Uterine endometrial stromal sarcoma located in uterine myometrium: MRI appearance

Two cases of uterine endometrial stromal sarcoma whose main mass was located in uterine myometrium are reported. They mimicked uterine leiomyoma with cystic degeneration or uterine leiomyosarcoma. Endometrial stromal sarcoma should be suggested in the differential diagnosis of mass lesion in uterine myometrium. Received: 9 March 1999; Revised: 2 September 1999; Accepted: 25 November 1999     

MRI appearance of mesenchymal tumors of the uterus

Purpose

Uterine leiomyomas are the most common uterine neoplasms. Statistically, a uterine mass with unusual imaging features is more likely to represent a leiomyoma than other uncommon uterine mesenchymal neoplasms such as leiomyosarcoma or endometrial stromal tumors. Several prior studies have attempted to identify objective imaging characteristics that differentiate these entities. The purpose of this study was to test these criteria on our patient population.

Methods and materials

This retrospective study was approved by the institutional Human Investigations Committee and was performed in compliance with HIPAA regulations. Four patients with uterine leiomyosarcoma, two with stromal tumors of uncertain malignant potential (STUMP), one with endometrial stromal sarcoma, and two with mixed endometrial stromal and smooth muscle tumors were included in the study. Seventeen additional control cases of leiomyomas were selected as controls. Cases were blindly evaluated by two experienced readers. Objective criteria included T1 and T2 signal characteristics, enhancement pattern, the presence of cystic changes, and ill defined margins. Subjective criteria included individual reader gestalt. All cases had pathologic correlation.

Results

None of the objective criteria were associated with the presence or absence of uterine mesenchymal neoplasm. Ill defined margins came closest to having statistical significance (p = 0.06). Reader gestalt was statistically associated with the presence of mesenchymal neoplasm for one of our readers (p = 0.02) but not for the other (p = 0.07).

Conclusion

We found poor accuracy for objective imaging criteria in distinguishing leiomyomas with atypical imaging features from more clinically significant uterine mesenchymal neoplasms.     

Vaginal metastasis from uterine leiomyosarcoma. Magnetic resonance imaging features with pathological correlation

Vaginal metastases, though more frequent than primary vaginal tumors, are rare. Except for isolated reports of metastatic disease arising from extragenital organs, the vagina is typically a site for metastatic disease from genitourinary tract sites, especially cervix, endometrium, and kidney. We present herein a case of a 68-year-old woman presenting with a vaginal metastasis as the first manifestation of a uterine leiomyosarcoma. The magnetic resonance imaging features are described and correlated with the pathological findings. To the best of our knowledge, this is the first case of vaginal metastasis from uterine leiomyosarcoma described in the literature.     

Osteogenic sarcoma with skeletal muscle metastases

Two cases of osteogenic sarcoma with skeletal muscle metastases are described. A 40-year-old woman presented with progressive swelling of both calves and a soft tissue back lump. She had been diagnosed with mandibular chondroblastic osteogenic sarcoma 6 years earlier. Radiographs showed calcified masses. MRI scans and bone scintigraphy revealed multiple soft tissue masses in both calves. Bone scintigraphy also showed uptake in the back lump, right thigh and left lung base. Biopsy confirmed metastatic chondroblastic osteogenic sarcoma, which initially responded well to chemotherapy. However, the metastatic disease subsequently progressed rapidly and she died 21 months after presentation. The second case concerns a 20-year-old man who presented with a pathologic fracture of the humerus, which was found to be due to osteoblastic osteogenic sarcoma. He developed cerebral metastases 17 months later, followed by metastases at other sites. Calcified masses were subsequently seen on radiographs of the abdomen and chest. CT scans confirmed the presence of densely calcified muscle metastases in the abdominal wall, erector spinae and gluteal muscles. The patient’s disease progressed rapidly and he died 30 months after presentation. Received: 16 November 1998 Revision requested: 28 December 1998 Revision received: 18 January 1999 Accepted: 18 January 1999     

Leiomyosarcoma in bone: Primary or secondary?

