Clinicopathologic features and outcomes of hepatic inflammatory pseudotumour (IPT) and hepatic IPT-like lesions.

AIMS: Hepatic inflammatory pseudotumours (IPTs) are nonneoplastic hepatic masses characterized by variably fibroblastic stroma and inflammatory infiltrate, hypothesized to arise as part of a response to infection or prior surgery. The aim of this study was to evaluate the clinicopathologic features and outcomes of biopsy-proven hepatic IPT as well as other cases with IPT-like histologic features. METHODS AND RESULTS: A database search at our institution identified cases with a pathologic diagnosis of hepatic IPT (n = 80) between 2000 and 2023. Histologic features (stromal quality, inflammatory cell components, granulomas, and necrosis) were evaluated. Past medical and surgical history, microbiologic studies, and outcomes were reviewed retrospectively. Patients frequently had a past medical history of malignancy (34%), biliary disease (15%), or prior intraabdominal surgery (24%), and often presented with multifocal hepatic lesions (36%). Variable inflammatory backgrounds were present, including histiocytic (36%), lymphoplasmacytic (34%), or neutrophilic (24%). Specific organisms were identified in 15% of cases, most commonly Klebsiella and Staphylococcus species. Most patients with available clinical follow-up demonstrated radiologic resolution and/or had repeat negative biopsy; a minority of patients (8%) were subsequently diagnosed with neoplastic hepatic lesions. No significant association was seen between histologic features and the subsequent clinical or pathologic diagnosis of hepatic neoplastic lesions. CONCLUSIONS: Hepatic IPT is a heterogeneous entity that can present in a variety of clinical scenarios and show a wide morphologic spectrum. These lesions often regress spontaneously or with antibiotics. A subset of cases with hepatic IPT-like histologic features were subsequently diagnosed with malignancy, emphasizing the need for continued follow-up and repeat biopsy depending on clinical and radiologic features.

Inflammatory myofibroblastic tumour of the colon: 2 case reports and a comprehensive review of the literature.

PURPOSE: Inflammatory myofibroblastic tumour (IMT), which is also named as plasma cell granuloma (PCG) or inflammatory pseudotumour (IPT), is a rare tumour which rarely develops in the colorectal region. We aimed to review all reported cases to draw attention about this rare tumour. METHODS: We present two new cases of colonic IMT with no recurrence during the follow-up period. We also reviewed previously reported colorectal IMT/IPT/PCG patients to investigate demographics, diagnosis and treatment modalities. RESULTS: A total of 60 patients which including our 2 patients and 58 patients from 42 published articles were analysed. Male/female ratio was 34/26. Mean age was found to be 31.84 ± 22.26 years (9 months-82 years). Abdominal pain (56.7%) and fever (23.3%) were the most common complaints in the first admission. Fifty-nine (98.3%) out of 60 patients underwent surgery. During follow-up, 7 (14.3%) patients developed a local recurrence. CONCLUSION: IMT may occur at any age. IMT is considered to be a borderline tumour with the potential for recurrence or distant metastasis. Complete resection of the tumour is recommended for treatment. Long-time follow-up is necessary due to recurrence potential of the tumour even many years after complete surgical resection. TRIAL REGISTRATION: The study follows the regulation of the Institutional Review Board for human research at Izmir Katip Celebi University Ataturk Training and Research Hospital. Written informed consents were obtained from the patients who participated in this study.

Clinicopathological features of inflammatory pseudotumour-like follicular dendritic cell tumour of the abdomen.

AIMS: Inflammatory pseudotumour-like follicular dendritic cell (FDC) tumour is an extremely rare neoplasm. Herein, we report 10 cases of inflammatory pseudotumour-like FDC tumours in the abdomen and analyse their clinicopathological features. METHODS AND RESULTS: Seven patients were male, and three patients were female. The patients' age ranged from 28 years to 68 years (mean age, 52.9 years; median age, 51.5 years). Grossly, the tumour was usually solitary, well circumscribed, and solid, ranging from 23 mm to 233 mm in greatest diameter. Microscopically, the tumour was composed of variable amounts of spindle tumour cells with intense lymphoplasmacytic infiltrate. Unusual morphological findings, including eosinophils focally aggregated in one tumour admixed with multinucleated histiocytes in small clusters, fibrinoid deposits in the walls of ectatic blood vessel with occasional perivascular cuffs of lymphocytes, hyalinized fibrosis, and epithelioid granuloma, were identified. The tumour cells were immunoreactive for CD21, CD23, CD35, smooth muscle actin, and Epstein-Barr virus (EBV)-encoded latent membrane protein 1 (7/10, 70%). EBV-encoded small RNA was positive in all cases by in situ hybridization. The recurrence rate was 10% with a median follow-up of 19 months. CONCLUSIONS: Inflammatory pseudotumour-like FDC tumour should be differentiated from inflammatory myofibroblastic tumour, Hodgkin lymphoma, interdigitating dendritic cell sarcoma, and gastrointestinal stromal tumour.

