Inflammatory Pseudotumor Research Articles

A diagnostically challenging case of inflammatory myofibroblastic tumor primary to the peritoneum

IntroductionInflammatory myofibroblastic tumors are difficult to diagnose because of the lack of specific indicators. We describe a diagnostically challenging case of an inflammatory myofibroblastic tumor primary to the peritoneum.Case presentationThe patient was a 25‐year‐old male who presented at our hospital with lower abdominal pain. Computed tomography revealed a mass lesion 80 mm in diameter just above the bladder. This was suspected to be a bleeding tumor of the urachus. Since malignancy could not be ruled out, surgery was planned. This revealed a fragile tumor arising from the peritoneum. Following its removal, the tumor was diagnosed by histopathological analysis as an inflammatory myofibroblastic tumor.ConclusionWe describe a case of inflammatory myofibroblastic tumor primary to the peritoneum diagnosed by histopathology. Inflammatory myofibroblastic tumor should be considered in the differential diagnosis of abdominal wall and anterior bladder tumors.

Tracheal resection anastomosis for rare tracheal inflammatory lesions mimicking malignancy: report of 2 cases

BackgroundTumor-like lesions of the trachea are rare and challenging in diagnosis and management. Inflammatory myofibroblastoma, also known as Inflammatory pseudo tumors (IPTs), as well as Rosai Dorfman Disease (RDD) are inflammatory lesions that may involve the central airways with variable non-specific clinical features mimicking tumors.Case presentationIn this study 2 cases with tumor-like lesions are presented. One case with an inflammatory pseudotumor and the other one with Rosai–Dorfman disease affecting the upper trachea. Both cases were successfully managed with tracheal resection anastomosis.ConclusionTracheal Inflammatory myofibroblastoma, and Rosai–Dorfman diseases are rare tumor like lesions that present with upper airway obstruction. Despite being benign, these lesions may have features suggestive of malignancy, requiring prompt management. Complete surgical excision by segmental resection and primary anastomosis (if feasible) is the treatment of choice with an optimum outcome.

Inflammatory myofibroblastic tumor of the heart in an older woman with paroxysmal atrial fibrillation: a case report and review of the literature

Inflammatory myofibroblastic tumors (IMTs) of the heart are rarely observed in the eldly. We report a case involving an elderly woman with an IMT situated on the right atrial wall. The tumor was fully excised. The patient had a smooth recovery post-surgery and remained free of recurrence for three years.

Inflammatory myofibroblastic tumor directly invading the right first rib treated with oral steroids: a case report

BackgroundWe present a case of an inflammatory myofibroblastic tumor cured with a short period of steroid administration, a treatment previously unreported for such cases.Case presentationA 49-year-old man had a chief complaint of chest pain for more than 3 days. Computed tomography (CT) revealed a tumoral lesion suspected to have infiltrated into the right first rib and intercostal muscles, with changes in lung parenchymal density around the lesion. The maximal standardized uptake value on 18 F-fluorodeoxyglucose positron emission tomography/computed tomography was high (16.73), consistent with tumor presence. CT-guided biopsy revealed an inflammatory myofibroblastic tumor with no distant metastases. Surgery was indicated based on the disease course. However, he had received an oral steroid before the preoperative contrast-enhanced CT scan due to a history of bronchial asthma, and subsequent CT showed that the tumor shrank in size after administration; he has been recurrence-free for more than a year.ConclusionsSurgery is still the first choice for inflammatory myofibroblastic tumors, as the disease can metastasize and relapse; however, this condition can also be cured with a short period of steroid therapy.

Anaplastic Lymphoma Kinase (ALK) in Posterior Cranial Fossa Tumors: A Scoping Review of Diagnostic, Prognostic, and Therapeutic Perspectives

Anaplastic Lymphoma Kinase (ALK) has been implicated in several human cancers. This review aims at mapping the available literature on the involvement of ALK in non-glial tumors localized in the posterior cranial fossa and at identifying diagnostic, prognostic, and therapeutic considerations. Following the PRISMA-ScR guidelines, studies were included if they investigated ALK’s role in primary CNS, non-glial tumors located in the posterior cranial fossa. A total of 210 manuscripts were selected for full-text review and 16 finally met the inclusion criteria. The review included 55 cases of primary, intracranial neoplasms with ALK genetic alterations and/or protein expression, located in the posterior fossa, comprising of medulloblastoma, anaplastic large-cell lymphoma, histiocytosis, inflammatory myofibroblastic tumors, and intracranial myxoid mesenchymal tumors. ALK pathology was investigated via immunohistochemistry or genetic analysis. Several studies provided evidence for potential diagnostic and prognostic value for ALK assessment as well as therapeutic efficacy in its targeting. The available findings on ALK in posterior fossa tumors are limited. Nevertheless, previous findings suggest that ALK assessment is of diagnostic and prognostic value in medulloblastoma (WNT-activated). Interestingly, a substantial proportion of ALK-positive/altered CNS histiocytoses thus far identified have been localized in the posterior fossa. The therapeutic potential of ALK inhibition in histiocytosis warrants further investigation.

