Clinical and Pathological Characteristics of Soft Tissue Sarcomas: A Retrospective Study From a Developing Country

Abstract

Introduction
Soft tissue sarcomas remain an exceedingly rare malignancy. While soft tissue sarcomas boast a high mortality rate, their characteristics and behavior patterns are poorly understood. This study aims to evaluate the various aspects that pertain to soft tissue sarcomas, including their histology, tumor characteristics, survival rates, and therapeutic modalities.

Methods
A retrospective study analyzing the data from 19 patients presenting over four years with a histologically confirmed diagnosis of soft tissue sarcomas was conducted. The patients were studied for various parameters, including tumor site and the particular pathological subtypes. The data obtained were analyzed using the SPSS 23.0 statistical software (IBM Corporation, Armonk, NY), and the results were then tabulated.

Results
A total of 19 patients with a confirmed diagnosis of a soft tissue sarcoma were included in the study. The mean age of the patients included was 45.32 ± 16.88 years. Wide local excision was the most common surgical procedure employed for the resection of these tumors. Within the cohort, the mortality rate was noted to hover at 10.52%. Gastrointestinal stromal tumors were observed in 21% of the patients and were therefore the most common histological subtype. Of the patients included, 42.10% required blood transfusion during the perioperative time. Most of the tumors were noted to be intermediate grade, with high-grade tumors observed in 26.3% of the cases.

Conclusion
Soft tissue sarcomas remain a rare but potent cause of death in developing countries. The diversity of the tissues that they afflict renders their prompt detection a diagnostic challenge. A meticulous exploration of the various characteristics honed by soft tissue sarcomas, such as the particular histological subtype and the associated mortality rates, can better elucidate the prognosis and the eventual disease outcomes.

Full text

Received 08/10/2020
Review began 08/13/2020
Review ended 08/13/2020
Published 08/21/2020
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Clinical and Pathological Characteristics of
Soft Tissue Sarcomas: A Retrospective
Study From a Developing Country
Talal Almas , Muhammad Kashif Khan , Muhammad Faisal Murad , Muneeb Ullah , Adil
Shafi , Maryam Ehtesham , Syed Muhammad Jawad Zaidi , Salman Hussain , Mehwish
Kaneez
1. Internal Medicine, Royal College of Surgeons in Ireland, Dublin, IRL 2. Surgical Oncology, Federal
Government Poly Clinic (Post Graduate Medical Institute), Islamabad, PAK 3. Surgical Oncology, Maroof
International Hospital, Islamabad, PAK 4. General Surgery, Maroof International Hospital, Islamabad ,
PAK 5. General Surgery, Maroof International Hospital, Islamabad, PAK 6. Internal Medicine, Rawalpindi
Medical University, Rawalpindi, PAK
Corresponding author: Muhammad Kashif Khan, kashifamc@gmail.com
Abstract
Introduction
Soft tissue sarcomas remain an exceedingly rare malignancy. While soft tissue sarcomas boast a
high mortality rate, their characteristics and behavior patterns are poorly understood. This
study aims to evaluate the various aspects that pertain to soft tissue sarcomas, including their
histology, tumor characteristics, survival rates, and therapeutic modalities.
Methods
A retrospective study analyzing the data from 19 patients presenting over four years with a
histologically confirmed diagnosis of soft tissue sarcomas was conducted. The patients were
studied for various parameters, including tumor site and the particular pathological subtypes.
The data obtained were analyzed using the SPSS 23.0 statistical software (IBM Corporation,
Armonk, NY), and the results were then tabulated.
Results
A total of 19 patients with a confirmed diagnosis of a soft tissue sarcoma were included in the
study. The mean age of the patients included was 45.32 ± 16.88 years. Wide local excision was
the most common surgical procedure employed for the resection of these tumors. Within the
cohort, the mortality rate was noted to hover at 10.52%. Gastrointestinal stromal tumors were
observed in 21% of the patients and were therefore the most common histological subtype. Of
the patients included, 42.10% required blood transfusion during the perioperative time. Most of
the tumors were noted to be intermediate grade, with high-grade tumors observed in 26.3% of
the cases.
Conclusion
Soft tissue sarcomas remain a rare but potent cause of death in developing countries. The
diversity of the tissues that they afflict renders their prompt detection a diagnostic challenge. A
meticulous exploration of the various characteristics honed by soft tissue sarcomas, such as the
particular histological subtype and the associated mortality rates, can better elucidate the
prognosis and the eventual disease outcomes.
1 2, 3 4 4
5 1 6 1
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Open Access Original
Article DOI: 10.7759/cureus.9913
How to cite this article
Almas T, Khan, Murad M, et al. (August 21, 2020) Clinical and Pathological Characteristics of Soft Tissue
Sarcomas: A Retrospective Study From a Developing Country. Cureus 12(8): e9913. DOI
10.7759/cureus.9913

