Case Of Inflammatory Myofibroblastic Tumor Research Articles

Inflammatory Myofibroblastic Tumours of the Urinary Bladder: Multi-Centre 18-Year Experience

Objective: To review a series of inflammatory myofibroblastic tumours (IMTs) of the urinary bladder in 10 hospitals in Hong Kong. Methods: A database search in the pathology archives of 10 hospitals in Hong Kong from 1995 to 2013 was performed using the key words ‘inflammatory myofibroblastic tumour', ‘inflammatory pseudotumour' and ‘spindle cell lesion'. Patient characteristics, clinical features, histological features, immunohistochemical staining results and treatment outcomes were reviewed. Results: Nine cases of IMT of the urinary bladder were retrieved. The mean age was 45.4 ± 22.8 years (range 11-78). Eight patients (88.9%) presented with haematuria and 5 patients (55.6%) had anaemia with a mean haemoglobin level of 6.8 ± 1.3 g/dl. Histologically, the majority of patients (77.8%) had a compact spindle cell pattern. Anaplastic lymphoma kinase staining was positive in 75% of cases. During a mean follow-up period of 43.4 months (range 8-94), none of them developed any local recurrence or distant metastasis. Conclusions: A high index of suspicion of IMT should be maintained for young patients presenting with bleeding bladder tumours and significant anaemia. IMTs of the urinary bladder run a benign disease course, and good prognosis can be achieved after surgical resection.

Aggressive Inflammatory Myofibroblastic Tumor of the Urinary Bladder in Children

Inflammatory myofibroblastic tumor (IMT) of the genitourinary tract is a well- known entity. The majority of the literature characterizes IMT of the bladder as a benign, slow growing tumor in children. We present two cases of aggressive IMT. Although rhabdomyosarcoma is more common, IMT should remain within the differential diagnosis for any bladder mass found in a child or young adult. The diagnosis of IMT is important in preventing unnecessary diagnostic procedures and guiding the appropriate treatment.

Inflammatory Myofibroblastic Tumor of the Aortic Valves Causing Sudden Cardiac Death: A Case Report and Review of the Literature

Cardiac inflammatory myofibroblastic tumor (IMT) is a rare entity affecting predominantly infants, children, and young adults. Although most tumors have a benign clinical course after complete surgical resection, some have significant clinical effects. We report the case of a 9-year-old girl who had sudden cardiac death as a result of occlusion of the left circumflex coronary artery. A review of 57 cases of cardiac IMTs reported in the literature in terms of epidemiology, clinical presentation, histologic and immunohistologic features, and outcome is presented. Recognition of this rare abnormality is important in order to initiate prompt surgical intervention.

ALK-Positive Inflammatory Myofibroblastic Tumor of the Abdomen With Widespread Microscopic Multifocality

Inflammatory myofibroblastic tumor (IMT) is a locally aggressive neoplasm, most frequently occurring in the abdominal cavity as multiple recurrent nodules. We report a case of IMT in a 24-year-old male presenting as multiple nodules involving the omentum, the liver, and the colon. Spindle tumor cells expressed ALK with a cytoplasmic granular distribution, the CLTC-ALK fusion gene was demonstrated by reverse-transcriptase polymerase chain reaction analysis, and break-apart fluorescence in situ hybridization (FISH) probes for the ALK gene showed a pathological pattern (single red signal associated with 1/2 normal fused signals) highly suggestive for combined gene fusion and deletion. To reduce the surgically unresectable liver mass, the patient was treated with crizotinib, and after 4 months of treatment the disease was defined stable according to RECIST criteria. Interestingly, ALK and FISH/FICTION analysis revealed that tumor cells were widely dispersed as multiple microscopic foci or as single cells beneath the omental mesothelium. These findings indicate that IMT multifocality might result either from dissemination from the main tumor mass or development of multiple independent neoplastic foci; furthermore, they underline the need of omentectomy in abdominal IMT to obtain surgical radicality.

胸壁皮下に発生した炎症性筋線維芽細胞性腫瘍の1例

胸壁皮下に発生した炎症性筋線維芽細胞性腫瘍の1例

Bronchoscopic resection of endobronchial inflammatory myofibroblastic tumor: A case report and systematic review of the literature.

