Infantile Inflammatory Myofibroblastic Tumor of Spleen.

Background: Inflammatory myofibroblastic tumor (IMT) is a mesenchymal neoplasm with unknown etiology and recurrent potential. They are widely reported in children and young adults. Nearly 50% of inflammatory myofibroblastic tumor harbor rearrangement in anaplastic lymphoma kinase (ALK) gene with the majority expressing ALK protein. ALK-negative IMTs harbor alteration in ROS1 gene in a subset of cases. Few reports have shown association of IMT with Epstein-Barr virus (EBV). Case report: We report a case of IMT of the spleen in an 18-month-old infant presenting with abdominal distention and failure to thrive. Workup for ALK-1, ROS1, and EBV small-encoded RNA in-situ hybridization using immunohistochemistry was negative. Conclusions: IMT can arise in an infant spleen.

Inflammatory pseudotumor associated with HSV infection of rectal vascular endothelium in a patient with HIV: a case report and literature review.

BACKGROUND: Herpes simplex virus (HSV) typically infects oral or anogenital squamous epithelium and causes blisters and ulcerations. Here we reported an unusual case of HSV induced exuberant rectal inflammatory pseudotumor with vascular endothelial involvement. CASE PRESENTATIONS: A 52-year old man with HIV presented with abdominal pain, rectal drainage and constipation. Proctoscopy and CT scans revealed an 8 × 5 × 4 cm circumferential, mid-lower rectal mass that was concerning for malignancy. PET-CT showed mild to moderate FDG uptake of the rectal mass. Repeated biopsies showed exuberant lymphoplasmacytic inflammation with rich eosinophils and necrosis in the submucosa and scattered single or multi-nucleated viral inclusions in vascular endothelial cells that were positive for HSV by immunostains. There was no evidence of malignancy on histology or by immunostains. The patient started valacyclovir for three weeks and symptoms resolved after the antiviral therapy. Follow-up CT and sigmoidoscopy with biopsy revealed no rectal mass or drainable collection. CONCLUSIONS: HSV may present as proctitis with exuberant inflammatory response and mass-like lesion, and damages vascular endothelial cells in patients with HIV. The HSV-associated mass-like lesion can be effectively treated by 3-week valacyclovir.

Retro-odontoid Degenerative Pseudotumour Causing Spinal Cord Compression and Myelopathy: Current Evidence on the Role of Posterior C1-C2 Fixation in Treatment.

BACKGROUND: A retro-odontoid pseudotumour compressing the spinal cord and causing myelopathy is often associated with an inflammatory condition such as rheumatoid arthritis. A degenerative non-inflammatory retro-odontoid pseudotumour responsible for clinically relevant spinal cord compression is a rare condition described in small clinical series and is likely associated with craniovertebral junction hypermobility or instability-like conditions. For several years, direct removal of the lesion through an anterior or lateral approach has been advocated as the best surgical option. However, in the last decade the posterior approach to the craniovertebral junction, to perform C1-C2 fixation and C1 laminectomy without removal of the retro-odontoid tissue, has demonstrated its efficacy in reducing retro-odontoid pannus as well as in obtaining improvement of myelopathy. METHODS: In this paper we analyse the clinical and radiological outcomes of seven patients (five males and two females) treated with posterior C1-C2 fixation and C1 laminectomy for a degenerative non-inflammatory retro-odontoid pseudotumour responsible for spinal cord compression. C1 laminectomy provided immediate spinal cord decompression. We also review the relevant literature focusing on associated cervical degenerative conditions that may contribute to triggering or acceleration of atlantoaxial hypermobility or 'instability', causing formation of the retro-odontoid tissue. RESULTS: The mean follow-up period (of six followed-up patients) was 55.8 months (range 10-96 months). In all cases the Nurick score at the latest follow-up visit demonstrated clinical improvement; magnetic resonance imaging during follow-up demonstrated progressive reduction of the retro-odontoid pseudotumour in all but one patient, who died of surgery-unrelated disease in the early postoperative period. No vascular or neural damage secondary to C1-C2 fixation was observed. CONCLUSION: C1-C2 fixation associated with C1 laminectomy is an effective surgical option to treat myelopathy secondary to a degenerative retro-odontoid pseudotumour. In these cases, direct removal of intracanalar tissue compressing the spinal cord is not required, as C1-C2 fixation is sufficient to cause its disappearance.

