Primary leiomyosarcoma of bone

OBJECTIVE: This study describes radiographic and MR imaging features of primary leiomyosarcoma of bone. SUBJECTS AND METHODS: Twelve patients (five men and seven women, 39-79 years old) who were treated at two oncology centers for primary leiomyosarcoma of bone involving the femurs, tibia, ilium, and inferior pubic ramus were studied. None of the patients had preexisting disease or disease elsewhere at the time of diagnosis. Pathologic diagnosis was obtained in all patients. RESULTS: Radiographs of all patients showed a matrix that was exclusively osteolytic. In long bones (seven patients), the tumor had an average length of 11 cm (range, 7-17 cm) and revealed an elongated configuration. In the pelvis (five patients), the average length of the tumor was 10 cm (range, 4-15 cm). MR imaging confirmed an intramedullary lesion in all patients, with extension into the soft tissues in eight patients and no identifiable soft-tissue mass in the remaining four patients. Four of the five pelvic tumors had a prominent soft-tissue mass, whereas only four of the seven long bone lesions revealed a soft-tissue mass that was, in all instances, small. The tumor was hypointense on T1-weighted images and showed heterogeneous signal intensity on T2-weighted conventional and fast spin-echo sequences. We saw low signal intensity (short T2) in eight patients and homogeneous hyperintense signal intensity in one patient. In the remaining three patients, T2-weighted spin-echo sequences obtained with fat saturation showed high signal intensity (long T2) in the tumors. CONCLUSION: Primary leiomyosarcoma of bone is a rare tumor that on radiography reveals no matrix and on MR imaging reveals areas of T2 shortening in relation to fat on conventional and fast spin-echo sequences.     

Primary leiomyosarcoma of bone

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.     

Primary leiomyosarcoma of bone: report of 4 cases

Primary leiomyosarcoma of bone is a very rare tumor. Four cases are reported: 3 tumors were located in the femur and 1 in the talus. Clinical and imaging findings including radiographs, CT, and MRI are described. The final diagnosis was made by histopathological, immunohistochemical, and/or ultrastructural study after biopsy.     

Primary leiomyosarcoma of bone: a report on three patients

Primary leiomyosarcoma of bone is a rare aggressive tumor that occurs mainly in older people. It resembles other malignancies clinically and radiologically, and differential diagnosis is based on histopathology, including immunohistochemistry. If leiomyosarcoma is found in bone, the possibility that it is a metastasis from a distant tumor should be investigated. The treatment is surgical excision with wide margins. Here we present three patients who had primary leiomyosarcoma of radius, ilium, and femur, respectively, and who were treated surgically.     

Primary leiomyosarcoma of the pancreas

Primary leiomyosarcoma of the pancreas is a very rare malignant tumor, with only 12 cases reported. A case of primary pancreatic leiomyosarcoma in a 55 year-old female with diabetes mellitus is described. US and CT demonstrated tumor masses in the head and tail of the pancreas. By angiography, abundant tumor vessels corresponding to the pancreatic tumor masses were revealed in the pancreas. The patient underwent surgical resection of the tumor in the tail of the pancreas, and then, microscopically it was diagnosed as leiomyosarcoma. The tumor in the head of the pancreas was not resected on account of the presence of diabetes mellitus and the possibility of poor prognosis. Macroscopically, it seemed to be the same as the tumor in the pancreatic tail. By operative findings, there was invasion from the pancreatic head to the duodenum and there was nothing to justify suspicion of the extra-ductal growing type of primary leiomyosarcoma of the duodenum. There were no metastases noted at the time.     

Primary leiomyosarcoma of the patella

We report on a case of primary leiomyosarcoma of the patella in a 59-year-old woman. To our knowledge, this is the first report of this rare tumor in a bone where malignant lesions rarely occur. Received: 21 November 2000 Revision requested: 8 January 2001 Revision received: 2 April 2001 Accepted: 4 April 2001     

Primary leiomyosarcoma of the spine

We present a 39-year-old man with tumour of the eighth thoracic vertebra, causing compression of the spinal cord. The tumour proved to be a primary leiomyosarcoma (LMS) of bone, an uncommon neoplasm; to our knowledge this is the first report of primary LMS in the spine. The lesion was documented by plain radiography, myelography, CT, MRI and digital subtraction angiography. These investigations did help to focus on the differential diagnosis and demonstrated the extent of the bony lesion, the findings were nonspecific, and the correct diagnosis was established by pathological examination.     

