Inflammatory Myofibroblastic Tumor in the Inguinal canal: A Rare Case Report

Background Inflammatory myofibroblastic tumors, previously known as inflammatory pseudotumors, are rare soft tissue carcinomas with variable presentation and location. Due to non-specific symptoms and location, the diagnosis of this condition is often clinically challenging. Only a handful of case reports have been published in the literature describing this tumor, and there is still a lack of consensus on pathogenesis, risk factors, and treatment strategy. Most tumors have shown mutation in the anaplastic lymphoma receptor tyrosine kinase (ALK) gene. In this article, we describe a case of ALK-negative malignant inflammatory myofibroblastic tumor. Case A 46 years old male with no risk factors presented with a mass in the inguinal region. The ultrasound was suggestive of a mixed echoic mass suggestive of inguinal hernia, which led to surgical repair with resection of the tumor segment. Subsequently, histopathology and immunohistochemistry confirmed that the mass was an inflammatory myofibroblastic tumor which then recurred in a few years and failed to respond to multiple chemotherapy regimens, and over time, it progressively metastasized to the anterior abdominal wall and lungs. The patient is currently receiving palliative chemotherapy and symptomatic treatment. Conclusion This rare soft tissue tumor has not received much attention, and clinicians often miss the diagnosis. We stress that further study should be carried out on these soft tissue tumors, and adequate diagnostic and therapy recommendations should be developed.

Nodal mycobacterial spindle cell pseudotumor: A diagnostic pitfall

Inflammatory pseudotumor (IPT) is a rare benign mass forming lesion that has been reported in virtually every organ, and can closely mimic spindle cell neoplasms. Mycobacterial spindle cell pseudotumor (MSP) represents a small proportion of IPT of the lymph node, which occur in immunocompromised patients, posing a diagnostic challenge. We report three cases of MSP involving two AIDS patients who presented with generalized lymphadenopathy, and one immunosuppressed patient with a mediastinal mass. Biopsy in these cases revealed effaced architecture replaced by proliferating fibrohistiocytic spindle cells, fibrosis and polymorphic inflammatory infiltrate. Inflammatory pseudotumors and other spindle cell neoplasms of the lymph node can show overlapping morphologic features, resulting in diagnostic confusion. A differential diagnosis of Mycobacterial spindle cell pseudotumor should be kept in mind when approaching localized or generalized lymphadenopathy in an immunocompromised patient.

Resección endoscópica bajo visión coledocoscópica de pólipo biliar. Reporte de un caso

Inflammatory pseudotumors of the biliary tract represent an infrequent biliary pathology, with few cases reported in literature. In radiological or surgical explorations, these tumors are usually diagnosed as unsuspected findings. They represent a challenge in preoperative diagnosis, due to the cost of specific technology such as Spyglass DS (Boston Scientific) and the need of trained personnel for its correct use. This means that most patients are usually treated with major resections with wrong preoperative diagnosis of cholangiocarcinoma, missing a chance of an endoscopic solution while adding unnecessary morbidity and mortality in benign pathology. We present a case that underwent endoscopic resection by ERCP under direct choledochoscopic view for Kehr’s biliary fístula in the General Surgery Department of a central hospital.

Chronic Expansive Retroperitoneal Hematoma

Chronic expansive retroperitoneal hematoma is a hematoma that grows progressively sometime after the original trauma. It can appear in different locations. The mechanism of onset is unclear, but experimental evidence favours an inflammatory cause. For diagnostic imaging, magnetic resonance imaging is more useful than computed tomography (CT). However, it is difficult to differentiate it from other soft tissue tumors, sarcomas, actinomycosis or inflammatory pseudotumors, due to the uneven central uptake. Histopathological analysis of the lesion usually shows a dense fibrous tissue capsule containing numerous old clots without finding clear atypia or signs suggestive of malignancy. The treatment of choice is complete resection of the lesion (including the capsule), as incomplete treatments (such as drainage or curettage of the hematoma contents) may cause recurrence.

