Intracranial angiomatoid fibrous histiocytoma presenting as recurrent multifocal intraparenchymal hemorrhage

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.

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The Vascular Swirls- Angiomatoid Fibrous Histiocytoma

Journal of Clinical and Diagnostic Pathology ◽

10.14302/issn.2689-5773.jcdp-20-3648 ◽

2021 ◽

Vol 1 (3) ◽

pp. 15-23

Author(s):

Anubha Bajaj

Keyword(s):

Soft Tissue ◽

Malignant Fibrous Histiocytoma ◽

Distant Metastasis ◽

Fibrous Histiocytoma ◽

Soft Tissue Neoplasm ◽

Undifferentiated Pleomorphic Sarcoma ◽

Angiomatoid Fibrous Histiocytoma ◽

Pleomorphic Sarcoma

Angiomatoid fibrous histiocytoma (AFH) is an exceptional, soft tissue neoplasm of indeterminate lineage and intermediate malignancy associated with minimal localized tumour reoccurrence and infrequent distant metastasis. Preliminarily contemplated to be a variant or derivative of malignant fibrous histiocytoma or undifferentiated pleomorphic sarcoma or an unusual fibrohistiocytic sarcoma, angiomatoid fibrous histiocytoma predominantly incriminates young po pulation and superficial sites although several extra-somatic sites can be implicated.

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Angiomatoid Fibrous Histiocytoma: A Review With Recent Genetic Findings

Archives of Pathology & Laboratory Medicine ◽

10.5858/2008-132-273-afharw ◽

2008 ◽

Vol 132 (2) ◽

pp. 273-277 ◽

Cited By ~ 4

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.

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Intramammary Angiomatoid Fibrous Histiocytoma, a Rare EWSR1 Rearranged Mesenchymal Neoplasm in a Previously Unreported Anatomic Location with Review of the Cleveland Clinic Experience

Case Reports in Pathology ◽

10.1155/2019/9012878 ◽

2019 ◽

Vol 2019 ◽

pp. 1-6

Author(s):

F. K. Bruehl ◽

K. L. Cooper ◽

S. E. Kilpatrick ◽

M. D. Weindel ◽

M. Ganea ◽

...

Keyword(s):

Fibrous Histiocytoma ◽

Cleveland Clinic ◽

Biological Behavior ◽

Spindle Cell Neoplasm ◽

Angiomatoid Fibrous Histiocytoma ◽

Anatomic Location ◽

Mesenchymal Neoplasm ◽

Subcutaneous Tissues ◽

Clinic Experience ◽

Morphological Variants

Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue tumor that is most commonly reported to arise in the subcutaneous tissues of the upper extremities in adolescents and young adults. At present, the WHO classifies this neoplasm as a tumor of uncertain differentiation. AFH is most often clinically regarded as a tumor of intermediate risk due to low reported rates of recurrence and only rare occurrences of metastases. Its histomorphological hallmarks are a prominent lymphoid cuff surrounding a spindle cell neoplasm with syncytial-appearing cytoplasm. Several variant morphologies have been described. Genetically, the tumor is characterized by translocations involving the EWSR1 gene in over 90% of cases. A widening range of anatomical locations and morphological variants of AFH has been reported in the literature; however, neither anatomic location nor specific morphologic features have been shown to correlate with clinical/biological behavior. We report a unique case of AFH arising in the parenchyma of the breast. The neoplasm showed the typical histomorphology including a peripheral lymphoid cuff. The lesional cells in this case were found to be immunoreactive with desmin, and a positive EWSR1 result was confirmed by break-apart fluorescence in situ hybridization testing. To our knowledge, this is the first report of AFH arising in the breast parenchyma of a postmenopausal female.

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Angiomatoid fibrous histiocytoma (AFH) unusual clinical presentation and unique radiological findings

BJR|case reports ◽

10.1259/bjrcr.20190069 ◽

2020 ◽

pp. 20190069

Author(s):

Mohammed Khader ◽

Tahiya Alyafei ◽

Sulafa Ibrahim ◽

Orwa Elaiwy

Keyword(s):

Fibrous Histiocytoma ◽

Final Diagnosis ◽

Minor Trauma ◽

Imaging Features ◽

Angiomatoid Fibrous Histiocytoma ◽

Mesenchymal Neoplasms ◽

History Of ◽

Children And Young Adults ◽

Vascular Channels ◽

Profunda Femoris

Angiomatoid fibrous histiocytoma (AFH) are rare soft tissue mesenchymal neoplasms that commonly affect children and young adults. They are classified as “intermediate tumours of uncertain differentiation”. We present a case of an 8-year-old child with a left thigh AFH and antecedent history of minor trauma showing perilesional oedema and enhancement at MRI, leading to an initial working diagnosis of infected haematoma that contributed to the challenge in reaching the final diagnosis. Although most of the imaging features of AFH previously described in the literature are demonstrated in this case, the presence of arterial vascular channels within the tumour and feeding branch from the left profunda femoris artery is unusual and to our knowledge the first to be published in the literature.

