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RESUMEN Existe una variedad de tumores epiteliales, mesoteliales y de tejidos blandos que pueden desarrollarse en la red testicular y paratesticular. Se presenta el caso clínico de paciente con tumoración paratesticular derecha: Miofibroblastoma Mamario Paratesticular. Importante recalcar la variedad de origen embrionario para el desarrollo de estos tumores, así como el diagnóstico oportuno para el tratamiento y seguimiento resolutivo.
ABSTRACT There is a variety of epithelial, mesothelial, and soft tissue tumors that can develop in the testicular and paratesticular area. We present the case of a patient with a right paratesticular tumor that was diagnosed as paratesticular mammary myofibroblastoma. It is important to emphasize the embryonic origin variety for the development of these tumors, as well as having a timely diagnosis for treatment and decisive follow-up.
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Objective:To investigate the efficacy of anaplastic lymphoma kinase-tyrosine kinase inhibitors (ALK-TKI) in treatment of inflammatory myofibroblastic tumor (IMT).Methods:The clinicopathological data of one recurrent abdominal IMT patient in Renmin Hospital of Wuhan University in 2018 were retrospectively analyzed. The clinicopathological and molecular characteristics, ALK-TKI treatment efficacy and prognosis of 41 patients with IMT reported in the literature from January 2010 to August 2020 were systematically reviewed.Results:This patient with abdominal IMT in Renmin Hospital of Wuhan University was a 27-year-old female who relapsed 2 months after surgery. Chemotherapy combined with bevacizumab was ineffective. After oral administration of crizotinib, the condition resolved after 1 month, and complete remission (CR) was achieved after 29 months. The median age of onset of 41 IMT cases reported in the literature was 22 years old (0-61 years old), of which 32 cases (78.0%) had multiple organ involvement, all of which had recurrence or metastasis. There were 38 cases of ALK mutation and 3 cases of TFG-ROS1 fusion gene-positive. Thirty-four patients treated with crizotinib in the first-line treatment of ALK-TKI, and the median resistance time of crizotinib was 8 months (2-48 months). The total clinical benefit rate of ALK-TKI was 85.3% (29/34), and 20 patients achieved CR. The median time for the first CR was 11 months (4-36 months), and the median duration time of medication for CR patients was 19.5 months (2-60 months). The median progression-free survival (PFS) time of 24 patients who underwent surgery and/or chemotherapy and radiotherapy was 4 months (1-45 months); after progression, ALK-TKI treatment was performed, and the median PFS time was 14 months (3-62 months).Conclusions:IMT is a true neoplasm with characteristics of recurrence and metastasis. Reasonable combination of ALK-TKI with surgery, radiotherapy and chemotherapy can improve the prognosis of IMT patients.
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Objective@#To study the clinicopathologic characteristics, immunophenotype and ALK gene alterations of gastrointestinal inflammatory myofibroblastic tumor.@*Methods@#Clinical data, histological features and immunohistochemical results were analyzed in 7 cases of gastrointestinal inflammatory myofibroblastic tumor at Zhejiang Province Taizhou Hospital from January 2005 to December 2016. ALK gene status was investigated by ALK fluorescence in situ hybridization.@*Results@#There were 4 female and 3 male patients. The age of patients ranged from 1 to 72 years (median age=53 years and mean age=40 years). The tumor was located in stomach (n=4), left hemicolon (n=1), right hemicolon (n=1) and rectum (n=1). Histologically, the tumors consisted of spindle fibroblast and myofibroblast cells growing in bundles with inflammatory infiltration primarily composed of plasma cells and lymphocytes. Immunohistochemical study showed spindle tumor cells were positive for vimentin (7/7), SMA (7/7), but were negative for CD34, CKpan, CD117, DOG1, S-100 and desmin. Two cases expressed ALK protein and fluorescence in-situ hybridization revealed the presence of ALK gene rearrangement in the both cases.@*Conclusions@#Gastrointestinal inflammatory myofibroblastic tumor is a rare neoplasm that is easily misdiagnosed. Its surgical removal is a reliable treatment. ALK may be a potential novel therapeutic target for inflammatory myofibroblastic tumor.
