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.

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 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 Large intraperitoneal lipoleiomyoma in a pre-menopausal woman: a case report

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Sara L. Schaefer ◽  
Amy L. Strong ◽  
Sheena Bahroloomi ◽  
Jichang Han ◽  
Michella K. Whisman ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Smooth Muscle ◽  
Case Report ◽  
Smooth Muscle Actin ◽  
Abdominal Mass ◽  
Histopathological Analysis ◽  
Menopausal Woman ◽  
En Bloc ◽  
Cross Sectional

Abstract Background Lipoleiomyoma is a rare, benign variant of the commonplace uterine leiomyoma. Unlike leiomyoma, these tumors are composed of smooth muscle cells admixed with mature adipose tissue. While rare, they are most frequently identified in the uterus, but even more infrequently have been described in extrauterine locations. Case presentation We describe a case report of a 45-year-old woman with a history of in vitro fertilization pregnancy presenting 6 years later with abdominal distention and weight loss found to have a 30-cm intra-abdominal lipoleiomyoma. While cross-sectional imaging can narrow the differential diagnosis, histopathological analysis with stains positive for smooth muscle actin, desmin, and estrogen receptor, but negative for HMB-45 confirms the diagnosis of lipoleiomyoma. The large encapsulated tumor was resected en bloc. The patients post-operative course was uneventful and her symptoms resolved. Conclusions Lipoleiomyoma should be considered on the differential diagnosis in a woman with a large intra-abdominal mass. While considered benign, resection should be considered if the mass is symptomatic, and the diagnosis is unclear or there is a concern for malignancy.

scholarly journals Intra-abdominal Solitary Myofibroma in a Child: A Rare Case Report

2021 ◽  
Author(s):  
Smita Singh ◽  
Jyoti Garg ◽  
Kusha Sharma ◽  
Kiran Agarwal
Keyword(s):  
Histopathological Examination ◽  
Smooth Muscle Actin ◽  
Iliac Fossa ◽  
Correct Diagnosis ◽  
Neck Region ◽  
Abdominal Distension ◽  
Malignant Tumours ◽  
Rare Presentation ◽  
Anaplastic Lymphoma ◽  
Abdominal Masses

Intra-abdominal masses in children are usually malignant. Benign tumours at this location are not seen frequently. It is even rarer to find solitary myofibromas intra-abdominally as these tumours are known to have predilection for the head and neck region. We present an unusual case of solitary myofibroma with abdominal localisation in a six-year-old male child who presented with abdominal distension since six months. Computed Tomography (CT) abdomen revealed a complex solid-cystic mass extending from umbilicus to right iliac fossa and measuring 9×6×4 cm. Histopathological examination revealed a tumour displaying biphasic nodular pattern with the presence of lighter staining fascicles of mature myoid cells along with darker staining and more cellular areas of smaller primitive cells. On Immunohistochemistry (IHC), tumour cells were positive for vimentin and Smooth Muscle Actin (SMA) with variable reactivity for desmin while these were negative for CD34 and Anaplastic Lymphoma Kinase 1 (ALK). Based on the histopathological and immunohistochemical findings, final diagnosis of myofibroma was made. Recognition of these lesions is extremely challenging owing to their rare presentation intra-abdominally and also because of their close morphological overlap with other spindle cell tumours commonly found at this site. This case highlights the combined role played by histopathology and IHC in making a clear distinction between different entities. It is imperative for both clinicians and histopathologists to establish the correct diagnosis as excision of the solitary myofibroma is curative in most cases and offers better clinical course than the more commonly found malignant tumours at this site.

scholarly journals Inflammatory myofibroblastic tumor of the pancreas in a dog

2019 ◽  
Vol 31 (6) ◽  
pp. 879-882
Author(s):  
Mariarita Romanucci ◽  
Sabrina V. P. Defourny ◽  
Marcella Massimini ◽  
Laura Bongiovanni ◽  
Giovanni Aste ◽  
...  
Keyword(s):  
Inflammatory Myofibroblastic Tumor ◽  
Distress Syndrome ◽  
Plasma Cells ◽  
Smooth Muscle Actin ◽  
S100 Protein ◽  
Variable Number ◽  
Postmortem Examination ◽  
Muscle Actin ◽  
Coagulative Necrosis ◽  
Spindle Cells

A large, ill-defined, firm, multinodular mass involving the pancreas was confirmed on postmortem examination of a 5-y-old, male Rottweiler that died following acute respiratory distress syndrome, after a period of anorexia and lethargy. Histologically, the mass consisted of plump spindle cells admixed with a variable number of macrophages, lymphocytes, plasma cells, and neutrophils. Foci of coagulative necrosis and hemorrhage were also observed. Spindle cells strongly reacted to antibodies against vimentin, α–smooth muscle actin, and calponin, whereas desmin was expressed only mildly and focally. Pan-cytokeratin, KIT, glial fibrillary acidic protein, and S100 protein were nonreactive. Variable numbers of MAC 387–positive cells, CD3+ lymphocytes, and numerous blood vessels were also detected throughout the mass. Histologic and IHC findings were consistent with a diagnosis of inflammatory myofibroblastic tumor of the pancreas.

