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Classical Hodgkin lymphoma (CHL) is a common lymphoid neoplasm with a wide range of differential diagnoses. Although it has a specific immunophenotype, aberrant expression of antigens can cause problems at its diagnosis. In this study we evaluated the usefulness of GATA3 in the differential diagnosis of CHL. One hundred cases of CHL and a control group of 106 lymphoma cases, which included anaplastic large-cell lymphoma both positive and negative for anaplastic lymphoma kinase (ALK), Epstein-Barr virus (EBV)-positive large B-cell lymphoma, T-cell/histiocyte-rich B-cell lymphoma, primary mediastinal large B-cell lymphoma, nodular lymphocyte-predominant Hodgkin lymphoma, and mediastinal gray-zone lymphoma, were included in the study. GATA3 immunohistochemistry was applied to all cases and nuclear expression was accepted as positive. Expression status of GATA3 was compared between the CHL group and the control group, as well as among each lymphoma subtype. In addition, whether the biopsy type affected diagnostic performance was assessed. For CHL, the relationship with EBV status and GATA3 expression was evaluated. GATA3 expression was significantly higher in CHL cases compared to the control group (p<0.001). When compared among individual subgroups, GATA3 was found to be useful in the differential diagnosis of all except for ALK-negative anaplastic large-cell lymphoma (p=0.678) and mediastinal gray-zone lymphoma (p=0.327). GATA3 expression was significantly higher in EBV-negative CHL (p=0.02). In core-needle biopsies, the diagnostic performance was limited (p=0.178). GATA3 is a useful marker for differentiating CHL from B-cell non-Hodgkin lymphomas but its efficiency is limited in ALK-negative anaplastic large-cell lymphoma and mediastinal gray-zone lymphoma. Due to heterogeneous reactions, its diagnostic value is limited in core-needle biopsies.

Progress in the field of pediatric thyroid pathology has linked DICER1 mutations to benign follicular cell–derived thyroid tumors (e.g., follicular adenoma with papillary architecture, follicular nodular disease), low-risk follicular cell–derived differentiated thyroid carcinomas and PDTCs enriched in fatal or recurrent/progressive disease. The dismal outcome of DICER1-harboring pediatric PDTCs stems from a limited number of reported patients’ data given the rarity of pediatric PDTCs. In light of the former observations, the current study assessed clinicopathological variables of a series of 5 pediatric (≤ 18 years old) PDTCs using the Turin criteria (WHO 2022) and also examined the status of DICER1 and TERT promoter mutations. Five PDTCs (3 males, 2 females) were included in the study. The mean age at the time of diagnosis was 15.4 years. No patients had a history of DICER1 syndrome-related tumors or other clinicopathological diagnostic features of DICER1 syndrome. The mean tumor size was 3.9 cm. All tumors were completely submitted for microscopic examination. There was increased mitotic activity ranging from 3 to 10 mitoses per 2 mm2. Tumor necrosis was present in two cases. No PDTC harbored TERT promoter mutation. DICER1 hot spot mutation was identified in one (20%) tumor. The DICER1-mutant tumor had neither associated differentiated thyroid carcinoma component nor other pathological findings in the adjacent thyroid parenchyma. The DICER1-mutant PDTC showed widely invasive growth confined to the thyroid parenchyma. Despite the widely invasive growth, the tumor lacked vascular invasion. Two DICER1 wild-type PDTCs had lymphocytic thyroiditis and another one had underlying follicular nodular disease and/or follicular adenomas. Three DICER1 wild-type PDTCs also had an associated differentiated thyroid carcinoma component with no high-grade features. No abnormal p53 expression (overexpression or global loss) was recorded in all tested tumors. Four patients had follow-up data with a mean follow-up time of 60.25 months (range: 18–86 months). One patient with no evidence of disease recurrence died of an unrelated cause after 18 months of the initial surgery, all remaining patients were alive with no distant metastasis at their last visit. Of the 4 patients with lymph node (LN) dissection, one DICER1 wild-type PDTC had recurrent nodal disease. During the follow-up period (72 months), no local recurrence or distant metastases was detected in the DICER1-mutant PDTC. Taken together all reported findings from earlier series, DICER1 mutations alone may not necessarily indicate dismal outcome in a subset of pediatric PDTCs. The occurrence of additional genomic alterations as discussed in some earlier reports may be contributing to tumor progression or aggressivity of pediatric PDTCs. The lack of vascular invasion in the current DICER1-mutant pediatric PDTC may also explain an indolent biologic outcome. The risk escalation of DICER1 mutations should integrate the status of additional genetic events and well-established pathologic variables in order to ensure predictive dynamic risk stratification in DICER1-mutant pediatric PDTCs. Additional studies are needed to corroborate the findings of this study and advance our knowledge in pediatric thyroid neoplasia.

