Solitary fibrous tumor/hemangiopericytoma of the cervical spine: A systematic review of the literature with an illustrative case.

Background: In the WHO 2016 classification of central nervous system tumors, solitary fibrous tumors (SFT) and hemangiopericytomas (HPC) were considered part of the same category given a shared mutation. Nevertheless, since the new 2021 WHO classification, the term "hemangiopericytoma" has been retired, and SFT is considered an independent pathological entity. Methods: We reviewed the literature following preferred reporting items for systematic reviews and meta-analyses guidelines focusing on the treatment options and prognosis of patients with cervical SFT. We also present a 68-year-old female with spinal intradural extramedullary SFT complicated by diffuse extension into paravertebral tissues and muscles. Results: We found 38 cervical SFT in the literature. Patients averaged 47.3 years of age and 47.4% were female. Typically, these lesions spanned two spinal levels resulting in cord compression and most frequently exhibited benign features (i.e., diagnosed as Grade I SFTs). Interestingly, two patients exhibited distant metastases and had initial pathology consistent with grade II SFT. Conclusion: SFT of the cervical spine is rare and its management varies according to the histological grade and the clinical behavior, generally warranting surgical excision and adjuvant radiation therapy and/or systemic chemotherapy.

Pfeifer-Weber-Christian Disease: A Case Report and Review of Literature on Visceral Involvements and Treatment Choices.

Pfeifer-Weber-Christian disease (PWCD) is a rare idiopathic disease characterized by lobular panniculitis of adipose tissue with systemic symptoms and multiple organ involvement. Even though the systemic involvement is rare, it is life-threatening and represent a treatment challenge for the clinicians. We report a case of PWCD characterized by hepatic, hematologic, and renal involvement, with good response to mofetil mycophenolate and prednisone treatment. A 47-year-old female presented several months' history of painful subcutaneous nodules, fever and lymphadenopathy with recent appearing of microcytic hypochromic anemia, leucopenia with neutropenia, and increase in transaminase. Skin biopsy showed lobular panniculitis with lymph-histiocytic and neutrophilic infiltrates with necrosis of adipocytes. A combination therapy of corticosteroid with mofetil mycophenolate was effective. Moreover, we discuss the clinical manifestation and the therapeutic choices in PWCD, from classical immunosuppressive drugs to new biotechnological agents, and we provide a comprehensive review of the available literature.

Whipple's disease: case report and review of the literature.

Whipple's disease (WD) is known as an infrequent, systemic, chronic infection caused by the actinomycete Tropherima whipplei (T. whipplei). The disease is frequently characterized by a long prodromal and protean extra-intestinal phase, which often causes misdiagnosis and inappropriate treatments. Herein, we describe the case a 62-year-old man with a histological diagnosis of WD established when oral steroid treatment was started due to rheumatic manifestations, triggering intestinal symptoms. Systematic review of the literature was performed to include studies where WD was eventually diagnosed on duodenal biopsies. Three patients' subgroups were identified according to the clinical presentation.

Multifocal Gastrointestinal Angiosarcoma: a Challenging Diagnosis?

Angiosarcoma rarely involves the gastrointestinal tract. Herein, we describe the case of a 68-year-old man with haemoptysis and melena who was eventually diagnosed with multifocal angiosarcoma of the stomach, small bowel, lungs, and thyroid. The peculiarity was that the histological feature of the polypoid lesions removed at endoscopy was initially misinterpreted as benign hyperplastic polyps, whilst their neoplastic nature was clinically suspected only when the videocapsule endoscopy revealed the presence of multiple variable-sized nodules with apical erosion or active bleeding in the small bowel. Based on the very low incidence, diagnosis of angiosarcoma involving the gastrointestinal tract may be misinterpreted by both the endoscopist and pathologist.

High Wilms' tumour gene (WT1) expression and low mitotic count are independent predictors of survival in diffuse peritoneal mesothelioma.

AIMS: To evaluate the use of the Wilms' tumour gene (WT1) marker and histomorphological parameters as indicators of prognosis in malignant peritoneal mesothelioma (MPM). METHODS AND RESULTS: Histological samples of 31 MPM were stained immunohistochemically for the WT1 protein. The results were quantified by recording the number of stained nuclei, and then correlated with patient survival. Statistical correlation was evaluated for tumour histotype, mitotic count (MC), nuclear grade (NG), necrosis, lymphoid response (grade of inflammation) and desmoplasia with regard to survival. High-grade histology (solid epithelioid, pure sarcomatoid or biphasic tumours), high NG, MC more than five per 10 per high-power field (HPF), necrosis and desmoplasia were associated with a significantly worse prognosis. Patients with MPM with low WT1 expression (≤25% of positive cells) survived for a significantly shorter time compared to those with high WT1 expression (>25% of positive cells) (P = 0.0001). The 50% survival time of subjects with low WT1 expression was 2.9 months [95% confidence interval (CI): 2.05-3.71] versus 31.5 months (95% CI: 20.4-42.5) for those with high WT1 expression. On multivariate analysis, WT1 and MC were found to be associated independently with survival (P = 0.002; P = 0.005, respectively). CONCLUSIONS: Our study suggests that low WT1 expression and high MC may be indicative of an unfavourable prognosis in patients with advanced malignant peritoneal mesothelioma.

IL15 can reverse the unresponsiveness of Wilms' tumor antigen-specific CTL in patients with prostate cancer.

PURPOSE: The Wilms' tumor antigen 1 (WT1) is overexpressed in several leukemias and solid tumors, but there is currently limited information regarding its role in prostate cancer. This study aimed to investigate WT1 expression in prostate cancer, and to determine the number and function of WT1-specific T cells in the peripheral blood of patients. EXPERIMENTAL DESIGN: Immunohistochemistry was used to assess WT1 expression in cancer tissues. Human leukocyte antigen A2 (HLA-A2) tetramers served to detect WT1-specific T cells, and peptide-specific stimulation was used to assess T-cell function in vitro. RESULTS: Immunohistochemistry of tissue arrays comprising 36 cancer and 8 normal prostate samples revealed nuclear WT1 staining in 39% of cancer samples, but not in normal prostate tissues. Tetramer analysis revealed a low frequency of WT1-specific T cells in 20 of 38 HLA-A2-positive patients. In vitro stimulation with WT1 peptide plus interleukin 2(IL2) and interleukin 7 (IL7) did not lead to an accumulation of WT1-specific T cells in any of the patient samples, although all patients were able to generate T-cell responses against Melan-A/MART1 control peptide. Stimulation with WT1 peptide in the presence of interleukin 15 (IL15), a cytokine that was shown to reverse tolerance of murine tumor-specific T cells, was able to restore the expansion and IFNgamma production of WT1-specific T cells in a subgroup of prostate cancer patients. CONCLUSION: The observation that IL15 can restore the function of WT1-specific T cells that were unresponsive to IL2 has implications for vaccination and immunotherapeutic strategies that aim to enhance WT1-specific T cell immunity in patients.

Survivin expression in renal cell carcinoma.

Deregulated expression of inhibitors of apoptosis may contribute to cancer by aberrantly extending cell viability and facilitating the insurgence of resistance to chemo- and radiotherapy. In this study, we have investigated, by immunohistochemical technique, the expression and potential prognostic significance of survivin in a series of 49 clear cell type renal cell carcinoma (ccRCC). Survivin expression was significantly associated with poorly differentiated, advanced stages and more aggressive ccRCCs (p < 0.05). Patients with low survivin expression had statistically significant better survival rates than patients with high survivin expression (p < 0.05). This may be relevant for follow-up protocols design and/or alternative therapeutic approaches.