A needle in the haystack: An unusual case presentation of ganglioneuroblastoma at a tertiary care center in Coastal Karnataka.

Key Clinical Message: This case report highlights the importance of recognizing and accurately diagnosing ganglioneuroblastoma, an uncommon variant of neuroblastic tumors in children. Ganglioneuroblastomas have diverse clinical and morphological presentations, and histopathological examination is paramount in guiding treatment decisions, especially in cases with ambiguous symptoms. Early detection is crucial, as the prognosis varies significantly based on the subtype and the presence of metastatic disease. Clinicians should maintain a high index of suspicion and utilize radiological examinations to promptly identify and treat these tumors. Abstract: Children are frequently affected by neuroblastic tumors, which grow from the sympathoadrenal lineage of the neural crest during its development. However, intermixed ganglioneuroblastomas are far less common within the same tumor spectrum, the diagnosis of which could become challenging amidst an unusual presentation. In our case report, we present a 4-year-old boy who had complaints of fever and difficulty in walking, with a supra-renal mass on ultrasound, which was diagnosed as ganglioneuroblastoma-intermixed type on histopathological examination. This report aims to contribute to the understanding of the diverse clinical and morphological spectrum of ganglioneuroblastomas and the importance of multidisciplinary collaboration and histopathological examination to enhance decision-making in such ambiguous scenarios.

Study of significance of bone marrow microvessel density in myeloproliferative neoplasms in correlation with CD34 blasts, mast cell count and fibrosis.

Background: Myeloproliferative neoplasms (MPN) are clonal hematopoietic stem cell diseases characterised by myeloid cell growth from one or more lineages. Angiogenesis, in contrast to other subtypes, plays a substantial role in the pathophysiology of primary myelofibrosis (PMF). Research expressing the correlation of microvessel density (MVD), blasts, fibrosis and mast cell count in MPN cases are rarely conducted. We aimed to study the significance of MVD in correlation with CD34 blasts, mast cells and fibrosis in bone marrow biopsies of MPN patients. Methods: The current research was a cross sectional study conducted on 66 cases diagnosed as MPN during a six-year period. This comprised of 32 chronic myeloid leukemia (CML), 31 PMF and three essential thrombocythemia (ET) cases. Routine staining along with reticulin stain to look for fibrosis and immunohistochemistry (IHC) using CD34 and mast cell tryptase (MCT) were performed. Results: We found increased MVD in PMF, when compared to CML and ET (p = 0.042). Further, mean MVD was observed to be increased with high blast counts (p = 0.036). On follow up, raised mean MVD was seen in those cases with relapse/deceased as compared to disease-free patients, which was highly significant (p = 0.000). Conclusions: Increased MVD score was mostly associated with PMF subtype among all the MPNs. Further, higher MVD was observed to be associated with increased blast count and poor prognosis. With angiogenesis playing a critical role in disease outcome, we now have drugs to regulate angiogenesis that are supported by contemporary research. However, further studies with larger cohorts to establish the theranostic role of MVD in MPNs is recommended.

A Quest for Accuracy: Evaluation of The Paris System in Diagnosis of Urothelial Carcinomas.

INTRODUCTION: Urine cytology is an important screening tool of patients for urothelial carcinoma (UC) and follow-up of patients with treated disease. Ease of procurement, cost-effectiveness, and lower turnaround time are the major advantages. OBJECTIVE: To compare current system of reporting (CSR) at our institute with The Paris System (TPS) and analyze utility of urine cytology based on TPS reporting in correlation with urine culture and histopathology. MATERIALS AND METHODS: One-year retrospective study of 90 cases was undertaken wherein cases presenting with painless hematuria and clinically suspicious of UC were included. Urine cytology slides were reviewed and reported with TPS guidelines. These findings were correlated with histopathological diagnosis and urine culture as indicated. Statistical analysis was done using SPSS 17 software. RESULTS: With TPS guidelines, 11.1% and 5.6% cases were reported as high-grade UC (HGUC) and low-grade urothelial neoplasm (LGUN), respectively. Suspicious for HGUC category included 17.8% of cases. The rate of reporting "atypical urothelial cells (AUC)" was significantly lower (11.1%) with TPS on comparison with CSR (16.7%). Histopathological correlation of positive predictive value for HGUC was better (100%) on using TPS when compared with CSR (64.3%). Among 11 cases with microbial growth on urine culture, 9.1% were reported as atypical. Sensitivity and accuracy of TPS in detecting UC were 83.3% and 86.52%, respectively. Both were higher when compared with CSR. CONCLUSION: In comparison to CSR, criteria of TPS limit the AUC category and enhance the sensitivity and accuracy of detecting HGUC. Adopting TPS for urinary cytology will ensure uniformity and accuracy of HGUC diagnosis.

Xanthomatous hypophysitis: A rare case report with review of literature.

Hypophysitis is classified into primary and secondary. Xanthomatous hypophysitis is one of the rare types of primary hypophysitis. A 55-year-old female presented with headache, vomiting, and blurring of vision. She also had endocrine dysfunction in the form of low serum T3, T4, and low cortisol levels. MRI scan showed a sellar expansile lesion suggestive of pituitary macroadenoma. Microscopy showed pituitary tissue replaced by inflammatory infiltrate made up of foamy histiocytes arranged in sheets along with lymphoplasmacytic infiltrate. Interspersed areas of fibrosis, hyalinization, few congested and sclerosed blood vessels were seen. Compressed residual pituitary tissue was identified at the periphery. Xanthomatous hypophysitis is a rare entity which can mimic as pituitary adenoma both clinically and radiologically. Accurate diagnosis at an early stage with postsurgical steroid therapy may help to prevent permanent pituitary damage.

