Journal of Cosmetics, Dermatological Sciences and Applications, 2013, 3, 1-3
Published Online November 2013 (http://www.scirp.org/journal/jcdsa)
http://dx.doi.org/10.4236/jcdsa.2013.33A2001
Open Access JCDSA
1
Undifferentiated Pleomorphic Sarcoma of the Cheek
with Surface Ulceration: Mimicking Spindle Cell
Squamous Cell Carcinoma
Fumiko Momma*, Chihiro Onami, Takayuki Konno, Tamio Suzuki
Department of Dermatology, Faculty of Medicine, Yamagata University, Yamagata, Japan.
Email: *fmonma@med.id.yamagata-u.ac.jp
Received August 9th, 2013; revised September 4th, 2013; accepted September 11th, 2013
Copyright © 2013 Fumiko Momma et al. This is an open access article distributed under the Creative Commons Attribution License,
which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
ABSTRACT
Undifferentiated pleomorphic sarcoma (UPS), also called malignant fibrous histiocytoma (MFH), belongs to the soft
tissue tumors and is more likely in elder patients. UPS currently accounts for approximately 18% of cutaneous soft tis-
sue sarcoma except Kaposi sarcoma. The most common sites are the extremities and head and n eck. UPS is rapidly en-
larging, and easily invades subcutaneous tissue and muscle without overlying skin changes. UPS tumors, which are
confined to subcutaneous tissue, have been reported to be less than 10%. On the other hand, the involvement of the ep-
idermis, occasionally with ulceration, is rare. Here, we describe a case of UPS of the cheek with surface ulceration. An
83-year-old man presented with a 1-year history of a red nodule on his left cheek. The size of the nodule gradually in-
creased. Clinical examination revealed a 55 × 45 mm red, hard mass in the center of his left cheek with a large crater-
shaped ulcer involving the subcutaneous tissue. The bottom of the ulcer contained necrotic tissue. No cervical lymph
nodes were palpable. A biopsy revealed a high-grade, pleomorphic spindle cell neoplasm and frequent abnormal mitotic
figures within the dermis. We temporarily diagnosed and suspected a soft tissue sarcoma, but we could not rule out
spindle cell squamous cell carcinoma (SCSCC). The patient underwent tumor resection and skin grafting. The tumor
had reached the muscle layer of his left cheek with invasion and ulceration of the overlying skin. Histopathological
examination of the totally resected tumor revealed a neoplasm of mesenchymal origin, rich in cells with morphology
and immunohistochemical characteristics compatible with UPS. Malignant cells were not found in the epidermis. Im-
munohistochemical analyses were positive for vimentin, α-anti-trypsin, α-smooth muscle actin (α-SMA), and CD10,
and negative for cytokeratins (CAM5.2 and AE1/AE3), lysozyme, epithelial membrane antigen (EMA), CD34, S-100,
desmin, and caldesmon. Two months after total resection, the tumor recurred, and the patient was treated with radiation
therapy. However, he died 2 months later from multiple lun g metastases. We thought that the metastases were from the
UPS, because his prostatic carcinoma had not progressed. According to published literatures, the UPS rarely accompa-
nies with surface ulceration. On the other hand, SCC commonly occurs on sun-exposed sites of the head and neck with
surface changes including scaling, ulceration, crusting, and the presence of a cutaneous horn. In our case, it was very
difficult to distinguish UPS from SCSCC by clinical examination and conventional histopathological staining (e.g. he-
matoxylin and eosin). Therefore, the immunohistochemistry was required to obtain the final diagnosis.
Keywords: Undifferentiated Pleomorphic Sarcoma; Ulcer; Spindle Cell Squamous Cell Carcinoma
1. Introduction
Undifferentiated pleomorphic sarcoma (UPS), also called
malignant fibrous histiocytoma (MFH), belongs to the
soft tissue tumors and is more likely in elder patients.
UPS currently accounts for approximately 18% of cuta-
neous soft tissue sarcoma except Kaposi sarcoma [1].
The most common sites are the extremities and head and
neck [1]. UPS is rapidly enlarging, and easily invades
subcutaneous tissue and muscle without overlying skin
changes [1]. UPS tumors, which are confined to subcu-
taneous tissue, have been reported to be less than 10%.
On the other hand, the involvement of the epidermis,
occasionally with ulceration, is rare [2]. Here, we de-
scribe a case of UPS of the cheek with surface ulceration.
*Corresponding author.
Undifferentiated Pleomorphic Sarcoma of the Cheek with Surface Ulceration:
Mimicking Spindle Cell Squamous Cell Carcinoma
2
2. The Case Report
An 83-year-old man presented with a 1-year history of a
red nodule on his left cheek. He had been treated with
antibiotics at ano ther dermatological clinic; however, the
size of the nodule gradually increased. He also suffered
from severe Alzheimer’s dementia and prostatic carci-
noma. He had no family history of skin disease. Clinical
examination revealed a 55 × 45 mm red, hard mass in the
center of his left cheek with a large crater-shaped ulcer
involving the subcutaneous tissue. The bottom of the
ulcer contained necrotic tissue. No cervical lymph nodes
were palpable (Figure 1). A biopsy revealed a high-
grade, pleomorphic spindle cell neoplasm and frequent
abnormal mitotic figures within the dermis. We tempo-
rarily diagnosed suspected a soft tissue sarcoma, but we
could not rule out spindle cell squamous cell carcinoma
(SCSCC).