Categories: Internal Medicine, General Surgery, Oncology
Keywords: soft tissue sarcomas, clinical characteristics, management
Introduction
Soft tissue sarcomas (STS) refer to a rare group of heterogeneous tumors of mesenchymal origin
and comprise less than 0.2% of all adult cancers [1]. Due to their predilection for evoking
malignant transformations in a plethora of various tissues, STS are believed to be one of the
most diverse malignancies [1,2]. In general, STS may involve the connective tissue of the head
and neck, trunk, and limbs, as well as the retroperitoneum [2]. STS manifest a spectrum of
tumor behaviour, ranging from indolent growth to widespread metastasis [3]. Pertinently, the
optimal management of STS remains a conundrum for clinicians, and depends on a multitude
of factors, including clinical characteristics, tumor characteristics, tumor size, and the
histopathological subtype [2,4]. Due to the complexity of STS, a multimodal approach is usually
followed, and the utilization of a multidisciplinary approach plays a vital role in the apt
management of these tumors [5].
Retroperitoneal sarcomas (RPS) are a rare group of soft tissue malignant neoplasms that
comprise merely 1%-2% of all solid cancers and only 10%-20% of all sarcomas [6-8]. These
tumors classically arise in the retroperitoneum and have the potential to reach exorbitant
proportions without eliciting any symptoms, and thus usually present late in the disease
course. When RPS present with symptoms, they are usually non-specific and include abdominal
pain, abdominal discomfort, fullness, and changes in urinary or bowel habits [6]. Complete
surgical resection is the standard of care for RPS, with conflicting data on the use of adjuvant
and neoadjuvant therapies [9-11]. The retroperitoneum contains multiple vital organs and
critical structures, including the aorta, vena cava, head of the pancreas, and duodenum [2,6].
Sarcomas generally have a poor prognosis, with a five-year overall survival rate hovering
around 36%-58% [6]. Overall survival is impacted by various prognostic factors that include
histologic subtype, grade, and completeness of tumor resection [12]. While these are
established prognosticators, additional parameters, such as tumor size, transfusion
requirements, and anatomical location of the tumor, are also conjectured to impact the overall
survival. Since surgical resection is the mainstay of management, different patterns of
resection, including the employment of complex compartmental resection, are often required
and are associated with varying complication rates and postoperative outcomes [13,14]. The
goal is to achieve complete resection with negative macroscopic and microscopic margins to
reduce the risk of local recurrence [6]. Due to the paucity of data elucidating the outcomes of
STS in developing nations such as Pakistan, there is an unmet need to analyze these
parameters as they pertain to STS. The present study therefore aims to delineate these
parameters.
Materials And Methods
A retrospective cross-sectional study was conducted in the department of Surgical Oncology,
Maroof International Hospital, Islamabad, Pakistan. A total of 19 patients who underwent
surgery for a myriad of sarcomas involving various sites from January 2016 till January 2020
were included in the study. The patients were studied for various parameters, including tumor
site, histopathological subtype, tumor grade, and the type of intervention employed. Patient
comorbidities and various other surgical outcomes were also evaluated. Thereafter, the
distribution of the various sarcomas in these patients was tabulated. The data were then
analyzed using the SPSS 23.0 software (IBM Corporation, Armonk, NY).
2020 Almas et al. Cureus 12(8): e9913. DOI 10.7759/cureus.9913 2 of 9

Results
In the present study involving 19 cases, the mean age of the study participants was 45.32 ±
16.88 years, with a range of 20 to 80 years. Table 1 delineates the characteristics of study
participants based on their gender, marital status, and comorbidities.
Parameter Frequency Percentage
Gender
Male 10 52.6%
Female 9 47.4%
Marital status
Married 15 78.9%
Unmarried 4 21.1%
Comorbidities
Hypertension 2 10.5%
Diabetes mellitus 2 10.5%
Ischemic heart disease 1 5.3%
TABLE 1: Background characteristics of the study participants.
Based on the clinical evaluation, baseline laboratory investigations, and radiological imaging,
the initial diagnosis was made. A plethora of various surgical procedures were deemed apt
based on the site implicated and the extent of the tumor. Wide local excision was the preferred
modality of surgical intervention in 16 patients, while compartmental excision was performed
in merely 3 patients. Table 2 further highlights the primary site of involvement, the closest
margins, the grade of the tumor, and the type of procedure employed.
2020 Almas et al. Cureus 12(8): e9913. DOI 10.7759/cureus.9913 3 of 9

