Bone destruction of orbital wall in idiopathic orbital inflammatory pseudotumor:does it always imply malignancy?

AIM:To assess the clinical presentations and outcomes of idiopathic orbital inflammatory pseudotumor(IOIP)patients with orbital wall bone destruction(OWBD)and to propose an expanded classification system that includes bone destruction.METHODS:The study retrospectively reviewed clinical presentations,imaging findings,treatment modalities,and outcomes of six patients diagnosed histopathologically with IOIP and OWBD at the Beijing Tongren Hospital,Capital Medical University between October 2018 and June 2021.RESULTS:Over two years,6(10%)of 60 IOIP patients at our hospital exhibited OWBD,but this may overrepresent severe cases.The cohort consisted of three men and three women,aged 17 to 60y(mean 35.5±16.1y).Presenting symptoms included proptosis,eyelid swelling,decreased visual acuity with pain,and palpable mass.Imaging revealed multiple anatomical structures involved with the medial wall being the most common site of bone destruction.Histopathological examination showed classic type in five patients and sclerosing type in one patient.All patients underwent surgical resection followed by methylprednisolone treatment.Follow-up(mean 30.3±3.1mo)indicated three patients had no recurrence,while others had varying degrees of symptom persistence or recurrence.CONCLUSION:IOIP with bone destruction is a rare but significant subtype that mimics malignancy,leading to potential diagnostic and therapeutic challenges.Our findings suggest that complete surgical resection combined with adjunctive glucocorticoid therapy can yield favorable outcomes.However,larger-scale studies are needed to further optimize therapeutic approaches.

Hepatic pseudotumor:A diagnostic challenge

Hepatic pseudotumors are rare lesions of unknown origin,characterized by the proliferation of fibrous connective tissue and inflammatory cell infiltrates.They mimic malignant lesions clinically,and radiologically,given their non-specific clinical and imaging features.The pathophysiology of hepatic pseudotumor is incompletely understood and there are no standardized criteria for diagnosis.Pseudotumors have been reported to develop in various organs in the body with the lung and liver being the most common site.Hepatic pseudotumors develop in patients with underlying triggers of liver inflammation and injury,including infections,autoimmune liver diseases,bile duct injury,or surgery.Hepatic pse-udotumors respond well to conservative treatment with antibiotics,and steroids and some may regress spontaneously,thus avoiding unnecessary resection.This condition is rewarding to treat.It is important to recognize pseudotumor as a distinct clinical entity and include it in the differential of liver masses with atypical imaging features.

Inflammatory pseudotumor of the liver and spleen diagnosed by percutaneous needle biopsy

An inflammatory pseudotumor (IPT) is a relatively rare lesion characterized by chronic inf iltration of inflammatory cells and areas of f ibrosis. IPTs are diff icult to diagnose because of the absence of specif ic symptoms or of characteristic hematological or radiological f indings. In this study, a case of a woman aged over 70 years was reported, who presented with a general malaise lasting more than two months. A computed tomography scan demonstrated a diffusely spread lesion of the liver with a portal vein occlusion and a splenic lesion surrounded by a soft density layer. Since the percutaneous liver biopsy showed f indings that suggested an IPT, although the radiological f indings did not exclude the possibility of a malignancy, we performed a percutaneous spleen biopsy to enable a more defi nitive diagnosis. The microscopic f indings from the spleen specimen lead us to a diagnosis of IPT involving the liver and spleen. Sub-sequent steroid pulse therapy was effective, and rapid resolution of the disease was observed.

Hepatic inflammatory pseudotumor presenting in an 8-year-old boy: A case report and review of literature

Hepatic inflammatory pseudotumors are uncommon benign lesions.Accurately diagnosing hepatic inflammatory pseudotumor can be very challenging because the clinical presentation and radiological appearances are nonspecific and cannot be certainly distinguished from malignant neoplastic processes.Herein,we present a case of hepatic IPT in an 8-yearold boy who presented to clinic with a 3-mo history of a tender hepatic mass,fever of unknown origin,and9-kg weight loss.The physical examination was notable for tender hepatomegaly.Laboratory investigations were notable for a normal hepatic profile and elevated erythrocyte sedimentation rate and C-reactive protein.A T2-attenuated magnetic resonance imaging scan of the abdomen showed a 4.7 cm x 4.7 cm x 6.6cm,contrast-enhancing,hyper-intense,well-defined lesion involving the right hepatic lobe.In view of the unremitting symptoms,tender hepatomegaly,thrombosed right hepatic vein,nonspecific radiological findings,and high suspicion of a deep-seated underlying infection or malignancy,a right hepatic lobectomy was recommended.Microscopically,the hepatic lesion exhibited a mixture of inflammatory cells(histiocytes,plasma cells,mature lymphocytes,and occasional multinucleated giant cells) in a background of dense fibrous tissue.Immunohistochemically,the cells stained negative for SMA,ALK-1,CD-21 and CD-23,diffusely positive for CD-68,and focally positive for lgG4.The final histopathological diagnosis was consistent with hepatic IPT.At the postoperative 4-mo follow-up,the patient was asymptomatic without radiological evidence of recurrence.