Primary epithelioid leiomyosarcoma of the endothoracic fascia

The case we describe is, to our knowledge, the first primary epithelioid leiomyosarcoma of the endothoracic fascia reported in the literature. The endothoracic fascia is localized between the costal parietal pleura and the inner face of the thoracic wall and contains connective, adipose, smooth muscular tissues, lymphoid aggregates and vessels. Malignant tumors of the endothoracic fascia are difficult to identify because they quickly infiltrate the lung, the chest wall and the mediastinum. When discovered, these tumors are classified as primary soft tissue sarcomas of these sites. In the present case, early diagnosis of an asymptomatic lesion allowed us to establish its origin in the endothoracic fascia. The CT characteristics suggested a non-aggressive lesion, with clearly defined profiles and without signs of infiltration of the adjacent lung, fat and cartilages. Histological differential diagnosis and role of radiology in the management of the case are discussed. As radiological techniques spread and improve, we expect the diagnosis of endothoracic fascia primary tumors could become more frequent.     

Primary leiomyosarcoma of the liver MR findings

The magnetic resonance (MR) features of a 67-year-old woman with a surgically and pathologically proved primary leiomyosarcoma of the liver studied at 1.0 T, using T1- (TR/TE = 450/15), and T2-weighted (TR/TE = 2200/45 to 90) spin-echo (SE) images, are described. On T1-weighted SE images, the tumor was well defined, was slightly heterogeneous, and displayed hypointensity to the adjacent hepatic parenchyma, with an area displaying hyperintensity. On T2-weighted SE images, the tumor was encapsulated, was heterogeneous, and displayed marked hyperintensity.     

Primary leiomyosarcoma of the spinal dura mater

Summary The case of a 64-year-old woman with a leiomyosarcoma arising from the upper thoracic spinal dura mater is presented: the CT and magnetic resonance image features are reported.     

Computed tomography appearance of bone metastases of leiomyosarcoma

Bone lesions in leiomyosarcoma are uncommon. When encountered, they are usually a late manifestation of disease seen in the setting of widespread systemic metastases. We present five cases of metastatic leiomyosarcoma, including one in which a bone lesion predated detection of a primary gastric leiomyosarcoma and one in which bone metastases signaled disease recurrence. Computed tomography in 14 of 16 osseous leiomyosarcomas demonstrated a lytic, non-expansile pattern.     

肺原发性平滑肌肉瘤1例

肺原发性平滑肌肉瘤极其罕见,笔者现将手术病理证实1例报告如下. 女,40岁, 因咳嗽、吐痰、痰中带血丝20 d而入院.20 d前无诱因而出现刺激性咳嗽,吐黄痰,每次约50 ml,无臭味,痰中带血丝,伴胸闷、憋气,无发热、盗汗及胸痛.在当地用抗生素治疗症状稍减轻.     

Primary leiomyosarcoma of the greater omentum: CT findings

Three cases of primary leiomyosarcoma arising from the greater omentum are described. On CT the tumors were multilobulated, flat, and pancake-like, with enhancing solid and multicystic densities. The masses in all cases were located in the most anterior part of peritoneal cavity. These CT findings are useful in the preoperative diagnosis of omental leiomyosarcoma.     

A primary leiomyosarcoma of bone containing pseudoepithelial plexiform elements

A rare but distinctive variant of smooth muscle tumours that occurs almost exclusively in the uterus is characterised by the presence of plexiform tumourlets, which are composed of clumps and cords of tumour cells that form a discrete pseudoepithelial component. We report on a case of a primary leiomyosarcoma of the proximal humerus, which, in addition to characteristic histological and immunophenotypic features of leiomyosarcoma, contained plexiform tumourlets. Tumour cells in the plexiform component focally expressed muscle/smooth muscle actin, calponin and cytokeratin. Spindle-shaped and epithelioid smooth muscle tumour cells also expressed the above antigens. This is the first report of a plexiform smooth muscle tumour arising in bone. This case is remarkable, not only for being only the second reported case of a malignant plexiform smooth muscle tumour, but also for being one of very few examples of this type of tumour arising outside the uterus; it also is unique in having arisen in a male patient. This variant of primary leiomyosarcoma needs to be distinguished from other bone tumours containing epithelial elements, notably metastatic carcinoma.     

