A young male with non-resolving consolidation and hepatosplenomegaly.

ABSTRACT: Non-resolving consolidation refers to the persistence of radiographic abnormalities beyond the anticipated timeframe. Conditions such as infection, malignancy, inflammatory disorders, and connective tissue diseases can all manifest as non-resolving consolidation. We share a clinicopathological case study involving a 30-year-old male who exhibits non-resolving consolidation accompanied by hepatosplenomegaly.

Quantification of Radiomics features of Peritumoral Vasogenic Edema extracted from fluid-attenuated inversion recovery images in glioblastoma and isolated brain metastasis, using T1-dynamic contrast-enhanced Perfusion analysis.

The peritumoral vasogenic edema (PVE) in brain tumors exhibits varied characteristics. Brain metastasis (BM) and meningioma barely have tumor cells in PVE, while glioblastoma (GB) show tumor cell infiltration in most subjects. The purpose of this study was to investigate the PVE of these three pathologies using radiomics features in FLAIR images, with the hypothesis that the tumor cells might influence textural variation. Ex vivo experimentation of radiomics analysis of T1-weighted images of the culture medium with and without suspended tumor cells was also attempted to infer the possible influence of increasing tumor cells on radiomics features. This retrospective study involved magnetic resonance (MR) images acquired using a 3.0-T MR machine from 83 patients with 48 GB, 21 BM, and 14 meningioma. The 93 radiomics features were extracted from each subject's PVE mask from three pathologies using T1-dynamic contrast-enhanced MR imaging. Statistically significant (< 0.05, independent samples T-test) features were considered. Features maps were also computed for qualitative investigation. The same was carried out for T1-weighted cell line images but group comparison was carried out using one-way analysis of variance. Further, a random forest (RF)-based machine learning model was designed to classify the PVE of GB and BM. Texture-based variations, especially higher nonuniformity values, were observed in the PVE of GB. No significance was observed between BM and meningioma PVE. In cell line images, the culture medium had higher nonuniformity and was considerably reduced with increasing cell densities in four features. The RF model implemented with highly significant features provided improved area under the curve results. The possible infiltrative tumor cells in the PVE of the GB are likely influencing the texture values and are higher in comparison with BM PVE and may be of value in the differentiation of solitary metastasis from GB. However, the robustness of the features needs to be investigated with a larger cohort and across different scanners in the future.

18F-Labeled WBC PET/CT Scan in a Case of Recurrent Glioblastoma Multiforme, Presented as Pyrexia of Unknown Origin.

ABSTRACT: Neoplastic causes account for approximately 10% to 20% cases of PUO (pyrexia of unknown origin). The mechanisms by which malignancies induce fever are not fully understood. The release of pyrogenic cytokines either directly from tumor cells or from macrophages responding to tumor are likely to play a major role, which acts on the hypothalamus, causing a change in the thermostatic set point. We present a case of recurrent glioblastoma multiforme, who presented with PUO. 18F-FDG-labeled leukocyte PET/CT scan done for localization of infective focus demonstrated significant tracer accumulation at the periphery of the recurrent brain lesion. Subsequent excisional biopsy from the lesion was suggestive of noninfected recurrent glioblastoma multiforme.

Role of intra-tumoral vasculature imaging features on susceptibility weighted imaging in differentiating primary central nervous system lymphoma from glioblastoma: a multiparametric comparison with pathological validation.

