scholarly journals Angiomatoid Fibrous Histiocytoma in the Forearm: A Case Report

2016 ◽  
Vol 6 (1) ◽  
pp. 36
Author(s):  
Hatim Mohammed A Alshareef ◽  
Ahmed Hassan A Alhasan ◽  
Mohammed G. Felemban ◽  
Ashwaq Mohammed J Almalki ◽  
Amer Mohammed A Alshareef
Keyword(s):  
Young Adults ◽  
Soft Tissue ◽  
Soft Tissue Tumor ◽  
Fibrous Histiocytoma ◽  
Full Range ◽  
Common Site ◽  
Tissue Mass ◽  
Skin Changes ◽  
Angiomatoid Fibrous Histiocytoma ◽  
Children And Young Adults

Background: Angiomatoid fibrous histiocytoma [AFH] is a rare soft tissue tumor most commonly occurring in children, adolescents, and young adults. It is considered to be a tumor of intermediate malignancy because of its less aggressive course. The extremities are the most common site of involvement, followed by the trunk and head and neck.Case presentation: A 28-Year-old male presented with a right forearm mass that has been present for 2 years. There was no associated fever, no trauma, no night pain and no constitutional symptoms. On examination he had a forearm mass dorsally measuring 2x3 cm, firm, rounded, mobile with no skin changes and full range motion joint above and joint below. Lab and imaging studies done which showed soft tissue mass, multilobulated complex heterogeneous with increased vascularity. Patient underwent biopsy and surgical excision.Conclusion: Angiomatoid fibrous histiocytoma is a neoplasm of intermediate biologic potential. Most often, the extremities are the most common site of involvement, followed by the trunk and head and neck of children and young adults. Its rarity may lead to misdiagnosis as either a reactive lesion or a benign or higher-grade tumor. This tumor has a relatively rare metastasis and its overall clinical outcome is excellent. It is clinically important to accurately diagnose this tumor so that wide local excision may be performed and patients may be appropriately monitored and followed up.

Angiomatoid Fibrous Histiocytoma: A Review With Recent Genetic Findings

2008 ◽  
Vol 132 (2) ◽  
pp. 273-277 ◽  
Author(s):  
Khin Thway
Keyword(s):  
Young Adults ◽  
Differential Diagnosis ◽  
Malignant Fibrous Histiocytoma ◽  
Fibrous Histiocytoma ◽  
Molecular Genetic ◽  
Angiomatoid Fibrous Histiocytoma ◽  
Lymphoplasmacytic Infiltrate ◽  
Children And Young Adults

Abstract Angiomatoid fibrous histiocytoma is a neoplasm of intermediate biologic potential most often arising in the extremities of children and young adults. Its rarity may lead to misdiagnosis as either a reactive lesion or a benign or higher-grade tumor. Originally described as a type of malignant fibrous histiocytoma, its differentiation remains enigmatic, with precise histogenesis still only hypothesized. Its morphology is distinct, as a circumscribed lesion with sheets of bland spindle to ovoid cells, peripheral lymphoplasmacytic infiltrate, and blood-filled cystic cavities, and half of the cases show strong desmin expression. Cytogenetically, 2 specific translocations, t(12:16)(q13:p11) and more recently t(12:22)(q13:q12), have been characterized. The literature on angiomatoid fibrous histiocytoma is reviewed, particularly with regard to recent molecular genetic developments and differentiation, and its morphology, immunohistochemistry, and differential diagnosis are summarized.

scholarly journals Cranial angiomatoid fibrous histiocytoma: A case report and review of literature

2020 ◽  
Vol 11 ◽  
pp. 295
Author(s):  
Amanda E. Sion ◽  
Rizwan A. Tahir ◽  
Abir Mukherjee ◽  
Jack P. Rock
Keyword(s):  
Case Report ◽  
Soft Tissue Tumor ◽  
Fibrous Histiocytoma ◽  
Subcutaneous Tissue ◽  
Low Grade ◽  
Case Description ◽  
Review Of Literature ◽  
Angiomatoid Fibrous Histiocytoma ◽  
Resected Tumor ◽  
Children And Young Adults

