Inflammatory myofibroblastic tumor of small bowel wall in childhood: Report of a case and a review of the literature

Benign intestinal tumors are rare in children, however we describe an inflammatory myofibroblastic tumor (IMT) of the jejunum in a 2-year-old girl who presented with an intestinal obstruction. During laparotomy, an annular mass around the jejunum was resected, from which a histological diagnosis of IMT was made. A review of the literature for this rare entity emphasizes the importance of histological confirmation of its benign nature. Because of the risk of local recurrence, IMT cases should have a long-term follow up.     

Inflammatory myofibroblastic tumor of the larynx: report of a case and review of the literature

Inflammatory myofibroblastic tumor (IMT) is a rare neoplasm, most commonly seen in children and adolescents. It can occur in nearly every part of the body. Inflammatory myofibroblastic tumor (IMT) is a rare neoplasm mostly seen in the lungs, but also in extrapulmonary sites. But it may rarely be seen in the vical cord. We report a case of a 73-year-old men presented with hoarseness and cough. Laryngoscopy reveals a large non-ulcerated, red subepithelial mass arising from the right vical cord. Magnetic resonance imaging (MRI) scan revealed a mass in the right vical cord, and magnetic resonance imaging (MRI) enhanced scan showed the mass of the right vical cord inhomogeneous enhancement. The patient underwent right cordectomy with KTP laser, and further assessment of the tissue demonstrated a pathologic diagnosis of IMT.     

21 例炎性肌纤维母细胞瘤的超声表现分析简

目的探讨炎性肌纤维母细胞瘤（IMT）的超声表现,旨在提高对其诊断水平。方法回顾性分析21例经手术病理组织证实的IMT患者超声图像,其中男性13例,女性8例：年龄0.5～76.0岁,平均年龄45.1岁。分析病变的发生部位、大小、形态、边界、内部回声、血流信号及有无转移等特点。仅有5例腹腔IMT和2例盆腔IMT进行增强CT检查,余14例只做了超声检查。结果位于腹腔7例,位于盆腔4例,位于泌尿道2例,位于前臂、颈部、甲状腺、眼眶、阴囊、腹股沟、锁骨下、小腿各1例。13例瘤体最大径〈5cm。8例瘤体最大径〉5cm。14例瘤体边界清楚,7例瘤体边界不清楚。15例瘤体为低回声.6例为囊实混合回声。14例瘤体血流信号不丰富.7例血流信号丰富。仅1例IMT发生腹腔大网膜多发转移,余20例IMT未发现转移灶。免疫组织化学检测显示：肿瘤细胞表达Vimentin、SMA、MSA、Desmin,其阳性率分别为100％（21／21）、100％（21／21）、100％（21／21）、47.6％（10／21）。结论超声检查能清楚显示IMT的部位、大小、形态、边界、内部回声、血流信号及其有无转移等.但超声图的表现无特异性.确诊仍依赖病理学及免疫组织化学检查。     

胃肠道钙化性纤维性肿瘤分子标志物的研究进展

钙化性纤维性肿瘤(calcifying fibrous tumor,CFT)是一种罕见的良性病变,其临床表现、影像学检查等不具有特异性.发生于胃肠道的CFT,易与其他胃肠道间叶源性肿瘤混淆,如胃肠道间质瘤(gastrointestinal stromal tumor,GIST)和炎性肌纤维母细胞瘤(inflammatory myofibroblastic tumor,IMT),在临床诊断时需进行分子标志物检测才可鉴别.CFT发病原因不明.近年来,越来越多的学者认为胃肠道CFT与IgG4相关性疾病(IgG4-RD)有关.     

Cystic nephroma: cytologic findings in fine-needle aspiration cytology.

This report presents the fine-needle aspiration cytology (FNAC) findings of a multicystic renal tumor found in a 3-year-old child. The smears contained benign epithelial cells isolated or arranged in sheets of uniform cells strongly suggesting the lining of the cysts. The combination of the imaging data with the FNAC findings favoured the diagnosis of cystic nephroma (CN), a benign renal tumor that is cured by surgery. Surgical pathology confirmed the diagnosis. CN should be added to the list of tumors of the kidney in infancy that appear to be diagnosable by FNAC/biopsy.     

Inflammatory myofibroblastic tumor of the posterior mediastinum: an older adult case with anaplastic lymphoma kinase abnormalities determined using immunohistochemistry and fluorescence in situ hybridization

Inflammatory myofibroblastic tumor (IMT) is a rare neoplasm that usually occurs in children and young adults. Anaplastic lymphoma kinase (ALK) abnormalities in IMT, determined using immunohistochemistry and/or molecular genetic studies, including fluorescence in situ hybridization (FISH), have almost been limited to children and young adults. In elderly cases of IMT, these ALK abnormalities are very rare. We report on a case of IMT arising in the posterior mediastinum of a 59-year-old Japanese man that showed ALK abnormalities determined using immunohistochemistry and FISH, suggesting the neoplastic nature of a subset of IMTs in older patients similar to those in younger ones and the presence of an additional mechanism(s) that allows them to start to grow late.     

