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Bone

Medical Journal Article Annotated Citations Latest Pubmed/Medline Search for Leiomyosarcoma bone primary Skeletal Radiol 1987;16(5):364-76 Primary leiomyosarcoma of bone. A clinical, radiographic, pathologic-anatomic, and prognostic study of 16 cases. Berlin O, Angervall L, Kindblom LG, Berlin IC, Stener B. Sixteen cases of primary leiomyosarcoma of bone are described. The patients, 11 males and 5 females, ranged in age from 9 to 74 years. The annual incidence of this tumor in Sweden was calculated to be 0.09 cases per million. This figure was obtained by reviewing a Swedish series of spindle cell sarcomas of bone of which one quarter (11/44) were diagnosed by us as primary leiomyosarcoma. The diagnosis was based on light- and electron-microscopic examinations using the same criteria as for leiomyosarcoma of soft tissues. Thirteen tumors were located in a long bone of an extremity (nine close to the knee joint) and three in the central skeleton. Radiographically, all the tumors presented as a purely osteolytic lesion, and three patients had sustained a pathologic fracture. In four of six cases angiography suggested malignancy by revealing hypervascularity, irregular tortuous vessels, and diffuse contrast opacification. Contrast-enhanced computed tomography, performed in two cases, showed hypervascular areas within the tumors. Scintigraphy showed a marked increase in radionuclide uptake in all five cases studied. The clinical behavior indicates that primary leiomyosarcoma of bone is highly malignant. Eight patients had died of the tumor and, of the eight patients who were alive at follow-up, two had metastases, and one had been operated on three times for a cutaneous metastasis, which had recurred locally twice. The remaining five patients had been continuously free of disease for 6.5 to 12.3 years. Publication Types: Review Fetch PMID: 3306938

Breast

Medical Journal Article Annotated Citations Latest Pubmed/Medline Search for Leiomyosarcoma and breast primary Orv Hetil 1997 Jan 26;138(4):195-8 [Experience in the treatment of breast sarcomas].[Article in Hungarian] Farkas E, Orosz Z, Kovacs T, Koves I. Orszagos Onkologiai Intezet Budapest. Authors operated on 6339 malignant breast tumors at the Department of Surgery, National Institute on Oncology between 1980 and 1994. The records of 14 patients with sarcomas of the breast were analyzed. These included 2 malignant phyllodes tumors, 3 malignant fibrous histiocytomas, 2-2 fibrosarcomas and carcinosarcomas and liposarcoma, angiosarcoma, leiomyosarcoma, osteosarcoma and dermatofibrosarcoma protuberans one of each. During this period 5 patients died, 9 are living without evidence of tumor. Analysing these 14 cases authors present their policy in surgical therapy of breast sarcomas compared with literature data. They emphasise the importance of wide resection margins for prevention of tumor progression. The above-mentioned principle is valid for local recurrences so radicalization of the previous conservative breast surgery may be necessary in selected cases. Intraoperative histological examination is recommended to verify that the resection margins are tumor free. Axillary block dissection is not necessary except in cases when palpable, firm lymph nodes are present in the region. Depending on the histological grade adjuvant radiotherapy could be considered but its effectiveness is not proven. Fetch PMID: 9072752 Aust N Z J Surg 1997 Jan;67(1):71-2 Breast metastases from primary leiomyosarcoma. Tulasi NR, Kurian S, Mathew G, Viswanathan FR, Roul RK. Department of Oncology, GKNM Hospital, Coimbatore, India. Two cases of metastatic leiomyosarcoma of the breast are presented. The reasons why they are considered as secondary tumours and not primary tumours are also discussed. Fetch PMID: 9033385 Neoplasma 1992;39(6):375-9 Sarcomas of the breast: a multicenter series of 70 cases. Ciatto S, Bonardi R, Cataliotti L, Cardona G. Center for Study and Prevention of Oncological Diseases, Firenze, Italy. A multicenter retrospective series of 70 breast sarcomas (,,, leiomyosarcoma (1)) was reviewed. The average follow-up was 5.9 years. Diagnostic tests (palpation, mammography, sonography and cytology) were poorly sensitive, and a large proportion of cases, appearing as regular, sharp bordered, rounded masses were diagnosed as benign fibroadenomas. Surgery (limited (29), mastectomy (41)) was the treatment of choice. Axillary nodes were rarely involved (2 of 31) at pathologic staging. No significant predictors of local recurrences (12 cases) were observed although recurrences were more frequent in larger lesions (0-20 mm = 1.1%, 21-50 mm = 1.7%, > 50 mm = 6.1% women-year) and in cases treated with limited surgery (limited surgery 4.6%, mastectomy 2.0% women-year). Distant metastases (16 cases) were less frequent in malignant cystosarcoma phyllodes or liposarcoma patients, but no other significant predictors of distant metastases were evidenced. Five-year disease-free or overall survival was 50% or 66%, respectively. The study confirms that breast sarcomas are rare, difficult to diagnose, but can be cured by surgical treatment in a considerable proportion of cases. Multicenter study Fetch PMID: 1491728 Am J Clin Pathol 1989 Oct;92(4):500-5 Primary