scholarly journals An Unusual Case of Systemic Inflammatory Myofibroblastic Tumor with Successful Treatment with ALK-Inhibitor

2014 ◽  
Vol 2014 ◽  
pp. 1-5 ◽  
Author(s):  
Sanjivini V. Jacob ◽  
John D. Reith ◽  
Angerika Y. Kojima ◽  
William D. Williams ◽  
Chen Liu ◽  
...  
Keyword(s):  
Inflammatory Myofibroblastic Tumor ◽  
Smooth Muscle Actin ◽  
Portal Tract ◽  
Fish Analysis ◽  
Anaplastic Lymphoma ◽  
Alk Inhibitor ◽  
Spindle Cell Proliferation ◽  
History Of ◽  
Subcutaneous Tissues ◽  
Systemic Manifestations

Systemic inflammatory myofibroblastic tumor is an exceedingly rare entity. A 45-year-old Hispanic female presented with a 6-month history of left-sided thigh pain, low back pain, and generalized weakness. PET/CT scan revealed abnormal activity in the liver, adrenal gland, and pancreas. MRI of the abdomen demonstrated two 6-7 cm masses in the liver. MRI of the lumbar spine demonstrated lesions in the L2 to L4 spinous processes, paraspinal muscles, and subcutaneous tissues, as well as an 8 mm enhancing intradural lesion at T11, all thought to be metastatic disease. A biopsy of the liver showed portal tract expansion by a spindle cell proliferation rich in inflammation. Tumor cells showed immunoreactivity for smooth muscle actin and anaplastic lymphoma kinase 1 (ALK1). Tissue from the L5 vertebra showed a process histologically identical to that seen in the liver. FISH analysis of these lesions demonstrated an ALK (2p23) gene rearrangement. The patient was successfully treated with an ALK-inhibitor, Crizotinib, and is now in complete remission. We present the first reported case, to our knowledge, of inflammatory myofibroblastic tumor with systemic manifestations and ALK translocation. This case is a prime example of how personalized medicine has vastly improved patient care through the use of molecular-targeted therapy.

scholarly journals Voluminous Omental Inflammatory Myofibroblastic Tumor in an Elderly Man: A Case Report and Literature Review

2015 ◽  
Vol 2015 ◽  
pp. 1-5 ◽  
Author(s):  
Pasquale Cianci ◽  
Antonio Ambrosi ◽  
Alberto Fersini ◽  
Nicola Tartaglia ◽  
Vincenzo Lizzi ◽  
...  
Keyword(s):  
Smooth Muscle ◽  
Inflammatory Myofibroblastic Tumor ◽  
Plasma Cells ◽  
Smooth Muscle Actin ◽  
Abdominal Mass ◽  
Abdominal Distension ◽  
Greater Omentum ◽  
Anaplastic Lymphoma ◽  
Mesenchymal Neoplasm ◽  
Spindle Cell Proliferation

Inflammatory myofibroblastic tumor (IMT) is a rare neoplasm of intermediate biologic potential, with uncertain etiology. This tumor occurs primarily in the lung, but the tumor may affect any organ system. A 75-year-old male was evaluated for voluminous palpable high abdominal mass with continuous and moderately abdominal pain, associated with abdominal distension for the last two months. Abdominal computed tomography showed a large (32×29×15 cm) heterogeneously enhanced mass with well-defined margins. At surgery, the mass originated from the greater omentum was completely excised. Histologically the tumor was a mesenchymal neoplasm in smooth muscle differentiation and was characterized by spindle-cell proliferation with lymphocytes, plasma cells, and rare eosinophils. Immunohistochemically, the tumor cells were positive for vimentin and smooth muscle actin and negative for anaplastic lymphoma kinase. Complete surgical resection of IMTs remains the mainstay of treatment associated with a low rate of recurrence. Final diagnosis should be based on histopathological and immunohistochemical findings. Appropriate awareness should be exercised by surgeons to abdominal IMTs in combination with constitutional symptoms, abnormal hematologic findings, and radiological definition, to avoid misdiagnosed.

