Angioleiomyoma of the Tongue- A Rare Peculiar Entity

Angioleiomyomas (ALs) are benign mesenchymal tumors which were considered to be of smooth muscle origin, until the latest WHO classification (2016) of soft tissue tumors, in which it was reclassified as a tumor of perivascular origin. The majority of ALs occurs in the uterus, gastrointestinal tract and skin; AL of the oral cavity is infrequent, and AL of the tongue is particularly rare. These usually appear as solitary, slow-growing masses and are seldom observed in oral cavity. Here, we present a case of a fifty four year old male with a lesion on the left lateral border of the tongue, clinically mimicking a neurofibroma/ schwannoma. In this article we highlight the importance of scrupulous and detailed histopathological analysis and differential immunohistochemical studies which are essential for the diagnosis of AL from other spindle cell lesions.

Tumors diagnosed as cerebellar glioblastoma comprise distinct molecular entities

In this multi-institutional study we compiled a retrospective cohort of 86 posterior fossa tumors having received the diagnosis of cerebellar glioblastoma (cGBM). All tumors were reviewed histologically and subjected to array-based methylation analysis followed by algorithm-based classification into distinct methylation classes (MCs). The single MC containing the largest proportion of 25 tumors diagnosed as cGBM was MC anaplastic astrocytoma with piloid features representing a recently-described molecular tumor entity not yet included in the WHO Classification of Tumours of the Central Nervous System (WHO classification). Twenty-nine tumors molecularly corresponded to either of 6 methylation subclasses subsumed in the MC family GBM IDH wildtype. Further we identified 6 tumors belonging to the MC diffuse midline glioma H3 K27 M mutant and 6 tumors allotted to the MC IDH mutant glioma subclass astrocytoma. Two tumors were classified as MC pilocytic astrocytoma of the posterior fossa, one as MC CNS high grade neuroepithelial tumor with BCOR alteration and one as MC control tissue, inflammatory tumor microenvironment. The methylation profiles of 16 tumors could not clearly be assigned to one distinct MC. In comparison to supratentorial localization, the MC GBM IDH wildtype subclass midline was overrepresented, whereas the MCs GBM IDH wildtype subclass mesenchymal and subclass RTK II were underrepresented in the cerebellum. Based on the integration of molecular and histological findings all tumors received an integrated diagnosis in line with the WHO classification 2016. In conclusion, cGBM does not represent a molecularly uniform tumor entity, but rather comprises different brain tumor entities with diverse prognosis and therapeutic options. Distinction of these molecular tumor classes requires molecular analysis. More than 30% of tumors diagnosed as cGBM belong to the recently described molecular entity of anaplastic astrocytoma with piloid features.

Histo-morphologic Spectrum of Non-Hodgkin Lymphoma According to WHO Classification 2016: A Cross-Sectional Study

Background: Geographic variations with regard to incidence and histological subtypes are known to occur in Non-Hodgkin lymphoma (NHL). This study was aimed to see the incidence and subtypes of NHL in a group of Bangladeshi population.Methods: This cross sectional study was carried out in Armed Forces Institute of Pathology (AFIP) Bangladesh, from 1st April 2017 to 31st March 2018. All nodal and extranodal tissues which were morphologically diagnosed as NHL were included in the study and immunohistochemistry was done for sub-classification according to WHO classification 2016. Bone marrow trephine biopsy samples were excluded.Results: Total cases: 106, mean age: 48.5 years ± 18.5 (range 2Y 9 M –82 years), male-female ratio: 2.2:1. Total subject of B cell Lymphoma (BNHL): 83 (78.3%) and T cell lymphoma (TNHL): 23 (21.7%). Among BNHL, total subjects of diffuse large B cell lymphoma (DLBCL) 50 (60%), follicular lymphoma 9 (11%), marginal zone lymphoma 8 (10%), small cell lymphoma and mantle cell lymphoma 5 (6%) each, Burkitt lymphoma 4 (5%) and B cell lymphoblastic lymphoma 2 (1.89%) in number. Among TNHL peripheral T-cell lymphoma NOS 11 (48%), anaplastic large cell lymphoma (ALCL) 5 (22%), T cell lymphoblastic lymphoma 4 (17%), and angio-immunoblastic T cell lymphoma 3 (13%) in number. Among 5 ALCL, 4 ALK positive and 1 ALK negative. Number of primary extra-nodal NHL were 29 (27%) with most common involved organ system being GIT and most common histological subtype being DLBCL.Conclusion: Quite similar pattern of age range with mean age, male to female ratio, subtypes and extra nodal NHL distribution prevailing in our subcontinent is found in our population with subtle increased incidence of TNHL indicating the necessity of further large epidemiological study.Birdem Med J 2018; 8(3): 215-222

Pre-operative chemotherapy (ctx) in plasmacytoid urothelial carcinoma (PUC).

522 Background: PUC is characterized by E-cadherin loss, diffuse growth pattern and aggressive natural history. Management of this entity is controversial. This retrospective study aims to evaluate the impact of PUC histology on clinical outcomes with ctx compared to UC-NOS. Methods: Consecutive cases of nonmetastatic PUC were identified as either (1) localized disease (LD: cT2-3 cN0) or (2) locally-advanced (LA: fixed bladder or radiographic nodal disease). All cases were reviewed by a GU pathologist to confirm PUC histology per WHO classification (2016). A separate cohort of neoadjuvant(NAC)-treated UC-NOS (Tully et al, ASCO GU 2014) served as comparator for clinical outcomes. Kaplan-Meier estimates and logrank test were used for analysis of recurrence free (RFS) and overall survival (OS) defined from date of cystectomy (RC). Results: Between 2000 and 2017, eighty one PUC were identified, with median age of 65 years (range: 22 - 84) and 65% male. Thirty patients (pts) had up-front RC; 51 pts had ctx. In the former group, all pts had LD but upstaging was seen on post-op pathology: 63% pT3, 17% pT4 and 37% pN+. Among ctx pts, 33/51(65%) were LD and had NAC: 70% had ctx doublet and 24% triplet; 82% had 4 cycles and 18% had up to 6 cycles. Response ( < pT2 pN0) was seen in 7/33(22%), four (12%) of which had no residual disease (pCR). Six RC were aborted after NAC for pT4b disease. NAC response was not associated superior RFS (NR vs. 17.3 months; HR 0.52 95% CI 0.18 – 1.47, p = .218) or OS (41.3 vs. 25.5 months; HR 0.65 95% CI 0.23 – 1.87, p = .428). Compared to UC-NOS, PUC responders had significantly inferior outcomes (table). In LA cohort, majority had triplet chemotherapy (72%) and up to 6 cycles (89%). Response was seen in 4/18(22%) pts, with two pCR (11%). Five RC were aborted for pT4b disease. Median RFS and OS were 5.8 and 10.5 months, respectively, with 19% alive at 2-years. Most common sites of recurrence/progression for PUC were peritoneal (42%) and locoregional invasion (26%). Conclusions: Outcomes for PUC are poor with conventional NAC-RC approach. [Table: see text]