Paratesticular fibrous pseudotumour: a rare cause of an intrascrotal mass.

This report describes a boy in his early adolescence who was referred to a urologist with a large, painless right scrotal mass. Following a thorough workup, the patient underwent surgical removal of the mass, which was revealed to be a paratesticular fibrous pseudotumour (PFP) on histopathological analysis. This diagnosis is rare and can often prove difficult to distinguish from a malignant lesion within the scrotum. We have conducted a review of the current literature surrounding PFP to compliment the case discussion.

Thoracic Epidural Chronic Inflammatory Lesion: A Case Report and Review of Literature.

Chronic inflammatory lesions (CIL) in the body are rare and inflammatory pseudotumor (IPT) is a subtype of CIL. Spine is an uncommon location of CIL, with most cases reported in the thoracic spine. Our objective was to review the current literature on IPT. To the best of our knowledge only about 13 cases are reported till date. A retrospective review of 13 existing cases was done. The etiopathogenesis of CIL is uncertain and total surgical excision remains the mainstay. It's a diagnosis of exclusion after ruling out commonly found lesions, both clinically and radiologically. Only histopathology can confirm the diagnosis. Total surgical resection remains the mainstay of treatment with adjuvant treatment only if there is a residue or a recurrence. CILs are rare lesions in the body with IPT being the commonest. Surgical excision remains the mainstay of treatment and a long-term follow up is warranted.

Inflammatory myofibroblastic tumours of the liver - a systematic review.

BACKGROUND: Hepatic inflammatory myofibroblastic tumours (HIMTs) are rare and poorly described in the literature. Most publications are single patient case reports and lack detailed reporting on characteristics, management, and outcomes. This systematic review aimed to assess the demography, clinical presentation, typical imaging features, histopathology, treatment, and outcomes of patients presenting with HIMTs. METHODS: A systematic literature search was performed in MEDLINE (PubMed), EMBASE (Scopus), JSTOR, Cochrane CENTRAL (Cochrane Library), and the databases included in the Web of Science for studies published between 1940 and 2023 on HIMTs, including its reported synonyms. Case series or cohort studies that reported on the management and outcomes of at least four patients with histologically confirmed HIMTs were included in the analysis. RESULTS: After screening 4553 publications, 22 articles including a total of 440 patients with confirmed HIMTs were eligible for inclusion. The average age was 53.4 years (range 42.0-65.0) with a male to female ratio of 1.7:1. Abdominal pain, discomfort, fever, and loss of weight were the most common presenting symptoms. Surgical resection is the standard of care for HIMTs and is associated with low mortality of 3.4% and low disease recurrence. CONCLUSION: HIMT is a disease more often affecting middle-aged males. The lesions are typically solitary with low recurrence after treatment. The relative roles of surgical versus medical treatment remain unclear. Differences in clinical presentation, histopathology, and treatment of HIMTs compared to inflammatory myofibroblastic tumour (IMT) at extrahepatic sites could challenge the current view of IMT as a single pathological entity.

Treatment and outcomes in pediatric inflammatory myofibroblastic tumors - A systematic review of published studies.

Inflammatory myofibroblastic tumor (IMT) is a soft tissue neoplasm which can be locally invasive, recur, or in rare cases metastasize. Often originating from the abdomen or thorax, IMT most commonly affects children and young adults. Due to its rarity comprehensive reports detailing clinical management and outcome(s) are sparse and often based on limited index case numbers. This study systematically analyzes outcome metrics of pediatric IMT and identifies risk factors for mortality. Medline/Embase databases were searched in accordance with PRISMA guidelines. Final analysis included 57 studies with 673 IMT patients (355 males, 53 %). Individual patient data was available for 405 cases with a median follow-up period of 36 months. Tumor sites included abdomen/pelvis (n = 233, 58 %), thorax (n = 125, 31 %), head/neck (n = 34, 8 %), and extremities (n = 13, 3 %). Surgical tumor resection was the mainstay of treatment, while only 20 patients (5 %) were treated non-operatively. Recurrence(s) were reported in 80 patients (20 %) with 34 (12 %) requiring reoperation. Positive tumor margins were a significant risk factor for tumor recurrence (p < 0.0001). Chemo/radiotherapy was reported in 98 patients (25 %). Most patients (94 %) survived; 81 % (n = 237) with no evidence of recurrent disease, 14 % (n = 41) were alive with disease, and 25 (6 %) died of disease. Positive margins at primary operation, and metastatic disease were associated with mortality (p < 0.0001 for both). IMT is a rare tumor with favorable outcome for the majority of patients. Whilst most patients will present with benign tumors, complete surgical resection (R0) is crucial, as positive surgical margins are a significant risk factor for tumor recurrence and mortality.

