scholarly journals The First Case Treated with Larotrectinib of a Novel TMTC2-NTRK3 Fusion Undifferentiated High-Grade Pleomorphic Sarcoma of the Chest

Author(s):  
Chujie Bai ◽  
Lu Zhang ◽  
Yaohui Wang ◽  
Taiyan Guo ◽  
Xia You ◽  
...  

Abstract Undifferentiated high-grade pleomorphic sarcoma (UPS) is a rare soft tissue sarcoma (STS) of mesenchymal origin, particularly the extremities and retroperitoneum, meanwhile it has been reported in almost all parts of the body. UPS is highly invasive and has a poor prognosis due to its clinical manifestations of painless mass and deep tumor site, which are usually found at an advanced stage. Patients with UPS tend to have a lower 5-year survival rate than patients with other types of STS. Recently, NTRK fusions were detected in many cancer types, such as thyroid cancer, colorectal cancer, non-small cell lung cancer, soft tissue tumors, uterine sarcomas, and melanomas. However, the mutation frequency of NTRK fusion in all cancers is only 0.1-1%. Targeted therapy with NTRK inhibitors, such as Larotrectinib and Entrectinib, leads to a response in most patients with NTRK1/2/3 gene fusion-positive tumors. Herein, we present a 68-years old man diagnosed with stage IIIA (T2N0M0G3) UPS. Next-generation sequencing (NGS) revealed a novel TMTC2-NTRK3 fusion. The NTRK3 positivity was also detected by immunohistochemistry (IHC) and fluorescence in situ hybridization (FISH). He had a response to Larotrectinib. This report broadens the spectrum of NTRK fusions in UPS and highlights a new target for treatment.

Author(s):  
Khadija S. Tapadar ◽  
Manoj K. Deka ◽  
R. N. Chaubey ◽  
Shah A. Sheikh ◽  
Gargi R. Choudhury ◽  
...  

Background: Soft tissue tumors are defined as mesenchymal proliferations which occur in the extraskeletal non-epithelial tissues of the body, excluding the viscera, coverings of brain and   lymphoreticular system. The objective of this study was to study the histopathological features of soft tissue tumors and to study the occurrence of soft tissue tumors in relation to age, sex and anatomical site.Methods: This study comprised of 89 cases studied over a period of two years. All soft tissue tumors, their gross features, microscopic findings were analysed in detail. Soft tissue tumors were divided into benign and malignant categories and further sub typing were done according to World Health Organization (WHO) classification. The distribution of soft tissue tumors according to the age, sex and site of occurrence was studied.Results: Out of 89 cases of soft tissue tumors, 76 cases were benign, 4 cases belonged to intermediate category and 9 cases were malignant. Adipocytic tumors formed the largest group constituting 39 cases. Vascular tumors were the second commonest (26 cases) followed by peripheral nerve sheath tumors (11 cases). The benign tumors were seen in younger age as compared to malignant tumors. Malignant soft tissue tumors was seen to be more common in male than female and pleomorphic sarcoma and liposarcoma was commonest (3 cases each).Conclusions: Benign tumors were more common than malignant. The most common benign tumors were lipoma followed by hemangioma and schwannoma. The most common malignant tumor was pleomorphic sarcoma. The benign tumors were seen in younger age as compared to malignant tumors.


2017 ◽  
Vol 03 (02) ◽  
pp. 133-135
Author(s):  
Pragati S. Upasham ◽  
Sharayu P. Dighavkar ◽  
Prakash M. Roplekar

AbstractMalignant soft-tissue tumors which were designated as malignant fibrous histiocytoma are regrouped by the WHO (in 2002) under the new entity termed as “undifferentiated pleomorphic sarcoma.”1 It accounts for less than 5% of all adult soft-tissue sarcomas. Here, we report the lesion in a 70-year-old man who presented with high-grade undifferentiated pleomorphic sarcoma in the lower extremity.


2014 ◽  
Vol 3 (2) ◽  
pp. 367-374 ◽  
Author(s):  
MANABU HOSHI ◽  
MASATSUGU TAKAMI ◽  
MAKOTO IEGUCHI ◽  
MASANARI AONO ◽  
JUN TAKADA ◽  
...  

Author(s):  
Kunhi Mohammed K. P. ◽  
Snehasis Pradhan ◽  
Supratim Bhattacharyya ◽  
Prafulla Kumar Das ◽  
Muhammed Navas N. K.

