<b>Medical Journal Article Annotated Citations 

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Am Surg 1998 Jan;64(1):53-60; discussion 60-1 
<b>Leiomyosarcoma: a 45-year review at Charity Hospital, New Orleans. </b>
Hill MA, Mera R, Levine EA. Section of Surgical Oncology, Louisiana State Univ Medical Center, NewOrleans 70112, USA. 

Approximately 2 to 9% of all soft tissue sarcomas are leiomyosarcomas (LMS). LMS arises nearly exclusively as tumors in adults, with peak incidence occurring in the fifth and sixth decades. The purpose of this study was to analyze disease-specific survival and define prognostic factors in patients with this disease who were treated and followed at a single institution. Fifty-eight cases of LMS were identified in the Tumor Registry of the Medical Center of Louisiana at New Orleans (charity Hospital) from 1950 to 1995. Charts were reviewed and tissue blocks reexamined to confirm the diagnosis. Follow-up information was available for 56 of 58 (96%) patients. Univariate and multivariate analyses were performed to analyze which factors predict outcome. The median survival time was 138 months. Univariate analysis identified age (> 48 years), location (retroperitoneal vs other sites), and extent of disease as prognostic factors. Multivariate analysis revealed that only age and the extent of disease at presentation are independent prognostic indicators. Race, sex, and adjuvant therapy were not significant prognostic factors. Surgical resection remains the therapeutic mainstay for patients with LMS. The value of other treatment modalities is largely limited to surgical failures. The data show that the age of the patient and the extent of disease at presentation are the best predictors of long-term survival. <b>LMS has a good prognosis when complete resection of localized lesions can be achieved. </b> 

&&url PMID: 9457038 


Cancer 1995 Oct 15;76(8):1398-405 
<b>High grade soft tissue sarcoma of the flexor fossae. Size rather than compartmental status determine prognosis.</b>
Yang RS, Lane JM, Eilber FR, Dorey FJ, al-Shaikh R, Schumacher LY, Rosen G, Forscher CA, Eckardt JJ. Department of Orthopaedic Surgery, UCLA School of Medicine, Cedars-Sinai Comprehensive Cancer Center, Los Angeles, California 90095-6902, USA. 

,,, High grade soft tissue sarcoma arising in the popliteal space, axilla, and antecubital fossae (flexor fossae tumors) have by convention been classified as extracompartmental tumors ,,, Advances in neoadjuvant chemotherapy and radiation therapy have made surgical resection more feasible. The hypothesis to be tested is that compartmental status may not be of prognostic significance if the tumor is adjusted for size, histologic grade, and distant metastasis after undergoing adjuvant chemotherapy and radiation. ,,, From ...1976 to ...1992, 22 patients with high grade soft tissue sarcomas of the flexor fossae (Group A) were treated at UCLA Medical Center. The histologic subtypes were ,,, leiomyosarcoma (two), ,,, The popliteal fossa was the location in 11, the axilla in 10, and the antecubital fossa in 1. Wide resection was attempted in all patients after preoperative chemotherapy and radiation therapy. ,,,  A group of 77 patients (Group B) with high grade soft tissue sarcoma located within an extremity compartment were chosen to test the hypothesis that survival of patients with tumors in the flexor fossae is equal to that of patients with intracompartmental tumors of similar size and grade if both are given adjuvant therapy. This group was chosen so that histologic subtype, size, sex, and location would be similar in the two groups,,, The median follow-up of survivors in Group A was 104 months and for patients in Group B was 79 months,,, The 5-year cumulative survival rate (Kaplan-Meier method) of patients in Group A was 76%, and 67% for those in Group B. The difference was not significant. 

Three patients in Group A (14%) and 17 (22%) in Group B had local tumor recurrence. Eight patients in Group A (36%) and 27 (35%) in Group B had lung metastases. Age, sex, histologic subtype, and surgical margins did not affect survival outcomes, lung metastasis, and local recurrence. However, patients with larger tumors (maximum dimension > or = 8 cm or cross-sectional area > or = 40 cm2) had significantly poorer survival, more metastases, and local recurrences. ,,,<b>Flexor fossae sarcomas do not have a poorer prognosis than extremity intracompartmental tumors when adjusted for size, distant metastasis, and histologic grade when they are treated with adjuvant radiation therapy, chemotherapy, and surgery. [NOTE: this is a group of mixed sarcomas, not LMS alone.  Ed.]</b> 

&&url PMID: 8620414 


Rev Chir Orthop Reparatrice Appar Mot 1995;81(4):338-43 
<b>[Bone metastasis of leiomyosarcoma. Apropos of a case]. </b>[Article in French] 
Mariconda M, Tacchetti C, Misiti CM, Lotti G, Ammendolia A, Millo R, Milano C. Clinique Orthopedique, Faculte de Medecine de Catanzaro, Universite de Reggio Calabria, Italie. 

