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Current Problems in Cancer Aug 2021Inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal tumor of intermediate malignant potential that predominantly affects children, adolescents and young... (Review)
Review
Inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal tumor of intermediate malignant potential that predominantly affects children, adolescents and young adults. IMT has a predilection for the lung, abdomen, pelvis, and retroperitoneum, however, can affect any part of the body. IMT is typically localized, and multifocal or metastatic disease is uncommon. Complete surgical resection is the treatment of choice when feasible. There is no established standard of care for unresectable and advanced IMT. Approximately half of IMTs harbor anaplastic lymphoma kinase (ALK) gene rearrangements, and fusions involving ROS1, PDGFRβ, RET and NTRK have also been described. Given the molecular landscape of IMT, management of these tumors has evolved to include tyrosine kinase inhibitors and novel targeted therapeutics. This review highlights the molecular characteristics, evolution of targeted therapies and the remaining challenges in the management of IMT.
Topics: Adolescent; Antineoplastic Agents; Child; Child, Preschool; Female; Humans; Infant; Male; Molecular Targeted Therapy; Neoplasms, Muscle Tissue; Pyrimidines; Sulfones; Treatment Outcome
PubMed: 34244015
DOI: 10.1016/j.currproblcancer.2021.100768 -
The Journal of Hand Surgery... Oct 2023Angioleiomyoma is a rare benign soft tissue tumour arising from smooth muscle, representing <1% of upper limb soft tissue tumours. We report a 54-year-old male... (Review)
Review
Angioleiomyoma is a rare benign soft tissue tumour arising from smooth muscle, representing <1% of upper limb soft tissue tumours. We report a 54-year-old male presenting with a progressively enlarging atraumatic lesion along the palmar side of the base of the ring and little finger. A biopsy was done to determine the diagnosis. Intraoperatively, the lump was found to be intimately related to the radial digital artery, it could not be excised en-bloc without transecting the radial digital artery of the little finger. Following excision, the ends of the digital artery were anastomosed. At 10-months follow-up, the hand was fully functional without any evidence of cold-intolerance or neurological deficit along the distribution of the digital nerve. We review the literature on angioleiomyoma and report careful resection of the tumour with digital artery transection and repair as a treatment option for angioleiomyoma of the digital artery. Level V (Therapeutic).
Topics: Male; Humans; Middle Aged; Angiomyoma; Soft Tissue Neoplasms; Hand; Peripheral Nerves; Fingers
PubMed: 37881819
DOI: 10.1142/S2424835523720207 -
Clinical Radiology Apr 2017There is a myriad of potential mass lesions that occur in the popliteal fossa, which present as palpable masses or are found incidentally on imaging. With a thorough... (Review)
Review
There is a myriad of potential mass lesions that occur in the popliteal fossa, which present as palpable masses or are found incidentally on imaging. With a thorough knowledge and understanding of the appearances and locations of these different entities, one can narrow the differential diagnoses in the majority of cases. This will eliminate unnecessary additional investigations and enable a more rapid management. We present a review of frequently encountered and less common entities using an anatomical sieve, with the aim of providing a diagnostic approach to popliteal fossa masses.
Topics: Bursa, Synovial; Diagnosis, Differential; Diagnostic Imaging; Humans; Joint Capsule; Joint Diseases; Knee Joint; Neoplasms, Adipose Tissue; Neoplasms, Muscle Tissue; Osteochondroma; Popliteal Cyst; Soft Tissue Neoplasms
PubMed: 28007292
DOI: 10.1016/j.crad.2016.11.010 -
Head and Neck Pathology Dec 2015Rhabdomyosarcoma is a relatively common soft tissue sarcoma that frequently affects children and adolescents and may involve the head and neck. Rhabdomyosarcoma is... (Review)
Review
Rhabdomyosarcoma is a relatively common soft tissue sarcoma that frequently affects children and adolescents and may involve the head and neck. Rhabdomyosarcoma is defined by skeletal muscle differentiation which can be suggested by routine histology and confirmed by immunohistochemistry for the skeletal muscle-specific markers myogenin or myoD1. At the same time, it must be remembered that when it comes to head and neck malignancies, skeletal muscle differentiation is not limited to rhabdomyosarcoma. A lack of awareness of this phenomenon could lead to misdiagnosis and, subsequently, inappropriate therapeutic interventions. This review focuses on malignant neoplasms of the head and neck other than rhabdomyosarcoma that may exhibit rhabdomyoblastic differentiation, with an emphasis on strategies to resolve the diagnostic dilemmas these tumors may present. Axiomatically, no primary central nervous system tumors will be discussed.
