Case Report Original Plexiform Fibrohistiocytic Tumors on Both

中華癌醫會誌 (J. Chinese Oncol. Soc.) 25(2), 153-156, 2009
journal homepage:www.cos.org.tw/web/index.asp
Case Report
Original Plexiform Fibrohistiocytic Tumors on Both Thighs
Chin-Hung Wei1, Nien-Lu Wang1*, Be-Fong Chen2
1
Department of Pediatric Surgery, Mackay Memorial Hospital, Taipei, Taiwan
Department of Pathology, Mackay Memorial Hospital, Taipei, Taiwan
2
Abstract.
Plexiform fibrohistiocytic tumor is a rare tumor with low grade-malignancy. It often presents with a slow-growing painless dermis or subcutaneous mass. The vast majority of occurrence is located at the upper extremity, followed by the lower extremity, chest, trunk and the region of head and neck. Gender distribution has female predominance. All primary lesions reported in the literature were solitary. We present a 5 year-old boy with tumor over bilateral
thighs. Excision was performed and mixed subtype plexiform fibrohistiocytic tumor was
proven. Wide excision was arranged and no recurrence was noted in the following 6 months.
Keywords : plexiform fibrohitiocytic tumor
病例報告
雙側股叢狀纖維組織細胞瘤
魏晉弘 1
1
2
王念陸 1*
陳碧芳 2
台北馬偕紀念醫院 小兒外科
台北馬偕紀念醫院 病理科
中文摘要
叢狀纖維組織細胞瘤為一稀少且低惡性腫瘤，其通常以緩慢成長且無痛之皮下腫
塊來表現，最好發部位為上肢，其次為下肢、胸部、軀幹及頭頸部，女性比例較高，
目前文獻中所有原發性病灶皆為單一性，我們介紹一個雙側大腿腫塊的病例，經切除
後病理証實為叢狀纖維組織細胞瘤，再一次廣泛性切除後，接下來六個月並無觀察到
復發。
關鍵字: 叢狀纖維組織細胞瘤
INTRODUCTION
extremity, the lower extremity, chest, trunk, and head
Plexiform fibrohistiocytic tumor (PFT) was first
and neck. To our knowledge, all previously reported
described by Enzinger and Zhang [1] in 1988. PFT
primary PFTs were solitary. We present a rare case of
usually affects children and young adults, with female
bilateral PFTs of the thighs.
predominance about six times to male. Asymptomatic
mass is the main presentation and involves the upper
154
C. H. Wei et al./JCOS 25(2009) 153-156
CASE REPORT
to affect children and young adults with median age of
A 5-year-old boy had two painless masses over the
14.5 years, from 2 months to 71 years [1]. The oldest
anterior-lateral aspect of bilateral thighs for several
case had PFT for 60 years without treatment. The
months, which grew slowly in size. Computed tomo-
prevalence of PFTs in females is 2~6 times to males
graphy revealed two masses that were adherent to
[1-3]. But in a recent serial study, male gender was
muscle and fascia. Compared with muscle, the masses
more frequent than female gender [4]. Totally, the ra-
showed isodensity to hyperdensity (Figure 1). Grossly,
tio women to men approaches 3:2. Most patients visit
the tumors were yellowish and infiltrated into the
clinics for painless indurated nodule. The upper ex-
quadriceps muscle and tensor fascia lata without clear
tremity is the most common site of PFTs, followed by
border. Excisional biopsy was performed. Pathology
the lower extremity, trunk, and head and neck [5].
report showed plexiform fibrohistiocytic tumor with
There was only one case report in which the tumor
positive pathologic margin involvement. Second op-
was located in the bone [6].
eration for wide excision was accomplished, and the
PFTs arise in dermis and extend into the subcuta-
margin was confirmed to be negative to tumor in-
neous layer, even fascia and muscle. The definite
volvement by multiple frozen sections. Associated
treatment is complete surgical excision. However, the
surveys, such as chest radiograph and bone scan, were
infiltrative appearance of PFTs results in difficulty to
negative for metastasis. Recurrence was not observed
recognize the microscopic margin and achieve com-
during 6 months of follow-up.
plete excision. Furthermore, it contributes to high lo-
Microscopically, the tumor was composed of mul-
cal recurrence rate. The local recurrence rate ranges
tiple small and medium-sized nodule on low-power
from 12.5% to 50%, with average of 18.6% [3]. All
view (Figure 2). Mononeclear histiocyte-like cells and
recurrence can be treated with wide re-excision suc-
multinucleated giant cells were arranged in a plexi-
cessfully. Some authors recommend application of
form pattern, surrounded by short fascicles of fibro-
Mohs micrographic surgery in primary and recurrent
clast-like cells (Figure 3). This is characterized by
PFTs [7,8].
mixed subtype of PFTs. Immunohistochemistry rec-
The typical histology of PFTs is characterized by
ognized strong cytoplasmic immunoreactivity for
multiple small nodules or elongated fascicles arranged
CD68 (Figure 4). The lesions were negative for cy-
in a plexiform pattern. These nodules include mono-
tokeratin, α-smooth muscle actin, desmin, and S-100
nuclear cells and oesteoclast-like giant cells. There
protein.
