Dramatic regression of an inflammatory urachal pseudotumor mimicking adenocarcinoma under conservative medical therapy

A previously healthy 15-year-old boy was admitted after 6 months of intermittent fevers, night sweats, and 9 kg weight loss. A systolic murmur was appreciated at the left upper sternal border. Laboratory data revealed elevated white blood cells (11 800/μL), erythrocyte sedimentation rate (75 mm/h), and C-reactive protein (10.5 mg/dL). The results of blood cultures were negative. Imaging demonstrated a hypermetabolic 2×2×4.5 cm pedunculated mass in the right ventricle, suggestive of a neoplasm with overlying thrombus (Figures 1A, 2A and 2B and online-only Data Supplement Movie I). Figure 1. Cardiac magnetic resonance imaging demonstrating the initial IMT originating from the interventricular septum near the right ventricular outflow tract ( A ) and the recurrent IMT near the tricuspid annulus ( B ). IMT indicates inflammatory myofibroblastic tumor. Figure 2. Transthoracic echocardiography before and after resection of the initial inflammatory myofibroblastic tumor. A and B , Preoperative images showed a large echogenic mass in the apex of the right ventricle extending superiorly through the right ventricular outflow tract. C and D , Postoperative echocardiography revealed no evidence of residual tumor. A4C indicates apical 4-chamber view; Ao, aorta; LA, left atrium; LV, left ventricle; PA, pulmonary artery; PSAX, parasternal short axis view; RA, right atrium; RV, right ventricle; RVOT, right ventricular outflow tract; Pre-op, preoperative; and Post-op, postoperative. Arrow denotes the tumor. At surgery, the mass was found to originate from the midportion of the interventricular septum and was covered with thrombus. …

ABSTRACTCONTEXT:Inflammatory myofibroblastic tumors are a rare type of soft-tissue tumor. Inflammatory myofibroblastic tumors are characterized by rearrangements involving the anaplastic lymphoma kinase gene locus on 2p23.CASE REPORT:We report the case of a 67-year-old Chinese male who presented with dysuria and fever. Magnetic resonance imaging showed an irregular prostatic mass with an isointense signal and obscure boundary. Histopathological evaluation showed that the mass consisted mainly of spindle-shaped cells. Immunohistochemical evaluation showed that the tumor cells were negative for anaplastic lymphoma kinase.CONCLUSIONS:Inflammatory myofibroblastic prostate tumors are rare lesions with unclear etiology. The pathological diagnosis is very important.

Inflammatory myofibroblastic tumors (IMTs) are a distinct entity of mesenchymal tumors. We present the challenges in their diagnosis and management. A retrospective study with detailed clinical, radiological, and histopathological (HPE) features along with management and outcome of 10 biopsy-proven patients with IMT, between 2001 and 2020. The location included intracranial (5), orbital (4), and spinal (1) with M:F = 7:3. The mean age of onset was in the third decade. The commonest symptom was headache, while proptosis and blurred vision occurred in orbital IMTs. HPE revealed diffuse infiltration of mixed inflammatory cells over proliferating myofibroblasts. Smooth muscle antigen immunoreactivity was noted in fibroblastic spindle cells of all IMTs. However, we did not find anaplastic lymphoma kinase expression in any of our cases, as this is only found in ~50% of all IMTs. Tumor infiltration into adjacent tissue was noted in 4 patients. Surgical excision was limited to orbital IMTs, as most central nervous system (CNS) tumors were not amenable for resection. Steroid administration showed moderate improvement in the IMT-CNS patients but also required additional immunomodulation. Four patients had a median long-term follow-up of 7 years. Two patients had recurrent lesions demonstrated by imaging after 2 years of initial presentation. IMTs are rare and ambiguous tumors of unknown etiology that can occur anywhere in the body. Clinical and radiological features may not be specific to determine the diagnosis, but it should be considered as a differential diagnosis. Extensive thorough workup with histopathology along with the help of immunohistochemistry is conducive to better clinical outcomes. Surgical biopsy with extensive and total resection of these tumors along with steroid and radiotherapy may enhance the survival outcomes.

Inflammatory myofibroblastic tumor (IMT) is a rare occurrence most commonly affecting the pediatric population. It is considered a diagnostic challenge because of nonspecific clinical symptoms and radiologic presentations. IMT is a soft-tissue tumor that can occur throughout the body but is most commonly found in the lung. This article discusses the case of a 10-year-old girl who presented to our hospital with abnormal chest radiograph. Imaging revealed a heterogenous mass in the superior aspect of the left lower lobe. It was found incidentally when the patient presented to the emergency department. Surgical resection proved a diagnostic and therapeutic measure confirming diagnosis of IMT. The tumor has potential to cause illness either by its rare malignant potential or by mass effect. Because lesions are often asymptomatic with a predilection for pediatric patients, cases describing appropriate management are essential. IMT is a rare tumor of borderline malignant potential. IMT of the lung may invade hilar structures, the mediastinum, and the pleura. Lesions range in diameter from 1.5 to 14 cm. Etiology of the tumor is unclear but may include immune response to infection, trauma, or surgery. Affected patients may present with cough and shortness of breath but are often asymptomatic. Histology is unique but not specific. Immunohistochemistry can differentiate the tumor, by staining for overexpression of the anaplastic lymphoma kinase oncogene. In conclusion, surgical resection is the preferred diagnostic and therapeutic regimen for IMT. The majority of IMTs follow a benign course, but exceptions qualify as low-grade malignancies. As a result, it is important to identify, treat, and maintain vigilance. This case encompasses presentation, diagnosis, and treatment of pulmonary IMT and highlights best practices in addressing IMT for adolescent patients.