recurrent leiomyosarcoma of the breast. Case report with ultrastructural and immunohistochemical study and review of the literature. Arista-Nasr J, Gonzalez-Gomez I, Angeles-Angeles A, Illanes-Baz E, Brandt-Brandt H, Larriva-Sahd J. Department of Pathology, Instituto Nacional de la Nutricion, Mexico, D.F. .. As a group, they have better prognosis than other sarcomas of the breast, although the possibilities of recurrence or dissemination exist, even many years after the primary extirpation. The size of the tumor and mitotic activity seem to be of little prognostic value. Mammary leiomyosarcoma shares clinical and pathologic similarities with subcutaneous leiomyosarcoma in other anatomic sites. Review of reported cases Fetch PMID: 2679042 Cancer 1981 Apr 1;47(7):1883-6 Leiomyosarcoma of the breast: a case of long survival and late hepatic metastasis. Chen KT, Kuo TT, Hoffmann KD. A case of leiomyosarcoma of the breast that metastasized to the liver 15 years after mastectomy is reported. Leiomyosarcoma appears to have less aggressive biologic behavior than other types of breast sarcomas. Analysis of the reported cases of smooth muscle tumors of the breast revealed some guidelines for the histologic diagnosis of these tumors. Tumors with three or more mitoses per 10 HPF are leiomyosarcomas, and those with no mitotic activity, necrosis, and significant cellular atypia are leiomyomas. The borderline cases are best considered smooth muscle tumors with uncertain malignant potential. [A mitotic rate of one per 10 HPF might be just a very low grade LMS. Best to have slides reviewed by a sarcoma oncologist at a sarcoma center. Ed. ] Fetch PMID: 7194732 Arch Surg 1980 Mar;115(3):244-8 Estrogen receptor proteins in diverse human tumors. Stedman KE, Moore GE, Morgan RT. One hundred three diverse benign and malignant human tissues have been assayed for estrogen receptor proteins. Receptors were detected in many endocrine and nonendocrine tumors. Tissues with estrogen receptor activity included four of five male breast carcinomas, 11 of 14 malignant melanomas, four of eight colon carcinomas, five of seven renal carcinomas, and various sarcomas and benign and normal tissues. Some tumors also had progesterone, androgen, and/or glucocorticoid receptors. These results suggest the use of hormones and hormone antagonists for therapy of a broad range of human cancer. Clinicians of diverse expertise should be aware of, and responsive to, potential endocrinological involvement in many dissimilar disease states. [Uterine, breast, vulval, and ovarian tumors should be tested for estrogen receptors. Sometimes estrogen blockers can be used for adjuvant or supplemental treatment. Ed.] Fetch PMID: 7356378

Chest

Medical Journal Article Annotated Citations Latest Pubmed/Medline Search on Leiomyosarcoma with chest primary Eur J Cardiothorac Surg 1999 Apr;15(4):456-60 Surgical treatment of primary pulmonary sarcomas. Bacha EA, Wright CD, Grillo HC, Wain JC, Moncure A, Keel SB, Donahue DM, Mathisen DJ. Thoracic Surgical Unit, Massachusetts General Hospital, Boston 02114, USA. OBJECTIVE: We sought to identify the long-term prognosis after surgical treatment for primary pulmonary sarcoma. METHODS: Twenty-three patients were retrospectively identified as having been treated surgically for primary pulmonary sarcoma between 1981 and 1996. The records of all patients were reviewed, and the histopathology reexamined by a pathologist. RESULTS: Fifteen patients were male and eight female; their ages ranged from 20 to 78 (mean 51) years. Tumors measured between 0.9 and 12.0 (mean 5.2) cm across the greatest diameter. The histologic diagnoses were ,,, leiomyosarcoma (3) [of 23] ,,, Three patients were found to be unresectable. All three underwent radiation and chemotherapy. Lobectomies or bilobectomies were performed in 13 patients including two sleeve resections, one carinal resection, and one chest wall resection. Four patients underwent radical pneumonectomies. Three patients with invasion of the pulmonary artery, pulmonary veins or atrial wall underwent extended resections with the use of cardiopulmonary bypass. In two, a homograft was used to reconstruct the right ventricular outflow tract. Of the resected patients, six had a positive resection margin, and four had at least one positive lymph node in the specimen. Three patients underwent repeat pulmonary resections for recurrences. Eleven patients received postoperative chemotherapy and eight had radiation therapy. Follow-up was available on 22 patients, and ranged from 2 to 183 (mean 48) months; 14 patients are disease free, six died of disease, one died of surgical complications (operative mortality 5%), and two are alive with disease. Actuarial 3- and 5-year survival of the resected patients was 69%. Size and grade were not found to be correlated with significantly increased survival, but completeness of resection was (P<0.05). Resection of primary pulmonary sarcomas can produce an acceptable survival rate if the resection is complete. Cardiopulmonary bypass can be a useful adjunct when tumors involve a resectable area of the heart or great vessels. Fetch PMID: 10371121 Nippon Kyobu Geka Gakkai Zasshi 1993 Sep;41(9):1562-6 [Leiomyosarcoma of the chest wall]. [Article in Japanese] Togashi K, Yazawa M, Sato Y. Division