Parapharyngeal space schwannoma of hypoglossal nerve

Parapharyngeal schwannomas are rare benign neoplasms located in a difficult anatomical region. Most of them are asymptomatic and some presents late. Neurological deficit is a late finding, and it occurs only when the lesion is very large and compresses contiguous structures. Computed tomography (CT) guided fine needle aspiration cytology along with preoperative CT and magnetic resonance imaging can detect and diagnose it correctly and helps in proper planning and management. Total surgical excision is the treatment of choice. The approach is different as per the site, but trans-cervical approach is preferred. Recurrence is rare after complete excision. We are presenting a very rare parapharyngeal schwannoma arising from the hypoglossal nerve that was excised by trans-cervical approach without any complications and less morbidity as compared to other described approaches

Lacrimal sac rhinosporidiosis

Rhinosporidiosis is caused by the organism Rhinosporidium seeberi. It is a rare aquatic protistan parasite. Though more prevalent in Asiatic regions, cases have also been reported in European countries. In India, it mostly affects the southern part. Rhinosporidium seeberi most commonly affects the mucous membranes, but can also affect other structures including the larynx, trachea, skin, genitalia, lungs and rectum. The typical presentation is that of a pinkish mass which bleeds profusely. Isolated lacrimal sac rhinosporidiosis is very rare. Computed tomography scans and magnetic resonance imaging are helpful in diagnosis, but histopathological study along with Gomori methenamine silver, periodic acid-Schiff, and potassium chloride are required for confirmation. Its mainstay of treatment is surgery. Prognosis is excellent, but recurrence is not unusua

Central Nervous System Blast Crisis in Chronic Myeloid Leukemia on Imatinib Mesylate Therapy: Report of Two Cases

Chronic myeloid leukaemia is a chronic myeloproliferative disorder characterised by a reciprocal translocation between chromosomes 9 and 22 and thereby formation of the Philadelphia chromosome. Imatinib mesylate (STI-571) is a potent and selective inhibitor of BCR-ABL tyrosine kinase and has emerged as a treatment of choice in chronic myeloid leukaemia (CML) patients in chronic phase. It has shown activity in CML patients in the chronic phase or blastic phase. However there is poor penetration of the central nervous system (CNS) by the drug or its active metabolites. Therefore the CNS acts as a sanctuary site for malignant cells for CML patients treated with Imatinib. We report cases of two CML patients on Imatinib therapy, who were in haematological remission but developed CNS disease

GATA3 expression in clear cell adenocarcinoma of the lower urinary tract: a potential diagnostic pitfall

Background Clear cell adenocarcinoma of the lower urinary tract (CCACLUT) is a rare primary malignant neoplasm with heterogenous morphology. There is a paucity of data in the literature regarding its immunohistochemical profile. Methods The immunohistochemical features (extent and intensity) of a multinational cohort of CCACLUT were evaluated with comparison between clear cell adenocarcinoma of the female genital tract (CCACFGT, tissue microarray) and nephrogenic adenoma (NA). Results 33 CCACLUT (24 female, 9 male; mean age 59 years) were collected. CCACLUT most commonly arose from the urinary bladder (26/33, 78%), particularly from the trigone (10/33, 30.3%) followed by the urethra (8/33, 22%). All 12 NA cases were located at the urinary bladder, whereas the most common CCACFGT location was the ovary (29/56, 52%). None of the CCACLUT patients had, intestinal metaplasia, NA, or urothelial carcinoma. One patient had concurrent endometriosis of the sigmoid colon. Most frequently observed morphology in CCACLUT was papillary/tubulocystic (9/3; 27.3%), followed by papillary/tubular (6/33; 18.2%) and papillary/solid (5/33; 15.2%). GATA3 expression was significantly higher in CCACLUT (18/33, 54.5%) and NA (6/12, 50%), when compared to CCACFGT cases 6/56, 11.7%)(p = 0.001 and p = 0.022, respectively). The extent of GATA3 was significantly higher in CCACLUT group (19.2 +/- 16.6%) than the other groups (9.6 +/- 22.5% in NA and 2.6 +/- 9% in CCACFGT group) (p = 0.001). 4/33 patients (12.1) had weak, 10/33 patients (30.3%) had moderate, and 4/33 patients (12.1%) had strong GATA3 intensity in CCACLUT group. In NA group, one patient (8.3%, 1/12) had weak, one patient (8.3%, 1/12) had moderate and 4 patients (33.3%, 4/12) had strong GATA3 intensity. Most cases (CCACLUT 29/33, 88%; NA 11/12, 92%; CCACFGT 46/56, 82.1%) had positive Napsin A expression, by which CCACLUT had significantly more cases with Napsin A expression (p = 0.034). p63 was consistently negative in all cases (30/33 (91.9%) CCACLUT; 12/12 (100%) NA; 42/56 (75%) CCACFGT. Ki67 (MIB) proliferation index was significantly higher in CCACLUT group (54.6 +/- 21%) when compared to NA group (4.5 +/- 2.7%) and CCACFGT group (35.5 +/- 25.8%) (p = 0.001). Conclusion CCACLUT has consistent GATA3 expression, which may cause challenge in the diagnosis of urothelial carcinoma but can be used to distinguish CCACLUT from CCACFGT

Advances, recognition, and interpretation of molecular heterogeneity among conventional and subtype histology of urothelial carcinoma (UC): a survey among urologic pathologists and comprehensive review of the literature

Aims Urothelial carcinoma (UC) demonstrates significant molecular and histologic heterogeneity. The WHO 2022 classification has hinted at adding molecular signatures to the morphologic diagnosis. As morphology and associated molecular repertoire may potentially translate to choices of and response to therapy and relapse rate, broader acceptability of recognizing these key features among uropathologists is needed. This prompted an international survey to ascertain the practice patterns in classical/subtype UC among uropathologists across the globe. Methods and Results A survey instrument was shared among 98 uropathologists using SurveyMonkey software. Anonymized respondent data were analysed. The response rate was 85%. A majority were in concordance with the profiles of luminal (93%) and basal (82%) types. Opinion on the FGFR3 testing platform was variable. While 95% concurred that TERT promoter mutation is the key driver in UC, 72% had the opinion that APOBEC mutagenesis is the main signature in muscle invasive bladder cancer (MIBC). Uropathologists have divergent opinions on MIBC and ERCC2 mutations. Among the participants, 94% would quantify aggressive micropapillary and sarcomatoid histology, while 88% would reevaluate another transurethral resection of the bladder tumour specimen in nonmuscle invasive tumour with micropapillary, small cell, or sarcomatoid histology. A leading number agreed to specific molecular signatures of micropapillary (93%), plasmacytoid (97%), and small cell (86%) subtypes. Ninety‐six percent of participants agreed that a small‐cell component portends a more aggressive course and should be treated with neoadjuvant chemotherapy and 63% would perform HER2/neu testing only on oncologist's request in advanced tumours. Ninety percent agreed that microsatellite instability testing, although not a standard protocol, should be considered in young patients with upper tract UC. Eighty‐six percent agreed that UC with high tumour mutational burden would be a better candidate for immunotherapy. Conclusion In the era of precision medicine, enhanced understanding of molecular heterogeneity of UC will contribute to better therapeutic options, novel biomarker discovery, innovative management protocols, and outcomes. Our survey provides a broad perspective of pathologists' perceptions and experience regarding incorporation of histomolecular approaches to “personalize” therapy. Due to variable clinical adoption, there is a need for additional data using uniform study criteria. This will drive generation of best practice guidelines in this area for widespread and consistent clinical utility