Sclerosing mesenteritis: A real manifestation or histological mimic of IgG4-related disease?

We present three cases of sclerosing mesenteritis and review the literature to learn whether or not sclerosing mesenteritis is an IgG4-related disease (IgG4-RD). Our patients were all adult males. Their mesenteric masses ranged from 6.5 to 14.5 cm in the greatest diameter. Tissue specimens showed moderate to severe lymphoplasmacytic infiltration with occasional eosinophils against a background of irregular fibrosis. Both obliterative phlebitis and storiform fibrosis were noted in all cases. IgG4+ plasma cells were moderately increased in number (46 to 85 cells/high-power field). However, unlike IgG4-RD, the IgG4+/IgG+ plasma cell ratio was <40% (28% to 35%). Serum IgG4 concentrations were also within the normal range (43.2 to 105 mg/dL; normal range <135 mg/dL). Two biopsy cases showed spontaneous regression on imaging approximately 5 months later. No sclerosing conditions were found in other organs. The literature review identified 11 additional cases of sclerosing mesenteritis with IgG4+ plasma cell infiltration. However, conclusive cases with four characteristic features (high serum IgG4 levels, tissue IgG4 elevation, multi-organ involvement, and effective response to glucocorticoid therapy) have never been reported. In conclusion, although sclerosing mesenteritis shares histological features with IgG4-RD, most cases are less likely to be IgG4-related. IgG4-RD seemingly seldom, if ever, affects this anatomical site.

[Giant inflammatory pseudotumor of the cranial base]. / Pseudotumor inflamatorio gigante de la base del cráneo.

The inflammatory pseudotumour (IPT) is a non-neoplastic entity of unknown origin, and is characterised by a proliferation of connective tissue and a polyclonal inflammatory infiltrate. Central nervous system involvement is uncommon, and usually represents a diagnostic and therapeutic challenge even for the experienced clinician. This reports deals with the case of a 56year-old woman diagnosed with a giant, infiltrating mass centred in the left cavernous sinus, who had a rapid clinical and radiological response to steroid therapy. Biopsy specimens were diagnostic for IPT. The progression of a small orbital residual lesion was detected after steroid withdrawal. Treatment with cyclophosphamide induced a complete response that remains stable after six years of follow-up.

Inflammatory myofibroblastic tumour of the urinary bladder: the role of immunoglobulin G4 and the comparison of two immunohistochemical antibodies and fluorescence in-situ hybridization for the detection of anaplastic lymphoma kinase alterations.

AIMS: We examined gene rearrangement and the expression of anaplastic lymphoma kinase (ALK) in urinary bladder inflammatory myofibroblastic tumour (IMT) using fluorescence in-situ hybridization (FISH) and two immunohistochemical antibodies to ALK. We also investigated whether IMT represents an immunoglobulin (Ig)G4-related disease. METHODS AND RESULTS: The performance of the Dako FLEX ALK monoclonal antibody (CD246) and the Cell Signaling Technology ALK (D5F3) XP monoclonal antibody were compared. Overall, 11 of 16 tumours showed ALK expression by immunohistochemistry (69%). Ten demonstrated ALK expression with both stains and one was positive with D5F3 but not CD246 (91% correlation). The D5F3 antibody yielded a stronger staining intensity and a higher sensitivity. Nine tumours demonstrated ALK rearrangements (56%) by FISH. Three were ALK(+) by immunohistochemistry but negative for rearrangement by FISH, whereas one showed rearrangement by FISH but was negative by immunohistochemistry. In total, 12 tumours were positive for ALK abnormalities (75%). Using current criteria, no cases were classified as an IgG4-related disease. CONCLUSIONS: The ALK D5F3 immunohistochemical stain showed superior staining characteristics compared with ALK CD246. Discrepancies in the results between FISH and immunohistochemistry for ALK abnormalities may have causes that are multifactorial. By current criteria, IMT does not represent an IgG4-related disease.

