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.

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.

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.

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.

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.

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)

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.

Inflammatory pseudotumors in the liver associated with influenza:A case report

BACKGROUND Inflammatory pseudotumor(IPT)is a rare and benign lesion that mimics malignancy and can develop in any part of the body.The pathophysiology and etiology of these quasineoplastic lesions remain unclear.CASE SUMMARY We report a case of a 65-year-old male who presented with fevers,night sweats,and unintentional weight loss following an influenza infection and was found to have multiple hepatic IPT’s following an extensive work up.CONCLUSION Our case highlights the importance of considering hepatic IPT’s in the differential in a patient who presents with symptoms and imaging findings mimicking malignancy shortly following a viral infection.

The radiological image of pulmonary inflammatory pseudotumor:an analysis of 36 cases

Objective:The aim of the study was to discuss the X-ray and CT manifestations and characteristics of pulmonary inflammatory pseudotumors.Methods:The 36 patients with pathological diagnosis of pulmonary inflammatory pseudotumor were tested by thoracic X-ray and CT scan,and their imaging characteristics were analyzed.Results:Most of masses had intact capsule and smooth border.It was very important to diagnose pulmonary inflammatory pseudotumor when the mass was closed to or adhered to the pleura with irregular shadow or being sharply circumscribed.Conclusion:X-ray and CT scan are very important ways to diagnose the pulmonary inflammatory pseudotumor,and to accurately diagnose it,the clinical data,complete analysis are also necessary.

Multiple inflammatory pseudotumor formation after craniopharyngioma resection via an extended nasal endoscopic approach:A case report

BACKGROUND This report describes a case of intracranial multiple inflammatory pseudotumors(IP)after endoscopic resection of a craniopharyngioma,which is relatively rarely reported in the literature,and neurosurgeons should be aware of its existence.CASE SUMMARY Herein,we report the case of a 56-year-old man who developed decreased visual acuity and blurred vision without obvious cause or inducement on April 27,2020.To seek further treatment,he went to the Department of Neurosurgery,Clinical Medical College,Yangzhou University.After falling ill,there was no nausea,vomiting,limb convulsions,obvious disturbance of consciousness,speech disorders,cough,or persistent fever.The neurological examination findings were normal,and pituitary magnetic resonance imaging(MRI)revealed multiple nodules with abnormal signals in the sellar region.The diagnosis was craniopharyngioma.We performed total resection of the tumor via transnasal endoscopy,and the postoperative pathology suggested that the type of tumor was craniopharyngioma.Six months after the operation,the patient experienced sudden hearing loss in the right ear,tinnitus in both ears,and numbness on the right side of the face and head.Meanwhile,cranial MRI showed multiple IP.After steroid hormone and anti-inflammatory therapy,the above symptoms did not significantly improve.Finally,the patient's symptoms were well improved by surgery,and the postoperative pathological diagnosis was multiple IP.CONCLUSION Intracranial inflammatory pseudotumor is a benign disease with slow progression,but the clinical symptoms and imaging findings are not typical,there are no pathological findings,and the diagnosis is relatively difficult.Most of the cases are treated by surgical resection,and the prognosis is good after surgery.

Diagnosis of hepatic inflammatory pseudotumor by fine-needle biopsy

Hepatic inflammatory pseudotumor(IPT)is a benign lesion characterized by chronic infiltration of inflammatory cells and fibrosis that clinically,radiologically,and pathologically mimics malignancy.However,the epidemiology of IPTs remains unclear.IPTs are often misdiagnosed as malignant lesions because of the lack of characteristic features.We present the case of a 32-year-old man that was misdiagnosed as intrahepatic cholangiocarcinoma by CECT,CEMRI,and CEUS,which was finally confirmed as IPT by fine-needle liver biopsy.In this report,the key factor in the diagnosis of liver inflammatory masses was the presence of hepatic perfusion disorder.

Pulmonary inflammatory pseudotumor caused by Cryptococcus presenting multiple nodules in bilateral lungs

By now there are a few reports describing a case of pulmonary inflammatory pseudotumor,presenting multiple nodules in bilateral lungs and a pseudotumor caused by fungus infection is also a very rare disease.Here we report a rare case of pulmonary inflammatory pseudotumor with confirmed cause by Cryptococcus infection presenting multiple nodules in bilateral lungs.

Intra-abdominal inflammatory myofibroblastic tumor:Spontaneous regression

Inflammatory myofibroblastic tumors are usually treated by surgical resection. We herein report two cases of intra-abdominal inflammatory myofibroblastic tumors that were unresectable and underwent spontaneous regression without any treatment. Our case report and literature review show that regression is more common in the middle-aged and older male populations. Abdominal discomfort and fever were the most common symptoms, but the majority of patients had no obvious physical signs. There was no specific indicator for diagnosis. The majority of the lesions regressed within3 mo and nearly all of the masses completely resolved within 1 year. We conclude that the clinical characteristics of inflammatory myofibroblastic tumors are variable and, accordingly, the disease needs to be subdivided and treated on an individual basis. Surgery is always the first-line treatment; however, for those masses assessed as unresectable, conservative therapy with in-tense follow-up should be considered.

