A rare case aggressive of uterine leiomyosarcoma: a case report.

Uterine leiomyosarcoma is a rare aggressive uterine malignancy that arises from a smooth muscle of the uterus which accounts for 2-5% of all uterine malignancies. Definitive treatment is surgery with a high rate of recurrences. Our patient presented with lower abdominal pain and mass per abdomen which was diagnosed to be uterine leiomyosarcoma. A 56-year-old woman of East Indian origin presented with abdominal pain and a huge rapidly growing suprapubic abdominal mass with an almost monthly doubling. Her CA 125 and Lactate dehydrogenase (LDH) level was elevated and Computed Tomography (CT) scan showed a large irregular-shaped abdominopelvic solid heterogeneously enhanced lesion with focal central hyperdensity and areas of necrosis causing mass effect. A primary cytoreductive surgery was performed and the histopathology report confirmed the diagnosis of uterine leiomyosarcoma. A combination chemotherapy of six cycles was given to prevent recurrence. No recurrence was detected during her more than two years follow-up period. As the cases are rare in nature, screening is impractical. Hence, the diagnosis of uterine leiomyosarcoma is done by histopathologic examination after surgery.

Primary ovarian leiomyosarcoma: a case report.

Primary ovarian sarcoma is a rare malignancy, with primary ovarian leiomyosarcoma being even rarer because of the lack of smooth muscle in the ovaries. We herein report a case of primary ovarian leiomyosarcoma in a woman in her late 50s who presented with a 6-month history of abdominal pain. Imaging revealed a pelvic mass. The patient underwent surgery and was diagnosed with ovarian leiomyosarcoma. One month postoperatively, she began gemcitabine and docetaxel chemotherapy and continued this treatment for 6 months. Eight months postoperatively, however, recurrence was detected in the pelvic cavity. This case is reported with the aim of raising awareness about this rare disease.

Primary osseous leiomyosarcoma of humerus misinterpreted as aneurysmal bone cyst: A case report and literature review.

RATIONALE: Primary leiomyosarcoma of the bone (LMSB) is a rare aggressive sarcoma with limited treatment options. Histopathologic and immunohistochemical features are similar to their more common uterine and soft tissue counterparts. However, its broader spectrum of histopathologic features and rarity make diagnostic challenges. PATIENT CONCERNS: We present a case of LMSB in a 20-year-old female who presented with left shoulder aching pain for 3 months. An osteolytic intramedullary lesion was found in the left proximal humeral epi-metaphysis. DIAGNOSES: Initial open biopsy showed a giant cell tumor of bone with aneurysmal bone cyst (ABC)-like changes. However, an open biopsy followed by extended curettage showed LMSB with ABC-like changes. INTERVENTIONS: Wide excision of the lesion and bipolar hemiarthroplasty followed by concomitant chemoradiation therapy was conducted. The mass was completely removed without significant problems. OUTCOMES: Complete mass excision and symptomatic improvements were achieved, and no subsequent relapses were observed. LESSONS: The authors encountered a rare case of LMSB. Most occurrences are in the lower extremity and trunk, respectively. ABC-like changes in bone tumors can lead to misdiagnosis. In this case, the ABC-like changes developed from the underlying LMSB as a secondary alteration. A careful examination of the underlying bone tumor is crucial to avoid misdiagnosing it as ABC or exhibiting ABC-like changes. Moreover, there has been no case report of LMSB with secondary ABC-like changes in bone.

[Rare case of leiomyosarcoma of the urinary bladder in a young man]. / Seltener Fall eines Leiomyosarkoms der Harnblase bei einem jungen Mann.

Leiomyosarcomas are rare, highly aggressive tumors of the urinary bladder. With approximately 200 cases reported in the literature, there is limited data on the prognosis and treatment of these neoplasms. Curative treatment approaches are primarily characterized by radical surgery, especially radical cystectomy. However, this procedure is associated with significant impairments in the quality of life for patients. This circumstance forms the basis for considering a curative treatment approach with partial cystectomy for a 19-year-old man with leiomyosarcoma of the urinary bladder.

Molecular genetics and research progress of uterine leiomyosarcoma.

