scholarly journals Primary Synovial Sarcoma of Kidney: A Rare Differential Diagnosis of Renomegaly

2014 ◽  
Vol 2014 ◽  
pp. 1-3 ◽  
Author(s):  
Gaurang Modi ◽  
Irappa Madabhavi ◽  
Harsha Panchal ◽  
Asha Anand ◽  
Apurva Patel ◽  
...  
Keyword(s):  
Poor Prognosis ◽  
Renal Mass ◽  
Epithelial Membrane Antigen ◽  
Palliative Chemotherapy ◽  
Soft Tissue Sarcomas ◽  
Rare Differential Diagnosis ◽  
Rare Tumours ◽  
Synovial Sarcomas ◽  
Poorly Differentiated ◽  
Histology And Immunohistochemistry

Synovial sarcomas (SS) are classified as subgroup of soft tissue sarcomas affecting mainly extremities of young adults. Primary SS of kidney are very rare tumours with poor prognosis. Though they have characteristic histology and immunohistochemistry (IHC) due to rarity of incidence it is difficult to diagnose them. Sometimes chromosomal rearrangement studies are required to confirm the diagnosis. We are presenting a case of 41-year-old male who was referred to our cancer centre for evaluation of left renal mass. CT scan of abdomen revealed a large left renal mass encasing the aorta. Biopsy of renal mass revealed poorly differentiated sarcoma and IHC was positive for vimentin, CD99, and BCL2 and negative for AE1, epithelial membrane antigen, and leukocyte common antigen. The patient was clinically inoperable as renal mass was encasing the aorta. So he was subsequently offered palliative chemotherapy in form of ifosfamide and adriamycin. CT abdomen shows partial response after 3 cycles of chemotherapy according to RECIST criteria.

Small cell carcinoma of the pancreas: a rare neuroendocrine tumour

2020 ◽  
Vol 13 (7) ◽  
pp. e235397
Author(s):  
Theakarajan Rajendran ◽  
Bhavana Katta ◽  
Oseen Hajilal Shaikh ◽  
Uday Shamrao Kumbhar
Keyword(s):  
Cell Carcinoma ◽  
Small Cell Carcinoma ◽  
Poor Prognosis ◽  
Neuroendocrine Tumours ◽  
Palliative Chemotherapy ◽  
Neuroendocrine Tumour ◽  
Small Cell ◽  
Immunohistochemical Markers ◽  
Poorly Differentiated

Primary small cell carcinoma (SCC) of the pancreas is a rare disease with poor prognosis. Very few cases have been reported in the literature. It is a type of poorly differentiated variety of neuroendocrine tumours of the pancreas with specific immunohistochemical markers. Imaging is not diagnostic for disease, and diagnosis is mainly by biopsy. We report a rare case of SCC of the pancreas who presented with features of obstructive jaundice without any paraneoplastic features. The patient is planned for palliative chemotherapy because of metastasis and is under regular follow-up.

scholarly journals Key Features in the Management of Pulmonary Carcinosarcoma

2016 ◽  
Vol 2016 ◽  
pp. 1-4 ◽  
Author(s):  
Nikolaos Panagiotopoulos ◽  
Davide Patrini ◽  
Benjamin Adams ◽  
Jonathan Pararajasingham ◽  
Rajeev Shukla ◽  
...  
Keyword(s):  
Cell Carcinoma ◽  
Poor Prognosis ◽  
Small Cell ◽  
Optimal Management ◽  
Review Of The Literature ◽  
Rare Tumours ◽  
Lung Malignancies ◽  
Poorly Differentiated ◽  
Pulmonary Carcinosarcoma ◽  
Key Features

Pulmonary carcinosarcoma represents a category of extremely rare tumours accounting for 0.1% of all lung malignancies. It is defined as a poorly differentiated non-small-cell carcinoma that contains a component of sarcoma or sarcoma-like elements. These biphasic tumours typically have a poor prognosis due to late diagnosis and early metastases. Preoperative tissue diagnosis is usually difficult due to the heterogeneity of the tumour, with biopsies often just reflecting one element of the tumour. By means of a case illustration and review of the literature, we discuss the optimal management of patients with pulmonary carcinosarcoma.

scholarly journals Gastric cancer with repeated metastasis in the colonic lumen: a case report and multi-surgical experience

2021 ◽  
Vol 49 (5) ◽  
pp. 030006052110184
Author(s):  
Yi Wang ◽  
Peiqing Ma ◽  
Kan Liu ◽  
Dongkui Xu ◽  
Qian Liu
Keyword(s):  
Gastric Cancer ◽  
Gastric Carcinoma ◽  
Poor Prognosis ◽  
Partial Gastrectomy ◽  
Radical Resection ◽  
Surgical Experience ◽  
Colonic Lumen ◽  
Poorly Differentiated ◽  
Gastric Stump Carcinoma ◽  
Peritoneal Spread