Our case demonstrates how metastases may be present and symptomatic long before they produce sufficient osseous changes to render the lesions radiologically detectable. A case of leiomyosarcoma arising in the uterus and initially presenting as a femoral lesion is presented. It required an intensive review of the case history and of the previous pathology in order to identify the primary site in the uterus. In the literature nine cases of metastatic leiomyosarcoma presenting initially in bone are reviewed. Ten additional cases from the literature showed bone metastases as the first site of recurrence. When this is assessed together with some 14 cases of primary leiomyosarcomas reported arising in bone, one is impressed that this is an uncommon tumour in bone either as a primary or secondary. In all patients in whom a leiomyosarcoma is histologically proven in bone, all primary sites should be specifically and categorically excluded before the lesion is accepted as an osseous primary. This should include special stains for myofibrils and electron microscopy if at all possible, in order to differentiate such tumours from other sarcomas such as fibrosarcoma. This applies especially to patients with uterine lesions that have been previously treated, even if they have been regarded as benign initially. All previous pathology and in particular hysterectomy specimens should be carefully reviewed. The need to specifically identify a leiomyosarcoma as a primary lies in the different modalities of treatment used: aggressive ablative surgery for primary lesions as opposed to radiation and chemotherapy for metastatic disease.     

Multiple Pulmonary Artery Pseudoaneurysms Secondary to Metastatic Uterine Leiomyosarcoma

Teaching point: The probable mechanism of pseudoaneurysm formation related to metastatic neoplasm is a tumor embolus penetrating and destroying the vessel wall.Pulmonary artery pseudoaneurysm (PAP) related to metastatic neoplasm is rare. We describe a unique case of multiple PAPs secondary to metastatic uterine leiomyosarcoma and demonstrate the serial chest computed tomography to support the theory that the tumor begins as a tumor embolus, followed by infiltration and breakdown of the vessel wall, leading to aneurysmal dilatation and invading the perivascular tissue.     

Primary leiomyosarcoma of the oral tongue: magnetic resonance and ultrasonography findings with histopathologic correlation

Leiomyosarcoma is a malignant smooth muscle tumor that frequently occurs in the gastrointestinal tract and female genital tract. It is aggressive and tends to recur and metastasize. Clinical behavior is unpredictable, mostly influenced by a proper surgical approach. Oral leiomyosarcoma, in particular of the tongue, is extremely rare and poorly documented in the radiology literature. Diagnostic assessment of oral leiomyosarcoma is often challenging, mostly founded on its peculiar immunohistological features. However, imaging evaluation is essential in staging and for preoperative planning. We illustrate the case of a 52-year-old woman with 2-months history of a painless growing mass on the left hemitongue, with magnetic resonance and ultrasonographic features correlated to histopathologic examination.     

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.     

Primary Renal Leiomyosarcoma Presenting with Subcutaneous and Osseous Metastases: Staging and Follow-Up with 18F-FDG PET/CT

A 60 year old woman who presented with multiple small subcutaneous nodules in the upper back and arms, was referred for an [18F] fluoro-2-deoxy-D-glucose (¹⁸F-FDG) positron emission tomography/computed tomography (PET/CT) after histological evaluation revealed metastatic leiomyosarcoma of unknown origin. The PET/CT showed multiple ¹⁸F-FDG-avid subcutaneous nodules, bone lesions, as well as a large left renal mass, which was biopsied to confirm a primary renal leiomyosarcoma arising from the renal parenchyma. A post therapy PET/CT showed overall progression of disease. The use of ¹⁸F-FDG PET/CT in the staging and evaluation of response to therapy of a renal leiomyosarcoma has not been previously described in the literature.     

Metastatic thymoma of the breast.

Breast metastasis from nonmammary malignant neoplasms is uncommon, and it accounts for approximately 2% of all breast tumors. Distant metastasis of thymoma is very rare, and especially to extrathorcic areas. We report a female who had a metastatic thymoma in her breast 20 years after undergoing resection for a non-invasive thymoma. She presented with a palpable mass in her left breast. Mammography and ultrasonogram showed a lobular mass at the anterior glandular portion. Histological examination after surgical excision revealed a metastatic thymoma.     

