Chao-Shiang Li
Wing P. Chan
Wei-Tsung Chen
Chih-Peng Chang
Li-Sun Shih
Ran-Chou Chen
Hsing-Yang Tu
MRI of angiomatoid fibrous histiocytoma
Received: 15 July 2003 Revised: 11 February 2004 Accepted: 12 February 2004 Published online: 16 July 2004 ISS 2004
C.-S. Li · W.-T. Chen · R.-C. Chen · H.-Y. Tu
Department of Radiology, Taipei Municipal Jen-Ai Hospital, Taipei, Taiwan, Republic of China W. P. Chan
Department of Radiology, School of Medicine, Taipei Medical University, Taipei, Taiwan, Republic of China W. P. Chan (
)
)Department of Radiology,
Taipei Municipal Wan-Fang Hospital, 111, Hsing-Long Road, Section 3, 116 Taipei, Taiwan, Republic of China e-mail: [email protected] Tel.: +886-2-29307930 ext. 1300 Fax: +886-2-29316809
C.-P. Chang
Department of Orthopedic Surgery, Taipei Municipal Jen-Ai Hospital, Taipei, Taiwan, Republic of China L.-S. Shih
Department of Pathology, Taipei Municipal Jen-Ai Hospital, Taipei, Taiwan, Republic of China
Abstract
Angiomatoid fibrous
his-tiocytoma is a rare soft tissue tumor
of low-grade malignancy. We present
the case of a 32-year-old man who
complained of soreness and
numb-ness over his left arm and hand over
the previous 2 months and of having
a palpable mass over his left upper
back for 4 years. Magnetic resonance
imaging (MRI) showed an
intramus-cular soft tissue mass in the left
scapular region. The tumor mass was
seen to have multiple cystic
compo-nents with fluid-fluid levels.
Histo-logical examination showed multiple
cystic spaces filled with blood lakes
and hemosiderin deposits in the solid
part of the tumor. After the initial
surgery, the patient had local
recur-rences over 2.5 years. The
immuno-histochemical study at the second
surgery showed that the recurrent
tumor was strongly positive for the
histiocytic marker CD68, and the
myoid trait desmin. Histological
di-agnosis was compatible with
an-giomatoid fibrous histiocytoma.
Keywords
Back · Angiomatoid
fibrous histiocytoma · Soft tissue
tumor · Computed tomography ·
Magnetic resonance imaging (MRI)
Introduction
Angiomatoid fibrous histiocytoma is a rare soft tissue
tumor. Enzinger in 1979 [1] first designated the tumor as
angiomatoid malignant fibrous histiocytoma. The tumor
was later renamed angiomatoid fibrous histiocytoma
be-cause of its slow growth and rare metastasis [2]. To our
knowledge, only one case of angiomatoid fibrous
histio-cytoma with magnetic resonance imaging (MRI) findings
has been described [3]. We present a case of angiomatoid
fibrous histiocytoma occurring in the muscles about the
scapula with striking MRI findings.