Inflammatory myofibroblastic tumour (IMT) is a rare tumour affecting the tracheo-bronchial tree in the adult population. The clinical presentation of this tumour is diverse and diagnosis can be definitively clinched by histopathological examination. Treatment of this tumour usually requires surgical resection with bronchoscopic resection being described in few cases. We describe a 32 year old male presenting with hemoptysis who was diagnosed to have IMT. Resection of the tumour was done with the help of rigid bronchoscopy. Post-resection, hemoptysis stopped and no recurrence of tumour was noted on subsequent follow-up. We also present a systematic review of literature of all the cases of tracheo-bronchial IMT treated with bronchoscopic resection and conclude it to be a useful alternative to surgery in such cases.

Inflammatory myofibroblastic tumor of the thyroid gland: a brief case report.

Inflammatory myofibroblastic tumor (IMT), also known as inflammatory pseudotumor (IPT) or plasma cell granuloma (PCG), is a histologically distinctive lesion that occurs primarily in the viscera and soft tissue, but has been reported in nearly every site in the body. 1 Until now, only 19 cases of IMT that have involved the thyroid have been reported in the English literature. 2 Moreover, most cases in the thyroid have been described as cases of PCG, 2 and the myofibroblastic component in the thyroid cases is usually not prominent. Only one case has reported IMT of the thyroid gland. 3 This study reports and discusses the differential diagnosis of an IMT of the thyroid gland that occurred in a 50-year-old Korean woman and which showed a predominantly myofibroblastic proliferation.

Inflammatory Myofibroblastic Tumor of the Larynx—A Case Report

Inflammatory myofibroblastic tumor (IMT) is a borderline neoplasm with uncertain malignant potential. It is a rare disease also referred to as an inflammatory pseudotumor, a plasma cell granuloma, and an inflammatory fibrosarcoma. IMT rarely also involves the head and neck region with only 50 cases of laryngeal IMT reported in the literature, and this is the first case with reported magnetic resonance imaging (MRI) findings. A 37-year-old man with a 1-year history of hoarseness, dysphagia, and fatigue presented with a right vocal fold submucosal mass and was treated conservatively. The MRI of the neck revealed a mildly spontaneously hyperintense right true vocal fold on GRE images and relative hyperintensity on fat-saturation T2-weighted images. A biopsy of the right-sided submucosal laryngeal mass was performed and the pathologic examination revealed a lesion consistent with an IMT. IMT is a borderline neoplasm with uncertain malignant potential. There are many variants of IMT and its etiology is not truly understood. In general, IMT of the larynx has a benign clinical course with low rates of recurrence.

Inflammatory myofibroblastic tumors of the duodenum

Inflammatory myofibroblastic tumors (IMTs) are rare soft-tissue tumors that can occur at virtually any anatomical site. We report the case of a 58-year-old male with an IMT of the fourth part of the duodenum who presented with signs and symptoms of high intestinal obstruction and bilious vomiting. The patient underwent a surgical resection of the fourth part of the duodenum with end-to-end duodenojejunal anastomosis. The follow-up period of 6months was uneventful with no evidence of recurrence. According to our knowledge, only six cases of duodenal IMTs have been reported in the literature thus far, and this is the first report of a duodenal IMT sited at the fourth part of the duodenum. The duodenum is among the rarest sites of IMTs. Signs and symptoms resulting from diagnostic imaging investigations are nonspecific and inadequate to obtain diagnosis accurately. In most cases, surgical treatment is considered a cure for IMTs. There is no evidence of deaths caused by duodenal IMT. IMT of the duodenum is a possible diagnosis in differential diagnosis of tumor-like lesions of the duodenum.

Huge Inflammatory Myofibroblastic Tumor of Pleura With Concomitant Nuchal Fibroma

Inflammatory myofibroblastic tumor (IMT) is a rare benign neoplasm. It is a challenging disease because the symptoms and radiologic findings are diverse and nonspecific. Although pulmonary IMT is the most common form, pleural origin is an extremely rare clinical entity. Nuchal fibroma (NF) is another rare benign neoplasm. We report herein a case of pleural IMT with concomitant NF in a 15-year-old girl. To the best of our knowledge, this is the first report suggesting an association between IMT and NF, and our case had the largest reported intrathoracic IMT. Moreover, we founda possible association between IMT and increased CA-125 levels.

