EBER-negative inflammatory pseudotumor-like follicular dendritic cell sarcoma of liver: A case report.

RATIONALE: Inflammatory pseudotumor-like follicular dendritic cell sarcoma (IPT-like FDCS) of the liver is rare. It was previously believed that Epstein-Barr virus (EBV) positivity was a necessary criterion for pathological diagnosis. However, we found that there were also cases of EBV negativity. Therefore, clinicians and pathologists are reminded that EBV positivity is not a necessary condition for diagnosis. PATIENT CONCERNS: A 70-year-old female underwent computed tomography (CT) examination for upper abdominal discomfort, which revealed the presence of a liver tumor. Follow-up revealed that the tumor had progressively increased in size. DIAGNOSIS: The final diagnosis was an IPT-like follicular cell sarcoma, based on CT, MRI, HE staining, and immunohistochemical staining. INTERVENTIONS: The patient underwent a laparoscopic left hemihepatectomy. OUTCOMES: The patient has not undergone any special treatment, such as radiotherapy and chemotherapy, and has been followed up for over 3 years without experiencing any recurrence. LESSONS: IPT-like FDCS is a rare tumor that lacks definitive criteria, and its diagnosis mainly relies on pathological findings. Previously, it was believed that being EBV-positive was an important condition for diagnosis. Primary IPT-like FDCS in the liver is even rarer, and the patient in this case tested negative for EBV. It may be necessary for pathologists to consider the role of EBV in the diagnosis of IPT-like FDCS.

A rare inflammatory myofibroblastic tumor appearing both inside and outside the heart.

BACKGROUND: Inflammatory myofibroblastic tumor (IMT) is an uncommon cardiac tumor that primarily affects infants, children, and young adults. While complete surgical resection generally leads to a favorable prognosis, accurate diagnostic tests remain limited. CASE PRESENTATION: We describe the case of a 26-year-old female who had a dual tumor inside and outside the heart and was misdiagnosed by echocardiography and MRI. We also review 71 cases of cardiac IMTs from the literature regarding their epidemiology, clinical presentation, and outcome. CONCLUSION: Early detection of this rare disorder is essential for optimal surgical management.

Ocular inflammatory myofibroblastic tumor in a pediatric patient.

Inflammatory myofibroblastic tumor (IMT) is a mesenchymal neoplasm that rarely metastasizes and is more commonly seen in children, adolescents, and young adults than older adults. These tumors, composed of myofibroblasts and inflammatory cells, are often confused for a local infection due to the inflammatory cell infiltration, and they form in mucosal surfaces but rarely arise in the orbit. We present the case of a 6-year-old girl with excisional biopsy-confirmed conjunctival stromal IMT. There was no evidence of recurrence 2 years following resection with no subsequent medical therapy.

A surgical case of inflammatory pseudotumor by hepatic anisakiasis.

Anisakiasis is a parasitic infection caused by the ingestion of raw or undercooked seafood infected with Anisakis larvae. It generally affects the gastrointestinal tract, particularly the stomach, but very rare cases have been reported in which infection of the liver leads to the formation of inflammatory pseudotumors. We herein report an extremely rare case of an inflammatory pseudotumor induced by hepatic anisakiasis that was laparoscopically resected for the purpose of both diagnosis and treatment. A 51-year-old woman underwent a routine medical checkup by ultrasound examination, which incidentally detected a 15-mm mass on the surface of S6 of the liver. Because a malignant tumor could not be ruled out on several preoperative imaging studies, laparoscopic partial resection of the liver was performed. Histopathological examination revealed Anisakis larva in the inflammatory pseudotumor, suggesting hepatic anisakiasis. This report describes an extremely rare case of an inflammatory pseudotumor induced by hepatic anisakiasis. Because the preoperative diagnosis could not be obtained by several imaging modalities, laparoscopic liver resection with a sufficient margin might be suitable for diagnosis and treatment of this disease.

Crizotinib combined with bronchoscopic interventional treatment in ALK-positive inflammatory myofibroblastic tumor of left main stem bronchus: a case report.

BACKGROUND: Inflammatory myofibroblastic tumor (IMT), also known as an inflammatory pseudotumor, is a unique type of intermediate soft tissue tumor that commonly occurred in the lung. Its unclear etiology and cellular activity brought about the confusion not only in naming of it, but also in diagnosis and treatment. CASE PRESENTATION: We reported the case of an 18-year-old male student who suffered from shortness of breath, chest tightness and chest pain. Chest computed tomography scan showed a spherical neoplasm blocking left main stem bronchus. After fiberoptic bronchoscopy procedure, the results of histopathological and immunohistochemical analysis indicated an IMT. The targeted next generation sequencing based genomic profiling of the tumor using formalin-fixed and paraffin embedded tissue was performed and a EML4-ALK fusion was detected. The patient began to receive Crizotinib, a ALK tyrosine kinase inhibitor, at a dose of 250 mg twice daily orally. The patient has recovered well after the operation, and no recurrence or metastasis has been found after 12 months' follow-up. CONCLUSION: By means of the diagnosis and treatment of this case, the characteristics and therapies of IMT are illustrated. In addition, it also provides a reference for the therapeutic strategy of IMT in the future.