Post-operative regression of retro-odontoid pseudotumors treated with and without fusion.

PURPOSE: Retro-odontoid pseudotumor is common in elderly people and is a cause of cervical myelopathy. The goal of the study was to investigate surgical procedures, outcomes, and post-operative spontaneous regression of posterior cervical retro-odontoid pseudotumors. METHODS: The subjects were 29 patients who underwent surgery for myelopathy due to a retro-odontoid pseudotumor around the craniocervical region at 9 facilities and were followed-up for an average of 54 months (range 12-96 months). Data were collected in a multicenter review of a retrospective database. Comparisons were performed between cases treated with and without fusion. RESULTS: The JOA recovery rate at final follow-up did not differ significantly between the fusion (n = 17, including all 15 patients with atlantoaxial subluxation) and non-fusion (n = 12) groups. However, pseudotumor regression was significantly more frequent in the fusion group (100% vs. 42%, p < 0.01). In all patients, regression cases had significantly higher rates of contrast enhancement of the pseudotumor on pre-operative T1 gadolinium-enhanced MRI (68% vs. 14%, p = 0.013) and of JOA recovery (50% vs. 30%, p < 0.01). CONCLUSIONS: Regression of pseudotumor occurred in all cases treated with fusion surgery. There was a significant difference in pseudotumor regression with or without fusion, and regression was significantly related to gadolinium enhancement on MRI. Therefore, it is preferable to use fusion surgery for a retro-odontoid pseudotumor that shows contrast enhancement, even if there is no apparent instability pre-operatively. These slides can be retrieved under Electronic Supplementary Material.

[Inflammatory Pseudotumor in the Cecum-A Case Report and Review of Literature].

A 42-year-old woman with complaints of fever and abdominal pain, was diagnosed to have perforative appendicitis, for which emergency surgery was performed. Marked thickening and edema around the cecum, ileum, and the mesentery was observed. Ileocecal resection was performed, as malignant disease could not be excluded. Histopathological examination revealed abscess formation in the lining membrane of the intestine, inflammatory granulation and proliferation of the spindleshaped cells in the serosal membrane extensively, and an inflammatory pseudotumor(IPT)was diagnosed. It is generally difficult to clearly distinguish IPT from a malignant tumor before surgery. We thus report this case, along with a review of literature.

Differentiating Primary Central Nervous System Lymphomas From Glioblastomas and Inflammatory Demyelinating Pseudotumor Using Relative Minimum Apparent Diffusion Coefficients.

OBJECTIVE: Our purpose was to evaluate the diagnostic performance of diffusion-weighted imaging, the relative minimum apparent diffusion coefficient (rADCmin) in differentiating primary central nervous system lymphomas (PCNSLs) from glioblastomas (GBMs) and inflammatory demyelinating pseudotumors (IDPs). MATERIALS AND METHODS: Magnetic resonance images were reviewed retrospectively in 82 patients including 39 PCNSLs, 35 GBMs, and 8 IDPs. Regions of interest were drawn around the tumor on contrast-enhanced axial images; these images were transferred onto coregistered ADC maps to obtain the ADCmin, and the normalized ADCmin ratios (rADCmin) were calculated using the formula rADCmin = ADCmin of the lesion / ADCmin of the normal white matter. The rADCmin values were compared between PCNSLs, GBMs, and IDPs using the analysis of variance test. Receiver operating characteristic curves were constructed to evaluate the diagnostic performance of rADCmin values and to determine the optimum thresholds. Simple logistic regression was analyzed to evaluate the relationship between ADCs and tumor cellularity. RESULTS: The rADCmin value was significantly lower in PCNSLs (0.675 ± 0.113) than GBMs (0.765 ± 0.059) and IDPs (0.834 ± 0.067) (PCNSL vs GBM, P < 0.001; PCNSL vs IDP, P < 0.001). Relative ADCmin was a significant assessor for differentiating PCNSLs from non-PCNCLs (P < 0.001). The optimal cutoff value was 0.722 (sensitivity, 74.5%; specificity, 74.1%; area under the curve, 0.803) on receiver operating characteristic analysis. A stronger negative correlation (r = -0.755, P = 0.000) was obtained between the cytoplasm and rADCmin. CONCLUSIONS: Relative ADCmin value is helpful in differentiating PCNSL from GBM and IDP. Thus, ADC values may provide a useful supplement to the information obtained from conventional contrast-enhanced magnetic resonance imaging and assist in future treatment planning.

