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PubMed1994

Abnormalities of the p53 MDM2 and DCC genes in human leiomyosarcomas.

In this study we have screened a series of 29 primary leiomyosarcomas for abnormalities of both the p53 gene and the MDM2 gene, which encodes a p53-associated protein. SSCP (single-strand conformation polymorphism) analysis and direct sequencing of polymerase chain reaction (PCR)-amplified DNA were used to establish that 6/29 tumours possessed point mutations of the p53 gene. Using a monoclonal antibody that recognises the p53 protein in immunohistochemical staining experiments, we observed overexpression of the p53 protein in five of the six tumours containing point mutations in the p53 gene. Southern analysis of tumour DNA revealed that 2/29 tumours demonstrated amplification of the MDM2 gene. When considered together, these results indicate that alterations in both the p53 gene and MDM2 gene are important in the development of a significant minority of leiomyosarcomas. In addition, we have demonstrated a significant association between the presence of abnormalities of the p53 gene or MDM2 genes in leiomyosarcomas and a more advanced clinicopathological stage (P = 0.03). We have also examined the role of the DCC tumour-suppressor gene in the development of human soft-tissue tumours in a variety of histological types. Except for evidence of a rearrangement in a single leiomyosarcoma cell line, SK-UT-1, we have found no direct evidence to support a role for mutation of the gene in the development of human soft-tissue tumours.

Adult↗

PubMed2002

Low frequency of somatic mutations in the FH/multiple cutaneous leiomyomatosis gene in sporadic leiomyosarcomas and uterine leiomyomas.

Germline mutations in the fumarate hydratase gene at 1q43 predispose to dominantly inherited skin and uterine leiomyomata and leiomyosarcomas. The enzyme, which is a component of the tricarboxylic acid cycle, acts as a tumour suppressor. To evaluate fumarate hydratase in respective sporadic tumours, we analysed a series of 26 leiomyosarcomas and 129 uterine leiomyomas (from 21 patients) for somatic mutations in fumarate hydratase and allelic imbalance around 1q43. None of the 26 leiomyosarcomas harboured somatic mutations in fumarate hydratase. Fifty per cent of leiomysarcomas tested showed evidence of allelic imbalance at 1q, but this was not confined to the vicinity of fumarate hydratase. Only 5% (seven out of 129) of the leiomyomas showed allele imbalance at 1q42-q43 and no somatic mutations in fumarate hydratase were observed. Our findings indicate that mutations in fumarate hydratase do not play a major role in the development of sporadic leiomyosarcomas or uterine leiomyomas

Adult↗

PubMed2002

Leiomyosarcoma of the head and neck: a clinicopathological study.

AIMS: The behaviour of leiomyosarcoma is site-related and there are few data on such tumours located in the head and neck. We studied the clinicopathological features of these lesions. METHODS AND RESULTS: Cases diagnosed as leiomyosarcoma of the head and neck were retrieved from the archives of three institutions. Immunohistochemistry was performed and follow-up information was obtained. There were seven men and six women, aged 21-73 years, and lesions involved the neck (n=3), maxilla (n=4), buccal area (n=3), and maxillary sinus, nose, and pharynx (n=1 each). Tumours ranged from 10 to 80 mm. All tumours showed at least focally typical histological features of leiomyosarcoma with perpendicularly arranged fascicles of smooth muscle cells with blunt-ended nuclei, eosinophilic cytoplasm and paranuclear vacuoles. They expressed muscle-specific actin (8/9), smooth muscle actin (7/9), and desmin (10/12). Follow-up information was available on nine patients. All had surgery, with radiation and/or chemotherapy in seven. Three (27%) recurred at 6-24 months; in one case twice. Five (56%) had metastases, including the three with prior recurrences at 1-128 months. Five (including two who received adjuvant therapy) were disease-free at a median of 47 months, one was alive with metastatic disease at 24 months), and three were dead of disease (median 13 months). CONCLUSIONS: Head and neck leiomyosarcomas are rare and aggressive neoplasms which metastasize. Adjuvant therapy has limited effect.

