Epitheloid hemangioendothelioma of the urinary bladder: A case report of a rare entity

Epithelioid hemangioendothelioma (EHE) is a rare, often multifocal, intermediate-grade vascular sarcoma arising from the endothelial cells. Their behavior is intermediate between hemangioma and conventional angiosarcoma. Their rarity and unpredictable clinical behavior result in our still limited understanding and treatment options for this variety of tumors. Molecular diagnostic tools like the presence of WWTR1-CAMTA1 fusion have been developed to aid this challenging diagnosis. There is no definitive consensus on the management of patients with this disease and no commonly accepted treatment strategies have been found to be effective, particularly in the metastatic setting. When possible, surgery with clear margins should be considered in case of localized disease. Here, we not only present such a rare case of EHE of the urinary bladder but also provide a comprehensive discussion on the present treatment modalities offered, as found through a thorough search of the published studies. Finally, more studies are required to establish a standard of care for this rare entity

HCC masquerading as gluteal mass: A rare presentation

Hepatocellular carcinoma (HCC) is the most common primary tumor of the liver. Extrahepatic metastasis occurs mostly through the hematogenous route and is seen in around one-third of patients with the common sites of involvement being the lungs, regional lymph nodes, bone, adrenal glands, and pancreas. Soft-tissue metastasis from HCC is an extremely rare condition. Here, we present a rare case of an elderly male, with HCC presenting as a soft-tissue mass in the gluteal region. We further provide a detailed discussion regarding the investigative approach used to arrive at the diagnosis and the treatment modalities offered. Case reports like this may offer insight into the possibilities of such unusual presentations and aid the clinician in his endeavor to the early diagnose and treat the patient.

CONCORDANCE: A real?world evidence study to evaluate the concordance of detecting epidermal growth factor receptor (EGFR) mutation by circulating tumor DNA* versus tissue biopsy in patients with metastatic non?small cell lung cancer

Background: Molecular tissue testing in non?small cell lung cancer (NSCLC) is done for the assessment of epidermal growth factor receptor (EGFR) mutation. EGFR mutation status is the basis for deciding the targeted treatment option for patients with metastatic NSCLC. The nonavailability of tissue samples and contraindications for biopsy pose a significant challenge. Hence circulating tumor DNA (ctDNA) by liquid biopsy can be a viable alternative for NSCLC patients. Methods: This study was conducted at 15 sites across India. EGFR mutation testing from plasma was done as part of the study at the central laboratory by the next?generation sequencing (NGS) method and EGFR mutation test results from tissue samples (done as part of routine practice) were recorded for all the patients. Results: Out of the total patients enrolled (N = 245) the majority (64.5% n = 158) were men. The median age of patients was 58.0 (range: 26�) years. The concordance between plasma and tissue testing was found to be 82.9% (95% confidence interval [CI]: 77.55 87.45). The sensitivity and specificity of NGS were 68.4% (95% CI: 56.92 78.37) and 90.1% [95% CI: 84.36 94.21) respectively. Plasma testing detected 1.2% (n = 3) and tissue sample testing detected 2.4% (n = 6) positive status of exon 20 T790M EGFR mutation. Out of the total number of patients enrolled 25 were tissue positive and plasma negative while 16 were plasma positive and tissue negative. Conclusions: This real?world study in Indian patients suggests that plasma testing for EGFR mutation analysis is a viable diagnostic option in newly diagnosed advanced/metastatic NSCLC patients. The noninvasive plasma procedure in patients without available/evaluable tumor sample may enable more patients to receive appropriate targeted therapies by providing clinicians with valuable insights into the patient抯 tumor mutation status. ClinicalTrials.gov Identifier: NCT03562819

Primary Merkel cell carcinoma of the oral mucosa in a young adult male: Report of a rare case.

Merkel cell carcinoma (MCC) is a highly aggressive neoplasm of skin with neuroendocrine differentiation. Primary MCC of the oral mucosa is exceedingly rare and even more unresponsive to therapy. A 15-year-old male presents with gradually increasing painless swelling in right side of the fl oor of mouth for 6 weeks. Computed tomography of head and neck region showed globular mass (4.6 cm × 1.7 cm) involving right side of the fl oor of mouth. Fine-needle aspiration from the upper deep cervical node suggested small round cell tumor. A trucut biopsy showed mass composed of trabeculae and nests of tumor cells with high N:C ratio, granular speckled chromatin, scanty to moderate amount of clear vacuolated cytoplasm. Cells were immunoreactive for cytokeratin-20, CD56, c-kit, CD99 and negative for p63, thyroid-transcription factor-1, CDX2, synaptophysin, neuron-specifi c enolase. Patient was started on chemotherapy with cyclophosphamide, doxorubicin and vincristine. The mass regressed in size and patient underwent wide local excision with pull-through approach. Patient is currently under combined chemoradiation regime and doing well.

Intra-cardiac angiosarcoma.

Intra-cardiac angiosarcoma is a challenging disease – difficult to diagnose early and an aggressive tumour to treat. Transoesophageal echocardiography (TEE) and cardiac computed tomography are the two most sensitive diagnostic tools. Though there are various multimodality treatment options available, the results are not impressive. We present a case of intra-cardiac angiosarcoma in a 62-year-old gentleman.