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Sclerosing angiomatoid nodular transformation (SANT) of the spleen is a rare benign vascular lesion of the spleen that was first described in 2004. SANT is associated with other concurrent diseases, mostly malignancies. Calcifying fibrous pseudotumor (CFPT) is a tumor-like lesion usually arising from soft tissue or peritoneal sub-serosa. Both SANT and CFPT are considered to be variants of the inflammatory myofibroblastic tumor. We report the rare case of a 24-year-old female presenting with a left abdominal mass that was clinically diagnosed as an extraintestinal gastrointestinal stromal tumor (GIST). Histopathological examination revealed SANT of spleen and CFPT. We report this case due to its rarity of occurrence and unusual association of SANT with CFPT.
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El tumor miofibroblástico inflamatorio primario gástrico es una neoplasia rara desarrollada de células madre mesenquimales, e infrecuentemente discutido en la literatura científica. El diagnóstico clínico a través de endoscopia y patología es desafiante para el equipo. Nosotros reportamos el caso de una paciente mujer con síndrome de obstrucción gástrica por un tumor de 10 cm diagnosticado con esta enfermedad usando histología e inmunohistoquímica.
Primary gastric inflammatory myofibroblastic tumor is a rare neoplasm developed from mesenchymal stem cells, infrequently discussed in the scientific literature. Clinical diagnosis through endoscopy and pathology is challenging for the medical team. We report the case of a female patient with gastric obstruction syndrome due to a 10 cm tumor diagnosed with this disease by histology and immunohistochemistry.
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Inflammatory myofibroblastic tumors (IMTs) are uncommon; intermediate grade soft tissue tumors occurring in young individuals with an uncertain behaviour. The incidence of pulmonary lymphangitis carcinomatosis (PLC) is around 6-8% of all pulmonary metastases. However, PLC due to papillary thyroid carcinoma (PTC) is very uncommon. We present a case of a 26-year-old male, who presented with a solitary left lung nodule on radiological scans. There was also a past history of thyroid surgery done two years back for PTC. Histology revealed a soft tissue tumor reminiscent of IMT. The periphery of the IMT nodule showed metastatic PTC in the form of extensive PLC. In view of this unusual histology, a diagnosis of PTC with nodular fasciitis-like stroma (PTC-NFS) was initially considered. However, molecular studies for anaplastic lymphoma kinase (ALK) gene rearrangement confirmed the diagnosis of IMT. This case highlights the unusual occurrence of tumor-to-tumor metastasis causing diagnostic challenges and also the importance of molecular testing.
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Mediastinal masses span a wide histopathological spectrum. Inflammatory pseudotumors are rare and most commonly described in the lungs but these are reported in almost all the organs in the body. Mediastinal involvement is rare and difficult to diagnose. Clinical manifestations and laboratory investigations and radiology are non-specific. Histomorphology and Immunohistochemistry provide a valuable aid. Complete resection usually provides definitive diagnosis and is treatment of choice.
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Inflammatory myofibroblastic tumor (IMT) of gastrointestinal tract is a rare and low malignant mesenchymal tumor, which is composed of differentiated myofibroblastoid spindle cells and often accompanied by numerous inflammatory cells such as plasma cells, lymphocytes, and eosinophils. Surgical resection is the preferred treatment for IMT of gastrointestinal tract, and patients can achieve a good prognosis after surgery. In recent years, with the progress of imaging examination and pathological diagnosis technology, the detection rate of IMT of gastrointes-tinal tract has been greatly improved, but its pathogenesis and mechanism have not been completely investigated, requiring further research results to confirm. At the same time, due to the lack of standardized strategies for diagnosis and treatment of IMT of gastrointestinal tract, it was inevitable to miss diagnosis or treatment in clinical diagnosis and treatment. By reviewing relevant literatures and combined with the team's practical experience, the authors summarize the research progress of the diagnosis and treatment of IMT of gastrointestinal tract from the aspects of pathogenesis, clinical diagnosis and treatment strategies of IMT, aiming to provide references for the clinical treatment of surgeons.