Relationship between dacryoadenitis subtype of idiopathic orbital inflammatory pseudotumor and paranasal sinusitis

AIM：To determine the clinical features of the dacryoadenitis subtype of idiopathic orbital inflammatory pseudotumor and its relationship with paranasal sinusitis.· METHODS：A retrospective analysis of 46 patients who received surgical treatment at the Department of Ophthalmology,Beijing Tongren Hospital,Capital Medical University for the dacryoadenitis subtype of idiopathic orbital inflammatory pseudotumor from October 2010 to December 2012.Each patient underwent magnetic resonance imaging（MRI） of the orbits and the 4paranasal sinuses.Disease status and the level of serum immunoglobulin G4（IgG4） was measured before and6 mo after surgery.· RESULTS：The initial clinical feature of the idiopathic dacryoadenitis type of orbital inflammatory pseudotumor was redness or swelling of the eyelids.Masses were palpated in the area of the lacrimal gland in some patients.Of the 46 patients,16 also suffered from sinusitis（34.8%）,with 14 cases of ethmoid sinusitis,8cases of maxillary sinusitis,9 cases of sphenoid sinusitis,and 8 cases of frontal sinusitis.Of the 16 patients with sinusitis,4 patients had a medical history of rhinitis（range：10mo to 15 y previously）,10 patients had occasional nasal congestion,and 2 patients had no nasal congestion.Thirteen of the 46 patients had elevated serum IgG4 levels.Nine of these 13 patients had MRI signs of sinusitis.All patients（n=46） received oral glucocorticoid treatment for approximately 3mo after surgery.No sign of recurrence was found in the orbital MRI 6mo after surgery.Of the 16 patients with sinusitis,9cases of elevated serum IgG4 levels improved after treatment with decreased serum IgG4 level and 7 cases of normal serum IgG4 levels remained unchanged.· CONCLUSION：Some patients with the dacryoadenitis subtype of idiopathic orbital inflammatory pseudotumor may also suffer from paranasal sinusitis.The incidence of paranasal sinusitis was much higher in patients with IgG4-elevated dacryoadenitis subtype orbital inflammatory pseudotumor than in those with normal IgG4 levels.Dacryoadenitis subtype orbital inflammatory pseudotumor and paranasal sinusitis may both the clinical manifestations of IgG4-related disease involved in different locations.

Role of MR in the differentiation of IgG4-related from non-IgG4-related hepatic inflammatory pseudotumor

BACKGROUND:Hepatic inflammatory pseudotumor(IPT)is classified into 2 types based on IgG 4 stain:IgG 4-related and non-IgG4-related; the two types differ not only in their pathological characteristics, but also in the clinical features. This study aimed to investigate the MR character of hepatic IPT,and differentiate the IgG4-related IPT from the non-IgG4-related IPT.METHODS:Twenty-five patients with 27 histologically proven hepatic IPTs were retrospectively analyzed. Ten lesions were diagnosed as IgG4-related IPT, and the other 17 as non-IgG4-related IPT. The MR signal features on T1,T2-weighted, dynamic-enhanced, and diffusion-weighted imaging were evaluated and compared. RESULTS:The dominant lesions were subcapsularly distributed(n=17, 63.0%) with clear boundary(n=20, 74.1%), and showed progressive enhancement pattern(n=21, 77.8%) with diffuse homogeneous(n=12, 44.4%) or heterogeneous(n=8,29.6%) hyperintensity, accompanied by delayed capsule-like enhancement(n=17, 63.0%) and central nonenhanced areas(n=18, 66.7%). Morphological features(P>0.05) were not sufficient to differentiate IgG4-related IPT from non-IgG4-related IPT; the wash-out pattern was only found in 2 IgG 4-related IPT, while the progressive enhancement pattern was more common in the non-IgG4-related lesions(n=16)(P=0.022).During portal and delayed phases, iso-/hypoenhanced lesions were only seen in 3 IgG4-related IPT, and circular-enhanced lesions(n=5) existed exceptionally in the non-IgG4-related group with significant differences(P=0.029 and 0.027). Most IgG4-related IPTs had lower apparent diffusion coefficient compared with the liver parenchyma(n=6), while most non-IgG4-related IPTs had higher apparent diffusion coefficient value(n=13)(P=0.046).CONCLUSIONS:Although MR images of hepatic IPT have certain characteristics, they are not enough to differentiate IgG4-related IPT from non-IgG4-related IPT. The enhancement pattern, signal features on portal and delayed phases, and the apparent diffusion coefficient value of the lesion may be helpful for the diagnosis.

