scholarly journals Case Report: Adult NTRK-Rearranged Spindle Cell Neoplasm: Early Tumor Shrinkage in a Case With Bone and Visceral Metastases Treated With Targeted Therapy

2022 ◽  
Vol 11 ◽  
Author(s):  
Federica Recine ◽  
Alessandro De Vita ◽  
Valentina Fausti ◽  
Federica Pieri ◽  
Alberto Bongiovanni ◽  
...  
Keyword(s):  
Spindle Cell ◽  
Tumor Regression ◽  
Wide Spectrum ◽  
World Health ◽  
Bone Lesions ◽  
Tumor Shrinkage ◽  
Spindle Cell Neoplasm ◽  
Cell Neoplasm ◽  
Early Tumor ◽  
Health Organization

BackgroundNTRK (neurotrophic tyrosine receptor kinase)-rearranged spindle cell neoplasms are a new group of tumors included in the new 5th edition of the World Health Organization (WHO) classification of soft Tissue and Bone Sarcomas. These tumors are characterized by NTRK gene fusions and show a wide spectrum of histologies and clinical behavior. Several targeted therapies have recently been approved for tumors harboring NTRK fusions, including STS.Case PresentationA 26-year-old male with advanced, pretreated NTRK rearranged spindle cell neoplasm and liver, lung and bone metastases was treated with larotrectinib on a continuous 28-day schedule, at a dose of 100 mg twice daily. An 18FDG-PET/CT scan performed after 7 days of treatment showed tumor shrinkage in both visceral and bone lesions. There was no drug-related toxicity. Subsequent evaluations confirmed continued tumor regression in disease sites. The patient is well and continues treatment.ConclusionThe clinical and radiological response of our patient with an uncommon TPM4 (exon 7)-NTRK1 (exon 12) gene fusion tumor treated with a first-generation TRK inhibitor could contribute to a better understanding of the biology of this new STS entity and help to improve patient management.

scholarly journals RARE-13. INFLAMMATORY MYOFIBROBLASTIC TUMOR MIMICKING DESMOPLASTIC INFANTILE GANGLIOGLIOMA (DIG) OF THE TEMPORAL LOBE

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii444-iii444
Author(s):  
Ibrahim Abukhiran ◽  
Gino Bardi Lora ◽  
Patricia Kirby ◽  
Julia Shelton ◽  
Joel Shilyanski ◽  
...  
Keyword(s):  
Temporal Lobe ◽  
Inflammatory Myofibroblastic Tumor ◽  
Spindle Cell ◽  
World Health ◽  
Lung Mass ◽  
Left Temporal Lobe ◽  
Desmoplastic Infantile Ganglioglioma ◽  
Spindle Cell Neoplasm ◽  
Cell Neoplasm ◽  
Temporal Lobe Tumor

Abstract Inflammatory myofibroblastic tumor (IMT) is a mesenchymal neoplasm composed of fascicles of myofibroblastic spindle cells in a background of prominent inflammatory infiltrate. It is categorized as ‘intermediate, rarely metastasizing’ in the World Health Organization classification of tumors of soft tissue and bone. We present a novel case of concurrent brain and lung tumor with diagnosis of TFG-ROS1-rearranged IMT in a 14 year old female patient, in which targeted next-generation sequencing became a powerful tool for detection of genomic alterations in both lung and brain tumors. At age 9, the patient’s lung mass was incidentally found and investigated for various infectious diseases with negative result. At age 14, she presented with seizure and was noted to have a stable size lung mass and a left temporal lobe tumor. The left temporal lobe tumor showed a desmoplastic spindle cell neoplasm involving the meninges and cerebral cortex and Desmoplastic Infantile Ganglioglioma (DIG) was considered one of differentials. Subsequently, her right lung mass was resected and showed a similar spindle cell neoplasm with a background of dense fibrosis and chronic inflammation, consistent with Inflammatory Myofibroblastic Tumor. Molecular microdissection revealed that both tumors shares TFG-ROS1 fusion which is associated with (t(3;6) (q12;q22)), thus it is strongly suggestive that two tumors arose from the same origin. No predisposition syndrome was identified.

