Unresectable multifocal omental and peritoneal inflammatory myofibroblastic tumor in a child: revisiting the role of adjuvant therapy

Cho

Pediatr Gastroenterol Hepatol Nutr

2014

Inflammatory myofibroblastic tumor (IMT) is rare mesenchymal solid tumor that consists of proliferating myofibroblasts with an inflammatory infiltrate background. It has a very low prevalence in infants and occurs mainly in children and young adults. IMT are mainly located in the thoracic cavity, but intra-abdominal lesions are rare. IMT can exhibit locally aggressive neoplastic processes and metastases similar to malignancies, so, have clinical importance. Herein, we describe two infantile intra-abdominal IMT cases presenting with incidentally found palpable abdominal mass. A 4-month-old male infant had IMT at the ileal mesentery and a 5-month-old male infant had IMT at liver. Both cases were successfully treated by complete surgical resection without complication or recurrence. Considering the biological behavior of the intermediate type of neoplasm in IMT, we expect good survivals when achieving appropriate surgical resection without adjuvant therapy in infantile intra-abdominal IMT.

Section: Discussionmentioning

confidence: 99%

Two Cases of Infantile Intra-abdominal Inflammatory Myofibroblastic Tumor

Cho

Pediatr Gastroenterol Hepatol Nutr

2014

“…However, this therapy may not be suitable in the present case due to the large size of the tumor surrounding large vessels, as well as due to the presence of metastatic regions. Although the number of previous reports on IMT is limited, a small number of cases have been reported (5,(12)(13)(14)(15)(16). A study reporting a case with ALK gene rearrangement-positive IMT demonstrated the failure of AI therapy followed by maintenance with imatinib subsequent to surgery (12).…”

Section: Discussionmentioning

confidence: 99%

“…Another case of retroperitoneal IMT underwent systemic chemotherapy with vincristine plus methotrexate and radiation, and no recurrence was observed for 2 years (5). In addition, certain studies on IMT cases in children reported the use of various chemotherapeutic drugs or regimens, including methotrexate, vinblastine, cisplatin, doxorubicin and ifosfamide, which demonstrated variable responses and thus their efficacy is unclear (13,17,18). Non-steroidal anti-inflammatory drugs (NSAIDs) and corticosteroids have also been studied as treatment options for IMT.…”

Section: Discussionmentioning

confidence: 99%

Successful combination chemotherapy for metastatic inflammatory myofibroblastic tumor: A case report

et al. 2015

Abstract.A 64-year-old male presented with increased abdominal fullness and fever. Radiological examination revealed moderate ascites, a tumor with a diameter of 12.5 cm in the mesenteric region, as well as multiple tumors in the thoracic and abdominal para-aortic regions and in the left supraclavicular regions. Pathohistological findings of the biopsy specimen revealed atypical spindle cells accompanied by infiltration of lymphocytes. The plasmacytes were positive for CD68, murine double minute 2 and S-100, while they were negative for α-smooth muscle actin, cyclin-dependent kinase 4 and anaplastic lymphoma kinase. Clinically, the patient presented systemic symptoms and laboratory results indicated an elevation in the inflammatory response, while the CT and MRI findings were consistent with an inflammatory myofibroblastic tumor (IMT). Based on the clinical and histological findings, the patient was diagnosed with IMT. In total, 4 cycles of combination chemotherapy with doxorubicin and ifosfamide were administered. Tumor size reduction by 50% was achieved subsequent to the 4th chemotherapy cycle. In conclusion, successful control of this rare metastatic IMT was achieved by systemic chemotherapy. IntroductionInflammatory myofibroblastic tumor (IMT) is a relatively rare disease that predominantly occurs in the peritoneum, retroperitoneum and lungs of young people; a previous study of 38 cases reported a median age of 8.5 years (1). This disease is histologically characterized by the proliferation of various spindle cells, which are of mesenchymal cell origin, in myxoid or collagenous stroma with prominent inflammatory infiltrates (2). IMT cells can potentially behave as locally invasive and are rarely metastatic (in <5% of cases) (3). In recent studies, a chromosomal translocation involving 2p23 and subsequent anaplastic lymphoma kinase (ALK) gene rearrangement was identified in ~50% of IMT cases (3,4). The ALK inhibitor, crizotinib, was demonstrated to be an effective therapy for ALK rearrangement-positive IMT cases.Complete surgical resection is typically conducted for localized IMTs (5); however, no standard therapeutic strategy has been established for inoperable or metastatic IMTs. In the present study, an extremely rare case of metastatic IMT that responded successfully to combination chemotherapy of doxorubicin and ifosfamide was reported. Case reportA 64-year-old male presented with abdominal fullness, fever and severe fatigue in March 2014. These symptoms had worsened within half a month. Following a visit to a primary care doctor in March 2014, a computed tomography (CT) scan revealed a moderate volume of ascites and a mass in the middle of the abdomen (Fig. 1A). Initially, lymphoma was suspected, and oral prednisolone (60 mg/day) was administered for 3 days as a therapy with diagnostic intent; however, no relief of the symptoms was observed. Exploratory laparotomy indicated a protruded large tumor in the mesenterium and 2,500 ml of chylous ascites. Microscopic examination of the tumor biopsy sample was ...

“…Most reported composition of chemotherapy referred to the soft tissue tumors, such as ifosfamide, etoposide, adriamycin, and cisplatin. Bertocchini et al [6] reported pediatric cases of unresectable multifocal omental and peritoneal IMT was followed by chemotherapy and ketorolac. The chemotherapy regimen consisted of 4 cycles of ifosfamide and adriamycin followed by two courses of ifosfomide.…”

Section: Discussionmentioning

confidence: 99%

Management of rectal inflammatory myofibroblastic tumor recurrence

Sun¹,

Tu²,

Wang³

et al. 2014

J Can Res Ther

Inflammatory myofibroblastic tumor (IMT) is an uncommon mesenchymal neoplasm of intermediate malignant potential. It may occur in various anatomic locations, but rarely in the rectum. This is a case discussion of a 36-year-old male patient with IMT of the rectum. After the patient underwent radical surgery, recurrence was seen after 18 months. Because the tumor was very close to the surrounding tissue, palliative tumor resection was performed followed by concurrent chemo-radiation and non-steroidal anti-inflammatory drugs (NSAID). After 2-year follow-up, the patient has no evidence of recurrence or metastasis. Surgical resection is very important for patient with rectal IMT, even in relapse cases. And adjuvant chemoradiotherapy and NSAID are in favor of the incompletely resected tumors as our case. But perhaps, the adjuvant treatments could be helpful after radical resection of rectal tumor.