Cardiac Inflammatory Pseudotumor: Rapid Appearance in an Infant with Congenital Heart Disease

The rapid growth of an inflammatory pseudotumor in the right atrium of an infant with pulmonary valve stenosis and atrial septal defect is documented. Systemic manifestations, including fever, weight loss, leukocytosis, and hypergammaglobulinemia, suggested infection; the diagnosis was made after surgical resection. Sixteen months after surgery the infant is asymptomatic, and the hypergammaglobulinemia and leukocytosis have resolved. Trauma may have been the inciting factor for this inflammatory pseudotumor.     

Mesenteric inflammatory pseudotumor: unusual presentation with leukemoid reaction and massive calcified mass

The authors describe a child with an unusual presentation of mesenteric inflammatory pseudotumor in association with leukemoid reaction. An 11-year-old-boy admitted with short stature was found to have an abdominal mass localized in the right lower quadrant. The leukocyte count was 92,000/mm3 with neutrophilic leukemoid reaction. Abdominal ultrasonography and computed tomography revealed a massive calcified mass in the pelvis. Total resection of the mass was performed and the pathologic diagnosis of inflammatory pseudotumor of the mesentery was made. Leukemoid reaction dramatically resolved within a few days after surgical resection. Physicians should be aware of the association of inflammatory pseudotumor, leukemoid reaction, and massive calcification.     

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.     

Metachronous Pulmonary and Cerebral Inflammatory Pseudotumor in a child

An 8-year-old boy had a right pneumonectomy performed for a large inflammatory pseudotumor. Over the subsequent 8 years he developed multiple similar lesions in the meninges and bilateral cerebral hemispheres as well as new growths within the ipsilateral pleural cavity and contralateral lung. Metachronous pulmonary and intracranial inflammatory pseudotumor has been reported only once. These lesions probably represent a multifocal, exaggerated inflammatory response to some as yet unidentified stimuli.     

Inflammatory pseudotumor of the lung manifesting as a posterior mediastinal mass

A case of inflammatory pseudotumor of the lung in a 6-year-old boy is presented. The respiratory illness presented as a mycoplasma pneumonia and there had been a similar episode of mycoplasma pneumonia one and a half years previously. Computed tomography revealed a large, calcified right posterior mediastinal mass and exploratory thoracotomy revealed a large posterior mediastinal mass that had an endobronchial component and grew exophytically from the lung. The present case suggests that inflammatory pseudotumor of the lung can manifest as a mediastinal mass and could be associated with mycoplasma pneumonia infection.     

Association of inflammatory pseudotumor of the liver and Papillon-Lefevre syndrome--case report.

A case of hepatic inflammatory pseudotumor mimicking malignancy in a 4-year-old girl with the Papillon-Lefevre syndrome (PLS) is reported. Only recently, an association between this inherited syndrome and liver abscesses has been found. Its possible pathogenesis is discussed and immunologic defects resulting from the Papillon-Lefevre syndrome are presented. The development of inflammatory pseudotumor of the liver might be caused by immunologic disturbances and staphylococcal infection. The picture of the hepatic tumor on imaging in patients with PLS should be attributed rather to inflammatory than neoplastic process.     

Unilateral pseudotumor of the orbit--an autoimmune disease?

An eleven-year-old boy with congenital paresis of the left superior rectus eye muscle developed symptoms of acute left orbital expansion: pain, ptosis, redness, extraocular muscle dysfunction. Computer assisted tomography of the orbit showed typical signs of inflammatory orbital pseudotumor and immunological screening cryoglobulinemia. Short-term treatment with prednisone (2 mg/kg bodyweight/day) rapidly improved symptoms. However several relapses occurred following discontinuation of therapy. Therefore a long-term treatment with corticosteroids was instituted. The simultaneous development of inflammatory orbital pseudotumor and cryoglobulinemia support the hypothesis that orbital pseudotumor is an autoimmune disease. The meaning of the congenital superior rectus muscle paresis in this case for the development of the inflammatory process remains uncertain.     