PD21-06 INFLAMMATORY MYOFIBROBLASTIC TUMOR OF THE GENITOURINARY TRACT: A SYSTEMATIC REVIEW

Rationale:Both inflammatory myofibroblastic tumor (IMT) and retroperitoneal fibrosis are rare lesions, but kidney involvement is more rare. It is the first study about IMT of the kidney in a patient with perinephric and periureteric fibrosis and we hold that fibroblast proliferation may be an intermediate status in oncogenesis of IMT. But further investigation is necessary in order to better clarify the relationship between fibroblast proliferation and IMT.Patient concerns:A 54-year-old female presented no positive signs except dull back pain after overwork.Diagnoses:On the basis of the urinary ultrasonography and computed tomography (CT) scan, we strongly suspected a renal cell carcinoma.Interventions:Considering the little remaining function of the right kidney and the possibility of malignancy, we performed a laparoscopic right radical nephrectomy.Outcomes:According to the analysis of the postoperative paraffin section and immunohistochemistry assay, a final diagnosis of IMT and retroperitoneal fibrosis nodules was made.Lessons:Both IMTs are rare lesions and its etiology and pathogeny are unclear. It is the first study about IMT of the kidney in a patient with perinephric and periureteric fibrosis. This report suggested that fibroblast proliferation may be an intermediate status in oncogenesis of IMT, but further investigation is necessary in order to better clarify the relationship between fibroblast proliferation and IMT. The preoperative diagnosis of renal IMT remains difficult. Preoperative fine-needle aspiration or percutaneous biopsy and intraoperative frozen section were applied to confirm the diagnosis to avoid unnecessary nephrectomy, especially in patients with renal insufficiency, bilateral masses, or a solitary kidney.

Correspondence

Inflammatory myofibroblastic tumor (IMT) is a rare mass-forming lesion characterized by fibroblastic or myofibroblastic spindle cell proliferation with varying degrees of inflammatory cell infiltration. Although it has been reported in virtually every organ in the body, the lung is the most common site of involvement. Extrapulmonary IMTs, although rare, have been reported and are characterized by different, more aggressive behavior. We report an extremely rare case of maxillary metastases of pulmonary IMT. Lung IMT was initially misdiagnosed, and oral lesion mimicked clinically and radiologically a radicular cyst. On histologic examination, cells exhibited diffuse and intense immunoreactivity for α-smooth muscle actin and vimentin whereas both pulmonary and oral IMTs presented absence of cellular atypia and lack of expressivity of oncogenic determinants. Distant metastases of lung IMT are extremely unusual, and this is the first report to our knowledge with this particular clinical course. Despite the possibility that the present case could also represent a metachronous multifocal IMT, with pulmonary and extrapulmonary lesions, similar histopathological and immunohistochemical patterns in lung and maxillary region suggest a metastatic course.

ALK negative inflammatory myofibroblastic tumor of the gallbladder origin as polyp: a rare case report

ObjectivesThe etiology of Inflammatory myofibroblastic tumor(IMT) is contentious. In this study, we used computed tomography (CT) to examine tonsillar IMT and further analyzed the etiology of this entity.MethodologyWe presented CT features of left tonsillar IMT and reviewed the English-language literature published between 1984 and 2011.ResultsTo our knowledge, there are only six published cases of tonsillar IMT including the present case. Two patients were asymptomatic at initial presentation. Two patients were taking immunosuppressants, and one was pregnant and in an immunomodulated state. CT of our patient revealed a 2.6 × 1.8 cm irregular soft tissue mass between the left tonsil and the base of the tongue. It did not invade surrounding structures and was not enhanced on contrast-enhanced imaging.ConclusionsTonsillar IMT may be a benign tumor. We suggest that preoperative recognition of tonsillar IMT by CT may be important to avoid unnecessary expanded surgery.

The rationale for nonsteroidal anti-inflammatory drug therapy for inflammatory myofibroblastic tumors: a Children's Oncology Group study

Although rare, extra-pulmonary inflammatory myofibroblastic tumors (IMTs) are becoming increasingly recognized. While surgical resection is currently an effective and accepted treatment for IMTs, the optimal management of unresectable or residual IMTs remains a clinical dilemma. We present the case of an incompletely resected IMT treated successfully with anti-inflammatory therapy alone, and describe the rationale for this approach.