Fig. 1 A Contrast-enhanced CT scan shows a soft tissue tumor with hypodense components (arrows) in the left scapular region. Note that there is no enhancement of the tumor. B Axial T1-weighted (TR/TE=554/15 ms) and C T2-T1-weighted (TR/TE=5091/ 100 ms) MR images show a multiloculated soft tissue mass with fluid-fluid levels indicative of layered blood serum. The high-sig-nal-intensity layer (curved arrows) represents serum in the non-dependent position. A focal low-signal-intensity area (open ar-row) is noted on both T1- and T2-weighted images, indicative of hemosiderin deposits. D Contrast-enhanced T1-weighted (TR/TE= 916/15 ms) MR image with fat saturation shows no enhancement of the soft tissue mass. The high signal intensity of the cystic com-ponent (arrow) is an effect of fat suppression. E Gross specimen (cut surface) shows a lobulated mass containing multiple cystic spaces filled with blood clot. F Photomicrograph shows multiple cystic spaces of blood lakes, and hemosiderin deposits (arrow) in
the solid part of the tumor (H&E, 20). G Dense fibrous tissue (fibrous pseudocapsule, thick arrows) is noted in the periphery of the tumor. Lymphoplasmacytic infiltrate with randomly distributed lymph follicles with germinal center formation (arrow) is also noted (H&E, 10). H The solid part of tumor is composed of irregular sheets of histiocyte-like cells with dense neutrophilic and lymphoplasmacytic infiltrate. The blood-filled cystic spaces are lined by tumor cells (H&E, 20). I The tumor cells have eosino-philic cytoplasm, round to oval nuclei, vesicular chromatin pattern, nuclear indentation or grooving, and the tumor is considered to be a histiocytic lesion (H&E, 40). J Immunohistochemical stain for CD68 shows a positive result (brown color in cytoplasm) (perox-idase-antiperoxidase immune complex method). K Desmin ex-pression is also noted (brown color in cytoplasm) (peroxidase-an-tiperoxidase immune complex method)
References
1. Enzinger FM. Angiomatoid malignant fibrous histiocytoma: a distinct fibro-histiocytic tumor of children and young adults simulating a vascular neoplasm. Cancer 1979; 44:2147–2157.
2. Enzinger FM, Weiss SW. Fibrohistio-cytic tumors of intermediate malignan-cy. In: Enzinger FM, Weiss SW, eds. Soft tissue tumors. St Louis: Mosby-Year Book, 1995:325–349.
3. Murphey MD, Gross TM, Rosenthal HG. From the archives of the AFIP. Musculoskeletal malignant fibrous his-tiocytoma: radiologic-pathologic corre-lation. Radiographics 1994; 14:807– 826.
Case report
A 32-year-old man complained of soreness and numbness over his left arm and hand over the past 2 months and of having a palpable mass over his left upper back for 4 years. Physical examination revealed a tender mass in the left scapular region, limitation of range of motion of the shoulder joint, and a normal deep tendon reflex. Laboratory examination revealed anemia (hemoglobin 7.3 gm/dl), normal serum carcinoembryonic antigen, alpha-feto-protein and CA-199, and normal complete coagulation profiles.
Computed tomography (CT) showed a lobulated soft tissue mass with heterogeneous density at the left scapula (Fig. 1A). MRI (1.5 T, Gyroscan ACS-NT, Philips, The Netherlands) showed a 10105 cm intramuscular soft tissue mass in the left scapular region (Fig. 1B—D). The mass was seen to have multiple cystic components with fluid-fluid levels indicative of layered serum. Foci of low signal intensity were noted on both T1- and T2-weighted images, suggesting hemosiderin deposits. Gadolinium-enhanced T1-weighted images showed no enhancement of the presumed areas with hemosiderin deposition.
The tumor was widely excised following the preoperative di-agnosis of hemorrhagic soft tissue tumor. At surgery, a circum-scribed, red-brown and elastic soft tissue mass was encountered. The cut surface of the tumor revealed grayish solid tissue with multiple intervening variable-sized, blood-filled cystic spaces (Fig. 1E). On microscopy, the most striking features were variable-sized blood lakes in the cystic component and prominent hemo-siderin deposition in the hypocellular fibrous tissue of the solid part (Fig. 1F).
One year later, local recurrence and regional metastasis to the left subclavicular region occurred. At the second operation, the recurrent tumor was noted to have more solid and cellular parts than the tumor at the initial operation (Fig. 1G—I). The tumor was composed of relatively bland-looking spindle and round tumor cells, mixed with intense infiltrates of plasma cells and lympho-cytes. The tumor cells were strongly positive for the histiocytic marker CD68 and the myoid trait desmin on immunohistochemical study (Fig. 1J, K). The histological diagnosis was angiomatoid fi-brous histiocytoma.
After the initial surgery, the patient experienced local recur-rences over 2.5 years. He received wide excision and adjuvant radiotherapy four times in this period.
