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LABORATORY OBSERVATIONS

Inflammatory Myofibroblastic Tumor of the Kidney and Bilateral Lung Nodules in a Child Mimicking Wilms Tumor With Lung Metastases Mehmet S. Dogan, MD,* Selim Doganay, MD,* Gonca Koc, MD,* Sureyya B. Gorkem, MD,* Ekrem Unal, MD,w Figen Ozturk, MD,z and Abdulhakim Coskun, MD*

Summary: Renal inflammatory myofibroblastic tumor (IMT) is an extremely rare lesion especially in children. This report describes a case of renal IMT accompanied by multiple lung nodules mimicking Wilms tumor with lung metastasis in a 3-year-old boy. To our knowledge, this is a unique case of IMT which has not been reported in the literature previously. Key Words: inflammatory myofibroblastic tumor, inflammatory pseudotumor, lung nodules, plasma cell granuloma

(J Pediatr Hematol Oncol 2015;37:e390–e393)

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nflammatory myofibroblastic tumor (IMT) is a rare benign condition which can be found in nearly every organ in the body.1 It is seen most commonly in the lung. The bladder is the most common site within the urogenital tract.1,2 Renal occurrence of IMT is extremely rare,1–7 with only 5 previously reported cases in children up to date.3–5,8,9 In the pediatric age group, it is usually difficult to differentiate a renal IMT from a malignancy such as Wilms tumor. We report a case of a 3year-old boy with a right renal mass accompanied by bilateral lung lesions mimicking Wilms tumor and its lung metastases on preoperative diagnostic images, which were pathologically revealed to be IMT lesions.

CASE REPORT A 3-year-old boy with complaints of right flank pain and recurrent fever for >1 month was referred to us. The findings in the physical examination were unremarkable except for right flank tenderness. Laboratory data showed a leukocytosis (12,300/mm3); microcystic anemia (hemoglobin, 10.1 g/dL; hematocrit, 33.2%; mean corpuscular volume, 68.7 fL; mean corpuscular hemoglobin concentration, 26.4 g/dL; red cell distribution width, 16.9; and thrombocytosis (569,000/mm3). Ertrocyte sedimentation rate was 8 mm/h. Blood urea, creatinine, and electrolytes were in normal limits. Urinalysis was normal. Renal ultrasonography revealed a hypoechoic, heterogeneous solid right upper pole mass, and pelvicaliectasis due to renal stone within the pelvis. Contrast-enhanced computed tomography (CT) and magnetic resonance imaging demonstrated a well-defined, solid heterogeneous mass measuring 6.0 5.5 5.5 cm in the superior portion of the right kidney, and Received for publication December 5, 2014; accepted April 13, 2015. From the Departments of *Radiology; wPediatric HematologyOncology; and zPathology, Faculty of Medicine, Erciyes University, Kayseri, Turkey. The authors declare no conflict of interest. Reprints: Mehmet S. Dogan, MD, Department of Radiology, Faculty of Medicine, Erciyes University, Kayseri 38039, Turkey (e-mail: [email protected]). Copyright r 2015 Wolters Kluwer Health, Inc. All rights reserved.

bilateral multiple lung nodules (Fig. 1). The renal mass was hypodense on CT images, it had iso-slight hypointensity on T2weighted images, iso-intensity on T1-weighted images, and showed heterogeneous enhancement after contrast media administration. On the basis of the clinical and radiologic findings, a malignancy such as Wilms tumor and lung metastases was considered. Right radical nephrectomy and CT-guided tru-cut biopsy of the lung nodules were performed. The histopathologic examinations of the nephrectomy and biopsy materials revealed spindle-shaped cells, plasma cells, and lymphocytes compatible with IMT (Fig. 2). The immunohistochemical findings of the nephrectomy material confirmed the myofibroblastic origin of the spindle cells; vimentin (+), smooth muscle actin (+), CD38 (+), CD68 (+), desmin (+), and anaplastic lymphoma kinase (ALK) (+) (Fig. 3). The thoracotomy was thought to be invasive to diagnose the pulmonary lesions; so that CT-guided tru-cut biopsy was performed. Given the insufficient/small amount of biopsy materials obtained from the lung nodules, immunohistochemical study could not be performed on these. However, the findings of the hematoxylin and eosin staining of both materials obtained from the lung nodules and kidney were similar. When considered together with the immunohistochemical findings of the nephrectomy materials confirming the myofibroblastic origin, as a part of the same process, all the lesions were diagnosed as IMT. At 6-month follow-up evaluation, the patient was asymptomatic with no evidence of local recurrence in the abdomen and with stable lung nodules on CT.