Topics: Head and Neck Neoplasms; Humans; Muscle, Skeletal; Neoplasms, Muscle Tissue; Rhabdomyosarcoma
PubMed: 25757816
DOI: 10.1007/s12105-015-0624-2 -
Veterinary Pathology Mar 2022Canine smooth muscle tumors (SMTs) commonly develop in the alimentary and female genital tracts and less frequently in soft tissue. The definition of histological...
Canine smooth muscle tumors (SMTs) commonly develop in the alimentary and female genital tracts and less frequently in soft tissue. The definition of histological criteria of malignancy is less detailed for SMTs in dogs than in humans. This study evaluated the clinicopathologic features of canine SMTs and compared the veterinary and human medical criteria of malignancy. A total of 105 canine SMTs were evaluated histologically and classified according to both veterinary and human criteria. The Ki67 labeling index was assessed in all SMTs. Estrogen receptor (ER) and progesterone receptor (PR) expression was evaluated for soft tissue SMTs. Follow-up data were available in 25 cases. SMTs were diagnosed in the female genital tract (42%), alimentary tract (22%), and soft tissue (20%). Soft tissue SMTs frequently arose in the perigenital area, pelvic cavity, and retroperitoneum. A subset of soft tissue SMTs expressed ER and/or PR, resembling the gynecologic type of soft tissue SMT in humans. SMTs were less frequently malignant when assessed with human criteria than with veterinary criteria, better reflecting their benign behavior, especially in the genital tract where human criteria tolerate a higher mitotic count for leiomyoma. Decreased differentiation was correlated with increased proliferation, necrosis, and reduced desmin expression. Mitotic count, Ki67 labeling index, and necrosis were correlated with metastases and tumor-related death. Further prognostic studies are warranted to confirm the better performance of the human criteria when assessing SMT malignancy, especially genital cases, to confirm their usefulness in ER/PR-expressing soft tissue SMTs, and to better define the most useful prognostic parameters for canine SMTs.
Topics: Animals; Dog Diseases; Dogs; Female; Ki-67 Antigen; Leiomyoma; Leiomyosarcoma; Male; Muscle, Smooth; Necrosis; Smooth Muscle Tumor
PubMed: 34955045
DOI: 10.1177/03009858211066862 -
Journal of Gastrointestinal Surgery :... Mar 2021
Topics: Granuloma, Plasma Cell; Humans; Neoplasms, Muscle Tissue
PubMed: 32632730
DOI: 10.1007/s11605-020-04711-6 -
RoFo : Fortschritte Auf Dem Gebiete Der... Aug 2017
Topics: Child; Diagnosis, Differential; Female; Humans; Lung Neoplasms; Neoplasms, Muscle Tissue; Pneumonia; Radiography, Thoracic; Rare Diseases; Tomography, X-Ray Computed
PubMed: 28683506
DOI: 10.1055/s-0043-108690 -
Surgical Pathology Clinics Sep 2015The diagnostic spectrum of spindle cell neoplasms arising in the retroperitoneum is wide and, in the presence of commonly shared morphologic features, it may be... (Review)
Review
The diagnostic spectrum of spindle cell neoplasms arising in the retroperitoneum is wide and, in the presence of commonly shared morphologic features, it may be challenging to establish a correct diagnosis in certain cases. Beyond seemingly undifferentiated spindle cell morphology, most neoplasms may reveal distinctive adipocytic, smooth muscle or myofibroblastic or nerve sheath differentiation and show additional diagnostic clues or characteristic molecular abnormalities. Obtaining sufficient and representative biopsy material, a thorough work-up, and extensive sampling of gross specimens followed by a combined histopathologic, immunohistochemical, and, if necessary, molecular work-up of these cases is advisable so as not to miss important diagnostic and/or prognostic indicators.