of Thoracic and Cardiovascular Surgery, Nagaoka Red Cross Hospital, Niigata, Japan. An unusual case of a primary leiomyosarcoma of the chest wall is presented. The patient was a 41-year-old man and had no symptoms. The chest x-ray showed a tumor with mediastinal involvement. A percutaneous needle biopsy indicated that it was histologically a leiomyosarcoma. Wide en bloc excision of the chest wall was done, including much of the third, fourth and fifth ribs and a piece of lung. The defect was closed with GORE-TEX soft tissue patch. There were three courses of chemotherapy added, but the patient had a local recurrence, which was resected again, two years after the first surgery. It may be thought that wide en bloc excision and closely follow-up are important in the management of a leiomyosarcoma of the chest wall because of its high recurrence rate. Review of reported cases Fetch PMID: 8409615

Cutaneous

Cutaneous (Skin) & Subcutaneous (just under the Skin) Medical Journal Article Annotated Citations Latest Pubmed/Medline Search for Leiomyosarcoma with cutaneous primary Latest Pubmed/Medline Search for Leiomyosarcoma with subcutaneous primary Gastroenterol Clin Biol 1999 Aug-Sep;23(8-9):991-2 [Hepatic leiomyosarcoma revealed by cutaneous metastasis]. [Article in French] Pequignot R, Thevenot T, Permal S, Tulliez M, Legman P, Dhote R, Christoforov B. Publication Types:Letter [Again---the first tumor you find may actually be a metastasis from a hidden primary LMS. Ed.] Fetch PMID: 10533154 Dermatol Surg 1999 Jun;25(6):489-91 Subcutaneous leiomyosarcoma on the face. Lin JY, Tsai RY. Department of Dermatology, Chang Gung Memorial Hospital, Taipei, Taiwan. .. Cutaneous leiomyosarcoma is a rare soft tissue sarcoma with a predilection for the lower extremities. Leiomyosarcoma of the face is very rare. Subcutaneous leiomyosarcoma has a higher likelihood of recurrence and metastases than that of the superficial dermal type. ,,, Leiomyosarcoma of the face is exceedingly rare. The deep subcutaneous type is thought to arise from the smooth muscle of the vascular wall. The neoplasm we report here has deep tumor invasion, high malignancy grade (3B), and large tumor size. Wide excision and postoperative radiotherapy were performed. ,,, Subcutaneous leiomyosarcoma has a higher likelihood of recurrence and metastases than that of the superficial dermal type. The most effective treatment is wide excision with 3-5 cm lateral margins and a depth that includes subcutaneous tissue and fascia. Fetch PMID: 10469099 Ann Dermatol Venereol 1999 Mar;126(3):235-42 Comment in: Ann Dermatol Venereol. 2000 Apr;127(4):411 Ann Dermatol Venereol. 2000 Apr;127(4):412-3 [Primary cutaneous leiomyosarcoma: 32 cases]. [Article in French] Auroy S, Contesso G, Spatz A, Genin J, Margulis A, Lecesne A, Avril MF. Service de Dermatologie, Institut Gustave-Roussy, Villejuif. ,,, Superficial leiomyosarcomas are rare tumors, which may be confined to the dermis or extend to subcutaneous tissues. ,,, We report the results of a retrospective study of 32 patients treated for leiomyosarcomas through a twenty-two year period (from 1975 to 1997). ,,, Mean age was 45 years, with 50% of patients less than 35 years of age. Forty seven percent of the tumors were located on the lower limbs and mean diameter was 2.8 cm. Three clinical types have been isolated: nodule beneath normal epidermis (50%), purple nodule ulcerated or not (28%), swelling tumor (22%). Sixteen percent were intradermal, whereas sixty nine percent involved subcutaneous tissues. With regard to tumor grade, 37% of tumors were grade I, 44% of tumors were grade II, and 19% were grade III. Immunohistochemical staining showed positive reactions for all tumors with anti-vimentin and anti-alpha smooth muscle actin. Main treatment was complete surgical excision. Follow-up informations were available for all patients and 75% of them had a follow up period longer than a year. Five patients with leiomyosarcomas involving the subcutis developed local recurrences, and two of them died of the disease. ,,, Leiomyosarcomas can occur at any age without predominant sex-ratio. Main prognostic factors are tumor size, distal location, depth of tumor invasion and pathological grade. Immunohistological staining with anti-alpha smooth muscle actin is more sensitive and specific than with anti-desmin or anti-HHF 35. Main treatment is surgical excision with wide margins. Fetch PMID: 10394436 Verh Dtsch Ges Pathol 1998;82:112-20 [Cutaneous and subcutaneous soft tissue tumors]. [Article in German] Meister P. ,,, Basically all types of soft tissue tumors can also be found in subcutaneous and cutaneous localisation. ,,,. Superficial leiomyosarcoma and atypical fibroxanthoma are examples of sarcomas, which in spite of histological criteria, which generally are associated with high grade malignancy, behave as low grade malignant tumors, i.e. chiefly with risk of recurrency albeit negligible risk of metastases. ,,, In conclusion it is evident that exact tumor classification is the basic requirement for an optimal, prognosis-adapted therapy. ,,, Fetch PMID: 10095423 Am J Surg Pathol 1997 Sep;21(9):979-87 Cutaneous leiomyosarcoma. Kaddu S, Beham A, Cerroni L, Humer-Fuchs U, Salmhofer W, Kerl H, Soyer HP. Department of Dermatology, University of Graz, Austria. We