DISCUSSION IMT was first described in the lung in 1937 and in the kidney in 1972.2,3 It has also been termed as inflammatory myofibroblastic proliferation, inflammatory pseudotumor, plasma cell granuloma, xanthomatous pseudotumor, myofibroblastoma, and pseudosarcamatous fibromyxoid tumor.2,3 Although the lung is the most commonly affected site, IMT can be encountered almost everywhere in the body including head and neck, gastrointestinal, hepatobiliary, genitourinary system, and soft tissues of the trunk and extremities.1,10–12 IMT is characterized histologically by the presence of acute and chronic inflammatory cells including lymphocytes, plasma cells, and myofibroblastic spindle cells with a variable fibrous response.1 Immunohistochemical studies have revealed consistent expression of vimentin and smooth muscle actin in almost all cases, indicating the myofibroblastic nature of this lesion as in the present case. Variable positivity has been identified for HHF-35, cytokeratins, and CD68.2,6 The cause and pathogenesis of inflammatory pseudotumor still remain unknown.1 Initially it was thought that IMT represented a reactive inflammatory process that may be secondary to trauma, surgery, or infection.2 Recent studies suggest a neoplastic cause showing cytogenetic clonal abnormality and ALK expression.2,3 Autoimmune origin is

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Renal Inflammatory Pseudotumor With Lung Involvement

FIGURE 1. A–E, Axial computed tomography scans revealed a hypodense mass with well-defined margins in the superior portion of the right kidney (A) and bilateral multiple lung nodules (B and C). On T2-weighted (D), precontrast (E), and postcontrast (F) T1-weighted coronal magnetic resonance images, right renal mass, caliectasis at the lower pole because of its mass effect, and the lung nodule in the left lower lobe were also seen.

FIGURE 2. A and B, Hematoxylin and eosin staining of nephrectomy material (A: 400 magnification), and lung biopsy materials (B:  200 magnification) revealed spindle-shaped cells, plasma cells, and lymphocytes.

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FIGURE 3. A–D, The immunohistochemical findings of the nephrectomy material using higher power view (magnification: 400). The tumor cells were positive for vimentin (A), smooth muscle actin (B), CD38 (C), and anaplastic lymphoma kinase (D).

another hypothesis.1,2 In this case, there were both renal stone and ALK expression that could be the cause of reactive inflammation and neoplastic process, respectively. Renal IMT is a very rare condition that may mimic other benign and malignant tumors of the kidney. Our search for literature revealed only 5 cases of renal IMT in children.3,4,5,8,9 Thus, to our knowledge, the present case represents the sixth case of renal IMT in a child and the first reported case accompanied by multiple lung nodules. The list of differential diagnosis for renal IMT includes benign tumors such as, xanthogranulomatous pyelonephritis, angyomyolipoma with minimal fat, plasma cell granuloma, and malignant tumors such as renal cell carcinoma, mesenchymal sarcomas, sarcomatoid carcinoma, malignant lymphoma, malignant fibrous histiocytoma, urethelial carcinoma, and Wilms tumor in children.2,6,7 The clinical findings of renal IMT include flank pain, hematuria, fever, growth retardation, weight loss, microcytic hypochromic anemia, and thrombocytosis. However, it may be asymptomatic without any signs.2,3,5 Our patient had flank pain, fever, and his laboratory data showed microcytic hypochromic anemia and thrombocytosis. Renal IMT presents various radiologic findings. Renal IMT can be seen as hypogeneous or heterogeneous echoic mass on sonography, homogeneous or heterogeneous low attenuation mass on CT.4,5 Nakamura et al7 stated that most IMTs of the kidney appear as an ill-defined,