Discussion
Angiomatoid fibrous histiocytoma is a low-grade
neo-plasm which primarily affects children and young adults.
Eighty-eight percent of patients are 30 years of age or
younger [4]. The tumor usually occurs in a superficial
location and most commonly in the extremities, followed
by the trunk and head and neck. Symptoms of anemia,
weight loss, and fever are observed in a minority of cases.
Accurate preoperative diagnosis of angiomatoid
fi-brous histiocytoma is difficult. CT and MRI findings of
angiomatoid fibrous histiocytoma can be similar to those
of malignant fibrous histiocytoma [5]. MRI is superior to
CT in demonstrating fluid-fluid levels within multiple
cystic components of the tumor, indicative of intralesional
hemorrhage [3]. The fluid-fluid levels are nonspecific
findings that may occur in malignant fibrous
histiocyto-ma, soft tissue hemangiohistiocyto-ma, and hematoma [6].
Malig-nant fibrous histiocytoma, however, usually occurs in
old-er adults. An entire lesion filled with blood is not typical
for malignant fibrous histiocytoma, which may have more
enhancing tissue about the hemorrhagic region.
The diagnosis of angiomatoid fibrous histiocytoma is
made on the basis of histopathology and
immunohis-tochemical studies [7, 8]. Four histological features are
commonly identified. These are a fibrous pseudocapsule,
round or spindle fibrohistiocytic proliferation, a
pseudo-angiomatous pattern, and plasmalymphocytic response.
Pseudovascular blood-filled spaces have been observed in
50% of cases [9]; this can be related to multiloculated
cystic components of the tumor, as shown on MRI in our
case. Smith and associates [10] reported that the
histio-cytic marker CD68 was positive in 9 of 19 (47%) cases of
angiomatoid fibrous histiocytoma. Immunopositivity for
myoid or myofibroblastic markers in more than 50% of
cases has also been reported [9].
Local recurrence has been reported in 11% of patients
and distant metastasis in 1%; wide excision is
recom-mended as the treatment of angiomatoid fibrous
histio-cytoma [4, 7]. Local recurrence is attributed to the
infil-trative margin and deep location of the tumor, as in our
case which had a deep location. Angiomatoid fibrous
his-tiocytoma in the head and neck also can frequently recur,
which may be a result of the difficulty of performing a
wide local excision [4].
In summary, angiomatoid fibrous histiocytoma is a
rare soft tissue tumor with low-grade malignancy.
Al-though it is a nonspecific finding, angiomatoid fibrous
histiocytoma should be included in the differential
di-agnosis in young patients having a soft tissue tumor with
multiple fluid-fluid levels occurring in the extremities
and trunk. Tumor location and clear surgical margins are
important factors affecting local recurrence or
metasta-sis.
ation of possible histologic predictors of outcome. Am J Surg Pathol 1990; 14:1126–1132.
5. De Beuckeleer L, Fibrohistocytic tu-mors. In: De Schepper AM, Parizel PM, De Beuckeleer L, Vanhoenacker F, eds. Imaging of soft tissue tumors. Berlin Heidelberg New York: Springer, 2001:181–193.
bone and soft tissue. Radiology 1990; 175:779–782.
7. Grossman LD, White RR 4th, Arber DA. Angiomatoid fibrous histiocytoma. Ann Plast Surg 1996; 36:649–651. 8. Asakura S, Tezuka N, Inoue S, Kihara
N, Fujino S. Angiomatoid fibrous his-tiocytoma in mediastinum. Ann Thorac Surg 2001; 72:283–285.
158 cases and further exploration of the myoid phenotype. Hum Pathol 1999; 30: 1336-1343.
10. Smith ME, Costa MJ, Weiss SW. Evaluation of CD68 and other histio-cytic antigens in angiomatoid malignant fibrous histiocytoma. Am J Surg Pathol 1991; 15: 757-763.
