Review
Oncology
Shijun Xia, Wenjiang Wu, Lijuan Ma
Summary: This case report describes a rare case of proximal-type epithelioid sarcoma of the perineum in a 31-year-old male who underwent wide excision. The disease is characterized by an indetectable early symptom and frequent recurrences. A review of the current literature on the differential diagnosis and management of this disease is also presented.
FRONTIERS IN ONCOLOGY
(2023)
Article
Oncology
Samuel Rasmussen, Jia Xiang Jin, Lissett R. Bickford, Andrew D. Woods, Felix Sahm, Kenneth A. Crawford, Kiyo Nagamori, Hiroaki Goto, Keila E. Torres, Angelo Sidoni, Erin R. Rudzinski, Khin Thway, Robin L. Jones, Alessio Ciulli, Hollis Wright, Melvin Lathara, Ganapati Srinivasa, Kavya Kannan, Paul H. Huang, Thomas G. P. Grunewald, Noah E. Berlow, Charles Keller
Summary: This study used a functional genomics approach to identify key signaling pathway vulnerabilities in EPS patient samples, revealing subtle differences between distal, pediatric/young adult-associated EPS and proximal, adult-associated EPS. These differences highlight the need to approach the two subtypes as molecularly and clinically distinct diseases.
CLINICAL AND TRANSLATIONAL MEDICINE
(2022)
Article
Oncology
Cristina R. Antonescu, Andrew E. Rosenberg, Ziyu Xie, Lei Zhang, Katharina Anne Perell, Anand C. Loya
Summary: Undifferentiated sarcomas remain challenging to classify, with the study presenting two high-grade sarcomas exhibiting an EWSR1-SSX1 fusion and sharing similar molecular and immunoprofile characteristics. One patient developed lymph node metastases while the other showed no evidence of disease.
GENES CHROMOSOMES & CANCER
(2021)
Article
Oncology
Caroline C. H. Siew, Kenneth Cardona, Winan J. van Houdt
Summary: Recurrent retroperitoneal sarcomas are challenging to manage due to the diverse patient characteristics, treatment profiles, and patterns of recurrence. While surgical resection can improve survival, the oncological outcomes are inferior to primary retroperitoneal sarcomas. Management options for unresectable disease include local ablative therapy, radiation, systemic therapy, and occasional palliative surgery. This review provides insights into the understanding and management of recurrent retroperitoneal sarcomas.
Article
Biochemistry & Molecular Biology
William G. J. Kerrison, Alexander T. J. Lee, Khin Thway, Robin L. Jones, Paul H. Huang
Summary: Immunotherapy in soft tissue sarcoma has become a popular and promising therapeutic strategy, with immune checkpoint inhibitors showing effectiveness in certain subtypes. Combination therapies have also shown enhanced response rates.
Review
Oncology
Harsimrat Kaur Birdi, Anna Jirovec, Serena Cortes-Kaplan, Joel Werier, Carolyn Nessim, Jean-Simon Diallo, Michele Ardolino
Summary: Sarcomas, a rare malignancy of mesenchymal tissues with over 60 subtypes, pose challenges in treatment due to disease heterogeneity. While traditional therapies are effective in early stages, immunotherapy is showing promise for advanced sarcoma treatment, sparking interest in further research and application.
JOURNAL FOR IMMUNOTHERAPY OF CANCER
(2021)
Review
Oncology
Pampina Pilavaki, Myrofora Panagi, Samia Arifi, Robin L. Jones, Triantafyllos Stylianopoulos, Anastasia Constantinidou
Summary: Sarcomas are a diverse group of malignant tumors that arise from mesenchymal tissue and account for 1% of adult malignancies. Surgical excision and conventional chemotherapy are the mainstay of treatment for local and advanced disease, respectively. Immunotherapy has shown promise in multiple cancers, but its efficacy in sarcomas remains uncertain. Clinical trials and studies are ongoing to determine the role of immunotherapy in specific sarcoma subtypes.
