Title: Evaluation of Childhood Soft Tissue Tumors of the Extremities in Iranian Population
Author(s): Sam Hajaliloo Sami MD 1, Tahmineh Mokhtari PhD 2, Gholamreza Shafiee MD 3*
1 Iran University of Medical Sciences, Tehran, Iran 2 Tehran University of Medical sciences, Tehran, Iran 3 Golestan University of Medical Sciences, Kalaleh, Iran
* Corresponding Author
Vol 2, Num 2, April 2015
Introduction:Benign and malignant soft tissue neoplastic lesions are observed in extremities of children. These tumors have different clinical and epidemiological characteristics. The aim of this study was to evaluate the epidemiological characteristics of childhood soft tissue tumors in extremities in Iranian population.
Materials and Methods:In a cross-sectional study, 69 cases (0-16 year old children) were evaluated for benign and malignant soft tissue tumors in their extremities referred to Shafa Orthopedic Hospital, from 2006 to 2012. The pathologic specimens were obtained and the pathologic reports were recorded for each case.
Results:Of all 69 patients with mean age of 12.1±3.1 years at the time of surgery, 30 (43.4%) were female and 39 (56.5%) were male. Pathologic reports of 76.8% of cases were benign lesion and 25.2% were malignant. Most of the lesions (37.68%) were in the hand, after that the most involved region was thigh (20.28%). Hemangioma was the most observed lesion of soft tissue tumors (23.18%) even after rhabdomyosarcoma. The recurrence of tumor was reported in 13 cases with rhabdomyosarcoma.
Conclusion:According to the results, hemangioma was the most observed childhood soft tissue tumors as a benign mass and rhabdomyosarcoma was the most observed as a malignant mass in Iranian population.
Keyword:Soft tissue neoplasms, Sarcoma, Childhood, Iranian population, Orthopaedics, A.12, A.13
There is a large and heterogeneous group of lesions in extremities are related to the children, including both neoplastic (including benign and malignant) and nonneoplastic lesions.
In general, in children, benign type of soft tissue tumors in extremities are more common than malignant ones. According to the literature, benign neoplasms are 100 times more common than malignant neoplasms.[2,3] These tumors have different clinical and epidemiological characteristics. Pain can't be helpful for differentiating between the benign and malignant tumors because most patients with soft tissue benign tumor have mild pain and often they are referred with a mass. Since there is no exact criterion for diagnosis of benign or malignant, two factors of size and depth of the tumors are more than others can affect deciding in this background including tumor.
Soft tissue sarcomas are a heterogeneous malignant tumors, this kind of tumors are rare (7% to 15 %of all childhood tumors) and mostly arise from the embryonic mesoderm. Generally, soft tissue sarcomas can occur anywhere in the body. However, they are mostly observed in extremities (59%), the trunk (19%), the retroperitoneum (15%), or the head and neck (9%).[5-7]
The recurrence of soft tissue sarcomas is prone to local despite its optimal treatment, the intermediate or high-grade Soft tissue sarcomas have significant metastatic potential.
Rhabdomyosarcoma is the most common soft tissue sarcoma in children. According to the studies, the cure rate of children with rhabdomyosarcoma, especially those with local or regional rhabdomyosarcoma, has dramatically improved, from 25% (1970) to over 70% (2001) in Europe and the United States. Some benign types of the soft tissues tumors such as desmoid tumor needs wide resection for its control.
Magnetic Resonance Imaging ( MRI) is the diagnostic method that is more useful for diagnosing lipoma and hemangioma and also mass growth or symptoms of the tumors. CT-scan isn't helpful in soft tissue tumors but it is useful in pelvic area and determination of tumor stage. This method is useful for determination of lymphatic spread of lesions as synovial sarcoma and epiteloid, sarcoma and rhabdomyosarcoma. The final diagnostic way is biopsy which should be performed carefully, because it can affects diagnosis and patient survival. Basically, extremities' biopsy should be performed in open form to determine the stage of tumor.
The primary treatment of soft tissue sarcomas is surgery methods including intraregional, marginal and wide resection. But still there is no agreement addition of radiation to surgery according to the patients gain profit. 
The aim of this study was to evaluate the epidemiological characteristics of childhood soft tissue tumors in extremities in Iranian population.
Materials and Methods
In a cross-sectional study, 69 cases (0-16 year old children) referred to Shafa Orthopedic Hospital were evaluated for benign and malignant soft tissue tumors in their extremities between 2006 to 2012. Samples were obtained from 69 patients and all data from each patient were recorded in the designed checklist. This checklist was designed on the basis of project aims. The recorded variables included age at the time of diagnosis, treatment, recurrence rate, place of tumor, related pathological information. All of the primary diagnosis were performed by MRI.
Data analysis: Data were analyzed by SPSS software. Descriptive statistics is presented in frequency distribution table and statistics' indexes (mean and standard deviation). P≤ 0.05 considered as significant level.