Clinical features, treatment strategies, and prognosis of epithelioid inflammatory myofibroblastic sarcoma in children: a multicenter experience.

Inflammatory myofibroblastic tumors (IMTs) are a spectrum of tumors that range in morphology and biological behavior from benign, intermediate, to apparently malignant and epithelioid inflammatory myofibroblastic sarcoma (EIMS) is one of the malignant subtypes. This study tried to provide experience and new ideas for treating this rare disease. This study retrospectively analyzed and followed up 12 children with EIMS admitted to Beijing Children's Hospital, Baoding Children's Hospital, and Children's Hospital of Chongqing Medical University from August 2016 to May 2022. Of the 12 children, 7 were male and 5 were female, with a median age of 74.50 [interquartile range (IQR), 61.50-90.00] months. Of these patients, eight had a single lesion and four had multiple lesions. The maximum diameter of the single tumor foci was 19.30 cm, the full meridian of the multiple tumor foci target lesions was 32.67 cm, and the median maximum tumor size was 11.99 (IQR, 7.80-15.70) cm. The site of disease was the abdominopelvic cavity in eight cases, the thoracic cavity in two cases, the maxillofacial region in one case, and the larynx in one case. The clinical manifestations were predominantly elevated body temperature (n=8). There was one case of ROS1 fusion mutation and nine cases of ALK fusion mutation. Of the 12 children, 6 were biopsied at the initial diagnosis and 6 were surgically treated. Follow-up treatment included preoperative neoadjuvant chemotherapy (n=4), peritoneal thermal perfusion therapy (n=2), targeted therapy (n=3), postoperative chemotherapy (n=5), and radiotherapy (n=3). The follow-up time was 14.50 (IQR, 10.50-31.50) months, with eight cases of tumor-free survival, two cases of death, and two cases of loss of follow-up. EIMS in children is extremely rare and clinically aggressive. The clinical presentation is nonspecific, and the initial diagnosis of the tumor is often large. Mutations in the ALK gene are common in EIMS. Surgery is the mainstay of EIMS treatment, and patients benefit from a multidisciplinary combination that includes targeted therapies, with long-term prognosis remaining subject to ongoing follow-up.

Retroperitoneal Castleman’s disease in a young Nepalese girl: A rare cause of childhood abdominal mass

Introduction: Castleman’s disease (CD), or benign angio-follicular lymph node hyperplasia, is an uncommon condition in childhood. When a child presents with a huge retroperitoneal mass and inconclusive findings on clinico-radiological evaluation or tissue sampling, management becomes exceedingly difficult. CD herein becomes an uncommon presentation of an uncommon diagnosis. Case presentation: A six-year-old girl with no past medical problems presented to the office with a slowly progressive, painless mass over the right lumbar region for a year. Abdominal ultrasound showed a well-defined oval mass in the right periumbilical region, further evaluation of which with a computed tomography scan suggested lymphoma. A preoperative core-cut biopsy could not confirm the findings and suggested a neoplastic lesion, probably an inflammatory myofibroblastic tumour or small round cell tumour. She underwent an exploratory laparotomy with in-toto excision of the mass. Intraoperatively, a solid retroperitoneal tumour measuring 8×8×6 cm was found. Histopathology and immunohistochemistry confirmed a unicentric CD of the hyaline-vascular type. At two years of follow-up, she remained asymptomatic and disease-free. Conclusion: While CD in children is rare, retroperitoneal localization of the same can further add to the diagnostic conundrum. However, if carefully considered, an en-bloc surgical resection offers complete treatment.