Actins↗

PubMed2001

Evaluation of putative molecular biomarkers in abdominal and retroperitoneal leiomyosarcomas.

AIMS: Leiomyosarcomas (LMS) are diverse tumours with different biological behaviour. To evaluate the biological nature of intraabdominal and retroperitoneal leiomyosarcomas we retrospectively examined the immunoreactivity of p53, bcl-2 and proliferative activity expressed as Ki-67-labelling index in 43 tumours. METHODS: Immunohistochemical staining was performed using a peroxidase-streptavidin method on paraffin-embedded sections using specific anti- p53, anti- bcl-2 and anti Ki-67 monoclonal antibodies. RESULTS: Of 43 tumours, seven were located in the stomach, 11 in the small or large bowel, 12 in the uterus, 11 in the retroperitoneum and two cases in the urinary bladder. Five-year disease-free survival was 46.5%. Twenty-three patients (53.4%) died of the disease. Positive immunoreactivity for p53 and bcl-2 was found in 18 (41.9%) and 26 patients (60.5%), respectively. Positive Ki-67 staining was observed in eight patients (18.6%). Proliferative indices were higher in LMS with high mitotic activity (P=0.004) and high grade (P=0.009). All Ki-67 positive LMS were intermediate or high-grade tumours. Ki-67-labelling index showed inverse relationship to bcl-2 expression. A trend towards higher survival and expression of bcl-2, p53 or Ki-67 was found. CONCLUSIONS: Our results demonstrate that p53 and bcl-2 are expressed in a substantial number of intraabdominal and retroperitoneal leiomyosarcomas. In our study, the expression of these biomarkers did not predict patient outcome. Higher Ki-67 labelling indices were found in more biologically aggressive leiomyosarcomas.

Abdominal Neoplasms↗

PubMed2004

[Diagnostic imaging of vascular leiomyosarcomas].

Primary vascular leiomyosarcomas are very rare tumors, with the venous variety most often arising from the inferior caval vein and the arterial variety from the pulmonary artery. The tumors show either an exclusive intra- or extravascular pattern or a mixed growth pattern. The clinical symptoms depend on tumor location, with intraluminal tumors of the inferior caval vein causing edema or a Budd-Chiari syndrome. Leiomyosarcomas of the pulmonary artery can mimic chronic central or recurrent peripheral pulmonary embolism. Contrast enhanced spiral CT with multiplanar reconstruction is the diagnostic method of choice when a vascular leiomyosarcoma is suspected. MRI with MR-angiography can be added. If a tumor of undetermined origin shows a broad contact with a vessel and/or an intraluminal component, possible primary vascular leiomyosarcoma should be included in the differential diagnosis.

Budd-Chiari Syndrome↗

PubMedDate not supplied

[Primary leiomyosarcoma of the bone].

In a 52-year-old female patient and a 50-year-old patient with a malign tumor of the right distal femur (stage IIIb) respectively the left distal tibia (stage Ia according to Enneking), the extremely rare primary leiomyosarcoma of the bone was diagnosed. The mortality of the primary leiomyosarcoma of the bone is 50%, the mean survival time after diagnosis is 3.4 years. Diagnosis of a primary leiomyosarcoma of the bone is only possible after a secondary leiomyosarcoma has been excluded as well as by histopathological including immunohistochemical examination. The operative procedure is the therapy of choice. A neoadjuvant and/or adjuvant radio- or chemotherapy can be useful.

Angiography↗

PubMed2000

Desmoplastic leiomyosarcoma of the skin.

This report focuses on two unusual cases of cutaneous leiomyosarcoma composed of sparse numbers of neoplastic cells embedded in abundant sclerotic stroma throughout the entire neoplasm. To the best of our knowledge, only one example of this rare lesion has been described previously as "sclerotic primary cutaneous leiomyosarcoma." However, the resemblance of this tumor to other desmoplastic tumors of the skin is striking and, therefore, we propose the term desmoplastic leiomyosarcoma of the skin for this neoplasm. Because of the sparse cellularity and the abundant stroma, desmoplastic leiomyosarcoma of the skin can be easily misinterpreted, especially in small biopsies. It should be included in the differential diagnosis of inflammatory skin diseases associated with sclerosis, such as radiation dermatitis, and of desmoplastic tumors of the skin, including desmoplastic melanocytic nevus, desmoplastic melanoma, and desmoplastic squamous cell carcinoma.