Intraocular Inflammatory Myofibroblastic Tumor With ALK Overexpression

2004 ◽  
Vol 128 (1) ◽  
pp. e5-e7
Author(s):  
Dennis P. O'Malley ◽  
Christopher Poulos ◽  
Magdalena Czader ◽  
Warren G. Sanger ◽  
Attilio Orazi
Keyword(s):  
Tumor Cells ◽  
Inflammatory Myofibroblastic Tumor ◽  
Plasma Cells ◽  
Smooth Muscle Actin ◽  
Chromosome 2 ◽  
Muscle Actin ◽  
Anaplastic Lymphoma ◽  
Spindle Cells ◽  
Multiple Copies

Abstract We report a case of an intraocular inflammatory myofibroblastic tumor nearly filling the vitreous cavity of the eye of a 50-year-old man. The tumor was composed of a mixture of spindle cells and mixed inflammatory elements, including numerous plasma cells. The differential diagnosis included inflammatory pseudotumor and neoplastic mimics of this condition. Further investigation with immunohistochemistry revealed the mass to be composed of myofibroblasts, positive for smooth muscle actin stains and with weak anaplastic lymphoma kinase (ALK) expression in some tumor cells. Evaluation by fluorescence in situ hybridization revealed the tumor cells to have multiple copies of chromosome 2 and ALK but no rearrangement of the ALK gene. The authors propose that multiple copies of the ALK gene may be involved in inflammatory myofibroblastic tumor tumorigenesis, in addition to ALK gene rearrangements.

Inflammatory myofibroblastic tumor: A multi-institutional study from the pediatric surgical oncology research collaborative.

2021 ◽  
Vol 39 (15_suppl) ◽  
pp. 10024-10024
Author(s):  
Barrie S. Rich ◽  
Joanna Stein Fishbein ◽  
Timothy Lautz ◽  
Nathan S. Rubalcava ◽  
Rodrigo L. P. Romao ◽  
...  
Keyword(s):  
Tumor Size ◽  
Respiratory Symptoms ◽  
Inflammatory Myofibroblastic Tumor ◽  
Surgical Oncology ◽  
Distant Disease ◽  
Palpable Mass ◽  
Anaplastic Lymphoma ◽  
Alk Inhibitor ◽  
Oncology Research ◽  
Mesenchymal Neoplasm

10024 Background: Inflammatory myofibroblastic tumor (IMT) is a mesenchymal neoplasm of intermediate malignancy, predominantly seen in children and young adults. Given its rarity, data are limited. We describe the largest cohort of patients with IMT to date, with an aim to further characterize this poorly understood tumor. Methods: A multi-institutional review was performed at 18 North American hospitals participating in the Pediatric Surgical Oncology Research Collaborative to identify IMT patients ≤39 years, diagnosed from 2000-2018. Descriptive statistics are described as median and interquartile range. Multivariable analysis was used to identify predictors of event free survival (EFS). Results: 182 patients were identified with a median age of 11 years (5-17); 52% were female. 33% of tumors were thoracic, 26% abdominal/pelvic, 20% head/neck, and 14% genitourinary. Common presenting symptoms included pain (29%), respiratory symptoms (24%), weight loss (12%), and a palpable mass (10%). Median tumor size was 3.9 cm (1.9-6.5). Anaplastic lymphoma kinase (ALK) overexpression was identified via immunohistochemistry in 53% of patients tested. One third of patients had abnormal cytogenetics, with 12% of the entire cohort having an ALK mutation. 7% of patients had distant disease at diagnosis. 13% of patients received neoadjuvant therapy including chemotherapy (3%), ALK inhibitor (4%), radiation (0.5%), non-steroidal anti-inflammatories (NSAIDs) (7%), or steroids (2%). Of those who underwent resection with known margin status (n = 158), 66% had complete resection, 20% had microscopic positive margins, and 14% had gross residual disease. Just over 40% of patients had an en bloc resection of involved organs, most commonly lung (26%). 21% of patients received adjuvant therapy, including chemotherapy (3%), ALK inhibitor (9%), radiation (0.5%), NSAIDs (8%), or steroids (5%). 12% of all patients received an ALK inhibitor: 24% neoadjuvant, 62% adjuvant, and 14% without surgery. Median follow-up time was 36 months (14-69). Overall 5-year survival (OS) was 95% and 5-year EFS was 80%. Predictors of recurrence included respiratory symptoms, larger tumor size, or distant disease at diagnosis. Gender, race, age and primary site were not predictive of EFS. Likewise, there was no association of ALK overexpression or ALK mutation with EFS. The presence of gross or microscopic margins following resection was not associated with recurrence. Conclusions: IMT is a rare tumor with favorable OS. Five year recurrence rate was 20%. Presenting with respiratory symptoms, larger tumor size, or metastatic disease was associated with recurrence, while ALK positivity was not. Notably, a positive margin after resection was not associated with increased recurrence, indicating that aggressive attempts at surgical resection that would compromise form or function may not be warranted.

scholarly journals Inflammatory Myofibroblastic Tumor of the Lung and the Maxillary Region: A Benign Lesion with Aggressive Behavior