EBER-negative inflammatory pseudotumor-like follicular dendritic cell sarcoma of liver: A case report.

RATIONALE: Inflammatory pseudotumor-like follicular dendritic cell sarcoma (IPT-like FDCS) of the liver is rare. It was previously believed that Epstein-Barr virus (EBV) positivity was a necessary criterion for pathological diagnosis. However, we found that there were also cases of EBV negativity. Therefore, clinicians and pathologists are reminded that EBV positivity is not a necessary condition for diagnosis. PATIENT CONCERNS: A 70-year-old female underwent computed tomography (CT) examination for upper abdominal discomfort, which revealed the presence of a liver tumor. Follow-up revealed that the tumor had progressively increased in size. DIAGNOSIS: The final diagnosis was an IPT-like follicular cell sarcoma, based on CT, MRI, HE staining, and immunohistochemical staining. INTERVENTIONS: The patient underwent a laparoscopic left hemihepatectomy. OUTCOMES: The patient has not undergone any special treatment, such as radiotherapy and chemotherapy, and has been followed up for over 3 years without experiencing any recurrence. LESSONS: IPT-like FDCS is a rare tumor that lacks definitive criteria, and its diagnosis mainly relies on pathological findings. Previously, it was believed that being EBV-positive was an important condition for diagnosis. Primary IPT-like FDCS in the liver is even rarer, and the patient in this case tested negative for EBV. It may be necessary for pathologists to consider the role of EBV in the diagnosis of IPT-like FDCS.

A rare inflammatory myofibroblastic tumor appearing both inside and outside the heart.

BACKGROUND: Inflammatory myofibroblastic tumor (IMT) is an uncommon cardiac tumor that primarily affects infants, children, and young adults. While complete surgical resection generally leads to a favorable prognosis, accurate diagnostic tests remain limited. CASE PRESENTATION: We describe the case of a 26-year-old female who had a dual tumor inside and outside the heart and was misdiagnosed by echocardiography and MRI. We also review 71 cases of cardiac IMTs from the literature regarding their epidemiology, clinical presentation, and outcome. CONCLUSION: Early detection of this rare disorder is essential for optimal surgical management.

Inflammatory myofibroblastic disease of right petrous apex: A rare case with review of literature.

ABSTRACT: Inflammatory myofibroblastic tumor (IMFT) is a rare tumor of unknown etiology. It can involve any part of the body. The IMFT involving the base of skull is rare with only 36 cases reported in the literature. We report a rare case of IMFT of temporal bone with review of literature. A 42 year old male presented with complaints of headache and double vision and MRI brain showed lesion in the right petrous apex region suggestive of a neurogenic mass. He had excision of lesion and histopathology was suggestive of IMFT with IgG4 and ALK positive. He had complete clinical response but a month later he presented with right eyelid ptosis and decreased rotation of eye medially with recurrent lesion on MRI. Patient received radiation by SRT technique and then started on Ceretinib with partial response. The IMFT is rare tumor of unknown etiology and tumors of temporal bone are more aggressive. It is benign but locally invasive tumor. Treatment of IMFT is controversial. Extensive surgery with complete excision has about 80% response rates and with intracranial extension, adjuvant radiation is need. In head and neck IMFT response rates are lower (30 to 40%). Monoclonal antibodies and steroids are used in IMFT at recurrence. In advanced or metastatic ALK positive tumors, Crizotinib is used with a response rate of 50%. Radiotherapy (25 to 30 Gy) induces remission and helps to taper the steroids. Temporal bone IMFT is a rare tumor with multimodality approach and variable response to treatment.

Ocular inflammatory myofibroblastic tumor in a pediatric patient.

Inflammatory myofibroblastic tumor (IMT) is a mesenchymal neoplasm that rarely metastasizes and is more commonly seen in children, adolescents, and young adults than older adults. These tumors, composed of myofibroblasts and inflammatory cells, are often confused for a local infection due to the inflammatory cell infiltration, and they form in mucosal surfaces but rarely arise in the orbit. We present the case of a 6-year-old girl with excisional biopsy-confirmed conjunctival stromal IMT. There was no evidence of recurrence 2 years following resection with no subsequent medical therapy.

