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.

Epstein-Barr virus-positive inflammatory follicular dendritic cell sarcoma with significant granuloma: case report and literature review.

BACKGROUND: Epstein-Barr virus-positive inflammatory follicular dendritic cell sarcoma (EBV+IFDCS) is a rare disease characterized by mild clinical symptoms and non-specific imaging findings. The diagnosis of the disease depends on pathological diagnosis. However, EBV+IFDCS has a very broad spectrum of histological morphology and immune phenotypes, and its histopathological features have not been fully described by pathologists. CASE PRESENTATION: A 59-year-old female, with no significant discomfort, was found to have a splenic mass during a routine physical examination. Microscopic examination at low magnification revealed numerous epithelioid granulomas, amidst which a substantial inflammatory response was observed. Interspersed among the dense inflammatory cells were spindle or oval-shaped cells, distributed sporadically with indistinct boundaries. Under high magnification, these spindle cells had subtle features: smooth and clear nuclear membranes, inconspicuous small nucleoli, and infrequent mitotic figures. Immunophenotypically, the spindle cells expressed CD21 and CD23, and Epstein-Barr encoding region (EBER) in situ hybridization yielded positive results. The inflammatory milieu predominantly consisted of T cells, with a minority of plasma cells expressing IgG4. The confluence of morphological and immunohistochemical findings led to the final pathological diagnosis of EBV+IFDCS in this case. CONCLUSIONS: The presentation of EBV+IFDCS with pronounced granulomatous changes is rare. This morphological variant poses a high risk of misdiagnosis, frequently leading to confusion with other granulomatous diseases. Accurate diagnosis necessitates a comprehensive analysis, integrating immunohistochemistry and in situ hybridization. The case presented here is instrumental in raising awareness and understanding of EBV+IFDCS, with the goal of reducing misdiagnoses and unrecognized cases.

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.

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.

[A case of pediatric tracheal inflammatory myofibroblastic tumor].

Inflammatory myofibroblastic tumor is a rare tumor of mesenchymal origin. A case of intratracheal inflammatory myofibroblastic tumor in a male child was reported. The clinical characteristics, diagnosis, treatment and prognosis of the disease were reviewed based on the literature, and a differential diagnosis between inflammatory myofibroblastic tumor and hamartoma was performed to ultimately confirm the nature of the tumor in the child.

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.

Un caso singular de granuloma de cèlulas plasmáticas y actinomicosis en encía / A singular case of plasma cell granuloma and actinomycosis in the gum

Antecedentes: El granuloma de cèlulas plasmáticas (GCP) es una patología poco común que se caracteriza por una lesión roja e hiperplasia que presenta una gran afinidad a cualquier tejido blando con mayor frecuencia a nivel pulmonar y de manera poco usual a nivel oral como los labios, lengua y encía como un caso muy singular, histológicamente se caracteriza por un gran número de cèlulas plasmáticas. Objetivo: Reportar un caso poco común de GCP en encía con el abordaje, complicaciones y èxito del tratamiento del mismo. Descripción del caso: paciente femenino de 60 años de edad, acude a consulta por una lesión roja nivel de la encía marginal y adherida del maxilar superior. Al examen intraoral se observa una hiperplasia localizada Con diagnostico presuntivo de granuloma de cèlulas plasmáticas, donde se le realizaron exámenes radiográficos, posteriormente una biopsia, confirmando dicho diagnóstico. Conclusión: GCP se caracteriza clínicamente por presentar una hipertrofia gingival en la encía adherida, una de los lugares menos frecuentes a nivel oral. Esta patología al presentar ciertas similitudes clínica e histológicamente es muy difícil realizar un correcto diagnóstico de dicha patología (AU)

Background: Plasma cell granuloma (PCG) is an uncommon pathology characterized by a red lesion and hyperplasia that has a high affinity to any soft tissue, more frequently at the lung level and in an unusual way at the oral level such as lips, tongue and gingiva as a very unique case, histologically characterized by a large number of plasma cells. Objective: To report a rare case of GCP in the gingiva with the approach, complications and success of its treatment. Description of the case: a 60-year-old female patient comes to the clinic for a red lesion at the level of the marginal and attached gingiva of the upper jaw. Intraoral examination revealed localized hyperplasia with a presumptive diagnosis of plasma cell granuloma, where radiographic examinations were performed, followed by a biopsy, confirming said diagnosis. Conclusion: GCP is clinically characterized by presenting gingival hypertrophy in the attached gingiva, one of the least frequent places at the oral level. This pathology, as it presents certain clinical and histological similarities, is very difficult to make a correct diagnosis of said pathology. (AU)

Role of myofibroblasts in oral plasma cell granuloma: Immunohistochemical evaluation of α-SMA and ALK in a retrospective study of 30 cases.