Inflammatory Myofibroblastic Tumor of the Larynx

Objectives: 1) Describe a case of inflammatory myofibroblastic tumor (IMFT) of the larynx. 2) Analyze the literature regarding the disease process. 3) Discuss appropriate management options and prognosis. Methods: Retrospective review of a single case of IMFT involving the subglottic region at the University of Texas Medical Branch. Results: Here we present IMFT of the larynx, a benign condition often believed to be a malignancy due to its aggressive proliferative nature. The majority of extra-laryngeal cases are pulmonary; however, only 25 cases in the larynx have been described. Involvement of the subglottis is even rarer, representing only 5 cases. The origin is still unknown; however, many believe IMFT to be of clonal origin with chromosomal rearrangements of 2p23 and the ALK1 gene. These abnormalities are found in up to 47% of cases either immunohistochemically or using fluorescence in situ hybridization. Microsuspension laryngoscopy with laser excision has been the mainstay of treatment, and long term results have been excellent. Our patient’s tumor was ALK1 positive on immunohistochemistry, confirming the diagnosis. As observed in previous cases, our patient has responded well to conservative resection with no recurrence at this time. Conclusions: IMFT is an aggressive tumor of the larynx. However, conservative excision is adequate for tumor control and good long term survival. Knowledge of the disease is important to prevent overtreatment.

Radiological and histopathological features of hepatic inflammatory myofibroblastic tumour: Analysis of 10 cases

To evaluate the radiological and histopathological features of hepatic inflammatory myofibroblastic tumours (IMTs), and improve the understanding of this tumour. A retrospective analysis of radiological and histopathological features of 10 cases of IMT was carried out from May 2003 to September 2011 at the Second Xiangya Hospital of Central South University. Ten cases (five male and five female patients; age range 4-68 years) were enrolled. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed that the lesions were regular, hypodense or hypointense (T1WI) masses with well-defined borders (n=8) or ill-defined borders (n=2). The maximum diameter ranged from 3.1-13.4cm (mean=6cm). The masses showed homogeneous (n=8) or inhomogeneous (n=2) density. Contrast-enhanced CT and MRI showed the lesions were mildly, irregularly enhanced (n=7) or not enhanced (n=2) in the arterial phase and markedly enhanced in the portal venous phase or delayed phase. Hepatic arteriography revealed that the lesions were hypovascular and had a well-defined border. One patient had lung metastasis with obvious arterial phase enhancement. None of the patients had a history of hepatitis, cirrhosis, or enlarged lymph nodes. Pathology showed that the gross appearance of the tumours was smooth. The tumour cells comprised spindle-shaped fibroblast and myofibroblast cells with abundant inflammatory cells. Immunohistochemistry showed that most were positive for vimentin, smooth muscle actin (SMA), and CD68, but negative for CD34, anaplastic lymphoma kinase (ALK), S-100, and CD117. Radiological features of IMT have some characteristics of an intermediate-grade malignant tumour. However, imaging alone cannot be used to diagnose IMT. Therefore, histopathological examination is necessary for confirmation.

Inflammatory Myofibroblastic Tumor: A Rapidly Growing Soft Tissue Mass in the Posterior Mandible

The term inflammatory myofibroblastic tumor (IMT) encompasses a diverse group of spindle cell entities that traverses a clinical and histologic spectrum, extending from reactive to benign neoplastic to highly aggressive with malignant inclinations. Head and neck IMTs are rarely seen and comprise less than 5% of tumors. Here we report a case of a 30year old male who presented with a rapidly enlarging and extremely painful growth in the right posterior mandible, post extraction. Histopathological examination revealed a highly cellular connective tissue stroma comprised of spindle shaped cells arranged in fascicles, admixed with inflammatory cells, predominantly plasma cells. Apart from routine hematological investigations, serum protein electrophoresis was also performed. The final diagnosis was confirmed by a panel of immunomarkers consisting of MPO, CD34, CD20, CD3, CD23, CD138, SMA and ALK. To the best of our knowledge, this is the third case of oral IMT arising from an extraction socket.

