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 Table of Contents  
CASE REPORT
Year : 2018  |  Volume : 6  |  Issue : 2  |  Page : 60-62

Subcutaneous leiomyosarcoma of anterior abdominal wall in young girl: Rare case report


1 Department of General Surgery, All India Institute of Medical Sciences, Patna, Bihar, India
2 Department of Trauma and Emergency, All India Institute of Medical Sciences, Bhubneshwar, Odisha, India
3 Department of Surgical Disciplines, All India Institute of Medical Sciences, New Delhi, India
4 Department of Paediatrics Surgery, All India Institute of Medical Sciences, Patna, Bihar, India

Date of Web Publication29-May-2018

Correspondence Address:
Dr. Anil Kumar
All India Institute of Medical Sciences, Patna, Type-5, Block-B, AIIMS Residential Complex, Hydraulic Road, Patna, Bihar
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ssj.ssj_3_18

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  Abstract 

Leiomyosarcoma (LMS) overall comprises 5%–10% of all soft-tissue sarcoma. Subcutaneous LMS accounts only for 1%–2% of all superficial soft-tissue sarcoma. Usually, subcutaneous LMS occur in lower extremity; however, it may occur anywhere in the body. Its occurrence in the abdominal wall is extremely rare. Here, we report a case of recurrent LMS of anterior abdominal wall in a 25-year-old female.

Keywords: Abdominal wall, leiomyosarcoma, recurrent, subcutaneous


How to cite this article:
Kumar A, Paswan SS, Chumber S, Kumar B. Subcutaneous leiomyosarcoma of anterior abdominal wall in young girl: Rare case report. Saudi Surg J 2018;6:60-2

How to cite this URL:
Kumar A, Paswan SS, Chumber S, Kumar B. Subcutaneous leiomyosarcoma of anterior abdominal wall in young girl: Rare case report. Saudi Surg J [serial online] 2018 [cited 2020 Jul 10];6:60-2. Available from: http://www.saudisurgj.org/text.asp?2018/6/2/60/233489


  Introduction Top


Soft-tissue sarcomas are one of the rare conditions in the medical world, with occurrence of approximately 1% of adult solid malignant cancer.[1] Leiomyosarcoma (LMS) is mesenchymal neoplasm which arises from smooth muscles and accounts for 5%–10% of soft-tissue sarcoma.[2] Subcutaneous LMSs account only for 1%–2% of all soft-tissue sarcomas and arise from small- to medium-sized blood vessels present in the subcutaneous tissue.[3] Although superficial LMSs can occur anywhere in the body, but the usual site is lower extremity, preferably thigh followed by trunk.[4],[5] The LMS of anterior abdominal wall is very rare to occur, and only few cases are reported in literature.[6],[7] Here, we report a case of recurrent subcutaneous LMS arising from the anterior abdominal wall.


  Case Report Top


A 25-year-old unmarried female referred to our institute with recurrent abdominal lump with ulceration. She had a history of surgical excision of lump from the anterior abdominal wall twice, first 7 years back and second 3 years back at primary health center (PHC). Biopsy reports were not available as histopathological examination was not carried out at PHC. There was no history of chemotherapy or radiotherapy. Physical examination revealed a lump of 27 cm × 22 cm in the middle of the abdomen with evidence of ulceration on the apex of the lump [Figure 1]. The tumor was fixed to the overlying skin. The lump was nonreducible and noncompressible, and also there was no impulse on coughing. The overlying skin was ulcerated. All laboratory investigations including tumor markers were within normal limits. Fine-needle aspiration cytology showed spindle cells in clusters with atypical and typical mitosis, suggesting the diagnosis of LMS. The computed tomography (CT) scan showed a large, lobulated heterogeneously contrast-enhancing soft-tissue mass in the middle of anterior abdominal wall not involving the underlying muscle and rectus sheath. All the intra-abdominal structures on CT were normal without any evidence of metastasis. She was planned for surgical excision of this lump. Under general anesthesia with full aseptic precaution, the mass was excised with 5 cm of healthy margin. The abdominal cavity was opened to make sure that neither there was an internal extension of tumor nor any internal organs were damaged [Figure 2]. The defect was closed primarily. The excised lump was measured 30 cm × 20 cm × 8 cm in diameter without central necrosis and hemorrhage on its cut surface [Figure 3]. Histopathological examination showed that the tumor is composed of spindle cells with scant-to-moderate amount of eosinophilic cytoplasm and elongated nuclei [Figure 4]. All resected margins were free from tumor. In immunohistochemistry, the tumor cells were positive for smooth muscle actin, but negative for desmin and S-100 [Figure 5]. With these findings, the diagnosis of subcutaneous LMS was confirmed. With this report, she referred to radiotherapy department for radiotherapy and chemotherapy. She has now been on our follow-up for the last 8 months and is doing well. Informed consent was taken from the patient for the publication of this case report and accompanying images.
Figure 1: Lump of 27 cm × 22 cm in the middle of abdomen with evidence of ulceration on the apex of the lump

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Figure 2: The open abdominal cavity after excision of the tumor

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Figure 3: Excised lump of 30 cm × 20 cm × 8 cm in diameter

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Figure 4: Spindle cells with scant-to-moderate amount of eosinophilic cytoplasm and elongated nuclei

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Figure 5: Immunohistochemistry showing the smooth muscle actin positivity and desmin and S-100 negativity of tumor cells

