Surgical Treatment of Childhood Inflammatory Myofibroblastic Tumors

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Abstract

Aim

A retrospective study was performed to evaluate the clinical features, diagnostic methods, and treatment alternatives of childhood inflammatory myofibroblastic tumors (IMTs).

Patients and Methods

Patients who underwent surgical treatment for IMT between 2000 and 2015 were evaluated for age, sex, presenting symptoms, physical examination findings, diagnostic methods, treatment modalities, histopathologic findings, and results of surgical treatment during long-term follow-up.

Results

Eleven patients who underwent surgical treatment were included in the study. Male:female ratio was 7:4 and the mean age of the patients was 6.09 years (1-10 years). Presenting symptoms were respiratory difficulty, cough (n = 7, 63.3%), abdominal pain, vomiting (n = 2, 18.8%), loss of body weight (n = 1, 9.09%), palpable mass (n = 1, 9.09%), and rectal bleeding (n = 1, 9.09%). Ultrasonography (n = 4, 36.3%) and computed tomography (n = 9, 81.1%) were used for diagnosis. Localizations of tumors were lungs (n = 5, 45.4%), mediastinum (n = 2, 18.1%), spleen (n = 1, 9.09%), neck (n = 1, 9.09%), colon (n = 1, 9.09%), and rectum (n = 1, 9.09%). The mean size of mass was 6.6 cm (2-12 cm) and six patients were diagnosed with preoperative biopsy. Lung lobectomy (right lower lobe; n = 3, right middle and lower lobe; n = 2), total resection of mass with adjacent bowel (n = 2), partial splenectomy (n = 1), total resection of neck mass (n = 1), and incomplete resection (n = 2) were the choice of surgical treatment. Incomplete resection was performed in masses closely adjacent to atrium and mediastinal structures. In histopathologic evaluation, surgical margins were free of tumor in four cases, positive in six cases, and were not reported in one case. Anaplastic lymphoma kinase (ALK) positivity was detected in six cases, negative in two cases, and was not evaluated in three cases. Two cases who had residual mass with positive ALK received chemotherapy. Mean follow-up time was 68.2 months (5 months to 12 years). During follow-up, there was no recurrence or distant metastasis. Ten patients survived and one patient was lost to follow-up.

Conclusion

IMT is a rare tumor of childhood with a spectrum of clinical findings because of variable localization. Surgical treatment is the first choice of treatment. Patients with residual mass and ALK positivity may require medical treatment. In our series, long-term survival of patients was favorable in patients with total resection.

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