report the clinical, histopathologic, immunohistologic, and prognostic findings in 19 patients with cutaneous leiomyosarcoma, eight males and 11 females (mean age, 66 years; age range, 41-93 years). The tumors presented mainly as solitary lesions and were located on the head and neck (eight lesions), trunk (four lesions), upper extremities (three lesions), and lower extremities (four lesions). Histopathologically, two predominant growth patterns were observed: nodular (12 cases) and diffuse (seven cases). ,,, Clinical follow-up revealed local recurrences in five patients (three cases with nodular pattern and two lesions with a diffuse pattern) after a period ranging from 8 months to 3 years after surgical excision. No distant metastases have been observed in our series. We conclude that cutaneous leiomyosarcoma with a diffuse growth pattern may constitute a pitfall in histopathologic diagnosis because of the presence of only subtle criteria for malignancy. ,,, Cutaneous leiomyosarcoma sometimes reveals local recurrences, but it has negligible potential for distant metastases. Fetch PMID: 9298873 Australas J Dermatol 1997 Feb;38(1):26-8 Primary leiomyosarcoma of the skin. Wargon O. Royal Prince Alfred Hospital, Sydney, New South Wales, Australia. Leiomyosarcomas of the skin are frequently misdiagnosed clinically. This tumour however, exhibits typical histological and immunohistochemical features. This collection of seven cases of cutaneous leiomyosarcoma on the trunk and limbs of adults with a mean age of 63 years and a range of 49-78 years demonstrates that the clinical diagnosis in each case, from seven different clinicians, did not include the correct diagnosis. In each case, the special immunohistochemical stains were helpful in making the diagnosis with actin and vimentin being positive and S100 and prekeratin stains being negative. Review of reported cases Fetch PMID: 9046649 Dermatol Surg 1996 Sep;22(9):761-8 Tumors with smooth muscle differentiation. Spencer JM, Amonette RA. Department of Dermatology and Cutaneous Surgery, University of Miami, FL 33101,USA. ,,,:This review article describes the clinical presentation and course, histology, and treatment recommendations for benign and malignant smooth muscle tumors. ,,, Benign tumors exhibiting differentiation towards smooth muscle include smooth muscle hamartoma and leiomyoma. ,,, Immunohistochemical stains are also helpful in establishing a diagnosis. With solitary tumors, treatment for cosmesis or for painful leiomyomas is not a problem. Multiple leiomyomas, which may be painful or sensitive to cold or touch, are a therapeutic challenge, with reports of multiple medications being attempted in the literature. Leiomyosarcoma are malignant tumors of smooth muscle. They may be cutaneous and presumably arise from the arrector pilorum muscle, or subcutaneous, where they are believed to arise from vascular smooth muscle. Cutaneous leiomyosarcomas frequently reoccur following excision, but rarely metastasize. Subcutaneous leiomyosarcomas frequently reoccur following excision and have a high metastatic rate. Several recent reports have documented an increased frequency of visceral leiomyosarcomas in immunosuppressed patients. These tumors have been found to contain the Epstein Barr virus. Treatment of leiomyosarcoma is wide excision.,,,. While leiomyomas are benign, their frequent sensitivity or pain necessitates treatment. Leiomyosarcomas are malignancies with a high reoccurrence rate, and when deep, a high metastatic rate. ... Fetch PMID: 8874523 Dermatol Surg 1996 Mar;22(3):268-73 Soft tissue sarcomas in dermatology. Fish FS. Department of Dermatology/Cutaneous Surgery, St. Paul RamseyMedicalCenter, MN55101,USA ,,, and cutaneous leiomyosarcoma are uncommon soft tissue sarcomas that tend to have a high recurrence rate when removed with standard excisional surgery, and in some cases have been reported to metastasize. ,,, Wide excision and surgical techniques involving meticulous surgical margin control appear to be the most efficacious treatment for these problematic tumors. ,,, Cutaneous leiomyosarcoma is a rare superficial soft tissue sarcoma that has a high incidence of recurrence after excision and can metastasize. ,,, Currently, wide local excision is recommended with a 3-5 cm margin and removal of the underlying subcutaneous tissue. Mohs micrographic surgery has been used in a small number of cases with good success. ,,, When removing these tumors it is very important to take adequate margins. Mohs micrographic surgery is proving to be very useful in treating these difficult neoplasms. Review, tutorial Fetch PMID: 8599739 An Med Interna 1993 Feb;10(2):98 [Cutaneous metastases as a form of presentation of a retroperitoneal leiomyosarcoma]. [Article in Spanish] Gironell Carrero A, Domingo Pedrol P, Costa Trachsel I. Publication Types: Letter Fetch PMID: 8452982 [Again---the first tumor you find may actually be a metastasis from a hidden primary LMS. Ed.] Cancer 1992 Jul 15;70(2):490-2 Recurrent cutaneous leiomyosarcoma. Wascher RA, Lee MY. Department of Surgery, Tripler Army Medical Center, Honolulu, HI 96859. ,,, Soft tissue sarcomas comprise approximately 0.7% of all malignant neoplasms. Superficial leiomyosarcoma, a rare malignant lesion, constitutes 4.0-6.5% of all soft