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

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.

2019 ◽  
Vol 31 (6) ◽  
pp. 879-882
Author(s):  
Mariarita Romanucci ◽  
Sabrina V. P. Defourny ◽  
Marcella Massimini ◽  
Laura Bongiovanni ◽  
Giovanni Aste ◽  
...  

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.


2021 ◽  
pp. 106689692110313
Author(s):  
Alexander M. Strait ◽  
Julia A. Bridge ◽  
Anthony J. Iafrate ◽  
Marilyn M. Li ◽  
Feng Xu ◽  
...  

Myofibroblastoma is a rare, benign stromal tumor with a diverse morphologic spectrum. Mammary-type myofibroblastoma (MTMF) is the extra-mammary counterpart of this neoplasm and its occurrence throughout the body has become increasingly recognized. Similar morphologic variations of MTMF have now been described which mirror those seen in the breast. We describe a case of intra-abdominal MTMF composed of short fascicles of eosinophilic spindle cells admixed with mature adipose tissue. The spindle cells stained diffusely positive for CD34, desmin, smooth muscle actin, and h-caldesmon by immunohistochemistry. Concurrent loss of RB1 (13q14) and 13q34 loci were confirmed by fluorescence in situ hybridization whereas anchored multiplex PCR and whole transcriptome sequencing did not reveal any pathognomonic fusions suggesting an alternative diagnosis. To the best of our knowledge this is the first documented case of leiomyomatous variant of MTMF.


2013 ◽  
Vol 2013 ◽  
pp. 1-4 ◽  
Author(s):  
Yuri Okumura ◽  
Kazuhiro Nomura ◽  
Takeshi Oshima ◽  
Atsuko Kasajima ◽  
Takahiro Suzuki ◽  
...  

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.


2016 ◽  
Vol 9 (2) ◽  
pp. 464-469 ◽  
Author(s):  
Toshiki Etani ◽  
Taku Naiki ◽  
Takashi Nagai ◽  
Keitaro Iida ◽  
Ryosuke Ando ◽  
...  

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.


2015 ◽  
Vol 2015 ◽  
pp. 1-5 ◽  
Author(s):  
Pasquale Cianci ◽  
Antonio Ambrosi ◽  
Alberto Fersini ◽  
Nicola Tartaglia ◽  
Vincenzo Lizzi ◽  
...  

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.


2001 ◽  
Vol 125 (11) ◽  
pp. 1494-1496
Author(s):  
Ravindra Veeramachaneni ◽  
Janis Gulick ◽  
Ari O. Halldorsson ◽  
Thanh T. Van ◽  
Ping L. Zhang ◽  
...  

Abstract This report describes a benign myoepithelioma of the lung that occurred in a 60-year-old woman. The patient had experienced hoarseness for 6 weeks, and a computed tomographic scan showed a nodule of approximately 2 cm in diameter at the peripheral portion of her right upper lung. Positron emission tomography showed no uptake of F-18 fluorodeoxyglucose in the nodule. Wedge biopsy of the lesion showed benign spindle cells arranged in a whorled pattern. The cells were positive for both cytokeratin and smooth muscle actin, which corresponded to the presence of tonofilaments and myofilaments that were identified ultrastructurally. The features of the present case of benign myoepithelioma that differ from features of previously reported benign and malignant cases of myoepithelioma in the lung are discussed in the report.


2018 ◽  
Vol 46 (10) ◽  
pp. 4343-4349 ◽  
Author(s):  
Yong-hao You ◽  
Yi Zhang

Objective This study was performed to discuss the characteristics, diagnosis, and treatment of primary prostatic extragastrointestinal stromal tumor (EGIST). Methods The case history data of a patient with an EGIST were analyzed and discussed with a literature review. Results The patient was diagnosed with a pelvic tumor, possibly malignant. We ascertained the diagnosis by exploratory surgery and pathological biopsy. The tumor was present in the prostate and infiltrated and pressed against the anterior rectal wall. Pathological biopsy showed that the tumor comprised spindle cells, which were also present at the junction of the tumor and prostate tissue. Immunohistochemically, the tumor cells were positive for CD117, DOG-1, CD34, and smooth muscle actin and negative for S100 and desmin; Ki-67LI was about 10%. These results support the diagnosis of primary prostatic EGIST. Conclusion The rarity and nonspecific clinical manifestation of prostatic EGIST facilitate misdiagnosis. Diagnosis mainly depends on imaging examination and characteristic histopathological and immunohistochemical features, and GIST must be excluded. Surgery is the main treatment method, and imatinib is suggested for unresectable and malignant EGISTs.