Uterine leiomyosarcoma (uLMS) is a type of malignant soft-tissue tumor, which is developed from myometrium in the female reproductive system. This disease is difficult to be distinguished from benign uterine leiomyoma in the early stages, but it progresses aggressively and relentlessly. Hence, uLMS has a dismal prognosis and high rates of both misdiagnosis and missed diagnosis. Unfortunately, current studies of uLMS pathogenesis and disease biology are inadequate. uLMS disease models are also very limited, hindering the development of effective therapeutics. In this review, we focus on the pathological molecular biology of uLMS, and systematically review the molecular genetic features, epigenetic variants, experimental models, and clinical research progress of uLMS. We further discuss the development direction and potential needs of uLMS in the fields of tumor evolution, tumor microenvironment, and tumor therapy, with the aim of providing a better understanding of the pathobiological mechanism of uLMS and providing a reference for the development of potential diagnostic and therapeutic strategies.

Canine primary ureteral leiomyosarcoma treated with unilateral left ureteronephrectomy.

Background: Primary ureteral neoplasms are extremely rare in dogs, and ureteral involvement usually occurs owing to the invasion of renal and bladder tumors. Case Description: This case report describes a 12-year-old intact male mixed-breed dog referred to a private clinic with a six-month history of abdominal distention. A physical examination revealed mild abdominal pain. Hematological tests detected normocytic-normochromic anemia (hematocrit 33.6% [reference interval-RI: 37%-55%], red blood cells 4.93 M/µl [RI: 5.5-8.5 M/µl], and hemoglobin 12.4 g/dl [RI: 12-18.0 g/dl]). The results from the leukogram, thrombogram, renal, and hepatic panels were within the reference intervals for dogs. Abdominal ultrasonography revealed a cavitary mass measuring approximately 12 cm in diameter as the largest tumor in the left abdominal region over the left hepatic lobe or mesenteric site. Chest radiography did not reveal any metastasis. Therefore, the patient underwent exploratory laparotomy, during which the left ureter was found to be affected by a 12-cm mass that adhered to the left kidney. A unilateral left ureteronephrectomy was performed, and histology and immunohistochemistry (IHC) confirmed well-differentiated primary ureteral leiomyosarcoma. The patient survived for 130 days but died of lung metastasis. Conclusion: Ureteral leiomyosarcoma should be investigated and included in the list of differential diagnoses for primary ureteral neoplasms. Regardless of the therapeutic modality, the prognosis of ureteral leiomyosarcoma may be unfavorable, as shown in this report.

POTENTIAL DIAGNOSTIC BIOMARKERS FOR HUMAN MESENCHYMAL TUMORS, ESPECIALLY LMP2/Β1I AND CYCLIN E1/MIB1 DIFFERENTIAL EXPRESSION: PRUM-IBIO STUDY.

Most mesenchymal tumors found in the uterine corpus are benign tumors; however, uterine leiomyosarcoma is a malignant tumor with unknown risk factors that repeatedly recurs and metastasizes. In some cases, the histopathologic findings of uterine leiomyoma and uterine leiomyosarcoma are similar and surgical pathological diagnosis using excised tissue samples is difficult. It is necessary to analyze the risk factors for human uterine leiomyosarcoma and establish diagnostic biomarkers and treatments. Female mice deficient in the proteasome subunit low molecular mass peptide 2 (LMP2)/ß1i develop uterine leiomyosarcoma spontaneously. MATERIAL AND METHODS: Out of 334 patients with suspected uterine mesenchymal tumors, patients diagnosed with smooth muscle tumors of the uterus were selected from the pathological file. To investigate the expression status of biomarker candidate factors, immunohistochemical staining was performed with antibodies of biomarker candidate factors on thin-cut slides of human uterine leiomyosarcoma, uterine leiomyoma, and other uterine mesenchymal tumors. RESULTS AND DISCUSSION: In human uterine leiomyosarcoma, there was a loss of LMP2/ß1i expression and enhanced cyclin E1 and Ki-67/MIB1 expression. In human uterine leiomyomas and normal uterine smooth muscle layers, enhanced LMP2/ß1i expression and the disappearance of the expression of E1 and Ki-67/MIB1 were noted. The pattern of expression of each factor in other uterine mesenchymal tumors was different from that of uterine leiomyosarcoma. CONCLUSIONS: LMP2/ß1i, cyclin E1, and Ki-67/MIB1 may be candidate factors for biomarkers of human uterine leiomyosarcoma. Further large-cohort clinical trials should be conducted to establish treatments and diagnostics for uterine mesenchymal tumors.

Cutaneous leiomyosarcoma in a case of hereditary leiomyomatosis and renal cell carcinoma syndrome.