tissue sarcomas, an overall incidence of approximately 0.04% among all cancers. Currently, less than 125 cumulative cases of cutaneous and subcutaneous leiomyosarcoma have been reported in the English literature,,,, The authors report the case of a 70-year-old Japanese man with recurrent cutaneous leiomyosarcoma who was treated by wide local excision. A comprehensive literature survey is also presented. ,,, The patient is free from recurrence 1 year after wide local excision of a second recurrence of cutaneous leiomyosarcoma. Moreover, superficial leiomyosarcoma can be subdivided into cutaneous leiomyosarcoma and subcutaneous leiomyosarcoma, based on histopathologic and prognostic differences. ,,, The treatment of choice of superficial leiomyosarcoma is wide local excision. Cutaneous leiomyosarcoma is associated with local recurrence only, although subcutaneous leiomyosarcoma undergoes metastatic spread in 30-60% of cases, with a 30-40% mortality rate. Fetch PMID: 1617597 Cancer 1981 Jan 1;47(1):156-69 Leiomyosarcoma of the skin and subcutaneous tissue. Fields JP, Helwig EB. In a study of 65 primary cutaneous leiomyosarcomas and 15 primary superficial subcutaneous leiomyosarcomas, tumors occurred at any age but were more common in middle age and most common on the extremities. They developed as solitary painful or tender intracutaneous or subcutaneous nodules. Microscopically, the cutaneous leiomyosarcomas consist of a poorly delineated proliferation of spindle-shaped atypical myomatous cells arranged in interlacing fascicles which merge into collagenous stroma. Subcutaneous leiomyosarcomas are more sharply circumscribed and typically include a vascular pattern. About 40% of the cutaneous leiomyosarcomas recurred, but none metastasized despite a high mitotic frequency and marked cytologic atypia. Among the 12 patients with subcutaneous leiomyosarcomas, one-half of the tumors recurred and one-third eventuated in metastasis or tumor-related death. Cutaneous leiomyosarcomas have a relatively benign biologic course and may be excised conservatively, but are less likely to recur if the local excision is wide enough to require a skin graft for closure of the surgical defect. For primary subcutaneous leiomyosarcoma, early wide local excision with adequate clear histologic borders constitutes rational treatment. Fetch PMID: 7459804 Pathol Eur 1974;9(4):307-15 Cutaneous and subcutaneous leiomyosarcoma. A clinicopathologic study of 47 patients. Dahl I, Angervall L. A retrospective study of 47 patients with leiomyosarcoma of superficial (skin and subcutaneous) soft tissue is presented. ,,, Forty patients had a solitary tumour which, in 19 patients, was situated entirely or almost entirely in the corium. There were two distinct growth patterns for the leiomyosarcomas. The cutaneous tumours were poorly delimited and the subcutaneous tumours more well-circumscribed or nodular. Some three quarters of the tumours were located in the extremities, the thigh and hip regions being the predilection sites; the highest frequency was noted in patients in their seventies; the ages ranged between 25 and 89 years. The sex ratio (male to female) was 2 to 1. In 37 patients follow-up information was available, the follow-up period ranging from 1 month to 16 1/2 years; the median time was 6 years. One or more local recurrences developed in 15 patients. Seven out of the 47 patients had multiple leiomyosarcomas; 4 of these patients had already been operated on for a retroperitoneal leiomyosarcoma. Fourteen patients in the whole series died with metastases especially in the lungs. Metastases were seen particularly in patients with subcutaneous and multiple leiomyosarcomas. Our study suggest that the size and the mitotic activity of the tumour appear to have some prognostic value. The initial surgical procedure was found to be the most important factor in influencing the outcome of the disease and it is stressed that leiomyosarcoma in superficial soft tissues should be treated by wide surgical excision. It is recommended that patients with multiple leiomyosarcomas in the superficial soft tissues should be subjected to further examination in order to exclude the possible occurrence of a retroperitoneal tumour. ,,, Fetch PMID: 4457785

External Genital

Medical Journal Article Annotated Citations Latest Pubmed/Medline Searches: Vulval LMS Scrotal LMS Testicular LMS Other site: replace the XXX with the site. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=search&db=PubMed&term=leiomyosarcoma%20XXX Arch Surg 1980 Mar;115(3):244-8 Estrogen receptor proteins in diverse human tumors. Stedman KE, Moore GE, Morgan RT. One hundred three diverse benign and malignant human tissues have been assayed for estrogen receptor proteins. Receptors were detected in many endocrine and nonendocrine tumors. Tissues with estrogen receptor activity included four of five male breast carcinomas, 11 of 14 malignant melanomas, four of eight colon carcinomas, five of seven renal carcinomas, and various sarcomas and benign and normal tissues. Some tumors also had progesterone, androgen, and/or glucocorticoid receptors. These results suggest the use of hormones and hormone antagonists for therapy of a broad range of human cancer. Clinicians of diverse expertise should be aware of, and responsive