Infantile inflammatory myofibroblastic tumour of the sigmoid colon: a diagnostic dilemma.

An inflammatory myofibroblastic tumour (IMT) is an uncommon neoplasm composed of inflammatory cells and myofibroblasts in a fibrous stroma. They are mostly seen in the lungs and rarely involve the gastrointestinal tract. An 8-month-old infant presented with a history of lower abdominal lump for 2 months. Her CT scan confirmed a large, lobulated mass in the retroperitoneum arising from the pelvis. The mass was found to be arising from the sigmoid colon on laparotomy which was excised. Histopathology showed a cellular tumour composed of spindle cells and inflammatory lymphocytic infiltrate. Immunohistochemistry revealed positive staining for anaplastic lymphoma kinase and smooth muscle actin, confirming the diagnosis of IMT. The patient is doing well at her 6-month follow-up. Ours is the youngest case of sigmoid IMT among the only other series of eight cases reported in the literature indicating its rarity.

Management Strategies of Plasma Cell Granuloma Involving the Central Nervous System: A Systematic Review of the Literature.

BACKGROUND: Plasma cell granuloma (PCG) is a rare clinical entity seen in the neurosurgical literature. It has often been referred to as inflammatory myofibroblastic tumor or inflammatory pseudotumor. No well-defined management guidelines exist in the literature. METHODS: Using PRISMA guidelines, we systematically reviewed the literature in PubMed and Google Scholar using MeSH terms: intracranial plasma cell granuloma, myofibroblastic tumor, intracranial pseudotumor, spinal plasma cell granuloma. We analyzed the clinical presentation, treatment strategies, clinical outcomes, and follow-up across different studies. RESULTS: Eighty-three studies were included presenting 108 cases. Primary extracranial disease was seen in 4 patients and primary central nervous system (CNS) disease in 104. In the combined cohort, multicompartmental disease was seen in 22 (20.8%) patients. Headache (n=40, 42.59%) was the most common clinical symptom. Surgical excision (n=86, 79.6%) was the most common primary treatment used. Radiation therapy, steroids, and chemotherapy (methotrexate/6-mercaptopurine/rituximab) were also used. Disease recurrence was noted in 25 (33.3%) patients and residual disease in 33 (30.5%). Mortality was seen in 4 (3.7%) patients. In the cranial PCG subgroup (n=87), 81 (93.1%) patients had solitary lesions, and 6 (6.8%) had multiple lesions. Recurrence after primary surgery was noted in 27.58% (n=24). In the spinal PCG subgroup (n=17), the thoracic spine was the most common location (n=9, 52.9%) and recurrence was seen in 5.84% (n=1). CONCLUSIONS: Combination of multiple treatment modalities is needed when approaching this complex disease. Spinal PCGs respond favorably to gross total excision, with a low recurrence rate. Cranial PCGs warrant intense follow-up with secondary chemotherapy/radiation/steroids in recurrent cases.

[A Case of Inflammatory Myofibroblastic Tumor of The Bladder Treated with Partial Cystectomy].

A 60-year-old woman with extensive hematuria visited our hospital. Cystoscopy revealed a tumor with an edematous surface on the left lateral wall of the urinary bladder. To diagnose the tumor, we performed a transurethral resection of the bladder tumor. Pathological examination suggested leiomyoma. Four weeks after the transurethral resection, magnetic resonance imaging revealed an increase in the bladder tumor. She received partial cystectomy. Pathological analysis revealed an inflammatory myofibroblastic tumor. No recurrence was observed 9 months after the initial operation.

Successful surgical management of an aggressive IgG4-related sclerosing esophageal inflammatory pseudotumor: a case report and review of literature.