FRONTIERS IN ONCOLOGY
(2023)
Article
Oncology
Jennine H. Weller, Carly Westermann, Palak Patel, Ross M. Beckman, Christine A. Pratilas, Carol D. Morris, Daniel S. Rhee
Summary: Synovial sarcoma has a lower incidence of lymph node metastasis compared to CCS and epithelioid sarcoma. Lymph node sampling can improve overall survival in children and young adults with CCS.
PEDIATRIC BLOOD & CANCER
(2022)
Article
Pathology
Inigo Espinosa, Emanuela D'Angelo, Louise De Brot, Jaime Prat
Summary: A study revealed that most extrarenal rhabdoid tumors of the vulva are proximal-type epithelioid sarcomas. By analyzing the clinicopathologic, immunohistochemical, and molecular features of these tumors, it was found that they have distinct expressions.
Article
Oncology
Lars H. Lindner, Jean-Yves Blay, Alexander M. M. Eggermont, Rolf D. Issels
Summary: Patients with adult-type soft tissue sarcoma face high risks of local recurrence and distant metastases. Complete tumor resection is the main treatment, with possible addition of radiotherapy. Perioperative chemotherapy can target micrometastatic disease. Multimodality approaches including chemotherapy, RHT, and immunotherapy have shown improved survival in high-risk patients.
EUROPEAN JOURNAL OF CANCER
(2021)
Article
Cardiac & Cardiovascular Systems
Edward Y. Chan, Areeba Ali, M. Mujeeb Zubair, Duc T. Nguyen, Sergio H. Ibarra-Cortez, Edward A. Graviss, Oz M. Shapira, Vinod Ravi, Thomas E. MacGillivray, Michael J. Reardon
Summary: This study retrospectively analyzed the surgical outcomes of 122 patients with primary cardiac sarcoma. The results showed that the survival rate of cardiac sarcoma patients gradually decreased within 5 years, and there was no significant difference in mortality between different time periods. This suggests that cardiac sarcoma is a systemic disease, and current treatment methods have not significantly improved patient survival.
JOURNAL OF THORACIC AND CARDIOVASCULAR SURGERY
(2023)
Review
Radiology, Nuclear Medicine & Medical Imaging
X. Hu, C. Zhang, K. Qian, R. Yu, T. Mei, J. Cai
Summary: This article reports a case of proximal-type epithelioid sarcoma of the groin and discusses its clinical and imaging features. Surgery seems to be the most effective treatment, but the effectiveness of adjuvant and radiation therapy needs further research.
INTERNATIONAL JOURNAL OF RADIATION RESEARCH
(2022)
Article
Medicine, General & Internal
Stefania Kokkali, Louiza Vini, Anastasia Stergioula, Anastasios Kyriazoglou, Nikolaos Vassos, Ioannis Boukovinas
Summary: Brain metastases in sarcoma patients are rare, often occurring with extracranial metastases. Treatment modalities include whole-brain radiation therapy, chemotherapy, and surgery, but most patients have a short survival period after brain metastases.
JOURNAL OF CLINICAL MEDICINE
(2021)
Review
Medicine, General & Internal
Elizabeth Zegarra Buitron, Daniel A. Vidal Panduro, Domingo Morales Luna
Summary: Epithelioid sarcoma is a rare and aggressive tumor. Timely and accurate diagnosis is crucial for improving the survival rate of patients. This article reports a case of a 41-year-old male patient with a 9-month disease duration, recurrence after surgery, and unsuccessful chemotherapy.
CUREUS JOURNAL OF MEDICAL SCIENCE
(2022)
Article
Biochemistry & Molecular Biology
Timur I. Fetisov, Sofya A. Khazanova, Polina A. Shtompel, Ekaterina S. Trapeznikova, Victoria Y. Zinovieva, Valeria I. Marshall, Anastasia A. Lovenger, Dmitriy V. Rogozhin, Tararykova A. Anastasia, Beniamin Yu. Bokhyan, Gennady A. Belitsky, Marianna G. Yakubovskaya, Kirill I. Kirsanov
Summary: Treatment of highly malignant soft tissue sarcomas (STSs) involves multicomponent therapy, but personalized selection of chemotherapy is lacking. In this study, in vitro chemosensitivity/resistance assays (CSRAs) were used to predict STS chemoresistance. Results showed that certain cancer histotypes had higher resistance to specific drugs and combinations, and the CSRAs were correlated with clinical responses and survival outcomes. CSRAs can assist physicians in avoiding ineffective treatments and aid researchers in developing new STS drugs.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Review
Pathology
Bharat Rekhi, Omshree Shetty, Prachi Bapat, Mamta Gurav, Sajid Qureshi
Summary: The article presents a case of a 1-year-old male infant with NTRK-positive mesenchymal tumor, who deteriorated after chemotherapy and eventually succumbed to the disease. The characteristics of the tumor were identified through techniques such as immunohistochemistry and genetic testing, highlighting the importance of next-generation sequencing in uncovering such tumors.