Wegener's granulomatosis presenting as orbital pseudotumor in children

The case of a nine-year-old white female who presented initially with idiopathic inflammatory pseudotumor and was ultimately diagnosed (22 months later) as having Wegener's granulomatosis is presented. A review of the literature reveals that, to date, there are 21 well-documented cases of Wegener's granulomatosis occurring in children under the age of 16. Of these 21 cases, eight patients (39%) manifested ocular or orbital signs during the course of the disease. Four of these cases (50%) presented initially with idiopathic inflammatory pseudotumor. The diagnosis of Wegener's granulomatosis should be considered in children with orbital pseudotumor. Long-term follow-up may be necessary to exclude this possibility.     

Inflammatory pseudotumor of the lung — fibrous histiocytoma type

A 7 1/2-year-old girl with an inflammatory pseudotumor of the lung is presented. The nature of the lesion was not recognized pre-or intraoperatively. The lesion was rubbery, yellowish, and well-defined but not encapsulated. Histologically, a spindle-cell lesion with a storiform pattern and chronic inflammatory-cell infiltrate was seen. The immunological and ultrastructural studies supported an inflammatory origin. The lesion corresponds to the fibrous histiocytoma variant of inflammatory pseudotumor of the lung, as defined recently by Matsubara et al. [17]. This must be distinguished from rare benign neoplasms of the lung such as benign fibrous histiocytoma, leiomyoma, Schwannoma, and histiocytosis.     

Clostridium difficile colitis associated with inflammatory pseudotumor in a liver transplant recipient

The aim of this report is to describe a rare complication of clostridium difficile (CD) disease, the occurrence of an inflammatory pseudotumor that caused intestinal obstruction in a liver transplant recipient. A 9-month-old girl underwent liver transplantation for biliary atresia. She was given tacrolimus as primary immunosuppressive therapy. Three months after liver transplantation, she presented with febrile protracted bloody diarrhea and failure to thrive. A diagnosis of post-transplant lymphoproliferative disease associated with Epstein-Barr virus infection was initially made on histological examination of duodenal biopsies. Tacrolimus was discontinued. Despite treatment with anti-CD20 monoclonal antibodies the child's condition deteriorated and she presented with intestinal occlusion. A mass at the ascending colon was seen on the computed tomography scan mimicking lymphoma and the child underwent surgical laparotomy. Histological examination of the mass showed typical pathological lesions of inflammatory pseudotumor and CD pseudomembranous colitis. Diagnosis of CD disease was confirmed upon the identification of CD toxin A in stools. She was successfully treated by metronidazole and gamma-globulin perfusions. Delayed diagnosis and anti-CD20 monoclonal antibodies therapy (associated with hypogammaglobulinemia) possibly played a major role in the severity of CD pseudomembranous colitis and in the occurrence of an inflammatory pseudotumor.     

Pulmonary blastoma

A three-and-half-year-old boy presented, with recurrent chest infections, fever and weight loss of three month duration not responding to antibiotics. The chest X-ray and CT scan revealed a large well-circumscribed mass in right upper thorax with collapse of right upper lobe. A preoperative diagnosis could not be made even after fine needle aspiration cytology. Thoracotomy and right upper lobectomy was done and the biopsy report was an inflammatory pseudotumor. The child remained well for three months after which his symptoms and the mass recurred. The histopathology slides were reviewed and revealed a biphasic malignant tumor suggestive of Pulmonary Blastoma (PB). Patient received four cycles of chemotherapy followed by re-exploration. The recurrent tumour could only be excised partly and the child succumbed to persistent shock postoperatively. The final histopathological diagnosis was confirmed as PB. Primary pulmonary neoplasms in children are rare and of these PB which is even rarer, constitutes less than 15%. The report highlights that the lack of familliarity with this entity still causes error in the diagnosis of PB.     