Sclerosing angiomatoid nodular transformation of spleen with calcifying fibrous pseudotumor: A rare case with unusual association

Sclerosing angiomatoid nodular transformation (SANT) of the spleen is a rare benign vascular lesion of the spleen that was first described in 2004. SANT is associated with other concurrent diseases, mostly malignancies. Calcifying fibrous pseudotumor (CFPT) is a tumor-like lesion usually arising from soft tissue or peritoneal sub-serosa. Both SANT and CFPT are considered to be variants of the inflammatory myofibroblastic tumor. We report the rare case of a 24-year-old female presenting with a left abdominal mass that was clinically diagnosed as an extraintestinal gastrointestinal stromal tumor (GIST). Histopathological examination revealed SANT of spleen and CFPT. We report this case due to its rarity of occurrence and unusual association of SANT with CFPT.

Gastric and cardiac inflammatory myofibroblastic tumor: an extremely rare case

BackgroundInflammatory myofibroblastic tumor (IMT) is a unique, rarely metastatic tumor composed of myofibroblasts and fibrous spindle cells with inflammatory cell infiltration that can affect any organ in the human body. By reviewing the relevant literature on PubMed, we found that this is the first case report of IMT with both gastric and cardiac involvement.Case presentationA 57-year-old male patient was admitted to the hospital with complaints of malaise, poor appetite, and epigastric pain with black stools. We found a mass in the patient’s stomach and left atrium by contrast-enhanced computed tomography, 18 F-fluorodeoxyglucose positron emission tomography/computed tomography, and other tests. The patient underwent laparoscopic Billroth II subtotal gastrectomy and Braun’s gastrointestinal reconstruction under general anesthesia. On the 46th day following stomach surgery, the cardiac tumor was removed under general anesthesia. The patient has treated with doxorubicin 70 mg of D1 chemotherapy two months after cardiac surgery. Postoperative pathological immunohistochemistry of the mass confirmed the diagnosis of an IMT. His review three months after the cardiac surgery suggested the progression of the left atrial mass, but he declined further treatment and finally died one month after the review.ConclusionsAs a unique class of tumors that rarely metastasize, IMTs have an unknown etiology and pathogenesis, and distant metastasis is primarily observed in patients with negative activin receptor-like kinase (ALK) expression. The preferred treatment for IMT is complete surgical resection, and the effectiveness of adjuvant therapy for patients with distant metastases is still being determined. The clinical presentation of IMT lacks specificity and is often related to the location of tumor growth, which poses a diagnostic challenge. Pathological immunohistochemistry is the only way to confirm the diagnosis at present. Our case report reminds clinicians that a category of ALK-negative IMT with a tendency toward distant metastasis should not be ignored.

Trap-Door Thoracotomy and Clamshell Thoracotomy as Surgical Approaches for Neuroblastoma and Other Thoracic Tumors in Children.

Solid tumors of the cervicothoracic junction, the posterior mediastinum, or bilateral dorsal thoracic tumors represent a challenge in pediatric surgical oncology. The aim of this study was to evaluate trap-door thoracotomy and clamshell thoracotomy as surgical approaches. A single-center retrospective study of children with solid tumors in these specific localizations was performed. From 2015 to 2023, 26 children (17 girls; 9 boys) were treated at a median age of 54 months (range 8-229). Tumor resection was performed for neuroblastoma (n = 11); metastatic disease (n = 7); malignant rhabdoid tumor (n = 4); Ewing sarcoma (n = 1); inflammatory myofibroblastic tumor (n = 1); rhabdomyosarcoma (n = 1); and neurofibroma (n = 1). The surgical goal of macroscopic complete excision was achieved in all of the 14 children who underwent trap-door thoracotomy and in 11 of the 12 children who underwent clamshell thoracotomy. There were no major complications. At a median follow-up of 8 months (range 0-60), the disease was under local control or in complete remission in 66.7% of the children. In conclusion, surgical resection of solid tumors of the cervicothoracic junction in children can be performed safely and successfully with trap-door thoracotomy and with clamshell thoracotomy for posterior mediastinal or bilateral dorsal thoracic tumors.

P-85 Inflammatory myofibroblastic tumor: An extremely rare tumor of the thyroid gland poses challenges in diagnosis and management