Infant biliary cirrhosis secondary to a biliary inflammatory myofibroblastic tumor: A case report and review of literature

BACKGROUND A biliary inflammatory myofibroblastic tumor(IMT)is a rare type of mesenchymoma that,although it has a broad age spectrum,usually occurs in adults.Diagnosis is difficult because biliary IMTs often exhibit nonspecific clinical symptoms and imaging features,resulting in delayed or inappropriate treatment.Although most IMTs are benign,some show malignant properties such as infiltration,recurrence,and metastasis.CASE SUMMARY Here,we retrospectively describe a 10-month-old infant who was admitted to our hospital due to stubborn jaundice.The patient responded poorly to routine medical treatment and his clinical manifestations and laboratory tests lacked specificity,so we turned to repeated ultrasound scans and other imaging examinations.As both hepatosplenic ultrasonography and diffusion-weighted magnetic resonance imaging demonstrated a space-occupying lesion,an exploratory laparotomy was performed.The final diagnosis made over two mo after the disease onset was infant biliary cirrhosis caused by a biliary IMT,which partially infiltrated into the liver.This infant is the youngest case of biliary IMTs that has been reported till now.The patient underwent an incomplete resection of the mass and Kasai Portoenterostomy.However,because of cirrhosis,he also received a paternal liver transplant.Since some IMTs show malignant properties,we proceeded with a three-year of follow-up;however,no recurrence or metastasis has been noted.CONCLUSION Neoplastic disease such as IMTs should be considered when routine medical treatment of obstructive jaundice is not successful.Observation of dynamic imaging changes is helpful for diagnosis.Periodic follow-up is necessary for IMTs.

Pyogenic liver abscess as the first manifestation of hepatobiliary malignancy

BACKGROUND:Pyogenic liver abscess(PLA)is commonly seen in patients in the Far East.Similarly,hepatobiliary neoplasms are also common.PLA as the initial manifestation of hepatobiliary neoplasms is extremely rare. METHOD:Three patients with PLA were identified from the ward registion files and were retrospectively reviewed. RESULTS:These patients(two men aged 74 and 80 years, and one woman aged 35 years)were treated(in the right lobe in two patients and in the left lobe in one)and subsequently followed up for 11-22 months.Two patients were diagnosed with hepatocellular carcinoma and one with metastatic cholangiocarcinoma.Two patients were subjected to biopsies showing features of inflammatory pseudotumor before a diagnosis of hepatocellular carcinoma.One patient underwent hepatic resection with good results.In the other two patients who declined any further interventions,one died a few weeks after diagnosis and the other is still alive on conservative treatment. CONCLUSIONS:This series of cases highlights the importance of considering underlying hepatobiliary malignancies in patients with PLA and a close follow-up necessary for non-resolving hepatic lesions.

Primary Inflammatory demyelinating pseudotumor in the left frontal lobe with meningeal involvement presenting as malignant neoplasms

Background:Tumor-like demyelinating lesion is a rare form of IP,often indistinguishable from brain tumors on CT or MR imaging.Case presentation:We report here a case of pathologically confirmed tumor-like demyelinating lesions.The clinical manifestations,image feature and pathology of this case are also discussed together with a review of the relevant literature.Conclusion:The correct diagnosis of tumor-like demyelinating lesion mainly depends on the postsurgical histopathologic examinations.We suggest complete resection of the intracranial lesion and involved dura,followed by administration of short term oral prednisolone therapy will be good for preventing recurrence.

Inflammatory Myofibroblastic Tumor of the Liver:A Diagnostic Challenge

Inflammatory myofibroblastic tumor (IMT) is an uncommon myofibroblastic neoplasm that was formerly included within the broad category of inflammatory pseudotumor (IPT).IMT is rarely encountered in the liver.Similar to IMT of other organs,the interchangeable use of the terms IMT and IPT in liver has made the analysis of these lesions difficult.In this review,clinical and pathological features of IMT of the liver are reviewed and the differential diagnosis of IMT is discussed,with emphasis on IPT and the other entities included in this large category.IMT can mimic malignant tumors.There are no known unique diagnostic clinical,laboratory,or radiological features.The definitive diagnosis of IMT depends on careful pathological examination.The histopathological evaluation of hepatic IMT reveals that,the myxoid/vascular pattern is the most frequently observed,followed by,in decreasing frequency,fibrous histiocytomalike pattern and hypocellular fibrous pattern.In IMT of the liver,anaplastic lymphoma kinase (ALK) expression reliably predicts the presence of an ALK gene rearrangement.The diagnosis of hepatic IMT depends on the dominant histopathological pattern,and the management of the disease is still controversial.IMTof the liver is a distinctive neoplasm of intermediate biological potential,and should be distinguished from the variety of lesions that are included under the broad category of IPT.Therefore,to avoid confusion regarding the true incidence and behavior of hepatic IMT,the term IPT should not be used interchangeably with IMT.The rarity of IMT in liver should not minimize its consideration in the differential diagnosis of liver tumors,especially in patients with tumor markers in normal range.

Inflammatory myofibroblastic tumor of the right frontal lobe

Background: We aim to present an unique case of inflammatory myofibroblastic tumor (IMT) of the brain parenchyma and study the clinical presentation, imagine characteristic, intraoperative findings, and histopathology features of IMT in the brain parenchyma. Case Presentation: A 36-year-old female with IMT come to see doctor presented with a 3-month history of progressively worsening weakness in the left upper limb along with alalia. Serial magnetic resonance imaging studies revealed a lesion within the right frontal lobe. The lesions were locally clear boundary with peripheral tissues. A right frontotemporal craniotomy was performed and the specimens were detected by immunohistochemical staining and light microscopy. Through the microscope, the tumor was composed of large number of chronic inflammatory cells and spindle cells. From immunohistochemical stains, it demonstrated CD34 (+), SMA (+), Vimentin (+), and actin (+) were positive meanwhile S-100(-) was negative. Conclusion: IMT of the right frontal lobe is a rare lesions. Surgical resection is the best and the most effective treatment.