Rare case of an abdominal mass: Reactive nodular fibrous pseudotumor of the stomach encroaching on multiple abdominal organs

Reactive nodular fibrous pseudotumor(RNFP), which presents abdominal clinical manifestations and malignant radiographic results, usually requires radical resection as the treatment. However, RNFP has been recently described as an extremely rare benign postinflammatory lesion of a reactive nature, which typically arises from the sub-serosal layer of the digestive tract or within the surrounding mesentery in association with local injury or inflammation. In addition, a postoperative diagnosis is necessary to differentiate it from the other reactive processes of the abdomen. Furthermore, RNFP shows a good prognosis without signs of recurrence or metastasis. A 16-year-old girl presented with a 3-mo history of epigastric discomfort, and auxiliary examinations suggested a malignant tumor originating from the stomach; postoperative pathology confirmed RNFP, and after a 2-year follow-up period, the patient did not display any signs of recurrence. This case highlights the importance of preoperative pathology for surgeons who may encounter similar cases.

Liver inflammatory pseudotumor or parasitic granuloma?

INTRODUCTION Liver pseudotumor is a very rare benign lesion.Since the first case reported by Pack and Baker in1953,only 40 cases had been reported up to 1996.The diagnostic challenge of hepatic inflammatorypseudotumor is emphasized by the fact that most ofthe reported cases were diagnosed by surgicalprocedures.Pathogenesis and etiology of

Inflammatory pseudotumor of the colon causing intussusception: A case report and literature review

Inflammatory pseudotumor(IPT) is a rare spaceoccupying lesion of unknown etiology that can mimic malignancy on clinic-radiological and pathological examination. We describe a rare case of ileocecal intussusception from clinically suspected malignancy of the right colon where the patient underwent righthemicolectomy. Histopathology of the resected specimen confirmed IPT of the colon. This patient was observed to have abnormally elevated total leukocyte count and platelets before and after surgery. In an adult with intussusception associated with an abdominal mass, the possibility of IPT of the colon should be considered. Considering the abnormally high total leukocyte and platelet counts and colonic IPT, it is necessary to prevent postoperative adverse effects due to these changes. Although IPT of the colon is usually a benign process, controversy regarding its management still exists. We consider hemicolectomy as a safe treatment approach for colonic IPT and review the existing literature.

Spontaneous regression of hepatic inflammatory pseudotumor with primary biliary cirrhosis:Case report and literature review

Hepatic inflammatory pseudotumor （IPT） is a rare benign non-neoplastic lesion characterized by proliferating fibrous tissue infiltrated by inflammatory cells. The exact etiology of IPT remains unclear. Although the association of IPT with systemic inflammatory disorders has been well established, a specific relationship with cholangitis is distinctly rare. We report a case of spontaneous regression of hepatic IPT with primary biliary cirrhosis （PBC）. To date, only two cases of IPT with PBC have been reported. In our case, however, IPT developed during the course of improvement of cholangitis of PBC induced by effective treatment, differing from two previously reported cases. Our case indicates that the development of IPT does not also relate to the activity of cholangitis and/or hyper gamma-globulinemia, since our case was confirmed radiologically to be free of IPT when biliary enzymes and immunoglobulins were much higher than the corresponding values on admission. Comparison of our case with the two previously reported cases suggests that IPT occurring with PBC does not represent the same disease entity or be a bystander for PBC.

Hilar inflammatory pseudotumor mimicking hilar cholangiocarcinoma

BACKGROUND: Inflammatory pseudotumor of the biliary tract is a benign disease, and is extremely rare. Its diagnosis often depends on pathological examination after operation. The histopathological examination shows inflammatory lesions with a polymorphous infiltration and variable amounts of fibrous tissue. This study was undertaken to elucidate that an inflammatory pseudotumor in the right hepatic duct is especially difficult to distinguish from hilar cholangiocarcinoma. METHOD: The clinical data of one patient with inflammatory pseudotumor of the right hepatic duct were analyzed. RESULTS: An occupying lesion of the right hepatic duct was revealed by abdominal ultrasound and magnetic resonance cholangiopancreatography. The right hepatic duct inflammatory pseudotumor was not identified during the operation but was confirmed by postoperative histopathological analysis. The patient recovered well without any serious complication. CONCLUSIONS: The preoperative evaluation for optimizing surgical management is important to the diagnosis of hepatobiliary occupying lesions. The evaluation involves clinical manifestations, imaging appearance and tumor markers. Malignant tumors and possible benign lesions should be considered to avoid aggressive surgical treatment. (Hepatobiliary Pancreat Dis Int 2010; 9: 219-221)