Aged↗

PubMed1996

Death due to a leiomyosarcoma of the pulmonary artery.

Leiomyosarcomas of the pulmonary arteries are rare neoplasms seldom diagnosed during the patient's lifetime. We report a case of unexpected death in a 44-year-old man due to a leiomyosarcoma originating from the main pulmonary trunk and involving the right and left arteries as far as the lobar ramifications. The patient died after a 2-year history of bronchiectasis and chronic bronchopneumonia with weight loss and occasional episodes of syncope during the last 4 months. Immunohistochemical demonstration for alpha-smooth muscle actin was necessary to make a histological diagnosis of leiomyosarcoma. A review of pulmonary artery leiomyosarcomas is also presented.

Adult↗

PubMed1997

Primary leiomyosarcoma of bone: a clinicopathologic, immunohistochemical, and ultrastructural study of 33 patients and a literature review.

Leiomyosarcoma of bone is a rare tumor in an unusual location. Previous analysis of this entity mostly involved small numbers of cases with limited follow-up. Thirty-three patients with leiomyosarcoma of bone between 1977 and 1996 were studied, and the histologic appearance and grade were correlated with subsequent treatment and clinical behavior. To be included in this study the tumor had to be intraosseous, with other primary sites of origin clinically excluded. Also, most of the sarcomatous tissue (> or =70%) had to be of intramedullary location with only limited extraosseous extension. The patient's age at diagnosis ranged from 13 to 77 years (average 44.4). The gender distribution was equal. The long bones were preferentially affected (64%), with the lower extremity, around the knee joint, predominantly involved. Five patients (15%) developed postradiation leiomyosarcomas. The histologic analysis showed that the osseous leiomyosarcomas are most commonly of the classic type, followed by the epithelioid, myxoid, and pleomorphic variants. Immunoreactivity for smooth muscle markers (smooth muscle actin, common muscle actin, desmin) was positive in all tumors, and ultrastructural confirmation was obtained in 21% of cases. All sarcomas were histologically graded, which accurately reflected the subsequent prognosis. Seventy-five percent of the lesions were high-grade and the rest low-grade. The histologic grade of the tumors correlated with both the recurrence as well as the metastatic rates and together with the clinicopathologic stage of disease represented the cornerstone on which prudent therapy should be based.

Actins↗

PubMed2000

Myxoid leiomyosarcoma of soft tissue, an underrecognized variant.

Myxoid leiomyosarcoma is an uncommon tumor which, although previously well described in the uterus, is recognized to a lesser extent at other sites. We describe 18 cases of soft tissue leiomyosarcoma in which myxoid stroma occupied >50% of the tissue examined. Patients ranged in age from 22 to 84 years old (median, 57.5 yrs) and female patients outnumbered male patients 14 to 4. Tumor locations included the limbs (6 cases), female external genitalia (4 cases), head and neck region (3 cases), chest (2 cases), nipple, paratesticular soft tissue, and perineum (one case each). The tumors had a grossly gelatinous appearance and adopted three major histologic architectures: fascicular, reticular/microcystic, and "myxofibrosarcoma-like." The tumor cells were predominantly spindled in all cases with typical features of smooth muscle differentiation; there was a mixture of spindle and epithelioid cells in one case. No cases with pure epithelioid cytology were seen. All tumors displayed immunoreactivity for smooth muscle markers (smooth muscle actin 16/17, desmin 8/18) and, in addition, four cases were positive for keratin CAM 5.2 and three for epithelial membrane antigen. The tumors had a tendency to be morphologically lower grade (9 tumors were grade I, 8 were grade II, and only 1 was grade III). Follow up was available in 13 patients with a duration of 8 months to 41 years (median, 39 mos), and revealed local recurrences (often repeated) in five cases and metastases in two cases. There were three tumor-related deaths, of which two were the result of uncontrolled local disease. The differential diagnosis of myxoid leiomyosarcoma is broad and encompasses both benign and malignant lesions. Accurate diagnosis is critical because therapies may differ widely for entities in the differential diagnosis of myxoid leiomyosarcoma.