Fibromyxomatous spindle cell neoplasm of the ethmoid sinus with extension into the optic cavity: Report of a case and review of the literature

2010 ◽  
Vol 120 (S3) ◽  
pp. S119-S119
Author(s):  
Joshua M Levy ◽  
Christian P Hasney ◽  
Paul L Friedlander ◽  
Michael S Ellis ◽  
Mary A Fazekas-May
Keyword(s):  
Spindle Cell ◽  
Ethmoid Sinus ◽  
Review Of The Literature ◽  
Spindle Cell Neoplasm ◽  
Cell Neoplasm

scholarly journals Spindle cell/sclerosing rhabdomyosarcoma with intracranial invasion without destroying the bone of the skull base: a case report and literature review

2017 ◽  
Vol 6 (8) ◽  
pp. 205846011772731
Author(s):  
Daichi Momosaka ◽  
Osamu Togao ◽  
Akio Hiwatashi ◽  
Koji Yamashita ◽  
Koji Yoshimoto ◽  
...  
Keyword(s):  
Spindle Cell ◽  
Bone Destruction ◽  
Japanese Woman ◽  
World Health ◽  
Imaging Characteristics ◽  
The World ◽  
Sclerosing Rhabdomyosarcoma ◽  
Elderly Japanese ◽  
Health Organization ◽  
Worse Prognosis

Spindle cell/sclerosing rhabdomyosarcoma (ssRMS) is a new subtype of rhabdomyosarcoma included in the World Health Organization soft tissue and bone tumor classification in 2013. Despite the increasing number of reported cases of ssRMS, the imaging characteristics of ssRMS are not established. Herein, we present the case of an elderly Japanese woman with ssRMS of the masticator space with intracranial invasion without destruction of the adjacent bone. Attention should be paid to the presence of intracranial infiltration that may indicate a worse prognosis. Tumor growth without bone destruction could be a key finding to differentiate ssRMSs from conventional subtypes of rhabdomyosarcoma.

scholarly journals Oral fibrosarcoma in jararaca (Bothrops pubescens): anatomopathological and immunohistochemical aspects

2015 ◽  
Vol 35 (7) ◽  
pp. 664-670 ◽  
Author(s):  
Ezequiel D. Santos ◽  
José R. Silva Filho ◽  
Tanise P. Machado ◽  
Stefano L. Dau ◽  
Rubens Rodriguez ◽  
...  
Keyword(s):  
Spindle Cell ◽  
Immunohistochemical Analysis ◽  
Pathological Examination ◽  
Neoplastic Cells ◽  
Spindle Cell Neoplasm ◽  
Two Samples ◽  
Cell Neoplasm ◽  
Morphology Characterization ◽  
Masson Trichrome Staining ◽  
Positive Content

Abstract A 4-year-old female captive-bred snake of the genus Bothrops showed swelling on the left side of the oral cavity, suggesting the development of neoplasia. The mass was removed surgically and sent for pathological examination. Two months later a new increase in volume in the same site was observed, suggesting recurrence. The lesion was completely removed and sent for pathological analysis. Histologically, the two-samples consisted of a mass with highly-cell density composed of spindle-shaped anaplastic cells arranged in interwoven bundles, distributed throughout the tissue extension and, occasionally, polygonal cells arranged in irregular fascicles. The Masson trichrome staining showed modest amount of collagen supporting the neoplastic cells. PAS-positive content was not observed in the cytoplasm of neoplastic cells. Histological and histochemical findings indicated that it was a spindle cell neoplasm, but the classification was not possible. Immunohistochemistry was requested and performed using the streptavidin-biotin-peroxidase method. The markers used were anti-vimentin, anti-PCNA, anti-EMA, anti-melan A and anti-melanosome, anti-desmin, anti-actin, anti-CD68 and anti- S100protein. The neoplastic cells were immunoreactive for vimentin and PCNA and negative for the other antibodies. The morphology characterization, histochemical and immunohistochemical analysis of neoplastic cells allowed the definitive diagnosis of oral fibrosarcoma.

Understanding Bone Conditions

2012 ◽  
Author(s):  
Anne Sutcliffe ◽  
Cameron Swift
Keyword(s):  
Hip Fracture ◽  
Wide Spectrum ◽  
Bone Fragility ◽  
World Health ◽  
Nursing Management ◽  
Skeletal Disease ◽  
Secondary Care Setting ◽  
Definition Of ◽  
Health Organization ◽  
The Uk

