Malignancy and mass-forming phenotypes of IgG4-related disease: a challenging diagnosis.

Mass-forming phenotypes of IgG4-related disease (IgG4-RD) mimic malignancy and histological confirmation can be challenging. A woman in her 70s with HIV infection presented with painless obstructive jaundice and weight loss. Magnetic resonance imaging was suggestive of unresectable cholangiocarcinoma. Tumour markers and serum IgG4 were normal. Percutaneous liver biopsy was consistent with IgG4-RD inflammatory pseudotumour, with complete response to glucocorticoid therapy. Two years later, a new episode of obstructive jaundice occurred, with CT showing a solid lesion in the head of the pancreas with double duct sign and encasement of the portal vein. Re-induction therapy was tried without response. Fine-needle biopsy was consistent with pancreatic cancer. Supportive care was offered and the patient died 8 months later, with no signs of disease progression on subsequent imaging. We discuss the challenges of IgG4-RD diagnosis and treatment and the differential diagnosis between mass-forming phenotypes and malignancy, highlighting the difficulties in managing such patients.

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.

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.

Nonspecific inflammatory pseudotumor of the maxillary and temporal fossa: a study of seven cases.

OBJECTIVE: Inflammatory pseudotumor (IPT) is a rare, locally aggressive, benign neoplasm of unknown etiology. Because of its aggressive clinical behavior and locally destructive or infiltrative features, it may be mistaken for a malignant tumor. Approximately 5%-44% of extrapulmonary IPT occur in the head and neck, primarily affecting the orbit. STUDY DESIGN: Between 2008 and 2021, our hospital received referrals for seven patients (three men and four women, aged 42-73 years) with pain, swelling, mass, and trismus. Computed tomography, magnetic resonance imaging, and biopsy were performed on all patients to diagnose IPT. RESULTS: Of the seven patients, four received low-dose prednisolone (PSL), one underwent surgery, and two were left untreated. The IPT disappeared in one of the two untreated cases, whereas it improved and later deteriorated in the other. The surgical patient had no recurrence. Low-dose PSL was effective in two patients; however, high-dose PSL and immunosuppressants were required in the remaining two cases owing to infiltration into each orbit or brain region. CONCLUSIONS: Low-dose PSL treatment was applicable in IPT cases affecting the maxillary to temporal fossa region, wherein symptoms did not improve without treatment. However, when low-dose PSL was ineffective, high-dose PSL and immunosuppressants were required.

Rare cardiac inflammatory pseudotumor in a toddler: Complementary roles of cardiac magnetic resonance and positron emission tomography.

We present a rare pediatric case of cardiac inflammatory pseudotumor (IPT) with a unique presentation of fever of unknown origin with markedly elevated inflammatory markers. A right atrial mass was discovered incidentally by echocardiography. The cardiac magnetic resonance (CMR) signal characteristics and mass location were not consistent with any of the common benign cardiac tumors of childhood. The presence of high signal intensity on T2 imaging and late gadolinium enhancement, in conjunction with intense metabolic activity at the mass site on positron emission tomography (PET), raised the possibility of an inflammatory or malignant mass. The diagnosis of IPT was confirmed by biopsy. Our case highlights the utility of PET imaging to confirm the inflammatory nature and extent of an IPT.

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.

The Potential Utility of 18 F-FDG PET/CT in Monitoring Corticosteroid Therapy in Inflammatory Pseudotumor.

ABSTRACT: A 69-year-old man with suspected gastrointestinal stromal tumor was referred to 18 F-FDG PET/CT. Images showed increased metabolism in a jejunal wall thickening, 2 liver lesions, and hepatic lymph nodes. The patient underwent wedge biopsy of the liver, which revealed inflammatory pseudotumor. The patient was treated with 20 mg/d prednisone, with a gradual dose reduction. A partial metabolic response was achieved after 2 months of therapy, and a final PET/CT showed complete metabolic response after 9 months. This clinical case shows the potential role of PET/CT in the assessment of the response of the inflammatory pseudotumor to corticosteroid therapy.

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.

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.

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.

IgG4-related hepatic inflammatory pseudotumor in a patient with serum IgG4-negative type 1 autoimmune pancreatitis.

IgG4-related disease (IgG4-RD) can cause heterogeneous lesion in various organs. Serum IgG4 levels are useful in monitoring patients with IgG4-RD; however, when it is negative, more careful observation is required. A 58-year-old woman who had been diagnosed with serum IgG4-negative type 1 autoimmune pancreatitis (AIP) 3 years prior visited our hospital for the evaluation of a liver tumor. She had visited a nearby hospital 1 month prior with complaints of a swelling in her right neck, and histological examinations were suggestive of IgG4-related sialadenitis. A positron emission tomography scan showed fluoro-deoxy-glucose accumulation in her right liver lobe; therefore, she was referred to our hospital. Liver tumor biopsy showed inflammatory cell infiltration and storiform fibrosis, without histological findings indicative of a malignancy. Many IgG4-positive cells were detected in immunostaining; thus, an IgG4-related hepatic inflammatory pseudo-tumor was diagnosed. After increasing in steroid dosage, the patient remained recurrence-free with 2 years. To our knowledge, this is the first report of mass-forming IPT for serum IgG4-negative type 1 AIP. Occasionally, IgG4-related IPT may appear in the periphery of the liver, and serum IgG4-negative cases should be more carefully observed because serum IgG4 is not an indicator.

Inflammatory Pseudotumour Of The Urachus: Case Report And Literature Review.

A 52 year old woman presented to the emergency department of Affiliated Hospital of Zunyi Medical University, Zunyi, China in May 2022, complaining of a palpable lower abdominal mass since two days. She denied haematuria, umbilical drainage, or any other urinary symptoms. Previous health record indicated that the patient was diagnosed with urachal inflammatory pseudotumour. Inflammatory pseudotumourous masses of the urachal canal are rare chronic inflammatory disorders with only a few case reports. Ultrasonography is the preferred method for diagnosing urachal lesions. Contrast- enhanced ultrasonography (CEUS) allows real-time visualization of the microvascular blood flow within the solid lesion, reducing the probability of misdiagnosis of the disease. We have reported a case of urachal inflammatory pseudotumour and analyzed its ultrasonographic findings from two-dimensional conventional ultrasonography and CEUS to provide support for the diagnosis of urachal inflammatory pseudotumour in the clinic and to assist clinical selection of effective treatment modalities.