PURPOSE: Primary objective of this study was to retrospectively evaluate the potential of a range of qualitative and quantitative multiparametric features assessed on T2, post-contrast T1, DWI, DCE-MRI, and susceptibility-weighted-imaging (SWI) in differentiating evenly sampled cohort of primary-central-nervous-system-lymphoma (PCNSL) vs glioblastoma (GB) with pathological validation. METHODS: The study included MRI-data of histopathologically confirmed ninety-five GB and PCNSL patients scanned at 3.0 T MRI. A total of six qualitative features (three from T2 and post-contrast T1, three from SWI: thin-linear-uninterrupted-intra-tumoral-vasculature, broken-intra-tumoral-microvasculature, hemorrhage) were analyzed by three independent radiologists. Ten quantitative features from DWI and DCE-MRI were computed using in-house-developed algorithms. For qualitative features, Cohen's Kappa-interrater-variability-analysis was performed. Z-test and independent t-tests were performed to find significant qualitative and quantitative features respectively. Logistic-regression (LR) classifiers were implemented for evaluating performance of individual and various combinations of features in differentiating PCNSL vs GB. Performance evaluation was done via ROC-analysis. Pathological validation was performed to verify disintegration of vessel walls in GB and rim of viable neoplastic lymphoid cells with angiocentric-pattern in PCNSL. RESULTS: Three qualitative SWI features and four quantitative DCE-MRI features (rCBVcorr, Kep, Ve, and necrosis-volume-percentage) were significantly different (p < 0.05) between PCNSL and GB. Best diagnostic performance was observed with LR classifier using SWI features (AUC-0.99). The inclusion of quantitative features with SWI feature did not improve the differentiation accuracy. CONCLUSIONS: The combination of three qualitative SWI features using LR provided the highest accuracy in differentiating PCNSL and GB. Thin-linear-uninterrupted-intra-tumoral-vasculature in PCNSL and broken-intra-tumoral-microvasculature with hemorrhage in GB are the major contributors to the differentiation.

18F-FDG PET/CT in Bilateral Breasts Metastasis From Ovarian Carcinoma.

ABSTRACT: Ovarian carcinomas generally metastasize within the peritoneal cavity due to exfoliation of malignant cells from primary tumor. Metastasis to the breasts is an unusual event and may mimic primary neoplastic disease. Usually, breast metastasis presents as a single isolated, well-circumscribed soft tissue lesion, and serous papillary carcinoma is the most common type of ovarian tumor that can metastasize to the breast. Concurrent bilateral breast metastasis is rare event. We present a follow-up case of metastatic carcinoma ovary, demonstrating FDG-avid soft tissue density masses in the bilateral breast parenchyma along with bilateral axillary lymphadenopathy, biopsy of which revealed metastatic deposits from carcinoma ovary.

A Rare Case of Isolated Intraventricular Primary Central Nervous System Lymphoma in an 85-Year-Old Man.

Primary CNS lymphoma (PCNSL) is rare malignant B cell lymphoid tumor of brain which predominantly occurs in supratentorial region in periventricular location. Majority of PCNSL are of DLBCL type and idiopathic in etiology. Here we are reporting a case of primary CNS lymphoma, DLBCL involving extremely uncommon intraventricular location. Central neurocytoma, subependymal giant cell astrocytoma, choroid plexus tumors and meningiomas are the common diagnosis at this site. Aim of reporting this case is to bring awareness of unusual intraventricular location of primary CNS lymphoma which should be kept in mind before considering gross total excision of lesion.

Secretory carcinoma of salivary gland origin: A recently established masquerader.

Secretory carcinoma (SC) of the salivary gland is considered to be a low-intermediate grade tumor with the potential of locally aggressive behavior. This tumor is similar both genetically (ETV6-NTRK3 fusion) and histologically to secretory carcinoma of the breast. We intent to share our experience of four cases of SC in terms of clinical behavior, pathological features, and treatment outcome. Medical records of four cases of SC were retrieved and analyzed for clinical presentation, surgical treatment, and outcome. Pathological data was reanalyzed along with immunohistochemistry. Out of the four identified SC cases, three were men. Two lesions originated from the parotid gland and two from minor salivary glands. Two of these cases exhibited features of locally aggressive pattern. SC of salivary gland origin has distinct histological and immunohistochemical features apart from the characteristic genetic translocation and fusion. Surgery with or without adjuvant radiotherapy is the treatment of choice. To establish the biological behavior of this tumor, larger case series with long-term follow-up is desirable.

Tectal Rosette-Forming Glioneuronal Tumor - A Case Report Focusing on a Possible Role for Radiotherapy in Inoperable Tumors.