Adult↗

PubMed2004

Uterine epithelioid leiomyosarcomas with clear cells: reactivity with HMB-45 and the concept of PEComa.

In this study, we investigated HMB-45 expression in epithelioid uterine leiomyosarcomas with clear cell areas. From 12 epithelioid leiomyosarcomas, we selected 5 that had: 1) clear cell areas and 2) spindle cell areas that were at least focally positive for desmin and caldesmon. The patients' ages ranged from 47 to 82 years (mean 64 years). Presenting symptoms were uterine bleeding (three), abdominal pain (one), and a pelvic mass (one). There was no history of tuberous sclerosis or lymphangioleiomyomatosis. One patient had stage II disease, one stage III, and three stage IV. All were treated with total abdominal hysterectomy and bilateral salpingo-oophorectomy. Two received radiotherapy, and three were also treated with chemotherapy. The tumors ranged in size from 4 x 3 x 3 cm to 10 x 7 x 6 cm; all had significant cellular atypia, areas of coagulative necrosis, and between 10 and 90 mitoses per 10 high power fields. Vascular invasion was seen in three cases. The epithelioid component varied from 50% to 90% in each case; and the percentage of clear cells was < 1% in one case, 5% in one case, and 10% to 80% in three cases. Smooth muscle actin and desmin were positive in all cases. Four cases were positive for HMB-45 only in the clear cell areas. The tumor with < 1% of clear cells was negative for HMB-45. All were negative for S-100 and c-kit. Three patients died of disease at 9, 30, and 32 months; one patient is alive with progressive disease at 6 months, and one patient (stage II disease) is alive with no evidence of disease at 8 months. Unequivocal uterine epithelioid leiomyosarcomas may have clear cells positive for HMB-45. These tumors might belong to the group of lesion designated as PEComas; however, it is advisable to designate them as uterine leiomyosarcomas. In uterine smooth muscle tumors, some epithelioid cells most likely undergo clear cell changes and become positive for HMB-45. It would be advisable to perform this stain in all epithelioid smooth muscle tumors of the uterus.

Adult↗

PubMedDate not supplied

Gastrointestinal leiomyoma and leiomyosarcoma: CT differentiation.

PURPOSE: Our goal was to evaluate CT efficacy in differentiating gastrointestinal leiomyoma and leiomyosarcoma. METHOD: We retrospectively reviewed CT scans of 45 patients (21 men, 24 women, mean age 55 years) with surgically proven gastrointestinal leiomyomas (n = 21) and leiomyosarcomas (n = 24) with respect to size, contour, enhancing pattern, mesenteric fat infiltration, calcification, ulceration, regional lymphadenopathy, direct invasion, distant metastasis, and growth pattern after visual inspection by two radiologists in agreement. On the basis of these CT features, subjective diagnosis was also categorized into three groups (Group I: probably benign, Group II: probably malignant, Group III: diagnostic malignant). The results were compared with the final histopathologic diagnosis. RESULTS: In addition to the features of direct invasion and distant metastasis suggesting diagnostic malignancy, the CT features favoring malignancy with statistical significance included larger size (> 5 cm), lobulated contour, heterogeneous enhancement, mesenteric fat infiltration, ulceration, regional lymphadenopathy, and exophytic growth pattern (p < 0.005). However, calcification was not significant in differentiating the two entities (p = 0.25163). A subjective analysis revealed 89% sensitivity, 85% specificity, and 87% accuracy for diagnosis of leiomyosarcoma. CONCLUSION: CT features are useful in differentiating leiomyoma and leiomyosarcoma in gastrointestinal tract.