Inflammatory pseudotumours in the abdomen and pelvis: a pictorial essay.

Inflammatory pseudotumours are uncommonly encountered lesions in the abdomen and pelvis that often present with variable and nonspecific imaging features. They may mimic other more common lesions, including malignancy. Within the appropriate clinical context, inflammatory pseudotumours merit consideration in the differential diagnosis of soft-tissue masses within the abdomen and pelvis. A preoperative diagnosis of inflammatory pseudotumour, established through biopsy, may help to differentiate this benign entity from malignancy. In this article, we reviewed the imaging features of inflammatory pseudotumours of the abdomen and pelvis, including liver, spleen, bowel, retroperitoneum, kidney, bladder, uterus, and adnexa.

Photodynamic therapy for cutaneous inflammatory pseudotumour: A case report.

Inflammatory pseudotumour (IP) is a rare proliferative disease characterized by a dense infiltrate of plasma cells, lymphocytes, eosinophils and neutrophils in the fibrous stroma. It primarily affects the lungs of pediatric patients or young adults. Cutaneous IP is an extremely rare condition, with limited documentation in the English literature. In this case report, we presented an unusual instance of a 62-year-old male endured recalcitrant cutaneous IP for 8 years and exhibited poor response to topical glucocorticoid therapy, as well as intralesional injections of pingyangmycin and/or corticosteroid. Notably, after undergoing four sessions of 5-aminolevulinic acid photodynamic therapy (ALA-PDT), the patient experienced a significant reduction in erythema and nodules. This observation suggests that ALA-PDT may represent a promising and safe treatment option for cutaneous IP.

A Case of Inflammatory Pseudotumour Masquerading as Hepatocellular Carcinoma.

Inflammatory pseudotumors (IPTs) of the liver can mimic malignant lesions. As the name implies, they are usually associated with an inflammatory process and usually regress with the treatment of the underlying pathology. We report a case of a 67-year-old female who presented with right upper quadrant pain, deranged liver enzymes, elevated tumor markers [alpha-fetoprotein (AFP) and CA 19-9], and a large liver mass on imaging, suspected to be hepatocellular carcinoma (HCC). She was eventually diagnosed with IPT complicating the liver inflammation due to autoimmune hepatitis (AIH). She responded well to treatment with steroids and immunosuppressive therapy.

Hydrocephalus associated to posterior fossa tumor-like sarcoidosis: A case report and literature review.

Background: Intracranial inflammatory pseudotumors (IIPT) are one of the differential diagnosis for the central nervous system (CNS) tumors. They represent a rare condition that may mimic clinically and radiologically intracranial tumors and induce their complications. Among their etiologies, neurosarcoidosis is one of the less known and less frequent. To the best of our knowledge, only two cases of posterior fossa IIPT have been reported in the literature. We present here the 3rd case related to a neurosarcoidosis. Case presentation: We report the case of a 55-year-old female patient who presented with an altered state of consciousness associated to severe intracranial hypertension syndrome for four months. Glasgow coma scale on admission was 14/15. Brain imaging revealed bilateral cerebellar micronodular meningeal enhancement regarding the mesencephalon and the pons, as well as a nodular lesion of the 4th ventricle causing a triventricular acute hydrocephalus. The patient had a ventriculo-peritoneal shunt with a favorable outcome. Afterwards, she underwent a salivary gland biopsy which confirmed the diagnosis of neurosarcoidosis. Conclusion: Posterior fossa IIPT are very rare, mainly when located in the posterior fossa, leading to confusion with other pathologies. MRI has an important role in the diagnosis of these lesions, and the determination of their etiology. It shows other than the IIPT itself, many other signs such as leptomeningeal enhancement, nodular lesions or pituitary stalk thickening. These signs can orientate towards the diagnosis. Treatment may associate to symptomatic approach, corticosteroids. Surgical resection may be proposed when the diagnosis remains doubtful.

Inflammatory Myofibroblastic Tumour of the Sinonasal Tract with Orbital and Intracranial Extensions Simulating a Malignancy: A Case Report and Review of Literature.

Inflammatory Myofibroblastic Tumour (IMT) is a rare pathology of unknown etiology. It is pathologically benign, but clinically locally invasive. We are herewith reporting a unique case of rapidly growing sinonasal IMT with orbital and intracranial extensions in a middle aged female patient masquerading as a malignancy. The lesion was excised endoscopically with margins and the patient is presently under close follow up. The report also reviews the common sites of involvement and management options available, based on a literature review.