BACKGROUND: Inflammatory pseudotumor (IPT) of the esophagus is a very rare benign lesions which clinical presentation is not clear and difficult to make a definitive diagnosis preoperatively. CASE PRESENTATION: In this report, we presented a case of a 24-year-old female with signs of severe malnutrition state due to dysphagia increasing gradually and losing 10 kg in weight for 2 months. Comprehensive preoperative radiologic investigations were proceeded with a circumferential severe stricture caused smooth submucosal swelling in the esophagus under 23 cm from the upper dental arch and two times of negative biopsy. Due to the aggressive clinical symptoms and gross lesion characteristics, the patient underwent laparoscopic-thoracoscopic esophagectomy and reconstruction with a gastric tube. Histopathological examination showed that the squamous epithelium of the esophagus had a small, benign nucleus, the submucosal layer and the smooth muscle layer increased fibrous, with infiltrating many lymphocytes, plasma cells, and macrophages. Immunohistochemical staining was negative for CD68, CD34, Desmin and ALK markers, and there was an increase in the number of IgG4-positive plasma cells. The final diagnosis was an aggressive IgG4-related sclerosing esophageal inflammatory pseudotumor. CONCLUSIONS: Inflammatory pseudotumor of the esophagus is an extremely rare benign lesion but could led to aggressive clinical presentation. The gold standard of diagnosis is histopathological examination of surgically removed specimens. Radical resection is still the most efficient treatment method.

Inflammatory myofibroblastic tumor of the urinary bladder: a case report.

The inflammatory myofibroblastic tumor (IMT) is a rare lesion, particularly in the urinary bladder. Inflammatory myofibroblastic tumor mainly affects children and young adults. It is unknown neoplastic potential, characterized by spindle cell proliferation with characteristic fibroinflammatory and pseudo-sarcomatous appearance. We describe a 36-year-old Moroccan man, who presented with hematuria the last week. The cystoscopy found a large bladder mass with necrotic-looking floating lesions, located in the trigonal area and left lateral wall on the dome of the urinary bladder. The patient underwent transurethral resection of the bladder tumor (TUR-BT). The histopathology and immunohistochemistry showed an IMT. No evidence of regrowth or residual tumor in 9 months of follow-up cystoscopy. In conclusion, even though, urinary bladder IMT is a rare occurrence, it is associated with a good prognosis. Histopathology investigation and immunohistochemistry analysis are essential to confirm the diagnosis. Complete TUR-BT is the treatment of choice.

Successful treatment with laparoscopic extended posterior sectionectomy for inflammatory pseudotumor in the liver with para-aortic lymphadenitis.

Inflammatory pseudotumor (IPT) is a rare disease that requires a differential diagnosis from malignancies. We describe a case of hepatic IPT with para-aortic lymphadenopathy, treated with a stepwise strategy of laparoscopic surgery. A 61-year-old woman was referred with a liver lesion. Computed tomography revealed a 13 cm well-defined lesion in segments VII-VI. The patient also had bead-like enlarged lymph nodes from the perihilar to the para-aortic regions. Although percutaneous lymph node biopsy showed no evidence of malignancy, 18 F-fluorodeoxyglucose positron emission tomography revealed accumulation in the lesion and lymph nodes. Lymph nodes were harvested laparoscopically for intraoperative pathological examination. With no evidence of malignancy, laparoscopic liver resection was continuously performed as a diagnostic treatment. The patient was given a pathological diagnosis of IPT and was discharged on the 16th day and is well 2 years after surgery. The minimally invasive laparoscopic approach to diagnostic treatment could be useful with secure advantages.

ALK-Negative Inflammatory Myofibroblastic Tumor: A Challenging Case in a Premature Newborn.

Inflammatory myofibroblastic tumors (IMT) are rare borderline tumors with a variable histological appearance that may mimic multiple mesenchymal tumors. We present a rare case of a challenging abdominal mass discovered in a premature newborn. The histopathology showed a bland myofibroblastic proliferation associated with an inflammatory infiltrate that was positive for smooth muscle actin and desmin but negative for anaplastic lymphoma kinase (ALK) protein. The diagnosis of an ALK-negative IMT was established. The tumor was partially resected. After six months of follow-up, the residual tumor remained stable, and the patient was asymptomatic. The correct diagnosis and subsequent treatment of ALK-negative IMT require appropriate histopathological, immunohistochemical, and sometimes genetic examination. Further research has to be conducted to help clinicians make an appropriate treatment plan.

Giant inflammatory pseudotumor of the lung: a case report.

The inflammatory myofibroblastic tumor, which was first described in 1939, is a rare neoplasm that accounts for 0.04%-0.7% of all lung neoplasms. These neoplasms occur most often in children, as they are the most common primary lung tumors in children. Preoperative diagnosis of such patients using bronchoscopy with endoluminal biopsy and transthoracic biopsy is not always informative and often the diagnosis can only be established during surgery. The presented case shows that on rare occasions, a giant myofibroblastic tumor of the lung may be encountered in adults, and radical intervention with subsequent rehabilitation can lead to full recovery.

