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RESEARCH POSTER PRESENTATION DESIGN © 2015
www.PosterPresentations.com
90 year old Caucasian man with history of several non-
melanoma skin cancers, presented with a 4.0 x 2.5 cm
ulcerated, friable, exophytic mass on the left mid
frontal scalp of two months duration.
The patient had previously presented two months
earlier with non-healing scalp lesion. At that time, the
lesion was a 0.9 cm ulcerated, erythematous, papule. A
shave biopsy of the lesion was performed however the
histopathology was non-diagnostic and demonstrated
marked parakeratosis, fibrosing granulation tissue in
the upper dermis, with a massive neutrophilic infiltrate.
A repeat biopsy was performed of the exophytic mass
for diagnostic and de-bulking purposes. The
histopathology of the re-biopsy demonstrated an
ulcerated tumor filling the dermis. The tumor cells
were pleomorphic, spindled, arranged in vague
fascicles, and extended to the deep margin. The
cytology was markedly atypical, with large irregular
nuclei, prominent nucleoli, and numerous mitoses,
including atypical forms. Immunohistochemical
analysis was performed. The tumor cells were
diffusely positive for CD10 and weakly positive for
CD68. Cytokeratin AE1/AE3, desmin, S-100, EMA,
cytokeratin 5, p63, Mart-1, smooth muscle myosin,
procollagen 1, ERG, CD31, and CD 34 were all
negative.
History
Physical Exam
Figure 2-4. Histopathology of the lesion. The excision specimen
reveals the extent of tumor invasion. Tumor cells infiltrate the
dermis and the deep subcutis (H&E, 40X total magnification). The
tumor cells are spindled and arranged in vague interlacing fascicles
with focal areas of necrosis (H&E, 200X total magnification). The
tumor cells are pleomorphic with large, irregular nuclei, prominent
nucleoli, and moderately abundant eosinophilic cytoplasm. Mitoses
are readily identified, including atypical forms (H&E, 400X total
magnification).
Histopathology
The patient subsequently underwent wide local
excision with one centimeter margins. The specimen
showed atypical spindled cells extending deep into
subcutaneous tissue. Due to the depth of invasion with
involvement of subcutaneous tissue and the presence
of necrosis, the lesion was diagnosed as an
undifferentiated pleomorphic sarcoma of skin. The
margins were free of tumor however the patient was
referred to oncology for further evaluation and
consideration of adjuvant therapy. The patient and
family declined oncology referral, and as of four
months post-excision, there was no evidence of
recurrence.
Clinical Course
Discussion
Undifferentiated pleomorphic sarcoma (UPS) of skin
can clinically and histopathologically mimic atypical
fibroxanthoma (AFX). Distinguishing between the
two is important, as the prognoses of these tumors are
vastly different. AFX follows more of a benign course,
typically recurs only after incomplete excision, and
rarely metastasizes. UPS, previously grouped into the
malignant fibrous histiocytoma (MFH) category, is
more aggressive in nature, and has a high rate of
recurrence along with malignant/metastatic potential.
Clinically, AFX presents as a rapidly growing solitary
nodule on sun-damaged, actinic skin of the elderly,
usually on the head and neck region. UPS is
considered a soft tissue tumor but can occur
superficially in the skin, with a presentation mimicking
AFX.
Histologically, UPS and AFX consist of spindle shaped
cells arranged in a fascicular pattern and can exhibit
multinucleation, pleomorphism, and mitotic figures.
UPS is distinguished from AFX by deep subcutaneous
involvement, perineural and/or lymphovascular
invasion, and necrosis. Immunohistochemically, both
stain negative for S-100/SOX-10, cytokeratin,
CD31/CD34, and desmin/myosin allowing
differentiation from other pleomorphic tumors in the
skin, such as melanoma, squamous cell carcinoma,
angiosarcoma, and leiomyosarcoma. AFX and UPS
are diagnoses of exclusion, requiring broad lineage-
specific immunohistochemical analysis to exclude
other poorly differentiated tumors.
References
1. Bolognia JL, Jorizzo JL and Rapini RR. Fibrous and
fibrohistiocytic proliferations of the skin and tendons, atypical
fibroxanthoma. In: Dermatology, 3rd ed, Elsevier, 2012. p
1973.
2. Goldblum JR, Folpe AL and Weiss SW. Undifferentiated
pleomorphic sarcoma. In: Enzinger and Weiss's Soft Tissue
Tumors, 6th ed, Elsevier, 2014 p 421-442.
3. McKee PH, Calonje E and Granter SR. Connective tissue
tumors, atypical fibroxanthoma, malignant fibrous
histiocytoma. In: Pathology of the Skin, 4th ed, Elsevier, 2012.
p 1658-1662.
4. Luzar B and Calonje E. Morphological and
immunohistochemical characteristics of atypical fibroxanthoma
with a special emphasis on potential diagnostic pitfalls: a
review. J Cutan Pathol. 2010 Mar;37(3):301-309.
5. Miller K, Goodlad JR and Brenn T. Pleomorphic dermal
sarcoma: adverse histologic features predict aggressive
behavior and allow distinction from atypical fibroxanthomas.
Am J Surg Pathol. 2012 Sep;36(9):1317-1326.
6. Beer TW, Drury P, and Heenan PJ. Atypical fibroxanthoma: a
histological and immunohistochemical review of 171 cases. Am
J Dermatopathol. 2010 Aug;32(6):533-540.
7. Mirza B, and Weedon D. Atypical fibroxanthoma: a
clinicopathological study of 89 cases. Australas J Dermatol.
2005 Nov;46(4):235-238.
There is significant overlap between AFX and UPS of
skin clinically, morphologically, and
immunohistochemically. Histologically, there are
identifiable differences found on excision of the lesion.
Distinguishing the two requires complete excision to
evaluate for aggressive features, specifically the
tumor’s extent of invasion, with AFX designated to
tumors restricted to the dermis. UPS of skin invades
deeply into subcutaneous tissue and can demonstrate
tumor necrosis, perineural or lymphovascular invasion.
These features are consistent with its more aggressive
course including recurrence and metastatic potential.
Undifferentiated pleomorphic sarcoma is a rare entity
with confusing, misleading, and changing
nomenclature previously named malignant fibrous
histiocytoma. Undifferentiated pleomorphic sarcoma
of skin is a diagnosis of exclusion made after complete
excision with histology aided by
immunohistochemistry. The correct diagnosis is crucial
to optimal outcome, preventing mismanagement of an
aggressive and potentially fatal tumor.
Figure 1. Clinical presentation of lesion. Left mid frontal scalp
with rapid growing, ulcerated, 4 x 2.5 cm exophytic mass.
1 University of North Texas Health Science Center, Forth Worth, TX; 2 ProPath, Dallas, TX
Michael Carletti DO1, Peter Malouf DO1, Zachary Ingersoll MS-III1, Greg Hosler MD, PhD2, Stephen Weis DO1
Undifferentiated Pleomorphic Sarcoma of Skin: Clinical and histopathologic emulator ofatypical fibroxanthoma, distinction imperative
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