Whole-body MRI using a rolling table platform for the detection of bone metastases

The aim of this study was to compare the results of whole-body MRI using a recently developed rolling table platform with findings of nuclear scintigraphy in patients with bone metastases. Twenty-six patients with known or suspected bone metastases who had undergone radionuclide scintigraphy were examined by MRI. Patients were placed on a rolling table platform with integrated phased-array surface coils [BodySURF (system for unlimited field of view)] capable of pulling the patient through the isocenter of the magnet. Using a five-station approach three different image sets (T1-weighted gradient recalled echo, half-Fourier acquired single-shot turbo spin echo, and short tau inversion recovery) were collected in the coronal plane. In addition, the spine was imaged in the sagittal plane. The MRI findings were compared with the results obtained by scintigraphy. The whole-body MR examination lasting merely 40 min was feasible in all 26 patients. The MRI revealed excellent correlation with scintigraphy regarding metastatic lesions. A total of 60 regions with metastatic lesions were identified on bone scintigraphy. Fifty-three regions were detected on identical locations by MRI. The regions missed by MRI were located mainly in ribs and skull. The MRI could identify additional bone metastases in spine, pelvis, and femur. The MRI screening for bone metastases correlated well with bone scintigraphy. Use of the rolling table platform permits rapid imaging based on three different contrast mechanisms of the entire skeletal system.     

A rare case of metastatic small cell carcinoma of breast from mixed types of cervical carcinoma

Metastatic small cell carcinoma to the breast from the uterine cervix is an extremely rare case. We report a case of a 58-year-old postmenopausal woman who presented with postmenopausal bleeding. Further investigations confirmed the diagnosis of mixed types of squamous and small cell cervical carcinoma. She underwent chemoradiotherapy and brachytherapy. A few months later, she presented with a palpable left breast lump three months after the diagnosis. Diagnosis of metastatic small cell carcinoma of the breast was confirmed by biopsy. Due to its rarity, we report on this case along with its relevant literature review.     

Huge uterine leiomyoma with adenomyotic cysts mimicking uterine sarcoma on MR imaging

Magnetic resonance imaging of a 39-year-old woman who presented with an abdominal mass revealed a tumor with hemorrhagic lesions extending from the intrauterine space to the subserosa. Hysterectomy was performed for probable uterine sarcoma. The histological examination diagnosed uterine leiomyoma with severe myxoid degeneration and without malignant components. Hemorrhagic lesions were diagnosed as adenomyotic cysts, resulting in findings similar to those of a uterine sarcoma.     

雌、孕激素受体蛋白在子宫肉瘤中的表达及其相关性研究

目的检测雌激素受体ERα、ERβ（estrogen receptorα、estrogen receptorβ）和孕激素受体PRA、PRB（progesteronereceptor A、progesterone receptor B）在子宫肉瘤中的表达及其相关性。方法选取子宫肉瘤患者66例,采用石蜡病理切片,用免疫组织化学染色法检测雌、孕激素受体蛋白的表达情况并分析其相关性。结果 3种肉瘤组分别与正常子宫肌层对照组ERα蛋白阳性率比较均有统计学差异（均P〈0.01）;子宫内膜间质肉瘤组分别与子宫平滑肌肉瘤组、子宫癌肉瘤组PRA蛋白、PRB蛋白阳性率比较均有统计学差异（均P〈0.05）。结论雌激素受体ERα、ERβ和孕激素受体PRA、PRB在3种肉瘤组织和正常子宫肌层组织中均有表达。在子宫内膜间质肉瘤组织中ERα与PRA、PRB表达有相关关系且均呈正相关。     

Uterus-like mass: MRI appearance of a very rare entity

We describe a 37-year-old patient who presented with right lower quadrant pain and intermenstrual bleeding. MRI demonstrated a 5 x 5 cm lobulated mass centered in the right uterine wall interpreted as suspicious for malignancy. A total hysterectomy was performed, and the gross and histologic features were consistent with the diagnosis of a uterus-like mass. Uterus-like mass is a benign entity that can be found in a variety of organs, and is characterized by endometrium surrounded by smooth muscle. It is an extremely rare lesion with only approximately 15 cases reported in the current literature. There is a lack of imaging literature on this entity, which is primarily described in the pathology literature. Its histogenesis is uncertain, but is theorized to be metaplastic change, congenital anomaly, and/or heterotopia. However, given the MRI appearance in this case, we feel that uterus-like mass could be prospectively diagnosed or listed in a differential diagnosis.     

Uterine leiomyomas: histopathologic features, MR imaging findings, differential diagnosis, and treatment.