BACKGROUND: Rosette-forming glioneuronal tumor (RGNT) is a rare and distinctive glioneuronal tumor. Although surgical excision is considered the standard treatment for these slow growing WHO Grade I tumors, gross-total resection is achieved in less than 50% of RGNTs due to its localisation amidst vital structures. With very few cases with long term follow-up reported, there is limited knowledge of the natural clinical course and the role of radiotherapy in inoperable RGNTs. CASE DESCRIPTION: A previously well 26-year old male, presented with long standing headache, increasing gait instability and fainting episodes. Imaging revealed a tectal plate mass with hydrocephalous. An endoscopic third ventriculostomy and biopsy was done, revealing RGNT. He received radiotherapy with a curative intent. The patient remained neurologically stable for 4 years. Follow-up imaging done after 4 years showed decrease in tumor size. CONCLUSIONS: The current case highlights a role for radiotherapy in RGNTs occurring in surgically challenging sites.

An institutional review of 10 cases of spinal hemangiopericytoma/solitary fibrous tumor.

BACKGROUND: Spinal hemangiopericytoma is very rare tumors with only a few case reports and one case series. We have treated ten patients between 2004 and 2017 and, thus, present a retrospective review of our patients with a focus on clinical presentation, radiological features, management, pathology, and outcome. MATERIALS AND METHODS: Histopathological data were reviewed in all the cases and clinical and follow-up details were collected from data available in our department. RESULTS: There were five males and five females, including one pediatric patient. The mean age of the patients was 34.7 years (Range 12-52 years). Dorsal, cervical, and lumbar spine involvement were found in five, four, and one patient, respectively. Intradural extramedullary tumor was the most common tumor and all patients presented motor weaknesses. Gross total resection of the tumor was done in seven patients and six patients received postoperative radiotherapy. Histopathology showed anaplastic tumor in two cases with high MIB-1 labelling index. Most patients were positive for CD34, vimentin, mic-2, and bcl-2. While the seven patients who underwent gross total resection improved significantly and were self-ambulatory in the follow-up period, two patients who underwent subtotal resection expired due to tumor metastasis. CONCLUSION: Spinal hemangiopericytoma is a very rare tumor. We present a series of cases treated at our institute for the same. Gross total resection is the goal and radiotherapy should be given in case of residual tumor or high-grade tumors. Prognosis is good after gross total excision and functional recovery can be expected in most patients.

Cytomorphology of hepatoblastoma with histological correlation and role of SALL4 immunocytochemistry in its diagnosis, subtyping, and prognostication.

BACKGROUND: Hepatoblastoma (HB) is the most common malignant pediatric liver tumor, and cytology material is often the only tissue available for evaluation before definitive therapy. Subcategorization of HB based on cytomorphological features thus carries an important role in its prognostication. Spalt-like transcription factor 4 (SALL4), a marker of embryonic stem cells that is also found in the fetal liver, is reactivated in certain liver tumors. Limited studies have evaluated its role in HB. This study was aimed at evaluating the cytomorphological features of HB and assessing the utility of SALL4 immunocytochemistry (ICC) in its subtyping and prognostication. METHODS: Pretherapy fine-needle aspiration smears from patients diagnosed with HB over a period of 9 years were retrieved. Aspirates were subclassified on the basis of the cytomorphology and were correlated with the histology wherever it was available. ICC for SALL4 was performed in 33 cases, and nuclear staining was considered positive. RESULTS: A total of 53 HB cases were included with 30 available postchemotherapy resection specimens. All the patients were diagnosed as epithelial HB on cytology, and the cases were subclassified as pure fetal (9 of 53), pure embryonal (2 of 53), or combined epithelial HB (42 of 53). There was good concordance between cytology and histology for subtyping. SALL4 immunostaining displayed strong and diffuse nuclear positivity in the embryonal component while focal and weak to negative staining in fetal cells. CONCLUSIONS: Fine-needle aspiration cytology serves as a rapid and effective tool for a correct diagnosis of HB before the implementation of chemotherapy, and SALL4 may serve as a useful diagnostic and prognostic marker.

Evolution of epidermoid cyst into dermoid cyst: Embryological explanation and radiological-pathological correlation.

Intracranial dermoid and epidermoid cysts are usually considered to be two different entities in the radiological and surgical literature. Epidermoid cysts are classically off midline in location, isointense to cerebrospinal fluid on T1 and T2-weighted images and have restricted diffusion, whereas dermoid cysts are classically midline in location, have T1-hyperintense regions due to the presence of fat and show facilitated diffusion. We report a case of radiological epidermoid cyst in baseline imaging, which evolved into a radiological dermoid cyst over time, and explain this unique occurrence with a review of the embryology and histopathogenesis of these cysts.