IgG4-Related Inflammatory Pseudotumor Involving the Clivus: A Case Report and Literature Review

IgG4-related inflammatory pseudotumors are very uncommon and are characterized histologically by the presence of inflammatory swellings with increasing IgG4-positive plasma cells and lymphocytes infiltrating the tissues. As reports of intracranial IgG4-related pseudotumors are very rare, we report a case of an IgG4-related inflammatory pseudotumor involving the clivus mimicking meningioma. A 46-year-old male presented with intermittent headache for 2 years and a sudden onset of dysphagia and dysphonia of 7 days’ duration along with lower limb weakness. Enhanced magnetic resonance imaging (MRI) of the skull base revealed an isointense signal on T1- and T2-weighted images from an enhanced mass located at the middle of the upper clivus region, for which a meningioma was highly suspected. Then, an endoscopic transsphenoidal approach was adopted and the lesion was partially resected, as the subdural extra-axial lesion was found to be very tough and firm, exhibiting fibrous scarring attaching to the brain stem and basal artery. After the surgery, brain stem and posterior cranial nerve decompression was achieved, and the patient’s symptoms, such as dysphagia, dysphonia and lower limb weakness, improved. Pathological findings showed many IgG4-positive plasma cells and lymphocytes surrounded by collagen-rich fibers. The patient was sent to the rheumatology department for further glucocorticoids after the diagnosis of an IgG4-related inflammatory pseudotumor was made. This case highlights the importance of considering IgG4-related inflammatory pseudotumors as a differential diagnosis in patients with lesions involving the clivus presenting with a sudden onset of symptoms of dysphagia and dysphonia along with lower limb weakness when other more threatening causes have been excluded. IgG4-related inflammatory pseudotumors are etiologically enigmatic and unpredictable, and total resection might not be warranted. Glucocorticoids are usually the first line of treatment after diagnosis.

A Rare Case of Plasma Cell Granuloma

Plasma cell granulomas (PCGs) or inflammatory pseudotumors are nonneoplastic lesions that consist of predominantly antibody-secreting plasma cells and innate immune cells such as neutrophils, macrophages, and eosinophils. Unlike in multiple myeloma, the plasma cells are polyclonal and present in a spindly fibroblast-rich stromal background. These lesions mainly occur in the lungs; however, they can arise in other organs. PCGs from the gingiva are extremely rare, and a proper diagnosis is crucial to treat these patients further. These tumors have an increased number of plasma cells that are immunoreactive with CD138 and are polyclonal for kappa and lambda light chains, confirming these proliferations’ nonneoplastic nature. Surgical resection with clear margins, when possible, is the primary choice of treatment. Radiation and anti-inflammatory steroid therapy are other therapeutic approaches. Critical and careful examination by a pathologist is necessary to rule out plasma cell neoplasms. Here, we report a rare occurrence of gingival PCG in an elderly male.

Soft Tissue Sarcoma Follow-up Imaging: Strategies to Distinguish Post-treatment Changes from Recurrence

Soft tissue sarcomas encompass multiple entities with differing recurrence rates and follow-up intervals. The detection of recurrences and their differentiation from post-therapeutic changes is therefore complex, with a central role for the clinical radiologist. This article describes approved recommendations. Prerequisite is a precise knowledge of the current clinical management and surgical techniques. We review recurrence rates and treatment modalities. An adequate imaging technique is paramount, and comparison with previous imaging is highly recommended. We describe time-dependent therapy-related complications on magnetic resonance imaging compared with the spectrum of regular post-therapeutic changes. Early complications such as seromas, hematomas, and infections, late complications such as edema and fibrosis, and inflammatory pseudotumors are elucidated. The appearance of recurrences and radiation-associated sarcomas is contrasted with these changes. This systematic approach in follow-up imaging of soft tissue sarcoma patients will facilitate the differentiation of post-therapeutic changes from recurrences.

Urologic manifestations of IgG4-related disease

IgG4-related disease (IgG4-RD) is a clinical entity characterized by elevated serum IgG4 and tumor-like inflammation, with tissue infiltration by IgG4 and plasma cells. IgG4-RD is rare, but clinically significant, and its urologic manifestations have been reported in the literature. The present review covers a broad spectrum, describing the pathologies related to the area of urology. In 2003, Terumi Kamisawa was the first to recognize IgG4-RD, characterized by multiorgan lesions in patients with autoimmune pancreatitis and classified as an inflammatory and fibrotic entity with a dense lymphoplasmacytic infiltrate, positive for IgG4.(1–3) It presents in middle-aged patients, between 59-68 years of age, with no clear distribution by sex, (4–6) and has different clinical presentations. The main urologic manifestations are inflammatory pseudotumors and lower urinary tract symptoms. The present article offers a clear, general overview of the disease, encompassing its pathophysiology, diagnosis, and treatment, from the perspective of urology.