to, potential endocrinological involvement in many dissimilar disease states. [Uterine, breast, vulval, and ovarian tumors should be tested for estrogen receptors. Sometimes estrogen blockers can be used for adjuvant or supplemental treatment. Ed.] Fetch PMID: 7356378 Histopathology 1991 Jun;18(6):523-9 Erratum in: Histopathology 1991 Aug;19(2):198 Smooth muscle tumours of the external genitalia: clinicopathological analysis of a series. Newman PL, Fletcher CD. Department of Histopathology, St. Thomas's Hospital (UMDS) London, UK. Smooth muscle tumours of the external genitalia (vulva, scrotum and nipple) are uncommon and have received little attention in the literature. Given their purported close relationship to cutaneous (pilar) leiomyomatous lesions, we have studied 32 cases (18 vulva, 10 scrotum, four nipple) from 31 patients in order to compare clinicopathological features at the different sites. Only those in the nipple were comparable in size and histology to pilar leiomyomas. Vulval and scrotal tumours tended to be much larger and better circumscribed. Purely epithelioid lesions were only identified in the vulva (two cases) and at this site myxoid change and hyalinization were substantially more common. Almost all cases showed immunohistochemical evidence of muscle differentiation. On histological grounds, nine cases were classified as leiomyosarcoma (six scrotum, three vulva) but only one of the five with follow-up has led to the patient's death. With the exception of nipple lesions, which are particularly uncommon, we would not support the traditional inclusion of genital smooth muscle tumours under the general heading of cutaneous leiomyoma/sarcoma, as vulval and scrotal lesions are clinicopathologically quite different. Fetch PMID: 1879812 Histopathology 1991 Jun;18(6):523-9 Erratum in: Histopathology 1991 Aug;19(2):198 Smooth muscle tumours of the external genitalia: clinicopathological analysis of a series. Newman PL, Fletcher CD. Department of Histopathology, St. Thomas's Hospital (UMDS) London, UK. Smooth muscle tumours of the external genitalia (vulva, scrotum and nipple) are uncommon and have received little attention in the literature. Given their purported close relationship to cutaneous (pilar) leiomyomatous lesions, we have studied 32 cases (18 vulva, 10 scrotum, four nipple) from 31 patients in order to compare clinicopathological features at the different sites. Only those in the nipple were comparable in size and histology to pilar leiomyomas. Vulval and scrotal tumours tended to be much larger and better circumscribed. Purely epithelioid lesions were only identified in the vulva (two cases) and at this site myxoid change and hyalinization were substantially more common. ,,, On histological grounds, nine cases were classified as leiomyosarcoma (six scrotum, three vulva) but only one of the five with follow-up has led to the patient's death. With the exception of nipple lesions, which are particularly uncommon, we would not support the traditional inclusion of genital smooth muscle tumours under the general heading of cutaneous leiomyoma/sarcoma, as vulval and scrotal lesions are clinicopathologically quite different. Fetch PMID: 1879812 J Urol 1994 Jun;151(6):1643-5 Leiomyosarcoma of the penis. Pow-Sang MR, Orihuela E. Department of Surgery, University of Texas Medical Branch, Galveston 77550. The third case of leiomyosarcoma of the foreskin is reported. A review of the literature indicates that this lesion, which is similar to cutaneous leiomyosarcoma that is seen elsewhere in the body, has a low grade of malignancy. This finding is in contrast to leiomyosarcoma of the penis, which arises in deep structures and tends to metastasize. Circumcision appears to be the most effective treatment for leiomyosarcoma of the foreskin. Fetch PMID: 8189587 J Surg Oncol 1986 Mar;31(3):191-8 Neoplasms in skin and subcutis over the breast, simulating breast neoplasms: case reports and literature review. Ilie B. Twenty cases of neoplasms in skin and subcutaneous tissue over the breast were reviewed. There were 17 women, from 15 to 70 years of age, and three men, from 25 to 66 years of age. Among the benign skin neoplasms, superficial leiomyoma, granular cell tumor, and eccrine acrospiroma were misdiagnosed clinically as primary breast carcinoma. Among the malignant neoplasms in subcutaneous tissue, there were three metastatic malignant melanomas, one metastatic epidermoid bronchogenic carcinoma, and two malignant lymphomas. It is interesting that four of these six patients had no prior history of malignant lesion, the subcutaneous nodule presenting as the first manifestation of an occult primary. It is concluded that histological diagnosis of such tumors may lead to avoidance of unnecessary radical surgery. Fetch PMID: 3014220

Gastrointestinal