Poorly differentiated gastric adenocarcinoma is commonly associated with lymph node metastasis, peritoneal spread, and liver metastasis but rarely with intraintestinal metastasis. Most patients with metastatic gastric carcinoma are unable to undergo surgical treatment and have a poor prognosis. A 42-year-old man with hunger-related abdominal pain was diagnosed as having gastric cancer. After the first surgery (distal partial gastrectomy) and the second surgery (gastric stump carcinoma (GSC) resection), the patient suffered repeated multiple intracolonic metastases and underwent three additional resection operations. The patient survived for 154 months after the first operation. In patients with gastric carcinoma that metastasizes to the colonic lumen, radical resection, if possible, can extend survival. Once patients develop extensive extraintestinal metastasis, radical resection cannot be performed, and patients often exhibit a poor prognosis.

scholarly journals Top-level MET gene copy number gain defines a subtype of poorly differentiated pulmonary adenocarcinomas with poor prognosis

2020 ◽  
Vol 9 (3) ◽  
pp. 603-616 ◽  
Author(s):  
Tobias Raphael Overbeck ◽  
Dana Alina Cron ◽  
Katja Schmitz ◽  
Achim Rittmeyer ◽  
Wolfgang Körber ◽  
...  
Keyword(s):  
Copy Number ◽  
Poor Prognosis ◽  
Gene Copy Number ◽  
Copy Number Gain ◽  
Gene Copy ◽  
Poorly Differentiated ◽  
Gene Copy Number Gain

scholarly journals Case Report: Metastatic Dedifferentiated Liposarcoma Presenting as Hypereosinophilia in an Adolescent

2020 ◽  
Author(s):  
Sadhana Balasubramanyam ◽  
Joud Hajjar
Keyword(s):  
Computed Tomography ◽  
Palliative Chemotherapy ◽  
Pediatric Population ◽  
Brain Lesion ◽  
Soft Tissue Sarcomas ◽  
Pulmonary Nodules ◽  
Dedifferentiated Liposarcoma ◽  
Positron Emission ◽  
Pet Ct ◽  
Metastatic Sarcoma

ABSTRACT Soft-tissue sarcomas associated with eosinophilia are rare, with limited cases reported in adults, and even fewer in the pediatric population. In this report, we highlight the importance of malignancy in the differential of hypereosinophilia in an adolescent. A 17-year-old boy presented with incidental findings of multiple bilateral pulmonary nodules on chest computed tomography (CT), and hypereosinophilia (absolute eosinophilic count [AEC] 7029 cells/mm3, hypereosinophilia defined as AEC >1500 cells/mm3). Lung biopsy showed high-grade metastatic sarcoma. A positron emission tomography–computed tomography (PET-CT) demonstrated a 7.9-cm mass in the left thigh, with biopsy revealing dedifferentiated liposarcoma. Subsequently, the patient was diagnosed with liposarcoma, with lung, mediastinal, and brain metastases. He completed six cycles of ifosfamide/doxorubicin, followed by surgical resection of primary thigh tumor and brain lesion. Given widely metastatic disease, he received palliative chemotherapy, and later transitioned to hospice. The patient died of respiratory failure from malignant pleural effusions. In conclusion, this case demonstrates the importance of a having a broad differential for hypereosinophilia, including malignancy, to expedite the diagnosis and initiate appropriate management promptly.

scholarly journals A Case of Giant Primary Pleuropulmonary Synovial Sarcoma

2020 ◽  
pp. 1-4
Author(s):  
Wickii T. Vigneswaran ◽  
Brittany C. Fields ◽  
Vijayalakshmi Ananthanarayanan ◽  
Wickii T. Vigneswaran
Keyword(s):  
Synovial Sarcoma ◽  
Soft Tissue Sarcomas ◽  
Extrapleural Pneumonectomy ◽  
Ct Guided ◽  
Prosthetic Reconstruction ◽  
Pathologic Features ◽  
Synovial Sarcomas ◽  
Primary Pulmonary Synovial Sarcoma ◽  
Rare Group

Synovial sarcomas represent an extremely rare subtype of an already rare group of malignancies, soft tissue sarcomas. Among these, primary pulmonary synovial sarcomas comprise an even smaller number, though they have become more frequently reported in the literature. This case report details a case of giant primary pulmonary synovial sarcoma in a 44-year-old male patient who presented with left-sided chest pain and shortness of breath and was found to have a large left-sided pleural effusion. No malignant cells were demonstrated on cytology of pleural fluid after thoracentesis; however, CT-guided needle biopsy of pleural nodules seen on imaging demonstrated pathologic features consistent with monophasic type synovial sarcoma. He was treated with neoadjuvant chemotherapy with minimal response; thus, he was referred for surgical management. A left extrapleural pneumonectomy with resection of the left hemi-diaphragm and Gore-Tex prosthetic reconstruction was performed. Imaging at six-month follow-up demonstrated a new nodule in the contralateral lung, suggestive of metastasis at that time and the patient later developed ascites at nine months, consistent with further intra-abdominal metastasis. Perhaps early diagnosis and aggressive multimodality therapy may have a place in the treatment of this aggressive disease.