Abstract Introduction Inflammatory myofibroblastic tumor (IMT) is an exceedingly rare mesenchymal tumor characterized by myofibroblastic spindle cells and an inflammatory cell infiltrate. The clinical presentation of IMT in the thyroid gland varies widely, ranging from an asymptomatic thyroid nodule to a diffuse, rigid, fixed, and painful mass. While IMT of the thyroid gland is generally considered a benign borderline tumor with very low metastatic potential, its diagnosis and management can be intricate. Surgery remains the preferred treatment, with recurrence being rare following successful removal. Nonetheless, the optimal management of residual tumors is contentious and may involve a second surgery or the use of immunosuppressants and chemotherapeutic agents. Clinical Case A 47-year-old female patient presented with painless discomfort and stiffness around the thyroid gland. A neck ultrasound revealed a hypoechoic nodule measuring 21×12 mm on the left lobe and a hypoisoechoic heterogeneous nodule 35×28 mm in size on the right lobe of the thyroid gland. These nodules displayed low vascularity, and no pathologic lymph nodes were detected. Fine needle aspiration biopsies (FNABs) were performed on the nodules in both lobes at different times, indicating thyroiditis in the right lobe and spindle-shaped cells with atypia of uncertain significance in the left lobe. Subsequent evaluation of FNAB materials at a referral center suggested a high suspicion of lymphoid malignancy. The patient underwent thyroidectomy; however, the surgeon chose to preserve the left thyroid lobe due to its extreme fibrotic and stone-like consistency, thereby preserving recurrent laryngeal nerve function. Histological analysis confirmed the presence of an inflammatory myofibroblastic tumor characterized by spindle cells accompanied by abundant histocytes, plasma cells, and lymphocytes. The tumor cells exhibited positivity for SMA. Postoperative ultrasound revealed a residual tumor approximately 2 cm in size located in the left isthmus and adjacent left lobe, which remained stable over a 6-month follow-up period. Conclusion Our case highlights the potential for misdiagnosing IMT of the thyroid gland as malignant thyroid tumors. Furthermore, the management of a residual tumor presents a complex clinical challenge. The surgical team is reluctant to pursue a second surgery due to potential risks to recurrent laryngeal nerve function. In this session, we aim to solicit expert opinions and engage in interactive discussions regarding the management of the residual tumor in this specific case.

Gastric inflammatory myofibroblastic tumor presented with severe anemia and inflammation: a case report

BackgroundInflammatory myofibroblastic tumor (IMT) is a rare stromal tumor, often found in children and young adults, and most commonly occurs in the lungs. Surgical resection is considered the standard treatment for localized IMT, although only limited data exist. Gastric IMT in adults is extremely rare, and there are no established guidelines for its treatment.Case presentationA 69-year-old male presented with persistent fatigue and weakness. Laboratory examination revealed severe anemia and inflammation. Upper gastrointestinal endoscopy at admission revealed a 40-mm type I softish tumor in the lesser curvature of the gastric body, without apparent hemorrhage. Repeated biopsies, including partial resection with snare, failed to give a definitive diagnosis. Computed tomography (CT) revealed a massive lesion at the gastric body, protruding into the gastric lumen, which was consistent with the gastric tumor. After admission, the patient developed anemia refractory to frequent blood transfusions despite the absence of apparent gastrointestinal bleeding. In addition, the patient had recurrent fevers of 38 °C or higher, and persistent high inflammatory levels. Fluorodeoxyglucose-positron emission tomography (FDG-PET) CT 1 month after the first visit exhibited an increased FDG uptake in the gastric tumor. In addition, this CT scan revealed a rapid increase in tumor size to 75 mm. It was suspected that the undiagnosed gastric tumor caused these serious clinical symptoms, and he underwent distal gastrectomy and cholecystectomy. The gross image of the tumor showed an 80-mm cauliflower-like shape with a gelatinous texture. The histopathological diagnosis was IMT. The postoperative course was uneventful, and the patient’s symptoms subsided drastically, improving both anemia and systemic inflammation. The patient has shown no recurrence or relapse of the symptoms over one and a half years.ConclusionsIn this case, the tumor resection finally enabled the diagnosis of IMT and resolved the clinical symptoms. Despite its predominantly benign morphological nature, some cases of IMT present clinically adverse courses. Surgical treatment may lead to its final diagnosis and improvement of clinical symptoms.

The Value of Amide Proton Transfer MRI in the Diagnosis of Malignant and Benign Urinary Bladder Lesions: Comparison With Diffusion-Weighted Imaging.