Hereditary leiomyomatosis and renal cell carcinoma (HLRCC) syndrome is an autosomal-dominant disorder that results from a germline pathogenic variant in the fumarate hydratase (FH) gene on chromosome 1, characterised by renal cell carcinoma (RCC), cutaneous leiomyoma and uterine leiomyoma. Leiomyosarcomas are reported in less than 1% of those with HLRCC. We report a case of a man in his 30s who had a long-standing plaque excised from the left upper arm after undergoing a radical nephrectomy for a fumarate-deficient RCC, with histological exam revealing a grade 1 leiomyosarcoma. Genetic testing confirmed a heterozygous pathogenic variant in the FH gene. This is a rare case of leiomyosarcoma associated with HLRCC, and our patient remains under surveillance with interval abdominal imaging and skin examination. Leiomyosarcomas are difficult to distinguish clinically from their benign counterpart; therefore, histopathological examination is paramount with a low threshold for excision.

Primary leiomyosarcoma of epididymis: a case report.

BACKGROUND: Leiomyosarcoma is a tumor that can develop in any organ that contains smooth muscles. Although leiomyosarcoma is common, its epididymal localization is quite rare. CASE PRESENTATION: A 79-year-old male Chinese Han patient presented with mild pain in the right groin and scrotum for 3 years concomitant with right scrotal swelling. Ultrasonography and magnetic resonance imaging of the scrotum showed a irregular and heterogeneous mass that was extratesticular. Right high orchiectomy was performed, and pathological examination of the resected specimen confirmed the diagnosis of leiomyosarcoma of the epididymis with surgical margins clear of tumor. CONCLUSION: Epididymal leiomyosarcoma is rare and difficult to diagnose preoperatively. The final diagnosis of epididymal leiomyosarcoma requires histologic examination. Resection must be extensive and complete. The effect of chemotherapy and radiation on the epididymal leiomyosarcoma remains unclear. Recurrence is common, so follow-up is necessary.

Leiomyosarcoma of the bone: Unveiling the mystery of a spindly ossein.

ABSTRACT: Leiomyosarcoma (LMS) represents one of the most common soft tissue sarcomas, involving various anatomical sites like the retroperitoneum, genitourinary tract, and extremities. LMS of the bone is extremely rare, with a 0.7% incidence of all primary malignant bone tumors. They are histologically identical to the leiomyosarcomas of other sites but pose a diagnostic dilemma due to their rarity and varied presentation when it manifests as a bony lesion.

Comprehensive Review of Uterine Leiomyosarcoma: Pathogenesis, Diagnosis, Prognosis, and Targeted Therapy.

Uterine leiomyosarcoma (uLMS) is the most common subtype of uterine sarcomas. They have a poor prognosis with high rates of recurrence and metastasis. The five-year survival for uLMS patients is between 25 and 76%, with survival rates approaching 10-15% for patients with metastatic disease at the initial diagnosis. Accumulating evidence suggests that several biological pathways are involved in uLMS pathogenesis. Notably, drugs that block abnormal functions of these pathways remarkably improve survival in uLMS patients. However, due to chemotherapy resistance, there remains a need for novel drugs that can target these pathways effectively. In this review article, we provide an overview of the recent progress in ascertaining the biological functions and regulatory mechanisms in uLMS from the perspective of aberrant biological pathways, including DNA repair, immune checkpoint blockade, protein kinase and intracellular signaling pathways, and the hedgehog pathway. We review the emerging role of epigenetics and epitranscriptome in the pathogenesis of uLMS. In addition, we discuss serum markers, artificial intelligence (AI) combined with machine learning, shear wave elastography, current management and medical treatment options, and ongoing clinical trials for patients with uLMS. Comprehensive, integrated, and deeper insights into the pathobiology and underlying molecular mechanisms of uLMS will help develop novel strategies to treat patients with this aggressive tumor.

Potential Markers to Differentiate Uterine Leiomyosarcomas from Leiomyomas.

Uterine leiomyomas (ULM) are the most common benign tumors of the female genitalia, while uterine leiomyosarcomas (ULMS) are rare. The sarcoma is diffuse growth, prone to hematogenous metastasis, and has a poor prognosis. Due to their similar clinical symptoms and morphological features, it is sometimes difficult to distinguish them, and the final diagnosis depends on histological diagnosis. Misdiagnosis of ULM as ULMS will lead to more invasive and extensive surgery when it is not needed, while misdiagnosis of ULMS as ULM may lead to delayed treatment and poor prognosis. This review searched and studied the published articles on ULM and ULMS, and summarized the potential markers for the differential diagnosis of ULMS. These markers will facilitate differential diagnosis and personalized treatment, providing timely diagnosis and potentially better prognosis for patients.

Primary left ventricular leiomyosarcoma: a case report.