The aim of this chapter is to provide nurses with the knowledge to be able to assess, manage, and care for people with bone conditions in an evidence-based and person-centred way. Bone conditions (a major category of musculoskeletal conditions) cover a wide spectrum of diseases, some of which may be considered mild and self-limiting, while others may have a significant impact upon the individual’s quality of life and ability to function. It is estimated that up to 30% of all GP consultations are about musculoskeletal complaints; many are age-associated, and population ageing will continue to increase this demand (Oliver, 2009). The chapter will focus on osteoporosis, hip fracture (perhaps the most serious and costly consequence of osteoporosis or osteopaenia), Paget’s disease, and osteoarthritis, respectively. The chapter will provide a broad overview of these common conditions, enabling a proactive approach to patient care within a multidisciplinary context, whether in the primary or secondary care setting. The nursing management of the symptoms and common health problems associated with bone conditions can be found in several Part 2 chapters, and these are highlighted throughout the chapter. Osteoporosis has been defined as:...A progressive systemic skeletal disease characterised by low bone mass and micro-architectural deterioration of bone tissue, with a consequent increase in bone fragility and susceptibility to fracture. (WHO, 1994)…The World Health Organization (WHO) has recommended a clinical definition of osteoporosis based on a bone mineral density (BMD) measurement of the spine or hip, expressed in standard deviation (SD) units called T scores. Using this definition, an individual is classified as having osteoporosis if his or her T score is ≤–2.5 SD at the spine or hip (WHO, 1994). It is estimated that osteoporosis occurs in approximately 3 million people in the UK, resulting in more than 230,000 fractures per annum, the most frequent being hip, vertebral body, and forearm fractures. In total, 75,000 hip fractures occur annually (British Orthopaedic Association, 2007), with the average age of incidence being 84 and 83 in men and women, respectively (National Hip Fracture Database, 2010).

scholarly journals Myofibroma of the oral cavity. A rare spindle cell neoplasm

2010 ◽  
pp. e596-e600 ◽  
Author(s):  
BF. Brasileiro ◽  
PR. Martins-Filho ◽  
MR. Piva ◽  
LC. da Silva ◽  
CF. Nonaka ◽  
...  
Keyword(s):  
Oral Cavity ◽  
Spindle Cell ◽  
Spindle Cell Neoplasm ◽  
Cell Neoplasm

scholarly journals Myopericytoma of the Neck Originating in the Middle Scalene Muscle: A Case Report

2017 ◽  
Vol 96 (10-11) ◽  
pp. E5-E7 ◽  
Author(s):  
Mark B. Chaskes ◽  
John W. Bishop ◽  
Matthew Bobinski ◽  
D. Gregory Farwell
Keyword(s):  
Needle Biopsy ◽  
Spindle Cell ◽  
Tissue Sample ◽  
Surgical Excision ◽  
Scalene Muscle ◽  
Spindle Cell Neoplasm ◽  
Sensory Deficits ◽  
Complete Surgical Excision ◽  
Supraclavicular Fossa ◽  
Cell Neoplasm

We report a case of myopericytoma of the neck. A 23-year-old woman noticed a small, nontender mass in her left supraclavicular fossa. The mass had grown over a period of 5 months, prompting her to seek evaluation. On examination, no motor or sensory deficits were present. Imaging suggested that a mass had originated in the middle scalene muscle. Computed-tomography–guided core needle biopsy demonstrated a spindle-cell neoplasm with smooth-muscle differentiation. Complete surgical excision was performed. Histopathologic and immunohistochemical evaluations of the tissue sample suggested a myopericytoma. Myopericytoma is an extremely rare tumor of the head and neck. To the best of our knowledge, this is the first reported case of a myopericytoma originating in a scalene muscle.

Solitary Fibrous Tumor of the Kidney. A Propos of a Case

2009 ◽  
Vol 76 (2) ◽  
pp. 112-114 ◽  
Author(s):  
M. Marzi ◽  
M. D'Alpaos ◽  
P. Piras ◽  
A. Paiusco ◽  
M.S. Minervini ◽  
...  
Keyword(s):  
Solitary Fibrous Tumor ◽  
Renal Pelvis ◽  
Spindle Cell ◽  
Left Kidney ◽  
Clinical Behavior ◽  
Spindle Cell Neoplasm ◽  
Solitary Fibrous Tumors ◽  
Genital Apparatus ◽  
Cell Neoplasm ◽  
Fibrous Tumor

Solitary Fibrous Tumors (SFT) are rare spindle cell neoplasm that typically originate from the pleura. However, cases of the SFT are described with origin in other organs, including the urinary and genital apparatus. Within the kidney, except from the renal pelvis, only 19 cases of SFT are described and such rarity of localization makes the histogenesis and the prognosis of the tumor rather unknown. We report the case of a 72-year-old lady who attended our Unit for a mass that was clinically palpable at the level of the left hemiabdomen. The tomodensitographic test indicated a 19cm-diameter mass of likely pertinence of the middle bystender of the left kidney. She had undergone left radical nephrectomy. The histological examination highlighted a solitary fibrous tumor (SFT): the presence of hypercellularity, of cellular pleiomorphism and of a high number of mitosis has led to a histological diagnosis of malignancy for the neoplasm analyzed. The SFT are of rare clinical comparison: this does not allow for a deep knowledge of the lesion histogenesis and prognosis; moreover, the clinical behavior should be more precisely defined.

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