2013 ◽  
Vol 2013 ◽  
pp. 1-5
Author(s):  
Lorena Gallego ◽  
Tania R. Santamarta ◽  
Verónica Blanco ◽  
Luis García-Consuegra ◽  
Tommaso Cutilli ◽  
...  
Keyword(s):  
Aggressive Behavior ◽  
Inflammatory Myofibroblastic Tumor ◽  
Smooth Muscle Actin ◽  
Benign Lesion ◽  
Distant Metastases ◽  
Oral Lesion ◽  
The Body ◽  
Spindle Cell Proliferation ◽  
Intense Immunoreactivity ◽  
Maxillary Region

Inflammatory myofibroblastic tumor (IMT) is a rare mass-forming lesion characterized by fibroblastic or myofibroblastic spindle cell proliferation with varying degrees of inflammatory cell infiltration. Although it has been reported in virtually every organ in the body, the lung is the most common site of involvement. Extrapulmonary IMTs, although rare, have been reported and are characterized by different, more aggressive behavior. We report an extremely rare case of maxillary metastases of pulmonary IMT. Lung IMT was initially misdiagnosed, and oral lesion mimicked clinically and radiologically a radicular cyst. On histologic examination, cells exhibited diffuse and intense immunoreactivity forα-smooth muscle actin and vimentin whereas both pulmonary and oral IMTs presented absence of cellular atypia and lack of expressivity of oncogenic determinants. Distant metastases of lung IMT are extremely unusual, and this is the first report to our knowledge with this particular clinical course. Despite the possibility that the present case could also represent a metachronous multifocal IMT, with pulmonary and extrapulmonary lesions, similar histopathological and immunohistochemical patterns in lung and maxillary region suggest a metastatic course.

scholarly journals Inflammatory Myofibroblastic Tumor of the Nasal Septum

2013 ◽  
Vol 2013 ◽  
pp. 1-4 ◽  
Author(s):  
Yuri Okumura ◽  
Kazuhiro Nomura ◽  
Takeshi Oshima ◽  
Atsuko Kasajima ◽  
Takahiro Suzuki ◽  
...  
Keyword(s):  
Nasal Septum ◽  
Inflammatory Myofibroblastic Tumor ◽  
Clinical Presentation ◽  
Smooth Muscle Actin ◽  
Imaging Studies ◽  
Posterior Edge ◽  
Juvenile Angiofibroma ◽  
Anaplastic Lymphoma ◽  
Spindle Cells ◽  
One Year

We report an extremely rare case of inflammatory myofibroblastic tumor of the posterior edge of the nasal septum. An 11-year-old boy presented with frequent epistaxis and nasal obstruction persisting for one year. Based on the clinical presentation and imaging studies, juvenile angiofibroma was suspected, but angiography suggested the possibility of another type of tumor. Transnasal endoscopic surgery found that the tumor protruded into the nasopharynx from the posterior end of the nasal septum. Histological examination identified spindle cells with immunoreaction for vimentin, smooth muscle actin, and anaplastic lymphoma kinase (ALK), but not for desmin and cytokeratin. This is a report of inflammatory myofibroblastic tumor mimicking juvenile angiofibroma. This case suggests that angiography is helpful in the differential diagnosis of epipharyngeal tumor in adolescence.

Anaplastic Lymphoma Kinase (ALK) Inhibitor in Treatment of Inflammatory Myofibroblastic Tumor of the Tongue in Infancy

2020 ◽  
Vol Publish Ahead of Print ◽  
Author(s):  
Karishma Rao ◽  
Sana Farooki ◽  
Erin Guest ◽  
Katherine Chastain ◽  
Melissa Gener ◽  
...  
Keyword(s):  
Anaplastic Lymphoma Kinase ◽  
Inflammatory Myofibroblastic Tumor ◽  
Anaplastic Lymphoma ◽  
Alk Inhibitor

scholarly journals Inflammatory Myofibroblastic Tumor of the Urinary Bladder: A Case Report

2016 ◽  
Vol 9 (2) ◽  
pp. 464-469 ◽  
Author(s):  
Toshiki Etani ◽  
Taku Naiki ◽  
Takashi Nagai ◽  
Keitaro Iida ◽  
Ryosuke Ando ◽  
...  
Keyword(s):  
Urinary Bladder ◽  
Inflammatory Myofibroblastic Tumor ◽  
Plasma Cells ◽  
Smooth Muscle Actin ◽  
Vascular Endothelial ◽  
Inflammatory Infiltration ◽  
Anaplastic Lymphoma ◽  
Resected Tumor ◽  
Muscle Invasion ◽  
Endothelial Growth Factor