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Inflammatory myofibroblastic tumor (IMT)is a potentially or low-grade malignant mesenchymal neoplasm, which is rare in clinic. Renomedullary interstitial cell tumor(RICT) is a clinically rare benign renal tumor. The combination of these two diseases in one patient has not been reported. A 25-year-old female patient was admitted to the hospital due to left back pain for 12 days and hematuria for 1 week. MRI of kidneys showed a mass in the left renal pelvis, which was considered as renal pelvic carcinoma. Urine cytopathological examination was negative. Robot-assisted laparoscopic radical left nephroureterectomy was performed. There was no tumor recurrence or metastasis during the follow-up for more than 6 months after operation.
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Objective:To investigate the efficacy and safety of targeted therapy with Crizotinib for children with ALK gene mutation positive inflammatory myofibroblastic tumor (IMT). Methods:A retrospective analysis was performed on 4 children with ALK gene mutation positive IMT admitted to Shanghai Children′s Hospital from January 2019 to June 2021.Among them, 3 cases were given the targeted drug Crizotinib[280 mg/(m 2· time), q12h] orally, and 1 case was observed after complete tumor resection to analyze the efficacy and adverse drug reactions. Results:All 4 cases were male, aged from 2 years and 3 months to 11 years and 3 months.The tumors originated from the abdominal cavity in 2 cases, the right orbit in 1 case, and the right lung in 1 case.Pathological immunohistochemistry and fluorescence in situ hybridization were both positive for ALK gene mutation, and complete remission was achieved after comprehensive treatment.Among them, 3 patients were treated with oral Crizotinib, and 2 patients were tried to stop taking the drug for 1 year, relapsed 1 month later, and still achieved complete remission after the second treatment.The 4 cases were followed up for 8-30 months, and all survived.All the cases showed no abnormalities in blood image, liver and kidney function, myocardial enzyme profile, cardiac function, hearing and vision, and 2 cases showed prolonged Q-T interval in the course of Crizotinib treatment, which could be recovered by temporary withdrawal of drug, and no abnormality in electrocardiogram was found in continued drug use. Conclusions:Crizotinib was used to treat ALK mutation positive IMT, shrink tumor and consolidate postoperative treatment, which is a good choice for IMT in children with difficult surgical resection and refractory recurrence.
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@#Anterior mediastinal inflammatory myofibroblastoma is a rare tumor with insidious onset and easy misdiagnosis. In this report, we presented a case of anterior mediastinal inflammatory myofibroblastoma with thymoma. The mediastinal tumor was found by physical examination, and the prognosis was good after surgical treatment. For this disease, operation is an effective method for definite diagnosis and treatment, and complete excision can achieve good outcomes.
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The incidence of inflammatory myofibroblastic sarcoma is low, and bladder origin is more rare. We reported a 58-year-old patient with painless gross hematuria for one week. Total abdominal CT examination showed soft tissue mass in the anterior wall of the bladder, which was considered as bladder cancer, and bladder tumor resection was performed. Postoperative pathology showed inflammatory myofibroblastic sarcoma. Therefore, radical cystectomy was performed because of the high degree of malignancy. There was no recurrence during 3 years 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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Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a rare entity and a novel variant of inflammatory myofibroblastic tumor (IMT), usually seen in children and nonsmoking young adults. Their occurrence in a posttransplant setting is still rare. These tumors are characterized by prominent epithelioid morphology, large histiocytoid “Reed Sternberg”-like cell, unique pattern of ALK immuno-reactivity, and aggressive clinical behavior. Their etiology and metastatic potential is controversial. In a post-transplant setting, many factors such as trauma, infections with EBV, HIV, Hepatitis C, mycobacteria, fungus, and chemotherapy-induced immunosuppression have been implicated in their etiology. We present the case of a 2-year-old female child who developed multiple omental and mesenteric tumor nodules, 8 months post liver transplant for progressive familial intrahepatic cholestasis (PFIC). Following a histopathological diagnosis of “mesenchymal neoplasm of possible malignant nature” on a trucut biopsy and frozen section, tumor debulking was performed. A final histological diagnosis of EMIS was made on the completely resected tumor. The patient remains in remission nearly 7 months after presentation, without any follow-up systemic chemotherapy. IMT after a solid organ transplant is rare, only 5 cases have been reported in the literature until now. Similar phenomenon has also been noted with hematopoietic stem cell transplant. However, to our knowledge, this case of EMIS in a post liver transplant patient is first of its kind.