In this study, mean age at the time of surgery was 12.6 ±3.1 years and the mean age at the diagnosis time was 9.45±4.2 years. Thirty cases (43.4%) were female and 39(56.5%) were male. Mean age and frequency of each type of tumors has been shown in table 1. Also, the frequency of involvement regions has been shown in table 2 and 3. Pathology report showed that 76.8 %of the lesions were benign and 23.2% were malignant. Most of the lesions (37.68%) were in the hand, after that the most involved region was thigh (20.28%) (table 3). In this study, hemangioma was reported in 21.2% cases that was the most frequency percentage. After this tumor ganglion cyst is 10.1% in another category.
During conducted study, hemangioma had more frequency in female and male groups but in male group after hemangioma, rhabdomyosarcoma tumors and ganglion cyst and fibromatosis had the most frequency.
In this study, surgical techniques were used in 78.3% of patients. Chemotherapy was performed for 11.6% of the patients who had microfibrosarcoma, sinovial cell sarcoma, clear cell sarcoma and rhabdomyosarcoma in their pathology report. 1.4 % of the patients received only radiotherapy and 8.7% patients received both chemotherapy and radiotherapy. In the present study, two cases with metastasis were reported. The pathologic reports showed rhabdomyosarcoma in these cases. In 13 cases, tumor recurrence was observed after the treatment. Rhabdomyosarcoma had the most recurrence and after that schwannomas had the most recurrence level after radiotherapy plus chemotherapy, also the most recurrence after chemotherapy was seen in synovial cell sarcoma.
40.6% of the patients had more than once surgical process and had higher recurrence level. Surgery complications such as venal and nervous injuries were reported in 10.1% of the patients due to extensive mass of tumors.
Exact prediction of soft tissue tumors prevalence is difficult because a lot of benign lesions are not sampled. But prevalence of soft tissue sarcomas is increasing. Malignant soft tissue tumors are rare and its prevalence is 30 cases in 1 million and they are about 10% of the cancers and 1% of the malignant tumors.
The pathology result was malignant in 23.2% cases that didn't have significant relationship with sex. 13 cases had tumor recurrence and tumor metastasis was reported in two patients that their primary tumor pathology reports of these cases were rhabdomyosarcoma. Soft tissue sarcoma has the higher risk of local recurrence and metastases. In the percent study, rhabdomyosarcoma had the most recurrence. Local recurrences, metastases and survival remain are the great concerns of high-grade soft tissue sarcomas after their surgical treatment.
Toro et al (2006) showed that the age-adjusted incidence rate of all kinds of soft tissue sarcomas increased 1.0 % per year over the last 2 decades in US population. They suggested that this increases may be a result of current shifts in the diagnostic criteria and soft tissue sarcoma classification. Arndt et al. (1999) showed that rhabdomyosarcoma is the third most common solid tumor in children fter neuroblastoma and Wilm's tumor.
Eilber et al (2003) evaluated the predictive factors of local recurrence of high-grade soft tissue sarcomas of extremities and their morbidity and mortality in 753 patients. They showed that the local recurrence of the tumors caused amputation in 38%of cases and can decrease the patients survival.
In the present study, surgery was the most technique for treatment of both benign and malignant masses. Radiotherapy and chemotherapy were used for controlling the malignant tumors. The basic treatment of soft tissue sarcomas is surgery. Surgical interventions are virus types and include the spectrum from simple wide local excision to radical amputation. In cases with chemotherapy the recurrence rate of the tumor was higher. According to the studies, radiotherapy can reduce the local recurrence rate of malignant tumors. It is indicate in the treatment of high grade tumors with deep to fascia and more than 5 cm diameter tumors.[8,18]
In this study, the most common region of soft tissue tumors in Iranian children was hand. Sadighi and Raafat (2003) evaluated Sarcoma in Iranian population. According to the results, the common place of sarcoma was in thigh in adult patients (48.6%).
In the present study, all diagnosis was made by MRI. According to the studies, MRI is more diagnostic method for soft tissue pathologies. In fact MRI shows soft tissue characteristic better. MRI is the choice diagnostic method for the preoperative evaluation of soft tissue lesions. This technique has superior soft-tissue contrast and ability to show anatomic relationship between lesion and surrounding structures.
Accordingly, some researchers believe that MRI can be a reliable method with high accuracy to determine if soft tissue lesions are malignant or not.[20-22] Chen et al.(2009) showed that MRI had sensitivity of 84.2% and specificity of 64.0% to diagnosis of benign lesions. Chung et al (2014) showed that MRI had sensitivity of 64%, specificity of 85%, positive predictive value of 32%, negative predictive value of 59% and an accuracy of 77% for diagnosis of benign soft tissue tumors.
This study gives us useful epidemiological information about soft tissue tumors in Iranian children. Hemangioma was the most benign tumor and rhabdomyosarcoma was the most malignant tumor.
Sam Hajaliloo Sami MD Associate Professor, Department of Orthopedic Surgery, Shafa Orthopedic Hospital, Iran University of Medical Sciences, Tehran, Iran. email@example.com
Tahmineh Mokhtari PhD Student of Anatomy, Department of anatomy, Medical school, Tehran University of Medical sciences, Tehran, Iran. firstname.lastname@example.org
Gholamreza Shafiee MD Orthopedic Surgeon, Rasool Akram Hospital, Golestan University of Medical Sciences, Kalaleh, Iran. Corresponding Author email@example.com
Acknowledgements: None declared.
Financial disclosure: None declared.
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