Inflammatory pseudo-tumour of the colon mimicking acute appendicitis: A case report.

INTRODUCTION: Inflammatory pseudo-tumour (IPT) of the colon is a rare entity with an obscure pathophysiology and largely indeterminate aetiology. PRESENTATION OF CASE: A young male patient presented with an Alvarado score of 9/10 and was admitted for appendectomy. An irregular hepatic flexure mass was discovered intraoperatively. The patient underwent an oncological right hemicolectomy with lymphadenectomy under the supposition that it was malignant and recovered with no short or long-term repercussions. Haemoxylin and eosin staining of the mass revealed features of a benign IPT. DISCUSSION: IPTs have clinical and radiological features that may be indistinguishable from those of malignancies, often resulting in extensive oncological resections despite recurrence and malignant transformation being negligibly rare. CONCLUSION: Benign pathologies such as IPT that mimic malignancy can sometimes result in extensive investigations or radical resections, the justification of which can only be a point of contention in retrospect. The following report explores our experience with one such patient and is accompanied by a review of the literature.

Application of 3D-printed prosthesis in revision surgery with large inflammatory pseudotumour and extensive bone defect: A case report.

BACKGROUND: Hip revision surgery is the final treatment option for the failure of artificial hip joints, but it is more difficult than the initial operation. For patients with hip joint loosening around the prosthesis combined with large inflammatory pseudotumours and large segment bone defects, hip revision is even more difficult, and clinical reports are rare. CASE SUMMARY: Male, 59 years old. The patient underwent left hip replacement 35 years ago and was now admitted to hospital due to massive masses in the left thigh, shortening of the left lower extremity, and pain and lameness of the left hip joint. X-ray, computed tomography and magnetic resonance imaging revealed prosthesis loosening, left acetabular bone defect (Parprosky IIIB type), and a bone defect of the left proximal femur (Parprosky IIIA type). Inflammatory pseudotumours were seen in the left hip and left thigh. Hip revision surgery was performed using a 3D-printed custom acetabular prosthesis was used for hip revision surgery, which was produced by Arcam Electron Beam Melting system with Electron Beam Melting technology. The operation was successful, and the patient was followed up regularly after the operation. The custom-made acetabular prosthesis was well matched, the inflammatory pseudotumour was completely removed, the postoperative hip prosthesis was stable, and the old greater trochanter fracture was well reduced and fixed. The patient was partially weight-bearing with crutches 3 mo after the operation and walked with full weight-bearing after 6 mo. The hip prosthesis was stable, and there was no recurrence of inflammatory pseudotumours at the last follow-up. The Visual Analogue Scale was 3, and the Harris hip score was 90. CONCLUSION: The use of 3D-printed personalized custom prostheses for complex hip revision surgery has satisfactory surgical results and has great clinical application value.

Inflammatory pseudotumor-like follicular/fibroblastic dendritic cell sarcoma: focus on immunohistochemical profile and association with Epstein-Barr virus.

Inflammatory pseudotumour-like follicular/fibroblastic dendritic cell sarcoma (IPT-like FDCS) is a rare EBV-associated variant of follicular dendritic cell sarcoma, usually arising in the liver or spleen and characterized by a favourable prognosis. The neoplastic cells show variable follicular dendritic cell or fibroblastic reticular cell differentiation and their immunoprofile is still poorly characterized. We describe a case of splenic IPT-like FDCS with unexpected CD31 expression and provide a concise review of English literature on the topic.

Case Report: Recurrence of Testicular Myofibroblastic Tumor After Surgery.

Inflammatory myofibroblastic tumour (IMT), also known as plasma cell granuloma (PCG) or inflammatory pseudotumour (IPT), is a distinctive, rarely metastasizing neoplasm composed of myofibroblastic and fibroblastic spindle cells accompanied by inflammatory infiltration of plasma cells, lymphocytes and/or eosinophils. IMT predominantly affects children and young adults, and the age at presentation ranges from 3 to 89 years. We present a very rare case of recurrent testicular IMT without ALK rearrangement. This case highlights the clinical characteristics and diagnostic factors associated with primary and recurrent foci of this rare tumour, along with key therapeutic approaches.

Paratesticular fibrous pseudotumour in a 26-year-old Nigerian man: A case report.