An ulcerated gastric ulcer and pseudotumour with pancreatic affectation associated with immunoglobulin G4-related disease: a case report and literature review.

We report the case of a 67 year old male who presented with a nine year history of a gastric ulcer with symptoms of hematemesis and melena. Histological analysis identified fibrotic lesions and the accumulation of immunoglobulin G4-positive plasma cells with no evidence of malignancy. The lesion extended into the pancreas, where histological lesions and gastric lesions were also observed. This is a case of an ulcerated gastric ulcer and pseudo-tumor with pancreatic affection that is associated with immunoglobulin G4-related disease.

An inflammatory pseudotumor in the thoracic epidural space presenting with progressive paraplegia: a histopathological diagnosis with clinical and radiological uncertainty. Case report with literature review.

INTRODUCTION: Inflammatory pseudotumors (IPTs) are benign lesions with unknown etiology, probably an immunological reaction to a traumatic or an infective insult or sometimes considered as an IgG4-related autoimmune disorder. It can occur as an isolated or multi-centric lesion and are reported to involve almost all parts of the human body. Although lung and orbital IPTs are reported commonly, central nervous system involvement is a rare occurrence. Only seven cases of spinal epidural IPTs have been reported to date. These are clinically and radiologically a diagnosis of exclusion. It is an exclusive histopathological diagnosis. CASE REPORT: We present here a 49-year-old female with 2 months history of progressive weakness in lower limbs, with no history suggestive of any traumatic, infective, inflammatory, or neoplastic pathology. Both clinical and radiological investigations were inconclusive. There was a mass lesion in the epidural space (predominantly in the posterior and right lateral space) at T1-T3 vertebral levels compressing the thoracic spinal cord. Considering the progressive nature of her neurological deficit, an emergency decompressive laminectomies of T1-T3 vertebrae were done with excision of the compressive mass lesion. Histopathological examination showed a rich lympho-plasmacytic cell infiltrates with storiform spindle cells and dense fibrosis, which was diagnostic of IPT. Post-operatively there was a rapid recovery in neurology and she became ambulatory at the end of 2 weeks. The purpose of this case report is to discuss the clinical, histopathological and radiological features, differential diagnosis, management, and prognosis of spinal IPT on the background of relevant literature review.

Hemoperitoneum secondary to perforated inflammatory myofibroblastic tumor: A case report of an unusual complication.

INTRODUCTION: Inflammatory myofibroblastic tumors (IMT) are rare neoplasms characterized by a proliferation of spindle-shaped cells with a stroma infiltrated by macrophages, lymphocytes and plasma cells. CASE REPORT: We report a case of 59 years old male who presented an acute abdomen due to a mass of the mesentery of the terminal ileum, which was perforated with active bleeding. Histopathology reported a low-grade TMI with clear margins. DISCUSSION: Inflammatory myofibroblastic tumors of the mesentery are rare entities whose etio-pathogenia remains unclear. It requires a histopathological diagnosis and inmunohistochemical evaluation and its treatment is based on complete resection of the tumor. These type of neoplasms require close monitoring due to local recurrence.

Gluteal muscle fatty atrophy is not associated with elevated blood metal ions or pseudotumors in patients with a unilateral metal-on-metal hip replacement.