Rosai-Dorfman disease (RDD) is a benign histiocytosis with unknown etiology. It generally occurs in cervical lymph nodes. Isolated central nervous system (CNS) RDD is very rare in the literature. We reported a case of no systemic involvement Rosai-Dorfmann which is rarely seen and shows CNS involvement by mimicking meningioma. A 32-year-old man presented with diplopia and a headache he has been experiencing for the past two years. His neurological examination showed left facial paresthesia, consistent with trigeminal nerve trace. Tendon reflexes were increased at the right side and the right plantar reflex was extensor. Brain magnetic resonance imaging demonstrated irregularly shaped, tumor-like lesions in the bilateral cerebellopontin area that were compressing pons. Rosai-Dorfman disease can be differentiated from IgG4 related disease (IgG4-RD) by its characteristic features such as plasma cell density and emperipolesis seen in its histopathology. Rosai-Dorfman disease can be confused with other diseases radiologically and histopathologically, especially the IgG4-RD, so be careful about differential diagnosis.

Abstract Context Noninvasive follicular thyroid neoplasm with papillary-like nuclear features (NIFTP) was introduced as a new entity replacing the diagnosis of noninvasive encapsulated follicular variant of papillary thyroid carcinoma (PTC). Significant variability in the incidence of NIFTP diagnosed in different world regions has been reported. Objective To investigate the rate of adoption of NIFTP, change in practice patterns, and uniformity in applying diagnostic criteria among pathologists practicing in different regions. Methods Two surveys distributed to pathologists of the International Endocrine Pathology Discussion Group with multiple-choice questions on NIFTP adoption into pathology practice and whole slide images of 5 tumors to collect information on nuclear score and diagnosis. Forty-eight endocrine pathologists, including 24 from North America, 8 from Europe, and 16 from Asia/Oceania completed the first survey and 38 the second survey. Results A 94% adoption rate of NIFTP by the pathologists was found. Yet, the frequency of rendering NIFTP diagnosis was significantly higher in North America than in other regions (P = .009). While the highest concordance was found in diagnosing lesions with mildly or well-developed PTC-like nuclei, there was significant variability in nuclear scoring and diagnosing NIFTP for tumors with moderate nuclear changes (nuclear score 2) (case 2, P < .05). Pathologists practicing in North America and Europe showed a tendency for lower thresholds for PTC-like nuclei and NIFTP than those practicing in Asia/Oceania. Conclusion Despite a high adoption rate of NIFTP across geographic regions, NIFTP is diagnosed more often by pathologists in North America. Significant differences remain in diagnosing intermediate PTC-like nuclei and respectively NIFTP, with more conservative nuclear scoring in Asia/Oceania, which may explain the geographic differences in NIFTP incidence.

Budd-Chiari syndrome (BCS) is an uncommon congestive hepatopathy caused by blockage of hepatic veins in the absence of cardiac/pericardial disorders as well as hepatic veno-occlusive disease [1]. In 75% of patients with BCS there is an underlying condition that predisposes to blood clotting [2]. More than one etiological factor may play a role in 25% of cases [2]. Coagulation problems mainly involving bleeding abnormalities are well recognized in AL amyloidosis while thrombosis is a less common feature [3]. Here, we report a rare case of AL amyloidosis complicated by BCS.

Myasthenia gravis (MG) is an immune-mediated disease frequently associated with thymic changes. Increased T helper 17 (Th17) cell activity and dysfunctional regulatory T (Treg) cells have been demonstrated in subgroups of MG. On the other hand, hypoxia-inducible factor 1 (HIF-1) has been shown to regulate the Th17/Treg balance by inducing Th17 differentiation while attenuating Treg development. To identify the underlying mechanisms of different thymic pathologies in MG development, we evaluated thymic samples from thymoma-associated myasthenia gravis (TAMG), MG with hyperplasia (TFH-MG) and thymoma without MG (TOMA) patients. Differential gene expression analysis revealed that TAMG and TFH-MG cells are associated with different functional pathways. A higher RORC/FOXP3 ratio provided evidence for Th17/Treg imbalance in TAMG potentially related to increased HIF1A . The hypoxic microenvironment in thymoma may be a driver of TAMG by increasing HIF1A . These findings may lead to new therapeutic approaches targeting HIF1A in the development of TAMG.