Parameter Frequency Percentages
Primary site of tumor
Gastrointestinal tract 3 15.8%
Abdominal/pelvic wall 3 15.8%
Breast 2 10.5%
Retroperitoneal 3 15.8%
Limbs 3 15.8%
Others 5 26.3%
Grade of tumor
Low 6 31.6%
Intermediate 8 42.1%
High 5 26.3%
Closest margin
1-10 mm 6 31.6%
10-20 mm 5 26.3%
Greater than 20 mm 5 26.3%
Involved 3 15.8%
Surgical procedure
Wide local excision 16 84.2%
Compartmental excision 3 15.8%
TABLE 2: A tabulation of the various parameters studied with pertinence to soft tissue
sarcomas.
The particular histological subtypes of the sarcomas were also evaluated. Table 3 delineates the
frequency of the various histopathological subtypes.
2020 Almas et al. Cureus 12(8): e9913. DOI 10.7759/cureus.9913 4 of 9

Histopathological subtype Frequency
Gastrointestinal stromal tumor 4
Undifferentiated pleomorphic sarcoma 2
Retroperitoneal and dedifferentiated liposarcoma 2
Retroperitoneal leiomyosarcoma 1
Fibrosarcoma 1
Carcinosarcoma of uterus 1
Solitary fibrous tumor (malignant) 1
Endometrial stromal sarcoma 1
Neurofibrosarcoma 1
Myxoid leiomyosarcoma 1
Malignant spindle cell sarcoma 1
Malignant phyllodes tumor 1
Malignant peripheral nerve sheath tumor 1
Recurrent dermatofibrosarcoma protuberans 1
TABLE 3: The frequency of the various histopathological subtypes of sarcomas.
Imperatively, merely 2 out of the 19 patients eventually died due to recurrent and persistent
disease. The postoperative outcomes of the surgical interventions performed are detailed in
Table 4.
2020 Almas et al. Cureus 12(8): e9913. DOI 10.7759/cureus.9913 5 of 9

Parameter Frequency Percentage
Mortality 2 10.5%
Blood transfusion required 8 42.1%
Need for re-exploration 2 10.5%
Need for re-admission 2 10.5%
Need for chemotherapy 4 21%
Need for radiotherapy 4 21%
Median operating time (range) 120 (60-240) minutes
Median hospital stay (range) 3 (1-6) days
TABLE 4: The postoperative outcomes of patients operated for various sarcomas.
Discussion
STS remain a rare but diverse malignancy, notably affecting a vast range of different tissues and
organs [1]. Imaging modalities, such as MRI, remain pivotal in detecting soft tissue tumors.
CT and standard radiographs are used to rule out other possible bone tumors or cystic lesions
[3,4]. After the appropriate radiological assessment, the gold standard diagnostic investigation
is the core needle or excisional biopsy [5]. Given that sarcomas are noted to elicit a multitude of
non-specific symptoms, they are often detected incidentally upon physical examination or
imaging [6]. Ascertainment of the particular histologic subtype is performed through the means
of image-guided percutaneous core needle biopsy, preferably with a co-axial technique to
minimize the risk of seeding [15].
Before the consideration of the optimal management plan for the patient, a thorough
preoperative evaluation of factors such as the patient’s age and comorbidity status should be
performed. The overarching goal, and the most efficacious treatment modality for soft tissue
tumors in general and RPS in specific, is complete resection of the tumor with negative
microscopic and macroscopic margins [6]. However, an accurate pathological assessment of
microscopic margins is often onerous and imprecise due to the exorbitant proportions that STS
can grow to. It is therefore more pragmatic to aim for a complete macroscopical resection [16].
In certain subtypes of RPS, extensive encasement of adjacent structures is noted, which often
warrants an en bloc compartmental resection approach for the excision of the tumor along with
the encased structures. This usually presents a formidable challenge to surgeons owing to the
proximity of the tumor to various vital organs [17].
Oncological literature vouches for the notion that the grade of the tumor remains the most
imperative prognostic factor [18]. Although all types of STS manifest a spectrum of behavior,
ranging from indolent to malignant, certain sarcomas have a lower metastatic potential or less
aggressive behavior than the other subtypes. Other prognosticators include tumor size, depth
of invasion, and tumor location, with retroperitoneal tumors often boasting a worse prognosis
[3,18]. Of note, completeness of tumor resection, tumor grade, and histologic subtype are all
factors that are intricately linked to the overall survival [19]. Avancés et al. reported that a high
histologic grade was associated with tumor recurrence and poor survival [20]. In our data, 40%
2020 Almas et al. Cureus 12(8): e9913. DOI 10.7759/cureus.9913 6 of 9