Conventional magnetic resonance imaging (MRI) has certain limitations in distinguishing between malignant and benign urinary bladder (UB) lesions. Amide proton transfer (APT) imaging may provide more diagnostic information than diffusion-weighted imaging (DWI) to distinguish between malignant and benign UB. To investigate the potential of APT imaging in the diagnosis of malignant and benign UB lesions and to compare its diagnostic efficacy with that of conventional DWI. Prospective. Eighty patients with UB lesions. A 3.0 T/turbo spin echo (TSE) T1-weighted and T2-weighted imaging, single-shot echo planar DWI, and three-dimensional TSE APT imaging. Patients underwent radical cystectomy or transurethral resection of the bladder lesions within 2 weeks after CT urography and MRI examination. APT signal intensity in UB lesions was quantified by the asymmetric magnetization transfer ratio (MTRasym). MTRasym and apparent diffusion coefficient (ADC) values were measured and compared between malignant and benign UB lesions. Kolmogorov-Smirnov test, Student's t test or Mann-Whitney U test, Spearman rank correlation coefficient, area under the receiver operating characteristic (ROC) curve (AUC), Delong test, and intraclass correlation coefficient (ICC). The significance threshold was set at P < 0.05. Thirty-two patients had pathologically confirmed benign UB lesions, including 2 bladder leiomyomas, 1 submucosal amyloidosis, 1 inflammatory myofibroblastic tumor, and 28 inflammatory lesions, and 48 patients had pathologically confirmed urothelial carcinoma. Urothelial carcinomas showed significantly higher MTRasym values (1.53% [0.74%] vs. 0.85% [0.23%]) and significantly lower ADC values (1.24 ± 0.34 × 10-3 mm2/s vs. 1.43 ± 0.22 × 10-3 mm2/s) than benign UB lesions. The MTRasym value (AUC = 0.928) was significantly better in differentiating urothelial carcinoma from benign UB lesions than the ADC value (AUC = 0.722). APT imaging may have value in discriminating malignant from benign UB lesions and has better diagnostic performance than DWI. 3 TECHNICAL EFFICACY: Stage 2.

P17 Hepatobiliary parasitic infestation: a case report

Abstract Background Hepatic parasitic lesions can present with multiple cystic lesions that may simulate inflammatory pseudo-tumour or malignancy in appropriate clinical settings. Here we present a known case of renal cell carcinoma with liver lesions that showed parasitic infestation on histology. Patient case A 70-year-old male with a history of right partial nephrectomy for clear cell renal cell carcinoma having an uneventful postoperative follow-up for 2 years underwent a surveillance CT scan. Multiple hepatic cystic lesions were identified, which on subsequent MRI and PET scan were suspicious for metastasis. Partial hepatectomy was performed for histological evaluation. Results Multiple discrete cystic hepatic lesions were noted on macroscopy. Histological examination revealed multiple necrotic hyalinized nodules with granulomatous inflammation within the liver and dense neutrophil infiltration with microabscess formation. Within the central necrotic area, there were non-viable parasite eggs and parts of worm, in keeping with parasitic infestation. On further expert opinion, this was diagnosed as Enterobius infestation of liver. Conclusions Hepatobiliary parasitic infestations may be encountered as clinically and biochemically asymptomatic lesions, especially in immunocompromised patients. Strong clinical history of travel and parasite serology screen is always helpful to clinch the diagnosis but may not be always available during histological examination. This case highlights the importance of thorough histological and microbiological examination as all nodular lesions in liver are not malignant and could be infective.

Unravelling the Enigma of Inflammatory Myofibroblastic Tumours: A Cross-sectional Study from a Regional Cancer Centre, Karnataka, India

Introduction: Inflammatory Myofibroblastic Tumour (IMT) is an unusual neoplasm with intermediate malignant potential, which rarely metastasises. IMT occurs in various anatomical sites, and the initial descriptions were primarily centered on its manifestation in the lungs. Although the age range is broad, IMT is most common in the first three decades of life, with a slight female predominance. Recent data and Anaplastic Lymphoma Kinase (ALK) gene aberrations confirm a neoplastic process for these lesions. Aim: To study the clinicopathological features of IMTs in a regional cancer centre in South India. Materials and Methods: A cross-sectional study included 17 cases of IMT over six years, from May 2014 to May 2020, at Memorial Kidwai Memorial Institute of Oncology (KMIO), Bengaluru, Karnataka, India. The clinicopathologic and immunophenotypic features were analysed on needle biopsies and resected specimens. Correlation between the expression of ALK-1 and histological patterns, mimickers, metastasis, and prognosis were described. Results were analysed using Microsoft Excel 2019 and Medcalc calculator. Results: Seventeen cases of IMTs were included in this study, out of which two were in the lung, and the rest were extrapulmonary. Sixteen cases were unifocal on presentation; however, one IMT of extremity showed evidence of secondaries on bone scan. The mean age at presentation was 33.47 years, and the maleto-female ratio was 1:1.12. Among the seventeen cases, sites are as follows: one each in the retroperitoneum, liver, soft-tissue (extremity), two each in the breast, lung, female genital tract, urinary bladder, and three each in the head and neck region (maxilla, trachea, and alveolus) and mesentery. Microscopically, IMTs showed various histological patterns. Immunostaining for ALK-1 was positive in seven out of seventeen cases, and the rest were diagnosed by excluding closer mimickers. Conclusion: The present study provides crucial insights into the clinicopathological features of IMTs. Immunohistochemistry (IHC) is an essential diagnostic tool that helps accurately differentiate IMT from its mimickers by identifying ALK protein expression. This distinction is essential for guiding targeted therapy in recurrent or metastatic cases.

