Immunoglobulin G4-related disease is an immune-mediated condition comprised of a number of various disorders sharing unique pathologic, serologic, and clinical features. Diagnosis of immunoglobulin G4-related sclerosing mastitis is challenging as the clinical and imaging findings mimic breast malignancies or other types of inflammatory mastitis. Herein, we describe a case of a female patient with a painless palpable mass in her right breast. An excisional core biopsy led to the rare diagnosis of immunoglobulin G4-related sclerosing mastitis, and the patient received steroid treatment for a month. To date, the patient has remained disease-free without any recurrence. As immunoglobulin G4-related sclerosing mastitis is a very rare disease, further studies are needed to reach conclusions about the pathogenesis, diagnosis, and treatment of this entity.

Liposarcoma is a malignant soft tissue tumor with several subtypes, the most common of which is well-differentiated liposarcoma (WDL) or atypical lipomatous tumor (ALT). WDL/ALTs are further divided into three histological subtypes, including lipoma-like, sclerosing, and inflammatory. While the majority of these tumors are predominantly fatty, the sclerosing variant demonstrates diverse histologic and radiographic characteristics, including variable amounts of fibrosis and fat. Because of this histological variability and relative rarity, the sclerosing WDL/ALT can present diagnostic dilemmas. We present two cases of sclerosing WDL/ALT, both of which demonstrated high degrees of fibrosis and a paucity of fat, mimicking desmoid fibromatosis and other fibrotic soft tissue tumors. Thus, it is important for radiologists to be aware of the subtypes of liposarcoma and their unique characteristics, and to consider sclerosing WDL/ALT in cases of fibrotic soft tissue tumors.

BACKGROUND: The tall cell, columnar, and diffuse sclerosing subtypes are aggressive histologic subtypes of papillary thyroid cancer (PTC) with increasing incidence, yet there is a wide variation in reporting. We aimed to identify and compare factors associated with the reporting of these aggressive subtypes (aPTC) to classic PTC (cPTC) and secondarily identify differences in outcomes.
METHODS: The National Cancer Database was utilized to identify cPTC and aPTC from 2004 to 2017. Patient and facility demographics and clinicopathologic variables were analyzed. Independent predictors of aPTC reporting were identified and a survival analysis was performed.
RESULTS: The majority of aPTC (67%) were reported by academic facilities. Compared to academic facilities, all other facility types were 1.4-2.0 times less likely to report aPTC (P < 0.05). Regional variation in reporting was noted, with more cases reported in the Middle Atlantic, despite there being more total facilities in the South Atlantic and East North Central regions. Compared to the Middle Atlantic, all other regions were 1.4-5 times less likely to report aPTC (P < 0.001). Patient characteristics including race and income were not associated with aPTC reporting. Compared to cPTC, aPTC had higher rates of aggressive features and worse 5-y overall survival (90.5% versus 94.5%, log rank P < 0.001).
CONCLUSIONS: Aggressive subtypes of PTC are associated with worse outcomes. Academic and other facilities in the Middle Atlantic were more likely to report aPTC. This suggests the need for further evaluation of environmental or geographic factors versus a need for increased awareness and more accurate diagnosis of these subtypes.

BACKGROUND: Intramedullary meningiomas are an exceptionally rare subtype of spinal tumors, accounting for only 5% of primary spinal neoplasms. Given their scarcity and unique characteristics, understanding optimal management approaches is crucial for improved clinical decision-making. This systematic review aims to consolidate existing literature and present a detailed case illustration to enhance understanding of this uncommon spinal tumor entity.
METHODS: A systematic search adhering to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines was conducted. Eligible studies included case reports, case series, cohort studies, reviews, and meta-analyses. Data extraction and synthesis focused on demographic characteristics, tumor location, clinical presentation, imaging findings, surgical interventions, histopathological features, and outcomes.
RESULTS: A total of 15 high-quality scientific articles were included in the systematic review, providing insights into various aspects of intramedullary meningiomas. Demographic analysis revealed a broad age distribution with an equal gender distribution among affected patients. Common clinical presentations included difficulty walking, sensory disturbances, spastic paraparesis, and urinary incontinence. Neuroimaging findings demonstrated heterogeneous signal intensity variations on T1- and T2-weighted images, with variable enhancement patterns on gadolinium-enhanced images. Surgical interventions, predominantly total resection, resulted in favorable postoperative outcomes in most cases.
CONCLUSIONS: Intramedullary meningiomas pose diagnostic and therapeutic challenges due to their rarity and unique characteristics. Tailored surgical approaches, incorporating techniques such as intraoperative neurophysiological monitoring and fluorescence-aided resection, are crucial for minimizing neurological deficits and optimizing patient outcomes. Despite their infrequency, recognizing intramedullary meningiomas in the differential diagnosis of spinal tumors is essential for prompt diagnosis and timely intervention, ultimately improving patient prognosis.