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INTRODUCCIÓN: El tumor miofibroblástico inflamatorio (TMI) es una neoplasia benigna infrecuente, de comportamiento clínico impredecible. OBJETIVOS: describir 3 casos de TMI diagnosticados entre marzo 2014 y enero 2018 en Hospital Clinico San Borja Arriaran, y realizar una revisión actualizada de la literatura. CASO 1: Adolescente de género masculino de 14 años de edad, hospitalizado por dolor abdominal, diagnosticado de invaginación yeyunoyeyunal secundaria a un tumor de pared intestinal. La histología fue compatible con un tumor miofibroblástico inflamatorio. CASO 2: Adolescente de género femenino, edad 12 años, hospitalizada por neumonía y dolor lumbar en estudio asociado a pérdida de peso. Se diagnosticó una masa retroperitoneal que comprometía el músculo psoas derecho, músculos paravertebrales, vértebras, riñón derecho y diafragma ipsilateral. Se efectuó biopsia por punción cuyo resultado fue compatible con un tumor miofibroblástico inflamatorio. CASO 3: Preadolescente de género femenino de 11 años de edad, hospitalizada para estudio de infección del tracto urinario a repetición. Se identificó un tumor vesical y la biopsia mostró ser compatible con tumor miofibroblástico inflamatorio. CONCLUSIÓN: Debido al comportamiento variable del tumor miofibroblástico inflamatorio, el manejo de este dependerá de la localización, la expresión del anaplasic like lymphoma (ALK), el comportamiento del tumor y la posibilidad de resección.
INTRODUCTION: The inflammatory myofibroblastic tumor is an infrequent benign neoplasm with unpredictable cli nical behavior. OBJECTIVES: to describe three clinical cases at the San Borja Arriarán Clinical Hospital between March 2014 and January 2018 and to carry out an updated review of the literature. CASE 1: 14-year-old male adolescent, hospitalized due to abdominal pain, diagnosed with jejunojejunal intus susception secondary to an intestinal wall tumor. The histology was compatible with an inflamma tory myofibroblastic tumor. CASE 2: 12-year-old female adolescent, hospitalized due to pneumonia and low-back pain under study associated with weight loss. A retroperitoneal mass was diagnosed involving the right psoas muscle, paravertebral muscles, vertebrae, right kidney, and ipsilateral dia phragm. A puncture biopsy was performed and the result was compatible with an inflammatory myofibroblastic tumor. CASE 3: 11-year-old female pre-adolescent, hospitalized to study recurrent urinary tract infection. A bladder tumor was identified, and the biopsy showed compatibility with inflammatory myofibroblastic tumor. CONCLUSION: Due to the variable behavior of the inflammatory myofibroblastic tumor, its management will depend on the location, expression of the anaplastic lymphoma kinase (ALK), tumor behavior, and the resection possibility.
Subject(s)
Humans , Male , Female , Child , Adolescent , Retroperitoneal Neoplasms/diagnosis , Urinary Bladder Neoplasms/diagnosis , Intestinal Neoplasms/diagnosis , Retroperitoneal Neoplasms/pathology , Urinary Bladder Neoplasms/pathology , Myofibroblasts/pathology , Inflammation/diagnosis , Inflammation/pathology , Intestinal Neoplasms/pathologyABSTRACT
Inflammatory myofibroblastic tumor (IMT) is a rare tumor of unknown etiology and pathogenesis. The lesion has been recognized to occur at various sites but rarely affects the head and neck region. A 29-year-old male presented with a 13 months' history of a slow growing, painless growth in maxillary left posterior gingiva. An excisional biopsy was performed under local anesthesia. Microscopic examination revealed a compact cellular spindle cell proliferation with collagenous stroma having storiform architecture. Immunohistochemistry revealed that the tumor cells were positive for smooth muscle actin, CD-68 and negative for anaplastic lymphoma kinase. Oral IMT should be included in the differential diagnosis of localized gingival enlargement mimicking oral hyperplastic/reactive lesions
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@#Inflammatory myofibroblastic tumors (IMT) are rare, benign spindle cell neoplasms of the urinary bladder with a presentation similar to that of a malignant disease. Oftentimes, these tumors pose a diagnostic dilemma because of a significant overlap with malignant spindle cell tumors in terms of clinical presentation, gross findings and immunohistopathologic profile. Due to its rarity, there are no guidelines regarding treatment and follow up. Reporting cases of IMT will bring awareness to the features of these rare tumors, and will help in overcoming limitations in diagnosis, and treatment protocols. Presented here is a case of a 28-year-old female who presented with gross hematuria. Upon work up, revealed the presence of a bladder mass. Patient underwent transurethral resection, then a partial cystectomy, and pathology demonstrated an IMT.