INTRODUCTION: Oral plasma cell granuloma (OPCG) is a rare reactive lesion with generally benign yet occasionally aggressive behavior. Myofibroblasts are important in many physiologic, and pathologic conditions. The role of myofibroblasts in the clinical behavior of OPCG was assessed as well as its usefulness in differentiating this lesion from the inflammatory myofibroblastic tumor mimicking plasma cell granuloma. MATERIAL AND METHODS: This retrospective study included 30 paraffin blocks of OPCG. Immunohistochemical evaluation of alpha-smooth muscle actin (α-SMA) and Anaplastic lymphoma kinase (ALK) antibodies was performed. The mean area of positive expression was calculated and scored semiquantitatively with clinicopathologic correlations. RESULTS: Most of the cases were clinically non-aggressive. Alveolar bone resorption was observed in nine cases, two of them showed severe resorption and stromal fibrosis. Negative α-SMA was observed in 70% of cases showing a predominance of plasma cells in the stroma. All cases of stromal fibrosis revealed positive α-SMA of a weak percentage. A statistically significant difference was observed between α-SMA expression and the clinicopathologic variables. Negative ALK expression was noted in all cases. DISCUSSION: Myofibroblasts were infrequently found in OPCG. Remarkably, the aggressive behavior in cases with intense fibrosis was related to the existence of myofibroblasts even of non-neoplastic nature and minimal amount. The number of myofibroblasts and their nature assessed via α-SMA and ALK immunohistochemical expression respectively might be valuable in predicting the biological behavior of OPCG and may hold diagnostic significance in challenging OPCG cases that might mimic inflammatory myofibroblastic tumor.

Inflammatory Pseudotumor of the Liver.

Hepatic inflammatory pseudotumor (IPT) describes a mass lesion composed of fibroblasts or myofibroblasts with a dense inflammatory infiltrate comprising lymphocyte, plasma cells, and histiocytes. These lesions are presumed to be an exuberant response to an infectious organism, although in most cases the causative agent is unknown. In specific circumstances, pathologists should consider ancillary techniques to exclude specific infections, such as mycobacteria, Candida, or syphilis. IgG4-related disease may cause a plasma-cell rich IPT. Finally, true neoplasms can mimic IPTs and must be excluded with appropriate ancillary studies, including inflammatory myofibroblastic tumor, follicular dendritic cell tumor, inflammatory angiomyolipoma, Hodgkin lymphoma, and inflammatory hepatocellular carcinoma.

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.

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.

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.

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.

Inflammatory myofibroblastic tumors: A short series with an emphasis on the diagnostic and therapeutic challenges.

BACKGROUND: Inflammatory myofibroblastic tumors (IMTs) are a distinct entity of mesenchymal tumors. We present the challenges in their diagnosis and management. MATERIALS AND METHODS: A retrospective study with detailed clinical, radiological, and histopathological (HPE) features along with management and outcome of 10 biopsy-proven patients with IMT, between 2001 and 2020. RESULTS: The location included intracranial (5), orbital (4), and spinal (1) with M : F = 7 : 3. The mean age of onset was in the third decade. The commonest symptom was headache, while proptosis and blurred vision occurred in orbital IMTs. HPE revealed diffuse infiltration of mixed inflammatory cells over proliferating myofibroblasts. Smooth muscle antigen immunoreactivity was noted in fibroblastic spindle cells of all IMTs. However, we did not find anaplastic lymphoma kinase expression in any of our cases, as this is only found in ~ 50% of all IMTs. Tumor infiltration into adjacent tissue was noted in 4 patients. Surgical excision was limited to orbital IMTs, as most central nervous system (CNS) tumors were not amenable for resection. Steroid administration showed moderate improvement in the IMT-CNS patients but also required additional immunomodulation. Four patients had a median long-term follow-up of 7 years. Two patients had recurrent lesions demonstrated by imaging after 2 years of initial presentation. CONCLUSION: IMTs are rare and ambiguous tumors of unknown etiology that can occur anywhere in the body. Clinical and radiological features may not be specific to determine the diagnosis, but it should be considered as a differential diagnosis. Extensive thorough workup with histopathology along with the help of immunohistochemistry is conducive to better clinical outcomes. Surgical biopsy with extensive and total resection of these tumors along with steroid and radiotherapy may enhance the survival outcomes.