BACKGROUND: Primary sclerosing cholangitis (PSC) is a chronic, cholestatic liver disease that is characterized by an inflammatory and fibrotic process affecting bile ducts which eventually develops into liver cirrhosis and liver failure. The aim of this study was to investigate serum IgG subclass distribution in patients with PSC and its possible association with PSC outcomes.
METHODS: We performed a retrospective analysis of 181 patients who had been diagnosed with PSC between January 1970 and December 2015 and followed at our outpatient clinic. Their demographic, immunological, and clinical characteristics were recorded and analyzed.
RESULTS: This study included 181 patients with PSC (120 males, 61 females). There was no association between IgGs and the development of autoimmune hepatitis, cirrhosis, cholangiocarcinoma, liver transplantation, inflammatory bowel disease, and colectomy. Patients with elevated IgG4 had statistically significant higher rates of cholangitis (p = 0.02) and endoscopic retrograde cholangiopancreatography (ERCP) (p = 0.009). High IgG4 values were observed in nine patients who underwent ERCP. In these nine patients, on average, IgG4 was evaluated 5 years after ERCP (min 3 days, max 11 years). Subanalysis considering only IgG4 values evaluated before ERCP showed no significant difference but remains significant if we consider IgG4 values after ERCP.
CONCLUSIONS: Elevated IgG4 in our study showed a possible association with higher rates of cholangitis and ERCP among patients with primary sclerosing cholangitis. It seems that IgGs may be a useful tool for the prediction of outcomes in patients with PSC. A prospective study is necessary, especially to study the trends of IgGs values during disease as well as the role of possible seroconversion.

Sclerosing meningiomas (SMs) represent a rare histological variant of meningiomas, first described in 1989 as invasive bulking masses of whorling collagen bundles with a minimum percentage of meningothelia-resembling cells, and they are often misdiagnosed. The literature reports only 30 cases of SMs, with only two of them being intramedullary. The authors present the case of a patient with a cervical intramedullary SM who presented with gait disturbances, sensory deficits, weakness in four extremities, and hyperreflexia. The surgery was performed under neurophysiological monitoring and after administration of sodium fluorescein, which allowed us to discriminate the exact myelotomy point. Intramedullary SMs are very rare entities whose correct management may result in a good outcome.

BACKGROUND: Primary soft tissue sarcoma of the ovary is uncommon, with most literature reports being in the format of a single case report or small case series.
METHODS: We report a case of a primary sclerosing liposarcoma of the ovary in a 67-year-old lady. She presented with a rapidly growing ovarian mass, which was surgically excised. Histologically, the lesion appeared nodular with alternating hypercellular and low-cellularity areas, with areas of necrosis. It comprised atypical spindled, oval and stellate cells set in abundant myxoid and fibrous hyalinised stroma. Immunohistochemistry showed expression of p16, CDK4, CD34, S-100 and MDM2. The case was sent to a referral centre where MDM2 gene amplification was detected by interphase fluorescent in-situ hybridisation (FISH) analysis, rendering a diagnosis of well-differentiated sclerosing liposarcoma with focal low-grade dedifferentiation.
CONCLUSIONS: To the best of our knowledge, this is the first reported example of a sclerosing variant of liposarcoma in the ovary. Differential diagnosis includes microcystic stromal tumour (MST), sclerosing stromal tumour (SST), atypical spindle cell lipoma, myxoid liposarcoma, myxofibosarcoma, solitary fibrous tumour and low grade fibromyxoid sarcoma.
CONCLUSIONS: Ovarian sarcomas are characterised by a rapid and aggressive clinical course, requiring multidisciplinary team input for ongoing management. Complete surgical resection with clear margins is the mainstay of treatment.

Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal uterine tumor and the histological variant, sclerosing PEComa is exceedingly rare. Sclerosing PEComas preferentially occur in the retroperitoneum and occurrence in the uterine corpus is seldom seen. These tumors pose a diagnostic challenge and need distinction from morphological mimickers such as epithelioid smooth muscle tumors, endometrial stromal sarcoma, and metastatic carcinoma. Accurate diagnosis can be established coupling histomorphology with immunostaining. The distinction from other entities is of prime importance considering the therapeutic and prognostic implications. Herein, we describe a case of uterine sclerosing variant of PEComa with diagnostic difficulties and key to diagnose this entity.

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OBJECTIVE: Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is considered a biliary manifestation of IgG4-related diseases. However, there has been a controversy on the clinical outcomes according to the location of the involved bile duct. We therefore compared the clinical outcomes and long-term prognosis of IgG4-SC with proximal bile duct involvement (proximal IgG4-SC) and IgG4-SC with distal bile duct involvement (distal IgG4-SC).
METHODS: We reviewed the data of patients with IgG4-SC that were prospectively collected at 10 tertiary centers between March 2002 and October 2020. Clinical manifestations, outcomes, association with autoimmune pancreatitis (AIP), steroid-responsiveness, and relapse of IgG4-SC were evaluated.
RESULTS: A total of 148 patients (proximal IgG4-SC, n = 59; distal IgG4-SC, n = 89) were analyzed. The median age was 65 years (IQR, 56.25-71), and 86% were male. The two groups were similar in terms of jaundice at initial presentation (51% vs 65%; P = 0.082) and presence of elevated serum IgG4 (66% vs 70%; P = 0.649). The two groups showed significant differences in terms of steroid-responsiveness (91% vs 100%; P = 0.008), association with AIP (75% vs 99%; P = 0.001), and occurrence of liver cirrhosis (9% vs 1%; P = 0.034). During a median follow-up of 64 months (IQR, 21.9-84.7), the cumulative relapse-free survival was significantly different between the two groups (67% vs 79% at 5 years; P = 0.035).
CONCLUSIONS: Relapse of IgG4-SC frequently occurred during follow-up. Proximal IgG4-SC and distal IgG4-SC had different long-term outcomes in terms of steroid-responsiveness, occurrence of liver cirrhosis, and recurrence. It may be advantageous to determine the therapeutic and follow-up strategies according to the location of bile duct involvement.