原发性肺动脉平滑肌肉瘤1例

患者女,50岁。因反复咳嗽,痰中带血1年,胸闷,气促,右胸痛5月,加重3月入院,患者一年前无明显诱因出现咳嗽,嗽痰,痰中带血,无低热盗汗,胸片示右肺感染,抗感染治疗后,咳嗽略缓解,于5月前开始出现胸闷、气短、右侧胸痛,以活动后明显,抗感染治疗无效。     

右肺平滑肌肉瘤1例

患者男,65岁.发现右肺肿物5月,间断性咳嗽、咯血、右胸痛2月入院.患者于5个月前体检时发现右肺阴影,未诊治.近2月出现右胸痛且深吸气时加重,伴咳嗽、咯血(以痰中带血为主),无发热;2年前曾患"右大腿纤维肉瘤",在其它医院行手术及局部放疗.入院后X线检查示:右下肺野可见11.2cm×8.3cm的团块状密度较均匀增高影,边缘较光整(图1).     

Primary leiomyosarcoma of the distal tibia: A case report

The authors present an unusual case of a leiomyosarcoma of the distal tibia. Leiomyosarcoma tumors typically originate from smooth muscle tissue. It is rare for it to derive from bone and even rarer to be found in a bone of the lower limb. Given this extreme rarity in addition to nonspecific findings on plain film radiographs and magnetic resonance imaging (MRI), biopsy was needed in this case. It was only through immunochemistry staining that a definitive diagnosis was made. As such, this case is an illustrative example of an aggressive, though rare, primary lesion of the bone which should be considered in the differential diagnosis of a lytic intramedullary lesion. This case also highlights the need for careful evaluation of imaging features suggesting a potentially aggressive lesion requiring appropriate work up in a timely fashion.     

Primary leiomyosarcoma of the pulmonary artery: a diagnostic dilemma

Primary leiomyosarcoma of the pulmonary artery is a rare malignancy arising from the multipotential mesenchymal cell of the intima of the pulmonary artery. Due to its rarity and nonspecific clinical symptoms, the correct diagnosis and proper management are often delayed. Furthermore, it is frequently misdiagnosed as pulmonary embolism, mediastinal mass, pulmonary stenosis and lung cancer. Therefore, it is important to consider primary leiomyosarcoma of the pulmonary artery a possibility when a persistent filling defect is present in the pulmonary artery and there is no response to optimal anticoagulation treatment. Radiologic findings such as a unilateral mass continuously filling the pulmonary artery, inhomogenous enhancement, vascular distension, extravascular invasion into adjacent structure or uptake in the area of tumor on the FDG-PET can be helpful when differentiating pulmonary artery sarcoma (PAS) from chronic thromboembolism.     

原发性下腔静脉平滑肌肉瘤1例

患者女,55岁。子宫肌瘤、子宫切除术后5年。33天前患者无明显诱因于夜间出现鲜红肉眼血尿,伴尿频、尿急,无尿痛、腰痛、发热等症状,潜血( ),蛋白≥3g/L,WBC 4~6个/Hp,抗感染治疗后血尿消失,但多次复查尿常规尿蛋白≥3g/L,肾功:Scr 151umol/L,Ccr 67.78ml/L,24h尿蛋白0·71~1.49g。腹部B超:下腔静脉中段腔内低回声灶,累及肾静脉水平以上分支,右肾静脉起始部受累,考虑栓子形成。CT平扫下腔静脉明显增粗,其内可见略低密度灶,CT值约为20~24HU,中心可见更低密度区,肝实质CT值降低,呈肝淤血改变。增强后病变呈不均匀强化,动脉期CT值约39…     

MRI appearance of a primary leiomyosarcoma of bone with epiphyseal involvement

Primary leiomyosarcoma of bone is a rarely reported tumor of elderly subjects. It usually shows an aggressive osteolytic pattern on plain radiographs and involves predominantly the metaphyses of long bones. We report a case of primary leiomyosarcoma of bone, which is atypical by its epiphyseal location, a non-aggressive pattern on plain radiographs and its MR imaging features.