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Objective To explore the CT finding of inflammatory myofibroblastic tumor (IMT)in the rare abdominal or pelvic, and compare their pathologies,to improve the accuracy of diagnosis and understanding of the disease.Methods The imaging,clinical and pathological data of 1 7 cases with IMT in the rare abdominal or pelvic were analyzed retrospectively.1 1 cases were performed with plain and enhanced scan,3 cases had plain scan only,and 3 cases had enhanced scan only.Results Among the 1 7 cases,8 cases were male,9 cases were female;1 5 cases were single,2 cases were multiple;4 cases were located in the stomach,colon or bladder,3 cases located in spleen,1 case located in kidney or urachus.Among those,2 lesions located in the colon cavity presented as soft tissue, it was difficult to distinguish the lesion from the adjacent intestinal contents since the small intestinal hypotonic CT examination was not performed and the intestinal filling was poor,thus those lesions were missed diagnosed with CT plain scan.The remaining 15 cases presented as soft tissue density as well,among those,9 cases presented with clear border while 6 cases with blurred border;the peripheral fat space of 4 cases were blurred and presented as infection and exudation,5 cases were accompanied with lymph node enlargement around the lesion;the density of 10 cases were uneven,among those,1 IMT lesion in bladder presented with linear calcification,1 IMT lesion in gastric antrum presented with nodular ossification in the center,9 cases showed obvious necrosis and cystic degeneration;5 cases showed even.After contrast administration,the lesions were enhanced mildly,moderately or significantly.The enhancement type were various,presented as uniform or nonuniform delayed enhancement.Vessel shadow was observed in 10 cases during arterial phase.All of the 17 lesions were primary, and no recurrence was found during follow-up.Conclusion The feature of IMT in the abdominal or pelivic including the following:the peripheral area of the lesion presenting as infection or exudation,the lesion showing uniform or nonuniform delayed enhancement, and vessel shadow observed in the lesion during arterial phase;in addition,calcification of the edge or central of the lesion occasionally happens,while ossification of the lesion is quite rare.
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Objective@#To investigate the clinicopathological features, diagnosis and differential diagnosis of dedifferentiated liposarcoma (DDLPS) with inflammatory myofibroblastic tumor (IMT)-like features.@*Methods@#Five cases of DDLPS with IMT-like features were collected from the First Affiliated Hospital of Nanjing Medical University, the Affiliated Hospital of Nanjing University of Traditional Chinese Medicine and the First People′s Hospital of Qinzhou between 2013 and 2018. EnVision method and fluorescence in situ hybridization (FISH) were used to detect the immunophenotype of the tumor cells and the profile of MDM2 gene amplification respectively.@*Results@#All five cases were male and the median age was 61 (range 53 to 65) years. The clinical symptoms were mainly related to the space-occupying lesions. The tumors were located in duodenal mesentery (two cases), intestinal wall (one case), retroperitoneum (one case), and spermatic cord (one case). Grossly, the tumors were not well encapsulated, ranging from 3 to 13 cm (median 6.7 cm) in diameter, with tan to gray and firm cut surface. Histologically, the dedifferentiated component closely resembled inflammatory myofibroblastic tumor (IMT), with spindle/polygonal/stellate-shaped cells arranged in storiform, sheet-like, or random pattern, with varying degrees of chronic inflammation and fibrosis. All three major patterns seen in IMT (myxoid, cellular and hypocellular fibrous) were observed, the hypocellular fibrous pattern was the most common. Well-differentiated liposarcomatous component was found in the peripheral areas of all the tumors. One case had high grade dedifferentiated component. Four cases were strongly positive for MDM2 and p16. Two cases were positive for SMA, and one case was focally positive for desmin and one for CD34. None of the cases stained for ALK-1. FISH demonstrated MDM2 gene amplification in all five cases. Clinical follow-ups were available in all five cases and the interval ranged from 3 to 66 months (median 23 months). Two patients developed recurrences and one patient had metastasis. The remaining two patients were alive with no evidence of tumor recurrence at 3 and 14 months after surgery respectively.@*Conclusions@#DDLPS with IMT-like features is a more aggressive neoplasm than its histological mimic (IMT), and should not be misdiagnosed as other intermediate or low-grade malignant tumors, such as IMT, sclerosing liposarcoma, inflammatory liposarcoma, aggressive fibromatosis, solitary fibrous tumors, low-grade myofibroblastic sarcoma, and low-grade fibrosarcoma.