INTERNATIONAL JOURNAL OF SURGICAL PATHOLOGY
(2021)
Article
Oncology
Kalasekhar Vijayasekharan, Subramaniam Ramanathan, Girish Chinnaswamy, Maya Prasad, Ashish Gulia, Amit Janu, Bharat Rekhi, Mukta Ramadwar, Nehal Khanna, Siddhartha Laskar, Nilendu Puranadare, Jyoti Bajpai, Ajay Puri, Shripad Banavali, Tushar Vora
Summary: This study presented outcomes of a novel hybrid salvage protocol using four active chemotherapeutic agents in relapsed ES patients. While some patients showed promising results, overall prognosis remains poor. Adolescents and young adults, as well as patients with longer disease-free intervals, had better survival post-relapse.
JOURNAL OF ADOLESCENT AND YOUNG ADULT ONCOLOGY
(2021)
Article
Orthopedics
Bharat Rekhi, Rushabh Kothari, Sanjeev Shah, Omshree Shetty, Mandip C. Shah
Summary: Adamantinoma-like Ewing sarcoma is a rare entity, particularly when occurring in the calcaneus. Diagnosis involves imaging studies, histopathological examination, and immunohistochemistry. Despite neoadjuvant chemotherapy, the patient had a poor response and ultimately required amputation.
SKELETAL RADIOLOGY
(2022)
Article
Oncology
Ashish Gulia, Vineet Kurisunkal, Ajay Puri, Prakash Nayak, Bharat Rekhi
Summary: Clear cell chondrosarcoma is a rare subtype of chondrosarcoma, mainly affecting males with an average symptom duration of around 6 months. It commonly occurs in long bones.
INDIAN JOURNAL OF SURGICAL ONCOLOGY
(2022)
Article
Pathology
Bharat Rekhi, Michael Michal, Fatma Bilge Ergen, Paromita Roy, Florian Puls, Hans Kristian Haugland, Figen Soylemezoglu, Kemal Kosemehmetoglu
Summary: Poorly differentiated chordomas are a newly recognized entity that displays a spectrum of features and aggressive outcomes. Timely diagnosis requires critical analysis of radiological and histopathological features, including necessary immunostains such as brachyury and SMARCB1/INI1. These tumors typically show loss of SMARCB1/INI1 immunostaining and homozygous deletion of the INI1/SMARCB1 gene, and are associated with a lower index of suspicion for diagnosis.
ANNALS OF DIAGNOSTIC PATHOLOGY
(2021)
Article
Medical Laboratory Technology
Bharat Rekhi, Leslie Dodd, Bhaskar Dharavath, Amit Dutt
Summary: This study presents one of the first documentations of cytomorphological features of two rare cases of spindle cell/sclerosing rhabdomyosarcoma (RMS). The differential diagnoses and treatment-related implications are discussed.
DIAGNOSTIC CYTOPATHOLOGY
(2022)
Article
Pathology
Bharat Rekhi, Adalet Elcin Yildiz, Anne Jennifer, Fisun Ardic Yukruk, Gokhan Gedikoglu, Devmalya Banerjee, Omshree Shetty, Kemal Kosemehmetoglu
Summary: This study presents six cases of adamantinoma with a prominent spindle cell component mimicking intraosseous synovial sarcomas. Clinical-radiological index of suspicion, immunostains (p63 and p40), and molecular tests are useful in accurately diagnosing adamantinomas with spindle cell morphology, which has treatment-related implications.