Paediatric diffuse leptomeningeal tumor with glial and neuronal differentiation harbouring chromosome 1p/19q co-deletion and H3.3 K27M mutation: unusual molecular profile and its therapeutic implications.

Diffuse leptomeningeal glioneuronal tumor (DL-GNT) is a newly introduced tumor entity of uncertain prognosis characterised by a primary diffuse leptomeningeal growth pattern, oligodendroglial-like morphology and dual glial/neuronal differentiation. Predominantly occurring in children, these tumors present as chronic meningitis and mimic infectious/inflammatory diseases. They are surgically challenging tumors with a high incidence of delayed morbidity and mortality despite low-grade histology. Their molecular genetic profile is not fully elucidated and few reports have identified chromosome 1p and 19q deletions, and BRAF alterations. We present a rare instance of a DL-GNT in a 13-year-old female who presented with slowly progressive and sequential neurological deficits over a 12-month duration. Imaging showed leptomeningeal thickening and spinal lesions. Biopsy from the spinal mass showed histomorphological features characteristic of DL-GNT. Further molecular analysis revealed 1p and 19q co-deletion and H3K27M mutation, while no mutation were identified in IDH, TERT, or BRAF genes. Patient died 4 months after diagnosis. Only one previous case of DL-GNT has been reported to harbour H3K27M mutation. Although H3K27M mutations have been described in rare examples of low-grade glial and glioneuronal tumors, whether DL-GNTs with H3K27M represent a rare growth pattern of the aggressive H3K27M-mutant diffuse midline gliomas needs further clarification.

Pigmented Ganglioglioma of the Cerebellum: Case Report and Review.

BACKGROUND: Gangliogliomas (GGs) are rare intra-axial tumors. Cerebellar seizures caused by GGs have been described only rarely. Pigmented neural cell tumors are well described in the literature but are infrequent, especially when presenting as primary neuroepithelial tumors. Only 5 cases of pigmented GG have been reported previously, including 4 in the pediatric population. CASE DESCRIPTION: A 17-year-old female presented to us with cerebellar seizures, which resolved after tumor excision. Histopathological examination revealed a pigmented GG. CONCLUSIONS: We present the sixth documented case of a pigmented ganglioglioma, the first such case reported in cerebellar location, associated with a rare presentation of cerebellar seizures.

A rare case of paediatric primary central nervous system lymphoma treated with high-dose methotrexate and rituximab-based chemoimmunotherapy and whole brain radiotherapy followed by tumour bed boost with three-dimensional conformal radiation technique.

BACKGROUND: Primary central nervous system lymphomas (PCNSL) are rare in the paediatric population. CLINICAL CASE: A 12-year-old boy presented to our clinic with complaints of multiple episodes of generalised tonic-clonic seizures for 1 year and gradual loss of vision in both eyes for 3 months. Baseline magnetic resonance imaging (MRI) of the brain showed a large (7.2 × 7 cm) enhancing soft tissue lesion in the right frontal lobe causing mass effect and midline shift. With a radiological diagnosis of supratentorial primitive neuroectodermal tumour, he underwent subtotal resection of tumour. The post-operative histopathology revealed diffuse large B cell lymphoma (DLBCL). Systemic lymphoma workup was essentially normal. He received five cycles of chemoimmunotherapy with rituximab, high-dose methotrexate (HDMTX), vincristine and procarbazine and had complete radiological response (CR). This was followed by whole brain radiotherapy (WBRT) to a dose of 36 Gy in 20 fractions and sequential tumour bed boost to a dose of 9 Gy in 5 fractions by three-dimensional conformal technique. Subsequently, he received two cycles of consolidation chemotherapy with high-dose cytarabine. At completion of treatment, 3 and 6 months thereafter, MRI brain showed CR. At last follow-up visit, 13 months from the date of diagnosis, he was disease-free and asymptomatic with the exception of dimness of vision in both eyes due to long-standing bilateral optic atrophy. CONCLUSION: This report highlights the fact that paediatric PCNSL may be effectively treated by a combination of HDMTX and rituximab-based chemoimmunotherapy followed by consolidation with conformal WBRT and tumour bed boost. Lack of awareness of this rare entity may lead to diagnostic delay and potential ramifications as exemplified by chronic atrophic papilloedema and visual loss in the illustrative case.