Medical Journal Article Annotated Citations NOTE: GISTs [GastroIntestinal Stromal Tumors] and GI LMS were often pooled and considered LMS. GISTs are very chemoresistant, and the survival statistics showed that. Since the development of Gleevec [Glivec] in 2000/2001, however, many GIST tumors are now managed and the disease is held stable. With the development of Gleevec, the statistics for survival for people with GIST are much better. With the removal of GISTs from the LMS tumor pool, it is clear that the statistics for survival for GI LMS are better as well. GIST and LMS are now considered two different tumors. Consequently, pre-2001 publications on survival statistics for GI LMS are seriously flawed. For Latest Pubmed/Medline Search on Leiomyosarcoma with gastrointestinal primary Am J Surg Pathol 2000 Oct;24(10):1339-52 Gastrointestinal stromal tumors and leiomyosarcomas in the colon: a clinicopathologic, immunohistochemical, and molecular genetic study of 44 cases. Miettinen M, Sarlomo-Rikala M, Sobin LH, Lasota J. Department of Soft Tissue Pathology, Armed Forces Institute of Pathology, Washington, DC 20306-6000, USA. Gastrointestinal stromal tumors (GISTs), mesenchymal tumors largely specific for the gastrointestinal tract, have been well defined in the stomach and small intestine, but have not been extensively documented or contrasted with true smooth muscle tumors in the colon. This study was undertaken to determine the clinicopathologic features of GISTs of the colon, excluding the rectum, and to compare them with leiomyosarcomas (LMSs) of the same location. A total of 37 colonic GISTs and seven LMSs from the files of the Armed Forces Institute of Pathology and the Haartman Institute of the University of Helsinki were analyzed. The GISTs occurred predominantly in adults older than 50 years of age (median, 67 yrs), and most were histologically malignant; four small benign tumors (< or = 1 cm) were incidentally detected, and 10 others had minimal mitotic activity (five or fewer mitoses per 50 high-power fields). The colonic GISTs were typically transmural tumors with frequent intraluminal and outward bulging components. Histologically, they usually showed a spindle cell pattern (92%), whereas 8% were epithelioid. Most tumors (19 of 25) were positive for CD117 (KIT) and for CD34 (16 of 27); six tumors coexpressed alpha-smooth muscle actin and CD117; none showed desmin or S-100 protein. C-kit mutations in exon 11 were seen in 5 (36%) of 14 colonic GISTs. None of the patients with incidental small tumors had a recurrence, whereas 2 of 10 patients with tumors larger than 1 cm but minimal mitotic activity died of the disease with liver metastasis. Nearly all patients whose tumor was larger than 1 cm and showed more than five mitoses per 50 high-power fields died of disease; half had evidence of metastasis. LMSs were typically intraluminally bulging, polypoid masses that showed a histologic likeness to differentiated smooth muscle cells. They occurred in five men and two women with a median age of 61 years. Most LMSs were high-grade histologically and showed smooth muscle actin, desmin, or both. All were negative for CD34 and CD117 and lacked c-kit mutations. Five of the seven patients died of disease, and two had a long-term survival, despite high mitotic activity. These results show that KIT-positive GISTs are more common than LMSs of the colon, and these tumor groups have clinicopathologic differences that warrant their separation. Fetch PMID: 11023095 J Clin Oncol 2000 Sep 15;18(18):3211-20 Soft tissue leiomyosarcomas and malignant gastrointestinal stromal tumors: differences in clinical outcome and expression of multidrug resistance proteins. Plaat BE, Hollema H, Molenaar WM, Torn Broers GH, Pijpe J, Mastik MF, Hoekstra HJ, van den Berg E, Scheper RJ, van der Graaf WT. Department of Pathology, University Hospital Groningen, The Netherlands. b.e.ch.plaat@kno.azg.nl ,,, Several studies have reported clinical behavior and chemotherapy resistance in leiomyosarcomas, but these studies did not differentiate between soft tissue leiomyosarcomas (LMS) and malignant gastrointestinal stromal tumors (GIST). Multidrug resistance (MDR) has been associated with the expression of P-glycoprotein (P-gp), multidrug resistance protein (MRP(1)), and lung resistance protein (LRP). The aim of the present study was to compare LMS and GIST with respect to clinical outcome and MDR parameters. ,,, Clinical outcome was evaluated in 29 patients with a primary deep-seated LMS and 26 patients with a primary malignant GIST. Paraffin-embedded material, available for 26 patients with LMS and 25 with GIST, was used for immunohistochemical detection of P-gp, MRP(1), LRP, and c-kit. ,,, Mean overall survival (OS) was 72 months for LMS patients and 31 months for GIST patients (P: <.05). Metastases occurred in 16 (59%) of 27 assessable LMS patients and in 10 (56%) of 18 assessable GIST patients. LMS predominantly metastasized to the lungs (14 of 16 patients), whereas GIST tended to spread to the liver (five of 10 patients) and the abdominal cavity (three of 10 patients; P: <.001). P-gp and MRP(1) expression was more pronounced in GIST than in LMS (P: <.05): the mean percentage of P-gp expressing cells was 13.4% in patients with LMS and 38.4% in patients with GIST, and the mean percentage MRP(1) expressing cells was 13.3% in patients with LMS and 35.4% in patients with GIST. LRP expression did not differ between LMS and GIST. c-kit was expressed in 5% of the LMS patients and in 68% of the GIST patients. ,,, LMS patients have a better survival than GIST patients, and the metastatic pattern is different. Expression of MDR proteins in LMS is less pronounced than in GIST. Fetch PMID: 10986053

Heart

