Exophytic growth of a neglected giant subcutaneous Leiomyosarcoma of the lower extremity. A case report
© Angeloni et al; licensee BioMed Central Ltd. 2008
Received: 15 December 2007
Accepted: 21 May 2008
Published: 21 May 2008
Superficial leiomyosarcoma is an exceedingly uncommon malignant tumor which could be located either to cutaneous or subcutaneous tissues. Increased mass size and depth, advanced tumor staging and inadequate surgical excision are the main prognostic factors for poor result.
We report a rare case of a 71-year-old man with an extensive exophytic lesion (12 × 10 cm) in the anterior-medial side of the proximal right tibia. The lesion was painless and consistently neglected by the patient until a skin trauma caused ulceration of the affected area. Magnetic Resonance Imaging revealed a soft-tissue mass which was well defined from the surrounding bone and muscles. As initial biopsy in another hospital hadn't clarified the true nature of the lesion, new samples were taken and the diagnosis of leiomyosarcoma was established. Laboratory examination showed no distant metastasis and wide excision of the neoplasm was decided. After tumor resection, the remaining soft tissue and skin defect was covered with a gastrocnemius myocutaneous flap. The postoperative period was uneventful and wound healing was followed by local radiotherapy and systemic chemotherapy. At 3 years follow up, no recurrence or metastasis was identified and the patient was able to walk and stand without impairment of his ambulatory status.
Proper surgical management of soft tissue leiomyosarcoma continues to remain the cornerstone of treatment efficacy and the most important prognostic factor for patients' survival. Reconstruction of the remaining soft tissue defect should be always performed at the same operative time when removal of giant size tumors leaves an uncovered cavity with an inadequate sleeve of muscular and skin tissues.
Superficial Leiomyosarcoma (LMS) is a quite uncommon tumor which is mainly developed in the proximal portion of the lower limbs [1–3]. The lesion can be appeared as a single nodular mass or multiple swelling tumours [2, 4]. Although it may occur in any age, superficial LMS is more prevalent between the fifth and seventh decade of life. Furthermore, a male predominance with a male-to-female ratio of 2:1 was also described [1, 2]. The tumor could be located either cutaneously or subcutaneously. In the first case, it is arisen from the limb extensor surfaces where a greater density of hair structures (arrectores pilorum muscles) exists [1, 2, 5]. It is frequently quite small-less 2 cm-causing local changes like skin discoloration, umblication and ulceration [1, 2].
Subcutaneous soft tissue Leiomyosarcomas (LMSs) are apparently larger and vascular in their origin, grow faster and they may produce similar changes in the skin. Their incidence is even rarer accounting for 2.3 – 5.3% of all malignant soft tissue tumors [1, 5] and 4 – 6,5% of all soft tissue sarcomas [6, 7]. As clinical signs are not strongly suggestive of malignancy and vary from person to person, the definitive diagnosis may be delayed until the later stage of the disease . Wide excision constitutes the current treatment of choice [1, 2, 5, 8–15] while radiotherapy (RTP) and chemotherapy (CTP) may be considered in high grade lesions [1, 2, 10, 12–15].
We report a rare case of a giant subcutaneous LMS of the proximal tibia. Wide excision of the tumour in combination with local RTP and systemic CTP led to good functional outcome and no recurrence until the latest follow up (3 yrs postoperatively).
The postoperative course was uneventful and no local or systemic complications were encountered. Due to the weakening of the proximal tibia from the excision of the anterior-medial cortex, partial weight bearing for 6 weeks was advised. After wound healing the patient was submitted to the Oncology Department for local RTP and systemic CTP.
At the latest follow up visit (3 years) clinical and laboratory examination (X-Rays, MRI, Bone Scan) didn't reveal any signs of tumor recurrence or metastasis. The patient could stand and walk without any restriction and he was able to perform his usual daily activities.
The distinction between cutaneous and subcutaneous LMS is of primary importance for prognostic reasons [2, 8, 10]. Although the cutaneous LMSs are associated with local recurrence in about 30–50% of cases only a low incidence of 0–10% of metastasis has been described [1, 2, 15, 18]. On the other hand, subcutaneous LMSs may develop local recurrence in about 40–60% of patients and distant metastasis in 20 to 60% of patients [1, 2, 5, 8, 10, 12, 15]. As a result, the mortality may be raised up to 30–40% [2, 18]. Despite the fact that tumor location and depth affect the recurrence rate and metastatic risk, the mitotic activity seems not to have a similar effect [1, 2, 8].
In the current patient, the size of the tumor (12 × 10 cm) was higher than the previously reported cases. Specifically, the mean size reported in literature for superficial LMSs is about 5 cm, with a wide range from 0.5 to 19 cm [2, 4, 8, 19]. Some authors described cases of LMSs with larger size (up to 35 cm), but in their series they didn't distinguish the superficial soft tissue LMSs from non-visceral deep LMSs [15, 20]. Large tumors (>5 cm) are considered to have worst prognosis in terms of recurrence and metastasis [1, 8, 12–15]. In a multivariate analysis of 267 patients with leiomyosarcoma, Miyajima et al. found that tumour size and advanced staging were the only factors that were correlated independently with decreased survival .
Wide excision of the lesion is of outmost importance as it is strongly correlated with disease-free survival [1, 2, 8, 10, 12–15]. Massi et al. in a retrospective study of 62 patients with extremity soft tissue leiomyosarcoma reported that seven out of the nine patients who had developed one or more local recurrences had been surgically treated with either marginal or intralesional excision . Similarly, Svarvar et al. in a large study of 225 patients with leiomyosarcoma which was based on Scandinavian Sarcoma Group Register showed that the inadequate local treatment was the main risk factor for local recurrence .
Many authors have been supported the application of postoperative RTP and CTP in high grade LMSs [1, 2, 10, 12–15]. However, the role of adjuvant therapy in LMSs is still controversial as no sufficient statistical evidence exists regarding their efficacy. The above treatment modalities may have a higher impact on local and systemic tumor respond when adequate excision of large and high grade lesions could not be achieved [13–15].
Even though delayed diagnosis and giant size of soft tissue leioymyosarcomas may adversely affect the final result, successful recovery could be anticipated. Special focus should be given to careful and meticulous wide excision of the mass as the role of chemotherapy and radiotherapy is still blur and unknown.
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal
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