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Postauricular subcutaneous leiomyosarcoma.

Superficial leiomyosarcomas are rare soft tissue tumors, most frequently involving the lower extremity. Leiomyosarcomas which could be misdiagnosis or delayed are very rare in the head and neck region. In this report, a 46-year-old man with postauricular leiomyosarcoma was presented. The tumor was totally excised with 3 cm margins. Immunohistochemically, the specimen showed reactivity for smooth muscle actin, vimentin, and desmin. No complications developed in the postoperative period and the patient was free of recurrence at the 26 months follow-up. Immunohistopathological examination is essential for an accurate histological diagnosis. The ENT surgeon should pay attention to superficial leiomyosarcoma with its specific behavior and treatment while evaluating the lesions in the head and neck region.

Ear, External↗

Foci of decreased signal on T2-weighted MR images in leiomyosarcomas of soft tissue: correlation between MR and histological findings.

OBJECTIVE: Leiomyosarcomas are rare soft tissue sarcomas with varying MR signal characteristics and histologic pictures. The purpose of this study was to investigate the histological features of foci, which showed decreased signal on T2-weighted images in leiomyosarcomas of soft tissue. MATERIAL AND METHODS: We reviewed the MR images of six histologically proved cases of leiomyosarcomas of soft tissue and correlated the foci, which showed decreased signal on T2-weighted images with the histologic findings. RESULT: Microscopic examination revealed that these foci were composed of hyalinization of neoplastic tissue, internal septations, deposition of hemosiderin, or corresponded to metaplastic bone. CONCLUSION: The authors explain that the foci of decreased signal on T2-weighted MR images correspond to tissue components of the lesion, particularly fibrous tissue, hemosiderin and metaplastic bone. So, the suggestion is that leiomyosarcoma should be considered in the differential diagnosis of soft-tissue tumors that show foci of decreased signal on T2-weighted MR images.

Aged↗

Leiomyosarcoma of scrotum--case report and review of literature.

Leiomyosarcoma of the scrotum is a rare tumor. Cutaneous and subcutaneous leiomyosarcomas constitute the two subtypes. We report a case of cutaneous leiomyosarcoma of the scrotum in a 73-year-old man. Cutaneous leiomyosarcoma arises from the smooth muscle of the dartos or arrectores pilorum. It is often mistaken for a benign lesion. We describe the clinical and pathologic features and review the published reports of this uncommon malignancy. It is best treated by wide local excision. Inguinal lymph node dissection is not advocated, unless a high degree of suspicion is present for lymph node metastasis. Long-term follow-up is essential, because of the risk of delayed local recurrence and distant metastasis.

Aged↗

Leiomyosarcoma of the cervix.

BACKGROUND: Epithelioid leiomyosarcoma arising from the uterine cervix is extremely rare, with only three cases reported in the world literature. We present the case report of a 14-cm epithelioid leiomyosarcoma arising from the uterine cervix. CASE: A 47-year-old female presented with a 1-year history of worsening menorrhagia and was found to have a large pelvic mass extending into and filling the upper vagina. She underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy, revealing a 14 x 10 x 9 cm cervical epithelioid leiomyosarcoma. CONCLUSION: Although exceedingly rare, leiomyosarcoma is able to arise primarily from the uterine cervix. Given its extreme rarity, management of cervical LMS must be extrapolated from the currently accepted management for uterine LMS.

Chemotherapy, Adjuvant↗

Epithelioid leiomyosarcoma of the ovary.

INTRODUCTION: Leiomyosarcoma of the ovary is a rare lesion usually seen in postmenopausal women. Epithelioid leiomyosarcoma of the ovary is exceptionally rare, with only one previously reported case. CASE: A 42-year-old nulliparous woman undergoing ovulation induction presented with a left adnexal mass. An exploratory laparotomy was performed. Frozen section diagnosis was read as fibrothecoma, and an oophorectomy was performed. The final diagnosis was epithelioid leiomyosarcoma of the ovary. Subsequently, a staging procedure including hysterectomy was performed with no residual disease. DISCUSSION: This is an unusual case for several reasons. Most leiomyosarcomas of the ovary have occurred in postmenopausal women and were of advanced stage at diagnosis. Our case had an epithelioid histology, occurred in a premenopausal woman, and was confined to the ovary. The fact that the patient was undergoing ovulation induction at the time of diagnosis raises the question of the risk of developing ovarian neoplasms with these medications.