BACKGROUND AND PURPOSE: There are no international guidelines to define adverse reaction to metal debris (ARMD). Muscle fatty atrophy has been reported to be common in patients with failing metal-on-metal (MoM) hip replacements. We assessed whether gluteal muscle fatty atrophy is associated with elevated blood metal ion levels and pseudotumors. PATIENTS AND METHODS: 263 consecutive patients with unilateral ASR XL total hip replacement using a posterior approach and with an unoperated contralateral hip were included in the study. All patients had undergone a standard screening program at our institution, including MRI and blood metal ion measurement. Muscle fatty atrophy was graded as being absent, mild, moderate, or severe in each of the gluteal muscles. RESULTS: The prevalence of moderate-to-severe gluteal muscle atrophy was low (12% for gluteus minimus, 10% for gluteus medius, and 2% for gluteus maximus). Muscle atrophy was neither associated with elevated blood metal ion levels (> 5 ppb) nor with the presence of a clear (solid- or mixed-type) pseudotumor seen in MRI. A combination of moderate-to-severe atrophy in MRI, elevated blood metal ion levels, and MRI-confirmed mixed or solid pseudotumor was rare. Multivariable regression revealed that "preoperative diagnosis other than osteoarthrosis" was the strongest predictor of the presence of fatty atrophy. INTERPRETATION: Gluteal muscle atrophy may be a clinically significant finding with influence on hip muscle strength in patients with MoM hip replacement. However, our results suggest that gluteal muscle atrophy seen in MRI is not associated with either the presence or severity of ARMD, at least not in patients who have been operated on using the posterior approach.

Inflammatory myofibroblastic tumor of the esophagus in childhood: a case report and a review of the literature.

Inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal disease of low malignancy consisting of inflammatory cells inside a mesenchymal stroma comprising myofibroblasts. Biological behavior is variable, recurrence is uncommon, metastatic disease is rare. Treatment strategy is based on the status of low-grade malignant tumor. Radical surgery is considered to be the principal treatment modality, except if it requires a mutilating procedure. Only 6 cases of pediatric patients treated for IMT of the esophagus have been reported in English-language literature. Herein, a case of a 13-year-old boy with an IMT of the esophagus is presented.

Inflammatory myofibroblastic tumours of the urinary bladder: multi-centre 18-year experience.

OBJECTIVE: To review a series of inflammatory myofibroblastic tumours (IMTs) of the urinary bladder in 10 hospitals in Hong Kong. METHODS: A database search in the pathology archives of 10 hospitals in Hong Kong from 1995 to 2013 was performed using the key words 'inflammatory myofibroblastic tumour', 'inflammatory pseudotumour' and 'spindle cell lesion'. Patient characteristics, clinical features, histological features, immunohistochemical staining results and treatment outcomes were reviewed. RESULTS: Nine cases of IMT of the urinary bladder were retrieved. The mean age was 45.4 ± 22.8 years (range 11-78). Eight patients (88.9%) presented with haematuria and 5 patients (55.6%) had anaemia with a mean haemoglobin level of 6.8 ± 1.3 g/dl. Histologically, the majority of patients (77.8%) had a compact spindle cell pattern. Anaplastic lymphoma kinase staining was positive in 75% of cases. During a mean follow-up period of 43.4 months (range 8-94), none of them developed any local recurrence or distant metastasis. CONCLUSIONS: A high index of suspicion of IMT should be maintained for young patients presenting with bleeding bladder tumours and significant anaemia. IMTs of the urinary bladder run a benign disease course, and good prognosis can be achieved after surgical resection.

Simultaneous inflammatory pseudotumors of the coronary arteries and abdominal aorta.