胸部炎性肌纤维母细胞瘤的CT及MRI评价

目的 探讨胸部炎性肌纤维母细胞瘤(IMT)的CT和MRI表现,提高其影像学诊断水平.方法 回顾性分析2005年2月至2009年11月广州医学院第一附属医院及中山大学附属肿瘤医院住院并经手术病理证实的13例胸部IMT的影像学表现.结果 13例病灶中,肺5例,纵膈2例,食道上段1例,右侧胸壁2例,左侧胸壁2例,气管1例.肺IMT 1例为中央型,4例为周围型,为边缘清楚或不清的单发肿块.肺外IMT均表现为大小不一的单个实性软组织肿块,密度均匀或不均匀.2例病灶内出现斑点状钙化,1例胸壁IMT伴肋骨破坏.MR表现为T1WI呈稍低信号,T2WI呈不均匀高信号.结论 CT、MRI检查可清晰显示IMT的大小、形态及与周围组织的关系,对选择手术方案及评价治疗效果有重要参考价值.     

Inflammatory myofibroblastic tumor with valvular involvement: a case report and review of the literature

BACKGROUND: Inflammatory myofibroblastic tumor (IMT) of the heart is extremely rare with unpredictable clinical expression. IMTs, characterized by spindle cells, plasma cells, and a polymorphic inflammatory infiltrate, have a predilection for children and young adults. Clinically, IMT mimics malignancy, making a definitive diagnosis difficult, prior to histopathological examination. METHODS AND RESULTS: We describe a case of an intracardiac IMT in a 69-year-old woman. Histopathological examination of excised specimens revealed a marked, polymorphic, infiltrate of lymphocytes and plasma cells. CONCLUSIONS: Cardiac IMTs are benign lesions with favorable prognosis. Herein, we present this case, along with a review of all cardiac IMTs published after the last review in 2002, with emphasis on clinical, pathologic, and immunohistochemical features.     

Inflammatory Myofibroblastic Tumor of the Kidney Misdiagnosed as Renal Cell Carcinoma

The inflammatory myofibroblastic tumor (IMT), also knowns as inflammatory pseuduotumor, is a soft tissue lesion of unknown etiology. In the urogenital tract, IMT mainly affects the urinary bladder or prostate, but rarely the kidney. It has been considered as a nonneoplastic reactive inflammatory lesion, but nowadays, it is regarded as a neoplasm due to its high recurrence rate and metastasis. We describe a case of a 61-yr-old woman that had originally been misdiagnosed as renal cell carcinoma, which was pathologically revealed to be an IMT.     

腹腔脏器炎性肌纤维母细胞瘤的临床病理观察

目的探讨腹腔脏器炎性肌纤维母细胞瘤(inflammatorymyofibroblastictumor,IMT)的临床病理特征、组织发生和预后。方法分析10例IMT的临床病理学资料,所有病例行HE染色和免疫组化染色。结果腹腔脏器IMT的组织学分为三型:(1)黏液样-血管型,(2)梭型细胞密集型,(3)少细胞纤维瘢痕型。肿瘤细胞表达vimentin、SMA、MSA、CKpan和ALK,其阳性率分别为100%、90%、90%、60%和20%。10例IMT中有5例做了根治性切除术,另有5例单纯肿瘤切除,随访无1例复发。结论腹腔脏器IMT易被临床医师误诊为晚期癌。免疫组化表型是该肿瘤与其他软组织肿瘤鉴别的重要依据。腹腔脏器IMT一般具有良性病变的生物学行为,根治性手术可能治愈。     

Inflammatory myofibroblastic tumor of the appendix arising after treatment of gastric cancer: a case report and review of the literature

Inflammatory myofibroblastic tumor (IMT) is a rare soft tissue neoplasm of uncertain malignant potential and unclear etiology. IMT involving the appendix is very rare. Herein, we report a case of IMT of the appendix in a gastric cancer patient who was treated with radical gastrectomy and adjuvant systemic chemotherapy. Rare cases of IMT associated with preceding events have been described in other organs/sites, but not in the appendix. A previous intra‐abdominal operation for gastric cancer may contribute to the development of IMT in the appendix as seen in the present patient. To our knowledge, this is the first case of appendiceal IMT arising after a previous operation.     