The patient underwent tumor resection, which in-
volved wide local excision, parotidectomy, and skin
grafting. The tumor had reached the muscle layer of his
left cheek with invasion and ulceration of the overlying
skin. Histopathological examination of the totally re-
sected tumor revealed a neoplasm of mesenchymal origin,
rich in cells with morphology and immunohistochemical
characteristics compatible with UPS (Figure 2). Malig-
nant cells were not found in the epidermis. Immunohis-
tochemical analyses were positive for vimentin, α-anti-
trypsin, α-smooth muscle actin (α-SMA), and CD10, and
negative for cytokeratins (CAM5.2 and AE1/AE3), ly-
sozyme, epithelial membrane antigen (EMA), CD34, S-
100, desmin, and caldesmon. The CD10 usually appears
Figure 1. The mass measured 55 × 45 mm on the left cheek,
it was firm to red color and hard in consistency and fixed
to underlying muscles. Overlying skin was ulcerated, in-
flamed with irregular margins.
Figure 2. In total resected sample of the tumor, tumor cells
were not found in the epidermis. There were tumors cell
from the epidermal right under to muscle. The tumor was
com p o s ed of a t ypical f ib r o b l a s t ic s pind l e c e l l s (H & E, ×100).
in UPS and also atypical fibroxanthoma (AFX) etc. [3,4].
Then we distinguished UPS from AFX by two reasons
following, 1) the tumor was larger than 2 cm, 2) the tu-
mor cells extensively involved the subcutaneous tissue
and penetrated the muscle [5]. Then, we finally diag-
nosed UPS.
Two months after total resection, the tumor recurred,
and the patient was treated with radiation therapy (total
of 68 Gray). However, he died 2 months later from mul-
tiple lung metastases. We thought that the metastases
were from the UPS, because his prostatic carcinoma had
not progressed.
3. Discussion
According to published literatures, the UPS rarely ac-
companies with surface ulceration [6]. On the other hand,
SCC commonly occurs on sun-exposed sites of the head
and neck with surface changes including scaling, ulcera-
tion, crusting, and the presence of a cutaneous horn [7].
4. Conclusion
In our case, it was very difficult to distinguish UPS from
SCSCC by clinical examination and conventional histo-
Open Access JCDSA
Undifferentiated Pleomorphic Sarcoma of the Cheek with Surface Ulceration:
Mimicking Spindle Cell Squamous Cell Carcinoma
Open Access JCDSA
3
pathological staining (e.g. hematoxylin and eosin). There-
fore, the immunohistochemistry was required to obtain
the final diagnosis.
REFERENCES
[1] S. T. Hollmig, R. Sachdev, C. J. Cockerell, W. Posten, M.
Chiang and J. Kim, “Spindle Cell Neoplasms Encoun-
tered in Dermatologic Surgery a Review,” Dermatologic
Surgery, Vol. 38, No. 6, 2012, pp. 825-850.
http://dx.doi.org/10.1111/j.1524-4725.2012.02296.x
[2] E. E. David, E. Rosalie, L. J. Bernett and F. M. George,
“Lever’s Histopathology of the Skin,” 9th Edition, Lip-
pincott Williams & Wilkins, Philadelphia, 2005, p. 989.
[3] D. Kemal, C. Ganime and O. Turhan, “Anti-CD10 (56C6)
Expression in Soft Tissue Sa rcomas,” Pathology, Research
and Practice, Vol. 208, No. 5, 2012, pp. 281-285.
http://dx.doi.org/10.1016/j.prp.2012.02.002
[4] C. N. Wieland, R. Dyck, R. H. Weenig and N. I. Comfere,
“The Role of CD10 in Distinguishing Atypical Fibroxan-
thoma from Sarcomatoid (Spindle Cell) Squamous Cell
Carcinoma,” Journal of Cutaneous Pathology, Vol. 38,
No. 11, 2011, pp. 884-888.
http://dx.doi.org/10.1111/j.1600-0560.2011.01768.x
[5] S. Shuji, W. Shoichi, K. Hiroshi, I. Hiroshi, H. Hideo and
A. Morita, “A Case of Cutaneous Malignant Fibrous
Histiocytoma with Multiple Organ Metastases,” Kaohsi-
ung Journal of Medical Sciences, Vol. 29, No. 2, 2013,
pp. 111-115.
http://dx.doi.org/10.1016/j.kjms.2012.08.019
[6] A. Prerna, W. Rachna, K. Nita, J. Shyama and N. S. Hadke,
“Secondary Malignant Fibrous Histiocytoma of Thigh
with Surface Ulceration Following Radiotherapy for Car-
cinoma Cervix,” Archives of Gynecology and Obstetrics,
Vol. 283, Suppl. 1, 2011, pp. S79-S82.
http://dx.doi.org/10.1007/s00404-010-1699-3
[7] H. M. Gloster and K. Neal, “Skin Cancer in Skin of Color,”
Journal of the American Academy of Dermatology, Vol.
55, No. 5, 2006, pp. 741-760.
http://dx.doi.org/10.1016/j.jaad.2005.08.063