Tertiary care center experience: An overview of primary mediastinal lymphomas.

BACKGROUND: Mediastinal lymphoma is uncommon, and only 10% of lymphomas occur primarily in the mediastinum. Very few Indian studies have discussed the frequency, presentation, and diagnostic approach of primary mediastinal lymphomas. AIMS: This study aims to detail the frequency and histological features of mediastinal lymphomas diagnosed in our Institute, discuss the close differentials, and compare the morphological and immunohistochemistry (IHC) features of primary mediastinal lymphomas. SETTINGS AND DESIGN: A retrospective observational study. MATERIAL AND METHODS: Fifty-nine cases of primary mediastinal lymphomas diagnosed over 5 years using hematoxylin and eosin (H and E)-stained and IHC slides. STATISTICAL ANALYSIS USED: Descriptive statistics. RESULTS AND CONCLUSIONS: Forty-five (76%) cases had predominant involvement of the anterior mediastinum; Hodgkin lymphoma, and non-Hodgkin lymphoma constituted 12 and 47 cases, respectively. T lymphoblastic lymphoma comprises the most common primary mediastinal lymphoma. There is a marked difference between the demographic profiles of cases with primary mediastinal lymphomas in India and worldwide. A meticulous histological examination to direct a limited panel of IHC markers can help arrive at the final diagnosis in settings where molecular and cytogenetic studies are not performed.

Pediatric and adolescent chronic myeloid leukemia: A follow-up study in Western India.

Background: Chronic myeloid leukemia (CML) is relatively rare in pediatric and adolescent age groups. The purpose of this study was to evaluate the clinical, hematopathological, and biochemical parameters of CML in pediatric and adolescent age groups, along with an assessment of the treatment response with first-line tyrosine kinase inhibitors (TKI) and its correlation with the prognostic scoring systems of adults. Materials and Methods: A retrospective study of 44 Breakpoint Cluster Region-Abelson leukemia virus (BCR-ABL1)-positive pediatric and adolescent CML cases registered at our hospital was done. The clinical and laboratory parameters were evaluated using hospital software. The treatment response was monitored and scoring was performed using mathematical calculations. Results: The mean age was 11.6 (±4.7) years. The median hemoglobin was 8.4 g/dL and 63.6% of the cases showed white blood cell (WBC) counts >250,000/µL. The average follow-up was 21 months. A total of 97.7 and 78.1% cases achieved complete hematological response (CHR) and molecular response, respectively, during the treatment course. The maximum number of patients had low Sokal and European treatment and Outcomes Study (EUTOS) scores. Seventy-five per cent of the cases achieved CHR at 3 months, while 73.6 and 78.6% CML-Chronic phase (CP) cases with low Sokal and EUTOS scores achieved CHR at 3 months, respectively. Conclusion: This study revealed that the CML cases in pediatric and adolescent age groups are normally present with higher WBC counts at the time of diagnosis. The association of the prognostic scoring system with treatment response was statistically insignificant. However, a larger cohort study is needed to determine the treatment response of TKI in children and adolescent CML and its correlation with the prognostic scoring systems.

The evolving role of molecular pathology in the diagnosis of salivary gland tumours with potential pitfalls.

Salivary gland tumors are diagnostically challenging owing to the morphological diversity within any tumor type and overlapping histomorphology and immunohistochemistry amongst different tumours. In past two decades, rapid progress has been made in the field of understanding the pathogenesis of these tumours with the discovery of many tumour specific translocations and rearrangements. This includes CRTC1-MAML2 and CRTC-MAML2 in mucoepidermoid carcinoma, MYBNFIB and MYBL1-NFIB fusions in adenoid cystic carcinoma, PLAG1 and HMGA2 in pleomorphic adenoma, ETV6-NTRK3 in secretory carcinoma, NR4A3 rearrangements in acinic cell carcinoma, PRKD1 mutations in polymorphous adenocarcinoma and EWSR1-ATF1 in clear cell carcinoma. This review is a lens for progress made till date in the molecular pathology of salivary gland tumours with a special focus on their role as diagnostic tools and implications on clinical management of the patient as prognostic and predictive markers.

Giant Cutaneous Horn: Is It a Tip of An Iceberg? Two Case Reports and a Review of Literature.

Cutaneous horns are uncommon lesions which consist of proliferation of keratotic material resembling that of an animal horn. This lesion most commonly occurs in sun-exposed areas, particularly the face, ear, nose, forearms and dorsum of hands. The purpose of the study was to highlight the atypical presentation of squamous cell carcinoma as a giant cutaneous horn along with the review of literature. Case details were obtained from hospital records and clinical and histopathological findings were noted. Herein we report two cases of giant cutaneous horn associated with an underlying carcinoma presenting at an uncommon site. Both the patients were adults and had a chronic history of a skin lesion over the affected sites. Wide local excision was performed in both cases and histopathological examination confirmed the diagnosis of a cutaneous horn with a well differentiated squamous cell carcinoma at the base of the horn. Cutaneous horns are commonly associated with benign lesions and rarely with malignant conditions. It is imperative to biopsy the base of the horn to rule out any underlying malignancy. Proper surgical excision with adequate margins is the preferred treatment in case of an underlying malignancy.