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  Discussion Top


Soft-tissue sarcomas are rare tumors comprising only 1% of all adult solid tumors. Histologically there is more than 50 subtypes of this tumour with most common subtypes are liposarcoma, malignant fibrous histiocytoma and LMS. LMS arises from smooth muscle cells of wall of blood vessels, internal organs, and skin. Although soft-tissue sarcoma can occur at any anatomic site, 45% occur in extremities, most commonly in thigh, followed by visceral (20%), retroperitoneal (15%), truncal or thoracic (10%), and other location (10%). The occurrence of subcutaneous LMS on the anterior abdominal wall is extremely rare [4] as in our case. Soft-tissue sarcoma becomes more common with increasing age but may occur in the pediatric age group.[8] Majority of the patients present with the pain which can be spontaneous or induced by pressure.[5] The patient usually presents with tumor mass of small to big size with well-defined or irregular border, pedunculated mass, and umblication with discoloration of skin. Dermal LMS presents as a small firm fibrous nodule of size <2 cm, whereas subcutaneous LMSs are larger in size with an average of 4 cm.[5] However, radiological imaging such as ultrasonography and CT are helpful, but confirmatory diagnosis can be made only histologically. On ultrasonography, tumor appears as hypoechoic solid mass with well-defined or ill-defined borders. Magnetic resonance imaging should be used for staging purpose. Surgical excision with wide margins in the early stage is the treatment of choice [6],[7] as done in the present case. Early surgical intervention as a wide local excision with safe surgical margins is the most important prognostic factor in patients with superficial LMSs. Histologically, the tumor shows perpendicularly arranged fascicles of spindle cell with abundant eosinophilic cytoplasm and vacuolated and elongated blunt-ended nuclei. Previously, it was reported that radiotherapy and chemotherapy had limited role in LMS,[4] and contrary to this, Massi et al. reported that adjuvant radiotherapy along with wide local excision has excellent result.[9] In the patients with subcutaneous LMSs, local recurrence has been reported from 14% to 42%.[4] In 30%–60% of patients with subcutaneous LMS, metastasis has been reported.[4] Lung is the most common site for the metastases, and hematogenous spread is the most common mode.[4] The long-term prognosis remains poor despite adequate local control with or without adjuvant radiation therapy in the patients with subcutaneous LMSs.[5] In immunosuppressed patients, tumor may be aggressive and require long-term follow-up.[10]

Soft-tissue sarcomas are rare adult solid tumors and anterior abdominal wall LMSs are even rarer. Misleading features of tumors and lack of knowledge about unusual site of these tumors lead to delay in diagnosis and management. Early surgical intervention along with chemotherapy, radiotherapy, and long follow-up is an ideal approach to prevent metastasis as well as recurrence.

Acknowledgment

We would like to thank Dr. Amit Kumar Sinha, Assistant Professor (Department of Pediatric Surgery), All India Institute of Medical Sciences, Patna.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patients understand that name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
van Vliet M, Kliffen M, Krestin GP, van Dijke CF. Soft tissue sarcomas at a glance: Clinical, histological, and MR imaging features of malignant extremity soft tissue tumors. Eur Radiol 2009;19:1499-511.  Back to cited text no. 1
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2.
Gustafson P, Willén H, Baldetorp B, Fernö M, Akerman M, Rydholm A, et al. Soft tissue leiomyosarcoma. A population-based epidemiologic and prognostic study of 48 patients, including cellular DNA content. Cancer 1992;70:114-9.  Back to cited text no. 2
    
3.
Yajima K, Shirai Y, Fujita N, Sato D, Umezu H, Hatakeyama K, et al. Agiant subcutaneous leiomyosarcoma arising in the inguinal region. World J Surg Oncol 2005;3:14.  Back to cited text no. 3
    
4.
Fields JP, Helwig EB. Leiomyosarcoma of the skin and subcutaneous tissue. Cancer 1981;47:156-69.  Back to cited text no. 4
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5.
Svarvar C, Böhling T, Berlin O, Gustafson P, Follerås G, Bjerkehagen B, et al. Clinical course of nonvisceral soft tissue leiomyosarcoma in 225 patients from the Scandinavian sarcoma group. Cancer 2007;109:282-91.  Back to cited text no. 5
    
6.
Eken H, Karagul S, Topgul K, Yoruker S, Ozen N, Gun S, et al. Giant cutaneous leiomyosarcoma originating from the anterior abdominal wall: A case report. Am J Case Rep 2016;17:35-8.  Back to cited text no. 6
    
7.
Jena S, Bhattacharya S, Roy S. Giant subcutaneous leiomyosarcoma of anterior abdominal wall. Case Rep Surg 2014;2014:308916.  Back to cited text no. 7
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8.
Koike Y, Imaoka H, Otake K, Inoue M, Uchida K, Kusunoki M. Primary leiomyosarcoma of the abdominal wall mimicking nodular fasciitis in a child. J Paediatr Surg Case Rep 2015;3:252-4.  Back to cited text no. 8
    
9.
Massi D, Beltrami G, Mela MM, Pertici M, Capanna R, Franchi A, et al. Prognostic factors in soft tissue leiomyosarcoma of the extremities: A retrospective analysis of 42 cases. Eur J Surg Oncol 2004;30:565-72.  Back to cited text no. 9
    
10.
Humphreys TR, Finkelstein DH, Lee JB. Superficial leiomyosarcoma treated with Mohs micrographic surgery. Dermatol Surg 2004;30:108-12.  Back to cited text no. 10
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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

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