Mantle Cell Lymphoma of the Palatine Tonsil: A Rare Case Report.

INTRODUCTION: Primary mantle cell lymphoma (MCL) of the palatine tonsil without involvement of the regional lymph nodes is rarely reported. CASE REPORT: A 52-year-old male presented with complaints of a change in his voice over 3 months, with neither sore throat nor fever. Physical examination revealed right-sided grade IV and left-sided grade III tonsillar enlargement with prominent vessels. The patient underwent bilateral tonsillectomy. An initial histopathological report revealed chronic tonsillitis on the left side and suspicion of atypical lymphoproliferative disorder on the right. Immunohistochemically, the neoplastic cells were positive for Bcl2, CD20, CD5 and Cyclin D1 and negative for CD10, Bcl6 and CD3; thus a diagnosis of MCL was confirmed. CONCLUSION: MCL of the tonsil is rare. The microscopic diagnosis is challenging as the picture is very similar compared with other types of small cell lymphomas. A detailed immunohistochemistry panel is required for an accurate diagnosis.

Histomorphologic Spectrum in Hyaline Vascular Variant of Castleman Disease.

INTRODUCTION: Castleman Disease (CD) is a rare lymphopro-liferative disorder with heterogenous clinical and pathological features. It is a rare disease with mention in the rare disease data of the orphanet. It can present as unicentric or multicentric disease. Hyaline vascular variant and plasma cell variant are the two pathological subtypes. Hyaline vascular variant accounts for nearly 80% to 90% of unicentric cases. Hyaline vascular subtype variant has follicular and stroma rich subtype. AIM: To study the histomorphologic spectrum of hyaline vascular variant of CD. MATERIALS AND METHODS: Retrospective cross-sectional, observational study was undertaken from the archival data between January 2009 and April 2015. Only ten cases of hyaline vascular CD were identified after studying the histomorphological characteristics. Both follicular and interfollicular changes were studied in detail. RESULTS: The age of presentation ranged from 17 years to 59 years. Seven out of 10 cases were female. Site of presentation included cervical, inguinal, retroperitoneal, intra-abdominal and axillary. Six cases showed predominant follicular change. Two cases were sclerotic subtype. Two cases showed both follicular and interfollicular changes in equal proportion. Distribution of follicles throughout the lymphnode was seen in eight cases. Uniform sized follicles seen in seven out of ten cases. Small germinal centre with lymphocyte depletion was one of the uniform features seen in all 10 cases. Numerous high endothelial vessels were seen in nine cases. Twinning of germinal centre was seen in two cases. All ten cases showed concentric rings of small lymphocytes. Lollipop pattern was relatively rare feature seen in only two cases. CONCLUSION: Hyaline vascular variant of CD has considerable morphologic variation with few consistent features seen in most of the cases.

Fraternal Twin Tumours of the Oesophagus and Stomach Presenting in the Advanced Stage - A Rare Case with a Fatal End.

Synchronous primary tumours of the aerodigestive tract presenting with different histologies is a very rare event. A case with such an association is presented here. A 50-year-old lady presented with symptoms of abdominal obstruction like abdominal pain, distention and vomiting since one month. Her radiological imaging and biopsies revealed a primary oesophageal squamous cell carcinoma and primary gastric adenocarcinoma. Western medical literature has shown the occurrence of multiple synchronous cancers; however, cases from India have seldom been reported. Early screening, correct diagnosis and appropriate plans of management are crucial to reduce the morbidity and mortality of such patients who bear the burden of not just one, but multiple primary cancers.

Clinicopathological profile of papillary carcinoma of thyroid: A 10-year experience in a tertiary care institute in North Karnataka, India.

CONTEXT: Papillary thyroid carcinoma (PTC) is the most common thyroid malignancy accounting for 80% of the thyroid cancers. Many histopathologic variants of PTC have been recognized, and few of these are of prognostic significance. The studies on clinicopathological features of PTC and its variants are so far seldom reported in India. AIM: The aim of the study was to study the percentage distribution of PTC among total thyroid specimens, the age and sex distribution of PTC, its histopathological features including frequency of nuclear findings, and various histological subtypes are also studied in detail. Methods: All cases of PTC diagnosed in our department from April 2003 to March 2013 formed the material for the study. The tissues were routinely processed and stained. On microscopic examination, tumors were classified according to 2004 WHO classification. RESULTS: PTC formed the predominant type of malignancy accounting to 71% of the total cases. Of these, about 75% of patients were in the second to fifth decade. Male to female ratio was 1:5.4. Other than the usual classic variant and follicular variant, we also found rare types such as clear cell variant, tall cell type, oncocytic type, and macrofollicular variant. Microscopically, nuclear overcrowding and ground glass nuclei were seen in more than 90% of cases. Nodular goiter, Hashimoto's thyroiditis, and follicular adenoma were associated lesions in some cases. CONCLUSION: PTC is the most common thyroid malignancy, and it can affect any age group though it presents mostly in the third to fourth decade of life. Recognition of histological subtype is crucial in patient prognosis.