|Year : 2012 | Volume
| Issue : 3 | Page : 448-450
Adult primary pleomorphic leiomyosarcoma of forearm with axillary lymph node metastasis: A case report and literature review
Seema Gupta1, Nuzhat Husain2, Ashish Kumar3, Sham Sunder1
1 Department of Radiotherapy, C.S.M. Medical University, Lucknow, Uttar Pradesh, India
2 Department of Pathology, C.S.M. Medical University, Lucknow, Uttar Pradesh, India
3 Department of Orthopaedic Surgery, C.S.M. Medical University, Lucknow, Uttar Pradesh, India
|Date of Web Publication||17-Nov-2012|
Department of Radiotherapy, C.S. M. Medical University, Erstwhile (King George's Medical University), Street- Chowk, Lucknow - 226003, Uttar Pradesh
Source of Support: None, Conflict of Interest: None
A rare case of advanced pleomorphic leiomyosarcoma of forearm with axillary lymph node metastasis in a young adult, diagnosed with the aid of immunohistochemistry and electron microscopic examinations together with a review of the literature are reported. The primary tumor involved the extensor and flexor aspect of forearm without bone involvement and metastasized to the axillary lymph nodes. Patient showed poor treatment outcome with adjuvant chemoradiotherapy following incomplete surgery.
Keywords: Immunohistochemistry, lymph node, pleomorphic leiomyosarcoma, treatment
|How to cite this article:|
Gupta S, Husain N, Kumar A, Sunder S. Adult primary pleomorphic leiomyosarcoma of forearm with axillary lymph node metastasis: A case report and literature review. J Can Res Ther 2012;8:448-50
|How to cite this URL:|
Gupta S, Husain N, Kumar A, Sunder S. Adult primary pleomorphic leiomyosarcoma of forearm with axillary lymph node metastasis: A case report and literature review. J Can Res Ther [serial online] 2012 [cited 2020 Jul 11];8:448-50. Available from: http://www.cancerjournal.net/text.asp?2012/8/3/448/103532
| > Introduction|| |
Pleomorphic leiomyosarcoma accounts for about 9% of all leiomyosarcomas, which are 5-10% of all soft tissue sarcomas, and these accounts for only 0.7% of all adult cancer. 
The overall survival of all variants of leiomyosarcoma is not very encouraging.  Lymph node metastasis which is an infrequent event in the natural history of pleomorphic leiomyosarcomas are characterized by aggressiveness and poor prognosis. 
Here we report a case of pleomorphic leiomyosarcoma of forearm in a young adult with axillary node metastasis which is a rare presentation, who showed poor outcome with multimodality treatment.
| > Case Report|| |
A 21-year-old young male presented to us in May 2011, with large diffuse swelling over the right forearm which had been progressively increasing for 5 months. On examination he had a painless swelling involving the extensor and flexor aspect of right forearm extending from elbow above to lower third of forearm [Figure 1]a. Contrast-enhanced MRI showed a large soft tissue mass lesion involving extensor and flexor muscles of right forearm along with intermuscular septa, blood vessel involvement and necrosis within the lesion [Figure 2]. Biopsy from the forearm lesion was suggestive of pleomorphic leiomyosarcoma with foci of necrosis. Immunohistochemistry was positive for smooth muscle actin [Figure 3]a and b.
|Figure 1: Pretreatment large tumor extending from elbow above to lower third of forearm (a), Progression of disease observed during second cycle of gemcitabine and docetaxel based chemotherapy following post operative radiotherapy (b).|
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|Figure 2: Pretreatment contrast enhanced T1, T2 axial (a, b) and T1,T2 coronal (c, d) of forearm showing large soft tissue mass lesion involving predominantly extensor aspect of right forearm, adherent to skin.|
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|Figure 3: (a) Trucut biopsy H and E stain showing a pleomorphic tumor (b). Smooth muscle actin expression in biopsied tissue. (c) Aspiration from axillary node showing neoplastic cells (d) Lymph node showing|
metastases from pleomorphic leiomyosarcoma (HandE x 10X)
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Since the primary lesion was unresectable, patient was given 2 cycles of doxorubicin(50mg/ m 2 in divided doses day 1, day 2), ifosfamide (5g/m 2 in divided doses day 1,2 and 3) and vincristine (1.4 mg/m 2 day 1) chemotherapy at 3 weeks interval, to which he did not show good response and hence was started on gemcitabine and docetaxal chemotherapy. Patient completed these 2 cycles of chemotherapy in August 2011, when he developed palpable axillary lymphnode without any significant benefit in primary forearm tumor [Figure 4]. Fine needle aspiration cytology from the axillary lymphnode was suggestive of metastasis from pleomorphic leiomyosarcoma [Figure 3]c, which was later confirmed by excisional biopsy [Figure 3]d. Hence, patient was considered for surgery, where gross resection of primary forearm tumor, along with excision of involved axillary lympnode was done. The margins of the excised primary tumor were microscopically positive and adherent to skin; capsular invasion of the involved axillary lymph node was also evident [Figure 5]a and b. Following surgery the patient received adjuvant external beam radiotherapy to a total dose of 60 Gy in 30 fractions in conventional fractionation schedule. Patient then received adjuvant gemcitabine and docetaxal based chemotherapy, during the second cycle in January 2012, progression of disease at the local site was observed [Figure 1]b. Hence, there was no significant benefit seen with adjuvant treatment, with only 5 months progression free survival observed from diagnosis of lymph node metastasis.
