UNASSIGNED: Hemangioblastoma originates in the central nervous system (CNS), usually in the cerebellum, and sporadic cases in the supratentorial region are extremely rare. In addition, there have been no previous reports of cases showing hyperintensity on diffusion weighted image (DWI) on magnetic resonance imaging (MRI) and negative immunostaining for inhibin-alpha. Here, we report a rare case of sporadic supratentorial hemangioblastoma arising in the parasagittal region and suggest a useful indicator for the exact diagnosis and pitfalls for surgical procedures.
UNASSIGNED: A 66-year-old woman was admitted to our hospital with a 6-month history of progressive numbness in the right lower extremities and gait disturbance. Neurological findings on admission revealed mild right-sided hemiparesis of the lower limbs (manual muscle test: 4/V). Neuroimaging demonstrated an abnormal lesion with clear boundaries in the left frontal lobe appearing hypointense on T1-weighted image (WI), hyperintense on T2-WI, and hyperintense on DWI, with strong enhancement on gadolinium (Gd)-enhanced T1-WI. Computed tomography (CT) showed no calcification, and cerebral angiography revealed strong staining from bilateral middle meningeal arteries and the left anterior cerebral artery (ACA). Surgical excision of the lesion was performed and gross total resection was achieved. Histological findings revealed a marked increase in vascular structures, and the round stroma contained tumor cells. Silver impregnation stains demonstrated abundant reticulin fibers. In addition, immunohistochemistry revealed that most tumor cells stained negatively for epithelial membrane antigen (EMA) and inhibin-alpha, and positively stained for podoplanin (D2-40), and the tumor was diagnosed as hemangioblastoma. The postoperative course was uneventful and follow-up neuroimaging after one year revealed no signs of recurrence.
UNASSIGNED: Supratentorial hemangioblastomas are extremely rare and display a strong infiltrative and aggressive nature. Careful identification from preoperative image and histopathological study for appropriate treatment selection are warranted for supratentorial hemangioblastoma.

BACKGROUND: Sjögren\'s syndrome (SjS) is a systemic autoimmune disease characterized by lymphocytic infiltration of the salivary and lacrimal glands associated with sicca syndrome. TAFRO syndrome is a systemic inflammatory disease of unknown cause, characterized by Thrombocytopenia, Anasarca, Fever, Reticulin fibrosis, Renal dysfunction and Organomegaly, first reported in 2010 in Japanese patients. Despite their rarity, both conditions have been concurrently reported in several patients during the recent years, hence questioning the existence of shared or related features.
METHODS: A systematic review of the literature regarding SjS associated with TAFRO syndrome (SjS-TAFRO) was performed. The 2019 updated Masaki diagnostic criteria were used for TAFRO syndrome and SjS was considered when the diagnosis was mentioned by the authors, necessarily with either anti-Sjogren\'s Syndrome A (SSA) ± anti-Sjogren\'s Syndrome B (SSB) antibodies and/or histological evidence of focal lymphocytic sialadenitis.
RESULTS: Ten cases of SjS-TAFRO have been reported in the literature to date. Compared to SjS patients without TAFRO syndrome, these 10 SjS-TAFRO had a lower female predominance (2.3:1 vs 9:1 women to man ratio) and a higher frequency of anti-SSA antibodies (90% vs 70%). All fulfilled the three major Masaki criteria i.e., anasarca, thrombocytopenia, and systemic inflammation. Seven of them (70%) had megakaryocyte hyperplasia or reticulin fibrosis in the bone marrow. Lymph node biopsy was performed in 8 out of 10 cases (80%) and results were consistent with Castleman disease in 6 (75%). Eight of them had developed renal failure (80%) within six months. Nine of them (90%) had organomegaly, with hepatosplenomegaly in 8 cases and splenomegaly alone in 1.
CONCLUSIONS: This review brings new insights regarding TAFRO syndrome and suggests it could be a severe manifestation of SjS. The identification of shared abnormal signaling pathways could help in the therapeutic management of both diseases, which face an unmet therapeutic need.

Fibroblastic reticular cells (FRCs), usually found and isolated from the T cell zone of lymph nodes, have recently been described as much more than simple structural cells. Originally, these cells were described to form a conduit system called the \"reticular fiber network\" and for being responsible for transferring the lymph fluid drained from tissues through afferent lymphatic vessels to the T cell zone. However, nowadays, these cells are described as being capable of secreting several cytokines and chemokines and possessing the ability to interfere with the immune response, improving it, and also controlling lymphocyte proliferation. Here, we performed a systematic review of the several methods employed to investigate the mechanisms used by fibroblastic reticular cells to control the immune response, as well as their ability in determining the fate of T cells. We searched articles indexed and published in the last five years, between 2016 and 2020, in PubMed, Scopus, and Cochrane, following the PRISMA guidelines. We found 175 articles published in the literature using our searching strategies, but only 24 articles fulfilled our inclusion criteria and are discussed here. Other articles important in the built knowledge of FRCs were included in the introduction and discussion. The studies selected for this review used different strategies in order to access the contribution of FRCs to different mechanisms involved in the immune response: 21% evaluated viral infection in this context, 13% used a model of autoimmunity, 8% used a model of GvHD or cancer, 4% used a model of Ischemic-reperfusion injury (IRI). Another four studies just targeted a particular signaling pathway, such as MHC II expression, FRC microvesicles, FRC secretion of IL-15, FRC network, or ablation of the lysophosphatidic acid (LPA)-producing ectoenzyme autotaxin. In conclusion, our review shows the strategies used by several studies to isolate and culture fibroblastic reticular cells, the models chosen by each one, and dissects their main findings and implications in homeostasis and disease.

