The leiomyosarcoma that occur on the skin includes : trivial and metastatic leiomyosarcoma . It is a malignant tumour of flaccid tissues , exceedingly rare , comprise only 4.0 % to 6.5 % of sarcoma .
What is Cutaneous Leiomyosarcoma?
The superficial leiomyosarcoma are subdivided into : dermal ( SCL ) and subcutaneous , depending on their location in the thickness of the skin .
They are called superficial dermal leiomyosarcomas when they are located mainly in the derma and infrequently extend to hypodermic fat ; it is believed that these tumour originate in the erector sinew of thehair follicle .
The hypodermic leiomyosarcomas are mainly found in the subcutaneous cellular tissue paper and rise in the smooth muscle of the stemma watercraft .
The superficial leiomyosarcoma is ofttimes found in individuals who are 40 - 60 long time older , with a maximal incidence in the sixth decade of life . It is twice more common in workforce than in women .
Clinically , superficial dermal leiomyosarcomas often correspond to firm , solitary nodules , deeply seated , with variable erythema and hyperpigmentation , and sometimes they show ulceration due to their obsession with the epidermis , while the subcutaneous leiomyosarcomas seem as a large roving mass , located in hypodermis without cuticular change ; however , they do not have a distinctive clinical aspect .
Regarding its location , there is a discrepancy , while the most frequent site in the superficial cutaneous leiomyosarcoma is head and neck ; the subcutaneous leiomyosarcomas are located rather in extremities .
They are often asymptomatic ( the patient role does not have any symptom ) , but in both types there are report of spontaneous bother , sensitivity , but also other uncouth symptom were : pruritus(itching ) , bleed andburning maven .
Among the predisposing broker that have been described : psychic trauma , radiation , chemicals and sunlight are the common ones . There is no clearly demonstrate peril of transformation or specialization of pre - existent leiomyoma in leiomyosarcomas . Although the development of various malignant tumors in scar tissue is well known , the mellow affiliation of trivial cutaneous leiomyosarcomas is found in continuing tan scars . The development of trivial cutaneous leiomyosarcomas on smallpox scratch was also described .
It is important to note that the depth of these neoplasms represents the most important predictive cistron . That is , the superficial cutaneous leiomyosarcoma have a significant content for local intrusion and subclinical university extension ; however , the metastatic potential of these tumors is considered to be extremely downhearted : 5 to 10 % , unlike subcutaneous leiomyosarcomas , in which local return has been reported from 50 % to 70 % and a potential development of metastasis to aloofness of 30 to 60 % , most commonly to lungs .
The differential diagnosing from the clinical pointedness of vista include : cysts , lipomas , fibroids , granulomas , dermal nevus , pyogenic granuloma , neurofibromas , dermatofibromas , carcinomas , amelanotic melanoma , benignant tumors derive from annexa and other soft tissue sarcomas .
The diagnosis is made by clinical suspicion and histopathological subject area . operative extirpation is found as a discourse for both entities . Chemotherapy , radiation therapy and drug - directed therapy have not yet replaced surgery .
There are several factors that correlate with the prognosis , these let in : tumor size of it , in high spirits mitotic index , presence or absence of gangrene , vascular invasion . The survival pace for tumors small than 2 curium was 95 % , whereas in tumors that outstrip 5 cm , survival was reduced to 30 % .
Recurrence is variable : 30 - 50 % in the cutaneous form , while in the subcutaneous it reach 70 % , being this deeper and mitotically more dynamic than the primary lesion . The period of intro of return is variable , they germinate from 1 to 5 geezerhood , after surgical process . It is then important to monitor affected role with chest x - ray , since the highest frequency of metastasis is lung , commonly by subcutaneous leiomyosarcomas in a third of patients and 25 % may have metastases in regional lymph node , which square off an unfavourable prognosis .
Conclusion
dermal leiomyosarcomas are infrequent malignant tumors , derive from smooth sinew ; they constitute 10 % of gentle tissue sarcomas . Its classification and forecast depend on the berth of origin and its anatomic position ; 85 % of the lesions come about in the extremity . The sanative direction is always surgical , subsist possibility of return and metastasis , although it is rare .
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