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Objective To investigate the ultrasound features of hepatic inflammatory myofibroblastic tumor (HIMT).Methods A retrospective analysis was performed for the ultrasound features of 4 patients who visit Shengjing Hospital from February 2007 to July 2017 with pathologically confirmed HIMT,including the location,size,boundary,morphology,internal echo,and blood flow of lesions.Results The patients were aged 2-69 years and there were 2 male and 2 female patients.All 4 patients had single tumor with a maximum diameter of 3.4 -21.2 cm.Two patients had clear boundaries and two had unclear boundaries.Two patients had solid tumor with inhomogeneous low echo and 2 had mixed cystic and solid type;color Doppler flow imaging found blood flow signal in tumor in all patients.Conclusion Ultrasound can clearly reveal the size,internal echo,and blood flow of HIMT,but the ultrasound images of HIMT lack specificity.Pathological examination is still needed for a confirmed diagnosis.
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Objective To analyze the CT and MRI features of splenic inflammatory myofibroblastoma (SIMF).Methods The CT and MRI images of 6 patients with SIMF were retrospectively analyzed.Results 5 cases underwent CT examination;2 cases did MRI.Six cases of SIMF were all single lesions;the average diameter was (3.9 ± 0.9) cm;the lesions were round or gourdshaped;most have clear boundaries with uneven scan density.On the MRI,the parenchymal part was slightly low signal on T1WI,inhomogeneous hypointensity on T2WI,or high signal on T2WI.After CT or MRI enhancement,lesions were progressively enhanced.Correct preoperative diagnosis was established in only 1 patient.Conclusion In this study,all SIMF cases present as single lesion.The circular appearance and clear boundaries are in common.Asymptotic intensification with map-unenhanced regions is the main enhancement feature.
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Objective@#To explore the clinical characteristics, diagnosis and treatment of pulmonary inflammatory myofibroblastic tumor in children.@*Methods@#The clinical data of 13 patients with pulmonary inflammatory myofibroblastic tumor between October 2009 and October 2015 were retrospectively analyzed, including 9 boys and 4 girls aged 1 to 12 years 4 months (mean 4 years 9 months). Seven patients underwent total tumor resection, 4 had lobectomy, 1 had partial tumor resection and 1 had biopsy. The follow-up time ranged from 2 months to 6 years and 2 months.@*Results@#Among the 13 patients, 9 were cured. Two patients lived with tumor. One of them had tumor which shrinked gradually and the other had stable disease. One patient with multiple lesions encountered recurrence after 3 operations and developed suspicious hepatic metastasis. One died during the operation because of airway mismanagement.@*Conclusions@#Pulmonary inflammatory myofibroblastic tumor in children has rather good prognosis while a few cases have the potential of recurrence and canceration. Surgery is the primary therapeutic approach. Close post-operative follow up is necessary.
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OBJECTIVE: To report various anatomic locations and clinical characteristics of pathologically proven myofibroblastoma in Koran patients. METHODS: Pathologic reports of patients who underwent surgeries at two centers between April 2003 and March 2016 were retrieved from the electronic medical record system of the hospital. Pathologic reports were included after performing a search using the keyword “myofibroblastoma”. RESULTS: The cohort consisted of 11 subjects and included eight female and three male individuals. The patients' ages ranged from 9 to 66 years. Tumors were located in the vagina in three patients and presented in the breast in seven patients. One case presented with an abdominal mass. The tumors ranged in mean size from 4.0 to 53.0 mm. Despite a relatively long-term follow-up, no case had evidence of tumor recurrence. CONCLUSION: We evaluated the various anatomic locations of pathologically proven myofibroblastoma in Korean patients. As an extremely rare tumor, physicians should pay special attention to differential diagnosis. Surgical resection is the preferred method for a cure, and the recurrence rate is extremely low.