Background: Angiomatoid fibrous histiocytoma (AFH) is a rare low-grade soft-tissue tumor that typically arises from the deep dermal and subcutaneous tissue of the extremities in children and young adults. Intracranial AFH is exceedingly rare, and only four cases of primary AFH tumors have been reported to date. Case Description: A 43-year-old male presented to our hospital with headaches, vision changes, and a known brain tumor suspected to be an atypical meningioma. After undergoing craniotomy for resection of the mass, the immunomorphologic features of the resected tumor showed typical features of AFH with ESWR1 (exon7) – ATF1 (exon 5) fusion. Conclusion: AFH is a difficult tumor to diagnose with imaging and histologic studies. Thus, further knowledge is necessary – particularly of intracranial cases – to aid clinicians in its diagnosis and management.

scholarly journals CT density of cervical thymus, in comparison with mediastinal thymus

2019 ◽  
Vol 10 (1) ◽  
Author(s):  
Anitha Sen ◽  
Jiji Valsalamony ◽  
Jubie Raj
Keyword(s):  
Young Adults ◽  
Soft Tissue ◽  
Older Age ◽  
List Type ◽  
Age Group ◽  
Positive Correlation ◽  
Children And Young Adults ◽  
Criteria For Diagnosis ◽  
Ct Density ◽  
Moderate Positive Correlation

Abstract Objectives Cervical component of thymus is noted more in children and young adults than in older age group. CT texture (lobules of soft tissue interspersed with fat), similarity with CT density of mediastinal thymus and continuity with mediastinal thymus on sagittal/coronal images, are given as the criteria for diagnosis of the cervical thymus. But CT densities of cervical and mediastinal components of the thymus may vary. The purpose of our study was to compare CT densities of cervical and mediastinal parts of the thymus, in cases where ultrasonography correlation was available. Methods We retrospectively identified 22 patients who had undergone CT between May 2015 and May 2017 and in whom ultrasonography (USG) correlation was available. CT densities of cervical and mediastinal components of thymus were measured. Results CT density of cervical thymus is lower than the CT density of mediastinal thymus by ~ 25 HU. There is a moderate positive correlation between CT densities of cervical and mediastinal parts of the thymus. CT densities of both cervical and mediastinal thymus were found to reduce with age, but the reduction was statistically significant only in the cervical thymus in this study. Conclusions CT densities of cervical and mediastinal components of the thymus may vary, with CT density of cervical thymus being lower. There is a positive correlation between CT densities of cervical and mediastinal parts of the thymus. CT density of cervical thymus reduces with age. Understanding these may help avoid confusion on CT and avoid the need for correlative USG, saving time and effort.

Primitive Neuroectodermal Tumor of the Stomach

2005 ◽  
Vol 129 (1) ◽  
pp. 107-110 ◽  
Author(s):  
Raoulin Soulard ◽  
Valère Claude ◽  
Philippe Camparo ◽  
Jean-Philippe Dufau ◽  
Patrick Saint-Blancard ◽  
...  
Keyword(s):  
Polymerase Chain Reaction ◽  
Young Adults ◽  
Soft Tissue ◽  
Ewing Sarcoma ◽  
Primitive Neuroectodermal Tumor ◽  
Neuron Specific Enolase ◽  
Neuroectodermal Tumor ◽  
Chain Reaction ◽  
Children And Young Adults ◽  
Polymerase Chain

Abstract Ewing sarcoma/primitive neuroectodermal tumor is classically a tumor of the soft tissue or bone in children and young adults, but several cases have been described in patients of all ages. Within the last decade, the clinicopathologic spectrum of Ewing sarcoma/primitive neuroectodermal tumor has been markedly expanded by recognition that the tumor may also have a visceral origin. We describe a case of primitive neuroectodermal tumor arising in the stomach of a 66-year-old woman. The neoplasm was excised using a radical surgical procedure. Microscopically, the tumor was made up of solid nests and sheets of round cells. Immunohistochemically, the tumor cells showed immunoreactivity for CD99, S100, neuron-specific enolase, and vimentin. A multiplex real-time polymerase chain reaction assay detected an EWS-ERG fusion. To our knowledge, this is the first description of a primitive neuroectodermal tumor arising in the stomach.

scholarly journals Subungual Glomus Tumor- Clinical Presentation And Treatment

2014 ◽  
Vol 3 (2) ◽  
pp. 45-48 ◽  
Author(s):  
Shyamal Chandra Debnath ◽  
Suman Kumar Roy ◽  
RR Kairy
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Tumor ◽  
Clinical Presentation ◽  
Glomus Tumor ◽  
Rare Condition ◽  
Surgical Excision ◽  
Tissue Mass ◽  
Delay In Diagnosis ◽  
Nail Deformity

