Angiomatoid fibrous histiocytoma: Clinicopathological spectrum of five cases, including EWSR1‑CREB1 positive result in a single case.
Loading...
Date
2016-04
Journal Title
Journal ISSN
Volume Title
Publisher
Abstract
Background: Angiomatoid fibrous histiocytoma (AFH) is an unusual soft tissue
tumor (STT), characterized by recurrences, but rarely metastasis. Later, certain
molecular signatures have been identified underlying this tumor, which at times,
is either underdiagnosed as a benign vascular tumor, or over diagnosed as a
high‑grade pleomorphic sarcoma, including a malignant fibrous histiocytoma.
Materials and Methods: Over a 14‑year‑period, five diagnosed cases of
AFH were analyzed. Results: Five tumors occurred in three males and two
females, over a wide age‑range (median = 21, mean = 30 years); mostly in
the extremities (4) (80%). Microscopically, most tumors were circumscribed,
comprising large, blood‑filed spaces with surrounding histiocytic cells and
a “cuff” of lymphoplasmacytic cells. Three tumors revealed solid growth
pattern with polygonal to spindle cells, including myxoid matrix in one of these
tumors. On molecular analysis, this tumor exhibited EWS‑CREB transcript.
Immunohistochemically, various tumors were positive for CD68 (n = 2/2),
epithelial membrane antigen (n = 3/4), CD99/MIC2 (n = 2/3), and desmin (n = 1/4).
All tumors were surgically excised. On follow‑up (n = 2), a single patient, who
underwent wide‑excision was free‑of‑disease (24 months), while another patient
had a recurrence 4 months post tumor excision. Conclusions: This forms as
the first documented series on clinicopathological features of AFH, a rare STT,
from our country. Significant clinicopathological features include younger age,
extremities as commonest site and histopathological appearance of blood‑filled
spaces with surrounding “cuff” of histiocytic cells and lymphocytes. Tumors with
unusual histopathological tumor patterns require molecular confirmation. Surgical
resection remains the treatment mainstay.
Description
Keywords
Angiomatoid fibrous histiocytoma, EWSR1‑ATF1, EWSR1‑CREB1, malignant fibrous histiocytoma
Citation
Rekhi Bharat, Adamane Shraddha, Ghodke Kiran, Desai Saral, Jambhekar Nirmala A. Angiomatoid fibrous histiocytoma: Clinicopathological spectrum of five cases, including EWSR1‑CREB1 positive result in a single case. Indian Journal of Pathology & Microbiology. 2016 Apr-June 59(2): 148-152.