Background: Soft tissue sarcomas are a rare and heterogeneous group of malignant tumors of mesenchymal origin that comprise less than 1 percent of all adult malignancies. Although they occur anywhere in the body, they involve most commonly in extremities, trunk, retroperitoneum and head and neck. The aim of the study was to analyze clinical and histopathological features of various soft tissue sarcomas.Methods: This was a retrospective study, conducted in tertiary cancer centre in Odisha during the period 2015 to 2018. We collected clinical parameters like age, sex, site of swelling, any associated pain and biopsy reports and these variables were correlated with final histopathology reports.Results: A total of 107 patients were included in the study, with male to female ratio of 2:1(71 and 36) and average age of 43.45 years. All of them presented with a swelling. The lower extremities were the most common sites i.e. 44.62%. Pleomorphic sarcoma was the most frequent histologic variety comprising 43% and less frequent variety were angiosarcoma, and myxoid sarcoma.Conclusions: Soft tissue sarcoma are predominant in males and middle aged population are frequently affected. Most common affected site is lower extremity and pleomorphic sarcoma is the prominent histologic type.


2017 ◽  
Vol 7 ◽  
pp. 38 ◽  
Author(s):  
Jamie Crist ◽  
Jacob R Hodge ◽  
Matthew Frick ◽  
Fiona P Leung ◽  
Eugene Hsu ◽  
...  

Schwannomas are benign soft-tissue tumors that arise from peripheral nerve sheaths throughout the body and are commonly encountered in patients with neurofibromatosis Type 2. The vast majority of schwannomas are benign, with rare cases of malignant transformation reported. In this pictorial review, we discuss the magnetic resonance imaging (MRI) appearance of schwannomas by demonstrating a collection of tumors from different parts of the body that exhibit similar MRI characteristics. We review strategies to distinguish schwannomas from malignant soft-tissue tumors while exploring the anatomic and histologic origins of these tumors to discuss how this correlates with their imaging findings. Familiarity with the MRI appearance of schwannomas can help aid in the differential diagnosis of soft-tissue masses, especially in unexpected locations.


1970 ◽  
Vol 1 (1) ◽  
pp. 29-32
Author(s):  
MD A Kalam ◽  
F Ahmad ◽  
M Hassan ◽  
Md Nasiruddin ◽  
SA Rahman ◽  
...  

The term fibromatosis refers to a group of benign soft tissue tumors (fibromas), which have certain characteristics in common, including absence of cytologic and clinical malignant features, a histology consistent with proliferation of well-differentiated fibroblasts, an infiltrative growth pattern, and aggressive clinical behavior with frequent local recurrence. Lymphangiomas are malformations of the lymphatic system, which is the network of vessels responsible for returning to the venous system of excess fluid from tissues. Here we report a case of 30 years old lady with history of multiple soft tissue tumours in thigh, axilla and abdomen. On physical examination the swelling of the thigh and abdomen were firm and those of axilla were soft. All the tumours were excised surgically and diagnosis of thigh swelling was fibromatosis and that of axilla was lymphangioma, on histopathology.DOI: http://dx.doi.org/10.3329/bdjps.v1i1.6490Bangladesh Journal of Plastic Surgery (2010) Vol. 1 (1) pp.29-32


10.12737/9072 ◽  
2015 ◽  
Vol 22 (1) ◽  
pp. 27-33 ◽  
Author(s):  
Садовникова ◽  
I. Sadovnikova ◽  
Зудов ◽  
Andrey Zudov

The research of effective methods of treatment of broncho-obstructive diseases is one of the most important questions of the pediatric pulmonology. General clinical manifestations are characterized by attacks of breathlessness, prolonged exhalation, unproductive cough, whistling and noisy breathing sometimes with a help of auxiliary muscles. Often this disease leads to bronchial asthma, obstructive bronchitis, and malformations of the lung and bronchus. In the pathogenesis of bronchial obstruction whilst respiratory infections the main factors are inflammatory infiltration of the bronchial mucosa, its swelling, hypersecretion of viscous mucus and bronchospasm due to hyperactivity of the bronchi of the inflammatory nature. To diagnose this disease it is important to establish the presence or absence of the effectiveness of the protective mechanisms of the body at different hierarchic levels. To protect the respiratory tract from exposure to adverse environmental factors in the process of ontogenesis pro-tective mechanisms formed. The first stage of purification of the bronchial system is mucociliary clearance carried out by the cells of the ciliated epithelial cells and glands that produce bronchial secret. If the cause of the disease is established, the etiotropic and pathogenetic treatment of the underlying disease should be carried out. Of special interest in pediatric pulmonology is the experience of the combined drug Kashnol. It simultaneously affects almost all parts of the pathogenesis of acute and chronic broncho-pulmonary diseases.


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