Hepatocellular carcinoma (HCC) is the most common primary malignancy of the liver, and extrahepatic metastases are typically found during disease progression. The incidence of adrenal metastasis (AM) from HCC in autopsy series ranges from 4.6 to 12.5%, and it is the second most common site of metastasis after the lungs. To date, there have been few reports of patients who underwent adrenalectomy for isolated AM from HCC after liver transplantation (LT). A woman aged 55 years was referred to our clinic for the evaluation of a right adrenal mass that was detected by abdominal ultrasonography at another center. She had undergone liver transplantation secondary to HCC and acute liver failure due to cryptogenic liver cirrhosis 138 months previously. She had been followed up for 5 years following LT after which she declined to continue with further follow-up. After radiologic and biochemical evaluation, she underwent adrenalectomy and the histopathologic examination revealed a 10 × 8 × 7-cm adrenal mass, which was considered to be an isolated AM from HCC. To our knowledge, this is the first case of isolated AM from HCC in the literature that was diagnosed 138 months after liver transplantation. Isolated AM from HCC after LT is rare and might be detected a long time after LT. Curative surgical resection of isolated metachronous AM from HCC in the absence of disseminated disease might provide for an acceptable disease-free period after adrenalectomy.

[LANGUAGE=”English”]Objective: Diffuse large B-cell lymphoma (DLBCL) is a biologically heterogeneous disease that is classified into germinal center B-cell (GCB) and non-GCB subtypes, which are prognostically different. The Hans algorithm is the most widely used tool based on CD10, BCL6, and MUM1 expression, but some cases with the non-GCB phenotype are still known to be misclassified. In this study, we investigate the extent to which GCET1, HGAL, and LMO2 protein expressions reflect GCB phenotype together with their roles in determining the GCB phenotype of DLBCL and their contributions to the performance of the Hans algorithm.Materials and Methods: Sixty-five cases of DLBCL-not otherwise specified, 40 cases of follicular lymphoma (FL), and 19 non-GC-derived lymphoma cases were included in this study. The DLBCL cases were grouped as CD10+ (Group A) or only MUM1+ (Group B), and the remaining cases constituted the intermediate group (Group C). GCET1, HGAL, and LMO2 expressions were evaluated.Results: In the FL group, GCET1, HGAL, and LMO2 were positive in 85%, 77.5%, and 100% of the cases, respectively. Among the non-GCderived lymphoma cases, all three markers were negative in cases of small lymphocytic lymphoma, plasmablastic lymphoma, peripheral T-cell lymphoma, and anaplastic large cell lymphoma. GCET1 and HGAL were negative in cases of marginal zone lymphoma (MZL) and mantle cell lymphoma (MCL). Two of the 3 MZL and 2 of the 4 MCL cases were positive for LMO2. In the DLBCL group, the number of cases with GCET1, HGAL, and LMO2 positivity was 18 (90%), 17 (85%), and 20 (100%), respectively, in Group A and 0 (0%), 2 (13.3%), and 2 (13.3%), respectively, in Group B. Considering these rates, when the cases in the intermediate group were evaluated, it was concluded that 13 cases typed as non-GCB according to the Hans algorithm may have the GCB phenotype.Conclusion: GCET1, HGAL, and LMO2 are highly sensitive markers for determining the germinal center cell phenotype and can increase the accuracy of the subclassification of DLBCL cases, especially for cases that are negative for CD10.[LANGUAGE=”Turkish”]Amaç: Diffüz büyük B-hücreli lenfoma (DBBHL), germinal merkez B (GMB) hücre ve non-GMB hücre olmak üzere prognostik olarak farklı alt grupları olan biyolojik olarak heterojen bir hastalıktır. CD10, BCL6 ve MUM1 ekspresyonuna göre yapılan Hans algoritması ile GMB olgularının bazıları yanlış sınıflandırılmaktadır. Çalışmamızda, immünohistokimyasal olarak GMB hücre belirteçleri olan GCET1, HGAL ve LMO2’nin folikül merkez hücre fenotipini ne derecede yansıttığını, DBBHL’de GMB hücre fenotipini belirlemedeki rolünü ve Hans algoritmasına katkısını araştırmaktayız.Gereç ve Yöntem: Altmış beş adet DBBHL-NOS, 40 adet foliküler lenfoma (FL) ve 19 adet non-GM kökenli lenfoma olgusu çalışmaya alındı. DBBHL olguları CD10+ (Grup A), sadece MUM1+ (Grup B) ve kalanlar ara grup (Grup C) olarak gruplandı. GCET1, HGAL ve LMO2 ekspresyonları değerlendirildi.Bulgular: FL grubunda, GCET1, HGAL ve LMO2 sırasıyla %85, %77,5, %100 olguda pozitif saptandı. Küçük lenfositik lenfoma, plazmablastik lenfoma, periferik T-hücreli lenfoma ve anaplastik büyük hücreli lenfoma olgularında 3 antikor da negatifti. Marjinal zon lenfoma (MZL) ve mantle hücreli lenfoma (MHL) olgularında GCET1 ve HGAL negatifken; LMO2, 2 MZL ve 2 MHL’de pozitif bulundu. DBBHL olgularında, Grup A’da GCET1, HGAL and LMO2 pozitif olgu sayısı sırasıyla 18 (%90), 17 (%85), 20 (%100) iken, Grup B’de sırasıyla 0 (%0), 2 (%13,3), 2 (%13,3) idi (p<0,001). Bu oranlar göz önüne alınarak ara gruptaki olgular değerlendirildiğinde, Hans algoritmasına göre non-GMB olarak tiplendirilen 13 olgunun GMB fenotipli olabileceği sonucuna varıldı.Sonuç: GCET1, HGAL ve LMO2, GMB fenotipini belirlemede duyarlı belirteçler olup, DBBHL’lerin (özellikle CD10 negatif olguların) doğru tiplendirilmesine katkı sağladıkları düşünülmüştür.