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Female , Humans , Male , Anatomic Variation , Breast , Cohort Studies , Diagnosis, Differential , Electronic Health Records , Follow-Up Studies , Methods , Neoplasms, Muscle Tissue , Recurrence , VaginaABSTRACT
Objective To retrospectively analyze the clinical data of 3 middle-aged and elderly cases with inflammatory myofibroblastic tumor (IMT) of the kidney,in order to improve its diagnosis and treatment.Methods The records of 3 patients who were diagnosed as IMT of the kidney by pathology in our hospital from Jan 2005 to Jun 2014 were reviewed to identify the characteristics including age,gender,clinical manifestation,tumor imaging and pathological features,treatment and prognosis.Results The mean age of the patients was 60 years.One patient underwent partial nephrectorny,1 patient underwent radical nephrectomy,and another patient received nephroureterectomy with bladder cuff excision.Patients were followed up for 8 months to 10 years,and no tumor recurrence was found.Conclusions IMT of the kidney is the tumor of low malignant potential with a good prognosis.Surgery is the first choice for the diagnosis and treatment.
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Objective To analyze the clinical features of rectal and perianal inflammatory myofibroblastic tumor and evaluate its diagnosis and treatment.Method Clinicopathological data of 3 cases diagnosed as inflammatory myofibroblastic tumor from January,2005 to June,2011 were retrospectively reviewed.Results Inflammatory myofibroblastic tumor presents as infiltrative growth mass with rich vascularization on CT or MRI,and is difficult to distinguish from hemangioma and other rectal tumors.Preoperative biopsy usually fails to ascertain the entity of mass,and pathological examination of the whole resected specimen with immunohistochemical staining is needed to make final diagnosis.All 3 cases underwent sphincter preserving surgery.One case received a second radical operation 16 months after primary resection because of local recurrence.All patients are followed up to now,with a survival time of 67 months,55 months,and 35 months respectively.Conclusions Rectal and perianal inflammatory myofibroblastic tumor is difficult to diagnose on preoperative imaging examinations or biopsy.Immunohistochemical staining is needed to make final diagnosis.Sphincter preserving surgery with complete tumor removal could achieve long term survival.
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Objective To describe MR findings in inflammatory myofibroblastic tumor(IMT)of the soft tissue.Methods MR manifestations of 11 cases of IMT of the soft tissue were retrospectively analyzed,and the MR findings were correlated with surgical and histological results.Results The pathological classification of the tumors was type Ⅰ in 1 case,type Ⅱ in 4 cases.mainly type Ⅱ admixed with type Ⅰ in 3 cases,and mainly type Ⅱ admixed with type Ⅲ in 3 cases.In 4 cases with primary tumor,the tumors were spheroid in shape,with well-defined margin and pseudocapsule.In 2 cases with primary axillary tumor and 5 cases with recurrent tumor,the tumors were irregular in shape,with ill-defined margin and invasion of adjacent structures.The tumors were mainly isointensive in T1-weighted images.Tumors of different pathological classifications had different signal intensities in T2-weighted images:1 case of type Ⅰ tumor was bright:4 cases of type Ⅱ tumor and 3 cases of type Ⅱ tumor admixed with type Ⅰ tumor were slig}ltly bright;3 cases of type Ⅱ tumor admixed with type Ⅲ were isointense or slightly hypointense in signal.All of the 11 eases in the study exhibited"pitaya cross-section sign"in T2-weighted sequence,which referred to discrete punctuate foci of relatively hypointensity in the background of hyperintensity,slightly hypointensity or isointensity.All of the 11 cases exhibited inhomogeneously significant enhancement after gadolinium administration.In the follow-up of the 6 cases of primary tumor,4 cases had recurrence,1 case had no recurrence,and 1 ease was lost in the follow-up process.In the follow-up of the 5 cases of recurrent tumor,4 cases showed recurrence again,and 3 cases were lost in the follow-up process.Conclusions The IMT of the soft tissue has characteristic MR features.The signal intensity of the tumor on T2-weighted sequence could reflect the pathological type of the tumor to some extent."pitaya cross-section sign"could be a pathogenomic sign of the IMT of the soft tissue.
