Epithelioid angiosarcoma of the pericardium: Case report of the challenges in the management of a rare malignancy with poor prognosis.

ABSTRACT: A primary epithelioid angiosarcoma of the pericardium is a rare soft tissue malignancy. This report describes a young adult woman who presented with progressive dyspnea and pericardial effusion. She was found to have pericardial mass on imaging along with extensive myocardial infiltration. The biopsy of the mass revealed epithelioid angiosarcoma, which was CD34 and CD31-immuno-positive. Due to unresectable disease, she was given a trial of immunotherapy followed by conventional chemotherapy. She showed partial response on interim assessment, but progressed soon after the completion of six cycles and succumbed to her rapidly progressive illness within nine months of diagnosis. This report discusses the diagnostic and therapeutic challenges faced while managing this disease of aggressive tumor biology.

To compare neoadjuvant concurrent chemo-radiotherapy followed by surgery and neoadjuvant chemotherapy followed by surgery in carcinoma esophagus patients: A single institutional study in the Indian population.

Objective: This single institutional study compared neoadjuvant concurrent chemo-radiotherapy (NACCRT) and neoadjuvant chemotherapy (NACT) followed by surgery in locally advanced middle and lower-1/3 carcinoma esophagus patients in terms of toxicity, clinical response, operative complications, disease downstaging, resection rates, pathological response, recurrence, and survival. Methods: This randomized prospective comparative study comprised 40 consecutive patients divided equally between two study arms NACCRT (n = 20; 41.4 Gy radiation dose; carboplatin area under the curve (AUC) 2/paclitaxel 50 mg/m2; 5 cycles) and NACT (n = 20; carboplatin AUC 5/paclitaxel 175 mg/m2; 2 cycles) from March 2014 to December 2016. Follow-up was done for 4 years. Chi-square test, Fischer's-exact test were used for comparative analysis and Kaplan-Meier analysis for survival. Results: Statistically significant esophagitis in NACCRT and peripheral-neuropathy in NACT was observed (P < 0.001). NACCRT recorded more postoperative complications, higher complete resection (R0) rates, and pathologically complete response (pCR). Tumor downstaging was significant in both study groups (n < 0.001). Four-year median disease-free survival (DFS) and overall survival (OS) were 28.50 months and 38 months in NACCRT versus 28 months and 35.5 months in NACT, respectively. In both NACCRT and NACT, pCR cases showed improved median DFS and OS compared to pathological partial response (pPR) (n < 0.001). Conclusion: This study demonstrated significant activity and tolerable toxicity of taxane-based therapy in NACCRT and NACT. Both groups recorded no survival benefit over each other, although pCR cases resulted in statistically significant survival advantage compared to clinical partial response. NACCRT resulted in lesser toxicity, numerically higher R0-resection, pCRs, median DFS, and OS compared to NACT.

Carcinoma Penis Manifesting as Upfront Supraclavicular Lymph Node Metastases Detected by 18 F-Fluorodeoxyglucose Positron Emission Tomography Scan: Report of an Extremely Rare and Aggressive Case.

Carcinoma penis is a rare malignancy accounting 0.5 to 1% cases in the developed countries with a slightly higher incidence in the developing nations. Slow locoregional progression is characteristic of penile carcinoma and distant metastases are very uncommon. We hereby report a case of highly aggressive squamous cell penile carcinoma in a 46-year-old male with fulminant upfront distant dissemination to left supraclavicular lymph nodes without involving the inguinal and pelvic nodes detected by whole-body 18 F-fluorodeoxyglucose positron emission tomography scan. The scan also detected lytic destructive lesion involving the pelvic and adjacent bones with infiltration of skeletal muscles. He was treated with palliative radiotherapy to the weight-bearing sites followed by systemic chemotherapy. A thorough review of literature reveals that our case may be one of the rarest cases ever reported in world literature where an asymptomatic penile carcinoma presents with upfront supraclavicular lymph node metastasis bypassing the inguinal, pelvic, and retroperitoneal lymph node chains.

Synchronous Detection of Extra-Adrenal Paraganglioma in a Follow-Up Case of High-Grade Gastrointestinal Stromal Tumor.