Mastocytosis is a very rare disorder and is divided into three prognostically distinct variants by World Health Organization: Cutaneous mastocytosis (CM), systemic mastocytosis (SM), and mast cell sarcoma or localized mast cell (MC) tumors. The wide range of complaints may cause patients to consult various clinics, with resulting mis- or underdiagnosis. Therefore, cooperation between different subspecialties is of paramount importance. In this article, we have compiled 104 adult mastocytosis cases diagnosed and followed in our Hematology and other clinics. 86 (82.7%) of 104 patients had systemic mastocytosis. Osteoporosis, disease-related complications, and secondary malignancies are important topics in this group. We know that indolent form has great survival. But smoldering or aggressive mastocytosis has a poor prognosis. CM and indolent SM have a significantly better prognosis compared to aggressive SM ( p  < 0.001). We found that the presence of more than 25% of mast cells in the bone marrow, the presence of concomitant marrow dysplasia, and the presence of disease-related complications affect survival ( p  < 0.001). In addition to the WHO classification, the IPSM scoring system is indicative of the prognosis in this rare disease.

Here, we present a rare case of primary ovarian lymphoma with bilateral ovarian involvement, which manifested with malignant hypercalcemia. A 39-year-old female patient presented to our hospital with lower abdominal pain. Hypercalcemia, anemia, and thrombocytopenia were observed during the examinations. Abdominal ultrasound revealed a mass lesion thought to be associated with an abscess or malignancy in both adnexal areas, as well as mass lesions in bilateral ovaries, along with paraaortic and paraaortocaval lymphadenomegaly on abdominal computed tomography (CT). In the positron emission tomography CT examination, increased fluorodeoxyglucose uptake (SUVmaxranging between 11.5 and 9.5) was observed in the lesions defined on abdominal CT. Bone marrow biopsy performed for thrombocytopenia was found to be compatible with diffuse large B-cell lymphoma (DLBCL). Although this case demonstrates that hypercalcemia can be observed in the course of DLBCL with bilateral ovarian involvement, which is a rare condition, it is also considered significant as it is inconsistent with most of the hypercalcemia mechanisms associated with malignancies described today.