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Objective To describe the CT and MR imaging features of hepatic inflammatory myofibroblastic tumor(IMT).Methods The CT(n=12)and MRI(n=2)findings of pathologically proved hepatic IMT in 12 patients were retrospectively analyzed.Results All the tumors of the 12 patients were solitary and located in right hepatic lobe.Six tumors were solid and 4 tumors were solid-cystic.The other 2 tumors were periportal soft-tissue infiltration.The tumors appeared as solid or solid-cystic hypodense mass on CT images.The lesions were dark in T1-weighted sequences and slightly bright on T2-weighted sequences.There was homogeneous or inhomogeneous moderate to significant enhancement in solid portion of the tumors.Honeycomb-like enhancement was demontrated both in the peripheral part and at the intratumoral septa of the solid-cystic mass.Conclusion CT and MRI can provide helpful information for the clinical diagnosis and differential diagnosis of hepatic IMT.
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Scrotal leiomyomas with atypical bizarre nuclei are rare, which might be misdiagnosed as malignant tumor. We describe a case of scrotal bizarre leiomyoma in a 65-yr-old man. The tumor was a 1 cm-sized, well circumscribed, oval mass arising from the tunica dartos muscle. Histologically, it was formed by whorling bundles of fusiform cells with occasional atypical, pleomorphic nuclei and pseudoinclusions. Mitosis was not found. Although morphologically atypical, scrotal bizarre leiomyomas take on a biologic behavior not different from that of conventional leiomyoma, they should be distinguished from leiomyosarcoma to avoid unnecessary treatment.
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Aged , Humans , Male , Genital Neoplasms, Male/pathology , Leiomyoma/pathology , Scrotum/pathologyABSTRACT
Objective To analyze the imaging manifestations of primary non-Hodgkin lymphoma of skeletal muscle and improve the recognition of this rare disease.Methods Five cases of primary non-Hodgkin lymphoma of skeletal muscle proved pathologically underwent imaging exam,including MRI and CT in 3 cases,only MRI in 1 case,only CT in 1 case,X-ray in 2 cases and bone scintigraphy in 2 cases.Results Diffuse enlargements of involved muscle with presentation of overall configuration were observed in all five cases.All 4 cases manifested as homogeneous soft masses,which is isoattenuating to normal muscle on unenhanced CT images.After intravenous injection of contrast media,the masses enhanced homogeneously and slightly(2 cases)or moderately(1 case)on CT images.The lesions were homogenous and had isointense or slightly low signal intensity compared with that of uninvolved muscle on T1-weighted images and high signal intensity on T2-weighted images.After intravenous injection of contrast media,all 2 cases enhanced homogeneously and moderately with the enhanced signal intensity of involved muscle greatly higher than that of uninvolved muscle on MR images.Two cases of X-ray plain showed no destruction of bone and 2 cases of bone scintigraphy exams showed increased radiotracer uptake of involved muscle with no infiltration of bone marrow.Conclusion There are several characteristics on the imaging of primary non-Hodgkin lymphoma of skeletal muscle.MRI is the optimal imaging method for the diagnosis of this disease.
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Objective To evaluate the clinical application of contrast-enhanced digital subtraction MRI in musculoskeletal malignant tumors.Methods Fifty patients with musculoskeletal malignant tumors were scanned by contrast enhanced T 1WI with an injection of Gd-DTPA (0.1 mmol/kg), and digital subtraction was performed between post-contrast-enhanced images and pre-contrast-enhanced images. Musculoskeletal malignant tumors were evaluated by means of a contrast-to-noise ratio (C/Ns). Imaging sign of the musculoskeletal malignant tumors were compared between subtraction MR images and conventional contrast enhanced MR images.Results Contrast-enhanced digital subtraction MR images were better than conventional contrast enhanced MR images. All cases were confirmed by operation or pathology. All the lesions had higher C/Ns on subtraction MR imaging than on conventional contrast enhanced MR images. C/Ns was 109.74?5.10 (?s) for subtraction MR imaging and 23.61?3.16 for conventional contrast enhanced MR images (t=101.51, P