Glomus tumor is a rare condition and constitutes only 1 % of all soft tissue tumor of body. Glomus tumor is familiar for its unusual presentation and long standing symptoms due to delay in diagnosis. Authors have presented the clinical behavior and treatment of 7 patients with subungual soft tissue mass. Following surgical excision, all patients cured of symptoms, at the end of follow up, no recurrence occurred; post operative nail deformity was insignificant. DOI: http://dx.doi.org/10.3329/bdjps.v3i2.18250 Bangladesh Journal of Plastic Surgery July 2012, 3(2): 45-48

scholarly journals Therapeutic Approaches Targeting PAX3-FOXO1 and Its Regulatory and Transcriptional Pathways in Rhabdomyosarcoma

Molecules ◽  
2018 ◽  
Vol 23 (11) ◽  
pp. 2798 ◽  
Author(s):  
Thanh Hung Nguyen ◽  
Frederic G. Barr
Keyword(s):  
Skeletal Muscle ◽  
Transcription Factor ◽  
Young Adults ◽  
Soft Tissue ◽  
Chromosomal Translocation ◽  
Murine Models ◽  
Therapeutic Approaches ◽  
Recent Developments ◽  
Children And Young Adults ◽  
Oncogenic Transcription Factor

Rhabdomyosarcoma (RMS) is a family of soft tissue cancers that are related to the skeletal muscle lineage and predominantly occur in children and young adults. A specific chromosomal translocation t(2;13)(q35;q14) that gives rise to the chimeric oncogenic transcription factor PAX3-FOXO1 has been identified as a hallmark of the aggressive alveolar subtype of RMS. PAX3-FOXO1 cooperates with additional molecular changes to promote oncogenic transformation and tumorigenesis in various human and murine models. Its expression is generally restricted to RMS tumor cells, thus providing a very specific target for therapeutic approaches for these RMS tumors. In this article, we review the recent understanding of PAX3-FOXO1 as a transcription factor in the pathogenesis of this cancer and discuss recent developments to target this oncoprotein for treatment of RMS.

Primary Pleural Synovial Sarcoma

2003 ◽  
Vol 127 (1) ◽  
pp. 85-90 ◽  
Author(s):  
Siok Bian Ng ◽  
Qasim Ahmed ◽  
Sim Leng Tien ◽  
Christina Sivaswaren ◽  
Lai Ching Lau
Keyword(s):  
Young Adults ◽  
In Situ Hybridization ◽  
Soft Tissue ◽  
Synovial Sarcoma ◽  
Fluorescent In Situ Hybridization ◽  
Soft Tissue Tumor ◽  
Differential Diagnoses ◽  
Positive Staining ◽  
Rare Occurrence

Abstract Synovial sarcoma (SS) is an uncommon soft tissue tumor that occurs primarily in the extremities of young adults, especially in the periarticular region. In this report, we describe the rare occurrence of primary SS of the pleura in a 15-year-old boy. Histologically, the tumor demonstrated monophasic morphologic findings and showed positive staining with vimentin and Bcl-2 and focally for cytokeratin CK7. Fluorescent in situ hybridization identified t(X;18) translocation. The patient developed recurrences 20 months following resection of the tumor. The literature on this uncommon entity is reviewed, and its histogenesis, differential diagnoses, and cytologic features are also discussed.

Adipocyte-rich CTNNB1-mutated Intramuscular Gardner Fibroma Progressing to Desmoid Fibromatosis

2020 ◽  
pp. 109352662096880
Author(s):  
Andrea Bakker ◽  
Jonathan C Slack ◽  
Mara Caragea ◽  
Kyle C Kurek ◽  
Marie-Anne Bründler
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Tumor ◽  
Soft Tissue Tumors ◽  
Molecular Testing ◽  
Tissue Mass ◽  
Desmoid Fibromatosis ◽  
Benign Soft Tissue Tumor ◽  
First Time ◽  
Generation Sequencing