of the tumors with a high histologic grade were associated with recurrence or death. In
contrast, a retrospective study observed that a high histologic grade was not associated with
recurrence or poor overall survival [8]. Hassan et al. demonstrated an association between
histologic subtype and overall survival, particularly outlining forbidding overall survival rates
and outcomes portended by leiomyosarcomas [16]. In concert with this notion, our study
divuled only two patients who were diagnosed with leiomyosarcomas, both of whom
demonstrated poor prognostic outcomes. Patients with liposarcomas and lower grade sarcomas
were found to have an improved overall survival [15].
Previous studies have established that complete surgical resection is the cornerstone curative
treatment for RPS [21]. To this end, Malinka et al. analyzed predictors of overall survival and
disease-free survival and identified surgical resection margins as one of the most important
predictors of disease-free survival [22]. In our study, all patients who received blood
transfusions had undergone either pelvic or abdominal surgery. Furthermore, half of those who
received blood transfusions did not develop recurrence, metastasis, or a poor survival outcome.
On the other hand, 25% of the patients who received transfusions eventually developed a
recurrence. Whether receiving a blood transfusion serves as a viable prognostic factor remains
shrouded in uncertainty and at the epicenter of an extensive oncological dilemma.
Although surgical excision remains the cornerstone of STS management, a combination of
radiotherapy and/or chemotherapy remains controversial. Despite an improvement in local
control, the use of chemotherapy shows no improvement in overall survival [23]. This is in
accordance with a retrospective analysis that reported no beneficial effects on survival with the
use of chemotherapy; however, postoperative adjuvant radiotherapy displayed beneficial effects
on overall survival for truncal sarcomas [24]. This notion is in contrast to a recent analysis of
the Nationwide Clinical Oncology Database (NCOD) by Nussbaum et al. that established
ameliorated survival outcomes with the uptake of radiotherapy in addition to surgery when
compared to surgery alone [25]. Although surgical excision remains pivotal for portending
favorable outcomes, the particular efficacy of radiotherapy and chemotherapy in the optimal
management of STS remains elusive. Furthermore, background patient characteristics should
also be factored into decisions pertaining to the optimal treatment regimens. There is thus an
overarching need for the curation of specific guidelines that can better inform the debate on
the most effective modality of management in patients with STS. Larger studies with greater
sample sizes are needed in order to better elucidate the outcomes and characteristics of STS,
especially as they prevail in developing nations such as Pakistan.
Conclusions
STS are a rare but important cause of cancer-related mortality in developed and developing
countries alike. While surgical excision remains the cornerstone treatment modality, there is
an ongoing debate on the efficacy of radiotherapy and chemotherapy in thwarting the
carcinogenesis of STS. Due to their non-specific symptoms, STS can evade prompt detection,
often presenting as high-grade tumors that portend grave disease outcomes. A meticulous
evaluation through the means of history, physical examination, and radiological imaging,
followed by a core biopsy to assess the local extent of disease, therefore remains imperative.
Additional studies with larger sample sizes are needed to better delineate the characteristics
and behavior patterns of these tumors in order to understand the disease prognosis more
comprehensively.
Additional Information
Disclosures
Human subjects: All authors have confirmed that this study did not involve human
participants or tissue. Animal subjects: All authors have confirmed that this study did not
2020 Almas et al. Cureus 12(8): e9913. DOI 10.7759/cureus.9913 7 of 9

involve animal subjects or tissue. Conflicts of interest: In compliance with the ICMJE uniform
disclosure form, all authors declare the following: Payment/services info: All authors have
declared that no financial support was received from any organization for the submitted work.
Financial relationships: All authors have declared that they have no financial relationships at
present or within the previous three years with any organizations that might have an interest in
the submitted work. Other relationships: All authors have declared that there are no other
relationships or activities that could appear to have influenced the submitted work.
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