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OJHAS Vol. 23, Issue 3:
July-September 2024 |
Case
Report
Navigating
Diagnostic Challenges in Cutaneous
Leiomyosarcoma: A Clinical Case Study
Authors:
Vijayashree
Raghavan, Professor and
Head,
Ayeesha Sithika T, Associate
Professor,
Kundhavai Chandrasekaran,
Assistant Professor,
Department of Pathology, Chettinad
Hospital and Research Institute,
Chettinad Academy of Research and
Education, Kelambakkam - 603103,
Chengalpattu District, Tamilnadu,
India.
Address for
Correspondence
Dr. Kundhavai
Chandrasekaran,
Assistant Professor,
Department of Pathology,
Chettinad Hospital and Research
Institute,
Chettinad Academy of Research and
Education,
Kelambakkam - 603103,
Chengalpattu District, Tamilnadu, India.
E-mail:
kundhavaipath@gmail.com.
Citation
Raghavan V, Sithika AT,
Chandrasekaran K. Navigating Diagnostic
Challenges in Cutaneous Leiomyosarcoma:
A Clinical Case Study. Online J
Health Allied Scs.
2024;23(3):11. Available at URL:
https://www.ojhas.org/issue91/2024-3-11.html
Submitted:
Sep
26, 2024; Accepted: Oct 7, 2024;
Published: Oct 15, 2024
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Abstract:
Cutaneous Leiomyosarcomas (CL) are rare,
constituting just 3% of malignant soft tissue
sarcomas. We present a case involving a
40-year-old male with a forearm nodule.
Histopathological examination revealed atypical
spindle cells with immunohistochemistry
confirming Cutaneous Leiomyosarcomas. While the
patient had a prior excision for a similar
swelling, reports were unavailable. Treatment
involved a wide resection with a lesion-free
specimen. Cutaneous Leiomyosarcomas commonly
presents as a nodular growth in extremities,
with a microscopic appearance characterized by
spindle-shaped cells exhibiting palisading and
atypia. Differential diagnoses include other
spindle cell lesions necessitating IHC for
confirmation. Recurrence rates underscore the
importance of wide excision. Distant metastasis,
primarily to the lungs, and identified
prognostic factors, such as lesion size and
histological grade, contribute to the complex
clinical landscape. This case emphasizes the
rarity of Cutaneous Leiomyosarcomas, emphasizing
the need for thorough diagnostic evaluation and
a multidisciplinary approach for effective
management.
Key
Words: Cutaneous Leiomyosarcoma,
multidisciplinary approach
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Introduction
Cutaneous
Leiomyosarcomas (CL) are a rare subset,
constituting a mere 3% of malignant soft tissue
sarcomas(1). Within superficial leiomyosarcomas,
two distinctive forms exist—cutaneous and
subcutaneous(2). Typically presenting as skin
nodules, precise diagnosis requires
histopathological examination complemented by
immunohistochemistry. This case report outlines an
instance of cutaneous leiomyosarcoma in the
forearm of a 40-year-old male.
Case Presentation
A 40-year-old male
presented with a forearm nodule persisting for a
year, with a history of a similar swelling three
years prior, excised without available reports. On
examination, the 3 x 3 cm swelling displayed firm
consistency with unremarkable overlying skin. A
0.5 x 0.4 x 0.3 cm skin biopsy revealed a dermal
neoplasm, comprising atypical spindle cells
exhibiting palisading in fascicles and bundles
(Figure.1). Individual cells featured eosinophilic
cytoplasm, pleomorphic, and hyperchromatic nuclei
(Figure.2). Focal areas of necrosis and a mitotic
count of approximately 28 mitoses/10 high power
fields were noted. Immunohistochemical analysis
confirmed positivity for smooth muscle actin
(Figure.3) and negativity for S100 (Figure.4),
establishing a diagnosis of cutaneous
leiomyosarcoma. Subsequent wide resection
confirmed tumor-free margins.
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Figure
1 : Dermal neoplasm showed
composed of atypical spindle cells
arranged in fascicles and bundles with
palisading.(100x) |
Figure
2 : Atypical spindle cells with
eosinophilic cytoplasm,pleomorphic and
hyperchromatic nuclei.(400 x) |
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Figure
3: Immunohistochemistry - The tumor cells
showing strong positivity for Smooth
Muscle Actin (100 x) |
Figure
4: Immunohistochemistry - The tumor cells
negative for S100 (100 x) |
Discussion
Comprising a mere 3%
of malignant soft tissue sarcomas(1), Cutaneous
Leiomyosarcomas predominantly affects the
elderly(3), with a male predilection and
extremities being the most common location(4).
Clinically, nodular growth is characteristic(5).
Histologically, Cutaneous Leiomyosarcomas is
defined by palisading spindle-shaped tumor cells
with cigar-shaped nuclei and atypia, frequent
mitotic figures, and areas of necrosis.
Differential diagnoses encompass spindle cell
lesions such as peripheral nerve sheath tumors,
fibrosarcoma, synovial sarcoma, and
dermatofibrosarcoma protuberans.
Immunohistochemistry plays a pivotal role in
precise diagnosis, demonstrating positivity for
vimentin, desmin, and smooth muscle actin in
Cutaneous Leiomyosarcomas, while neural origin
lesions exhibit S100 positivity.
Recommended
treatment involves wide excision(6,7), but
recurrences are common(8). Distant metastasis,
primarily to the lungs(1,5), and poor prognostic
factors such as lesion size, fascial involvement,
inadequate margin clearance, extremity location,
and high histological grade are significant
considerations. Subcutaneous Cutaneous
Leiomyosarcomas poses higher risks of local
recurrence and metastasis. The role of
radiotherapy is emphasized to reduce relapse,
while chemotherapy administration remains
contentious. Pazopanib, a tyrosine kinase
inhibitor targeting VEGF and PDGF, is FDA-approved
for advanced cases.
Conclusion
In the domain of
malignant spindle cell neoplasms of the skin,
cutaneous leiomyosarcoma, though rare, demands
consideration and confirmation through a
comprehensive IHC panel. Awareness of prognostic
factors guides tailored management, advocating for
a multidisciplinary approach to ensure optimal
patient outcomes.
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