Post-traumatic inflammatory pseudotumor of the breast with atypical morphological features: A potential diagnostic pitfall. Report of a case and a critical review of the literature

Although inflammatory pseudotumor (IPT) and inflammatory myofibroblastic tumor (IMT) may share a common morphology, they are distinct clinico-pathologic entities. Unfortunately, the terms IMT and IPT are still used interchangeably, especially when lesions occur in unusual sites, including breast. All the cases of IMT/IPT involving the breast have raised spontaneously without any apparent prior injury. We herein report the first case of a post-traumatic IPT of the breast parenchyma in a 22-year-old male. Histologically, the lesion was highly cellular and composed of spindle cells arranged in a predominant fascicular pattern. Notably, mono- or multi-nucleated large pleomorphic cells were observed. Inflammatory cells, especially plasma cells and lymphocytes, were closely admixed with the spindle cell proliferation. The overall picture was reminiscent of an “IMT with atypical features”, typically seen in lung, abdomen, pelvis, and retroperitoneum of children. Immunohistochemically, the spindle-shaped and large pleomorphic cells were immunoreactive to vimentin, α-smooth muscle actin, and desmin. No immunoreactivity was obtained with ALK-1 protein. The present case contributes to widening the morphological spectrum of IPT of the breast, emphasizing the possibility that a reactive lesion may contain large pleomorphic cells that may represent a potential diagnostic pitfall. Lastly, we suggest that the diagnosis of IMT of the breast should be rendered with caution when dealing with ALK-negative spindle cell lesions in adult patients, and alternative diagnoses, including IPT, should be seriously considered.     

Malignant lymphoma of the stomach in association with inflammatory myofibroblastic tumor of the spleen. A case report

We report on a case of a 40-year-old male patient who underwent a gastrectomy because of a biopsy-proven large B-cell lymphoma of the stomach. On surgery, a nodule in the spleen also was noted. Grossly and microscopically, the two lesions were different: the tumor of the stomach appeared white-gray on the cut surface and was a centroblastic variant of diffuse large B-cell lymphoma. Histologically, one perigastric lymph node was involved. Grossly, the splenic nodule was gray-yellow and had a histological appearance of an inflammatory myofibroblastic tumor (IMT). The association between malignant tumor and IMT is rare. In such an association, the latter lesion most often has been reported in the spleen. As EBV may be involved in the genesis of both lymphoma and IMT, we tested both lesions for its presence using in situ hybridization, but the tests were negative. It remains to be verified whether the association between lymphoma and IMT is more than fortuitous.     

Cytology of fine‐needle aspiration of inflammatory myofibroblastic tumor

Inflammatory Myofibroblastic Tumor (IMT) is a rare spindle cell neoplasm with a relatively indolent course. Its morphology may be confused with both reactive processes and/or malignant neoplasms on FNA specimens. Herein we discuss the cytologic features and IHC studies of IMT. The archives of the Department of Pathology at the Johns Hopkins Hospital were searched for IMT. A total of 257 cases were identified over a period of 11 years. Among them, 20 cases had cytology material. The patients' ages ranged from 7 to 81 years old with a median age of 54 years. The locations of the tumor in descending order were: liver (9/20, 45%), lung (8/20, 40%), abdomen (1/20, 5%), pelvis (1/20, 5%), and kidney (1/20, 5%). On FNA, the majority of tumors consisted of bland spindle cells with oval nuclei and small prominent nucleoli in a background of lymphocytes and plasma cells. Focal cytological atypia and “ganglion‐like” cells were identified in 7 cases, likely related to the risk of metastases and malignant transformation. The lesional cells expressed ALK (8/17, 47.1%) and actin (10/10, 100%), but with variable expression of cytokeratin. Ki‐67 showed low proliferative indices. ALK gene rearrangement was detected by FISH in three out of three cases and correlated with ALK protein expression by IHC. The cytologic diagnosis of IMT is challenging. When encountering a spindle cell lesion with prominent inflammatory component, a high index of suspicion in combination with the use of ancillary studies increases the diagnostic yield of IMT. Diagn. Cytopathol. 2010. © 2010 Wiley‐Liss, Inc.     

