2018
DOI: 10.1136/bcr-2017-221553
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Treatment-refractory ALK-positive inflammatory myofibroblastic tumour of the oral cavity

Abstract: We present a challenging case of a previously healthy 23-year-old man who developed an inflammatory myofibroblastic tumour of the hard palate, harbouring a rearrangement of the anaplastic lymphoma kinase (ALK) locus. Despite surgical intervention, radiotherapy and ALK-inhibition therapy, the tumour recurred locally and metastasised to regional lymph nodes, and the patient passed away roughly 9 months after diagnosis from local progression. The rapid progression of this patient's disease and its resistance to t… Show more

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Cited by 6 publications
(2 citation statements)
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“…Multinodular intraabdominal tumors are prone to the highest recurrence rate, as well as IMTs that are surgically challenging anatomical locations such as the head and neck region where complete surgical resection can pose great difficulty for a surgeon. Statistically, en-bloc excision with clear margins (R0) of a single IMT has a very low recurrence rate [27,61,33,[161][162][163][164]. Several studies advocate for re-excision of local recurrence as a treatment of choice, with little evidence available for optimal therapy for inoperable, relapsed, or metastatic IMT [11,162].…”
Section: Surgical Treatmentmentioning
confidence: 99%
“…Multinodular intraabdominal tumors are prone to the highest recurrence rate, as well as IMTs that are surgically challenging anatomical locations such as the head and neck region where complete surgical resection can pose great difficulty for a surgeon. Statistically, en-bloc excision with clear margins (R0) of a single IMT has a very low recurrence rate [27,61,33,[161][162][163][164]. Several studies advocate for re-excision of local recurrence as a treatment of choice, with little evidence available for optimal therapy for inoperable, relapsed, or metastatic IMT [11,162].…”
Section: Surgical Treatmentmentioning
confidence: 99%
“…Systemic symptoms and laboratory abnormalities described in up to 30% of IMTs overall [75] are not typically reported in sinonasal or laryngeal IMTs [76,77]. Laryngeal IMTs generally arise in the glottis and follow a benign clinical course following excision [77,78], while sinonasal and oral cavity IMTs are clinically more aggressive, with higher rates of recurrence, metastasis and mortality despite treatment [79,80]. Other reported sites include pharynx, neck, skull base, salivary glands, trachea, and orbit [74,79].…”
Section: Inflammatory Myofibroblastic Tumormentioning
confidence: 99%