Topics: Diagnosis, Differential; Humans; Leiomyoma; Leiomyosarcoma; Liposarcoma; Neoplasms, Muscle Tissue; Nerve Sheath Neoplasms; Neurilemmoma; Prognosis; Retroperitoneal Neoplasms; Sarcoma; Soft Tissue Neoplasms
PubMed: 26297061
DOI: 10.1016/j.path.2015.05.007 -
Revista de La Facultad de Ciencias... Sep 2022Primary skin leiomyosarcomas are infrequent neoplasms. They correspond to 2-3% of skin sarcomas and are most frequently located on the lower extremities, trunk and...
INTRODUCTION
Primary skin leiomyosarcomas are infrequent neoplasms. They correspond to 2-3% of skin sarcomas and are most frequently located on the lower extremities, trunk and genitals.
METHODS
We present a case of a 73-year-old man with a 4-month evolution of foreskin leiomyosarcoma. The lesion was biopsied for histopathological study with HE and immunohistochemistry with smooth muscle actin, specific muscle actin, CD34, p63 and S-100 (-).
RESULTS
We observed a leiomyosarcoma of high histological grade and mitotic count. It was positive by immunohistochemistry for smooth muscle actin, while the other markers were negative. Surgical limits were compromised so a reoperation with wide margins of healthy tissue was necessary.
CONCLUSION
The skin lesions should be removed all, without exception, since they can be neoplasms of variable biological behavior. The histological study must be complemented with immunohistochemistry to differentiate them from other neoplasms. For the prognosis, the histological grade, size, location and the possibility of resection with wide margins must be taken into account.
Topics: Actins; Aged; Foreskin; Humans; Immunohistochemistry; Leiomyosarcoma; Male; Prognosis
PubMed: 36149076
DOI: 10.31053/1853.0605.v79.n3.34628 -
Current Treatment Options in Oncology Apr 2023Leiomyosarcoma arises from smooth muscle and represents one of the most common soft tissue sarcomas. Despite aggressive multimodality care, over half of the patients... (Review)
Review
Leiomyosarcoma arises from smooth muscle and represents one of the most common soft tissue sarcomas. Despite aggressive multimodality care, over half of the patients will ultimately develop metastatic and incurable disease with a median survival of 12-18 months. At present, there is no standard system to classify leiomyosarcoma, which itself is a heterogeneous disease. Classification by tumor location is the most simplistic approach and is most frequently utilized in clinical practice. Tumor location impacts diagnosis (recognition pre-operatively versus at the time of surgery) as well as treatment (ability to completely resect with clear margins with minimal morbidity). While tumor location can impact prognosis, for example, extremity tumors would generally be considered as lower risk than inferior vena cava tumors, leiomyosarcoma can exhibit a heterogeneous behavior irrespective of tumor location. Specifically, some patients have rapidly progressing disease despite aggressive chemotherapy, while others display a more indolent course even in the metastatic setting. The pathogenic drivers of the heterogeneity observed in tumor behavior are not well understood. As we learn more about the molecular composition of leiomyosarcoma, various classification groups have been proposed as discussed here. Ultimately, it is unlikely that one variable will be adequate for tumor classification, and a combination of location and molecular composition will be necessary to develop appropriate risk stratification nomograms and treatment strategies.
Topics: Humans; Leiomyosarcoma; Sarcoma; Prognosis; Vena Cava, Inferior; Soft Tissue Neoplasms
PubMed: 36884163
DOI: 10.1007/s11864-023-01067-2