scholarly journals Improving Immunotherapy Efficacy in Soft-Tissue Sarcomas: A Biomarker Driven and Histotype Tailored Review

2021 ◽  
Vol 12 ◽  
Author(s):  
Matthieu Roulleaux Dugage ◽  
Elise F. Nassif ◽  
Antoine Italiano ◽  
Rastislav Bahleda
Keyword(s):  
Soft Tissue ◽  
Immune Checkpoint ◽  
Response Rate ◽  
Checkpoint Inhibitors ◽  
Early Stage ◽  
Soft Tissue Sarcomas ◽  
Oncolytic Viruses ◽  
Cellular Therapies ◽  
M1 Macrophage ◽  
Synovial Sarcomas

Anti-PD-(L)1 therapies yield a disappointing response rate of 15% across soft-tissue sarcomas, even if some subtypes benefit more than others. The proportions of TAMs and TILs in their tumor microenvironment are variable, and this heterogeneity correlates to histotype. Tumors with a richer CD8+ T cell, M1 macrophage, and CD20+ cells infiltrate have a better prognosis than those infiltrated by M0/M2 macrophages and a high immune checkpoint protein expression. PD-L1 and CD8+ infiltrate seem correlated to response to immune checkpoint inhibitors (ICI), but tertiary lymphoid structures have the best predictive value and have been validated prospectively. Trials for combination therapies are ongoing and focus on the association of ICI with chemotherapy, achieving encouraging results especially with pembrolizumab and doxorubicin at an early stage, or ICI with antiangiogenics. A synergy with oncolytic viruses is seen and intratumoral talimogene laherpavec yields an impressive 35% ORR when associated to pembrolizumab. Adoptive cellular therapies are also of great interest in tumors with a high expression of cancer-testis antigens (CTA), such as synovial sarcomas or myxoid round cell liposarcomas with an ORR ranging from 20 to 50%. It seems crucial to adapt the design of clinical trials to histology. Leiomyosarcomas are characterized by complex genomics but are poorly infiltrated by immune cells and do not benefit from ICI. They should be tested with PIK3CA/AKT inhibition, IDO blockade, or treatments aiming at increasing antigenicity (radiotherapy, PARP inhibitors). DDLPS are more infiltrated and have higher PD-L1 expression, but responses to ICI remain variable across clinical studies. Combinations with MDM2 antagonists or CDK4/6 inhibitors may improve responses for DDLPS. UPS harbor the highest copy number alterations (CNA) and mutation rates, with a rich immune infiltrate containing TLS. They have a promising 15-40% ORR to ICI. Trials for ICB should focus on immune-high UPS. Association of ICI with FGFR inhibitors warrants further exploration in the immune-low group of UPS. Finally translocation-related sarcomas are heterogeneous, and although synovial sarcomas a poorly infiltrated and have a poor response rate to ICI, ASPS largely benefit from ICB monotherapy or its association with antiangiogenics agents. Targeting specific neoantigens through vaccine or adoptive cellular therapies is probably the most promising approach in synovial sarcomas.

scholarly journals Adenosquamous Carcinoma of the Pancreas

2019 ◽  
Vol 13 ◽  
pp. 117955491988658 ◽  
Author(s):  
Hélène Del Arco ◽  
Camille Chakiba-Brugère ◽  
Laura Salabert ◽  
Dominique Béchade
Keyword(s):  
Poor Prognosis ◽  
Palliative Chemotherapy ◽  
Adenosquamous Carcinoma ◽  
Needle Aspiration ◽  
Massive Weight Loss ◽  
Rare Tumor ◽  
Imaging Studies ◽  
Fine Needle ◽  
Carcinoma Of The Pancreas ◽  
Line Chemotherapy

A 60-year-old woman presented to hospital with abdominal pain and massive weight loss. Imaging studies confirmed the presence of a tumor of the pancreas. Histologic analysis of the sampling performed by echoendoscopic ultrasound fine-needle aspiration found aspects evocative of adenosquamous carcinoma. This case report highlights the difficulties of clinical pathologic diagnosis for these occasionally composite tumors. The patient underwent palliative chemotherapy based on platinum and 5-fluorouracil, followed by second-line chemotherapy with FOLFIRI after progression. Adenosquamous carcinoma of the pancreas remains a rare tumor with very poor prognosis and limited therapeutic options.

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