Cardiac leiomyosarcomas are a rare subset of the already infrequent, primary malignant cardiac neoplasia spectrum. The most common site for a primary leiomyosarcoma of the ventricle is on the right with fewer than five globally reported cases in the left ventricle. Most present with non-specific symptoms but attention is usually sought after the appearance of compressive symptoms or arrhythmias. We present a case of a left ventricular leiomyosarcoma in a 50-year old female patient that had a delayed diagnosis and its subsequent surgical resection and oncological management with docetaxel and gemcitabine. This case highlights the need for a high index of suspicion for cardiac masses especially if there are competing chronic diseases with similar symptomatology. Given the rare presentation of left ventricular leiomyosarcomas, case reports may provide valuable information that is otherwise unavailable.

A case report of uterine leiomyosarcoma: unusual clinical presentation with unilateral hydronephrosis and importance of an appropriate diagnosis.

Uterine leiomyosarcoma is a rare malignant gynecologic tumor that arises from the myometrial or endometrial stromal precursor cells. This tumor has the highest prevalence in the pre- and post-is more frequent between 40 and 60 years old. It has a very unfavorable prognosis: only early-stage tumors have an acceptable prognosis; unfortunately, it is often diagnosed accidentally, typically on an advanced stage, when hematological metastases have already spread. Surgery is the main treatment strategy, while systemic treatment and radiotherapy are not recommended due to the lack of results. Since metastatization is mainly hematological, lymphadenectomy is not recommended. Recent progresses have been achieved in advanced and recurrent disease, often inoperable, thanks to new chemotherapies, target therapies and immunotherapies. We reported the case of a 51-year-old woman evaluated for lumbar pain in the right region compatible with renal colic. The ultrasound evaluation revealed right hydronephrosis and the presence of a paraovarian or intraligamentary mass compatible with fibroma. The abdominal CT confirmed the presence of a mass with heterogeneous vascularization. Therefore, the patient underwent laparoscopic surgery to remove the lesion which resulted to be a leiomyosarcoma G2. During the following week the patient underwent a laparoscopic hysterectomy. The first step for differential diagnosis consists in the evaluation of clinicopathological features, followed by the analysis of preoperative imaging. Pelvic MRI represents the gold standard, while CT is used to detect metastases. The main issue is that imaging shows limited ability in differential diagnosis between benign and malign smooth muscle tumor. The definitive diagnosis is confirmed by histological analysis; this implies the necessity of improved attentions on the surgical procedure, which is often performed by steps with prolongation of the treatment pathway. To distinguish which fibroids presents a major risk to be misdiagnosed, some risk scores were developed (rPRESS in 2014 and pLMS in 2019), though actually they are not applied in clinical practice. Uterine leiomyosarcoma (uLMS) is rare but causes several deaths in perimenopausal women due to lack of effective treatments, although target therapies represent a future hope. Furthermore, clinical practice needs support through the development and improvement of diagnostic risk scores and their integration into guidelines.

Tumor nasal de músculo liso de comportamiento maligno incierto: informe de un caso / Nasal smooth muscle tumor of uncertain malignant potential: A case report

Los tumores de músculo liso que no pueden ser clasificados según su histología como leiomiomas o leiomiosarcomas se denominan tumores de músculo liso de comportamiento maligno incierto. La localización nasal de estos tumores es muy infrecuente y la extensión adecuada de la cirugía para tratar estas neoplasias no está bien definida. Se describe el caso clínico de una adolescente de 16 años, que consultó por padecer un tumor de aspecto vascular en la cavidad nasal derecha y que fue tratada con éxito mediante cirugía intranasal. El diagnóstico histológico fue tumor de músculo liso de comportamiento maligno incierto. Por la rareza de estas neoplasias, su infrecuente localización nasal y la falta de evidencia que soporte cuál debe ser la extensión de la cirugía, es relevante la descripción y discusión del caso clínico.

Smooth muscle tumors that cannot be histologically classified as leiomyomas or leiomyosarcomas are defined as smooth muscle tumors of uncertain malignant potential. The location of these tumors in the nose is very rare, and the appropriate surgical extent to manage these neoplasms has not been adequately defined. Here we describe the case of a 16-year-old female adolescent who consulted due to a vascular-like tumor in the right nasal cavity who was successfully treated with intranasal surgery. The histological diagnosis was smooth muscle tumor of uncertain malignant potential. Given that these neoplasms are rare, the uncommon location in the nose, and the lack of evidence indicating the extent of surgery, it is relevant to describe and discuss this clinical case.