Adult↗

PubMed1991

Leiomyosarcoma of the inferior vena cava. Experience with 7 patients and literature review.

Leiomyosarcoma of the inferior vena cava (IVC) is a rare malignant tumor originating in the smooth muscle of the media. Although rare, it is the most common malignancy in the IVC. One hundred and six cases have been reported thus far in the world literature, usually as isolated case reports. Clinical, radiologic, and therapeutic management and follow-up, including 7 additional cases, have been reviewed and summarized. Clinical manifestations are dependent upon the location of the tumor. The main symptom was a palpable mass for a tumor in segment I, abdominal pain for segment II, the presence of Budd-Chiari syndrome for segment III. Segment II was the most frequent site of leiomyosarcoma of the IVC, alone (n = 41) or with other segments (n = 39). Before laparotomy, clinical recognition was difficult or impossible. Recently, however, newer imaging modalities including ultrasound and CT scan have permitted earlier diagnosis. Metastases, when diagnosed, were either present at diagnosis (n = 20) or appeared as the disease progressed (n = 18). Metastatic disease frequently involved the liver, lung, lymph nodes, or bone. The small number of patients alive without metastases (16/113) must be analyzed all the more carefully because these patients were followed for less than 2 years. When prolonged follow-up is possible, the number of patients alive without neoplastic disease is significantly reduced. We found the prognosis of patients with LMS of the IVC to be poor. Diagnosis was made at autopsy for 27 patients. Among the 86 patients with follow-up information, 59 died within a mean of 16 months, and 26 were alive 25 months after the diagnosis. The main prognostic factor is topography, particularly the highest level of extension of the tumor. The upper-segment tumors have the poorest prognosis. The best therapeutic management is difficult to recommend because most of the cases in the literature did not include a sufficient follow-up. Given the very small number of patients completely free of neoplastic disease after sufficient follow-up, it seems unlikely that leiomyosarcoma of the IVC can now be cured. Patients who received a combination of surgery, radiotherapy and chemotherapy remained free of disease for longer periods. The unanswered question is: what is the best timing for each of these treatments? We recommend diagnosis of leiomyosarcoma of the IVC through biopsy guided by ultrasonography or computed tomographic scan. Therapeutic management should include large doses of chemotherapy preoperatively with or without radiotherapy to reduce tumor size.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

PubMed1999

Primary dural leiomyosarcoma in a patient infected with human immunodeficiency virus: case report.

INTRODUCTION: We report the magnetic resonance imaging, angiographic, and immunohistochemical characteristics of a dural leiomyosarcoma in a patient infected with human immunodeficiency virus. METHODS AND RESULTS: A 38-year-old homosexual man presented with a recent history of headaches. Magnetic resonance imaging of the brain revealed an enhancing dural based right lateral wing mass that was thought to be a meningioma. The tumor had a signal intensity similar to the adjacent gray matter on T1-, T2-, and proton-weighted images. Angiography revealed that the tumor was vascular, supplied by the middle meningeal artery, but with contrast puddling as if there were small vascular lakes within the tumor. This evoked the possibility of a cavernous hemangioma. A craniotomy was performed, and the mass was resected. The pathological finding was consistent with a leiomyosarcoma. Immunohistochemistry revealed that the tumor was positive for alpha smooth muscle actin. Repeat testing for human immunodeficiency virus 2 months postoperatively was positive. Dural leiomyosarcomas are thought to take origin from the smooth muscles of the blood vessel walls. Another possible source is pluripotential mesenchymal cells. There may be an association with immunosuppression. CONCLUSION: Primary dural leiomyosarcomas simulate meningiomas on preoperative magnetic resonance images. They should be included in the differential diagnosis of dural based enhancing lesions.

Adult↗

PubMed1996

Successful treatment of a primary cardiac leiomyosarcoma with ifosfamide and etoposide.