A surgical case of inflammatory pseudotumor by hepatic anisakiasis.

Anisakiasis is a parasitic infection caused by the ingestion of raw or undercooked seafood infected with Anisakis larvae. It generally affects the gastrointestinal tract, particularly the stomach, but very rare cases have been reported in which infection of the liver leads to the formation of inflammatory pseudotumors. We herein report an extremely rare case of an inflammatory pseudotumor induced by hepatic anisakiasis that was laparoscopically resected for the purpose of both diagnosis and treatment. A 51-year-old woman underwent a routine medical checkup by ultrasound examination, which incidentally detected a 15-mm mass on the surface of S6 of the liver. Because a malignant tumor could not be ruled out on several preoperative imaging studies, laparoscopic partial resection of the liver was performed. Histopathological examination revealed Anisakis larva in the inflammatory pseudotumor, suggesting hepatic anisakiasis. This report describes an extremely rare case of an inflammatory pseudotumor induced by hepatic anisakiasis. Because the preoperative diagnosis could not be obtained by several imaging modalities, laparoscopic liver resection with a sufficient margin might be suitable for diagnosis and treatment of this disease.

Crizotinib combined with bronchoscopic interventional treatment in ALK-positive inflammatory myofibroblastic tumor of left main stem bronchus: a case report.

BACKGROUND: Inflammatory myofibroblastic tumor (IMT), also known as an inflammatory pseudotumor, is a unique type of intermediate soft tissue tumor that commonly occurred in the lung. Its unclear etiology and cellular activity brought about the confusion not only in naming of it, but also in diagnosis and treatment. CASE PRESENTATION: We reported the case of an 18-year-old male student who suffered from shortness of breath, chest tightness and chest pain. Chest computed tomography scan showed a spherical neoplasm blocking left main stem bronchus. After fiberoptic bronchoscopy procedure, the results of histopathological and immunohistochemical analysis indicated an IMT. The targeted next generation sequencing based genomic profiling of the tumor using formalin-fixed and paraffin embedded tissue was performed and a EML4-ALK fusion was detected. The patient began to receive Crizotinib, a ALK tyrosine kinase inhibitor, at a dose of 250 mg twice daily orally. The patient has recovered well after the operation, and no recurrence or metastasis has been found after 12 months' follow-up. CONCLUSION: By means of the diagnosis and treatment of this case, the characteristics and therapies of IMT are illustrated. In addition, it also provides a reference for the therapeutic strategy of IMT in the future.

Infantile inflammatory myofibroblastic tumour of the sigmoid colon: a diagnostic dilemma.

An inflammatory myofibroblastic tumour (IMT) is an uncommon neoplasm composed of inflammatory cells and myofibroblasts in a fibrous stroma. They are mostly seen in the lungs and rarely involve the gastrointestinal tract. An 8-month-old infant presented with a history of lower abdominal lump for 2 months. Her CT scan confirmed a large, lobulated mass in the retroperitoneum arising from the pelvis. The mass was found to be arising from the sigmoid colon on laparotomy which was excised. Histopathology showed a cellular tumour composed of spindle cells and inflammatory lymphocytic infiltrate. Immunohistochemistry revealed positive staining for anaplastic lymphoma kinase and smooth muscle actin, confirming the diagnosis of IMT. The patient is doing well at her 6-month follow-up. Ours is the youngest case of sigmoid IMT among the only other series of eight cases reported in the literature indicating its rarity.

Management Strategies of Plasma Cell Granuloma Involving the Central Nervous System: A Systematic Review of the Literature.