INTERNATIONAL JOURNAL OF SURGICAL PATHOLOGY
(2022)
Article
Pathology
Vidya Rao, Bharat Rekhi, Latha K. Abraham, Prachi Bapat
Summary: This article reports a rare case of solitary fibrous tumor in a pediatric patient, presenting as a congenital mass, confirmed by immunostaining and molecular testing.
INTERNATIONAL JOURNAL OF SURGICAL PATHOLOGY
(2023)
Article
Oncology
Souvik Mukherjee, Asmita Sakpal, Megha Mehrotra, Pratham Phadte, Bharat Rekhi, Pritha Ray
Summary: This study demonstrates the role of Notch3 in ovarian cancer metastasis using a co-culture model. Incremental expression of Jagged1 leads to activation of Notch3 in SNFT, and differential activation is observed in co-cultures with other EOC cell lines and cancer-associated fibroblasts. Furthermore, VEGFA and p21 are identified as the top upregulated genes in co-cultures, and secreted VEGFA reduces cancer stem cell differentiation.
Article
Cell Biology
Bharat Rekhi
Summary: This article reports the cytomorphologic features of a superficial CD34-positive fibroblastic tumor and discusses its differential diagnoses, molecular updates, and treatment-associated implications.
Review
Pathology
Bharat Rekhi, Fatma Bilge Ergen, Vaibhavi Vengurlekar, Rachna Rumde, Omshree Shetty, Gaye Guler, Kemal Kosemehmetoglu
Summary: This article presents the clinicopathological features of 12 cases of BCOR::CCNB3-positive undifferentiated sarcomas, including immunohistochemical and molecular data, as well as radiological profiles. The study found specific clinicopathological features and molecular alterations in these tumors, which are important for accurate diagnosis and treatment.
INTERNATIONAL JOURNAL OF SURGICAL PATHOLOGY
(2023)
Review
Medicine, General & Internal
Sourav Chakraborty, Priti S. Shenoy, Megha Mehrotra, Pratham Phadte, Prerna Singh, Bharat Rekhi, Pritha Ray
Summary: Epithelial ovarian cancer (EOC) is a deadly gynaecological malignancy, and its late-stage diagnosis and metastasis contribute to a high mortality rate. Current diagnostic tools only aid in diagnosing advanced-stage patients, highlighting the need for novel approaches for early diagnosis and prognosis. Biomarkers such as CA-125 and HE4 have shown promise in early-stage detection and administration of chemotherapy. This review provides a summary of existing and potential biomarkers for ovarian cancer.
Review
Cell Biology
Bharat Rekhi, Xiaohua Qian, Henryk A. Domanski, Jerzy Klijanienko, Andrew Field
Summary: Fine needle aspiration biopsy (FNAB) is an effective method for evaluating suspicious soft tissue masses, but accurately subtyping primary soft tissue tumors can be difficult. This review discusses the diagnostic challenges in soft tissue cytopathology, including pitfalls and strategies for overcoming these challenges by integrating clinical details, key cytomorphological features, and ancillary techniques.
Article
Pathology
Bharat Rekhi, Prachi Bapat, Arpita Jindal
Summary: This case is a rare vulvar solitary fibrous tumor confirmed by STAT6 immunostaining and NAB2-STAT6 gene fusion. The histopathologic features include patchy CD34 and diffuse STAT6 immunostaining in tumor cells, as well as NAB2ex6-STAT6ex17 fusion.
INDIAN JOURNAL OF PATHOLOGY AND MICROBIOLOGY
(2021)
Article
Orthopedics
Jagandeep Singh Virk, Manish Pruthi, Bharat Rekhi, Sunil Pasricha
Summary: This case report presents a 24-year-old male with adamantinoma initially mistaken for fibrous dysplasia, eventually confirmed through histological and immunohistochemical examination. The patient underwent internal hemipelvectomy with successful outcome. Careful evaluation of histomorphologic features, immunohistochemical stains, and clinical correlation is essential for accurate diagnosis of this rare tumor at an unusual site.
INDIAN JOURNAL OF ORTHOPAEDICS
(2021)