Extraadrenal paragalionoma at multiple sites and its association with GIST is a rare finding. Although 18F-FDG PET-CT is not a modality of choice for primary diagnosis of paraganglioma, and its use is restricted for detection of metastases/multiple sites and disease staging. However, in this case that we describe here, its role in an already-proven/recurrent case of paraganglioma is emphasized by its simultaneous assessment of disease at several different sites.

Carcinoma penis manifesting as upfront supraclavicular lymph node metastases detected by 18F-fluorodeoxyglucose positron emission tomography scan: Report of an extremely rare and aggressive case.

Carcinoma penis is a rare malignancy accounting 0.5%-1% of cases in the developed countries with a slightly higher incidence in the developing nations. Slow locoregional progression is characteristic of penile carcinoma (PC) and distant metastases are very uncommon. We hereby report a case of highly aggressive squamous cell PC in a 46-year-old male with fulminant upfront distant dissemination to left supraclavicular lymph nodes (LNs) without involving the inguinal and pelvic nodes detected by whole-body 18F-fluorodeoxyglucose positron-emission tomography scan. The scan also detected lytic destructive lesion involving the pelvic and adjacent bones with infiltration of skeletal muscles. He was treated with palliative radiotherapy to the wight-bearing sites followed by systemic chemotherapy. A thorough review of literature reveals that our case may be one of the rarest cases ever reported in world literature where an asymptomatic PC presents with upfront supraclavicular LN metastasis bypassing the inguinal, pelvic, and retroperitoneal LN chains.

Tumor Thrombi in Multiple Tributaries of the Mesenteric Vein in a Case of Carcinoma Rectosigmoid: A Rare Entity.

Treatment for tumor thrombus in multiple blood vessels can be a challenge for management and thus FDG PET CT is a potent tool in evaluation of such cases in differentiating between bland and tumor thrombus and commenting on the extent of the involvement.

Malignant peripheral nerve sheath tumor of thigh with sphenoid and brain metastases: Extremely rare occurrence with dismal prognosis despite significant response to palliative chemoradiotherapy.

Malignant peripheral nerve sheath tumor (MPNST) is a neurogenic tumor arising from peripheral nerves or nerve sheaths. MPNSTs are highly aggressive sarcomas mainly associated with neurofibromatosis type-1 (NF-1) with high rates of local recurrence and distant metastasis carrying a dismal prognosis. Lung is the most common metastatic site. Bone metastasis although documented in the literature is still very rare, while dissemination to brain without the involvement of lungs and that too in a non-NF-1 case is extremely unusual. A 48-year-old female was diagnosed with a case of non-NF-1 MPNST left thigh with bone metastases including sphenoid. Despite showing complete resolution of skeletal and primary lesions postpalliative chemoradiotherapy, she developed brain metastases and succumbed to her disease. This case is discussed to highlight an unusual scenario we encountered, the clinical course of the disease with its management, and overall poor prognosis. To the best of our knowledge, this may be the earliest case of MPNST with sphenoid metastases detected by 18-fluorodeoxyglucose positron-emission computed tomography scan and a sporadic case of brain metastases reported in the world literature.

Upfront Skeletal Muscle Metastases from Non-small Cell Lung Carcinoma: Report of an Extremely Rare Occurrence Detected by 18F-Fluorodeoxyglucose Positron Emission Computed Tomography Scan.

Adenocarcinoma lung with upfront metastases to skeletal muscle is rarely encountered in clinical practice since skeletal muscles are highly resistant to dissemination from solid organs. Moreover, these muscle metastatic lesions generally present with pain and palpable mass to get detected clinically. However, silent skeletal muscle metastases without any symptoms or signs getting detected by functional imaging with whole body 18F-fluorodeoxyglucose positron emission/computed tomography (18FDG-PET/CT) scan have been scarcely described in literature, while we present such an interesting case in a 45-year-old female. She was diagnosed as a case of biopsy-proven metastatic adenocarcinoma lung after evaluation by 18FDG-PET/CT. Despite treatment with palliative chemoradiotherapy, her disease progressed, and she finally succumbed to her illness. This case is discussed to highlight an unusual scenario we encountered, the clinical course of the disease with its management and overall poor prognosis.