BACKGROUND: Mitotically active cellular fibroma (MACF) of the ovary, characterized by relatively high mitotic activity without severe atypia, represents a relatively new disease entity. MACF is categorized as a benign ovarian tumor. However, due to a limited number of case reports, its clinical and pathological features and optimum management remains largely undetermined. Herein, we report on a rare case of MACF that grew rapidly in size and was diagnosed on detailed pathological examination.
METHODS: A 44-year-old Japanese woman, who detected a myoma-like lesion 1-year earlier, was referred to our hospital when the follow-up examination demonstrated that the mass had increased in size. Magnetic resonance imaging revealed a T1 isointense and T2 hyperintense tumor (11 cm in diameter) in the right pelvic cavity. Laparoscopy confirmed the presence of a right ovarian tumor and laparoscopic right adnexectomy was performed. The tumor cells consisted of dense cellular proliferations of spindle fibroblast-like cells without significant cytological atypia. The mitotic activity index was estimated at >15 mitotic figures per 10 high-power fields. Reticulin staining and FOXL2 mutation analysis excluded the possibility of an adult granulosa cell tumor, and the patient was diagnosed with a MACF of the ovary.
CONCLUSIONS: To the best of our knowledge, we are the first to report on a case of rapid growth of a MACF of the ovary during follow-up. When an increase in the size of a solid ovarian mass is detected, a MACF should be considered as a differential diagnosis.

Pathologic increase in bone marrow reticulin fibrosis can be present in many malignant hematopoietic diseases. In acute leukemia, one-third of patients have some degree of marrow reticulin fibrosis at presentation, which is thought to be related to cytokine release from blasts. Marrow fibrosis is particularly common in acute megakaryoblastic leukemia, while this change is rarely seen in acute promyelocytic leukemia. Six case reports of acute promyelocytic leukemia with marrow reticulin fibrosis have been described so far in the literature. Herein, we present three cases of classical acute promyelocytic leukemia with increased marrow reticulin fibrosis encountered in our institution, summarizing their clinicopathologic features, treatment, and outcome to date. Awareness of the features of acute promyelocytic leukemia with marrow reticulin fibrosis is important as it may guide treatment options.

暂无摘要。

Celiac disease (CD) is an autoimmune disorder that occurs in genetically susceptible individuals of all ages and is triggered by immune response to gluten and related proteins. The disease is characterized by the presence of HLA-DQ2 and/or -DQ8 haplotypes, diverse clinical manifestations, gluten-sensitive enteropathy, and production of several autoantibodies of which endomysial, tissue transglutaminase, and deamidated gliadin peptide antibodies are considered specific. Although antireticulin antibodies (ARA) have historically been used in the evaluation of CD, these assays lack optimal sensitivities and specificities for routine diagnostic use. This minireview highlights the advances in CD-specific serologic testing and the rationale for eliminating ARA from CD evaluation consistent with recommendations for diagnosis.

We report a case of enteropathy-associated T-cell lymphoma (EATL), diagnosed by small intestine and gastric biopsies, who presented with manifestations of hypocalcemia and malabsorption. Immunological assessment revealed increased expression levels of tumor necrosis factor system components and eotaxin, an observation that is consistent with the cytotoxic T-cell phenotype characteristic of EATL, and decreased numbers of circulating activated (CD8+CD38+ and CD4+CD25+) and suppressor (CD11b+) T cells, a feature which can contribute to lymphomagenesis in patients with celiac disease. The acute clinical presentation of the patient resolved with mineral and vitamin supplementation and a gluten-free diet. The novel immunological findings described are discussed in the context of a review of our current knowledge of the immunopathogenesis of celiac disease and associated intestinal neoplasia.

暂无摘要。

Primary renal angiosarcoma is very rare. To our knowledge, only 15 cases have been reported to date. A 77-year-old Japanese man with a unilateral kidney presented with massive hematuria followed by renal failure. A renal tumor was suspected and a left nephrectomy was performed. The histopathological diagnosis was angiosarcoma of the kidney. A hemorrhagic tumor measuring 10 x 5 cm and clotted blood was found in the medullary area. The atypical tumor cells had a sinusoidal and solid appearance, and showed immunohistochemically positive reactions for some of the endothelial markers. The patient died about 21 months after the nephrectomy and the autopsy revealed massive metastases to the liver and retroperitoneum. One of the differential diagnoses of the case was angiomyolipoma, because the tumor cells were relatively bland in their histological appearance with entrapped fat cells in the pelvic area. Fifteen case reports with titles that included the term \'hemangiosarcoma/angiosarcoma\', \'hemangioendothelioma/endothelioma\' or \'vascular sarcoma\' of the kidney were reviewed and compared to the present case.