,,, Bone leiomyosarcoma is a rare tumor, whether it may be primary or secondary. The authors report on the case of a woman, aged 67, admitted in January 1992 complaining of pain in the left hip and the upper end of the femur. ,,, In 1985 the patient underwent surgical excision of a soft tissue tumor in the right thigh, histologically diagnosed as a benign fibrous tumor. This lesion recurred locally four times and repeated excisions were performed throughout the years, always with a histological diagnosis of a benign lesion. On admission to hospital, the physical examination as well as laboratory data and plain roentgenograms were unremarkable.<b>[ !  Ed.] </b> Both tomography and MRI showed a lesion in the upper end of the left femur. An isotopic bone scan showed marked increased uptake in the left hip extending to the femoral diaphysis. An open biopsy was performed for histology, immunohistochemistry and electron microscopy. A diagnosis of metastatic leiomyosarcoma was made. The retrospective histological examination of specimens of the soft tissue tumor excised in 1985 showed the same immunohistochemical features of the contralateral leiomyosarcoma. On this basis, one stage resection of the left hip and the upper end of the femur was performed and a Kotz modular prosthesis was inserted. Postoperative healing was achieved without any complications and the function of the operated limb was satisfactory. Three months after the operation pulmonary lesions were noted on chest radiographs and CT scan. The patient died two years after the first admission for widespread metastasis. ,,, In the reported case, the bony metastasis appeared to be the presenting finding of the soft tissue tumor of the contralateral thigh. This presentation is rare in previously published series. <b>The misdiagnosis of the primary tumor had caused local recurrences, and an increased malignity occurred. According to the literature, a soft tissue leiomyosarcoma can be easily confused with other spindle cell lesions. Therefore an accurate histological and ultrastructural diagnosis is necessary for adequate surgical treatment.</b>  

&&url PMID: 8560004 


Cancer 1992 Jul 1;70(1):114-9 
<b>Soft tissue leiomyosarcoma. A population-based epidemiologic and prognostic study of 48 patients, including cellular DNA content. </b>
Gustafson P, Willen H, Baldetorp B, Ferno M, Akerman M, Rydholm A. Department of Orthopedics, University Hospital, Lund, Sweden. 

,,, Leiomyosarcoma of soft tissue is a rare tumor. ,,,Epidemiology and prognosis were analyzed in a consecutive, population-based series of 48 patients with subcutaneous and deep-seated leiomyosarcoma in the extremities and trunk wall with a complete follow-up of a minimum of 3 years. Cutaneous tumors were not included. ,,, The annual incidence was 0.13/10(5). The ratio of men to women was 1.2, and the median age was 65 years. The thigh was the most common location. Almost half of the tumors were subcutaneous. The median tumor size was 6 cm (range, 1-25 cm). All patients were treated with surgery, and in 19 cases it was combined with adjuvant radiation therapy or chemotherapy. The cumulative 5-year survival rate was 64%. Multivariate analysis indicated that age of 60 years or greater (relative risk [RR] = 8) and intratumoral vascular invasion (RR = 4) were independent risk factors for death resulting from tumor. DNA aneuploidy (RR = 4) and tumor necrosis (RR = 3) were associated with poor prognosis, but did not reach statistic significance. ,,, 

&&url PMID: 1606532 


Cancer 1986 Apr 1;57(7):1395-400
<b>Intramural venous leiomyosarcomas.</b> 
Leu HJ, Makek M. 

Five cases of intramural venous leiomyosarcomas are described. Only one was localized in the inferior vena cava, the classic site; the other four cases were situated in smaller veins, namely, the short and long saphenous veins, the external jugular vein, and a superficial vein at the back of the hand. Light and, in two cases, electron microscopy, as well as immunohistochemical examinations for factor VIII, helped to establish the diagnosis. The fate of these cases indicates that size and localization are the main determining factors of outcome, whereas the level of mitotic activity is informative only if it is high.<b> Small tumors in superficial veins that are detected early may have an excellent prognosis, even after limited removal of the tumor.</b> 

&&url PMID: 3081244 
