BACKGROUND:: Inflammatory myofibroblastic tumor (IMT) is a rare benign cancer that can express a more aggressive phenotype related to the genetic mutation of the anaplastic lymphoma kinase receptor (ALK). Involvement of trachea is extremely rare and due to the clinical and radiologic nonspecificity, the definitive diagnosis is based on the histologic evaluation of tissue specimens. Total surgical excision is curative and chemotherapy or radiotherapy has been employed in the treatment of unresectable tumors or as adjuvant therapies. CASE PRESENTATION:: The case described here is being reported because of the rare tracheal location and the atypical treatment approach used for an ALK-positive IMT. A 7-week pregnant woman voluntary interrupted pregnancy and underwent total surgical excision that resulted to have close margins. Although ALK-positive expression indicated the use of ALK inhibitors, she refused any type of adjuvant therapy that could affect ovarian function. Thus, 3D conformational external beam radiotherapy was performed with a daily dose of 180 cGy, 5 times per week, up to 45 Gy at the level of trachea. A total of 62 months of follow-up showed and no signs of disease recurrence or late radiation therapy-related toxicity. CONCLUSIONS: This report describes an extremely rare case of a tracheal IMT, underlying the key role of radiotherapy as adjuvant treatment able to definitively cure IMT, limiting systemic chemotherapy-related toxicity.

Successful role of adjuvant radiotherapy in a rare case of tracheal inflammatory myofibroblastic tumor: a case report / Lisi, Roberto; Abate, Gessica; D'Urso, Pasqualina; Martinetti, Maria Teresa; Siniscalchi, Benedetta; Marampon, Francesco; Bulzonetti, Nadia; Tombolini, Vincenzo. - In: TUMORI. - ISSN 0300-8916. - 105:6(2019), pp. NP1-NP3. [10.1177/0300891619838333]

Successful role of adjuvant radiotherapy in a rare case of tracheal inflammatory myofibroblastic tumor: a case report

Abate, Gessica
Secondo
;
D'URSO, PASQUALINA;Martinetti, Maria Teresa;SINISCALCHI, BENEDETTA;Marampon, Francesco;Tombolini, Vincenzo
Ultimo
2019

Abstract

BACKGROUND:: Inflammatory myofibroblastic tumor (IMT) is a rare benign cancer that can express a more aggressive phenotype related to the genetic mutation of the anaplastic lymphoma kinase receptor (ALK). Involvement of trachea is extremely rare and due to the clinical and radiologic nonspecificity, the definitive diagnosis is based on the histologic evaluation of tissue specimens. Total surgical excision is curative and chemotherapy or radiotherapy has been employed in the treatment of unresectable tumors or as adjuvant therapies. CASE PRESENTATION:: The case described here is being reported because of the rare tracheal location and the atypical treatment approach used for an ALK-positive IMT. A 7-week pregnant woman voluntary interrupted pregnancy and underwent total surgical excision that resulted to have close margins. Although ALK-positive expression indicated the use of ALK inhibitors, she refused any type of adjuvant therapy that could affect ovarian function. Thus, 3D conformational external beam radiotherapy was performed with a daily dose of 180 cGy, 5 times per week, up to 45 Gy at the level of trachea. A total of 62 months of follow-up showed and no signs of disease recurrence or late radiation therapy-related toxicity. CONCLUSIONS: This report describes an extremely rare case of a tracheal IMT, underlying the key role of radiotherapy as adjuvant treatment able to definitively cure IMT, limiting systemic chemotherapy-related toxicity.
inflammatory myofibroblastic tumor; trachea; radiotherapy; ALK
01 Pubblicazione su rivista::01i Case report
Successful role of adjuvant radiotherapy in a rare case of tracheal inflammatory myofibroblastic tumor: a case report / Lisi, Roberto; Abate, Gessica; D'Urso, Pasqualina; Martinetti, Maria Teresa; Siniscalchi, Benedetta; Marampon, Francesco; Bulzonetti, Nadia; Tombolini, Vincenzo. - In: TUMORI. - ISSN 0300-8916. - 105:6(2019), pp. NP1-NP3. [10.1177/0300891619838333]
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11573/1261323
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