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Primary intracranial angiomatoid fibrous histiocytoma presenting with anaemia and migraine-like headaches and aura as early clinical features

Cephalalgia ◽

10.1177/0333102415583988 ◽

2015 ◽

Vol 35 (14) ◽

pp. 1334-1336 ◽

Cited By ~ 10

Author(s):

Jakob Møller Hansen ◽

Vibeke Andrée Larsen ◽

David Scheie ◽

Arie Perry ◽

Jane Skjøth-Rasmussen

Keyword(s):

Fibrous Histiocytoma ◽

Brain Mri ◽

Tumor Removal ◽

Visual Aura ◽

Angiomatoid Fibrous Histiocytoma ◽

Hospital Referral ◽

Resection Specimen ◽

Intracerebral Tumor ◽

Systemic Symptoms ◽

Rare Diagnosis

A 17-year-old female with migraine with aura complained of fatigue and was diagnosed with anemia. Three years later, changes in her headache pattern prompted hospital referral. Brain MRI showed a bi-lobed extra-axial intracerebral tumor encroaching both parieto-occipital regions. The resection specimen yielded a rare diagnosis of primary intracranial angiomatoid fibrous histiocytoma (AFH). Tumor removal resulted in cessation of her migraine and anemia. AFH may cause systemic symptoms – in this case fatigue and anemia – long before focal neurological symptoms are present. This is the first report of an intracranial AFH presenting as migraine with visual aura and anemia.

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Angiomatoid Fibrous Histiocytoma in the Forearm: A Case Report

Cancer and Clinical Oncology ◽

10.5539/cco.v6n1p36 ◽

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.

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

Surgical Neurology International ◽

10.25259/sni_282_2020 ◽

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.

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Angiomatoid Fibrous Histiocytoma: The Current Status of Pathology and Genetics

Archives of Pathology & Laboratory Medicine ◽

10.5858/arpa.2014-0234-ra ◽

2015 ◽

Vol 139 (5) ◽

pp. 674-682 ◽

Cited By ~ 45

Author(s):

Khin Thway ◽

Cyril Fisher

Keyword(s):

Fibrous Histiocytoma ◽

Case Reports ◽

Correct Diagnosis ◽

Molecular Genetic ◽

Case Series ◽

Current Status ◽

Gene Fusions ◽

Soft Tissue Neoplasm ◽

Medical Databases ◽

Angiomatoid Fibrous Histiocytoma

Context Angiomatoid fibrous histiocytoma (AFH) is a rare soft tissue neoplasm of intermediate biologic potential and uncertain differentiation, most often arising in the superficial extremities of children and young adults. While it has characteristic histologic features of nodular distributions of ovoid and spindle cells with blood-filled cystic cavities and a surrounding dense lymphoplasmacytic infiltrate, there is a significant morphologic spectrum, which coupled with its rarity and lack of specific immunoprofile can make diagnosis challenging. Angiomatoid fibrous histiocytoma is associated with 3 characteristic gene fusions, EWSR1-CREB1 and EWSR1-ATF1, which are also described in other neoplasms, and rarely FUS-ATF1. Angiomatoid fibrous histiocytoma is now recognized at an increasing number of sites and is known to display a variety of unusual histologic features. Objective To review the current status of AFH, discussing putative etiology, histopathology with variant morphology and differential diagnosis, and current genetics, including overlap with other tumors harboring EWSR1-CREB1 and EWSR1-ATF1 fusions. Data Sources Review of published literature, including case series, case reports, and review articles, in online medical databases. Conclusions The occurrence of AFH at several unusual anatomic sites and its spectrum of morphologic patterns can result in significant diagnostic difficulty, and correct diagnosis is particularly important because of its small risk of metastasis and death. This highlights the importance of diagnostic recognition, ancillary molecular genetic confirmation, and close clinical follow-up of patients with AFH. Further insight into the genetic and epigenetic changes arising secondary to the characteristic gene fusions of AFH will be integral to understanding its tumorigenic mechanisms.

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