Rare Pediatric Cardiac Tumor Presentation

A case of cardiac inflammatory pseudotumor with a unique presentation is reported. This especially rare case belongs to a subset of pseudotumor caused by an infectious etiology. Furthermore, it is unique in that the patient experienced unexplained sinus tachycardia, which resolved with resection of the tumor. This report also emphasizes the importance of multiple imaging methods combined with the usefulness of surgery for treatment and diagnosis.     

Pancreatic pseudotumor in an 11-year-old child: imaging findings

Background. An inflammatory pseudotumor is a benign, solid lesion of unclear etiology. Some authors believe it is a true neoplasm, while others consider it a post-infectious or post-traumatic process. It is most commonly found in the lung; an inflammatory pseudotumor of the pancreas is rare. This case report is the sixth of a pancreatic pseudotumor in a child.¶Patients and methods. An 11-year-old girl presented with obstructive jaundice due to a mass in the head of the pancreas. The mass was identified by sonography. This was confirmed by MRI and CT. The mass enhanced with gadolinium, but its enhancement at CT was similar to the remainder of the pancreas.¶Results. At operation, a pancreatic inflammatory pseudotumor was totally resected.     

Inflammatory pseudotumor of the omentum

Inflammatory pseudotumor is an unusual quasineoplastic lesion, of uncertain natural history and etiopathogenesis, which consists of inflammatory cells and myofibroblastic spindle cells; and is usually found in children and young adults. Clinical manifestations and radiological feature of this rare entity, can be indistinguishable from a malignant sarcomatous or lymphoproliferative disorders. Here, a case of surgically proven inflammatory pseudotumor of the omentum in a child and its review of literature is presented. Conservative surgery is curative in most cases of inflammatory pseudotumors. Awareness of this lesion is essential for clinicians, radiologists and pathologists, as pre-operative recognition may spare the child from unnecessary radical surgery or harmful chemoradiotherapy.     

Intraosseous hemophilic pseudotumor of the orbit

Intraosseous hemophilic pseudotumor involving the orbit has not been described previously. A 14-year-old white male presented with a rapidly progressive, expansile right orbital mass consisting of hematoma contained within bony cortex. Computerized tomography revealed findings consistent with intraosseous hemophilic pseudotumor. The patient responded to conservative therapy of factor VIII replacement.     

Pulmonary inflammatory pseudotumor with esophageal obstruction

A 6-year-old boy with an unusually agressive pulmonary inflammatory pseudotumor is presented. The tumor invaded the mediastinum and resulted in an esophageal obstruction, initially necessitating a pneumonectomy, then radiation therapy, and eventually colonic substitution of the esophagus. The previously reported 8 cases of pulmonary inflammatory pseudotumors with involvement of the mediastinum are reviewed. Offprint requests to: M. S. Slim at the above address     

Parapharyngeal Inflammatory Pseudotumor Presenting as Fever of Unknown Origin in a 3-Year-Old Girl

A parapharyngeal inflammatory pseudotumor in a 3-year-old girl who presented with high fever, anemia, and weight loss is described. The histologic differentiation from other fibroblastic lesions occurring in the head and neck region of a child is discussed. Symptoms improved dramatically after removal of the mass. The lesion may represent an exaggerated local response to an as yet unidentified agent with concomitant systemic manifestations.     

Aggressive manifestations of inflammatory pulmonary pseudotumor in children

We present three cases that illustrate the locally invasive radiographic appearance that inflammatory pulmonary pseudotumor can assume. Awareness and inclusion of inflammatory pseudotumor in the differential diagnosis of aggressive pleuropulmonary and mediastinal processes may have critical treatment implications. Received: 5 February 1998 Accepted: 29 June 1998     

Extraperitoneal abdominopelvic inflammatory pseudotumor: report of four cases

We describe four cases of inflammatory pseudotumor seen at our institution in the past 4 years. Four children were each found to have a large extraperitoneal mass on imaging studies, three of which were in the pelvis. Malignant sarcomatous tumors were suspected. Surgical biopsy of each mass, however, revealed inflammatory pseudotumor. Received: 6 October 1995 Accepted: 7 June 1996