with CNL of VHL gene that benefits from Sunitinib treatment. This case illustrates the potential of clinicalNGS to open unexpected avenues for treatment and thereby improve patient outcomes.

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“…Overall, 4/8 patients who underwent a complete resection were alive after a median follow-up time of 30 months, while the remaining 4 patients with extended disease and marginal surgery succumbed to the disease. Demir et al (18) showed that patients who underwent a complete resection exhibited a higher 5-year survival rate compared with patients who underwent an incomplete resection (56 vs. 25%; P=0.13). With regard to repeated relapse cases, a surgical resection combined with chemoradiotherapy resulted in satisfactory survival rates.…”

Section: Discussionmentioning

confidence: 99%

“…Sawin et al (33) demonstrated that pre-operative chemotherapy resulted not only in a reduced tumor volume (from 7,054 to 911 cm 3 ), but also in prolonged survival rates. Demir et al (18) reported that neoadjuvant chemotherapy significantly increased the complete resection rate (P=0.027) and that the 5-year survival rates of the patients with or without neoadjuvant therapy were 77 and 37%, respectively (P=0.22). Concerning older patients, radical surgery may be avoided when a complete response is achieved following neoadjuvant chemotherapy (34).…”

Section: Discussionmentioning

confidence: 99%

Treatment of an Askin tumor: A case report and review of the literature

2013

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Askin tumors are rare malignant neoplasms that are localized in the thoracopulmonary region and mainly occur in children and adolescents. Further investigation with regard to the effective treatment of this disease is required, since the disease has a low incidence and limited knowledge is available on the biological activity and prognostic factors of this type of tumor. The present study describes the case of a 30-year-old male patient with a histologically and morphologically proven Askin tumor who was treated in Shandong Cancer Hospital and Institute (Jinan, China). A chest computed tomography (CT) scan demonstrated a large mass filling the entire left lung, associated with mediastinum and right lung compression and accompanied by destruction of the 2nd rib. The patient accepted chemotherapy and radiotherapy instead of a radical mass resection since the mass was irresectable. A good clinical response was achieved to the chemotherapy and radiotherapy. The diagnosis and treatment of Askin tumors remains a challenge for clinicians and surgeons due to the absence of standard therapeutic guidelines for the treatment of this disease. According to the experience obtained from the cases encountered to date, treatment strategies should aim to reduce local recurrence and distant metastasis. Moreover, surgery, chemotherapy and radiotherapy or a combination of these methods appears to constitute an effective treatment strategy for Askin tumors.

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Surgical Treatment and Prognosis of Primitive Neuroectodermal Tumors of the Thorax

Abstract: PNET is an aggressive malignancy that often requires multimodal therapy. Induction chemotherapy leads to a greater complete resection rate and better disease-free survival, while involvement of the costovertebral junction indicates a poorer survival.

Cited by 34 publications

References 16 publications

Ewing's sarcoma family of tumors originating in the main bronchus

Ewing's sarcoma family of tumors originating in the main bronchus

Benefit of Sunitinib in the treatment of pulmonary primitive neuroectodermal tumors: a case report and literature review

Treatment of an Askin tumor: A case report and review of the literature

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