An inflammatory myofibroblastic tumor (IMT) is a distinctive neoplasm composed of myofibroblastic and fibroblastic spindle cells, accompanied by inflammatory infiltration of plasma cells, lymphocytes, and eosinophils. IMTs rarely occur in the urinary bladder. It is important to distinguish this tumor from other malignant spindle cell tumors. Herein, we report a patient with an IMT showing muscle invasion, who underwent a transurethral resection of the bladder tumor and, at a later date, partial cystectomy. The resected tumor specimen revealed a proliferation of spindle-shaped cells on a background of plasma cells and lymphocytes. Immunohistochemical staining showed the tumor to be positive for anaplastic lymphoma kinase (ALK), smooth muscle actin, and vascular endothelial growth factor (VEGF). Such histopathological findings were indicative of an IMT, suggesting the use of inhibitors of ALK and VEGF as pharmacotherapy.

scholarly journals Unusual Case of Inflammatory Myofibroblastic Tumor in Maxilla

2013 ◽  
Vol 2013 ◽  
pp. 1-4 ◽  
Author(s):  
Jaana Rautava ◽  
Tero Soukka ◽  
Esko Peltonen ◽  
Petri Nurmenniemi ◽  
Markku Kallajoki ◽  
...  
Keyword(s):  
Inflammatory Myofibroblastic Tumor ◽  
Unusual Case ◽  
Root Resorption ◽  
Histopathological Examination ◽  
Smooth Muscle Actin ◽  
Clinical Manifestations ◽  
Anaplastic Lymphoma ◽  
First Case ◽  
Rare Lesion ◽  
Children And Young Adults

Inflammatory myofibroblastic tumor (IMT) is a rare lesion found mostly in children and young adults and originates from the lung, abdominopelvic region, and retroperitoneum. Clinical manifestations of IMT or imaging are nonspecific and diagnosis is based on histopathological and immunohistochemical findings. Minority of all IMTs will metastasize. IMT in the oral cavity is an extreme rarity and this is a first case report of IMT in maxilla causing delayed tooth eruption and multiple cervical root resorption with an 11-year-old child. The IMT reported here was positive for smooth muscle actin, vimentin, and anaplastic lymphoma kinase (ALK1) with immunohistochemistry. Only three IMTs of the jaws have been reported so far and none of them had delayed root eruption and tooth resorption. This unusual case of IMT in a child was also ALK1- positive supporting neoplastic origin of her tumor. The case presented here underscores the importance of histopathological examination of the tissue found in any root resorption especially in the case of multiple resorptions.

scholarly journals Anaplastic lymphoma kinase-negative uterine inflammatory myofibroblastic tumor containing the ETV6-NTRK3 fusion gene: a case report

2018 ◽  
Vol 46 (8) ◽  
pp. 3498-3503 ◽  
Author(s):  
Akimasa Takahashi ◽  
Manabu Kurosawa ◽  
Mao Uemura ◽  
Jun Kitazawa ◽  
Yoshihiko Hayashi
Keyword(s):  
Anaplastic Lymphoma Kinase ◽  
Fusion Gene ◽  
Smooth Muscle Actin ◽  
Side Wall ◽  
Definitive Treatment ◽  
Maximum Diameter ◽  
Tyrosine Kinase Receptor ◽  
Kinase Gene ◽  
Anaplastic Lymphoma ◽  
Spindle Cell Proliferation

Inflammatory myofibroblastic tumors (IMTs) are neoplasms with low malignant potential, and the most common tumor in the lung and orbit. Their occurrence in the uterus is rare. Approximately 50% of IMT patients have anaplastic lymphoma kinase gene ( ALK) rearrangements. Recent studies described novel fusions involving ROS1, platelet-derived growth factor receptor beta ( PDGFR-β), and ETS translocation variant ( ETV6) genes in a subset of ALK-negative patients. We report a 44-year-old woman with anemia and uterine IMT. Ultrasonography and magnetic resonance imaging revealed a myxoid degenerative myoma-like mass, 7.4 cm in maximum diameter, on the left uterine side wall. Hysterectomy was performed as a definitive treatment. Microscopic examination revealed spindle cell proliferation with numerous lymphocytes and plasma cells. Immunohistochemically, the spindle cells were negative for ALK-1, desmin, and smooth muscle actin. The pathological diagnosis was IMT arising from the uterus. Fluorescence in situ hybridization demonstrated an ETV6–neurotrophic tyrosine kinase, receptor, type 3 gene ( NTRK3) translocation but no ALK, ROS1, or PDGFR-β translocations. Lung and abdomen computed tomography at 31 months postoperatively revealed no disease recurrence. This association of an ETV6–NTRK3 fusion oncogene with an ALK-negative uterine IMT increases our understanding of this neoplasm, which may help the development of specific therapies.

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