Medical Journal Article Annotated Citations Latest Pubmed/Medline Search for Leiomyosarcoma with heart primary Can J Cardiol 2001 Mar;17(3):331-6 Myxoid leiomyosarcoma of the left atrium: a rare malignancy of the heart and its comparison with atrial myxoma. Morin JE, Rahal DP, Huttner I. Department of Cardiothoracic Surgery, McGill University Health Centre and Royal Victoria Hospital, Montreal, Canada. jean.morin@muhc.mcgill.ca Primary tumours of the heart are rare. The majority of these tumours are benign, with myxomas located in the left atrium being the most common form. Almost all malignant tumours are sarcomas and occur preferentially in the right side of the heart. An exception to this rule is leiomyosarcoma, a rare form of primary cardiac sarcoma that occurs predominantly in the left atrium, as does cardiac myxoma. The case of a 53-year-old woman who presented with symptoms of mitral valve stenosis and pulmonary hypertension is reported. Cardiac catheterization, angiography and echocardiography revealed a left atrial mass that was interpreted as atrial myxoma. ,,, The tumour, including a wide rim of atrial septum, was removed with cautery. Histopathological examination unexpectedly showed that the tumour was not an atrial myxoma but rather a myxoid variant of a primary leiomyosarcoma. Immunohistochemistry and electron microscopy confirmed the diagnosis. Local radiotherapy was considered but deemed contraindicated in view of the longstanding pulmonary hypertension. Two months after excision, a repeat echocardiogram indicated recurrence of tumour in the left atrium, and the patient died a few days later. The preferential left atrial location and the frequently myxoid appearance of primary leiomyosarcomas of the heart make it particularly difficult to differentiate them preoperatively from atrial myxomas. The authors recommend resection of all atrial myxoid tumours with a wide (at least 1 cm) margin, combined with intraoperative frozen section diagnosis, because complete surgical resection appears to correlate with prolonged survival in the few reported cases of atrial leiomyosarcomas. In cases of incomplete initial resection or local recurrence in the absence of metastatic disease, heart transplantation may be a valid option in appropriately selected patients. Fetch PMID: 11264566 Cardiologia 1999 Nov;44(11):1001-3 Left ventricular metastasis from uterine leiomyosarcoma. Cordioli E, Pizzi C, Bugiardini R. Dipartimento di Medicina Interna, Cardioangiologia, Epatologia, Universita degli Studi, Bologna. Cardiac metastases are uncommon but seem to be increasing in incidence, possibly in relation to prolonged survival of cancer patients. Leiomyosarcoma metastatic to the heart is extremely rare. We report the case of a 57-year-old woman previously treated for uterine leiomyosarcoma who presented with dyspnea, electrocardiographic changes mimicking myocardial infarction, and normal enzymes. A left intraventricular mass, suspected as cardiac metastasis, was revealed by echocardiography. The patient died 1 week later. At autopsy the mass proved to be histologically a metastasis of the uterine tumor. Fetch PMID: 10686777 Int J Cardiol 1997 Jun 27;60(1):91-4 Primary cardiac leiomyosarcoma: seven-year survival with combined surgical and adjuvant therapy. Pessotto R, Silvestre G, Luciani GB, Anselmi M, Pasini F, Santini F, Mazzucco A. Division of Cardiac Surgery, OCM B.go Trento, Verona, Italy. Primary cardiac sarcomas constitute a rare entity that has been uniformly associated with poor long-term survival. A case of left atrial leiomyosarcoma involving the interatrial septum and the right atrial free wall and presenting with syncope and atrial fibrillation, is described. Two extensive surgical excisions followed by adjuvant radiation and chemotherapy improved survival with a good quality of life. This approach of combined surgical, medical and radiation therapy may offer better longterm outcome, since our patient is the longest survivor thus far reported. Fetch PMID: 9209944 Cancer 1992 Jan 15;69(2):387-95 Primary sarcomas of the heart. Burke AP, Cowan D, Virmani R. Department of Cardiovascular Pathology, Armed Forces Institute of Pathology, Washington, D.C. 20306-6000. Seventy-five primary sarcomas of the heart were classified by histologic appearance as,,, leiomyosarcoma (4 cases,,, The ages of the patients ranged from 1 to 75 years at the time of presentation (mean, 39 years). Angiosarcomas were predominantly right-sided and osteosarcomas left-sided. Forty patients treated surgically were examined, and survival correlated with clinical and histologic parameters. the survival rate was poor, with a mean of 11 months and median of 6 months. By univariate analysis, the survival rate was more favorable for patients with tumors located on the left side of the heart, without necrosis, with a low mitotic count, and without metastasis at diagnosis. Survival rates were better in patients receiving chemotherapy and radiation therapy.[BUT this was a mixed group of sarcomas, not leiomyosarcoma alone. Ed.]Age, gender, presence of differentiation, and histologic type did not affect prognosis. By multivariate analysis, a low level of mitotic activity and any therapy were the only significant factors affecting survival rate,,, Fetch PMID: 1728367

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