Inflammatory pseudo tumor (IPT) is a rare benign lesion that can occur in any organ in the body. IPT is histologically characterized by the presence of inflammatory cells, especially, plasma cells, spindle-shaped cells, and myofibroblasts.Many cases of IPTs affecting multiple organs in both adults and children have been documented in the literature. Fifty-five cases of hepatic IPTs have been reported in children, and all of them were managed by surgical resection. Limited data are available on IPTs in Arabs.Our aim was to report the case of an 8-year-old Saudi boy who was referred to our hospital with fever of unknown origin since 3 months with associated weight loss and a hepatic mass, and was found to have an IPT of the liver, which was confirmed after surgical resection.8 years old Saudi boy who presented with fever and liver mass. Ultrasound and MRI abdomen showed heterogeneous liver mass. After surgical resection, his mass histology going with inflammatory pseudo tumor. Following surgical resection his fever subsided.The findings of the present case report show that fever and liver mass in children can be manifestations of a rare disease such as IPT, which should be considered in the differential diagnosis when all investigations are inconclusive. Based on the literature review, surgical excision seems to be the best treatment strategy for this condition. However, the imaging findings, especially the size and location of the mass, must be carefully discussed with the surgical team before the operation.

1. Aditi Bagchi, MD* 2. Ashraf Elnawawi, MD† 3. Swayam Sadanandan, MD‡ 1. *Resident (PGY-3), Pediatrics, The Brooklyn Hospital Center, Brooklyn, NY. 2. †Attending Physician, Department of Pathology, The Brooklyn Hospital Center, Brooklyn, NY. 3. ‡Associate Chairperson, Department of Pediatrics, Chief, Division of Pediatric Hematology and Oncology, The Brooklyn Hospital Center, Brooklyn, NY. * Abbreviations: CFP: : calcifying fibrous pseudotumor IMT: : inflammatory myofibroblastic tumor A 3½-year-old boy with a history of sickle β-thalassemia presents to the hematology/oncology clinic for evaluation of a mass in his left groin. The child’s mother had noted a small lump in his left inguinal region 2 years ago. At that time, the pediatrician recommended observing the lump and monitoring any progression. Subsequently, the family moved out of town and did not seek further medical attention until now. Two weeks ago, his mother thought the mass had grown in size and brought him to a nearby hospital for evaluation. He was prescribed a 5-day course of oral antibiotics for a presumed local infection. However, there was no significant change in the size of the mass; so his mother brought him to the hematology/oncology clinic. There is no history of groin pain or erythema, abdominal pain, fever, weight loss, or change in bowel or bladder habits. There is no history of trauma. The child was born at term after an uncomplicated pregnancy and delivery, but newborn screening indicated sickle cell disease. On further evaluation, his mother was found to have β-thalassemia trait and his father had sickle cell trait. The patient was diagnosed as having sickle β-thalassemia and placed on penicillin prophylaxis and folic acid supplementation. Since then, he has had four hospitalizations for febrile illnesses but no history of documented bacteremia. He has not had any vaso-occlusive crises. His motor milestones are appropriate for age, with the exception of speech delay. On physical examination, the child is well nourished …

The inflammatory myofibroblastic tumor (IMT) is a very rare lesion with an incidence of less than 0.1% of total neoplasms and with main affection in the lungs. Involvement in the central nervous system is extremely rare, but with a much more aggressive course than IMT diagnosed in the rest of the body. We report the 2 cases presented in our neurosurgery department to date; both were treated satisfactorily without intercurrences in 10years of follow-up. The World Health Organization described the IMT as a distinctive lesion composed of myofibroblastic spindle cells accompanied by an inflammatory infiltrate of plasma cells, lymphocytes, and eosinophils. Clinical manifestations of patients with CNS IMT vary and may consist of headache, vomiting, seizures, and blindness. Seizures are the most common symptom in patients with focal lesions. The true origin of this entity remains to be elucidated, but to date, etiologies ranging from chromosomal alterations to autoimmune or postinfectious mechanisms have been described. Due to its rarity and non-specificity in imaging, the final diagnosis of IMT in the brain parenchyma relies on pathological examination. Treatment options are controversial and include total or subtotal removal, high-dose steroids, and radiation therapy. In the last decade, the development of ALK Tyrosine Kinase Inhibitors allows the possibility of chemotherapy in those patients harboring ALK mutations. IMT is a rare tumor that can exceptionally be found in the CNS. The cause is still unknown although the different studies focus on a neoplastic origin. The diagnosis is based in the use of different modalities of imaging and with histological confirmation. Optimal management is gross total resection whenever possible, is the only established curative treatment. Further research with longer follow-up is needed to clarify the natural history of this rare tumor.