Recurrence of inflammatory pseudotumor in the distal bile duct: Lessons learned from a single case and reported cases

Inflammatory myofibroblastic tumors （IMTs） or inflammatory pseudotumors （IPs） have been extensively discussed in the literature. They are usually found in the lung and upper respiratory tract. However, reporting of cases involving the biliopancreatic region has increased over recent years. Immunohistochemical study of these lesions limited to the pancreatic head or distal bile duct seems to be compatible with those observed in a new entity called autoimmune pancreatitis, but usually intense fibrotic reaction （zonation） predominates producing a mass. When this condition is limited to the pancreatic head, the common bile duct might be involved by the inflammatory process and jaundice may occur often resembling adenocarcinoma of the pancreas. We have previously reported a case of IMT arising from the bile duct associated with autoimmune pancreatitis which is an extremely rare entity. Four years after Kaush-Whipple resection, radiological examination on routine follow-up revealed a tumor mass, suggesting local recurrence. Ultrasound-guided FNA confirmed our suspicious diagnosis. This present case, as others, suggests that persistent follow-up is necessary in order to prevent irreversible liver damage at this specific location.

Coumadin ridge: An incidental finding of a left atrial pseudotumor on transthoracic echocardiography

Coumadin ridge is a normal anatomic variant that is occasionally found in the left atrium. It can present as a linear or nodular mass which can undulate with cardiac motion and if particularly prominent, can easily be mistaken for a tumor or thrombus. Careful evaluation and consideration of the common variants discussed in this review can help limit misdiagnosis, as well as unnecessary workup and treatment. We present a case of coumadin ridge that was found on a patient using two-dimensional transthoracic echocardiography.

Pseudotumoral acute cerebellitis associated with mumps infection in a child

Pseudotumoral cerebellitis in childhood is an uncommon presentation of cerebellitis mimicking a brain tumor. It often follows an inflammatory or infectious event, particularly due to varicella virus. Patients could have a wide clinical spectrum on presentation. Some patients may be asymptomatic or present at most with mild cerebellar signs, whereas others may suffer severe forms with brainstem involvement and severe intracranial hypertension mimicking tumor warranting surgical intervention. Imaging techniques especially multimodal magnetic resonance imaging represent an interesting tool to differentiate between posterior fossa tumors and acute cerebellitis. We describe a case of pseudotumoral cerebellitis in a 6-year-old girl consequent to mumps infection and review the literature on this rare association.

Successful emergency resection of a massive intra-abdominal hemophilic pseudotumor

An intra-abdominal pseudotumor is a rare complication of hemophilia. Surgical treatment is associated with high morbidity and mortality rates and reported cases are scarce. We present a 66-year-old Caucasian male suffering from severe hemophilia type A treated for 10 years with Factor Ⅷ. Major complications from the disease were chronic hepatitis B and C, cerebral hemorrhage and disabling arthropathy. Twenty-three years ago, retro-peritoneal bleeding led to the development of a large intra-abdominal pseudotumor, which was followed-up clinically due to the high surgical risk and the lack of clinical indication. The patient presented to the emergency department with severe sepsis and umbilical discharge that had appeared over the past two days. Abdominal computed tomography images were highly suggestive of a bowel fistula. The patient was taken to the operating room under continuous infusion of factor Ⅷ. Surgical exploration revealed a large infected pseudotumor with severe intra-abdominal adhesions and a left colonic fistula. The pseudotumor was partially resected en bloc with the left colon leaving the posterior wall intact. The postoperative period was complicated by septic shock and a small bowel fistula that required reoperation. He was discharged on the 73 rd hospital day and is well 8 mo after surgery. No bleeding complications were encountered and we consider surgery safe under factor Ⅷ replacement therapy.

Hemophilic Pseudotumor: A Case Report and Review of Literature

A hemophilic pseudotumor is one of the rarer complications of hemophilia that results from repetitive bleeding resulting in an encapsulated mass of clotted blood and necrosed tissue. These have become rarer over the years with better treatment modalities for bleeding disorders like factor replacement. In this case report, we describe the natural history and clinical course of a patient with hemophilia who presented with a massive pseudotumor. A review of literature follows which explores all the relevant data to date on the presentation, complications, diagnostic modalities and therapies that have been implemented in the management of this rare complication of a common bleeding disorder.