Adult↗

Array comparative genomic hybridization analysis of uterine leiomyosarcoma.

PURPOSE: Using a genome-wide array-based comparative genomic hybridization (array-CGH), DNA copy number changes in uterine leiomyosarcoma were analyzed. MATERIALS AND METHODS: We analyzed 4 cases of uterine leiomyoma and 7 cases of uterine leiomyosarcoma. The paraffin-fixed tissue samples were microdissected under microscope and DNA was extracted. Array-based CGH and fluorescence in situ hybridization (FISH) were carried out with Genome database (Gene Ontology). RESULTS: Uterine leiomyoma showed no genetic alterations, while all of 7 cases of uterine leiomyosarcoma showed specific gains and losses. The percentage of average gains and losses were 4.86% and 15.1%, respectively. The regions of high level of gain were 7q36.3, 7q33-q35, 12q13-12q15, and 12q23.3. And the regions of homozygous loss were 1p21.1, 2p22.2, 6p11.2, 9p21.1, 9p21.3, 9p22.1, 14q32.33, and 14q32.33 qter. There were no recurrent regions of gain, but recurrent regions of loss were 1p21.1-p21.2, 1p22.3-p31.1, 9p21.2-p22.2, 10q25-q25.2, 11q24.2-q25, 13q12-q12.13, 14q31.1-q31.3, 14q32.32-q32.33, 15q11-q12, 15q13-q14, 18q12.1-q12.2, 18q22.1-q22.3, 20p12.1, and 21q22.12-q22.13. In the high level of gain regions, BAC clones encoded HMGIC, SAS, MDM2, TIM1 genes. Frequently gained BAC clone-encoded genes were TIM1, PDGFR-beta, REC Q4, VAV2, FGF4, KLK2, PNUTL1, GDNF, FLG, EXT1, WISP1, HER-2, and SOX18. The genes encoded by frequently lost BAC clones were LEU1, ERCC5, THBS1, DCC, MBD2, SCCA1, FVT1, CYB5, and ETS2/E2. A subset of cellular processes from each gene was clustered by Gene Ontology database. CONCLUSION: Using array-CGH, chromosomal aberrations related to uterine leiomyosarcoma were identified. The high resolution of array-CGH combined with human genome database would give a chance to find out possible target genes present in the gained or lost clones.

Adult↗

Leiomyosarcoma of the penis.

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.

Adult↗

Uterine leiomyosarcomas and benign smooth muscle tumors: usefulness of nuclear DNA patterns studied by flow cytometry.

Flow cytometry was used to determine the DNA ploidy pattern of paraffin-embedded archival tissue specimens from 90 surgically resected uterine smooth muscle tumors (49 leiomyosarcomas and 41 leiomyomas). The technique of Hedley was used for preparation of paraffin-embedded tissue into single dissociated nuclei, and the method of Vindeløv was used for staining with propidium iodide. Among the 41 leiomyomas, most tumors (88%) had a DNA diploid pattern; the exceptions were two DNA tetraploid/polyploid and three DNA aneuploid samples. The DNA histograms of the 49 leiomyosarcomas (including 6 epithelioid leiomyosarcomas) were classified as follows: 9 cases (18%) exhibited a DNA diploid pattern, 29 cases (59%) had a DNA tetraploid/polyploid pattern, and 11 cases (23%) had DNA aneuploid peaks. Although DNA ploidy pattern cannot be used diagnostically to distinguish malignant from benign uterine smooth muscle tumors, the nuclear DNA ploidy pattern is an easily measured, objective determination that may have important prognostic significance for patients with uterine leiomyosarcomas.

Adult↗

Aborted leiomyosarcoma after treatment with leuprolide acetate.

BACKGROUND: Leuprolide acetate has been used to decrease uterine size and shrink leiomyomata. In carefully selected patients, its treatment benefits are well recognized. However, if leuprolide acetate is inadvertently given to a patient with an unsuspected leiomyosarcoma, complications may occur. CASE: A patient presumed to have leiomyomata was treated with monthly injections of leuprolide acetate. In the third month of treatment, unusual manifestations, including increased bleeding, aborting mass, urinary retention, and severe pain, occurred suggesting a possible malignancy and requiring immediate operation. CONCLUSION: The use of leuprolide acetate can delay the diagnosis and treatment of leiomyosarcoma and thus may increase the risk of morbidity and affect the treatment outcome of patients with leiomyosarcoma. The histologic changes ascribed to leuprolide acetate treatment in leiomyomata also were seen in this leiomyosarcoma.