PURPOSE: Primary cardiac leiomyosarcoma is a rare malignant tumor in childhood. Patients with unresectable or partially resected cardiac leiomyosarcoma typically have a poor prognosis. The role of chemotherapy in the treatment of these patients has not been well defined. PATIENT AND METHODS: A 6-week-old infant with an incompletely resected cardiac leiomyosarcoma was treated postoperatively with ifosfamide and etoposide. RESULTS: The patient is disease-free and without apparent late effects 5 years following the completion of therapy. CONCLUSION: The combination of ifosfamide and etoposide warrants further evaluation in patients with leiomyosarcoma.

Antineoplastic Combined Chemotherapy Protocols↗

PubMed2003

Leiomyosarcoma of urinary bladder following cyclophosphamide therapy: report of two cases.

Leiomyosarcoma of urinary bladder is rare, although it is the most common mesenchymal tumor in adults. We report two cases of this tumor following cyclophosphamide therapy. The first case is from a 53-year-old man with Wegener's granulomatosis treated for 6 years with cyclophosphamide. He presented with painless hematuria, and the initial biopsy of the bladder tumor revealed a malignant spindle cell neoplasm. A final diagnosis of leiomyosarcoma was made on radical cystoprostatectomy. The second example is from a 21-year-old man who had received cyclophosphamide in early infancy for a bilateral retinoblastoma. He also presented with painless hematuria, and a bladder tumor was resected transurethrally and diagnosed as leiomyosarcoma. He underwent partial cystectomy two months later. Cyclophosphamide, when used for a neoplastic or non-neoplastic condition, is associated with an increased risk of developing bladder cancer. The distribution of histologic subtypes differs from that seen in spontaneous bladder tumors. A review of the literature shows an increased proportion of squamous cell carcinomas and sarcomas, especially leiomyosarcomas in cyclophosphamide exposed patients. Acrolein, a cytotoxic metabolite of cyclophosphamide excreted in urine, is regarded as the most likely causative agent.

Adult↗

PubMed2006

Primary cutaneous diffuse leiomyosarcoma with desmoplasia.

A 65-year-old man presented with an indurated plaque over his left cheek and left neck. An initial punch biopsy of skin showing increased smooth muscle bundles was consistent with a diagnosis of smooth muscle hamartoma. A second incisional skin biopsy revealed a well-differentiated smooth muscle proliferation invading into the dermis and subcutaneous fat in a diffusely infiltrative pattern and with a desmoplastic component, suggesting a diagnosis of desmoplastic leiomyosarcoma. Resection of the tumour confirmed the presence of a cytologically low grade leiomyosarcoma with an insidious infiltrative growth pattern. This rare pattern of diffuse leiomyosarcoma is important to recognize as the histological features are subtle and may potentially constitute a pitfall in histological diagnosis in a small biopsy specimen. In addition, our case illustrates overlapping morphology between diffuse and desmoplastic types of leiomyosarcoma.

Aged↗

PubMed2000

A case of a primary ovarian leiomyosarcoma.

A primary ovarian leiomyosarcoma is extremely rare. Moreover, there is no established treatment modality other than surgery, and the prognosis is extremely poor. We report a case of a primary ovarian leiomyosarcoma. The patient was a gravida 4, para 2, 73-year-old female. She first presented with chief complaints of constipation and a pelvic mass. A physical examination revealed a solid tumor in the pelvic cavity. Which was about the size of an infant's head and had an irregular shape. The patient was suspected of having a subserosal myoma of the uterus or an ovarian tumor, and she was subjected to a laparotomy. A solid tumor about the size of an infant's head was found in the left uterine adnexa, and an intraoperative rapid pathological diagnosis of an ovarian leiomyosarcoma was made. A total abdominal hysterectomy, a bilateral salpingo-oophorectomy, a pelvic lymphadenectomy, and an omentectomy were then performed. The final pathological diagnosis confirmed a left primary ovarian leiomyosarcoma. After a series of discussions with the patient's family about her therapeutic plan, we decided to let the patient be followed-up without adjuvant therapy. Forty-two months after the surgery, the patient accumulated massive amounts of pleural effusion and ascites, with extensive organ metastasis. She received terminal care and soon died.

Aged↗