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Objective To investigate clinical, pathological and radiographic features of inflammatory myofibroblastic tumor (IMT) of the nasal cavity. Methods Four cases of pathologically confirmed IMT in the Shengjing hospital of China Medical University from April 2015 to April 2017 were retrospectively analyzed. Three dimensional CT and enhanced MRI was performed in all patients. Results The main symptoms were local obstruction, oppression or infringement. In 4 cases, IMT was single, with 2 cases of primary maxillary sinus, 1 case of nasal cavity, and 1 case of nasal septum;Pathological morphology presented diversity, consisting mainly of spindle-shaped cells which distributed in bundles or whirlpools, and showed inflammatory cell infiltration. Immunohistochemical results showed SMA (+) in 4 cases, Desmin (-) in 4 cases , and ALK (-) in 3 cases. CT plain tumors showed mostly uniform soft tissue densities. MR showed more enhancement of tumors, 1 case with necrotic cystic changes in the maxillary sinus IMT tumors, showed a significant ring after enhancement. Conclusions The IMT of nasal cavity and paranasal sinuses is rare and the clinical symptoms are non-specific. The diagnosis mainly depends on pathological examination and immunohistochemical staining. Preoperative CT and MRI examinations can not only provide information on the lesion location, extent, and adjacent tissue invasion, but also on the blood supply of the tumor and the internal components of the tissue, which will facilitate the choice of treatment options.
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An inflammatory myofibroblastic tumor (IMT) is a solid tumor of unknown etiology frequently affecting children and young adults and commonly affecting the lung or orbital region. We present a case involving a 41-year-old man who had an IMT combined with Mycobacterium tuberculosis infection in the retroperitoneum. He presented with only pain in the right lower abdomen without accompanying symptoms; a retroperitoneal mass was found on computed tomography. The tumor had invaded the end of the ileum and was attached to the omentum, so mass excision could not be performed. The tumor was completely excised surgically and had histological features diagnostic of an IMT. Histologic findings of the omentum were positive for Ziehl-Nielsen staining for acid-fast bacilli and for a positive polymerase chain reaction for M. tuberculosis. The patient had no apparent immune disorder. These findings made this case exceptional because IMTs, which are mostly due to atypical mycobacteria, have been found mainly in immunocompromised patients.
Subject(s)
Adult , Child , Humans , Young Adult , Abdomen , Ileum , Immune System Diseases , Immunocompromised Host , Inflammation , Lung , Mycobacterium tuberculosis , Myofibroblasts , Nontuberculous Mycobacteria , Omentum , Orbit , Polymerase Chain Reaction , TuberculosisABSTRACT
Inflammatory myofibroblastic tumor (IMT) is a rare benign tumor, that is composed of myofibroblastic spindle cells with inflammatory cells. IMTs usually occur in lungs, intestine organs, orbits and paranasal sinuses, however, it may rarely be seen in the larynx. We present two cases of patients with laryngeal IMT that had different causes and prognosis.
Subject(s)
Humans , Intestines , Larynx , Lung , Myofibroblasts , Orbit , Paranasal Sinuses , Prognosis , RecurrenceABSTRACT
An inflammatory myofibroblastic tumor (IMT) is a rare disease that can occur in a variety of locations, including the lung, orbit, parotid, pleura, and stomach. Despite multiple reports in various organs, a duodenal IMT is rare with limited case reports. We encountered a case of a 49-year-old male with a duodenal IMT. The patient underwent a laparoscopic wedge resection under the impression of a duodenal mesenchymal tumor, such as gastrointestinal stromal tumor, but the final diagnosis was a duodenal IMT. The patient was treated successfully with an oral nonsteroidal anti-inflammatory drug for the residual lesions. He was free of recurrence during the 12 month follow-up period.