Antineoplastic Agents, Hormonal↗

Leiomyosarcoma of the breast: a pathologic and comparative genomic hybridization study of two cases.

Leiomyosarcoma is an extremely rare form of primary breast sarcoma. We present the pathologic and genetic findings of two cases of leiomyosarcoma of the breast. The patients were 44 and 52 years of age and they presented with circumscribed masses of 3.0 and 4.5 cm, (greatest dimension) respectively. Microscopically, the two tumors showed diffuse proliferation of spindle cells with oval and blunt-ended nuclei arranged in short fascicles or bundles. There was moderate cytologic atypia in both cases, and 6 and 12 mitotic figures per 10 high power fields, respectively. No epithelial component was identified. The tumor cells were strongly immunoreactive for markers of smooth-muscle differentiation, including desmin, muscle-specific actin, and smooth-muscle actin. Comparative genomic hybridization analysis showed losses of 10q (two of two cases), 13q (two of two cases), 17p (one of two cases), and gains of 1q (one of two cases) and 17p (one of two cases). The patterns of chromosomal imbalances identified in leiomyosarcoma of the breast are similar to those reported in leiomyosarcoma of soft tissue and uterus and are different from those reported for leiomyoma, indicating that these alterations may be important for development of malignant smooth-muscle tumors regardless of site or organ of origin.

Adult↗

Primary leiomyosarcoma with the invasion into the intertrabecular space of bone: a case report and the review of the literatures.

Primary leiomyosarcoma of bone is very rare. Most of reported cases were osteolytic. We report a primary leiomyosarcoma arising from the neck of the femur of a 43-year-old woman who had no remarkable abnormalities on plain radiographs. Magnetic resonance imaging (MRI) clearly depicted the lesion. Biopsy revealed it leiomyosarcoma. Gynecological and gastrointestinal examinations ruled out metastatic leiomyosarcoma. Wide excision of the tumor was performed and followed by endoprosthetic replacement of the proximal femur. Microscopic examination revealed tumor cells infiltrating into the intertrabecular space.

Adult↗

Pedunculated exogastric leiomyosarcoma: case report and brief literature review.

A case report and review of the literature on pedunculated exogastric leiomyosarcomas are presented. Although about one-fourth of the stromal tumors (common leiomyomas and leiomyosarcomas) of the stomach grow in an exogastric configuration, pedunculated exogastric leiomyosarcomas are extremely rare. At present there is no evidence of intraperitoneal seeding from exogastric leiomyosarcomas. Consequently, a local resection with an adequate margin is sufficient when no invasion to the adjacent structures, is observed.

Diagnosis, Differential↗

Primary leiomyosarcoma of the mandible in a 7-year-old girl: report of a case and review of the literature.

Leiomyosarcoma is a malignant neoplasm of smooth muscle origin that manifests itself uncommonly in the oral cavity because of the paucity of smooth muscle in that location. To the best of our knowledge, only 10 cases of leiomyosarcoma primary to the jawbones have been reported in the English language literature. We report the first pediatric case of leiomyosarcoma arising from the mandible. Facial asymmetry and swelling were accompanied by a rapidly growing exophytic soft tissue mass that caused buccal displacement of the mandibular left permanent first molar. The lesion, observed radiographically as an extensive ill-defined area of osteolytic alveolar destruction, perforated the lingual cortex, displaced the inferior alveolar nerve canal inferiorly, and produced a "floating-in-air" appearance of the first molar. Diagnosis of leiomyosarcoma was made after initial incisional biopsy of the lesion. A 5-cm segmental mandibulectomy and supraomohyoid neck dissection were followed by reconstruction with a dynamic mandibular reconstruction plate and placement of a multidimensional mandibular distraction device in a transport rectangle of bone to promote bifocal distraction osteogenesis. Forty millimeters of distraction (the technical limit of the device) were performed; this was followed by terminal iliac crest bone grafting. Seventeen months after the definitive surgical procedure, the patient remains free of disease.

Actins↗

Complete hepatic resection of metastases from leiomyosarcoma prolongs survival.