Intraosseous Inflammatory Myofibroblastic Tumor of the Twelfth Thoracic Vertebra

Inflammatory myofibroblastic tumor (IMT) consisting of a spindle cell proliferation admixed with an inflammatory component is an uncommon mesenchymal lesion that can arise in multiple anatomic locations1,2. Because of its heterogenic histologic appearance, different synonyms have been used throughout the literature, including inflammatory pseudotumor, fibromyxoid lesion, pseudosarcomatous myofibroblastic tumor, and plasma cell granuloma2. Although IMTs have been considered benign proliferation of myofibroblasts, they have potential for recurrence, persistent local growth3, and local aggressive behavior2,4. Their malignant potential is controversal5. The rare entity of multilocality has been described6,7 and might even be interpreted as metastatic occurrence8. Although very rare, even paraneoplastic symptoms have been attributed to IMT9. While IMTs seem to localize predominantly in the viscera (e.g., lung) and soft tissue (e.g., mesentery and omentum)2, they arise very infrequently in bone. Their occurrence has been reported in cranial bones10-12, long bones13,14, the sacrum9, and the iliac bone15. IMTs in the spinal system are also reported16-19, but primary occurrence in a vertebral body is extremely rare1. Roberts et al. described a case of an IMT of the T10 vertebra compressing the thecal sac and involving the paravertebral muscles20, but it was unclear whether the IMT originated from the vertebral bone or from the surrounding soft tissues. We present a rare case of a localized IMT in the T12 vertebra, and we describe the difficulties involved in histological diagnosis and treatment. The patient was informed that data concerning the case would be submitted for publication, and she provided consent. A thirty-seven-year-old woman presented with persistent and localized constant pain at …

Intraosseous Inflammatory Myofibroblastic Tumor of the Twelfth Thoracic Vertebra: Report of a Rare Case with Histological Diagnosis and Surgical Treatment.

Inflammatory myofibroblastic tumor (IMT) consisting of a spindle cell proliferation admixed with an inflammatory component is an uncommon mesenchymal lesion that can arise in multiple anatomic locations1,2. Because of its heterogenic histologic appearance, different synonyms have been used throughout the literature, including inflammatory pseudotumor, fibromyxoid lesion, pseudosarcomatous myofibroblastic tumor, and plasma cell granuloma2. Although IMTs have been considered benign proliferation of myofibroblasts, they have potential for recurrence, persistent local growth3, and local aggressive behavior2,4. Their malignant potential is controversal5. The rare entity of multilocality has been described6,7 and might even be interpreted as metastatic occurrence8. Although very rare, even paraneoplastic symptoms have been attributed to IMT9. While IMTs seem to localize predominantly in the viscera (e.g., lung) and soft tissue (e.g., mesentery and omentum)2, they arise very infrequently in bone. Their occurrence has been reported in cranial bones10-12, long bones13,14, the sacrum9, and the iliac bone15. IMTs in the spinal system are also reported16-19, but primary occurrence in a vertebral body is extremely rare1. Roberts et al. described a case of an IMT of the T10 vertebra compressing the thecal sac and involving the paravertebral muscles20, but it was unclear whether the IMT originated from the vertebral bone or from the surrounding soft tissues. We present a rare case of a localized IMT in the T12 vertebra, and we describe the difficulties involved in histological diagnosis and treatment. The patient was informed that data concerning the case would be submitted for publication, and she provided consent. A thirty-seven-year-old woman presented with persistent and localized constant pain at …

Inflammatory myofibroblastic tumor of parotid in infancy—A new entity

Inflammatory myofibroblastic tumor is commonly found in lungs, mesentery or omentum in the younger population. We hereby report a hitherto unreported case of inflammatory myofibroblastic tumor, arising from the parotid gland in an infant. Diagnosis by histopathology and its treatment by superficial parotidectomy is being presented.