Leiomyomas are the most common uterine neoplasm and are composed of smooth muscle with varying amounts of fibrous connective tissue. As leiomyomas enlarge, they may outgrow their blood supply, resulting in various types of degeneration: hyaline or myxoid degeneration, calcification, cystic degeneration, and red degeneration. Leiomyomas are classified as submucosal, intramural, or subserosal; the latter may become pedunculated and simulate ovarian neoplasms. Although most leiomyomas are asymptomatic, patients may present with abnormal uterine bleeding, pressure on adjacent organs, pain, infertility, or a palpable abdominalpelvic mass. Magnetic resonance (MR) imaging is the most accurate imaging technique for detection and localization of leiomyomas. On T2-weighted images, nondegenerated leiomyomas appear as well-circumscribed masses of decreased signal intensity; however, cellular leiomyomas can have relatively higher signal intensity on T2-weighted images and demonstrate enhancement on contrast material-enhanced images. Degenerated leiomyomas have variable appearances on T2-weighted images and contrast-enhanced images. The differential diagnosis of leiomyomas includes adenomyosis, solid adnexal mass, focal myometrial contraction, and uterine leiomyosarcoma. For patients with symptoms, medical or surgical treatment may be indicated. MR imaging also has a role in treatment of leiomyomas by assisting in surgical planning and monitoring the response to medical therapy.     

Posterior mediastinal epithelioid leiomyosarcoma: Case report and literature review

Epithelioid type leiomyosarcoma is rarely encountered outside of the abdomen or uterus. We present a case of posterior mediastinal leiomyosarcoma in a 45-year-old male with back pain and bilateral lower extremity weakness. Magnetic Resonance Imaging of the thoracic spine revealed a heterogeneous posterior mediastinal soft tissue mass infiltrating the vertebral body and epidural space with resultant spinal cord compression and edema. Positron Emission Tomography showed no evidence of distant metastatic spread. Histopathological characterization revealed epithelial type leiomyosarcoma. Despite multiple subtotal resections, radiotherapy, and salvage chemotherapy with successful restoration of the patient''s neurological function, the tumor burden remained significant. The patient was subsequently lost to follow up and the clinical outcome remains unknown. To our knowledge this is the first reported case of epithelioid type posterior mediastinal leiomyosarcoma presenting with spinal cord compression and edema.     

Rectal malignant stromal tumor (leiomyosarcoma): echoendoscopic, computed tomographic aspects and anatomopathologic correlations]

We report the CT and endorectal us features of a malignant stromal tumor of the rectum (leiomyosarcoma). On CT scan, rectal leiomyosarcoma appeared as a large soft tissue mass, arising from the anterior rectal wall with exorectal extension. On endorectal US, the rectal leiomyosarcoma presented as a well-defined hypoechogenic and heterogeneous mass arising from the rectal muscularis propria. The uncommon location of leiomyosarcoma to the rectum may simulate other rectal tumors such as adenocarcinoma on CT. As suggested by this report, endorectal us demonstrated the muscular origin of the tumor.     

Primary lung carcinoma metastatic to a solitary fibrous tumor

We report the case of a 78-year-old man with a 2 month history of newly diagnosed metastatic lung adenocarcinoma, who presented with a left gluteal soft tissue mass. Histological examination of the mass revealed a solitary fibrous tumor containing metastases from adenocarcinoma.     

C—myc和P~(53)与子宫平滑肌肿瘤的关系

应用地高辛精标记C—myc和P~(53)基因探针原位杂交技术检测18例子宫肌瘤,48例交界性子宫平滑肌瘤和6例子宫平滑肌肉瘤中C—myc和P~(53)的定位及表达,探讨肿瘤基因与子宫平滑肌肿瘤的发生和发展有无相关性,以及交界性子宫平滑肌瘤与子宫肌瘤和平滑肌肉瘤间的内在联系。结果显示C—myc和P~(53)的表达产物均定位于细胞核中,在三型肿瘤中C—myc的表达率分别为5.6％、33.3％、50％;P~(53)的表达率分别为11.1％、39.8％、66.7％(P<0.05)。在肿瘤中的表达强度与细胞增殖程度成正比。推测C—myc和P~(53)突变及过量表达可能是子宫平滑肌肿瘤发生和发展的一个因素,交界性子宫平滑肌瘤可能为平滑肌肉瘤的前驱病变,具有特殊的生物学行为,经手术治疗,预后良好。