IgG4 inflammatory pseudotumor mimicking primary lung cancer.

Introduction: IgG4 related disease (IgG4-RTD) is an infrequent disease with possible multiple organ involvement. It is characteristic to find inflammatory nodules with IgG4 positive plasma cell infiltration, storiform fibrosis and obliterative phlebitis. We present a patient with an inflammatory pseudotumor in the right upper lobe, mimicking a primary lung tumor. Case report: Our patient, a 48-year old heavy smoker (25 pack/year) with no relevant medical background, referred chest pain, non-productive cough and sporadic nightly fever. Image findings revealed a mass in the right upper lobe, with increased SUV in PET-scan, and mediastinal lymphadenopathies. Primary lung tumor was suspected and right upper lobectomy was performed. Due to absence of cellular atypia and the intense plasmacytic activity in the lesion, immunohistochemical analysis was performed: abundant IgG4 plasma cells were identified, with a IgG4/IgG relation of 74%. Diagnosis of IgG4- inflammatory pseudotumor was made. Discussion: After an extensive bibliographic review, we found just one similar case reported with an IgG4-lung pseudotumor without systemic disease.  Due to the broad spectrum of clinical features of IgG4-RTD, and the  potential multiple organ involvement, it is hard to find a classification and diagnostic criteria with high sensitivity and specificity, nevertheless they can be useful in clinical practice. Conclusion: There are several benign inflammatory diseases which can mimic a primary lung tumor. Although incidence is low, IgG4 pseudotumor should be considered as a differential diagnosis in the absence of malignancy.

Introducción: La enfermedad relacionada con IgG4 (IgG4-RTD) es una enfermedad poco frecuente con posible afectación multiorgánica. La presencia de infiltrados linfoplasmocitarios con células plasmáticas positivas para IgG4, fibrosis y flebitis obliterante. Presentamos el caso de un paciente con un pseudotumor inflamatorio en el lóbulo superior derecho, con presentación clínica compatible con tumor primario de pulmón. Caso clínico: Nuestro paciente de 48 años de edad, tabaquista severo (25 paquetes / año) sin antecedentes médicos relevantes, consulta por dolor torácico, tos no productiva y registros subfebriles aislados. Presenta una masa en el lóbulo superior derecho en estudio por imagen, con aumento de la captación en el PET, asociado a adenopatías mediastínicas. Con diagnóstico inicial de cáncer de pulmón, se realizó lobectomía superior derecha. Debido a la ausencia de atipia celular y la presencia de infiltrados linfoplasmocitarios en la lesión, se realizó análisis inmunohistoquímico: se identificaron abundantes células plasmáticas positivas para IgG4, con una relación IgG4 / IgG del 74%. Se realizó el diagnóstico de pseudotumor inflamatorio por IgG4. Discusión: Tras una extensa revisión bibliográfica, sólo encontramos un caso similar, de una paciente con un pseudotumor pulmonar IgG4 sin enfermedad sistémica. Debido a la variabilidad de la presentación clínica de la enfermedad relacionada a IgG4, y su potencial afectación multiorgánica, es difícil encontrar una clasificación y criterios diagnósticos con alta sensibilidad y especificidad, sin embargo estos suelen ser útiles en la práctica clínica. Conclusión: Múltiples enfermedades inflamatorias son diagnóstico diferencial de tumor primario de pulmón. Si bien la incidencia es baja, el pseudotumor IgG4 debe considerarse como un diagnóstico diferencial cuando no hay evidencia de enfermedad neoplásica.

Large Inflammatory Myofibroblastic Tumor of the Esophagus: A Case Report and Literature Review.

Inflammatory myofibroblastic tumor (IMT) is a rare tumor composed of myofibroblasts with inflammatory blood cell infiltration. It commonly occurs in the lungs and rarely in the esophagus. We herein report a valuable case of IMT originating in the esophagus. A 60-year-old Japanese woman with dysphagia had a large subepithelial lesion in the cervical esophagus, which was 15 cm in length. Surgical resection was performed to confirm the pathological diagnosis and improve the symptoms. The postoperative diagnosis was IMT composed of multiple nodules. There was no recurrence or metastasis within one year after surgery.

Superior caval vein syndrome and cardiac inflammatory myofibroblastic tumour in an infant.

The inflammatory myofibroblastic tumour, although very rare, must be considered in the differential diagnosis of intracardiac masses in children as it has systemic implications. We present a case of an infant whose diagnosis was suspected on clinical basis and echocardiogram, but the anatomopathological analysis with immunohistochemical study was essential for the conclusion of the histological type and orientation of the clinical follow-up.