Clinical analysis of 14 patients with extraorbital inflammatory myofibroblastic tumor of the head and neck

To summarize the clinical features of extraorbital inflammatory myofibroblastic tumor (IMT) of the head and neck. Fourteen cases of extraorbital IMT treated in recent 20 years were analyzed retrospectively. Of the 14 patients, 9 cases with limited lesion in maxilla (n = 5), mandible (n = 2) or neck (n = 2) underwent local resection, and no recurrences were found after 1.5 to 20.0 years; 3 cases diagnosed as maxillary IMT involved in orbit, hard palate or pterygopalatine fossa received conservative therapy (prednisone, prednisone plus radiotherapy or prednisone plus chemotherapy), and no disease progression was found after 6, 9 or 2 years respectively; and 1 case diagnosed as maxillary IMT involved in orbit and pterygopalatine fossa was confirmed with cervical metastases after two operations and died of brain invasion within 17 months. One patient with localized lesion around the common carotid artery was treated with prednisone and had no disease progression with a 2-year follow-up. Extraorbital IMT of the head and neck is a rare clinical entity. Pathology examination is required for final diagnosis. Corticosteroid administration may be a choice of treatments, and radical resection should be taken selectively for limited lesions.

English

Inflammatory myofibroblastic tumor (IMT) comprise of rare group of lesions characterized histologically by acute and chronic inflammatory cells with variable degree of fibrous stroma . Also called as Plasma cell granuloma or Inflammatory pseudotumor IMTs are most often seen in lung of young adults(2). The extrapulmonary sites are rare , and are found mostly in liver, spleen and small intestinal mesentery. Large bowel mesentery , rectum and appendenceal sites of IMTs are not often known(1,3,4).We present a case of IMT of sigmoid mesocolon in 27 years old young female patient whose symptoms improved after surgical resection of tumor.

Use of PAX8 and GATA3 in diagnosing sarcomatoid renal cell carcinoma and sarcomatoid urothelial carcinoma

Immunohistochemistry for PAX8 and GATA3 are sensitive markers for renal cell carcinoma and urothelial carcinoma, respectively. However, there are limited data on these markers in sarcomatoid renal cell carcinoma (SARCRCC) and sarcomatoid urothelial carcinoma (SARCUC). Tissue microarrays (TMAs) were constructed from 45 cases of SARCRCC and 45 cases of SARCUC of the lower urinary tract, with an additional 11 SARCUCs of the upper tract. PAX8 and GATA3 were also evaluated in TMAs from 161 sarcomas from other sites, 14 atypical epithelioid angiomyolipomas (AMLs) of the kidney, 23 bladder inflammatory myofibroblastic tumors (IMTs), and 2 bladder and 4 renal leiomyosarcomas. In the SARCRCC, PAX8 and GATA3 were positive in the sarcomatoid areas in 31 (69%) and 0 (0%) of cases, respectively. In the bladder SARCUC, GATA3 and PAX8 were positive in 14 (31%) and 2 (4%) of cases, respectively. Of the 11 SARCUCs of the upper urinary tract, 2 (18%) cases were PAX8 positive and 2 (18%) separate cases were GATA3 positive. Only 1 tumor present on the sarcoma TMAs, a Ewing sarcoma/primitive neuroectodermal tumor, was PAX8 positive, and all sarcomas were GATA3 negative. Of the AMLs, IMTs, and leiomyosarcoma, only 1 case of IMT showed moderate GATA3 positivity, and all were negative for PAX8. PAX8 can be used to distinguish SARCCRCC from atypical epithelioid AMLs and primary renal or retroperitoneal sarcomas. However, in a kidney/renal pelvic tumor, PAX8 shows overlap in staining between SARCUC and SARCRCC. GATA3 lacks sensitivity but is more specific for SARCUC.

上顎癌根治治療後に発生した炎症性筋線維芽細胞性腫瘍の1例

症例は68歳，男性。上顎癌に対し，20年前に手術と放射線加療を行い経過観察していた。上顎の切除部に肉芽様組織が出現し増大傾向を示したため，手術を行った。病理診断は炎症性筋線維芽細胞性腫瘍（Inflammatory myofibroblastic tumor；IMT）であった。組織学的所見は筋線維芽細胞様の紡錘形細胞が増生し，炎症細胞の浸潤が散見された。免疫組織染色ではvimentinとSMAは陽性，ALK，desmin，サイトケラチンは陰性であった。これまで炎症性偽腫瘍や形質細胞性肉芽腫などと診断されていた病変がIMTに該当すると考えられている。