Immunohistochemical and molecular genetic study on epithelioid glioblastoma: Series of seven cases with review of literature.

Epithelioid glioblastoma (e-gbm) is a recently described variant of glioblastoma (GBM) which is associated with short survival and now added as a provisional entity to WHO 2016 classification of CNStumors. About half of these tumors show characteristic BRAF-V600E mutation. However, unlike conventional GBMs, e-gbm lack specific diagnostic and prognostic markers. Hence, we aimed to molecularly characterize these tumors. An extensive review of literature was performed.In a multi-institutional effort, all the cases of glioblastoma of year 2017 were reviewed. Cases with predominant epithelioid morphology were analysed. Seven cases of e-gbm (adults:4 and pediatric: 3) were identified. Duration of symptoms varied from 2 weeks to one month. Radiologically, all cases were supratentorial, contrast enhancing with solid and cystic appearance. Majority of the cases were immunopositive for GFAP (71%), EMA (71%), S100 (71%) and vimentin (85%). All the cases showed ATRX, INI-1 and H3K27me3 expression. BRAFV600Emutation was seen in 28% of cases. TERT mutation was seen in 40% cases, while one case showed EGFR amplification. H3F3A mutations and PTEN deletions were seen in none. Although e-gbms are rare, epithelioid morphology of a CNS tumor in a young adult or children with areas of necrosis needs thorough histomorphological and genetic workup.

Tuberculosis of the cervix: An uncommon cause of vaginal discharge in a post-menopausal woman.

Discharge per vaginum is a common symptom of a variety of gynaecological diseases. Among post-menopausal women, atrophic vaginitis, cervicitis and cervical carcinoma are common causes of this symptom. We present a 64-year-old woman who had foul-smelling discharge per vaginum for the past 1 year. Per speculum examination revealed an unhealthy-looking cervix and white discharge. On bi-manual examination, the cervix was flush with the vagina. There was no obvious growth felt, the exact uterine size could not be ascertained and the finger was stained with thick discharge. A biopsy of the cervix showed epithelioid cell granulomas and a diagnosis of tuberculosis was made. The patient responded to antitubercular therapy. Tuberculosis of the cervix may be a rare cause of foul- smelling discharge per vaginum in post-menopausal women but there should be a high index of suspicion of this condition, especially in areas where tuberculosis is common. We report this post-menopausal woman owing to the rarity of tuberculosis of the cervix mimicking a gynaecological malignancy.

Pancreatic heterotropia in wall of extra-hepatic choledochal cysts: A retrospective analysis of thirteen of such cases from north India.

INTRODUCTION: Heterotopic pancreas (HP) has rarely been identified in the wall of choledochal cyst (CC). METHODS: Retrospectively we screened 200 excised specimens of CC received at our Institute over a period of last eight years and looked for presence of HP rests in them. All the specimens were processed in their entirety. RESULT: HP was identified in the wall of 13 (6.5%) CCs, out of which 11 were Heinrich Type 2, and two were Heinrich Type 1. In half of the cases peribiliary mucous glands were observed intermingled with the HP rests. Features of chronic fibrosing pancreatitis were identified in these rests, with ulceration of overlying cyst lining. CONCLUSIONS: HP rests in the wall of CC though rare; their coexistence with peribiliary glands may possibly indicate their common embryonic origin. As a common site of inflammation, HP rest may be one of the common causes of CC.

Intrahepatic biliary cystadenoma with ciliary metaplasia: Report of a rare morphological variant.

Histological diagnosis of biliary cystadenoma is based on the type of epithelial cells lining the cyst and radiological features. The cyst-lining cells are not usually ciliated. We herein report a very rare example of an intrahepatic biliary cystadenoma with ciliated epithelial lining, which had taken us away from this diagnosis toward an intrahepatic foregut duplication cyst. Radiologically, also the lesion was deceptive, and a possibility of hydatid cyst was considered. However, immunohistochemical workup finally led us to this diagnosis. This report would document this rare morphological variant, which may pose diagnostic difficulty.