Inflammatory myofibroblastic tumor from molecular diagnostics to current treatment.

Inflammatory myofibroblastic tumor (IMT) is a rare neoplasm with intermediate malignancy characterized by a propensity for recurrence but a low metastatic rate. Diagnostic challenges arise from the diverse pathological presentation, variable symptomatology, and lack of different imaging features. However, IMT is identified by the fusion of the anaplastic lymphoma kinase (ALK) gene, which is present in approximately 70% of cases, with various fusion partners, including ran-binding protein 2 (RANBP2), which allows confirmation of the diagnosis. While surgery is the preferred approach for localized tumors, the optimal long-term treatment for advanced or metastatic disease is difficult to define. Targeted therapies are crucial for achieving sustained response to treatment within the context of genetic alteration in IMT. Crizotinib, an ALK tyrosine kinase inhibitor (TKI), was officially approved by the US Food and Drug Administration (FDA) in 2020 to treat IMT with ALK rearrangement. However, most patients face resistance and disease progression, requiring consideration of sequential treatments. Combining radiotherapy with targeted therapy appears to be beneficial in this indication. Early promising results have also been achieved with immunotherapy, indicating potential for combined therapy approaches. However, defined recommendations are still lacking. This review analyzes the available research on IMT, including genetic disorders and their impact on the course of the disease, data on the latest targeted therapy regimens and the possibility of developing immunotherapy in this indication, as well as summarizing general knowledge about prognostic and predictive factors, also in terms of resistance to systemic therapy.

Lorlatinib for the Treatment of Inflammatory Myofibroblastic Tumor after Allogeneic Hematopoietic Stem Cell Transplantation: A Case Report.

Inflammatory myofibroblastic tumors (IMTs) are rare sarcomas composed of myofibroblastic and fibroblastic cells, accompanied by inflammatory cell infiltration. Many IMTs exhibit clonal rearrangement of anaplastic lymphoma kinase (ALK). We herein report a 56-year-old woman with uterine IMT harboring a thrombospondin-1::ALK fusion that developed after allogeneic hematopoietic stem cell transplantation (allo-HSCT). Laboratory data before systemic therapy indicated increased interleukin-6 and severe leukocytosis. The patient was treated with lorlatinib; however, the response duration was approximately two months. Similar case reports need to be compiled and evaluated to elucidate the efficacy of lorlatinib in post-allo-HSCT IMT with ALK rearrangement.

Inflammatory myofibroblastic tumor: An uncommon pathology in pediatrics

Inflammatory myofibroblastic tumor: An uncommon pathology in pediatrics

Pregnancy Complicated by Inflammatory Myofibroblastic Tumor: A Case Report

Pregnancy Complicated by Inflammatory Myofibroblastic Tumor: A Case Report

INFLAMMATORY RENAL PSEUDOTUMOR IN WEGENER'S GRANULOMATOSIS: INITIAL MANIFESTATION OF A CASE

We report a case of a 37-year-old woman who was treated in the emergency department with low back pain and intermittent cough for 7 days. Computed tomography and magnetic resonance imaging of the abdomen and pelvis revealed an expansive, heterogeneous formation with ill-defined limits, measuring 6.3 x 5.1 cm, in the upper third of the left kidney and a second expansive formation located in the lower pole of the left kidney, measuring 5.1 x 5.9 x 6.4 cm. She then underwent uneventful left videolaparoscopic radical nephrectomy. After the procedure, the patient progressed with prostration, decreased general condition and cough with brownish secretion, in addition to complaining of decreased visual and hearing acuity. She underwent laboratory and imaging tests, revealing bilateral cavitated lung masses that proved to be a case of Wegener's granulomatosis. Treatment with pulse therapy was initiated, but the patient died days later. This is the 11th case described in the scientific literature and serves as a warning to guide the management of this rare clinical condition, especially with regard to the presentation of inflammatory renal pseudotumor.