Gardner fibroma (GF) is a benign soft-tissue tumor that is associated with Gardner syndrome and can progress to, or co-occur with, desmoid fibromatosis (DF). Herein, we report a unique case of an 11-year-old boy who presented with a rapidly growing soft-tissue mass after biopsy of a stable fat-rich lesion present in the calf muscles since infancy, with Magnetic resonance imaging findings suggesting an intramuscular adipocytic tumor. The resection showed GF and DF. DF arising from a preexisting GF (the so-called “GF-DF sequence”) is a well-documented phenomenon. Although immunohistochemistry was negative for nuclear β-catenin expression, a CTTNB1 S45F mutation, which has been associated with aggressive behavior in DF, was identified in both components using a next-generation sequencing-based molecular assay. This is the first time a mutation in CTNNB1 has been identified in GF and the GF–DF sequence, thus expanding our knowledge of the molecular pathogenesis of the GF–DF sequence and highlighting the role of molecular testing in pediatric soft-tissue tumors. The histologic findings of an adipocyte-rich intramuscular GF also are unique, expanding the morphological spectrum of GF and adding GF to the differential diagnosis of intramuscular lesions with an adipocytic component.

Intracranial angiomatoid fibrous histiocytoma presenting as recurrent multifocal intraparenchymal hemorrhage

2010 ◽  
Vol 112 (5) ◽  
pp. 978-982 ◽  
Author(s):  
Pawel G. Ochalski ◽  
James T. Edinger ◽  
Michael B. Horowitz ◽  
William R. Stetler ◽  
Geoffrey H. Murdoch ◽  
...  
Keyword(s):  
Cavernous Malformation ◽  
Fibrous Histiocytoma ◽  
Biological Behavior ◽  
Intraparenchymal Hemorrhage ◽  
Soft Tissue Neoplasm ◽  
Recurrent Hemorrhage ◽  
Angiomatoid Fibrous Histiocytoma ◽  
Molecular Studies ◽  
Resection Specimen ◽  
Children And Young Adults

Angiomatoid fibrous histiocytoma (AFH) is a rare soft-tissue neoplasm that most commonly appears in the limbs, typically affecting children and young adults. The tumor has a propensity for local recurrence and recurrent hemorrhage but rarely for remote metastasis. To date, only 2 reports have documented an intracranial occurrence of the tumor (1 of which was believed to be metastatic disease). This is the second report of primary intracranial AFH. Additionally, hemorrhage from an intracranial AFH lesion has yet to be reported, and little is known about the radiographic characteristics and biological behavior of these lesions. In this report, the authors describe the case of a patient with recurrent hemorrhage due to primary multifocal intracranial AFH. Initially misdiagnosed as a cavernous malformation and then an unusual meningioma, the tumor was finally correctly identified when there was a large enough intact resection specimen to reveal the characteristic histological pattern. The diagnosis was confirmed using immunohistochemical and molecular studies.

scholarly journals Subcutaneous Angiomatoid Fibrous Histiocytoma Mimicking Metastatic Melanoma

2012 ◽  
Vol 2012 ◽  
pp. 1-6 ◽  
Author(s):  
E. Sparreboom ◽  
C. Wetzels ◽  
M. Verdijk ◽  
S. Mulder ◽  
W. Blokx
Keyword(s):  
Lymph Node ◽  
Metastatic Melanoma ◽  
Molecular Diagnostics ◽  
Soft Tissue Tumor ◽  
Fibrous Histiocytoma ◽  
Case Reports ◽  
Relevant Literature ◽  
Node Metastasis ◽  
Angiomatoid Fibrous Histiocytoma ◽  
The Past

Angiomatoid fibrous histiocytoma is an uncommon soft-tissue tumor of intermediate malignancy that is often misdiagnosed initially. As there is not one immunohistochemical marker that consequently stains positive or negative for angiomatoid fibrous histiocytoma, molecular diagnostics are becoming more widely used. So far three translocations have been reported to arise in angiomatoid fibrous histiocytoma: FUS-ATF1, EWSR1-CREB1, or EWSR1-ATF1. We present a case of angiomatoid fibrous histiocytoma on the upper arm of a 40-year-old female, which was initially misdiagnosed as metastatic melanoma in a lymph node. Revision of the pathology revealed an angiomatoid fibrous histiocytoma, which was later confirmed by a EWSR1-CREB1 translocation with molecular diagnostics. Furthermore, we review the relevant literature and provide an overview of all available case reports in the past ten years. This case report illustrates the importance for pathologists of knowing the typical pathology features of AFH and integrating immunohistochemical and molecular findings in order to prevent overdiagnosis of lymph node metastasis of a malignancy.

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