have been three subtypes of PFT: 1) fibrohistiocytic
subtype, that is composed of clusters of mononuclear
DISCUSSION
histiocyte-cells and multinucleated giant cells, 2) fi-
Since an initial report of 65 cases of PFTs by
broblastic subtype, that is composed of elongated
Enzinger and Zhang in 1988, the pathologic charac-
clusters and short fascicles of spindle fibroblast-like
teristics have been described clearly. The tumor tends
cells, and 3) mixed subtype, which has both patterns
equally. Salamanca et al described a unique case with
PFT without multinucleated giant cell [9]. Immuno-
*Corresponding author: Nien-Lu Wang M.D., Ph.D.
histiochemically, the rounded mononuclear cells and
*通訊作者：王念陸醫師
giant cells are usually positive for CD68. In our case,
Tel: +886-2-25433535
both fibriohistiocytic and fibroblastic cells existed, in-
Fax: +886-2-25433642
dicating a mixed subtype of PFTs. Some authors at-
E-mail: nienlu.wang@msa.hinet.net
tempted to find out the relationship between subtypes
C. H. Wei et al./JCOS 25(2009) 153-156
155
of PFTs and recurrence, but failed (1-3).
Other fibrohistiocytic tumors, including malignant
and benign fibrous histiocytoma, should be considered
in the differential diagnosis. Malignant fibrous histiocytoma, also termed angiomatoid fibrous histiocytoma,
resembles a benign nodule. But systemic symptoms
may appear, such as fever, anemia and body weight
loss. It is thought to be caused by release of cytokines
by tumor. Malignant fibrous histiocytoma is classified
as a fibrohistiocytic tumor of intermediate malignance
by the World Health Organization. Histologically, malignant fibrous histiocytoma has a dense and thick
capsule with surrounding of infiltrative lymphocyte .
The cystic content is composed of blood due to hemorrhage. Benign fibrous histiocytoma, also termed
Figure 1. Computed tomography: bilateral tumors ad-
aneurysmal fibrous histiocytoma or dermatofibroma,
herent to fascia over anterior-lateral aspect
may mimic malignant fibrous histiocytoma. It also has
of the thighs, which revealed isodensity to
hemorrhage and hemosiderin staining but lacks thick
hyperdensity in comparison to muscle
capsule and surrounding lymphocyte infiltration.
Characteristic spindled cells infiltrate among collagen
bundles in a storiform pattern. Whether benign or malignant fibrous histiocytoma, it is not difficult to be
differentiated from PFTs histologically [10].
Jafarian et al. reviewed 123 patients with PFTs in
the English literatures [3], and Moosavi et al. published 66 cases of PFTs [4]. All primary lesions were
solitary, whereas the metastatic lesions could be multiple. This case offers the concept that PFTs can be bilateral, and clinicians should be careful not to ignore
another lesion.
CONCLUSIONS
Plexiform fibrohistoicytic tumor is a slow-growing
lesion with low-grade malignancy. Gross appearance
is infiltrative into tissue without definite border. Wide
Figure 2. Low-power view (HE x40) of plexiform fi-
excision with negative pathologic margin cannot be
brohistiocytic tumor shows multiple small
overemphasized. Doctors should be careful not to
to medium-sized nodules
miss bilateral lesions. Long-term follow-up is necessary for early diagnosis of pulmonary or nodal metastasis.
156
C. H. Wei et al./JCOS 25(2009) 153-156
Figure 3. High-power view (HE x200) of plexiform
Figure 4. CD68 immunohistochemical stain shows st-
fibrohistiocytic tumor shows histiocyte-like
rong immunoreactivity in plexiform fibro-
cells and multinucleated giant cells, cir-
histiocytic tumor
cumscribed with short fascicles of spindle
cells
REFERENCES
cytic tumor: a brief review. Arch Pathol Lab
1. Enzinger FM, Zhang RY. Plexiform fibrohistio-
Med 131: 1135-1138, 2007.
cytic tumor presenting in children and young
6. Povysil C, Habanec B. Plexiform fibrohistiocytic
adults: an analysis of 65 cases. Am J Surg Pathol
tumor of soft tissues and bone. Cesk Pathol 36:
2: 818-826, 1988.
106-110, 2000.
2. Remstein ED, Arndt CA, Nascimento AG. Plexiform fibrohistiocytic tumor: clinicopathologic analysis of 22 cases. Am J Surg Pathol 23: 662670, 1999.
3. Jafarian F, McCuaig C, Kokta V, et al. Plexiform
fibrohistiocytic tumor in three children. Pediatr
Dermatol 1: 7-12, 2006.
4. Moosavi C, Jha P, Fanbrug-Smith JC. An update
on plexiform fibrohistiocytic tumor and addition
7. Herring SM. Plexiform fibrohistiocytic tumor of
skin. Ann Plast Surg 30: 459-461, 1993.
8. Rahimi AD, Shelton R, Dumas A, et al. Mohs
micrographic surgery of a plexiform fibrohistiocytic tumor. Dermatol Surg 27: 768-771, 2001.
9. Salamanca J, Rodriguez-Peralto JL, Garcia de la
Torre JP, et al. Plexiform fibrohistiocytic tumor
without multinucleated giant cells: a case report.
Am J Dermatopathol 24: 399-401, 2002.
of 66 new cases from the Armed Forces Institute
10. Billings SD, Folpe AL. Cutaneous and subcuta-
of Pathlogy, in honor of Franz M. Enzinger, MD.
neous fibrohistiocytic tumors of intermediate ma-
Ann Diagn Pathol 11: 313-9, 2007.
lignancy: an update. Am J Dermatopathol 26:
5. Taher A, Pushpanathan C. Plexiform fibrohistio-
141-55,2004.