Elsevier

World Neurosurgery

Volume 96, December 2016, Pages 403-409
World Neurosurgery

Original Article
Intracranial Angiomatoid Fibrous Histiocytoma: Case Report and Literature Review

https://doi.org/10.1016/j.wneu.2016.09.059Get rights and content

Background

Angiomatoid fibrous histiocytoma (AFH) is a rare and low-grade soft tissue lesion that typically arises from subcutaneous and deep dermal tissue of extremities. The first case was reported in 1979 by Enzinger and has since become known as a distinct entity. AFH has been increasingly reported in different organ systems, with rare reports of primary intracranial AFH. To date there have been 3 reports of intracranial AFH and 1 report of metastasis to the brain, most of which were in young adults.

Case Description

In this paper, we present a case of an older patient with a large, petrous apex AFH that was clinically mistaken for a trigeminal nerve schwannoma. We discuss radiographic and histologic features initially found and the findings that ultimately led to the diagnosis of AFH. We also discuss the findings noted in all other reports of intracranial AFH.

Conclusion

We present a rare case of intracranial AFH in a patient relatively old for onset of diagnosis. To date, only 3 prior cases of AFH have been reported. The radiographic findings were nonspecific and initially pointed toward a diagnosis of schwannoma, whereas histopathology seemed to initially suggest meningioma. Further pathologic consultation finally confirmed AFH as the diagnosis. We suspect there are more cases of intracranial AFH that are misdiagnosed due to variability of findings on pathology. The behavior of this tumor remains in question as 1 of the 3 reported cases demonstrated significant recurrence. As such, gross total resection of this lesion is preferable.

Introduction

Angiomatoid fibrous histiocytoma (AFH) is a rare, low-grade soft tissue tumor that arises primarily from deep dermal and subcutaneous tissue of the extremities in children and young adults.1, 2, 3 It accounts for nearly 0.3% of all soft tissue tumors. Most patients present in the first 3 decades of life, but there is a wide distribution of age.4 AFH has an indolent behavior with 2%–11% local recurrence and <1% metastasis. No factors have been identified to alter prognosis with the exception of wide local excision of tumor.5

Before Enzinger's first official report of AFH in 1979, this entity was misdiagnosed as sclerosing hemangioma, dermatofibroma, and fibrous histiocytoma, among other diagnoses.3 It was later defined as a variant of malignant fibrous histiocytoma arising in younger populations and at superficial sites as opposed to the typical presentation of malignant fibrous histiocytoma, a tumor within deep soft tissues of older adults.2 AFH lesions tend to be small, ranging from 2–4 cm and reaching a maximum reported size of 10 cm.3 Larger studies of AFH have shown a favorable prognosis with gross total resection (GTR), leading to its identification as a separate entity from malignant fibrous histiocytoma. As more specific markers are becoming available, this rare tumor is being identified in more locations than previously noted.4

AFH rarely presents intracranially. Four reports of intracranial AFH exist in the literature to date, 3 primary lesions and 1 that was thought to be a metastasis on the basis of imaging alone.6, 7, 8 These primary lesions were initially misdiagnosed as cavernous malformations or unusual meningioma, but the correct diagnosis was made after using a combination of histologic, immunohistochemical, and molecular features characteristic of AFH.

In this report, we present a rare case of primary, intracranial petrous apex AFH in an older female patient.

Section snippets

Case Report

A 58-year-old female presented with right facial weakness, pain, and numbness. Notably, she had also experienced a 30-pound unintentional weight loss over the previous year and a previous tooth infection several months prior. The facial pain had developed shortly after her tooth infection but persisted for several months and became unbearable. The patient attributed her weight loss to the loss of taste sensation on the right side of her tongue and intolerable pain while eating. On physical

Discussion

AFH is a benign, soft tissue tumor arising in dermal and subcutaneous tissue that tends to affect patients in the first few decades of life. This rare tumor shares histologic and radiographic features with other fibrous lesions, thus lending to its diagnostic difficulty. As mentioned previously, the first diagnosis of AFH was made in 1979 by Enzinger.3 This first case was initially misdiagnosed as multiple other pathologies. Since then, there have been numerous case reports classifying AFH as a

Conclusion

In this report, we present a rare case of primary intracranial AFH in a 58-year-old patient. Before this, only 3 cases of primary intracranial AFH had been reported in individuals ranging from 17–35 years old. The radiographic and histopathologic findings were nonspecific and initially pointed toward a schwannoma, clinically, and meningioma, pathologically. Further pathologic consultation finally supported the diagnosis of AFH. We suspect there are more cases of intracranial AFH that are

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Conflict of interest statement: The authors have no conflicts of interest with regard to this submission.

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