hypovascular, homogeneous tumor on CT images, with variable signal intensity on T1-weighted, and low signal intensity on T2-weighted images. In our case the renal mass was hypodense on CT, isointense on T1-weighted images, iso-slightly hypointense on T2-weighted images, and showed heterogeneous enhancement after contrast media administration. As discussed above, generally imaging characteristics of renal IMT are nonspecific.1,3,5 Therefore, the preoperative exclusion of malignancy in these lesions is extremely challenging. Although regression of bilateral renal IMT has been reported after long-term steroid therapy, nephrectomy is considered to be the best treatment modality especially in unilateral cases.2,5,7 Surgery with radical excision is also the recommended treatment for thoracic inflammatory pseudotumors.1 In this case, the preoperative diagnosis was Wilms tumor with lung metastases so the patient underwent nephrectomy and CT-guided tru-cut biopsies of the lung nodules. Because of the multiplicity and prevalence of lung lesions, surgical excision was not implemented. Currently crizotinib, an oral drug that inhibits the ALK had been reported to be effective for IMT in children.13 The option of crizotinib was preserved for either progression or recurrence of the disease in reported case. According to case reports no recurrences or associated metastases were identified in renal IMT previously.14 We do not know whether these lung nodules are metastases or

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synchronized lesions with IMT. Regardless of this controversy, close follow-up seems mandatory for this case. In conclusion, renal IMT in pediatric age group is an extremely rare lesion, and is difficult to differentiate from a malignancy such as Wilms tumor. Here, we report a case of renal IMT accompanied with lung nodules which is an original contribution in the literature. We suggest that renal IMT with lung involvement should be included in the differential diagnosis of Wilms tumor in the kidney with lung metastases.

REFERENCES 1. Patnana M, Sevrukov AB, Elsayes KM, et al. Inflammatory pseudotumor: the great mimicker. AJR. 2012;198:W217–W227. 2. Lee NG, Alexander MP, Xu H, et al. Renal inflammatory myofibroblastic tumor: a case report and comprehensive review of literature. World J Oncol. 2011;2:85–88. 3. Boo YJ, Kim J, Kim JH, et al. Inflammatory myofibroblastic tumor of the kidney in a child: report of a case. Surg Today. 2006;36:710–713. 4. Czerwinski M, Dave S. Pediatric renal inflammatory myofibroblastic tumours: a case report and review of the etiology and management options. Can Urol Assoc J. 2012;6: E150–E153. 5. Tarhan F, Gul AE, Karadayi N, et al. Inflammatory pseudotumor of the kidney: a case report. Int Urol Nephrol. 2004;36:137–140.

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Renal Inflammatory Pseudotumor With Lung Involvement

6. Ryu KH, Im CM, Kim MK, et al. Inflammatory myofibroblastic tumor of the kidney misdiagnosed as renal cell carcinoma. J Korean Med Sci. 2010;25:330–332. 7. Nakamura Y, Urashima M, Nishihara R, et al. Inflammatory pseudotumor of the kidney with renal artery penetration. Radiat Med. 2007;25:541–547. 8. Itoh H, Namiki M, Yoshioka T, et al. Plasma cell granuloma of the renal pelvis. J Urol. 1982;127:1177–1178. 9. Vujanic GM, Berry PJ, Frank JD. Inflammatory pseudotumor of the kidney with extensive metaplastic bone. Pediatr Pathol. 1992;12:557–561. 10. Homaei-Shandiz F, Jafarzadeh-Esfehani R, Moazzen N, et al. Inflammatory myofibroblastic tumor of salpinx: a very rare case treated with a less aggressive method. Iran J Cancer Prev. 2014;7:244–277. 11. Gurzu S, Bara T, Jung I. Inflammatory myofibroblastic tumor of the colon. J Clin Oncol. 2013;31:e155–e158. 12. Dousek R, Tuma J, Planka L, et al. Inflammatory myofibroblastic tumor of the esophagus in childhood: a case report and a review of the literature. J Pediatr Hematol Oncol. 2015;37:e121–124. 13. Mosse´ YP, Lim MS, Voss SD, et al. Safety and activity of crizotinib for paediatric patients with refractory solid tumours or anaplastic large-cell lymphoma: a Children’s Oncology Group phase 1 consortium study. Lancet Oncol. 2013;14:472–480. 14. Kapusta LR, Weiss MA, Ramsay J, et al. Inflammatory myofibroblastic tumors of the kidney—a clinicopathologic and immunohistochemical study of 12 cases. Am J Surg Pathol. 2003;27:658–666.

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e393

Inflammatory Myofibroblastic Tumor of the Kidney and Bilateral Lung Nodules in a Child Mimicking Wilms Tumor With Lung Metastases.

Renal inflammatory myofibroblastic tumor (IMT) is an extremely rare lesion especially in children. This report describes a case of renal IMT accompani...
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