Inflammatory pseudotumor of the liver and peripheral eosinophilia in autoimmune pancreatitis

AIM: Inflammatory pseudotumor (IPT) of the liver is a rare benign lesion, the etiology of which remains obscure.It is not associated with any particular diseases apart from phlebitis and Crohn's disease.METHODS: A middle-aged male with hepatic IPT and peripheral eosinophilia associated with autoimmune pancreatitis (AIP) was selected for this study and review of literature.RESULTS: A 59-year-old male was admitted with obstructive jaundice, marked eosinophilia (1 343/mm3)and hypergammaglobulinemia (4 145 mg/dL). Imaging techniques revealed dilatation of the intrahepatic bile duct,stenosis of the common bile duct with diffuse wall thickening, gallbladder wall thickening, irregular narrowing of the pancreatic duct, and swelling of the pancreatic parenchyma. Multiple liver masses were also demonstrated and diagnosed as IPT by biopsy specimens. Six months later, the abnormal features of the biliary tree remarkably improved by the oral administration of prednisolone, and the liver masses disappeared. The swelling of the pancreatic head also improved. The peripheral eosinophil count normalized. IPT associated with AIP, as we know,has not been reported in the literature. The clinical features of the present case mimicked those of pancreatic cancer with liver metastasis. This case deserves to be documented to prevent misdiagnosis of similar cases.

Splenic inflammatory pseudotumor mimicking angiosarcoma

Splenic tumors are rare. Differentiation of the tumors before operation is of great value regarding the outcome. A case of a 32-year-old man with a splenic inflammatory pseudotumor (IPT) mimicking splenic angiosarcoma is described. The tumor was highly suspected of being splenic angiosarcoma based on radiological findings preoperatively. However, after splenectomy, histopathological examinations revealed splenic IPT. Splenic IPT and angiosarcoma are rare and often pose diagnostic difficulties because the clinical and radiological fi ndings are obscure. Due to large differences in prognosis, we briefl y reviewed the clinical, radiological, and pathological features of both of the tumors.

Pseudotumor recurrence in a post-revision total hip arthroplasty with stem neck modularity: A case report

BACKGROUND This unique presentation of hip swelling is only the seventh reported case of pseudotumor recurrence in a non-metal-on-metal total hip arthroplasty(THA)construct.The constellation of patient symptoms and laboratory findings contradict the expected elevated serum metal ion levels associated with the formation of pseudotumor.The presentation,lab trends,and imaging findings contribute to the growing base of knowledge surrounding the effects of corrosion in arthroplasty constructs with stem-neck modularity.CASE SUMMARY A 74-year-old man status post primary THA presented with left hip swelling and elevated serum metal ions five years after implantation of a modular stem-neck prosthesis.The swelling was diagnosed as pseudotumor based on laboratory trends and imaging findings and was treated with revision arthroplasty that completely resolved the initial hip swelling.The patient presented with recurrent hip swelling and recurrent pseudotumor findings on imaging in the same hip four months later.Non-operative management with ultrasound-guided hip aspiration resulted in symptom relief and resolution of the recurrent swelling.After one year of follow-up,the patient had no further recurrences of hip swelling.CONCLUSION This case of post-revision pseudotumor recurrence elucidates attributable patient,surgical,and implant factors with a discussion of diagnostics,management,and prognosis for patients with pseudotumor in non-metal-on-metal arthroplasty constructs.

A Clinical Analysis of Idiopathic Orbital Inflammatory Pseudotumor

Purpose: To observe the clinical findings and response to treatment in patients with a diagnosis of idiopathic orbital inflammatory pseudotumor.Methods: 209 idiopathic orbital inflammatory pseudotumor cases seen between Jan 1, 1978 and Dec 31, 1999 in our hospital were evaluated retrospectively. Results: Of the 209 cases, 118 were male and 91 were female; there were 90 in the right eye, 81 left eye and 38 both eyes. Patients age ranged from 4 to 80 years (mean 44.4). Proptosis (66%), palpable mass (65%), swollen eyelid (55%), increased orbital pressure (55%) and motility restriction (48%) were the five most common presenting signs. According to radiologic and surgical findings, focal mass within orbit was the most frequent subtype (43% ), followed by lacrimal inflammatory pseudotumor (32%), diffuse orbital inflammation (10%), myositis (8%) . Perineuritis (2%), periscleritis(2%), acute inflammation (2%) and eyelid pseudotumor (1%) were rare clinical findings. The response to treatment (with a mean