|Figure 4: Contrast enhanced CT scan of upper extremity and axilla after completion of 4 cycles of doxorubicin and docetaxel based chemotherapy; the tumor metastasized to axillary lymph node without signifi cant benefit in the primary tumor (a,b)|
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|Figure 5: Macroscopic examination of excised primary lesion of forearm showing large necrotic mass adherent to skin (a) Macroscopic examination of axillary lymph node showing mass in lymph node involving the capsule with hemorrhage (b)|
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| > Discussion|| |
Lymph node metastasis is considered an infrequent event in the natural history of soft tissue sarcomas with an overall prevalence of 2.6% reported from the recent prospective study, which is much less than reported earlier mainly from retrospective, small and heterogenous studies showing an overall incidence of 9.1% to 10.8%. 
There are a limited number of studies and case reports of pleomorphic leiomyosarcoma with regional lymph node involvement. The impact of lymph node metastasis in soft tissue sarcoma on the prognosis and treatment outcome is difficult to assess due limited number of cases and studies available.  Some investigators have concluded from retrospective and small clinical studies that lymph node metastasis is an indication of disseminated disease, and any treatment of these lymph nodes is only palliative,  whereas others believe an aggressive approach of treatment can bring about long-term survival.  However, there are convincing evidences which show that median survival with patients treated with radical therapeutic lymphadenectomy with curative intent was 12.7-16.3 months, with an overall 5-year survival of 34%-46%, whereas less than curative radical procedure like biopsy was associated with median survival of 5.9 months.  This survival data is almost similar to that seen in patients without lymph node involvement with complete and incomplete surgery of the primary tumor, suggesting that lymph node involvement in LMS should not be considered a contraindication for radical treatment as this may provide long-term survival. 
Therefore, success of the initial surgical management is an important prognostic factor, as complete surgical excision is associated with longer survival. As seen in our patient who presented with advanced pleomorphic leiomyosarcoma of forearm with axillary lymphnode metastasis, with incomplete resection of tumor resulted in poor outcome even with adjuvant combination chemotherapy and radiotherapy.
Hence adjuvant radiation treatment or chemotherapy appears to be ineffective in achieving local control when there is residual tumor postoperatively,  but studies have shown that if surgical margins are narrow or positive, chemotherapy and radiotherapy are beneficial. 
Although leiomyosarcoma tends to be resistant to radiation and chemotherapy, each case is different and varying results are seen, however, there are little published data available to validate the role of chemoradiotherapy.
Recent reported studies have shown beneficial role of combination chemotherapy in unresectable leiomyosarcoma. Combined ifosfamide, doxorubicin and Paclitaxel based chemotherapy yielded response rate ranging from 16 to 30.3% with minimal 3-month progression-free rate of 58%. ,
Hensley et al. has reported 6-month progression-free rate of 47% with combined gemcitabine and docetaxel chemotherapy with better response rate of 53% and median overall survival of 17.9 months in these unresectable patients.  Our patient with unresectable disease showed a minimal progression free survival of 3 months even with neoadjuvant chemotherapy and adjuvant radiotherapy following incomplete surgery. This proves the role of complete surgery as the main curative treatment modality with long term survival. Adjuvant treatment in unresectable tumors can be employed but is not beneficial in most instances, and seems appropriate only for palliation.
| > References|| |
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]