2005 ◽  
Vol 129 (10) ◽  
pp. 1322-1325
Author(s):  
Samson W. Fine ◽  
North J. Davis ◽  
Lawrence E. Lykins ◽  
Elizabeth Montgomery

Abstract Myofibromas are benign mesenchymal neoplasms of myofibroblastic origin. Most present as solitary lesions at any age, but the presentation of multiple lesions in newborns and infants is known as infantile myofibromatosis. Multicentric lesions commonly involve soft tissues and bone and may involve internal organs, where they are associated with an unfavorable prognosis. Solitary lesions involving the viscera are rare. We report a case of a 3-month-old male infant with a left testicular mass detected during an evaluation for suspected torsion. The patient underwent orchiectomy, revealing a nodular mass with grossly evident foci of necrosis. Histologically, the lesion exhibited small fascicles of plump eosinophilic, smooth muscle actin–positive spindle cells, alternating with larger areas of primitive cells with vesicular nuclei and scant cytoplasm arranged around a hemangiopericytoma-like vasculature. To our knowledge, this is the first report of a myofibroma localized within the testis.


2004 ◽  
Vol 128 (3) ◽  
pp. e46-e49 ◽  
Author(s):  
Vilkesh R. Jaiswal ◽  
Julie G. Champine ◽  
Suash Sharma ◽  
Kyle H. Molberg

Abstract Glomangiomas are a subset of glomus tumors that have a rich vascular network. Although a majority of the glomus tumors occur in the skin of the hand, they have also been reported in the deep soft tissue, bone, lungs, and gastrointestinal tract, especially the stomach. To our knowledge, only one such case has previously been reported primarily occurring in the liver. We report a case of a glomangioma primarily arising in the liver of a 57-year-old man who presented with right flank pain of several months' duration. A 3.0-cm hepatic mass was excised and consisted of numerous, small-to-medium branched vessels with the stroma containing small, round, regular cells with sharply outlined round-to-oval nuclei. Immunostains showed the tumor cells to be diffusely positive for vimentin and smooth muscle actin and to be focally positive for calponin. Collagen IV stained the pericellular matrix. The immunostain for CD34 highlighted the vascular network as well as outlined the tumor cells in many areas. Coexpression of actin and CD34 in glomus tumors, although unusual, has recently been reported in the literature. Despite its bland histology, the large tumor size and deep visceral location were suggestive of aggressive behavior; thus, a close clinical follow-up was recommended. The patient had an unremarkable postoperative course and has no evidence of metastatic disease 12 months after the procedure. An accurate diagnosis and an understanding the biology of this rare disease, especially in an unusual location, are crucial to its management.


1998 ◽  
Vol 275 (5) ◽  
pp. L998-L1005 ◽  
Author(s):  
Bruce D. Uhal ◽  
Carlos Ramos ◽  
Iravati Joshi ◽  
Antonio Bifero ◽  
Annie Pardo ◽  
...  

Primary human lung fibroblasts were separated into small ( group I), intermediate ( group II), and large ( group III) subpopulations by unit gravity sedimentation (1 G). The three subsets retained differences in cell size for up to 15 days of primary culture. Flow cytometric (fluorescence-activated cell sorter) measurements of forward-angle light scatter agreed well with fibroblast volume measured by image analysis and confirmed the utility of forward-angle light scatter for discriminating size subpopulations. Group II fibroblasts accumulated most rapidly by 8 days of culture and also contained the greatest proportion of S and G2/M phase cells as determined by fluorescence-activated cell sorter. Fibroblasts that were immunoreactive with antibodies to α-smooth muscle actin (α-SMA) were found only in group III. In situ end labeling of fragmented DNA detected apoptotic cells in both groups II and III, but double labeling for in situ end labeling and α-SMA revealed apoptotic cells in both the α-SMA-positive and -negative populations. These results demonstrate that primary human lung fibroblasts behave as predicted by classic models of cell cycle progression and differentiation. However, they do not support the hypothesis that the expression of α-actin is related to apoptosis. We also describe a simple and reproducible method for the high-yield isolation of human lung fibroblast subsets of differing proliferative potential and phenotype.


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