Clinical Features And Hypoxic Marker Expression Of Inflammatory Myofibroblastic Tumors Of The Head And Neck

Part 1 Case Analysis of Inflammatory Myofibroblastic Tumors of The Head and NeckBackground:Inflammatory myofibroblastic tumors (IMTs) rarely affect the head and neck region. IMTs of the head and neck regions account for 14-18% of extra-pulmonary IMTs. Most commonly, they are located in the region of the orbits and upper airways, and less often at other sites. In the present study, we investigated clinicopathological features of 15 patients with head and neck IMTs, aiming to review and present useful information regarding the etiology, clinical manifestations, pathological phenotype, imaging characteristics, treatment and prognosis, thus to provide experience and reference for clinicians.Materials and Methods:We collected 15 cases of inflammatory myofibroblastic tumors of the head and neck admitted in Otolaryngology Department of First Affiliated Hospital of Zhejiang University during February 2002 to August 2014, including related Chinese-and English-language literature, and retrospectively analysis the clinical manifestations, imaging features, treatment and prognostic factors of IMTs, thus to proide appropriate treatment of IMTs for clinicians.Results:We collected 15 cases, including 6 males and 9 females and aged 22-81 years old, with average age of 47.5 years old. IMTs in the present study located in maxillary (n=5), right upper mandible (n=1), left nasal dorsum and upper lip (n=1), larynx (n=2), hypopharynx (n=1), oropharyngeal tonsil (n=2), floor of the mouth (n=1), root of tongue (n=1), neck (n=1). According to the pathological classification suggested by Coffin,4 cases belong to myxiod type (4/10),5 cases belong to compact type (5/10), while 1 case belongs to mixed type (1/10). Immunohistochemical analysis exhibited the positive expression rate of vimentin was (6/6), of smooth muscle actin (SMA) was (6/9), of desmin (DES) was (3/8), of HHF35 was (1/1), of Calponin (CP) was (1/4), of ALK was (0/6). Patients were followed up until June 2015.7 cases accepted radical surgery and no recurrence and metastasis reported. One case of maxillary IMT, one case of hypopharynx IMT and one case of right vocal cord accepted local tumor resection as the first treatment, and recurred 6 years,2 years and month respectively after surgery. They then received another radical surgery, without chemotherapy, radiotherapy and systemic high-dose glucocorticoid therapy before or after surgery, and reported no recurrence after the second surgery until lately (41 months,35 months,9 months).5 cases of maxillary IMTs accepted local tumor resection as the first treatment,4 of which recurred 1 month,2 months,6 months and 8 months respectively after surgery and then received radical surgery, combined with radiotherapy and systemic glucocorticoid therapy.1 of the 4 invasive cases responsed well to the subsequent therapy but the other 3 cases experienced several more recurrences and died 35 months,3 months,11 months respectively after diagnosis.Conclusions:Inflammatory myofibroblastic tumors rarely affect the head and neck region. Patients with an IMT generally present with non-specific symptoms and a mass. The diagnosis mainly depends on histopathological and immunohistochemical staining. CT and MRI images help to identify the primary lesion and the range, to predict benign or malignant lesion, thus to provide more information for drawing the treatment decision. The treatment of IMTs of the head and neck remains unclear and the main therapy is empirical therapy currently, based on the site, range, pathological type, metastasis of the lesion. Surgery is the main therapy of IMTs of the head and neck. Resection should be complete as possible to avoid recurrence, and there is no need to perform adjuvant therapy (radiotherapy, chemotherapy, systemic glucocorticoid therapy) if radical surgery is available.Part 2 Expression of hypoxia-related markers in inflammatory myofibroblastic tumors of thehead and neckBackground:The etiology of inflammatory myofibroblastic tumors (IMTs) is controversial and the prognosis is unpredictable. Previous studies have not investigated the expression of hypoxia-related markers in IMTs.Methods:Between 2002 and 2012,12 consecutive patients with histologically proven IMTs were enrolled in the study. Immunohistochemistry was used to detect GLUT-1, HIF-1α, PI3K, and p-Akt expression in paraffin-embedded tumor specimens. Associations among GLUT-1, HIF-la, PI3K, and p-Akt protein expression and clinical parameters were investigated.Results:The mean duration of follow-up was 52.1 months (range,11 to 132 months). Six patients had local recurrence. GLUT-1, HIF-la, PI3K, and p-Akt expression were detected in 41.7%,50.0%,33.3%, and 41.7% of patients, respectively. Fisher’s exact test revealed significant correlations between recurrence of IMT and PI3K expression (P=0.01) and p-Akt expression (P= 0.015). Univariate analyses revealed significant correlations between survival and GLUT-1 expression (P= 0.028), PI3K expression (P= 0.006), and p-Akt expression (P= 0.028). Multivariate analysis did not show a significant relationship between survival and GLUT-1, HIF-la, PI3K, or p-Akt. Spearman rank correlation analysis showed significant correlations between HIF-la and PI3K expression (r=0.707,P= 0.01) and between p-Akt and PI3K expression (r=0.837, P=0.001).Conclusions:Although our results are inconclusive owing to the small sample size, they suggest that PI3K and p-Akt expression may play a role in the recurrence of IMTs of the head and neck.