BACKGROUND: Plasma cell granuloma (PCG) is a rare clinical entity seen in the neurosurgical literature. It has often been referred to as inflammatory myofibroblastic tumor or inflammatory pseudotumor. No well-defined management guidelines exist in the literature. METHODS: Using PRISMA guidelines, we systematically reviewed the literature in PubMed and Google Scholar using MeSH terms: intracranial plasma cell granuloma, myofibroblastic tumor, intracranial pseudotumor, spinal plasma cell granuloma. We analyzed the clinical presentation, treatment strategies, clinical outcomes, and follow-up across different studies. RESULTS: Eighty-three studies were included presenting 108 cases. Primary extracranial disease was seen in 4 patients and primary central nervous system (CNS) disease in 104. In the combined cohort, multicompartmental disease was seen in 22 (20.8%) patients. Headache (n=40, 42.59%) was the most common clinical symptom. Surgical excision (n=86, 79.6%) was the most common primary treatment used. Radiation therapy, steroids, and chemotherapy (methotrexate/6-mercaptopurine/rituximab) were also used. Disease recurrence was noted in 25 (33.3%) patients and residual disease in 33 (30.5%). Mortality was seen in 4 (3.7%) patients. In the cranial PCG subgroup (n=87), 81 (93.1%) patients had solitary lesions, and 6 (6.8%) had multiple lesions. Recurrence after primary surgery was noted in 27.58% (n=24). In the spinal PCG subgroup (n=17), the thoracic spine was the most common location (n=9, 52.9%) and recurrence was seen in 5.84% (n=1). CONCLUSIONS: Combination of multiple treatment modalities is needed when approaching this complex disease. Spinal PCGs respond favorably to gross total excision, with a low recurrence rate. Cranial PCGs warrant intense follow-up with secondary chemotherapy/radiation/steroids in recurrent cases.

[A Case of Inflammatory Myofibroblastic Tumor of The Bladder Treated with Partial Cystectomy].

A 60-year-old woman with extensive hematuria visited our hospital. Cystoscopy revealed a tumor with an edematous surface on the left lateral wall of the urinary bladder. To diagnose the tumor, we performed a transurethral resection of the bladder tumor. Pathological examination suggested leiomyoma. Four weeks after the transurethral resection, magnetic resonance imaging revealed an increase in the bladder tumor. She received partial cystectomy. Pathological analysis revealed an inflammatory myofibroblastic tumor. No recurrence was observed 9 months after the initial operation.

Successful surgical management of an aggressive IgG4-related sclerosing esophageal inflammatory pseudotumor: a case report and review of literature.

BACKGROUND: Inflammatory pseudotumor (IPT) of the esophagus is a very rare benign lesions which clinical presentation is not clear and difficult to make a definitive diagnosis preoperatively. CASE PRESENTATION: In this report, we presented a case of a 24-year-old female with signs of severe malnutrition state due to dysphagia increasing gradually and losing 10 kg in weight for 2 months. Comprehensive preoperative radiologic investigations were proceeded with a circumferential severe stricture caused smooth submucosal swelling in the esophagus under 23 cm from the upper dental arch and two times of negative biopsy. Due to the aggressive clinical symptoms and gross lesion characteristics, the patient underwent laparoscopic-thoracoscopic esophagectomy and reconstruction with a gastric tube. Histopathological examination showed that the squamous epithelium of the esophagus had a small, benign nucleus, the submucosal layer and the smooth muscle layer increased fibrous, with infiltrating many lymphocytes, plasma cells, and macrophages. Immunohistochemical staining was negative for CD68, CD34, Desmin and ALK markers, and there was an increase in the number of IgG4-positive plasma cells. The final diagnosis was an aggressive IgG4-related sclerosing esophageal inflammatory pseudotumor. CONCLUSIONS: Inflammatory pseudotumor of the esophagus is an extremely rare benign lesion but could led to aggressive clinical presentation. The gold standard of diagnosis is histopathological examination of surgically removed specimens. Radical resection is still the most efficient treatment method.

Successful treatment with laparoscopic extended posterior sectionectomy for inflammatory pseudotumor in the liver with para-aortic lymphadenitis.

Inflammatory pseudotumor (IPT) is a rare disease that requires a differential diagnosis from malignancies. We describe a case of hepatic IPT with para-aortic lymphadenopathy, treated with a stepwise strategy of laparoscopic surgery. A 61-year-old woman was referred with a liver lesion. Computed tomography revealed a 13 cm well-defined lesion in segments VII-VI. The patient also had bead-like enlarged lymph nodes from the perihilar to the para-aortic regions. Although percutaneous lymph node biopsy showed no evidence of malignancy, 18 F-fluorodeoxyglucose positron emission tomography revealed accumulation in the lesion and lymph nodes. Lymph nodes were harvested laparoscopically for intraoperative pathological examination. With no evidence of malignancy, laparoscopic liver resection was continuously performed as a diagnostic treatment. The patient was given a pathological diagnosis of IPT and was discharged on the 16th day and is well 2 years after surgery. The minimally invasive laparoscopic approach to diagnostic treatment could be useful with secure advantages.