Although liver resection has been shown to prolong survival in selected patients with metastases from colorectal cancer, the benefit for other metastatic tumors is unproved. To determine whether hepatic resection has a role in the management of metastatic leiomyosarcoma, medical records from 11 consecutive patients who underwent resection of isolated metastases from leiomyosarcoma between 1984 and 1995 were reviewed. All liver resections were for leiomyosarcomas originating in the viscera (n = 6) or retroperitoneum (n = 5). The average disease-free interval was 16 months. Five of 11 primary tumors were classified as low grade, whereas six were high grade. Hepatic resections included lobectomy or extended lobectomy (n = 4), segmentectomy and/or wedge resection (n = 5), and complex resection (n = 2). There were no operative deaths. Median survival of all patients after liver resection was 39 months. Patients who underwent complete resection of hepatic metastases (n = 6) had a significantly longer survival than those who had incomplete resections (n = 5) (P = 0.03, log-rank test). Furthermore, five of six patients who underwent complete resection are alive after hepatectomy with a median follow-up of 53 months. Therefore, in selected patients with isolated liver metastases from visceral and retroperitoneal leiomyosarcomas, complete resection of hepatic metastases results in prolonged survival.

Adult↗

[Leiomyosarcoma of the paranasal sinuses with intracranial involvement: report of a clinical case and review of the literature].

We describe a 53 year old man with a two months history of frontal headache and right visual loss, with complete right blindness at the moment of diagnosis. Neither nasal obstruction nor endocrine symptoms were found. Computerized Tomography showed a neoplasm involving both paranasal sinuses and sellar region, with bilateral orbital extension. The patient underwent a bifrontal craniotomy with apparent complete excision. Histopathologic examination proved smooth muscle cells sarcoma (leiomyosarcoma). Clinical evolution was unfavorable, with rapid local recurrence. The patient died of cerebellar metastasis 4 months after the initial diagnosis and treatment. Leiomyosarcoma of the sinonasal tract is an unusual tumor, and we have found only 63 cases previously reported. The most frequent clinical presentation is nasal obstruction. Surgery is the treatment of choice, as far as radiotherapy or chemotherapy do not appear to slow disease progression. No relationship has been found between the aggressiveness of leiomyosarcoma of the sinonasal tract and morphologic parameters; instead, prognosis is dependent on the distribution of disease at clinical onset. Leiomyosarcoma of the sinonasal tract may be regarded as a locally aggressive neoplasm with only limited metastatic potential.

Brain Neoplasms↗

Oral leiomyosarcoma: review of the literature and report of two cases with assessment of the prognostic and diagnostic significance of immunohistochemical and molecular markers.

Leiomyosarcoma of the oral cavity is a very rare tumor that is associated with aggressive clinical behavior and low survival. In this paper, we report two new cases of leiomyosarcoma affecting the mandibular gingiva and mandible of a 35-year-old male and the mandible of a 51-year-old female. Given the difficulty in the histopathologic discrimination between benign and malignant smooth muscle tumors and the absence of reliable histologic parameters for prognostication of leiomyosarcomas, we evaluated the diagnostic and prognostic value of various immunohistochemical and molecular markers. By means of immunohistochemistry and quantitative real-time PCR analysis, we detected protein expression of PCNA, bcl-2, CDK4, p53 and MDM2 in both our cases and MDM2 amplification in our second case. The literature, pertinent to oral leiomyosarcoma and to molecular analysis of smooth muscle tumors, is reviewed.

Adult↗

Leiomyosarcoma of the auricle: case report and literature review.

Leiomyosarcoma is a malignant tumour of smooth muscle origin. These tumours are rarely encountered in the head and neck region. A case of leiomyosarcoma of the auricle is presented. There are two cases of external auditory canal leiomyosarcoma in the literature. To our knowledge the present case is the first example of leiomyosarcoma of the auricle.

Aged↗

Intracranial leiomyosarcoma: a neuro-oncological consequence of acquired immunodeficiency syndrome.

Leiomyosarcoma has recently been identified as an AIDS-related tumor, usually occurring in children in the chest or abdomen. We describe a case of intracranial leiomyosarcoma in an adult with AIDS. An enhancing mass adjacent to the right occipital cortical surface was diagnosed during a work-up for left arm weakness. Imaging characteristics were suggestive of a malignant neoplasm versus meningioma. The patient had a craniotomy for total excision of the lesion. Pathology showed a leiomyosarcoma. Metastatic work-up was negative. The patient refused radiation therapy, but is well without evidence of recurrence at 8 months follow-up. The differential diagnosis for intracranial lesions in AIDS should be expanded to include leiomyosarcoma. Surgical resection of the lesion is recommended.

Acquired Immunodeficiency Syndrome↗