We herein report a rare case of cardiac and abdominal aortic inflammatory pseudotumors (IPTs). A 64-year-old male presented with a loss of appetite, abdominal distension and general fatigue. A cardiac tumor was suspected on the basis of computed tomography scans. A needle biopsy was performed, but it did not lead to a definitive diagnosis. At the same time, a 70-mm abdominal aortic aneurysm (AAA) was also detected. A full sternotomy was performed, and a huge, elastic hard tumor was found around the bilateral coronary arteries, anterior side of the right atria, ascending aorta and pulmonary artery. The pathological diagnosis was IPT, which was judged to be inoperable because of its anatomical location and the fact that the patient was a Jehovah's Witness, which precluded the administration of heterologous blood transfusions. The AAA was surgically treated, and the pathological diagnosis of the aneurysmal tissue also revealed IPT. Perioral steroid therapy was initiated, and the size of the tumor did not change for 1-2 years, but then gradually increased. The patient eventually died 8 years later, and the cause of his sudden death was considered to be heart failure caused by the pressure on the right atrium and ventricle due to the enlarged cardiac tumor.

Inflammatory pseudotumour of the maxillary sinus mimicking malignancy.

Inflammatory pseudotumours of the paranasal sinuses are rare entities and can be easily confused with aggressive malignant tumours. In this report we describe the characteristic behavior of inflammatory pseudotumours in the maxillary sinus. We present a case of a 47-year-old Caucasian woman, who presented in our ENT department because of progressive nasal obstruction, epistaxis, and epiphora. The clinical as well as the radiological findings suggested a maxillary sinus neoplasm, most probably of malignant nature. The histopathological examination showed an inflammatory pseudotumour of the maxillary sinus with polypoid structure and no signs of malignancy.

Peripheral inflammatory pseudotumor of the liver and extensive thrombosis of the portal venous system in a child.

We report a case of a peripheral inflammatory pseudotumor of the right lobe of the liver with extensive thrombosis of the portal venous system in a 9-year-old boy. Local thrombosis of the portal vein is a known complication of the inflammatory pseudotumor, especially in the hepatic hilum. The extent of the thrombosis in this case was unexpected, considering the peripheral location and the benign nature of the lesion. To our knowledge, thrombosis of this degree has not been described.

Budd-Chiari syndrome secondary to inflammatory pseudotumor of the liver: Report of a case with a 10-year follow-up.

Inflammatory pseudotumor (IPT) of the liver is a rare benign tumor of unknown origin, it has the appearance of a malignant tumor but has a benign histology and clinical course. We report a case of a 63-year-old man diagnosed of IPT of the liver and followed for 10 years. During the clinical course, he developed a secondary Budd-Chiari syndrome, with a successful response to a transjugular intrahepatic portosystemic shunt over a 5-year follow-up period.

Inflammatory pseudotumour of urinary bladder - a rare cause of massive macroscopic haematuria.

PURPOSE: Authors analyzed their experience with urinary bladder tumours. This article discusses clinical and histopathological diagnostics and treatment procedures, and follow up of patients with rare benign urinary bladder tumours. METHODS: 406 patients with bladder tumours were treated in our department between January 2000 and December 2008. 322 patients had superficial tumours and 84 had primary invasive tumours. All patients who underwent transurethral resections of these tumours were operated under general or spinal anaesthesia. The resected specimens were histologically examined in the department of Pathology. RESULTS: 399 of the 406 patients had urothelial bladder cancer, 7 patients had a histologically uncommon type of bladder tumour, one female was diagnosed with sarcomatoid bladder cancer, one patient had a histologically confirmed feochromocytoma of the urinary bladder. Two males had epidermoid carcinoma. One female had a histologically described uncommon benign pseudoneoplastic lesion, chararacteristic for endosalpingiosis. Another two patients were diagnosed with inflammatory myofibroblastic tumour of the urinary bladder. Both patients presented with gross macroscopic haematuria. Authors performed complete transurethral tumour resections, which required several sessions and the deliberation of a blocked ureter through nephrostomy in one case. CONCLUSION: More than 98 % of all treated patients had urothelial bladder cancer in different stages and grades. Two patients had rare benign inflammatory proliferation of the bladder wall which formed large tumorous bleeding masses obstructing the ureter in one case. These types of bladder tumour could be treated conservatively with meticulous long term follow up similarly to patients with bladder cancer (Tab. 1, Fig. 4, Ref. 15). .