Inflammatory myofibroblastic tumor of the central nervous system and its relationship to inflammatory pseudotumor

Inflammatory myofibroblastic tumor (IMT) is a distinctive spindle cell lesion and occurs primarily in soft tissue. Recent evidence suggests a neoplastic nature, although historically, both neoplastic and nonneoplastic processes were combined in this category. Originally described as a nonneoplastic process, the term inflammatory pseudotumor (IP) has been used synonymously with IMT. IMTs have been linked to ALK gene (2p23) rearrangements, and some have suggested an association with the human herpesvirus 8 (HHV-8). IMT in the central nervous system (CNS) is rare, its characteristics are poorly defined, and its relation to similar tumors at other sites is unclear. To better characterize IMT within the CNS, we studied clinicopathologic features of 6 IMTs and compared them with 18 nonneoplastic lesions originally classified as IP. The IMT group consisted of 2 male and 4 female patients with a median age of 29 years. Of the six IMTs, 5 occurred within the cerebral hemispheres, and one was in the posterior fossa. All tumors were composed of neoplastic spindle cells and a variable amount of inflammatory infiltrate. Eighteen IPs included in this study consisted of predominantly inflammatory masses occasionally seen in the setting of systemic diseases. Only 1 IMT and none of the IPs recurred during the follow-up period. Four IMTs had either ALK protein overexpression or 2p23 rearrangement, and 1 case demonstrated both. None of the IPs were positive for ALK. Neither IMT nor IP cases demonstrated HHV-8 expression. We suggest that IMT in the CNS is distinct from the nonneoplastic IP, and distinguishing IMT from nonneoplastic lesions should enable better decisions for patient management.     

Inflammatory myofibroblastic tumor of the lung with unique histological pattern and association with Sjögren's disease and systemic lupus erythematosus

Inflammatory myofibroblastic tumor (IMT) of the lung is a rare condition. Radiological properties and clinical presentation of this disease can mimic malignant process. We present a case of IMT of the lung in a 58 year old female patient with a single lung nodule. Tumor was unencapsulated, firm, and well circumscribed. Microscopically tumor had multinodular structure with single or multiple small blood vessels in the center of each nodule surrounded in circular pattern by connective tissue containing spindle cells embedded into the thick layers of extracellular matrix. Extracellular matrix was identified as type I and type III collagen fibrils embedded into type IV collagen and laminin. The tumor was surrounded by T-, B-lymphocytes and polyclonal plasma cells. Histological organization of this lesion's stromal component was unique, but cell composition was similar to inflammatory pseudotumor of the lung. In addition, tumor tissue sections exhibited strong positivity for IgG, weak positivity for IgA, 1Cq, but were negative for IgM, and C3. Mutational analysis of the EGFR, KRAS genes and ALK locus rearrangement were performed and did not reveal any mutations. This is the first report of an IMT associated with Sjögren's disease, systemic lupus erythematosus and Non-Hodgkin lymphoma developing in the lungs. Patient was clinically followed up for 18 months and no recurrence of the tumor observed.     

Kidney carcinoid tumor: Histological,immunohistochemical and ultrastructural features

Primary carcinoid tumor of the kidney is uncommon. Only limited morphologic studies have been reported in the literature. In general, renal carcinoid tumor reveals typical morphological features as present in carcinoid tumors in the other organs, i.e. growing as trabeculae intermixed with nests of monotonous cuboidal cells that show “salt and pepper” nuclear appearance. As reported in other organs, different morphological expressions may also present in renal carcinoid tumors, such as papillary, pseudoglandular or solid growth patterns and clear/eosinophilic cytoplasmic changes. These morphological variations may mimic other benign and malignant primary neoplasms in the kidney. Therefore, in some cases, ancillary studies are necessary or even crucial to differentiate carcinoid tumors from other primary renal neoplasms.

Herewith, we report two primary renal carcinoid tumors with long follow-up, one showed typical morphological features of carcinoid tumor, and the second one revealed some uncommon histological findings. To differentiate these carcinoid tumors from other primary renal neoplasms and confirm neuroendocrine differentiation, immunohistochemical studies are valuable. In case 2 immunohistochemical stain results were not definitive and electron microscopy was performed confirming the diagnosis of carcinoid tumor.     

Fat-forming solitary fibrous tumor of the kidney: a case report and literature review

Fat-forming solitary fibrous tumor (SFT) is a rare soft tissue tumor. Herein, we reported a 30-year-old woman was found to have a solid mass measuring 60×45 mm in the right kidney on an abdominal computed tomography scan. The tumor was well-circumscribed and composed of cellular nodules with the classic SFT admixed with clusters and lobules of mature adipocytes. Immunohistochemistry staining showed that the tumor cells were diffusely and strongly positive for CD34 and Bcl-2, focally and weakly positive for CD99 and EMA. Mature adipocytes were positive for S-100 protein. Ki-67 expression was found in approximately 2% of tumor cells. However, tumor cells were negative for cytokeratin, S-100 protein, HMB-45, Melan-A, SMA, and CD117. We made the pathological diagnosis of fat-forming SFT of the right kidney. The differential diagnosis includes angiomyolipoma, liposarcoma, spindle cell lipoma, sarcomatoid renal cell carcinoma, synovial sarcoma, and gastrointestinal stromal tumor. The patient was alive and well without evidence of recurrence or metastasis at 19 months after tumor resection.