scholarly journals Splenic malignant mesenchymoma in a dog – immunophenotypic features and clinicopathological ramifications: a case report

2014 ◽  
Vol 59 (No. 1) ◽  
pp. 55-61 ◽  
Author(s):  
Z. Dokic ◽  
W. Pirog ◽  
J. Benak ◽  
D. Lorinson
Keyword(s):  
Smooth Muscle ◽  
Retrospective Studies ◽  
Smooth Muscle Actin ◽  
Abdominal Distension ◽  
Alpha Smooth Muscle Actin ◽  
Abdominal Ultrasonography ◽  
Tumor Entity ◽  
The Common ◽  
Well Differentiated

A 13-year-old spayed bitch was referred for evaluation of an abdominal distension with a palpable, continuously growing mass. Abdominal ultrasonography revealed a 30 × 20 cm mass directly connected to the spleen. Surgical exploration confirmed the sonographic diagnosis with adhesions to the omentum and the liver. Pathohistological samples revealed well differentiated adipose tissue and variably differentiated collagenous and myxomatous tissue. Immunohistochemically, vimentin and in some regions alpha smooth muscle actin were expressed indicating smooth muscle differentiation. The results support the diagnosis of a malignant mesenchymoma composed of liposarcoma, mixosarcoma and leiomyosarcoma. No local recurrence or metastasis occurred during a nine month follow-up. So far, only two pathological retrospective studies describing the common prevalence and properties of canine splenic malignant mesenchymomas were found in the literature. However, this rare tumor entity has to be considered as a differential diagnosis in cases of large splenic masses.  

scholarly journals Solitary Fibrous tumor arising from the Omentum

2016 ◽  
Vol 9 (4) ◽  
Author(s):  
Salem A ◽  
Madden M ◽  
Bateson P
Keyword(s):  
Ct Scan ◽  
Solitary Fibrous Tumor ◽  
Abdominal Mass ◽  
Abdominal Distension ◽  
Abdominal Ultrasound ◽  
Greater Omentum ◽  
Midline Laparotomy ◽  
Tender Mass ◽  
History Of ◽  
Fibrous Tumor

A 60-year old man presented with a one week history of intermittent periumbilical pain. He had weight loss of half a stone over 6 weeks and abdominal distension for 2 weeks, there was no other systemic complaint. General examination revealed bilateral Dupuytren`s contracture and grade 4 finger clubbing. Abdominal palpation revealed a very large non tender mass. The Liver and spleen were difficult to assess because of the size of the mass. Rectal examination was normal. An abdominal Ultrasound showed a central abdominal mass of mixed echogenicity. CT scan showed a large lobulated mass with overlying serpinginous vessels with a clear plane posteriorly separating it from the retro-peritoneum. Liver, spleen and pancreas showed no abnormality. CT scan of the chest showed no abnormality. Core biopsy under ultrasound guidance revealed features consistent with a solitary fibrous tumor, haemangiopericytoma or angiosarcoma. The patient underwent a midline laparotomy. The huge mass was attached to the greater omentum by a pedicle with minimal adhesions to the lateral peritoneum. It was excised compeletly. The post-operative course was uneventful. Gross pathological findings macroscopically revealed the mass measuring 24x19x10 cm, weighing 3870 grams and on section it was a fleshy lobulated tumour with a few cystic areas. There was some attenuated fat on part of the surface. Histologically, the architecture was pattern-less with prominent stromal hyalinization, varying cellularity (mainly spindle and ovoid cells) and branching (haemangiopericytoma-like) vessels.

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.

Glomus Tumor of the Colon: A Rare Case Report and Review of Literature

2020 ◽  
Vol 28 (6) ◽  
pp. 691-695 ◽  
Author(s):  
Irene Y. Chen ◽  
Bushra G. Fazili ◽  
Xiaoyan Liao
Keyword(s):  
Smooth Muscle ◽  
Tumor Cells ◽  
Glomus Tumor ◽  
Smooth Muscle Actin ◽  
English Literature ◽  
Screening Colonoscopy ◽  
Significant Past Medical History ◽  
Sessile Polyp ◽  
Mesenchymal Neoplasm ◽  
Eosinophilic Cytoplasm

Glomus tumor is a rare mesenchymal neoplasm originating from the modified smooth muscle cells of the glomus body. Primary colonic glomus tumor is extremely rare with only 5 cases published in the English literature. In this article, we report the sixth case of primary colonic glomus tumor in a 50-year-old female with no significant past medical history who presented with routine screening colonoscopy. The entire colon was endoscopically unremarkable except an incidental 6-mm sessile polyp located in the descending colon. Biopsy showed a densely cellular neoplasm composed of small, bland, slightly spindled to predominantly epithelioid cells with clear to eosinophilic cytoplasm arranged in nests and sheets. The tumor cells were interspersed with slit-like thin-walled vessels and scattered short nerve bundles. Immunohistochemically, the tumor cells were positive for smooth muscle actin, h-caldesmon, and CD34 (focal), but completely negative for HMB45, S100, EMA, desmin, DOG-1, and CD117. The histologic features and immunohistochemical profile supported a diagnosis of primary colonic glomus tumor. The patient was asymptomatic and disease free after the procedure.

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