INTRODUCTION AND IMPORTANCE: Benign fibroblastic proliferation located within the scrotum are uncommon. They are also identified as fibrous pseudotumour and usually arise from the paratesticular region. Their pathogenesis is unknown. These tumours are difficult to diagnose pre-operatively and resemble intra-scrotal malignancy. CASE PRESENTATION: We managed a 26-year-old Nigerian man with a five-year history of left sided hemiscrotal swelling. The mass was excised, and histology showed benign fibroblastic proliferation. DISCUSSION: Most paratesticular tumours are benign. Imaging studies that show the mass as separate from the testis, normal levels of tumour markers and clinical features not suggestive of malignancy make the case for organ-sparing mass excision, in the treatment of paratesticular fibrous pseudotumour, very strong. CONCLUSION: Intra-scrotal benign fibrous proliferation is a challenge to diagnose pre-operatively but excision of this benign mass without an orchidectomy is practicable when pre-operative assessment suggests a benign disease.

Computed tomographic features of inflammatory myofibroblastic tumour of the stomach in adult patients: An analysis of five multicentre cases with literature review.

INTRODUCTION: The aim of this study was to describe the clinical and computed tomography (CT) features of gastric inflammatory myofibroblastic tumour (IMT) in adult patients and provide a review of the literature. METHODS: Five adult patients with surgically resected and pathologically confirmed gastric IMT were included in the study from four tertiary referral centres. Clinical history was assessed for determination of patient demographics and symptoms at presentation. All patients underwent contrast-enhanced abdominal CT, and two radiologists assessed the CT features by consensus. Also, abdominal CT findings from previous case reports of nine adult patients with gastric IMT were reviewed and summarised. RESULTS: Of five gastric IMTs, four (80%) appeared as a well-defined subepithelial mass and only one (20%) appeared as an irregular wall thickening. All of five gastric IMTs showed strong enhancement. When we analysed the CT features of nine cases from previously published literatures combined with our five cases, predominant imaging feature of gastric IMT was a well-defined subepithelial mass with strong enhancement. Perigastric infiltration or direct invasion of the neighbouring organs was rarely seen. CONCLUSION: Gastric IMT in adult patients appeared either as a well-defined subepithelial mass or irregular wall thickening, with mostly strong enhancement. Although rare, these imaging feature may be helpful for diagnosis of gastric IMT.

Bilateral Inflammatory Pseudotumour of the Breast: A Case Report and Review of the Literature.

Inflammatory tumour (IPT) consists of spindle cells, mature plasma cells, histiocytes, lymphocytes and eosinophils. Most frequently presenting in the respiratory tract it can also affect other sites such as breast. This case was a 73-year old woman presenting with a left breast lump, clinically indeterminate (P3), proven on biopsy to be IPT. Seven years later she returned with bilateral breast lumps and underwent triple assessment followed by wide excisions which confirmed the diagnosis of IPTs. Because it can be difficult to differentiate IPT from a low-grade spindle cell metaplastic breast carcinoma (SpCMBC) wide excision to achieve clear margins should be achieved to exclude malignancy.

Inflammatory pseudotumour of the infratemporal fossa visualized with 18F-FDG PET/CT.

The inflammatory pseudotumour of the head and neck is a benign lesion, extremely rare outside the cranial orbits. A case is presented of an inflammatory pseudotumour not associated with the IgG4-related disease. The pseudotumour was found as a solitary mass in the infratemporal fossa of a young woman who complained of otalgia and hearing loss. A clear image of the lesion was obtained using an 18F-fluoro-deoxy-glucose (18F-FDG) PET. After the histopathological diagnosis, and treatment with corticosteroids, a second 18F-FDG PET was performed. The metabolic image had returned to normal, and the previously observed mass disappeared. A brief review is presented of the studies examining this type of lesion.

Hepatic inflammatory pseudotumour-like follicular dendritic cell tumor: A case report.

Hepatic inflammatory pseudotumor (IPT)-like follicular dendritic cell (FDC) tumor is a rare neoplasm. We herein report the case of 19-year-old female patient with an IPT-like FDC tumor and summarize 24 cases of hepatic FDC tumors previously reported in the English literature. The patient presented with complaints of abdominal discomfort, without significant laboratory abnormalities, and underwent surgical removal of a hepatic tumor. The resected tumor was 6 cm in the longest diameter and the tumor cells were positive for CD21, CD35 and Epstein-Barr virus (EBV). The postoperative course was uneventful and there have been no metastases or recurrence during 1 year of follow-up. The majority of the cases of hepatic IPT-like FDC tumors have a female predominance and exhibit an association with EBV infection. Their clinical manifestations and image findings are usually non-specific and the diagnosis of this disease mainly relies on pathology.